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From Maude to Claude: the musings of an insomniac in the era of evidence-based medicine

Published online by Cambridge University Press:  19 August 2008

Robert M. Freedom*
Affiliation:
The University of Toronto Faculty of Medicine Head. Division of Cardiology. The Hospital for Sick Children. Toronto, Ontario, Canada
*
Robert M Freedom, MD, FRCPC, Dept of Paediatrics (Cardiology), The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8 Tel 416 813 6132, Fax 416 813 7547

Abstract

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Type
The Edgar Mannheimer Memorial Lecture
Copyright
Copyright © Cambridge University Press 1998

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References

1.Taussig, HB. Congenital Maformations of the Heart. Vols. 1 & 2. Harvard University Press. Cambridge, 1960, pp.1049Google Scholar
2.Nadas, AS. Pediatric Cardiology. WE Saunders, Philadelphia, 1957, p. 475Google Scholar
3.Kjellberg, Mannheimer E, Rudhe, U,Jonsson, B. Diagnosis of Congenital Heart Disease. The Year Book Publishers, Inc., Chicago, 1955, pp 649Google Scholar
4.Rashkind, WJ. Congenital Heart Disease. Benchmark Papers in Human Physiology. No. 16. Hutchinson Ross Publishing Co., Stoudsburg, PA, 1982, pp. 390Google Scholar
5.Shumacker, HB JrThe Evolution of Cardiac Surgery. Indiana University Press, Bloomington, 1992; pp 476Google Scholar
6.Abbott, ME. Atlas of Congenital Cardiac Disease. Am Heart Assoc., 1939, pp. 62Google Scholar
7.Gross, RE, Hubbard, JP. Surgical ligation of a patent ductus arteriosus: Report of first successful case. Am Med Assoc J 1939; 112: 729731CrossRefGoogle Scholar
8.Blalock, A, Taussig, HB. The surgical treatment of malforma tions of the heart in which there is pulmonary stenosis or pulmonary atresia. J Am Med Assoc 1945; 128: 189192CrossRefGoogle Scholar
9.Crafoord, C, Nylin, G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg 1945; 14: 347361CrossRefGoogle Scholar
10.Brock, RC. Pulmonary valvotomy for the relief of congenital pulmonary stenosis. Report of three cases. Br Med J 1948; 1: 11211126CrossRefGoogle Scholar
11.Senning, A. Surgical correction of transposition of the great vessels. Surg 1959; 45:966980.Google ScholarPubMed
12.Mustard, WT. Successful two-stage correction of transposition of the great vessels. Surg 1964; 55: 469472.Google ScholarPubMed
13.Rashkind, WJ. Congenital Heart Disease. Benchmark Papers in Human Physiology. No. 16. Hutchinson Ross Publishing Co., Stoudsburg, PA, 1982, pp. 390Google Scholar
14.Fontan, F and Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.CrossRefGoogle ScholarPubMed
15.Jatene, AD, Fontes, VF, Paulista, PP, Souza, LCB, Neger, F, Galantier, M, Sousa, JEMR. Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 1976; 72: 364370.CrossRefGoogle ScholarPubMed
16.Jatene, AD. Anatomic correction of transposition of the great arteries: Historical aspects. Pediatr Cardiol 1983; 4: 38.Google Scholar
17.Castaneda, AR, Jonas, RA, Mayer, JE Jr., Hanley, FL. Cardiac Surgery of the Neonate and Infant. WB Saunders, Philadelphia, 1994, 409438Google Scholar
18.Rashkind, WJ and Miller, WW. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA 1966; 196: 991992CrossRefGoogle ScholarPubMed
19.Olley, PM, Coceani, F, Bodach, E. E-type prostaglandins: A new emergency therapy for certain cyanotic congenital heart malformations. Circulation 1976; 53: 728731.CrossRefGoogle ScholarPubMed
20.Williams, WG, Trusler, GA, Kirklin, JW, Blackstone, EH, Coles, JG, Izukawa, T, Freedom, RM. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988; 95: 717726.CrossRefGoogle Scholar
21.Jonas, RA, Quaegebeur, JM, Kirklin, JW, Blackstone, EH, Daicoff, G. Outcomes in patients with interrupted aortic arch and ventricular septal defect. A multi-institutional study. J Thorac Cardiovasc Surg 1994; 107:10991113CrossRefGoogle Scholar
22.Wernovsky, G, Mayer, JE Jr., Jonas, RA, Hanley, FL, Blackstone, EH, Kirklin, JW, Castaneda, AR. Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 1995; 109: 289302.CrossRefGoogle ScholarPubMed
23.Kirklin, JW, Blackstone, EH, Tchervenkov, CI, Castaneda, AR. Clinical outcomes after the arterial switch operation for transposition. Patient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society. Circulation 1992; 86:1501–15.CrossRefGoogle ScholarPubMed
24.Norwood, WI, Dobell, AR, Freed, MD, Kirklin, JW, Blackstone, EH. Intermediate results of the arterial switch repair. A 20–institution study. J Thorac Cardiovasc Surg 1988; 96:854–63.CrossRefGoogle ScholarPubMed
25.Hanley, FL, Sade, RM, Freedom, RM, Blackstone, EH, Kirklin, JW. Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. Congenital Heart Surgeons Society. J Am Coll Cardiol 1993;22:183192CrossRefGoogle ScholarPubMed
26.Hanley, FL, Sade, RM, Blackstone, EH, Kirklin, JW, Freedom, RM, Nanda, NC. Outcomes in Neonatal Pulmonary Atresia with Intact Ventricular Septum. J Thorac Cardiovasc Surg 1993; 105:406427CrossRefGoogle ScholarPubMed
27.Hoffman, JIE. Incidence of congenital heart diseae: I. Postnatal Incidence. Pediatr Cardiol 1995; 16: 103113CrossRefGoogle Scholar
28.Van Praagh, R. The segmental approach to diagnosis in congenital heart disease. Birth Defects (Original Article Series) 1972; 8: 423Google Scholar
29.Van Praagh, R. Terminology of Congenital Heart Disease. Glossary and Commentary. Circulation 1977; 56: 139143.CrossRefGoogle ScholarPubMed
30.Van Praagh, R. Diagnosis of Complex Congenital Heart Disease: Morphologic-Anatomic Method and Terminology. Cardiovasc Intervent Radiol 1984; 7: 115120CrossRefGoogle ScholarPubMed
31.Van Praagh, R, Ongley, PA, Swan, HJC. Anatomic types of single or common ventricle in man. Morphologic and geometric aspects of sixty autopsied cases. Am J Cardiol 1964; 13: 367386CrossRefGoogle Scholar
32.Van Praagh, R, Van Praagh, S, Vlad, P, Keith, JD. Anatomic types of congenital dextrocardia. Diagnostic and embryologic implications. Am J Cardiol 1964; 13: 510531.CrossRefGoogle Scholar
33.Anderson, RH, Yen Ho, S. Sequential segmental analysis-description and categorization for the millenium. Cardiol Young 1997; 7:98116CrossRefGoogle Scholar
34.Anderson, RH, Becker, AE, Freedom, RM, Macartney, FJ, Quero-Jiminez, M, Shinebourne, EA, Wilkinson, JL, Tynan, M. Sequential Segmental Analysis of Congenital Heart Disease. Pediatr Cardiol 1984; 5: 281288.CrossRefGoogle ScholarPubMed
