Patients with pulmonary arterial hypertension (PAH) refractory to conventional pharmaceutical intervention with PAH-specific medications remain a significant challenge to providers. Generally, such patients are those who have failed combination therapy including infusion prostanoid. The presentation may be gradual decline or acute deterioration with right heart failure requiring critical care to resuscitate and stabilize the patient. Options for management entail a deliberate approach in PAH specialty centers and encompass complex interventions such as lung transplantation, atrial septostomy, Potts anastomosis, and bridging therapies such as extracorporeal circulation.
For that very reason, I am quite pleased that Dr. Harrison (who we all know as Hap) Farber accepted the gauntlet of challenge to serve as guest editor of the current issue. He has orchestrated a spectrum of articles and discussions that span the range of considerations for patients with refractory disease. I would also call your attention to the roundtable discussion that provides insight and perspective beyond the basic criteria for selection of patients for lung transplantation.
Unfortunately, patients may fail all medical interventions; therefore, guidance on the role of palliative care serves an important role. This issue includes 2 informative articles on this topic as well. Hopefully, the reader will be better equipped to care for these patients!