Medication Summary

Congenital structural malformations such as tricuspid atresia cannot be corrected with drug therapy. However, some of the pathophysiologic abnormalities are amenable to treatment, and some functions can improve.

Neonates with arterial hypoxemia, pulmonary oligemia, and ductal-dependent pulmonary flow can experience improvement with intravenous administration of alprostadil (PGE1). PGE1 helps to dilate the ductus arteriosus with resultant augmentation of pulmonary blood flow and improvement in systemic arterial oxygen saturation. This effect allows for stabilization of the neonate's condition so that a detailed workup can be performed and important issues regarding management can be defined. PGE1 is effective only in the neonatal period; therefore, a more permanent solution involving a Blalock-Taussig type of shunt should be offered to resolve pulmonary oligemia.

In neonates with severe aortic coarctation, PGE1 may bypass the aortic obstruction by opening the ductus. After the patient's condition is stabilized, surgical repair of coarctation may be undertaken. An alternative approach is balloon angioplasty, especially if the coarctation is discrete. If balloon angioplasty is contemplated on an urgent basis, PGE1 should not be administered.

In the presence of congestive heart failure, decongestive medications such as digoxin, furosemide, and afterload-reducing agents may be administered. Angiotensin-converting enzyme inhibitors reduce afterload and are generally thought to be useful in augmenting left ventricular function, especially after a bidirectional Glenn procedure and Fontan operation. Anticoagulants and platelet-inhibiting drugs are thought to be useful in preventing thrombus formation after a Fontan operation.

The American Heart Association recommends that prophylaxis with antibiotics be given before any bacteremia-producing procedures are performed. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Class Summary

These agents are used to dilatate the ductus. Alprostadil (PGE1) is used for treatment of ductal-dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.

Alprostadil IV (Prostin VR)

View full drug information

Used to open ductus in young neonates with ductal-dependent pulmonary flow or severe aortic coarctation. First-line palliative therapy to temporarily maintain patency of ductus arteriosus prior to surgery. Produces vasodilation and increases cardiac output. Inhibits platelet aggregation and stimulates intestinal and uterine smooth muscle. Each 1-mL ampule contains 500 mcg/mL.

Rao PS. Tricuspid atresia: anatomy, imaging, and natural history. Braunwald E, Freedom R, eds. Atlas of Heart Disease: Congenital Heart Disease. Philadelphia, PA: Current Medicine; 1997. Vol 12: 14.1.
Rao PS. Tricuspid atresia. Curr Treat Options Cardiovasc Med. 2000 Dec. 2(6):507-20. [QxMD MEDLINE Link].
Kuhne M. Uber zwei falle kongenitaler atreside des ostium venosum dextrum. Jahrb Kinderh. 1906. 63:235.
Rashkind WJ. Tricuspid atresia: a historical review. Pediatr Cardiol. 1982. 2(1):85-8. [QxMD MEDLINE Link].
Rao PS. Terminology: is tricuspid atresia the correct term to use?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 3-15.
Rao PS. Classification of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 59-79.
Rao PS. Classification of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 41-7.
Rao PS. Terminology: tricuspid atresia or univentricular heart?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 3-6.
Anderson RH, Wilkinson JL, Gerlis LM, et al. Atresia of the right atrioventricular orifice. Br Heart J. 1977 Apr. 39(4):414-28. [QxMD MEDLINE Link].
Bharati S, Lev M. The concept of tricuspid atresia complex as distinct from that of the single ventricle complex. Pediatr Cardiol. 1979. 1:57.
Bharati S, McAllister HA Jr, Tatooles CJ, et al. Anatomic variations in underdeveloped right ventricle related to tricuspid atresia and stenosis. J Thorac Cardiovasc Surg. 1976 Sep. 72(3):383-400. [QxMD MEDLINE Link].
Rao PS. Is the term "tricuspid atresia" appropriate?. Am J Cardiol. 1990 Nov 15. 66(17):1251-4. [QxMD MEDLINE Link].
Wenink AC, Ottenkamp J. Tricuspid atresia. Microscopic findings in relation to "absence" of the atrioventricular connexion. Int J Cardiol. 1987 Jul. 16(1):57-73. [QxMD MEDLINE Link].
Gessner IH. Embryology of atrioventricular valve formation and embryogenesis of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992: 25-40.
Ando M, Santomi G, Takao A. Atresia of tricuspid and mitral orifice: anatomic spectrum and morphogenetic hypothesis. Van Praagh R, Takao A, eds. Etiology and Morphogenesis of Congenital Heart Disease. NY: Futura: Mount Kisco; 1980: 421-87.
Van Mierop LH, Gessner IH. Pathogenetic mechanisms in congenital cardiovascular malformations. Prog Cardiovasc Dis. 1972 Jul-Aug. 15(1):67-85. [QxMD MEDLINE Link].
Wilson AD, Rao PS. Embryology. Kambam J, ed. Cardiac Anesthesia for Infants and Children. St Louis, Mo: Mosby; 1994. 3-9.
Rao PS. Tricuspid atresia. Long WA, ed. Fetal and Neonatal Cardiology. Philadelphia, Pa: WB Saunders; 1990. 525-40.
