A new study has shown that patients with portopulmonary hypertension (PoPH)—a form of pulmonary arterial hypertension associated with liver cirrhosis—may successfully undergo liver transplantation when carefully evaluated and appropriately treated.
Portopulmonary hypertension is a condition in which elevated portal pressure due to liver cirrhosis is accompanied by a concurrent rise in pulmonary arterial pressure. During liver transplantation, massive fluid shifts can place a sudden, severe burden on the heart, carrying a high risk of fatal complications such as right ventricular failure. A mean pulmonary arterial pressure (mPAP) of 45 mmHg or higher is generally considered a contraindication to liver transplantation, and many patients in the borderline range of 35–45 mmHg have also forgone transplantation due to the associated risk.
A multi-institutional research team—including Professor Sung-A Chang and Fellow Yechan Kim of the Division of Cardiology at Samsung Medical Center, and Professors Hyung-Kwan Kim and Soon Gu Kwak of the Department of Cardiology at Seoul National University Hospital—analyzed 43 patients diagnosed with portopulmonary hypertension between 2009 and 2023. The team reported that with comprehensive evaluation and optimized treatment, the possibility of liver transplantation can be expanded even among patients with portopulmonary hypertension. The findings were published in a recent issue of the Korean Circulation Journal (IF=3.1), the official international journal of the Korean Society of Cardiology.
Among the 43 patients with portopulmonary hypertension analyzed, 9 underwent liver transplantation during the study period. Notably, 6 of these 9 patients had a mean pulmonary arterial pressure of 35–45 mmHg, placing them in the high-risk category for whom transplant eligibility had previously required extremely cautious judgment. In contrast, among 7 patients who needed a liver transplant but had it deferred due to pulmonary hypertension-related risk, 5 died within one year—a stark contrast to the 7 of 9 transplant recipients who survived.
The research team explained that these findings indicate that transplant eligibility in patients with portopulmonary hypertension should not be determined by pulmonary arterial pressure values alone, but rather through a comprehensive assessment that includes pulmonary vascular resistance, right ventricular function, response to pulmonary hypertension treatment, and the severity of liver disease. Indeed, in this study, mean pulmonary arterial pressure itself showed no significant association with mortality risk, whereas the severity of cirrhosis, as measured by the Child-Pugh score, was closely associated with prognosis. Each one-point increase in the Child-Pugh score was associated with a 1.53-fold increase in mortality risk.
The team also reported that in patients with portopulmonary hypertension, resolving the underlying cause—liver cirrhosis—through liver transplantation could also lead to improvement in pulmonary hypertension. Among patients who survived after liver transplantation, peak pulmonary artery systolic pressure decreased significantly from 58.4 mmHg before surgery to 38.6 mmHg after surgery. Of the 7 survivors, 4 experienced complete resolution of pulmonary hypertension, to the point that they were able to discontinue related medication.
The research team emphasized that in order to consider liver transplantation in patients with portopulmonary hypertension, it is necessary to carefully assess the severity of pulmonary hypertension and right ventricular function before surgery, and to jointly review the patient's response to pulmonary hypertension treatment along with peri-transplant management strategies. They noted that it is important for the divisions of cardiology, transplant surgery, and critical care medicine to collaborate in assessing patient-specific risk and establishing treatment and management plans, and to actively administer pulmonary arterial hypertension-targeted therapy before surgery to lower pulmonary arterial pressure as much as possible. They added that active postoperative management could resolve hemodynamic issues that arise after surgery.
Professor Hyung-Kwan Kim of the Department of Cardiology at Seoul National University Hospital said, "This study provides evidence that even borderline patients can safely undergo liver transplantation," adding, "The decision should not be based on pulmonary arterial pressure values alone."
Professor Sung-A Chang of the Division of Cardiology, who led the study, said, "Portopulmonary hypertension has long been regarded as a condition requiring cautious judgment in the liver transplantation process, but this study shows that liver transplantation may be a viable option for selected patients following appropriate treatment and thorough evaluation." She added, "For this to happen, multidisciplinary care involving cardiology, transplant surgery, and critical care medicine together is essential."