Korean J Thorac Cardiovasc Surg.  2004 Dec;37(12):1015-1018.

Single Lung Transplantation in the Patient with End-stage Lymphangioleiomyomatosis: Report of 1 case

• Yang HC
• Kim K
• Choi YS
• Kim J
• Shim YM

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease in women in childbearing age leading to progressive respiratory failure. LAM is characterized by an abnormal harmartomatous proliferation of smooth muscle cells surrounding the blood vessels, lymphatics and airways in the lung. This proliferation leads to airway obstruction, cystic alveolar change and lymphatic obstruction. Patients present with dyspnea, pneumothorax, cough, chest pain, hemoptysis, and chylous effusion. Although lung transplantation is the only therapeutic modality in end-stage LAM with respiratory failure, there has not been any report of successful treatment in Korea. We report one case of successful left single lung transplantation in a 40 year old woman suffering from end-stage LAM.

Keyword

Lung transplantation; Lymphangioleiomyomatosis

MeSH Terms

Adult
Airway Obstruction
Blood Vessels
Chest Pain
Cough
Dyspnea
Female
Hemoptysis
Humans
Korea
Lung Transplantation*
Lung*
Lymphangioleiomyomatosis*
Myocytes, Smooth Muscle
Pneumothorax
Rare Diseases
Respiratory Insufficiency