Keefe JF, Wolk MJ, Levine HJ. Isolated tricuspid valvular stenosis. Am J Cardiol. 1970 Feb. 25(2):252-7. [QxMD MEDLINE Link].
Van Praagh R, Ando M, Dungan WT. Anatomic types of tricuspid atresia: clinical and developmental implications [abstract]. Circulation. 1971. 44:115.
Rao PS, Jue KL, Isabel-Jones J, Ruttenberg HD. Ebstein's malformation of the tricuspid valve with atresia. Differentiation from isolated tricuspid atresia. Am J Cardiol. 1973 Dec. 32(7):1004-9. [QxMD MEDLINE Link].
Scalia D, Russo P, Anderson RH, et al. The surgical anatomy of hearts with no direct communication between the right atrium and the ventricular mass--so-called tricuspid atresia. J Thorac Cardiovasc Surg. 1984 May. 87(5):743-55. [QxMD MEDLINE Link].
Rao PS. Natural history of ventricular septal defects in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 32(7): 261-93.
Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg. 1980 Apr. 29(4):306-11. [QxMD MEDLINE Link].
Weinberg PM. Pathologic anatomy of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Futura: Mount Kisco, NY; 1992. 49-67.
Rao PS. Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. Br Heart J. 1977 Mar. 39(3):276-88. [QxMD MEDLINE Link]. [Full Text].
Rao PS. Physiologically advantageous ventricular septal defects. Pediatr Cardiol. 1983 Jan-Mar. 4(1):59-61. [QxMD MEDLINE Link].
Ottenkamp J, Wenink AC, Quaegebeur JM, et al. Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications. J Thorac Cardiovasc Surg. 1985 Apr. 89(4):597-603. [QxMD MEDLINE Link].
Rao PS, Covitz W, Chopra PS. Principles of palliative management of patients with tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 4(1): 297-320.
Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF. Tricuspid atresia: association with persistent truncus arteriosus. Am Heart J. 1991 Sep. 122(3 Pt 1):829-35. [QxMD MEDLINE Link].
Van Praagh R. Discussion after paper by Vlad P: Pulmonary atresia with intact ventricular septum. Barrett-Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy: Diagnosis and Surgical Treatment. 2nd ed. London: Churchill Livingstone; 1992. 1973:236.
Astley R, Oldham JS, Parsons C. Congenital tricuspid atresia. Br Heart J. 1953 Jul. 15(3):287-97. [QxMD MEDLINE Link]. [Full Text].
Dick M, Fyler DC, Nadas AS. Tricuspid atresia: clinical course in 101 patients. Am J Cardiol. 1975 Sep. 36(3):327-37. [QxMD MEDLINE Link].
Edwards JE, Burchell HB. Congenital tricuspid atresia: a classification. Med Clin North Am. 1949. 33:1117.
Keith J, Rowe RD, Vlad P. Heart Disease in Infancy and Childhood. Tricuspid Atresia. 2nd ed. New York: Macmillian; 1967. 434: 664.
Vlad P. Tricuspid atresia. Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Childhood. 3rd ed. New York: Macmillian; 1977. 518-41.
Rao PS. A unified classification for tricuspid atresia. Am Heart J. 1980 Jun. 99(6):799-804. [QxMD MEDLINE Link].
Rudolph AM. Tricuspid atresia with hypoplastic right ventricle. Congenital Disease of the Heart. Chicago, Ill: Year Book Medical; 1974. 429-61.
Rao PS. Perinatal circulatory physiology. Indian J Pediatr. 1991 Jul-Aug. 58(4):441-51. [QxMD MEDLINE Link].
Rao PS. Tricuspid atresia. Moller JH, Hoffman JIE, eds. Pediatric Cardiovascular Medicine. 2nd ed. Oxford, UK: Wiley-Blackwell; 2012. 487-508.
Dick M, Rosenthal A. The clinical profile of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1982. 83-111.
Rosenthal A, Dick M, II. Tricuspid atresia. Adams FH, Emmanouilides GC, eds. Moss' Heart Disease in Infants, Children, and Adolescents. 3rd ed. Baltimore, Md: Lippincott Williams & Wilkins; 1983. 271.
La Corte MA, Dick M, Scheer G, La Farge CG, Fyler DC. Left ventricular function in tricuspid atresia. Angiographic analysis in 28 patients. Circulation. 1975 Dec. 52(6):996-1000. [QxMD MEDLINE Link].
Graham TP Jr, Erath HG Jr, Boucek RJ Jr, Boerth RC. Left ventricular function in cyanotic congenital heart disease. Am J Cardiol. 1980 Jun. 45(6):1231-6. [QxMD MEDLINE Link].
Rao PS, Alpert BS, Covitz W. Left ventricular function in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 52(6): 247-59.
Tzifa A, Gauvreau K, Geggel RL. Factors associated with development of atrial septal restriction in patients with tricuspid atresia involving the right-sided atrioventricular valve. Am Heart J. 2007 Dec. 154(6):1235-41. [QxMD MEDLINE Link].
Rao PS, Sissman NJ. Spontaneous closure of physiologically advantageous ventricular septal defects. Circulation. 1971 Jan. 43(1):83-90. [QxMD MEDLINE Link].
