Modern management of portopulmonary hypertension in children: case series of an expert center

Abstract

Background: Portopulmonary hypertension (PoPH) may develop in portal hypertension or portosystemic shunting and require liver transplantation (LT). This procedure is contraindicated in patients with severe hemodynamic impairment. We describe a serie of children with PoPH and their management. Methods: Diagnostic workup was performed within the current guidelines and PoPH was confirmed by right heart catheterization. Patient data was collected via chart review. Results: 4 patients were identified in our pediatric center since 2013. Mean age was 15.0 yo. The underlying liver conditions were cirrhosis of unknown origin, portocaval shunt, biliary atresia, and portal vein cavernoma. 3 patients had dyspnea and 1 had no cardiopulmonary symptom. Mean pulmonary artery pressure was 39.7mmHg and the indexed mean pulmonary vascular resistance (PVRi) was 10.0 WU. 2 patients, 1 in functionnal class (FC) III and 1 with no cardiopulmonary symptom, were initially treated by upfront bitherapy with an endothelin receptor antagonist (ERA) and a phosphodiesterase-5 inhibitor (PDE5i). Both showed hemodynamic improvement allowing LT. The 3rd patient was in FC IV and treated by triple combination therapy with ERA, PDE5i and intravenous prostacyclin. PoPH improved and the patient underwent LT. Prostacyclin was withdrawn 9 months after LT and PoPH is now controlled (FC I) under oral triple combination therapy. The 4th patient underwent a surgical mesocaval shunt. After 7 years, he presented in FC III with midly elevated PVRi. He was treated with an ERA treatment and LT was performed. Conclusion: Outcome of PoPH may be improved when managed at an expert center offering PH and LT multidisciplinary team. Further studies are needed to better define its prognosis and treatment