Marshall I. Hertz, M.D.;([unkeyable...

Marshall I. Hertz, M.D.;([unkeyable]) Robert s Bonser, F.R.C.S.;([unkeyable]) Stuart W. Jamieson, MB F.R.C.S.;([unkeyable]) Joseph Tashjian, M.D.(Section) and Robert A. Halvorsen, M.D.(Section)

Pulmonary emphysema inferences in hyperinflation of the lung and concomitant changes in the configuration of the thoracic cavity. We describe a patient who underwent bilateral lung transplantation for emphysema proper to [alpha.sub.1] antitrypsin deficiency. Dramatic changes in chest dimensions and configuration occurr following transplantation, demonstrating the dynamic and reversible nature of the thoracic cavity abnormalities of emphysema.

Pulmonary emphysema be deriveds in hyperinflation of the lung and concomitant changes in the configuration of the thoracic cavity.[1-3] The advent of clinical lung transplantation has given us the opportunity to determine the standing of plasticity of the chest wall changes of emphysema. In this report, we ready a patient who underwent bilateral lung transplantation for emphysema to be ascribed to [alpha.sub.1]-antitrypsin deficiency, and describe the dramatic changes in the thoracic cavity configuration which occurr following transplantation.

CASE REPORT

A 43-year-old man was evaluated prior to lung transplantation. A diagnosis of [alpha.sub.1]-antitrypsin deficiency had been made in 1981 when he neared with exertional dyspnea. At that time, his [FEVsub1] was 09 liters and increased by the agency of 20 percent with inhaled bronchodilators. He was treated with theophylline, inhaled [unkeyable]-adrenergic agonists, prednisone, and oxygen above the ensuing years, his exercise tolerance gradually decreased and he was forced to take a desk piece of work In December, 1986 he was referr to the University of Minnesota Medical Center for evaluation. The patient had a 15 pack-year cigarette smoking history and quit smoking in 1980 He has a brother with unadorned emphysema.

Physical examination revealed a large-chested man in mild respiratory distress at pause The chest was hyperresonant to percussion. The breath unimpaireds were markedly diminished and no wheezes, rales or rhonchi were heard. Jugular venous distention, hepatomegaly and edema were absent.

The [alpha.sub.1]-antitrypsin of the same height was 54 mg/dl, and the Pi phenotype was Z Pulmonary function exhibitions revealed an [FEV.sub.1] of 05 liters (15 percent of predicted) and an FVC of 23 liters (54 percent of predicted). The Dco was 109 ml/min/mm Hg (35 percent of predicted). field air arterial blood gas analysis showed pH 742; [Posub2] 65; and [Pcosub2] 36 The chest film (Fig 1) showed sinked diaphragms, increased retrosternal air space, and decreased pulmonary vascular markings.

The patient underwent felicitous bilateral lung transplantation in January, 1988 A chest X-ray film taken immediately postoperatively revealed a marked change in the configuration of the chest with elevation of the diaphragms as compared with the preoperative film. The patient required reoperation upon the 6th postoperative day to repair a small pleural air leak and was discharged from the hospital in succession the 21st postoperative day in succession cyclosporine A, azathioprine (Imuran) and prednisone. single month after transplantation, he was luckily treated for episodes of cytomegalovirus pneumonia and lung rejection. Since that time his course has been remarkable simply for steady improvement in pulmonary function and exercise tolerance. Seven month after transplantation, pulmonary function standards show an [FEV.sub.1] of 32 liters (88 percent of predicted), an FVC of 35 liters (80 percent of predicted), and a Dco of 166 ml/min/mm Hg (54 percent of predicted). Arterial vital current gas levels drawn while the patient was breathing latitude air showed pH, 7.36; [Posub2] 96; and [Pcosub2] 35

In comparing the preoperative and postoperative chest films (Fig 1 and 2) the diaphragms have been elevated brace full intercostal spaces, the lung height has been reduc from 287 cm to 226 cm and the retrosternal air space has decreased from 65 cm to 31 cm CT scans (Fig 3) indicate that the anterior-posterior diameter of the chest has decreased on 2.5 cm and the width of the chest has decreased by dint of 1.5 cm.

DISCUSSION

This patient put up withed from severe emphysema due to [alpha.sub.1]-antitrypsin dificiency. Emphysema has been defined from the American Thoracic Society as "a condition of the lung characterized according to abnormal, permanent enlargement of airspaces distal to the terminal bronchiole, accompanied by dint of the destruction of their walls. ."[4] The chest X-ray findings in emphysema bring reproach these processes: pulmonary parenchymal destruction is manifested on decreased vascular and interstitial markings; airspace enlargement is manifested according to signs of lung hyperinflation, with detrudeed diaphragms, increased lung height, increased size of the retrosternal air space; and increased anterior-posterior dimension of the chest.[1-3] Typical CT scan findings in emphysema patients include non-peripheral cheap attenuation areas and vascular pruning to be ascribed to parenchymal destruction, and increased anterior-posterior and transverse chest wall dimensions reflecting hyperinflation.[5-7]