| 1. Khoshnood B, Lelong N, Houyel L, et al. Prevalence, timing of diagnosis and mortality of newborns with congenital heart defects: a population-based study. Heart 2012;98:1667-73. |
Review/Other-Dx |
317,538 (live births and stillbirths) |
To assess the prevalence, timing of diagnosis and infant mortality of congenital heart defects (CHD) with population-based data and using a classification that allows regrouping of the International Paediatric and Congenital Cardiac Code into a manageable number of categories based on anatomic and clinical criteria (ACC-CHD). |
The total number of CHD was 2867, including 2348 live births (82%), 466 TOPFA (16.2%) and 53 foetal deaths (1.8%). The total prevalence of CHD was 90 per 10,000. After exclusion of ventricular septal defects (VSD), 40% of ‘isolated’ CHD was diagnosed prenatally with about one half of the remaining diagnosed before 7 days of age. Nevertheless, one in five cases of these major CHD was diagnosed after the fourth week. Infant mortality of ‘isolated’ CHD-VSD excluded was 8.5% with 40% of deaths occurring after the fourth week of life. These outcomes varied substantially across categories of ACC-CHD. |
4 |
| 2. van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011;58:2241-7. |
Meta-analysis |
114 studies |
In this systematic review and meta-analysis, we provide a complete worldwide overview of the reported birth prevalence of total congenital heart disease (CHD) and the 8 most common subtypes of CHD from 1930 until 2010. |
Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since1930 and in continent and income groups since 1970 using the inverse variance method. Reported total CHD birth prevalence increased substantially over time, from 0.6 per 1,000 live births (95% confidence interval [CI]: 0.4 to 0.8)in 1930 to 1934 to 9.1 per 1,000 live births (95% CI: 9.0 to 9.2) after 1995. Over the last 15 years, stabilization occurred, corresponding to 1.35 million newborns with CHD every year. Significant geographical differences were found. Asia reported the highest CHD birth prevalence, with 9.3 per 1,000 live births (95% CI: 8.9 to 9.7), with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions. Reported total CHD birth prevalence in Europe was significantly higher than in North America (8.2 per 1,000 live births [95% CI: 8.1 to 8.3] vs.6.9 per 1,000 live births [95% CI: 6.7 to 7.1]; p<0.001). Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries. Observed differences may also be of genetic, environmental, socioeconomical, or ethnic origin, and there needs to be further investigation to tailor the management of this global health problem |
Good |
| 3. Mahle WT, Sutherland JL, Frias PA. Outcome of isolated bicuspid aortic valve in childhood. J Pediatr 2010;157:445-9. |
Review/Other-Dx |
981 children |
To evaluate the outcomes associated with isolated bicuspid aortic valve (BAV) during childhood and adolescence. |
The median age of the subjects at diagnosis was 8.3 years. At the time of the last pediatric follow-up, 7% of the subjects had moderate aortic regurgitation or greater, and the median Z score for the ascending aorta was +2.31. There were 9427 patient years of follow-up. Primary cardiac events occurred in 38 subjects, yielding an event rate of 0.004 per patient year. Eleven subjects (1.1%) underwent aortic valve surgery. Thirty subjects (3.0%) underwent balloon dilatation of the aortic valve. There was a single case of endocarditis. There were no cardiac-related deaths and no cases of aortic dissection. |
4 |
| 4. Gilboa SM, Devine OJ, Kucik JE, et al. Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation 2016;134:101-9. |
Review/Other-Dx |
N/A |
To estimate the congenital heart defects (CHD) prevalence across all age groups in the United States in the year 2010. |
No results stated in the abstract. |
4 |
| 5. American College of Radiology. ACR–NASCI–SIR–SPR Practice Parameter for the Performance and Interpretation of Body Computed Tomography Angiography (CTA). Available at: https://www.acr.org/-/media/ACR/Files/Practice-Parameters/body-cta.pdf. |
Review/Other-Dx |
N/A |
Guidance document to promote the safe and effective use of diagnostic and therapeutic radiology by describing specific training, skills and techniques. |
No abstract available. |
4 |
| 6. Kim SJ, Park SA, Song J, Shim WS, Choi EY, Lee SY. The role of transesophageal echocardiography during surgery for patients with tetralogy of Fallot. Pediatr Cardiol 2013;34:240-4. |
Observational-Dx |
340 patients |
To analyze the cost-benefit of routine transesophageal echocardiography (TEE) during the repair of tetralogy of Fallot (TOF). |
TEE was performed for 340 patients (85 %). Residual problems were detected in 17.9 % (61/340), and a return to bypass was needed for 10 % (34/340) of the patients. The degree of agreement between the intraoperative TEE and early postoperative transthoracic echocardiography (TTE) was relatively high. Surgeons with less surgical experience more frequently used intraoperative TEE (p = 0.007) and performed repeat bypass surgery at a higher rate (p = 0.00). Even relatively unskilled surgeons might be able to achieve surgical outcomes similar to those of experienced surgeons using intraoperative TEE. By avoiding late surgical revision, the possible cost savings were estimated to be 1,726,000 Korean won (US$1,489) per TEE examination. |
3 |
| 7. Mouws E, de Groot NMS, van de Woestijne PC, et al. Tetralogy of Fallot in the Current Era. Semin Thorac Cardiovasc Surg 2019;31:496-504. |
Observational-Dx |
177 patients |
To investigate a 15-year outcome of total Tetralogy of Fallot (ToF) correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated between 2000 and 2015. |
All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients. |
3 |
| 8. Hoashi T, Kagisaki K, Meng Y, et al. Long-term outcomes after definitive repair for tetralogy of Fallot with preservation of the pulmonary valve annulus. J Thorac Cardiovasc Surg 2014;148:802-8; discussion 08-9. |
Review/Other-Dx |
84 patients |
To evaluate the long-term outcomes after definitive repair of tetralogy of Fallot with preservation of the pulmonary valve (PV) annulus. |
The actuarial survival and freedom from reoperation rates at 20 years was 98.6% and 95.8%. The freedom from ventricular arrhythmia at 5, 10, 15, and 20 years was 98.7%, 89.6%, 74.1%, and 58.0%, respectively. All detected ventricular arrhythmias were isolated monofocal premature ventricular contractions. Freedom from moderate or greater pulmonary regurgitation at 5, 10, 15 and 20 years was 50.4%, 44.9%, 38.4%, and 35.7%, respectively. A bicuspid PV (hazard ratio, 2.910; 95% confidence interval, 1.404-6.204, P = .004) and a Z-value of less than -2 (hazard ratio, 1.948; 95% confidence interval, 0.915-5.857; P = .034) were the risk factors for developing moderate or greater pulmonary regurgitation. |
4 |
| 9. Ammash NM, Dearani JA, Burkhart HM, Connolly HM. Pulmonary regurgitation after tetralogy of Fallot repair: clinical features, sequelae, and timing of pulmonary valve replacement. Congenit Heart Dis 2007;2:386-403. |
Review/Other-Dx |
N/A |
To discuss pulmonary regurgitation following repair of tetralogy of Fallot and the clinical features, sequelae, and timing of pulmonary valve replacement. |
No results stated in the abstract. |
4 |
| 10. Geva T. Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006:11-22. |
Review/Other-Dx |
N/A |
To reviews the pathophysiology of chronic right ventricular volume load after tetralogy of Fallot repair and the risks and benefits of pulmonary valve replacement. |
No results stated in the abstract. |
4 |
| 11. Geva T, Gauvreau K, Powell AJ, et al. Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation 2010;122:S201-8. |
Observational-Dx |
64 patients |
To investigate whether the addition of surgical right ventricular (RV) remodeling with exclusion of scar tissue to pulmonary valve replacement (PVR) would result in improved RV function and laboratory and clinical parameters, as compared with PVR alone. |
Between February 2004 and October 2008, 64 patients who underwent RV outflow tract procedures in early childhood, had =moderate PR, and fulfilled defined criteria for PVR were randomly assigned to undergo either PVR alone (n = 34) or PVR with surgical RV remodeling (n = 30). No significant difference was observed in the primary outcome (change in RV ejection fraction: -2±7% in the PVR alone group and -1±7% in the PVR with RV remodeling group, P = 0.38) or in any of the secondary outcomes at 6-month postoperative follow-up. Multivariable analysis of the entire cohort identified preoperative RV end-systolic volume index <90 ml/m2 and QRS duration <140 ms to be associated with optimal postoperative outcome (normal RV size and function), and RV ejection fraction <45% and QRS duration =160 ms to be associated with suboptimal postoperative outcome (RV dilatation and dysfunction). |
1 |
| 12. Valente AM, Cook S, Festa P, et al. Multimodality imaging guidelines for patients with repaired tetralogy of fallot: a report from the AmericanSsociety of Echocardiography: developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. Journal of the American Society of Echocardiography. 27(2):111-41, 2014 Feb.J Am Soc Echocardiogr. 27(2):111-41, 2014 Feb. |
Review/Other-Dx |
N/A |
To describe the role of each diagnostic modality in the care of patients with repaired TOF and to provide guidelines for a multimodality approach that takesinto account patient-related and modality-related considerations. |
No results stated in abstract. |
4 |
| 13. Han BK, Rigsby CK, Hlavacek A, et al. Computed Tomography Imaging in Patients with Congenital Heart Disease Part I: Rationale and Utility. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT): Endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr 2015;9:475-92. |
Review/Other-Dx |
N/A |
This is an expert consensus document created to provide information about the current use of cardiovascular computed tomography (CT) in patients of all ages with proven or suspected congenital heart disease (CHD). The goals of this document apply to both pediatric and adult CHD patients and are to: 1) Review the current use of cardiovascular CT. 2)Assess the most up to date information on risks, benefits, as well as limitations of cardiovascular CT. 3) Provide disease-specific indications for cardiovascular CT imaging. 4) Outline a consensus opinion on the essential skills and knowledge needed to optimally perform and interpret cardiovascular CT. |
Successful cardiovascular CT imaging of CHD requires an in depth understanding of the core teaching elements of both cardiology and radiology. The ability to perform and interpret high quality congenital cardiovascular CT in a clinical context requires focused time and effort regardless of the previous background of the cardiac imager. This is reflected by a writing committee that consists of pediatric and adult radiologists and cardiologists, all whom have extensive experience in performing CT in this patient population. Cardiovascular CT is complementary to other imaging modalities and its optimal use will be in centers where all diagnostic modalities are available. The choice of modality for an individual patient should be determined by age, diagnosis, clinical condition, clinical question and patient preference.1–4 Use of CT in CHD should be reserved for situations in which it is expected to provide unique diagnostic information for the individual patient or clinical indication, and/or less risk than other modalities. This multi-disciplinary document is intended to guide the optimal selection of CHD patients for cardiovascular CT. |
