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Fetal diagnosis of lethal dysfunction of the right heart in three siblings

Published online by Cambridge University Press:  19 August 2008

Tim S. Hornung ,
Alison Heads ,
Chris Wright  and
Stewart Hunter
Tim S. Hornung*
Affiliation:
Department of Paediatric CardiologyFreeman Hospital, Newcastle-upon-Tyne, UK
Alison Heads
Affiliation:
Department of EchocardiographyFreeman HospitalNewcastle-upon-Tyne, UK
Chris Wright
Affiliation:
Dept of Histopathology, Royal Victoria Infirmary, Newcastle-upon-Tyne, UK
Stewart Hunter
Affiliation:
Department of Paediatric CardiologyFreeman Hospital, Newcastle-upon-Tyne, UK
*
Dr. T.S.Hornung, Adult Congenital Cardiac Centre, Royal Brompton Hospital, Sydney Street, London. SW3 6NPUK. Tel: (0)20 7351 8121; E-mail: [email protected]
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Abstract

A woman, having already delivered one child, underwent fetal echocardiography during thre subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.

Keywords

Uhl's anomalyparchment right ventriclefamilial right ventricular cardiomyopathy
Type
Original Articles
Information
Cardiology in the Young , Volume 10 , Issue 6 , November 2000 , pp. 621 - 624
Copyright
Copyright © Cambridge University Press 2000

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References

1. Allan, LD, Chita, SK, Anderson, RH, Fagg, N, Crawford, DC, Tynan, MJCoarctauon of the aorta in prenatal life: an echocardiographic, anatomical and functional study. Br Heart J 1988; 59: 356–360.CrossRefGoogle ScholarPubMed
2. Benacerraf, BR, Saltzman, DH, Sanders, SP. Sonographic signs suggesting the prenatal diagnosis of coarctation of the aorta. Jltrasound Med 1989; 8: 65–69.CrossRefGoogle ScholarPubMed
3. Uhl, NS. A previously undiagnosed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952, 91: 197–205.Google Scholar
4. Cumming, GR, Bowman, JM, Whitehead, L. Congenital aplasia of the myocardium of the right ventricle (Uhl's anomaly). Am Heart J 1985; 10: 671–676.Google Scholar
5. Gerlis, LM, Schmidt-Ott, SC, Ho, SY, Anderson, RH. Dysplastic conditions of the right ventricular myocardium' Uhl's anomaly versus arrhythmogenic right ventricular dysplasia. Br Heart J 1993; 69: 142–150.CrossRefGoogle Scholar
6. Hoback, J, Adicoff, A, From, AH, Smith, M, Shafer, R, Chesler, E.A report of Uhl's disease in identical adult twins: evaluation of right ventricular dysfunction with echocardiography and nuclear angiography. Chest 1981; 79: 306–310.CrossRefGoogle ScholarPubMed
7. Fischer, DR, Zuberbuhler, JR. Familial Uhl's anomaly. Am J Cardiol 1984; 54: 940.CrossRefGoogle ScholarPubMed
8. Digglemann, U, Baur, HR. Familial Uhl's anomaly in the adult Am J Cardiol 1984; 53: 1402–1403.CrossRefGoogle Scholar
9. Benson, CB, Brown, DL, Roberts, DJ. Uhl's anomaly of the heart mimicking Ebstein's anomaly in utero. J Ultrasound Med 1995; 14: 781–783.CrossRefGoogle ScholarPubMed
10. Wager, GP, Couser, RJ, Edwards, OP; Gmach, C, Bessinger, B Jr. Antenatal ultrasound findings in a case of Uhl's anomaly. Am J Perinatol 1988; 5: 164–167CrossRefGoogle Scholar
11. Fitchett, DH, Sugrue, DD, MacArthur, CG, Oakley, CM. Right ventricular dilated cardiomyopathy. Br Heart J 1984; 51: 25–29.CrossRefGoogle ScholarPubMed
12. Ibsen, HHW, Baandrup, U, Simonsen, EE. Familial right ventricular dilated cardiomyopathy. Br Heart J 1985; 54: 156–159.CrossRefGoogle ScholarPubMed
13. Marcus, FI, Fontaine, GH, Guiraudon, G, Frank, R, Laurenceau, JL, Malergue, C, Grosgogeat, Y. Right ventricular dysplasia: areport of 24 adult cases. Circulation 1982; 65: 384–398.CrossRefGoogle Scholar