Patients with idiopathic pulmonary artery hypertension (PAH) undergoing a lung transplant in which extracorporeal life support was needed experienced poorer outcomes compared with patients with other diagnoses, according to study results published in the Journal of Thoracic Disease.

The rate of overall and bronchiolitis obliterans syndrome (BOS)-free survival following lung transplant at one clinic was retrospectively reviewed. Secondary outcomes of interest were postoperative characteristics such as the need for the use of postoperative extracorporeal life support. These outcomes were then compared with patient diagnosis and associations were investigated using IBM SPSS Statistics.

Of the 321 lung transplants, 15 were performed for idiopathic PAH as a cause of end-stage lung disease. Of these, 13 were double and 2 were single lung transplants. BOS-free rates at one, , and 5 years was 75%, 50.0%, and 50.0%, whereas overall cumulative survival at 1, 3, and 5 years was 57.8%, 39.6% and, 19.8%, respectively. Two patients who received lung transplantation were bridged via extracorporeal life support; one patient died on day 38 after transplantation as a result of bowel ischemia that led to multi-organ failure. After propensity score matching, overall cumulative survival was still significantly poorer in patients with idiopathic PAH, whereas there were no statistically significant differences in terms of freedom from BOS.

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“Despite utilization of [extracorporeal life support] in the management of idiopathic PAH, the outcomes in terms of [primary graft dysfunction] and survival remain poor,” the researchers wrote. “Idiopathic PAH patients with end stage lung disease should be referred for lung transplant assessment at [an] early stage and a higher lung allocation score should be attributed to idiopathic PAH patients on waiting lists so that they receive [lung transplants] before it is too late.”

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Sabashnikov A, Mohite PN, Zeriouh M, et al. The role of extracorporeal life support in the management with severe idiopathic pulmonary artery hypertension undergoing lung transplantation: are those patients referred too late?J Thorac Dis. 2019;11(Suppl 6):S929-S937.

This article originally appeared on Pulmonology Advisor