Double-Chambered Right Ventricle
A 26-year-old pregnant woman was admitted to the hospital because of premature rupture of the membranes at gestational week 26. She had given birth to a child previously without any problems and denied ever having had symptoms of heart failure. Because a ventricular septal defect had been surgically corrected during childhood and a systolic heart murmur was now present, she was referred for transthoracic echocardiography. The interventricular septum appeared normal and did not show any shunt after ventricular septal defect closure. Significant valve abnormalities could be excluded. However, a prominent turbulent flow pattern was apparent in the middle of the right ventricle (Figure, panel A and online-only Data Supplement Movie I). Transesophageal echocardiography revealed a pressure gradient (Figure, panel B) of 21 mm Hg that resulted from a midventricular stenosis (Figure, panel C and online-only Data Supplement Movie II). Three muscular bands crossed the right ventricle from a protrusion at the free wall toward the interventricular septum (Figure, panel D). Cardiac magnetic resonance imaging recapitulated all findings (Figure, panels E and F and online-only Data Supplement Movie III) and demonstrated mild hypertrophy of the proximal right ventricle. In summary, results were consistent with a double-chambered right ventricle. One week later, the patient gave birth to a premature but healthy child without any problems.
A double-chambered right ventricle is a rare congenital anomaly. The majority of cases are diagnosed during childhood and are associated with other defects, primarily a ventricular septal defect. It does not necessarily cause symptoms, but right-sided heart failure may develop over time. Cardiac magnetic resonance imaging is the method of choice to confirm the diagnosis. Cardiac surgery should be considered when heart failure or a high midventricular gradient (>40 mm Hg) develops.1,2 A double-chambered right ventricle should be considered in all cases of abnormal Doppler flow signals in the right ventricle during echocardiographic assessment.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.154054/-/DC1.
- © 2013 American Heart Association, Inc.