Abstract
Tricuspid atresia is the 19th most common congenital heart defect (0.7–1.6% of all congenital heart defects in clinical series, and up to 2.9% in autopsy series), but is the third most common cyanotic malformation presenting in the neonatal period, after transposition of the great arteries and tetralogy of Fallot. A slight male preponderance exists for tricuspid atresia, particularly in the presence of associated ventriculoarterial discordance.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Acar P, Saliba Z, Sidi D, Kachaner J (2000) New insight into left ventricular function in tricuspid atresia after total cavopulmonary connection: a three-dimensional echocardiographic study. Cardiol Young 10:83–89
Anderson RH, Wilkinson JL, Gerlis LM, Smith A, Becker AE (1979) Atresia of right atrioventricular orifice. Br Heart J 39:414
Anderson RH, Becker AE, Macartney FJ, Shinebourne EA, Wilkinson JL, Tynan MJ (1979) Is “tricuspid atresia” a univentricular heart? Pediatr Cardiol 1:51–56
Anderson RH, Rigby ML (1987) The morphologic heterogeneity of “tricuspid atresia”. Int J Cardiol 16:67–73
Annecchino FP, Fontan F, Chauve A, Quaegebeur J (1980) Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: a report of 5 patients. Ann Thorac Surg 29:317–321
Ashrafian H, Swan L (2002) The mechanism of formation of pulmonary arteriovenous malformations associated with the classic Glenn shunt (superior cavopulmonary anastomosis). Heart 88:639
Azzolina G, Eufrate S, Pensa P (1972) Tricuspid atresia: experience in surgical management with a modified cavopulmonary anastomosis. Thorax 27:111–115
Bargeron LM, Karp RB, Barcia A, Kirklin JW, Hunt D, Deverall PB (1972) Late deterioration of patients after superior vena cava to right pulmonary artery anastomosis. Am J Cardiol 30:211
Bakuliev AN, Kolesnikov SA (1959) Anastomosis of the superior vena cava and pulmonary artery in the surgical treatment of certain congenital defects of the heart. J Thorac Cardiovasc Surg 37:693
Bharati S, Lev M (1979) The concept of tricuspid atresia complex as distinct form that of the single ventricle complex. Pediatr Cardiol 1:57–62
Binotto MA, Higuchi MdL, Aiello VD (2003) Left ventricular remodeling in hearts with tricuspid atresia: morphologic observations and possible basis for ventricular dysfunction after surgery. J Thorac Cardiovasc Surg 126:1026–1032
Bjork VO, Olin CL, Bjarke BB, Thoren CA (1979) Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 77:452–458
Bonnet D, Sidi D, Vouhé PR (2001) Absorbable pulmonary artery banding in tricuspid atresia. Ann Thorac Surg 71:360–361
Bowman FO, Malm JR, Hayes CJ, Gersony WM (1978) Physiological approach to surgery for tricuspid atresia. Circulation 58(Suppl.I):83–86
Bradley SM, Simsic JM, Mulvihill DM (2003) Hypoventilation improves oxygenation after bidirectional superior cavopulmonary connection. J Thorac Cardiovasc Surg 126:1033–1039
Bridges ND, Jonas RA, Mayer JE, Flanagan MF, Keane JF, Castaneda AR (1990) Bidirectional cavopulmonary anastomosis as interim palliation for high risk Fontan candidates. Circulation 82(Suppl IV:)70
Bridges ND, Lock JE, Castaneda AR (1990) Baffle fenestration with subsequent transcatheter closure, modification of the Fontan operation for patients at increased risk. Circulation 82:1681
Brown JW (1936) Congenital tricuspid atresia. Arch Dis Child 11:275
Bull C, de Leval MR, Stark J, Taylor JFN, Macartney FJ, McGoon DC (1983) Use of a subpulmonary ventricular chamber in the Fontan circulation. J Thorac Cardiovasc Surg 85:21
Burkhart HM, Dearani JA, Mair DD, Warnes CA, Rowland CC, Schaff HV, Puga FJ, Danielson GK (2003) The modified Fontan procedure: early and late results in 132 adult patients. J Thorac Cardiovasc Surg 125:1252–1259
Campbell M (1961) Tricuspid atresia and its prognosis with and without surgical treatment. Br Heart J 23:699
Carlon CA, Mondini PG, de Marchi R (1951) Surgical treatment of some cardiovascular diseases. J Int Coll Surg 16:1
