Heterotaxy Visceral 6 Autosomal C3553676
A congenital condition affecting organ arrangement in the body caused by genetic mutations. Patients usually have heart abnormalities and may need multiple surgeries and ongoing medical management.
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
Gastrointestinal malrotation
Complex congenital heart disease
(cardiovascular)Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent respiratory infections
(respiratory)Abnormal spleen (asplenia/polysplenia)
(hematologic)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
Individualized Cardiac Surgical Planning
Custom surgical approach based on detailed pre-operative imaging and genetic findings specific to this heterotaxy variant. May involve complex repairs of transposition patterns and septal defects.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Situs and Organ Function Assessment
Regular assessment of organ arrangement and function, including abdominal ultrasound for asplenia or polysplenia, and monitoring for associated gastrointestinal complications.
Additional treatment options
Interventional Cardiac Catheterization
Minimally invasive procedures to address specific cardiac lesions identified on imaging. May include device closure of septal defects, valve interventions, or vascular stenting.
procedureActivity and Self-Management Education
Comprehensive education on activity limitations, symptom monitoring, infection prevention (important with asplenia), and when to seek medical attention. Promotes self-management and independence.
lifestyleHeart Failure Medication Optimization
Pharmacologic management targeting heart failure symptoms when present, including diuretics, ACE inhibitors, and beta-blockers as tolerated. Regular medication reconciliation required.
medicationWhen 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 6 Autosomal C3553676?
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 20, 2026. · Read our editorial policy