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The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum

Published online by Cambridge University Press:  16 September 2005

Robert M. Freedom
Affiliation:
Division of Cardiology, Department of Paediatrics, The University of Toronto Faculty of Medicine, Toronto, Ontario, Canada Department of Paediatric Laboratory Medicine and Pathology, The University of Toronto Faculty of Medicine, Toronto, Ontario, Canada Division of Cardiac Imaging, Department of Diagnostic Imaging, The Hospital for Sick Children and Departments of Paediatrics, Laboratory Medicine and Pathology and Medical Imaging, The University of Toronto Faculty of Medicine, Toronto, Ontario, Canada
Robert H. Anderson
Affiliation:
Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
Don Perrin
Affiliation:
Department of Paediatric Laboratory Medicine and Pathology, The University of Toronto Faculty of Medicine, Toronto, Ontario, Canada

Abstract

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Type
Continuing Medical Education
Copyright
© 2005 Cambridge University Press

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References

Hunter J. Three cases of mal-conformation of the heart. Medical observations and inquiries 1784; 6: 291294.Google Scholar
Hare CJ. Malformation of the heart. Complete closure of the orifice of the pulmonary artery. Very small foramen ovale. Cyanosis. Trans Pathol Soc Lond 1853; 4: 8185.Google Scholar
Peacock TB. Cases of malformation of the heart. Case 1 – Entire obliteration of the orifice and trunk of the pulmonary artery; cyanosis; death from cancrum oris. Trans Pathol Soc Lond 1871; 22: 8588.Google Scholar
Abercrombie J. Congenital atresia of right ventricle, ductus arteriosus patent (card specimen). Trans Pathol Soc Lond 1883; 34: 78.Google Scholar
Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 1926; 13: 273283.Google Scholar
Lauer RM, Fink HP, Petry EL, Dunn MI, Diehl AM. Angiographic demonstration of intramyocardial sinusoids in pulmonary-valve atresia with intact ventricular septum and hypoplastic right ventricle. New Engl J Med 1964; 271: 6872.Google Scholar
Freedom RM, 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.Google Scholar
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.Google Scholar
Kasznica J, Ursell PC, Blanc WA, Gersony WM. Abnormalities of the coronary circulation in pulmonary atresia and intact ventricular septum. Am Heart J 1987; 114: 14151420.Google Scholar
Finegold MJ, Klein KM. Anastomotic coronary vessels in hypoplasia of the right ventricle. Am Heart J 1971; 82: 678683.Google Scholar
MacMahon HE, Dickinson PCT. Occlusive fibroelastosis of coronary arteries in the newborn. Circulation 1967; 35: 39.Google Scholar
Calder AL, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987; 59: 437442.Google Scholar
Sauer U, Bindl L, Pilossoff V, et al. Pulmonary atresia with intact ventricular septum and right ventricle–coronary artery fistulae: Selection of patients for surgery. In: Pediatric Cardiology. Springer-Verlag, New York, 1986.
Freedom RM, Benson L, Wilson GJ. The coronary circulation and myocardium in pulmonary and aortic atresia with an intact ventricular septum. In: Marcelletti C Anderson RH Becker AE, Corno A, di Dicarlo D, Mazzera E (eds). Paediatric Cardiology, Vol. 6. Churchill Livingstone, Edinburgh 1986, pp 7896.
Burrows PE, Freedom RM, Benson LN, Moes CAF. Coronary angiography of pulmonary atresia, Hypoplastic right ventricle, and ventriculocoronary communications. AJR 1990; 154: 789795.Google Scholar
O'Connor WN, Cottrill CM, Johnson GL, Noonan JA, Todd EP. Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance. Circulation 1982; 65: 805809.Google Scholar
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. J Am Coll Cardiol 1988; 11: 10611072.Google Scholar
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, pp 3844.Google Scholar
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
Freedom RM, 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. University Park Place, Baltimore, 1984, pp 217255.
Freedom RM, Culham JAG, Moes CAF. Angiocardiography of Congenital Heart Disease. Macmillan Publishing Co., New York, 1984, pp 221253.
Wilson GJ, Freedom RM, Koike K, Perrin D. The Coronary Arteries: Anatomy and Histopathology. In: Freedom RM (ed.). Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Co., Mt. Kisco, New York, 1989, pp 7588.
