Estenne M, Ketelbant P, Primo G, Yernault J C
Am Rev Respir Dis. 1987 Apr;135(4):976-8. doi: 10.1164/arrd.1987.135.4.976.
Eighteen sequential follow-up measurements of pulmonary function were obtained over a period of 21 months after heart-lung transplantation in a patient who had undergone surgery for end-stage pulmonary lymphangioleiomyomatosis. In the early postoperative period, there was a moderate decrease in VC and TLC but gas exchange was maintained at essentially normal levels. The most conspicuous features of postoperative lung function were a very low airway resistance and an increase in FEV1/VC ratio above 95%. These alterations were associated with an unusual shape of the maximal expiratory flow-volume (MEFV) curve. Instead of showing a uniform decrease in expiratory flow as expiration proceeds to residual volume, the post-transplant MEFV curve showed a peak followed by a gently sloping plateau ending at a knee where flow suddenly fell. The knee occurred after exhalation of 80% VC. From the sixth postoperative month, the patient developed rapidly increasing air-flow obstruction, which proved to be due to obliterative bronchiolitis. As air-flow obstruction worsened, the knee on the MEFV curve progressively occurred at a higher lung volume, the flow plateau shortened, and flow after the knee became smaller at a given volume. From the ninth postoperative month, it was no longer possible to identify a plateau-knee configuration on the MEFV curve, which resembled that seen in severe obstructive airway disease.(ABSTRACT TRUNCATED AT 250 WORDS)