Update on the modified Fontan procedure

Current Opinion in Cardiology

Volumn 12, Issue 1, 1997, Pages 51-62

  Geggel, Robert L ^a  

^a TUFTS MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ARTERIOVENOUS MALFORMATION; CYANOSIS; EFFUSION; EXERCISE; FONTAN PROCEDURE; HEART ARRHYTHMIA; HEART VENTRICLE FUNCTION; HUMAN; MORBIDITY; PALLIATIVE THERAPY; PRIORITY JOURNAL; PROTEIN LOSING GASTROENTEROPATHY; REVIEW; SHUNTING; SURGICAL TECHNIQUE; THROMBOEMBOLISM; TRICUSPID VALVE ATRESIA;

EID: 0031044303     PISSN: 02684705     EISSN: None     Source Type: Journal    
DOI: 10.1097/00001573-199701000-00009     Document Type: Review

References (80)

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14. van Son JAM, Reddy M, Hanley FL: Extracardiac modification of the • Fontan operation without use of prosthetic material. J Thorac Cardiovasc Surg 1995, 110:1766-1768. Modification applicable for patients with L-malposition or L-transposition of the aorta (or the situs inverses equivalent) if and adequate-sized main pulmonary artery is present.
15. Laks H, Ardehali A, Grant PW, Permut L, Aharon A, Kuhn M, Isabel-Jones • J, Galindo A: Modification of the Fontan procedure- superior vena cava to left pulmonary artery connection and inferior vena cava to right pulmonary artery connection with adjustable atrial septal defect. Circulation 1995, 91:2943-2947. Report of results of this procedure in 18 patients considered by the authors to be especially applicable to the high-risk patient.
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17. Serraf A, Conte S, Lacour-Gayet F, Bruniaux J, Sousa-Uva M, Roussin R, •• Planché C: Systemic obstruction in univentricular hearts: surgical options for neonates. Ann Thorac Surg 1995, 60:970-977. Excellent presentation of surgical options available for this challenging group of patients.
18. Bu'Lock FA, Stümper O, Jagtap R, Silove ED, de Giovanni JV, Wright •• JGC, Sethia B, Brawn WJ: Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure. Br Heart J 1995, 73:456-461. Description of new surgical technique in 17 patients.
19. van Son JAM: Importance of early relief of aortic outflow obstruction in univentricular hearts. Ann Thorac Surg 1995, 60:492-493.
20. Donofrio MT, Jacobs ML, Norwood WI, Rychik J: Early changes in •• ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery. J Am Coll Cardiol 1995, 26:1008-1015. Preoperative and postoperative echocardiograms were reviewed after pulmonary artery banding and cavopulmonary connection. The latter was associated with greater reduction in ventricular septal defect area, which contributes to early development of subaortic stenosis.
21. Cohen DM, Wheller JJ, Davis JT, Allen HD: Obstruction of the systemic • venous pathway after closure of an adjustable atrial septal defect in the modified Fontan operation. Am Heart J 1995, 130:617-618. Case report describing obstruction in a lateral tunnel after closure of an adjustable atrial septal defect. The obstruction was relieved by balloon dilation.
22. Jahnke AW, Kraatz EG, Blömer F, Paul J, Pfister G, Sievers HH: A new • experimental model and formula for flow determination and possible application in sizing of interatrial communication. J Cardiovasc Surg 1995, 36:313-318. In vitro flow studies to evaluate flow through an interatrial communication.
23. Rao IM, Swanson JS, Hovaguimian H, McIrvin DM, King DH, Furnary AP, Livermore J, Starr A: Intrahepatic steal after Fontan operation with partial hepatic exclusion. J Thorac Cardiovasc Surg 1995, 109:180-181.
24. Reed MK, Leonard SR, Zellers TM, Nikaidoh H: Major intrahepatic • venovenous fistulas after a modified Fontan operation. Ann Thorac Surg 1996, 61:713-715. Case report describing progressive cyanosis caused by shunting from the inferior vena cava to the hepatic veins in a patient with partial exclusion of the hepatic veins. This was treated by realigning the inferior portion of the lateral tunnel patch to include all the hepatic veins.
25. Waterbolk TW, Talsma MD, Loef BG, Slooff MJH, Ebels T: Increasing cyanosis after total cavopulmonary connection treated by banding a separate liver vein. Ann Thorac Surg 1995, 59:1226-1228.
