J Cardiovasc Ultrasound.  2015 Mar;23(1):48-51. 10.4250/jcu.2015.23.1.48.

Double Chambered Right Ventricle with Ventricular Septal Defect in Adults: Case Series and Review of the Literature

• Moustafa S
• Patton DJ
• Alvarez N
• Al Shanawani M
• AlDossari
• Connelly M
• Prieur T
• Mookadam F

• KMID: 2135408
• DOI: http://doi.org/10.4250/jcu.2015.23.1.48

Abstract

Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

Keyword

Double chambered right ventricle; Ventricular septal defect; Echocardiography; Magnetic resonance

MeSH Terms

Adult*
Echocardiography
Heart Septal Defects, Ventricular*
Heart Ventricles*
Humans

Figure

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  Fig. 1 A: Transesophageal echocardiogram mid esophageal view showing muscle band in the right ventricular outflow tract (arrow). B, C, and D: Transthoracic echocardiogram short axis views showing right ventricular muscle band (arrow). C: Posterior pericardial effusion was noted. E: Transesophageal echocardiogram mid esophageal view showing muscle band in the right ventricular outflow tract (left, black arrow) with color turbulence across (right). Pulmonic valve was shown (white arrow). LA: left atrium, LV: left ventricle, PA: pulmonary artery, RV: right ventricle, RVOT: right ventricular outflow tract.

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  Fig. 2 A and B: Cardiac magnetic resonance: sagittal steady-state free precession views demonstrating prominent right ventricular outflow tract muscle bundles causing significant dephasing (arrow). C, D, and E: Cardiac magnetic resonance: sagittal steady-state free precession views demonstrating prominent right ventricular outflow tract muscle bundles without significant dephasing (arrow). C: Pericardial effusion was noted as well. LV: left ventricle, RV: right ventricle, PE: pericardial effusion.

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