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Tricuspid and pulmonary atresia with coarctation of the aorta: a rare combination possibly explained by persistence of the fifth aortic arch with a systemic-to-pulmonary arterial connection

  • Robert M. Freedom
    Correspondence
    Correspondence to: R.M. Freedom, M.D., The Hospital for Sick Children, 555 University Avenue, Division of Cardiology, Room 1503, Toronto, Ontario, Canada, M5G 1X8.
    Affiliations
    Departments of Pathology and Pediatrics, The University of Toronto Faculty of Medicine, and the Division of Cardiology, Canada

    Department of Pathology, the Hospital for Sick Children, Toronto, Canada
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  • Meredith Silver
    Affiliations
    Department of Pathology, the Hospital for Sick Children, Toronto, Canada
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  • Haruo Miyamura
    Affiliations
    Department of Thoracic and Cardiovascular Surgery, Niigata University School of Medicine, Niigata, Japan
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      Abstract

      A ten-day old baby was found to have tricuspid and pulmonary atresia, and a “ductal-dependent” pulmonary circulation that was prostaglandin-sensitive. An unusual coarctation of the aorta was not recognised until an autopsy was performed, some 11 days after construction of an aortopulmonary shunt. A previously undefined anomaly in this neonate was persistence of the fifth aortic arch with a systemic-to-pulmonary artery connection on the same side as the definitive aorta. The histology of the fifth aortic arch is consistent with the pattern of an arterial duct.

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