Coartación de aorta y su asociación con la patología mitral / Aortic Coartaction and its association with the mitral pathology

Coartaction of the aorta is characterized by a localized deformity narrowing the aortic lumen. The zone of coartaction is characteristically located beyond the origin of the left subclavian artery. Most of them are located in the zone of the posterior wall of the oposit aorta of the invertion of the ductus. The coartaction of the aorta amounts up to 5.1% of the congenital cardiac malformations and constitutes the eigth cardiac malformation in frequency; Is a malformation tipically associated with the Turner syndrome. The aims of this report were to describe and to analyze the mitral pathology and the clinicosonographic experience in patients with coarctation of the aorta associated to congenital mistral pathology during the last 10 years. An observtiona, descriptive and retrospective study was performed. The history of 103 patients with a diagnosis of coartaction of the aorta assisted in our Hospital were studied. All the patients were confirmed by color Doppler echocardiography. Assuming that the mitral pathology is a frequent association that affects strongly the morbi-mortality and the prognosis of such patients is an indisensable condition to investigate its presence in, front of a diagnosis of coartaction of the aorta (AU)

Compromiso cardiaco en pacientes con Mucopolisacaridosis tipo II (Enfermedad De Hunter) / Cardiac commitment in patients with type II Mucopolysaccharidosis. (Hunter's disease)

This report describes the cardiac involvement of patients with mucopolysaccharidoses Type II (Hunter disease). Mucopolysaccharidoses Type II are an uncommon group of about 50 rare inherited metabolic disorders, that result from defects in lysosomal dysfunction, usually as a consequence of deficiency of a single enzyme required for the metabolism of lipids, glycoproteins or so called mucoplysaccharides. Most of this diorders are autosomal recesively inherited such as Hunter syndrome Mucopolysacharidosis. Tuype II is a lisosomal storage disease caused by a deficiency of the lysosomal ensyme iduronate 2 sulfatase. its frequency is 1 to 100.000 to 150.000 male births; is farmore common in boys. Clinical, electrocardiographical and sonographical variables were determined. As a result 18 patients were evaluated; all the patients presented cardiac involvement. Color Doppler sonocardiogram was pathological in the 100% of the patients, and 4 of them, showed mitral/and or aortic, and 4 patients with miocardic hypoertrophy, and 1 patient, pulmonary hipertension. A clinical review is prsented, and a guide for management is detailed (AU)