A 2-Decade Cardiac Magnetic Resonance Imaging Journey in Heritable Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive disease that leads to right heart failure. We report on the long-term follow-up of a male patient diagnosed at the age of 26 years with heritable PAH. Over 2 decades, he underwent several targeted therapies and repeated multimodal assessments, including cardiac magnetic resonance imaging (CMR). Despite a gradual clinical deterioration with right ventricular (RV) decompensation and an increase in pulmonary arterial pressure, the administration of sotatercept over 6 months led to a complete reversal of disease progression in terms of RV function and clinical status. This case demonstrates the evolving therapeutic landscape in PAH and the central role of CMR in the detailed assessment of RV function. Modern drugs can significantly modify the course of PAH. Regular CMR examinations allow detailed monitoring of the disease course in PAH. Sotatercept can achieve impressive benefits even in late-stage PAH.