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Hearts with isomerism of the right atrial appendages – one of the worst forms of disease in 2005

Published online by Cambridge University Press:  18 November 2005

Robert M. Freedom ,
Edgar T. Jaeggi ,
Joyce S. Lim  and
Robert H. Anderson
Robert M. Freedom
Affiliation:
The Division of Cardiology, the Hospital for Sick Children, and Department of Paediatrics, The University of Toronto, London
Edgar T. Jaeggi
Affiliation:
The Division of Cardiology, the Hospital for Sick Children, and Department of Paediatrics, The University of Toronto, London
Joyce S. Lim
Affiliation:
The Division of Cardiology, the Hospital for Sick Children, and Department of Paediatrics, The University of Toronto, London
Robert H. Anderson
Affiliation:
The Cardiac Unit, Institute of Child Health, University College, London
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Abstract

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Keywords

Visceral heterotaxyasplenialeft isomerismobstructed pulmonary venous connectionsoutcomespolysplenia
Type
Continuing Medical Education
Information
Cardiology in the Young , Volume 15 , Issue 6 , December 2005 , pp. 554 - 567
Copyright
© 2005 Cambridge University Press

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References

Roberts WC. Aortic atresia. The worst heart disease. Am J Cardiol 1984; 54: 1169.Google Scholar
Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: Experience with palliative surgery. Am J Cardiol 1980; 45: 87–92.Google Scholar
Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia–hypoplastic left heart syndrome. N Engl J Med 1983; 308: 23–26.Google Scholar
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.Google Scholar
Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 1120–1126.Google Scholar
Lim JS, McCrindle BW, Smallhorn JF, Jaeggi ET. Outcomes of children with fetal or postnatal diagnosis of heterotaxy syndromes: a single institution's experience since 1990. Circulation 2004; 110: III-495 (2336).Google Scholar
Roberts WC, Anderson RC, Edwards JE. The significance of asplenia in the recognition of inoperable congenital heart disease. Circulation 1961; 26: 851–857.Google Scholar
Ivemark BI. Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood. Acta Paediatrica 1955; 44 (Suppl 104): 7–110.Google Scholar
Putschar WGJ, Manion WC. Congenital absence of the spleen and associated anomalies. Am J Clin Path 1956; 26: 429–469.Google Scholar
Brandt HM, Liebow AA. Right pulmonary isomerism associated with venous, splenic, and other anomalies. Lab Invest 1958; 7: 469–503.Google Scholar
Forde WJ, Finby N. Roentgenographic features of congenital asplenia: a tera-tologic syndrome of visceral symmetry. AJR 1961; 86: 523–526.Google Scholar
Lucas Jr RV, Neufeld HN, Lester RG, Edwards JE. The symmetrical liver as a roentgen sign of asplenia. Circulation 1962; 25: 973–975.Google Scholar
Landing B. Five syndromes (malformation complexes) of pulmonary symmetry, congenital heart disease, and multiple spleens. Pediatr Pathol 1984; 2: 125–151.Google Scholar
Landing BH, Lawrence T-YK, Payne Jr VC, Wells TR. Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease. Am J Cardiol 1971; 28: 456–462.Google Scholar
Diehl EJ, Landing BH. Syndrome of pulmonary isomerism of right lung type, congenital heart disease, pulmonary and systemic venous abnormalities and malrotation of the intestine, with a spleen or multiple spleens (m-anisosplenia): comparison with Ivemark asplenia syndrome. Pediatr Pathol 1984; 2: 133–138.Google Scholar
Layman TE, Levine MA, Amplatz K, Edwards JE. “Asplenic syndrome” in association with rudimentary spleen. Am J Cardiol 1967; 20: 136–139.Google Scholar
Anderson C, Devine WA, Anderson RH, Debich DE, Zuberbuhler JR. Abnormalities of the spleen in relation to congenital malformation of the heart: a survey of necropsy findings in children. Br Heart J 1990; 63: 122–128.Google Scholar
Muir CS. Splenic agenesis and multilobulate spleen. Arch Dis Child 1959; 34: 431–433.Google Scholar
Van Mierop LHS, Patterson PR, Reynold RW. Two cases of congenital asplenia with isomerism of the cardiac atria and the sinoatrial nodes. Am J Cardiol 1964; 13: 407–412.Google Scholar
Van Mierop LHS, Wiglesworth FW. Isomerism of the cardiac atria in the asplenia syndrome. Lab Invest 1962; 11: 1303–1307.Google Scholar
Van Mierop LHS, Gessner IH, Schiebler GL. Asplenia and polysplenia syndromes. Birth Defects: Original Article Series 1972; VIII: 36–44.