35.Macartney, FJ, Shinebourne, EA, Anderson, RH. Connexions, relations, discordance, and distorsions. Br Heart J 1976; 38: 323326CrossRefGoogle ScholarPubMed
36.Shinebourne, EA, Macartney, FJ, Anderson, RH. Sequential chamber localisation-logical approach to diagnosis in congenital heart disease. Br Heart J 1976; 38: 327340CrossRefGoogle ScholarPubMed
37.Freedom, RM. The Changing Nature of Neonatal Cardiovascular Surgery: The Medical Perspective. The Second World Congress of Pediatric Cardiology and Cardiovascular Surgery. Abstracts. May 11–15, 1997, p.60Google Scholar
38.Cook, DH, Izukawa, T, Muiholland, HC,Rowe, RD. Unmet need for pediatric cardiology services and correlates of referral in Ontario, 1975–1977. Ped Cardiol 1982; 3:275282.CrossRefGoogle ScholarPubMed
39.Roy, DL, Mclntyre, L, Human, DG, Nanton, MA, Sherman, GJ, Allen, LM, Finley, JP. Trends in the prevalence of congenital heart disease: comprehensive observations over a 24–year period in a defined region of Canada. Can J Cardiol 1994; 10:821–6Google Scholar
40.Van Arsdell, GS, Williams, WG, Einarson, KD, Oag, L, McCrindle, BW, McDonough, J, Rebeyka, I, Coles, JC, Black, MD, freedom, RM. Interventions associated with minimal Fontan mortality. Can cardiovasc Society Proc., in press, 1997.Google Scholar
41.Hashmi, A, Abu-Sulaiman, R, McCrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RM. Management and outcomes of right atrial isomerism. JACC, in press, 1997Google Scholar
42.Tam, CKH, Lightfoot, NE, Finlay, CD, Coles, J, Williams, WG, Trusler, GA, Freedom, RM. Course of Tricuspid Atresia in the Fontan Era. Am J Cardiol 1989; 63: 589593.CrossRefGoogle ScholarPubMed
43.Franklin, RCG, Spiegalhalter, DJ, Sullivan, ID, Anderson, RH, Thoele, DG, Shinebourne, EA, Deanfield, JE. Tricuspid atresia presenting in infancy. Survival and suitability for the Fontan operation. Circulation 1993; 87:427439CrossRefGoogle ScholarPubMed
44.Hofbeck, M, Sunnegardh, JT, Burrows, PE, Moes, CA, Lightfoot, N, Williams, WG, Trusler, GA, Freedom, RM. Analysis of survival in patients with pulmonic valve atresia and ventricular septal defect. Am J Cardiol 1991; 67:737–43CrossRefGoogle ScholarPubMed
45.Coles, JG, Freedom, RM, Lightfoot, NE, Dasmahapatra, HK, Williams, WG, Trusler, GA, Burrows, PE, Himansu, K. Long-term Results in Neonates with Pulmonary Atresia and Intact Ventricular Septum. Ann Thorac Surg. 1989; 47: 213237.CrossRefGoogle ScholarPubMed
46.Lightfoot, NE, Coles, JG, Dasmahapatra, HK, Williams, EG, Chin, K, Trusler, GA, Freedom, RM. Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically. Int J Cardiol 1989; 24: 159164CrossRefGoogle ScholarPubMed
47.Soongswang, J, Adatia, I, Newman, C, Smalihorn, JF, Williams, WG.Freedom, RM. Mortality in potential arterial switch candidates with d-transposition of the great arteries. JACC 1997; 29(No. 2, Suppl. A): 106A (Abstr).Google Scholar
48.Freedom, RM and Harrington, DP. Contribution of intramyocardial sinusoids in pulmonary atresia and intact ventricular septum to a right-sided circular shunt. Br Heart J 1974; 36: 10611065.CrossRefGoogle ScholarPubMed
49.Freedom, RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Press Publishing Co., Mt. Kisco, NY, 262 pages, 1989Google Scholar
50.Freedom, RM, Burrows, PE, Smallhorn, JF. Pulmonary atresia and intact ventricular septum. In, Neonatal Heart Disease. Eds. Freedom, RM, Benson, LN, Smallhorn, JF. Springer-Verlag, London, 1992, 285307.CrossRefGoogle Scholar
51.Freedom, RM, Culham, JAG, Moes, CAF. Angiocardiography of Congenital Heart Disease. Macmillan Publishing Co., New York, 1984, 221253.Google Scholar
52.Freedom, RM and Moes, CAF. The Hypoplastic Right Heart Complex. Sem in Roeneg 1985; 20: 169183.Google ScholarPubMed
53.Rowe, RD, Freedom, RM, and Mehrizi, A. The Neonate with Congenital Heart Disease. WB Saunders Co., New York, 1981, 328349.Google ScholarPubMed
54.Freedom, RM, Mawson, JB, Yoo, S-J, Benson, LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co, Armonk, NY, 1997, 617662Google Scholar
55.Freedom, RM. Angiocardiography of the Right Ventricle. In Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 163206Google Scholar
56.Patel, R, Freedom, RM, Moes, CAF, Bloom, K, Olley, PM, Williams, WG, Trusler, GA, Rowe, RD. Right Ventricular Volume Determinations in 18 Patients with Pulmonary Atresia and Intact Ventricular Septum. Analysis of Factors Influencing Right Ventricular Growth. Circulation 1980; 61: 428440.CrossRefGoogle ScholarPubMed
57.Freedom, RM and Perrin, D. The right ventricle: morphologic considerations. In Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 5374Google Scholar
58.Hanley, FL, Sade, RM, Freedom, RM, Blackstone, EH, Kirklin, JW. Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. Congenital Heart Surgeons Society. J Am Coll Cardiol 1993;22:183192CrossRefGoogle ScholarPubMed
59.Freedom, RM, Dische, MR, Rowe, RD. The tricuspid valve in pulmonary atresia and intact ventricular septum. Arch Pathol Lab Med 1978; 102: 2831Google ScholarPubMed
60.Freedom, RM and Perrin, D. The tricuspid valve: morphologic considerations. In Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 3752.Google Scholar
61.Freedom, RM and Wilson, GJ. The anatomic substrate of pulmonary atresia and intact ventricular septum. Third clinical conference on congenital heart disease. Obstructive lesions of the right heart. Baltimore, University Park Place, 1984, 217255Google Scholar
62.Freedom, RM, Wilson, G, Trusler, GA, Williams, WG, Rowe, RD. Pulmonary Atresia And Intact Ventricular Septum. A Review of the Anatomy, Myocardium, and Factors Influencing Right Ventricular Growth and Guidelines for Surgical Intervention. Scand J Thor Cardiovasc Surg 1983; 17: 128.Google Scholar
63.Williams, WG, Burrows, P, Freedom, RM, Trusler, GA, Coles, JG, Moes, CAF, Smallhorn, JF. Thromboexclusion of the Right Ventricle in a Subset of Children with Pulmonary Atresia and Intact Ventricular Septum. J Thorac Cardiovasc Surg 1991; 101:222229.CrossRefGoogle Scholar
64.Freedom, RM, Benson, L, Wilson, GJ. The coronary circulation and myocardium in pulmonary and aortic atresia with an intact ventricular septum. In , Paediatric Cardiology, Vol. 6. Eds. Marcelletti, C, Anderson, RH, Becker, AE, Corno, A, di Dicarlo, D, Mazzera, E. Churchill Livingstone, Edinburgh, 1986, 7896.Google Scholar
65.Freedom, RM, Benson, LN, Trusler, GA. Pulmonary atresia and intact ventricular septum: a consideration of the coronary circulation and ventriculo-coronary connections. Annual of Cardiac Surgery, 1989, 3844Google Scholar