26. Robotin MC, Edis BD, Weintraub RG, Pawade A, Karl TR: Heart transplantation for chyloptysis after Fontan operation. Ann Thorac Surg 1995, 59:1570-1571.
27. Cecchin F, Johnsrude CL, Perry JC, Friedman RA: Effect of age and •• surgical technique on symptomatic arrhythmias after the Fontan procedure. Am J Cardiol 1995, 76:386-391. Retrospective review of 151 patients operated at Texas Children's Hospital between 1987 to 1993.
28. Gelatt M, Hamilton RM, McCrindle BW, Gow RM, Williams WG, Trusler GA, Freedom RM: Risk factors for atrial tachyarrhythmias after the Fontan operation. J Am Coll Cardiol 1994, 24:1735-1741.
29. Kavey REW, Gaum WE, Byrum CJ, Smith FC, Kveselis DA: Loss of sinus •• rhythm after total cavopulmonary connection. Circulation 1995, 92(suppl II):II-304-II-308. Retrospective review of 36 patients operated at the State University of New York Helath Science Center. Syracuse between 1982 and 1994. Patients with total cavopulmonary connection had a lower incidence of sinus rhythm (40%) than patients with atriopulmonary connection (76%) 2 years after surgery.
30. Manning PB, Mayer JE Jr, Wernovsky G, Fishberger SB, Walsh EP: •• Staged operation to Fontan increases the incidence of sinoatrial node dysfunction. J Thorac Cardiovasc Surg 1996, 111:833-840. Retrospective review of 227 patients operated at Children's Hospital, Boston between 1988 and 1992. Patients having a preceding cavopulmonary shunt had a higher incidence of early and late sinoatrial node dysfunction.
31. Drant SE, Klitzner TS, Shannon KM, Wetzel GT. Williams RG: Guidance • of radiofrequency catheter ablation by transesophageal echocardiography in children with palliated single ventricle. Am J Cardiol 1995, 76:1311-1312. Describes the utility of transesophageal echocardiography in performing radiofrequency ablation in six patients with single ventricle and atrial tachyarrhythmias.
32. Jonas RA: Advances in surgical care of infants and children with • congenital heart disease. Curr Opin Pediatr 1995, 7:572-579. Annual review including results and complications after bidirectional Glenn and Fontan procedures.
33. Rosenthal E, Qureshi SA, Crick JCP: Successful long-term ventricular pacing via the coronary sinus after the Fontan operation. PACE Pacing Clin Electrophysiol 1995, 18:2103-2105.
34. Alejos JC, Williams RG, 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, 75:1048-1050.
35. Reddy VM, Liddicoat JR, Hanley FL: Primary bidirectional superior •• cavopulmonary shunt in infants between 1 and 4 months of age. Ann Thorac Surg 1995, 59:1120-1126. Review of nine infants, aged 5 weeks to 4 months, who had a bidirectional Glenn shunt as initial palliation. The role of this procedure is reviewed as well as potential disadvantages in this age group.
36. Uemura H, Yagihara T, Kawashima Y, Okada K, Kamiya T, Anderson RH: • Use of the bidirectional Glenn procedure in the presence of forward flow from the ventricles to the pulmonary arteries. Circulation 1995, 92(suppl II):II-228-II-232. Presentation of outcome in 27 high-risk patients who had accessory flow maintained at the time of a bidirectional Glenn shunt. There was no control group. The authors contend that pulmonary growth is preserved and arterial oxygenation improved by this technique.
37. Salim MA, DiSessa TG, Arheart KL, Alpert BS: Contribution of superior •• vena caval flow to total cardiac output in children: a Doppler echocardiographic study. Circulation 1995, 92:1860-1865. Measurement of proportion of cardiac output contributed by the superior vena cava in 145 patients ranging in age from neonates to 7 years using two-dimensional and Doppler echocardiography. The authors document a maturational change in superior vena cava contribution.
38. Webber SA, Horvath P, LeBlanc JG, Slavik Z, Lamb RK, Monro JL, Reich •• O, Hruda J, Sandor GGS, Keeton BR, Salmon AP: Influence of competitive pulmonary blood flow on the bidirectional superior cavopulmonary shunt: a multi-institutional study. Circulation 1995, 92(suppl II):II-279-II286. Review of 108 patients operated between 1987 and 1993 at three institutions.
