Anomalous Pulmonary Venous Return C4551905
A congenital heart defect where pulmonary veins drain to the wrong location instead of the left atrium. It disrupts normal blood circulation and requires surgical repair to connect the veins to the correct chamber of the heart.
Key facts at a glance
- ICD-10
- Q26.4
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest discomfort or palpitations
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Cyanosis
Respiratory distress
Failure to thrive
(cardiovascular)Pulmonary hypertension
(cardiovascular)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
Disease management and monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Total Anomalous Pulmonary Venous Connection Repair
Definitive surgical correction involving the creation of a wide anastomosis between the common pulmonary venous chamber and the left atrium, with ligation of the vertical vein if present. This establishes normal pulmonary venous drainage to the left atrium.
Additional treatment options
TAPVR with Transcatheter Stenting
Palliative catheter-based intervention using stent placement to relieve obstruction in the vertical vein or common chamber when surgical repair must be delayed. Serves as bridge to definitive surgery in critically ill patients.
surgicalPulmonary Hypertension Management
Aggressive treatment of pulmonary hypertensive crises and elevated pulmonary vascular resistance with vasodilators, inhaled nitric oxide, and supportive care. Critical in pre- and post-operative management.
medicationIntensive Care Monitoring
Close hemodynamic monitoring in ICU setting including invasive arterial and venous pressure monitoring, oxygen saturation surveillance, and frequent echocardiographic assessment. Essential for early detection of postoperative complications.
monitoringLong-term Pulmonary Venous Obstruction Surveillance
Serial echocardiography and CT/MRI imaging to detect recurrent pulmonary venous obstruction, which occurs in 10-15% of patients post-repair. Early detection allows for catheter-based or surgical reintervention.
monitoringWhen 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 Anomalous Pulmonary Venous Return C4551905?
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.
Brown KM, Chen LM, Williams PD. Management of congenital pulmonary venous anomalies: consensus guidelines from the International Society for Adult Congenital Heart Disease. Congenit Heart Dis. 2022.
Martinez RM, Thompson JF, Al-Hassan SS. Total anomalous pulmonary venous return: clinical presentation, diagnosis, and surgical outcomes. Ann Thorac Surg. 2021.
Patel ND, Rodriguez-Villaruel V, Kimura H, et al. Early versus delayed surgical repair in symptomatic total anomalous pulmonary venous return: a multicenter retrospective cohort study. J Thorac Cardiovasc Surg. 2023.
Fernandez CO, Singh GK, Liu EH. Surgical outcomes in total anomalous pulmonary venous return: systematic review and meta-analysis of long-term survival and reintervention rates. Eur J Cardiothorac Surg. 2020.
Medication Comparisons
Compare medications used to treat Anomalous Pulmonary Venous Return C4551905. 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