Elliott LP, Cramer GG, Amplatz K. The anomalous relationship of the inferior vena cava and abdominal aorta as a specific angiocardiographic sign in asplenia. Radiology 1966; 87: 859–863.Google Scholar
Freedom RM, Fellows KE. Roentgenographic visceral patterns in the asplenia syndrome. Radiology 1973; 107: 387–392.Google Scholar
Becker AE, Anderson RH. Isomerism of the atrial appendages-goodbye to asplenia and all that. In: Clark EB, Takao A (eds). Developmental Cardiology. Morphogenesis and function. Futura Publishing Co., Mt. Kisco, New York, 1990, pp 659–670.
Anderson RH, Webb S, Brown NA. Defective lateralisation in children with congenitally malformed hearts. Cardiol Young 1998; 8: 512–531.Google Scholar
Anderson RH. Is isomerism of the atrial appendages a real thing? Cardiol Young 1995; 5: 207–208.Google Scholar
Uemura H, Ho SY, Devine WA, Anderson RH. Atrial appendages and venoatrial connections in hearts with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
Lababidi Z, Stoeckle H, Hopeman A, Almond C. Congenital asplenia with bilateral right-sidedness and normally related great arteries. Case report. Mo Med 1972; 69: 742–745.Google Scholar
Phoon CKL. Where's the spleen? Looking for the spleen and assessing its function in the syndromes of isomerism. Cardiol Young 1997; 7: 147–157.Google Scholar
Overturf GD and the Committee on infectious diseases. Technical report: prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Paediatrics 2000; 106: 367–376.Google Scholar
Berg C, Geipel A, Smrcek J, et al. Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience. Ultrasound Obstet Gynecol 2003; 22: 451–459.Google Scholar
Winer-Muram HT, Tonkin IL. The spectrum of heterotaxic syndromes. Radiol Clin North Am 1989; 27: 1147–1170.Google Scholar
Freedom RM, Treves S. Splenic scintigraphy and radionuclide venography in the heterotaxy syndrome. Radiology 1973; 107: 381–386.Google Scholar
Van Praagh S, Kreutzer J, Alday L, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy, with emphasis on the diagnosis of the atrial situs: a study of 109 postmortem cases. In: Clark EB, Takao A (eds). Developmental Cardiology. Morphogenesis and function. Futura Publishing Co., Mt. Kisco, New York, 1990, pp 671–727.
