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The trilogy of double-chambered right ventricle, perimembranous ventricular septal defect and subaortic narrowing—a more common association than previously recognized

Published online by Cambridge University Press:  19 August 2008

Cameron J. B. Ward ,
J. A. Gordon Culham ,
Michael W. H. Patterson  and
George G. S. Sandor
Cameron J. B. Ward
Affiliation:
From the Division of Paediatric Cardiology and the Department of Radiology, The University of British Columbia, Vancouver
J. A. Gordon Culham
Affiliation:
From the Division of Paediatric Cardiology and the Department of Radiology, The University of British Columbia, Vancouver
Michael W. H. Patterson
Affiliation:
From the Division of Paediatric Cardiology and the Department of Radiology, The University of British Columbia, Vancouver
George G. S. Sandor*
Affiliation:
From the Division of Paediatric Cardiology and the Department of Radiology, The University of British Columbia, Vancouver
*
Dr. George G. S. Sandor, 4480 Oak Street, Room 1C49, Vancouver, B.C. V6H 3V4, Canada. Tel.(604) 875-2345.
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Abstract

The records of nine patients with double-chambered right ventricle, ventricular septal defect and subaortic narrowing were reviewed to clarify the natural history of the components of this pathological combination. All nine patients presented with heart failure related to a perimembranous ventricular septal defect, which underwent spontaneous reduction in eight and closed in one. Echocardiographic diagnoses of double-chambered right ventricle and subaortic narrowing were delayed in eight of the nine cases. The ratio of the distances from the pulmonary valve to moderator band/tricuspid valve annulus (the displacement index) was less than one in all patients. Subaortic narrowing was always discrete and was present at the diagnosis of double-chambered right ventricle. The subaortic narrowing was always recognized echocardiographically, and was seen in eight of nine patients at angiography. The subaortic gradient was 0–20 mm Hg and the subpulmonary gradient 0–110 mm Hg at catheterization. Five patients demonstrated aortic regurgitation. Subaortic narrowing should be diligently sought among patients with double-chambered right ventricle, and vice versa, even in the absence of a ventricular septal defect which may have closed. Both the subaortic narrowing and subpulmonary obstruction may progress, but probably at different rates. Patients with a perimembranous ventricular septal defect, particularly with a displacement index of less than one, should be monitored carefully for evolving subpulmonary obstruction, subaortic narrowing and aortic regurgitation. The subpulmonary obstruction should be resected at the time of any other surgical intervention. The indications for resection of the subaortic narrowing remain ill-defined.

Keywords

Double-chambered right ventriclesubaortic narrowing
Type
Original Articles
Information
Cardiology in the Young , Volume 5 , Issue 2 , April 1995 , pp. 140 - 146
Copyright
Copyright © Cambridge University Press 1995

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