66.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: 914920.CrossRefGoogle Scholar
67.Koike, K, Perrin, D, Wilson, GJ, Freedom, RM. Myocardial ischemia and coronary arterial involvement in newborn babies less than one week old with pulmonary atresia and intact ventricular septum. In Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 101108.Google Scholar
68.Razzouk, AJ, Freedom, RM, Cohen, AJ, Williams, WG, Trusler, GA, Coles, JG, Burrows, PE, Rebeyka, I. The recognition, identification of morphological substrate, and treatment of subaortic stenosis after a Fontan operation: an analysis of 12 patients. J Thorac Cardiovasc Surg 1992; 104:938944.CrossRefGoogle Scholar
69.Akagi, T, Benson, LN, Williams, WG, Trusler, GA, Freedom, RM. Ventriculo-Coronary arterial connections in pulmonary atresia with intact ventricular septum, and their influences on ventricular performance and clinical course. Am J Cardiol 1993; 72: 586590CrossRefGoogle ScholarPubMed
70.Burrows, PE, Freedom, RM, Benson, LN, Moes, CAP. Coronary Angiography of Pulmonary Atresia, Hypoplastic Right Ventricle, and Ventriculo-coronary Communications. AJR 1990; 154: 789795,CrossRefGoogle Scholar
71.Burrows, PE, Benson, LN, Freedom, RM. Coronary angiography in pulmonary atresia with intact ventricular septum. In Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Press Publishing Co., Mt. Kisco, NY, 1989, 207226Google Scholar
72.Freedom, RM. How can something so small cause so much grief? Some thoughts about the underdeveloped right ventricle in pulmonary atresia and intact ventricular septum. JACC 1992; 19:10381040.CrossRefGoogle ScholarPubMed
73.Freedom, RM, Sondheimer, H, Dische, R, Rowe, RD. Development of “Subaortic Stenosis” After Pulmonary Arterial Banding for Common Ventricle. Am J Cardiol 1977; 39: 7883.CrossRefGoogle ScholarPubMed
74.Freedom, RM, Dische, MR, Rowe, RD. Pathologic Anatomy of Subaortic Stenosis and Atresia in the First Year of Life. Am J Cardiol 1977; 39: 10351044CrossRefGoogle ScholarPubMed
75.Freedom, RM, Culham, JAG, Rowe, RD. Angiocardiography of Subaortic Obstruction in Infancy. Am J Roentgenol 1977; 129: 813824.CrossRefGoogle ScholarPubMed
76.Freedom, RM, Rowe, RD. Morphological and Topographical Variations of the Outlet Chamber in Complex Congenital Heart Disease: An Angiographic Study. Cath and Cardiovasc Diagnd 1978; 4: 345371.CrossRefGoogle Scholar
77.Freedom, RM. The morphologic and therapeutic implications of an obstructive anomaly of the aortic arch in infants with complex congenital heart disease. In Parenzan, L, Crupi, GC, Graham, G, editors, Congenital heart disease in the first three months of life: medical and surgical aspects. Bologna, Patron Editore, 1981, 341361.Google Scholar
78.Penkoske, PA, Freedom, RM, Williams, WG, Trusler, GA, Rowe, RD. Surgical palliation of subaortic stenosis in the univentricular heart. J Thorac Cardiovasc Surg 1984; 87: 767781.CrossRefGoogle ScholarPubMed
79.Cohen, AJ, Cleveland, DC, Dyck, J, Poppe, D, Smallhorn, JF, Freedom, RM, Trusler, GA, Coles, JG, Moes, CAF, Rebeyka, I, Williams, WG. Results of the Fontan procedure for patients with univentricular heart. Ann Thorac Surg 1991; 52:12661271CrossRefGoogle ScholarPubMed
80.Freedom, RM. Invited Commentary. Ann Thorac Surg 1994; 58: 11121113.Google Scholar
81.Freedom, RM and Smallhorn, JF. Hearts with a univentricular atrio-ventricular connection. In, Freedom, RM, Benson, LN, Smallhorn, JF. Neonatal Heart Disease. Springer-Verlag, London, 1992,497521.CrossRefGoogle Scholar
82.Akagi, T, Benson, LN, Williams, WG, Freedom, RM. The relation between ventricular hypertrophy and clinical outcome in patients with double inlet left ventricle after atrial to pulmonary anastomosis. Herz 1992; 17:220227.Google ScholarPubMed
83.Freedom, RM. 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. JACC 1987; 10: 427429.CrossRefGoogle ScholarPubMed
84.Freedom, RM, Williams, WG, Fowler, RS, Trusler, GA, Rowe, RD. Tricuspid atresia, transposition of the great arteries, and banded pulmonary artery. Repair by arterial switch, coronary artery reimplantation, and right atrioventricular valved conduit. J Thorac Cardiovasc Surg 1980; 80: 621628.CrossRefGoogle ScholarPubMed
85.Freedom, RM, Benson, LN, Smallhorn, JF, Williams, WG, Trusler, GA, Rowe, RD. Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding. Circulation 1986; 73: 758764.CrossRefGoogle ScholarPubMed
86.Trusler, GA and Freedom, RM. Management of Subaortic Stenosis in the Univentricular Heart. Ann Thor Surg 1989; 47: 643645.CrossRefGoogle ScholarPubMed
87.Caspi, J, Coles, JG, Rabinovitch, M, Cohen, D, Trusler, GA, Williams, WG, Wilson, GJ, Freedom, RM. Morphological Findings Contributing to a failed Fontan Procedure in the Current Era. Circulation 1990; 82(Suppl. IV: IV-177–IV-182.Google Scholar
88.Freedom, RM and Trusler, GA. Arterial switch for palliation of subaortic stenosis in single ventricle and transposition: no mean feat! Ann Thorac Surg 1991; 52: 415416.CrossRefGoogle ScholarPubMed
89.Freedom, RM, Akagi, T, Benson, LN. The potentially obstructive subaortic region and banding of the pulmonary trunk-selected observations in the patient considered for a Fontan procedure. Cardiol Young 1993; 3:9197.CrossRefGoogle Scholar
90.Lui, RC, Williams, WG, Trusler, GA, Freedom, RM, Coles, JG, Rebeyka, IM, Smallhorn, J. Experience with the Damus Kaye-Stansel procedure for children with Taussig-Bing hearts or univentricular hearts with subaortic stenosis. Circulation 1993; 88: II170–6.Google ScholarPubMed
91.Grant, RT. An Unusual Anomaly of the Coronary Vessels in the Malformed Heart of a Child. Heart 1926; 13: 273283Google Scholar
92.Wearn, JT, Mettier, SR, Klumpp, TG, Zschiesche, U. The Nature of the Vascular Communications Between the Coronary Arteries and the Chambers of the Heart. Am Heart J 1933; 9: 143164.CrossRefGoogle Scholar
93.Anselmi, G, Munoz, S, Blanco, P, Carbonell, L, Puigbo, JJ. Anomalous coronary artery connecting with the right ventricle associated with pulmonary stenosis and atrial septal defect. Am Heart J 1961; 62: 406414.CrossRefGoogle ScholarPubMed
94.Williams, RR, Kent, GBJ, Edwards, JE. Anomalous Cardiac Blood Vessel Communicating With the Right Ventricle Arch Path 1951; 52: 480487.Google Scholar
95.Guidici, C and Becu, L. Cardio-Aortic Fistula through Anomalous Coronary Arteries. Br Heart J 1960; 22: 729733CrossRefGoogle ScholarPubMed
