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The Journal of Thoracic and Cardiovascular Surgery, Vol 106, 1126-1132, Copyright © 1993 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
AH Cromme-Dijkhuis, J Hess, K Hahlen, CM Henkens, MT Bink-Boelkens, AA Eygelaar and E Bos
Patients who have undergone a Fontan-type operation usually have an
elevated systemic venous pressure. To determine the sequelae of this
nonphysiologic condition, we evaluated 66 patients 1 to 14 years after a
Fontan-type operation. Fifty-one patients were apparently in good clinical
condition, and 15 patients had symptoms and were restricted in their daily
life. Bicycle exercise capacity, tested in 41 patients, ranged from 50% to
110% (mean 85%) of the predicted value for length. In 16 patients, a
decreased capacity (< 85%) was, among others, related to arrhythmias and
the presence of protein-losing enteropathy. A 24- hour ambulatory
electrocardiogram was available in 56 patients and found to be normal in 32
(57%) patients. Arrhythmias were present in 21 patients, six of whom had
symptoms. Three patients had previous pacemaker implantation. One or more
abnormalities in liver enzyme and function tests were present in 40
patients (61%) and in coagulation factors in 46 patients (69%). The most
pronounced was a protein C deficiency, a known thrombotic risk factor,
present in 41 patients. The occurrence of arrhythmias increased with time
of follow-up (p < 0.004), the occurrence of protein C deficiency
decreased with time (p < 0.0001), and the occurrence of abnormal liver
enzyme and function tests was not related to time of follow-up. With regard
to age at operation, arrhythmias did not occur in patients who underwent
operation at a mean age of 4 +/- 1.9 years (standard deviation), in
contrast to patients who underwent operation at a mean age of 7.6 +/- 4
years (standard deviation) (p < 0.001). The occurrence of the two other
types of sequelae was not related to the age at operation. With regard to
the type of operation, only patients with a valved right atrium-to-
pulmonary artery connection had a higher prevalence of arrhythmias than
patients with a nonvalved or direct right atrium-to-pulmonary artery
connection (p < or = 0.001). The latter patients also had a higher
prevalence of protein C deficiency (p < or = 0.001). No relationship was
found among the other types of operation, the underlying structure, or the
hemodynamic condition measured at rest and the presence of arrhythmias,
abnormal liver enzyme and function tests, or protein C deficiency. This
point survey shows that even patients with an apparently good clinical
condition are at risk for arrhythmias, abnormal liver enzyme and function
tests, and coagulation factor abnormalities. Serial statement of affairs is
recommended to ensure that adequate preventive measures can be taken.
ARTICLES
Specific sequelae after Fontan operation at mid- and long-term follow- up. Arrhythmia, liver dysfunction, and coagulation disorders
Division of Pediatric Cardiology, Sophia Children's Hospital, Rotterdam, The Netherlands.
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 C. D. McClain, F. X. McGowan, and P. G. Kovatsis Laparoscopic Surgery in a Patient with Fontan Physiology Anesth. Analg., October 1, 2006; 103(4): 856 - 858. [Abstract] [Full Text] [PDF]
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D. L.S. Morales, D. J. Dibardino, B. E. Braud, A. L. Fenrich, J. S. Heinle, W. K. Vaughn, E. D. McKenzie, and C. D. Fraser Jr Salvaging the Failing Fontan: Lateral Tunnel Versus Extracardiac Conduit Ann. Thorac. Surg., October 1, 2005; 80(4): 1445 - 1452. [Abstract] [Full Text] [PDF]
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A.M. Sheikh, A.T.M. Tang, K. Roman, K. Baig, R. Mehta, J. Morgan, B. Keeton, J. Gnanapragasam, J.V. Vettukattil, A.P. Salmon, et al. The failing Fontan circulation: Successful conversion of atriopulmonary connections J. Thorac. Cardiovasc. Surg., July 1, 2004; 128(1): 60 - 66. [Abstract] [Full Text] [PDF]
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K. C. Odegard, F. X. McGowan Jr, D. Zurakowski, J. A. DiNardo, R. A. Castro, P. J. del Nido, and P. C. Laussen Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: Increased factor VIII may predispose to thrombosis J. Thorac. Cardiovasc. Surg., June 1, 2003; 125(6): 1260 - 1267. [Abstract] [Full Text] [PDF]
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A. T. Yetman, J. Drummond-Webb, W. P. Fiser, M. L. Schmitz, M. Imamura, S. Ullah, R. J. Gunselman, C. W. Chipman, C. E. Johnson, and S. H. Van Devanter The extracardiac Fontan procedure without cardiopulmonary bypass: technique and intermediate-term results Ann. Thorac. Surg., October 1, 2002; 74(4): S1416 - S1421. [Abstract] [Full Text] [PDF]
