Chronic Thromboembolic Pulmonary Hypertension: An Analysis of the Australian and New Zealand Experience

Purpose Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as pulmonary hypertension resulting from non-resolving fibro-thrombotic obstructions of pulmonary arteries. Pulmonary endarterectomy (PEA) remains the treatment of choice for disease that is technically operable. The epidemiology and long-term outcomes of CTEPH has not been previously described in Australia and New Zealand. Methods Using PHSANZ registry, data was extracted for all CTEPH patients diagnosed between January 2004 and March 2020. We analysed baseline characteristics, treatment strategies, outcome data, and long-term survival. Results A total of 404 patients were included with 146 (36.1%) undergoing PEA and 258 (63.9%) in the non-PEA group. PEA patients were younger (55±16yr vs. 62±16yr) with higher baseline 6MWD (405±122m vs. 323±146m), whilst both groups had similar baseline pulmonary haemodynamics. Pulmonary vasodilator therapy was used in 50% of patients post-PEA, and 76% in the non-PEA group. Actual 1, 5, and 10-yr transplant-free survivals were 93%, 84% and 74% for the PEA group compared to 87%, 63% and 42% for the non-PEA group (log rank test, p Conclusion In this first multicentre report of CTEPH in Australia and New Zealand, long-term survival is comparable to other contemporary registries. However, PEA was only offered to a minority of CTEPH patients and significantly less than overseas reports. Greater awareness and improved patient access to experienced CTEPH surgical centres are important priorities. Multivariate survival analysis showed baseline 6MWD was an independent predictor of survival in both operated and medically managed patients.