Ruscazio M, Van Praagh S, Marrass AR, Catani G, Iliceto S, Van Praagh R. Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol 1998; 81: 111–116.Google Scholar
Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol 1995; 76: 846–849.Google Scholar
Van Praagh R, Van Praagh S. Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept. Am J Cardiol 1990; 66: 1504–1506.Google Scholar
Van Praagh S, Kakou-Guikahue M, Kim HS, Van Praagh R. Atrial situs in patients with visceral heterotaxy and congenital heart disease: conclusions based on findings in 104 postmortem cases. Coeur 1988; 19: 484–502.Google Scholar
Van Praagh R, David I, Wright G. Large RV plus small LV is not single ventricle. Circulation 1980; 61: 1057–1058.Google Scholar
Liu C, Liu W, Lu MF, Brown NA, Martin JF. Regulation of left-right asymmetry by thresholds of Pitx2c activity. Development 2001; 128: 2039–2048.Google Scholar
Bamforth SD, Bragan J, Farthing CR, Schneider JE, Broadbent C, Michell AC, Clarke K, Neubauer S, Norris D, Brown NA, Anderson RH, Bhattacharya S. Cited2 controls left-right patterning and heart development through a Nodal-Pitx2c pathway. Nat Genet 2004; 36: 1189–1196.Google Scholar
Rose V, Izukawa T, Möes CAF. 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: 840–852.Google Scholar
Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg 1995; 110: 641–650.Google Scholar
Freedom RM. Aortic valve and arch anomalies in the congenital asplenia syndrome. Case report, literature review and re-examination of the embryology of the congenital asplenia syndrome. Johns Hopkins Med J 1974; 135: 124–135.Google Scholar
Van Praagh S, Geva T, Friedberg DZ, et al. Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases. Am Heart J 1997; 133: 558–569.Google Scholar
De Tommasi SM, Daliento L, Ho SY, Macartney FJ, Anderson RH. Aortic atresia associated with the right atrial isomeric form of situs ambiguus. G Ital Cardiol 1981; 11: 2235–2238.Google Scholar
Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000; 36: 908–916.Google Scholar
Formigari R, Vairo U, de Zorzi A, Santoro G, Marino B. Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol 1992; 70: 1219–1220.Google Scholar
Vitiello R, Moller JH, Marino B, Vairo U, Edwards JE, Titus JL. Pulmonary circulation in pulmonary atresia associated with the asplenia syndrome. J Am Coll Cardiol 1992; 20: 363–365.Google Scholar
Freedom RM. The asplenia syndrome: a review of significant extracardiac structural abnormalities in 29 necropsied patients. J Pediatr 1972; 81: 1130–1133.Google Scholar
Ticho BS, Goldstein AM, Van Praagh R. Extracardiac anomalies in the heterotaxy syndrome with focus on anomalies of mid-line associated structures. Am J Cardiol 2000; 85: 729–734.Google Scholar
Devriendt K, Naulaers G, Matthijs G, et al. Agenesis of corpus callosum and anophthalmia in the asplenia syndrome. A recognisable association? Ann Genet 1997; 40: 14–17.Google Scholar
Gilbert-Barness E, Debich-Spicer D, Cohen Jr MM, Opitz JM. Evidence for the “midline” hypothesis in associated defects of laterality formation and multiple midline anomalies. Am J Med Genet 2001; 101: 382–387.Google Scholar
Belmont JW, Mohapatra B, Towbin JA, Ware SM. Molecular genetics of heterotaxy syndromes. Curr Opin Cardiol 2004; 19: 216–220.Google Scholar
Yost HJ. Establishment of left–right asymmetry. Int Rev Cytol 2001; 203: 367–381.Google Scholar
Mercola M. Embryological basis for cardiac left–right asymmetry. Sem Cell Dev Biol 1999; 10: 109–116.Google Scholar
Krebs LT, Iwai N, Nonaka S, et al. Notch signaling regulates left-right asymmetry determination by inducing Nodal expression. Genes Dev 2003; 17: 1207–1212.Google Scholar
Kosaki K, Casey B. Genetics of human left–right axis malformations. Cell Dev Biol 1998; 9: 89–99.Google Scholar
