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June 1965

Isolated Free-Wall Hypoplasia of the Right Ventricle

Author Affiliations

From the Children's Hospital and the Children's Hospital Research Foundation, the departments of pediatrics and pathology of the University of Cincinnati College of Medicine, and the Department of Pediatrics of The Johns Hopkins University School of Medicine. Associate Pathologist and Assistant Professor of Pediatrics and Pathology (Dr. Perrin), and Assistant Professor of Pediatrics, The Johns Hopkins University School of Medicine (Dr. Mehrizi).

Am J Dis Child. 1965;109(6):558-566. doi:10.1001/archpedi.1965.02090020560011

IN 1952, Henry S. M. Uhl published observations of a case of focal fibrosis or muscular hypoplasia of the right ventricle in a 7-month-old child.30 Possibly related to this, if only grossly, are: Osler's case of "parchment heart,"27 reports of coronary insufficiency with or without healed myocardial infarct,7 congenital ventricular aneurysms, healed or healing myocarditis,8 and atypical extreme degrees of congenital endocardial fibrosis. Since Uhl's report, only three similar observations have been published.2,20,23,28 In these case reports, we offer morphologically proved cases of partial fibrous replacement of the right ventricular myocardium. In one instance, two brothers of the patient had died in infancy with morphological abnormalities of the heart. These are the fourth and fifth cases of "Uhl's anomaly" with autopsy and the second with a "positive" family history. The second case is an elaboration of a previously recorded but briefly noted example.28 Two references2,20 record the same case

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