Rao PS, Linde LM, Liebman J, Perrin E. Functional closure of physiologically advantageous ventricular septal defects. Observations in three cases with tricuspid atresia. Am J Dis Child. 1974 Jan. 127(1):36-40. [QxMD MEDLINE Link].
Gallaher ME, Fyler DC. Observations on changing hemodynamics in tricuspid atresia without associated transposition of the great vessels. Circulation. 1967 Feb. 35(2):381-8. [QxMD MEDLINE Link].
Rao PS. Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: surgical implications. Ann Thorac Surg. 1983 Feb. 35(2):121-31. [QxMD MEDLINE Link].
Sauer U, Hall D. Spontaneous closure or critical decrease in size of the ventricular septal defect in tricuspid atresia with normally connected great arteries: surgical implications. 1980. 5:369.
Bloomfield DK. The natural history of ventricular septal defect in patients surviving infancy. Circulation. 1964 Jun. 29:914-55. [QxMD MEDLINE Link].
Hoffman JI, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965 Nov. 16(5):634-53. [QxMD MEDLINE Link].
Rao PS. Demographic features of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 23-37.
Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circulation. 1971 Mar. 43(3):323-32. [QxMD MEDLINE Link].
Schrire V. Experience with congenital heart disease at Groote Schuur Hospital, Cape Town. An analysis of 1,439 patients over an eleven-year period. S Afr Med J. 1963 Nov 23. 37:1175-80. [QxMD MEDLINE Link].
Rao PS. Management of neonate with suspected serious heart disease. King Faisal Spec Hosp Med J. 1984. (4):209.
Marcelletti CF, Hanley FL, Mavroudis C, et al. Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience. J Thorac Cardiovasc Surg. 2000 Feb. 119(2):340-6. [QxMD MEDLINE Link].
Marcano BA, Riemenschneider TA, Ruttenberg HD, Goldberg SJ, Gyepes M. Tricuspid atresia with increased pulmonary blood flow. An analysis of 13 cases. Circulation. 1969 Sep. 40(3):399-410. [QxMD MEDLINE Link].
Rao PS. Pathophysiologic consequences of cyanotic congenital heart disease. Indian J Pediatr. Sep-Oct 1983. 50(406):479-87.
Covitz W, Rao PS. Non-invasive evaluation of patients with tricuspid atresia (roentgenography, echocardiography and nuclear angiography). Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992: 165-82.
Rao PS. Tricuspid atresia. Vijayalakshmi IB, Rao PS, Chugh R, eds. A Comprehensive Approach to Congenital Heart Diseases. New Delhi, India: Jaypee Brothers Medical Publishers; 2013. 397-413.
Rao PS. Tricuspid atresia. Rao PS, Vidyasagar D, eds. Perinatal Cardiology: A Multidisciplinary Approach: A Multidisciplinary Approach. Minneapolis, Mn: Cardiotext Publishing; 2015. Chapter 30.
Gamboa R, Gersony WM, Nadas AS. The electrocardiogram in tricuspid atresia and pulmonary atresia with intact ventricular septum. Circulation. 1966 Jul. 34(1):24-37. [QxMD MEDLINE Link].
Rao PS, Kulungara RJ, Boineau JP. Electrovectorcardiographic features of tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 141-64.
Rao PS. Cardiac catheterization in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 153-78.
Rao PS. Cardiac catheterization in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, New York: Futura; 1992: 193-221.
Rao PS. The femoral route for cardiac catheterization of infants and children. Chest. 1973 Feb. 63(2):239-41. [QxMD MEDLINE Link].
Rao PS. Left to right atrial shunting in tricuspid atresia. Br Heart J. 1983 Apr. 49(4):345-9. [QxMD MEDLINE Link].
Rao PS, Sissman NJ. The relationship of pulmonary venous wedge to pulmonary arterial pressures. Circulation. 1971 Oct. 44(4):565-74. [QxMD MEDLINE Link].
Mair DD, Hagler DJ, Puga FJ, Schaff HV, Danielson GK. Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation. 1990 Nov. 82(5 Suppl):IV164-9. [QxMD MEDLINE Link].
Schwartz DC, Rao PS. Angiography in tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992. 1(3): 223-46.
Rao PS. Value of pulmonary vein wedge angiography in visualization of obstructed ipsilateral pulmonary artery. Cardiovasc Radiol. 1978 Jul 25. 1(3):151-2. [QxMD MEDLINE Link].
Rao PS. Principles of management of the neonate with congenital heart disease neonatology Today. 2007. 2(8):1-10.
Freed MD, Heymann MA, Lewis AB, Roehl SL, Kensey RC. Prostaglandin E1 infants with ductus arteriosus-dependent congenital heart disease. Circulation. 1981 Nov. 64(5):899-905. [QxMD MEDLINE Link].
Rao PS. Transcatheter blade atrial septostomy. Cathet Cardiovasc Diagn. 1984. 10(4):335-42. [QxMD MEDLINE Link].
Rao PS. Role of interventional cardiology in neonates: Part I. Non-surgical atrial septostomy. Congenital Cardiol Today. 2007 Dec. 5(12):1-12.
Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002 Sep 24. 106(12 Suppl 1):I82-9. [QxMD MEDLINE Link].
Yates MC, Rao PS. Pediatric cardiac emergencies. Emerg Med. 2013. 3(164):doi:10.4172/2165-7548.1000164. [Full Text].
Tsounias E, Rao PS. Stent therapy for clotted Blalock-Taussig shunts. Congenital Cardiol Today. 2010. 8(7):1-9.
Lichtman SW, Caravano M, Schneyman M, Howell B, King ML. Successful outpatient cardiac rehabilitation in an adult patient post-surgical repair for tricuspid valve atresia and hypoplastic right ventricle: a case study. J Cardiopulm Rehabil Prev. 2008 Jan-Feb. 28(1):48-51. [QxMD MEDLINE Link].
Strong WB, Morera JA, Rao PS. Sexuality, contraception and pregancy in patients with cyanotic congenital heart disease with special reference to tricuspid atresia. Rao PS, ed. Tricuspid Atresia. 2nd ed. NY: Futura: Mount Kisco; 1992. 16(1): 415-27.
Rashkind W, Waldhausen J, Miller W, Friedman S. Palliative treatment in tricuspid atresia. Combined balloon atrioseptostomy and surgical alteration of pulmonary blood flow. J Thorac Cardiovasc Surg. 1969 Jun. 57(6):812-8. [QxMD MEDLINE Link].
McCredie RM, Swinburn MJ, Lee CL, Warner G. Balloon dilatation pulmonary valvuloplasty in pulmonary stenosis. Aust N Z J Med. 1986 Feb. 16(1):20-3. [QxMD MEDLINE Link].
Rao PS, Levy JM, Chopra PS. Balloon angioplasty of stenosed Blalock-Taussig anastomosis: role of balloon-on-a-wire in dilating occluded shunts. Am Heart J. 1990 Nov. 120(5):1173-8. [QxMD MEDLINE Link].
Rao PS. Current status of balloon angioplasty for neonatal and infant aortic coarctation. Progress Pediat Cardiol. 2001. 14:35-44.
Rao PS, Jureidini SB, Balfour IC, Singh GK, Chen S. Severe aortic coarctation in infants less than 3 months: Successful palliation by balloon angioplasty. J Invasive Cardiol. 2003. 15:203-208.
Rao PS, Balfour IC, Singh GK, Jureidini SB, Chen S. Bridge stents in the management of obstructive vascular lesions in children. Am J Cardiol. 2001. 88:699-702.
Rao PS. Stents in the management of congenital heart disease in pediatric and adult patients. Indian Heart J. 2001 Nov-Dec. 53(6):714-30. [QxMD MEDLINE Link].
Siblini G, Rao PS. Coil Embolization in the Management of Cardiac Problems in Children. J Invasive Cardiol. 1996. 8:332-40.
Rao PS. Transcatheter embolization of unwanted blood vessels in children. Rao PS, Kern MJ, eds. Catheter Based Devices for Treatment of Noncoronary Cardiovascular Disease in Adults and Children. Philadelphia, PA: Lippincott, Williams & Wilkins; 2003. 251(16): 2123-38.
Rao PS, Chandar JS, Sideris EB. Role of inverted buttoned device in transcatheter occlusion of atrial septal defects or patent foramen ovale with right-to-left shunting associated with previously operated complex congenital cardiac anomalies. Am J Cardiol. 1997 Oct 1. 80(7):914-21. [QxMD MEDLINE Link].
Goff DA, Blume ED, Gauvreau K, Mayer JE, Lock JE, Jenkins KJ. Clinical outcome of fenestrated Fontan patients after closure: the first 10 years. Circulation. 2000 Oct 24. 102(17):2094-9. [QxMD MEDLINE Link].
Boudjemline Y, Bonnet D, Sidi D, Agnoletti G. [Closure of extrocardiac Fontan fenestration by using the Amplatzer duct occluder]. Arch Mal Coeur Vaiss. 2005 May. 98(5):449-54. [QxMD MEDLINE Link].
Rothman A, Evans WN, Mayman GA. Percutaneous fenestration closure with problematic residual native atrial septum. Catheter Cardiovasc Interv. 2005 Oct. 66(2):286-90. [QxMD MEDLINE Link].
Sugiyama H, Yoo SJ, Williams W, Benson LN. Characterization and treatment of systemic venous to pulmonary venous collaterals seen after the Fontan operation. Cardiol Young. 2003 Oct. 13(5):424-30. [QxMD MEDLINE Link].
Tsounias E, Rao PS. Versatility of Amplatzer Vascular Plug in occlusion of different types of vascular channels. Catheter Cardiovasc Interv. 2008. 71:63.
Alsoufi B, Schlosser B, Mori M, et al. Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 2015 Oct. 100 (4):1403-9; discussion 1409-10. [QxMD MEDLINE Link].
Blalock A, Taussig HB. Landmark article May 19, 1945: The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. By Alfred Blalock and Helen B. Taussig. JAMA. 1984 Apr 27. 251(16):2123-38. [QxMD MEDLINE Link].
de Leval MR, McKay R, Jones M, Stark J, Macartney FJ. Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts. J Thorac Cardiovasc Surg. 1981 Jan. 81(1):112-9. [QxMD MEDLINE Link].