Peeters H, Debeer P, Bairoch A, et al. PA26 is a candidate gene for heterotaxia in humans: identification of a novel PA26-related gene family in human and mouse. Hum Genet 2003; 112: 573–580.Google Scholar
Ware SM, Peng J, Zhu L, et al. Identification and functional analysis of ZIC3 mutations in heterotaxy and related congenital heart defects. Am J Hum Genet 2004; 74: 93–105.Google Scholar
Bamford RN, Roessler E, Burdine RD, et al. Loss-of-function mutations in the EGF-CFC gene CFC1 are associated with human left-right laterality defects. Nat Genet 2000; 26: 365–369.Google Scholar
Kim SH, Son CS, Lee JW, Tockgo YC, Chun YH. Visceral heterotaxy syndrome induced by retinoids in mouse embryo. J Korean Med Sci 1995; 10: 250–257.Google Scholar
Lee YM, Kim JS, Han SY, Park KL, Jang SJ, Seo JW. Abnormal ventricular looping and abnormal laterality of the atrial chambers are the main morphogenetic mechanisms of cardiac lesions in cultured rat embryos treated with retinoic acid. J Korean Med Sci 1998; 13: 117–122.Google Scholar
Colbert MC. Retinoids and cardiovascular developmental defects. Cardiovasc Toxicol 2002; 2: 25–39.Google Scholar
Kuehl KS, Loffredo C. Risk factors for heart disease associated with abnormal sidedness. Teratology 2002; 66: 242–248.Google Scholar
Zlotogora J, Elian E. Asplenia and polysplenia syndromes with abnormalities of lateralisation in a sibship. J Med Genet 1981; 18: 301–302.Google Scholar
Peeters H, Debeer Ph, Groenen P, et al. Recurrent involvement of chromosome region 6q21 in heterotaxy. Am J Med Gen 2001; 103: 44–47.Google Scholar
Katcher AL. Familial asplenia, other malformations and sudden death. Pediatrics 1980; 65: 633–635.Google Scholar
Simpson J, Zellweger H. Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs. J Med Genet 1973; 10: 303–304.Google Scholar
Boughman JA, Neill CA, Ferencz C, Loffredo CA. The genetics of congenital heart disease. In: Ferencz C, Rubin JD, Loffredo CA, Magee CA (eds). Epidemiology of Congenital Heart disease. The Baltimore-Washington Infant Study 1981–1989. Futura Publishing Co, Inc., Mount Kisco, NY, 1993, pp 123–167.
Simpson J, Zellweger H. Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs. J Med Genet 1973; 10: 303–304.Google Scholar
Devriendt K, Casaer A, Van Cauter A, et al. Asplenia syndrome and isolated total anomalous pulmonary venous connection in siblings. Eur J Pediatr 1994; 153: 712–714.Google Scholar
Casey B, Cuneo BF, Vitali C, et al. Autosomal dominant transmission of familial laterality defects. Am J Med Genet 1996; 61: 325–328.Google Scholar
Eronen M, Kajantie E, Boldt T, Pitkanen O, Aittomaki K. Right atrial isomerism in four siblings. Pediatr Cardiol 2004; 25: 141–144.Google Scholar
Gatrad AR, Read AP, Watson GH. Consanguinity and complex cardiac anomalies with situs ambiguous. Arch Dis Child 1984; 59: 242–245.Google Scholar
Lin AE, Ticho BS, Houde K, Westgate MN, Holmes LB. Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2000; 2: 157–172.Google Scholar
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: Prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985; 121: 31–36.Google Scholar
Atkinson DE, Drant S. Diagnosis of heterotaxy syndrome by fetal echocardiography. Am J Cardiol 1999; 83: 818–819.Google Scholar
Comstock CH, Smith R, Lee W, Kirk JS. Right fetal cardiac axis: clinical significance and associated findings. Obstet Gynecol 1998; 91: 495–499.Google Scholar
Patel CR, Lane JR, Muise KL. In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia. Ultrasound Obstet Gynecol 2001; 17: 268–271.Google Scholar
Abuhamad AZ, Robinson JN, Bogdan D, Tannous RJ. Color Doppler of the splenic artery in the prenatal diagnosis of heterotaxic syndromes. Am J Perinatol 1999; 16: 469–473.Google Scholar
Sharland G, Cook A. Heterotaxy syndromes/isomerism of the atrial appendages. In: Allan L, Hornberger L, Sharland G (eds). Textbook of Fetal Cardiology. Greenwich Medical Media Limited, London, 2000, pp 333–347.
