Findings from the federally funded Women’s Ischemia Syndrome Evaluation (WISE) study — a landmark investigation into ischemic heart disease (meaning reduced blood supply to the heart muscle) – are helping us to understand that, as the Harvard Women’s Health Watch puts it: heart disease – like cancer – is not one, but several disorders.
While I was at Mayo Clinic shortly after my heart attack, I also learned that at least two of these disorders are far more commonly seen in women than in men’s “Hollywood heart attacks”. These two heart conditions are coronary microvascular disease (MVD) and coronary artery spasm (CAS).
For example, these also happen to be the diagnoses delivered to an Australian woman who suffered a serious heart attack at age 51 – despite having no identifiable cardiac risk factors.
It took her a year after surviving that heart attack before she was finally appropriately diagnosed* with a coronary artery spasm disorder sometimes known as Prinzmetal’s variant angina, and then another two years before she received a further diagnosis of coronary microvascular disease (MVD) – missed because most standard coronary artery tests are designed to identify only obstructive heart disease in major coronary arteries.
CAROLYN’S NOTE: After my heart attack in 2008, I too was subsequently diagnosed with MVD following a second trip to the cath lab ruled out “stent failure” (gotta love that terminology!) See also: My Love-Hate Relationship With my Little Black Box
Frustrated with the lack of awareness of these conditions by many of the medical staff that she had encountered during this time, the Australian woman set out to gather a solid list of credible journal references about these under-appreciated conditions in women to share with other women, and with her health care providers. As she explained:
“At my last visit to our local hospital, I suspected that all the nurses were treating me as if I had an anxiety disorder, even though my medical chart clearly indicated Prinzmetal’s variant angina. Some staff were very gentle and some were extremely rude. Some ignored my requests for chest pain relief and some were helpful.
“Overall, it was a really bad experience. I decided that I would have to do something about it. So after cooling down, I put together some very relevent material for ‘the powers that be’ in the hope that the hospital staff may actually be educated about coronary artery spasm (CAS) and microvascular disease (MVD).
“To be honest, I hold out little hope that anything will change, but at least I can say to myself that I have made a genuine effort to better my situation.”
To help her better her situation – and especially that of other women whose debilitating symptoms may have been blown off by uninformed medical staff because of “normal” cardiac test results – I asked her permission to republish her comprehensive resource list here.
If you (like this Australian survivor and I both experienced) had “normal” cardiac test results during what turned out to be a heart attack, please print off her resource list below and share it with your friendly neighbourhood physicians and nurses:
UPDATE November 2019: Diagnostic tools used to identify coronary microvascular dysfunction
UPDATE March 2021: International Heart Spasm Alliance website
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Negative Stress Test Does Not Rule Out MVD – American Heart Association: Circulation: Cardiovascular Interventions
“The presence of a negative non-invasive stress test does not rule out coronary vasomotor dysfunction (MVD) in symptomatic patients with non-obstructive coronary artery disease.”
Andrew Cassar, M.D., M.R.C.P.(U.K.), Panithaya Chareonthaitawee, M.D., F.A.C.C., Charanjit S. Rihal, M.D., F.A.C.C., Abhiram Prasad, M.D., F.R.C.P., F.A.C.C., Ryan J. Lennon, M.S., Lilach O. Lerman, M.D., Ph.D., and Amir Lerman, M.D., F.A.C.C.
Standard Tests Don’t Detect Microvascular Disease – Harvard Women’s Health Watch
“Heart disease, like cancer, is not one, but several disorders. Discoveries from the WISE study suggest that many women have a form of Heart disease called coronary microvascular dysfunction (MVD) that isn’t detected by standard diagnostic procedures and thus goes unrecognised and untreated.”
“The research, led by Dr. Noel Bairey Merz at Cedars-Sinai Medical Center in Los Angeles, is laying the groundwork for better ways to evaluate and treat women with heart disease.”
“Because small vessel disease can make it harder for the heart to pump blood to the rest of the body, small vessel disease can cause serious problems if left untreated, such as coronary artery spasm, heart attack, sudden cardiac death, heart failure.”
MayoClinic.com, Small Vessel Disease
Coronary Microvascular Disease is Difficult to Diagnose – PBS Second Opinion transcripts
“Coronary microvascular disease is an elusive disease. It doesn’t show up on many standard tests. MVD is a disease that’s just as dangerous as coronary artery disease, and it increases the risk of heart attacks and heart failure. The characteristics of microvascular dysfunction include:
no obvious blockages but blood flow to the heart is still low;
vessels that don’t expand or dilate properly during physical or emotional stress; vessels that spasm abnormally.
“Diagnosis remains the biggest hurdle for people with coronary MVD.”
Louis J. Papa, MD, FACP, Kathy Kastan, LCSW, MAEd Kim Kachmann-Geltz, Noel Bairey Merz, MD, Matthew Burg, PhD, Gladys Velarde, MD
Coronary Spasm–Induced Non–ST‐Segment Elevation Acute Coronary Syndrome – Journal of the American Heart Association
“The study showed frequent involvement of coronary spasm in the pathogenesis of Non-ST Elevation Myocardial Infarction- Acute Coronary Syndrome (NSTE-ACS). Variant angina was observed in one third of patients with spasm‐induced NSTE‐ACS. Compared with the obstructive group, patients of the spasm group were younger and were less likely to be hypertensive or to have dyslipidemia, diabetes, or previous myocardial infarction. Coronary spasm should be considered even in patients with fewer coronary risk factors and non-obstructive coronary arteries.”
Koichi Kaikita et al. J Am Heart Assoc. 2014; 3: e000795
Vasospastic Angina and Coronary Microvascular Spasm – Journal of the American College of Cardiology
“Coronary microvascular spasm causes myocardial ischemia in patients with vasospastic angina. The result suggests that coronary microvascular disease may also contribute to angina in patients with “vasospastic angina (CAS).”
