The rudimentary left ventricle does not impact on right ventricular size and function in hypoplastic left heart syndrome during serial follow up after Fontan completion.

Background: Previous studies in patients with hypoplastic left heart syndrome (HLHS) suggested that a larger left ventricle (LV) might negatively impact right ventricular (RV) function. This study aimed to analyse the impact of the presence of an LV remnant on RV size and function during serial follow up. Methods: Serial cardiovascular magnetic resonance (CMR) examinations after completion of the total cavopulmonary connection were retrospectively analysed. Patients were divided into those with and those without a rudimentary LV. RV and LV end diastolic and end systolic volumes as well as stroke volume, ejection fraction (RVEF, LVEF) and end diastolic mass were measured. Results: 90 HLHS patients (female: 26) who had at least two CMR examinations were included. 51 patients had three examinations. 56 patients had an LV remnant, 34 did not. RV volumes and mass indexed to body surface area as well as RVEF did not differ significantly between both groups. LV volumes showed no association with RV volumes and RVEF. Conclusion: Analysis of serial CMR examination suggests that the presence of an LV remnant does not have a major impact on RV size and function during longer-term follow-up. Future studies might focus on regional RV function.