Efficacy and long-term prognosis of liver transplantation for porto-pulmonary hypertension
Li-Na Wu1,2,3,4, Hao-Su Zhan1,2,3,4, Lin Wei1,3,4, Wei Qu1,3,4, Zhi-Gui Zeng1,3,4, Liang Zhang1,3,4,5, En-Hui He1,3,4,6, Zhi-Jun Zhu1,3,4, Li-Ying Sun1,2,3,4.
1Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 2Department of Critical Liver Diseases, Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 3Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 4Clinical Center for Pediatric Liver Transplantation, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 5Department of Anesthesia, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 6Department of Ultrasound, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China
Objective: Porto-pulmonary hypertension (PoPH) refers to pulmonary hypertension based on severe portal hypertension. Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for liver transplantation (LT). We analyze efficacy and long-term prognosis of liver transplantation for porto-pulmonary hypertension from our center.
Methods: A retrospective analysis was performed on 5 patients with PoPH who received liver transplantation in Beijing Friendship Hospital from Jan. 2013 to March 2022, in order to analyze postoperative follow-up results and long-term survival.
Results: Five patients with PoPH with a mean age of 35.6 ± 18.6 years (60% females), were included in the analysis. Their primary diseases were hepatic spongiosis, Caroli disease, hepatitis B cirrhosis, drug-induced cirrhosis and primary sclerosing cholangitis. All patients were measured by Swan-Ganz catheterization of right heart, among them, the mean of the highest mean pulmonary artery pressure(mPAP) level before LT were 46.6mmHg(27-58mmHg). The mean of their highest systolic pulmonary artery pressure(SPAP) level measured under echocardiography was 101.36mmHg(51-154mmHg) before LT. The median Model for End-Stage Liver Disease (MELD) score was 16(14-20) at PoPH diagnosis. All patients had been on pulmonary vasodilator therapy(including ambrisentan, remodulin and sildenafil) for more than six months to lower pulmonary artery pressure to mPAP<35mmHg, that can achieve surgical indications before LT. The operations went smoothly, except in one case where pulmonary artery pressure suddenly increased during surgery ended smoothly after the addition of remodulin. Two patients underwent a second liver transplant at 6 months and 65 months postoperatively, and one patient died of acute heart failure after the second transplantation, while the remaining four patients survived. The follow-up time of the survived patients were 29.7, 46.9, 56, 85.2 months. The longest follow-up among them was over seven years. SPAP of them decreased 15.4, 129, 65.3, 20mmHg after LT. Two patient was discontinued pulmonary arterial pressure medications within 1 year after surgery because SPAP were down to normal, and other patients still on medication.
Conclusion: Patients with pulmonary vasodilator therapy before LT can have excellent long-term outcomes post-transplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify patients for LT.
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