The "wall-to-wall" heart in the patient with pulmonary atresia and intact ventricular septum

Cardiology in the Young

Volumn 16, Issue 1, 2006, Pages 18-29

  Freedom, Robert M ^a,b   Jaeggi, Edgar ^a   Perrin, Don ^b   Yoo, Shi Joon ^a,c   Anderson, Robert H ^d  

^a UNIVERSITY OF TORONTO   (Canada)

^b HOSPITAL FOR SICK CHILDREN   (Canada)

^c UNIVERSITY OF TORONTO   (Canada)

^d UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Ebstein's malformation regurgitation; Uhl's anomaly of the right ventricle

Indexed keywords

NITRIC OXIDE; PROSTAGLANDIN;

ARTICLE; CARDIOMEGALY; CARDIOPULMONARY INSUFFICIENCY; EBSTEIN ANOMALY; FETUS OUTCOME; HEART SURGERY; HEART VENTRICLE SEPTUM; HUMAN; INCIDENCE; LUNG ATRESIA; PATHOLOGICAL ANATOMY; PRENATAL DIAGNOSIS; PRENATAL PERIOD; PREVALENCE; PROGNOSIS; SURGICAL ANATOMY; SURVIVAL RATE; TRANSLUMINAL VALVULOPLASTY; TREATMENT OUTCOME; TRICUSPID VALVE REGURGITATION;

ECHOCARDIOGRAPHY, DOPPLER; FEMALE; FETAL HEART; HEART SEPTAL DEFECTS, VENTRICULAR; HEART VENTRICLES; HUMANS; PREGNANCY; PULMONARY ATRESIA; SEVERITY OF ILLNESS INDEX; ULTRASONOGRAPHY, PRENATAL;

EID: 32044442286     PISSN: 10479511     EISSN: None     Source Type: Journal    
DOI: 10.1017/S1047951105002040     Document Type: Article

References (97)

