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Anomalous mitral 'cleft' with abnormal ventriculo-arterial connection: anatomical findings and surgical implications
Insights
This study describes a rare congenital heart defect involving a cleft mitral valve with abnormal connections. These anomalies can lead to mitral regurgitation or left ventricular outlet obstruction.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- A 'cleft' mitral valve is a rare congenital anomaly.
- Associated anomalies include abnormal chordal fixation and ventriculo-arterial connection.
- Understanding these variations is crucial for diagnosis and treatment.
Purpose of the Study:
- To present and characterize seven cases of cleft mitral valve with specific associated anomalies.
- To differentiate these cleft mitral valves from those seen in atrioventricular defects.
- To highlight potential clinical consequences.
Main Methods:
- Descriptive case series.
- Anatomical examination of seven cardiac specimens.
- Detailed description of mitral valve morphology, chordal attachments, and ventriculo-arterial connections.
Main Results:
- Seven specimens exhibited cleft mitral valve with anomalous chordal fixation and abnormal ventriculo-arterial connection.
- Four cases had discordant ventriculo-arterial connection, two had double-outlet right ventricle, and one had double-outlet left ventricle.
- Chordal attachments varied, with most connected to a ventricular septal defect.
Conclusions:
- This specific type of cleft mitral valve differs from atrioventricular defects.
- The anomaly is characterized by anterior cleft, absence of atrioventricular canal defects, and intact basal ventricular septum.
- Potential complications include mitral regurgitation and left ventricular outlet obstruction.
Abstract:
Seven specimens with a 'cleft' mitral valve associated with anomalous chordal fixation and abnormal ventriculo-arterial connection are presented. Four were associated with a discordant ventriculo-arterial connection, two with double-outlet right ventricle, and one with double-outlet left ventricle. In six the chordae from the cleft were attached to the anterior border of a small ventricular septal defect. In the seventh the chordae were attached to an accessory papillary muscle in the left ventricular outflow tract. These clefts differ from those of atrioventricular defects. The cleft is more anterior, there are no septal defects of the atrioventricular canal type, and no deficiency of the basal ventricular septum. Mitral regurgitation or left ventricular outlet obstruction are possible consequences of the anomaly.