CTICasesCleft Mitral Valve with Associated Cardiac Anomalies
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Imaging findings
CT shows a thickened, calcified, dysplastic aortic valve. The mitral valve anterior leaflet is tethered to the interventricular septum by two straps, and a chunk of tissue is missing from this leaflet (cleft mitral valve). An abnormal cord (aberrant left atrial cordae) is seen within the left atrium, coursing inferiorly to the posterior and inferior wall. The coronary sinus is seen draining completely into the left atrium, with no connection to the right atrium.
Key takeaways
Cleft mitral valve (usually anterior leaflet) is often associated with endocardial cushion defects and an abnormal attachment or false tendon to the interventricular septum, which can obstruct the left ventricular outflow tract and lead to abnormal aortic valve development. Other rare associations can include aberrant left atrial cordae and anomalous coronary sinus drainage to the left atrium. These complex congenital anomalies often require surgical repair.
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