Abstract Introduction Sotatercept was FDA-approved for Group 1 pulmonary arterial hypertension (PAH) in spring 2024, but patients with portopulmonary hypertension (POPH) were not included in major clinical trials that led to its approval. Therefore, safety and efficacy of sotatercept is not well-understood among patients with POPH. We present a case of severe POPH treated with sotatercept. Case Report A 61 year-old female presented to pulmonary hypertension (PH) clinic for follow-up of POPH. History notable for cirrhosis in setting of Hepatis C virus and portosystemic shunts status post embolization. She was on triple PAH therapy with sildenafil, macitentan, and IV epoprostenol. She had functional class 2 dyspnea on exertion, and transthoracic echocardiogram (TTE) demonstrated mild-moderate RV enlargement and mild dysfunction, with estimated right ventricular systolic pressure (RVSP) 76 mmHg. Right heart catheterization (RHC) revealed mean pulmonary arterial pressure (MPAP) 46 mmHg, pulmonary vascular resistance (PVR) 7.9 wood units (WU), cardiac output (CO) 4.9 L/minute, and low biventricular filling pressures. She was started on sotatercept, with goal of improving hemodynamics, RV function, and ultimately qualifying for POPH Model for End-Stage Liver Disease (MELD) exception. She returned for assessment several months later and reported progressive dyspnea with decreasing oxygen saturations. Arterial blood gas (ABG) revealed decreased PaO2 (56 mmHg, compared with 64 mmHg approximately 3 months prior before sotatercept initiation). She was noted to have cutaneous telangiectasias on face, chest, upper extremities, and back (Figure 1). TTE showed decreased RV size and improved function and RVSP (62 mmHg) but large intrapulmonary shunt per agitated saline (“bubble”) study. RHC demonstrated improvement in hemodynamics but remained inadequate to meet POPH MELD exception criteria (MPAP 36 mmHg, PVR 4.6 WU). Sotatercept was discontinued. Follow-up ABG demonstrated increase in PaO2 (63 mmHg), similar to prior baseline, and TTE approximately 6 weeks after stopping sotatercept demonstrated notable decrease in intrapulmonary shunting, now described as small. Telangiectasias improved as well. Discussion The use of sotatercept in patient with POPH and/or liver disease remains an exciting area of investigation, and additional clinical studies are warranted. Potential sequelae such as increased intrapulmonary shunting may particularly affect this patient population, given their unique and complex multiorgan pathology. In current clinical practice, close monitoring is essential when using sotatercept among patients with POPH. This abstract is funded by: n/a
Valle et al. (Fri,) studied this question.