Pulmonary vascular disease & thromboembolism
Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension
Authors' conclusion
A primary defect in human SSc-PAH resides in depressed sarcomere function, whereas this is enhanced in IPAH. These disparities correlate with in vivo RV contractility and contractile reserve and are consistent with worse clinical outcomes in SSc-PAH.
Study context
- Population
- Patients with SSc-PAH, idiopathic PAH (IPAH) or systemic sclerosis (SSc)-d (right ventricular (RV) septal endomyocardial biopsies); donor-heart controls6–7 per group
- Comparison
- RV myocyte function in SSc-PAH vs IPAH vs SSc-d vs control
- Outcome
- Isolated RV myocyte myofilament function
Main finding
As reported in the abstract
- Fmax vs control: higher in IPAH
- 28%
- Fmax vs control: lower in SSc-PAH
- 37%
- Fmax correlation with RV end-systolic elastance
- R2 =0.46, P=0.002
- Fmax correlation with change in end-systolic elastance with exercise
- R2 =0.49, P=0.008
- Fmax inverse relation with exercise-induced chamber dilation
- R2 =0.63, P<0.002
Original publication
Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension. Circulation. Published 19 January 2018. doi:10.
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