Annecchino FP, Fontan F, Chauve A, Quaegebeur J. Palliative reconstruction of the right ventricular outflow tract in tricuspid atresia: a report of 5 patients. Ann Thorac Surg. 1980 Apr. 29(4):317-21. [QxMD MEDLINE Link].
Gibbs JL, Rothman MT, Rees MR, Parsons JM, Blackburn ME, Ruiz CE. Stenting of the arterial duct: a new approach to palliation for pulmonary atresia. Br Heart J. 1992 Mar. 67(3):240-5. [QxMD MEDLINE Link]. [Full Text].
Alwi M, Choo KK, Latiff HA, Kandavello G, Samion H, Mulyadi MD. Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation. J Am Coll Cardiol. 2004 Jul 21. 44(2):438-45. [QxMD MEDLINE Link].
Rao PS. Subaortic obstruction after pulmonary artery banding in patients with tricuspid atresia and double-inlet left ventricle and ventriculoarterial discordance. J Am Coll Cardiol. 1991 Nov 15. 18(6):1585-6. [QxMD MEDLINE Link].
Bonnet D, Sidi D, Vouhe PR. Absorbable pulmonary artery banding in tricuspid atresia. Ann Thorac Surg. 2001 Jan. 71(1):360-1; discussion 361-2. [QxMD MEDLINE Link].
Rao PS. Absorbable pulmonary artery band in tricuspid atresia (Editorial). Ann Thorac Surg. 2001. 71:361-362.
Park SC, Neches WH, Zuberbuhler JR, et al. Clinical use of blade atrial septostomy. Circulation. 1978 Oct. 58(4):600-6. [QxMD MEDLINE Link].
Seliem M, Muster AJ, Paul MH, Benson DW Jr. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coll Cardiol. 1989 Sep. 14(3):750-5. [QxMD MEDLINE Link].
Smolinsky A, Castaneda AR, Van Praagh R. Infundibular septal resection: surgical anatomy of the superior approach. J Thorac Cardiovasc Surg. 1988 Mar. 95(3):486-94. [QxMD MEDLINE Link].
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May. 26(3):240-8. [QxMD MEDLINE Link]. [Full Text].
Kreutzer G, Bono H, Galindez E. Una operacion para la correccion de la atresia tricuspidea. Ninth Argent Congress of Cardiology; Buenos Aires, Argentina. October 31-November 6, 1971.
Glenn WW. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application. N Engl J Med. 1958 Jul 17. 259(3):117-20. [QxMD MEDLINE Link].
Chopra PS, Rao PS. Corrective surgery for tricuspid atresia: which modification of Fontan-Kreutzer procedure should be used? A review. Am Heart J. 1992 Mar. 123(3):758-67. [QxMD MEDLINE Link].
Rao PS, Chopra PS. Modifications of Fontan-Kreutzer procedure for tricuspid atresia: can a choice be made?. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, New York: Futura; 1992. 34(2): 361-75.
Haller JA Jr, Adkins JC, Worthington M, Rauenhorst J. Experimental studies on permanent bypass of the right heart. Surgery. 1966 Jun. 59(6):1128-32. [QxMD MEDLINE Link].
Azzolina G, Eufrate S, Pensa P. Tricuspid atresia: experience in surgical management with a modified cavopulmonary anastomosis. Thorax. 1972 Jan. 27(1):111-5. [QxMD MEDLINE Link]. [Full Text].
Choussat A, Fontan F, Besse P, et al. Selection criteria for Fontan procedure. Anderson RH, Shinebourne EA, eds. Pediatric Cardiology. White Plains, NY: Churchill Livingstone; 1978. 559.
Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1989 May. 97(5):746-54. [QxMD MEDLINE Link].
Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990 Nov. 82(5):1681-9. [QxMD MEDLINE Link].
Laks H, Pearl JM, Haas GS, et al. Partial Fontan: advantages of an adjustable interatrial communication. Ann Thorac Surg. 1991 Nov. 52(5):1084-94; discussion 1094-5. [QxMD MEDLINE Link].
Thompson LD, Petrossian E, McElhinney DB, et al. Is it necessary to routinely fenestrate an extracardiac Fontan?. J Am Coll Cardiol. 1999 Aug. 34(2):539-44. [QxMD MEDLINE Link].
de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988 Nov. 96(5):682-95. [QxMD MEDLINE Link].
Sharma S, Goudy S, Walker P, et al. In vitro flow experiments for determination of optimal geometry of total cavopulmonary connection for surgical repair of children with functional single ventricle. J Am Coll Cardiol. 1996 Apr. 27(5):1264-9. [QxMD MEDLINE Link].
Kumar SP, Rubinstein CS, Simsic JM, Taylor AB, Saul JP, Bradley SM. Lateral tunnel versus extracardiac conduit Fontan procedure: a concurrent comparison. Ann Thorac Surg. 2003 Nov. 76(5):1389-96; discussion 1396-7. [QxMD MEDLINE Link].
