Prospective longitudinal study of coagulation profiles in children with hypoplastic left heart syndrome from stage I through Fontan completion

Abstract

This longitudinal study in patients with identical cardiac disease and staged surgical procedures confirms the increase in factor VIII level after the Fontan procedure. This is an acquired defect, and although the cause remains to be determined, monitoring factor VIII levels after the Fontan operation could indicate a subset of patients at risk for thrombosis.

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

“…In cyanotic heart disease, this abnormality is hypothesized to be a result of cyanosis, low cardiac output, or a genetic predisposition. 136 Hepatic synthetic ability does not appear to be abnormal. 136 In children with acyanotic CHD, certain coagulation factors have been shown to be decreased in an age-dependent manner, making them similar to those measured in cyanotic CHD 143 : • At0 to 3 months of age: decreased levels of protein However, in children with acyanotic CHD, normal ageappropriate levels are achieved by 5 years of age, which differs from children with cyanotic CHD.…”

“…136 Hepatic synthetic ability does not appear to be abnormal. 136 In children with acyanotic CHD, certain coagulation factors have been shown to be decreased in an age-dependent manner, making them similar to those measured in cyanotic CHD 143 : • At0 to 3 months of age: decreased levels of protein However, in children with acyanotic CHD, normal ageappropriate levels are achieved by 5 years of age, which differs from children with cyanotic CHD. 136,143 In children with cyanotic CHD, studies before 1998 reported decreased protein C, protein S, and FVII levels; however, these studies did not use age-appropriate normal values, which renders the study results invalid.…”

“…Cyanotic CHD is more commonly reported to have known hemostatic abnormalities [136][137][138][139][140][141][142] compared with acyanotic CHD 143 and acquired heart disease (Kawasaki disease, cardiomyopathy).…”

“…• Coagulation proteins: II, V, VII, VIII, IX, and X, and fibrinogen: decreased; VIII: increased [136][137][138][139][140][141][142][143] • Inhibitors of coagulation: proteins C and S and antithrombin: decreased [136][137][138][139][140][141][142][143] • Fibrinolytic proteins: plasminogen: decreased 143 • Prothrombotic genetic polymorphisms identified: factor V Leiden, prothrombin gene 20120, plasminogen G4/ G4, methylene tetrahydrofolate reductase 677 144,145 Abnormalities in hemostatic function include the following:…”

“…[136][137][138][139][140][141][142][143] Depending on the abnormality or abnormalities present, these children can be at risk for bleeding or thrombosis. The reason for the decreased production and delayed normalization of coagulation protein levels (anticoagulant and procoagulant factors) is unknown in cyanotic and acyanotic heart disease.…”