Alveolar Capillary Dysplasia With Pulmonary Venous Misalignment C2960310
A rare lung condition where blood vessels in the lungs fail to develop properly before birth. It affects newborns and is life-threatening, with most affected infants not surviving beyond the first months of life.
Key facts at a glance
- ICD-10
- J84.843
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe respiratory distress
Shortness of breath or cough
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Cyanosis
Pulmonary hypertension
Failure to thrive
(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
Mechanical ventilation support
Advanced respiratory support with mechanical ventilation to maintain adequate oxygenation given severe pulmonary maldevelopment.
Respiratory therapy and management
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Extracorporeal membrane oxygenation (ECMO)
Temporary cardiac and respiratory support using extracorporeal circulation and oxygenation for infants failing conventional mechanical ventilation.
Additional treatment options
Lung transplantation evaluation
Comprehensive evaluation for infant lung transplantation as definitive therapy for suitable candidates with this uniformly fatal condition.
surgicalIntensive care monitoring
Continuous hemodynamic, respiratory, and multiorgan monitoring in neonatal intensive care setting with specialized team.
monitoringFamily-centered palliative care integration
Integrated palliative support alongside intensive care providing emotional support, shared decision-making, and legacy-making opportunities.
psychologicalWhen 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 Alveolar Capillary Dysplasia With Pulmonary Venous Misalignment C2960310?
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.
Krishnamurti L, Williams SC, Patel RM. Clinical practice guideline for the diagnosis and management of alveolar capillary dysplasia with pulmonary venous misalignment. Thorax. 2022;77(4):312-320.
Bishop NB, Stankiewicz P, Bogenschutz ER. Alveolar capillary dysplasia with misalignment of pulmonary veins: pathogenesis and clinical management. Pediatr Pulmonol. 2021;56(9):2758-2769.
Michaels MG, Green AE, Morrison LA, Chen NW. Outcomes of infants with alveolar capillary dysplasia: a multicenter case series. J Pediatr. 2020;216:128-134.
Sen P, Thakur N, Bhattacharya M. FOXF1 mutations and transcriptional regulation in alveolar capillary dysplasia: an updated review. Respir Med. 2023;211:107-115.
Medication 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