Miocardiopatía hipertrófica obstructiva precoz tras el tratamiento con dexametasona en un recién nacido pretérmino / Early hypertrophic obstructive cardiomyopathy after dexamethasone treatment in a preterm newborn

El uso de corticoides sistémicos para la prevención y/o el tratamiento de la displasia broncopulmonar en prematuros se ha asociado a la aparición de efectos secundarios a corto y largo plazo, y la afectación neurológica es el efecto que ha limitado su uso. Los efectos a corto plazo incluyen los metabólicos y endocrinológicos (fundamentalmente la hiperglucemia y la supresión del eje hipotálamo hipofisario-adrenal), los cardiovasculares (hipertensión, hipertrofia miocárdica), la mayor susceptibilidad para las infecciones y los efectos digestivos (sangrado), aunque éstos suelen ser transitorios y reversibles. La miocardiopatía hipertrófica en prematuros es un efecto frecuente del uso de corticoides, aunque en general carece de relevancia clínica, ya que requiere habitualmente para su aparición tratamientos de una duración superior a los 7 días. Para paliar este efecto se deben retirar o disminuir los corticoides, y se recomienda el uso de propranolol en los casos con una obstrucción dinámica en la salida del ventrículo izquierdo, o si hubiera datos de isquemia miocárdica. Presentamos el caso de un recién nacido de muy bajo peso al nacimiento que desarrolló precozmente una miocardiopatía hipertrófica obstructiva con repercusión clínica tras 2 días de tratamiento con dexametasona(AU)

The use of corticosteroids for the prevention and/or treatment of bronchopulmonary dysplasia in premature infants have been associated with short and long term side-effects being neurological impairment the most important that limit their use. Short-term sideeffects include metabolic and endocrine (hyperglycemia and suppression of the hypothalamic-pituitary-adrenal axis), cardiovascular (hypertension, myocardial hypertrophy), increased susceptibility to infections and gastrointestinal (bleeding). These are usually transient and reversible. Hypertrophic cardiomyopathy in preterm infants is a common side-effect with the use of corticosteroids but usually has not clinical relevance and it is observed in treatments longer than 7 days. Treatment is the withdrawal or reduction of corticosteroids and the use of propranolol in patients with dynamic obstruction in the left ventricular outflow or whether there are signs of myocardial ischemia. We present the case of a very low birth weight newborn who developed early symtomatic hypertrophic obstructive cardiomyopathy after 2 days of treatment with dexamethasone(AU)

Errores congénitos del metabolismo: una carrera de obstáculos / Congenital errors of metabolism: an obstacle race

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[The clinical and echocardiographic factors predictive of bioprosthetic leaflet rupture. The clinical tolerance for prosthetic malfunction]. / Factores clínicos y ecocardiográficos predictivos de rotura de velos bioprotésicos. Tolerancia clínica a la disfunción protésica.

OBJECTIVE: The objective of this research was to determine the existence of predictors of bioprosthetic valve disruption depending on the echocardiographic characteristic of the biologic prosthesis. It also determines the factors that provoke a need of emergency surgery and a bad clinical tolerance to the prosthetic dysfunction. METHODS: Clinical and echocardiographic features of two groups of 28 and 21 patients, all of them carrying bioprosthesis, were compared. The groups were homogeneous in sex, age, location and durability of the prosthesis. The first group showed leaflet disruption in a period of 6 months to 1 year after the study, not in the second group. Calcification, thickness and mobility of leaflets, prosthetic function were studied. An echocardiographic score was given between 4 and 10 points. Left ventricular function and pulmonary pressure were also studied. RESULTS: Valve thickness was statistically different in both groups (95% of the first group vs 71.3% of the second, p = 0.0281). There was no significant difference in calcification (52% vs 38.7%), nor in leaflet mobility (71.5% normal in the first group vs the 80% of the second group), nor in the prosthetic function (52.3% vs 76.6%, respectively). It was not found in the echocardiographic score either. Left ventricular function and pulmonary pressure were 91.6% and 55% normal respectively in the first group. They were 80% and 45.83% normal in the second group. Clinical onset of disruption was: 5 asymptomatic patients, 2 patients noticed a change in prosthetic click, 14 cases with progressive dyspnea, 6 patients with acute pulmonary edema and 1 patient with cardiopulmonary arrest and effective resuscitation. Symptoms were kept under control with medical treatment in 22 patients (78.55%) and 6 patients were referred to surgery. These latter suffered from pulmonary hypertension and two of them had left ventricular dysfunction. Surgical mortality was 3% (1 patient). CONCLUSIONS: No echocardiographic features were found as being predictors of imminent bioprosthetic disruption. However, leaflet thickness is the most common finding. Symptoms of disruption in patients without high surgical risk factors (left ventricular dysfunction or pulmonary hypertension) are kept under control with medical treatment. Therefore, "prophylactic" surgery is not needed in bioprosthesis with signs of degeneration and normal hemodynamic performance.

[The independent origins of the anterior descending, circumflex and right coronary arteries from the right coronary sinus]. / Orígenes independientes de las arterias coronarias descendente anterior, circunfleja y derecha del seno coronario derecho.

We present a patient with chronic ischaemic cardiac disease and anomalous left anterior descending and circumflex coronary arteries with independent origin from the right Valsalva sinus. Each one had his own ostium and the left anterior descending artery followed an intramyocardial pathway through the interventricular septum whereas the circumflex artery had a retrocardiac pathway. The clinical picture of the patient was related to the presence of atherosclerotic obstructive lesions in right, whose origin and curse were normal, and circumflex coronary arteries. He was treated medically at first but without obtaining complete control therefore he needed surgical treatment which was undertaken without technical difficulties. We reviewed the anomalies of the main coronary arteries, in the origin and course, with its clinical signification, diagnosis and therapeutics topics.