Korean J Thorac Cardiovasc Surg.  2007 Aug;40(8):569-573.

Aggressive Surgical Treatment for Complex Cardiac Anomalies Associated with Right Atrial Isomerism

• Hwang UD
• Yun TJ
• Jung SH
• Jhang WK
• Kim YH

Abstract

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The postoperative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.

Keyword

Heterotaxy; Pulmonary vein, total anomalous return; Pulmonary artery, collateral; Extracorporeal membrane oxygenation

MeSH Terms

Arteries
Drainage
Echocardiography
Extracorporeal Membrane Oxygenation
Female
Heterotaxy Syndrome*
Humans
Hyperemia
Infant
Intubation, Intratracheal
Oxygen
Oxygenators
Oxygenators, Membrane
Scimitar Syndrome
Tracheal Stenosis
Tracheostomy
Oxygen