Table 4. [Treatment of Manifestations in Individuals with LMNA-related DCM]. - GeneReviews®

Manifestation/Concern	Treatment
Cardiac conduction system disease & arrhythmias	For chronic atrial fibrillation unresponsive to cardioversion: Anticoagulants Agents for ventricular rate control
For other symptomatic supraventricular arrhythmias: Pharmacologic agents May be augmented with electrophysiologic intervention (e.g., atrial or atrioventricular node ablations)
For symptomatic bradyarrhythmias or asymptomatic but significant heart block: An implantable electronic pacemaker Strongly consider an ICD (not an electronic pacemaker) due to risk of mortality from sudden cardiac death.
For symptomatic ventricular arrhythmias, ventricular tachycardia, ventricular fibrillation, & resuscitated sudden cardiac death: Use of an ICD Drug therapy as needed
Dilated cardiomyopathy	ACE inhibitors, beta blockers, diuretics, & other conventional approaches, as summarized in Yancy et al [2013] (full text)
When DCM is present & left ventricular ejection fraction is <35% (w/or w/o arrhythmia): use of an ICD
For progressive deterioration in left ventricular function despite full medical & device therapy, consider: Cardiac transplantation; Durable mechanical circulatory support if cardiac transplantation is contraindicated.

Manifestation/Concern

Treatment

Cardiac conduction
system disease &
arrhythmias

For chronic atrial fibrillation unresponsive to cardioversion:

Anticoagulants
Agents for ventricular rate control

For other symptomatic supraventricular arrhythmias:

Pharmacologic agents
May be augmented with electrophysiologic intervention (e.g., atrial or atrioventricular node ablations)

For symptomatic bradyarrhythmias or asymptomatic but significant heart block:

An implantable electronic pacemaker
Strongly consider an ICD (not an electronic pacemaker) due to risk of mortality from sudden cardiac death.

For symptomatic ventricular arrhythmias, ventricular tachycardia, ventricular fibrillation, & resuscitated sudden cardiac death:

Use of an ICD
Drug therapy as needed

Dilated
cardiomyopathy

ACE inhibitors, beta blockers, diuretics, & other conventional approaches, as summarized in Yancy et al [2013] (full text)

When DCM is present & left ventricular ejection fraction is <35% (w/or w/o arrhythmia): use of an ICD

For progressive deterioration in left ventricular function despite full medical & device therapy, consider:

Cardiac transplantation;
Durable mechanical circulatory support if cardiac transplantation is contraindicated.

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Adam MP, Feldman J, Mirzaa GM, et al., editors.

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