S5507 Schistosomiasis-Induced Cirrhosis With Multifactorial Pulmonary Hypertension: A Case Report

Introduction: Schistosomiasis, caused by parasitic blood fluke, afflicts about 200 million people globally, primarily in Africa. Though rare in the U.S., some cases arise, often in immigrants. Pulmonary arterial hypertension (PAH) is a known complication of hepatosplenic schistosomiasis. We present a case of liver schistosomiasis complicated by multifactorial PAH. Case Description/Methods: A 64-year-old Yemeni man with decompensated cirrhosis suspected to be from schistosomiasis, was repeatedly hospitalized for dyspnea. Initial right heart catheterizations (RHC) showed mean pulmonary artery pressure (mPAP) of 33 mmHG. Transjugular biopsy revealed hepatic venous pressure gradient (HVPG) 16 mmHg. Biopsy confirmed cirrhosis with scattered portal and lobule inflammation. Model for End-Stage Liver Disease 3.0 score was 18. He was evaluated for liver transplant, but did not qualify for MELD exception points. After starting macitentan-sildenafil, repeat RHC showed mPAP 50 mmHg, still ineligible for exception points. Despite diuresis and pulmonary vasodilators, he remained hypoxic. Echo with contrast and bubble study revealed an intrapulmonary shunt, diagnosing hepatopulmonary syndrome (HPS). A PAH specialist classified his condition as multifactorial: WHO Group 1 PAH due to portopulmonary hypertension (POPH), as well as WHO Group 2 PAH from heart failure from poorly controlled diabetes and hypertension. He is symptomatically managed with lactulose, beta blockers, and diuretics. He was referred to Transplant Infectious Disease for vaccinations in case of future eligibility. Discussion: This case illustrates a rare combination of PAH types: Group 1 POPH, and Group 2 heart failure. PAH affects 5-10% of those with hepatosplenic schistosomiasis, often caused by S. mansoni and S. japonicum. Schistosoma-associated PAH is a form POPH, occurring from eggs shunted into the pulmonary vasculature due to portal hypertension, creating obstruction and immune-driven vascular remodeling. Managing cirrhosis with POPH, in this case due to schistosomiasis, is challenging. Per guidelines, MELD exception requires mPAP between 35-45 mmHg post treatment. Initially, his mPAP was too low to qualify, crucial for transplant listing. Post treatment it exceeded the threshold and posed a surgical risk, excluding him from transplant eligibility. The case highlights the severe complications of schistosomiasis, the narrow eligibility window on POPH, and complexity in managing such multifactorial disease.