Junior comment:

The recent clinical consensus statement1 published in the European Heart Journal – Cardiovascular Pharmacotherapy offers a valuable framework for clinicians navigating the complexities of managing cardiovascular (CV) diseases in patients with congenital bleeding disorders (CBDs).

Safe clotting factor replacement therapies have extended the life expectancy of patients with CBDs, leading cardiologists to more frequently encounter these high-risk individuals with age-related CV conditions such as atrial fibrillation (AF), acute and chronic coronary syndromes, and valvular heart disease. In this aging population with CBDs, the management of CV comorbidities presents a significant clinical challenge. Antithrombotic therapy decisions are particularly complex, requiring careful evaluation of treatment intensity and duration, selection of appropriate agents, and consideration of the individual’s bleeding risk and clinical phenotype. Bleeding disorders vary in severity, with milder forms often associated with a higher thrombotic risk and more severe forms dominated by bleeding risk and recognizing this spectrum is essential when making decisions regarding cardiovascular prevention or treatment. Moreover, improved risk stratification tools tailored to bleeding disorders are urgently needed, as current scoring systems such as CHA₂DS₂-VASc may not adequately reflect their unique risk profiles2.

First, CV prevention remains a priority with careful management of modifiable CV risk factors. Subsequently, close collaboration with a hematologist is essential for all therapeutic decision steps including the assessment of clotting factor levels, platelet count, and evaluation of platelet function using methods such as platelet aggregometry and flow cytometry to characterize the bleeding disorder. Therefore, the use of antiplatelet therapy should be evaluated on a case-by-case basis.

From a cardiologist’s perspective, optimizing percutaneous coronary intervention techniques is critical in minimizing bleeding risk. Strategies such as radial access, intravascular imaging, and the use of new-generation drug-eluting stents help reduce the incidence of stent thrombosis and support the adoption of very short-duration dual antiplatelet therapy (typically one month)1, which is especially advantageous in patients at high bleeding risk. Similarly, in patients requiring valve intervention, minimally invasive procedures, transcatheter approaches, or valve repair should be favored over open-heart surgery when feasible. Bioprosthetic valves are preferred to lower both perioperative and long-term bleeding risks3. However, robust data from large-scale studies are still needed to validate the safety and efficacy of these approaches in patients with CBDs.
The potential protective effect of congenital bleeding disorders (CBDs) against thrombosis remains a subject of debate. Existing data on the prevalence of atherothrombosis in this population are inconsistent, with studies reporting lower, similar, or even higher rates compared to the general population. Prospective, large-scale studies are needed to clarify the true incidence of thrombotic and bleeding events in individuals with CBDs. Moving forward, close interdisciplinary collaboration among cardiologists, hematologists, and thrombosis specialists will be critical for developing individualized treatment strategies. The integration of genetic and phenotypic profiling may further enhance risk stratification and therapeutic decision-making, advancing the field toward truly personalized care for this high-risk group.

Senior comment:

This consensus document contributes to an increased understanding of rare congenital bleeding disorders (CBDs). The tables in the paper are helpful and describe in detail the frequencies, as well as the bleeding and thromboembolic risks, for patients with primary hemostasis defects and the rarer CBDs affecting specific coagulation factors. The authors provide practical advice on how to manage antithrombotic treatment in these patients to balance the risks of bleeding and thrombosis. They emphasize collaboration with hematologists experienced in bleeding disorders to individualize antithrombotic treatment and improve outcomes. Advances in the primary treatment of bleeding disorders have increased early survival, and as a result, patients with CBDs are becoming more common in clinical cardiology practice.