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R. Kaulitz, G. Ziemer, T. Paul, M. Peuster, H. Bertram, and G. Hausdorf Fontan-type procedures: residual lesions and late interventions Ann. Thorac. Surg., September 1, 2002; 74(3): 778 - 785. [Abstract] [Full Text] [PDF]
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B. J. Deal, C. Mavroudis, C. L. Backer, S. H. Buck, and C. Johnsrude Comparison of Anatomic Isthmus Block With the Modified Right Atrial Maze Procedure for Late Atrial Tachycardia in Fontan Patients Circulation, July 30, 2002; 106(5): 575 - 579. [Abstract] [Full Text] [PDF]
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K. C. Odegard, F. X. McGowan Jr, D. Zurakowski, J. A. DiNardo, R. A. Castro, P. J. del Nido, and P. C. Laussen Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the fontan procedure Ann. Thorac. Surg., June 1, 2002; 73(6): 1770 - 1777. [Abstract] [Full Text] [PDF]
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K. C. Odegard, F. X. McGowan Jr, J. A. DiNardo, R. A. Castro, D. Zurakowski, C. M. Connor, D. D. Hansen, E. J. Neufeld, P. J. del Nido, and P. C. Laussen Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure J. Thorac. Cardiovasc. Surg., March 1, 2002; 123(3): 459 - 465. [Abstract] [Full Text] [PDF]
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M. L. Jacobs, K. K. Pourmoghadam, E. M. Geary, A. T. Reyes, N. Madan, L. B. McGrath, and J. W. Moore Fontan's operation: is aspirin enough? Is coumadin too much? Ann. Thorac. Surg., January 1, 2002; 73(1): 64 - 68. [Abstract] [Full Text] [PDF]
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S. Tokunaga, H. Kado, Y. Imoto, M. Masuda, Y. Shiokawa, K. Fukae, N. Fusazaki, S. Ishikawa, and H. Yasui Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients Ann. Thorac. Surg., January 1, 2002; 73(1): 76 - 80. [Abstract] [Full Text] [PDF]
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C. Mavroudis, C. L. Backer, B. J. Deal, C. Johnsrude, and J. Strasburger Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation J. Thorac. Cardiovasc. Surg., November 1, 2001; 122(5): 863 - 871. [Abstract] [Full Text] [PDF]
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 A. Ghai, L. Harris, D. A. Harrison, G. D. Webb, and S. C. Siu Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation J. Am. Coll. Cardiol., February 1, 2001; 37(2): 585 - 592. [Abstract] [Full Text] [PDF]
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S. Tokunaga, H. Kado, Y. Imoto, Y. Shiokawa, and H. Yasui Successful staged-Fontan operation in a patient with ectopia cordis Ann. Thorac. Surg., February 1, 2001; 71(2): 715 - 717. [Abstract] [Full Text] [PDF]
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H B Ravn, V E Hjortdal, E V Stenbog, K Emmertsen, O Kromann, J Pedersen, and K E Sorensen Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection Heart, January 1, 2001; 85(1): 61 - 65. [Abstract] [Full Text]
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C. P.R. Brizard, N. Goussef, J. C. Chachques, and A. F. Carpentier Model of complete separation of the hepatic veins from the systemic venous system Ann. Thorac. Surg., December 1, 2000; 70(6): 2096 - 2101. [Abstract] [Full Text] [PDF]
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M. Jahangiri, J. Kreutzer, D. Zurakowski, E. Bacha, and R. A. Jonas Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation J. Thorac. Cardiovasc. Surg., October 1, 2000; 120(4): 778 - 782. [Abstract] [Full Text] [PDF]
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B. Airan, R. Sharma, S. K. Choudhary, S. R. Mohanty, A. Bhan, U. K. Chowdhari, R. Juneja, S. S. Kothari, A. Saxena, and P. Venugopal Univentricular repair: is routine fenestration justified? Ann. Thorac. Surg., June 1, 2000; 69(6): 1900 - 1906. [Abstract] [Full Text] [PDF]
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G. Balling, M. Vogt, H. Kaemmerer, A. Eicken, H. Meisner, and J. Hess INTRACARDIAC THROMBUS FORMATION AFTER THE FONTAN OPERATION J. Thorac. Cardiovasc. Surg., April 1, 2000; 119(4): 745 - 751. [Abstract] [Full Text] [PDF]
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R Kaulitz, P Bergman, I Luhmer, T Paul, and G Hausdorf Instantaneous pressure-flow velocity relations of systemic venous return in patients with univentricular circulation Heart, September 1, 1999; 82(3): 294 - 299. [Abstract] [Full Text] [PDF]
