Geoff Strange1, Edmund M Lau2, Eleni Giannoulatou3, Carolyn Corrigan4, Eugene Kotlyar4, Fiona Kermeen5, Trevor Williams6, David S Celermajer7, Nathan Dwyer8, Helen Whitford6, Jeremy P Wrobel9, John Feenstra5, Melanie Lavender10, Kenneth Whyte11, Nicholas Collins12, Peter Steele13, Susanna Proudman14, Vivek Thakkar15, Dominic Keating6, Anne Keogh4. 1. School of Medicine, University of Notre Dame, Perth, WA, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Pulmonary Hypertension Society of Australia and New Zealand, Sydney, NSW, Australia. 2. Sydney Medical School, University of Sydney and Royal Prince Alfred Hospital, Sydney, NSW, Australia. Electronic address: edmund.lau@sydney.edu.au. 3. Computational Genomics Laboratory, Victor Chang Cardiac Research Institute, Sydney, NSW, Australia. 4. Heart Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia. 5. Queensland Lung Transplant Service, Prince Charles Hospital, Brisbane, Qld, Australia. 6. Department of Allergy, Immunology and Respiratory Medicine, The Alfred Hospital, Melbourne, Vic, Australia. 7. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney and Royal Prince Alfred Hospital, Sydney, NSW, Australia. 8. Department of Cardiology, Royal Hobart Hospital, Hobart, Tas, Australia. 9. School of Medicine, University of Notre Dame, Perth, WA, Australia; Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia. 10. Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia. 11. Greenlane Clinical Centre, Auckland City Hospital, Auckland, New Zealand. 12. Department of Cardiology, John Hunter Hospital, Newcastle, NSW, Australia. 13. Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia. 14. Rheumatology Unit, Royal Adelaide Hospital, Adelaide, SA, Australia. 15. Department of Rheumatology, Liverpool Hospital, Sydney, NSW, Australia.
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±18.7years, 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.
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±18.7years, 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.
Authors: Manreet K Kanwar; Mardi Gomberg-Maitland; Marius Hoeper; Christine Pausch; David Pittrow; Geoff Strange; James J Anderson; Carol Zhao; Jacqueline V Scott; Marek J Druzdzel; Jidapa Kraisangka; Lisa Lohmueller; James Antaki; Raymond L Benza Journal: Eur Respir J Date: 2020-08-27 Impact factor: 16.671
Authors: Sophia Emmons-Bell; Catherine Johnson; Alexandra Boon-Dooley; Paul A Corris; Peter J Leary; Stuart Rich; Magdi Yacoub; Gregory A Roth Journal: Pulm Circ Date: 2022-01-18 Impact factor: 2.886