Coarctation in Taussig-Bing malformation of the heart. Surgical significance
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Coarctation of the aorta in infants suggests Taussig-Bing heart malformation over simple transposition with VSD. Initial management for these critical newborns involves pulmonary artery banding and coarctation repair.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Background:
- Transposition of the great arteries (TGA) with ventricular septal defect (VSD) and Taussig-Bing heart malformation are complex congenital heart defects.
- Differentiating these conditions is crucial for appropriate surgical management and improving patient outcomes.
Observation:
- A review of 126 simple TGA with VSD specimens showed 6% with coarctation.
- In contrast, 53% of 105 Taussig-Bing heart specimens exhibited coarctation and/or aortic outflow tract obstruction (p < 10^-10).
- Surgical data corroborated these findings, with coarctation present in 6 of 9 Taussig-Bing patients but none of 17 TGA with VSD patients.
Findings:
- The presence of coarctation of the aorta strongly indicates Taussig-Bing heart malformation rather than simple TGA with VSD.
- Initial surgical management for critically ill newborns with Taussig-Bing heart and coarctation should prioritize pulmonary artery banding and coarctation repair.
- Closure of the VSD through the tricuspid valve is technically challenging in infants; pulmonary artery banding with a Senning procedure is preferred over right ventriculotomy.
Implications:
- Early identification of coarctation can guide diagnosis towards Taussig-Bing malformation, enabling timely and appropriate intervention.
- Pulmonary artery banding and coarctation repair serve as effective initial strategies for managing these complex neonatal cardiac conditions.
- Delayed VSD closure may be necessary, with staged procedures offering a potentially safer approach than immediate VSD closure in certain infant populations.
Abstract:
In a review of 126 heart specimens of simple complete transposition of the great arteries with ventricular septal defect (VSD), coarctation was noted in eight (6%). In 105 Taussig-Bing heart specimens, coarctation and/or aortic outflow tract obstruction was noted in 56 (53%) (p less than 10(-10). This was similar to our surgical experience in 26 patients with transposition and VSD or Taussig-Bing heart. Six of nine patients with Taussig-Bing heart also had coarctation of the aorta, whereas all 17 patients with transposition and VSD had a normal aorta. Four patients with Taussig-Bing heart underwent coarctation repair and pulmonary artery banding at 2 to 7 days of age. The remaining two patients with Taussig-Bing heart did not have a hemodynamically significant coarctation. Five of the six patients with Taussig-Bing heart and coarctation underwent a Senning procedure between the ages of 7 weeks and 3 1/2 years. In four (all less than 4 months of age) the VSD could not be closed through the tricuspid valve. A right ventriculotomy was done in two and contributed to their deaths. In the other two patients the VSD was left alone and the pulmonary artery banded. Both remain well. The fifth child, aged 3 1/2 years, had the VSD closed through the tricuspid valve but died in the postoperative period of renal failure. The sixth patient is awaiting further operation following coarctation repair and pulmonary artery banding. We conclude that the presence of coarctation strongly suggests that Taussig-Bing heart malformation exists rather than transposition and VSD. Pulmonary artery banding and coarctation repair are the initial procedures of choice in the management of these critically ill newborn infants. Increasing cyanosis and failure to thrive will necessitate further operation. Closure of the VSD through the tricuspid valve in these infants is difficult. Pulmonary artery banding in association with a Senning procedure is preferable to a right ventriculotomy. Closure of the VSD is then planned at a later stage.