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Coagulation profile, hepatic function, and hemodynamics following Fontan-type operations

Published online by Cambridge University Press:  01 July 2011

Hideshi Tomita
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
Osamu Yamada
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
Hideo Ohuchi
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
Yasuo Ono
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
Yoshio Arakaki
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
Toshikatsu Yagihara
Cardiovascular surgery, National Cardiovascular Center, Osaka, Japan
Shigeyuki Echigo
Department of Pediatrics, National Cardiovascular Center, Osaka, Japan


We analyzed retrospectively the relationship between coagulation profile, and either hepatic function or hemodynamics, in patients who had undergone a Fontan-type procedure, comparing them, first, with a control group of 12 patients without significant hemodynamic abnormality, and, second, with a group of 14 patients who had not undergone a Fontan procedure, but whose mean right atrial pressure exceeded 8 mmHg. Follow-up catheterization had been performed in all 30 patients submitted to the Fontan-type operation. Prothrombin time, and factor XIII, were significantly lower in those who had undergone the Fontan procedure than in the other groups. Those submitted to the Fontan operation also had lower levels of protein C than controls, and their levels of plasminogen were lower than the patients with high right atrial pressure. Both aspartate aminotransferase and alanine aminotransferase were higher in those undergoing the Fontan procedure than in the other groups, while gamma-glutamyltranspeptidase in these patients was higher than in the control group. Mean right atrial pressure was highest in those under-going the Fontan procedure, while cardiac index was lowest. Prothrombin time was correlated to some extent with aspartate aminotransferase, mean right atrial pressure, and cardiac index. Protein C correlated with both aspartate aminotransferase and mean right atrial pressure, while factor XIII correlated with alanine aminotransferase, mean right atrial pressure, and cardiac index. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltranspeptidase, parameters of hepatic function, correlated significantly with mean right atrial pressure. In those who had undergone the Fontan procedure, decreased synthesis of pro-and anti-coagulant factors is a risk factor for both thrombosis and bleeding. Abnormal hemodynamics, in the absence of a right sided pumping chamber, may predispose to subclinical hepatic dysfunction, leading to selective disturbances of protein synthesis.

Original Articles
Copyright © Cambridge University Press 2001

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1.Fontan, F, Baudet, E.Surgical repair of tricupid atresia. Thorax 1971;26:240248.CrossRefGoogle Scholar
2.Fontan, F, JW, Kirklin, Fernandez, G. Outcome after a ‘perfectFontan operation. Circulation 1990;81:15201536.CrossRefGoogle ScholarPubMed
3.JE, Mayer, Helgason, H, RD, Jonas, Lang, V, FJ, Vargas, Cook, N, AR., CastanedaExtending the limits of modified Fontan procedures. J Thorac Cardiovasc Surg 1986;92:10211028.Google Scholar
4.DD, Mair, DJ, Hagler, FJ, Pugs, HV, Schaff, GK., DanielsonFontan operation in 176 patients with tricuspid atresia. Circulation 1990;82(suppl IV):IV–l46-IV–169.Google Scholar
5.Bartmus, D, DJ, Driscoll, KP, Offord, RA, Humer, DD, Mair, HV, Schaff, FJ, Puga, GK, Danielson. The modified Fontan operation for children less than 4 years old. J Am Coll Cardiol 1990;15:429435.CrossRefGoogle Scholar
6.DJ, Driscoll, KP, Offord, RH, Feldt, HV, Schaff, FJ, Puga, Danielson. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992;85:469496.Google Scholar
7.AH, Cromme-Dijkhuis, CM, Henkes, CM, Bijleveld, HL, Hillege, VJ, Bom, van der Meer, J.Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations. Lancet 1990;336:10871090.Google Scholar
8.AH, Cromme-Dijkhuis, Hess, J, Hahlen, K, CM, Henkes, MT, Bink-Boelkens, AA, Eygelaar, Bos, E.Specific sequelae after Fontan operation at mid- and long-term follow-up. Arrhythmia, liver dysfunction, and coagulation disorders. J Thorac Cardiovasc Surg 1993;106:11261132.Google Scholar
9.Jahangiri, M, DB, Ross, AN, Redington, Lincoln, C, Shinebourne, E.Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg 1994;58:14091413.CrossRefGoogle ScholarPubMed
10.Kaulitz, R, Ziemer, G, Bergmann, F, Luhmer, I, HC., KallfelizAtrial thrombus after the Fontan-operation: predisposing factors, treatment and prophylaxis. Cardiol Young 1997;7:3743.CrossRefGoogle Scholar
11.Kaulitz, R, Luhmer, I, Bergmann, F, Rodeck, B, Hausdorf, G.Sequelae after modified Fontan operation: postoperative haemodynamics and organ function. Heart 1997;58:154159.CrossRefGoogle Scholar
12.Jahangiri, M, Shore, D, Kakkar, V, Lincoln, C, Shinebourne, E.Coagulation factor abnormality after the Fontan procedure and its modifications. J Thorac Cardiovasc Surg 1997;113:989–93.CrossRefGoogle ScholarPubMed
13.RCV, Nieuwenhuizen, Peters, M, LJ, Lubbers, MD, Trip, JGP, Tijssen, BJM., MulderAbnormality in liver function and coagulation profile following the Fontan procedure. Heart 1999;82:4046.Google Scholar
14.CG, LaFarge, OS., MicttinenThe estimation of oxygen consumption. Cardiovascular Research 1970;4:2330.Google Scholar
15.Andrew, M, Paes, B, Milner, R, Johnston, M, Mitchell, L, DM, Tollefsen, Powers, P.Development of the human coagulation system in the fullterm infant. Blood 1987;70:165–72.CrossRefGoogle Scholar
16.Andrew, M, Vegh, P, Johnston, M, Bowker, J, Ofosu, F, Mitchell, L.Maturation of the hemostatic system during childhood. Blood 1992;80:19982005.CrossRefGoogle ScholarPubMed