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. 1979 Jun;41(6):676–682. doi: 10.1136/hrt.41.6.676

Uhl's anomaly.

R J Vecht, D J Carmichael, R Gopal, G Philip
PMCID: PMC482090  PMID: 465242

Abstract

Uhl's anomaly of the heart is a rare condition. Another well-documented case is presented with a review of the published reports outlining the main clinical features and the bad overall prognosis. Right atriotomy should be avoided if closure of the atrial septal defect is attempted.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ARCILLA R. A., GASUL B. M. Congenital aplasia or marked hypoplasia of the myocardium of the right ventricle (Uhl's anomaly). Clinical, angiocardiographic, and hemodynamic findings. J Pediatr. 1961 Mar;58:381–388. doi: 10.1016/s0022-3476(61)80269-2. [DOI] [PubMed] [Google Scholar]
  2. Aherne W. A. Uhl's anomaly. Arch Dis Child. 1973 Feb;48(2):160–160. doi: 10.1136/adc.48.2.160-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Cumming G. R., Bowman J. M., Whytehead L. Congenital aplasia of the myocardium of the right ventricle (Uhl's anomaly). Am Heart J. 1965 Nov;70(5):671–676. doi: 10.1016/0002-8703(65)90396-0. [DOI] [PubMed] [Google Scholar]
  4. Côté M., Davignon A., Fouron J. C. Congenital hypoplasia of right ventricular myocardium (Uhl's anomaly) associated with pulmonary atresia in a newborn. Am J Cardiol. 1973 May;31(5):658–661. doi: 10.1016/0002-9149(73)90339-1. [DOI] [PubMed] [Google Scholar]
  5. FISHER E. R., DAVIS E. R. [Observations concerning the pathogenesis of endocardial thickening in the adult heart]. Am Heart J. 1958 Oct;56(4):553–561. doi: 10.1016/0002-8703(58)90083-8. [DOI] [PubMed] [Google Scholar]
  6. French J. W., Baum D., Popp R. L. Echocardiographic findings in Uhl's anomaly. Demonstration of diastolic pulmonary valve opening. Am J Cardiol. 1975 Sep;36(3):349–353. doi: 10.1016/0002-9149(75)90488-9. [DOI] [PubMed] [Google Scholar]
  7. Froment R., Perrin A., Loire R., Dalloz C. Ventricule droit papyracé du jeune adulte par dystrophie congénitale. A propos de 2 cas anatomo-cliniques et de 3 cas cliniques. Arch Mal Coeur Vaiss. 1968 Apr;61(4):477–503. [PubMed] [Google Scholar]
  8. Gould L., Guttman A. B., Carrasco J., Lyon A. F. Partial absence of the right ventricular musculature. A congenital lesion. Am J Med. 1967 Apr;42(4):636–641. doi: 10.1016/0002-9343(67)90064-2. [DOI] [PubMed] [Google Scholar]
  9. Haworth S. G., Shinebourne E. A., Miller G. A. Right-to-left interatrial shunting with normal right ventricular pressure. A puzzling haemodynamic picture associated with some rare congenital malformations of the right ventricle and tricuspid valve. Br Heart J. 1975 Apr;37(4):386–391. doi: 10.1136/hrt.37.4.386. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Kinare S. G., Panday S. R., Deshmukh S. M. Congenital aplasia of the right ventricular myocardium (Uhl's anomaly). Dis Chest. 1969 May;55(5):429–431. doi: 10.1378/chest.55.5.429. [DOI] [PubMed] [Google Scholar]
  11. NEIMANN N., PERNOT C., RAUBER G. APLASIE DU MYOCARDE DU VENTRICULE DROIT (VENTRICULE DROIT PAPYRAC'E CONG'ENITAL) Arch Mal Coeur Vaiss. 1965 Mar;58:421–430. [PubMed] [Google Scholar]
  12. PERRIN E. V., MEHRIZI A. ISOLATED FREE-WALL HYPOPLASIA OF HE RIGHT VENTRICLE. Am J Dis Child. 1965 Jun;109:558–566. doi: 10.1001/archpedi.1965.02090020560011. [DOI] [PubMed] [Google Scholar]
  13. Pomerance A., Yacoub M. H., Gula G. The surgical pathology of thoracic aortic aneurysms. Histopathology. 1977 Jul;1(4):257–276. doi: 10.1111/j.1365-2559.1977.tb01665.x. [DOI] [PubMed] [Google Scholar]
  14. Pérez Díaz L., Quero Jiménez M., Moreno Granados F., Pérez Martínez V., Merino Batres G. Congenital absence of myocardium of right ventricle: Uhl's anomaly. Br Heart J. 1973 May;35(5):570–572. doi: 10.1136/hrt.35.5.570. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. REEVE R., MACDONALD D. PARTIAL ABSENCE OF THE RIGHT VENTRICULAR MUSCULATURE--PARTIAL PARCHMENT HEART. Am J Cardiol. 1964 Sep;14:415–419. doi: 10.1016/0002-9149(64)90087-6. [DOI] [PubMed] [Google Scholar]
  16. SACKNER M. A., ROBINSON M. J., JAMISON W. L., LEWIS D. H. Isolated right ventricular hypoplasia with atrial septal defect or patent foramen ovale. Circulation. 1961 Dec;24:1388–1402. doi: 10.1161/01.cir.24.6.1388. [DOI] [PubMed] [Google Scholar]
  17. SEGALL H. N. Parchment heart (Osler). Am Heart J. 1950 Dec;40(6):948–950. doi: 10.1016/0002-8703(50)90192-x. [DOI] [PubMed] [Google Scholar]
  18. UHL H. S. M. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp. 1952 Sep;91(3):197–209. [PubMed] [Google Scholar]
  19. Van der Hauwaert L. G., Michaelsson M. Isolated right ventricular hypoplasia. Circulation. 1971 Sep;44(3):466–474. doi: 10.1161/01.cir.44.3.466. [DOI] [PubMed] [Google Scholar]

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