Double-Outlet Ventricle

Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult

Volumn , Issue , 2009, Pages 417-438

  Lopez, Leo ^a,b  

^a ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^b CHILDREN S HOSPITAL AT MONTEFIORE   (United States)

Author keywords

Conal morphology and outflow tract obstruction; DORV uncommon congenital heart disease; DORV with subpulmonary ventricular septal defect (VSD); Double outlet left ventricle (DOLV) rare ventriculo arterial alignment; Double outlet right ventricle (DORV); Double outlet ventricle; Great artery relationships; Transthoracic imaging

Indexed keywords

EID: 84884904601     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1002/9781444306309.ch25     Document Type: Chapter

References (116)

1. Van Praagh R. What is the Taussig-Bing malformation? Circulation 1968;28:445-9.
2. Shinebourne EA, Macartney FJ, Anderson RH. Sequential chamber localization-logical approach to diagnosis in congenital heart disease. Br Heart J 1976;38:327-40.
3. Wilcox BR, Ho SY, Macartney FJ et al. Surgical anatomy of double-outlet right ventricle with situs solitus and atrioventricular concordance. J Thorac Cardiov Surg 1981;82:405-17.
4. Edwards WD. Double-outlet right ventricle and tetralogy of Fallot. Two distinct but not mutually exclusive entities. J Thorac Cardiov Surg 1981;82:418-22.
5. Van Praagh S, Davidoff A, Chin A et al. Double outlet right ventricle: Anatomic types and developmental implications based on a study of 101 autopsied cases. Coeur 1982;13: 389-440.
6. Neufeld HN, Dushane JW, Wood EH et al. Origin of both great vessels from the right ventricle. I. Without pulmonary stenosis. Circulation 1961;23:399-412.
7. Neufeld HN, Dushane JW, Edwards JE. Origin of both great vessels from the right ventricle. II. With pulmonary stenosis. Circulation 1961;23:603-12.
8. Neufeld HN, Lucas RV Jr, Lester RG et al. Origin of both great vessels from the right ventricle without pulmonary stenosis. Br Heart J 1962;24:393-408.
9. McGoon D. Origin of both great vessels from the right ventricle. Surg Clin N Am 1961;41:1113-20.
10. Lecompte Y, Batisse A, Di Carlo D. Double-outlet right ventricle: a surgical synthesis. Adv Cardiac Surg 1993;4:109-36.
11. Sakata R, Lecompte Y, Batisse A et al. Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. I. Criteria of surgical decision. J Thorac Cardiov Surg 1988;95:90-5.
12. Aoki M, Forbess JM, Jonas RA et al. Result of biventricular repair for double-outlet right ventricle. J Thorac Cardiov Surg 1994;107:338-49; discussion 49-50.
13. Belli E, Serraf A, Lacour-Gayet F et al. Biventricular repair for double-outlet right ventricle. Results and long-term follow-up. Circulation 1998;98(19 Suppl.):II360-5; discussion II5-7.
14. Abernathy J. Surgical and Physiological Essays, Part II. London: James Evans, 1793:162.
15. Peacock TB. On Malformations of the Human Heart. London: John Churchill and Sons 1866:32.
16. Vierordt H. Die angeborenen Herzkrankheiten. Northnagel's Handb du spez Pathhol u Therap. Wien: 1898:244.
17. Spitzer A. Uber den Bauplan des normalen und missbildeten Herzens. Versuch einer phylogenetischen theorie. Virchows Arch F Path Anat 1923;243:81-272.
18. Taussig HB, Bing RJ. Complete transposition of the aorta and levoposition of the pulmonary artery. Am Heart J 1949;37: 551-9.
19. Beuren A. Differential diagnosis of the Taussig-Bing heart from complete transposition of the great vessels with a posteriorly overriding pulmonary artery. Circulation 1960;21:1071-87.
20. Braun K, de Vries A, Feingold DS et al. Complete dextroposition of the aorta, pulmonary stenosis, interventricular septal defect, and patent foramen ovale. Am Heart J 1952;43:773-80.
21. Witham AC. Double outlet right ventricle. A partial transposition complex. Am Heart J 1957;53:928-39.