Cheung YF, Penny DJ, Redington AN (2000) Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 83:420–424
Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H (1977) Selection criteria for Fontan’s procedure. In: Anderson RH, Shinebourne EA (eds): Paediatric Cardiology. Churchill Livingstone, Edinburgh, pp 559–566
Cloutier A, Ash JM, Smallhorn JF, Williams WG, Trusler GA, Rowe RD, Rabinovitch M (1985) Abnormal distribution of pulmonary blood after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 72:471
Coles JG, Leung M, Kielmanowicz S, Freedom RM, Benson LN, Rabinovitch M, Sherret H, Dasmahapatra H, Trusler GA, McLaughlin PR (1988) Repair of tricuspid atresia: utility of right ventricular incorporation. Ann Thorac Surg 45:384–389
Corno AF, Laks H, Davtyan H, Flynn WM, Chang P, Drinkwater DC (1988) The heterotopic right heart assist transplant. J Heart Transplant 7:183–190
Corno AF, Carta MG, Giannico S (1989) Pulmonary artery banding through median sternotomy. Cl Res 37:91A
Corno AF, Mazzera E, Marino B, Picardo S, Marcelletti C (1989) Bidirectional cavopulmonary anastomosis. J Am Coll Cardiol 13:74A
Corno AF, Giannico S (1993) Echo evaluation of total extracardiac right heart bypass. G Ital Ecogr Cardiovasc 3:1
Corno AF, Bertucci C, Ranucci M, Rosti L (1994) Bidirectional superior vena cava to pulmonary artery anastomosis. Int Soc Cardiothorac Surg, 4th World Congr, Abstracts: 150
Corno AF, Da Cruz E, Lal AB, Milella L, Wilson N (1998) “Controlled reoxygenation” for cyanotic patients. In: Imai Y, Momma K (eds) Proceedings of 2nd World Congress of PCCS. Futura Publishing Co., Armonk, NY, pp 1127–1129
Corno AF (1996) Considerations on the cavopulmonary connection. Am Coll Cardiol Curr J Rev 5:38
Corno AF, von Segesser LK (1999) Is hypothermia necessary in pediatric cardiac surgery? Eur J Cardiothorac Surg 15:110–111
Corno AF (2000) Surgery for congenital heart disease. Curr Opinion Cardiol 15:238–243
Corno AF (2001) Palliative bilateral unidirectional Glenn shunts. Asian Cardiovasc Thorac Ann 9:141 (invited commentary)
Corno AF, Horisberger J, Jegger D, von Segesser LK (2004) Right atrial surgery with unsnared inferior vena cava. Eur J Cardiothorac Surg 26:219
Crupi GC, Locatelli G, Tiraboschi R, Villani M, de Tommasi M, Parenzan L (1980) Protein-loosing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve). Thorac Cardiovasc Surg 28:359–363
Deanfield JE, Tommassini G, Anderson RH, Macartney FJ (1982) Tricuspid atresia: analysis of coronary arteries distribution and ventricular morphology. Br Heart J 48:485
de Leval MR, Kilner P, Gewillig M, Bull C, McGoon DC (1988) Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 96:682–695
de Leval MR (1998) The Fontan circulation: what have we learned? What to expect? Pediatr Cardiol 19:316–320
di Carlo D, Williams WG, Freedom RM, Trusler GA, Rowe RD (1982) The role of cava-pulmonary (Glenn) anastomosis in the palliative treatment of congenital heart disease. J Thorac Cardiovasc Surg 83:437
di Carlo D, Marcelletti C, Nijveld A, Lubbers LJ, Becker AE (1983) The Fontan procedure in the absence of the interatrial septum. J Thorac Cardiovasc Surg 85:923–927
Dick M, Fyler DC, Nadas AS (1975) Tricuspid atresia: clinical course in 101 patients. Am J Cardiol 36:327–337
DiSessa TG, Child JS, Perloff JK, Wu L, Williams RG, Laks H, Friedman WF (1984) Systemic venous and pulmonary arterial flow patterns after Fontan’s procedure for tricuspid atresia or single ventricle. Circulation 70:898
Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK (1992) Five-to fifteen-year follow-up after Fontan operation. Circulation 85:469–496
Edwards JE, Burchell HB (1949) Congenital tricuspid atresia: a classification. Med Clin North Am 33:1177–1196
Eicken A, Fratz S, Gutfried C, Balling G, Schwaiger M, Lange R, Busch R, Hess J, Stern H (2003) Hearts late after Fontan operation have normal mass, normal volume, and reduced systolic function. A magnetic resonance imaging study. J Am Coll Cardiol 42:1061–1065