1. Roberts WC. Aortic atresia. The worst heart disease. Am J Cardiol 1984; 54: 1169.
2. Freedom RM, Yoo S-J. The hypoplastic left heart syndrome. In: Freedom RM, Yoo SJ, Mikailian H, Williams WG. The Natural and Modified History of Congenital Heart Disease. Blackwell-Futura Publishing Co., Inc, Oxford, UK and New York, 2004, pp 397-407.
3. Freedom RM, Jaeggi ET, Lim J, Anderson RH. Hearts with isomerism of the right atrial appendages - one of the worst forms of disease in 2005. Cardiol Young 2005; 15: 554-567.
4. 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: 213-237.
5. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary stresia with intact ventricular septum. J Thorac Cardiovasc Surg 1993; 105: 406-427.
6. Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 2004; 127: 1000-1007.
7. Freedom RM, Perrin D. Ventriculocoronary arterial connections in pulmonary atresia and intact ventricular septum. Cardiol Young 2005; 15: 447-468.
8. Greenwold WE, DuShane JW, Burchell HB, Bruwer A, Edwards JE. Congenital pulmonary atresia with intact ventricular septum: Two anatomic types (Abstract). Circulation 1956; 14: 945.
9. Davignon AL, Greenwold WE, DuShane JW, Edwards JE. Congenital pulmonary atresia with intact ventricular septum. Clinicopathologic correlation of two anatomic types. Am Heart J 1961; 62: 591-602.
10. Bharati S, McAllister HAJ, Chiemmongkoltip P, Lev M. Congenital pulmonary atresia with tricuspid insufficiency: Morphologic study. Am J Cardiol 1977; 40: 70-75.
11. Freedom RM, Dische MR, Rowe RD. The tricuspid valve in pulmonary atresia and intact ventricular septum. Arch Pathol Lab Med 1978; 102: 28-31.
12. Freedom RM, Culham G, Moes F, Olley PM, Rowe RD. Differentiation of functional and structural pulmonary atresia: Role of aortography. Am J Cardiol 1978; 41: 914-920.
13. Long WA, Willis PW. Maternal lithium and neonatal Ebstein's. Am J Perinatol 1984; 1: 182-184.
14. Correa-Villasenor A, Ferencz C, Neill CA, Wilson PD, Boughman JA, and the Baltimore-Washington infant study group. Ebstein's malformation of the tricuspid valve: Genetic and etiologic factors. Teratology 1994; 50: 137-147.
15. Zalzstein E, Koren G, Einarson T, Freedom RM. A case-control study on the association between first trimester exposure to lithium and Ebstein's anomaly. Am J Cardiol 1990; 65: 817-818.
16. Hoffman JIE, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am Heart J 2004; 147: 425-439.
17. Steeg CN, Ellis K, Bransilver B, Gersony W. Pulmonary atresia and intact ventricular septum complicating corrected transposition of the great vessels. Am Heart J 1971; 82: 382-386.
18. Shimizu T, Ando M, Takao A. Pulmonary atresia with intact ventricular septum and corrected transposition of the great arteries. Br Heart J 1981; 45: 471-474.
19. Freedom RM, Culham JAG, Moes CAF. Angiocardiography of Congenital Heart Disease. Macmillan Publishing Co., New York, 1984, pp 221-253.
20. Patel CR, Spector ML, Zahka KG. Congenitally corrected transposition with pulmonary atresia and intact ventricular septum. Cardiol Young 2000, 10: 268-270.
21. Brenner JI, Bharati S, Winn Jr WC, Lev M. Absent tricuspid valve with aortic atresia in mixed levocardia (atria situs solitus, L-loop). A hitherto undescribed entity. Circulation 1978; 57: 836-840.
22. Celermajer DS, Seamus C, Deanfield JE, Sullivan ID. Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: Association with aortic arch obstruction. JACC 1991; 18: 1056-1058.
23. Deanfield JE, Anderson RH, Macartney FJ. Aortic atresia with 'corrected transposition of the great arteries (atrioventricular and ventriculoarterial discordance). Br Heart J 1981; 46: 683-686.
24. Matsukawa T, Yoshii S, Miyamura H, Eguchi S. Aortic atresia with Ebstein's and Uhl's anomaly in corrected transposition of the great arteries: Clinicopathologic findings. Japanese Circ J 1985; 49: 325-328.
