J Thorac Cardiovasc Surg 1994;108:793-794
© 1994 Mosby, Inc.
Double-chambered right ventricle associated with left ventricular dysfunction
Klaus Langes, MD
Volker Siglow
Dietmar Koschyk, MD,
Detlef G. Mathey, MD
Medical Clinic
Department of Cardiology
University Hospital Eppendorf
D-20246 Hamburg, Germany
To the Editor:
A 31-year-old man was admitted because of orthopnea (New York Heart Association functional class IV) with progression of symptoms for the past 8 years. On physical examination the apical impulse was displaced to the anterior axillary line in the sixth intercostal space and was enlarged. The second heart sound was widely split. He had marked peripheral edema and chronic dermatosis of the lower legs. The electrocardiogram showed atrial fibrillation and right bundle branch block with poor R-wave progression in the precordial leads.
Transthoracic echocardiography revealed an additional right ventricular chamber, located below and in front of the left ventricle. It was separated from the hypertrophied right ventricle by a permeable, thick, septumlike structure (Figs. 1 and 2), creating an additional right ventricular chamber. The additional intraventricular muscular mass was located adjacent to the moderator band, low in the body of the right ventricle. The double right ventricle wrapped around the left ventricle, which was ball-shaped and appeared to be hypoplastic. On M-mode echocardiography the left atrial diameter was 75 mm and the right atrial 50 mm. Transesophageal echocardiography demonstrated spontaneous echo contrast (smoke) in the markedly dilated left atrium. The additional right ventricle measured 4.9 X 1.4 cm on transesophageal echocardiography (Fig. 2). The left ventricular end-diastolic diameter was 75 mm on transthoracic echocardiography and the left ventricle was slightly hypertrophic. During right and left heart catheterization the pulmonary artery pressure was 84/54 mm Hg (mean 55 mm Hg), mean capillary wedge pressure was 26 mm Hg (with a v-wave of 30 mm Hg), right ventricular pressure was 70/5 mm Hg, and left ventricular end-diastolic pressure was 28 mm Hg. No evidence of intracardiac shunting, pulmonary stenosis, or subvalvular pulmonary stenosis was found.
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Fig. 1. Transthoracic echocardiography. A,Apical four-chamber view. B,Apical two-chamber view. LA, Left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; RV II, additional right ventricle.
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Fig. 2. Transesophageal echocardiography. Spontaneous echo contrast (smoke) in the markedly dilated left atrium. The additional right ventricle was 4.9x 1.4 cm. LA, Left atrium; LV, left ventricle; RA, right atrium; RV,right ventricle; RV II, additional right ventricle.
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Contrast cineangiography demonstrated a ball-shaped left ventricle with markedly impaired wall motion. The ejection fraction was 25%. The regular right ventricle was dilated and hypertrophied and had normal wall motion. The additional chamber was immediately filled with contrast medium. It was trabecularized and located ventrocaudally to the left ventricle. Washout from the additional ventricle to the right ventricle was delayed and sluggish. Right ventricular endomyocardial biopsy specimens revealed both hypertrophy and atrophy and a moderate degree of interstitial fibrosis, consistent with dilative cardiomyopathy.
The patient was placed on a regimen of digoxin (0.2 mg a day), captopril (25 mg three times daily), and furosemide (40 mg twice daily). His condition improved on this regimen to functional class II.
Comparable subinfundibular anomalies of the right ventricle were described previously as anomalous muscle bundles. Other reports have also described two-chambered right ventricles
1 or double-chambered right ventricles. 2 Other concomitant cardiac defects were noted in connection with the two-chambered right ventricle, such as obstruction of the right ventricular outflow tract, 3 ventricular septal defects, 4 aorticregurgitation, or mild subaortic stenosis. 5 That our patient did not have any concomitant malformations suggests that the additional right interventricular wall was not the result of compensatory hypertrophy caused by right ventricular pressure or volume overload. Interestingly, in contrast to the "original" right and left ventricles, the additional chamber was contracting normally. Furthermore, the double-chambered right ventricle was associated with marked left ventricular dysfunction.
References
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Hartmann AF, Tsifutis AA, Arvidsson H, et al. The two-chambered right ventricle: report of nine cases. Circulation 1962;26:279-87.[Abstract/Free Full Text]
-
Matina D, van Doesburg NH, Fouron J-C, et al. Subxiphoid two-dimensional echocardiographic diagnosis of double-chambered right ventricle. Circulation 1983;4:885-8.
-
Lucas RV, Marshall RJ, Morgan DZ, et al. Anomalous muscle bundle of the right ventricle with intact ventricular septum: a newly recognized cause of right ventricular obstruction. Circulation 1963;28:759-60.
-
Benjamin JD, Haitas B, Cassel GA, et al. Double chambered right ventricle, ventricular septal defect and aortic regurgitation: a report of 2 cases. South Afr Med J 1978;54:74-6.[Medline]
-
Baumstark A, Fellows KE, Rosenthal A. Combined double chambered right ventricle and discrete subaortic stenosis. Circulation 1978;57:299-303.[Abstract/Free Full Text]