4 |
| 14. Han BK, Rigsby CK, Leipsic J, et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT): Endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr 2015;9:493-513. |
Review/Other-Dx |
N/A |
To provide recommendations on patient preparation and technical scan acquisition for the most commonly referred CHD lesions, and to provide a brief description of radiation dose reduction techniques specific to CT in CHD. |
No results stated in abstract. |
4 |
| 15. Helbing WA, Bosch HG, Maliepaard C, et al. Comparison of echocardiographic methods with magnetic resonance imaging for assessment of right ventricular function in children. Am J Cardiol 1995;76:589-94. |
Observational-Dx |
33 children |
This study compares echocardiographic methods for the assessment of right ventricular (RV) volumes and function, and compares these methods to magnetic resonance imaging (MRI). |
Transthoracic 2-dimensional echocardiography from 3 different views and gradient-echo tomographic MRI were performed in 16 children with congenital heart disease and 17 age-matched healthy children. RV volumes and ejection fraction were calculated with 5 mono- and biplane area-length and multiple-slice echocardiographic methods. Adequate MRI and echocardiographic apical 4-chamber images could be obtained in all 33 children. The best correlation between MRI and echocardiographic volumes was with the biplane pyramidal approximation method. End-diastolic volume by MRI was 92 ± 27 ml: systematic difference with echocardiography was +14 ± 16 ml (r = 0.86). End-systolic volume by MRI was 33 ± 13 ml: systematic difference with echocardiography was -4 ± 7 ml (r = 0.82). Ejection fraction by MRI was 65 ± 8%: systematic difference with echocardiography was +5 ± 7% (r = 0.72), using monoplane ellipsoid approximation. For all echocardiographic methods, significant effects of RV geometry were noted. Echocardiographic mono- and biplane area-length and multiple-slice calculations demonstrated moderate correlation and significant systematic errors compared with MRI-derived RV volumes. Echocardiographic results were influenced by RV geometry. The relatively simple monoplane area-length method provides ejection fraction results acceptable for clinical practice; results are not improved by more complex biplane and/or multislice methods. |
3 |
| 16. Mohamed I, Stamm R, Keenan R, Lowe B, Coffey S. Assessment of Disease Progression in Patients With Repaired Tetralogy of Fallot Using Cardiac Magnetic Resonance Imaging: A Systematic Review. Heart, Lung & Circulation. 29(11):1613-1620, 2020 Nov. |
Review/Other-Dx |
14 studies |
To determine the most useful cardiac magnetic resonance imaging (CMR) predictors of disease progression and the optimal frequency of CMR. |
We systematically reviewed PubMed from inception until 29 April 2019 for longitudinal studies assessing the relationship between CMR features and disease progression in repaired ToF. Fourteen (14) studies were identified. Multiple studies showed that impaired right and left ventricular function predict subsequent disease progression. Right ventricular end diastolic volume, while being associated with disease progression when analysed alone, was generally not associated with disease progression on multivariate analysis. Severity of tricuspid regurgitation and pulmonary regurgitation likewise did not show a consistent association with subsequent events. A number of non-CMR factors were also identified as being associated with disease progression, in particular QRS duration and older age at repair. Restrictive right ventricular physiology was not consistently an independent predictor of events. |
4 |
| 17. Ghonim S, Ernst S, Keegan J, et al. Three-Dimensional Late Gadolinium Enhancement Cardiovascular Magnetic Resonance Predicts Inducibility of Ventricular Tachycardia in Adults With Repaired Tetralogy of Fallot. Circ Arrhythm Electrophysiol 2020;13:e008321. |
Observational-Dx |
69 patients (43 male) |
To prospectively examine whether three-dimensional late gadolinium enhancement (3D LGE) cardiovascular magnetic resonance (CMR) extent predicts inducible ventricular tachycardia (VT) at programmed electrical stimulation (PES). |
VT was induced in 22 (31%) patients. Univariable predictors of inducible VT included increased RV LGE (odds ratio [OR], 1.15; P=0.001 per cm3), increased nonapical vent LV LGE (OR, 1.09; P=0.008 per cm3), older age (OR, 1.6; P=0.01 per decile), QRS duration =180 ms (OR, 3.5; P=0.02), history of nonsustained VT (OR, 3.5; P=0.02), and previous clinical sustained VT (OR, 12.8; P=0.003); only prior sustained VT (OR, 8.02; P=0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR, 1.14; P=0.003). An RV LGE volume of 25 cm3 had 72% sensitivity and 81% specificity for predicting inducible VT (area under the curve, 0.81; P<0.001). At the extreme cutoffs for ruling-out and ruling-in inducible VT, RV LGE >10 cm3 was 100% sensitive and >36 cm3 was 100% specific for predicting inducible VT. |
3 |
| 18. Kim HK, Kim WH, Hwang SW, et al. Predictive value of intraoperative transesophageal echocardiography in complete atrioventricular septal defect. Ann Thorac Surg 2005;80:56-9. |
Observational-Dx |
35 patients |
To determine whether this discrepancy exists and to assess whether it is possible to predict follow-up results using intraoperative transesophageal echocardiography. |
In left-sided atrioventricular valve regurgitation, 34.3% (12 of 35) of patients showed discrepancy during follow-up, and 28.6% (10 of 35) showed progression of regurgitation (from grade I to II). In right-sided atrioventricular valve, 11.4% (4 of 35) of patients showed discrepancy, 9.6% (3 of 35) showed progression of regurgitation (from grade I to II). |
3 |
| 19. Gaydos SS, Varga-Szemes A, Judd RN, Suranyi P, Gregg D. Imaging in Adult Congenital Heart Disease. [Review]. J Thorac Imaging. 32(4):205-216, 2017 Jul. |
Review/Other-Dx |
N/A |
To highlight the most common conditions in adult Congenital Heart Disease (CHD) and the clinical questions that imaging hopes to answer, as well as the advantages and disadvantages of available imaging modalities. |
No results stated in the abstract. |
4 |
| 20. Patel S, Shah D, Chintala K, Karpawich PP. Atrial baffle problems following the Mustard operation in children and young adults with dextro-transposition of the great arteries: the need for improved clinical detection in the current era. Congenit Heart Dis 2011;6:466-74. |
Observational-Dx |
59 patients |
To reviews the effectiveness of these guidelines in detection of baffle issues pre-electrophysiology studies (EPS) catheterization and need for ancillary vascular interventions. |
Of 59 patients (34 pacemaker, 9 ablation, 16 routine hemodynamic) ages 8–39 years (mean 22.8), only three (5%) had symptoms of obstruction. However, baffle complications were found in 33 patients (56%), some with more than one problem: superior vena cava (SVC) obstruction in 32, inferior VC in two and leak in four. Baffle stenting was required in 24 patients and leak closure in two. Precatheterization TTE was available in 51 patients and showed 34% sensitivity, 61% specificity, 63% negative predictive value, and only 37% positive predictive value in recognizing baffle complications when compared with the actual catheterization findings. |
3 |
| 21. Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 29(7):571-621, 2016 07. |
Review/Other-Dx |
N/A |
To present evidence based practice guidelines for multimodality imaging in this cohort of patients with Transposition of the Great Arteries (TGA). |
No abstract available. |
4 |
| 22. Babu-Narayan SV, Goktekin O, Moon JC, et al. Late gadolinium enhancement cardiovascular magnetic resonance of the systemic right ventricle in adults with previous atrial redirection surgery for transposition of the great arteries. Circulation 2005;111:2091-8. |
Observational-Dx |
36 adults |
To determine if late gadolinium enhancement (LGE) would be present in the systemic right ventricle (RV) and that it would be associated with markers of adverse clinical outcome. |
We performed CMR on 36 consecutive adult patients (mean age, 27 years) after atrial redirection surgery for transposition of the great arteries. Late gadolinium RV enhancement was seen in 22 patients (61%) with various patterns. Patients with RV LGE were older (30 versus 22 years; P<0.001) and had increased RV end-systolic volume index (43 versus 35 mL/m2; P=0.03), decreased RV ejection fraction (57% versus 62%; P=0.02), increased QRS duration (108 versus 97 ms; P=0.01), and increased QT dispersion (93 versus 71 ms; P=0.002). The extent of LGE correlated with age (r=0.59, P<0.001) and QRS duration (r=0.67, P<0.001) and inversely with RV ejection fraction (r=-0.76, P<0.001). The incidence of documented arrhythmia and/or syncope (10 of 36) was significantly higher in the late gadolinium-positive group (9/22 versus 1/14; P=0.03) |
3 |
| 23. Morfaw F, Leenus A, Mbuagbaw L, Anderson LN, Dillenburg R, Thabane L. Outcomes after corrective surgery for congenital dextro-transposition of the arteries using the arterial switch technique: a scoping systematic review. Syst Rev 2020;9:231. |
Meta-analysis |
245 studies |
To summarize the evidence on short- (less than 1 year), medium- (1-20 years), and long-term (more than 20 years) outcomes of children with dextro-transposition of the great arteries (D-TGA) treated with the arterial switch operation (ASO). |
Following ASO for TGA, short-term survival was 92.0% (95% CI 91.0-93.0%; I2 = 85.8%, 151 studies, 30,186 participants; moderate certainty evidence). Medium-term survival was 90.0% (95% CI 89.0-91.0%; I2 = 84.3%, 133 studies; 23,686 participants, moderate certainty evidence), while long-term survival was 87.0% (95% CI 80.0-92.0 %; I2 = 84.5%, 4 studies, 933 participants, very low certainty evidence). Evaluation of the different secondary outcomes also showed satisfactory results in the short, medium and long term. Subgroup analysis suggests slightly higher survival following ASO for TGA in the second surgical era (1998 to 2018) than in the first surgical era (1975 to 1997) in the short and medium term [93.0% (95% CI 92.0-94.0) vs 90.0% (95% CI 89.0-92.0) and 93.0% (95% CI 91.0-94.0) vs 88.0% (87.0-90.0%) respectively] but not in the long term [81.0% (95% CI 76.0-86.0%) vs 89.0% (80.0-95.0%)]. |
Good |
| 24. Szymczyk K, Moll M, Sobczak-Budlewska K, et al. Usefulness of Routine Coronary CT Angiography in Patients with Transposition of the Great Arteries After an Arterial Switch Operation. Pediatr Cardiol. 39(2):335-346, 2018 Feb. |
Observational-Dx |
750 patients |
To assess the results of an initial series of coronary computed tomography angiography (CCTA) examinations that were routinely performed in asymptomatic patients with Transposition of the Great Arteries (TGA) after an Arterial Switch Operation (ASO). |
