PURPOSE: Although there is a high prevalence of impaired LV relaxation (diastolic dysfunction grade I) in patients with pulmonary arterial hypertension (PAH), little is known about its association with severity of disease 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 had Doppler echocardiography and RHC. Echocardiographic recordings were reviewed and diastolic parameters 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 ± 18 years, 84% of the subjects were women and 48% were on PAH-targeted therapies. Normal diastolic function, impaired relaxation and pseudonormal pattern were seen in 10%, 88% and 2% of the patients, respectively. Peak E velocity (early diastolic velocity) and E/A ratio (early / late diastolic velocity) directly correlated with left ventricular end-diastolic volume. Peak E velocity was directly associated with LV end-diastolic volume and cardiac index, and indirectly associated with the degree of RV dilation, RA pressure and pulmonary vascular resistance. Peak E velocity was inversely associated with mortality when adjusted by age and gender (HR: 0.96 (95%CI: 0.93-0.99), p=0.03) or mean PAP, RA pressure and CI (HR: 0.08 (95%CI: 0.01-0.77), p=0.03).
CONCLUSIONS: 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.
CLINICAL IMPLICATIONS: The evaluation of diastolic function by echocardiography can provide additional prognostic information in idiopathic and heritable PAH.
DISCLOSURE: The following authors have nothing to disclose: Adriano Tonelli, Juan Plana, Gustavo Heresi, Raed Dweik
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