A Rare Case Of Thoracic Ectopia Cordis: Staged Early Complete Intracardiac Repair
Madonna E. Lee, MD, Bhawna Arya, MD, Amy H. Schultz, MD, Patrick Healey, MD, David M. McMullan, MD.
Seattle Children's Hospital, Seattle, WA, USA.
Objective: Thoracic ectopia cordis (TEC) is a rare (~5.5-7.9/million) congenital defect in fusion of the anterior chest wall. The heart is located outside the thoracic cavity and clinically significant intracardiac lesions may be present. Few neonates survive to or beyond surgical repair. We present a 3.4 kg newborn with complete ectopia cordis, double-outlet right ventricle (DORV), omphalocele, and heterotaxy syndrome [Fig. 1A]. Methods: On the first day of life, the patient underwent extensive mobilization of the thoracoabdominal organs. The inferior vena cava was congenitally displaced anteriorly, which prevented repositioning of the heart into the thorax [Fig. 1B]. The patient became unstable and a pulmonary artery (PA) band was placed. The thoracoabdominal organs were covered with a Jackson-Pratt bulb (heart) and Silastic silo (liver and small bowel) [Fig. 1C]. Fourteen days later the patient underwent left lower lobectomy and division of the right superior vena cava to facilitate positioning of the heart within the thoracic cavity. [Fig. 1D] Seven days later the patient underwent PA band removal and complete intracardiac baffle repair of DORV. At one-month of age, biologic mesh was used to separate and cover the thoracic and abdominal cavities, and a V.A.C. dressing was applied. Results: The patient remains alive at two months. Conclusion: Using a multidisciplinary approach, early staged-repair for complex TEC is achievable, and may result in survival beyond the neonatal period.
Back to 2020 Abstracts