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: 213237.Google Scholar
Lightfoot NE, Coles JG, Dasmahapatra HK, et al. Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically. Int J Cardiol 1989; 24: 159164.Google Scholar
Hawkins JA, Thorne JK, Boucek MM, et al. Early and late results in pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1990; 100: 492497.Google Scholar
Niederhuser U, Bauer EP, von Segesser LK, et al. Pulmonary atresia and intact ventricular septum: results and predictive factors of surgical treatment. Thorac Cardiovasc Surg 1992; 40: 130134.Google Scholar
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: 733738.Google Scholar
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: 406427.Google Scholar
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: 359366.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: 924931.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: 10461052.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: 299308.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: 10001007.Google Scholar
Cornell SH. Myocardial sinusoids in pulmonary valvular atresia. Radiology 1966; 86: 421424.Google Scholar
Sissman NJ, Abrams HL. Bidirectional shunting in a coronary artery–right ventricular fistula associated with pulmonary atresia and an intact ventricular septum. Circulation 1965; 32: 582588.Google Scholar
Gittenberger-de Groot AC, Tennstedt C, Chaoui R, Lie-Venema H, Sauer U, Poelmann RE. Ventriculo coronary arterial communications (VCAC) and myocardial sinusoids in hearts with pulmonary atresia with intact ventricular septum: two different diseases. Prog Pediatr Cardiol 2001; 13: 157164.Google Scholar
Van Praagh R, Ando M, Van Praagh S, et al. Pulmonary atresia: Anatomic considerations. In: Kidd BSL, Rowe RD (eds). The Child With Congenital Heart Disease After Surgery. Futura Publishing Co., Mount Kisco, NY, 1976, pp 103135.
Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979; 41: 281288.Google Scholar
Freedom RM, Burrows PE, Smallhorn JF. Pulmonary atresia and intact ventricular septum. In: Freedom RM, Benson LN, Smallhorn JF (eds). Neonatal Heart Disease. Springer-Verlag, London, 1992, pp 285307.
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: 382386.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: 471474.Google Scholar
Patel CR, Spector ML, Zahka KG. Congenitally corrected transposition with pulmonary atresia and intact ventricular septum. Cardiol Young 2000; 10: 268270.Google Scholar
Milanesi O, Daliento L, Thiene G. Solitary aorta with bilateral ductal origin of non-confluent pulmonary arteries in pulmonary atresia with intact ventricular septum. Int J Cardiol 1990; 29: 9092.Google Scholar
Luciani GB, Swilley S, Starnes VA. Pulmonary atresia, intact ventricular septum, and major aortopulmonary collaterals: morphogentic and surgical implications. J Thorac Cardiovasc Surg 1995; 110: 853854.Google Scholar
Serra A Jr, Chamie F, Freedom RM. Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: 5th aortic arch with a systemic-to-pulmonary arterial connection. Cardiol Young 2000; 10: 419422.Google Scholar
Albanese SB, Carotti A, Toscano A, Marino B, Di Donato RM. Pulmonary atresia with intact ventricular septum and systemic-pulmonary collateral arteries. Ann Thorac Surg 2002; 73: 13221324.Google Scholar
Mildner RJ, Kiraly L, Sreeram N. Pulmonary atresia, “intact ventricular septum”, and aortopulmonary collateral arteries. Heart 1997; 77: 173175.Google Scholar
Patel CR, Spector ML, Zahka KG. Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery. Cardiol Young 1999; 9: 512515.Google Scholar
Freedom RM. Pulmonary Atresia and Intact Ventricular Septum. Futura Publishing Company, Mt. Kisco, NY, 1989, 262 pp.
Kauffman SL, Andersen DH. Persistent venous valves, maldevelopment of the right heart, and coronary artery–ventricular communications. Am Heart J 1963; 66: 664669.Google Scholar
Schutte DA, Rowland DG, Bharati S. Prominent venous valves in hypoplastic right hearts. Am Heart J 1997; 134: 527531.Google Scholar
Bharati S, McAllister HAJ, Chiemmongkoltip P, Lev M. Congenital pulmonary atresia with tricuspid insufficiency: morphologic study. Am J Cardiol 1977; 40: 7075.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: 914920.Google Scholar
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.Google Scholar
Freedom RM, Mawson J, Yoo S-J, Benson LN. Congenital Heart Disease: Textbook of Angiocardiography. Futura Publishing Co., Armonk, NY, 1997, pp 617672.
