Abstract
In patients with HCM, arrhythmias including atrial fibrillation (AF), supraventricular tachycardia (SVT), and ventricular tachycardia (VT) are common. Many of these arrhythmias are asymptomatic, especially if of short duration, but some can precipitate hemodynamic collapse and sudden cardiac death (SCD). Therefore, the evaluation of such arrhythmias, both proactively and reactively, and their clinical significance in patients with HCM are of paramount importance. In this chapter, we will discuss the incidence, diagnosis, medical management, and role of invasive testing and ablation for arrhythmias in HCM patients.
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Questions
Questions
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1.
All of the following are true regarding AF except:
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A.
Roughly 25% of HCM will evidence AF over the course of their disease.
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B.
It is associated with stroke.
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C.
Stroke risk tracks with standard scoring systems and some patients may reasonably avoid anticoagulation.
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D.
It may be associated with clinical deterioration.
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E.
Patients may receive convergent therapy as second-line ablation therapy.
Answer: C. All patients with AF, either PAF or chronic AF, must be anticoagulated because the estimated risk of stroke is > 4%, and therefore there does not appear to be a low-risk cohort for which anticoagulation can be safely withheld. Further studies are necessary to validate standard scoring systems in this population of patients.
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A.
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2.
The following are true about SVTs in HCM except:
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A.
WPW is more common in HCM than in the general population.
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B.
WPW should be treated by ablation whenever possible.
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C.
SVTs are generally well tolerated.
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D.
AV nodal blockers are first-line therapy for SVTs.
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E.
Patients with fast SVT should be anticoagulated.
Answer: E. There is no evidence that fast SVTs should be anticoagulated. However, all of the other responses are true. WPW is seen frequently and responds well to catheter ablation. This also allows safe institution of AV nodal blocking agents in these patients who require these medications for control of obstructive physiology and/or diastolic dysfunction.
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A.
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3.
Major risk factors in the US 2011 AHA guidelines that prompt ICD consideration include the following except:
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A.
Maximal wall thickness > 2.5 cm
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B.
Recent unexplained syncope
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C.
FH of SCD in a first-degree relative < 50 years of age
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D.
Sustained VT
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E.
Resuscitated cardiac arrest
Answer: A. Maximal wall thickness > 3.0 cm is a major risk factor and should prompt consideration of ICD. When thickness is > 2.5 cm, then other risk modifiers should be evaluated, including blood pressure response to exercise by treadmill test, the presence of obstruction, LGE scar burden on MRI, and others.
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A.
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4.
The best anti-arrhythmic medication in HCM or AF or VT is:
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A.
Disopyramide
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B.
Amiodarone
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C.
Propafenone
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D.
Flecainide
Answer: B. Amiodarone is considered the best anti-arrhythmic medication to be used in HCM, although disopyramide may be used in patients with AF and LVOT obstruction to help control both aspects of the disease. When used for this purpose, an AV nodal blocker must also be used, in order to avoid rapid conduction while in AF. The other medications are not used in the management of HCM.
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A.
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5.
The following is true about EPS in HCM:
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A.
EPS is recommended to risk stratify patients into intermediate or high risk for SCD and to guide ICD placement in borderline cases.
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B.
EPS may be helpful in the evaluation of conduction disease and whether PPM may be indicated in patients with HCM.
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C.
EPS is a Class 2b in the US 2011 AHA guidelines for risk stratification.
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D.
All of the above.
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E.
None of the above.
Answer: B. EPS may be helpful for the evaluation of conduction disease, as in other patients without HCM. In patients with surgical myectomy or alcohol ablation, conduction studies and the appropriate timing of conduction studies have not been confirmed, however.
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A.
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Zakhary, D.R., Germano, J.J. (2019). Management of Arrhythmia: Medications, Electrophysiology Studies, and Ablation. In: Naidu, S. (eds) Hypertrophic Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-319-92423-6_21
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