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MANAGEMENT OF BILIARY ATRESIA COMPLICATED WITH PORTOPULMONARY HYPERTENSION TOWARD LIVER TRANSPLANTATION

Kato H, Kawarasaki H, Yanagisawa M, Igarashi T

¹The University of Tokyo Hospital, Tokyo, Japan

Background and Objective: Portopulmonary hy pertension ((PPHTN) is considered to a contraindication for liver transplantation (LT), especially when pulmonary arterial systolic pressure (sPAP) is in the moderate to severe range (moderate, 35-45 mmHg, severe, >45 mmHg). Additionally, there is still controversy as to the reversibility of PPHTN after LT. Our object is to examine the efficacy of epoprostenol infusion therapy (EPO) for PPHTN and verify the management for biliary atresia (BA) with complicated PPHTN through our 2BA cases.

Cases: Case l; 14 years-old-boy. Main PAP was 48/22 (mean:32) mmHg Pulmonary vascular resistance (PVR) was 6.3 WU·m² Because PAP was deteriorated even with 60μg/day of Beraprost (PGI₂ derivative) for 6 months, we performed living-related LT (LRLT) from his mother. 3 months after LRLT, PAP and PVR had decreased to 36/20(26)and 4.9, alternatively. ECG showed reducement of RV overloading Case 2; 12 years-old-girl. PAP: 105/64(82), PVR:24.9: cardiac index (CI): 2.6 L/min/m². We administered 35ng/kg/min of EPO over 6 weeks. After that, PAP, PVR and CI changed to 84/45(58), 8.9, and 5.5, alternatively. Although BNP was decreased to normal value, we decided that this case was not adequate for LRLT. EPO was ceased over 6 weeks.

Conclusion: 1. Moderate PPHIN might be reversible after LT. 2. Effect of Beraprost per os is questionable. 3, EPO can improve PPHTN even in severe type, but do not guarantee LT. 4, We should start to use EPO in moderate PPHTN as a bridging therapy to LT. 5, EPO can be ceased safely.