Abstract Introduction Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are pulmonary vascular syndromes with nearly opposite physiology despite sharing the same stimulus: portal hypertension. HPS, characterized by intrapulmonary vascular dilatation, intrapulmonary shunting, and orthodeoxia, occurs in 10-30% of adults with advanced liver disease. Liver transplantation remains the only definitive therapy. In contrast, POPH is a subtype of pulmonary arterial hypertension caused by imbalanced vasoactive mediators, leading to a vasoconstrictive, pro-proliferative state. POPH affects 5-6% of liver transplant candidates and is managed medically, with or without liver transplantation. Although rare, overlap between HPS and POPH can occur and remains underrecognized. This case highlights the clinical overlap of these conditions and a complicated post-transplant course. Case The patient was a 69-year-old female with hepatitis C-related cirrhosis complicated by hepatocellular carcinoma (status post embolization) followed in pulmonary hypertension clinic. She was diagnosed with POPH and mild HPS based on right heart catheterization (RHC), elevated hepatic vein gradient, and bubble echocardiography showing intrapulmonary shunt. She could not tolerate endothelin receptor antagonists (ERA) due to volume overload. POPH was managed medically with tadalafil 40 mg daily, IV treprostinil 40 ng/kg/min, and torsemide while awaiting transplant. She underwent orthotopic liver transplantation in 02/2025, complicated by wound infection. Postoperatively, examination revealed a loud P2, hyperdynamic precordium, no jugular venous distension, a 2/6 murmur at the left upper sternal border, and no peripheral edema. Her post-transplant course was complicated by severe gastrointestinal bleeding, acute-on-chronic hypoxemic respiratory failure, volume overload, acute kidney injury, and infections. Seven months post-transplant, echocardiography showed persistently elevated PA pressures and RVSP, although right ventricular size and function had improved. She continued to demonstrate signs of HPS with an intrapulmonary shunt but was not stable enough for repeat RHC. IV treprostinil, ERA, and continuous renal replacement therapy were continued for hemodynamic support. Her course was further complicated by renal failure, biliary stricture, and mixed cardiogenic and septic shock, ultimately resulting in multiorgan failure and death. Significance While both HPS and POPH significantly increase mortality in cirrhosis, HPS causes abnormal pulmonary vasodilation, while POPH involves vasoconstriction and vascular remodeling. HPS doubles mortality risk compared to cirrhotic patients without HPS, though liver transplantation often resolves HPS within 6-12 months. POPH is less reliably curative, with about 50% of patients able to discontinue PAH therapies. Unfortunately, our patient did not survive post-transplant complications; however, her clinical case elucidates the complex pathophysiology of opposing vascular forces in cirrhosis-related pulmonary disease. This abstract is funded by: None
Segura et al. (Fri,) studied this question.