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Anterior chest wall deformities and congenital heart disease
R C Shamberger1, K J Welch, A R Castaneda
1Department of Surgery, Children's Hospital, Boston, Mass. 02115.
Insights
Surgical repair of pectus excavatum and pectus carinatum in children with congenital heart defects is safe. Chest wall deformity repair after cardiac surgery yields good results, but repair before cardiac surgery is recommended for those needing extracardiac conduits.
Area of Science:
- Pediatric Surgery
- Cardiothoracic Surgery
- Congenital Abnormalities
Background:
- Pectus excavatum and pectus carinatum are typically isolated chest wall deformities.
- Associated congenital heart defects are rare, reported in only 19 cases.
- Uncorrected pectus excavatum can cause complications during or after cardiac surgery.
Purpose of the Study:
- To review surgical repair outcomes for combined chest wall deformities and congenital heart defects.
- To assess the risks of pectus repair before and after cardiac anomaly correction.
- To establish optimal surgical timing for these coexisting conditions.
Main Methods:
- Retrospective review of 36 pediatric patients with congenital heart disease and thoracic deformities.
- Analysis of 22 patients who underwent surgical correction for pectus excavatum or carinatum.
- Comparison of outcomes based on the timing of pectus repair relative to cardiac surgery.
Main Results:
- Pectus repair after cardiac surgery was performed in 10 patients with no pleural/pericardial entry or blood transfusions.
- 10 patients had pectus repair before cardiac surgery or without subsequent cardiac repair.
- Simultaneous repair was avoided due to high complication risk; repair before cardiac surgery recommended for those with extracardiac conduits.
Conclusions:
- Congenital chest wall deformities can be safely repaired after congenital heart defect correction.
- Pectus repair after cardiac surgery shows good results.
- For patients requiring extracardiac conduits, initial pectus repair is recommended to prevent conduit compression.
Abstract:
Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.