Prevalencia y espectro de las enfermedades que predisponen a la muerte súbita cardiaca en niños mexicanos: una muestra obtenida del Hospital Infantil de México Federico Gómez / Prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children: a sample obtained from The Federico Gomez Children's Hospital of Mexico

Resumen Objetivo: Determinar la prevalencia y espectro de las enfermedades que predisponen la muerte súbita cardiaca en niños mexicanos e identificar los principales signos y síntomas tempranos que pueden permitir al personal de salud sospechar acerca de estas enfermedades y referir a los pacientes a un hospital de tercer nivel de manera temprana. Métodos: La incidencia, prevalencia y prevalencia de periodo, así como los primeros síntomas, los datos clínicos y el seguimiento, se describen en todos los niños con enfermedades que predisponen a la muerte súbita cardiaca en el Hospital Infantil de México. Resultados: Cincuenta y nueve pacientes de 8 ± 5 años, 40 con miocardiopatías y 19 con enfermedades arritmogénicas hereditarias. La prevalencia del periodo fue de 9.5/1,000 pacientes/año. Los primeros síntomas más comunes fueron disnea, palpitaciones y síncope. En 9 casos se encontró un patrón de herencia mendeliana. Tres pacientes fallecieron de muerte súbita cardiaca durante el periodo de estudio. Conclusión: Las enfermedades que predisponen a la muerte súbita cardiaca en los niños no son muy conocidas por la comunidad médica y general. Todo niño con disnea, palpitaciones y/o síncope debe referirse para la búsqueda intensiva de estas enfermedades. Una evaluación cardiológica completa en todos los miembros de la familia está indicada.

Abstract Objective: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. Methods: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. Results: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. Conclusion: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso / Persistent fifth aortic arch with patent ductus arteriosus

La persistencia del quinto arco aórtico es una rara anomalía congénita vascular que consiste en la presencia de una comunicación entre la aorta ascendente y la aorta descendente a través de un conducto arterial; se diagnostica de manera incidental. Informamos de un caso asociado a persistencia de conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding ocasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

Evaluación del flujo de las venas pulmonares con ecocardiografía transtorácica en niños con cardiomiopatía dilatada / Pulmonary venous flow in children with dilated cardiomyopathy using transthoracic echocardiography

OBJECTIVE: To evaluate the pulmonary venous flow in children with dilated cardiomyopathy and to establish its correlation with mitral Doppler flow and with the functional class. METHODS: It is a descriptive and transversal study in which we evaluated the pulmonary venous flow and its correlation with mitral Doppler flow and the functional class in 14 children with dilated cardiomyopathy. RESULTS: Nine patients were female. The mean age was 5.58 years and the mean follow up was 17 months. The functional class was I in 7 patients, II in 4 and III in 3. When diastolic function was evaluated using mitral Doppler flow it was abnormal in 9 of 14 patients, while the pulmonary venous flow was altered in all 14 patients since S/D ratio was abnormal in all. S wave was reduced in 12 patients; D wave was elevated in 8 patients, A reverse wave was elevated in 4 patients and in 8 the A reverse wave length was higher than mitral A wave length. CONCLUSIONS: Systolic function of the left ventricle is reduced in children with dilated cardiomyopathy. Diastolic function is also frequently abnormal in this group of patients. Pulmonary venous flow identifies better the abnormal diastolic function and correlates better with functional class than mitral Doppler flow.