Presentation, management and outcomes of thrombosis for children with cardiomyopathy

McCrindle, Brian W.; Karamlou, Tara; Wong, Herbert S.; Gangam, Nirupama; Trivedi, Kalyani R.; Lee, Kyong Jin; Benson, Lee

doi:10.1016/s0828-282x(06)70937-0

Cited by 27 publications

(36 citation statements)

References 31 publications

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“…577 A series of 66 patients with dilated cardiomyopathy reported a prevalence of thrombosis of 14%. 578 There are no studies of anticoagulant prophylaxis in pediatric patients. However, based on adult studies and the apparent risk of PE and stroke in children with cardiomyopathy, primary prophylaxis with warfarin (target INR, 2.5; range, 2.0-3.0) often is used.…”

Section: Dilated Cardiomyopathymentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

Chest

1,236

559

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Section: Dilated Cardiomyopathymentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

Chest

1,236

559

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“…Of the 402 patients included in these studies, 16% had an intracardiac thrombus and 8% experienced an embolism. Most of the embolisms reported appeared to be arterial events, with 60% of all embolic events being cerebral [4,[9][10][11][12]14]. Intracardiac thrombosis appears to be a significant risk factor for embolism considering that, on average, half of those with an intracardiac thrombus experienced a subsequent embolism.…”

Section: Incidencementioning

confidence: 99%

“…In the reviewed literature, the mean fractional shortening of the group with thrombotic and embolic complications was frequently under 15% [4,11,12,14]. The mean left ventricular ejection fraction (LVEF) of the group that developed intracardiac thrombus was usually under 25% at the time of presentation; however, few studies were able to find statistically significant differences between the LVEFs of the groups with and without thrombotic complications [10,11,14]. This may be due to small sample sizes.…”

Section: Risk Factorsmentioning

confidence: 99%

Thrombosis and embolism in pediatric cardiomyopathy

Chen

Williams

Chan

et al. 2013

Blood Coagulation &Amp; Fibrinolysis

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The management of cardiomyopathy in pediatric patients is complicated by the risk of cardiac-associated embolism. This review examines the incidence, risk factors, and treatment of embolism in dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM), and noncompaction of the left ventricular myocardium (NLVM) in children. The reported incidence of embolism for DCM ranges from 1 to 16%. Left ventricular ejection fraction below 25% or fractional shortening below 15% are major risk factors for intracardiac thrombus formation in this group. The risk of embolism for RCM ranges from 12 to 33%. Atrial dilation is considered the major risk factor. The reported incidence of embolism for NLVM ranges from 0 to 38%, with most studies indicating an absence of detectable thrombus or embolus. Severe systolic dysfunction exacerbates the risk of embolism in this group. On the basis of these risk factors, we propose an algorithm for the management of embolism in these groups of patients.

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“…[280][281][282][283] There are no similar prospective data available in children with systolic ventricular dysfunction, and the true risk of thromboembolism is unknown, regardless of NMD status. [284][285][286][287][288][289][290][291] It is well established that thrombosis prevention is indicated in adults with AF/atrial flutter in the absence of ventricular dysfunction, and extremely detailed riskbased guidelines for anticoagulation in AF/atrial flutter exist 205 ; however, data in children and those with NMDs are lacking. A study of arrhythmias in NMD described AF/atrial flutter in 139 patients with laminopathy (lamin A/C), MFM, DM1 and DM2, DMD, BMD, EDMD, LGMD, or facioscapulohumeral muscular dystrophy.…”

Section: Anticoagulationmentioning

confidence: 99%

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Feingold¹,

Mahle²,

Auerbach³

et al. 2017

Circulation

211

195

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For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes. N euromuscular diseases (NMDs) encompass a broad spectrum of diagnoses with overlapping but distinct phenotypes. Common to many NMDs is cardiac involvement. Although the past 3 decades have seen marked advances in our understanding of many NMDs, significant gaps in knowledge remain on how best to approach cardiac care in these patients. For example, survival in Duchenne muscular dystrophy (DMD) has been extended through the use of glucocorticoid use and respiratory support, yet cardiac complications remain a significant cause of morbidity and mortality.1-3 To achieve further gains in care, we will need to improve our understanding of the pathophysiologies driving cardiac involvement in NMDs and advance treatments aimed at preventing the progression of heart failure (HF) and sudden death in NMDs. The recently published findings of an expert working group on cardiac involvement in DMD, which outlined key gaps in knowledge and made specific recommendations aimed at improving diagnosis and management, are a step toward this goal. 4 In this statement, we include a comprehensive overview of the major categories of NMDs with cardiac involvement. For each, a brief background of the gene defect(s), common clinical manifestations (particularly cardiac findings), and current therapies is summarized. Gaps in knowledge are highlighted, and where possible, clinical treatment suggestions are made by the expert writing group appointed by the American Heart Association to review the available literature. Selection of the writing group was performed in accordance with the American Heart Association's conflict-of-interest management policy. Participants volunteered to write sections relevant to their expertise and experience. CLINICAL STATEMENTS AND GUIDELINESconducted a general search of the literature, restricted to human subjects research published between 1980 and 2016. Drafts of each section were written and sent to the chair of the writing group for incorporation into a single document, which was then edited. The edited document was discussed electron...

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Presentation, management and outcomes of thrombosis for children with cardiomyopathy

Cited by 27 publications

References 31 publications

Antithrombotic Therapy in Neonates and Children

Antithrombotic Therapy in Neonates and Children

Thrombosis and embolism in pediatric cardiomyopathy

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

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