Hypertrophic cardiomyopathy invasive therapy guidelines

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D. [2]; Caitlin J. Harrigan [3]; Martin S. Maron, M.D.; Barry J. Maron, M.D.; Lakshmi Gopalakrishnan, M.B.B.S. [4]

Overview

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.[1][2][3] 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.

Efficacy and Procedural Success

Relief of obstruction is noted immediately in the majority of appropriately selected patients. Clinical success is defined as a 50% or more reduction in peak gradient across the outflow tract, predicting continued improvement in gradient and cardiac remodeling over the ensuing 1 to 2 years. Over 90% of patients experience a successful procedure, with improvement in outflow tract gradient and mitral regurgitation. Patients typically report progressive reduction in symptoms, including improved shortness of breath, lightheadedness and chest pain.

Follow-Up

Serial echocardiograms are routinely obtained to follow the cardiac remodeling over time, and document reduction in outflow tract gradient.

Comparison to Myectomy

Non-randomized data from the Netherlands suggests caution in the utilization of alcohol septal ablation, which may be inferior to surgical myectomy [4]. The outcomes (the risk of cardiac death and aborted sudden cardiac death including appropriate cardioverter-defibrillator discharges for fast ventricular tachycardia/ventricular fibrillation) in 91 consecutive alcohol septal ablation patients were compared with 40 patients 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 a 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 propensity adjusted multivariable model (p=0.02). Based upon non-randomized data, myectomy may be prefferable to alcohol septal ablation.

It is important to note that patients who fail to respond to alcohol septal ablation may still be candidates for surgical myectomy. Likewise, patients who fail surgical myectomy may still respond to alcohol septal ablation.

2011 ACCF/AHA Guideline Recommendations: Alcohol Septal Ablation [5][6]

Class IIa

1. Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: C)

2. When surgery is contraindicated or the risk is considered unacceptable because of serious comorbidities or advanced age, alcohol septal ablation, when performed in experienced centers, can be beneficial in eligible adult patients with HCM with LVOT obstruction and severe drug-refractory symptoms (usually NYHA functional classes III or IV).(62,153,277–281) (Level of Evidence: B)

Class IIb

1. Alcohol septal ablation, when performed in experienced centers, may be considered as an alternative to surgical myectomy for eligible adult patients with HCM with severe drug-refractory symptoms and LVOT obstruction when, after a balanced and thorough discussion, the patient expresses a preference for septal ablation.(153,273,278,280,281) (Level of Evidence: B)

2. The effectiveness of alcohol septal ablation is uncertain in patients with HCM with marked (i.e., >30 mm) septal hypertrophy, and therefore the procedure is generally discouraged in such patients. (Level of Evidence: C)

Class III (Harm)

1. Alcohol septal ablation should not be done in patients with HCM with concomitant disease that independently warrants surgical correction (e.g., coronary artery bypass grafting for CAD, mitral valve repair for ruptured chordae) in whom surgical myectomy can be performed as part of the operation. (Level of Evidence: C)

2. Alcohol septal ablation should not be done in patients with HCM who are less than 21 years of age and is discouraged in adults less than 40 years of age if myectomy is a viable option. (Level of Evidence: C)

Septal Myectomy

Septal myectomy is a surgical treatment for hypertrophic cardiomyopathy (HCM). Septal myectomies have been successfully performed for more than 25 years.

Outcomes

Septal myectomy is associated with a low perioperative mortality and a high late survival rate. A study at the Mayo Clinic found surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. The results are shown below:[7]

Survival (all-cause mortality) *
Years With surgery Without surgery
1 98% 90%
5 96% 79%
10 83% 61%
Survival (HCM-related death)
Years With surgery Without surgery
1 99% 94%
5 98% 89%
10 95% 73%
Survival (sudden cardiac death)
Years With surgery Without surgery
1 100% 97%
5 99% 93%
10 99% 89%

* Includes 0.8% operative mortality.

Comparison with alcohol ablation

Either alcohol septal ablation or myectomy offers substantial clinical improvement for patients with hypertrophic obstructive cardiomyopathy.

Hemodynamic resolution of the obstruction and its sequelae is more complete with myectomy.[8]

2011 ACCF/AHA Guideline Recommendations: Septal Myectomy [5][6]

Class IIa

1. Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: C)

2. Surgical septal myectomy, when performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction.(61,62,155,273–275) (Level of Evidence: B)

3. Surgical septal myectomy, when performed at experienced centers, can be beneficial in symptomatic children with HCM and severe resting obstruction (>50 mm Hg) for whom standard medical therapy has failed.(276) (Level of Evidence: C)

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 [9]. Dual chamber pacing does not decrease the risk of sudden cardiac death in these patients.

2011 ACCF/AHA Guideline Recommendations: Pacing [5][6]

Class IIa

1. In patients with HCM who have had a dual-chamber device implanted for non-HCM indications, it is reasonable to consider a trial of dual-chamber atrial-ventricular pacing (from the right ventricular apex) for the relief of symptoms attributable to LVOT obstruction.(292,294,295,366) (Level of Evidence: B)

Class IIb

1. Permanent pacing may be considered in medically refractory symptomatic patients with obstructive HCM who are suboptimal candidates for septal reduction therapy.(283,292,294,295,366) (Level of Evidence: B)

Class III (No Benefit)

1. Permanent pacemaker implantation for the purpose of reducing gradient should not be performed in patients with HCM who are asymptomatic or whose symptoms are medically controlled.(283,284,367) (Level of Evidence: C)

2. Permanent pacemaker implantation should not be performed as a first-line therapy to relieve symptoms in medically refractory symptomatic patients with HCM and LVOT obstruction who are candidates for septal reduction.(283,284,367) (Level of Evidence: B)

2007 ESC Guidelines- Cardiac Pacing in HCM [10]

Class IIa

1. Symptomatic bradycardia due to beta-blockade when alternative therapies are unacceptable. (Level of Evidence: C)

Class IIb

1. Patients with drug refractory hypertrophic cardiomyopathy with significant resting or provoked LVOT gradient [11][12][13] and contraindications for septal ablation or myectomy. (Level of Evidence: A)

Class III

1. Asymptomatic patients. (Level of Evidence: C)

2. Symptomatic patients who do not have LVOT obstruction. (Level of Evidence: C)

Automatic Implantable Cardiac Defibrillator (AICD) 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.

2011 ACCF/AHA Guideline Recommendations: Invasive Therapies

[5][6]

Class I

1. Septal reduction therapy should be performed only by experienced operators in the context of a comprehensive HCM clinical program and only for the treatment of eligible patients with severe drug-refractory symptoms and LVOT obstruction.† (272) (Level of Evidence: C)

Class III (Harm)

1. Septal reduction therapy should not be done for adult patients with HCM who are asymptomatic with normal exercise tolerance or whose symptoms are controlled or minimized on optimal medical therapy. (Level of Evidence: C)

2. Septal reduction therapy should not be done unless performed as part of a program dedicated to the longitudinal and multidisciplinary care of patients with HCM. (Level of Evidence: C)

3. Mitral valve replacement for relief of LVOT obstruction should not be performed in patients with HCM in whom septal reduction therapy is an option. (Level of Evidence: C)

Guideline Resources

References

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