96.Lauer, RM, Fink, HP, Petty, EL, Dunn, MI, Diehl, AM. Angiographic Demonstration of Intramyocardial Sinusoids in Pulmonary-Valve Atresia with Intact Ventricular Septum and Hypoplastic Right Ventricle. N Eng J Med 1964; 271: 6872.CrossRefGoogle ScholarPubMed
97.MacMahon, HE and Dickinson, PCT. Occlusive fibroelastosis of coronary arteries in the newborn. Circulation 1967; 35:39.CrossRefGoogle ScholarPubMed
98.Vigorita, V. Epicardial Nodules: A Possible Sign of Coronary Endarteritis with Hypoplastic Right Heart Syndrome. Johns HopkMed J 1978; 142: 215217.Google ScholarPubMed
99.Dusek, J, Ostadal, B, Duskova, M. Postnatal Persistence of Spongy Myocardium with Embryonic Blood Supply. Arch Pathol 1975; 99: 312317.Google ScholarPubMed
100.Essed, CE, Klein, HW, Krediet, P, Vorst, EJ. Coronary and Endocardial Fibroelastosis of the Ventricles in the Hypoplastic Left and Right Heart Syndromes. Virchows Arch A Path Anat and Histol 1975; 368: 8797.CrossRefGoogle ScholarPubMed
101.Calder, AL, Co, EE, Sage, MD. Coronary Arterial Abnormalities in Pulmonary Atresia with Intact Ventricular Septum. Am J Cardiol 1987; 59: 437442.CrossRefGoogle ScholarPubMed
102.Waldman, JD, Lamberti, JJ, Mathewson, JW, George, L. Surgical closure of the tricuspid valve for pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications. Ped Cardiol 1984; 5: 221224.CrossRefGoogle ScholarPubMed
103.Kauffman, SL and Andersen, DH. Persistent venous valves, mal-development of the right heart, and coronary artery-ventricular communications. Am Heart J 1963; 66: 664669.CrossRefGoogle Scholar
104.Hamazaki, M. Congenital Coronary Arterio-Ventricular Fistulae, Associated with Absence of Proximal Coronary Artery from Aorta. J pn Heart J 1982; 23: 271277.CrossRefGoogle ScholarPubMed
105.Ho, SY, De, SCarvalho, J, Sheffield, E. Anomalous origin of single coronary artery in association with pulmonary atresia. Int J Cardiol 1988; 20: 125128.CrossRefGoogle ScholarPubMed
106.Lenox, CC and Briner, J. Absent Proximal Coronary Arteries Associated with Pulmonic Atresia.Am J Cardiol 1972; 30: 666669.CrossRefGoogle ScholarPubMed
107.Oppenheimer, EH and Esterly, JR. Some Aspects of Cardiac Pathology in Infancy and Childhood. II. Unusual Coronary Endarteritis with Congenital Cardiac Malformations. Bull Johns Hopk Hosp 1966;. 19: 343354.Google Scholar
108.Sissman, NJ and Abrams, HL. Bidirectional Shunting in a Coronary Artery-Right Ventricular Fistula Associated with Pulmonary Atresia and an Intact Ventricular Septum. Circulation 1965; 32: 582588.CrossRefGoogle Scholar
109.Ueda, K, Saito, A, Nakano, H, Hamazaki, Y. Absence of proximal coronary arteries associated with pulmonary atresia. Am Heart J 1983; 106: 596598.CrossRefGoogle ScholarPubMed
110.Gerlis, LM, Yen Ho, S, Milo, S. Three anomalies of the coronary arteries co‐;existing in a case of pulmonary atresia with intact ventricular septum. Int J Cardiol 1990; 29: 9395.CrossRefGoogle Scholar
111.Laks, H, Gates, RN, Grant, PW, Drant, S, Allada, V, Harake, B. Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1995; 59: 342347.Google ScholarPubMed
112.Freeman, JE, DeLeon, SY, Lai, S, Fisher, EA, Ow, EP, Pifarre, R. Right ventricle-to-aorta conduit in pulmonary atresia with intact ventricular septum and coronary sinusoids. Ann Thorac Surg 1993; 56; 13931394.CrossRefGoogle ScholarPubMed
113.De Leval, M. Myocardial perfusion in congenital heart disease: surgical implications. In, Marcelletti, C, Anderson, RH, Becker, AE, Corno, A, di Carlo, D, Mazzera, E, eds. Paediatric Cardiology, New York, Churchill Livingstone, 1986; 6: 97107Google Scholar
114.Blackman, MS, Schneider, B, Sondheimer, HM. Absent proximal left main coronary artery in association with pulmonary atresia. Br Heart J 1981; 46: 449451.CrossRefGoogle ScholarPubMed
115.Garcia, OL, Gelband, H, Tamer, DF, Fojaco, RM. Exclusive origin of both coronary arteries from a hypoplastic right ventricle complicating an extreme tetralogy of Fallot: Lethal myocardial infarction following a palliative shunt. Am Heart J 1988; 115: 198201.CrossRefGoogle ScholarPubMed
116.Rigby, ML, Salgado, M, Silva, C. Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period. Cardiol Young 1992; 2: 377381.CrossRefGoogle Scholar
117.Van der Wal, HJCM, Smith, A, Becker, AE, Wilkinson, JL, Hamilton, DI. Morphology of pulmonary atresia with intact ventricular septum in patients dying after operation. Ann Thorac Surg 1990; 50: 98102.CrossRefGoogle Scholar
118.Vosa, C, Arciprete, P, Caianello, G, Palms, G. Pulmonary atresia with intact ventricular septum: Is it possible to improve survival? Cardiol Young 1992; 2: 391394CrossRefGoogle Scholar
119.Bull, C, Kostelka, M, Sorensen, K, de Leval, M. Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1994; 107: 359–66.CrossRefGoogle ScholarPubMed
120.Daubeney, PEF, Delany, DJ, Slavik, Z, Anderson, RH, Keeton, BR, Webber, SA. Pulmonary atresia with intact ventricular septum: range of morphology in a population based study. Circulation 1995; 92 (Suppl.): 1126 (Abstract # 0595)Google Scholar
121.Mair, DD, Julsrud, PR, Puga, FJ, Danielson, GK. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results. J Am Coil Cardiol 1997; 29: 13591364.CrossRefGoogle ScholarPubMed
122.Van Praagh, R, Ando, M, Van Praagh, S, Senno, A, Hougen, TJ, Novak, G, Hastreiter, AR. Pulmonary Atresia: Anatomic Considerations. In, The Child With Congenital Heart Disease After Surgery. Mount Kisco, Futura Publishing Co., 1976, 103135Google Scholar
123.Elliott, LP, Adams, PJ, Edwards, JE. Pulmonary Atresia with Intact Ventricular Septum. Br Heart J 1963; 25: 489501.CrossRefGoogle ScholarPubMed
124.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: 591602.CrossRefGoogle ScholarPubMed
125.Giglia, TM, Jenkins, KJ, Matitiau, A, Mandell, VS, Sanders, SP, Mayer, JE Jr, Lock, JE. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation 1993; 88 [part I]: 22482256.CrossRefGoogle ScholarPubMed
126.Zuberbuhler, JR and Anderson, RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281288.CrossRefGoogle ScholarPubMed
127.Anderson, RH, Anderson, C, Zuberbuhler, JR. Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum. Cardiol Young 1991; 1: 105113.CrossRefGoogle Scholar
128.Bull, C, De Leval, M, Mercanti, C, MacArtney, FJ, Anderson, RH. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation 1982; 66: 266272.CrossRefGoogle ScholarPubMed
