Apical Right Ventricular Sequestration with Left Ventricular Hypertrabeculation: Multimodal Imaging Evaluation in an Adolescent.

BACKGROUND

Apical right ventricular sequestration is a rare, nonobstructive variant of double-chamber right ventricle with complex anatomy that may be underrecognized.

CASE SUMMARY: A 14-year-old female presented with palpitations, failure to thrive, and recurrent respiratory infections, with a history of cyanosis in early childhood. Evaluation demonstrated cardiomegaly, biatrial enlargement, severe atrioventricular valve regurgitation, and biventricular dysfunction. Multimodality assessment identified apical sequestration of the right ventricle as a variant of double-chamber right ventricle, associated with a large ventricular septal defect, reduced functional right ventricular volume, and severe pulmonary hypertension. Given advanced hemodynamic involvement, medical therapy was selected, resulting in clinical improvement.

DISCUSSION: Apical right ventricular sequestration differs from the classic obstructive form of double-chamber right ventricle and poses diagnostic challenges.

TAKE-HOME MESSAGES: Apical right ventricular sequestration represents a rare variant of double-chamber right ventricle. Accurate anatomic characterization is essential to guide management.