ArticleChest2023
Evidence of Advanced Pulmonary Vascular Remodeling in Obstructive Hypertrophic Cardiomyopathy With Pulmonary Hypertension.
Article in Chest, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 22 citations in OpenAlex.
- Ambient PMEnvironment & health (Washington, D.C.) · 2026Article
- Clinical Course and Predictors of Heart Failure in Asymptomatic Obstructive Hypertrophic Cardiomyopathy.Journal of the American Heart Association · 2026Article
- Pulmonary Venous Vasculopathy in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Reduced Diffusion Capacity for Carbon Monoxide.Pulmonary circulation · 2026Article
- HYAL3 as a therapeutic target for pulmonary arterial Hypertension-Cardiomyopathy comorbidity: an integrative analysis combined with machine learning and SHAP value interpretation.Frontiers in pharmacology · 2026Article
- Long-Term Real-World Outcomes of Mavacamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy up to 108 Weeks.Journal of clinical medicine · 2025Article
- Sex differences in the prevalence and survival of pulmonary hypertension in obstructive hypertrophic cardiomyopathy following septal myectomy.International journal of cardiology. Heart & vasculature · 2025Article
- Therapeutic challenges and new therapeutic targets for combined capillary pulmonary hypertension: a review.Frontiers in medicine · 2025Review
- Pulmonary hypertension associated with left heart disease.The European respiratory journal · 2024Review
- Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications.Journal of cardiovascular development and disease · 2024Review
- CD8 T cells promote heart failure progression in mice with preexisting left ventricular dysfunction.Frontiers in immunology · 2024Article
- Pulmonary Hypertension: A Contemporary Review.American journal of respiratory and critical care medicine · 2023Review
- Revised Definition of Pulmonary Hypertension and Approach to Management: A Clinical Primer.Journal of the American Heart Association · 2023Review
Corrections and comments
- Erratum issued
Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundElevated mean pulmonary artery pressure (mPAP) is common in patients with hypertrophic cardiomyopathy (HCM) and heart failure symptoms. However, dynamic left ventricular (LV) outflow tract obstruction may confound interpretation of pulmonary hypertension (PH) pathophysiologic features in HCM when relying on resting invasive hemodynamic data alone. RESEARCH QUESTION: Do structural changes to the lung vasculature clarify PH pathophysiologic features in patients with HCM with progressive heart failure? STUDY DESIGN AND
methodsClinical data and ultrarare lung autopsy specimens were acquired retrospectively from the National Institutes of Health (1975-1992). Patients were included based on the availability of lung tissue and recorded mPAP. Discarded tissue from rejected lung donors served as control specimens. Histomorphology was performed on pulmonary arterioles and veins. Comparisons were calculated using the Student t test and Mann-Whitney U test; Pearson correlation was used to assess association between morphometric measurements and HCM cardiac and hemodynamic measurements.
resultsThe HCM cohort (n = 7; mean ± SD age, 43 ± 18 years; 71% men) showed maximum mean ± SD LV wall thickness of 25 ± 2.8 mm, mean ± SD outflow tract gradient of 90 ± 30 mm Hg, median mPAP of 25 mm Hg (interquartile range [IQR], 6 mm Hg), median pulmonary artery wedge pressure (PAWP) of 16 mm Hg (IQR, 4 mm Hg), and median pulmonary vascular resistance of 1.8 Wood units (WU; IQR, 2.4 WU). Compared with control samples (n = 5), patients with HCM showed greater indexed pulmonary arterial hypertrophy (20.7 ± 7.2% vs 49.7 ± 12%; P < .001) and arterial wall fibrosis (11.5 ± 3.4 mm vs 21.0 ± 4.7 mm; P < .0001), which correlated with mPAP (r = 0.84; P = .018), PAWP (r = 0.74; P = .05), and LV outflow tract gradient (r = 0.78; P = .035). Compared with control samples, pulmonary vein thickness was increased by 2.9-fold (P = .008) in the HCM group, which correlated with mPAP (r = 0.81; P = .03) and LV outflow tract gradient (r = 0.83; P = .02).
interpretationTo the best of our knowledge, these data demonstrate for the first time that in patients with obstructive HCM, heart failure is associated with pathogenic pulmonary vascular remodeling even when mPAP is elevated only mildly. These observations clarify PH pathophysiologic features in HCM, with future implications for clinical strategies that mitigate outflow tract obstruction.
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