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. 1977 Mar;39(3):276–288. doi: 10.1136/hrt.39.3.276

Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications.

P S Rao
PMCID: PMC483232  PMID: 849388

Abstract

We have previously reported isolated cases of anatomical and functional closure of ventricular septal defects in tricuspid atresia. To study this phenomenon further, clinical, angiographic, and pathological findings in 20 consecutive cases of tricuspid atresia were reviewed. Sixteen cases were found to have normally related great arteries (type I) and 4 had transposition (type II). In 6 of these patients there was evidence of closure of a ventricular septal defect; in 3 this was complete and in the other 3, partial. Five of these ventricular septal defect closures occurred in type I patients and one among type II. Increasing cyanosis and polycythaemia and/or disappearance of a previously heard murmur were observed in all patients; these signs are more conspicuous in complete closure than in partial. The incidence of closure of ventricular septal defect in tricuspid atresia is 38 per cent and approximates to the incidence of spontaneous closure of isolated ventricular septal defects. Progressive muscular "encroachment" of the margins of ventricular septal defects with subsequent fibrosis and covering by endocardial proliferation is the most likely mechanism of closure in tricuspid atresia. The factors initiating the closure of ventricular septal defect remain unknown, but because of its occurrence in patients both with and without previous shunt operations, it is reasonable to assume that closure of ventricular septal defect in tricuspid atresia is not initiated or accelerated by these surgical shunts. Recent developments in surgical technique permit total surgical correction of tricuspid atresia at least in a physiological sense, but these operations can be successfully performed only in older children (over 8 years). Thus, palliation is essential in younger patients until they reach the age for total correction. A Blalock-Taussig shunt is recommended in preference to a Glenn anastomosis because the latter may leave the left pulmonary circuit without blood supply if the ventricular septal defect closes. In type II cases, a large and non-restrictive ventricular septal defect is essential for survival of the patient after the Fontan operation. For this reason, the size of the ventricular septal defect should be evaluated before and at the time of surgical correction. If the ventricular septal defect is small in a type II case, resection of the ventricular septum or a complete bypass of the ventricular septal defect and right ventricle is necessary at the time of the Fontan operation.

