Ventricular septal defect with severe pulmonary hypertension treated with two-step surgical repair involving ventricular septal defect closure and fenestrated atrial septal defect creation and closure: a case report

Abstract

　Ventricular septal defects (VSDs) that progress to Eisenmenger's syndrome are not usually an indication for surgical repair. However, some patients with atrial septal defects (ASDs) and severe pulmonary hypertension (PH) can be curatively treated with transcatheter ASD closure combined with pulmonary vasodilatation.

　A 65-year-old man presented with a double-outlet right ventricle. He had been diagnosed with Eisenmenger's syndrome in childhood and subjected to a long-term treatment with pulmonary vasodilators. We performed a surgical closure of the VSD accompanied by ASD creation with fenestration. Consequently, his PH ameliorated, and we performed ASD closure with a transcatheter device 12 months later.

　The present case indicates a possible comprehensive treatment strategy for patients with adult congenital heart disease despite progression to Eisenmenger's syndrome, placed on long-term medications for PH.