Abbott ME. Atlas of Congenital Cardiac Disease. Am Heart Assoc., 1939, p 62.Google Scholar
Abbott ME. McGill University Publications, Series VIII. New accessions in cardiac anomalies. 1. Pulmonary atresia of inflammatory origin. Montreal, 1924, pp 111116.Google Scholar
Bauer DdeF, Astbury EC. Congenital cardiac disease. Bibliography of the 1000 cases analyzed in Maude Abbott's Atlas. With an index. Am Heart J 1942; 22: 688732.Google Scholar
Vigorita V. Epicardial nodules: a possible sign of coronary endarteritis with hypoplastic right heart syndrome. Johns Hopkins Med J 1978; 142: 215217.Google Scholar
Wearn JT, Mettier SR, Klumpp TG, Zschiesche LJ. The nature of the vascular communications between the coronary arteries and the chambers of the heart. Am Heart J 1933; 9: 143164.Google Scholar
Keith JD, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. The Macmillan Co., New York, 1958; 2nd edn, 1967; 3rd edn, 1978.
Taussig HB. Congenital Malformations of the Heart. The Commonwealth Fund, New York, 1947, p 618.
Nadas AS. Pediatric Cardiology. WB Saunders, Philadelphia, 1957, p 475.
Kjellberg SR, Mannheimer E, Rudhe U, Jonsson B. Diagnosis of Congenital Heart Disease. The Year Book Publishers, Inc., Chicago, 1955, p 649.
Kjellberg SR, Mannheimer E, Rudhe U, Jonsson B. Diagnosis of Congenital Heart Disease, 2nd edn. The Year Book Publishers, Inc., Chicago, 1959, p 866.
Taussig HB. Congenital Malformations of the Heart, Vol 2. Harvard University Press, Cambridge, 1960, p 1049.
Nadas AS. Pediatric Cardiology. WB Saunders, Philadelphia, 1963, p 828.
Nadas AS, Fyler DC. Pediatric Cardiology. WB Saunders, Philadelphia, 1972, p 749.
Williams RR, Kent GBJ, Edwards JE. Anomalous cardiac blood vessel communicating with the right ventricle. Arch Path 1951; 52: 480487.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: 591602.Google Scholar
Oppenheimer EH, Esterly JR. Some aspects of cardiac pathology in infancy and childhood. II. Unusual coronary endarteritis with congenital cardiac malformations. Bull Johns Hopkins Hosp 1966; 19: 343354.Google Scholar
Esterly JR, Oppenheimer EH. Some aspects of cardiac pathology in infancy and childhood. IV. Myocardial and coronary lesions in cardiac malformations. Pediatrics 1967; 39: 896903.Google Scholar
Franciosi RA, Blanc WA. Myocardial infarcts in infants and children. I. A necropsy study in congenital heart disease. J Pediatr 1968; 73: 309319.Google Scholar
Setzer E, Ermocilla R, Tonkin I, John E, Sansa M, Cassady G. Papillary muscle necrosis in a neonatal autopsy population: incidence and associated clinical manifestations. J Pediatr 1980; 96: 289294.Google Scholar
Fyfe DA, Edwards WD, Driscoll DJ. Myocardial ischaemia in patients with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 1986; 8: 402406.Google Scholar
Gersony WM, Bernhard WF, Nadas AS, Gross RE. Diagnosis and surgical treatment of infants with critical pulmonary outflow obstruction. Study of thirty-four infants with pulmonary stenosis or atresia, and intact ventricular septum. Circulation 1967; 35: 765776.Google Scholar
Gamboa R, Gersony WM, Nadas AS. The electrocardiogram in tricuspid and pulmonary atresia with intact ventricular septum. Circulation 1966; 34: 2437.Google Scholar
Trusler GA, Fowler RS. The surgical management of pulmonary atresia with intact ventricular septum and hypoplastic right ventricle. J Thorac Cardiovasc Surg 1970; 59: 740743.Google Scholar
Williams WG, Burrows P, Freedom RM, et al. Thromboexclusion of the right ventricle in a subset of children with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1991; 101: 222229.Google Scholar
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. Pediatr Cardiol 1984; 5: 221224.Google Scholar
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: 933940.Google Scholar
Freedom RM, Yoo S-J, Dyamenahalli U. Pulmonary atresia and intact ventricular septum. In: Freedom RM, Yoo S-J, Mikailian H, Williams WG (eds). The Natural and Modified History of Congenital Heart Disease. Blackwell Publishing Co, Oxford, 2004, pp 386396.
