Cardiovascular symptoms of MCAS

MCAS patients often have a number of cardiovascular symptoms.  In true mast cell disease fashion, these symptoms often represent both ends of the spectrum.
Heart palpitations are the most common cardiac complaint, with true rhythmic abnormalities being fairly rare.  Tachycardia is also very common, but occasionally slow heart rate (bradycardia) is reported.  In bradycardic patients, no obvious cause for this can be identified.  Both low and high blood pressure can be seen, many times in the same patient, sometimes even following one after the other in a short period of time.  These changes in blood pressure often have no clear trigger.
True syncope (fainting) is uncommon in MCAS, but presyncope (lightheadedness, weakness, dizziness or vertigo) affects the majority of patients.  These presyncope episodes can be distinct from POTS symptoms, and may not be related to position.  Some patients experience as many as several episodes a day.  When tested for POTS with tilt table, MCAS patients may or may not be positive.  However, when treated for POTS, mast cell patients in general only see mild reduction in their presyncope episodes, with little improvement in their other symptoms.
MCAS patients often complain of chest pain, which may or may not reveal ECG abnormalities.  This type of pain is generally localized specifically to the chest and does not radiate down the arm.  Chest pain must be carefully evaluated due to the potential for two rare cardiac syndromes.  Additionally, mast cell disease can indirectly cause congestive heart failure by the long term action of histamine. 
Takotsubo syndrome, or stress-induced cardiomyopathy, is caused by sudden weakening of the myocardium that causes ballooning of the left ventricle.  It can cause acute heart failure, ventricular arrhythmias, and acute heart failure.  Angiography shows that there is no coronary artery defect to explain the left ventricular abnormalities.  If the patient survives, the left ventricle typically returns to normal after about eight weeks.  This does not occur as a result of an allergic reaction, but is sometimes seen in patients with idiopathic anaphylaxis.  In 75% of patients, serum catecholamines are elevated, a finding sometimes seen in MCAS patients.  Due to severe emotional stress frequently being the trigger for the cardiac event, Takotsubo syndrome is also known as broken heart syndrome.
Kounis syndrome is also known as allergic angina or allergic myocardial infarction.  In these patients, there are no obstructive lesions in the coronary artery.  Patients suffer severe chest pain or heart attack as an extension of an allergic reaction.  Kounis syndrome is caused by mast cell activation causing vasospasm of the coronary artery.  It is not known if the mast cells effecting this pathology are normally developed mast cells or improperly developed, such as seen in mastocytosis and MCAS.  This syndrome accounts for about 0.002% of all acute heart attacks.  (An in depth post on Kounis syndrome is on the way.)
MCAS patients often experience coronary and peripheral atherosclerosis.  Some have pain due to narrowing of the vessels.  Sclerosis and poor healing is seen in many MCAS patients.  Due to the importance of mast cells in angiogenesis, long term mast cell activation can contribute to aneurysms, hemorrhoids, varicosities, hemangiomas, arteriovenous malformations and telangiectasias. 
Edema is a common finding.  Most MCAS patients who have edema have no heart abnormalities and do not have pitting edema, indicating that the edema is likely not from heart disease.  MCAS patients often have widespread edema that can shift to different parts of the body.  There is usually no detectable low albumin.  This is thought to be due to third spacing. 

Afrin, Lawrence B. Presentation, diagnosis and management of mast cell activation syndrome.  2013.  Mast cells.
Molderings GJ, Brettner S, Homann J, Afrin LB. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J. Hematol. Oncol.2011; 4:10-17.
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5 Responses

  1. sabrina November 2, 2015 / 4:55 pm

    Hello; great article. Do you recommend a specific type of doctor to go for testing for mast cell?

  2. Anthony March 15, 2016 / 9:32 pm

    Hey Lisa

    I wrote an email to you about this issue awhile ago inquiring about the type of edema that’s associated with mast cell disorder. I don’t think it’s true that the third spacing/edema in mast cells disorders is always the non pitting type. According to all of the professional I have spoken with the third spacing/edema associated with mast cell disorders can indeed be a pitting type edema, especially in the lower limbs . I am currently under the care of an immunologist out of Mt Sinai for suspected mast cell disorder and I have been dealing with chronic pitting lower leg and non pitting generalized edema for years. I have no heart, kidney or liver disease or protein deficiency issues, either. The pitting in the lower legs just means that the fluid is rich in protein. I get very dehydrated from the fluid retention as well (hypovolemia)

    • Lisa Klimas March 18, 2016 / 3:29 pm

      Hi, Anthony,

      Thanks for commenting. You are correct that protein concentration is a factor on the quality of edema. Pitting edema is well associated with cardiovascular disease, whereas other types of edema, such as histamine driven, idiopathic non-histamine driven, and hereditary angioedema, generally have a non-pitting quality. The reason for histamine/allergic edema being non-pitting is that the main immediate effect is to increase movement of fluid out of the bloodstream and into the tissues, but it doesn’t immediately allow large molecules to move into the tissues. In this scenario, the edema would not be pitting.

      However, I do think in a more chronic situation where mediator release is allowing larger molecules through, that it could eventually develop a pitting component. I am only aware of this happening in a few instances, and in those instances, the patients had hypertension and other cardiovascular issues.

      Out of curiosity, do you also have POTS? Thanks for this information. I will keep my eyes open for more information about this.


      • Anthony March 18, 2016 / 5:03 pm

        Well, I have quite a few ongoing health issues, the main one being generalized edema, most likely due to permeable capillaries but my immunologist hasn’t figured out a cause yet. She is suspecting that it is an inflammatory type response (possibly masto?) but can’t seem to get to the bottom of it. I actually become very dehydrated when the edema is severe and my blood pressure is low. I basically “third space” fluids throughout my body from head to foot but I don’t have ascites or anything like that. The edema pits/indent in my lower legs but nowhere else. I’ve been screened very thoroughly for heart, liver, kidney disease and protein losses and everything turns up normal. I’ve been struggling with this edema for years. It waxes and wanes but is always an issue with me.

        It all started after I got sick with gi issues. Short of the long is that I wound up seeing my current immunologist who diagnosed me with an igg-1 (an antibody) deficiency. I’d been running total white blood cells counts in the 15-20k range, after I got sick, but nobody could figure out what was going on. She put me on a broad spectrum antibiotic and my wbc count dropped to 6k and I lost about 20 pounds of fluid. I am still dealing with fluid retention issues but it’s improved markedly since the antibiotic therapy so it is likely related to some type of systemic inflammatory process that is causing my capillaries to become permeable/leaky.

        My blood pressure has always been on the low side but has become even lower since the edema started. Just based on subjective feelings and symptoms, it seems like some fluid from my vascular system is leaking into spaces beneath my skin, where it doesn’t belong, so even though I am retaining extra fluid, I am actually depleted/dehydrated intravascularly (very dry mucous membranes, dry mouth, decreased perspiration, poor skin turgor, etc) . That’s all I know at the moment.

        As far as I know I don’t have POTS. I don’t really experience any of those orthostatic type symptoms

  3. Anthony March 15, 2016 / 9:40 pm

    *meaning that the fluid isn’t rich in protein

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