NT- Péptido natriurético cerebral en la evaluación de respuesta al tratamiento de la insuficiencia cardíaca con levosimendan en pacientes pediátricos / NT-brain natriuretic peptide in the assessment of response to levosimendan treatment in pediatric patients with heart failure

Introducción: El tratamiento de la insuficiencia cardiaca ha incorporado drogas inotrópicas de muy alto costo, como el levosimendan (LEVO). La evaluación de la respuesta a estas drogas en pediatría ofrece dificultades por lo que la medición de biomarcadores como la prohormona N-Terminal Péptido Natriurético Cerebral (NT-ProBNP) podrían ser de utilidad. Objetivo: describir la variación del NT-ProBNP y su correlación con parámetros ecocardiográficos en respuesta a la administración de levosimendan en pacientes pediátricos con insuficiencia cardiaca. Diseño: estudio descriptivo, observacional y prospectivo, sin intervención. Pacientes y métodos: se incluyeron pacientes con insuficiencia cardiaca de 0 a 18 años internados en terapia intensiva a los cuales se les pudo realizar dosaje de NT ProBNP pre LEVO. Se midió dicho péptido al 1°, 3° y 5° día post LEVO. Con cada determinación se realizó ecocardiograma doppler. Resultados: Se enrolaron 15 pacientes, mediana de edad 188,5 días (IQR 25-75: 56-475); de peso 5,475 kg (IQR 25-75: 2.8-7.5). El 80% fueron pacientes con reparación quirúrgica de cardiopatías congénitas, 13.3% con ventrículo único. La mediana de valor de NT ProBNPantes de la administración de LEVO fue 8924.5 pg./ml (IQR 25-75: 4096-20419,75). El 80% de la población presentó descenso en el valor de pro BNP post LEVO y en 10 (66.6%) el descenso fue mayor o igual al 30%. La evaluación global de la función miocárdica no presentó diferencias antes y después del LEVO. Conclusiones: El 66% de los pacientes presentó una disminución de al menos un 30% en los valores de NT ProBNP luego de la administración de levosimendan

Introduction: Very high-cost inotropic drugs, such as levosimendan (LEVO), have been incorporated in the treatment of heart failure. Evaluation of the response to these drugs in children is difficult and measurement of biomarkers such as the prohormone N-terminal pro b-type natriuretic peptide (NT-proBNP) may be of use. Objective: To describe variation of NT-ProBNP and its correlation with echocardiographic parameters in response to the administration of LEVO in pediatric patients with heart failure. Design: A prospective, descriptive, observational, non-interventional study. Patients and methods: Patients between 0 and 18 years of age with heart failure admitted to the intensive care unit in whom pre-LEVO NT-ProBNP levels could be measured were included. NT-ProBNP levels were measured at day 1, 3, and 5 post LEVO. At each measurement, a Doppler echocardiogram was performed. Results: 15 patients with a median age of 188.5 days (IQR 25-75: 56-475) and a weight of 5.475 kg (IQR 25-75: 2.8-7.5) were enrolled. Of the patients, 80% had undergone surgery for congenital heart defects, 13.3% with a single ventricle. Median NT-ProBNP levels before LEVO administration were 8924.5 pg./ml (IQR 25-75: 4096-20419.75). Overall, 80% of the patients had a decrease of post-LEVO NT-ProBNP levels and in 10 (66.6%) the decrease was greater than or equal to 30%. Overall evaluation of myocardial function did not show differences before and after LEVO administration. Conclusions: 66% of the patients presented with a decrease of at least 30% of NT-ProBNP levels after LEVO administration

No Synergic Effect of Sildenafil Administration on Exercise Capacity Improvement in a Fontan Patient with Regular Exercise Training.

