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Clinical Investigations: PULMONARY HYPERTENSION |

Effect of Orally Active Prostacyclin Analogue on Survival in Patients With Chronic Thromboembolic Pulmonary Hypertension Without Major Vessel Obstruction*

Fumiaki Ono; Noritoshi Nagaya; Hiroyuki Okumura; Yoshito Shimizu; Shingo Kyotani; Norifumi Nakanishi; Kunio Miyatake
Author and Funding Information

*From the Department of Internal Medicine (Drs. Ono, Nagaya, Okumura, Kyotani, Nakanishi, and Miyatake), National Cardiovascular Center, Osaka; and Second Department of Internal Medicine (Dr. Shimizu), Hiroshima University, Hiroshima, Japan.

Correspondence to: Noritoshi Nagaya, MD, Department of Internal Medicine, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan; e-mail: nagayann@hsp.ncvc.go.jp



Chest. 2003;123(5):1583-1588. doi:10.1378/chest.123.5.1583
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Objectives: This study investigated whether treatment with beraprost sodium (BPS), an orally active prostacyclin analog, improves hemodynamics and survival in patients with peripheral-vessel chronic thromboembolic pulmonary hypertension (CTEPH), for which there is no surgical option.

Background: Oral administration of BPS has been shown to improve the hemodynamics and prognosis in patients with primary pulmonary hypertension; however, whether BPS has beneficial effects in CTEPH remains unknown.

Methods: Forty-three patients with peripheral-vessel CTEPH were classified into two groups: patients treated with BPS (BPS group, n = 20) and those without BPS (conventional group, n = 23). Baseline demographic and hemodynamic data did not significantly differ between the two groups.

Results: BPS therapy improved New York Heart Association functional class in 10 patients (50%) and significantly decreased total pulmonary resistance from 18 ± 6 to 15 ± 8 Wood units (p < 0.05) [mean ± SD]. Sixteen patients died of cardiopulmonary causes in the conventional group during a mean follow-up period of 58 ± 45 months. In contrast, only three patients died of cardiopulmonary causes in the BPS group during a mean follow-up period of 44 ± 30 months. The absence of BPS therapy, elevated total pulmonary resistance, heart rate, and age were independently related to the mortality by Cox proportional hazard analysis. The 1-year, 3-year, and 5-year survival rates for the BPS group were 100%, 85%, and 76%, respectively, compared to 87%, 60%, and 46% in the conventional group.

Conclusions: This preliminary study suggests that oral administration of BPS may improve hemodynamics and survival in patients with peripheral-vessel CTEPH, for which there is no surgical option.

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