Converting an infant with borderline left heart structures to biventricular circulation after an initial hybrid Norwood approach: A case report

Abstract

Background: Hybrid approach in hypoplastic left heart syndrome (HLHS) implies placement of bilateral pulmonary artery bands and ductal stenting. This approach serves as a less invasive surgical-interventional alternative to the Norwood procedure with reported equivalent survival. Potential benefits include deferrable surgical risks and in some borderline left ventricle patients, when combined with a restrictive inter-Atrial communication, it offers a chance for biventricular circulation. Case summary: Term newborn with total anomalous pulmonary venous connection (TAPVC), hypoplastic left heart, small aortic arch, and atrial septal defect (ASD) who was multi-disciplinary deemed eligible for a hybrid palliation performed on day six of life after TAPVC surgical correction. Subsequently, percutaneous ductal stent implantation was performed (two Sinus Superflex-DS stents) on day 11 of life. Magnetic resonance imaging (MRI) showed a hypoplastic non-Apex-forming left ventricle (LV) [left ventricular end-diastolic volume index (LVEDVI) 15mL/m2]. On day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MRI showed striking LV growth (LVEDVI 37mL/m2). Herein an interventional hybrid take-down was performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with an Amplatzer Vascular plug II, thus achieving biventricular circulation. Discussion: Hybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multi-disciplinary care in high-risk two ventricular patients by means of LV rehabilitation. Further studies are needed to systematically define and understand the intricacies of potential candidates for staged biventricular repair initially managed with hybrid approach.