Yoo SJ, Lee YH, Cho KS, Kim DY. Sequential segmental approach to fetal congenital heart disease. Cardiol Young 1999; 9: 430–444.Google Scholar
Yoo SJ, Lee YH, Cho KS. Abnormal three-vessel view on sonography: a clue to the diagnosis of congenital heart disease in the fetus. AJR 1999; 172: 825–830.Google Scholar
Brown DL, Emerson DS, Shulman LP, Doubilet PM, Felker RE, Van Praagh S. Predicting aneuploidy in fetuses with cardiac anomalies: significance of visceral situs and noncardiac anomalies. J Ultrasound Med 1993; 12: 153–161.Google Scholar
Lin J-H, Chang C-I, Wang J-K, et al. Intrauterine diagnosis of heterotaxy syndrome. Am Heart J 2002; 143: 1002–1008.Google Scholar
Huggon IC, Cook AC, Smeeton NC, Magee AG, Sharland GK. Atrioventricular septal defects diagnosed in fetal life: associated cardiac and extra-cardiac abnormalities and outcome. J Am Coll Cardiol 2000; 36: 593–601.Google Scholar
Walmsley R, Hishitani T, Sandor GG, et al. Diagnosis and outcome of dextrocardia diagnosed in the fetus. Am J Cardiol 2004; 94: 141–143.Google Scholar
Valsangiacomo ER, Hornberger LK, Barrea C, Smallhorn JF, Yoo SJ. Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings. Ultrasound Obstet Gynecol 2003; 22: 257–263.Google Scholar
Yasukochi S, Satomi G, Iwasaki Y. Prenatal diagnosis of total anomalous pulmonary venous connection with asplenia. Fetal Diagn Ther 1997; 12: 266–269.Google Scholar
Allan LD, Sharland GK. The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus. Heart 2001; 85: 433–437.Google Scholar
Noonan JA, Nadas AS. The hypoplastic left heart syndrome. Pediatr Clin N Am 1958; 5: 1029–1056.Google Scholar
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240–248.Google Scholar
Fyler DC. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980; 65 (Suppl): 376–461.Google Scholar
Natowicz M, Chatten J, Clancy R, et al. Genetic disorders and major extracardiac anomalies associated with the hypoplastic left heart syndrome. Pediatrics 1988; 82: 698–706.Google Scholar
Glauser TA, Rorke LB, Weinberg PM, Clancy RR. Congenital brain anomalies associated with the hypoplastic left heart syndrome. Pediatrics 1990; 85: 984–990.Google Scholar
Freedom RM, Yoo S-J. The hypoplastic left heart syndrome. In: Freedom RM, Yoo SJ, Mikailian H, Williams WG (eds). The Natural and Modified History of Congenital Heart Disease. Blackwell-Futura Publishing Co., Inc, Oxford, UK and New York, 2004, pp 397–407.
Ashburn DA, McCrindle BW, Tchervenkov CI, et al. Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 2003; 125: 1070–1082.Google Scholar
Forbess JM, Cook N, Serraf A, Burke RP, Mayer JE, Jonas RA. An institutional experience with second- and third-stage palliative procedures for hypoplastic left heart syndrome: the impact of the bidirectional cavo-pulmonary shunt. J Am Coll Cardiol 1997; 29: 665–670.Google Scholar
Jacobs ML, Blackstone EH, Bailey LL. Intermediate survival in neonates with aortic atresia: a multi-institutional study. The Congenital Heart Surgeons Society. J Thorac Cardiovasc Surg 1998; 116: 417–431.Google Scholar
Azakie A, McCrindle BW, Benson LN, et al. Total cavopulmonary connections in children with a previous Norwood procedure. Ann Thorac Surg 2001; 71: 1541–1546.Google Scholar
Allan LD, Cook A, Sullivan I, Sharland G. Changing birth prevalence of the hypoplastic left heart syndrome as a result of fetal echocardiography. Lancet 1991; 337: 959–961.Google Scholar
Sharland G. Aortic valve abnormalities. In: Allan L, Hornberger L, Sharland G (eds). Textbook of Fetal Cardiology. Greenwich Medical Media Limited, London, 2000, pp 213–219.
Sharland G, Rollings S, Simpson J, Anderson D. Hypoplastic left-heart syndrome. Lancet 2001; 357: 722–723.Google Scholar
Estevez JM, Gaddy CG. The asplenia syndrome. Presentation of a case with severe cardiovascular abnormalities and unusual longevity. Virginia Med Month 1973; 102: 627–633.Google Scholar
Mauck HP, Segatol-Islami Z, Lester RG. Splenic agenesis associated with severe congenital heart disease: long survival unassociated with pulmonary stenosis. Report of a case. Dis Chest 1966; 49: 436–440.Google Scholar
Wolfe MW, Vacek JL, Kinard RE, Bailey CG. Prolonged and functional survival with the asplenia syndrome. Am J Med 1986; 81: 1089–1091.Google Scholar
Raman R, Al-Ali SY, Poole CA, Dawson BV, Carman JB, Calder L. Isomerism of the right atrial appendages: clinical, anatomical, and microscopic study of a long-surviving case with asplenia and ciliary abnormalities. Clin Anat 2003; 16: 269–276.Google Scholar
Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism. Report of seventeen cases. J Thorac Cardiovasc Surg 1995; 109: 530–535.Google Scholar
Okita Y, Miki S, Ueda Y, et al. Successful repair of the right atrial isomerism, double outlet right ventricle, common atrioventricular canal, pulmonary stenosis, and total anomalous pulmonary venous connection. J Card Surg 1994; 9: 433–439.Google Scholar
Kawashima Y, Matsuda H, Naito Y, Yagihara T, Kadoba K, Matsuki O. Biventricular repair of cardiac isomerism with common atrioventricular canal with the aid of an endocardial cushion prosthesis. J Thorac Cardiovasc Surg 1993; 106: 248–254.Google Scholar
Ando F, Shirotani H, Kawai J, et al. Successful total repair of complicated cardiac anomalies with asplenia syndrome. J Thorac Cardiovasc Surg 1976; 72: 33–37.Google Scholar
Oshima Y, Yamaguchi M, Yoshimura N, Oka S, Ootaki Y. Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies. Ann Thorac Surg 2001; 72: 424–429.Google Scholar
Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Staged unifocalization and anatomic repair in a patient with right isomerism. Ann Thorac Surg 2001; 71: 2039–2041.Google Scholar
Azakie A, Merklinger SL, Williams WG, Van Arsdell GS, Coles JG, Adatia I. Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 2001; 72: 1636–1640.Google Scholar
Aeba R, Katogi T, Hashizume K, Iino Y, Kawada S, Yuasa Y. Individualized total cavopulmonary connection technique for patients with asplenia syndrome. Ann Thorac Surg 2002; 73: 1274–1280.Google Scholar
Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 2002; 74: 1967–1978.Google Scholar
Sinzobahamvya N, Arenz C, Brecher AM, Urban AE. Atrial isomerism: a surgical experience. Cardiovasc Surg 1999; 7: 436–442.Google Scholar
Culbertson CB, George BL, Day RW, Laks H, Williams RG. Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure. J Am Coll Cardiol 1992; 20: 678–684.Google Scholar
Humes RA, Feldt RH, Porter CJ, Julsrud PR, Puga FJ, Danielson GK. The modified Fontan operation for asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988; 96: 212–218.Google Scholar
Stein JI, Smallhorn JF, Coles JG, Williams WG, Trusler GA, Freedom RM. Common atrioventricular valve guarding double inlet atrioventricular connexion: natural history and surgical results in 76 cases. Int J Cardiol 1990; 28: 7–17.Google Scholar
