Korean J Thorac Cardiovasc Surg.
2000 Jan;33(1):88-95.
Bilateral Sequential Lung Transplantation for a case with Respiratory Failure
due to Lymphagioleiomyomatosis
- Affiliations
-
- 1Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul,Korea.
- 2Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
- 3Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
- 4Department of Anesthesiology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
- 5Department of Radiology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Abstract
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Pulmonary lymphangioleiomyomatosis is a chronic destruct8ive disease of the lung affecting
women of childbearing ages which eventually leads to respiratory failure. Lung transplantation
is the only conclusive therapeutic measure because this disease responds poorly to other
therapies, To date only a few reports in the literature describes the clinical experience of
the bilateral sequential lung transplantation of this rare condition. We performed a bilateral
sequential lung transplantation on a 32-year-old woman suffering from lymphangioleiomyo-matosisw.
The heart-lung block was harvested from a 51-year-old donor. We transplanted the left lung
first through the clam-shell incision. As the hemodynamics deteriorated suddenly during the
dissection of the right lung the right lung was transplanted under the cardio-pulmonary bypass.
Although the patient's lung function was initially satisfactory the patient died of sepsis and
subsequent cardiogenic shock at the postoperative 18th day. Autopsy findings showed infection
of Candida albicans on the pericardium and the left lung which had been initiated possibly
from the left bronchial anastomosis site,. Through detailed review of the clinical course we
concluded that lung transplantation could have been performed safely on this disease provided
that early diagnosis and proper management or the opportunistic infection have been carried
out.