Facchini M, Guldenschub I, Turina J, Jenni R, Schalcher C, Attenhofer Jost CH (2000) Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia. J Cardiovasc Surg 41:567–670
Ferencz C, Rubin JD, McCarter RJ (1985) Congenital heart disease: prevalence at livebirth. The Baltimore-Washington infant study. Am J Epidemiol 121:31–36
Fesslova V, Hunter S, Stark J, Taylor JF (1989) Long-term clinical outcome of patients with tricuspid atresia: “natural history”. J Cardiovasc Surg 30: 262–272
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
Fontan F, Kirklin JW, Fernandez G, Costa F, Naftel DC, Tritto F, Blackstone EH (1990) Outcome after a “perfect” Fontan operation. Circulation 81:1520–1536
Franklin RC, Spieghalter NE, Sullivan ID, Anderson RH, Thoele DG, Shinebourne EA, Deanfield JE (1993) Tricuspid atresia presenting in infancy: survival and suitability for the Fontan operation. Circulation 87:427–439
Fredriksen PM, Therrien J, Veldtman G, Warsi MA, Liu P, Siu S, Williams WG, Granton J, Webb GD (2001) Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 85:295–299
Freedom RM (1987) The dinosaur and banding of the main pulmonary trunk in the heart with functionally one ventricle and transposition of the great arteries: a saga of evolution and caution. J Am Coll Cardiol 10:427–429
Freedom RM, Nykanen D, Benson LN (1998) The physiology of the bidirectional cavopulmonary connection. Ann Thorac Surg 66:664–667
Freedom RM, Hamilton R, Yoo SH, Mikailian H, Benson L, McCrindle B, Justino H, Williams WG (2000) The Fontan procedure: cohort analysis and late complications. Cardiol Young 10:307–331
Fyler DC, Buckley LP, Hellenbrand WE, Cohn HE (1980) Report of the New England Regional Infant Care Program. Pediatrics 65(Suppl):375–461
Gabriele OF (1970) Progressive obstruction of pulmonary blood flow in tricuspid atresia. J Thorac Cardiovasc Surg 59:447–449
Gale AW, Danielson GK, McGoon DC, Wallace RB, Mair DD (1980) Fontan procedure for tricuspid atresia. Circulation 62:91
Gallaher ME, Fyler DC (1967) Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels. Circulation 35:381–388
Gatzoulis MA, Munk MD, Williams WG, Webb GD (2000) Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart 83:51–57
Gersbach Ph, Friedli B, Hahn C (1981) Treatment of tricuspid atresia with small pulmonary flow (type I-B) by surgical enlargement of the ventricular septal defect. Thorac Cardiovasc Surg 29:82
Ghai A, Harris L, Harrison DA, Webb GD, Siu SC (2001) Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation. J Am Coll Cardiol 37:585–592
Giannico S, Corno AF, Nava S, Marino B, Guccione P, Marcelletti C (1989) Inferior vena cava to pulmonary artery extracardiac conduit: echo-Doppler patterns of systemic venous flow. J Am Soc Echocard 2:218
Giannico S, Corno AF, Marino B, Cicini MP, Gagliardi MG, Amodeo A, Picardo S, Marcelletti C (1992) Total extracardiac right heart bypass. Circulation 86(Suppl-II):110–117
Glenn WWL (1958) Circulatory bypass of the right side of the heart. II. Shunt between superior vena cava and distal right pulmonary artery: report of clinical application. N Engl J Med 259:117–120
Grabitz RG, Joffres MR, Collins-Nakai RL (1988) Congenital heart disease: incidence in the first year of life. The Alberta heritage pediatric cardiology program. Am J Epidemiol 128:381–388
Harris JA, Francannet C, Pradat P, Robert E (2003) The epidemiology of cardiovascular defects, part 2: a study based on data from three large registries of congenital malformations. Pediatr Cardiol 24:222–235
Hopkins RA, Armstrong BE, Serwer GA, Peterson FJ, Oldham HW (1985) Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 90:391
Ilbawi MN, Idriss FS, DeLeon SY, Kucich VA, Muster AJ, Paul MH, Zales VR (1989) When should the hypoplastic right ventricle be used in a Fontan operation? An experimental and clinical correlation. Ann Thorac Surg 47:533–538
Jahangiri M, Kreutzer J, Zurakowski D, Bacha E, Jonas RA (2000) Evaluation of hemostasis and coagulation factor abnormalities in patients undergoing the Fontan operation. J Thorac Cardiovasc Surg 120:778–782
