Background: Dilated cardiomyopathy (DCM) is a leading cause of heart transplantation (HTx) in children. Surgical pulmonary artery banding (PAB) is used worldwide to achieve functional heart regeneration and remodelling.
Case summary: We report for the first-time successful bilateral transcatheter implantation of bilateral pulmonary artery flow restrictors in a case series of three infants with severe DCM based on left-ventricular non-compaction morphology associated with Barth syndrome in one and a non-classified syndrome in another. Functional cardiac regeneration was observed in two patients after almost 6 months of endoluminal banding, and in the neonate with Barth syndrome already after 6 weeks. Accompanied by an improvement in functional class (Class IV to Class I), the left ventricular end-diastolic dimensions z-score normalized, as did the elevated serum brain natriuretic peptide levels. A listing for HTx could be avoided.
Discussion: Percutaneous bilateral endoluminal PAB is a novel minimally invasive approach that enables functional cardiac regeneration in infants with severe DCM and preserved right ventricular function. Interruption of the ventriculo-ventricular interaction, the key mechanism for recovery, is avoided. Intensive care for these critically ill patients is reduced to a minimum. However, investing in 'heart regeneration to avoid transplantation' remains a challenge.
Keywords: Case series; Endovascular pulmonary artery banding; Functional cardiac regeneration; Paediatric dilated cardiomyopathy; Pulmonary flow restrictors.
© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.