22. Baron MG. Radiologic notes in cardiology. Angiographic differentiation between tetralogy of Fallot and double-outlet right ventricle. Relationship of the mitral and aortic valves. Circulation 1971;43:451-5.
23. Sridaromont S, Ritter DG, Feldt RH et al. Double-outlet right ventricle. Anatomic and angiocardiographic correlations. Mayo Clin Proc 1978;53:555-77.
24. Van Praagh R, Layton WM, Van Praagh S. The morphogenesis of normal and abnormal relationships between the great arteries and the ventricles: pathologic and experimental data. In: Van Praagh R, Takao A (eds) Etiology and Morphogenesis of Congenital Heart Disease. Mount Kisco, NY: Futura, 1980; 271-316.
25. Lev M, Bharati S, Meng CC et al. A concept of double-outlet right ventricle. J Thorac Cardiov Surg 1972;64:271-81.
26. Kirklin JW, Pacifico AD, Bargeron LM Jr, Soto B. Cardiac repair in anatomically corrected malposition of the great arteries. Circulation 1973;48:153-9.
27. Howell CE, Ho SY, Anderson RH, Elliott MJ. Fibrous skeleton and ventricular outflow tracts in double-outlet right ventricle. Ann Thorac Surg 1991;51:394-400.
28. Walters HL 3rd, Mavroudis C, Tchervenkov CI et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg 2000;69(4 Suppl.):S249-63.
29. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.
30. Fyler DC, Bucklet LP, Hellenbrand WE, Cohn HE. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65(Suppl.):375-461.
31. Ferencz C, Rubin JD, McCarter RJ et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985;121:31-6.
32. Grabitz RG, Joffres MR, Collins-Nakai RL. Congenital heart disease: incidence in the first year of life. The Alberta Heritage Pediatric Cardiology Program. Am J Epidemiol 1988;128:381-8.
33. Botto LD, Correa A, Erickson JD. Racial and temporal variations in the prevalence of heart defects. Pediatrics 2001;107: E32.
34. Perry LW, Neill CA, Ferencz C et al. Infants with congenital heart disease: the cases. In: Ferencz C, Rubin JD, Loffredo CA, Magee CA (eds) Perspectives in Pediatric Cardiology Vol. 4: Epidemiology of Congenital Heart Disease The Baltimore-Washington Infant Study 1981-1989. Mount Kisco, NY: Futura 1993;33-62.
35. Triedman JK. Methodological issues for database development: trends. In: Keane JF, Lock JE, Fyler DC (eds) Nadas' Pediatric Cardiology, 2nd edn. Philadelphia: Saunders Elsevier, 2006; 323-36.
36. Ferencz C, Correa-Villasenor A, Loffredo CA, Wilson PD. Malformations of the cardiac outflow tract. In: Ferencz C, Correa-Villasenor A, Loffredo CA, Wilson PD (eds) Perspectives in Pediatric Cardiology Vol. 5: Genetic and Environmental Risk Factors of Major Cardiovascular Malformations The Baltimore-Washington Infant Study 1981-1989. Armonk, NY: Futura, 1997: 59-102.
37. Vuillemin M, Pexieder T, Winking H. Pathogenesis of various forms of double outlet right ventricle in mouse fetal trisomy 13. Int J Cardiol 1991;33:281-304.
38. Van Praagh S, Truman T, Firpo A et al. Cardiac malformations in trisomy-18: a study of 41 postmortem cases. J Am Coll Cardiol 1989;13:1586-97.
39. Goldmuntz E, Clark BJ, Mitchell LE et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol 1998;32:492-8.
40. Brannan CI, Perkins AS, Vogel KS et al. Targeted disruption of the neurofibromatosis type-1 gene leads to developmental abnormalities in heart and various neural crest-derived tissues. Gene Dev 1994;8:1019-29.
41. Epstein JA. Pax3, neural crest and cardiovascular development. Trends Cardiovas Med 1996;6:255-60.
42. Gruber PJ, Kubalak SW, Pexieder T et al. RXR alpha deficiency confers genetic susceptibility for aortic sac, conotruncal, atrioventricular cushion, and ventricular muscle defects in mice. J Clin Invest 1996;98:1332-43.
43. Goldmuntz E, Bamford R, Karkera JD et al. CFC1 mutations in patients with transposition of the great arteries and doubleoutlet right ventricle. Am J Hum Genet 2002;70:776-80.
44. Franco D, Campione M. The role of Pitx2 during cardiac development. Linking left-right signaling and congenital heart diseases. Trends Cardiovasc Med 2003;13:157-63.
45. Pu WT, Ishiwata T, Juraszek AL et al. GATA4 is a dosagesensitive regulator of cardiac morphogenesis. Dev Biol 2004; 275:235-44.
46. Bartram U, Molin DG, Wisse LJ et al. Double-outlet right ventricle and overriding tricuspid valve reflect disturbances of looping, myocardialization, endocardial cushion differentiation, and apoptosis in TGF-beta(2)-knockout mice. Circulation 2001;103:2745-52.
47. Gu H, Smith FC, Taffet SM, Delmar M. High incidence of cardiac malformations in connexin40-deficient mice. Circ Res 2003;93:201-6.
48. Henderson DJ, Phillips HM. Outflow tract remodeling-a role for tissue polarity? In: Artman M, Benson DW, Srivastava D, Nakazawa M (eds) Cardiovascular Development and Congenital Malformations: Molecular and Genetic Mechanisms. Malden, MA: Balckwell Futura, 2005;155-7.
49. Phillips HM, Murdoch JN, Chaudhry B et al. Vangl2 acts via RhoA signaling to regulate polarized cell movements during development of the proximal outflow tract. Circ Res 2005;96: 292-9.
50. Arteaga M, de la Cruz MV, Sanchez C, Diaz GF. Double outlet right ventricle: experimental morphogenesis in the chick embryo heart. Pediatr Cardiol 1982;3:219-27.
51. Kirby ML, Turnage KL 3rd, Hays BM. Characterization of conotruncal malformations following ablation of "cardiac" neural crest. Anat Rec 1985;213:87-93.
52. Ferencz C, Rubin JD, McCarter RJ, Clark EB. Maternal diabetes and cardiovascular malformations: predominance of double outlet right ventricle and truncus arteriosus. Teratology 1990; 41:319-26.
53. Zierler S, Rothman KJ. Congenital heart disease in relation to maternal use of Bendectin and other drugs in early pregnancy. N Engl J Med 1985;313:347-52.
54. Arey LB. The heart and circulation changes. In: Developmental Anatomy: A Textbook and Laboratory Manual of Embryology, revised 7th edn. Philadelphia: WB Saunders, 1974;375-95.
55. Moore KL. The circulatory system: the cardiovascular and lymphatic systems. In: The Developing Human: Clinically Oriented Embryology, 3rd edn. Philadelphia: WB Saunders, 1982;298-343.
56. Manner J. Cardiac looping in the chick embryo: a morphological review with special reference to terminological and biomechanical aspects of the looping process. Anat Rec 2000;259: 248-62.
57. de la Cruz MV, Markwald RR, Krug EL et al. Living morphogenesis of the ventricles and congenital pathology of their component parts. Cardiol Young 2001;11:588-600.
58. Moorman A, Webb S, Brown NA et al. Development of the heart: (1) formation of the cardiac chambers and arterial trunks. Heart (British Cardiac Society) 2003;89:806-14.
59. Anderson RH, Webb S, Brown NA et al. Development of the heart: (2) Septation of the atriums and ventricles. Heart (British Cardiac Society) 2003;89:949-58.
60. Anderson RH, Webb S, Brown NA et al. Development of the heart: (3) formation of the ventricular outflow tracts, arterial valves, and intrapericardial arterial trunks. Heart (British Cardiac Society) 2003;89:1110-8.
61. van den Hoff MJ, Moorman AF, Ruijter JM et al. Myocardialization of the cardiac outflow tract. Dev Biol 1999;212: 477-90.
62. Zamora R, Moller JH, Edwards JE. Double outlet right ventricle: anatomic types and associated anomalies. Chest 1975;68: 672-7.
63. Sridaromont S, Feldt RH, Ritter DG et al. Double outlet right ventricle: hemodynamic and anatomic correlations. Am J Cardiol 1976;38:85-94.
64. Patrick DL, McGoon DC. An operation for double-outlet right ventricle with transposition of the great arteries. J Cardiovasc Surg 1968;9:537-42.
65. Wilkinson JL. Double outlet ventricle. In: Anderson RH, Baker EJ, Macartney FJ et al. (eds) Paediatric Cardiology, 2nd edn. London: Churchill Livingstone, 2002;1353-81.
66. Takeuchi K, Del Nido PJ. Surgical management of doubleoutlet right ventricle with subaortic ventricular septal defect. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2000;3: 34-42.
67. Sondheimer HM, Freedom RM, Olley PM. Double outlet right ventricle: clinical spectrum and prognosis. Am J Cardiol 1977; 39:709-14.
68. Kirklin JW, Barratt-Boyes BG. Double-outlet right ventricle. In: Cardiac Surgery, 2nd edn. New York: Churchill Livingstone, 1993;1469-500.
69. Kleinert S, Sano T, Weintraub RG et al. Anatomic features and surgical strategies in double-outlet right ventricle. Circulation 1997;96:1233-9.
70. Troise DE, Ranieri L, Arciprete PM. Surgical repair for double outlet right ventricle and intact ventricular septum. Ann Thorac Surg 2001;71:1018-9.
71. Anderson RH, McCarthy K, Cook AC. Double outlet right ventricle. Cardiol Young 2001;11:329-44.
72. Van Praagh R, Perez-Trevino C, Reynolds JL et al. Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis. Am J Cardiol 1975;35:42-53.
73. Uemura H, Yagihara T, Kadohama T et al. Repair of double outlet right ventricle with doubly committed ventricular septal defect. Cardiol Young 2001;11:415-9.
74. Pasquini L, Sanders SP, Parness IA et al. Conal anatomy in 119 patients with D-loop transposition of the great arteries and ventricular septal defect: an echocardiographic and pathologic study. J Am Coll Cardiol 1993;21:1712-21.
75. Snider AR, Serwer GA, Ritter SB. Double outlet right ventricle. In: Echocardiography in Pediatric Heart Disease, 2nd edn. St Louis, MO: Mosby, 1997;323-42.
76. Castaneda AR, Jonas RA, Mayer JE, Hanley FL. Double-outlet right ventricle. In: Cardiac Surgery of the Neonate and Infant. Philadelphia: WB Saunders, 1994;445-59.
77. Kawashima Y, Matsuda H, Yagihara T et al. Intraventricular repair for Taussig-Bing anomaly. J Thorac Cardiov Surg 1993;105:591-6; discussion 6-7.
78. Keane JF, Fyler DC. Double-outlet right ventricle. In: Keane JF, Lock JE, Fyler DC (eds) Nadas' Pediatric Cardiology, 2nd edn. Philadelphia: Saunders Elsevier, 2006;735-41.
79. Comas JV, Mignosa C, Cochrane AD, et al. Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome. Eur J Cardiothorac Surg 1996;10:1114-9.
80. Takeuchi K, McGowan FX Jr, Moran AM et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg 2001;71:49-52; discussion 53-3.
81. Serraf A, Belli E, Lacour-Gayet F et al. Biventricular repair for double-outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2000;3:43-56.
82. Fraisse A, del Nido PJ, Gaudart J, Geva T. Echocardiographic characteristics and outcome of straddling mitral valve. J Am Coll Cardiol 2001;38:819-26.
83. Serraf A, Nakamura T, Lacour-Gayet F et al. Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves. J Thorac Cardiov Surg 1996;111:527-35.
84. Aeba R, Katogi T, Takeuchi S, Kawada S. Surgical management of the straddling mitral valve in the biventricular heart. Ann Thorac Surg 2000;69:130-4.
85. Russo P, Danielson GK, Puga FJ et al. Modified Fontan procedure for biventricular hearts with complex forms of doubleoutlet right ventricle. Circulation 1988;78:III20-5.
86. Delius RE, Rademecker MA, de Leval MR et al. Is a high-risk biventricular repair always preferable to conversion to a single ventricle repair? J Thorac Cardiov Surg 1996;112:1561-8; discussion 8-9.
87. Serraf A, Jonas RA, Burke RP et al. Univentricular repair for complex double right ventricle and transposed great arteries. Cardiol Young 1997;7:207-14.
88. Geva T, Van Praagh S, Sanders SP et al. Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations. J Am Coll Cardiol 1991;17:1603-12.
89. Uemura H, Yagihara T, Kawashima Y et al. Coronary arterial anatomy in double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg 1995;59:591-7.
90. Lacour-Gayet F, Haun C, Ntalakoura K et al. Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch. Eur J Cardiothorac Surg 2002;21:1042-8.
