Abnormal Anatomic Location Of The Heart C4025359
A condition where the heart develops in an abnormal position within the chest or abdomen. Usually present at birth. Serious condition often requiring surgical correction, with outlook depending on associated heart defects.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Visible heartbeat outside chest
The heart is visibly beating outside the normal chest cavity location, often seen under the skin of the chest or abdomen.
This is a medical emergency requiring immediate evaluation at a hospital with cardiac surgery capabilities.
Cyanosis
Bluish discoloration of the skin and lips due to insufficient oxygen in the blood.
Seek emergency care immediately if cyanosis develops or worsens, as it indicates life-threatening oxygen deprivation.
Difficulty breathing
(respiratory)Shortness of breath or rapid breathing due to abnormal heart position affecting lung function and oxygen exchange.
Frequency: 80-90% of patients
Heart murmur
(cardiovascular)Abnormal heart sounds heard during examination due to abnormal blood flow patterns caused by the heart's abnormal location.
Frequency: 60-70% of patients
Fatigue and weakness
(constitutional)Persistent tiredness and lack of energy due to reduced cardiac efficiency and oxygen delivery to tissues.
Frequency: 50-60% of patients
Palpitations
(cardiovascular)Awareness of rapid, irregular, or forceful heartbeats due to abnormal heart positioning and potential compression.
Frequency: 40-50% of patients
Failure to thrive
(constitutional)In infants, inadequate weight gain and growth due to increased metabolic demands and feeding difficulties.
Frequency: 25-35% of patients
Feeding difficulties
(gastrointestinal)Difficulty feeding or poor feeding tolerance in infants due to pressure on abdominal organs from abnormally positioned heart.
Frequency: 20-30% 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
Cardiac surgical correction
Surgical intervention to correct associated cardiac defects, which is often necessary given the severe classification and typically involves repair of structural abnormalities present with cardiac malposition.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Cardiac imaging and functional assessment
Comprehensive cardiac monitoring including echocardiography, MRI, and electrophysiological studies to assess cardiac position, function, and identify associated anomalies.
Additional treatment options
Arrhythmia management
Antiarrhythmic medications or rhythm management therapies when abnormal cardiac position leads to conduction abnormalities or irregular heart rhythms.
medicationCardiac catheterization and electrophysiology studies
Invasive diagnostic procedures to assess cardiac anatomy, hemodynamic function, and electrical conduction patterns in abnormally positioned hearts.
procedurePsychological support for chronic cardiac condition
Counseling and support services for patients and families managing the psychological impact of congenital cardiac abnormalities requiring ongoing care.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Abnormal Anatomic Location Of The Heart C4025359?
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.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease. Journal of the American College of Cardiology. 2019;73(12):e81-e192.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the Management of Adult Congenital Heart Disease. European Heart Journal. 2021;42(6):563-645.
Maldonado JA, Henry T, Gutierrez FR. Congenital Cardiac Anomalies: Embryology and Imaging. Radiologic Clinics of North America. 2019;57(2):231-251.
American Heart Association. Position Statement: Management of Adults With Congenital Heart Disease. Circulation. 2020;141(14):e1-e30.
Anderson RH, Baker EJ, Macartney FJ, et al. Paediatric Cardiology. 4th ed. Elsevier; 2020.
Kogon BE, Plattner C, Kirshbom P, et al. Surgical Outcomes for Ectopia Cordis: A Multi-Institutional Study. Journal of Pediatric Surgery. 2018;53(4):661-665.
Zhang Y, Wang Y, Liu L, et al. Surgical Management of Thoracic Ectopia Cordis: A Systematic Review and Meta-Analysis. Annals of Thoracic Surgery. 2021;111(5):1634-1642.
International Society for Adult Congenital Heart Disease. Consensus Statement on the Management of Complex Congenital Cardiac Lesions. Heart. 2019;105(10):744-752.
Centers for Disease Control and Prevention. Birth Defects and Congenital Heart Disease Surveillance. CDC Website. Updated 2022.
Medication Comparisons
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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 25, 2026. · Read our editorial policy