Ro PS, Rychik J, Cohen MS, Mahle WT, Rome JJ. Diagnostic assessment before Fontan operation in patients with bidirectional cavopulmonary anastomosis: are noninvasive methods sufficient?. J Am Coll Cardiol. 2004 Jul 7. 44(1):184-7. [QxMD MEDLINE Link].
Adachi I, Yagihara T, Kagisaki K, et al. Fontan operation with a viable and growing conduit using pedicled autologous pericardial roll: serial changes in conduit geometry. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1517-22. [QxMD MEDLINE Link].
Yalcinbas YK, Erek E, Salihoglu E, Sarioglu A, Sarioglu T. Early results of extracardiac fontan procedure with autologous pericardial tube conduit. Thorac Cardiovasc Surg. 2005 Feb. 53(1):37-40. [QxMD MEDLINE Link].
Lemler MS, Ramaciotti C, Stromberg D, Scott WA, Leonard SR. The extracardiac lateral tunnel Fontan, constructed with bovine pericardium: comparison with the extracardiac conduit Fontan. Am Heart J. 2006 Apr. 151(4):928-33. [QxMD MEDLINE Link].
Sreeram N, Alvarado O, Peart I. Tricuspid atresia with common arterial trunk: surgical palliation in a neonate. Int J Cardiol. 1991 Aug. 32(2):251-3. [QxMD MEDLINE Link].
Gonzalez-Lopez MT, Crucean A, Seale A, McGuirk S. Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart. Interact Cardiovasc Thorac Surg. 2015 Aug. 21(2):252-3. [QxMD MEDLINE Link].
Milovanovic V, Stefanovic I, Ilic S. Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas. Cardiol Young. 2016 Sep 29. 1-4. [QxMD MEDLINE Link].
Futuki A, Fujiwara K, Yoshizawa K, Sakazaki H. Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful Fontan completion. World J Pediatr Congenit Heart Surg. 2016 Aug 22 [Epub ahead of print]. [QxMD MEDLINE Link].
Yokoyama S, Kaneda K, Nagasaka S, et al. Tricuspid atresia IIc with a vascular ring: novel approach for Fontan completion. Ann Thorac Surg. 2016 Mar. 101(3):1188-90. [QxMD MEDLINE Link].
McMahon CJ, Nolke L. Successful palliation of a child with left ventricular noncompaction cardiomyopathy and tricuspid atresia to Fontan procedure. Ann Thorac Surg. 2014 Aug. 98(2):719-21. [QxMD MEDLINE Link].
Shimada M, Sakamoto T, Umezu K, Harada Y. Successful staged Fontan completion for a tricuspid atresia patient with left ventricular non-compaction. Interact Cardiovasc Thorac Surg. 2016 Mar. 22(3):387-9. [QxMD MEDLINE Link].
Konertz W, Schneider M, Herwig V, Kampmann C, Waldenberger F, Hausdorf G. Modified hemi-Fontan operation and subsequent nonsurgical Fontan completion. J Thorac Cardiovasc Surg. 1995 Sep. 110(3):865-7. [QxMD MEDLINE Link].
Hausdorf G, Schneider M, Konertz W. Surgical preconditioning and completion of total cavopulmonary connection by interventional cardiac catheterisation: a new concept. Heart. 1996 Apr. 75(4):403-9. [QxMD MEDLINE Link]. [Full Text].
Sidiropoulos A, Ritter J, Schneider M, Konertz W. Fontan modification for subsequent non-surgical Fontan completion. Eur J Cardiothorac Surg. 1998 May. 13(5):509-12; discussion 512-3. [QxMD MEDLINE Link].
Galantowicz M, Cheatham JP. Fontan completion without surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004. 7:48-55. [QxMD MEDLINE Link].
van den Bosch AE, Roos-Hesselink JW, Van Domburg R, Bogers AJ, Simoons ML, Meijboom FJ. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol. 2004 May 1. 93(9):1141-5. [QxMD MEDLINE Link].
Freedom RM, et al. The Fontan procedure for patients with tricuspid atresia: long-term follow-up. Rao PS, ed. Tricuspid Atresia. 2nd ed. Mount Kisco, NY: Futura; 1992. 128(1): 377.
Stamm C, Friehs I, Mayer JE Jr, et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001 Jan. 121(1):28-41. [QxMD MEDLINE Link].
Lubiszewska B, Rozanski J, Demkow M, et al. Long-term results of Fontan procedure in 43 patients. Kardiol Pol. 2003 Mar. 58(3):207-16. [QxMD MEDLINE Link].
Alphonso N, Baghai M, Sundar P, Tulloh R, Austin C, Anderson D. Intermediate-term outcome following the Fontan operation: a survival, functional and risk-factor analysis. Eur J Cardiothorac Surg. 2005 Oct. 28(4):529-35. [QxMD MEDLINE Link].
Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S, Spray TL. Intermediate outcomes after the Fontan procedure in the current era. J Thorac Cardiovasc Surg. 2006 Jan. 131(1):172-80. [QxMD MEDLINE Link].
McCrindle BW, Williams RV, Mitchell PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation. 2006 Feb 28. 113(8):1123-9. [QxMD MEDLINE Link].