129.De Leval, M, Bull, C, Stark, J, Anderson, RH, Taylor, JFN, MacArtney, FJ. Pulmonary Atresia and Intact Ventricular Septum: Surgical Management Based on a Revised Classification. Circulation 1982; 66: 272–28.CrossRefGoogle ScholarPubMed
130.De Leval, M, Bull, C, Hopkins, R, Rees, P, Deanfield, J, Taylor, JFN, Gersony, W, Stark, J, MacArtney, FJ. Decision making in the definitive repair of the heart with a small right ventricle. Circulation 1985; 72(Suppl II): 5260.Google ScholarPubMed
131.Pawade, A, Capuani, A, Penny, DJ, Karl, TR, Mee, RB. Pulmonary atresia with intact ventricular septum:surgical management based on right ventricular infundibulum. J Card Surg 1993; 8: 371383.CrossRefGoogle ScholarPubMed
132.Pawade, A, Karl, T. Management strategy in neonates presenting with pulmonary atresia with intact ventricular septum. Curr Opin Pediatr 1994, 6: 600–5.CrossRefGoogle ScholarPubMed
133.Drant, SE, Allada, V, Williams, RG. Infundibular diameter predicts the presence of right ventricular‐dependent coronary communications in pul-monary atresia and intact ventricular septum. JACC 1995; Abstracts, Feb., 140A.CrossRefGoogle Scholar
134.Freedom, RM and Perrin, D. The tricuspid valve: morphologic considerations. In Freedom, RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 3752.Google Scholar
135.Biackstone, EH, Kirklin, JW, Hanley, FE. What proportion of neonates with pulmonary atresia and intact ventricular septum reach definitive repair? Circulation 1996; 1173.Google Scholar
136.Fyler, DC. Nadas‐ Pediatric Cardiology. Mosby-Year Book, Boston, 1992, 557576.Google Scholar
137.Gentles, TL, Colan, SD, Giglia, TM, Mandell, VS, Mayer, JE, Sanders, SP. Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies. Circulation 1993; 88 [part 2]: 183188.Google ScholarPubMed
138.Giglia, TM, Mandell, VS, Connor, AR, Mayer, JE Jr., Lock, JE. Diagnosis and management of right ventricular-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992; 86: 15161528.CrossRefGoogle Scholar
139.Dyamenali, U, Hanna, B, Sharratt, GP. Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies. Cardiol Young 1997; 7: 80–8.CrossRefGoogle Scholar
140.Gittenberger-De Groot, AC, Sauer, U, Bindl, L, Babic, R, Essed, CE, Buhlmeyer, K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988; 18: 243258.CrossRefGoogle ScholarPubMed
141.Mainwaring, RD, Lamberti, JJ. Pulmonary atresia with intact ventricular septum. Surgical approach based on ventricular size and coronary anatomy. J Thorac Cardiovasc Surg 1993; 106: 733–8.CrossRefGoogle ScholarPubMed
142.Amodeo, A, Keeton, BR, Sutherland, GR, Monro, JL. Pulmonary atresia with intact ventricular septum: is neonatal repair advisable? Eur J Cardiothorac Surg 1991; 5: 1721.CrossRefGoogle ScholarPubMed
143.Battistessa, SA, Jackson, M, Hamilton, DI, Patel, RG, McKay, R. Staged surgical management of pulmonary atresia with intact ventricular septum. Cardiol Young 1992; 2: 395402.CrossRefGoogle Scholar
144.Waldman, JD, Karp, RB, Lamberti, JJ, Sand, ME, Ruschhaupt, DG, Agarwala, B. Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections. Ann Thorac Surg 1995; 59: 933–40.CrossRefGoogle ScholarPubMed
145.Koike, K, Perrin, D, Wilson, GJ, Freedom, RM. Myocardial ischemia and coronary arterial involvement in newborn babies less than one week old with pulmonary atresia and intact ventricular septum. In Freedom, RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, 101108.Google Scholar
146.OConnor, WN, Cottrill, CM, Johnson, GL, Noonan, JA, Todd, EP. Pulmonary atresia with intact ventricular septum and Ventriculocoronary Communications: Surgical Significance. Circulation 1982; 65: 805809.CrossRefGoogle ScholarPubMed
147.O'Connor, WN, Stahr, BJ, Cottrill, CM, Todd, EP, Noonan, JA. Ventriculocoronary Connections in Hypoplastic Right Heart Syndrome: Autopsy Serial Section Study of Six Cases. JACC 1988; 11: 10611072.CrossRefGoogle ScholarPubMed
148.Akiba, T and Becker, AE. Disease of the left ventricle in pulmonary atresia with intact ventricular septum. The limiting factor for long-lasting successful surgical intervention. J Thorac Cardiovasc Surg 1994; 108: 18.CrossRefGoogle ScholarPubMed
149.Sideris, EB, Olley, PM, Spooner, E, Farina, M, Foster, E, Trusler, GA, Shaher, R. Left ventricular function and compliance in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1982; 84: 192199.CrossRefGoogle ScholarPubMed
150.Corno, A, Mazzera, E. Marino, B, Parisi, F, Marcelletti, C. Simultaneous patency of ductus arteriosus and surgical shunt in pulmonary atresia with intact ventricular septum. A cause of acutemyocardial failure? Scand J Thorac Cardiovasc Surg 1986; 20: 123–7.CrossRefGoogle Scholar
151.Oosthoek, PW, Moorman, AF, Sauer, U, Gittenberger-de Grout, AC. Capillary distribution in the ventricles of hearts with pulmonary atresia and intact ventricular septum. Circulation 1995; 91: 1790–8CrossRefGoogle ScholarPubMed
152.Bharati, S, McAllister, HAJ, Chiemmongkoltip, P, Lev, M. Congenital Pulmonary Atresia With Tricuspid Insufficiency: Morphologic Study. Am J Cardiol 1977; 40: 70–7.CrossRefGoogle ScholarPubMed
153.Anderson, RH, Silverman, NH, Zuberbuhler, JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia andintact ventricular septum. Pediatr Cardiol 1990; 11: 8690CrossRefGoogle Scholar
154.Haworth, SG, Shinebourne, EA, Miller, GAH. Right-to-left interatrial shunting with 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: 386391.CrossRefGoogle ScholarPubMed
155.Van Praagh, R, Plett, JA, Van Praagh, S. Single ventricle. Pathology, embryology, terminology and classification. Herz 1979; 4: 113150.Google ScholarPubMed
156.Anderson, RH, Penkoske, PA, Zuberbuhler, JR. Variable Morphology oi Ventricular Septal Defect in Double Inlet Left Ventricle. Am J Cardiol 1985; 55: 15601565.CrossRefGoogle Scholar
157.Kirklin, JK, Blackstone, EH, Kirklin, JW, Pacifico, AD, Bargeron, LM. The Fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors. J Thorac Cardiovasc Surg 1986; 92: 1049–64.CrossRefGoogle ScholarPubMed
158.Barber, G, Hagler, DJ, Edwards, WD, Puga, FJ, Danielson, GK, McGoon, DC, Driscoli, DJ. Surgical Repair of Univentricular Heart (Double Inlet Left Ventricle) With Obstructed Anterior Subaortic Outlet Chamber. JACC 1984; 4: 771777.CrossRefGoogle ScholarPubMed
159.Malcic, I, Sauer, U, Stern, H, Kellerer, M, Kuhlein, B, Locher, D, Buhlmeyer, K, Sebening, F. The influence of pulmonary artery banding on outcome after the Fontan operation. J Thorac Cardiovasc Surg 1992; 104:743747.CrossRefGoogle ScholarPubMed
