Heterotaxy Visceral 9 Autosomal With Male Infertility C5394551
A condition combining abnormal organ arrangement with male infertility. Treatment addresses organ positioning problems along with reproductive health concerns in affected males.
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.
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)Male infertility
(reproductive)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 Defect Surgical Repair
Surgical correction of cardiovascular malformations associated with heterotaxy syndrome, addressing both immediate life-threatening lesions and staged reconstructive procedures.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive Multi-System Surveillance
Lifelong monitoring of cardiac, gastrointestinal, and other organ systems affected by heterotaxy including spleen status, liver configuration, and gastrointestinal positioning.
Additional treatment options
Reproductive Health Consultation
Specialized fertility assessment and reproductive counseling addressing male infertility component, including evaluation of hormonal function and potential assisted reproductive technologies.
complementaryImmunological Support
Management of immunocompromised states associated with asplenia or hyposplenia through prophylactic antibiotics, vaccination protocols, and infection surveillance.
medicationNutritional Optimization
Specialized nutritional support addressing malabsorption concerns, growth requirements in pediatric patients, and maintenance of adequate nutritional status through supplementation when needed.
dietaryWhen 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 9 Autosomal With Male Infertility C5394551?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Heterotaxy Visceral 9 Autosomal With Male Infertility C5394551. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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