Converting an Infant with Borderline Left Heart Structures to Biventricular Circulation after an initial Hybrid Norwood Approach: Case Report

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 interatrial communication, it offers a chance for biventricular circulation.

Method: We present the case of a term newborn with total anomalous pulmonary vein connection (TAPVC), hypoplastic left heart, small aortic arch and ASD who was multidisciplinary deemed eligible for a hybrid palliation performed on day 5 of life after TAPVC surgical correction. Consecutively, percutaneous ductal stent implantation was performed (2 Sinus Superflex DS stents) on day 11 of life. Initial cardiac MR imaging showed a hypoplastic non-apex forming left ventricle (LV) (left ventricular end-diastolic volume index (LVEDVI) 15 mL/m²). Subsequently, on day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MR showed striking LV growth (LVEDVI: 37 mL/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.

Conclusion: Hybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multidisciplinary 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.

Publication History

Article published online:
28 January 2023