Changes in serum chromogranin A and urotensin II levels in children with chronic heart failure / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To examine the changes in serum chromogranin A (CgA) and urotensin II (U II) levels in children with chronic heart failure (CHF) and their clinical significance.</p><p><b>METHODS</b>A total of 58 children with CHF, among whom 17 had endocardial fibroelastosis (EFE) and 41 had dilated cardiomyopathy (DCM), were selected as CHF group, and 20 healthy children were selected as control group. Serum levels of CgA and U II were measured using enzyme-linked immunosorbent assay, and the level of N-terminal pro-brain natriuretic peptide (NT-proBNP) was determined by bi-directional lateral flow immunoassay. Ventricular remodeling indices were measured using echocardiography. The correlation between serum CgA and U II levels and ventricular remodeling was evaluated by Pearson correlation or Spearman's rank correlation analysis.</p><p><b>RESULTS</b>There were no significant differences in serum CgA and NT-proBNP levels between children with grade II heart function and the control group (P>0.05). However, the serum CgA and NT-proBNP levels gradually increased as the heart function grade increased, and were significantly higher in grade III and IV children compared to those in the control group (P<0.05). U II levels were lower in children with grade II, III, or IV heart function than those in the control group (P<0.05), and significantly decreased with the aggravation of CHF (P<0.05). There were no significant differences in CgA and U II levels between patients with EFE and DCM (P>0.05). Serum CgA concentration was positively correlated with left ventricular mass index (LVMI), NT-proBNP, and cardiac function classification (r=0.279, 0.649, and 0.778 respectively; P<0.05), but was negatively correlated with left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), and U II (r=-0.369, -0.322, and -0.718 respectively; P<0.05). Serum U II concentration was negatively correlated with NT-proBNP and cardiac function classification (r=-0.472 and -0.591 respectively; P<0.05), but was not correlated with LVMI, LVEF, and LVFS (P>0.05).</p><p><b>CONCLUSIONS</b>CgA may play a role in ventricular remodeling in children with CHF. Serum CgA and U II may serve as a reference for the diagnosis and functional classification of heart failure.</p>

Congenital atresia of left main coronary artery in 4 children: case report and literature review / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestations and treatment of congenital atresia of the left main coronary artery (CLMCA-A).</p><p><b>METHOD</b>Four patients were diagnosed to have CLMCA-A from June 2010 to June 2012 in Beijing Anzhen Hospital. Clinical manifestations, ultrasound, ECG and angiographic characteristics were analyzed and summarized.</p><p><b>RESULT</b>Of the 4 cases, age of onset was 3 months to 2 yrs. Three cases were diagnosed by angiography, and 1 case by CTA . All 4 cases had chronic heart failure symptoms and signs, such as sweating, shortness of breath, easily choked by milk, predispose to pneumonia, activity intolerance. ECG showed abnormal Q wave and other ischemic signs such as ST-T segment depression. Ultrasonography showed left ventricular enlargement, left ventricular systolic function was normal or slightly reduced, and there was moderate to large amount of mitral valve regurgitation. Left ventricular trabeculations increased. Coronary collateral circulation increased. Left coronary artery appeared to be slender and disconnected with left coronary artery sinus. Aortic root angiography was the golden diagnostic standard. Angiography was performed in 3 patients and showed that left main coronary artery had a blind end, diameter 1.1-2.0 mm. The right coronary artery was found rising from the right coronary sinus and visible on coronary collateral circulation. Contrast agent developing sequence: right coronary artery-collateral vessels-left coronary artery distal branches-left main coronary artery. CTA exam was performed in 2 cases and in 1 case the diagnoses was confirmed. All the 4 patients are currently in the close follow-up, digoxin and diuretics were taken everyday and clinical symptoms were improved.</p><p><b>CONCLUSION</b>CLMCA-A is not rare, its clinical manifestations should be differentiated from those of cardiomyopathy, endocardial fibroelastosis, congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc. For pediatric patients with cardiac enlargement, abnormal heart function, mitral valve regurgitation etc, attention must be paid to consider the developmental abnormality of coronary artery, particularly the CLMCA-A diagnosis.</p>

