This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by FRIEDMAN, W. F. Articles by PUSCH, A. L. Search for Related Content PubMed PubMed Citation Articles by FRIEDMAN, W. F. Articles by PUSCH, A. L.

(Circulation. 1965;32:35.)
© 1965 American Heart Association, Inc.

---

Multiple Muscular Ventricular Septal Defects

WILLIAM F. FRIEDMAN M.D.¹; ALI MEHRIZI M.D.¹; ALLEN L. PUSCH M.D.¹

¹ From the Department of Pediatrics, The Harriet Lane Home Cardiac Clinic and the Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland.

Defects in the muscular portion of the interventricular septum are frequently multiple. Although their incidence is significant, scant attention has been paid them as a distinct entity. This report concerns seven patients with multiple muscular ventricular septal defects seen between 1956 and 1962. The patients ranged in age from 4 months to 9 years; five were less than 2 years old. Symptoms and signs of growth retardation, failure to thrive, and marked cardiac decompensation were present in each case. Electrocardiographic evidence of combined ventricular hypertrophy and incomplete right bundle-branch block was noted in each instance. Right heart catheterization demonstrated marked pulmonary hypertension of the high-flow, low-resistance type. These routine studies could not distinguish multiple muscular ventricular septal defects from the more usual membranous septal defect. All patients had cardiac surgery. There was one survivor. In three instances difficulty in identifying all of the multiple fenestrations prevented complete surgical repair. Postmortem examination was done in six patients. The sievelike appearance of the muscular ventricular septum was the most striking gross finding in the majority of the cases. Histologic analysis of the pulmonary vascular structural alterations did not reveal marked changes in any of the cases.

In contrast to the widespread belief that most isolated muscular septal defects are innocuous, these seven examples of multiple muscular defects were associated with a picture of severe heart disease. Every effort should be made to diagnose the lesion prior to surgery. Recognition of the multiple nature of the defect is a necessary preliminary to successful therapy. It is hoped that preoperative left heart angiocardiography will successfully demonstrate the multiple lesions.

This article has been cited by other articles:

				N. M. Ammash and C. A. Warnes Ventricular Septal Defects in Adults Ann Intern Med, November 6, 2001; 135(9): 812 - 824. [Abstract] [Full Text] [PDF]

				F. Seddio, V. M. Reddy, D. B. McElhinney, W. Tworetzky, N. H. Silverman, and F. L. Hanley MULTIPLE VENTRICULAR SEPTAL DEFECTS: HOW AND WHEN SHOULD THEY BE REPAIRED? J. Thorac. Cardiovasc. Surg., January 1, 1999; 117(1): 134 - 140. [Abstract] [Full Text] [PDF]

				T. Kitagawa, L. A. Durham III, R. S. Mosca, and E. L. Bove Techniques and results in the management of multiple ventricular septal defects J. Thorac. Cardiovasc. Surg., April 1, 1998; 115(4): 848 - 856. [Abstract] [Full Text] [PDF]