Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the fontan procedure

Odegard, Kirsten C.; McGowan, Francis X.; Zurakowski, David; DiNardo, James A.; Castro, Robert A.; Nido, Pedro J. del; Laussen, Peter C.

doi:10.1016/s0003-4975(02)03580-4

Cited by 84 publications

(68 citation statements)

References 19 publications

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“…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).…”

Section: Propensity To Coagulopathy In Children With Heart Diseasementioning

confidence: 99%

“…• 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:…”

Section: Propensity To Coagulopathy In Children With Heart Diseasementioning

confidence: 99%

“…[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.…”

Section: Abnormalities In Hemostatic Proteins In Childrenmentioning

confidence: 99%

“…138,139 Recent prospective studies using homogeneous patient cohorts demonstrate decreased protein C, antithrombin III (AT III), FII, FV, FVII, FX, and plasminogen before Fontan surgery and increased FVIII and decreased protein C after Fontan surgery. 136,[140][141][142]146 Increased FVIII levels have been demonstrated to be associated with an increased risk for thrombosis. 15 Nowak-Gottl et al 144 reported 44 neonates and infants with increased levels of plasminogen activator inhibitor-1, indicating that fibrinolysis may be decreased in some children with CHD.…”

Section: Abnormalities In Hemostatic Proteins In Childrenmentioning

confidence: 99%

“…83 Several studies have described intrinsic and acquired alterations in thrombophilia factors such that single-ventricle patients may be predisposed to both bleeding and thrombosis. 140,141,146,[268][269][270] These alterations may be related to inflammation, ventricular dysfunction, lower cardiac output, higher venous pressures, nonpulsatile systemic venous flow, chamber dilatation, subclinical hepatic dysfunction, and lower systemic oxygenation. The role of these alterations in clinical decision making is unknown in terms of their contribution to risk and requirement for screening and management.…”

Section: Long-term Anticoagulation In the Patient With A Palliated Simentioning

confidence: 99%

See 4 more Smart Citations

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

Giglia¹,

Massicotte²,

Tweddell³

et al. 2013

Circulation

267

242

View full text Add to dashboard Cite

Section: Propensity To Coagulopathy In Children With Heart Diseasementioning

confidence: 99%

Section: Propensity To Coagulopathy In Children With Heart Diseasementioning

confidence: 99%

Section: Abnormalities In Hemostatic Proteins In Childrenmentioning

confidence: 99%

Section: Abnormalities In Hemostatic Proteins In Childrenmentioning

confidence: 99%

Section: Long-term Anticoagulation In the Patient With A Palliated Simentioning

confidence: 99%

See 3 more Smart Citations

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

Giglia¹,

Massicotte²,

Tweddell³

et al. 2013

Circulation

267

242

View full text Add to dashboard Cite

Current and future management of pediatric venous thromboembolism

Kerlin

2012

American J Hematol

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Venous thromboembolism (VTE) is an increasingly common complication encountered in tertiary care pediatric settings. The purpose of this review is to summarize the epidemiology, current and emerging pharmacotherapeutic options, and management of this disease. Over 70% of VTE occur in children with chronic diseases. Although they are seen in children of all ages, adolescents are at greatest risk. Pediatric VTE is associated with an increased risk of in-hospital mortality; recurrent VTE and post-thrombotic syndrome are commonly seen in survivors. In recent years, anticoagulation with low molecular weight heparin has emerged as the mainstay of therapy, but compliance is limited by its onerous subcutaneous administration route. New anticoagulants either already approved for use in adults or in the pipeline offer the possibility of improved dose stability and oral routes of administration. Current recommended anticoagulation course durations are derived from very limited case series and cohort data, or extrapolations from adult literature. However, the pathophysiologic underpinnings of pediatric VTE are dissimilar from those seen in adults and are often variable within groups of pediatric patients. Clinical studies and trials in pediatric VTE are underway which will hopefully improve the quality of evidence from which therapeutic guidelines are derived.

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Venous thromboembolism in chronic pediatric heart disease is associated with substantial health care burden and expenditures

Woods

Boulet

Texter

et al. 2019

Research and Practice in Thrombosis and Haemostasis

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Venous thromboembolism (VTE) is a known complication in chronic pediatric heart disease (CPHD). The effect of certain VTE risk factors on VTE and its health care burden in CPHD is unknown. VTE in CPHD is associated with significantly increased health care resource utilization. Recent cardiac or noncardiac surgery is a risk factor that infers the highest VTE risk in CPHD. Abstract BackgroundVenous thromboembolism (VTE) is a complication in children with chronic pediatric heart disease (CPHD). The influence of acute VTE risk factors and the health care burden associated with VTE in CPHD is unknown. MethodsChildren <18 years of age with a CPHD diagnostic code were identified from the 2003‐2013 MarketScan Commercial Databases. VTE diagnoses were identified either concomitantly with initial CPHD diagnoses or during a 6‐month follow‐up. The associations between demographic and clinical characteristics and VTE among children with CPHD, stratified by recent cardiac surgery, were assessed by multivariable logistic regression models. Estimates of health care utilization were compared using Wilcoxon rank‐sum tests. ResultsVTE events occurred in 957 of 120 884 children with CPHD (0.8%). In‐hospital mortality was significantly higher in children with VTE. Single‐ventricle physiology had the highest VTE rate (2.3%). All comorbid conditions were significantly associated with VTE, but the prevalence was highest in children with recent cardiac (11.1%) or noncardiac surgery (7.8%). The magnitude of association between noncardiac comorbidities and acquired acute cardiovascular conditions and VTE were larger for children without a recent cardiac surgery. Children with VTE had significantly higher health care utilization. ConclusionsVTE in CPHD is associated with significantly increased health care resource utilization and in‐hospital mortality. All of the comorbid conditions examined were significantly associated with VTE, but a recent surgical procedure, especially cardiac surgery, conferred the highest VTE risk. Although confounding inherently limits observational studies, these findings provide practical information about the health care costs among patients with CPHD and VTE.

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Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the fontan procedure

Cited by 84 publications

References 19 publications

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

Current and future management of pediatric venous thromboembolism

Venous thromboembolism in chronic pediatric heart disease is associated with substantial health care burden and expenditures

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