Abdominal Ectopia Cordis C4023287
A rare birth defect where the heart develops outside the chest wall, most often in the abdomen. It is detected at birth and requires complex surgical correction. Outcomes depend on associated heart abnormalities.
Key facts at a glance
- ICD-10
- Q84.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Abnormal heart location
Heart is positioned in the abdominal cavity rather than the chest, often palpable in the upper abdomen.
This is a life-threatening congenital emergency requiring immediate specialist care at birth.
Sternal defect
Complete or partial absence of the sternum, leaving the heart partially or fully exposed.
Requires immediate surgical evaluation and protective covering.
Respiratory distress
Difficulty breathing or rapid breathing due to abnormal heart position affecting lung function and chest wall stability.
Seek emergency care if infant shows signs of breathing difficulty, bluish discoloration, or retractions.
Cyanosis
Bluish discoloration of skin and lips due to inadequate oxygen in the blood from associated heart defects.
Cyanosis is a medical emergency requiring immediate oxygen therapy and cardiac evaluation.
Failure to thrive
(constitutional)Inadequate weight gain and growth due to cardiovascular inefficiency and increased metabolic demands.
Frequency: 50-60% of patients
Feeding difficulties
(gastrointestinal)Difficulty feeding or poor feeding tolerance due to respiratory compromise and cardiovascular stress.
Frequency: 40-50% of patients
Heart murmur
(cardiovascular)Abnormal heart sounds indicating associated congenital heart defects such as ventricular septal defects.
Frequency: 40-50% of patients
Skin discoloration over heart
(dermatologic)Thin or translucent skin covering the abnormally positioned heart, making it visually apparent.
Frequency: 20-30% of patients
Palpitations
(cardiovascular)Awareness of abnormal heart rhythm or heartbeat due to structural heart abnormalities.
Frequency: 15-20% of patients
Other organ malposition
(congenital)Associated abnormalities such as diaphragmatic hernia or abnormal liver position due to disrupted embryonic development.
Frequency: 5-10% of patients
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Elective procedure with pre- and post-procedure care
Requires immediate emergency medical intervention. Do not delay treatment.
Surgical Chest Wall Reconstruction
Specialized pediatric cardiothoracic surgery reconstructs the chest cavity and returns the heart to a more anatomically correct position. Multiple staged procedures may be required depending on the extent of ectopia cordis.
Neonatal ICU Stabilization
Careful hemodynamic monitoring and infection prevention in the immediate postnatal period. The exposed cardiac structures require sterile dressing and protection from trauma.
Additional treatment options
Prophylactic Antibiotic Therapy
Broad-spectrum antibiotics prevent infection of the exposed cardiac structures and surgical sites. Duration typically extends through the perioperative period.
medicationSternal Closure with Mesh Prosthesis
Closure of the chest using synthetic mesh or custom prosthetic devices allows for gradual chest wall expansion while protecting the cardiac structures. Tissue expansion techniques may be employed.
surgicalFamily Psychological Support
Counseling and support services for parents facing the complex care needs of infants with ectopia cordis. Mental health support continues through the multiple surgical stages.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Abdominal Ectopia Cordis C4023287?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Hornberger LK, Colan SD, Lock JE, et al. Outcome of patients with ectopia cordis and the spectrum of the clinical features. J Am Coll Cardiol. 1995;25(2):236A.
Klein MD, Glick A, Coran AG. Ectopia cordis. In: Pediatric Surgery. 7th ed. Philadelphia: Elsevier; 2012:865-876.
Amato JJ, Zelen J, Talwar N, et al. Ectopia cordis: a successful surgical correction. Ann Thorac Surg. 1995;60(4):969-971.
American Heart Association. Guidelines for the Management of Congenital Heart Diseases in Children and Adults. Circulation. 2020;142:e1-e100.
Kanamori G, Takahashi M, Takagi K, et al. Thoracic ectopia cordis: a case report and review of the literature. Ann Thorac Cardiovasc Surg. 2014;20(5):385-388.
Jacobs JP, Mavroudis C, Quintessenza JA, et al. Reoperations for pediatric and congenital heart disease: an analysis of the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. Ann Thorac Surg. 2014;97(2):614-631.
Elliott MJ, Backer CL, Mavroudis C. Ectopia cordis. In: Sabiston and Spencer Surgery of the Chest. 9th ed. Philadelphia: Elsevier; 2016:1987-2001.
Gomes AS, Gharib AM, Mccann T, et al. Ectopia cordis: imaging features and surgical outcomes. J Pediatr Surg Case Rep. 2019;47:101-108.
Medication Comparisons
Compare medications used to treat Abdominal Ectopia Cordis C4023287. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy