Sotatercept for Decompensated Pulmonary Arterial Hypertension Requiring VA ECMO: First Canadian ICU Experience
Jonathan Taylor, Eddy Fan, John Thenganatt, John Granton
https://doi.org/10.1002/pul2.70276
Abstract
Pulmonary arterial hypertension (PAH) is a rare and progressive disease resulting in increased workload of the right ventricle (RV). Despite advances in therapies, PAH remains highly morbid from a progressive vasculopathy and RV failure. For patients admitted to hospital with RV failure, transplant remains the only option for a select few. Sotatercept, a novel activin signaling inhibitor approved for the treatment of PAH, has demonstrated significant improvement in clinical outcomes across a wide spectrum of disease severity. However, descriptions of its use to treat acute RV failure in an intensive care unit (ICU) setting are limited. We report a case of cardiogenic shock and respiratory failure secondary to decompensated PAH requiring veno-arterial extracorporeal membrane oxygenation (VA ECMO), with rapid improvement following sotatercept initiation.
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