Combined double-chambered right ventricle with double-chambered left ventricle: a rare anomaly

Abstract

ABSTR ACTDouble-chambered right ventricle with double-chambered left ventricle is a very rare congenital anomaly. An 18-year-old female presented with dyspnea, chest pain, and palpitation. Diagnosis was made using transthoracic echocardiography and was confirmed by angiography. Surgical excision of the anomalous muscle bundles in the right ventricular outflow tract along with excision of the fibrous band from the left ventricle was performed. The postoperative course was uneventful and repeated echocardiogram before discharge showed a right ventricular outflow tract gradient of 8 mmHg, no gradient across the left ventricular cavity, and improved biventricular functions. The management strategy is dependent on presence of symptom, associated anomalies and type of pathology.Keywords: Angiography; double-chambered left ventricle; double-chambered ventricle; echocardiography; infundibular stenosis; subaortic stenosis. Double-chambered ventricle is a rare congenital cardiac anomaly, where the ventricular chamber is wholly or partially partitioned usually by abnormal muscular ridges or fibrosis.[1-9] Unlike double-chambered right ventricle (DCRV), division of the left ventricle (LV) is a rare anomaly. [1][2][3][4][5] Previously, the description of the double-chambered left ventricle (DCLV) was given by Gerlis et al. [1] and Kay et al. [2] Herein, we describe a case of DCRV and DCLV in an 18-year-old female. CASE REPORTAn 18-year-old female patient presented with dyspnea, chest pain, and palpitation. The physical examination revealed a Grade 4/6 ejection systolic murmur at the left upper sternal border. Chest X-ray showed a cardiothoracic ratio of 50% with reduced pulmonary blood f low. Electrocardiogram showed right ventricular hypertrophy. Transthoracic echocardiography revealed levocardia and DCRV with a gradient of 70/58 mmHg associated with a ledge of muscle tissue in the LV cavity below the mitral valve (Figure 1a) with 30 mmHg gradient across the constriction with figure of 8 appearance in apical 4 chamber view. No regional wall motion abnormality and improved biventricular functions were observed. Cardiac catheterization revealed a right atrial pressure of 21/0 mmHg with a mean of 10 mmHg, a right ventricular pressure of 157/10 mmHg, a right ventricular outf low tract of 31/10 mmHg, a pulmonary artery of 33/11 mmHg, a pulmonary artery wedge of 17/6 mmHg, a LV apex of 162/11 mmHg, a LV outf low tract of 152/76 mmHg, an aortic pressure of 141/83 mmHg, and a femoral artery pressure of 157/77 mmHg. The gradient between the right ventricular body and the outf low tract was 126 mmHg, while the gradient across the fibrous ridge between the LV apex and outf low was 10 mmHg. Left ventricular angiography showed a ledge of tissue in the mid-LV cavity below the mitral valve (Figure 1b), separating the LV with a hypertrophied distal chamber and thin-walled proximal chamber. Right ventricular angiography revealed the presence of anomalous septal and parietal bands, producing DCRV with a good-sized main pulmonary artery and c...