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Ectopia cordis: a rare congenital anomaly
Abigail Gabriel1, Joseph Donnelly, Alexander Kuc
1Department of Anatomical Sciences, St. George's University, School of Medicine, Grenada, West Indies; Department of Internal Medicine, Harlem Hospital, New York, New York.
Insights
Ectopia cordis (EC) is a rare congenital heart anomaly where the heart is outside the chest. This review covers its history, causes, symptoms, and management.
Area of Science:
- Cardiology
- Developmental Biology
- Medical Genetics
Background:
- Ectopia cordis (EC) is a rare congenital anomaly characterized by the heart's abnormal positioning outside the thoracic cavity.
- This condition presents a spectrum of anatomical locations, including cervical, thoracic, and abdominal regions, often associated with a split sternum.
Observation:
- Since the early 1600s, only 91 cases of EC have been documented in medical literature, highlighting its extreme rarity.
- The review synthesizes historical data, prevalence rates, and etiological factors contributing to EC.
Findings:
- Morphological variations, clinical presentations, and associated symptoms of EC are detailed.
- Complications, diagnostic strategies, and current treatment and management approaches are discussed.
Implications:
- Understanding EC's multifaceted aspects is crucial for improving diagnostic accuracy and therapeutic interventions.
- This review provides a comprehensive resource for clinicians and researchers managing this complex congenital defect.
Abstract:
Ectopia cordis (EC) is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. The ectopic heart can be found along a spectrum of anatomical locations, including the cervical, thoracic and abdominal regions and in most cases, it protrudes outside the chest through a split sternum. Although the first case of EC was identified during the early 1600s only 91 cases have been reported since then in the literature. This review will discuss the history and prevalence of EC, its etiology, morphology, presentation and symptoms, complications, diagnosis, treatment and management and prognosis.
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