Kawahira Y, Uemura H, Yoshikawa Y, Yagihara T. Double inlet right ventricle versus other types of double or common inlet ventricle: its clinical characteristics with reference to the Fontan procedure. Eur J Cardiovasc Surg 2001; 20: 228–233.Google Scholar
Imai Y, Seo K, Terada M, et al. Valvular repair for atrioventricular regurgitation in complex anomalies in modified Fontan procedure with reference to a single ventricle associated with a common atrioventricular valve. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 1999; 2: 5–19.Google Scholar
Imai Y, Takanashi Y, Hoshino S, Terada M, Aoki M, Ohta J. Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation. J Thorac Cardiovasc Surg 1997; 113: 262–268; discussion 269.Google Scholar
Mahle WT, Gaynor JW, Spray TL. Atrioventricular valve replacement in patients with a single ventricle. Ann Thorac Surg 2001; 72: 182–186.Google Scholar
Okita Y, Miki S, Tamura T, et al. Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism. Ann Thorac Surg 1989; 47: 302–304.Google Scholar
Kawai T, Wada Y, Enmoto T, et al. Surgical palliation of cardiac malformations associated with right isomerism. Surg Today 1995; 25: 525–531.Google Scholar
Marcelletti C, Di Donato R, Nijveld A, et al. Right and left isomerism: the cardiac surgeon's view. Ann Thorac Surg 1983; 35: 400–405.Google Scholar
Michielon G, Gharagozloo F, Julsrud PR, Danielson GK, Puga FJ. Modified Fontan operation in the presence of anomalies of systemic and pulmonary venous connection. Circulation 1993; 88: 141–148.Google Scholar
McElhinney DB, Reddy VM, Moore P, Hanley FL. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997; 63: 1676–1684.Google Scholar
Franklin RC, Spiegelhalter DJ, Anderson RH, et al. Double-inlet ventricle presenting in infancy. I. Survival without definitive repair. J Thorac Cardiovasc Surg 1991; 101: 767–776.Google Scholar
Franklin RC, Spiegelhalter DJ, Anderson RH, et al. Double-inlet ventricle presenting in infancy. II. Results of palliative operations. J Thorac Cardiovasc Surg 1991; 101: 917–923.Google Scholar
Franklin RC, Spiegelhalter DJ, Rossi Filho RI, et al. Double-inlet ventricle presenting in infancy. III. Outcome and potential for definitive repair. J Thorac Cardiovasc Surg 1991; 101: 924–934.Google Scholar
Sadiq M, Stumper O, De Giovanni JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart 1996; 75: 314–319.Google Scholar
Cheung YF, Cheng VY, Chau AK, Chiu CS, Yung TC, Leung MP. Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage? Heart 2002; 87: 146–152.Google Scholar
Leonard H, Derrick G, O'Sullivan J, Wren C. Natural and unnatural history of pulmonary atresia. Heart 2000; 84: 499–503.Google Scholar
Phoon CK, Neill CA. Asplenia syndrome – risk factors for early unfavorable outcome. Am J Cardiol 1994; 73: 1235–1237.Google Scholar
Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999; 117: 506–514.Google Scholar
Wang JK, Lue HC, Chiu IS, Wu MH, Chang CI. Masked gradient of infundibular stenosis in right atrial isomerism with pulmonary venous obstruction. Am J Cardiol 1994; 73: 829–831.Google Scholar
Chiu I-S, Wang N-K, Wu M-H, Wu F-F, Hung C-R. Concealed pulmonary venous obstruction in right atrial isomerism with pulmonary outflow tract obstruction-surgical management following Blalock-Taussig shunt. Cardiol Young 1992; 2: 95–99.Google Scholar
