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EMGuideWire's Radiology Reading Room: Hypertrophic Cardiomyopathy

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EMGuideWire's Radiology Reading Room: Hypertrophic Cardiomyopathy

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The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Hypertrophic Cardiomyopathy and is brought to you by Ashley Moore-Gibbs, DNP, Claire Lawson, NP, Laszlo Littmann, MD, and John Symanski, MD.

The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Hypertrophic Cardiomyopathy and is brought to you by Ashley Moore-Gibbs, DNP, Claire Lawson, NP, Laszlo Littmann, MD, and John Symanski, MD.

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EMGuideWire's Radiology Reading Room: Hypertrophic Cardiomyopathy

  1. 1. Ashley Moore-Gibbs, DNP, Claire Lawson, NP, Laszlo Littmann, MD, John Symanski, MD Departments of Emergency Medicine & Internal Medicine Sanger Heart & Vascular Institute Carolinas Medical Center Michael A. Gibbs, MD, Lead Editor Carolinas Medical Center Imaging Mastery Project Hypertrophic Cardiomyopathy
  2. 2. Visit Our Educational Website www.EMGuidewire.com
  3. 3. Amyloid Dilated • Ischemic • Peripartum • Hypertensive • Iron overload Genetic • Hypertrophic • LV Noncompaction • ARVC1 Inflammatory (Myocarditis) • Viral • Giant cell • Eosinophilic • Chagas • COVID-19 Metabolic • Diabetic • Hypothyroid • Acromegalic • Cardiac Sarcoid Stress-Induced (Takotsubo) Tachycardia-Induced Toxic • Alcoholic • Chemotherapy-induced • Cocaine-induced • Other drug related 1Arrhythmogenic Right Ventricular Cardiomyopathy Classification Of Cardiomyopathies
  4. 4. Embedded References Ommen SR. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy. Journal of the American College of Cardiology. Article In Press. Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. New England Journal of Medicine. 2018. Volume 379, Number 7. Geske JB. Hypertrophic Cardiomyopathy: Clinical Update. Journal of the American College of Cardiology. 2018. Volume 6, Number 5.
  5. 5. See Appendix 1 For AHA/ACC Top Ten Hypertrophic Cardiomyopathy Recommendations.
  6. 6. Hypertrophic Cardiomyopathy (HCM) Cases Studies From Carolinas Medical Center
  7. 7. HCM: Imaging Essentials • The cardiac silhouette may or may not be enlarged on chest X-ray • Thickening of the interventricular septum is seen on cardiac ultrasound and cardiac MRI (CMR) • On CMR “late phase gadolinium enhancement” is seen on T1 images
  8. 8. Gadolinium Enhancement • Gadolinium containing contrast is widely used in cardiac MRI to assess the integrity of the myocardium • Gadolinium is typically taken up and rapidly washed out from healthy myocardial cells • In the presence of disease (e.g.: acute and chronic ischemia, prior infarct, myocarditis, cardiomyopathies) gadolinium remains in abnormal cardiomyocytes, thus causing late phase enhancement gadolinium enhancement (LGE) on T1 images • In hypertrophic cardiomyopathy, LGE is most prominent in the septum (or apex in patients with apical variant HCM)
  9. 9. Case #1 43-Year-Old Male
  10. 10. Case #1 Cardiac MRI
  11. 11. Case #1 Asymmetric Septal Hypertrophy Septum Cardiac MRI (CMR) Cardiac Apex
  12. 12. Case #1 Asymmetric Septal Hypertrophy 26 mm 25 mm Septum
  13. 13. Case #1 T1 Images: Late Phase Gadolinium Enhancement Areas Of Gadolinium Enhancement (Arrow) Represent Tissue Fibrosis & Scaring Cardiac Apex
  14. 14. Case #2 47-Year-Old Male
  15. 15. Case #2 Cardiac MRI
  16. 16. Case #2 Septum Asymmetric Septal Hypertrophy Cardiac Apex
  17. 17. T1 Images: Late Phase Gadolinium Enhancement Areas Of Gadolinium Enhancement (Arrow) Represent Tissue Fibrosis & Scaring
  18. 18. Case #3 54-Year-Old Male
  19. 19. “Ace of Spade” LV Chamber Geometry Case #3 Cardiac MRI
  20. 20. “Ace of Spade” LV Chamber Geometry Case #3 Apical Variant HCM Cardiac Apex