Jacobs ML, Rychik J, Rome JJ, Apostopoulou S, Pizarro C, Murphy JD, Norwood WI (1996) Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg 62:456–462
Kirklin JK, Blackstone EH, Kirklin JW, Pacifico AD, Bargeron LM (1986) The Fontan operation: ventricular hypertrophy, age, and date of operation as risk factors. J Thorac Cardiovasc Surg 92:1049–1064
Kopf GS, Laks H, Stansel HC, Hellenbrand WE, Kleinman CS, Talner NS (1990) Thirty year follow up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 100:662–671
Kreutzer GO, Galindez E, Bono H, de Palma C, Laura JP (1973) An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 66:613–621
Lamberti JJ, Thilenius O, de la Fuente D, Lin CY, Arcilla R, Replogle RL (1976) Right atrial partition and right ventricular exclusion. J Thorac Cardiovasc Surg 71:386–391
Lan YT, Chang RK, Laks H (2004) Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries. J Am Coll Cardiol 43:113–119
Lee CN, Schaff HV, Danielson GK, Puga FJ, Driscoll DJ (1986) Comparison of atriopulmonary versus atrioventricular connections for modified Fontan/Kreutzer repair of tricuspid valve atresia. J Thorac Cardiovasc Surg 92:1038–1043
Mainwaring RD, Lamberti JJ, Moore JW (1996) The bidirectional Glenn and Fontan procedures-integrated management of the patient with functionally single ventricle. Cardiol Young 6:198–207
Mair DD, Puga F J, Danielson GK (2001) The Fontan procedure for tricuspid atresia: early and late results of a 25 year experience with 216 patients. J Am Coll Cardiol 37:933–939
Marcelletti C, Corno AF, Losekoot TG, Olthof H, Schuller J, Bulterijs AHK, Becker AE (1980) Condotti extracardiaci: indicazioni, tecniche e risultati immediati. G Ital Cardiol 10:1041–1054
Marcelletti C, Corno AF (1981) Extracardiac conduits: indications, techniques and early results. 33th Herhalings Cursus Kindergeneeskunde, Amsterdam (Abstracts)
Marcelletti C, Corno AF, Giannico S, Marino B (1990) Inferior vena cava to pulmonary artery extracardiac conduit: a new form of right heart bypass. J Thorac Cardiovasc Surg 100:228–232
Marino B, Corno AF, Pasquini L, Guccione P, Carta MG, Ballerini L, De Simone G, Marcelletti C (1987) Indication for systemic-pulmonary artery shunts guided by two-dimensional and Doppler echocardiography: criteria for patient selection. Ann Thorac Surg 44:495–498
Marino B, Giannico S, Pasquini L, Corno AF, Picardo S (1988) Balloon-occlusion of the carotid artery for the angiographic visualization of Blalock-Taussig shunts and pulmonary arteries. Chest 94:267–269
Mavroudis C, Deal BJ, Backer CL (2002) The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 5:12–24
Mazzera E, Corno AF, Picardo S, Di Donato RM, Marino B, Costa D, Marcelletti C (1989) Bidirectional cavopulmonary shunts: clinical applications as staged or definitive palliation. Ann Thorac Surg 47:415–420
Mochizuki T, Ohtani T, Higashino H, Sugawara Y, Tsuda T, Sekiya M, Miyagawa M, Ohmoto K, Ikezoe J (2000) Tricuspid atresia with atrial septal defect, ventricular septal defect, right ventricular hypoplasia demonstrated by multidetector computed tomography. Circulation 102:164–165
Monagle P, Cochrane A, McCrindle B, Benson L, Williams WG (1998) Thromboembolic complications after Fontan procedures: the role of prophylactic anticoagulation. J Thorac Cardiovasc Surg 115:493–498
Pradat P, Francannet C, Harris JA, Robert E (2003) The epidemiology of cardiovascular defects, part 1: a study based on data from three large registries of congenital malformations. Pediatr Cardiol 24:195–221
Rao PS (1977) Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. Br Heart J 39:276–288
Rao PS (1983) Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: surgical implications. Ann Thorac Surg 35:121–131
Rao PS (1990) Is the term “tricuspid atresia” appropriate? Am J Cardiol 66:1251–1254
Rao PS (1992) Demographic features of tricuspid atresia. In: Rao PS (ed) Tricuspid atresia. Futura Publishing Co., Mt. Kisco, N.Y.
