Challenges in treating elderly patients with haemophilia: a focus on cardiology

Seventy years ago, the average life expectancy for patients with severe haemophilia A was less than 17 years. Today, due to the availability of safe and effective clotting factor concentrates, life expectancy is nearly normal, at least in patients without viral infections. More individuals are living into their 70s and 80s, acquiring a range of diseases that are common in elderly persons. One of the most important challenges includes the treatment of comorbidities, especially cardiovascular diseases. Although most evidence suggests that haemophilia, at least the severe manifestation, partially protects against myocardial infarction, stroke and venous thromboembolism, typical cardiovascular risk factors can still be present despite the clotting defect. Patients with haemophilia are equally or even more prone to obesity, hypertension, diabetes, and dyslipidaemia, and this is especially true for HIV-infected individuals using highly active antiretroviral therapy. The management of elderly haemophilia patients with cardiovascular comorbidities is hampered by a lack of evidence-based guidelines. Nevertheless, experience in treating cardiovascular disease is growing amongst the haemophilia community, and several authors have published their own recommendations for managing a variety of commonly encountered cardiovascular scenarios in haemophilia patients. Basic recommendations exist for risk-factor management, the adaptation of factor replacement therapy in the at-risk elderly, management of coronary revascularization, the management of acute coronary syndrome and atrial fibrillation. This review outlines our current knowledge about cardiovascular risk in elderly haemophilia patients, recommendations for clinical decision making, and our own experiences of managing individuals with coronary heart disease and atrial fibrillation.