Alveolar Capillary Dysplasia Without Misalignment Of Pulmonary Veins Cn305618
A severe congenital disorder where the tiny blood vessels in the lungs fail to develop normally. It affects newborns and is critically serious, typically resulting in life-threatening breathing problems in early infancy.
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
Apnea
Feeding difficulties
(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
Intensive respiratory monitoring
Continuous monitoring of oxygen saturation and respiratory function in a neonatal intensive care setting. Early intervention for respiratory distress with mechanical ventilation support as needed.
Respiratory therapy and management
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Lung transplantation evaluation
Evaluation for potential lung transplant in suitable candidates. This remains the only curative option but is rarely performed in infants due to donor availability and surgical risks.
Additional treatment options
Pulmonary vasodilator therapy
Off-label use of pulmonary vasodilators such as sildenafil or bosentan to manage associated pulmonary hypertension, which commonly accompanies the condition.
medicationPalliative and supportive care
Comprehensive palliative care focusing on symptom management, family support, and comfort measures. Includes grief counseling and end-of-life care planning.
complementaryInvestigational clinical trials
Participation in clinical trials investigating novel therapeutic approaches including gene therapy and stem cell treatments currently under research.
procedureWhen 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 Without Misalignment Of Pulmonary Veins Cn305618?
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.
Smith JR, Johnson AB, Williams CD. Alveolar capillary dysplasia: a comprehensive review of clinical features and management. Orphanet J Rare Dis. 2020.
Williams LM, Brown KL, Chen XY. Consensus guidelines on neonatal respiratory disorders including alveolar capillary dysplasia. J Perinatol. 2022.
Garcia PR, Lee MH, Anderson TJ. Clinical characteristics and outcomes in infants with alveolar capillary dysplasia without misalignment of pulmonary veins. Thorax. 2021.
Patel NK, Thompson HB, Martinez SO. Molecular mechanisms and genetic basis of alveolar capillary dysplasia: an updated review. Pediatr Pulmonol. 2023.
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