Lung Transplantation and Atrial Septostomy for Pulmonary Arterial Hypertension

Improvements in medical therapy for PAH patients have led to dramatic changes in the way PAH is managed, with much less emphasis on surgical techniques than in the past. Nevertheless, the current drug regimens are still ineffective or lose effectiveness in many patients, related to the progression of the underlying disease. For these patients, lung transplantation and atrial septostomy offer the hope of improved function, prolonged survival, and, most important to some patients, enhanced quality of life. However, both procedures are risky, with the potential for substantial morbidity and mortality, and patients must be selected carefully. Most lung transplant recipients now undergo double-lung transplants and experience higher perioperative mortality rates than those undergoing transplants for other diagnoses. Infection and acute rejection are early causes of morbidity, and most patients later suffer from bronchiolitis obliterans, thought to be related to chronic rejection. This is the main reason for a survival rate that approaches only 50% at five years’ posttransplant. Septostomies are reserved for patients with high-rate atrial pressures who are suffering from low cardiac output syndromes or syncope. They have been associated with mortality rates of 16% in the past, but more recent studies using graded balloon septostomy report rates approaching 5%.