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Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand
- Source :
- Heart, Lung and Circulation. 27:1368-1375
- Publication Year :
- 2018
- Publisher :
- Elsevier BV, 2018.
-
Abstract
- Background Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era. Methods Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. Results Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2 ± 18.7 years, female 69.5%) and followed for a median duration of 26 months (IQR17–39). Co-morbidities were common such as obesity (34.1%), systemic hypertension (30.5%), coronary artery disease (16.4%) and diabetes mellitus (19.5%). Initial combination therapy was used in 54 patients (dual, n = 50; triple, n = 4). Estimated survival rates at 1-year, 2-years and 3-years were 95.6% (CI 92.8–98.5%), 87.3% (CI 82.5–92.4%) and 77.0% (CI 70.3–84.3%), respectively. Multivariate analysis showed that male sex and lower 6-minute distance at diagnosis independently predicted worse survival, whereas obesity was associated with improved survival. Co-morbidities other than obesity did not impact survival. Initial dual oral combination therapy was associated with a trend towards better survival compared with initial oral monotherapy (adjusted HR = 0.27, CI 0.06-1.18, p = 0.082) Conclusions The epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America. Male sex and poorer exercise capacity are predictive of mortality whereas obesity appears to exert a protective effect. Despite current therapies, PAH remains a life-threatening disease associated with significant early mortality.
- Subjects :
- Male
Pulmonary and Respiratory Medicine
Cardiac Catheterization
medicine.medical_specialty
Multivariate analysis
Combination therapy
Hypertension, Pulmonary
Disease
030204 cardiovascular system & hematology
Coronary artery disease
03 medical and health sciences
0302 clinical medicine
Diabetes mellitus
Internal medicine
Epidemiology
medicine
Humans
Prospective Studies
Pulmonary Wedge Pressure
Registries
Aged
business.industry
Australia
Middle Aged
Prognosis
medicine.disease
Pulmonary hypertension
Obesity
Surgery
Survival Rate
030228 respiratory system
Female
Cardiology and Cardiovascular Medicine
business
New Zealand
Subjects
Details
- ISSN :
- 14439506
- Volume :
- 27
- Database :
- OpenAIRE
- Journal :
- Heart, Lung and Circulation
- Accession number :
- edsair.doi.dedup.....0b7a86521e6ad8c8b705cfaad63063a3