160.Kirklin, JW and Barratt-Boyes, BG. Cardiac Surgery. 2nd Edition. Churchill Livingstone, New York, 1993, 15491580.Google ScholarPubMed
161.Jonas, RA; Castaneda, AR; Lang, P. Single ventricle (single- or double-inlet) complicated by subaortic stenosis: surgical options in infancy. Ann Thorac Surg 1985; 39:361366.CrossRefGoogle ScholarPubMed
162.Cheung, HC; Lincoln, C; Anderson, RH; Ho, SY; Shinebourne, EA; Pallides, S; Rigby, ML. Options for surgical repair in hearts with univentricular atrioventricular connection and subaortic stenosis. J Thorac Cardiovasc Surg 1990, 100: 672–81.CrossRefGoogle ScholarPubMed
163.O'leary, PW, Driscoll, DJ, Connor, AR, Puga, FJ, Danielson, GK. Subaortic obstruction in hearts with a univentricular connection to a dominant left ventricle and an anterior subaortic outlet chamber. Results of a staged approach. J Thorac Cardiovasc Surg 1992; 104:12311237.CrossRefGoogle Scholar
164.Di Donato, R, Di Dicarlo, D, Giannico, S, Marcelletti, C. Palliation of Complex Cardiac Anomalies with Subaortic Stenosis: New Operative Aproach. JACC 1989; 13: 406412.CrossRefGoogle Scholar
165.Ross, DB, Cheung, HC, Lincoln, C. Direct relief of subaortic obstruction in patients with univentricular atrioventricular connection and discordant ventriculoarterial connection: intermediate results. S Thorac Cardio-vasc Surg 1994; 6: 3338.Google ScholarPubMed
166.van Son, JAM, Reddy, VM, Haas, GS, Hanley, FL. Modified surgical techniques for relief of aortic obstruction in (S, L, L) hearts with rudimentary right ventricle and restrcitive bulboventricular foramen. J Thorac Cardiovasc Surg 1995; 110: 909915.CrossRefGoogle Scholar
167.Puga, FJ. Appropriate palliative intervention for infants with double inlet ventricle and tricuspid atresia with discordant ventriculoarterial connection: role of pulmonary artery banding [editorial; comment]. J Am Coil Cardiol 1990, 16:1465–6.CrossRefGoogle ScholarPubMed
168.Franklin, RC; Sullivan, ID; Anderson, RH; Shinebourne, EA; Deanfield, JE. Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis. J Am Coil Cardiol 1990; 16:1455–64.CrossRefGoogle ScholarPubMed
170.Brawn, WJ, Sethia, B, Jagtap, R, Stumper, OFN, Wright, JGC, De Giovanni, JV, Silove, ED, Jackson, M, Sreeram, N. Univentricular heart with systemic outflow obstruction: palliation by primary Damus procedure. Ann Thorac Surg 1995; 59: 14411447.CrossRefGoogle ScholarPubMed
171.Seliem, M, Muster, AJ, Paul, MH, Benson, DW. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coil Cardiol 1989; 14:750755.CrossRefGoogle ScholarPubMed
172.Di Donato, RM, Amodeo, A, di Carlo, DD, Gailetti, L, Rinelli, G, Pasquini, L, Marcelletti, C. Staged Fontan operation for complex cardiac anomalies with subaortic obstruction. J Thorac Cardiovasc Surg 1993, 105:398404.CrossRefGoogle ScholarPubMed
173.Anderson, RH, Yen Ho, S, Wilcox, BR. The surgical anatomy of ventricular septal defects with univentricular atrioventricular connection. J Card Surg 1994; 9: 408426.CrossRefGoogle ScholarPubMed
174.de Vivie, R, Van Praagh, S, Bein, G, Eigster, G, Vogt, J, Van Praagh, R. Transposition of the great arteries with straddling tricuspid valve: Report of two rare cases with acquired subaortic stenosis after main pulmonary artery banding. J Thorac Cardiovasc Surg 1989; 98:205213.CrossRefGoogle ScholarPubMed
175.Rao, PS. Subaortic obstruction after pulmonary artery banding in patients with tricuspid atresia and double-inlet left ventricle and ventriculoarterial discordance. JACC 1991; 18: 15851586(letter).CrossRefGoogle ScholarPubMed
176.Rothman, A, Lang, P, Lock, JE, Jonas, RA, Mayer, JE, Castaneda, AR. Surgical Management of Subaortic Obstruction in Single Left Ventricle and Tricuspid Atresia. JACC 1987; 10: 421426.CrossRefGoogle ScholarPubMed
177.Rychik, J, Murdison, KA, Chin, AJ, Norwood, WI. Surgical management of severe aortic outflow tract obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. JACC 1991; 18:809816.CrossRefGoogle Scholar
178.Jacobs, ML, Rychik, J, Murphy, JD, Nicolson, SC, Steven, JM, Norwood, WI. Results of Norwood's operation for lesions other than hypoplastic left heart syndrome. J Thorac CardiovascSurg 1995; 110: 15551562.CrossRefGoogle ScholarPubMed
179.Ilbawi, MN, De Leon, SY, Wilson, WR Jr, Quinones, JA, Roberson, DA, Husayni, TS, Thilenius, OG, Arcilla, RA. Advantages of early relief of subaortic stenosis in single ventricle equivalents. Ann Thorac Surg 1991; 52:842–9.CrossRefGoogle ScholarPubMed
180.Serraf, A, Conte, S, Lacour-Gayet, F, Bruniaux, J, Sousa-Uva, M, Roussin, R, Planche, C. Systemic obstruction in univentricular hearts: surgical options for neonates. Ann Thorac Surg 1995; 60: 970977.CrossRefGoogle ScholarPubMed
181.Huddleston, CB, Canter, CE, Spray, TL. Damus-Kaye-Stansel with cavo-pulmonary connection for single ventricle and subaortic obstruction. Ann Thorac Surg 1993; 55:339–45; discussion 346.CrossRefGoogle Scholar
182.Matitiau, A, Geva, T, Colan, SD, Sluysmans, T, Parness, IA, Spevak, PJ, Van Der Veide, M, Mayer, JE Jr, Sanders, SP. Bulboventricular foramen size in infants with double-inlet left ventricle or tricuspid atresia with transposed great arteries: influence on initial palliative operation and rate of growth. JACC 1992; 19:142–8.CrossRefGoogle ScholarPubMed
183.Donofrio, MT, Jacobs, ML, Norwood, ML, Rychik, J. Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery. JACC 1995; 26: 10081015.CrossRefGoogle ScholarPubMed
184.Choussat, A, Fontan, F, Besse, P, Valiot, F, Chauve, A, Bricaud, H. Selection criteria for Fontans procedure. In, Paediatric Cardiology, 1977. Eds. Anderson, RH and Shinebourne, EA. Churchill Livingstone, Edinburgh, 1978, 559566.Google Scholar
185.Freedom, RM and Benson, LN. The Fontan Operation: Indications, Outcome, and Survival Data. In, Atlas of Heart Diseases: Congenital Heart Disease. Braunwald, E., Series Editor, Current Science, 1997; 17–1 to 17–10Google Scholar
186.Mayer, JE Jr., Bridges, ND, Lock, JE, Hanley, FL, Jonas, RA, Castaneda, AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992; 103: 444452.CrossRefGoogle ScholarPubMed
187.Bridges, ND, Lock, JE, Castaneda, AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 1990; 82: 16811689.CrossRefGoogle ScholarPubMed
188.Castaneda, AR and Bridges, ND. The Fontan operation with a fenestrated patch-rationale and results. Cardiol Young 1993; 3: 202–20.CrossRefGoogle Scholar