Sheikh AM, Tang AT, Roman K, et al. The failing Fontan circulation: successful conversion of atriopulmonary connections. J Thorac Cardiovasc Surg. 2004 Jul. 128(1):60-6. [QxMD MEDLINE Link].
Hill DJ, Feldt RH, Porter C. Protein losing enteropathy after Fontan operation: a preliminary report. Circulation. 1989. 80(Suppl II):490.
Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998 May. 115(5):1063-73. [QxMD MEDLINE Link].
Rao PS. Protein-losing enteropathy following the Fontan operation. J Invasive Cardiol. 2007 Oct. 19(10):447-8. [QxMD MEDLINE Link].
Feldt RH, Driscoll DJ, Offord KP, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996 Sep. 112(3):672-80. [QxMD MEDLINE Link].
Giannico S, Hammad F, Amodeo A, et al. Clinical outcome of 193 extracardiac Fontan patients: the first 15 years. J Am Coll Cardiol. 2006 May 16. 47(10):2065-73. [QxMD MEDLINE Link].
Chiu NT, Lee BF, Hwang SJ, Chang JM, Liu GC, Yu HS. Protein-losing enteropathy: diagnosis with (99m)Tc-labeled human serum albumin scintigraphy. Radiology. 2001 Apr. 219(1):86-90. [QxMD MEDLINE Link].
Masetti P, Marianeschi SM, Cipriani A, Iorio FS, Marcelletti CF. Reversal of protein-losing enteropathy after ligation of systemic-pulmonary shunt. Ann Thorac Surg. 1999 Jan. 67(1):235-6. [QxMD MEDLINE Link].
Zellers TM, Brown K. Protein-losing enteropathy after the modified Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol. 1996 Mar-Apr. 17(2):115-7. [QxMD MEDLINE Link].
Therrien J, Webb GD, Gatzoulis MA. Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?. Heart. 1999 Aug. 82(2):241-3. [QxMD MEDLINE Link]. [Full Text].
Guariso G, Cerutti A, Moreolo GS, Milanesi O. Protein-losing enteropathy after Fontan operation: treatment with elementary diet in one case. Pediatr Cardiol. 2000 May-Jun. 21 (3):292. [QxMD MEDLINE Link].
Kim SJ, Park IS, Song JY, Lee JY, Shim WS. Reversal of protein-losing enteropathy with calcium replacement in a patient after Fontan operation. Ann Thorac Surg. 2004 Apr. 77(4):1456-7. [QxMD MEDLINE Link].
Donnelly JP, Rosenthal A, Castle VP, Holmes RD. Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr. 1997 Mar. 130(3):474-8. [QxMD MEDLINE Link].
Kelly AM, Feldt RH, Driscoll DJ, Danielson GK. Use of heparin in the treatment of protein-losing enteropathy after Fontan operation for complex congenital heart disease. Mayo Clin Proc. 1998 Aug. 73(8):777-9. [QxMD MEDLINE Link].
Facchini M, Guldenschuh I, Turina J, Jenni R, Schalcher C, Attenhofer Jost CH. Resolution of protein-losing enteropathy with standard high molecular heparin and urokinase after Fontan repair in a patient with tricuspid atresia. J Cardiovasc Surg (Torino). 2000 Aug. 41(4):567-70. [QxMD MEDLINE Link].
Ringel RE, Peddy SB. Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol. 2003 Apr 15. 91(8):1031-2, A9. [QxMD MEDLINE Link].
Uzun O, Wong JK, Bhole V, Stumper O. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg. 2006 Dec. 82(6):e39-40. [QxMD MEDLINE Link].
Connor FL, Angelides S, Gibson M, et al. Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy. Pediatrics. 2003 Sep. 112(3 Pt 1):e242-7. [QxMD MEDLINE Link].
Mertens L, Dumoulin M, Gewillig M. Effect of percutaneous fenestration of the atrial septum on protein-losing enteropathy after the Fontan operation. Br Heart J. 1994 Dec. 72(6):591-2. [QxMD MEDLINE Link]. [Full Text].
Lemes V, Murphy AM, Osterman FA, Laschinger JC, Kan JS. Fenestration of extracardiac Fontan and reversal of protein-losing enteropathy: case report. Pediatr Cardiol. 1998 Jul-Aug. 19(4):355-7. [QxMD MEDLINE Link].
Fraisse A, Bonnet JL. Protein-losing enteropathy: radiofrequency fenestration of the atrial septum after failure of transseptal needle puncture. Pediatr Cardiol. 2004 Jan-Feb. 25(1):84-6. [QxMD MEDLINE Link].
Kreutzer J, Keane JF, Lock JE, et al. Conversion of modified Fontan procedure to lateral tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 1997. 1169-117.
Brancaccio G, Carotti A, D'Argenio P, Michielon G, Parisi F. Protein-losing enteropathy after Fontan surgery: resolution after cardiac transplantation. J Heart Lung Transplant. 2003 Apr. 22(4):484-6. [QxMD MEDLINE Link].
Estner HL, Kolb C, Schmitt C, et al. Long-term transvenous AV-sequential pacing in a failing atriopulmonary Fontan patient. Int J Cardiol. 2008 Jul 4. 127(2):e93-5. [QxMD MEDLINE Link].
