Asian Cardiovasc Thorac Ann 1999;7:111-114
© 1999 Asia Publishing EXchange Pte Ltd
Double-Outlet Right Ventricle Coexisting With Double-Chambered Right Ventricle
Manoj K Agarwala, MD,
Mandeep Singh, MD,
Anil Grover, MD,
Jagmohan S Varma, MD,
Vijay Pathak, MD
Department of Cardiology
Postgraduate Institute of Medical Education and Research
Chandigarh, India
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For reprint information contact: Anil Grover, MD, DM Tel: 91 172 74 7585 Fax: 91 172 74 4401 Deptartment of Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
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Abstract
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Of 60 consecutive patients with double-chambered right ventricle studied prospectively during a 42-month period, 7 had coexisting double-outlet right ventricle. There were 5 males and 2 females aged 4 to 20 years old. The clinical profile was similar to that of patients with tetralogy of Fallot. Echocardiography is recommended for the diagnosis of this condition.
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Introduction
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The combination of double-outlet right ventricle (DORV) with obstructive anomalous muscle bundles in the right ventricle, also called double-chambered right ventricle (DCRV), is a rare occurrence that has been described previously in a few case reports.1–3 In this study, we present the clinical, hemodynamic, and angiographic data of 7 patients with this combination and describe the associated anomalies.
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Patients and Methods
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Sixty consecutive patients with DCRV were studied prospectively during a 42-month period between 1994 and 1997. Seven of these 60 patients were found to have coexisting DORV and underwent clinical, electro-cardiographic, echocardiographic, hemodynamic, and angiographic evaluations. The diagnosis of DCRV + DORV was based on the following criteria: pressure gradients between the right ventricular inflow and outflow areas; demonstration of anomalous muscle bundles by right ventricular angiography; right ventricular origin of both great arteries; and an absence of aortic-mitral or mitral-pulmonary continuity. There were 5 males and 2 females aged 4 to 20 years old (mean, 12.4 ± 6 years). DCRV was not suspected clinically in any of these patients. All patients had cyanosis and breathlessness on exertion as the presenting symptoms. On examination, 5 patients had a grade 5/6 ejection systolic murmur and the other 2 had a grade 3/6 ejection systolic murmur at the 2nd and 3rd left intercostal spaces. One patient also had an early diastolic murmur of aortic regurgitation. The clinical diagnosis was tetralogy-like physiology in all patients. One of the patients had received a standard Blalock-Taussig shunt in childhood.
Detailed two-dimensional and Doppler echocardiographic examinations were performed using Sonos 1500 equip-ment (Hewlett Packard, Inc., Anaheim, CA, USA). Echo-cardiographic evaluation was not possible in 2 patients because of inadequate echocardiographic window. Right and left heart catheterization was carried out in all 7 patients. Pressure gradients were recorded by withdrawal tracings using an end-hole catheter. Right ventricular angiograms were carried out in anteroposterior and lateral views. Left ventricular angiograms and aortograms were performed in left anterior oblique projections.
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Results
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In the 5 patients in whom a detailed echocardiographic study could be performed, both great vessels were seen to arise from the morphological right ventricle with the aorta anterior and to the right, and all had aortic-mitral discontinuity (Table 1
). All of these 5 patients had a large subaortic ventricular septal defect, narrowed infundibulum, and small confluent pulmonary arteries. In one patient, additional multiple muscular ventricular septal defects were observed. Parasternal short-axis view at the aortic valve level clearly revealed an anomalous muscle bundle with flow acceleration on pulsed-wave Doppler with the first appearance of a color mosaic across it. The ventricular septal defect invariably opened into the proximal high-pressure chamber.
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Table 1. Clinical, Echocardiographic, Hemodynamic Data, and Associated Anomalies of Patients With Double-Outlet Double-Chambered Right Ventricle
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All 7 patients had abnormal electrocardiograms with the frontal QRS axis to the right. Right ventricular hypertrophy was seen in all 7 and one had a counterclockwise loop. Upright T waves in the right precordial leads were not found in any patient. Plain chest radiographs were of little additional benefit in the diagnosis of DCRV. All patients had situs solitus. Cardiac size was normal, except in one patient who had aortic regurgitation. Pulmonary vascularity was either normal or decreased.
Associated anomalies are summarized in Table 1
. Ventricular septal defect was considered to be a part of DCRV. Atrial septal defects were found in 2 patients. Valvular and supravalvular pulmonary stenosis was seen in 2 patients. A coronary artery anomaly where a single coronary artery arose from the left coronary sinus was present in one patient. Mild aortic regurgitation was detected in 2 patients (28.5%). During right heart catheterization, the pulmonary valve could not be crossed in 2 patients who had severe right ventricular outflow obstruction. There was no gradient across the pulmonary valve in the other 5 patients and pulmonary artery pressures were normal. On withdrawal of the catheter in the body of the right ventricle, a gradient was obtained in all 7 patients.
