Cardiovascular magnetic resonance parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation

Background: We sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts. Methods: Patients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation. Results: In the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4–84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3–51). The minimum CMR values were a LV EDV of 22 ml/m2 and a LV-to-RV stroke volume ratio of 0.19. Conclusions: In BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified

Outcomes of inferior sinus venosus defect repair

ObjectiveInferior sinus venosus defect is an unusual form of interatrial communication with few published data on surgical outcomes. We sought to compare outcomes of surgical repair of inferior sinus venosus defect with those of large secundum atrial septal defects.MethodsPatients undergoing surgical closure of an isolated interatrial defect were reviewed, and those with inferior sinus venosus defect were identified on the basis of predetermined anatomic criteria. For each case, 2 controls with secundum atrial septal defect, matched for age and year of surgery, were selected. Technical outcome scores and other perioperative outcomes were compared.ResultsCompared with the secundum atrial septal defect group (n = 90), the inferior sinus venosus defect group (n = 45) had worse technical outcome scores (P = .02), a higher rate of reintervention (9% vs 1%, P = .04), longer median total cardiopulmonary bypass (48 vs 39 minutes, P < .001) and crossclamp (29 vs 20 minutes, P < .001) times, and were more likely to stay more than 1 day in the intensive care unit (20% vs 8%, P = .04) and more than 3 days in the hospital (29% vs 13%, P = .03). Only 16 (36%) of the patients with inferior sinus venosus defect had a correct diagnosis preoperatively. Patients with an incorrect diagnosis had worse technical outcome scores than the secundum atrial septal defect group (P = .003), whereas those with a correct diagnosis had scores similar to those of the secundum atrial septal defect group (P = .55).ConclusionsCompared with patients with secundum atrial septal defect, patients with inferior sinus venosus defect have more residual defects and longer durations of cardiopulmonary bypass and hospitalization. Rates of misdiagnosis of inferior sinus venosus defect are high and associated with worse technical outcome scores. Accurate preoperative diagnosis of this lesion may lead to improved outcomes

Integrated Clinical and Magnetic Resonance Imaging Assessments Late After Fontan Operation

BACKGROUND Several clinical and cardiac magnetic resonance (CMR)-derived parameters have been shown to be associated with death or heart transplant late after the Fontan operation. OBJECTIVES The objective of this study was to identify the relative importance and interactions of clinical and CMR-based parameters for risk stratification after the Fontan operation. METHODS Fontan patients were retrospectively reviewed. Clinical and CMR parameters were analyzed using univariable Cox regression. The primary endpoint was time to death or (listing for) heart transplant. To identify the patients at highest risk for the endpoint, classification and regression tree survival analysis was performed, including all significant variables from Cox regression. RESULTS The cohort consisted of 416 patients (62% male) with a median age of 16 years (25th, 75th percentiles: 11, 23 years). Over a median follow-up of 5.4 years (25th, 75th percentiles: 2.4, 10.0 years) after CMR, 57 patients (14%) reached the endpoint (46 deaths, 7 heart transplants, 4 heart transplant listings). Lower total indexed end-diastolic volume (EDVi) was the strongest predictor of transplant-free survival. Among patients with dilated ventricles (EDVi >= 156 ml/BSA(1.3)), worse global circumferential strain (GCS) was the next most important predictor (73% vs. 44%). In patients with smaller ventricles (EDVi = II was the next most important predictor (30% vs. 4%). CONCLUSIONS In this cohort of patients late after Fontan operation, increased ventricular dilation was the strongest independent predictor of death or transplant (listing). Patients with both ventricular dilation and worse GCS were at highest risk. These data highlight the value of integrating CMR and clinical parameters for risk stratification in this population. (C) 2021 by the American College of Cardiology Foundation

Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology

This paper aims to provide information and explanations regarding the clinically relevant options, strengths, and limitations of cardiovascular magnetic resonance (CMR) in relation to adults with congenital heart disease (CHD). Cardiovascular magnetic resonance can provide assessments of anatomical connections, biventricular function, myocardial viability, measurements of flow, angiography, and more, without ionizing radiation. It should be regarded as a necessary facility in a centre specializing in the care of adults with CHD. Also, those using CMR to investigate acquired heart disease should be able to recognize and evaluate previously unsuspected CHD such as septal defects, anomalously connected pulmonary veins, or double-chambered right ventricle. To realize its full potential and to avoid pitfalls, however, CMR of CHD requires training and experience. Appropriate pathophysiological understanding is needed to evaluate cardiovascular function after surgery for tetralogy of Fallot, transposition of the great arteries, and after Fontan operations. For these and other complex CHD, CMR should be undertaken by specialists committed to long-term collaboration with the clinicians and surgeons managing the patients. We provide a table of CMR acquisition protocols in relation to CHD categories as a guide towards appropriate use of this uniquely versatile imaging modalit

Interactive Whole-Heart Segmentation in Congenital Heart Disease

We present an interactive algorithm to segment the heart chambers and epicardial surfaces, including the great vessel walls, in pediatric cardiac MRI of congenital heart disease. Accurate whole-heart segmentation is necessary to create patient-specific 3D heart models for surgical planning in the presence of complex heart defects. Anatomical variability due to congenital defects precludes fully automatic atlas-based segmentation. Our interactive segmentation method exploits expert segmentations of a small set of short-axis slice regions to automatically delineate the remaining volume using patch-based segmentation. We also investigate the potential of active learning to automatically solicit user input in areas where segmentation error is likely to be high. Validation is performed on four subjects with double outlet right ventricle, a severe congenital heart defect. We show that strategies asking the user to manually segment regions of interest within short-axis slices yield higher accuracy with less user input than those querying entire short-axis sliceNatural Sciences and Engineering Research Council of Canada (Alexander Graham Bell Canada Graduate Scholarships-Doctoral Program (CGS D))Wistron CorporationNational Institute for Biomedical Imaging and Bioengineering (U.S.) (NAMIC U54-EB005149)Boston Children's Hospital (Translational Research Program Fellowship)Boston Children's Hospital. Office of Faculty DevelopmentHarvard Catalys