Medical and surgical management of a pulmonary hypertensive adult patient with unrepaired complex congenital heart disease: a case report

Abstract

BackgroundSome patients with congenital heart defects are considered inoperable because of severe pulmonary arterial hypertension (PAH) at birth, and some of these patients are followed for a long period. Recently, PAH-specific drugs have been developed to treat PAH. These drugs might make it possible to operate in adult patients with congenital heart defects who were previously considered inoperable. However, it is unclear which cases are appropriate for treatment and how they should be managed.Case presentationWe describe a 32-year-old woman who was diagnosed with a ventricular septal defect, patent ductus arteriosus, and coarctation of the aorta, as well as severe PAH at birth. The attending physician at the time decided not to perform total repair. We re-evaluated the patient’s haemodynamics at 32 years of age. Her mean pulmonary artery pressure was 105 mmHg, pulmonary vascular resistance was 14.5 Wood units and pulmonary blood flow-to-systemic blood flow ratio (Qp/Qs) was 2.8. However, she had good vasoreactivity to oxygen. Vasoreactivity testing with 100% oxygen showed decreases in the following measures: the mean pulmonary artery pressure was 88 mmHg, pulmonary vascular resistance was 3.8 Wood units, and Qp/Qs was 10.8. We repaired the cases of coarctation of the aorta, patent ductus arteriosus and ventricular septal defect without the occurrence of a pulmonary hypertension crisis after surgery by using PAH-specific drugs. Furthermore, the continuous use of PAH-specific drugs improved the patient’s symptoms and the 6-min walk distance and decreased the mean pulmonary artery pressure and pulmonary vascular resistance. Her mean pulmonary artery pressure was 22 mmHg, and her pulmonary vascular resistance was 4.7 Wood units at the one-year follow-up.ConclusionsWith perioperative use of PAH-specific drugs, CHDs can be repaired without the occurrence of a pulmonary hypertension crisis after surgery in patients with a good vasoreactivity response to oxygen, even if the PVR is high. Medical treatment and surgery can therefore treat PAH and improve patients’ exercise capacity over time.