Marked clinical and haemodynamic improvement with sotatercept in severe refractory pulmonary arterial hypertension associated with corrected complex congenital heart disease - PubMed
6 hours ago
- #Pulmonary arterial hypertension
- #Sotatercept
- #Congenital heart disease
- Sotatercept, an activin signaling inhibitor, showed significant clinical and hemodynamic benefits in a patient with severe refractory pulmonary arterial hypertension (PAH) associated with corrected complex congenital heart disease (PAH-CHD).
- A 37-year-old woman with repaired pulmonary atresia and ventricular septal defect developed severe PAH despite treatment with tadalafil, ambrisentan, and high-dose treprostinil.
- After initiating sotatercept, the patient improved from WHO-FC III to II, with NT-proBNP levels dropping from 3773 pg/mL to 230 pg/mL and 6MWD increasing from 480 m to 540 m.
- Right heart catheterization revealed a reduction in mean pulmonary artery pressure (48 to 26 mmHg), PVR (14 to 5.4 Wood units), and an increase in cardiac index (1.17 to 2.91 L/min/m²).
- Sotatercept may serve as an effective rescue therapy for severe PAH-CHD patients unresponsive to maximal conventional treatment.