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Epicardial and Microvascular Ischemia: Implications, Diagnosis, and Management

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Hypertrophic Cardiomyopathy

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Abstract

Angina is a common symptom in hypertrophic cardiomyopathy and occurs in approximately 20% of patients. More than 50–75% of patients with HCM have myocardial ischemia on noninvasive testing. Of those patients with angina, approximately 25% have significant epicardial coronary artery disease. It is important to diagnose concomitant epicardial CAD in these patients as it is a predictor of increased mortality in HCM. The majority of HCM patients with myocardial ischemia do not have epicardial CAD, however. The proposed mechanisms of ischemia in the absence of epicardial CAD include increased oxygen demand, microvascular medial hypertrophy and dysfunction, and perturbations of cardiac-coronary coupling with reduction in systolic myocardial blood flow due to microvascular compression from a hypertrophic and hyperdynamic left ventricle and reduced diastolic myocardial blood flow due to blunting of diastolic suction secondary to impaired diastolic relaxation. Repetitive ischemic insults leads to myocardial injury and subsequent myocardial fibrosis. Myocardial ischemia in HCM patients is associated with increased mortality, ventricular tachycardia, left ventricular remodeling and dysfunction, and congestive heart failure. It is unknown whether the treatment of myocardial ischemia in HCM will influence outcomes.

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Author information

Authors and Affiliations

  1. Department of Cardiovascular Medicine, Beaumont Health System, Royal Oak, MI, USA

    George S. Hanzel

Authors
  1. George S. Hanzel
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Corresponding author

Correspondence to George S. Hanzel .

Editor information

Editors and Affiliations

  1. Westchester Medical Center, Valhalla, NY, USA

    Srihari S. Naidu

Questions

Questions

  1. 1.

    Which of the following is incorrect?

    1. A.

      20% of HCM patients have angina.

    2. B.

      90% of HCM patients have ischemia.

    3. C.

      50–75% of HCM patients have chronic troponin elevations.

    4. D.

      Repetitive ischemia induces myocardial fibrosis.

  • Answer: B. 50–75% of HCM patient have ischemia.

  1. 2.

    Which of the following are proposed mechanisms for microvascular ischemia in HCM?

    1. A.

      Supply-demand mismatch

    2. B.

      Microvascular hypertrophy and dysfunction

    3. C.

      Perturbations of cardiac-coronary coupling

    4. D.

      All of the above

  • Answer: D. All are proposed causes of myocardial ischemia in HCM.

  1. 3.

    According to wave intensity analysis (i.e., cardiac-coronary coupling) which of the following are important determinants of ischemia in HCM?

    1. A.

      Systolic reversal of flow due to microvascular compression.

    2. B.

      Decreased driving pressure due to LVOT obstruction.

    3. C.

      Blunted diastolic sucking wave due to impaired ventricular relaxation

    4. D.

      All of the above

  • Answer: D. All of the above mechanisms are thought to impact myocardial blood flow in HCM.

  1. 4.

    Which of the following is false regarding myocardial blood flow (indexed per gram of myocardial tissue) in HCM?

    1. A.

      Resting myocardial blood flow is normal.

    2. B.

      Resting myocardial blood flow is decreased.

    3. C.

      Hyperemic myocardial blood flow is normal.

    4. D.

      Hyperemic myocardial blood flow is deceased.

  • Answer: C. Coronary resistance is low in HCM with reduction in hyperemic coronary flow reserve. Coronary vasodilation at rest is necessary to meet metabolic demands of the hypertrophied ventricle.

  1. 5.

    Microvascular ischemia is associated with all the following but:

    1. A.

      Mortality

    2. B.

      Syncope

    3. C.

      Atrial fibrillation

    4. D.

      Congestive heart failure

  • Answer: C. Microvascular ischemia has been associated with increased mortality, ventricular arrhythmias, syncope, adverse ventricular remodeling, and CHF but not atrial fibrillation at this time.

  1. 6.

    Which of the following is the best imaging modality to detect epicardial CAD in the HCM patient?

    1. A.

      Angiography (invasive or CTA)

    2. B.

      Myocardial perfusion imaging

    3. C.

      Positron-emission tomography

    4. D.

      Exercise echocardiogram

  • Answer: A. Perfusion imaging studies and exercise echo suffer from poor specificity in detecting epicardial CAD in HCM patients. Therefore angiography is the preferred imaging modality. The choice of invasive angiography versus CTA is dictated by clinical suspicion for epicardial disease.

  1. 7.

    Which of the following is true regarding epicardial CAD?

    1. A.

      Epicardial CAD is seen in 25% of HCM patients.

    2. B.

      HCM contributes to premature atherosclerosis.

    3. C.

      Advanced age is protective in HCM patients with CAD.

    4. D.

      Epicardial CAD increases mortality to a greater extent in HCM vs non-HCM population.

  • Answer: D. In HCM patients with epicardial CAD the annual mortality rate is 6.4%, far higher than the typical CAD population.

  1. 8.

    Which of the following is associated with increased mortality in the adult HCM population?

    1. A.

      Microvascular ischemia

    2. B.

      Epicardial CAD

    3. C.

      Myocardial bridging

    4. D.

      A and B

    5. E.

      A, B, and C

  • Answer: D. Although myocardial bridging may cause angina is some patients with HCM, it has not been shown to influence mortality or other outcomes.

  1. 9.

    Which of the following if true:

    1. A.

      All HCM patients should undergo screening for microvascular ischemia.

    2. B.

      Medical therapy reduces myocardial ischemia in HCM.

    3. C.

      Medical therapy improves outcomes in HCM patients with microvascular ischemia.

    4. D.

      Septal reduction therapy should be considered the treatment of choice in patients with microvascular ischemia since it has been show to improve outcomes.

  • Answer: B. Medical therapy has been shown to reduce perfusion abnormalities in HCM. It is unknown whether medical or septal reduction therapy influences outcomes in HCM patients with microvascular ischemia. Since it is unknown whether treatment of silent microvascular ischemia improves outcomes, routine testing to detect ischemia is not recommended.

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Hanzel, G.S. (2019). Epicardial and Microvascular Ischemia: Implications, Diagnosis, and Management. In: Naidu, S. (eds) Hypertrophic Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-319-92423-6_19

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  • DOI: https://doi.org/10.1007/978-3-319-92423-6_19

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