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Combination of left ventricular noncompaction and partial atrioventricular canal defect in a 21-year-old male: a case
Malick Bodian1, Modou Jobe, Mohamed Lèye
1Department of Cardiology, Aristide Le Dantec Teaching Hospital, Dakar, Senegal.
Insights
This case report details a rare combination of left ventricular noncompaction (LVNC) and partial atrioventricular canal defect in a young male presenting with heart failure. Early diagnosis and management are crucial for this genetic cardiomyopathy.
Area of Science:
- Cardiology
- Genetics
- Pediatric Cardiology
Background:
- Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy.
- It is characterized by progressive systolic dysfunction.
- LVNC can occur with congenital cardiac anomalies.
Observation:
- A 21-year-old male presented with heart failure symptoms.
- Echocardiography revealed LVNC with prominent trabeculations.
- A partial atrioventricular canal defect was also identified.
Findings:
- The study describes a rare co-occurrence of LVNC and partial atrioventricular canal defect.
- Biventricular systolic dysfunction was present.
- The patient showed a positive response to medical management.
Implications:
- Highlights the importance of suspecting LVNC in heart failure patients.
- Emphasizes the need to screen for associated cardiac anomalies.
- Informs optimal short- and long-term management strategies for this rare condition.
Introduction:
Left ventricular noncompaction (LVNC) is classified as a genetic cardiomyopathy characterized by a progressive systolic dysfunction. It may occur alone or in association with congenital cardiac anomalies. The combination of left ventricular noncompaction with partial atrioventricular canal defect is rare and has not, to our knowledge, been described previously.
Case Presentation:
A 21-year-old male who traveled to our center from a neighboring country presented with signs of heart failure. Transthorarcic echocardiography showed prominent trabeculations in the left ventricle predominantly in the left ventricle involving the apical lateral and mid anterolateral segments associated with a partial atrioventricular canal defect. There was a biventricular systolic dysfunction. There was good response to medical treatment.
Conclusion:
This case stresses the importance of maintaining a high degree of suspicion for this rare cardiomyopathy and the need to systematically look for other associated anomalies in order to institute proper short- and long-term managements.
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