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Unguarded tricuspid orifice with Uhl's malformation

  • Brojendra Agarwala (a1), J. Deane Waldman (a1) and Michele Carbone (a1)


A case is reported where unguarding of the tricuspid orifice was associated with Uhl's malformation. Echocardiographic features of the condition permitted the diagnosis during life. A therapeutic plan is proposed which involves surgical closure of the unguarded tricuspid orifice.


Corresponding author

Dr. Brojendra Agarwala, Pediatric Cardiology, University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA. Tel. 312-702-6172; Fax. 312-702-2319.


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1.Uhl, HS. Previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull John Hopkins Hosp 1952; 91: 197205.
2.Kanjuh, VI, Stevenson, JE, Amplatz, K, Edwards, JE. Congenitally unguarded tricuspid orifice with co-existent pulmonary atresia. Circulation 1964; 30: 911917.
3.Gussenhoven, EJ, Essed, CE, Bos, E. Unguarded tricuspid orifice with two-chambered right ventricle. Pediatr Cardiol 1986; 7: 175177.
4.Anderson, RH, Silverman, NH, Zuberbuhler, JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 1990; 11: 8690.
5.Muñoz, CL, Salinas, Ch, Kuri, NM, Garcia, AF. Absence of the tricuspid valve: a case report. Archivos del Instituto de Cardiología de México 1992; 62: 6167. [Spanish]
6.Zuberbuhler, JR, Allwork, SP, Anderson, RH. The spectrum of Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 77: 202211.
7.Cote, M, Davignon, A, Fouron, J-C. Congenital hypoplasia of right ventricular myocardium (Uhl's anomaly) associated with pulmonary atresia. Am J Cardiol 1973; 31: 658661.
8.Pérez Díaz, L, Jiménez, MQ, Moreno Granados, F, Pérez Martínez, V, Merino Batres, G. Congenital absence of myocardium of right ventricle (Uhl's anomaly). Brit Heart J 1973; 35: 570572.
9.Gerlis, LM, Schmidt-Ott, SC, Ho, SY, Anderson, RH. Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia. Br Heart J 1993; 69: 142150.
10.Waldman, JD, Karp, RB, Lamberti, JJ, Sand, M, Ruschhaupt, DG, Agarwala, B. Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections. Ann Thor Surg 1995; 59: 933941.


Unguarded tricuspid orifice with Uhl's malformation

  • Brojendra Agarwala (a1), J. Deane Waldman (a1) and Michele Carbone (a1)


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