189.Fontan, F, Kirklin, J, Fernandez, G, Blackstone, E. Outcome after a “perfect” Fontan operation. Circulation 1990; 81: 15201536.CrossRefGoogle ScholarPubMed
190.Somerville, J. Changing form and function in one ventricle hearts. Herz 1979; 4: 206212.Google ScholarPubMed
191.Somerville, J. Congenital heart disease-changes in form and function. Br Heart J 1979; 41: 122.CrossRefGoogle ScholarPubMed
192.Somerville, J, Becu, L, Ross, D. Common Ventricle with Acquired Subaortic Obstruction. Am J Cardiol 1974; 34: 206214.CrossRefGoogle ScholarPubMed
193.Chin, AJ, Franklin, WH, Andrews, BAA, Norwood, WI Jr. Changes in ventricular geometry early after Fontan operation. Ann Thorac Surg 1993; 56: 13591365.CrossRefGoogle ScholarPubMed
194.Finta, KM, Beekman, RH, Lupinetti, FM, Bove, EL. Systemic ventricular outflow obstruction progresses after the Fontan operation. Ann Thorac Surg 1994; 58: 11081112.CrossRefGoogle ScholarPubMed
195.Freedom, RM, Mawson, JB, Yoo, S-J, Benson, LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co, Armonk, NY, 1997, 12011260Google Scholar
196.Karl, TR, Watterson, KG, Sano, S, Mee, RBB. Operations for subaortic stenosis in univentricular hearts. Ann Thorac Surg 1991; 52:420427.CrossRefGoogle ScholarPubMed
197.Hopkins, RA, Armstrong, BE, Serwer, GA, Peterson, RJ, Oldham, HN Jr. Physiological rationale for a bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg 1985; 90:391398.CrossRefGoogle ScholarPubMed
198.Nykanen, DG and Freedom, RM. Variations on the theme of Fontan: Consideration of the bidirectional cavopulmonary connection or the fenestrated Fontan. ACC Current Journal Review 1995; 4: 4952.CrossRefGoogle Scholar
199.Lamberti, JJ; Spicer, RL; Waldman, JD; Grehl, TM; Thomson, D; George, L; Kirkpatrick, SE; Mathewson, JW. The bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg 1990; 100:2230CrossRefGoogle ScholarPubMed
200.Bridges, ND; Jonas, RA; Mayer, JE; Flanagan, MF; Keane, JF; Castaneda, AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation 1990; 82(5 Suppl):IV:170–6.Google ScholarPubMed
201.Alejos, JC, Williams, WG, Jarmakani, JM, Galindo, AJ, Isabel-Jones, JB, Drinkwater, D, Laks, H, Kaplan, S. Factors influencing survival in patients undergoing the bidirectional Glenn anastomosis. Am J Cardiol 1995; 75: 10481050.CrossRefGoogle ScholarPubMed
202.Webber, SA; Sett, SS; Le Blanc, JG. Univentricular atrioventricular connection with subaortic stenosis: a staged surgical approach. Ann Thorac Surg 1992; 54:344–7.CrossRefGoogle ScholarPubMed
203.Webber, SA, Le Blanc, JG, Keeton, BR, Salmon, AP, Sandor, GGS, Lamb, RK, Monro, JL. Pulmonary artery banding is not contraindicated in double inlet left ventricle with transposition and aortic arch obstruction. Europ J Cardiothorac Surg 1995; 9: 515520CrossRefGoogle Scholar
204.Ivemark, BI. Implications of Agenesis of the Spleen on the Pathogenesis of Cono-Truncus Anomalies in Childhood. Acta Paediatrica 1955; 44: 711.CrossRefGoogle Scholar
205.Brandt, HM and Liebow, AA. Right Pulmonary Isomerism Associated with Venous, Splenic, and Other Anomalies. Lab Invest 1958; 7: 469503.Google ScholarPubMed
206.Landing, BH, Lawrence, T-YK, Payne, VC Jr, Wells, TR. Bronchial Anatomy in Syndromes with Abnormal Visceral Situs, Abnormal Spleen and Congenital Heart Disease. Am J Cardiol 1971; 28: 456462.CrossRefGoogle ScholarPubMed
207.Landing, B. Five Syndromes (Malformation Complexes) of Pulmonary Symmetry, Congenital Heart Disease, and Multiple Spleens. Pediatric Pathology 1984; 2:125151.CrossRefGoogle ScholarPubMed
208.Becker, AE and Anderson, RH. Isomerism of the atrial appendages-goodbye to asplenia and all that. In, Developmental Cardiology. Morpho-genesis and Function. Eds. Clark, EB and Takao, A, Futura Publishing Co., Mt. Kisco, New York, 1990, 659670.Google Scholar
209.De Tommasi, SM, Daliento, L, Ho, SY, MacArtney, FJ, Anderson, RH. Analysis of atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 specimens with atrial isomerism. Br Heart J 1981; 45: 236247.CrossRefGoogle ScholarPubMed
210.Yoo, S-J, Nykanen, DG, Freedom, RM, Benson, LN, Moes, CAF, Burrows, PE. Retrobronchial vertical vein in totally anomalous pulmonary venous connection to the innominate vein and its specific occurrence in right isomerism. Am J Cardiol 1993; 71: 11981203.CrossRefGoogle Scholar
213.Freedom, RM, Culham, JAG, Moes, CAE. Angiocardiography of Congenital Heart Disease. MacMillan Publishing Co., New York, 1984, 643654.Google Scholar
214.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: 9194.CrossRefGoogle ScholarPubMed
215.Freedom, RM and Smallhorn, JF. Syndromes of right or left atrial isomerism. In, Freedom RM, Benson LN, Smallhorn JE Neonatal Heart Disease. Springer-Verlag, London, 1992, 543560.CrossRefGoogle Scholar
216.Phoon, CK and Neill, CA. Asplenia syndrome: Insight into embryology through an analysis of cardiac and extracardiac anomalies. Am J Cardiol 1994; 73: 581587.CrossRefGoogle ScholarPubMed
217.Freedom, RM, Mawson, JB, Yoo, S-J, Benson, LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co, Armonk, NY, 1997, pp. 12611288.Google Scholar
218.Roberts, WC, Anderson, RC, Edwards, JE. The Significance of Asplenia in the Recognition of Inoperable Congenital Heart Disease. Circulation 1961; 26: 851857.Google Scholar
219.Culbertson, CB, George, BL, Day, RW, Laks, H, Williams, RG. Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure. JACC 1992; 20: 678684.CrossRefGoogle ScholarPubMed
220.Di Donoto, R, Di Carlo, D, Squitieri, C, Rossi, E, Ammirati, A, Marino, B, Marcelletti, C. Palliation of Cardiac Malformations Associated with Right Isomerism (Asplenia Syndrome) in Infancy. Ann Thorac Surg 1987; 44: 3539.CrossRefGoogle Scholar
221.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; 96:212–8.CrossRefGoogle ScholarPubMed
222.Julsrud, PR, Danielson, GK. A modification of the Fontan procedure incorporating anomalies of systemic and pulmonary venous return. J Thorac Cardiovasc Sueg 1990; 100: 233–9CrossRefGoogle ScholarPubMed
223.Lamberti, JJ, Waidman, JD, Mathewson, JW, Kirkpatrick, SE. Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass. J Thorac Cardiovasc Surg 1984; 88: 627630.CrossRefGoogle ScholarPubMed
224.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; 106:248254.CrossRefGoogle ScholarPubMed