Hongtao Sun, MD; Masahiro Mohri, MD, PhD; Hiroaki Shimokawa, MD, PhD; Makoto Usui, MD; Lemmy Urakami, MD; Akira Takeshita, MD, PhD
Coronary Artery Spasm and Coronary Microvascular Spasm – Cardiovascular Sciences Research Centre
“Microvascular angina affects a large number of people. These patients have typical chest pain and some evidence for myocardial ischemia, but the coronary arteries are absolutely clean. Many of these patients are considered to have non-cardiac problems, who are then ‘reassured’ that there is nothing wrong with the heart. They are sent back home with the same problem that they came with.
“It is an important condition to be taking into consideration. There is a connection between coronary artery spasm and microvascular angina, particularly when the microvascular angina patients develop coronary microvascular spasm.”
Professor Juan-Carlos Kaski, Head of the Cardiovascular Sciences Research Centre and the Microvascular Angina Clinic, St. George’s University of London, U.K.
The ACOVA Study: Abnormal COronary VAsomotion in patients with stable angina and unobstructed coronary arteries – Journal of the American College of Cardiology
“Nearly 50% of patients undergoing diagnostic angiography for assessment of stable angina had angiographically normal or near normal coronary arteriograms. The ACH test triggered epicardial or microvascular coronary spasm in nearly two-thirds of these patients.”
Ong P, Athanasiadis A, Borgulya G, Mahrholdt H, Kaski JC, Sechtem U. Robert-Bosch-Krankenhaus, Department of Cardiology, Stuttgart, Germany.
Coronary Artery Spasms Often Occur At Rest – Mayo Clinic
“A coronary artery spasm is a brief, temporary tightening (contraction) of the muscles in an artery wall in your heart. This can narrow and decrease or even prevent blood flow to part of the heart muscle. If the spasm lasts long enough, it can lead to chest pain (angina) and possibly a heart attack (myocardial infarction). These spasms may also be referred to as Prinzmetal’s angina or variant angina. Unlike typical angina, which usually occurs with physical activity, coronary artery spasms often occur at rest.
“Only about 2 percent of angina cases are Prinzmetal’s angina. Coronary artery spasms are more common in people with risk factors for heart disease, but the spasms also happen in people who have no risk factors. Coronary artery spasms can also occur in people who have conditions that affect their immune systems, such as lupus.”
Martha Grogan, M.D. Mayo Clinic cardiologist
Joan Jahnke Shares Her Story – Emory University
“Joan Jahnke, a patient of Dr. Habib Samady (Professor of Medicine at Emory University School of Medicine and Director of Interventional Cardiology at the Emory Heart and Vascular Centre), shares her journey through cardiac microvascular endothelial dysfuntion with vasospastic angina:
“A big frustration with many MVD patients is that our complaints and symptoms often bring us to an urgent or emergent care setting where none of our signs show on any EKG, echocardiogram, ultrasound or treadmill stress tests.
“We look well, present with chest pain and shortness of breath – perhaps anxious – but the tests are generally negative, even the cardiac enzymes. We repeatedly return seeking help, knowing that it is our heart, but we repeatedly fail the best current technology tests.”
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* Diagnosing non-obstructive coronary artery disease:
If a physician is not familiar with coronary artery spasm or coronary microvascular disease, it’s often unlikely that additional cardiac tests will be ordered once conventional EKGs, stress tests or cardiac enzyme blood tests are interpreted as “normal” in the Emergency Department.
Coronary Artery Spasm is explained by Mayo Clinic cardiologists as a temporary tightening (contraction) of the muscles in a coronary artery wall in your heart. This can narrow and decrease or even prevent blood flow to part of the heart muscle. Unfortunately, spasm disorders are often missed using conventional cardiac diagnostic tools like cardiac catheterization (angiogram) – unless accompanied by what’s known as “provocative testing” to try to provoke a spasm during the procedure using a type of medication or hyperventilation. Unlike typical angina, which usually occurs with physical activity, coronary artery spasms often occur at rest.
Similarly, diagnosing Coronary Microvascular Disease is difficult using standard cardiac diagnostics that are designed to identify large blockages in large coronary arteries. Dr. Noel Bairey Merz believes that the best current diagnostic test for detecting MVD may be something called coronary reactivity testing. This angiogram-like test lasts 60-90 minutes and allows doctors to see how very small vessels supplying the heart respond to different “challenges” from medications.
UPDATE: Coronary vasospasm disorders have also been found to be caused by a rare allergic reaction called Kounis Syndrome, treated with corticosteroids and antihistamines. See also: Mast Attack by microbiologist Dr. Lisa Klimas who has written a 4-part series of articles on Kounis Syndrome.
ANOTHER UPDATE: There may also be a line between hypothyroid issues and spasm disorders. Learn how this heart patient tracked down the culprit here.
Q: Have you had “normal” cardiac test results despite what turned out to be a serious heart issue?
UPDATE November 2019: Diagnostic tools used to identify coronary microvascular dysfunction
Watch the conference speakers (11 presentations, about 20-25 minutes each) attending the 2019 INOCA “Meeting of the Minds meeting on non-obstructive coronary artery disease in London England in this fantastic series of videos.
NOTE FROM CAROLYN: I wrote more about coronary microvascular disease in my book, “A Woman’s Guide to Living with Heart Disease”. You can ask for it at your local library or favourite bookshop, or order it online (paperback, hardcover or e-book) at Amazon, or order it directly from my publisher, Johns Hopkins University Press (use their code HTWN to save 20% off the list price).