  21. 21. Case #3 T1 Images: Late Phase Gadolinium Enhancement Areas Of Gadolinium Enhancement (Arrow) Represent Tissue Fibrosis & Scaring Cardiac Apex
  22. 22. Prominent Apical Hypertrophy Case #3 Cardiac Apex Apical Variant HCM
  23. 23. Apical Scarring (Late Gadolinium Enhancement) Case #3 T1 Images: Late Phase Gadolinium Enhancement Areas Of Gadolinium Enhancement (Arrow) Represent Tissue Fibrosis & Scaring
  24. 24. Definition • Hypertrophied, nondilated left ventricle – in the absence of another cardiac, systemic, or syndromic disease state as an explanation • A wall thickness of ≥15 mm by echocardiogram, CT, or CMR suggests HCM • Extensive late phase gadolinium enhancement on CMR is diagnostic
  25. 25. Epidemiology • Prevalence of 1 in 200 - 500 persons • Reported in 122 countries on all continents • Highly variable penetrance, disease expression, and clinical course • Under recognition disproportionately affects women and underserved minorities
  26. 26. Epidemiology
  27. 27. Genetics • Autosomal dominant mutation of the cardiac sarcomere • To date, mutations of 11(+) genes have been identified • Offspring of an affected person have a 50% risk of developing HCM • Genetic testing is typically limited to next-generation (cascade) family screening
  28. 28. 2020 ACC/AHA Genetic Screening Guidelines • Clinical screening (with or without genetic testing) recommended in first-degree relatives of patients with HCM (Class I, LOE: B) • Genetic testing reasonable in index patient to facilitate identification of first-degree relatives at risk for developing HCM (Class IIA, LOE: B) • Two or more mutations identified in < 5% Journal Of The American College of Cardiology. 2020. Article In Press.
  29. 29. Clinical Manifestations • Patients may be asymptomatic • 1 in 5 patients have atrial fibrillation - high stroke risk if undetected Symptoms include: • Dyspnea on exertion • Chest pain • Fatigue • Syncope or presyncope
  30. 30. Systematic Literature Review Assessing The Use Of Different Bedside Maneuvers To Discern The Likelihood That A HCM Murmur Is Present. Intensity decreased with passive leg elevation [+] LR 8.0 (95% CI 3-21) No change in intensity with leg elevation [-] LR 0.22(95% CI 0.06-0.77) Decreased from standing to squatting [+] LR 4.5 (95% CI 2.3-8.6) Increased from standing to squatting [-] LR 0.13 (95% CI 0.02.81) JAMA. 1997; 277:564-571. Does This Patient Have An Abnormal Systolic Murmur? JAMA: The Rational Clinical Examination
  31. 31. ECG Hallmarks • Left ventricular hypertrophy • Deep Q waves in the inferior and anterolateral leads See Appendix 1 at the end of this presentation: “Hypertrophic Cardiomyopathy ECG Case Studies.” Created by the master ECG educator, Dr. Laszlo Littmann.
  32. 32. A 34-YO HEALTHY, PHYSICALLY ACTIVE WOMAN PRESENTED WITH THREE RECENT EPISODES OF EXERTIONAL SYNCOPE
  33. 33. ECG DIAGNOSIS  Sinus bradycardia, left axis deviation  Left ventricular hypertrophy (aVL = 13 mm)  Deep Q waves in the inferior and anterolateral leads
  34. 34. Echocardiography • Left ventricular hypertrophy: • LV wall thickness 13-15mm = mild; > 15mm = severe • Diffuse LV wall thickening evident in ~50% patients • 10%-20% will have segmental hypertrophy • Truly symmetrical LVH is rare • Mild right ventricular hypertrophy • Mitral valve systolic anterior motion • Hyper-dynamic left ventricle
  35. 35. Echocardiography Class I: • TTE recommended in the initial evaluation of all patients with suspected HCM (LOE: B) • TTE recommended in screening of all family members of patients with HCM unless the family member is genotype negative in a family with known definitive mutations (LOE: B) • Repeat TTE recommended with a change in clinical status or new cardiovascular event (LOE: B) Journal Of The American College of Cardiology. 2020. Article In Press.