Mair DD, Julsrud PR, Puga FJ, Danielson GK. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results. J Am Coll Cardiol 1997; 29: 13591364.Google Scholar
Freedom RM, Wilson GJ. Endomyocardial abnormalities. In: Freedom RM (ed.). Pulmonary Atresia and Intact ventricular Septum. Futura Publishing Co., Mt. Kisco, NY, 1989, pp 8999.
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.Google Scholar
Bryan C, Oppenheimer EH. Ventricular endocardial fibro-elastosis. Basis for its presence or absence in cases of pulmonic and aortic atresia. Arch Path 1969; 87: 8286.Google Scholar
Freedom RM, White RI Jr, Ho CS, Gingell RL, Hawker RE, Rowe RD. Evaluation of patients with pulmonary atresia and intact ventricular septum by double catheter technique. Am J Cardiol 1974; 33: 892895.Google Scholar
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.Google Scholar
Pawade A, Karl T. Management strategy in neonates presenting with pulmonary atresia with intact ventricular septum. Curr Opin Pediatr 1994; 6: 600605.Google Scholar
Goor DA, Lillehei CW. Congenital Malformations of the Heart. Grune and Stratton, New York, 1975, pp 1115.
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: 272280.Google Scholar
Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992; 86: 15161528.Google Scholar
Giglia TM, Jenkins KJ, Matitiau A, et al. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation 1993; 88: 22482256.Google Scholar
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: 183188.Google Scholar
L'Ecuyer TJ, Poulik JM, Vincent JA. Myocardial infarction due to coronary abnormalities in pulmonary atresia with intact ventricular septum. Pediatr Cardiol 2001; 22: 6870.Google Scholar
Hubbard JF, Girod DA, Caldwell RL, Hurwitz RA, Mahony LA, Waller BF. Right ventricular infarction with cardiac rupture in an infant with pulmonary atresia with intact ventricular septum. J Am Coll Cardiol 1983; 2: 363368.Google Scholar
Dusek J, Ostadal B, Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 1975; 99: 312317.Google Scholar
Bulkley BH, D'Amico B, Taylor AL. Extensive myocardial fiber disarray in aortic and pulmonary atresia: relevance to hypertrophic cardiomyopathy. Circulation 1983; 67: 191198.Google Scholar
Akiba T, 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.Google Scholar
Oosthoek PW, Moorman AFM, Sauer U, Gittenberger-de Groot AC. Capillary distribution in the ventricles of hearts with pulmonary atresia and intact ventricular septum. Circulation 1995; 91: 17901798.Google Scholar
Razzouk AJ, Freedom RM, Cohen AJ, et al. The recognition, identification of morphologic substrate, and treatment of subaortic stenosis after a Fontan operation. J Thorac Cardiovasc Surg 1992; 104: 938944.Google Scholar
Giannico S. Successful balloon avulsion of tricuspid valve in a neonate with pulmonary atresia and intact ventricular septum [Letter]. J Thorac Cardiovasc Surg 1988; 96: 488491.Google Scholar
Squitieri C, Di Carlo D, Giannico S, Marion B, Giamberti A, Marcelletti C. Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1989; 97: 779784.Google Scholar
Schroeder JK, Wiggins JW. Inducing tricuspid regurgitation as palliation. Pediatr Cardiol 1992; 13: 110111.Google Scholar
Lenox CC, Briner J. Absent proximal coronary arteries associated with pulmonic atresia. Am J Cardiol 1972; 30: 666669.Google Scholar
Ueda K, Saito A, Nakano H, Hamazaki Y. Absence of proximal coronary arteries associated with pulmonary atresia. Am Heart J 1983; 106: 596598.Google Scholar
Freedom RM, Yoo SJ, Javois A. A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum. Cardiol Young 2000; 10: 6063.Google Scholar
Selamet SE, Hsu DT, Thaker HM, Gersony WM. Complete atresia of coronary ostia in pulmonary atresia and intact ventricular septum. Pediatr Cardiol 2004; 25: 6769.Google Scholar
Lajos P, Love J, Salim MA, Wang W, Cardarelli MG. Total right ventricular dependent coronary circulation in pulmonary atresia with intact ventricular septum. Ann Thorac Surg 2004; 77: 10871088.Google Scholar
Blackman MS, Schneider B, Sondheimer HM. Absent proximal left main coronary artery in association with pulmonary atresia. Br Heart J 1981; 46: 449451.Google Scholar
Freedom RM, Culham JAG, Rowe RD. Left atrial to coronary sinus fenestration. (Partially unroofed coronary sinus.) Morphological and angio-cardiographic observations. Br Heart J 1981; 46: 6368.Google Scholar