225.Marcelletti, C, Di Donato, R, Nijveld, A, Squitieri, C, Bulterijs, AH, Naeff, M, Schuller, J, Becker, AE. Right and Left Isomerism: The Cardiac Surgeon's View. Ann Thorac Surg 1983; 35: 400405.CrossRefGoogle ScholarPubMed
226.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(S Pt 2):II–141–8.Google ScholarPubMed
227.Rose, V, Izukawa, T, Moes, CAE. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac m alformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840–85.CrossRefGoogle Scholar
228.Okita, Y, Miki, S, Tamura, T, Kusuhara, K, Ueda, Y, Tahata, T, Yamanaka, K, Mitani, Y, Tamura, M. Bidirectional cavopulmonary anastomosis using vertical vein for right isomerism, pulmonary atresia and Tapvr. Ann Thorac Surg 1991; 52: 544546.CrossRefGoogle ScholarPubMed
229.Okita, Y, Miki, S, Tamura, T, Kusuhara, K, Ueda, Y, Tahata, T, Yamanaka, K, Shiraishi, S, Tamura, T. Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism. Ann Thorac Surg 1989; 47: 302304.CrossRefGoogle ScholarPubMed
230.Mizuhara, H; Yokota, M; Sakamoto, K; Ikai, A; Kado, M; Niwa, H; Nagato, H. [Relief of pulmonary venous obstruction for asplenia syndrome associated with total anomalous pulmonary venous connection in neonates and infants]. Nippon Kyobu Geka Gakkai Zasshi (Japan), Mar 1994,42(3) p379–84Google ScholarPubMed
231.Sadiq, M, Stumper, O, De Giovanni, JV, Wright, JGC, Sethia, B, Brawn, WJ, Silove, ED. Management and outcome of infants and chldren with right atrial isomerism. Heart 1996; 75: 314–31.CrossRefGoogle Scholar
232.Phoon, CK, Neill, CA. Asplenia syndrome-risk factors for early unfavorable outcome. Am J Cardiol 1994; 73: 12351237CrossRefGoogle ScholarPubMed
233.Franklin, RCG, Spiegaihalter, DJ, Sullivan, ID, Anderson, RH, Thoele, DG, Shinebourne, EA, Deanfield, JE. Tricuspid atresia presenting in infancy. Survival and suitability for the Fontan operation. Circulation 1993; 87:427439.CrossRefGoogle ScholarPubMed
234.Liebman, J, Cullum, L, Belloc, NB. Natural history of transposition of the great arteries. Anatomy and birth and death characteristics. Circulation 1969; XL: 237262.CrossRefGoogle Scholar
235.Freedom, RM, Mawson, JB, Yoo, S-J, Benson, LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co, Armonk, NY, 1997, pp. 9871070Google Scholar
236.Trusler, GA. Mustard operation for transposition: historical aspects and results. In, Transposition of the great arteries 25 years after Rashkind ba-Iloon septostomy. Eds, Vogel, M and Buhlmeyer, K. Springer-Verlag, New York, 1992; 113117.CrossRefGoogle Scholar
237.Trusler, GA, Gonzales, JC, Craig, BG, Williams, WG, Freedom, RM, and Rowe, RD. Isolated transposition of the Great arteries: the present unnatural history. In, Paediatric Cardiology. Eds. Doyle, EF, Engle, MA, Gersony, WM, Rashkind, WJ, Talner, NS. Springer-Verlag, New York, 1986, 13151319.CrossRefGoogle Scholar
238.Soongswang, P, Adatia, , Newman, C, Smallhorn, J, Williams, W, Freedom, R. Mortality in potential arterial switch candidates with d-transposition of the great arteries. JACC 1997; 29, No.2:106A (932–68)Google Scholar
239.Gutgesell, HP, Massaro, TA, Kron, IL. The arterial switch operation for transposition of the great arteries in a consortium of university hospitals. Am J Cardiol 1994; 74:959–60.CrossRefGoogle Scholar
240.Jenkins, K, Newburger, J, Lock, J, Davis, R, Coffman, G, lezzoni, L. In-Hospital Mortality for Surgical Repair of Congenital Heart Defects: Preliminary Observations of Variation by Hospital Caseload. Pediatrics 1995; 95 323330Google ScholarPubMed
241.Fulton, DR and Geggel, RL. Letter-to-the editor. In-hospital mortality for surgical repair of congenital heart defects: preliminary observations of variation by hospital caseload. Pediatrics 1995; 96: 980CrossRefGoogle Scholar
242.The Canadian Pediatric Cardiology Association. Paediatric cardiac investigation and surgery in Canada. Canadian Journal of Cardiology 1991; 7:10444446Google Scholar
243.Adams, H, Blumenthal, S, DuShane, J, Lurie, P, McNamara, D, Rudolph, A, Abrahamson, S, Foster, J. Manpower and Training Requirements in Pediatric Cardiology Pediatrics 1973; 51:5813821Google Scholar
244.Molier, J, Adams, EPaediatric cardiology manpower in the 1980s. Am Heart J 1996; 112:3599604CrossRefGoogle Scholar
245.Williams, R, Kennedy, T, Moller, J. Pediatric Cardiology in the 1990s. JACC 1994; 23: 4977980.CrossRefGoogle ScholarPubMed
246.Srivastava, D, Preminger, TJ, Lock, JE, Mandell, V, Keane, JF, Mayer, JE Jr., Kozakewich, H, Spevak, PJ. Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 1995; 92: 12171222.CrossRefGoogle ScholarPubMed
247.Laberge, J-M, Brandt, ML, Lebecque, P, Moulin, D, Veykemans, F, Paradis, K, Pelletier, L et al. Reversal of cirrhosis-related pulmonary shunting in two children by orthotopic liver transplantation. Transplantation 1992; 53: 11351138.Google ScholarPubMed
248.Fewtrell, MS, Noble-Jamieson, G, Revell, S, Valente, J, Friend, P, Johnston, P, Rasmussen, A, Jamieson, N, Caine, RY; Barnes, ND. Intrapulmonary shunting in the biliary atresia/ polysplenia syndrome: reversal after liver transplantation. Arch Dis Child 1994; 70:501–4.CrossRefGoogle ScholarPubMed
249.Knight, WB and Mee, RBB. A cure for pulmonary arteriovenous fistulas. Ann Thorac Surg 1995; 59: 9991001.CrossRefGoogle ScholarPubMed
250.Moore, JW; Kirby, WC; Madden, WA; Gaither, NS. Development of pulmonary arteriovenous malformations after modified Fontan operations. J Thorac Cardiovasc Surg 1989; 98:1045–50.CrossRefGoogle ScholarPubMed
251.Davis, CA, Driscoll, DJ, Perrault, J, Greenwood, DH, Schaff, HV, Puga, FJ, Danielson, GK, Feldt, RH. Enteric protein loss after the Fontan operation. Mayo Clin Proc 1994; 69:112–4CrossRefGoogle ScholarPubMed
252.Colloridi, V; Roggini, M; Formigari, R; Ventriglia, F; Giglioni, E. Plastic bronchitis as a rare complication of Fontan's operation. Pediatr Cardiol 1990; 11:228.CrossRefGoogle ScholarPubMed
253.Jett, JR, Tazelaar, HD, Keim, LW, Ingrassia, TS 3d. Plastic bronchitis: an old disease revisited. Mayo Clin Proc 1991; 66:305–11CrossRefGoogle ScholarPubMed
254.Seear, M, Hui, H, Magee, F, Bohn, D, Cutz, E. Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crir Care Med 1997; 155: 364–37.CrossRefGoogle Scholar
255. Section of Cardiology. American Academy of Paediatric Guidelines for Paediatric Cardiology Diagnostic and Treatment Centers. Pediatrics 1991; 87: 576580CrossRefGoogle Scholar
256.Somerville, J. Personal View. Future of paediatric cardiology and irs patients. European Heart Journal 1997; 18 198207CrossRefGoogle Scholar