JACC Case Rep. 2026 Jun 29:108873. doi: 10.1016/j.jaccas.2026.108873. Online ahead of print.
ABSTRACT
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.
PMID:42376712 | DOI:10.1016/j.jaccas.2026.108873