Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand

on behalf of PHSANZ Registry

Research output: Contribution to journalArticleResearchpeer-review

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1368-1375
Number of pages8
JournalHeart Lung and Circulation
Volume27
Issue number11
DOIs
Publication statusPublished - 1 Nov 2018

Keywords

  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Survival

Cite this

@article{7fab66e5f5de4a2cbd0274225ea5cf4d,
title = "Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand",
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.",
keywords = "Pulmonary arterial hypertension, Pulmonary hypertension, Survival",
author = "Geoff Strange and Lau, {Edmund M.} and Eleni Giannoulatou and Carolyn Corrigan and Eugene Kotlyar and Fiona Kermeen and Trevor Williams and Celermajer, {David S.} and Nathan Dwyer and Helen Whitford and Wrobel, {Jeremy P.} and John Feenstra and Melanie Lavender and Kenneth Whyte and Nicholas Collins and Peter Steele and Susanna Proudman and Vivek Thakkar and Dominic Keating and Anne Keogh and {on behalf of PHSANZ Registry}",
year = "2018",
month = "11",
day = "1",
doi = "10.1016/j.hlc.2017.08.018",
language = "English",
volume = "27",
pages = "1368--1375",
journal = "Heart Lung and Circulation",
issn = "1443-9506",
publisher = "Elsevier",
number = "11",

}

Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand. / on behalf of PHSANZ Registry.

In: Heart Lung and Circulation, Vol. 27, No. 11, 01.11.2018, p. 1368-1375.

Research output: Contribution to journalArticleResearchpeer-review

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

AU - on behalf of PHSANZ Registry

PY - 2018/11/1

Y1 - 2018/11/1

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

VL - 27

SP - 1368

EP - 1375

JO - Heart Lung and Circulation

JF - Heart Lung and Circulation

SN - 1443-9506

IS - 11

ER -