Miscellaneous
Survival of Japanese Patients With Idiopathic/Heritable Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.amjcard.2017.01.015Get rights and content

Idiopathic/heritable pulmonary arterial hypertension has a poor prognosis despite the available therapeutic options. Survival of Japanese patients with this disease entity has not been reported in the multicenter setting. A retrospective study of 141 patients with idiopathic/heritable pulmonary arterial hypertension treated at 3 pulmonary hypertension centers in Japan from 1992 to 2012 investigated survival and determinants of survival. Mean survival time from treatment initiation was 14.7 ± 0.8 years (95% confidence interval, 13.1 to 16.3 years) and the 1-, 3-, 5-, and 10-year survival rates were 97.9%, 92.1%, 85.8%, and 69.5%, respectively. Patients showed significant improvement in exercise capacity and hemodynamics after treatment. Patients with 6-minute walk distance >372 m, mean pulmonary arterial pressure ≤46 mm Hg, and cardiac index >2.5 L/min/m2 at follow-up had a significantly better prognosis. Most patients (99.2%) were receiving pulmonary hypertension-targeted drugs at follow-up. Use of endothelin receptor antagonists and intravenous epoprostenol were related to survival in the univariate analysis. Among the patients who were on intravenous epoprostenol therapy, those with endothelin receptor antagonists had a significantly better prognosis, whereas patients on warfarin had a significantly worse prognosis. In conclusion, survival of Japanese patients with idiopathic/heritable pulmonary arterial hypertension in this study was good, showing improvement in hemodynamic parameters supported by pulmonary hypertension-targeted drugs.

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Methods

We conducted a retrospective chart review of patients with I/HPAH. Patients were treated at 3 pulmonary hypertension centers in Japan (National Hospital Organization Okayama Medical Center, Kyorin University Hospital, and Keio University Hospital) between November 1992 and August 2012. Diagnosis was performed using a standard approach for the diagnosis of PAH including physical examination and right heart catheterization.7, 8 The study protocol was approved by the institutional review board of

Results

We conducted a retrospective chart review of 141 consecutive patients with I/HPAH. Patients' characteristics are listed in Table 1. Patients were predominantly women and in their 30s at diagnosis. At baseline, 86.5% of patients were in WHO functional class III or IV. Hemodynamic parameters were severely impaired, with mPAP >60 mm Hg and PVR >1,500 dyn⋅s/cm5.

Data of 130 patients who underwent follow-up right heart catheterization were collected. At follow-up, WHO functional class, 6MWD, and BNP

Discussion

This is the first multicenter report on survival of Japanese patients with I/HPAH treated at 3 referral centers. The results confirmed our previous report on the high survival rate of Japanese patients with I/HPAH and significant improvement in hemodynamic parameters after treatment.6 In patients receiving epoprostenol therapy, concomitant use of ERA was beneficial and warfarin was related to worse survival.

Survival of patients with PAH improved after progress in the use of PAH-targeted drugs,

References (26)

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