An initial series of 50 CCTAs performed in asymptomatic patients with TGA after an ASO were evaluated. In each case, a detailed examination of the coronary anatomy, its relationship to the surrounding structures, its exact position in the neoaortic sinus, and the presence of significant coronary abnormalities was performed. The CT scans revealed significant coronary abnormalities in 12 asymptomatic patients: three had acute proximal angulation and stenosis, four had an intra-arterial course, seven had a muscular bridge, one had a left anterior descending artery with an intramuscular course, and one had coronary fistulas to the pulmonary arteries. Additionally, in 25 patients, proximal acute angulation of at least one coronary artery was detected, and four of them had a high ellipticity index. Most of the potentially severe anatomical features were related to the left coronary artery or the left anterior descending artery. CCTA routinely performed on asymptomatic patients with TGA after an ASO provides accurate and useful information for postoperative management. The frequency of coronary anomalies and potentially dangerous anatomical features in this group of patients is high, and their impact on postoperative follow-up remains unknown. |
3 |
| 25. Ou P, Celermajer DS, Marini D, et al. Safety and accuracy of 64-slice computed tomography coronary angiography in children after the arterial switch operation for transposition of the great arteries. JACC Cardiovasc Imaging 2008;1:331-9. |
Observational-Dx |
130 children |
To investigate the accuracy of 64-slice computed tomography (CT) angiography, as compared to invasive angiography, to evaluate reimplanted coronary arteries in children after arterial switch operation (ASO) for transposition of the great arteries (TGA). |
The CT was fully evaluable in 126 of 130 patients (97%), allowing assessment of ostia and proximal segments of all coronary arteries. The CT correctly detected all 12 patients (9.2%) in whom invasive coronary angiography had identified significant coronary lesions, with a sensitivity, specificity, and negative predictive value of 100%. In addition, CT showed nonsignificant coronary lesions (<30% luminal narrowing) in 6 patients and allowed determination of the underlying reasons for coronary luminal narrowing, such as stretching or compression of the re-implanted coronary arteries caused by their anatomic relationship to the adjacent great vessels. |
2 |
| 26. Taylor AM, Dymarkowski S, Hamaekers P, et al. MR coronary angiography and late-enhancement myocardial MR in children who underwent arterial switch surgery for transposition of great arteries. Radiology 2005;234:542-7. |
Observational-Dx |
32 children |
To prospectively evaluate the feasibility of magnetic resonance (MR) coronary artery imaging and to define myocardial damage with late-enhancement myocardial MR imaging in children who underwent arterial switch surgery for transposition of the great arteries. |
In 23 (72%) of 32 coronary arteries imaged, diagnostic-quality images of the coronary ostium and proximal coronary artery course were acquired; this increased to 100% in subjects older than 11 years. No coronary ostial stenoses were seen. In all subjects, the proximal course of the coronary arteries was visualized. Two subendocardial viability defects were detected, which corresponded to known compromise of the artery that supplied that territory at the time of surgery. Global left and right ventricular function were preserved, with no regional wall abnormalities. |
2 |
| 27. Angeli E, Formigari R, Pace Napoleone C, et al. Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg 2010;38:714-20. |
Observational-Tx |
119 patients |
To analyze the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). |
The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p=0.04) and logistic regression (p=0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p=0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p=0.1). |
2 |
| 28. Noel CV, Krishnamurthy R, Masand P, et al. Myocardial Stress Perfusion MRI: Experience in Pediatric and Young-Adult Patients Following Arterial Switch Operation Utilizing Regadenoson. Pediatr Cardiol. 39(6):1249-1257, 2018 Aug. |
Observational-Dx |
36 patients (24 male, 12 female) |
To report on our initial experience with the use of regadenoson as a pharmacologic stress agent in a pediatric and young-adult population of arterial switchoperation (ASO) patients with signs or symptoms concerning for possible ischemia, and to compare the results to nuclear stress testing and cardiac catheterization, when available. |
We reviewed our initial experience with regadenoson stress cardiac MR in 36 pediatric and young-adult patients 15.1 ± 4.5 years (range 0.2–22 years) with history of ASO. The weight was 61.6 ± 21.5 kg (range 3.8–93 kg). All patients underwent cardiac MR because of concern for ischemia. Subjects’ heart rate and blood pressure were monitored and pharmacologic stress was induced by injection of regadenoson. We evaluated their hemodynamic response and adverse effects using changes in vital signs and onset of symptoms. A pediatric cardiologist and radiologist qualitatively assessed myocardial perfusion and viability images. All stress cardiac MR examinations were completed without adverse events. Resting heart rate was 72 ± 13 beats per minute (bpm) and rose to peak of 120 ± 17 bpm(95 ± 50% increase, p < 0.005) with regadenoson. Image quality was considered good or diagnostic in all cases. A total of 11/36 (31%) patients had a perfusion defect on the stress FPP images. 14 of the 36 patients (39%) underwent cardiac catheterization within 6 months of the CMR and the findings showed excellent agreement. |
3 |
| 29. Manso B, Castellote A, Dos L, Casaldaliga J. Myocardial perfusion magnetic resonance imaging for detecting coronary function anomalies in asymptomatic paediatric patients with a previous arterial switch operation for the transposition of great arteries. Cardiol Young 2010;20:410-7. |
Review/Other-Dx |
28 patients (13-16 years) |
To report a single centre experience with myocardial perfusion magnetic resonance imaging for detecting ischaemia after the arterial switch operation for transposition of great arteries. |
All patients were symptom free with no ischaemic signs on the electrocardiogram. All magnetic resonance imaging examinations were generally well tolerated with minor adenosine secondary effects in 36% of the patients. Two stress myocardial perfusion magnetic resonance studies were excluded from analysis for technical reasons. No perfusion stress defects were detected at the remaining 26. Myocardial delayed enhancement was performed in all 28 patients. In five subjects, a subendocardial late enhancement consistent with patch tissue for septal defect closure at the time of repair was indentified. |
4 |
| 30. Bernsen MLE, Koppes JCC, Straver B, Verberne HJ. Left ventricular ischemia after arterial switch procedure: Role of myocardial perfusion scintigraphy and cardiac CT. J Nucl Cardiol 2020;27:651-58. |
Review/Other-Dx |
2 boys (13 year old and 15 year old) |
To discuss two cases illustrating the added value of myocardial perfusion imaging (MPI) and cardiac computed tomography (CT) for the assessment of these patients. |
No results stated in the abstract. |
4 |
| 31. Cheezum MK, Liberthson RR, Shah NR, et al. Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva. [Review]. J Am Coll Cardiol. 69(12):1592-1608, 2017 Mar 28. |
Review/Other-Dx |
77 studies |
With increasing recognition of anomalous aortic origin of a coronary artery (AAOCA), we aimed to review the following: 1) the observed prevalence of AAOCA arising at or above the inappropriate sinus of Valsalva with attention to the interarterial course subtype; 2) the use of cardiac testing to examine AAOCA; 3) outcomes of interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA) patients; and 4) recommendations and knowledge gaps in current management. |
No results stated in the abstract. |
4 |
| 32. Lim JC, Beale A, Ramcharitar S, Medscape. Anomalous origination of a coronary artery from the opposite sinus. Nat Rev Cardiol 2011;8:706-19. |
Review/Other-Dx |
N/A |
To discuss the anomalous origination of a coronary artery from the opposite sinus. |
N/A |
4 |
| 33. van Stijn D, Planken N, Kuipers I, Kuijpers T. CT Angiography or Cardiac MRI for Detection of Coronary Artery Aneurysms in Kawasaki Disease. Front Pediatr 2021;9:630462. |
Observational-Dx |
965 patients |
The objective of this single center, retrospective study is to explore the diagnostic potential of coronary artery assessment of coronary computed tomographic angiography (cCTA) vs. cardiac MRI (CMR) in children with KD. |
Out of 965 KD patients from our database, a total of 111 cCTAs (104 patients) and 311 CMR (225 patients) have been performed since 2010. For comparison, we identified 54 KD patients who had undergone both cCTA and CMR. CMR only identified eight patients with CAAs compared to 14 patients by cCTA. CMR missed 50% of the CAAs identified by cCTA. |
2 |
| 34. McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. [Review]. Circulation. 135(17):e927-e999, 2017 Apr 25. |
Review/Other-Dx |
N/A |
To provide updated discussion and recommendations using best evidence-based guidance to healthcare providers for the diagnosis, acute treatment, and long-term management of Kawasaki Disease (KD). |
To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. |
4 |
| 35. Krishnamurthy R, Masand PM, Jadhav SP, et al. Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery. Pediatr Radiol 2021;51:1299-310. |
Observational-Dx |
25 patients |
To facilitate clinical risk stratification, the diagnostic accuracy of computed tomography (CT) angiography for individual risk factors in the setting of anomalous aortic origin of coronary artery (AAOCA) must be established. |
CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. |
2 |
| 36. Doan TT, Wilkinson JC, Loar RW, Pednekar AS, Masand PM, Noel CV. Regadenoson Stress Perfusion Cardiac Magnetic Resonance Imaging in Children With Kawasaki Disease and Coronary Artery Disease. Am J Cardiol 2019;124:1125-32. |
Observational-Dx |
32 patients |
To assess the safety, feasibility, and diagnostic utility of vasodilator stress perfusion cardiac magnetic resonance (CMR) using regadenoson in children with Kawasaki disease (KD) and Coronary artery (CA) abnormalities. |
The safety, feasibility, and diagnostic utility of regadenoson stress CMR was assessed in children with KD and CA abnormalities. A retrospective review of regadenoson stress CMR in children with convalescent KD was performed. Hemodynamics changes after regadenoson administration and adverse effects were recorded. First-pass perfusion was evaluated at rest and during pharmacologic stress. The results were compared with anatomic CA imaging. Forty-one stress CMR (18 sedated examinations, 44%) were performed successfully in 32 patients. Median age was 11.2 years (range 2.2 to 18.6) and weight 41 kg (range 13 to 93.4). Heart rate increased 66 ± 25% (p <0.005) after regadenoson. Minor adverse events occurred in 6 sedated and 1 unsedated patients. Hypoperfusion during stress occurred in 16 of 41 (39%), including 5 inducible, 9 inducible and fixed, and 2 fixed lesions. Late gadolinium enhancement was present in 10 of 16 with hypoperfusion and in 1 without hypoperfusion. Stress CMR had 100% positive agreement and >90% negative and overall agreement with moderate-to-severe CA stenoses. Four patients with hypoperfusion underwent revascularization for severe CA stenoses. |
3 |
| 37. Scannell CM, Hasaneen H, Greil G, et al. Automated Quantitative Stress Perfusion Cardiac Magnetic Resonance in Pediatric Patients. Front Pediatr 2021;9:699497. |
Observational-Dx |
14 patients |
To assess the feasibility of fully automated, high resolution, quantitative stress myocardial perfusion cardiac magnetic resonance (CMR) in a cohort of pediatric patients and to evaluate its agreement with the coronary anatomical status of the patients. |