Rao PS (2000) Tricuspid atresia. Curr Treat Options Cardiovasc Med 2:507–520
Rashkind WJ (1982) Tricuspid atresia: a historical review. Pediatr Cardiol 2:85–88
Reddy SC, Zuberbuhler JR (2003) Images in cardiovascular medicine. Himalayan P-waves in a patient with tricuspid atresia. Circulation 107:498
Robicsek A, Temesvari A, Kadar RL (1956) A new method for the treatment of congenital heart disease associated with impaired pulmonary circulation. Acta Med Scand 154:151–161
Ross DN, Somerville J (1973) Surgical correction of tricuspid atresia. Lancet 11:845–849
Scholl FG, Alejos JC, Laks H (1997) Revision of the traditional atriopulmonary Fontan connection. Adv Card Surg 9:217–2327
Seliem M, Muster AJ, Paul MH, Benson DW (1989) Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coll Cardiol 14:750–755
Sittiwangkul R, Azakie A, van Arsdell GS, Williams WG, McCrindle BW (2004) Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg 77:889–894
Somerville J (1979) Congenital heart disease: changes in form and function. Br Heart J 41:1–22
Tandon R, Edwards JE (1974) Tricuspid atresia: a re-evaluation and classification. J Thorac Cardiovasc Surg 67:530–542
Thiene G, Anderson RH (1981) The clinical morphology of tricuspid atresia: atresia of the right atrioventricular valve. G Ital Cardiol 11:1845–1859
Tokunaga S, Kado H, Imoto Y, Masuda M, Shiokawa Y, Fukae K, Fusazaki N, Ishikawa S, Yasui H (2002) Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients. Ann Thorac Surg 73:76–80
Varma C, Warr MR, Hendler AL, Paul NS, Webb GD, Therrien J (2003) Prevalence of “silent” pulmonary emboli in adults after the Fontan operation. J Am Coll Cardiol 41:2252–2258
Veldtman GR, Nishimoto A, Siu S, Freeman M, Fredriksen PM, Gatzoulis MA, Williams WG, Webb GD (2001) The Fontan procedure in adults. Heart 86:330–335
Vettukattil JJ (2002) Pathogenesis of pulmonary arteriovenous malformations: role of hepatopulmonary interactions. Heart 88:561–563
Yasuda K, Tomita H, Kimura K, Yagihara T, Echigo S (2002) Stenting pulmonary artery stenosis in an infant with tricuspid atresia: approach via the foramen ovale and ventricular septal defect using a long flexible sheath. Pediatr Int 44:690–692
Walther T, Theune P, Sullivan I, de Leval MR (2003) Successful medical treatment of persistent pleural drainage after the Fontan operation. Interact Cardiovasc Thorac Surg 2:348–349
Warnes CA, Somerville J (1986) Tricuspid atresia in adolescents and adults: current state and late complications. Br Heart J 56:535–543
Weinberg PW (1980) Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg 29:306–311
Weinstein S, Cua C, Chan D, Davis JT (2003) Outcome of symptomatic patients undergoing extracardiac Fontan conversion and cryoablation. J Thorac Cardiovasc Surg 126:529–536
Wenink ACG, Ottenkamp J (1987) Tricuspid atresia: microscopic findings in relation to “absence” of the atrioventricular connexion. Int J Cardiol 16:57–65
Woods RK, Dyamenahalli U, Duncan BW, Rosenthal GL, Lupinetti FM (2003) Comparison of extracardiac Fontan techniques: pedicled pericardial tunnel versus conduit reconstruction. J Thorac Cardiovasc Surg 125:465–471
Yeh T, Williams WG, McCrindle BW, Benson LN, Coles JG, van Arsdell GS, Webb GD, Freedom RM (1999) Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure. Eur J Cardiothorac Surg 16:111–116
Yetman AT, Drummond-Webb J, Fiser WP, Schmitz ML, Imamura M, Ullah S, Gunselman RJ, Chipman CW, Johnson CE, van Devanter SH (2002) The extracardiac Fontan procedure without cardiopulmonary bypass technique and intermediate-term results. Ann Thorac Surg 74:S-1416–1421
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2004 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Corno, A.F. (2004). Tricuspid atresia. In: Congenital Heart Defects. Steinkopff, Heidelberg. https://doi.org/10.1007/978-3-7985-1934-3_2
Download citation
DOI: https://doi.org/10.1007/978-3-7985-1934-3_2
Publisher Name: Steinkopff, Heidelberg
Print ISBN: 978-3-642-62158-1
Online ISBN: 978-3-7985-1934-3
eBook Packages: Springer Book Archive