Local data from the Pulmonary Hypertension Society of Australian and New Zealand (PHSANZ) Registry confirms that Australian patients with PAH aren’t doing too badly considering limited access to combination therapy.
In a study of 220 cases of predominantly idiopathic PAH diagnosed between the beginning of 2012 and the end of 2016, one-year survival was 95.6%, two-year survival 87.3% and three-year survival 77%.
Co-author Dr Edmund Lau, from the University of Sydney and Royal Prince Alfred Hospital, said the findings compared favourably to patient survival rates from PAH registries in other Western countries.
For example, a French study from 2002-2003 reported a one-year survival of 83% and three-year survival of 58%.
“However their data comes from an earlier time than our population and treatment has evolved quite rapidly in this field over the past decade,” Dr Lau said.
The Australian study reported 74% of patients started treatment on monotherapy – typically an endothelin receptor antagonist – 24% on dual combination therapy and 2% on triple therapy
Dr Lau said the low rates of combination therapy were consistent with the time period before stronger evidence was available for the benefits of initial combination therapy.