Automated perfusion quantification was successful in 15/16 cases. The coronary perfusion territories supplied by vessels affected by a medium/large aneurysm or stenosis (according to the AHA guidelines), induced by Kawasaki disease, an anomalous origin, or interarterial course had significantly reduced myocardial blood flow (MBF) (median (interquartile range), 1.26 (1.05, 1.67) ml/min/g) as compared to territories supplied by unaffected coronaries [2.57 (2.02, 2.69) ml/min/g, p < 0.001] and territories supplied by vessels with a small aneurysm [2.52 (2.45, 2.83) ml/min/g, p = 0.002]. |
3 |
| 38. Tacke CE, Kuipers IM, Groenink M, Spijkerboer AM, Kuijpers TW. Cardiac magnetic resonance imaging for noninvasive assessment of cardiovascular disease during the follow-up of patients with Kawasaki disease. Circ Cardiovasc Imaging 2011;4:712-20. |
Observational-Dx |
63 patients |
To apply within a single procedure a comprehensive cardiac magnetic resonance imaging (CMRI) protocol including adenosine stress testing to assess coronary artery pathology, reversible ischemia, and myocardial infarction during the follow-up of patients with Kawasaki disease (KD) and secondly, to assess to what extent CMRI identifies coronary artery lesions missed by echocardiography. |
Patients with KD aged =8 years were consecutively included. Sixty-three patients (median age, 14.6 years; 74.6% male sex) underwent a comprehensive CMRI protocol including adenosine stress testing to evaluate coronary artery anatomy, ischemia, and myocardial infarction. All patients underwent CMRI without significant complications. On CMRI, 23 coronary artery aneurysms (CAAs) were identified in 15 patients. CMRI detected thrombus formation in 6 CAAs in 4 patients, wall motion disturbances and ischemia in 4 patients, and delayed hyperenhancement indicating myocardial infarction in 5 patients. Wall motion and perfusion abnormalities were noted in territories supplied by affected coronary arteries. CMRI results were compared with recent echocardiography findings. In 6 of the 15 patients with CAAs on CMRI, CAAs were not detected by echocardiography. |
2 |
| 39. Molossi S, Agrawal H, Mery CM, et al. Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach. Circ., Cardiovasc. interv.. 13(2):e008445, 2020 02. |
Observational-Dx |
163 patients |
To evaluate outcomes in a prospective study of anomalous aortic origin of coronary artery (CA) patients following a standardized algorithm. |
Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7-2.8) years. |
2 |
| 40. Grani C, Buechel RR, Kaufmann PA, Kwong RY. Multimodality Imaging in Individuals With Anomalous Coronary Arteries. [Review]. JACC Cardiovasc Imaging. 10(4):471-481, 2017 04. |
Review/Other-Dx |
N/A |
To highlight current challenges and future perspectives with a special focus on the role of noninvasive multimodality imaging in patients with Anomalous Coronary Arteries (ACA). |
No results stated in the abstract. |
4 |
| 41. Goldstein BH, Holzer RJ, Trucco SM, et al. Practice Variation in Single-Ventricle Patients Undergoing Elective Cardiac Catheterization: A Report from the Congenital Cardiac Catheterization Project on Outcomes (C3PO). Congenit Heart Dis 2016;11:122-35. |
Review/Other-Tx |
1,459 patients |
The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease. |
Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts. |
4 |
| 42. Margossian R, Schwartz ML, Prakash A, et al. Comparison of echocardiographic and cardiac magnetic resonance imaging measurements of functional single ventricular volumes, mass, and ejection fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study). Am J Cardiol 2009;104:419-28. |
Observational-Dx |
546 total patients: 100 echocardiograms, 50 CMR studies, 124 subjects with paired studies |
To compare echocardiographic and cardiac MRI measurements of functional single ventricular volumes, mass, and ejection fraction. |
Volumes by echocardiography averaged 70% of CMR values. Interobserver reproducibility for the ejection fraction was similar for the 2 modalities. Although the absolute mean difference between modalities for the ejection fraction was small (<2%), 95% limits of agreement were wide. In conclusion, agreement between observers of qualitative functional single ventricular function by echocardiography is modest. Measurements of functional single ventricular volume by 2D echocardiography underestimate CMR measurements, but their reproducibility is high. Echocardiographic and CMR measurements of functional single ventricular ejection fraction demonstrate similar interobserver reproducibility, whereas measurements of functional single ventricular mass and left ventricular diastolic volume are more reproducible by CMR. |
2 |
| 43. Han BK, Vezmar M, Lesser JR, et al. Selective use of cardiac computed tomography angiography: an alternative diagnostic modality before second-stage single ventricle palliation. J Thorac Cardiovasc Surg 2014;148:1548-54. |
Review/Other-Dx |
16 patients |
To assess the accuracy and risk of substituting cardiac computed tomography for cardiac catheterization in select patients for evaluation of anatomy before second-stage single ventricle palliation. |
General anesthesia was used for 16 of 16 cardiac catheterization studies and 1 of 16 computed tomography studies. Vascular access was central venous and/or arterial for all cardiac catheterization studies and a peripheral intravenous line for all computed tomography studies. Median age- and size-adjusted radiation dose was 14.0 mSv for cardiac catheterization and 1.1 mSv for computed tomography. Contrast dose was 4.8 mL/kg for the cardiac catheterization group and 2 mL/kg for the computed tomography group. There were no computed tomography discrepancies and 1 discrepancy between cardiac catheterization and surgical findings. There were 8 adverse events in 6 patients in the cardiac catheterization group and 1 adverse event in the computed tomography group. There was no difference between groups in postoperative course or need for repeat intervention. |
4 |
| 44. Vargas D, Zhou H, Yu X, et al. Cangrelor PK/PD analysis in post-operative neonatal cardiac patients at risk for thrombosis. J Thromb Haemost 2021;19:202-11. |
Observational-Tx |
22 patients |
To evaluate the pharmacokinetics (PK), pharmacodynamics (PD), and safety of cangrelor in neonates undergoing stage 1 palliation. |
Twenty-two patients were consented and 15 received a 1-hour infusion of cangrelor at either 0.5 µg/kg/min (cohort 1) or 0.25 µg/kg/min (cohort 2). Whereas the primary PD endpoint was achieved at the higher dose (ie, reduction in maximal platelet aggregation by =90% in 60% of participants), only 29% of those in cohort 2 attained this goal. Comparable and statistically significant results were obtained in MF assays (P < .0001 vs. baseline). Drug levels during infusion were 3-fold higher in cohort 1 vs. cohort 2 (P < .001). Most participants (70%) had undetectable drug levels by 10 minutes postinfusion with full recovery in platelet function at 1 hour. No drug-related bleeding events occurred. |
2 |
| 45. Prakash A, Khan MA, Hardy R, Torres AJ, Chen JM, Gersony WM. A new diagnostic algorithm for assessment of patients with single ventricle before a Fontan operation. J Thorac Cardiovasc Surg 2009;138:917-23. |
Observational-Dx |
151 children |
To propose and evaluate a diagnostic algorithm that does not use routine cardiac catheterization for low-risk subjects before the Fontan operation. |
According to the algorithm, 95 (63%) of 151 subjects had no risk factors (‘‘low risk’’) whereas 56(37%) of 151 had 1 risk factor or more (‘‘high risk’’). Nine (6%) of 151 subjects were found to be inoperable after catheterization and all 9 were correctly classified as high risk by the algorithm. In the 135 of 151 subjects who underwent a Fontan operation, the algorithm predicted an adverse postoperative outcome with a sensitivity of51%and specificity of 78%. However, this prediction was not improved by including elevated pulmonary artery pressure or ventricular filling pressure as additional risk factors. |
2 |
| 46. Ait-Ali L, De Marchi D, Lombardi M, et al. The role of cardiovascular magnetic resonance in candidates for Fontan operation: proposal of a new algorithm. J Cardiovasc Magn Reson 2011;13:69. |
Observational-Dx |
44 patients |
To propose a new diagnostic algorithm for candidates for Fontan and identify those who can skip cardiac catheterization (CC). |
In Group I ("CC not required") no unexpected new information affecting surgical planning was provided by CC. Conversely, in Group II new information was provided by CC in three patients (0 vs 11.5%, p = 0.35) and in six an interventional procedure was performed. During CC, minor complications occurred in one patient from Group I and in three from Group II (6 vs 14%, p = 0.7). Radiation Dose-Area product was similar in the two groups (Median 20 Gycm(2), range: 5-40 vs 26.5 Gycm(2), range: 9-270 p = 0.37). All 18 Group I patients and 19 Group II patients underwent a total cavo-pulmonary anastomosis; in the remaining seven group II patients, four were excluded from Fontan; two are awaiting Fontan; one refused the intervention |
2 |
| 47. Han BK, Huntley M, Overman D, et al. Cardiovascular CT for evaluation of single-ventricle heart disease: risks and accuracy compared with interventional findings. Cardiol Young 2018;28:9-20. |
Observational-Dx |
122 patients |
To evaluate the risk and image quality from cardiovascular computed tomography (CT) in patients across all stages of single-ventricle palliation, and to define accuracy by comparing findings with intervention and surgery. |
From January, 2010 to August, 2015, 132 CT scans were performed in single-ventricle patients of whom 20 were neonates, 52 were post-Norwood, 15 were post-Glenn, and 45 were post-Fontan. No sedation was used in 76 patients, 47 were under minimal or moderate sedation, and nine were under general anaesthesia. The median image quality score was 1.2. The procedural dose-length product was 24 mGy-cm, and unadjusted and adjusted radiation doses were 0.34 (0.2, 1.8) and 0.82 (0.55, 1.88) mSv, respectively. There was one adverse event. No major and two minor discrepancies were noted at the time of 79 surgical and 10 catheter-based interventions. |
3 |
| 48. Ghadimi Mahani M, Agarwal PP, Rigsby CK, et al. CT for Assessment of Thrombosis and Pulmonary Embolism in Multiple Stages of Single-Ventricle Palliation: Challenges and Suggested Protocols. [Review]. Radiographics. 36(5):1273-84, 2016 Sep-Oct.Radiographics. 36(5):1273-84, 2016 Sep-Oct. |
Review/Other-Dx |
N/A |
To provide an overview of the multiple stages of single-ventricle repair; illustrate the technique of pulmonary Computed tomographic (CT) angiography at each stage, including suggested protocols for different types of CT scanners; and discuss common interpretive pitfalls in diagnosing thrombosis and pulmonary embolism in the setting of single-ventricle palliation. |
No results stated in the abstract. |
4 |
| 49. Brown DW, Powell AJ, Geva T. Imaging complex congenital heart disease — functional single ventricle, the Glenn circulation and the Fontan circulation: A multimodality approach. Progress in Pediatric Cardiology 2010;28:45-58. |
Review/Other-Dx |
N/A |
To review the surgical management of the patient with functional single ventricle, and then explores the role of various imaging modalities in this setting with an emphasis on these newer techniques. |
No results stated in the abstract. |
4 |
| 50. Fogel MA, Khiabani RH, Yoganathan A. Imaging for preintervention planning: pre- and post-Fontan procedures. Circ Cardiovasc Imaging 2013;6:1092-101. |
