What does the presence of a gooseneck sign suggest?

The presence of a gooseneck sign is indicative of an endocardial cushion defect, which is a type of congenital heart disease characterized by a combination of atrial septal defect and ventricular septal defect along with a single common atrioventricular valve. This defect leads to abnormal blood flow between the heart chambers and can result in various clinical features, including the gooseneck sign.

The gooseneck sign itself refers to the characteristic appearance of the left atrium on echocardiogram or other imaging studies due to the dilation and malformation of the structures associated with the endocardial cushion defect. It suggests significant hemodynamic compromise and is typically observed in pediatric patients with this condition, as they may develop heart failure and other related complications.

Other conditions listed, such as cardiac tamponade, congenital heart block, and atrial fibrillation, do not present with the gooseneck sign and are associated with different clinical findings and pathophysiological mechanisms. Thus, recognizing the gooseneck sign is crucial for diagnosing endocardial cushion defects and facilitating appropriate management strategies in affected patients.