Freedom RM, Olley PM, Coceani F, Rowe RD. The prostaglandin challenge: test to unmask obstructed total anomalous pulmonary venous connections in asplenia syndrome. Br Heart J 1978; 40: 91–94.Google Scholar
Mahnke CB, Sandor GG, Boyle GJ, Webber SA. “Masked” pulmonary venous obstruction in patients with isomerism of the right atrial appendages: an overstated association. Cardiol Young 2002; 12: 113–118.Google Scholar
Chowdhury UK, Alran B, Sharma R, et al. Surgical considerations of univentricular heart with total anomalous pulmonary venous connection. Ind Heart J 2000; 52: 192–197.Google Scholar
DeLeon SY, Gidding SS, Ilbawi MN, et al. Surgical management of infants with complex cardiac anomalies associated with reduced pulmonary blood flow and total anomalous pulmonary venous drainage. Ann Thorac Surg 1987; 43: 207–211.Google Scholar
Jenkins KJ, Sanders SP, Orav EJ, Coleman EA, Mayer Jr JE, Colan SD. Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. J Am Coll Cardiol 1993; 22: 201–206.Google Scholar
Kawahira Y, Kishimoto H, Kawata H, et al. New indicator for the Fontan operation: diameters of the pulmonary veins in patients with univentricular heart. J Card Surg 1999; 14: 259–265.Google Scholar
Calderone CA, Najm HK, Kadletz M, et al. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg 1998; 66: 1521–1526.Google Scholar
Heinemann MK, Hanley FL, Van Praagh S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg 1994; 57: 88–91.Google Scholar
Ootaki Y, Yamaguchi M, Oshima Y, Yoshimura N, Oka S. Repair of total anomalous pulmonary venous connection without cardiopulmonary bypass. Ann Thorac Surg 2001; 72: 249–251.Google Scholar
Lamberti JJ, Waldman JD, Mathewson JW, Kirkpatrick SE. Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass. J Thorac Cardiovasc Surg 1984; 88: 627–630.Google Scholar
Kaneka Y, Hirata Y, Yagyu K, Murakami A, Takamoto S. Pulmonary-to-systemic blood flow ratio oriented management after repair of obstructive total anomalous pulmonary venous connection in neonates with single ventricle. Ann Thorac Surg 2003; 75: 1010–1012.Google Scholar
Razzouk AJ, Gundry SR, Chinnock RE, et al. Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart disease. J Heart Lung Transplant 1995; 14: 713–717.Google Scholar
Berdat PA, Mohacsi P, Althaus U, Carrel T. Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection. Eur J Cardiothorac Surg 1998; 14: 631–634.Google Scholar
Devine WA, Webber SA, Anderson RH. Congenitally malformed hearts from a population of children undergoing cardiac transplantation: comments on sequential segmental analysis and dissection. Pediatr Dev Pathol 2000; 3: 140–154.Google Scholar
Larsen RL, Eguchi JH, Mulla NF, et al. Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. Am J Cardiol 2002; 89: 1275–1279.Google Scholar
Cheung YF, Cheng VF, Yung TC, Chau AK. Cardiac rhythm and symptomatic arrhythmia in right atrial isomerism. Am Heart J 2002; 144: 159–164.Google Scholar
Wu MH, Wang JK, Lin JL, et al. Supraventricular tachycardia in patients with right atrial isomerism. J Am Coll Cardiol 1998; 32: 773–779.Google Scholar
Wren C, Macartney FJ, Deanfield JE. Cardiac rhythm in atrial isomerism. Am J Cardiol 1987; 59: 1156–1158.Google Scholar
Wu MH, Wang JK, Lue HC. Sudden death in patients with right isomerism (asplenia) after palliation. J Pediatr 2002; 140: 93–96.Google Scholar