Review/Other-Dx |
N/A |
To discuss imaging for pre-intervention planning for pre and post-Fontan procedures. |
No abstract available |
4 |
| 51. Downing TE, Allen KY, Glatz AC, et al. Long-term survival after the Fontan operation: Twenty years of experience at a single center. J Thorac Cardiovasc Surg 2017;154:243-53 e2. |
Observational-Dx |
773 patients |
To describe long-term post-Fontan survival in a modern patient cohort. |
Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (=1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P = .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). |
3 |
| 52. Yeong M, Loughborough W, Hamilton M, Manghat N. Role of cardiac MRI and CT in Fontan circulation. Journal of Congenital Cardiology 2017;1:8. |
Review/Other-Dx |
N/A |
The purpose of this review is to understand the role of cardiac MRI and CT in Fontan circulation and information that can be obtained with each cross-sectional modality as well as highlight the challenges that each modality faces. |
No results stated in the abstract. |
4 |
| 53. Fratz S, Hess J, Schwaiger M, Martinoff S, Stern HC. More accurate quantification of pulmonary blood flow by magnetic resonance imaging than by lung perfusion scintigraphy in patients with fontan circulation. Circulation 2002;106:1510-3. |
Observational-Dx |
15 patients and 12 controls |
To determine whether PV-MRI is more accurate than scintigraphy for quantitative evaluation of pulmonary perfusion ratios in patients with APA, TCPC, or PCPC. |
Bland-Altman analysis showed a clinically unacceptable difference of 7.1% right pulmonary blood flow (27.2% upper and -13.0% lower limit of agreement) between the two methods in the study group. The two methods agreed excellently in the control group (difference, 1.6%; 4.0% upper and -7.2% lower limit of agreement), showing that the bad agreement in the study group was caused by the problems encountered using pulmonary scintigraphy in patients with APA, TCPC, or PCPC. |
3 |
| 54. Ginde S, Goot BH, Frommelt PC. Imaging adult patients with Fontan circulation. Curr Opin Cardiol 2017;32:521-28. |
Review/Other-Dx |
N/A |
To discuss the role of noninvasive imaging for surveillance and early detection of anatomic and functional abnormalities of the Fontan circulation that can impact the risk for Fontan failure over time. |
Echocardiography is the first-line imaging modality for the adult Fontan patient. Use of established techniques, such as tissue Doppler imaging, and newer techniques, such as myocardial deformation and three-dimensional imaging, has improved the ability of echocardiography to serially assess ventricular and valvular function in this population. Strain imaging, in particular, is effective for early detection of subclinical ventricular dysfunction, is reproducible and can be incorporated into a routine clinical echocardiography protocol. Cardiac magnetic resonance (CMR) imaging complements echocardiography and overcomes the limitation of poor acoustic windows in adult patients, especially with regards to visualizing the cavopulmonary anastomoses and pulmonary arteries. High resolution imaging with CMR provides reliable assessment of ventricular size and function. Novel techniques utilizing CMR, such as computational fluid dynamics, have provided important insights into Fontan fluid dynamics, and the impact of Fontan geometry on flow efficiency through the circulation. |
4 |
| 55. Hong SH, Kim YM, Lee C-H, Park S-J, Kim SH. CT and MRI Evaluation of the Fontan Pathway: Pearls and Pitfalls. Cardiovasc Imaging Asia 2017;1:133-45. |
Review/Other-Dx |
N/A |
To review the normal anatomy of common variations of the Fontan pathway, various multidetector computed tomography (MDCT) and cardiac magnetic resonance imaging (CMR) techniques for optimal imaging diagnosis of the Fontan pathway, and various spectra of imaging findings regarding potential complications in patients with failing Fontan. |
No results stated in the abstract. |
4 |
| 56. Raimondi F, Martins D, Coenen R, et al. Prevalence of Venovenous Shunting and High-Output State Quantified with 4D Flow MRI in Patients with Fontan Circulation. Radiol Cardiothorac Imaging 2021;3:e210161. |
Observational-Dx |
75 patients (46 female, 29 male) |
To assess the ability of four-dimensional (4D) flow magnetic resonance imaging (MRI) to quantify flow volume of the Fontan circuit, including the frequency and hemodynamic contribution of systemic-to-pulmonary venovenous collateral vessels. |
Seventy-five patients (mean age, 20 years; range, 5-58 years; 46 female and 29 male patients) were included. Interobserver agreement was high for aortic output, pulmonary arteries, pulmonary veins, superior vena cava (Glenn shunt), and inferior vena cava (Fontan conduit) (range, ? = 0.913-0.975). Calculated shunt volume also showed strong agreement, on the basis of the difference between aortic and pulmonary flow (? = 0.935). A total of 37 of 75 (49%) of the patients exhibited shunts exceeding 1.00 L/min, 81% (30 of 37) of whom had pulmonary venous or atrial flow volume step-ups and corresponding venovenous collaterals. A total of 12% of patients (nine of 75) exhibited a high-output state (>4 L/min/m2), most of whom had venovenous shunts exceeding 30% of cardiac output. |
2 |
| 57. Averin K, Hirsch R, Seckeler MD, Whiteside W, Beekman RH, 3rd, Goldstein BH. Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique. Heart 2016;102:1109-14. |
Observational-Tx |
46 patients |
To identify Fontan patients with occult diastolic dysfunction (DD) using 'ventricular stress testing' with rapid volume expansion (RVE). |
Forty-six Fontan patients (48% female, median age 14.1 (IQR 9.1 to 21.3) years) were included. The median Fontan duration was 10.8 (IQR 5.1 to 17.8) years and dominant left ventricular morphology was present in 63% of patients. Volume expansion increased mean Fontan pressure (15.2±2.5 vs 12.4±2.2 mm Hg, p<0.001), pulmonary capillary wedge pressure (11.3±2.6 vs 7.9±2 mm Hg, p<0.001) and EDP (12.7±3.3 vs 8.5±2.1 mm Hg, p<0.001). Sixteen patients (35%) had occult DD, demonstrating higher baseline EDP (10.3±1.9 vs 7.6±1.5 mm Hg, p<0.001) and greater increase in EDP (6.3±2.4 vs 3.1±1.4 mm Hg, p<0.001) compared with patients without DD. Higher baseline EDP, lower baseline cardiac index and longer duration of Fontan circulation were associated with higher post-stress EDP. There were no complications related to RVE. |
2 |
| 58. Schmitt B, Steendijk P, Ovroutski S, et al. Pulmonary vascular resistance, collateral flow, and ventricular function in patients with a Fontan circulation at rest and during dobutamine stress. Circ Cardiovasc Imaging 2010;3:623-31. |
Observational-Dx |
10 patients |
To determine if the exposure to dobutamine stress would have a specific impact on pulmonary vascular resistance (PVR) and aortopulmonary collateral, systolic and diastolic ventricular function and, hence, improve our understanding of cardiovascular pathophysiology in patients with a Fontan circulation. |
Using an MRI catheterization technique, we performed a differential analysis of pulmonary vascular resistance and aortopulmonary collateral blood flow in conjunction with global ventricular pump function, myocontractility (end-systolic pressure-volume relation), and diastolic compliance (end-diastolic pressure-volume relation) in 10 patients with a Fontan circulation at rest and during dobutamine stress. Pulmonary and ventricular pressures were measured invasively and synchronized with velocity-encoded MRI-derived pulmonary and aortic blood flows and cine MRI-derived ventricular volumes. Pulmonary vascular resistance and end-systolic and end-diastolic pressure-volume relations were then determined. Aortopulmonary collateral flow was calculated as the difference between aortic and pulmonary flow. Compared to rest, dobutamine caused a small increase in mean pulmonary pressures (P<0.05). Collateral flow was significantly augmented (P<0.001) and contributed importantly to an increase in pulmonary flow (P<0.01). Pulmonary vascular resistance decreased significantly (P<0.01). Dobutamine did not increase stroke volumes significantly despite slightly enhanced contractility (end-systolic pressure-volume relation). Active early relaxation (t) was inconspicuous, but the end-diastolic pressure-volume relation shifted upward, indicating reduced compliance. |
3 |
| 59. Shi X, Lu Y, Sun K. Research Progress in Pathogenesis of Total Anomalous Pulmonary Venous Connection. Methods Mol Biol 2020;2204:173-78. |
Review/Other-Dx |
N/A |
To systematically review the epidemiology, anatomy, and pathophysiology of Total Anomalous Pulmonary Venous Connection (TAPVC) and give an overview of the research progress of TAPVC pathogenesis. |
No results stated in the abstract. |
4 |
| 60. Files MD, Morray B. Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction. Semin Cardiothorac Vasc Anesth 2017;21:123-31. |
Review/Other-Dx |
N/A |
To explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications. |
No results in abstract. |
4 |
| 61. Dyme JL, Prakash A, Printz BF, Kaur A, Parness IA, Nielsen JC. Physiology of isolated anomalous pulmonary venous connection of a single pulmonary vein as determined by cardiac magnetic resonance imaging. Am J Cardiol 2006;98:107-10. |
Observational-Dx |
6 patients |
To determine physiological effects of isolated anomalous pulmonary venous return using MR. |
Patients with isolated anomalous pulmonary venous return were found to have modest left-to-right shunt (Qp/Qs ratio, 1.3 to 1.6) with mild dilation of the right ventricle. |
3 |
| 62. Lock JE, Bass JL, Castaneda-Zuniga W, Fuhrman BP, Rashkind WJ, Lucas RV, Jr. Dilation angioplasty of congenital or operative narrowings of venous channels. Circulation 1984;70:457-64. |
Review/Other-Tx |
10 infants and children |
To report the success of angioplasty in relieving vena caval obstructions and its failure in relieving pulmonary venous obstructions, and to analyze the potential reasons for those successes and failures. |
In five children the obstructions were "vena caval" and followed repair for transposition of the great vessels (four patients) or orthotopic liver transplantation (one patient). In the four patients with fixed vena caval or baffle obstructions, balloon angioplasty was successful in relieving the obstruction, decreasing the average gradient (16.0 to 4.5 mm Hg), and increasing the average diameter (3.0 to 8.9 mm) of the obstructed site. One child died 1 week later from an unrelated cerebral hemorrhage; the three survivors have had persistent clinical and angiographic improvement. The fifth child had severe systolic narrowing of the superior baffle limb caused by marked tricuspid regurgitation, which ballooned the superior limb of the baffle against the atrial roof. Angioplasty was unsuccessful in relieving this type of obstruction, which resolved with tricuspid valve replacement. Of the five infants with obstructed pulmonary veins, three had congenitally narrowed vessels associated with total anomalous pulmonary venous connection, one had acquired stenosis, and one had postoperative obstruction after repair of a mixed type of total anomalous pulmonary venous connection. Dilation was unsuccessful in all five patients, but for different reasons: in the congenitally narrowed veins, the waist in the balloon could not be eliminated, even with high dilating pressures; in the infant with acquired stenosis, the vein stretched but did not tear at low dilating pressures; and in the postoperative obstruction, angioplasty increased the diameter of the obstruction but did not increase flow to the affected lung. |
