Role of Left Ventricular Dysfunction in Systemic Sclerosis-Related Pulmonary Hypertension.

Autor:	Lui JK; Pulmonary Center, Boston University Chobanian & Avedisian School of Medicine, Boston, MA. Electronic address: justin.lui@bmc.org., Cozzolino M; Section of Cardiovascular Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA., Winburn M; Section of Cardiovascular Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA., Trojanowski MA; Section of Rheumatology, Boston University Chobanian & Avedisian School of Medicine, Boston, MA., Wiener RS; Pulmonary Center, Boston University Chobanian & Avedisian School of Medicine, Boston, MA; Center for Healthcare Organization & Implementation Research, VA Boston Healthcare System, Boston, MA., LaValley MP; Section of Rheumatology, Boston University Chobanian & Avedisian School of Medicine, Boston, MA; Department of Biostatistics, Boston University School of Public Health, Boston, MA., Bujor AM; Section of Rheumatology, Boston University Chobanian & Avedisian School of Medicine, Boston, MA., Gopal DM; Section of Cardiovascular Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA., Klings ES; Pulmonary Center, Boston University Chobanian & Avedisian School of Medicine, Boston, MA.
Jazyk:	angličtina
Zdroj:	Chest [Chest] 2024 Jun; Vol. 165 (6), pp. 1505-1517. Date of Electronic Publication: 2023 Dec 19.
DOI:	10.1016/j.chest.2023.12.018
Abstrakt:	Background: In systemic sclerosis (SSc), pulmonary hypertension remains a significant cause of morbidity and mortality. Although conventionally classified as group 1 pulmonary arterial hypertension, systemic sclerosis-related pulmonary hypertension (SSc-PH) is a heterogeneous disease. The contribution of left-sided cardiac disease in SSc-PH remains poorly understood. Research Question: How often does left ventricular (LV) dysfunction occur in SSc among patients undergoing right heart catheterization and how does coexistent LV dysfunction with SSc-PH affect all-cause mortality in this patient population? Study Design and Methods: We conducted a retrospective, observational study of 165 patients with SSc who underwent both echocardiography and right heart catheterization. LV dysfunction was identified using LV global longitudinal strain (GLS) on speckle-tracking echocardiography based on a defined threshold of > -18%. SSc-PH was defined by a mean pulmonary artery pressure > 20 mmHg. Results: Among patients with SSc who have undergone right heart catheterization, LV dysfunction occurred in 74.2% with SSc-PH and 51.2% without SSc-PH. The median survival of patients with SSc-PH and LV dysfunction was 67.9 (95% CI, 38.3-102.0) months, with a hazard ratio of 12.64 (95% CI, 1.73-92.60) for all-cause mortality when adjusted for age, sex, SSc disease duration, and FVC compared with patients with SSc without pulmonary hypertension with normal LV function. Interpretation: LV dysfunction is common in SSc-PH. Patients with SSc-PH and LV dysfunction by LV GLS have increased all-cause mortality. This suggests that LV GLS may be helpful in identifying underlying LV dysfunction and in risk assessment of patients with SSc-PH. Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: J. K. L. receives research support from United Therapeutics and served as a member of the Early Career Editorial Board Member program for the pulmonary vascular section of CHEST. E. S. K. received research support from Bayer, Novartis, Novo Nordisk, and United Therapeutics; received royalties for three topic cards in UpToDate; and is a consultant/advisory board member for Bluebird Bio, Novo Nordisk, Vertex, Pfizer, and CSL Behring for sickle cell disease-related clinical trials (no conflict with the present work). None declared (M. C., M. W., M. A. T., R. S. W., M. P. L., A. M. B., D. M. G.). (Copyright © 2023 American College of Chest Physicians. All rights reserved.)
Databáze:	MEDLINE
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