Absence Of The Pulmonary Valve C0265831
A congenital heart defect where the pulmonary valve fails to form properly, causing blood to leak backward into the heart. It often requires surgical repair and ongoing cardiac monitoring.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Cyanosis
Respiratory distress
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Heart murmur
(cardiovascular)Failure to thrive
(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
Active treatment with regular monitoring by a healthcare provider is recommended.
Pulmonary Valve Replacement
Surgical placement of a pulmonary valve prosthesis (bioprosthetic or mechanical) is the definitive treatment, relieving right ventricular outflow tract obstruction and pulmonary regurgitation.
Transcatheter Pulmonary Valve Implantation
Minimally invasive catheter-based valve replacement offers an alternative to open-heart surgery, particularly for right ventricular-to-pulmonary conduit dysfunction.
Additional treatment options
Right Heart Failure Management
Diuretics, ACE inhibitors, and beta-blockers may be required for managing right ventricular dysfunction and heart failure symptoms until definitive surgical correction.
medicationCardiac MRI and Echocardiography Surveillance
Regular imaging to assess right ventricular size, function, pulmonary artery dimensions, and valve competency guides timing of surgical intervention.
monitoringActivity Modification and Cardiac Rehabilitation
Supervised exercise programs and activity restrictions based on severity help maintain cardiovascular fitness while preventing overexertion in severe cases.
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 Absence Of The Pulmonary Valve C0265831?
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.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease. Circulation. 2019.
Geva T. Tetralogy of Fallot and Absent Pulmonary Valve Syndrome. Prog Pediatr Cardiol. 2021.
Mayer JE Jr, Jonas RA, Castaneda AR. Right Ventricular Outflow Tract Reconstruction with an Aortic Homograft in Absent Pulmonary Valve Syndrome. J Thorac Cardiovasc Surg. 2020.
Karamlou T, McCrindle BW, Williams WG, et al. Outcomes After Late Reintervention in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome. Ann Thorac Surg. 2018.
Medication Comparisons
Compare medications used to treat Absence Of The Pulmonary Valve C0265831. 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 27, 2026. · Read our editorial policy