Key result
Sotatercept escalation improves haemodynamics and CMR-derived ventricular function in severe pulmonary arterial hypertension.
Why the study?
Cardiovascular magnetic resonance-derived atrioventricular plane displacement and left ventricular pressure-volume loops may serve as non-invasive markers to track treatment response as novel remodelling therapies like sotatercept emerge for pulmonary arterial hypertension.
Case Report (n=1)
Serial cardiovascular magnetic resonance-derived atrioventricular plane displacement and left ventricular pressure-volume loops may serve as sensitive non-invasive biomarkers for tracking longitudinal treatment response to therapies including sotatercept in idiopathic pulmonary arterial hypertension.
May support CMR monitoring of Sotatercept response in PAH; hypothesis-generating and requires prospective validation.
Background Pulmonary arterial hypertension (PAH) causes progressive right ventricular (RV) pressure overload and left ventricular (LV) underfilling. Sotatercept is an activin-signal inhibitor, approved by FDA in 2024 and EMA in 2025, as an add-on treatment in adult patients with PAH. Cardiovascular magnetic resonance (CMR) derived atrioventricular plane displacement (AVPD) and left ventricular pressure-volume (PV) loops may represent non-invasive markers of treatment response, particularly important as novel therapies addressing remodelling emerge. Case summary A 30-year-old woman was diagnosed with idiopathic PAH (IPAH), after presenting with dyspnoea and peripheral oedema. Baseline CMR showed severe RV dilation, reduced RV ejection fraction, and low indexed LV stroke volume, with reduced bilateral AVPD and markedly impaired LV PV-loop indices, with low stroke work, low mean external power, and high ventriculo-arterial coupling. Initial dual PAH therapy, with an endothelin receptor antagonist (ERA) and a phosphodiesterase five inhibitor (PDE5i) initially improved symptoms, pulmonary vascular resistance, as well as AVPD and PV-loop derived indices, whereas conventional CMR indices showed only minimal change. One year later, despite triple therapy, including prostacyclin analogue, the patient deteriorated clinically and CMR showed worsening of AVPD and PV-loop indices. Treatment escalation with Sotatercept immediately improved clinical status, haemodynamic, AVPD, and PV-loop indices despite persistent RV remodelling. Discussion Serial CMR-derived AVPD and LV PV-loops may provide clinically useful additional information during longitudinal follow-up of PAH patients, especially in those approaching maximal available medical therapies. In the present case, both AVPD and LV PV-loops reflected treatment response, and showed significant improvement after initiation of Sotatercept.
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Castiglione et al. (2026) conducted a case report in Idiopathic pulmonary arterial hypertension (IPAH) (n=1). Sotatercept (added to triple PAH therapy: ERA, PDE5i, and prostacyclin analogue) tracked by serial CMR-derived AVPD and LV PV-loops was evaluated on Clinical status, haemodynamics, and CMR-derived atrioventricular plane displacement (AVPD) and left ventricular pressure-volume (PV) loop indices. Sotatercept escalation in a 30-year-old woman with IPAH after 1 year of background therapy immediately improved clinical status, haemodynamics, and CMR-derived AVPD and LV PV-loop indices.
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