Patients with SM may experience cardiovascular symptoms

Up to 80% of individuals with mast cell activation disease report cardiovascular symptoms, though the association remains understudied.

A recent study published in Frontiers in Cardiovascular Medicine explores the cardiovascular symptoms that can occur in systemic mastocytosis (SM) and other mast cell conditions, and how they can be treated.

“Cardiologists and angiologists are frequently confronted with mast cell activation disease in their daily clinical practice, often without recognizing it,” the authors wrote.

Mast cells can be found in the heart and surrounding blood vessels. While no large-scale studies have assessed the occurrence of cardiovascular symptoms in patients with SM, up to 80% of individuals with mast cell activation disease are estimated to experience one or more of these symptoms.

In their review, the investigators highlight the main cardiac manifestations of mast cell disorders, primarily SM and mast cell activation syndrome, as well as potential treatment options.

For patients experiencing cardiac fibrosis, a condition in which scarring of the heart tissue occurs, the authors recommend mast cell stabilizing therapies.

Read more about SM signs and symptoms

In the case of abnormal heart rhythm, treatment depends on the subtype. While antihistamines can be administered for sinus tachycardia, more invasive treatments such as catheter ablation may be needed for ventricular tachycardia.

Heart failure has also been reported in some patients with SM, the authors explained. This could be due to fibrosis and other changes in heart function due to mast cell accumulation. Oftentimes, angiotensin-converting enzyme (ACE) inhibitors are prescribed for heart failure. However, the researchers caution that ACE inhibitors can worsen symptoms of SM and should generally be avoided. Sodium-glucose cotransporter 2 (SGLT2) inhibitors may be suitable for patients with SM, though.

Both low blood pressure and high blood pressure may also arise in some patients. Antihistamines can treat symptoms of mast cell-mediated low blood pressure. In a subset of patients, non-steroidal anti-inflammatory drugs may improve symptoms, too. Deciding on a therapeutic regimen for patients with SM and high blood pressure is more complicated, the authors explained. In many cases, trial and error must be used.

In addition to cardiac and blood pressure symptoms, vascular symptoms can arise in patients with SM. These include atherosclerosis, which may be treated with statins, and a variety of coronary syndromes.

Raynaud phenomenon is another vascular condition that occurs in around 10% of people with mast cells disorders, the authors estimated. However, there is very little research on the link these two disorders and what treatment options work best.

Lastly, the study describes clotting abnormalities, recommending tranexamic acid to prevent surgical bleeding complications. Protamine can be used in severe cases, but should be avoided when possible in patients with SM.

The investigators conclude by calling for further research to develop more standardized guidelines that address the cardiovascular symptoms of mast cell disorders.

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