Heterotaxy Visceral 2 Autosomal C1415817
A genetic condition causing improper organ placement in the chest and abdomen. Both males and females can be affected with autosomal inheritance. Cardiac abnormalities typically need surgical intervention.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Congenital cardiac malformation
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Visceral heterotaxy
(congenital)Biliary atresia
(gastrointestinal)Bronchial isomerism
(respiratory)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 intervention
Surgical management of the complex cardiac defects characteristic of autosomal heterotaxy, similar to the X-linked form. This may include repair of transposition patterns, ventricular septal defects, and Fontan-type palliation when indicated.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Cardiac rhythm surveillance
Systematic monitoring for arrhythmias and conduction abnormalities through regular electrocardiograms, Holter monitors, and event recorders. Early detection allows for timely intervention and device placement.
Additional treatment options
Prophylactic antibiotic coverage
Lifelong prophylactic antibiotics and comprehensive immunization protect against infections in patients with functional asplenia. Education about infection signs and urgent care access is essential.
medicationCardiac catheterization
Interventional catheterization procedures may be used for diagnostic evaluation, balloon valvuloplasty, or stent placement. These less invasive approaches can delay or reduce the need for open heart surgery.
procedurePsychosocial support and family counseling
Comprehensive psychological support addresses the chronic nature of heterotaxy and its impact on quality of life. Family counseling helps navigate the complexities of managing a lifelong condition with genetic implications.
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 Heterotaxy Visceral 2 Autosomal C1415817?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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