Disproportionate Right Ventricular Dysfunction and Poor Survival in Group 3 Pulmonary Hypertension

Prins, Kurt W.; Rose, Lauren; Archer, Stephen L.; Pritzker, Marc; Weir, Ε. Kenneth; Kazmirczak, Felipe; Misialek, Jeffrey R.; Thenappan, Thenappan

doi:10.1164/rccm.201712-2405le

Cited by 30 publications

(42 citation statements)

References 10 publications

(13 reference statements)

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“…While the significance of RV remodeling in IPAF patients remained to be determined, RV systolic dysfunction has been associated with increased risk of hospitalization and death in patients with ILDs. 23 The pathogenesis of PH in IPAF remains unknown. However, there may be overlapping mechanisms in CTDassociated PH and IPAF-associated PH as both entities have an autoimmune component.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

et al. 2020

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Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

et al. 2020

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“…20 Survival of subjects with presumed Group 2 and 3 PH was significantly worse than those with presumed PAH, a finding consistent with recent reports from other single centers. 5,8 Maron et al. identified veterans who had RHC, the gold standard diagnostic test for PH.…”

Section: Discussionmentioning

confidence: 99%

Mortality in US veterans with pulmonary hypertension: a retrospective analysis of survival by subtype and baseline factors

et al. 2019

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Pulmonary hypertension (PH) occurs when the pulmonary vasculature is itself diseased or becomes affected secondarily by comorbid conditions, commonly left heart or lung disease. The high prevalence of chronic cardiopulmonary conditions among patients served by Veterans Health Administration (VHA) suggests this population may be particularly susceptible to PH. We sought to identify clinical features and outcomes in veterans diagnosed with PH. We utilized the VHA Corporate Data Warehouse to identify veterans diagnosed between January 1, 2003 and September 30, 2015, assess relevant patient characteristics and their survival time. The effects of PH subtype and baseline factors on outcome were estimated by Cox modeling. There were 110,564 veterans diagnosed with PH during the study period. These veterans were predominantly male, had median age 70.2, and had a high burden of comorbid conditions. PH was frequently due to left heart and/or lung disease. Average survival after PH diagnosis was 3.88 years. Compared with other types, PH due to left heart disease, lung disease or both had shorter survival. This large retrospective study of veterans demonstrates the significance of PH due to left heart and/or lung disease which was common and had high risk of death. Multi-comorbidity was common and added to risk. These findings underscore the need for risk assessment tools for subjects with non-Group 1 PH and novel management strategies to improve their outcome. This study details the largest retrospective cohort assembled for evaluation of secondary PH and allows hypothesis-generating inquiries into these common conditions that are rarely prospectively studied.

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“…RV function predicts mortality ( 12 , 13 ) and is affected independent of afterload in Group 2 PH ( 14 ). Despite having less severe pulmonary vascular disease than patients with PAH, Group 3 PH patients have disproportionate RV dysfunction and poor survival ( 15 ). The presence of RV dysfunction in Group 3 PH also identifies patients at high risk of poor outcomes ( 11 , 16 ).…”

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confidence: 99%

Treatment Targets for Right Ventricular Dysfunction in Pulmonary Arterial Hypertension

Prisco

Thenappan

Prins

2020

JACC: Basic to Translational Science

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Disproportionate Right Ventricular Dysfunction and Poor Survival in Group 3 Pulmonary Hypertension

Cited by 30 publications

References 10 publications

Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

Mortality in US veterans with pulmonary hypertension: a retrospective analysis of survival by subtype and baseline factors

Treatment Targets for Right Ventricular Dysfunction in Pulmonary Arterial Hypertension

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