RESUMEN
Cardiac amyloidosis (CA) is a form of cardiomyopathy characterized by the extracellular deposit of protein fibers in the myocardium, leading to the development of heart failure, arrhythmias, and electrical conduction system alterations. It is known that most cardiomyopathies have a close relationship with heart rhythm abnormalities, however, CA is specially related to different kinds of arrhythmias even in pre-diagnosis stages. Arrhythmias like atrial fibrillation are present in up to 70% of patients with CA associated with a high risk of cardioembolic complications independent of the risk stratification. Ventricular arrhythmias are frequent, but the use of implantable cardioverter defibrillator has not been demonstrated to improve survival. The Atrial-Ventricular node disease is also common, and is frequently associated with the implantation of a pacemaker, even in asymptomatic patients. In this review, we clarify the recommendations of the most current guidelines, summarize historical and contemporaneous data and describe evidence-based strategies for the management of arrhythmias and their complications in CA.
RESUMEN
The risk of sudden death in hypertrophic cardiomyopathy is related to the presence of ventricular arrhythmias in most cases. Finding the best schemes to assess the probability of arrhythmic complications will remain a challenge for modern Cardiology. Meanwhile, the multifactorial approach is the best strategy to avoid the unnecessary implantation of devices such as the implantable cardioverter defibrillator. Although the electrocardiogram remains an excellent diagnostic tool, even before echocardiographic expression, it does not have a clear role as a risk factor. However, the identification of associated arrhythmias such as preexcitation or long QT and variants of presentation as apical hypertrophic cardiomyopathy, allows identifying patients with high probability of sudden death. During the last few years, cardiac resonance and quantification of intramyocardial fibrosis (the basic mechanism of ventricular arrhythmias) have gained an important role in the evaluation of these patients.In particular, pediatric patients must have an individualized approach due to the poor prognosis at early ages and the uncertain role of different tools for risk assessment and treatment.
RESUMEN
Se describe el caso de una mujer de 57 años de edad, con diagnóstico de síndrome de Wolf Parkinson White, en tratamiento con amiodarona 200 mg/día; que acudió por presentar palpitaciones, fatiga y aumento de sueño. Al examen físico no se encontró bocio, pero los reflejos osteotendinosos estaban prolongados. Los exámenes auxiliares mostraron TSH elevado, T4 libre disminuido y anticuerpos antiperoxidasa tiroidea negativos. Se suspendió la amiodarona y se inició levotiroxina. Después de dos meses; los niveles de TSH y T4 libre eran normales y la paciente no presentaba molestias. La amiodarona está asociada a diversos efectos adversos que pueden limitar su uso. Entre estos efectos adversos, se describe el hipotiroidismo inducido, que se caracteriza por TSH elevado, T4 libre disminuido y síntomas inespecíficos como fatiga, intolerancia al frío y piel seca. El tratamiento de elección es la levotiroxina.
We report a case of a Wolf Parkinson White syndrome in a 57-yearl-old woman receiving amiodarone (200 mg/ day) who presented with tachycardia, fatigue and somnolence. The physical examination did not reveal goiter, but the osteotendinous reflexes were brisk. Laboratory examinations revealed high TSH level, low T4 free level and negative antiperoxidase antibodies. Amiodarone was stopped and levotiroxine was started. Two months after, TSH and T4 free levels were normal and the patient was asymptomatic. Amiodarone is associated with several side effects that can preclude its indication; one of these side effects is hypothiroidism, characterized by high TSH levels, low T4 free levels and unspecific symptoms such as fatigue, cool intolerance and dry skin. The treatment of choice is levotiroxine.