Changes of heart-type fatty acid-binding protein in children with chronic heart failure and its significance / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study serum levels of heart-type fatty acid-binding protein (h-FABP) in children with chronic heart failure (CHF), and the correlation between heart function and the level of h-FABP, with the aim of studying the significance of h-FABP in CHF.</p><p><b>METHODS</b>Thirty-six children with CHF, including 16 cases of endocardial fibroelastosis (EFE) and 20 cases of dilated cardiomyopathy (DCM) were enrolled in the study. Thirty healthy children sevred as the control group. Serum levels of h-FABP were determined using ELISA, and left ventricular ejection fraction (LVEF), cardiac index (CI) and fractional shortening of the left ventricle (LVSF) were measured by two-dimensional echocardiography in the CHF group.</p><p><b>RESULTS</b>Mean levels of h-FABP in the CHF group were significantly higher than in the control group (21.7±4.3 ng/mL vs 6.2±1.7 ng/mL; P<0.01). The worse the heart function, the higher the h-FABP levels (P<0.01). Mean levels of h-FABP in both the EFE and DCM groups were significantly higher than in the control group (P<0.01). Serum h-FABP concentrations were negatively correlated with LVEF, CI and LVSF (r=-0.65, -0.64 and -0.71 respectively; P<0.01) in the CHF group.</p><p><b>CONCLUSIONS</b>Serum h-FABP levels increase in children with CHF and are closely related to the severity of the condition. Serum h-FABP levels can be used as a biomarker for the diagnosis of heart failure and the evaluation of its severity.</p>

Advance in research on endocardial fiborelastosis / 中国当代儿科杂志

Endocardial fiborelastosis (EFE) is a common infantile myocardiosis. The pathogenesis of EFE may be associated with viral infection, genetic factors, immune factors and endocardial dysplasia. The fundamental pathological changes of EFE include hyperplasia of endocardium elastic fibers and collagen fibers. Acute EFE is a frequent type. Clinical manifestations of EFE are non-specific and children with EFE mainly present with congestive heart failure. Echocardiography is very helpful to the diagnosis of EFE. It is necessary to differentiate EFE from pneumonia complicated by acute congestive heart failure, viral myocarditis and anomalous origin of the left coronary artery. Treatment is meant to control symptoms of congestive heart failure. Patients who respond well to digitalis and have good medication compliance have a favorable prognosis.

Fibroelastosis cardíaca fetal / Fetal cardiac fibroelastosis

Se detalla el caso clínico de una gestante de 30 años (segundo trimestre del embarazo), residente en el poblado Siboney, quien asistió a la consulta de ecocardiografía fetal del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, donde se le diagnosticó una fibroelastosis cardíaca fetal a las 23 semanas y malformación renal. Fue remitida al Cardiocentro de esta ciudad y a la consulta de genética provincial, de manera que al confirmarse la miocardiopatía congénita en el feto, la pareja optó por interrumpir la gravidez. La necropsia reveló fibroelastosis endocárdica, estenosis aórtica moderada, hipoplasia ligera de la aorta ascendente y displasia renal bilateral.

The case report of a 30 year-old pregnant woman (second trimester) is detailed, who lives in Siboney neighborhood and attended the fetal ultrasonography department from Dr Juan Bruno Zayas Alfonso Teaching General Hospital of Santiago de Cuba, where she was diagnosed with fetal cardiac fibroelastosis at the 23 weeks and renal malformation. She was referred to the Cardiology Center of this city and to the provincial genetic department, and when congenital fetal myocardiopathy was confirmed the couple decided to terminate the pregnancy. The autopsy revealed endocardial fibroelastosis, moderate aortic stenosis, mild hypoplasia of the ascending aorta and bilateral renal dysplasia.

