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Hypertrophic Cardiomyopathy pp 59–81Cite as

Cardiac MRI in Diagnosis and Management

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Abstract

Cardiac magnetic resonance imaging (CMR) is an essential tool for diagnosis, risk stratification, and treatment of patients with hypertrophic cardiomyopathy (HCM). CMR works by manipulating protons found in myocytes and contrast agents such as gadolinium, using magnetic pull to create and detect energy differences in order to obtain images. CMR is ideal for determining location and extent of hypertrophy, presence of membranes, distribution of fibrosis, as well as for identifying anatomy and physiology of the mitral valve, all of which are crucial for management of HCM and can be missed on standard echocardiography. This is especially true for areas of the heart that are difficult to evaluate on echocardiography, such as the apex and lateral wall, as well as atypical presentations, e.g., focal segmental hypertrophy. Additionally, CMR enables accurate assessment of ventricular volumes, mass, and function, which have diagnostic, as well as prognostic value. Magnetic resonance tagging and delayed enhancement with gadolinium contrast agents allow for strain and perfusion analysis, further increasing the utility of CMR to detect regional function and cardiac microvascular ischemia. Delayed enhancement may also improve risk stratification for sudden cardiac death. Limitations of CMR include difficulty to assess left ventricular outflow tract gradients and the ability to identify highly mobile structures on the mitral valve, although newer protocols and improved technology may be able to compensate for these deficits in the future.

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Author information

Authors and Affiliations

  1. Division of Cardiology, Montefiore-Einstein Center for Heart and Vascular Care, Albert Einstein College of Medicine, Bronx, NY, USA

    Daniele Massera & Mario J. Garcia

  2. Department of Cardiology, Winthrop University Hospital, Mineola, NY, USA

    Jonathan Kahan & Juan Gaztanaga

Authors
  1. Daniele Massera
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  2. Jonathan Kahan
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  3. Juan Gaztanaga
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  4. Mario J. Garcia
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Corresponding author

Correspondence to Mario J. Garcia .

Editor information

Editors and Affiliations

  1. Westchester Medical Center, Valhalla, NY, USA

    Srihari S. Naidu

Questions

Questions

  1. 1.

    The manipulation of which of the following particles is responsible for MRI technology?

    1. A.

      Electrons

    2. B.

      Protons

    3. C.

      Neutrons

    4. D.

      X-rays

    5. E.

      Magnets

  • The correct answer is B. In magnetic resonance imaging, protons (in the form of hydrogen atoms contained in water) are aligned by application of a strong magnetic field. The energy emitted by the aligned protons is measured and used to generate MRI images.

  1. 2.

    Cardiac MR is superior to echocardiography at all of the following except:

    1. A.

      Measurement of left ventricular outflow tract gradients

    2. B.

      Ventricular volume measurement

    3. C.

      Detection of myocardial crypts

    4. D.

      Diagnosis of apical variant hypertrophic cardiomyopathy

    5. E.

      Evaluation of fibrosis

  • The correct answer is A. CMR is superior to echocardiography at measuring atrial and ventricular volumes and identifying LV wall thickness, segments of hypertrophy, as well as associated features, such as myocardial crypts. Late gadolinium enhancement enables identification and quantification of myocardial fibrosis. Echocardiography is superior to CMR at measuring LVOT gradients.

  1. 3.

    The most dangerous adverse reaction of gadolinium contrast media is

    1. A.

      Contrast-induced nephropathy

    2. B.

      Angioedema

    3. C.

      Ototoxicity

    4. D.

      Nephrogenic systemic fibrosis

    5. E.

      Osteoporosis

  • The correct answer is D. Nephrogenic systemic fibrosis is a dreaded complication of gadolinium contrast media and has only been seen in individuals with decreased glomerular filtration rate. While contrast-induced nephropathy is an adverse reaction to iodinated contrast radiocontrast media, it is not seen after administration of gadolinium contrast agents.

  1. 4.

    Which of the following is an absolute contraindication to cardiac MRI?

