Heart 1999;82:40-46 ( July )

Abnormalities in liver function and coagulation profile following the Fontan procedure

R C van Nieuwenhuizen^a, M Peters^b, L J Lubbers^c, M D Trip^a, J G P Tijssen^d, B J M Mulder^a

^a Department of Cardiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, ^b Department of Paediatric Haematology, Academic Medical Centre, University of Amsterdam, ^c Department of Paediatric Cardiology, Academic Medical Centre, University of Amsterdam, ^d Department of Clinical Epidemiology and Biostatistics, Academic Medical Centre, University of Amsterdam

Correspondence to: Dr Mulder.

Accepted for publication 2 November 1998

OBJECTIVETo investigate liver function and coagulation disorders in patients with a Fontan circulation at different time intervals after surgery.
DESIGNRetrospective analysis of clinical data and cross sectional study relating liver function and coagulation profile to time since surgery, in 28 surviving patients after the modified Fontan procedure.
PATIENTS20 patients (71%) with atriopulmonary anastomosis, seven (25%) with atrioventricular anastomosis, and one (4%) with total cavopulmonary connection. Follow up ranged from 2.0 to 21.8 years (mean 11.1).
RESULTSAbnormal liver function tests, mainly reflecting cholestasis, were present in 21 patients who had a significantly longer follow up (p < 0.01). Protein synthesis was normal in almost all patients. Coagulation profile showed abnormalities in 22 patients. "Procoagulant" abnormalitiesthat is, decreased plasminogen and protein C activitywere found in 11 and five patients, respectively. The extent of these abnormalities was less in patients with a longer follow up. Anticoagulant abnormalities were factor V deficiency in 16 patients and factor VII deficiency in 17, resulting in a prolonged prothrombin time in 19 patients. Thirteen patients had both pro- and anticoagulant abnormalities. A prethrombotic state was present in five patients, with a significantly longer mean time interval since surgery (p = 0.05). Thus, although the individual procoagulant indices decreased with increasing time intervals since surgery, a prethrombotic state was found particularly in patients with a long term follow up.
CONCLUSIONSMild cholestasis was mainly present in Fontan patients with a long duration of follow up. Along with laboratory procoagulant abnormalities indicating a prethrombotic state, anticoagulant abnormalities were also present. The coagulation profile varied at different time intervals after surgery. Thus detailed evaluation should be performed regularly, and the use of anticoagulants should be considered in every patient. Long term prospective studies are needed to evaluate the individual fluctuations of coagulation profile over time following a Fontan procedure.


Keywords: Fontan procedure; coagulation; liver function; congenital heart disease

