When clinicians have a clear treatment objective like transplantation or recovery for patients with pulmonary arterial hypertension (PAH), advanced intensive care treatment and extracorporeal life support (ECLS) is warranted; however, advanced intensive care can be futile when these treatment goals are not realistic, and best supportive care becomes the superior choice, according to an article published in the European Respiratory Journal.
Although there has been therapeutic progress, PAH remains an incurable, chronic disease that is often fatal. Intensive care of these patients focuses on careful fluid management, management of factors that can cause or contribute to heart failure, and strategies to improve cardiac function and reduce ventricular afterload. In distinct situations with clear treatment goals, such as in candidates for lung transplantation, extracorporeal membrane oxygenation (ECMO) should be considered. However, ECMO is not a good option in cases of end-state disease when patients do not have a realistic chance for transplantation or recovery.
Lung transplantation is still an important treatment option for patients with refractory disease. Patients who have received optimized PAH therapy yet remain in a high- or intermediate-risk category should be considered for transplantation. Primary graft dysfunction can be prevented by prolonging postoperative veno-arterial ECMO after transplantation, and at centers experienced in these techniques, 1-year survival rates are now >90%.
Study investigators concluded that PAH will remain a chronic, incurable, progressive disease for the foreseeable future. They noted that “[r]everse remodelling of the pulmonary vasculopathy is a main target of ongoing research, but success in human disease has been limited… future studies should aim at developing new drugs to affect the disease itself, but also at developing new means to support the failing right ventricle. The development of (awake) ECMO as a bridge to transplantation has been a first step… For the time being, lung transplantation remains an important treatment option for patients with otherwise refractory PAH.”
Disclosures: Authors report receiving grants and personal fees from Actelion, Bayer, GSK, MSD, Maquet, Merck, Pfizer, and Zoll.
Hoeper MM, Benza RL, Corris P, et al. Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension [published online December 13, 2018]. Eur Respir J. doi:10.1183/13993003.01906-2018