Journal of Pediatric Cardiology and Cardiac Surgery

The optimal treatment for unrepaired congenital heart disease in adults with pulmonary arterial hypertension (PAH) has not yet been clarified. We here report a case of a 42-year-old man who underwent successful surgical repair of isolated partial anomalous pulmonary venous connection (PAPVC) following target therapy for PAH. He presented with progressive exertional dyspnea. Enhanced computed tomography showed the right upper and middle pulmonary veins draining into the superior vena cava (SVC). Right heart catheterization showed that mean pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) were 91 mmHg and 20.3 Wood unit, respectively. He was subsequently treated with three specific PAH drugs (macitentan, tadalafil and selexipag) for 9 months. Follow-up catheterization showed that his mean PAP and PVR decreased to 45 mmHg and 2.7 Wood unit, respectively. Side effects of the PAH specific drugs, however, eventually caused poor medication adherence. As interruption of these drugs was likely to cause re-progression of PAH, the patient underwent surgical repair. The anomalous pulmonary veins were redirected into the left atrium through a surgically created atrial septal defect. The SVC was divided and reconstructed using a right atrial wall flap and an autologous pericardial patch. His exercise tolerance and PAH gradually improved thereafter. Catheter examination 15 months after surgery showed mean PAP was 34 mmHg. Specific target therapy for PAH could improve severe PAH related to isolated PAPVC in adults patients, and could make management of their circumstances easy and safe during the perioperative period.