Person:

Geva, Tal

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.

OBJECTIVES The Norwood procedure for hypoplastic left heart syndrome (HLHS) is performed either via a right ventricle-to-pulmonary artery (RVPA) conduit or a modified Blalock–Taussig (MBT) shunt. Cardiac magnetic resonance (CMR) data was used to assess the effects of the RVPA conduit on ventricular shape and function through a computational analysis of anatomy and assessment of indices of strain. METHODS A retrospective analysis of 93 CMR scans of subjects with HLHS was performed (59 with MBT shunt, 34 with RVPA conduit), incorporating data at varying stages of surgery from two congenital centres. Longitudinal and short-axis cine images were used to create a computational cardiac atlas and assess global strain. RESULTS Those receiving an RVPA conduit had significant differences (P< 0.0001) in the shape of the RV corresponding to increased ventricular dilatation (P = 0.001) and increased sphericity (P = 0.006). Differences were evident only following completion of stage II surgery. Despite preserved ejection fraction in both groups, functional strain in the RVPA conduit group compared with that in the MBT shunt group was reduced across multiple ventricular axes, including a reduced systolic longitudinal strain rate (P< 0.0001), reduced diastolic longitudinal strain rate (P = 0.0001) and reduced midventricular systolic circumferential strain (P < 0.0001). CONCLUSIONS Computational modelling analysis reveals differences in ventricular remodelling in patients with HLHS undergoing an RVPA conduit insertion with focal scarring and volume loading leading to decreased functional markers of strain. The need for continued surveillance is warranted, as deleterious effects may not become apparent until later years.