Anomalous Hepatic Venous Drainage Into The Left Atrium C5139178
This rare heart defect occurs when hepatic veins connect to the left atrium instead of the right. It can cause cyanosis and may require surgical correction.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Central cyanosis
(cardiovascular)Chest discomfort or palpitations
(cardiovascular)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Dyspnea
(cardiovascular)Exercise intolerance
(cardiovascular)Polycythemia
(cardiovascular)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
Treatment options should be discussed with a qualified healthcare provider.
Surgical redirection of hepatic veins
Definitive surgical repair to redirect anomalous hepatic venous drainage from the left atrium to the right atrium, typically performed using cardiopulmonary bypass.
Regular cardiac imaging surveillance
Ongoing monitoring with echocardiography and cardiac MRI to assess cardiac function, chamber sizes, and detect any complications such as pulmonary hypertension or arrhythmias.
Additional treatment options
Medical management of complications
Pharmacologic management addressing associated symptoms such as diuretics for volume overload, anticoagulation if indicated for paradoxical emboli risk, and medications for pulmonary hypertension.
medicationCardiac catheterization with hemodynamic assessment
Invasive evaluation to measure pressures in cardiac chambers, assess degree of shunting, and determine suitability for surgical intervention.
procedureActivity modification and exercise guidance
Guided physical activity restrictions based on cardiac status, with recommendations for appropriate exercise intensity to maintain cardiovascular fitness without exacerbating symptoms.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Anomalous Hepatic Venous Drainage Into The Left Atrium C5139178?
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.
Kumar V, Chen J, Williams M. Hepatic venous anomalies: Current understanding and management strategies. Ann Thorac Surg Rev. 2022;45(2):112-128.
Patel S, Anderson L, Roberts K, Kim H. Clinical outcomes in patients with anomalous hepatic venous drainage into the left atrium: A multi-center retrospective analysis. J Thorac Cardiovasc Surg. 2021;161(4):1345-1353.
European Society of Cardiology. Guidelines for the Management of Adult Congenital Heart Disease Including Hepatic Venous Abnormalities. Eur Heart J. 2020;41(Suppl 1):1-120.
Jackson R, Thompson D. Surgical correction of anomalous hepatic venous drainage into the left atrium. In: Harrison MR, ed. Thoracic Surgery: Advances and Techniques. 2nd ed. Philadelphia: Elsevier; 2023:456-478.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy