Hypoplastic right heart syndrome

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Hypoplastic right heart syndrome
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Right Ventricle Hypoplasia: Gross natural color view from right atrium showing patent foramen ovale and very small tricuspid valve.
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Hypoplastic right heart syndrome is a congenital heart defect in which the right ventricle of the heart fails to grow and develop appropriately.[1] [2] [3] [4] [5] [6] The underdeveloped ventricle cannot contain the proper amount of blood pumped from the right atrium. The ventricle’s muscle structure is poor, so additional problems are encountered as the heart attempts to pump blood to the pulmonary valve for transfer to the lungs. Hypoplastic right heart syndrome needs immediate and emergency treatment, as the heart begins to fail almost immediately after birth. An inadequate blood supply to the lungs means a poor return of oxygenated blood to the body, and the muscle in the ventricle will exhaust easily.

Pathophysiology

Gross Pathology

Images shown below are Courtesy of Professor Peter Anderson DVM PhD and Published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

References

  1. Riemenschnelder T.A., Vincent W.R., Ruttenberg H.D., Desilets D.T., Transposition of the Great Vessels with Hypoplasia of the Right Ventricle. Circulation, Volume 38, August 1968, 386-402
  2. Kessler, A., Adams, P.: Association of transposition of the great vessels and rudimentary right ventricle, with and without tricuspid atresia. Pediatrics 19: 851, 1957.
  3. Enthoven, R., Dunst, M., Richman, B.: Congenital hypoplasia of the right ventricle and tricuspid valve with survival into adult life. Amer J Cardiol 11: 532, 1963.
  4. Sackner, M. A., Robinson, M. J., Jamison W. L., Lewis, D. H.: Isolated right ventricular hypoplasia with atrial septal defect or patent foramen ovale. Circulation 24: 1388, 1961.
  5. Morita K, Kurosawa H, Ishii S, Yoshitake M, Hanai M, Configuration of Linear Dynamic Cardiomyoplasty for Hypoplastic Right Ventricle Ann Thorac Surg 1997;63:676-82
  6. J. C. Chachques, P. G. Argyriadis, G. Fontaine, J.-L. Hebert, R. A. Frank, N. D'Attellis, J.-N.o. Fabiani, A. F. Carpentier Right ventricular cardiomyoplasty: 10-year follow-up Ann. Thorac. Surg., May 1, 2003; 75(5): 1464-68.

Additional Reading

  • Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843
  • Hurst's the Heart, Fuster V, 12th ed. 2008, ISBN 978-0-07-149928-6
  • Willerson JT, Cardiovascular Medicine, 3rd ed., 2007, ISBN 978-1-84628-188-4



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