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Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan
- Source :
- Life Sciences. 118(2):414-419
- Publication Year :
- 2014
- Publisher :
- Elsevier BV, 2014.
-
Abstract
- AimsIdiopathic/heritable pulmonary arterial hypertension (I/HPAH) carries a poor prognosis despite the therapeutic options available. Patient survival from Western countries has been reported, but data from Asia are scarce.Main methodsWe retrospectively reviewed 56 patients with I/HPAH treated at a single referral center in Japan. Survival analyses were conducted using the Kaplan–Meier method with the log-rank test. Variables associated with survival were determined using a Cox proportional hazard model.Key findingsThere were 41 women (73%) and the mean age at the diagnosis was 32±17years. Mean survival time from the diagnosis was 14.9±0.8years (95% CI, 13.4–16.4years), with 1-, 2-, 3-, 5- and 10-year survival rates of 98, 96, 96, 96 and 78%, respectively. In patients who underwent follow-up right-heart catheterization >3months after initial catheterization, mean pulmonary arterial pressure (mPAP) was decreased significantly from 63±15 to 35±10mmHg with an improved cardiac index. Patients with high levels of brain natriuretic peptide (BNP) or low oxygen saturation at baseline showed worse survival. At follow-up, 98% of patients were on PAH-targeted drugs. WHO functional classes I and II, mPAP 2.5L/min/m2, BNP 347m at follow-up were predictors of good prognosis in the univariate analysis.SignificanceThe study revealed a long-term survival of Japanese patients with I/HPAH. Hemodynamic parameters improved significantly after treatment, which might be related to high prescription rates of PAH-targeted drugs. Multicenter studies are needed to reveal the prognostic factors for I/HPAH.
- Subjects :
- Adult
Male
medicine.medical_specialty
Time Factors
Survival
Adolescent
Cardiac index
Hemodynamics
Single Center
General Biochemistry, Genetics and Molecular Biology
Pulmonary hypertension
Pharmacology, Toxicology and Pharmaceutics(all)
Japan
Internal medicine
medicine
Humans
Heritable pulmonary arterial hypertension
Familial Primary Pulmonary Hypertension
Genetic Testing
General Pharmacology, Toxicology and Pharmaceutics
Child
Aged
Univariate analysis
business.industry
Proportional hazards model
Biochemistry, Genetics and Molecular Biology(all)
General Medicine
Middle Aged
Prognosis
Brain natriuretic peptide
medicine.disease
Survival Analysis
Surgery
Survival Rate
Child, Preschool
Peripheral vascular disease
Cardiology
Female
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 00243205
- Volume :
- 118
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Life Sciences
- Accession number :
- edsair.doi.dedup.....27c8564a0d5cda4d57de1b536a34c547
- Full Text :
- https://doi.org/10.1016/j.lfs.2014.01.077