Right ventricular angiograms in the anteroposterior and lateral view showed both of the great vessels arising from the morphological right ventricle (Figure 1). A thick infundibular septum separated the two great arteries and there was infundibular narrowing in all cases. The pulmonary valve was thickened and domed in 2 patients and 2 had peripheral pulmonary artery stenosis. Anomalous muscle bundles were best seen as tilling defects in systolic frames in the anteroposterior view. These bundles were observed to run horizontally from the ventricular septum to the tricuspid orifice. Left ventriculograms showed aortic-mitral discontinuity and large subaortic ventricular septal defects. Aortic root angiography in one patient showed a single coronary artery originating from the left sinus of Valsalva. Two of the patients underwent surgery and the preoperative findings were confirmed. Postoperative echocardiography showed no gradient across the anomalous muscle bundles.
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Figure 1. Right ventricular angiogram in anteroposterior view showing anomalous bundles crossing the right ventricular cavity. Both the aorta and pulmonary artery are seen in the angiogram. The infundibular septum is seen as a "tear drop". Small arrows indicate the anomalous muscle bundle and the large arrow indicates the infundibular septum.
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Discussion
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Double-outlet right ventricle encompasses features of a variety of clinical entities such as simple ventricular septal defect, tetralogy of Fallot, or transposition of the great arteries. Van Praagh and colleagues4 relied on subaortic and subpulmonary conus for diagnosis of DORV. All patients in our study had aortic-mitral discontinuity. The obstruction of flow into the pulmonary arteries from the right ventricle in DORV is usually at the infundibular or pulmonary valve levels.5 Obstruction in the right ventricular cavity due to anomalous muscle bundles in DORV has been described and has become increasingly recognized as a distinct angiographic entity.6 The clinical recognition of DCRV is difficult because a variety of other defects are associated with this anomaly. The variability in presentation is due to differences in the associated lesions. Beitzke and colleagues3 found DORV associated with subpulmonic ventricular septal defect and a band in the right ventricle in one of 2 autopsied cases. The distal chamber of the right ventricle communicated with the left ventricle via a subpulmonary ventricle septal defect. In their other case, the distal chamber supported the aorta and the pulmonary artery arose from the proximal chamber. The distal right ventricular chamber resembled the rudimentary chamber seen in univentricular hearts. Judson and colleagues1 described 2 cases of DORV with anomalous muscle bundles, which were successfully treated surgically.
In a recent study, Cil and colleagues7 reported that 4 of 52 patients (8%) with DCRV had associated DORV. The incidence in our study was 11.7%. Four of our 7 patients presented in their second decade. An important difference of this combination from the usual variety of DCRV is the presence of severe infundibular narrowing. In cases of DCRV, absence of infundibular hypoplasia is an accepted diagnostic criterion but in all our cases of DORV with DCRV, infundibular hypoplasia of the pulmonary annulus and central pulmonary arteries was present.
The combination of DORV with DCRV is being recognized with increasing frequency. Identification of anomalous muscle bundles is possible by two-dimensional and Doppler echocardiography and confirmation of DCRV in patients with DORV is of immense surgical importance.
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References
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Judson JP, Danielson GK, Ritter DG, Hagler DJ. Successful repair of coexisting double outlet right ventricle and two chambered right ventricle. J Thorac Cardiovasc Surg 1982;84:113–21.[Abstract]
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Hindle WV, Engle MA, Hanstrom JWC. Anomalous right ventricular muscles. A clinicopathologic study. Am J Cardiol 1968;21:487–95.[Medline]
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Beitzke A. Anderson RH, Wilkinson JL, Shinebourne EA. Two chambered right ventricle simulating two chambered left ventricle. Br Heart J 1979;42:22–6.[Abstract/Free Full Text]
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Van Praagh S, Davidoff A, Chin A, Shiel FS, Reynolds J, Van Praagh R. Double outlet right ventricle: anatomic types and developmental implications based on a study of 101 cases. Coeur (Paris) 1982;12:389–439.
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Venables AW, Campbell PE. Double outlet right ventricle. A review of 16 cases with 10 necropsy specimens. Br Heart J 1966;28:461–71.[Free Full Text]
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Gale GE, Heiman KW, Barlon JB. Double chambered right ventricle: a report of five cases. Br Heart J 1969;31:291–7.[Free Full Text]
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Cil E, Saraclar M, Ozkutlu S, Ozme S, Bilgic A, Ozer S, et al. Double chambered right ventricle: experience with 52 cases. Int J Cardiol 1995;50:19–29.[Medline]
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Abstract
Introduction
