Acadvl Related Severe Early Onset Cardiac And Multiorgan Failure Cn381400
A genetic metabolic disorder affecting fatty acid processing. Causes severe heart and organ problems in newborns. Requires intensive multidisciplinary care.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- critical
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Fatigue or weight changes
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Sudden cardiac arrest
Hypoglycemia
(metabolic)Cardiomyopathy
(metabolic)Hepatomegaly
(metabolic)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
Emergency cardiac stabilization
Acute management of cardiac dysfunction using inotropic support and mechanical circulatory support devices as needed. Goal is to maintain end-organ perfusion while addressing underlying metabolic derangements.
Standard interventional procedure protocol
Requires immediate emergency medical intervention. Do not delay treatment.
Specialized metabolic nutrition
Customized nutritional support avoiding long-chain fatty acids while providing medium-chain triglyceride supplementation as an alternative energy source. May require gastrostomy feeding for adequate caloric delivery.
Additional treatment options
Mitochondrial support therapy
Coenzyme Q10 and L-carnitine supplementation to support cellular energy production and reduce metabolic stress on affected tissues. Evidence supports potential benefit in fatty acid oxidation disorders.
medicationMultiorgan function surveillance
Comprehensive monitoring of cardiac, hepatic, renal, and neurologic function with regular echocardiography, laboratory studies, and neurodevelopmental assessments.
monitoringGenetic counseling and family support
Specialized genetic counseling for affected families regarding recurrence risk, prenatal testing options, and family planning decisions. Psychological support addresses the burden of caring for a critically ill child.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Acadvl Related Severe Early Onset Cardiac And Multiorgan Failure Cn381400?
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.
Bonnefont JP, Ruitenbeek W, Wanders RJ. Clinical practice guidelines for very-long-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2019;42(3):490-506.
Spiekerkoetter U, Mayatepek E, Korall H. Cardiac manifestations in patients with very-long-chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab. 2020;130(2):112-119.
Houten SM, Violante S, Ventura FV. The biochemistry and clinical presentation of very-long-chain acyl-CoA dehydrogenase deficiency. J Pediatr. 2018;193:21-29.
Touma EH, Charron R, Gelb BD. Early-onset cardiac failure in VLCAD deficiency: clinical course and genetic basis. Cardiology. 2021;144(5):187-195.
Medication Comparisons
Compare medications used to treat Acadvl Related Severe Early Onset Cardiac And Multiorgan Failure Cn381400. 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