Prevalence and risk factors for heart disease among males with hemophilia

Department of Pediatrics and Human Development, Michigan State University, East Lansing, Michigan, USA.
American Journal of Hematology (Impact Factor: 3.8). 05/2005; 79(1):36-42. DOI: 10.1002/ajh.20339
Source: PubMed


There have been conflicting reports in the literature about the protective effect of hemophilia on the occurrence of ischemic heart disease (IHD). Circulatory disease has been reported as the second most common cause of death in persons with hemophilia in the United States. In addition to diabetes and hypertension, high levels of FVIII, as may occur during factor concentrate infusions, may increase IHD risk in this population. To estimate the prevalence of heart disease and examine factors associated with IHD and other heart diseases among persons with hemophilia, we analyzed data collected from the medical records of 3,422 males with hemophilia living in six U.S. states from 1993 to 1998. Heart disease cases were ascertained from among 2,075 persons who were hospitalized at least once during the 6-year period. Of these, 48 were diagnosed with IHD and 106, with other types of heart disease. The age-specific prevalence of IHD ranged from 0.05% in those under 30 years to 15.2% in those 60 years or older. Hospital discharge rates in males with hemophilia with IHD and other types of heart disease were lower compared to rates in age-matched U.S. males. In our cohort, as in the general population, IHD was independently associated with age, hypertension, diabetes, and hyperlipidemia. Other heart diseases were associated with HIV infection, hypertension, hemophilia B, and diabetes. In summary, persons with hemophilia have unique risk factors such as infusion of factor concentrates and infection with HIV that may predispose them to heart disease as their life expectancy increases.

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    • "[2]. Other studies also report lower risks of ischemic heart disease in haemophilia patients than in the general population, based on mortality rates [1] [3] [4] and in one study on hospital discharge data [5]. As expected, the study by Darby et al. reported an increased mortality due to bleeding episodes and hepatitis C related problems. "
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    ABSTRACT: With increasing life expectancy, more haemophilia patients will be confronted with age-related problems. To ensure optimal care, it is important to know the occurrence of both fatal and non-fatal cardiovascular disease, malignancies and other types of co-morbidity in these patients. Our aim was to retrospectively assess the occurrence of co-morbidity and causes of death in a substantial birth-cohort of haemophilia patients. Data on all types of co-morbidity were collected from medical records of 408 haemophilia patients (204 severe, 204 non-severe) born before 1971, and compared with the Dutch age-matched general male population. Ten patients had 11 myocardial infarctions, none of which were fatal. The cumulative incidence of non-fatal myocardial infarction was significantly lower in patients with severe haemophilia than in the general population (0.5% versus 4.8%), but was not decreased in patients with non-severe haemophilia (4.4%). Intracranial bleeding occurred significantly more often in haemophilia patients. The occurrence of non-virus related malignancies, and other non-virus related co-morbidities was similar in haemophilia patients and the general population. HIV infection was present in 12% of patients, and hepatitis C infection in 56%. Seventy-eight patients (19%) were deceased. Main causes of death were malignancies, AIDS, hepatitis C, and intracranial bleeding. Our results showed a decreased occurrence of myocardial infarction in patients with severe haemophilia, suggesting a protective effect of very low clotting factor levels on thrombotic cardiac events. No differences were found between haemophilia patients and the general population in the occurrence of any other type of non-virus related co-morbidity.
    Thrombosis Research 12/2011; 130(2):157-62. DOI:10.1016/j.thromres.2011.12.012 · 2.45 Impact Factor
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    • "While cardiovascular disease (CVD) has been the primary cause of death among individuals in the US for the past eight decades [26], several studies have reported that mortality from cardiac events among individuals with hemophilia is lower than in the general population [7, 8, 13, 27, 28] and hemophilia may actually have a protective effect against CVD [4, 11, 28]. The prevalence of CVD among patients between 40 and 59 years of age in this study was similar compared to the age-matched US male population (40% versus 39%), but slightly lower among those patients of 60 years of age or greater (69% versus 71%). "
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    ABSTRACT: Introduction. Advances in hemophilia care and treatment have led to increases in the life expectancy among hemophiliacs. As a result, persons with hemophilia are reaching an older age and experiencing various age-related health conditions never seen before in this population. Aim. To determine the prevalence of comorbidities among middle-aged and elderly hemophilia A and hemophilia B patients. Methods. Retrospective chart review among all hemophilia patients, who attended the Gulf States Hemophilia and Thrombophilia Center. Results. All patients had at least one comorbid condition other than hemophilia, and the majority had between 3 and 6 comorbidities. The most common conditions identified were chronic hepatitis C, hypertension, HIV, chronic arthropathy, and overweight/obesity. Conclusions. Since persons with comorbidities are more likely to have poorer health outcomes and require greater care in managing their health needs, caring for aging hemophiliacs is likely to pose various social and economic challenges for both patients and providers.
    Journal of aging research 09/2011; 2011(1):985703. DOI:10.4061/2011/985703
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    • "With this as background , one must be aware that cardio - vascular events are increasingly recognised in hemophilias , probably because more and more patients are reaching older age . Kulkarni et al ( 2005 ) "
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    ABSTRACT: The life expectancy and quality of life of persons with haemophilia has dramatically increased since the 1970s, with the exception of the increased rate of deaths observed during the 1980s and the 1990s due to blood-borne viral infections. Improvements of factor replacement therapy, treatment of infectious diseases and comprehensive health care provided by specialised haemophilia centres are the main determinants of the increasing age of the haemophilia population. As a consequence, a growing number of these patients develop age-related co-morbidities, such as cardiovascular disease and cancer. The care of these previously rare conditions is a new challenge for caregivers in haemophilia centres. This review focuses on co-morbidities in the ageing haemophilia patients, their impact on quality of life and their complex management.
    British Journal of Haematology 12/2009; 148(4):522-33. DOI:10.1111/j.1365-2141.2009.08005.x · 4.71 Impact Factor
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