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Updated: Sep 12, 2025

Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension
Published on: February 11, 2017
A 2-Decade Cardiac Magnetic Resonance Imaging Journey in Heritable Pulmonary Arterial Hypertension: Reversal With
Bruno R Thal1, Nils C Kremer2, Sebastian Harth3
1Department of Internal Medicine, Justus-Liebig-University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Cardio-Pulmonary Institute (CPI), Institute for Lung Health (ILH), Giessen, Germany. Electronic address: https://twitter.com/BrunoRaphaeldD.
Background:
Pulmonary arterial hypertension (PAH) is a progressive disease that leads to right heart failure.
Case Summary:
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.
Discussion:
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.
Take-Home Message:
Regular CMR examinations allow detailed monitoring of the disease course in PAH. Sotatercept can achieve impressive benefits even in late-stage PAH.
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