989-09 - WHEN THE LUNGS BREAK THE RULES: A RARE CASE OF ESOPHAGEAL VARICEAL BLEED IN NON-CIRRHOTIC PORTAL HYPERTENSION DRIVEN BY PULMONARY ARTERIAL HYPERTENSION

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Author Block: Ajay Kerai, Yusuf Chang, Lauren East, Prinka Perswani, Jose Antonio Tallaj, Joanna M. Joly, University of Alabama at Birmingham, Birmingham, AL, USA
Background: Portopulmonary hypertension is a subset of pulmonary arterial hypertension (PAH) that is secondary to portal hypertension, usually linked to cirrhosis. PAH alone rarely causes portal hypertension or esophageal varices. We report a rare case of severe PAH and acute on chronic cor pulmonale leading to non-cirrhotic portal hypertension and hematemesis requiring esophageal variceal banding.
Case: A previously healthy 38-year-old female with 10 pack year smoking history presented with acute hematemesis, melena and progressive worsening dyspnea and volume overload. She was hemodynamically stable, with a hemoglobin of 6.8 and lactic acidosis. Esophagogastroduodenoscopy revealed actively bleeding esophageal varices, requiring four bands. Imaging ruled out pulmonary embolism, parenchymal lung disease, cirrhosis, and confirmed patent portal veins. Transthoracic echocardiography showed a normal left ventricle, severely dilated right ventricle with severely reduced function, severe tricuspid regurgitation, and a grade II intra-pulmonary shunt. She experienced a near-PEA arrest event with bradycardia and syncope shortly after arriving to the hospital. Right heart catheterization confirmed severe PAH. Extensive rheumatologic workup, hypercoagulability testing, and testing for common variable immunodeficiency, were all negative.
Decision‐making: Due to concern for acute on chronic cor pulmonale secondary to severe PAH, the patient was initiated on dobutamine for right ventricular support and underwent aggressive diuresis. Pulmonary vasodilator therapy was initiated with inhaled epoprostenol, which was later transitioned to intravenous epoprostenol, anticipating longterm management of PAH. Vasopressors were required initially for hypotension, which limited the use of additional pulmonary vasodilators. This treatment regime lead to significant symptomatic improvement.
Conclusion: Severe PAH can cause non-cirrhotic portal hypertension, leading to esophageal variceal bleeding. Treating PAH and managing cor pulmonale are essential to prevent further complications like secondary cirrhosis and recurrent varices.