[Heart-lung transplantation for idiopathic pulmonary fibrosis]

Diffuse idiopathic pulmonary fibrosis (DIPF) carries a poor prognosis, with an average survival of 4 years. A 37-year-old man had been followed by our department for over 5 years for biopsy-proven DIPF. The disease progressed relentlessly, unresponsive to corticosteroid or colchicine treatment. Marked pulmonary hypertension and right heart failure developed. Arterial pO2 decreased to 30 mmHg and he became bedridden and totally dependent on oxygen inhalation. A heart-lung transplantation was successful in June 1987. The anti-rejection regimen included cyclosporine and azathioprine. Subsequently his functional capacity improved remarkably and pO2 reached 87 mmHg on ambient air. Regular weekly followup visits have included physical examination, chest X-ray, cyclosporine blood levels, blood counts and determination of renal function. During a year of followup there has been no rejection episode nor any other major complication. Although experience with lung transplantation for end-stage DIPF is still limited, it is gradually being accepted as the only successful therapeutic approach. The case presented is the first in Israel of successful heart-lung transplantation for DIPF.