91. Gordillo L, Faye-Petersen O, de la Cruz MV, Soto B. Coronary arterial patterns in double-outlet right ventricle. Am J Cardiol 1993;71:1108-10.
92. Tynan MJ, Becker AE, Macartney FJ et al. Nomenclature and classification of congenital heart disease. Br Heart J 1979; 41:544-53.
93. Smallhorn JF. Double-outlet right ventricle: An echocardiographic approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2000;3:20-33.
94. Sanders SP, Bierman FZ, Williams RG. Conotruncal malformations: diagnosis in infancy using subxiphoid 2-dimensional echocardiography. Am J Cardiol 1982;50:1361-7.
95. Van Praagh S. Cardiac malpositions and the heterotaxy syndromes. In: Keane JF, Lock JE, Fyler DC (eds) Nadas' Pediatric Cardiology, 2nd edn. Philadelphia: Saunders Elsevier, 2006; 675-95.
96. Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975;37:840-52.
97. Rubino M, Van Praagh S, Kadoba K et al. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiov Surg 1995;110: 641-50.
98. Lai WW, Ravishankar C, Gross RP et al. Juxtaposition of the atrial appendages: a clinical series of 22 patients. Pediatr Cardiol 2001;22:121-7.
99. Allan L, Hornberger LK, Sharland G. Appendix A.2. In: Allan L, Hornberger LK, Sharland G (eds) Textbook of Fetal Cardiology London: Greenwich Medical Media, 2000;581.
100. Hafner E, Scholler J, Schuchter K et al. Detection of fetal congenital heart disease in a low-risk population. Prenatal Diag 1998;18:808-15.
101. Buskens E, Stewart PA, Hess J et al. Efficacy of fetal echocardiography and yield by risk category. Obstetr Gynecol 1996;87: 423-8.
102. Kim N, Friedberg MK, Silverman NH. Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenatal Diag 2006; 26:740-5.
103. Preminger TJ, Sanders SP, van der Velde ME et al. "Intramural" residual interventricular defects after repair of conotruncal malformations. Circulation 1994;89:236-42.
104. Dorfman AL, Geva T. Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson 2006;8:645-59.
105. Tchervenkov CI, Walters HL 3rd, Chu VF. Congenital Heart Surgery Nomenclature and Database Project: double outlet left ventricle. Ann Thorac Surg 2000;69(4 Suppl.):S264-9.
106. Grant RP. The morphogenesis of transposition of the great vessels. Circulation 1962;26:819-40.
107. Van Mierop LHS, Wiglesworth FW. Pathogenesis of transposition complexes: II. Anomalies due to faulty transfer of the posterior great artery. Am J Cardiol 1963;12:226-32.
108. Sakakibara S, Arai T, Hashimoto A, Nogi M. Both great vessels arising from the left ventricle. Bull Heart Inst Jpn 1967:66-86.
109. Paul MH, Muster AJ, Sinha SN et al. Double-outlet left ventricle with an intact ventricular septum: clinical and autopsy diagnosis and developmental implications. Circulation 1970;41:129-39.
110. Anderson R, Galbraith R, Gibson R, Miller G. Double outlet left ventricle. Br Heart J 1974;36:554-8.
111. Manner J, Seidl W, Steding G. Embryological observations on the formal pathogenesis of double-outlet left ventricle with a right-ventricular infundibulum. Thorac Cardiov Surg 1997; 45:172-7.
112. Bharati S, Lev M, Stewart R et al. The morphologic spectrum of double outlet left ventricle and its surgical significance. Circulation 1978;58:558-65.
113. Van Praagh R, Weinberg PM, Srebro JP. Double outlet left ventricle. In: Adams FH, Emmanoulides GC, Riemenschneider TA (eds) Moss's Heart Disease in Infants, Children, and Adolescents, 4th edn. Baltimore: Williams & Wilkins, 1989;461-85.
114. Kirklin JW, Barratt-Boyes BG. Double outlet left ventricle. In: Cardiac Surgery, 2nd edn. New York: Churchill Livingstone, 1993:1501-9.
115. Freedom RM, Yoo S, Williams WG. Double-outlet ventricle. In: Freedom RM, You S, Makailian H, Williams WG (eds) The Natural and Modified History of Congenital Heart Disease. New York: Futura, 2004;370-80.
116. DeLeon SY, Ow EP, Chiemmongkoltip P et al. Alternatives in biventricular repair of double-outlet left ventricle. Ann Thorac Surg 1995;60:213-6.