Pulmonary vascular disease & thromboembolism
Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 Single Nucleotide Polymorphism (SNPs) in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension
Authors' conclusion
Our data suggest that a change of cell identity (cFBs to cMFBs) in the RV is a driver of RV decompensation.
Study context
- Population
- Rat models of RV pressure overload and patients with pulmonary arterial hypertension (PAH) or group 2 pulmonary hypertension
- Participants
- 81 participants3 patient cohorts
- Comparison
- Monocrotaline (MCT) vs pulmonary artery banding (PAB) rat models; PAH vs group 2 pulmonary hypertension patients
Methodological details2 rat models, 3 patient cohorts
- 2 rat models
- 3 patient cohorts
Main finding
As reported in the abstract
- Significant increase in RV cardiac myofibroblasts (cMFBs) in MCT but not PAB RV (PAB decompensated much later)
- MCT RV contractility lower in decompensated RV (dRV) in isolated hearts but not in isolated cardiomyocytes (CMs), pointing to a non-CM cause
- Mitochondrial respiration lower in MCT dRV cMFBs than control and compensated right ventricle (cRV) cardiac fibroblasts (cFBs); increased in MCT-RV CMs
- mCa++ and c(M)FB (not CM) UCP2 progressively decreased from normal to MCT-cRV to dRV
- TNF-α decreased UCP2 mRNA/protein in RV cFBs but not RV CMs
- In human PAH, not pulmonary hypertension (PHT-2), dRVs had more cMFBs and less UCP2 than control and cRVs
- Decreased UCP2 and rs659366 associated with worse RV performance (TAPSE, cardiac index), even with similar mean pulmonary arterial pressure
Original publication
Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 SNPs in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension. Circulation. Published 9 March 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →