Hypertrophic cardiomyopathy invasive therapy guidelines

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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1], Cafer Zorkun, M.D. [2], Caitlin J. Harrigan [3], Martin S. Maron, M.D., and Barry J. Maron, M.D.

Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [4] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Interventional cardiology and device based therapy in hypertrophic cardiomyopathy

Alcohol septal ablation

Alcohol septal ablation, introduced by Ulrich Sigwart in 1994, is a percutaneous technique that involves injection of alcohol into the first septal perferator of the left anterior descending artery. This is a technique with results similar to the surgical septal myectomy procedure but is less invasive, since it does not involve general anaesthesia and opening of the chest wall and pericardium (which are done in a septal myomectomy). In a select population with symptoms secondary to a high outflow tract gradient, alcohol septal ablation can reduce the symptoms of HCM. When performed properly, an alcohol septal ablation induces a controlled heart attack, in which the portion of the interventricular septum that involves the left ventricular outflow tract is infarcted and will contract into a scar.

Non-randomized data from the Netherlands suggests caution in the utilization of alcohol septal ablation, which may be inferior to surgical myectomy . Outcomes (the risk of cardiac death and aborted sudden cardiac death including appropriate cardioverter-defibrillator discharges for fast ventricular tachycardia/ventricular fibrillation)of 91 consecutive alcohol septal ablation patients were compared with 40 patients with hypertrophic cardiomyopathy who underwent septal myectomy. The 1-, 5-, and 8-year event free survival was 96%, 86%, and 67%, respectively in the alcohol septal ablation patients which was poorer than the 100%, 96%, and 96%, event free rates in the myectomy patients over 6.6±2.7 years (P=0.01). Stated differently, the alcohol septal ablation patients faced 5-fold increase in the risk of the primary endpoint on an annual basis (4.4% versus 0.9%) even when adjustments were made in a multivariable model that adjusted for a propensity score (p=0.02). Based upon these non-randomized data, myectomy appears to be preffered to alcohol septal ablation.

Ventricular pacing

The use of a pacemaker has been advocated in a subset of individuals, in order to cause asynchronous contraction of the left ventricle. Since the pacemaker activates the interventricular septum before the left ventricular free wall, the gradient across the left ventricular outflow tract may decrease. The AV interval must be shortened to do this, but not at the expense of diastolic filling. This form of treatment has been shown to provide less relief of symptoms and less of a reduction in the left ventricular outflow tract gradient when compared to surgical myectomy . Dual chamber pacing does not decrease the risk of sudden cardiac death in these patients.

Recommendations for Pacing in Patients With Hypertrophic Cardiomyopathy (DO NOT EDIT)

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Class I 1. Permanent pacing is indicated for SND or AV block in patients with HCM as described previously (see Section 2.1.1, “Sinus Node Dysfunction,” and Section 2.1.2, “Acquired Atrioventricular Block in Adults”). (Level of Evidence: C) Class IIb 1. Permanent pacing may be considered in medically refractory symptomatic patients with HCM and significant resting or provoked LV outflow tract obstruction. (Level of Evidence: A) As for Class I indications, when risk factors for SCD are present, consider a DDD ICD (see Section 3, “Indications for Implantable Cardioverter-Defibrillator Therapy”). Class III 1. Permanent pacemaker implantation is not indicated for patients who are asymptomatic or whose symptoms are medically controlled. (Level of Evidence: C) 2. Permanent pacemaker implantation is not indicated for symptomatic patients without evidence of LV outflow tract obstruction. (Level of Evidence: C)

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AICD (automatic implantable cardiac defibrillator) placement

The role of AICD (automatic implantable cardiac defibrillator) placement in HCM is controversial. It offers the best potential benefit for survival and should probably be implanted in survivors of SCD and those deemed at high risk by clinical parameters. Nonetheless, the impact on prognosis is unclear because tachyarrhythmias may not always be the mechanism for syncope and sudden death. In addition, older patients may be self-selected “survivors” that stand to gain less from ICD placement. One recent retrospective study showed that at an average follow-up of 128 patients at 3.1 years, 23 percent had shocks for VT (ventricular tachycardia) and 25% had inappropriate shocks. Of those receiving the ICD prophylactically, 5% were shocked per year. This study did not evaluate the role of clinical predictors, evaluate total mortality and was a non-randomized retrospective design that does not establish the need for ICD placement in all patients with HCM or superiority to amiodarone therapy.