Korean J Thorac Cardiovasc Surg.  2004 Oct;37(10):856-860.

Interrupted Aortic Arch with Apical Muscular Ventricular Septal Defect Associating Esophageal Atresia with Tracheoesophageal Fistula

• Sung SC
• Cho JS
• Lee HD

Abstract

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

Keyword

Aortic arch, interrupted; Heart septal defect, ventricular; Esophageal stresia; Tracheoesophageal fistula; Aortic arch

MeSH Terms

Airway Obstruction
Aorta, Thoracic*
Esophageal Atresia*
Heart Septal Defects, Ventricular*
Humans
Infant, Newborn
Pulmonary Artery
Pyloric Stenosis, Hypertrophic
Thoracotomy
Tracheoesophageal Fistula*