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

Published online by Cambridge University Press:  03 February 2006

Robert M. Freedom ,
Edgar Jaeggi ,
Don Perrin ,
Shi-joon Yoo  and
Robert H. Anderson
Robert M. Freedom
Affiliation:
Division of Cardiology, the Hospital for Sick Children and Department of Paediatrics and Pathology, the University of Toronto, Canada Department of Pathology and Laboratory Medicine, the Hospital for Sick Children, and Department of Paediatrics and Pathology, the University of Toronto, Canada
Edgar Jaeggi
Affiliation:
Division of Cardiology, the Hospital for Sick Children and Department of Paediatrics and Pathology, the University of Toronto, Canada
Don Perrin
Affiliation:
Department of Pathology and Laboratory Medicine, the Hospital for Sick Children, and Department of Paediatrics and Pathology, the University of Toronto, Canada
Shi-joon Yoo
Affiliation:
Division of Cardiology, the Hospital for Sick Children and Department of Paediatrics and Pathology, the University of Toronto, Canada Division of Radiology, the Hospital for Sick Children and Department of Paediatrics and Pathology, the University of Toronto, Canada
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, University College London, United Kingdom
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Abstract

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Keywords

Ebstein's malformationtricuspid regurgitationUhl's anomaly of the right ventricle
Type
Continuing Medical Education
Information
Cardiology in the Young , Volume 16 , Issue 1 , January 2006 , pp. 18 - 29
Copyright
© 2006 Cambridge University Press

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References

Roberts WC. Aortic atresia. The worst heart disease. Am J Cardiol 1984; 54: 1169.Google Scholar
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.
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.Google Scholar
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.Google Scholar
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.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
Freedom RM, Perrin D. Ventriculocoronary arterial connections in pulmonary atresia and intact ventricular septum. Cardiol Young 2005; 15: 447–468.Google Scholar
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.Google Scholar
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.Google Scholar
Bharati S, McAllister HAJ, Chiemmongkoltip P, Lev M. Congenital pulmonary atresia with tricuspid insufficiency: morphologic study. Am J Cardiol 1977; 40: 70–75.Google Scholar
Freedom RM, Dische MR, Rowe RD. The tricuspid valve in pulmonary atresia and intact ventricular septum. Arch Pathol Lab Med 1978; 102: 28–31.Google Scholar
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.Google Scholar
Long WA, Willis PW. Maternal lithium and neonatal Ebstein's. Am J Perinatol 1984; 1: 182–184.Google Scholar
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.Google Scholar
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.Google Scholar
Hoffman JIE, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am Heart J 2004; 147: 425–439.Google Scholar
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.Google Scholar
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.Google Scholar
Freedom RM, Culham JAG, Moes CAF. Angiocardiography of Congenital Heart Disease. Macmillan Publishing Co., New York, 1984, 221–253.
Patel CR, Spector ML, Zahka KG. Congenitally corrected transposition with pulmonary atresia and intact ventricular septum. Cardiol Young 2000, 10: 268–270.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
Muster AJ, Idriss FS, Bharati S, et al. Functional aortic valve atresia in transposition of the great arteries. JACC 1985; 6: 630–634.Google Scholar
Chan KC, Da Costa P, Dickinson DF. Functional aortic atresia in congenitally corrected transposition. Int J Cardiol 1989; 25: 237–239.Google Scholar
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.Google Scholar
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.Google Scholar
Freedom RM, Mawson J, Yoo S-J, Benson LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co., Armonk, NY, 1997; pp 617–672.
Haworth SG, Shinebourne EA, Miller GAH. Right-to-left interatrial 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.Google Scholar
Rowlatt JF, Rimoldi MJA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963; 10: 499–588.Google Scholar
Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281–288.Google Scholar
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.Google Scholar
Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation 1964; 30: 911–917.Google Scholar
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.Google Scholar
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.Google Scholar
Freedom RM, Benson LN. Neonatal expression of Ebstein's anomaly. Prog Pediatr Cardiol 1993; 2: 22–27.Google Scholar
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.Google Scholar
Gerlis LM, Anderson RH. Cor triatriatum dexter with imperforate Ebstein's anomaly. Br Heart J 1976; 38: 108–111.Google Scholar
Zielinsky P, Pilla CB. Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis. Arq Bras Cardiol 2000; 75: 62–64.Google Scholar
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.Google Scholar
Fuertes J, Salazar J, Mengual J, et al. Anomalie d'Uhl associee a une atresia de la valvule piulmonaire. Pediatrie 1984; 39: 213–217.Google Scholar
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.Google Scholar
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.Google Scholar
Uhl HSM. Uhl's anomaly revisited. Circulation 1996; 93: 1483–1484.Google Scholar
Smallhorn JF, Izukawa T, Benson L, Freedom RM. Noninvasive recognition of functional pulmonary atresia by echocardiography. Am J Cardiol 1984; 54: 925–926.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
Haworth SG, Reid L. Quantitative structural study of pulmonary circulation in the newborn with pulmonary atresia. Thorax 1977; 32: 129–133.Google Scholar
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.Google Scholar
Naeye RC. Perinatal changes in the pulmonary vascular bed with stenosis and atresia of the pulmonic valve. Am Heart J 1961; 61: 586–592.Google Scholar
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.Google Scholar
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.Google Scholar
Rice MJ, McDonald RW, Reller MD. Progressive pulmonary stenosis in the fetus: two case reports. Am J Perinatol 1993; 10: 424–427.Google Scholar
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.Google Scholar
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.Google Scholar
Maeno Y, Himeno W, Fujino H, et al. Progression of congenital heart disease in the prenatal period. Pediatr Int 1999; 41: 709–715.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
Roberson DA, Silverman NH. Ebstein's Anomaly: Echocardiographic and Clinical features in the Fetus and Neonate. J Am Coll Cardiol 1989; 14: 1300–1307.Google Scholar
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.Google Scholar
Allan LD, Cook A. Pulmonary atresia with intact ventricular septum in the fetus. Cardiol Young 1992; 2: 367–376.Google Scholar
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.Google Scholar
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.Google Scholar
Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol 1998; 81: 749–754.Google Scholar
Rowe RD, Freedom RM, Mehrizi A. The Neonate with Congenital Heart Disease, Second Edition; WB Saunders Co., New York, 1981, pp 515–528.
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.Google Scholar
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.Google Scholar
Dunbar I, Loehr J, Schaffer M. Usefulness of prolonged prostaglandin infusion in neonates with Ebstein's anomaly. Am J Cardiol 1993; 72: 1327–1329.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
McCrindle BW. Commentary to Ref. 96. J Thorac Cardiovasc Surg 1999; 118: 1052–1055.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.Google Scholar
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.
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.
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.Google Scholar
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.Google Scholar
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.Google Scholar
Tworetzky W, Marshall AC. Balloon valvuloplasty for congenital heart disease in the fetus. Clin Perinatol 2003, 30: 541–550.Google Scholar
Tworetzky W, Marshall AC. Fetal interventions for cardiac defects. Pediatr Clin North Am 2004; 51: 1503–1513.Google Scholar
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.Google Scholar
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.Google Scholar