A hammock in the left atrium

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Shone's anomaly is a rare congenital heart defect characterized by multiple left-sided obstructive lesions.
• These obstructions typically include parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta.

Observation:

• This case presents a unique constellation of Shone's anomaly features.
• Key findings include aortic recoarctation, a mitral supravalvular membrane, bicuspid aortic valve, and complete atrioventricular block.
• The patient also experienced bradycardia-induced nonsustained polymorphic ventricular tachycardia.

Findings:

• 3D transesophageal echocardiography was crucial in accurately visualizing the mitral supravalvular membrane.
• The combination of these specific anomalies and arrhythmias makes this a particularly complex presentation of Shone's anomaly.

Implications:

• This case highlights the importance of advanced imaging techniques like 3D TEE in diagnosing complex congenital heart defects.
• Understanding such rare presentations can improve diagnostic accuracy and guide management strategies for Shone's anomaly.
• Further research into the genetic and developmental factors underlying these complex cardiac malformations is warranted.