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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., AGUSTSSON M. H., BICOFF J. P., LYNFIELD J., WEINBERG M., Jr, FELL E. H., GASUL B. M. FURTHER OBSERVATIONS ON THE NATURAL HISTORY OF ISOLATED VENTRICULAR SEPTAL DEFECTS IN INFANCY AND CHILDHOOD. SERIAL CARDIAC CATHETERIZATION STUDIES IN 75 PATIENTS. Circulation. 1963 Oct;28:560–571. doi: 10.1161/01.cir.28.4.560. [DOI] [PubMed] [Google Scholar]
  2. Achtel R. A., Kaplan S., Benzing G., 3rd, Helmsworth J. A. Superior vena cava-right pulmonary artery anastomosis. Long-term results. Ann Thorac Surg. 1969 Dec;8(6):511–519. doi: 10.1016/s0003-4975(10)66088-2. [DOI] [PubMed] [Google Scholar]
  3. BLOOMFIELD D. K. THE NATURAL HISTORY OF VENTRICULAR SEPTAL DEFECT IN PATIENTS SURVIVING INFANCY. Circulation. 1964 Jun;29:914–955. doi: 10.1161/01.cir.29.6.914. [DOI] [PubMed] [Google Scholar]
  4. Bargeron L. M., Jr, Karp R. B., Barcia A., Kirklin J. W., Hunt D., Deverall P. B. Late deterioration of patients after superior vena cava to right pulmonary artery anastomosis. Am J Cardiol. 1972 Aug;30(3):211–216. doi: 10.1016/0002-9149(72)90060-4. [DOI] [PubMed] [Google Scholar]
  5. Boruchow I. B., Bartley T. D., Elliott L. P., Schiebler G. L. Late superior vena cava syndrome after superior vena cava-right pulmonary artery anastomosis. Report of four cases. N Engl J Med. 1969 Sep 18;281(12):646–650. doi: 10.1056/NEJM196909182811204. [DOI] [PubMed] [Google Scholar]
  6. Boruchow I. B., Swenson E. W., Elliott L. P., Bartley T. D., Wheat M. W., Jr, Schiebler G. L. Study of the mechanisms of shunt failure after superior vena cava-right pulmonary artery anastomosis. J Thorac Cardiovasc Surg. 1970 Oct;60(4):531–539. [PubMed] [Google Scholar]
  7. DE AZEVEDO A. C., TOLEDO A. N., DE CARVALHO A. A., ZANIOLO W., DOHMANN H., ROUBACH R. Ventricular septal defect; an example of its relative diminution. Acta Cardiol. 1958;13(5):513–518. [PubMed] [Google Scholar]
  8. Dick M., Fyler D. C., Nadas A. S. Tricuspid atresia: clinical course in 101 patients. Am J Cardiol. 1975 Sep;36(3):327–337. doi: 10.1016/0002-9149(75)90484-1. [DOI] [PubMed] [Google Scholar]
  9. Dolara A., Fazzini P. F., Marchi F., Tordini B. Changing clinical features in tricuspid atresia without transposition of great vessels. Report of two cases. Acta Cardiol. 1969;24(3):275–284. [PubMed] [Google Scholar]
  10. EDWARDS J. E., JAMES J. W., DuSHANE J. W. Congenital malformation of the heart; origin of transposed great vessels from the right ventricle associated with atresia of the left ventricular outlet, double orifice of the mitral valve, and single coronary artery. Lab Invest. 1952;1(2):197–207. [PubMed] [Google Scholar]
  11. EVANS J. R., ROWE R. D., KEITH J. D. Spontaneous closure of ventricular septal defects. Circulation. 1960 Dec;22:1044–1054. doi: 10.1161/01.cir.22.6.1044. [DOI] [PubMed] [Google Scholar]
  12. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240–248. doi: 10.1136/thx.26.3.240. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Freedom R. M., White R. D., Pieroni D. R., Varghese P. J., Krovetz L. J., Rowe R. D. The natural history of the so-called aneurysm of the membranous ventricular septum in childhood. Circulation. 1974 Feb;49(2):375–384. doi: 10.1161/01.cir.49.2.375. [DOI] [PubMed] [Google Scholar]
  14. Gallaher M. E., Fyler D. C. Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels. Circulation. 1967 Feb;35(2):381–388. doi: 10.1161/01.cir.35.2.381. [DOI] [PubMed] [Google Scholar]
  15. Guller B., Titus J. L. Morphological studies in tricuspid atresia. Circulation. 1968 Nov;38(5):977–986. doi: 10.1161/01.cir.38.5.977. [DOI] [PubMed] [Google Scholar]
  16. Henry J. N., Devloo R. A., Ritter D. G., Mair D. D., Davis G. D., Danielson G. K. Tricuspid atresia. Successful surgical "correction" in two patients using porcine xenograft valves. Mayo Clin Proc. 1974 Nov;49(11):803–810. [PubMed] [Google Scholar]
  17. Hoffman J. I., Rudolph A. M. The natural history of isolated ventricular septal defect with special reference to selection of patients for surgery. Adv Pediatr. 1970;17:57–79. [PubMed] [Google Scholar]
  18. Hoffman J. I., Rudolph A. M. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965 Nov;16(5):634–653. doi: 10.1016/0002-9149(65)90047-0. [DOI] [PubMed] [Google Scholar]
  19. Kreutzer G., Galíndez E., Bono H., De Palma C., Laura J. P. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1973 Oct;66(4):613–621. [PubMed] [Google Scholar]
  20. Marcano B. A., Riemenschneider T. A., Ruttenberg H. D., Goldberg S. J., Gyepes M. Tricuspid atresia with increased pulmonary blood flow. An analysis of 13 cases. Circulation. 1969 Sep;40(3):399–410. doi: 10.1161/01.cir.40.3.399. [DOI] [PubMed] [Google Scholar]
  21. Meng C. C. Spontaneous closure of ventricular septal defect in tricuspid atresia. J Pediatr. 1969 Oct;75(4):697–700. doi: 10.1016/s0022-3476(69)80470-1. [DOI] [PubMed] [Google Scholar]
  22. NADAS A. S., SCOTT L. P., HAUCK A. J., RUDOLPH A. M. Spontaneous functional closing of ventricular septal defects. N Engl J Med. 1961 Feb 16;264:309–316. doi: 10.1056/NEJM196102162640701. [DOI] [PubMed] [Google Scholar]
  23. NEILL C. A., BRINK A. J. Left axis deviation in tricuspid atresia and single ventricle; the electrocardiogram in 36 autopsied cases. Circulation. 1955 Oct;12(4):612–619. doi: 10.1161/01.cir.12.4.612. [DOI] [PubMed] [Google Scholar]
  24. ROBERTS W. C., MORROW A. G., MASON D. T., BRAUNWALD E. Spontaneous closure of ventricular septal defect, anatomic proof in an adult with tricuspid atresia. Circulation. 1963 Jan;27:90–94. doi: 10.1161/01.cir.27.1.90. [DOI] [PubMed] [Google Scholar]
  25. Rao P. S., Jue K. L., Isabel-Jones J., Ruttenberg H. D. Ebstein's malformation of the tricuspid valve with atresia. Differentiation from isolated tricuspid atresia. Am J Cardiol. 1973 Dec;32(7):1004–1009. doi: 10.1016/s0002-9149(73)80173-0. [DOI] [PubMed] [Google Scholar]
  26. Rao P. S. Left ventricular obstruction in double outlet right ventricle. Am Heart J. 1972 Feb;83(2):289–290. doi: 10.1016/0002-8703(72)90155-x. [DOI] [PubMed] [Google Scholar]
  27. Rao P. S., Linde L. M. Pressure and energy in the cardiovascular chambers. Chest. 1974 Aug;66(2):176–178. doi: 10.1378/chest.66.2.176. [DOI] [PubMed] [Google Scholar]
  28. Rao P. S., Sissman N. J. Spontaneous closure of physiologically advantageous ventricular septal defects. Circulation. 1971 Jan;43(1):83–90. doi: 10.1161/01.cir.43.1.83. [DOI] [PubMed] [Google Scholar]
  29. Ross D. N., Somerville J. Surgical correction of tricuspid atresia. Lancet. 1973 Apr 21;1(7808):845–849. doi: 10.1016/s0140-6736(73)91418-9. [DOI] [PubMed] [Google Scholar]
  30. Sakiyalak P., Ankeney J. L., Liebman J., DeMeules J. Results of superior vena cava-to-pulmonary artery shunt in the treatment of cyanotic heart disease. Ann Thorac Surg. 1971 Nov;12(5):514–526. doi: 10.1016/s0003-4975(10)65799-2. [DOI] [PubMed] [Google Scholar]
  31. Silove E. D., Vogel J. H., Grover R. F. The pressure gradient in ventricular outflow obstruction: influence of peripheral resistance. Cardiovasc Res. 1968 Jul;2(3):234–242. doi: 10.1093/cvr/2.3.234. [DOI] [PubMed] [Google Scholar]
  32. Stanford W., Armstrong R. G., Cline R. E., King T. D. Right atrium-pulmonary artery allograft for correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1973 Jul;66(1):105–111. [PubMed] [Google Scholar]
  33. Stansel H. C., Jr A new operation for d-loop transposition of the great vessels. Ann Thorac Surg. 1975 May;19(5):565–567. doi: 10.1016/s0003-4975(10)64433-5. [DOI] [PubMed] [Google Scholar]
  34. Taussig H. B., Keinonen R., Momberger N., Kirk H. Long-time observations of the Blalock-Taussig operation. IV. Tricuspid atresia. Johns Hopkins Med J. 1973 Mar;132(3):135–145. [PubMed] [Google Scholar]
  35. Varghese P. J., Izukawa T., Celermajer J., Simon A., Rowe R. D. Aneurysm of the membranous ventricular septum. A method of spontaneous closure of small ventricular septal defect. Am J Cardiol. 1969 Oct;24(4):531–536. doi: 10.1016/0002-9149(69)90496-2. [DOI] [PubMed] [Google Scholar]
  36. Walker D. R., Sbokos C. G., Lennox S. C. Correction of tricuspid atresia. Br Heart J. 1975 Mar;37(3):282–286. doi: 10.1136/hrt.37.3.282. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. William V. L., Barner H. B., Mudd J. G., Fagan L. F., Kaiser G. C. Superior vena cava-pulmonary artery anastomosis. J Thorac Cardiovasc Surg. 1974 Mar;67(3):380–386. [PubMed] [Google Scholar]

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