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R C van Nieuwenhuizen, M Peters, L J Lubbers, M D Trip, J G P Tijssen, and B J M Mulder Abnormalities in liver function and coagulation profile following the Fontan procedure Heart, July 1, 1999; 82(1): 40 - 46. [Abstract] [Full Text] [PDF]
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J. A. M. van Son, F. W. Mohr, J. Hambsch, P. Schneider, H. Hess, and G. S. Haas Conversion of atriopulmonary or lateral atrial tunnel cavopulmonary anastomosis to extracardiac conduit Fontan modification Eur J Cardiothorac Surg, February 1, 1999; 15(2): 150 - 158. [Abstract] [Full Text] [PDF]
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T. W. Waterbolk, M. T. E. Bink-Boelkens, N. J. Elzenga, G. C. M. Beaufort-Krol, and T. Ebels The right auricle tunnel as intercaval tunnel in total cavopulmonary connection may prevent atrial flutter Eur J Cardiothorac Surg, December 1, 1998; 14(6): 590 - 595. [Abstract] [Full Text] [PDF]
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P. Monagle, A. Cochrane, B. McCrindle, L. Benson, W. Williams, and M. Andrew Editorial: Thromboembolic Complications After Fontan Procedures--The Role Of Prophylactic Anticoagulation J. Thorac. Cardiovasc. Surg., March 1, 1998; 115(3): 493 - 498. [Full Text]
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C. Mavroudis, C. L. Backer, B. J. Deal, and C. L. Johnsrude Fontan Conversion To Cavopulmonary Connection And Arrhythmia Circuit Cryoablation J. Thorac. Cardiovasc. Surg., March 1, 1998; 115(3): 547 - 556. [Abstract] [Full Text]
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P. Monagle and M. Andrew Coagulation abnormalities after Fontan procedures J. Thorac. Cardiovasc. Surg., March 1, 1998; 115(3): 732 - 732. [Full Text]
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K. BULL The Fontan procedure: lessons from the past Heart, March 1, 1998; 79(3): 213 - 214. [Full Text]
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T. L. Gentles, K. Gauvreau, J. E. Mayer Jr., S. B. Fishberger, J. Burnett, S. D. Colan, J. W. Newburger, and G. Wernovsky FUNCTIONAL OUTCOME AFTER THE FONTAN OPERATION: FACTORS INFLUENCING LATE MORBIDITY J. Thorac. Cardiovasc. Surg., September 1, 1997; 114(3): 392 - 403. [Abstract] [Full Text]
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M. Jahangiri, D. Shore, V. Kakkar, C. Lincoln, and E. Shinebourne COAGULATION FACTOR ABNORMALITIES AFTER THE FONTAN PROCEDURE AND ITS MODIFICATIONS J. Thorac. Cardiovasc. Surg., June 1, 1997; 113(6): 989 - 993. [Abstract] [Full Text]
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R. N. Gates, H. Laks, D. C. Drinkwater Jr, L. Lam, A. Blitz, J. S. Child, and J. K. Perloff The Fontan Procedure in Adults Ann. Thorac. Surg., April 1, 1997; 63(4): 1085 - 1090. [Abstract] [Full Text]
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D. B. McElhinney, V. M. Reddy, P. Moore, and F. L. Hanley Revision of Previous Fontan Connections to Extracardiac or Intraatrial Conduit Cavopulmonary Anastomosis Ann. Thorac. Surg., November 1, 1996; 62(5): 1276 - 1282. [Abstract] [Full Text]
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R. Kaulitz, G. Ziemer, I. Luhmer, and H.-C. Kallfelz MODIFIED FONTAN OPERATION IN FUNCTIONALLY UNIVENTRICULAR HEARTS: PREOPERATIVE RISK FACTORS AND INTERMEDIATE RESULTS J. Thorac. Cardiovasc. Surg., September 1, 1996; 112(3): 658 - 664. [Abstract] [Full Text]
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R. Kaulitz, G. Ziemer, I. Luhmer, T. Paul, and H. C. Kallfelz Total cavopulmonary anastomosis in patients less than three years of age Ann. Thorac. Surg., December 1, 1995; 60(suppl_3): S563 - S567. [Abstract] [PDF]
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D. N. Rosenthal, A. H. Friedman, C. S. Kleinman, G. S. Kopf, L. E. Rosenfeld, and W. E. Hellenbrand Thromboembolic Complications After Fontan Operations Circulation, November 1, 1995; 92(9): 287 - 293. [Abstract] [Full Text]
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J. M. Kao, J. C. Alejos, P. W. Grant, R. G. Williams, K. M. Shannon, and H. Laks Conversion of atriopulmonary to cavopulmonary anastomosis in management of late arrhythmias and atrial thrombosis Ann. Thorac. Surg., November 1, 1994; 58(5): 1510 - 1514. [Abstract] [PDF]
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R. A. Jonas Indications and timing for the bidirectional Glenn shunt versus the fenestrated Fontan circulation J. Thorac. Cardiovasc. Surg., September 1, 1994; 108(3): 522 - 524. [Full Text]
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