25. Muster AJ, Idriss FS, Bharati S, et al. Functional aortic valve atresia in transposition of the great arteries. JACC 1985; 6: 630-634.
26. Chan KC, Da Costa P, Dickinson DF. Functional aortic atresia in congenitally corrected transposition. Int J Cardiol 1989; 25: 237-239.
27. Craig BG, Smallhorn, JF, Rowe RD, Freedom RM. Severe obstruction to systemic blood flow in congenitally corrected transposition of the great arteries: An analysis of 14 patients. Int J Cardiol 1986; 11: 209-215.
28. Galal O, Hentrich F, Przybilla C, Donhuijsen K. Aortic atresia and pulmonary artery hypoplasia in congenitally corrected transposition of the large arteries, aortic isthmus stenosis and Uhl's anomaly of the left-sided ventricle. [Aortenatresie und Pulmonalarterienhypoplasie bei kongenital korrigierter Transposition der grossen Gefasse, Aortenisthmusstenose und Uhl'scher Anomalie des linksseitigen Ventrikels.] Pathologe 1987; 8: 168-171.
29. Freedom RM, Mawson J, Yoo S-J, Benson LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co., Armonk, NY, 1997; pp 617-672.
30. Haworth SG, Shinebourne EA, Miller GAH. Right-to-left inter-atrial 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; 37: 386-391.
31. Rowlatt JF, Rimoldi MJA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963; 10: 499-588.
32. Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281-288.
33. 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: 86-90.
34. Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation 1964; 30: 911-917.
35. Choi YH, Seo JW, Choi JY, Yun YS, Kim SH, Lee HJ. Morphology of tricuspid valve in pulmonary atresia with intact ventricular septum. Pediatr Cardiol 1998; 19: 381-389.
36. Becker AE, Becker MJ, Edwards JE. Pathologic spectrum of dysplasia of the tricuspid valve: Features in common with Ebstein's malformation. Arch Pathol 1971; 91: 167-178.
37. Freedom RM, Benson LN. Neonatal expression of Ebstein's anomaly. Prog Pediatr Cardiol 1993; 2: 22-27.
38. Rao PS, Jue KL, Isabel-Jones J, Ruttenberg HD. Ebstein's malformation of the tricuspid valve with atresia. Differentiation from isolated tricuspid atresia. Am J Cardiol 1973; 32: 1004-1009.
39. Gerlis LM, Anderson RH. Cor triatriatum dexter with imperforate Ebstein's anomaly. Br Heart J 1976; 38: 108-111.
40. Zielinsky P, Pilla CB. Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis. Arq Bras Cardiol 2000; 75: 62-64.
41. Cote 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; 31: 658-661.
42. Fuertes J, Salazar J, Mengual J, et al. Anomalie d'Uhl associee a une atresia de la valvule piulmonaire. Pediatrie 1984; 39: 213-217.
43. Descalzo A, Casadas M, Cintado C, Castillo CA, Garcia E, Ariza S. Uhl's anomaly associated with pulmonary atresia. Human Pathol 1980; 11: 575-576.
44. Uhl HSM. 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.
45. Uhl HSM. Uhl's anomaly revisited. Circulation 1996; 93: 1483-1484.
46. Smallhorn JF, Izukawa T, Benson L, Freedom RM. Noninvasive recognition of functional pulmonary atresia by echocardiography. Am J Cardiol 1984; 54: 925-926.
47. Lee CL, Hsieh KS, Huang TC, Lin CC, Choong CS. Recognition of functional pulmonary atresia by color Doppler echocardiography. Am J Cardiol 1999; 83: 987-988.
48. Hiraumi Y, Watanabe K, Tomita H, et al. Doppler echocardiographic differentiation of functional from anatomic pulmonary atresia-analysis using quantitative parameters. Circ J 2002; 66: 665-667.
49. Weinhaus L, Jureidini S, Nouri S, Connors RH. Functional pulmonary atresia: Color flow recognition and treatment with extracorporeal membrane oxygenation. Am Heart J 1990; 119: 980-982.
50. Tanaka T, Yamaki S, Ohno T, Ozawa A, Kakizawa H, Iinuma R. The histology of the lung in neonates with tricuspid valve disease and gross cardiomegaly due to severe regurgitation. Pediatr Cardiol 1998; 19: 133-138.
