Thrombo-hemorrhagic liability in children with congenital heart diseases

Shebl, Shebl Said; El-Shehaby, Walid Ahmed; Said, Yasmin Shebl; Darwish, Amira Hamed; EL-Fadaly, N.; Amer, Eman

doi:10.1016/j.hemonc.2017.07.001

Cited by 9 publications

(6 citation statements)

References 14 publications

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“…Additionally, CHD may be independently associated with abnormal hemostasis. In a study by Shebl, et al, 14) abnormal platelet counts, coagulation times, and selectin and platelet factor 4 levels were common in patients with CHD and constituted high-risk factors for both bleeding and thromboembolism. Similarly, Giang, et al 15) reported an approximately 8-fold increase in the relative risk of hemorrhagic stroke in children and young adults with CHD, compared to matched controls from the general population.…”

Section: Discussionmentioning

confidence: 98%

Spontaneous Spinal Epidural Hematoma in an Adult Patient with Complex Congenital Heart Disease

Ifuku

Nishiguchi

2021

Int. Heart J.

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Spontaneous spinal epidural hematoma (SSEH) is considered to be a relatively rare disease that can result in serious neurological sequelae. The pathogenesis and risk factors of SSEH are still unknown, and its differential diagnosis varies widely. Misdiagnosis with more common conditions such as stroke or aortic syndromes can occur. We report the case of a 27-year-old man who developed sudden upper back pain with no specific precipitant. Five days later, he visited our emergency department complaining of weakness in both lower limbs and dysuria. He had a history of intracardiac repair and a Blalock-Park procedure for an interrupted aortic arch and ventricular septal defect in infancy. Additionally, he had undergone an aortic root dilatation and aortic valve replacement at the age of 10 because of progression of aortic and supra-aortic stenosis and had received chronic anticoagulation and antiplatelet therapy with warfarin and aspirin, respectively. An emergency spine magnetic resonance imaging scan indicated a mass at the Th3-Th5 level with severe compression of the dural sac and the spinal cord. Emergency excision showed a spinal epidural hematoma. Mild postoperative gait disturbance and dysuria persisted, requiring rehabilitation and intermittent self-urethral catheterization. As patients with adult congenital heart disease have an increased risk of bleeding, they may be at risk of developing SSEH. However, this is the first report to describe such an association.

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Section: Discussionmentioning

confidence: 98%

Spontaneous Spinal Epidural Hematoma in an Adult Patient with Complex Congenital Heart Disease

Ifuku

Nishiguchi

2021

Int. Heart J.

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“…Many subsequent studies demonstrated the prolongation of clotting times in children with CHD. However, these studies demonstrated a more global impairment of the hemostatic profile, including decreased levels of FII, FV, FVIII, FIX, FX, and natural inhibitors of coagulation [4,[10][11][12][13][14][15][16][17].…”

Section: Discussionmentioning

confidence: 99%

“…The prolongation of PT in association with reduced FVII levels observed in our study indicates that a derangement towards hemorrhage is evident early in the course of the disease, even preoperatively. Shebl et al reported an increased liability both to hemorrhage and thrombosis in children with CHD, as demonstrated by thrombocytopenia, prolongation of clotting times, and increased levels of soluble markers of platelet and endothelial activation [17].…”

Section: Discussionmentioning

confidence: 99%

Coagulation Profile in Neonates with Congenital Heart Disease: A Pilot Study

Papadogeorgou,

Valsami,

Boutsikou

et al. 2024

Medicina

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Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.

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“…These patients are prone to both hypercoagulability and bleeding. Simple alteration of coagulation profiles may not accurately predict either event due to the complex dynamics influencing thrombosis and bleeding ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

Hematologic Derangements among Children with Unoperated Cyanotic Congenital Heart Disease in Ethiopia

Selamawit Alemseged,

Endale Tefera

2023

Ethiop J Health Sci

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BACKGROUND: Surgical treatment has transformed the course and outcome of congenital heart defects in high-income countries, but children with congenital heart diseases in sub-Saharan Africa, where access to cardiac surgery is limited, often experience the natural course of untreated lesions and their complications. The objective of this study was to determine the prevalence of hematologic derangements among Ethiopian children with unoperated cyanoticcongenital heart diseases, to identify factors associated with coagulopathy in this population, and to describe how these complications are managed in this setting. METHODS: In this single-center cross-sectional study, we prospectively collected clinical and demographic data from children (<18 years) with cyanotic congenital heart diseases. Blood samples were collected to measure hematologic parameters. Polycythemia was defined as hematocrit >50% and thrombocytopenia as <150,000 per microliter. RESULTS: Among 70 children recruited, the overall prevalence of polycythemia and thrombocytopenia was 63% (n=44) and 26% (n=18), respectively. On multivariate logistic regression analysis, hematocrit ³65% (p-value=.024), and oxygen saturation <85% (p-value=.018) were independently associated with moderate or severe thrombocytopenia. Thirty-one (44%) patients had undergone therapeutic phlebotomy, and 84% (26/31) of these patients received iron supplementation. CONCLUSION: We report a high prevalence of polycythemia and thrombocytopenia in Ethiopian children with untreated cyanotic congenital heart diseases. There was variable implementation of iron supplementation and therapeutic phlebotomy, highlighting the need to optimize supportive management strategies in this population to mitigate the risk of life-threatening complications.

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Thrombo-hemorrhagic liability in children with congenital heart diseases

Cited by 9 publications

References 14 publications

Spontaneous Spinal Epidural Hematoma in an Adult Patient with Complex Congenital Heart Disease

Spontaneous Spinal Epidural Hematoma in an Adult Patient with Complex Congenital Heart Disease

Coagulation Profile in Neonates with Congenital Heart Disease: A Pilot Study

Hematologic Derangements among Children with Unoperated Cyanotic Congenital Heart Disease in Ethiopia

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