4 |
| 63. Meadows J, Marshall AC, Lock JE, Scheurer M, Laussen PC, Bacha EA. A hybrid approach to stabilization and repair of obstructed total anomalous pulmonary venous connection in a critically ill newborn infant. J Thorac Cardiovasc Surg 2006;131:e1-2. |
Review/Other-Tx |
1 infant |
To report a hybrid approach to stabilization and repair of obstructed total anomalous pulmonary venous connection in a critically ill newborn infant. |
No abstract available. |
4 |
| 64. Kim TH, Kim YM, Suh CH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol 2000;175:1381-6. |
Observational-Dx |
14 patients (7 boys, 7 girls) |
The objective of this study was to investigate the usefulness of helical computed tomography (CT) angiography in the evaluation of total anomalous pulmonary venous connections. |
In all patients, helical CT angiography correctly depicted total anomalous pulmonary venous connections. Seven cases were the supracardiac type, four cases were the cardiac type, one case was the infracardiac type, and two cases were the mixed type. The detection rate of the pulmonary vein in 3D reconstruction images (95-98%) was slightly lower than that of the pulmonary vein in the axial images (100%), but the difference between axial and 3D reconstruction images was not statistically significant (p > 0.1). No statistically significant differences were noted among 3D reconstruction images in the detection rates of the pulmonary vein (p > 0.1). The extent of contrast enhancement of the pulmonary vein was good or excellent in all patients. In five patients, there were contrast-induced artifacts that made some surrounding vascular distortion but did not interfere with the pulmonary vein analysis, except in one patient. Motion-induced artifacts were observed in nine patients. One of them had an obstacle in pulmonary vein analysis. |
3 |
| 65. Shen Q, Pa M, Hu X, Wang J. Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatr Radiol 2013;43:827-35. |
Observational-Dx |
18 children (11 male, 7 female) |
The purpose of this study was to describe the chest radiographic features of obstructed total anomalous pulmonary venous connection (TAPVC) and compare computed tomography (CT) angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC. |
The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P = 0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P = 0.0002). |
2 |
| 66. Oh KH, Choo KS, Lim SJ, et al. Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography. Pediatr Radiol 2009;39:950-4. |
Observational-Dx |
23 patients (13 male, 10 female) |
To compare multidetector CT (MDCT) with echocardiography in the evaluation of total anomalous pulmonary venous connection (TAPVC). |
In all patients, MDCT correctly depicted the drainage site of the common pulmonary vein, stenosis of the vertical vein and the course of the atypical vessel into the systemic vein (sensitivity 100%, specificity 100%). The specificity of echocardiography was 100% for the three defined findings. The sensitivity of echocardiography, however, was 87%, 71% and 0%, respectively. |
3 |
| 67. Masrani A, McWilliams S, Bhalla S, Woodard PK. Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr 2018;12:286-89. |
Review/Other-Dx |
16 patients (3 males, 13 females) |
To report the anatomical associations and radiological characteristics of Scimitar syndrome on Computed Tomography (CT) and Magnetic Resonance (MR). |
Sixteen patients (3 males, 13 females; mean age 39.5 years, range 14 days-72 years) with confirmed Scimitar syndrome on CT and MR imaging were identified. The Scimitar vein drained to the infra-diaphragmatic inferior vena cava (IVC) in ten patients and to the supra-diaphragmatic IVC in six patients. The most common associated anomalies were right ventricle enlargement (93.3%), variant lobar pattern of the right lung (92.9%), enlarged pulmonary arteries (60%), and cardiac dextroposition (50%). |
4 |
| 68. Vyas HV, Greenberg SB, Krishnamurthy R. MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. Radiographics 2012;32:87-98. |
Review/Other-Dx |
N/A |
To describe various congenital pulmonary vein anomalies with an emphasis on embryology, morphology, appropriate indications for use of magnetic resonance (MR) imaging and computed tomography (CT) in neonates and infants, MR imaging and CT technique, and pertinent imaging manifestations. |
No results stated in the abstract. |
4 |
| 69. Riesenkampff EM, Schmitt B, Schnackenburg B, et al. Partial anomalous pulmonary venous drainage in young pediatric patients: the role of magnetic resonance imaging. Pediatr Cardiol 2009;30:458-64. |
Observational-Dx |
26 children |
To evaluate whether magnetic resonance imaging (MRI), performed in conscious sedation, is suited to accurately image partial anomalous pulmonary vein anatomy in infants and young children. |
Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed. The MRI protocol included shunt quantification by velocity-encoded cine as well as morphological and functional assessment by multislice multiphase and contrast-enhanced MR techniques. MRI was performed in the compliant patient in breath-hold (n = 8; age range, 4.6–9.5 years) and in the noncompliant patient in conscious-sedation free breathing (n = 18; age range, 0.4 to 7.5 years). In 22 patients, PAPVD was diagnosed with MRI and confirmed during surgery. In four patients with large atrial septal defects not accessible to percutaneous closure, normal pulmonary venous return was demonstrated by MRI and confirmed during surgery. MRI under conscious sedation accurately specifies the anatomy of pulmonary veins in infants and small children. |
4 |
| 70. Choe YH, Lee HJ, Kim HS, Ko JK, Kim JE, Han JJ. MRI of total anomalous pulmonary venous connections. J Comput Assist Tomogr 1994;18:243-9. |
Review/Other-Dx |
13 patients (8 males, 5 females) |
To report magnetic resonance (MR) findings of total anomalous pulmonary venous connections (TAPVC) and to evaluate the capability of MR in the diagnosis of TAPVC in comparison with those of echocardiography and cardiac angiography. |
Magnetic resonance detected TAPVC in four cases without prior echocardiographic and angiographic diagnosis. In seven surgically proven cases, the diagnostic accuracy of preoperative MR, echocardiography, and cardiac angiography was 100 (7 of 7), 57 (4 of 7), and 25% (1 of 4), respectively. In the remaining cases including three cases for postoperative evaluation, MR findings correlated well with those of echocardiography or angiography. The combination of axial and coronal MRI visualized 96% of the individual anomalous pulmonary veins and 100% of the common pulmonary veins. Stenosis of a common pulmonary vein (three cases) or the superior vena cava (one case) was identified on MR in all cases. |
4 |
| 71. Powell AJ, Tsai-Goodman B, Prakash A, Greil GF, Geva T. Comparison between phase-velocity cine magnetic resonance imaging and invasive oximetry for quantification of atrial shunts. Am J Cardiol 2003;91:1523-5, A9. |
Observational-Dx |
20 patients (12 females, 8 males) |
To prospectively evaluate the accuracy, reproducibility, and interobserver variability of phase-velocity cine magnetic resonance imaging (PVC MRI) measurements of pulmonary-to-systemic blood flow ratio (Qp/Qs) in subjects with a known shunt. |
We have previously reported that subjects without a shunt (n = 20) had a mean Qp/Qs of 0.99 ± 0.10 by PVC MRI. |
2 |
| 72. Stumper O, Vargas-Barron J, Rijlaarsdam M, et al. Assessment of anomalous systemic and pulmonary venous connections by transoesophageal echocardiography in infants and children. Br Heart J 1991;66:411-8. |
Review/Other-Dx |
76 unoperated infants and children |
To assess the value of transoesophageal echocardiography in the preoperative definition of systemic and pulmonary venous connections. |
Transoesophageal studies showed anomalous venous connections in 14 patients. Two had both anomalous systemic and pulmonary venous connections. Transoesophageal studies showed 12 anomalous systemic venous connections in nine patients. In eight patients these were confirmed at operation or catheterisation: one patient is awaiting operation. Six anomalous systemic venous connections were missed during earlier transthoracic studies. Anomalous pulmonary venous connections (one mixed total, six partial) were shown in seven patients. These were confirmed at operation in six and by cardiac catheterisation in one. Four of these patients were missed during earlier transthoracic ultrasound studies. No patient defined as having normal venous connections by the transoesophageal study was subsequently shown to have anomalous venous connections at operation or angiography. |
4 |
| 73. Ammash NM, Seward JB, Warnes CA, Connolly HM, O'Leary PW, Danielson GK. Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography. J Am Coll Cardiol 1997;29:1351-8. |
Review/Other-Dx |
43 patients |
To demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE). |
A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation. |
4 |
| 74. Chang YY, Chang CI, Wang MJ, et al. The safe use of intraoperative transesophageal echocardiography in the management of total anomalous pulmonary venous connection in newborns and infants: a case series. Paediatr Anaesth 2005;15:939-43. |
Review/Other-Dx |
28 neonates and infants |
To review the safety and efficacy of intraoperative transesophageal echocardiography (TEE) during total anomalous pulmonary venous connection (TAPVC) repair. |
Four patients received immediate surgical revision after primary surgery for residual anastomotic stenosis diagnosed by TEE. In addition, two unsuspected ventricular septal defects and three persistent ductus arteriosus were detected before surgery. Eight infants (29%) had hypotension and hypoxemia associated with TEE probe insertion before surgery, but this hemodynamic disturbance returned to baseline value after withdrawing the TEE probe from the esophagus. However, these eight patients had uneventful TEE probe insertion following sternotomy. The mechanism was probably because of the reduction of intrathoracic pressure when the chest was opened. |
4 |
| 75. Kenny D, Hijazi ZM. Coarctation of the aorta: from fetal life to adulthood. Cardiol J 2011;18:487-95. |
Review/Other-Dx |
N/A |
To outline the evaluation and treatment of this disease from pre-natal to adult life. |
No results in abstract. |
4 |
| 76. Marek J, Skovranek J, Hucin B, et al. Seven-year experience of noninvasive preoperative diagnostics in children with congenital heart defects: comprehensive analysis of 2,788 consecutive patients. Cardiology 1995;86:488-95. |
Observational-Dx |
2,788 children |
To assess prospectively the proportion of invasive and noninvasive diagnostic procedures used preoperatively in all children operated on between 1986 and 1992. |
The overall percentage of surgery based solely on noninvasive preoperative examination increased from 63% in 1986 to 81% in 1990 and decreased to 72% in 1992. There were no differences in the preoperative diagnostic approach between groups of newborn, infants and children. A high percentage of patients with patent ductus arteriosus (96.5%), atrial septal defect (94%), incomplete atrioventricular septal defect (88.6%), ventricular septal defect (86.3%), coarctation of the aorta (80.2%) and total anomalous pulmonary venous connection (79.3%) was referred for surgery without prior invasive examination, while a lower percentage was found in univentricular heart (48.4%), pulmonary atresia (34.6%) and double outlet right ventricle (27.7%). More patients with pulmonary and tricuspid atresia were catheterized before complete repair compared to those who underwent palliative surgery (p < 0.01 and p < 0.0001, respectively). The echocardiographic diagnosis was correct in 96% of patients. Two patients of those with incomplete preoperative diagnosis died early postoperatively, both with missed apical ventricular septal defect. One with tetralogy of Fallot died after reoperation, the other with persistent truncus arteriosus due to sepsis. |
