PAH-Associated Congenital Heart Disease Outcomes Predicted by Pulmonary Artery Compliance

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Decreased pulmonary vascular compliance predicts worse prognosis in patients with PAH-associated congenital heart disease.
Decreased pulmonary vascular compliance predicts worse prognosis in patients with PAH-associated congenital heart disease.

Pulmonary artery compliance (Cp) may be an independent predictor of survival and prognosis in patients with pulmonary artery hypertension (PAH) associated with congenital heart disease (APAH-CHD), according to a study published in the International Heart Journal

Researchers performed a prospective analysis of 175 patients in Beijing, China, who underwent a comprehensive clinical evaluation and were followed up in 6-month intervals. The primary end point of the study was cause of mortality. 

Study results showed overall predictors of survival were Cp (hazard ratio [HR], 1.359, P <.001), pulmonary artery resistance (HR, 0.972; P= .001), pulmonary blood flow (HR, 1.092; P =.001), heart rate (HR, 1.038; P =.028), and 6-minute walking distance (HR, 1.003; P =.037). In addition, after adjustment by bivariate analysis, it was found that Cp was an independent predictor, with Kaplan-Meier survival curves showing a worse prognosis in patients with Cp <1.04 mL/mm Hg.

Investigators concluded that Cp is an independent reflection of disease severity, with a decreased Cp associated with a poorer prognosis in patients with APAH-CHD. Cp and pulmonary artery resistance were found to be inversely correlated in patients with APAH-CHD.

Researchers also found it reasonable to suggest that “not just pulmonary vascular resistance, but pulmonary arterial stiffness influences the mortality and morbidity in patients with CHD and PAH.” Clinicians should consider the use of Cp to predict mortality and disease prognosis in patients with APAH-CHD.

Reference

Cheng, XL, Liu ZH, GU Q, et al.  Prognostic value of pulmonary artery compliance in patients with pulmonary arterial hypertension associated with adult congenital heart disease. Intl Heart J. 2017;58(5):731-738.

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