Hostname: page-component-76fb5796d-9pm4c Total loading time: 0 Render date: 2024-04-27T00:26:27.072Z Has data issue: false hasContentIssue false

Severe stenosis of a long tracheal segment, with agenesis of the right lung and left pulmonary arterial sling

Published online by Cambridge University Press:  03 February 2006

Hamish M. Munro
Affiliation:
Department of Cardiovascular Anesthesia, The Congenital Heart Institute, Arnold Palmer Hospital for Women and Children/Miami Children's Hospital, Orlando, Florida, United States of America
Andrea M. C. Sorbello
Affiliation:
Department of Cardiology, The Congenital Heart Institute, Arnold Palmer Hospital for Women and Children/Miami Children's Hospital, Orlando, Florida, United States of America
David G. Nykanen
Affiliation:
Department of Cardiology, The Congenital Heart Institute, Arnold Palmer Hospital for Women and Children/Miami Children's Hospital, Orlando, Florida, United States of America

Abstract

A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.

Type
Brief Report
Copyright
© 2006 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Kwan Kim H, Tae Kim Y, Whan Sung S, et al. Management of congenital tracheal stenosis. Eur J Cardiothorac Surg 2004; 25: 10651071.Google Scholar
Grillo HC, Wright CD, Vlahakes GJ, MacGillivray TE. Management of congenital tracheal stenosis by means of slide tracheoplasty or resection and reconstruction, with long-term follow-up of growth after slide tracheoplasty. J Thorac Cardiovasc Surg 2002; 123: 145152.Google Scholar
Lin JH, Chen SJ, Wu MH, Wang JK, Li YW, Lue HC. Right lung agenesis with left pulmonary artery sling. Pediatr Pulmonol 2000; 29: 239241.Google Scholar
Pu WT, Chung T, Hoffer FA, Jonas RA, Geva T. Diagnosis and management of agenesis of the right lung and left pulmonary artery sling. Am J Cardiol 1996; 78: 723727.Google Scholar
Weber TR, Connors RH, Tracy TF Jr. Congenital tracheal stenosis with unilateral pulmonary agenesis. Ann Surg 1991; 213: 7074.Google Scholar
Mardini MK, Nyhan WL. Agenesis of the lung. Report of four patients with unusual anomalies. Chest 1985; 87: 522527.Google Scholar