2 |
| 77. Lee EY, Siegel MJ, Hildebolt CF, Gutierrez FR, Bhalla S, Fallah JH. MDCT evaluation of thoracic aortic anomalies in pediatric patients and young adults: comparison of axial, multiplanar, and 3D images. AJR Am J Roentgenol. 182(3):777-84, 2004 Mar. |
Observational-Dx |
14 patients |
To compare accuracies of axial, multiplanar, and 3D volume-rendered images in the diagnosis of thoracic aortic anomalies in pediatric patients and young adults. |
Average accuracies (average of the three observers for a correct diagnosis) were greater than or equal to 96% for diagnoses of aortic position and airway narrowing on all image types. For the diagnosis of coarctation, average sensitivities (average of the three observers for a true diagnosis) were 73% for axial, 100% for multiplanar, and 100% for 3D volume-rendered images. For the diagnosis of patent ductus arteriosus, average sensitivities were 78% for axial, 94% for multiplanar, and 89% for 3D volume-rendered images. No patients in this study had collateral vessel formation. For the diagnosis of absence of collateral vessel formation, average sensitivities were 100% for axial, 100% for multiplanar, and 100% for 3D volume-rendered images. There were no significant statistical differences in diagnostic performances, agreement with truth, or confidence scores among observers or imaging formats (p > 0.05). |
3 |
| 78. Dijkema EJ, Leiner T, Grotenhuis HB. Diagnosis, imaging and clinical management of aortic coarctation. [Review]. Heart. 103(15):1148-1155, 2017 08. |
Review/Other-Dx |
N/A |
To provide an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algorithms, treatment and follow-up of patients with coarctation of the aorta (CoA). |
No results stated in abstract. |
4 |
| 79. Karaosmanoglu AD, Khawaja RD, Onur MR, Kalra MK. CT and MRI of aortic coarctation: pre- and postsurgical findings. [Review]. AJR Am J Roentgenol. 204(3):W224-33, 2015 Mar. |
Review/Other-Dx |
N/A |
To summarize the roles of CT and MRI in the diagnosis and follow-up of patients with aortic coarctation. |
No results stated in abstract. |
4 |
| 80. Teien DE, Wendel H, Bjornebrink J, Ekelund L. Evaluation of anatomical obstruction by Doppler echocardiography and magnetic resonance imaging in patients with coarctation of the aorta. Br Heart J 1993;69:352-5. |
Observational-Dx |
32 patients |
To evaluate a new Doppler echocardiographic index of obstruction in patients with native coarctation or recoarctation. |
There was a close correlation between the MRI index of obstruction and the Doppler velocity ratio (r = 0.92). The sensitivity and specificity of this ratio in detecting a CoA index equal to or less than 0.25 were better than those obtained with gradients only or the combination of gradients and diastolic half time. |
3 |
| 81. Nielsen JC, Powell AJ, Gauvreau K, Marcus EN, Prakash A, Geva T. Magnetic resonance imaging predictors of coarctation severity. Circulation. 111(5):622-8, 2005 Feb 08. |
Observational-Dx |
31 subjects |
To evaluate the ability of MRI to distinguish between mild versus moderate and severe coarctation (CoA) as determined by cardiac catheterization. |
Patients were divided into 2 groups on the basis of peak coarctation gradient by catheterization: <20 mm Hg (n=12) and > or =20 mm Hg (n=19). Patients with cardiac index <2.2 L x min(-1) x m(-2) by catheterization were excluded. By logistic regression analysis, the following variables simultaneously predicted coarctation gradient > or =20 mm Hg: (1) smallest aortic cross-sectional area (adjusted for body surface area) measured by planimetry from gadolinium-enhanced 3D magnetic resonance angiography (OR 1.71 for 10 mm2/m2 decrease, P=0.005) and (2) heart rate-corrected mean flow deceleration in the descending aorta measured by phase-velocity cine MRI (OR 1.68 for 100 mL/s(1.5) increase, P=0.018). For the combination of these variables, a predicted probability >0.38 had 95% sensitivity, 82% specificity, 90% positive and negative predictive values, and an area under the receiver-operator characteristics curve of 0.938. In a subsequent validation study, the prediction model correctly classified 9 of 10 patients, with no false-negatives. |
3 |
| 82. Muzzarelli S, Meadows AK, Ordovas KG, et al. Prediction of hemodynamic severity of coarctation by magnetic resonance imaging. Am J Cardiol 2011;108:1335-40. |
Observational-Dx |
79 patients |
To derive a simple and clinically practical algorithm to predict severe coarctation of the aorta (CoA) from data obtained by cardiovascular magnetic resonance. |
Seventy-nine consecutive patients who underwent cardiovascular magnetic resonance and cardiac catheterization for the evaluation of native or recurrent CoA at Children's Hospital Boston (n = 30) and the University of California, San Francisco (n = 49), were retrospectively reviewed. The published formula derived from data obtained at Children's Hospital Boston was first validated from data obtained at the University of California, San Francisco. Next, pooled data from the 2 institutions were analyzed, and a refined model was created using logistic regression methods. Finally, recursive partitioning was used to develop a clinically practical prediction tree to predict transcatheter systolic pressure gradient =20 mm Hg. Severe CoA was present in 48 patients (61%). Indexed minimal aortic cross-sectional area and heart rate–corrected flow deceleration time in the descending aorta were independent predictors of CoA gradient =20 mm Hg (p <0.01 for both). A prediction tree combining these variables reached a sensitivity and specificity of 90% and 76%, respectively. |
3 |
| 83. LoPresti MA, Ghali MZ, Srinivasan VM, et al. Neurovascular findings in children and young adults with Loeys-Dietz syndromes: Informing recommendations for screening. J Neurol Sci 2020;409:116633. |
Observational-Dx |
47 patients |
To review our institution’s experience with special focus on neurovascular imaging to better understand the pathology and guide screening. |
Of 47 patients with LDS identified, 39 (83.0%) were found to have neuroimaging. Intracranial and cervical vascular tortuosity were seen in 79.5% and 64.1%, respectively. Twenty-one patients (44.7%) received follow-up screening, of which 3 were found to have progression. Time to progression was an average of 2.1 years. Average follow-up was 607 days (range 123–3070 days). Mean Arterial Tortuosity Index for the right ICA, left ICA, right VA, and left VA were 18, 20, 49, and 47, respectively. Comparison of interval percent change in Arterial Tortuosity Index over the course of follow-up demonstrated small changes in the right ICA (mean 5%), left ICA (mean 1%), right VA (mean 1%), and left VA (mean 2%). |
3 |
| 84. Tzemos N, Therrien J, Yip J, et al. Outcomes in adults with bicuspid aortic valves. JAMA 2008;300:1317-25. |
Review/Other-Dx |
642 adults |
To determine the frequency and predictors of cardiac outcomes in a large consecutive series of adults with bicuspid aortic valve. |
During the follow-up period, there were 28 deaths (mean [SD], 4% [1%]). One or more primary cardiac events occurred in 161 patients (mean [SD], 25% [2%]), which included cardiac death in 17 patients (mean [SD], 3% [1%]), intervention on aortic valve or ascending aorta in 142 patients (mean [SD], 22% [2%]), aortic dissection or aneurysm in 11 patients (mean [SD], 2% [1%]), or congestive heart failure requiring hospital admission in 16 patients (mean [SD], 2% [1%]). Independent predictors of primary cardiac events were age older than 30 years (hazard ratio [HR], 3.01; 95% confidence interval [CI], 2.15-4.19; P<.001), moderate or severe aortic stenosis (HR, 5.67; 95% CI, 4.16-7.80; P<.001), and moderate or severe aortic regurgitation (HR, 2.68; 95% CI, 1.93-3.76; P<.001). The 10-year survival rate of the study group (mean [SD], 96% [1%]) was not significantly different from population estimates (mean [SD], 97% [1%]; P = .71). At last follow-up, 280 patients (mean [SD], 45% [2%]) had dilated aortic sinus and/or ascending aorta. |
4 |
| 85. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010;121:e266-369. |
Review/Other-Dx |
N/A |
To assist healthcare providers in clinical decision making by describing a range of generally acceptable approaches for diagnosis, management, and prevention of specific diseases or conditions. This guideline includes diseases involving any or all parts of the thoracic aorta with the exception of aortic valve diseases5 and includes the abdominal aorta when contiguous thoracic aortic diseases are present. |
No abstract available. |
4 |
| 86. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: executive summary. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Catheter Cardiovasc Interv 2010;76:E43-86. |
Review/Other-Dx |
N/A |
No abstract available. |
No abstract available. |
4 |
| 87. Landis BJ, Ware SM, James J, Shikany AR, Martin LJ, Hinton RB. Clinical Stratification of Pediatric Patients with Idiopathic Thoracic Aortic Aneurysm. J Pediatr 2015;167:131-7 e1-5. |
Observational-Dx |
69 patients |
To describe the global phenotypes of pediatric patients with thoracic aortic aneurysm (TAA) who do not have a clinical diagnosis of Marfan syndrome (MFS) or related connective tissue disorders. We hypothesized that the presence of noncardiovascular abnormalities correlate with TAA severity and that medical therapy reduces TAA progression. |
Sixty-nine patients with TAA at mean age 12.5 ± 5.3 years were included. Noncardiovascular abnormalities, including skeletal (65%) or craniofacial (54%) findings, were frequently observed. Increased rate of aortic root enlargement was associated with ocular (P = .002) and cutaneous (P = .003) abnormalities, and increased rate of ascending aorta enlargement was associated with craniofacial (P < .001) abnormalities. Beta blocker or angiotensin receptor blocker therapy (n = 41) was associated with reduction in the rate of aortic root growth (P = .018). |
3 |
| 88. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg 2014;148:e1-e132. |
Review/Other-Dx |
N/A |
Evidence-based guidelinesto assist referring physicians and other providers in making the most appropriate imaging or treatment decision for the management of patients with valvular heart disease. |
No abstract available. |
4 |
| 89. Meindl C, Achatz B, Huber D, et al. Coronary Artery Ectasia Are Frequently Observed in Patients With Bicuspid Aortic Valves With and Without Dilatation of the Ascending Aorta. Circ., Cardiovasc. interv.. 9(10), 2016 10. |
Observational-Dx |
177 patients |
To determine if coronary artery ectasia (CAE) is commonly observed in patients with bicuspid aortic valve (BAV). |
