How Would We Treat Our Own Cystic Fibrosis With Lung Transplantation?

Abstract

Lung transplantation is the only therapy for patients with end-stage lung disease. In advanced lung diseases such as cystic fibrosis (CF) life expectancy increases and it is important to recognize extra-pulmonary comorbidities. Cardiovascular involvement includes pulmonary hypertension, right heart failure and myocardial dysfunction are becoming manifest in late stages of the CF disease. Besides right heart failure, left heart dysfunction seems to be underestimated. Therefore, an optimal anesthesia and surgical management risk evaluation in this high-risk patient population is mandatory, especially with regard to the perioperative use of mechanical circulatory support. The use of an index case of an older patient with the diagnosis of cystic fibrosis demonstrates the importance of early risk stratification and strategy planning in a multidisciplinary team approach to guarantee successful lung transplantation.