Current status of pediatric electrophysiology in Latin America: a LAHRS survey.

PURPOSE: Over the last years, pediatric electrophysiology (EP) has evolved as a consolidated subspecialty of pediatric cardiology. In the USA, Canada, and Europe, there is a clear picture of the status of pediatric EP, but the situation in Latin America is largely unknown. METHODS: A descriptive cross-sectional observational study was performed. A survey was conducted by the Latin American Heart Rhythm Society in Spanish and Portuguese to assess the status of development of pediatric EP across Latin American countries. RESULTS: There are physicians practicing pediatric EP in 11 Latin American countries. The scope of the practices includes clinical and non-invasive EP as well as performing invasive EP procedures. All the current pediatric EP interventions are performed in most of Latin American countries. CONCLUSIONS: Pediatric electrophysiology is present as a subspecialty in half of the countries in Latin America, and all the current electrophysiology procedures and therapeutic technologies are available in most Latin American countries.

Miocardiopatía arritmogénica del ventrículo derecho: particularidades del enfrentamiento diagnóstico en pediatría / Arrhythmogenic right ventricle cardiomyopathy: particularities of the diagnostic approach in pediatrics

Resumen: La miocardiopatía arritmogénica del ventrículo derecho es una patología de origen genético cuya base molecular se encuentra a nivel de los desmosomas, caracterizada por una sustitución progresiva de los miocitos del ventrículo derecho por tejido graso, que conduce a arritmias potencialmente graves y disfunción miocárdica. Es causa de muerte súbita asociada al ejercicio. Es rara la aparición de síntomas antes de los 10 años de edad, lo que obliga a un alto grado de sospecha clínica. Existe afectación familiar hasta en el 50% de los casos, por lo que el estudio en cascada está recomendado y constituye un criterio mayor de enfermedad. Se cree que esta investigación es la forma más frecuente de pesquisa en pediatría, junto con la realización de un electrocardiograma como parte del screening preparticipativo en competencia deportiva. Se debe sospechar ante la objetivación de taquicardia ventricular con imagen de bloqueo de rama izquierda. Las alteraciones clásicas descritas en el ecocardiograma o la resonancia magnética son raras en niños, y deben considerarse las anomalías segmentarias en estas técnicas. La estratificación de riesgo ha debido ser extrapolada de los estudios realizados en adultos, y de acuerdo a esto se debe decidir la pertinencia de la instalación de un desfibrilador automático implantable, sobre todo en caso de muerte súbita cardíaca recuperada o taquicardia ventricular sostenida. La pertinencia del uso de fármacos debe analizarse individualmente. En los casos de diagnóstico definitivo, se debe abolir el ejercicio intensivo, recomendándose solo la actividad física de baja intensidad.

Summary: Arrhythmogenic right ventricle cardiomyopathy is a disease of genetic origin, whose molecular basis is at the level of desmosomes, characterized by a progressive replacement of right ventricle myocytes by fatty tissue, which leads to potentially serious arrhythmias and myocardial dysfunction. It is a cause of sudden death associated with exercise. The appearance of symptoms before 10 years old is rare, which compels a high degree of clinical suspicion. In more than 50% of cases there is a family history of those affected, so the cascade study of the probands is recommended (major criterion of disease). In pediatrics, it is believed that most often is that patients are investigated by this ground, as well among asymptomatic children in whom an electrocardiogram is performed as part of the screening of sports competition. One should suspect arrhythmogenic right ventricle cardiomyopathy with ventricular tachycardia with appearance of left bundle branch block. Classical alterations described in echocardiography or magnetic resonance imaging are rare in children, and segmental abnormalities in these techniques should be considered. The stratification of risk has been extrapolated from the studies of elderly patients, and according to this, the indication of an implantable cardioverter defibrillator must be decided, especially in case of sudden cardiac death recovered or sustained ventricular tachycardia. The relevance of the use of drugs must be individually analyzed. In definite cases, intensive exercises should be abolished, recommending only those of low intensity.