---

© 1999 by Heart
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Camposilvan, S., Milanesi, O., Stellin, G., Pettenazzo, A., Zancan, L., D'Antiga, L. (2008). Liver and Cardiac Function in the Long Term After Fontan Operation. Ann. Thorac. Surg. 86: 177-182 [Abstract] [Full Text]  
• Kendall, T J, Stedman, B, Hacking, N, Haw, M, Vettukattill, J J, Salmon, A P, Cope, R, Sheron, N, Millward-Sadler, H, Veldtman, G R, Iredale, J P (2008). Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J. Clin. Pathol. 61: 504-508 [Abstract] [Full Text]  
• Procelewska, M., Kolcz, J., Januszewska, K., Mroczek, T., Malec, E. (2007). Coagulation abnormalities and liver function after hemi-Fontan and Fontan procedures -- the importance of hemodynamics in the early postoperative period. Eur. J. Cardiothorac. Surg. 31: 866-872 [Abstract] [Full Text]  
• Kiesewetter, C. H, Sheron, N., Vettukattill, J. J, Hacking, N., Stedman, B., Millward-Sadler, H., Haw, M., Cope, R., Salmon, A. P, Sivaprakasam, M. C, Kendall, T., Keeton, B. R, Iredale, J. P, Veldtman, G. R (2007). Hepatic changes in the failing Fontan circulation. Heart 93: 579-584 [Abstract] [Full Text]  
• Engelfriet, P., Tijssen, J., Kaemmerer, H., Gatzoulis, M. A., Boersma, E., Oechslin, E., Thaulow, E., Popelova, J., Moons, P., Meijboom, F., Daliento, L., Hirsch, R., Laforest, V., Thilen, U., Mulder, B. (2006). Adherence to guidelines in the clinical care for adults with congenital heart disease: The Euro Heart Survey on Adult Congenital Heart Disease. Eur Heart J 27: 737-745 [Abstract] [Full Text]  
• Engelfriet, P., Boersma, E., Oechslin, E., Tijssen, J., Gatzoulis, M. A., Thilen, U., Kaemmerer, H., Moons, P., Meijboom, F., Popelova, J., Laforest, V., Hirsch, R., Daliento, L., Thaulow, E., Mulder, B. (2005). The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period: The Euro Heart Survey on adult congenital heart disease. Eur Heart J 26: 2325-2333 [Abstract] [Full Text]  
• Riemer, R. K., Amir, G., Reichenbach, S. H., Reinhartz, O. (2005). Mechanical support of total cavopulmonary connection with an axial flow pump. J. Thorac. Cardiovasc. Surg. 130: 351-354 [Abstract] [Full Text]  
• Walker, H A, Gatzoulis, M A (2005). Prophylactic anticoagulation following the Fontan operation. Heart 91: 854-856 [Full Text]  
• Chaloupecky, V, Svobodova, I, Hadacova, I, Tomek, V, Hucin, B, Tlaskal, T, Janousek, J, Reich, O, Skovranek, J (2005). Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up. Heart 91: 73-79 [Abstract] [Full Text]  
• Lovell, A. T. (2004). Anaesthetic implications of grown-up congenital heart disease. Br J Anaesth 93: 129-139 [Abstract] [Full Text]  
• Varma, C., Warr, M. R., Hendler, A. L., Paul, N. S., Webb, G. D., Therrien, J. (2003). Prevalence of "silent" pulmonary emboli in adults after the Fontan operation. J Am Coll Cardiol 41: 2252-2258 [Abstract] [Full Text]  
• Odegard, K. C., McGowan, F. X. Jr, Zurakowski, D., DiNardo, J. A., Castro, R. A., del Nido, P. J., Laussen, P. C. (2003). Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: Increased factor VIII may predispose to thrombosis. J. Thorac. Cardiovasc. Surg. 125: 1260-1267 [Abstract] [Full Text]  
• Odegard, K. C., McGowan, F. X. Jr, Zurakowski, D., DiNardo, J. A., Castro, R. A., del Nido, P. J., Laussen, P. C. (2002). Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the fontan procedure. Ann. Thorac. Surg. 73: 1770-1777 [Abstract] [Full Text]  
• Aeba, R., Katogi, T., Hashizume, K., Iino, Y., Kawada, S., Yuasa, Y. (2002). Individualized total cavopulmonary connection technique for patients with Asplenia syndrome. Ann. Thorac. Surg. 73: 1274-1280 [Abstract] [Full Text]  
• Odegard, K. C., McGowan, F. X. Jr, DiNardo, J. A., Castro, R. A., Zurakowski, D., Connor, C. M., Hansen, D. D., Neufeld, E. J., del Nido, P. J., Laussen, P. C. (2002). Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure. J. Thorac. Cardiovasc. Surg. 123: 459-465 [Abstract] [Full Text]  
• Coon, P. D., Rychik, J., Novello, R. T., Ro, P. S., Gaynor, J. W., Spray, T. L. (2001). Thrombus formation after the Fontan operation. Ann. Thorac. Surg. 71: 1990-1994 [Abstract] [Full Text]  
• Gaca, J. A., Douglas, W. I., Barnes, S. D. (2001). Anesthetic Implications of the Fontan Procedure for Single Ventricle Physiology. SEMIN CARDIOTHORAC VASC ANESTH 5: 31-39 [Abstract]  
• Hsia, T.-Y., Khambadkone, S., Deanfield, J. E., Taylor, J. F. N., Migliavacca, F., de Leval, M. R. (2001). Subdiaphragmatic venous hemodynamics in the Fontan circulation. J. Thorac. Cardiovasc. Surg. 121: 436-447 [Abstract] [Full Text]