Treating Portopulmonary Hypertension with Macitentan: Smoking Gun or Magic bullet?
Portopulmonary hypertension (PPHTN) is detected in about 5% of patients being evaluated for liver transplantation (LT). PPHTN is associated with a 5-year survival of only 45% in patients receiving medical therapy only. It is crucial to successfully bridge patients to LT by improving pulmonary hemodynamics, increase the number of patients eligible for LT model for end-stage liver disease (MELD) exception points and avoid waitlist and perioperative mortality. Although there is small observational and retrospective evidence that LT can be safely accomplished with careful patient selection and medical therapy directed at PPHTN, there has not been a lack of randomized and controlled data.
School of Medicine