Overview
Congenital right ventricular anomaly, specifically double-chambered right ventricle (DCRV), is a rare congenital heart defect characterized by abnormal partitioning of the right ventricle, leading to potential hemodynamic disturbances and obstruction 2.Diagnosis
Transthoracic echocardiography (TTE): Initial imaging modality to identify structural abnormalities 2.
Multiplane transoesophageal echocardiography (TOE): Provides detailed visualization of the right ventricular anatomy, particularly useful in pediatric patients, avoiding the need for angiography 2.
Angiography: Historically used for confirmation but can be avoided with TOE 2.
Surgical inspection: Gold standard for definitive diagnosis and surgical planning 2.Management
Surgical intervention: Primary treatment for symptomatic patients or those with significant obstruction 2.
Preoperative assessment: Utilize multiplane TOE to delineate the anatomy precisely, reducing reliance on angiography 2.
Postoperative care: Standard cardiac surgical follow-up protocols apply, including monitoring for complications and functional recovery 2.Special Populations
Pediatrics: Multiplane TOE is particularly valuable in pediatric patients due to its detailed imaging capabilities and reduced invasiveness compared to angiography 2.Key Recommendations
Utilize multiplane transoesophageal echocardiography for preoperative assessment of double-chambered right ventricle to minimize the need for angiography (Evidence: Moderate) 2.
Surgical correction is indicated for symptomatic patients or those with significant hemodynamic compromise due to DCRV (Evidence: Expert opinion) 2.
Detailed echocardiographic evaluation, especially with multiplane TOE, is essential in pediatric cases to guide surgical planning accurately (Evidence: Moderate) 2.References
1 Yavuz ST, Inan D, Pamuk FO, Sisman A, Kılıcgedik A. A Double-Chambered Left Ventricle Mimicking a Left Ventricular Aneurysm. Echocardiography (Mount Kisco, N.Y.) 2026. link
2 Sreeram N, de Bruijn D, Hitchcock JF. Double chambered right ventricle: delineation by multiplane transoesophageal echocardiography. International journal of cardiology 1998. link00229-0)