Sotatercept therapy in a 67-year-old female with pulmonary arterial hypertension was associated with the development of a large pericardial effusion and subsequent bilateral pleural effusions.
Case Report (n=1)
Sotatercept therapy in pulmonary arterial hypertension may be associated with the development of significant pericardial and pleural effusions requiring drainage, mandating clinician vigilance.
Abstract Pulmonary arterial hypertension (PAH) is a progressive disease characterized by an obliterative arteriopathy in the medium to small pulmonary arteries, resulting in right ventricular (RV) dysfunction, failure, and eventually, death1. While vasodilators remain first-line therapies for patients with PAH, Sotatercept is a first-in-class Activin signaling inhibitor (ASI) that was recently approved for the treatment of PAH2. We present the evaluation and management of a case of pericardial effusion in a patient on Sotatercept. A 67-year-old female with PAH (due to systemic sclerosis), hypothyroidism, and former tobacco use, being treated with quadruple therapy (Ambrisentan, Sildenafil, Treprostinil, Sotatercept), presented to a routine appointment when her echocardiogram revealed an enlarging pericardial effusion and early signs of tamponade. A small effusion was noted earlier in the year after initiation of Sotatercept and was observed conservatively with serial examination. Given her overall improvement in her PAH by hemodynamics and right ventricular function, this large pericardial effusion was considered an ‘off-target’ effect of Sotatercept. She was admitted to the intensive care unit (ICU), and a Swan-Ganz catheter (SGC) was placed. The pericardial fluid was aspirated in a controlled fashion with aliquots of 100ccs at a time, while monitoring hemodynamic parameters from the SGC. After pericardial fluid was safely aspirated and the pericardial catheter removed, she developed bilateral pleural effusions (right side larger than the left). She underwent thoracentesis and right chest tube placement for drainage. An immediate echocardiogram revealed a small, residual effusion post-thoracentesis. Sotatercept, the newest PAH therapy and first in its class, acts as a ligand trap for activins and growth differentiation factors, balancing the pro-proliferative and antiproliferative signaling in the pulmonary vasculature. However, there have been several reports of pericardial effusions in patients on sotatercept3-6. Sahay et. al found that most effusions were identified on routine surveillance echocardiography; severity ranged from small, asymptomatic effusions to moderate/large effusions requiring drainage6. Furthermore, the TROPOS phase-2 trial of cibotercept, another ASI, was halted after similar findings, raising concerns for the safety of this drug class3. Additionally, the development of bilateral pleural effusions associated with sotatercept therapy in this patient underscores a novel and important area for investigation. Sotatercept is an important advancement in PAH therapy; however, emerging reports of pericardial effusions mandate clinician vigilance. Our case report adds to the growing literature identifying novel adverse effects of ASIs and highlights the need for continued surveillance so patients who benefit from Sotatercept can be treated safely. This abstract is funded by: None
Puri et al. (Fri,) conducted a case report in Pulmonary Arterial Hypertension (PAH) (n=1). Sotatercept was evaluated. Sotatercept therapy in a 67-year-old female with pulmonary arterial hypertension was associated with the development of a large pericardial effusion and subsequent bilateral pleural effusions.