Transcatheter ventricular septal shunt creation in end-stage paediatric pulmonary arterial hypertension: lessons from a rescue decompression strategy

Abstract

A 12-year-old girl with end-stage suprasystemic pulmonary arterial hypertension and progressive right ventricular failure underwent transcatheter ventricular septal shunt creation after deterioration despite targeted therapy and prior atrial septostomy. Right-to-left shunting was established, but haemodynamic improvement was limited; pulmonary pressures remained suprasystemic, persistent pericardial effusion developed, and the patient died despite intensive treatment, illustrating the complexity of rescue decompression in advanced disease.