Shirouzu Yasumasa, Kasahara Mureo, Takada Yasutsugu, Taira Kaoru, Sakamoto Seisuke, Uryuhara Kenji, Ogawa Kohei, Doi Hiraku, Egawa Hiroto, Tanaka Koichi
Department of Transplant Surgery, Kyoto University Hospital, Kyoto, Japan.
Liver Transpl. 2006 May;12(5):870-5. doi: 10.1002/lt.20758.
The development of portopulmonary hypertension (PH) in a patient with end-stage liver disease is related to high cardiac output and hyperdynamic circulation. However, PH following liver transplantation is not fully understood. Of 617 pediatric patients receiving transplants between June 1990 and March 2004, 5 (median age 12 yr, median weight 24.5 kg) were revealed to have portopulmonary hypertension (PH) after living-donor liver transplantation (LDLT), as confirmed by echocardiography and/or right heart catheterization. All children underwent LDLT for post-Kasai biliary atresia. In 2 patients with refractory biliary complications, PH developed following portal thrombosis; 2 with stable graft function, who had had intrapulmonary shunting (IPS) before LDLT, were found to have PH in spite of overcoming liver dysfunction due to hepatitis. PH developed shortly after distal splenorenal shunting in 1 patient, who suffered liver cirrhosis due to an intractable outflow blockage. The onset of PH ranged from 2.8 to 11 yr after LDLT, and mean pulmonary artery pressure (mPAP) estimated by echocardiography at the time of presentation ranged from 43 to 120 mmHg. Three of the 5 patients are alive under prostaglandin I2 (PGI2) treatment. Of these, 1 is prepared for retransplantation for an intractable complications of liver allograft, while the other 2 with satisfactory grafts are being considered for lung transplantation. Even after LDLT, PH can develop with portal hypertension. Periodic echocardiography is essential for early detection and treatment of PH especially in the recipients with portal hypertension not only preoperatively but also postoperatively.
终末期肝病患者发生门脉性肺动脉高压（PH）与高心输出量和高动力循环有关。然而，肝移植后发生的PH尚未完全明确。在1990年6月至2004年3月接受移植的617例儿科患者中，有5例（中位年龄12岁，中位体重24.5 kg）在活体肝移植（LDLT）后经超声心动图和/或右心导管检查确诊为门脉性肺动脉高压（PH）。所有儿童均因Kasai术后胆道闭锁接受LDLT。2例患有难治性胆道并发症的患者在门静脉血栓形成后发生PH；2例移植功能稳定、LDLT前存在肺内分流（IPS）的患者，尽管克服了肝炎所致的肝功能障碍，但仍被发现患有PH。1例因顽固性流出道梗阻导致肝硬化的患者在远端脾肾分流术后不久发生PH。PH的发病时间在LDLT后2.8至11年之间，就诊时经超声心动图估计的平均肺动脉压（mPAP）在43至120 mmHg之间。5例患者中有3例在接受前列腺素I2（PGI2）治疗下存活。其中，1例因肝移植的难治性并发症准备再次移植，而另外2例移植情况良好的患者正在考虑进行肺移植。即使在LDLT后，PH仍可因门静脉高压而发生。定期进行超声心动图检查对于早期发现和治疗PH至关重要，尤其是对于不仅术前而且术后都存在门静脉高压的受者。