A long-term follow-up study on the clinical treatment of 75 cases with primary endocardial fibroelastosis / 中华儿科杂志

<p><b>OBJECTIVE</b>To summarize and analyze the effects of treatment and prognosis of infants with endocardial fibroelastosis (EFE) in different states of the illness undergone relevant therapies, and to understand the roles of different treatments for improving the prognosis of the disease.</p><p><b>METHODS</b>Data of 75 cases with EFE admitted into Anzhen Hospital Affiliated to Capital Medical University from August 1984 to June 2006 were analyzed retrospectively.</p><p><b>RESULTS</b>(1) Of the 75 cases with EFE (40 males and 35 females), with the onset age ranged from 20-days to two years and eight months, 69 cases were treated normally and followed up in the Outpatient Department of the Hospital after discharge, the follow-up rate was 92%, with the follow-up span from six months to 23 years (5.7 years in average). During the follow-up, six cases (8.7%) died. (2) The total curative rate of EFE patients was 46.4% (32/69), while the improvement rate was 40.6% (28/69), the total rate of the cure and improvement was 87%. (3) The average value of ejection fraction (EF) of left ventricle of all the patients returned to normal two years after treatment (EF value was 55.86 ± 2.85), the percentage of patients with normal left ventricle EF at 1 year, 3 years, 5 years and 10 years after treatment was 42.6% (26/61), 64.4% (29/45), 70.7% (29/41) and 84.6% (22/26), respectively. The average value of cardiothoracic (C/T) ratio became normal three years after treatment through X ray examination (0.50 ± 0.01), however the average value of the LVDD had not been returned to normal 3 years after treatment. At 1 year, 3 years, 5 years and 10 years after treatment, the proportion of patients with normal LVDD was 0% (0/61), 13.3% (6/45), 53.7% (22/41) and 84.6% (22/26), respectively. (4) The average value of EF became normal one year after treatment in the glucocorticoid group (EF value 58.44 ± 5.10) in 37 cases scored < 22 at the first visit, while the average value of C/T normalized two years after treatment (0.50 ± 0.00); The average value of EF became normal three years after treatment in the glucocorticoid plus cyclophosphamide group (EF 57.33 ± 3.43) in 29 cases scored < 22 at the first visit, however the average value of the C/T and the LVDD did not return to normal 3 years after treatment. (5) Use of IVIG reduced the percentage of patients who received cyclophos-phamide. (6) The recovery of intimal thickness was slow in EFE patients, the span was four years on the average (1 - 8 years), the percentage of patients whose endocardium became normal 1 year, 3 years, 5 years and 10 years after treatment was 9.85% (6/61), 22.2% (10/45), 51.2% (21/41), 100% (29/29).</p><p><b>CONCLUSION</b>The long-term continuous normal treatment of patients with EFE showed good therapeutic effects. For severe and refractory cases, immunotherapy must be strengthened and maintained for longer time. For those who clinically recovered, the quantity of activity should be restricted after the treatment is discontinued, and the re-examination should be done timely for further management.</p>

Endocardial Fibroelastosis in a 57-Year-Old Transplant Recipient

Endocardial fibroelastosis (EFE) is characterized by deposition of collagen and elastin leading to ventricular hypertrophy and diffuse endocardial thickening. Here we report (for the first time in Korea) the case of a EFE presenting with heart failure. The patient was a 57-year-old woman who had complained of dyspnea on exertion {New York Heart Association (NYHA) functional class 3} and abdominal distension at the time of hospital admission. Echocardiography showed severe diastolic dysfunction with normal systolic function. On MRI, the contrast-enhanced delayed myocardial image demonstrated hyperenhancement in the endocardium. Owing to progressive heart failure, the patient was transplanted. Histological examination of the explanted heart showed irregularly thickened endocardium with fibrosis and elastosis in the both ventricles, compatible with the diagnosis of EFE.