  36. 36. Cardiac Magnetic Resonance Class I: • Indicated in patients with suspected HCM when echocardiography is inconclusive for the diagnosis (LOE: B) • Indicated in patients with known HCM when additional information impacting decision-making regarding invasive management is not adequately defined by echocardiography (LOE: B) Class IIa: • CMR imaging is reasonable in HCM patients to define apical hypertrophy and/or aneurysm if echocardiography is inconclusive (LOE: B) Journal Of The American College of Cardiology. 2020. Article In Press.
  37. 37. Therapies: Reduction OF LVOT Obstruction 𝛽 -blockers are the mainstay of therapy for symptomatic patients: • Negative inotropy reduced left ventricular outflow track obstruction • Negative chronotropy prolongs diastole (which improves filling hemodynamics) and blunts adrenergic-mediated tachycardia Calcium channel blockers can be used if 𝛽-blockers are ineffective or not well tolerated Disopyramide and a 𝛽 -blockers is an alternative approach
  38. 38. Therapies: Treatment Of Atrial Fibrillation • Up to 20% of patients with HCM will develop atrial fibrillation (AF) • Silent cases are common, increasing the risk of stroke
  39. 39. Sudden Cardiac Death Risk Stratification • The incidence of sudden cardiac death (SCD) is ∼1% per year • The highest risks include a history of prior SCD, ventricular fibrillation, or sustained ventricular tachycardia. These are associated with an annual risk of SCD ∼10% • Massive hypertrophy (>30 mm), a family history of SCD, and unexplained syncope also important risk factors Journal Of The American College of Cardiology. 2020. Article In Press.
  40. 40. European Society Of Cardiology
  41. 41. Lifestyle Modifications • 2020 ACC/AHA guidelines encourage moderate-intensity aerobic exercise to increase overall cardiovascular health • Avoidance of alcohol, dehydration, and temperature extremes, e.g.: hot tubs, saunas • Avoidance of drugs that worsen left ventricular outflow track (LVOT) obstruction: diuretics, digitalis, phosphodiesterase inhibitors Journal Of The American College of Cardiology. 2020. Article In Press.
  42. 42. Improved Outcomes Over The Last 50 Years
  43. 43. APPENDIX 1: Top 10 Take Home Points
  44. 44. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points 1. Shared decision-making, a dialogue between patients and their care team that includes full disclosure of all testing and treatment options, discussion of the risks and benefits of those options and, importantly engagement of the patient to express their own goals is particularly relevant. 2. Although the primary cardiology team can initiate evaluation, treatment, and longitudinal care, referral to multidisciplinary HCM centers with graduated levels of expertise can be important to optimizing care for patients with HCM. Challenging treatment decisions – where reasonable alternatives exist, where the strength of the evidence or is particularly nuanced, and for invasive procedures that are specific to patients with HCM – represent crucial opportunities to refer patients to HCM center.
  45. 45. 3. Counseling patients with HCM regarding the potential for genetic transmission of HCM is one of the cornerstones of care. Screen first-degree family members of patients with HCM, using either genetic testing of an imaging electrocardiographic surveillance protocol can begin at any age and can be influenced by specifics of the patient/family history and family preference. As screening recommendations for family members hinge on the pathogenicity of any detected variants, the reported pathogenicity should be reconfirmed every 2 to 3 year. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  46. 46. 4. Optimal care for patients with HCM requires cardiac imaging to confirm the diagnosis, characterize the pathophysiology for the individual, and identify risk markers that may inform decisions regarding interventions for left ventricular outflow tract obstruction and sudden cardiac death (SCD) prevention. Echocardiography continues to be the foundational imaging modality for patients with HCM. Cardiovascular magnetic resonance imaging will also be helpful in many patients, especially those in whom there is diagnostic uncertainty, poor echocardiographic imaging windows, or where uncertainty persists regarding decisions around implantable cardioverter-defibrillator (ICD) placement. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  47. 47. 5. Assessment of an individual patient’s risk for SCD continues to evolve as new markers emerge (e.g.: apical aneurysm, decreased left ventricular systolic function, and extensive gadolinium enhancement). In addition to a full accounting of an individual’s risk markers, communication with patient’s regarding not just the presence of risk markers but also the magnitude of the individual’s risk is key. This enables the informed patient to fully participate in the decision-making regarding ICD placement, which incorporates their own level of risk tolerance and treatment goals. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  48. 48. 6. The risk factors for SCD in children with HCM carry different weights than those observed in adult patients, they vary with age and must account for different body sizes. Coupled with the complexity of placing ICDs in young patients with anticipated growth and a higher risk of device complications, the threshold for ICD implantation in children often differs from adults. These differences are best addressed at primary or comprehensive HCM centers with expertise in children with HCM. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  49. 49. 7. Septal reduction therapies (surgical myectomy and alcohol septal ablation), when performed by experience HCM teams at dedicated centers, continue to improve in safety and efficacy such that earlier intervention my be possible in select patients with drug-refractory or severe outflow tract obstruction causing signs of cardiac decompensation. Given the data on the significantly improved outcomes at comprehensive HCM centers, these decisions represent an optimal referral opportunity. 8. Patients with HCM and persistent or paroxysmal atrial fibrillation have a sufficiently increased risk of stroke such that oral anticoagulants should be considered as default treatment options independent of the CHA2DS2VASc score. As rapid atrial fibrillation is often poorly tolerated in patients with HCM, maintenance of sinus rhythm and rate control are key pursuits in successful treatment. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  50. 50. 9. Heart failure symptoms in patients with HCM, in the absence of left ventricular outflow tract obstruction should be treated similarly to other patients with heart failure symptoms, including consideration of advanced treatment options (e.g.: cardiac resynchronization therapy, left ventricular assist device, transplantation). In patients with HCM, an ejection fraction <50% connotes significantly impaired systolic function and identifies individuals with poor prognosis and who are at increased risk for SCD. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  51. 51. 10. Increasingly, data affirm that the beneficial effects of exercise on general health can be extended to patients with HCM. Healthy recreational exercise (moderate intensity) has not been associated with increased risk of ventricular arrhythmia events in recent studies. Whether an individual patient with HCM wises to pursue more rigorous exercise/training is dependent on a comprehensive shared discussion between that patient and their expert HCM care team regarding the potential risks of that level of training/participation but with the understanding that exercise-related risk cannot be individualized for any given patient. 2020 AHA/ACC Guidelines: Top 10 Take-Home Points
  52. 52. HYPERTROPHIC CARDIOMYOPATHY ECG CASE STUDIES Dr. Laszlo Littmann, MD Department of Internal Medicine Carolinas Medical Center September 2020 APPENDIX 2
  53. 53. 1. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY
  54. 54. A 34-YO HEALTHY, PHYSICALLY ACTIVE WOMAN PRESENTED WITH THREE RECENT EPISODES OF EXERTIONAL SYNCOPE
  55. 55. A 34-YO HEALTHY, PHYSICALLY ACTIVE WOMAN PRESENTED WITH THREE RECENT EPISODES OF EXERTIONAL SYNCOPE EXERTIONAL SYNCOPE: PROBABLY NOT VASOVAGAL
  56. 56. ECG DIAGNOSIS  Sinus bradycardia, left axis deviation  Left ventricular hypertrophy (aVL = 13 mm)  Deep Q waves in the inferior and anterolateral leads
  57. 57. COMPUTER INTERPRETATION:
  58. 58.  In a young, a normotensive patient, the causes of left axis deviation and LVH are:  Aortic stenosis  HCM  Causes of non-infarction Q waves:  HCM  Cardiac amyloidosis  Scleroderma  Pneumothorax  WPW syndrome  Hyperkalemia
  59. 59. Hypertrophic Cardiomyopathy  In a young, normotensive patient, causes of left axis deviation and LVH:  Aortic stenosis  HCM  Causes of non-infarction Q waves:  HCM  Cardiac amyloidosis  Scleroderma  Pneumothorax  WPW syndrome  Hyperkalemia  The Q waves are usually narrow but deep  As in this case, they are usually present in the inferior and lateral leads  Their etiology is uncertain:  Myocardial fibrosis?  Marked thickening of the base of the septum?  Their clinical significance is also uncertain
  60. 60. ECG DIAGNOSIS CLINICAL DIAGNOSIS: HOCM  Sinus bradycardia, left axis deviation  Left ventricular hypertrophy (aVL = 13 mm)  Deep Q waves in the inferior and anterolateral leads
  61. 61. 2. APICAL VARIANT OF HYPERTROPHIC CARDIOMYOPATHY
  62. 62. 56-year-old with multiple hospitalizations over 5 years for chest pain and syncope. Most recent admission for paroxysmal atrial fibrillation demonstrated: normal BP and cardiac examination. A 48-hour Holter demonstrated brief episodes of ventricular tachycardia. 3 echocardiograms all showed LVH and abnormal relaxation. Coronary angiogram was negative.
