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)

Detección prenatal de cardiopatías congénitas y factores relacionados. Experiencia en un hospital nacional / Prenatal detection of congenital cardiopathies and related factors. Experience in a national hospital

The authors present a estudy related to the prenatal detection of congenital heart diseases. The congenital anomalies (CA) are morphological or functional disorders of prenatal origin, present since the birth, even they can be detected later on, during their lifes. They constitute the second cause of infantile death in our country, following, prenatal diseases (prematurity, perinatal infections, etc.) The most severe CA have relevance for the health and require usually clinical a surgical treatment. Congenital cardiopaties (CC) are the most frequent and represent a great impact in the mobimortality both neonatal and pediatric. Foetal echocardiography is a sensitive and specific method of investigation for prenatal detection of cardiac malformations. Experience has shown that echocardiographic screening can demonstrate, the presence of probably various cardiac disease in the foetus as early as 18-20 th weeks of pregnancy. It is recommended that screening should be carried out as part of other forms of obstetric ultrasonic screening. Our aims were to analyze the characteristies of the pregnants at risk factors to CC. To determine the associated factors to the fetal pathology of the fetal electrocardiogram, and to correlate the postnatal diagnosis by cardiac echodoppler, which impoves neonate survival and reduces morbidity. The results obtained are detailed in the article (AU)

Sindrome de corazón izquierdo hipoplásico: análisis de las características de una población en un hospital público / Hipoplastic left heart syndrome: analysis of the characteristics of a population in a public hospital

The authors present a study on prenatal diagnosis of hipoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome, and an analysis of the characteristics of a population in a public hospital. The hypoplastic left heart syndrome (HLHS) is a rare congenital heart deficit consisting in the hypodevelopment of the left ventricle, aortic valve, mitral valve and the ascendent aorta. Its incidence is 0,016% to 0.034% of the total of newborns alive. It represents between 1 - 3% of the whole congenital cardiopaties (cc). Its natural evolution is severe and it represents the greater cause of death by CC during the neonatal period. There is a predominance in males between the 55 to 67%. The etiology is multifactorial. Presents risk of recurrency between brothers, and because of that circumstance it could have genetical basis. The ais of this report were to analyze the clinical variables, electrocardiographis, echocardiographic and therapeutical, of a population with diagnosis fo HLHS. The incidence of HLHS in the population studied was 0.06% of life births. The results obtained are detailed (AU)

Diagnóstico prenatal de cardiopatías congénitas / PRENATAL DIAGNOSIS OF CONGENITAL HEART DEFECTS FOR DETECTING CARDIAC MALFORMATIONS

Congenital heart defects or congenital cardiac malformations represent one of the major causes of death in children younger than 1 year. The incidence varies between 4 - 13 for each 1000 newborns. Represents the severe structural malformation of the higher prevalence worldwide. In the "Professor A. Posadas Hospital" during the year 2015, it was registered a frequency of congenital anomalies of 7,1 % , being in the first place the musculoskeletal malformations with 19,4 %, and in the second place we can find the cardiovascular manifestations with 18.1 %. It is important the prenatal diagnosis in order to carry out an assessment to their parents, to reach the birth in a high complexity center and in the possible case, to plan the possibility of performing a fetal therapy. There exists scientific evidence that proves that the prenatal diagnosis improves the neonatal survival, even being this anomalies the less diagnosticated during the prenatal period These considerations are discussed in the article.