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Natural history of ventricular septal defect with right-sided aortic arch
  1. P. J. Varghese,
  2. J. R. Allen,
  3. G. C. Rosenquist,
  4. R. D. Rowe
  1. Department of Pediatrics, The Johns Hopkins University School of Medicine, and Cardiac Clinic, Children's Medical and Surgical Center, The Johns Hopkins Hospital, Baltimore, Maryland, U.S.A.


    The natural history of 14 patients with ventricular septal defect and right-sided aortic arch is reviewed. Patients are divided into two groups. Group A consisted of 10 patients, all of whom developed progressive infundibular stenosis; group B consisted of 4 patients, none of whom developed infundibular stenosis. The clinical features, x-rays, electrocardiograms, haemodynamic data, and angiocardiograms in these two groups are compared.

    The right ventricular cine-angiocardiogram seems to be helpful in differentiating the course of the two groups. The patients who developed progressive infundibular stenosis showed an abnormal right ventricular cine-angiocardiogram even in the absence of obstruction. Various aetiological factors for the development of infundibular stenosis are discussed.

    The importance of medical management in group A patients is emphasized, even though all of them presented in infancy in congestive heart failure. The role of propranolol in preventing cyanotic spells in group A patients is also discussed.

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    • 1 Supported by United States Public Health Service grants.

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