Ono M, Otake S, Fukushima N, et al. Huge right ventricular–right coronary artery fistula compromising right ventricular function in a patient with pulmonary atresia and intact ventricular septum. A case report. J Thorac Cardiovasc Surg 2001; 122: 10301032.Google Scholar
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.Google Scholar
Ho SY, De S Carvalho J, Sheffield E. Anomalous origin of single coronary artery in association with pulmonary atresia. Int J Cardiol 1988; 20: 125128.Google Scholar
Yokose T, Doi M, Kimura Y, Ogata Tl. Ventriculo-coronary micro-communications in pulmonary atresia and sequential changes of coronary arteries. Acta Pathol Jpn 1987; 37: 10331040.Google Scholar
Law Y, Mawson J, Mikailian H, Nykanen D, Freedom RM, Adatia I. Transatrial selective coronary arteriography in pulmonary atresia with intact ventricular septum. Cathet Cardiovasc Diagn 1998; 43: 174176.Google Scholar
Drant S. The echocardiographic evaluation of pulmonary atresia with intact ventricular septum. Progress in Pediatric Cardiology 2001; 13: 165175.Google Scholar
Garcia JA, Zellers TM, Weinstein EM, Mahony L. Usefulness of Doppler echocardiography in diagnosing right ventricular coronary arterial communications in patients with pulmonary atresia and intact ventricular septum and comparison with angiography. Am J Cardiol 1998; 81: 103104.Google Scholar
Satou GM, Perry SB, Gauvreau K, Geva T. Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol 2000; 85: 13191324.Google Scholar
Minich LL, Tani LY, Ritter S, Williams RV, Shaddy RE, Hawkins JA. Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum. Am J Cardiol 2000; 85: 13251328.Google Scholar
Fyler DC. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980; 65 (Suppl): 376461.Google Scholar
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985; 121: 3136.Google Scholar
Samanek M, Voriskova M. Congenital heart disease among 815, 569 children born between 1980 and 1990 and their 15-year survival: A prospective Bohemia survival study. Pediatr Cardiol 2000; 20: 411417.Google Scholar
Raunikar RA, Strong 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 257270.
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: 562566.Google Scholar
Leonard H, Derrick G, O'Sullivan J, Wren C. Natural and unnatural history of pulmonary atresia. Heart 2000; 84: 499503.Google Scholar
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: 586590.Google Scholar
Yoshimura N, Yamaguchi M, Ohashi H, et al. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg 2003; 126: 14171426.Google Scholar
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.Google Scholar
Olley PM, Coceani F, Bodach E. E-type prostaglandins: A new emergency therapy for certain cyanotic congenital heart malformations. Circulation 1976; 53: 728731.Google Scholar
Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB (eds). Tricuspid atresia and management of single-ventricle physiology. In: Kirklin/Barratt-Boyes Cardiac Surgery, 3rd edn. Churchill Livingstone, Philadelphia, 2003, pp 11131175.
Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Pulmonary atresia with intact ventricular septum: results of the Fontan procedure. Ann Thorac Surg 1997; 63: 669675.Google Scholar
Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB (eds). Pulmonary atresia and intact ventricular septum. In Kirklin/Barratt-Boyes Cardiac Surgery, 3rd edn. Churchill Livingstone, Philadelphia, 2003, pp 10951112.
Lewis AB, Wells W, Lindesmith GG. Evaluation and surgical treatment of pulmonary atresia and intact ventricular septum in infancy. Circulation 1983; 67: 13181323.Google Scholar
Patel R, Freedom RM, Moes CAF, et al. 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.Google Scholar
Allan LD, Cook A. Pulmonary atresia with intact ventricular septum in the fetus. Cardiol Young 1992; 2: 367376.Google Scholar
Patel CR, Dahms BB, Sallee D. Pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection – prenatal echocardiographic diagnosis. Cardiol Young 2001; 11: 352354.Google Scholar
Sharland G. Pulmonary valve abnormalities. In: Allan L, Hornberger L, Sharland G (eds). Textbook of Fetal Cardiology. Greenwich Medical Media Limited, London, 2000, pp 333347.
