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Original Research: CARDIOVASCULAR DISEASE |

Prevalence and Prognostic Value of Left Ventricular Diastolic Dysfunction in Idiopathic and Heritable Pulmonary Arterial HypertensionLeft Ventricular Diastolic Dysfunction

Adriano R. Tonelli, MD; Juan Carlos Plana, MD; Gustavo A. Heresi, MD; Raed A. Dweik, MD, FCCP
Author and Funding Information

From the Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute (Drs Tonelli, Heresi, and Dweik), and the Department of Cardiovascular Medicine, Heart and Vascular Institute (Dr Plana), Cleveland Clinic, Cleveland, OH.

Correspondence to: Raed Dweik, MD, FCCP, 9500 Euclid Ave A-90, Cleveland, OH, 44195; e-mail: dweikr@ccf.org


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

Funding/Support: This study was funded by CTSA KL2 [Grant RR024990] (salary support to Dr Tonelli) from the National Center for Research Resources, a component of the National Institutes of Health (NIH), and NIH Roadmap for Medical Research. Dr Dweik receives salary support from the NIH [Grants HL081064, HL107147, HL095181, and RR026231] and a Biomedical Research and Commercialization Program 08-049 Third Frontier Program grant from the Ohio Department of Development.


© 2012 American College of Chest Physicians


Chest. 2012;141(6):1457-1465. doi:10.1378/chest.11-1903
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Background:  Little is known about the association between left ventricular (LV) diastolic dysfunction and outcomes in patients with idiopathic or heritable pulmonary arterial hypertension (PAH). Our rationale was to investigate the prevalence of LV diastolic dysfunction, and its association with disease severity and outcomes, in patients with idiopathic or heritable PAH.

Methods:  Using the Cleveland Clinic Pulmonary Hypertension Registry, we identified subjects with heritable or idiopathic PAH who underwent Doppler echocardiography and right-sided heart catheterization. Echocardiographic diastolic parameters were assessed in each patient.

Results:  A total of 61 patients met the inclusion criteria (idiopathic 85%, heritable 15%). The age at the time of echocardiography was 48.3 ± 18 years, 84% of the subjects were women, and 48% were on PAH-targeted therapies. Normal LV diastolic function, impaired relaxation, and pseudonormalization were seen in 10%, 88%, and 2% of the patients, respectively. Peak early diastolic (peak E) velocity was directly associated with LV end-diastolic volume and cardiac index and inversely associated with the degree of right ventricular dilation, right atrial pressure, and pulmonary vascular resistance. Peak E velocity was associated with mortality adjusted for age and sex (hazard ratio [HR], 1.5; 95% CI, 1.1-2 per 10 cm/s decrease; P = .015) and age, sex, 6-min walk distance, and cardiac output (HR, 1.8; 95% CI, 1.2-2.9 per 10 cm/s decrease; P = .01).

Conclusions:  LV diastolic dysfunction of the impaired relaxation type is observed in the majority of patients with advanced idiopathic or heritable PAH. A decrease in transmitral flow peak E velocity is associated with worse hemodynamics and outcome.

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