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

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.