Outcome of biventricular repair of congenital cardiac malformations with a borderline left ventricle

Introduction: Biventricular repairs are occasionally done on hearts with a borderline-sized left ventricle to avoid univentricular palliation. We aim to assess the outcome. Material and methods Data of 27 patients with aortic arch hypoplasia, coarctation and borderline left ventricle that had undergone biventricular repair in our centers in the past 20 years (median follow-up: 5 years) were analyzed retrospectively. Results Successful primary repair was performed in 17 (63%) patients. Four (15%) patients initially underwent primary repairs, which were converted to Norwood reconstructions, hoping to re-attempt biventricular repair later. Primary Norwood reconstruction with a restrictive atrial septal defect or its hybrid equivalent were performed in 6 (22%) patients for ventricular rehabilitation, with subsequent biventricular repair a few months later. The total number of procedures was 89 (average: 3.3 per patient). This strategy failed in 8 (30%) patients: 7 died (4 within 1 year, 1 at 18 months, 1 at 3 years, 1 at 3.5 years), and 1 was converted to univentricular palliation eventually reaching Fontan completion. Three (11%) patients reached favorable outcomes: alive and asymptomatic with biventricular circulation, good ventricular function and normal-sized valves. The remaining 16 (59%) patients are alive but with residual lesions, which may include ventricular diastolic dysfunction, somewhat small mitral and/or aortic valves and pulmonary hypertension; 9 are in NYHA class I and 7 in class II. Conclusion The results are suboptimal. Further studies are needed to identify ways of predicting who would benefit most from aggressively pursuing a biventricular strategy and offer it to them selectively.