  63. 63. HYPERTROPHIC CARDIOMYOPATHY, APICAL VARIANT
  64. 64.  A rare form of hypertrophic cardiomyopathy; highest prevalence in Asia  Typical presentation: chest pain, syncope, supraventricular and ventricular arrhythmias  Despite normal BP and negative physical exam, there is LVH both in the ECG and echo ECG: sharp, deep negative T waves in the chest leads
  65. 65.  Easy to miss by routine echocardiogram: the LV apex is frequently not well visualized  Imaging studies show an “Ace of Spades” pattern  Treatment is controversial
  66. 66. ECHO CONTRAST ECHO ACE OF SPADES
  67. 67. LV ANGIO
  68. 68. 3. HYPERTROPHIC CARDIOMYOPATHY, MIDVENTRICULAR VARIANT WITH APICAL ANEURYSM
  69. 69. A 66-YEAR-OLD MAN EXPERIENCED SUDDEN-ONSET PALPITATION, SHORTNESS OF BREATH, DIZZINESS AND ATYPICAL CHEST PAIN
  70. 70. VENTRICULAR TACHYCARDIA FOLLOWING IV PROCAINAMIDE, SINUS RHYTHM WITH ST ELEVATION IN THE ANTEROLATERAL LEADS  Heart rate: 200/min; ECG: wide-complex tachycardia  200 mg procainamide IV: sinus rhythm and symptom resolution  Subsequent ECG c/w anterolateral STEMI  Emergent cardiac catherization: clean coronaries  This was the patient’s third negative caths in the last 8 years
  71. 71. 12-LEAD ECG
  72. 72. 2007 2015
  73. 73. 2007 2015  STABLE ST ELEVATION SPANNING 8 YEARS SUGGESTS STRUCTURAL ABNORMALITY  LVH SUGGESTS HYPERTROPHIC CARDIOMYOPATHY  ST ELEVATION SUGGESTS LV APICAL ANEURYSM  THIS COMBINATION IS MOST FREQUENTLY SEEN IN HCM WITH MIDVENTRICULAR OBSTRUCTION  FREQUENT COMPLICATIONS: VENTRICULAR TACHYCARDIA, THROMBOEMBOLIC EVENTS, SUDDEN CARDIAC DEATH  TREATMENT: BETA BLOCKER, ICD, CATHETER ABLATION OF VT, ANTICOAGULATION
  74. 74. 2007 2015  STABLE ST ELEVATION SPANNING 8 YEARS SUGGESTS STRUCTURAL ABNORMALITY  LVH SUGGESTS HYPERTROPHIC CARDIOMYOPATHY  ST ELEVATION SUGGESTS LV APICAL ANEURYSM  THIS COMBINATION IS MOST FREQUENTLY SEEN IN HCM WITH MIDVENTRICULAR OBSTRUCTION  FREQUENT COMPLICATIONS: VENTRICULAR TACHYCARDIA, THROMBOEMBOLIC EVENTS, SUDDEN CARDIAC DEATH  TREATMENT: BETA BLOCKER, ICD, CATHETER ABLATION OF VT, ANTICOAGULATION
  75. 75. CARDIAC MRI
  76. 76. THE ECG IN DIFFERENT TYPES OF HYPERTROPHIC CARDIOMYOPATHY SUMMARY
  77. 77.  HCM  Left axis deviation  Abnormal Q waves  Bizarre repolarization abnormalities  Apical variant  Deep symmetrical negative T waves in the chest leads  Mid-ventricular variant with apical aneurysm  Stable, persistent ST elevation in the anterolateral leads II V4 V4 HYPERTROPHIC CARDIOMYOPATHY AND THE ECG LVH AND:
  78. 78. If You Have Interesting Cases Of Hypertrophic Cardiomyopathy, We Invite You To Send A Set Of Digital PDF Images And A Brief Descriptive Clinical History To: michael.gibbs@atriumhealth.org Your De-Identified Case(s) Will Be Posted On Our Education Website And You And Your Institution Will Be Recognized!

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