Javier De la cruz-Pelayo, Gabriela Mélendez-Ramírez, Regina de la Mora-Cervantes, José Martín Alanís-Naranjo, Ana Laura Trujeque-Ruiz, Omar Antonio Gamboa-Abundis
Instituto Nacional de Cardiología Ignacio Chávez. Centro Médico Nacional 20 de Noviembre ISSSTE.
Mexico
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2026;
DOI: 10.1016/j.jaccas.2026.108873
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.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Class II. Pulmonary Hypertension Associated with Left Ventricular Systolic or Diastolic Dysfunction
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes