Progress in Pediatric Cardiology
Volume 22, Issue 2 , Pages 187-204 , September 2006

Right ventricle to coronary artery connections (fistulae) in pulmonary atresia with intact ventricular septum: Clinical and histopathological correlations

  • Ursula Sauer

      Affiliations

    • Klinik für Kinderkardiologie und angeborene Herzfehler und Experimentelle Chirurgie, Deutsches Herzzentrum München, Technische Universität München, Germany
    • Corresponding Author InformationCorresponding author. Klinik für Kinderkardiologie und angeborene Herzfehler Deutsches Herzzentrum München, Technische Universität München Lazarettstrasse 36, D-80636 München, Germany.
  • ,
  • Adriana C. Gittenberger-de Groot

      Affiliations

    • Department of Anatomy and Embryology, University of Leiden, The Netherlands
  • ,
  • Werner Heimisch

      Affiliations

    • Klinik für Kinderkardiologie und angeborene Herzfehler und Experimentelle Chirurgie, Deutsches Herzzentrum München, Technische Universität München, Germany
  • ,
  • Lutz Bindl

      Affiliations

    • Zentrum für Kinderheilkunde, Universität Bonn, Germany

References 

  1. Goor DA, Lillehei CW. The anatomy of the heart. In:  Goor DA,  Lillehei CW editor. Congenital malformations of the heart. 1st ed.. New York: Grune and Stratton; 1975;p. 1–37
  2. Bull C, De Leval MR, Mercanti C, Macartney FJ, Anderson RH. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation. 1982;66:266–272
  3. De Leval MR, Bull C, Stark J, Anderson RH, Taylor JFN, Macartney FJ. Pulmonary atresia and intact ventricular septum: surgical management based on revised classification. Circulation. 1982;66:272–280
  4. Freedom RM, Wilson C, Trusler GA, Williams WG, Rowe RD. Pulmonary atresia and intact ventricular septum. A review of the anatomy, myocardium and factors influencing right ventricular growth and guidelines for surgical intervention. Scand J Thorac Cardiovasc Surg. 1983;17:1–28
  5. De Leval M, Bull C, Hopkins R, et al. Decision making in the definitive repair of the heart with a small right ventricle. Circulation. 1985;72(Suppl II):52–60
  6. Foker JE, Braunlin EA, St. Cyr JA, et al. Management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. 1986;92:706–715
  7. Freedom RM, Perrin D. The right ventricle: morphological considerations. In:  Freedom RM editors. Pulmonary atresia with intact ventricular septum. New York Mount Kisko: Futura Publishing Company, Inc.; 1989;p. 53–74
  8. Freedom RM. How can some thing so small cause so much grief? Some thoughts about the underdeveloped right ventricle in pulmonary atresia and intact septum. J Am Coll Cardiol. 1992;19:1038–1040
  9. Rigby ML, Salgado M, Silva C. Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period. Cardiol Young. 1992;2:377–381
  10. Giglia TM, Jenkins KJ, Matitiau A, et al. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation. 1993;88(part 1):2248–2256
  11. Leung MP, Mok C, Lee J, Lo RNS, Cheung H, Chiu C. Management evolution of pulmonary atresia and intact ventricular septum. Am J Cardiol. 1993;71:1331–1336
  12. Bull C, Kostelka M, Sorensen K, De Leval MR. Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum. Thorac Cardiovasc Surg. 1994;107:359–366
  13. Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart. 1926;13:73–83
  14. Kauffman SL, Anderson DH. Persistent venous valves, maldevelopment of the right heart and coronary artery-ventricular communications. Am Heart J. 1962;66:664–669
  15. Lenox C, Briner J. Absent proximal coronary arteries associated with pulmonic atresia. Am J Cardiol. 1972;30:666–669
  16. O'Connor WN, Cottrill CM, Johnson GL, Noonan JA, Todd EP. Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance. Circulation. 1982;65:805–809
  17. Sauer U, Bindl L, Babic R, et al. Communications entre le ventricule droit et les artères coronaires chez des nourissons atteints d'atrésie pulmonaires à septum interventriculaire intact. Corrélation anatomo-clinique. In:  Bex JP, et al. editor. Pathologie des artères coronaires. Paris: Masson; 1984;p. 60–72
  18. Sauer U, Bindl L, Pilossoff V, et al. Pulmonary atresia with intact ventricular septum and right ventricle–coronary artery fistulae: selection of patients for surgery. In:  Doyle EF,  Engle MA,  Gersony WM,  Rashkind WJ,  Talner NS editor. Pediatric cardiology. New York: Springer Verlag; 1986;p. 566–578
  19. Calder AL, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol. 1987;59:436–442
  20. Kasznica J, Ursell PhC, Blanc A, et al. Abnormalities of the coronary circulation in pulmonary atresia and intact ventricular septum. Am Heart J. 1987;1415–1420
  21. Gittenberger-de Groot AC, Sauer U, Bindl L, Babic R, Essed CE, Bühlmeyer K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol. 1988;18:243–258
  22. O'Connor WN, Stahr BJ, Cottrill CM, Todd EP, Noonan JA. Ventriculocoronary connections in hypoplastic right heart syndrome: autopsy serial section study of six cases. JACC. 1988;11:1061–1072
  23. Freedom RM, Benson LN, Trusler GA. Pulmonary atresia and intact ventricular septum: consideration of the coronary circulation and ventriculo-coronary artery connections. Annu Card Surg. 1989;38–44
  24. Wilson GJ, Freedom RM, Koike K, Perrin D. The coronary arteries: anatomy and histopathology. In:  Freedom RM editors. Pulmonary atresia with intact ventricular septum. New York Mount Kisko: Futura Publishing Company, Inc.; 1989;p. 75–88
  25. Burrows PE, Freedom RM, Benson LN, et al. Coronary angiography of pulmonary atresia, hypoplastic right ventricle, and ventriculocoronary communications. AJR. 1990;154:789–795
  26. Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricular–dependent coronary circulation in pulmonary atresia with intact septum. Circulation. 1992;86:1516–1528
  27. Gentles TL, Colan SD, Giglia TM, et al. Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies. Circulation. 1993;88(part 2):183–189
  28. Oosthoek PW, Moorman AFM, Sauer U, Gittenberger-de Groot AC. Capillary distribution in the ventricles of hearts with pulmonary atresia and intact ventricular septum. Circulation. 1995;91:1790–1797
  29. Gittenberger Groot AC, Tennstedt C, Chaoui H, Lie-Venema H, Sauer U, Poelmann RE. Ventriculo coronary arterial communications (VCAC) and myocardial sinusoids in hearts with pulmonary atresia with intact ventricular septum: two different diseases. Progr in Ped Card. In Press.
  30. Wood JC, Reyes-Migica M, Kopf G. Vascular aneurysm producing divided right atrium in a patient with pulmonary atresia and intact ventricular septum. Cardiol Young. 2000;10:281–285
  31. Coles JG, Freedom RM, Lightfoot NE, et al. Long-term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg. 1989;47:213–217
  32. Hawkins JA, Thorne JK, Boucek MM, et al. Early and late results in pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1990;100:492–497
  33. Termignon JL, Bruniaux J, Lacour-Gayet F, Serraf A, Planché C. Atrésie pulmonaire à septum interventriculaire intact. Résultats à moyen terme du traitement chirurgical à propos de 45 observations. Arch Mal Coeur. 1992;85:589–596
  34. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. 1993;105:406–427
  35. Satou GM, Perry SB, Gauvreau K, Geva T. Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol. 2000;85:1319–1324
  36. Squittieri C, di Carlo C, Giannico S, Marino B, Giamberti A, Marcelletti C. Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. 1989;97:779–784
  37. Williams WG, Burrows P, Freedom RM, et al. Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1991;101:222–229
  38. Waldman JD, Lamberti JJ, Mathewson JW, George L. Surgical closure of the tricuspid valve for pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications. Pediatr Cardiol. 1984;5:221–224
  39. Waldman JD, Karp RB, Lamberti JJ, Sand ME, Ruschhaupt DG, Agarwala B. Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections. Ann Thorac Surg. 1995;59:933–941
  40. Freeman JE, DeLeon SY, Lai S, Fischer EA, Pifarré R. Right ventricle-to-aorta conduit in pulmonary atresia with intact ventricular septum and coronary sinusoids. Ann Thorac Surg. 1993;56:1393–1395
  41. Laks H, Gates RN, Grant PW, Drant S, Allada V, Harake B. Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum. Ann Thorac Surg. 1995;59:342–347
  42. Cho Y, Fukuda T, Suzuki T, Aki A. A case of total cavopulmonary connection with preceding tricuspid valve closure in pulmonary atresia and intact ventricular septum, right ventricular-coronary artery fistulae, and coronary artery stenoses. Nippon Kyobu Geka Gakkai Zasshi. 1996;44:1929–1934
  43. Sissman NJ, Abrams HL. Bidirectional shunting in a coronary artery–right ventricular fistula associated with pulmonary atresia with intact ventricular septum. Circulation. 1965;32:382–388
  44. Freedom RM, Harrington DP. Contribution of intra-myocardial sinusoids in pulmonary atresia and intact ventricular septum to a right-sided circular shunt. Br Heart J. 1974;36:1062–1065
  45. Freedom RM. General morphological considerations. In:  Freedom RM editors. Pulmonary atresia with intact ventricular septum. New York Mount Kisko: Futura Publishing Company, Inc.; 1989;p. 17–36
  46. Graham TP, Jarmakani JM, Atwood GF, Canent RV. Right ventricular volume determinations in children. Normal values and observations with pressure overload. Circulation. 1973;47:144–153
  47. Graham TP, Jarmakani JM, Canent RV, Morrow MN. Left heart volume estimation in infancy and childhood. Reevaluation of methodology and normal values. Circulation. 1971;43:895-04.
  48. Pesonen E, Hirvonen J, Laaksonen H, Möttönen M, Raekallio J, Akerblom HK. Morphometry of coronary arteries. Its use in children one year of age and older. Arch Pathol Lab Med. 1982;106:181–184
  49. Bindl L. Ventrikulokoronare Verbindungen (“Fisteln”) bei Pulmonalatresie mit intaktem Ventrikelseptum (PA-IVS). Dissertation. 1987;
  50. Garcia OL, Gelband H, Tamer DF, Fojaco RM. Exclusive origin of both coronary arteries from a hypoplastic right ventricle complicating extreme tetralogy of Fallot: lethal myocardial infarction following a palliative shunt. Am Heart J. 1988;115:198–201
  51. Patel CR, Spector ML, Zahka KG. Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery. Cardiol Young. 1999;9:512–515
  52. Sauer U, Mocellin R, Henglein D. Pulmonalklappenatresie und hochgradige (kritische) Pulmonalklappenstenose mit intaktem Ventrikelseptum. Volumenbestimmung des rechten und linken Ventrikels. Herz. 1977;2:398–410
  53. Graham TP, Erath HG, Boucek RT, Boerth RC. Left ventricular function in cyanotic congenital heart disease. Am J Cardiol. 1980;45:1231–1236
  54. Sideris EB, Olley PM, Spooner E, et al. Left ventricular function and compliance in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. 1982;84:192–199
  55. Hausdorf G, Grävinghoff L, Keck EW. Effects of persisting myocardial sinusoids on left ventricular performance in pulmonary atresia with intact ventricular septum. Eur Heart J. 1987;8:291–296
  56. Scognamiglio R, Daliento L, Razzolini R, et al. Pulmonary atresia with intact ventricular septum: a quantitative cineventriculographic study of the right and left ventricular function. Pediatr Cardiol. 1986;7:183–187
  57. Daliento L, Scognamiglio R, Thiene G, et al. Morphologic and functional analysis of myocardial status in pulmonary atresia with intact ventricular septum. Cardiol Young. 1992;2:261–266
  58. Akiba T, Becker AE. Disease of the left ventricle in pulmonary atresia with intact ventricular septum: the limiting factor for long-lasting successful surgical intervention?. J Thorac Cardiovasc Surg. 1994;108:1–8
  59. Koike K, Perrin D, Wilson GJ, Freedom RM. Myocardial ischemia and coronary arterial involvement in newborn babies less than one week old with pulmonary atresia and intact ventricular septum. In:  Freedom RM editors. Pulmonary atresia with intact ventricular septum. New York Mount Kisko: Futura Publishing Company, Inc.; 1989;p. 101–107
  60. Esterly JR, Oppenheimer EH. Some aspects of cardiac pathology in infancy and childhood. IV. Myocardial and coronary lesions and cardiac malformations. Pediatrics. 1967;39:896–903
  61. Essed CD, Klein HW, Krediet P. Coronary and endocardial fibroelastosis of the ventricles in the hypoplastic left and right heart syndromes. Virchows Arch Pathol Anat Histopathol. 1975;368:87–97
  62. Sauer U, Gittenberger-de Groot AC, Geishauser M, Babic R, Bühlmeyer K. Coronary arteries in the hypoplastic left heart syndrome. Histopathologic and histometrical studies and implications for surgery. Circulation. 1989;80(Suppl.I):168–176
  63. Chaoui R, Tennstedt C, Göldner B, Bollman R. Prenatal diagnosis of ventriculo–coronary communications in a second-trimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol. 1997;4:194–197
  64. Balling G, Vogt M, Kaemmerer H, Eicken A, Meisner H, Hess J. Intracardiac thrombus formation after the Fontan operation. J Thorac Cardiovasc Surg. 2000;119:745–752

PII: S1058-9813(06)00052-X

doi: 10.1016/j.ppedcard.2006.07.009

Progress in Pediatric Cardiology
Volume 22, Issue 2 , Pages 187-204 , September 2006