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J Thorac Cardiovasc Surg 2002;123:459-465
© 2002 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease (CHD)

Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure

From the Departments of Anesthesia^a Cardiac Surgery^b Biostatistics^c and Division of Hematology^d Children's Hospital, Boston, and Harvard Medical School,^e Boston, Mass.

Supported in part by National Institutes of Health grants HL04184,^d HL52589, and HL66186^a,e and the Children's Hospital Anesthesia Foundation.

Received for publication May 29, 2001; revisions requested Aug 6, 2001; revisions received Aug 27, 2001; accepted for publication Aug 31, 2001. Address for reprints: Kirsten C. Odegard, MD, Cardiac Anesthesia Service, 300 Longwood Ave, Children's Hospital, Boston, MA 02115 (E-mail: kirsten.odegard{at}tch harvard.edu.).

Objective: Thromboembolic events in patients who have undergone the Fontan operation have been reported to be as high as 20% to 33%. A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair.
Methods: After informed consent had been obtained, coagulation factors were assayed in 36 infants (mean age, 7.7 ± 3.6 months) with single-ventricle cardiac defects immediately before undergoing the bidirectional Glenn procedure; 34 infants (mean age, 8.4 ± 2.6 months) without cardiac disease were assayed as control subjects. Concentration of factors II, V, VII, VIII, IX, and X; antithrombin III; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals on the basis of the control subjects were determined by using 95% confidence limits. Patient demographic and hemodynamic variables were evaluated as possible predictors of coagulation abnormalities.
Results: Concentrations of protein C; factors II, V, VII, IX, and X; plasminogen; fibrinogen; and antithrombin III were significantly lower in the pre-Glenn infants compared with in the age-matched control subjects (all P < .001, Student t test). On the basis of multiple logistic regression, no specific hemodynamic variables were predictive of a procoagulant or anticoagulant deficiency. Ventricular dysfunction did predict the presence of multiple coagulation abnormalities (P < .001).
Conclusion: Procoagulant and anticoagulant factor abnormalities occur early in the course of single-ventricle repair and precede the cavopulmonary connection.

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