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Published on: July 18, 2014
Adolescent Survival in Thoracoabdominal Ectopia Cordis With Complex Single Ventricle Physiology and Complete
Anil Kumar Singhi1, Kaushik Mukherjee2
1Department of Pediatric Cardiology and Adult Congenital Heart Disease, Manipal Hospital-EM Bypass, Kolkata, India.
Clinical Condition:
A 13-year-old child with unrepaired thoracoabdominal ectopia cordis presented with cyanosis, effort intolerance, and recurrent respiratory infections. Evaluation demonstrated tricuspid atresia, a hypoplastic right ventricle, pulmonary atresia with hypoplastic pulmonary arteries supplied by major aortopulmonary collateral arteries, a large subaortic ventricular septal defect, an unrestricted atrial septal defect, bilateral superior vena cavas, and complete atrioventricular block.
Key Questions:
What is the spectrum and natural history of thoracoabdominal ectopia cordis with complex congenital heart disease? What is the role of multimodality imaging in defining the feasibility of intervention? What therapeutic options should the multidisciplinary team consider, and what key information is required before choosing among them? What anatomical and physiological factors influenced the choice between intracardiac repair, palliation, and conservative management, and how does published experience inform these decisions? How does the presence of complete atrioventricular block modify management considerations in this setting?
Outcome:
Multimodality imaging defined severe anatomical constraints, including limited intrathoracic space and a major aortopulmonary collateral artery-dependent pulmonary circulation. After multidisciplinary discussion, the family elected to continue conservative management.
Take-Home Messages:
Prolonged survival to adolescence is possible in unrepaired thoracoabdominal ectopia cordis with severe single-ventricle physiology and complete atrioventricular block through a precarious physiological equilibrium. Multimodality imaging and multidisciplinary assessment are essential for identifying cases suitable for intervention vs those best managed conservatively.
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