One hundred seventy-seven patients with suspected aortic valve disease (n=94 BAV, n=83 tricuspid aortic valve) underwent both cardiac magnetic resonance imaging and coronary angiography. To confirm the association of CAE with BAV, the frequency of CAE was evaluated in an in-house BAV registry (n=600, n=231 with available coronary angiogram) and compared with the frequency of CAE in the German Myocardial Infarction (MI) Family Study, in which the heritability of CAE was formerly established (n=899). Furthermore, the frequency of CAE was investigated in an observational registry of real-life patients undergoing coronary angiography for clinically indicated reasons (n=3.097) and in a subgroup of the KORA MI study (Cooperative Health Research in the Region of Augsburg), which is a population-based MI registry (n=403).Compared with tricuspid aortic valve disease, CAE occurred more than twice as frequently in cardiac magnetic resonance-confirmed BAV disease (17% versus 44%; P<0.0001) and CAE was observed similarly often in subjects with BAV with (37%) and without (54%, P=0.11) ascending aortic pathology. The common appearance of CAE in patients with BAV could be independently confirmed in the BAV registry (frequency 37%), whereas CAE was found less frequently in family history of positive MI patients (21%), sporadic MI without familial disposition (10%), and rarely in unrelated real-life catheterization patients (6%). |
2 |
| 90. Jensen CJ, Jochims M, Hunold P, et al. Assessment of left ventricular function and mass in dual-source computed tomography coronary angiography: influence of beta-blockers on left ventricular function: comparison to magnetic resonance imaging. Eur J Radiol 2010;74:484-91. |
Observational-Dx |
32 patients |
To quantify left ventricular (LV) function and mass (LVM) derived from dual-source computed tomography (DSCT) and the influence of beta-blocker administration compared to cardiac magnetic resonance imaging (CMR). |
Thirty-two patients undergoing cardiac DSCT and CMR were included, where of fifteen received metoprolol intravenously before DSCT. LV parameters were calculated by the disc-summation method (DSM) and by a segmented region-growing algorithm (RGA). All data sets were analyzed by two blinded observers. Interobserver agreement was tested by the intraclass correlation coefficient. RESULTS.: 1. Using DSM LV parameters were not statistically different between DSCT and CMR in all patients (DSCT vs. CMR: EF 63+/-8% vs. 64+/-8%, p=0.47; EDV 136+/-36 ml vs. 138+/-35 ml, p=0.66; ESV 52+/-21 ml vs. 52+/-22 ml, p=0.61; SV 83+/-22 ml vs. 87+/-19 ml, p=0.22; CO 5.4+/-0.9l/min vs. 5.7+/-1.2l/min, p=0.09, LVM 132+/-33 g vs. 132+/-33 g, p=0.99). 2. In a subgroup of 15 patients beta-blockade prior to DSCT resulted in a lower ejection fraction (EF), stroke volume (SV), cardiac output (CO) and increase in end systolic volume (ESV) in DSCT (EF 59+/-8% vs. 62+/-9%; SV 73+/-17 ml vs. 81+/-15 ml; CO 5.7+/-1.2l/min vs. 5.0+/-0.8 l/min; ESV 52+/-27 ml vs. 57+/-24 ml, all p<0.05). 3. Analyzing the RGA parameters LV volumes were not significantly different compared to DSM, whereas LVM was higher using RGA (177+/-31 g vs. 132+/-33 g, p<0.05). Interobserver agreement was excellent comparing DSM values with best agreement between RGA calculations. |
2 |
| 91. Gordon DZ, Abbasi MA, Lee J, et al. Four-dimensional Flow Magnetic Resonance Imaging Quantification of Blood Flow in Bicuspid Aortic Valve. J Thorac Imaging 2020;35:383-88. |
Observational-Dx |
30 patients |
To test the efficiency and inter-observer reproducibility of a dedicated 4D flow magnetic resonance imaging (MRI) analysis workflow. |
4D flow analysis workflow time for both observers: 5.0±1.4 minutes per case (range 3–10 minutes). Valve outflow jets and flow derangement was visible in all 30 BAV patients (both observers). Net flow, peak velocity, and regurgitant fraction was significantly elevated in BAV patients compared to controls except for regurgitant fraction in plane 4 (91.1±29.7ml/s versus 62.6±19.6ml/s, 37.1% difference; 121.7±49.7cm/s versus 90.9±26.4cm/s, 28.9% difference; 9.3±10.1% versus 2.0±3.4%, 128.0% difference, respectively; p<0.001). Excellent ICC agreement for net flow: 0.979, peak velocity: 0.931 and regurgitant fraction: 0.928. |
2 |
| 92. Galian-Gay L, Rodriguez-Palomares J, Guala A, Michelena HI, Evangelista A. Multimodality imaging in bicuspid aortic valve. Prog Cardiovasc Dis 2020;63:442-51. |
Review/Other-Dx |
N/A |
To review the advantages and limitations of the imaging techniques in the diagnosis and management of Bicuspid aortic valve (BAV) and the best strategies in the use of multimodality imaging. |
No results stated in the abstract. |
4 |
| 93. Berhane H, Scott M, Elbaz M, et al. Fully automated 3D aortic segmentation of 4D flow MRI for hemodynamic analysis using deep learning. Magn Reson Med 2020;84:2204-18. |
Observational-Dx |
1,018 patients |
To generate fully automated and fast 4D-flow MRI-based 3D segmentations of the aorta using deep learning for reproducible quantification of aortic flow, peak velocity, and dimensions. |
Convolutional neural network segmentation required 0.438 ± 0.355 seconds versus 630 ± 254 seconds for manual analysis and demonstrated excellent performance with a median Dice score of 0.951 (0.930-0.966), Hausdorff distance of 2.80 (2.13-4.35), and average symmetrical surface distance of 0.176 (0.119-0.290). Excellent agreement was found for flow, peak velocity, and dimensions with low bias and limits of agreement less than 10% difference versus manual analysis. For aortic volume, limits of agreement were moderate within 16.3%. Interobserver variability (median Dice score: 0.950; Hausdorff distance: 2.45; and average symmetrical surface distance: 0.145) and convolutional neural network-based analysis (median Dice score: 0.953-0.959; Hausdorff distance: 2.24-2.91; and average symmetrical surface distance: 0.145-1.98 to observers) demonstrated similar reproducibility. |
2 |
| 94. Morris SA, Orbach DB, Geva T, Singh MN, Gauvreau K, Lacro RV. Increased vertebral artery tortuosity index is associated with adverse outcomes in children and young adults with connective tissue disorders. Circulation 2011;124:388-96. |
Experimental-Dx |
90 patients |
To hypothesize that arterial tortuosity is a marker for vascular fragility and that an objective measure of tortuosity for patients with connective tissue disorders (CTD) could be developed that would be associated with clinical outcomes |
We performed a retrospective analysis of 90 patients =50 years of age with Marfan syndrome, LDS, Ehlers-Danlos syndrome, or nonspecific connective tissue disorder who underwent thoracic contrast-enhanced magnetic resonance angiography. Controls (n=30) underwent magnetic resonance imaging to exclude arrhythmogenic right ventricular dysplasia. Using a volume-rendered angiogram, vertebral arteries were measured along the curvature of the vessel (actual length) and linearly (straight length), and distance factor was calculated: [(actual/straight length-1)×100]. Each subject's maximum distance factor was designated the Vertebral Tortuosity Index (VTI). The VTI was compared among diagnostic groups and among patients with cardiac surgery, dissection, and death. Median age at magnetic resonance imaging was 19.6 years (range 0.2 to 50.1). VTI interrater reliability was excellent (intraclass correlation coefficient =0.987). The VTI was higher in Marfan syndrome (n=57, median 26; interquartile range 10 to 49) and LDS (n=13, median 58; interquartile range 18 to 92) compared with controls (median 4.5; interquartile range 3 to 6; P<0.001 for both). Higher VTI was associated with younger age at surgery even when controlling for root size (adjusted P=0.002). Vertebral tortuosity index =50 was associated with earlier age at dissection and death compared with VTI <50 (P=0.001 versus P<0.001). We found no difference in age at surgery, dissection, or death in Marfan syndrome compared with LDS. |
2 |
| 95. Roman MJ, Devereux RB, Kramer-Fox R, Spitzer MC. Comparison of cardiovascular and skeletal features of primary mitral valve prolapse and Marfan syndrome. Am J Cardiol 1989;63:317-21. |
Review/Other-Dx |
177 patients |
To compare cardiovascular and skeletal features of primary mitral valve prolapse and Marfan syndrome. |
Echocardiographic, skeletal and anthropometric findings in 59 subjects with primary MVP and 59 age- and sex-matched patients with Marfan syndrome were compared with those in 59 control subjects. Subjects with mitral prolapse were similar to control subjects and differed (p < 0.025 to p < 0.001) from the patients with Marfan syndrome in aortic root dimensions, height, arm span, upper/lower segment ratio and prevalences of arachnodactyly, scoliosis and pectus carinatum. Subjects with mitral prolapse and patients with Marfan syndrome had similar body mass indexes and prevalences of pectus excavatum and straight back. All 3 groups were similar in arm span/height ratio. The 5 subjects with MVP and arachnodactyly had lower weights, smaller body surface areas and smaller aortic root dimensions, and were more likely to have scoliosis than subjects with MVP without arachnodactyly. |
4 |
| 96. Stefek HA, Berhane H, Robinson JD, et al. Comprehensive MR Analysis of Cardiac Function, Aortic Hemodynamics and Left Ventricular Strain in Pediatric Cohort with Isolated Bicuspid Aortic Valve. Pediatr Cardiol 2019;40:1450-59. |
Observational-Dx |
58 patients |
To use cardiac magnetic resonance imaging (CMR) to evaluate left ventricular (LV) parameters, myocardial strain and aortic hemodynamics in pediatric bicuspid aortic valve (BAV) patients with and without aortic stenosis (AS) or regurgitation (AR) compared to tricuspid aortic valve (TAV) controls. |
BAV patients with no valvulopathy or isolated AS had similar LV parameters to controls excepting cardiac output(p<0.05). AS+AR and post-surgical patients had abnormal LV volumetric and mass indices(p<0.01). Post-surgical patients had decreased global longitudinal strain(p=0.02); other subgroups had comparable strain to controls. Patients with valvulopathy demonstrated elevated velocity and wall shear stress(WSS) in the ascending aorta(AAo) and arch(p<0.01), while those without valve dysfunction had only elevated AAo velocity(p=0.03). Across the cohort, elevated AAo velocity and WSS correlated to higher LV mass(p<0.01), and abnormal hemodynamics correlated to decreased strain rates(p<0.045). |
3 |
| 97. Alpendurada F, Wong J, Kiotsekoglou A, et al. Evidence for Marfan cardiomyopathy. Eur J Heart Fail 2010;12:1085-91. |
Observational-Dx |
68 adult patients |
To evaluate the existence of a Marfan-related cardiomyopathy using cardiovascular magnetic resonance. |
One quarter (25.0%) of Marfan patients had reduced left ventricular ejection fraction (LVEF), with 25.0% having increased left ventricular end-diastolic and 30.8% having increased end-systolic volumes. The right ventricular ejection fraction was reduced in 10.3%, with increased right ventricular end-diastolic volumes in 11.8% and increased end-systolic volumes in 13.2%. On univariate analysis, no association was found between reduced LVEF and age, gender, indexed aortic dimensions, presence of mitral valve prolapse, or valve regurgitation. |
3 |
| 98. Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-85. |
Review/Other-Dx |
N/A |
To discuss the revised Ghent nosology for the Marfan syndrome. |
No results stated in the abstract. |
4 |
| 99. American College of Radiology. ACR Appropriateness Criteria® Radiation Dose Assessment Introduction. Available at: https://www.acr.org/-/media/ACR/Files/Appropriateness-Criteria/RadiationDoseAssessmentIntro.pdf. |
Review/Other-Dx |
N/A |
To provide evidence-based guidelines on exposure of patients to ionizing radiation. |
No abstract available. |
4 |
