cardiovascular disorders
Lamin A C Cardiomyopathy (Cardiolaminopathy)
Treatment of Lamin A C Cardiomyopathy
Moderation of physical activity Usually an implantable cardioverter-defibrillator (ICD) Sometimes antiarrhythmic drug therapy Heart failure therapy (including transplantation) as required Treatment focuses on prevention of sudden death and prevention of symptomatic ventricular tachyarrhythmias. Patients should avoid athletic exertion because such activities may hasten the progression of the cardiomyopathy and may foster life-threatening arrhythmias. Because of a high risk of sudden death, an ICD is typically implanted earlier in the course of illness in patients with lamin A/C cardiomyopathies than in others with dilated cardiomyopathy (see table Indications for Implantable Cardioverter-Defibrillators in Ventricular Tachycardia and Ventricular Fibrillation ). In addition to the normal recommendations for ICD use in patients with dilated cardiomyopathy and left ventricular ejection fraction of < 35% and in patients with sustained VT/VF or resuscitated cardiac arrest, placement of an ICD may also be useful (class IIa recommendation) for patients with lamin A/C cardiomyopathy who have unexplained syncope, an independent A beta-blocker should be used in most patients but may increase the possibility of AV block. Antiarrhythmic drug therapy with a class III drug, particularlysotaloloramiodarone, may reduce symptomatic ventricular tachyarrhythmias but is not a substitute for an ICD. However, these drugs may benefit patients who have frequent appropriate ICD discharges despite adequate beta-blocker therapy. Standard measures for Treatment are used as required. 