Abnormal Connection Of The Cardiac Segments C4023306
Heart segments that form abnormally connected during fetal development. Present from birth. Complex defects usually requiring surgical repair, with prognosis depending on specific anatomy.
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
Cyanosis
Bluish discoloration of the skin, lips, and nail beds due to inadequate oxygen in the blood.
Seek immediate emergency care if cyanosis appears suddenly or worsens rapidly.
Hypoxic spells
Sudden episodes of oxygen deprivation causing increased cyanosis, irritability, or limpness.
This is a medical emergency; call emergency services immediately if hypoxic spells occur.
Shortness of breath
(respiratory)Difficulty breathing or feeling out of breath, especially during activity or feeding in infants.
Frequency: 75-85% of patients
Rapid heartbeat
(cardiovascular)Abnormally fast heart rate, often exceeding normal ranges for age and activity level.
Frequency: 60-80% of patients
Fatigue and weakness
(constitutional)Persistent tiredness and lack of energy that interferes with daily activities.
Frequency: 50-65% of patients
Poor feeding
(gastrointestinal)Difficulty feeding or reduced intake in infants, often due to shortness of breath during feeding.
Frequency: 45-60% of patients
Exercise intolerance
(constitutional)Inability to perform physical activities that would be normal for age without excessive fatigue or breathlessness.
Frequency: 50-70% of patients
Failure to thrive
(constitutional)Inadequate weight gain and growth in infants and children due to increased metabolic demands.
Frequency: 20-35% of patients
Heart murmur
(cardiovascular)Abnormal heart sounds heard during examination due to turbulent blood flow through abnormal connections.
Frequency: 10-20% of patients
Digital clubbing
(dermatologic)Enlargement of the fingertips and nails due to chronic low oxygen levels in the blood.
Frequency: 5-15% 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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Surgical correction of cardiac segment abnormality
Open heart surgery to repair or reconstruct the abnormal cardiac connections, often involving cardiopulmonary bypass. The specific technique depends on the exact nature of the abnormal connections.
Cardiac catheterization with intervention
Minimally invasive procedure using catheters to visualize and potentially treat certain abnormal cardiac connections without open surgery.
Additional treatment options
Cardiac medication management
Pharmacological support including beta-blockers, anticoagulants, or diuretics to manage symptoms and prevent complications associated with abnormal cardiac hemodynamics.
medicationLong-term cardiac surveillance
Regular echocardiograms, ECGs, and clinical follow-up to monitor cardiac function and detect any deterioration or need for additional interventions.
monitoringActivity restrictions and cardiac rehabilitation
Structured exercise programs and activity limitations tailored to cardiac capacity to maintain cardiovascular health while avoiding excessive strain on the repaired heart.
lifestyleWhen 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 Connection Of The Cardiac Segments C4023306?
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: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14):e698-e800.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the Management of Adult Congenital Heart Disease. Eur Heart J. 2021;42(6):563-645.
Warnes CA. Transposition of the great arteries. Circulation. 2006;114(24):2699-2709.
Connelly MS, Webb GD, Somerville J, et al. Canadian Consensus Statement on Adult Congenital Heart Disease. Can J Cardiol. 1998;14(3):395-452.
Anderson RH, Baker EJ, Macartney FJ, et al. Paediatric Cardiology. 3rd ed. London: Churchill Livingstone; 2002.
American Heart Association. Management of Adults With Congenital Heart Disease: A Scientific Statement From the AHA. Circulation. 2020;141(14):e841-e869.
Michaelsson M, Ruckman RN. Congenitally Corrected Transposition of the Great Arteries. In: Gatzoulis MA, Webb GD, Daubeney PEF, eds. Diagnosis and Management of Adult Congenital Heart Disease. 3rd ed. Elsevier; 2017:341-352.
Oster ME, Lee KA, Honein MA, et al. Temporal Trends in Survival Among Individuals With Congenital Heart Defects. Pediatrics. 2013;132(2):e417-e423.
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