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In rodent models of pulmonary hypertension (PH) and right ventricular hypertrophy (RVH), the QTc interval is prolonged, reflecting downregulation of repolarizing Kv channels in RV myocytes. The significance of QTc prolongation in human PH is unknown. We hypothesized that QTc prolongation occurs in human PH, is associated with RVH and decreased RV function, and predicts adverse prognosis.

Patients receiving a PAH-specific therapy (a prostanoid, endothelin-receptor antagonist and/or a phosphodiesterase-5 inhibitor), who had a 12-lead electrocardiogram (ECG) (n = 202) were compared to age- and sex-matched controls (n = 100). The duration of QTc on ECG was correlated with invasive hemodynamics (n = 156) and with the status of the RV, as measured by Brain Natriuretic Peptide (NT-proBNP, n = 145) and magnetic resonance imaging (n = 24). Survival of the entire PH cohort and …