Coagulation in Cyanotic Congenital Heart Disease

Wedemeyer, Anne L.

doi:10.1001/archpedi.1972.02110170034005

Cited by 36 publications

(29 citation statements)

References 17 publications

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“…Comparisons are also difficult because whole blood viscosity cannot be quantified as a single entity. Many studies were conducted in polycythemic subjects (8 -10), who, unlike cyanotic patients with secondary erythrocytosis, have elevated leukocyte and platelet counts that contribute significantly to hyperviscosity and symptoms (22,23). Our finding that iron deficiency does not elevate viscosity is in harmony with the current understanding of erythropoiesis and the general determinants of viscosity.…”

Section: Discussionsupporting

confidence: 70%

Blood Viscosity and its Relationship to Iron Deficiency, Symptoms, and Exercise Capacity in Adults With Cyanotic Congenital Heart Disease

Broberg

Bax

Okonko

et al. 2006

Journal of the American College of Cardiology

148

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Section: Discussionsupporting

confidence: 70%

Blood Viscosity and its Relationship to Iron Deficiency, Symptoms, and Exercise Capacity in Adults With Cyanotic Congenital Heart Disease

Broberg

Bax

Okonko

et al. 2006

Journal of the American College of Cardiology

148

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“…226 Prothrombin time, partial thromboplastin time, and activated partial thromboplastin time are abnormal in ≈20% and are related to hepatic congestion, abnormalities in the vitamin K-dependent coagulation factors, and depletion of von Willebrand multimers. 111,227,228 In Eisenmenger syndrome, bleeding often presents as epistaxis, which is typically manageable, but can also present as hemoptysis, which can be catastrophic. 212,229 A majority of patients with Fontan physiology have at least 1 abnormality in coagulation factors.…”

Section: Pathogenesismentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

180

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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“…Children with cyanotic congenital heart disease have an increased incidence of thrombocytopenia when the hematocrit level is above 65% [Wedemeyer et al, 1972]. Congenital heart disease is found in approximately 40-50% of children with DS, and only a small proportion of this is cyanotic heart disease.…”

Section: Platelets Thrombophilia and Bleeding Disordersmentioning

confidence: 99%

Clinical manifestations of hematologic and oncologic disorders in patients with Down syndrome

Dixon

Kishnani

Zimmerman

2006

American J of Med Genetics Pt C

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Hematologic abnormalities are common in individuals with Down syndrome (DS). Increased erythrocyte mean corpuscular volume (MCV) is frequently found among DS infants and remains elevated throughout life in two-thirds of patients, making interpretation of red cell indices for diagnosis of nutritional anemias or bone marrow failure disorders more challenging. Transient myeloproliferative disorder (TMD) associated with pancytopenia, hepatosplenomegaly, and circulating immature WBCs, is found almost exclusively in DS infants with an incidence of approximately 10%. In most cases, TMD regresses spontaneously within the first 3 months of life, but in some children, it can be life threatening or even fatal. Despite the high rate of spontaneous regression, TMD can be a preleukemic disorder in 20-30% of children with DS. The types of malignancy, response to therapy, and clinical outcome in children with DS are also unique. There is an increased risk of leukemia with an equal incidence of lymphoid and myeloid leukemia. Acute megakaryocytic leukemia (AMKL) subtype is the most common form of acute myeloid leukemia (AML) in this setting, and is uncommon in children without DS. Somatic mutations of the gene encoding the hematopoetic growth factor GATA1 have been shown to be specific for TMD and AMKL in children with DS. Myelodysplastic syndrome can precede AML. Children with DS and leukemia are more sensitive to some chemotherapeutic agents such as methotrexate than other children which requires careful monitoring for toxicity. Although the risk for leukemia is higher in individuals with DS, these patients have a lower risk of developing solid tumors, with the exception of germ cell tumors, and perhaps retinoblastoma and lymphoma.

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Coagulation in Cyanotic Congenital Heart Disease

Cited by 36 publications

References 17 publications

Blood Viscosity and its Relationship to Iron Deficiency, Symptoms, and Exercise Capacity in Adults With Cyanotic Congenital Heart Disease

Blood Viscosity and its Relationship to Iron Deficiency, Symptoms, and Exercise Capacity in Adults With Cyanotic Congenital Heart Disease

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Clinical manifestations of hematologic and oncologic disorders in patients with Down syndrome

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