Abstract
Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.
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The data that support the findings of this study are available from the corresponding author, KB, upon reasonable request.
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KB conceived the study concept and design. YI, KB, MK, TE, YS, TF, and KH conducted follow-up of patients and performed echocardiography and catheter interventions. SK performed cardiovascular surgeries. TK performed anesthesia during surgery and catheter intervention. SO conducted review and editing. YI collected the electronic data, conducted data interpretation and statistical analyses. YI was a major contributor in writing the manuscript. All authors read and approved the final manuscript.
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Imai, Y., Baba, K., Otsuki, S. et al. Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion. Pediatr Cardiol 43, 756–763 (2022). https://doi.org/10.1007/s00246-021-02782-z
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DOI: https://doi.org/10.1007/s00246-021-02782-z