Two-stage surgery for a large ventricular septal defect and patent ductus arteriosus associated with severe pulmonary arterial hypertension in an adult patient

Abstract

Introduction Adult patients with pulmonary arterial hypertension (PAH) associated with congenital left-to-right shunting are often considered inoperable. Case outline A 26-year-old man presented with effort intolerance and palpitations. The diagnosis of PAH in the presence of a large perimembranous ventricular septal defect (VSD) and patent ductus arteriosus (PDA) was established. The patient was managed with a two-stage surgical approach involving an initial ligation of PDA, followed by VSD closure using unidirectional valved patch. Treatment decisions were based on the results of both invasive and non-invasive investigations. At follow-up, the patient was asymptomatic with pulmonary arterial pressure and vascular resistance returning to normal levels. Conclusion There is a possibility for an adult patient to have a congenital heart disease associated with marked pulmonary overcirculation that is still amenable to surgical repair. This implies that there is an individual response to a long-standing left-to-right shunt, and that the therapy should be considered on a case-by-case basis.