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Clinicopathological correlation—pulmonary atresia with imperforate tricuspid valve and aneurysmal dilatation of ascending aorta

Published online by Cambridge University Press:  19 August 2008

Rafael Hirsch ,
Leon Gerlis  and
Jane Somerville
Rafael Hirsch*
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
Leon Gerlis
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
Jane Somerville
Affiliation:
From the Grown-Up Congenital Heart Unit and the Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, London
*
Dr. Rafael Hirsch, Department of Cardiac Morphology, Royal Brompton National Heart and Lung Hospitals, Sidney Street, London, United Kingdom.
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Abstract

A patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.

Keywords

Pulmonary atresia with intact ventricular septumimperforate tricuspid valveMarfan-like diseasesingle coronary arteryhypoplastic pulmonary arteries
Type
Clinico-Pathological Correlation
Information
Cardiology in the Young , Volume 3 , Issue 3 , July 1993 , pp. 220 - 224
Copyright
Copyright © Cambridge University Press 1993

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References

1.Warnes, CA, Somerville, J. Tricuspid atresia in adolescents and adults: current state and late complications. Br Heart J 1986; 56: 535–543.CrossRefGoogle ScholarPubMed
2.Freedom, RM, Wilson, G, Trusler, G, Rowe, RD. Pulmonary atresia and intact ventricular septum: A review of the anatomy, myocardium and factors influencing right ventricular growth and guidelines for surgical intervention. Scand J Thorac Card Surg 1983; 17: 1–28.Google Scholar
3.Freedom, RM, Dische, MR, Rowe, RD. The tricuspid valve in pulmonary atresia with intact ventricular septum. A morpho logical study of 60 cases. Arch Pathol Lab Med 1978; 102: 28–31.Google Scholar
4.Zuberbuhler, JR, Anderson, RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281–288.CrossRefGoogle ScholarPubMed
5.Bull, C, de Leval, MR, Mercanti, C, Macartney, FJ, Anderson, RH. Pulmonary atresia and intact ventricular septum; a re vised classification. Circulation 1982; 66: 266–272.CrossRefGoogle Scholar
6.O'Connor, WN, Stahr, BJ, Cotrrill, CM, Todd, EP, Noonan, JA. Ventriculocoronary connections in hypoplastic right heart syndrome: autopsy serial section study of six cases. J Am Coil Cardiol 1988; 11: 1061–1072.CrossRefGoogle ScholarPubMed
7.Pyeritz, RE, McKusick, VA. The Marfan syndrome: Diagnosis and management. New Eng J Med 1979; 300: 772–777.CrossRefGoogle ScholarPubMed
8.Atkins, DA, Marvin, WJ Jr, Clark, EB. Foramen ovaleatrial septum area ratio: a marker of transatrial blood flow. Circulation 1982; 66: 281–283.CrossRefGoogle ScholarPubMed