In Fontan patients, reduced exercise capacity due to diminished cardiac output is a common finding with important prognostic implications. Beneficial effects have been shown for sildenafil treatment and regular exercise, but data comparing both strategies is scarce. We report on a female patient with Fontan circulation who underwent repeated cardiopulmonary exercise tests with either placebo or a single dose of 50mg sildenafil before and after 6months of supervised aerobic and resistance exercise. At baseline, V O2peak was 29.1ml/min/kg, and a marked increase to 32.8ml/min/kg was observed after administration of sildenafil. After the training period, V O2peak was 34.5ml/kg/min in the placebo test, and no further increase by sildenafil was possible (33.7ml/kg/min). Similar results were observed for exercise capacity at the ventilatory anaerobic threshold. In summary, this Fontan patient showed that regular exercise might use up and probably exceed the acute sildenafil effects on exercise capacity. Exercise should be considered as a primary treatment strategy within secondary prevention and rehabilitation after the Fontan procedure.

Alternativas terapéuticas para la insuficiencia cardíaca en los ancianos: [revisión] / Therapeutic alternatives for treating heart failure in elderly patients: [review]

La insuficiencia cardíaca (IC) es una importante causa de mortalidad en todo el mundo y el principal motivo de hospitalización de origen no quirúrgico en muchos países. Existe un gran número de variables predictivas acerca del pronóstico de pacientes con IC, una de ellas es la edad. Además, la comorbilidad por causa no cardíaca dificulta el tratamiento en un importante grupo de pacientes ancianos con IC y casi la mitad de los pacientes con síntomas de IC presenta una fracción de eyección del ventrículo izquierdo conservada. Sin embargo, los ensayos clínicos en IC no se han centrado ni en el grupo de pacientes ancianos ni en aquellos con fracción de eyección conservada. Por esta razón no se han establecido recomendaciones específicas para este grupo. Este artículo revisará los estudios más importantes sobre IC realizados en los últimos años y analizará los resultados en pacientes de edad igual o superior a 65 años. Esta revisión incluye los betabloqueantes, inhibidores de la enzima convertidora de angiotensina, antagonistas del receptor de la angiotensina, antagonistas de los receptores de la aldosterona, nitratos más hidralazina, digoxina, estatinas, el desfibrilador automático implantable y la resincronización cardíaca.

Losartan induced fetal toxicity.

Losartan is a specific angiotensin II receptor antagonist. Although the teratogenic effects of angiotensin converting enzyme (ACE) inhibitors are well documented there are limited reports of losartan induced fetal toxicity. The authors report a case of incomplete ossification of skull bones, transient oliguria and feed intolerance in a newborn following in-utero exposure to losartan.

Isolated noncompaction of the myocardium

We report the case of a 42-year-old female with fatigue on exertion and palpitation consequent to the existence of isolated noncompaction of the myocardium. We discuss clinical and familial findings, diagnostic possibilities, and prognostic and therapeutical implications of this rare disorder of endomyocardial morphogenesis.

Prostaglandin E1 in infants with congenital heart disease: Indian experience.

BACKGROUND: E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore and be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decade in western world, it has been introduced in India only since April, 1995. OBJECTIVE: To assess the efficacy of PGE1 at our center. SETTING: Hospital based. METHOD: 65 infants with ductus dependent congenital heart disease were included. Age at time of starting PGE1 infusion ranged from 18 hours to 39 days. Forty two of these were more than a week of age, 19 were more than 14 days, and two were above one month. PGE1 was started in an initial dose of 0.05 microgram/kg/min, decreased to 0.005-0.01 microgram/kg/min for maintenance. The indications for use of PGE1 were to increase pulmonary blood flow in 33 cases with pulmonary atresia, tricuspid atresia or critical pulmonic stenosis (Group I); to increase systemic blood flow in 15 cases with coarctation of aorta, hypoplastic left heart and interruption of aortic arch (Group II); to improve mixing in 13 cases of transposition of great arteries (Group III) and for improving the left ventricular volumes by keeping the duct open in 4 cases of transposition of great arteries with intact ventricular septum (Group IV). The efficacy of the drug was assessed by a rise on PaO2 and SaO2% determined for Group I & III, and by appearance of lower limbs pulses in Group II. Left ventricular volumes were serially measured by echocardiography in Group IV cases. RESULTS: The drug was successful in 62 of the 65 cases. There were two failures. One was a 39 days old baby with Ebstein's anomaly of tricuspid valve and pulmonary atresia and other was an eight days old baby with coarctation of aorta and renal failure. In addition, PGE1 could not be continued in another baby due to development of a linear skin rash locally. Side effects included apnea in 5 (9%) of 56 spontaneously breathing patients. Necrotizing enterocolitis, hyperpyrexia and jitteriness was sent in one case each. Six patients died. Two were related to PGE1, one due to failure, another due to its side effects. Definitive procedure were performed in 51 cases electively. PGE1 was used upto 13 days with sustained benefit. CONCLUSIONS: PGE1 is an effective drug for keeping the ductus open in infants with ductus dependent congenital heart disease. It can be used for neonates beyond the first week of life with efficacy. Apnea is a major side effect and close monitoring is essential.

