ArticleCirculation2018
Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis-Associated Versus Idiopathic Pulmonary Arterial Hypertension.
Article in Circulation, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 90 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
90 citing papers in PubMed, 1 synthesis or guideline pooled it, 140 citations in OpenAlex.
- Assessment of Right Ventricular Function in the Research Setting: Knowledge Gaps and Pathways Forward. An Official American Thoracic Society Research Statement.American journal of respiratory and critical care medicine · 2018Guideline
- The ALPHA phase 1 study: pulmonary ArteriaL hypertension treated with CardiosPHere-Derived allogeneic stem cells.EBioMedicine · 2024Trial
- Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study).Arthritis research & therapy · 2019Trial
- Vascular compliance phenotyping in pulmonary arterial hypertension (a PVDOMICS study).JHLT open · 2026Article
- Echocardiographic characterization of combined postcapillary and precapillary pulmonary hypertension in systemic sclerosis with preserved ejection fraction.Echo research and practice · 2026Article
- Severe obesity in human HFpEF alters contractile protein function and organization.Science (New York, N.Y.) · 2026Article
- Cardiac involvement in systemic sclerosis: mechanisms, manifestations and management.Rheumatology (Oxford, England) · 2026Review
- TAPSE/PASP combined with IVC can identify poor prognosis in PAH patients with low and intermediate risk.Clinical rheumatology · 2026Article
- Risk of ischaemic and non-ischaemic heart failure in patients with systemic sclerosis: a population-based study.Rheumatology (Oxford, England) · 2025Article
- Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2025Article
- Leveraging Artificial Intelligence for the Diagnosis of Systemic Sclerosis Associated Pulmonary Arterial Hypertension: Opportunities, Challenges, and Future Perspectives.Advances in respiratory medicine · 2025Review
- ATP directly modulates thick filament structure and function in porcine myocardium.Biophysical journal · 2025Article
- Lung Single-Cell Transcriptomics Reveal Diverging Pathobiology and Opportunities for Precision Targeting in Scleroderma-Associated Versus Idiopathic Pulmonary Arterial Hypertension.Circulation. Genomic and precision medicine · 2025Article
- Long-term prognostic value of native myocardial tissue relaxation parameters (T1, T2, and T1ρ) in patients with precapillary pulmonary hypertension.The international journal of cardiovascular imaging · 2025Article
- What can skinned cardiomyocytes teach us about heart disease? Response to letter to the editor: Challenges in assessing myoflament calcium sensitivity and contractile function in HFpEF by Christophe Maack and Vasco Sequeira.Journal of molecular and cellular cardiology · 2025Article
- A Word of Caution-Potential Limitations of Pulmonary Artery Pressure Monitoring in Detecting Congestion Caused by Right-Sided Heart Failure.Biomedicines · 2025Article
- Molecular Pathogenesis of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Narrative Review.Biomolecules · 2025Review
- The effect of hematopoietic stem cell transplantation on cardiac mechanics in systemic sclerosis.The international journal of cardiovascular imaging · 2025Article
- Mechanisms and treatment of pulmonary arterial hypertension.Nature reviews. Cardiology · 2025Review
- Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease.Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2025Article
30 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
14 authors at 3 institutions in 2 countries.
Funding
Abstract
backgroundPatients with systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility compared with IPAH. We tested whether this disparity involves underlying differences in myofilament function.
methodsCardiac myocytes were isolated from RV septal endomyocardial biopsies from patients with SSc-PAH, IPAH, or SSc with exertional dyspnea but no resting PAH (SSc-d); control RV septal tissue was obtained from nondiseased donor hearts (6-7 per group). Isolated myocyte passive length-tension and developed tension-calcium relationships were determined and correlated with in vivo RV function and reserve. RV septal fibrosis was also examined.
resultsMyocyte passive stiffness from length-tension relations was similarly increased in IPAH and SSc-PAH compared with control, although SSc-PAH biopsies had more interstitial fibrosis. More striking disparities were found between active force-calcium relations. Compared with controls, maximal calcium-activated force (F
conclusionsA 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. The existence of sarcomere disease before the development of resting PAH in patients with SSc-d suggests that earlier identification and intervention may prove useful.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.