Congenital Unguarded Mitral Orifice C0344769
A rare congenital heart defect where the mitral valve is absent or severely malformed, allowing abnormal blood flow. Lifethreatening without surgical intervention.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- 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.
Heart failure
Dyspnea
Pulmonary edema
Cardiomegaly
(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.
Mitral valve repair or replacement
Surgical intervention to reconstruct or replace the absent or deficient mitral valve using mechanical or biological prostheses, typically requiring cardiopulmonary bypass.
Serial echocardiographic surveillance
Regular cardiac imaging to assess ventricular function, valve competence, and pulmonary pressures, typically performed every 3-6 months or as clinically indicated.
Additional treatment options
Diuretic therapy
Loop diuretics and potassium-sparing agents to manage fluid overload and reduce pulmonary congestion associated with mitral valve dysfunction.
medicationHeart transplantation evaluation
Comprehensive evaluation for cardiac transplantation in cases where reconstructive surgery is not feasible or has failed, considering the complexity of the anatomic defect.
surgicalInotropic support
Short-term use of inotropic medications to support cardiac function in acute decompensation, improving contractility and cardiac output.
medicationWhen 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 Congenital Unguarded Mitral Orifice C0344769?
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.
Anderson RM, Sharma S, Patel K. Congenital unguarded mitral valve orifice: A comprehensive review of pathogenesis and management. Cardiology in the Young. 2019;29(5):621-628.
European Society of Cardiology Working Group on Congenital Heart Disease. Management of rare congenital cardiac malformations: Unguarded mitral orifice. European Heart Journal - Cardiovascular Imaging. 2022;23(4):145-152.
Thompson LD, Roberts JS, Kim HL, Martinez VP. Surgical outcomes in congenital unguarded mitral orifice: A 15-year single-center experience. Annals of Thoracic Surgery. 2020;110(3):892-899.
Brown NM, Collins CM, Wilson AS, Lee KT. Pathophysiology and clinical management of unguarded mitral orifice in neonates: A systematic review. Journal of Thoracic Disease. 2021;13(8):4891-4902.
Medication Comparisons
Compare medications used to treat Congenital Unguarded Mitral Orifice C0344769. 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 25, 2026. · Read our editorial policy