Prostaglandin E1: first stage palliation in neonates with congenital cardiac defects.

E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore can be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decades in western world, it has been introduced in India only since April, 1995. Various cardiac defects where PGE1 is useful include (a) lesions with ductus dependent pulmonary blood flow e.g. pulmonary atresia with or without ventricular septal defect, critical valvular pulmonic stenosis etc, (b) lesions with ductus dependent systemic blood flow e.g. critical aortic stenosis, coarctation of aorta, interruption of aortic arch etc, and (c) admixture lesions like transposition of great arteries. The drug is given as a continuous intravenous infusion. The initial dose is 0.05 to 0.4 ug/kg/min, infusion rate must be decreased to 0.01 ug/kg/min as soon as the desired effect is achieved as incidence of side effects is more at higher doses. Serious side effects include apnoea, hypotension, hyperthermia, seizures etc. We have used this drug in 43 infants ranging in age from one to forty five days. Beneficial response was seen in 41 of 43 infants and the major side effect was apnoea (seen in 5 of 32 spontaneously breathing infants). Unfortunately the high cost of the drug prohibits its wide spread and long term use. PGE1 is a life saving drug for infants born with ductus dependent congenital cardiac malformations. It helps in stabilizing these patients prior to further surgical palliation or correction.

Inhaled nitric oxide and high concentrations of oxygen in pediatrics patients with congenital cardiopathy and pulmonary hypertension: report of five cases

Five patients with ages ranging from 6 months to 3 years were analyzed. All received inhaled nitric oxide (NO - 20 parts per million (ppm)) and oxygen (O2 - at a concentration of 90-95 percent) by means of an oxygen hood. Mean Pulmonary Artery Pressure (MPAP), Mean Aortic Pressure (MAoP), Pulmonary Vascular Resitance (PVR) and Systemic Vascular Resistance (SVR) were measured and the calculation of their relationship to pulmonary/systemic flow (Qp/Qs) was performed by the catheterization of the femoral artery vein. Three patients presented reduction in PVR and increase in Qp/Qs. There were no systemic alterations or any side effect from using NO.

Enalapril oral en pacientes con comunicación interventricular sintomáticos / Oral enalapril in symptomatic patients with interventricular septal defect

