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We present a rare case of a double-chambered right ventricle masquerading as a double-chambered left ventricle, which was found incidentally on cardiac imaging in an adult female patient with atypical chest pain. The most common form of double-chambered right ventricle is characterized by compartmentalization of the right ventricle by muscular bands into 2 distinct chambers. The main features of this malformation are a pressure gradient between the 2 compartments, and the frequent (up to 90%) association with a membranous ventricular septal defect. In our case, the muscular band dividing the right ventricle was located in the inferoseptal part of the latter, creating a diminutive cavity that had no communication with the main right ventricle but communicated with the left ventricle creating the false impression of a double-chambered left ventricle. This constitutes a rare variant of double-chambered right ventricle with unknown clinical implications.

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A 40-year-old man with previous cardiac surgery was referred to the adult congenital heart disease department at our hospital because of progressive dyspnoea during the previous 3 months. He had had successful pulmonary valvotomy when he was 9 because of a congenital pulmonary stenosis. As the initial approach in a patient with suspected heart failure (HF), a transthoracic echocardiography was performed in which a high-pressure gradient was measured by Doppler …

Double-outlet right ventricle (DORV) and two-chambered right ventricle (TCRV) are both uncommon cardiac malformations; even more rare is the coexistence of these lesions. This communication describes the first two patients in whom this unusual combination of anomalies was recognized during life and successfully repaired with the aid of extracorporeal circulation. Late results in both patients are excellent.

A 34 year old woman with a history of five spontaneous abortions and a systemic lupus-like illness underwent cardiotomy for resection of a calcified right ventricular thrombus. At operation an anomalous right ventricular muscle bundle was discovered under the calcified thrombus. Clinical chemistry showed lupus anticoagulant activity and cardiolipin antibodies in the plasma.

Two cases are described of a most unusual variant of two-chambered right ventricle. In both the ventricular septal defect was between the distal chamber of the right ventricle and the left ventricle. However the extensive dividing 'septum' between proximal and distal parts of the right ventricle converted the latter, haemodynamically, into part of the left ventricle. In the first case the distal chamber supported the aorta in the left anterior position, the pulmonary artery arising from the proximal part of the right ventricle. In the second the pulmonary artery arose from the distal chamber and the aorta from the proximal chamber. Though in both the ventriculoarterial connection was double outlet right ventricle, functionally there was arterial concordance in case 1 and discordance in case 2. A further disconcerting feature was the resemblance of the distal right ventricular chamber to the rudimentary chamber of a univentricular heart of left ventricular type.

A 19 day old infant in extremis with congestive failure was shown to have a two-chambered right ventricle, ventricular septal defect and tricuspid regurgitation. There have been previous reports of patients with two-chambered right ventricle in whom an anomalous muscle bundle obstructed outflow of the right ventricle, creating a tetralogy-like function. By contrast, in this patient there was a partitioned off high-pressure apical chamber secondary to hypertrophy of an abnormally oriented septal band of the crista supraventricularis and moderator band. The infant was resuscitated, and over several days improved remarkably with medical management. Digitalis was discontinued at age 6 months, and at the age of 1 year there were no significant cardiac findings. Cardiac catheterization confirmed spontaneous closure of the ventricular septal defect; the high-pressure, apical chamber was absent, and there was no tricuspid regurgitation.

A relatively rare congenital cardiac malformation is described in nine patients and is characterized by aberrant hypertrophied muscular bands that divide the right ventricular cavity into two chambers. These hypertrophied muscular bands produce an effective stenosis and obstruct the outflow of blood from the right ventricle. An interventricular septal defect is usually seen in association with this abnormality. This cardiac malformation is anatomically distinct from the classic tetralogy of Fallot but may be mistakenly diagnosed as an acyanotic tetralogy of Fallot or an interventricular septal defect. The use of selective angiocardiography in conjunction with right-sided cardiac catheterization will establish the correct diagnosis. This congenital lesion is amenable to surgical correction, and five of the patients in this series were successfully operated upon with the aid of extracorporeal circulation. Surgery has been recommended for the remaining four patients because of the encouraging results.

Hearts with ventricular septal defect may undergo division of the right ventricle into two chambers. Recent observations indicate that this division can be caused by congenital, aberrant, hypertrophie muscle bundles that cross the main channel of the ventricular cavity. The degree of obstruction presents a continuum of from nil to severe. Clinical, hemodynamic, angiocardiographic and postmortem data are described in a patient who not only had ventricular septal defect with a “two-chambered right ventricle”, but in whom shunt operations apparently permitted survival after complete closure of the pathway connecting the two chambers.

Catheterization and anatomical data are presented of an infant with cyanosis due to a most unusual variant of unguarded tricuspid valve orifice in association with a two-chambered right ventricle. The inlet portion of the right ventricle ended blindly and was completely separated from the outlet portion by a muscular wall. The outlet chamber, which supported the pulmonary trunk, received its blood from the left ventricle through an outlet foramen. The condition is considered to be related to imperforate Ebstein's anomaly.

Seven patients with anomalous right ventricular muscle bundle are presented to emphasize possible pitfalls in the clinical diagnosis as based on physical examination, chest x ray, and electrocardiogram. Echocardiography, on the other hand, revealed abnormalities in all four of the patients on whom the study was done. The echocardiogram was directly responsible for a change in diagnosis and referral for a cardiac catheterization in one patient who had been followed for 19 years with the clinical diagnosis of a small ventricular septal defect. Four of the seven patients with anomalous right ventricular muscle bundle had extracardiac anomalies, one girl with an absent left thumb, and three patients, one girl and two boys, with left-sided renal anomalies. This high incidence of renal anomalies has not been previously reported in clinical or postmortem series.