JCK E-Oral Presentation 1 (II-JCKEOP01)

[II-JCKEOP01-04] Improvement of portopulmonary hypertension after liver transplantation

^○Shiro Baba¹, Daisuke Yoshinaga¹, Kouichi Matsuda¹, Kentaro Akagi¹, Shinji Uemoto², Takuya Hirata¹ (1.Department of Pediatrics, Kyoto University, Japan 2.Division Hepato-Billary-Pancreatic Surgery and Transplantation Department, Kyoto University, Japan)

Portopulmonary hypertension (POPH), associated with hepato-biliary diseases, is believed to improve after treatments of these liver diseases. However, not all patients improve their POPH even after the treatments. Thus, we retrospectively investigated 19 POPH patients (male: 8, female 11) in Kyoto university hospital whether their POPH improved after liver transplantation (LTx).
Diagnoses of these patients were biliary atresia (16), portosystemic shunt (2) and fulminant hepatitis (1). Accompanied diagnoses were tetralogy of Fallot (1), atrial septal defect (1), Alagille syndrome (1), Byler disease (1) and left isomerism (1). LTx was performed at the age of 4 months to 21 years old to all the patients, average was 8.6 years old. Mean pulmonary artery pressure (mPAp) and pulmonary artery resistance (Rp) at the timing of before LTx, 5.8±4.0 months and 6.5±4.9 years after LTx were 36.2±7.3, 30.3±7.5, 31.2±9.4mmHg、4.2±2.2, 5.4±2.6, 5.4±3.6U・m2, respectively. In these, only 5 patients’ mPAp normalized, <25mmHg, after LTx. Previously, it is reported that reduction rate of mPAp of portosystemic shunt patients was 24.7±17.5% in 3 months after LTx. Comparing with this splendid results, mPAp of our patients reduced only around 15% after LTx and still most of the patients use anti-pulmonary hypertension drugs.
It is said that POPH improves after treatment of primary hepato-biliary diseases. But there are many patients whose mPAp still high even after radical treatments including LTx. For these patients, we have to pay more attention to re-worsening of POPH.