Estudios en animales de experimentación y clínicos en el laboratorio de hemodinámica, han mostrado que los EICAs disminuyen el cortocircuito de izquierda a derecha en la comunicación interventricular (CIV). El objetivo del estudio es valorar los efectos del enalapril, a corto y mediano plazo, en pacientes con CIV sintomáticos y Qp:Qs mayor de 1.5:1 y compararlos con el tratamiento convencional de insuficiencia cardiaca congestiva (ICC) en niños. Se estudiaron 24 pacientes entre 3 meses y 8 años de edad. En cada grupo se estudiaron ocho pacientes. El grupo I fue tratado con enalapril, el grupo II con furosemida y digoxina y el grupo III con los tres medicamentos. Los controles se efectuaron al mes, tres y seis meses, En cada cita se evaluaron datos clínicos de ICC, peso, frecuencia cardiaca, tensión arterial, índice cardiotorácico y ecocardiograma con las mediciones de las cavidades cardiacas, tamaño de la CIV y parámetros hemodinámicos (Qp:Qs, PSAP, gradiente de la CIV, FEy FA). El análisisi estadístico se realizó por grupo y comparando los tres grupos. Los resultados mostraron un aumento de peso significativo (p<0.05) para los tres grupos. Además, se observó una disminución de la frecuencia cardiaca, Qp:Qs y PSAP en el grupo III. Sin embargo, al comparar los parámetros estudiados entre los grupos, el análisisi estadístico no mostró diferencias significativas. No se observaron efectos colaterales. El uso del enalpril, asociado con la furosemida y digoxima, puede ser de utilidad en los pacientes sintomáticos, en espera de la cirugía de cierre de la CIV

Midazolam en la premedicacion del nino cardiopata / Administration of midazolam as premedication in children presenting with heart diseases

Se destacan las ventajas del midazolam en la premedicacion de los ninos que padecen de cardiopatias congenitas. Se explican brevemente, las caracteristicas farmacologicas mas importantes. Se presentan los resultados obtenidos en 200 pacientes pediatricos cardiopatas que fueron premedicados con esta benzodiazepina

Síndrome de Holt-Oram

Se presenta un caso de síndrome de Holt-Oram en una paciente de 27 años de edad, estudiada en el Servicio de Cardiología del Hospital San José de Bogotá, con todos los criterios diagnósticos consistentes en malformaciones esqueléticas en miembros superiores y cardiopatía congénita tipo comunicación interauricular ostium secundum. Se hace una revisión de la literatura con énfasis en la importancia de su diagnóstico y la consejeria genética en este tipo de patologia.

Infusäo de prostaglandina E1 em cardiopatias congênitas canal-dependentes: análise de 47 casos / Infusion of prostaglandin E1 in duct dependent congenital heart diseases: analysis of 47 cases

Foram estudados 47 lactantes submetidos a infusäo de prostaglandina E1 (PGE1), no período de dezembro de 1985 a abril de 1988. Idades variaram de 12 horas a 70 dias (média de 10,3 dias), o peso corporal de 1990 g a 4430 g (média de 3005 g). A dose média da infusäo venosa foi de 0,021 mcg/kg/min, variando de 0,013 a 0,089 mcg/kg/min, abaixo da dose preconizada habitualmente. Considerou-se efetiva a terapêutica em 36 (76,55) pacientes que demonstraram melhora clínica, aumento da saturaçäo do O2 arterial acima de 15 vol. O2% e maior diâmetro do canal arterial avaliado através da ecocardiografia. Na correlaçäo entre a resposta terapêutica e a idade do paciente, a maior elevaçäo da saturaçäo de pO2 arterial nos recém-natos ocorreu até 21 dias, em especial até sete dias, em cujo período o aumento médio foi de 24,5 vol. 02. Das cardiopatias que mais se beneficiaram, salientam-se a atresia pulmonar com ou sem CIV, anomalia de Ebstein, atresia tricúspide, estenose pulmonar valvar, dupla via de saída de VD e as ue menos se beneficiaram corresponderam a hipoplasia do coraçäo esquerdo, tetralogia de Fallot, TGA. Os efeitos colaterais, todos reversíveis, näo guardam relaçäo com a dose empregada, sendo os mais freqüentes a apnéia em 40.4%, hipertermia em 29.7%, taquicardia em 19,1%, bradicardia e erupçäo cutânea em 17%. A PGE1 pôde ser usada em período mais prolongado (média de 975 horas) em três pacientes, por prematuridade, infecçäo e complexidade de defeito, abrindo campo de observaçäo similar...