Thrombosis in children with cardiac pathology: analysis of acquired and inherited risk factors

Abstract

The present study was conducted to analyze the features and risk factors of childhood thrombotic events in patients with cardiac defect followed-up at our hospital. The clinical and laboratory findings of 59 patients diagnosed with cardiac defects and thromboses between 1997 and 2006 were retrospectively analyzed. Thirty-one children (52.5%) had venous system thromboses, 21 (35.6%) had arterial system thromboses, and seven (11.9%) had venous and arterial system thromboses. Presence of congenital heart disease and cardiomyopathy (CMP) were significant risk factors for developing intracardiac thrombosis. In addition, presence of congenital heart disease was the significant statistical risk factor for developing left atrium and right ventricle thromboses. Presence of congenital heart disease was a significant risk factor for developing a central nervous system thrombosis. Presence of pulmonary stenosis and aortic coarctation were significant risk factors for developing a peripheral arterial system thrombosis. Acquired risk factors including major surgery, angiography, central venous catheter, systemic infection, and hypoxia were identified in 49 of the 59 patients. Many patients had more than one of these acquired risk factors. Analysis of the relationship between thrombosis and type of major surgery demonstrated a statistically significant relationship between an intracardiac thrombosis and total correction of tetralogy of Fallot and a peripheral venous system thrombosis and a Blalock Taussig shunt. Twenty-three of the 52 patients (44.2%) had at least one thrombophilic mutation. Overall, a heterozygous factor V Leiden mutation was found in nine patients (17.3%), a methylenetetrahydrofolate reductase 677C-T mutation in 15 patients (28.8%), and a PT 20210G-A mutation in three patients (5.8%). Our data suggest that cardiac defects are common risk factors for developing a childhood thrombosis. The type of disorder determines the site of thrombosis. Acquired risk factors may contribute to the development of a thrombosis. The results of this study also indicate that to ensure early diagnosis, routine screening for thrombosis should be performed in patients with a cardiac defect and that screening for factor V Leiden and PT 20210G-A mutations and other genetic risk factors should be included when assessing all patients with cardiac defects who present with a thrombosis, whether or not a predisposing factor has been identified.

Manifestations and clinical impact of pediatric inherited thrombophilia

“…The frequencies of inherited thrombophilic factors as well as the quality assessment are shown in Table 1. [8][9][10][11][12] The studies are analyzed descriptively.…”

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

“…• 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:…”

Role of thrombophilia factors in acute systemic–pulmonary shunt obstruction

“…It is well known that cyanotic congenital heart disease and cardiomyopathy are the two most common causes of cardiac thrombosis in childhood. Particularly in cyanotic congenital heart disease, there may be associated secondary erythrocytosis, activation of procoagulant pathways depending on hypoxemia, chronic acidosis, increase in the release of tissue factor, inhibition of fibrinolysis, acquired protein C and S deficiency, need to perform modified Blalock–Taussig shunt and Fontan operations, use of cardiopulmonary bypass, infection, increased anticardiolipin antibody titer, prothrombin G20210A mutation, and a risk of thrombosis depending on factor V Leiden mutation . Fifty‐nine patients with cardiac pathology and thrombosis were examined in a study conducted by Alioglu et al ., and a significant correlation between cardiac thrombosis and modified Blalock–Taussig shunt was reported .…”

“…and Gurgey et al . studies, infection, catheterization and the aforementioned thrombophilia factors increased the likelihood of thrombosis associated with cardiac pathology, in patients with congenital heart disease . Therefore, the investigation of inherited and acquired thrombophilia factors in patients with congenital heart disease combined with thrombosis is recommended …”