Maeno YV, Boutin C, Hornberger LK, et al. Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart 1999; 81: 661668.Google Scholar
Sandor GSS, Cook AC, Sharland G, Yen Ho S, Potts JE, Anderson RH. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life. Cardiol Young 2002; 12: 436444.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: 228233.Google Scholar
Khoshhal S, Sandor GSS, Duncan WJ. Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure. Cardiol Young 2004; 14: 335337.Google Scholar
Robicsek F, Bostoen H, Sanger PW. Atresia of the pulmonary valve with normal pulmonary artery and intact ventricular septum in a 21-year-old woman. Angiology 1966; 17: 896901.Google Scholar
McArthur JD, Munsi SC, Sukumar IP, Cherian G. Pulmonary valve atresia with intact ventricular septum. Report of a case with long survival and pulmonary blood supply from an anomalous coronary artery. Circulation 1971; 44: 740745.Google Scholar
Ekman Joelsson BM, Sunnegardh J, Hanseus K, et al. The outcome of children born with pulmonary atresia and intact ventricular septum in Sweden from 1980 to 1999. Scand Cardiovasc J 2001; 35: 192198.Google Scholar
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 acute myocardial failure? Scand J Thorac Cardiovasc Surg 1986; 20: 123127.Google Scholar
Blackstone EH, Kirklin JW, Hanley FH. What proportion of neonates with pulmonary atresia and intact ventricular septum reach definitive repair? Circulation 1996; 94 (Suppl): I173 [Abstract].Google Scholar
Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol 2000; 86: 12721274.Google Scholar
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, Vol 6. Churchill Livingstone, New York, 1986, pp 97107.
Freeman JE, DeLeon SY, Lai S, Fisher EA, Ow P, Pifarre R. Right ventricle-to-aorta conduit in pulmonary atresia with intact ventricular septum and coronary sinusoids. Ann Thorac Surg 1993; 56: 13931395.Google Scholar
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 Scholar
Asou T, Matsuzaki K, Matsui K, Karl TR, Mee RB. Veno-venous bypass to prevent myocardial ischemia during right heart bypass operation in PA, IVS, and RV dependent coronary circulation. Ann Thorac Surg 2000; 69: 955956.Google Scholar
Freedom RM, Yoo S-J. Hypoplastic left heart syndrome. In: Freedom RM, Yoo S-J, Mikailian H, Williams WG (eds). The Natural and Modified History of Congenital Heart Disease. Blackwell Publishing Co, Oxford, 2004, pp 397407.
Fenton KN, Pigula FA, Gandhi SK, Russo L, Duncan KF. Interim mortality in pulmonary atresia with intact ventricular septum. Ann Thorac Surg 2004; 78: 19941998.Google Scholar
Freedom RM, Yoo S-J, Williams WG. The Fontan-Kreutzer procedure. In: Freedom RM, Yoo S-J, Mikailian H, Williams WG (eds). The Natural and Modified History of Congenital Heart Disease. Blackwell Publishing Co, Oxford, 2004, pp 449459.
Freedom RM, Yoo S-J. Complications of the Fontan procedure. In: Freedom RM, Yoo S-J, Mikailian H, Williams WG (eds). The Natural and Modified History of Congenital Heart Disease. Blackwell Publishing Co, Oxford, 2004, pp 450470.
Hausdorf G, Gravinghoff L, Keck EW. Effects of persisting myocardial sinusoids on left ventricular performance in pulmonary atresia with intact ventricular septum. Eur Heart J 1987; 8: 291296.Google Scholar
Sideris EB, Olley PM, Spooner E, et al. Left ventricular function and compliance in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1982; 84: 192199.Google Scholar
Tanoue Y, Kado H, Maeda T, Shiokawa Y, Fusazaki N, Ishikawa S. Left ventricular performance of pulmonary atresia with intact ventricular septum after right heart bypass surgery. J Thorac Cardiovasc Surg 2004; 128: 710717.Google Scholar
Ekman-Joelsson B-M, Berntsson L, Sunnegardh J. Quality of life in children with pulmonary atresia and intact ventricular septum. Cardiol Young 2004; 14: 615623.Google Scholar