51. Lang D, Oberhoffer R, Cook A, et al. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life. J Am Coll Cardiol 1991; 17: 1161-1167.
52. Haworth SG, Reid L. Quantitative structural study of pulmonary circulation in the newborn with pulmonary atresia. Thorax 1977; 32: 129-133.
53. Tanaka T, Yanaki S, Kakizawa H. Histologic studies of the small pulmonary arteries in 38 patients with pulmonary atresia and intact ventricular septum. Jpn Circ J 1996; 60: 293-299.
54. Naeye RC. Perinatal changes in the pulmonary vascular bed with stenosis and atresia of the pulmonic valve. Am Heart J 1961; 61: 586-592.
55. Gerlis LM, Ho SY, Sweeney AE. Mitral valve anomalies associated with Ebstein's malformation of the tricuspid valve. Am J Cardiovasc Pathol 1993; 4: 294-301.
56. Attenhofer Jost CH, Connolly HM, Warnes CA, et al. Noncompacted myocardium in Ebstein's anomaly: Initial description in three patients. J Am Soc Echocardiogr 2004; 17: 677-680.
57. Rice MJ, McDonald RW, Reller MD. Progressive pulmonary stenosis in the fetus: Two case reports. Am J Perinatol 1993; 10: 424-427.
58. Todros T, Paladini D, Chiappa E, et al. Pulmonary stenosis and atresia with intact ventricular septum during prenatal life. Ultrasound Obstet Gynecol 2003; 21: 228-233.
59. Hsiao SM, Wu MH, Shih JC, Hsieh FJ. In utero progressive pulmonary stenosis successfully treated with transcatheter intervention after delivery. J Formos Med Assoc 2001; 100: 347-349.
60. Maeno Y, Himeno W, Fujino H, et al. Progression of congenital heart disease in the prenatal period. Pediatr Int 1999; 41: 709-715.
61. Tulzer G, Arzt W, Franklin RC, Loughna PV, Mair R, Gardiner HM. Fetal pulmonary valvuloplasty for critical pulmonary stenosis or atresia with intact septum. Lancet 2002; 360: 1567-1568.
62. Hornberger LK, Benacerraf BR, Bromley BS, Spevak PJ, Sanders SP. Prenatal detection of severe right ventricular outflow tract obstruction: Pulmonary stenosis and pulmonary atresia. J Ultrasound Med 1994; 13: 743-750.
63. Nishibatake M, Matsuda Y, Kamitomo M, Ibara S, Sameshima H. Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero. Pediatr Int 1999; 41: 716-721.
64. Sahn DJ, Heldt GP, Reed KL, Kleinman CS, Meijboom EJ. Fetal heart disease with cardiomegaly may be associated with lung hypoplasia as a determinant of poor prognosis (Abstract). J Am Coll Cardiol 1988; 11: 9A.
65. Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: Diagnosis and outcome. J Am Coll Cardiol 1991; 17: 167-173.
66. Chaoui R, Bollmann R, Goldner B, Heling KS, Tennstedt C. Fetal cardiomegaly: Echocardiographic findings and outcome in 19 cases. Fetal Diagn Ther 1994; 9: 92-104.
67. Roberson DA, Silverman NH. Ebstein's Anomaly: Echocardiographic and Clinical features in the Fetus and Neonate. J Am Coll Cardiol 1989; 14: 1300-1307.
68. Pavlova M, Fouron J-C, Drblik SP, et al. Factors affecting the prognosis of Ebstein's anomaly during fetal life. Am Heart J 1998; 135: 1081-1085.
69. Allan LD, Cook A. Pulmonary atresia with intact ventricular septum in the fetus. Cardiol Young 1992; 2: 367-376.
70. Daubeney PE, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA. Pulmonary atresia with intact ventricular septum: Impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation 1998; 98: 562-566.
71. Daubeney PE, Delany DJ, Anderson RH, et al. Pulmonary atresia with intact ventricular septum. Range of morphology in a population-based study. J Am Coll Cardiol 2002; 39: 1670-1679.
72. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol 1998; 81: 749-754.
73. Rowe RD, Freedom RM, Mehrizi A. The Neonate with Congenital Heart Disease, Second Edition; WB Saunders Co., New York, 1981, pp 515-528.
74. Atz AM, Munoz RA, Adatia I, Wessel DL. Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly. Am J Cardiol 2003; 91: 906-908.
