X Linked Intellectual Disability Cardiomegaly Congestive Heart Failure Syndrome C3550913
A rare genetic syndrome in males combining intellectual disability with heart enlargement and heart failure. Treatment requires cardiac management alongside developmental support.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Seizures
(neurologic)Hypotonia
(neurologic)Developmental delay
(neurologic)Feeding difficulties
(neurologic)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 Function Surveillance
Regular echocardiograms, ECG monitoring, and BNP testing to track cardiomegaly progression and heart failure development. Frequency adjusted based on disease severity.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Heart Failure Pharmacotherapy
Standard heart failure medications including diuretics, ACE inhibitors, beta-blockers, and aldosterone antagonists to reduce cardiac workload and manage congestion. Selection based on individual tolerance and contraindications.
Additional treatment options
Cardiac Device Implantation
Pacemaker or defibrillator implantation for arrhythmias or conduction abnormalities associated with cardiomegaly. Device selection based on specific rhythm disturbance and cardiac function.
surgicalCardiac-Specific Nutrition
Sodium-restricted diet with appropriate fluid management to reduce cardiac workload. Includes heart-healthy omega-3 fatty acids and potassium-rich foods as tolerated.
dietaryActivity Modification
Structured activity program balancing rest with appropriate physical activity. Includes energy conservation techniques, graduated exercise programs, and avoidance of strenuous exertion.
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 X Linked Intellectual Disability Cardiomegaly Congestive Heart Failure Syndrome C3550913?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy