Hostname: page-component-586b7cd67f-rcrh6 Total loading time: 0 Render date: 2024-11-26T15:16:23.099Z Has data issue: false hasContentIssue false
  • English
  • Français

Absence of the left ventricular myocardium in a newborn

Published online by Cambridge University Press:  19 August 2008

Angelika Lindinger ,
Yvonne Masur  and
Hans-Gerhard Limbach
Angelika Lindinger*
Affiliation:
From the Departments of Pediatric Cardiology and Pathology, the University of Hamburg/Soar
Yvonne Masur
Affiliation:
From the Departments of Pediatric Cardiology and Pathology, the University of Hamburg/Soar
Hans-Gerhard Limbach
Affiliation:
From the Departments of Pediatric Cardiology and Pathology, the University of Hamburg/Soar
*
Priv.-Doz. Dr. Angelika Lindinger, Abt. Pädiatrische Kardiologie, Universitätskliniken, D-66421 Homburg/Saar, Germany. Tel. 49 6841 16 8306; Fax. 49 6841 16 8330.
Get access Rights & Permissions [Opens in a new window]

Summary

Absence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.

Keywords

Uhl's anomaly of the left ventricleabsence of left ventricular myocardiumnewborn
Type
Brief Reports
Information
Cardiology in the Young , Volume 6 , Issue 4 , October 1996 , pp. 344 - 347
Copyright
Copyright © Cambridge University Press 1996

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.UhlHSM. A previously undescribed 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
2.Salazar, J, Alonso-Lej, F, Plaza, L, Gutiérrez, A, Garcia, MD, Ibarra, F, Felipe, J. Almost total absence of the left ventricular myocardium with dextro-transposition of the great arteries. Pediatr Cardiol 1986; 6: 283–285.Google ScholarPubMed
3.Vecht, RJ, Carmichael, DJS, Gopal, R, Philip, G. Uhl's anomaly. Br Heart J 1979; 41: 676–682.CrossRefGoogle ScholarPubMed
4.Anderson, RH, Becker, AE. Cardiac Anatomy. Gower Medical Publishing, London, 1980.Google Scholar
5.Allmann, J, Keiser, G, Schneider, J. Linksventrikuläre arrhyth-mogene Dysplasie.Schweiz Med Wschr 1993; 123:1615–1619.Google Scholar
6.Miani, D, Pinamonti, B, Bussani, R, Silvestri, F, Sinceinagra, G, Camerini, F. Right ventricular dysplasia: a clinical and patho logical study of two families with left ventricular involvement. Br Heart J 1993; 69: 151–157.CrossRefGoogle Scholar
7.Pinamonti, B, Sinagra, G, Salvi, A, Di Lenarda, A, Morgera, T, Silvestri, F, Bussani, R, Camerini, F. Left ventricular involve-ment in right ventricular dysplasia. Am Heart J 1992; 123: 711–724.CrossRefGoogle Scholar
8.Bharati, S, Ciraulo, DA, Bilitch, M, Rosen, KM, Lev, M. Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease. Circulation 1978; 57: 636–644.CrossRefGoogle ScholarPubMed
9.Tabib, A, Loire, R: Etude anatomo-clinique de 100 cas d'hypoplasie du muscle ventriculaire droit (dont 89 morts subites inattendues). Arch Mal Coeur 1992; 85: 1789–1795.Google Scholar
10.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: 142–150CrossRefGoogle Scholar