39. Salim MA, Case CL, Sade RM, Watson DC, Alpert BS, DiSessa TG: •• Pulmonary/systemic flow ratio in children after cavopulmonary anastomosis. J Am Coll Cardiol 1995, 25:735-738. Measurement of flow ratio using catheterization data based on the equation: pulmonary-to-systemic flow ratio equals (systemic saturation minus inferior vena cava saturation) divided by (pulmonary venous saturation minus inferior vena cava saturation).
40. Slavik Z, Webber SA, Lamb RK, Horvath P, LeBlanc JG, Keeton BR, •• Monro JL, Tax P, Tuma S, Reich O, et al.: Influence of bidirectional superior cavopulmonary anastomosis on pulmonary arterial growth. Am J Cardiol 1995, 76:1085-1087. Retrospective analysis of 108 procedures performed in three centers between 1987 and 1993. The authors emphasize the need to divide patients into two groups, those with initially small or large pulmonary arteries, in any analysis of the effect of bidirectional Glenn shunt on pulmonary artery growth.
41. Bernstein HS, Brook MM, Silverman NH, Bristow J: Development of •• pulmonary arteriovenous fistulae in children after cavopulmonary shunt. Circulation 1995, 92(suppl II):II-309-II-314. Using contrast echocardiography, the prevalence of pulmonary arteriovenous malformations in 20 patiens with cavopulmonary shunt was 60% and in nine patients with total cavopulmonary connection 33%, higher than previous reports using angiography. The prevalence was higher in patients having surgery less than 6 months of age.
42. Mainwaring RD, Lamberti JJ, Uzark K, Spicer RL: Bidirectional Glenn: is •• accessory pulmonary blood flow good or bad? Circulation 1995, 92(suppl II):II-294-II-297. Review of 92 patients operated at Children's Heart Institute, Children's Hospital, San Diego between 1986 and 1994. Morbidity and mortality were lower in patients who had accessory pulmonary blood flow eliminated at the time of a bidirectional Glenn shunt.
43. Frommelt MA, Frommelt PC, Berger S, Pelech AN, Lewis DA, Tweddell JS, •• Litwin SB: Does an additional source of pulmonary blood flow alter outcome after a bidirectional cavopulmonary shunt? Circulation 1995, 92(suppl II):II-240-II-244. Review of 43 patients operated at Children's Hospital of Wisconsin between 1991 and 1993. Patients with maintained additional sources of pulmonary flow after the shunt had higher postoperative central venous pressure and oxygen saturations and greater incidence of late chylothorax.
44. Rosenthal DN, Bulbul ZR, Friedman AH, Hellenbrand WE, Kleinman CS: • Thrombosis of the pulmonary artery stump after distal ligation. J Thorac Cardiovasc Surg 1995, 110:1563-1565. Three case reports are presented outlining the development of thrombosis in the main pulmonary artery stump after its ligation at the time of a bidirectional Glenn procedure. The authors suggest varying surgical technique to include patch or primary closure of the pulmonary valve.
45. Jonas RA: Intracardiac thrombus after the Fontan procedure. J Thorac • Cardiovasc Surg 1995, 110:1502-1503. Editorial accompanying the previous reference outlining factors associated with thrombus formation after the Fontan procedure.
46. Rychik J, Jacobs ML, Norwood WI Jr: Acute changes in left ventricular •• geometry after volume reduction operation. Ann Thorac Surg 1995, 60:1267-1274. Retrospective review of preoperative and perioperative echocardiograms performed in nine patients after a Fontan procedure, 10 patients after a bidirectional Glenn shunt, and 13 patients after surgical closure of a large ventricular septal defect. Ventricular geometry changed in all patients with greater increases in wall thickness and decreases in cavity dimension in the Fontan group.
47. Miura T, Hiramatsu T, Forbess JM, Mayer JE Jr: Effects of elevated coro•• nary sinus pressure on coronary blood flow and left ventricular function: implications after the Fontan operation. Circulation 1995, 92(suppl II):II-298-II-303. Using isolated blood-perfused juvenile lamb hearts, acute elevation in coronary sinus pressure decreased coronary blood flow and ventricular diastolic function.
48. Fogel MA, Gupta KB, Weinberg PM, Hoffman EA: Regional wall motion •• and strain analysis across stages of Fontan reconstruction by magnetic resonance tagging. Am J Physiol 1995, 269:H1132-H1152. A new imaging technique, magnetic resonance tagging, was used to assess ventricular function after a bidirectional Glenn or modified Fontan procedure. Regional wall motion and strain analyses were different in single ventricles regardless of ventricular morphology or surgical subgroup as compared with normal hearts.
49. Fogel MA, Weinberg PM, Fellows KE. Hoffman EA: A study in ventricu•• lar-ventricular interaction: single right ventricles compared with systemic right ventricles in a dual-chamber circulation. Circulation 1995, 92:219-230. Ventricular-ventricular interactions were assessed in 18 patients using the magnetic resonance tagging technique. Different characteristics in regional wall motion and strain in a systemic right ventricle were found depending on whether a functional single ventricle was present.
50. Yacoub MH: Two hearts that beat as one. Circulation 1995, • 92:160-161. Editorial accompanying the previous reference reviewing earlier research concerning ventricular-ventricular interactions and providing commentary regarding the potential role of magnetic resonance tagging in assessing surgical outcome of congential heart disease.
51. Osmers R, Osmers M, Bartmus D, Ruschewski W, Kuhn W: Ausgetragene Schwangerschaft nach maternaler Fontan'scher-operation bei univentrikulärem herzen. Z Geburtsh Neonatol 1995, 199:195-198.
52. Harrison DA, Liu P, Wlaters JE, Goodman JM, Siu SC, Webb GD, •• Williams WG, McLaughlin PR: Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardrol 1995, 26:1016-1021. Bicycle ergometry and gated radionuclide angiography were performed in 30 adults 6.7±3.9 years after a modified Fontan procedure. Despite few subjective symptoms, maximal workload, maximal ventilation, and anaerobic threshold were each approximately 50% of control levels, whereas maximal oxygen consumption was 35% of control levels.
53. Rosenthal M, Bush A, Deanfield J, Redington A: Comparison of Cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures. Circulation 1995, 91:372-378.
54. Reybrouck T, Rogers R, Weymans M, Dumoulin M, VanhoveM, Daenen W, •• van der Hauwaert L, Gewillig M: Serial cardiorespiratory exercise testing in patients with congenital heart disease. Eur J Pediatr 1995, 154:801-806. Twelve patients after a modified Fontan procedure are included in this analysis of exercise function in 79 patients after repair of various forms of congenital heart disease. Anaerobic threshold in the Fontan group decreased 6% over a 2-year period, representing either deconditioning or disease progression.
55. Norgard G, Gatzoulis MA: Value of exercise testing in the assessment • of the Fontan operation. Cardiol Young 1996, 6:106-108. Editorial accompanying the paper by Mertens et al. (Cardiol Young 1996, 6:136-142). This is a thorough critique of the limitations and findings from numerous investigations. The importance of performing longitudinal studies is stressed.
56. Mertens L, Rogers R, Reybrouck T, Dumoulin M, Vanhees L, Gewillig M: • Cardiopulmonary response to exercise after the Fontan operation: a cross-sectional and longitudinal evaluation. Cardiol Young 1996, 6:136-142. Eighteen patients were assessed with graded treadmill testing 2.3±1.4 years after a modified Fontan procedure. Twelve patients were reevaluated 2.4±2.1 years later. Exercise ability was reduced but varied widely in severity of limitations. There was minimal change in the intermediate follow-up period.
57. Kiani A, Shakibi JG: Fontan physiology. Circulation 1995, 92:3149-3150.
58. Kelley JR, Mack GW, Fahey JT: Diminished venous vascular capaci• tance in patients with univentricular hearts after the Fontan operation. Am J Cardiol 1995, 76:158-163. Most assessments of exercise ability in patients who have undergone Fontan procedures have measured cardiorespiratory function. This study documents increased venous tone and decreased venous capacitance in this patient population that may contribute to exercise limitations.
59. Slavik Z, Lamb RK, Webber SA, Delaney DJ, Salmon AP: A rare cause of • profound cyanosis after Kawashima modification of bidirectional cavopulmonary anastomosis. Ann Thorac Surg 1995, 60:435-437. Case report describing development of intrahepatic venovenous malformations producing cyanosis after this procedure. The shunt flow was reduced by placement of two Rashkind (Bard Ltd., Galway, Ireland) umbrella devices at separate catheterizations.
60. Srivastava D, Preminger T, 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, 92:1217-1222. Detailed review of a 23-year experience in the development of pulmonary arteriovenous malformations after a modified Fontan procedure. This complication was seen only in patients who had hepatic exclusion from the pulmonary circulation or hepatic disease.
61. Bernstein HS, Ursell PC, Brook MM, Hanley FC, Silverman NH, Bristow J: • Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt. Pediatr Cardiol 1996, 17:46-50. Case report describing fulminant and fatal development of pulmonary arteriovenous malformation in a 7-month-old infant 5 months after bilateral bidirectional Glenn shunts in a patient with complex heart disease and interrupted inferior vena cava. Histopathology demonstrated thin walled vessels likely to be the site of shunting.
62. Rosenthal DN, Friedman AH, Kleinman CS, Kopf GS, Rosenfeld LE. •• Hellenbrand WE: Thromboembolic complications after Fontan operations. Circulation 1995, 92(suppl II):II-287-II-293. Retrospective review of 70 patients undergoing a modified Fontan operation between 1978 and 1994 at Yale-New Haven Hospital. Twenty percent of patients developed thromboembolic complications, 43% of whom were asymptomatic. The rate of thrombosis was similar in patients having total cavopulmonary, atriopulmonany, or extracardiac conduit connections.
63. Stümper O, Wright JGC, Sadiq M, de Giovanni JV: Late systemic desat• uration after total cavopulmonary shunt operations. Br Heart J 1995, 74:282-286. Review of six patients with interrupted inferior vena cava who developed cyanosis after total cavopulmonary connection caused by systemic venous to hepatic venous collaterals. The right-to-left shunt was successfully closed in three patients using stainless steel coils or detachable silicone balloons.
64. Hsu H, Nykanen DG, Williams WG, Freedom RM, Benson LN: Right to •• left interatrial communications after the modified Fontan procedure: identification and management with transcatheter occlusion. Br Heart J 1995, 74:548-552. Describes three unusual types of right-to-left shunting occurring at the atrial level after total cavopulmonary connection using a lateral tunnel technique. Shunt occlusion or reduction in flow was achieved with double umbrella device or stainless steel coils.
65. Kuhn MA, Jarmakani JM, Laks H, Alejos JC, Permut LC, Galindo A. Isabel•• Jones JB: Effect of late postoperative atrial septal defect closure on modynamic function in patients with a lateral tunnel Fontan procedure. J Am Coll Cardiol 1995, 26:259-265. Prospective review of 12 patients having late closure of an adjustable interatrial communication at University of California, Los Angeles. Patients with good diastolic ventricular function had a greater decrease in cardiac output and oxygen delivery with closure than patients with poor ventricular function or elevated transpulmonary gradient.
66. Bridges ND, Lock JE, Mayer JE Jr, Burnett J, Castaneda AR: Cardiac •• catheterization and test occlusion of the interatrial communication after the fenestrated Fontan operation. J Am Coll Cardiol 1995, 25:1712-1717. Retrospective review of 60 patients undergoing a fenestrated Fontan procedure before July 1991 and postoperative catheterization before March 1992 at Children's Hospital, Boston. Catheterization was useful in selecting patients for fenestration closure and in identifying hemodynamically important branch pulmonary artery stenosis and aortopulmonary collaterals.
67. Macé L, Dervanian P, Weiss M, Daniel JP, Losay J, Neveux JY: • Hemodynamics of different degrees of right heart bypass: experimental assessment Ann Thorac Surg 1995, 60:1230-1237. Three different types of right heart bypass were assessed in dogs to determine the effect on cardiac index and left atrial pressures.
68. Ichikawa H, Yagihara T, Kishimoto H, Isobe F, Yamamoto F, Nishigaki K, Matsuki O, Fujita T: Extent of aortopulmonary collateral blood flow as a risk factor for Fontan operations. Ann Thorac Surg 1995, 59:433-437. The percent of cardiac return contributed by aortopulmonary collaterals was calculated in 33 patients by measuring blood flow rate returning to the heart and cardiopulmonary blood flow rate during cardioplegic arrest. The percent cardiac return correlated positively with postoperative venous pressure and patient age.
69. Ward CJB, Mullins CE, Nihill MR, Grifka RG, Vick W III: Use of intravas• cular stents in systemic venous and systemic venous baffle obstructions: short-term follow-up results. Circulation 1995, 91:2948-2954. Report of the use of intravascular stents to relieve obstruction in various forms of congenital heart disease in 12 patients. Two patients with classic Glenn shunts with or without inferior vena cava to left pulmonary artery anastomotic obstruction are included.
70. Moore JW, Spicer RL, Perry JC, Mathewson JW, Kirkpatrick SE, George L, •• Uzark K, Mainwaring RL, Lamberti JJ: Percutaneous use of stents to correct pulmonary artery stenosis in young children after cavopulmonary anastomosis. Am Heart J 1995, 130:1245-1249. Reviews placement of stent(s) to relieve left pulmonary artery stenosis after a bidirectional Glenn shunt in eight patients. The internal jugular or subclavian vein were used for access. The stents did not interfere with subsequent completion of the Fontan procedure.
71. 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, 110:865-867. Eight patients initially had total cavopulmonary connections with multiple fenestrations and banding of the superior vena cava at the right atrial junction. The Fontan procedure was completed by occluding the fenestrations with atrial septal defect devices and balloon dilation of the superior vena cava.
72. . Jahangiri M, Ross DB, Redington AN, Lincoln C, Shinebourne EA: Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg 1994, 58:1409-1414.
73. du Plessis AJ, Chang AC, Wessel DL, Lock JE, Wernovsky G, Newburger •• JW, Mayer JE Jr: Cerebrovascular accidents following the Fontan operation. Pediatr Neurol 1995, 12:230-236. Retrospective review of 645 patients undergoing a modified Fontan procedure at Children's Hospital, Boston between 1978 and 1993. There was a 2.6% incidence of stroke. The authors propose that the decision for prophylactic anticoagulation be individualized and that a prospective study be coordinated to establish clinical guidelines.
74. Wilson DG, Wisheart JD, Stuart AG: Systemic thromboembolism • leading to myocardial infarction and stroke after fenestrated total cavopulmonary connection. Br Heart J 1995, 73:483-485. Case report describing initially partial occlusion of the left main stem and left anterior descending coronary artery and cerebral infarction probably arising from femoral vein and inferior vena cava thrombosis and paradoxical embolization across a fenestration. A follow-up catheterization documented complete occlusion of the left anterior descending coronary artery.
75. Day RW, Boyer RS, Tait VF, Ruttenberg HD: Factors associated with •• stroke following the Fontan procedure. Pediatr Cardiol 1995, 16:270-275. Retrospective review of 68 consecutive patients undergoing a modified Fontan procedure between 1978 and 1993. There was an 8.8% incidence of stroke. Some of these patients were receiving antiplatelet agents or warfarin.
76. Danielson GK: Invited commentary. Ann Thorac Surg 1994, 58:1413-1414.
77. Zellers TM, Brown K: Protein-losing enteropathy after the modified • Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol 1996, 17:115-117. Successful use of oral prednisone in a 15-year-old adolescent girl who developed protein-losing enteropathy 4 years after surgery. Diagnosis and relapse after prednisone was discontinued were confirmed by small bowel biopsy. Chronic low-dose steroids produced clinical control.
78. Warnes CA, Feldt RH, Hagler DJ: Protein-losing enteropathy after the • Fontan operation: successful treatment by percutaneous fenestration of the atrial septum. Mayo Clin Proc 1996, 71:378-379. Fenestration was performed in a 42-year-old man who had a modified Fontan procedure involving anastomosis between the right atrial appendage and pulmonary artery. Transesophageal echocardiography was used to aid in site selection for blade septostomy. Follow-up was limited to 5 months.
79. Jacobs ML, Rychik J, Byrum CJ, Norwood WI Jr: Protein-losing enteropa• thy after Fontan operation: resolution after baffle fenestration. Ann Thorac Surg 1996, 61:206-208. First report of surgical creation of baffle fenestration in two patients, aged 4 and 6 years. Clinical symptoms of protein-losing enteropathy rapidly resolved. Follow-up was limited to 2 to 5 months.
80. 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, 72:591-592.