    1. A.

      Implantable cardioverter defibrillator

    2. B.

      Claustrophobia

    3. C.

      End-stage renal disease

    4. D.

      Pregnancy

    5. E.

      None of the above

  • The correct answer is E. Safe MRI imaging is possible in all of the above scenarios, provided that safety precautions are taken. MRI-conditional implantable cardioverter defibrillators are on the market, but devices that have been implanted after the year 2001 have been shown to be safe when following a dedicated protocol involving reprograming of the device and careful monitoring by skilled personnel. Claustrophobia is considered a relative contraindication, and end-stage renal disease is not a contraindication for MRI, unless gadolinium contrast media is used. Pregnancy is not a contraindication to non-contrast MRI imaging, though administration of gadolinium contrast agents should be avoided given the risk of teratogenicity.

  1. 5.

    Delayed enhancement sequences are obtained ___ min after administration of gadolinium contrast media.

    1. A.

      1–3

    2. B.

      5–10

    3. C.

      10–20

    4. D.

      20–30

    5. E.

      45

  • The correct answer is C. First-pass perfusion images are obtained immediately following injection of gadolinium contrast agents, though delayed enhancement sequences are taken 10–20 min after injection.

  1. 6.

    Which of the following answers is correct?

    1. A.

      Late gadolinium enhancement extent >15% of LV mass is associated with a twofold increase in sudden cardiac death in patients with HCM.

    2. B.

      Late gadolinium enhancement is associated with fourfold increase in dynamic LVOT gradients.

    3. C.

      There is no linear relationship of LGE extent and sudden cardiac death.

    4. D.

      Absence of LGE confers a worse prognosis.

  • The correct answer is A. There is a linear relationship between late gadolinium enhancement extent and sudden cardiac death risk. More specifically, LGE extent >15% of LV mass is associated with twofold increase in sudden cardiac death in patients with HCM. LGE extent is not associated with LVOT gradients.

  1. 7.

    CMR can help distinguish HCM from the following phenocopies, except:

    1. A.

      Anderson-Fabry disease

    2. B.

      Hypereosinophilic syndrome

    3. C.

      Hypertensive heart disease

    4. D.

      Left ventricular noncompaction cardiomyopathy

    5. E.

      None of the above

  • The correct answer is E. All of the above mimickers of HCM can be distinguished on CMR. Anderson-Fabry disease typically exhibits LGE in the basal inferolateral wall. Hypereosinophilic syndrome is characterized by diffuse subendocardial LGE and LV apical thrombus. In hypertensive heart disease, the LV wall thickness usually does not exceed 15 mm. Left ventricular noncompaction cardiomyopathy presents with an abnormally thick layer of noncompacted myocardium.

  1. 8.

    Which of the following findings on CMR is associated with increased risk of sudden cardiac death?

    1. A.

      Myocardial crypt

    2. B.

      Accessory apical-basal muscle bundle

    3. C.

      Accessory papillary muscle

    4. D.

      Apical aneurysm

    5. E.

      Apical-variant HCM

  • The correct answer is D. Apical aneurysm is a high-risk feature and has been found to be associated with increased risk of sudden cardiac death. All other findings are not currently known to be associated with an increased risk of sudden cardiac death.

  1. 9.

    In HCM, abnormal papillary morphology can cause systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction in the presence of normal septal wall thickness.

    1. A.

      True

    2. B.

      False

  • The correct answer is A. Abnormal positioning of a papillary muscle, e.g., anteroapical displacement, can lead to systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction, even when septal wall thickness is normal or only mildly increased.

  1. 10.

    CMR is recommended in all patients with HCM by current guidelines.

    1. A.

      True

    2. B.

      False

  • The correct answer is B. Current guidelines do not recommend CMR in all patients. However, CMR can provide additional information by better characterizing morphology and pattern of LV hypertrophy, mitral valve and subvalvular anatomy, associated pathologies, presence and extent of myocardial fibrosis, and distinguish between other causes of myocardial hypertrophy.

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Massera, D., Kahan, J., Gaztanaga, J., Garcia, M.J. (2019). Cardiac MRI in Diagnosis and Management. In: Naidu, S. (eds) Hypertrophic Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-319-92423-6_5

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  • DOI: https://doi.org/10.1007/978-3-319-92423-6_5

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  • Online ISBN: 978-3-319-92423-6

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