TY - JOUR
T1 - Survival of idiopathic pulmonary arterial hypertension patients in the modern era in Australia and New Zealand
AU - Strange, Geoff
AU - Lau, Edmund M.
AU - Giannoulatou, Eleni
AU - Corrigan, Carolyn
AU - Kotlyar, Eugene
AU - Kermeen, Fiona
AU - Williams, Trevor
AU - Celermajer, David S.
AU - Dwyer, Nathan
AU - Whitford, Helen
AU - Wrobel, Jeremy P.
AU - Feenstra, John
AU - Lavender, Melanie
AU - Whyte, Kenneth
AU - Collins, Nicholas
AU - Steele, Peter
AU - Proudman, Susanna
AU - Thakkar, Vivek
AU - Keating, Dominic
AU - Keogh, Anne
PY - 2018/11
Y1 - 2018/11
N2 - 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.
AB - 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.
KW - Pulmonary arterial hypertension
KW - Pulmonary hypertension
KW - Survival
UR - http://www.scopus.com/inward/record.url?scp=85030761188&partnerID=8YFLogxK
U2 - 10.1016/j.hlc.2017.08.018
DO - 10.1016/j.hlc.2017.08.018
M3 - Article
C2 - 29029950
SN - 1443-9506
VL - 27
SP - 1368
EP - 1375
JO - Heart Lung and Circulation
JF - Heart Lung and Circulation
IS - 11
ER -