Lopez JA. Transvenous right atrial and left ventricular pacing after the Fontan operation: long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization. Texas Heart Institut. 2007. 34(1):96-101.
Gamba A, Merlo M, Fiocchi R, et al. Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg. 2004 Feb. 127(2):555-62. [QxMD MEDLINE Link].
Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004 Nov 16. 44(10):2065-72. [QxMD MEDLINE Link].
Petko M, Myung RJ, Wernovsky G, et al. Surgical reinterventions following the Fontan procedure. Eur J Cardiothorac Surg. 2003 Aug. 24(2):255-9. [QxMD MEDLINE Link].

Media Gallery

Cardiac specimen from a patient with the muscular type of tricuspid atresia. The right atrium was opened by cutting through the right atrial appendage (RAA). Note the dimple (arrow) in the floor of the right atrium with muscle fibers radiating around it. An atrial septal defect (ASD) is also shown. From Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF. Tricuspid atresia: association with persistent truncus arteriosus. Am Heart J 1991, 122:829, with permission.
Systemic arterial saturations. Left ventricular (LV) and aortic (Ao) values are plotted against the pulmonary-to-systemic blood-flow ratio (Qp:Qs). Both type I and type II anatomy are included. Note the curvilinear relationship between the parameters. At low Qp:Qs levels, a slight increase in Qp:Qs produces large increase in systemic oxygen saturation; at high Qp:Qs levels, a further increase does not produce a notable increase in oxygen saturation. The ideal Qp:Qs appears to be 1.5-2.5, which results in oxygen saturations in the low 80s. From Rao PS. Cardiac catheterization in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co: 1982:153, with permission.
Geographic prevalence of tricuspid atresia by continent. Note that prevalences are similar on all continents except for Australia. This lone exception is thought to be related to the small sample size from Australia. CHD = congenital heart defects; TA = tricuspid atresia. From Rao PS. Demographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:23, with permission.
Actuarial survival curves from 3 reported clinical series compiled by Dick and Rosenthal (1992) show a high initial mortality rate in the first year of life, a plateau between the first year and middle of the second decade of life, and a second increase in the mortality rate after the middle of the second decade of life; this second rise is presumably related to impaired left ventricular function. From Dick M and Rosenthal A. The clinical profile of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. Mt Kisco, NY: Futura Publishing Co; 1982:83, with permission.
Actuarial survival rate of 100 patients who had tricuspid atresia and who underwent a Fontan operation at the Hospital for Sick Children, Toronto, in 1975-1989. The survival rate 5 years after the operation is 70%. From Freedom RM, et al. The Fontan procedure for patients with tricuspid atresia: long-term follow-up. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:377, with permission.
Use of electrocardiographic mean QRS vector (axis) in the frontal plane for the differential diagnosis of a cyanotic newborn with decreased pulmonary blood flow. Associated ventricular hypertrophy patterns (as marked in each quadrant) and decreased right ventricular (RV) forces are also helpful. From Rao PS. Management of neonate with suspected serious heart disease. King Faisal Spec Hosp Med J 1984 (4):209, with permission.
Frontal plane mean QRS vector in 308 patients, plotted by anatomic type. Most patients with tricuspid atresia type I (normally related great arteries) have an abnormally superior vector, also called left axis deviation. Only one half of patients with tricuspid atresia type II have an abnormally superior vector. Most patients with tricuspid atresia type III (subtype A) have an inferiorly oriented frontal plane vector. From Rao PS, Kulungara RJ, Boineau JP. Electrovectorcardiographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:141, with permission.
Subcostal 4-chamber 2-dimensional echocardiographic view of a neonate with tricuspid atresia shows an enlarged left ventricle (LV), a small right ventricle (RV), and a dense band of echoes where the tricuspid valve echo should be. Atrial and ventricular septal defects and the mitral valve are also seen. Note the attachment of the anterior leaflet of the detectable atrioventricular valve to the left side of the interatrial septum. Reprinted from Rao, Fetal and Neonatal Cardiology, 1990, with permission from Elsevier Science.
Selected cineangiographic frames from superior vena caval (SVC) and right atrial (RA) frontal angiography in 2 patients in the frontal view. Note sequential opacification of the left atrium (LA) and left ventricle (LV) without opacification of the right ventricle. The RA on the right, the LA superiorly, and the LV on the left form a nonopacified right ventricular window (arrow). This is a classic appearance of the muscular variety of tricuspid atresia. From Rao PS. Tricuspid atresia: anatomy, imaging, and natural history. In: Brawnwald E, Freedom RM, eds. Atlas of Heart Disease: Congenital Heart Disease. Vol 12. Philadelphia, PA: Current Medicine; 1997:14.1 with permission.
Selective superior vena caval (SVC) injection for a 4-chamber projection (hepatoclavicular) shows tricuspid atresia and filling of the left atrium (LA) through a somewhat restrictive atrial septal defect (arrows). Note the retrograde filling of the coronary sinus (CS). RA = right atrium. From Schwartz DC, Rao PS. Angiography in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:223, with permission.