75. Bruckheimer E, Bulbul Z, Pinter E, Gallani M, Kleinman CS, Fahey JT. Inhaled nitric oxide therapy in a critically ill neonate with Ebstein's anomaly. Pediatr Cardiol 1998; 19: 477-479.
76. Dunbar I, Loehr J, Schaffer M. Usefulness of prolonged prostaglandin infusion in neonates with Ebstein's anomaly. Am J Cardiol 1993; 72: 1327-1329.
77. Dyamenahalli U, McCrindle BW, McDonald CM, et al. Pulmonary atresia with intact ventricular septum: Management and outcomes of 210 consecutive patients. Cardiol Young 2004; 14: 299-308.
78. Rychik J, Levy H, Gaynor JW, De Campli WM, Spray TL. Outcome after operations for pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1998; 116: 924-931.
79. Jahangiri M, Zurakowski D, Bichell D, Mayer JE, del Nido PJ, Jonas RA. Improved results with selective management in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1999, 118: 1046-1052.
80. McCrindle BW. Commentary to Ref. 96. J Thorac Cardiovasc Surg 1999; 118: 1052-1055.
81. Stellin G, Santini F, Thiene G, et al. Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve. Anatomic and surgical considerations. J Thorac Cardiovasc Surg 1993; 106: 255-261.
82. Guzzo D, Castellano C, Munoz-Castellanos L, de Rubens J, Calderon J, Attie F. Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve. Arch Inst Cardiol Mex 1989; 59: 133-138.
83. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991; 101: 1082-1087.
84. Endo M, Ohmi M, Sato K, Tanaka T, Kakizawa H, Tabayashi K. Tricuspid valve closure for neonatal Ebstein's anomaly. Ann Thorac Surg 1998, 65: 540-542.
85. DeLeon MA, Gidding SS, Gotteiner N, Backer CL, Mavroudis C. Successful palliation of Ebstein's malformation on the first day of life following fetal diagnosis. Cardiol Young 2000; 10: 384-387.
86. Watanabe M, Harada Y, Takeuchi T, Satomi G, Yasukouchi S. Modified Starnes operation for neonatal Ebstein's anomaly. Ann Thorac Surg 2002; 74: 916-917.
87. Van Son JAM, Falk V, Black MD, Haas GS, Mohr FW. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiovasc Surg 1998; 13: 280-285.
88. Adachi I, Ishizaka T, Ichikawa H, Kagisaki K, Kogaki S, Matsuda H. Midterm result of a case after Starnes operation. Jpn Thorac Cardiovasc Surg 2004; 52: 272-275.
89. Raunikar RA, Stron WB. Pulmonary atresia with intact ventricular septum. In: Moller JH (ed.). Surgery of Congenital Heart Disease. Pediatric Cardiac Care Consortium 1984-1995. Futura Publishing Company, Inc., Armonk, NY, 1998, pp 257-270.
90. Chandar JS, Tamer DF, Young M-L. Ebstein's anomaly. In: Moller JH (ed). Surgery of Congenital Heart Disease. Pediatric Cardiac Care Consortium. Perspectives in Pediatric Cardiology, Vol. 6. Futura Publishing Company, Inc., Armonk, New York, 1998; 287-295.
91. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Razook JD. Neonatal repair of Ebstein's anomaly: Indications, surgical technique, and medium-term follow-up. Ann Thorac Surg 2000; 69: 1505-1510.
92. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein's anomaly in the symptomatic neonate: An evolution of technique with 7-year follow-up. Ann Thorac Surg 2002; 73: 1786-1792.
93. Arzt W, Tulzer G, Aigner M, Mair R, Hafner E. Invasive intrauterine treatment of pulmonary atresia/intact ventricular septum with heart failure. Ultrasound Obstet Gynecol 2003; 21: 186-188.
94. Tworetzky W, Marshall AC. Balloon valvuloplasty for congenital heart disease in the fetus. Clin Perinatol 2003, 30: 541-550.
95. Tworetzky W, Marshall AC. Fetal interventions for cardiac defects. Pediatr Clin North Am 2004; 51: 1503-1513.
96. Ozkutlu S, Ceviz N, Ayabakan C, Akcoren Z. A case of severe Ebstein's anomaly with incompetent pulmonary valve. Turk J Pediatr 2002; 44: 162-167.
97. Andelfinger G, Wright KN, Lee HS, Siemens LM, Benson DW. Canine tricuspid valve malformation, a model of human Ebstein anomaly, maps to dog chromosome 9. J Med Genet 2003; 40: 320-324.