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Study Focuses on Cardiovascular Disease in Hemophilia Patients

July 20, 2015
Study Focuses on Cardiovascular Disease in Hemophilia Patients

As life expectancy in people with hemophilia (PWH) continues to rise closer to the national average, hemophilia healthcare providers have grown increasingly interested in the conditions most commonly linked to aging. One of the more pervasive of these is cardiovascular disease (CVD), and associated conditions such as ischemic heart disease (hardening of the arteries) and atrial fibrillation (irregular heartbeat rate/rhythm). A multidisciplinary team of investigators conducted a scan and review of medical literature associated with CVD in PWH published between 1980-2013. Their findings, “Consensus Review of the Treatment of Cardiovascular Disease in People with Hemophilia A and B,” were published in the March/April 2015 issue of the journal Cardiology in Review.

The lead author of the review was Victor Ferraris, MD, PhD, Tyler Gill Professor of Surgery, Division of Cardiovascular and Thoracic Surgery at the University of Kentucky in Lexington. Ferraris and his coauthors acknowledged that data relevant to CVD in PWH is limited. That’s because of the low numbers of hemophilia patients who have been documented with complications related to heart disease. The result is a lack of evidence-based guidelines from which to base treatment decisions.

“Accordingly, current recommendations for the medical and surgical management of common cardiovascular conditions in PWH derive from anecdotal experience and expert opinion. Most recommendations reflect guidelines and common practices for people without hemophilia,” said Ferraris. “Ultimately, the rigorous, systematic investigation of management strategies for many cardiovascular conditions is unobtainable, given the relative rarity of hemophilia and even smaller numbers of PWH with any given cardiovascular condition.”

However, Ferraris and colleagues did arrive at some conclusions. An examination of the literature suggested that low levels of factor VIII or IX did not necessarily offer hemophilia A or B patients extra protection against CVD conditions, including  ischemic heart disease. In fact, the authors anticipate that older PWH will experience CVD rates comparable to the general population. Investigators added that recommendations relevant to the medical/surgical management of CVD in the aging PWH will be largely comparable to what is recommended for unaffected patients, as long as factor levels remain high enough to ensure adequate control of bleeds. They also acknowledged that the presence of an inhibitor to infused factor VIII or IX will complicate treatment and management in PWH/CVD considerably. The authors concluded that close collaboration between cardiology specialists and the comprehensive care team is crucial for quality clinical management.

“As the population of PWH ages, cardiovascular healthcare providers will encounter increasing numbers of PWH presenting with typical age-related cardiovascular conditions, in addition to other acquired or congenital conditions spanning all ages,” reported the authors. “To optimize resource utilization and clinical outcome, and to minimize bleeding risk and complications, close consultation with a hematologist, ideally in association with a hemophilia treatment center, is essential.”

Source:, June  29, 2015