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. 2017 Sep;33(5):498-509.
doi: 10.6515/acs20170612a.

Long-Term Survival of Patients with Pulmonary Arterial Hypertension at a Single Center in Taiwan

Le-Yung Wang  1 , Kuang-Tso Lee  2 , Chia-Pin Lin  2 , Lung-An Hsu  2 , Chun-Li Wang  2 , Tsu-Shiu Hsu  2 , Wan-Jing Ho  2
Affiliations

Long-Term Survival of Patients with Pulmonary Arterial Hypertension at a Single Center in Taiwan

Le-Yung Wang et al. Acta Cardiol Sin. 2017 Sep.

Abstract

Background: Clinical studies have suggested predictive parameters in mortality risk assessment for pulmonary arterial hypertension (PAH) patients. However, these studies predominantly include Caucasian population; information in Asian population is relatively deficient. In this study, we investigated the long-term survival of PAH patients and the predictors of mortality in our population.

Methods: We prospectively collected 70 patients with PAH at the Chang Gung Memorial Hospital between March 2002 and February 2015. Baseline data including functional class (FC), 6-minute walk distance (6MWD), hematological and biochemical data, echocardiography and cardiac catheterization were obtained before commencing PAH- targeted treatment. The follow-up period for analyses of survivors ended in October 2015.

Results: The mean age at diagnosis was 40.7 ± 15.2 years. Mean follow-up period was 4.6 ± 3.4 years, with 1-, 2-, 3-, and 5-year survival rates of 93%, 88%, 84%, and 77%, respectively. The baseline FC was worse in non-survivors than in survivors. More frequent presence of pericardial effusion, higher serum glucose levels, higher estimated systolic pulmonary artery pressure (SPAP) by echocardiography, and higher right atrial pressure (RAP) were found in non-survivors. Higher FC, lower 6MWD, and presence of pericardial effusion were associated with risk of mortality. Patients with worsening FC and increased serum uric acid had an increased risk of mortality during follow-up.

Conclusions: The overall survival remained unsatisfactory in PAH patients. Baseline FC, 6MWD, pericardial effusion, RAP, and a worsening FC and an increased serum uric acid levels during follow-up were significant prognostic parameters.

Keywords: Functional class; Pericardial effusion; Pulmonary arterial hypertension; Serum glucose; Uric acid.

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Figures

Figure 1
Figure 1
Functional class in patients with pulmonary arterial hypertension at baseline and follow-up.
Figure 2
Figure 2
Kaplan-Meier estimates of overall survival in 70 patients with pulmonary arterial hypertension. The 1-, 2-, 3-, and 5-year survival rates were 93%, 88%, 84%, and 77%, respectively.
Figure 3
Figure 3
Survival rate of patients stratified by baseline parameters. (A) WHO functional class (FC), (B) pericardial effusion, (C) right atrial pressure.
Figure 4
Figure 4
Survival rate of different PAH subgroups including IPAH, CTD-PAH and CHD-PAH. CHD, congenital heart disease; CTD, connective tissue disease; IPAH, idiopathic pulmonary arterial hypertension; PAH, pulmonary arterial hypertension.
Figure 5
Figure 5
Survival rates of the patients who did and did not receive PAH-targeted treatment. PAH, pulmonary arterial hypertension.
See this image and copyright information in PMC

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