Mitral Atresia with Normal Aortic Root
1Children's Heart Institute, Children's Memorial Hermann Hospital, McGovern Medical School, University of Texas-Houston, Houston, TX 77030, USA.
Insights
Mitral atresia with a normal aortic root is a rare congenital heart defect. Treatment involves staged surgical repair, typically the Fontan procedure, with careful monitoring for complications.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Mitral atresia with normal aortic root is a rare congenital heart defect (CHD), accounting for less than 1% of all CHDs.
- This condition involves an atretic mitral valve, a patent foramen ovale for left atrial blood egress, and either a single ventricle or a hypoplastic left ventricle, with a normal aortic valve/root by definition.
Purpose of the Study:
- To define the anatomic and pathophysiologic components of mitral atresia with normal aortic root.
- To outline the treatment strategy, including staged surgical interventions.
- To discuss potential complications and monitoring recommendations.
Main Methods:
- Diagnosis primarily relies on echo-Doppler studies, with minimal need for other imaging modalities.
- Treatment focuses on addressing the defect and associated anomalies in early infancy.
- Management involves a staged total cavo-pulmonary connection (Fontan procedure).
Main Results:
- Clinical, roentgenographic, and electrocardiographic features are non-distinctive.
- Echo-Doppler studies are crucial for accurate diagnosis and understanding pathophysiology.
- Staged Fontan procedure is the eventual surgical requirement.
Conclusions:
- Early diagnosis and management of associated anomalies are critical.
- Staged Fontan procedure is the standard treatment, with potential complications arising during and after the stages.
- Continuous monitoring for early detection and prompt management of complications is recommended.
Abstract:
Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended.
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