ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal disease that results in oxygen dependence, severe disability, and ultimately death, with a median survival time of 3 to 5 years after diagnosis. Although several pharmacologic avenues are available to slow the progression of this aggressive disease, lung transplantation (LTx) remains the only option to improve survival and quality of life because failure in medical management is exceedingly common. 1 Preoperative optimization is paramount to maximize patient benefit following surgery and requires the concerted efforts of both medical and surgical staff. This chapter outlines the basic pathophysiology of IPF, the surgical considerations required when approaching this disease, and the expected short- and long-term outcomes following LTx.
