Diagnosis, surgical management, and long-term outcomes of double chambered right ventricle: a rare and challenging disease in the adult population

• Published in: Progress in pediatric cardiology Vol. 62; p. 101417
• Main Authors: Otero-Luna, Andrea, Ghiroli, Stephen, Hsia, Tain-Yen, Fahey, John, Elder, Robert W.
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 01-09-2021
• Subjects: Adult congenital heart disease; Double chambered right ventricle; Late outcomes; Ventricular septal defect; Late outcomes; Ventricular septal defect; Adult congenital heart disease; Double chambered right ventricle
• ISSN: 1058-9813; 1558-1519
• DOI: 10.1016/j.ppedcard.2021.101417

Double Chambered right ventricle is a rare disease in adults and can be a late and incidental presentation in patients with history of ventricular septal defect.
To describe our institution's experience with late presentation of double chambered right ventricle.
We performed a review of demographic and clinical data and review of echocardiographic studies of patients with diagnosis of double chambered right ventricle. We examined pre-operative assessment, surgical outcomes, and clinical status at latest follow up.
Six adults with mean age of 40 years were identified to have and underwent surgical repair of double chambered right ventricle in our institution in the last decade. Two patients were older than 60 years of age. In all the patients, the pre-operative gradient from right ventricular inflow to outflow chamber was greater than 70 mm Hg. All patients underwent successful repair without significant residual gradient. Arrhythmia was present in three of our patients in the postoperative period.
Double chambered right ventricle is a rare disease but may be diagnosed in patients well into later adulthood who have a prior history of ventricular septal defect. The diagnosis requires a high index of suspicion in this patient population, but surgical repair can be done safely with satisfactory post-operative results.
•Double chambered right ventricle is a rare finding in the adult population with history of ventricular septal defect.•Double chambered right ventricle can develop even when the ventricular septal defect has spontaneously or surgically closed.•Diagnosis of double chambered right ventricle requires high index of suspicion.•Surgical repair of double chambered right ventricle can be done safely with satisfactory post-operative results.