Miocárdio não compactado de ventrículo esquerdo: relato de caso com estudo de necropsia / Noncompaction of ventricular myocardium: case report with autopsy

O miocárdio não compactado de ventrículo esquerdo é cardiomiopatia genética em decorrência de uma anormalidade da morfogênese endomiocárdica. Quando presente, geralmente está associado a outras anomalias cardíacas congênitas, sendo raro seu achado isolado sem outras alterações estruturais cardíacas associadas. Desde seu primeiro relato em 1990, a não compactação do miocárdio vem sendo amplamente estudada a fim de se estabelecerem caráter genético, história natural, métodos de diagnóstico, repercussões hemodinâmicas e prognóstico. O objetivo deste estudo foi relatar um caso de miocárdio não compactado isolado de ventrículo esquerdo com estudo de necrópsia.

Noncompaction of the ventricular myocardium is a genetic cardiomyopathy due to an alteration in endomyocardial morphogenesis. When present, it is commonly associated with other congenital cardiac diseases, whereas it is rare as an isolated finding without other associated cardiac structural alterations. Since its first report in 1990, the noncompaction of the ventricular myocardium has been widely studied in order to establish its genetic character, natural history, diagnostic methods, hemodynamic repercussions and prognosis. This study aims to report the case of an isolated noncompaction of the left ventricular myocardium with autopsy.

Preliminary study on the effect of carvedilol on children with primary endocardial fibroelastosis / 中华儿科杂志

<p><b>OBJECTIVE</b>Endocardial fibroelastosis (EFE), a common pediatric cardiovascular disease, often results in chronic heart failure (CHF) and death. Clinical trials have shown that the regimen of combining beta-adrenoreceptor blocker with traditional medicines against CHF can improve left ventricular function and prevent the ventricle from remodeling in patients with CHF. The present study aimed to observe the effect of carvedilol on concentration of plasma brain-type natriuretic peptide (BNP), and safety in children with EFE.</p><p><b>METHODS</b>Twenty-one children with EFE were randomly divided into two groups: (1) treated with traditional regimen (digoxin, prednisone and/or diuretics) (n = 10); (2) treated with carvedilol plus traditional regimen (n = 11). Measurement of plasma concentration of BNP by ELISA, cardiac function by ultrasound were performed before and after 6 months of treatment. The changes in clinical symptom, heart rate, heart function, side effect and maximal tolerance dose after treatment with carvedilol were observed.</p><p><b>RESULTS</b>Plasma concentration of BNP was much higher in the group of patients with EFE [(865 +/- 702) ng/L] than that of control group [(154 +/- 78) ng/L] (P < 0.01), and there was a positive correlation between plasma concentration of BNP and cardiac function classification, and cardiac function grades II, III, and IV corresponded to plasma concentration of BNP (286 +/- 125) ng/L, (437 +/- 386) ng/L, (1673 +/- 859) ng/L respectively in children with EFE. Compared with the group treated with traditional medicines, plasma concentration of BNP [(403 +/- 216) ng/L vs. (219 +/- 87) ng/L] significantly decreased, the clinical symptom was significantly improved, cardio-thoracic ratio (CTR) (0.60 +/- 0.05 vs. 0.54 +/- 0.06) (P < 0.05) and heart rate [(115 +/- 20) bpm vs. (90 +/- 14) bpm] (P < 0.01) decreased, ejection fraction (EF) (46.6% +/- 13.4% vs. 54.5% +/- 12.9%), fractional shortening (21.6% +/- 8.1% vs. 24.1% +/- 7.5%), mean velocity of circumferential fiber shortening [(0.8 +/- 0.5) cir/s vs. (0.9 +/- 0.4) cir/s] were significantly increased (P < 0.01), left ventricular end-systolic dimension [(34.0 +/- 8.6) mm vs. (32.2 +/- 9.1) mm] (P < 0.05), left ventricular mass [(65.9 +/- 34.1) g vs. (65.9 +/- 34.1) g], interventricular septal thickness at end-systole [(6.0 +/- 1.0) mm vs (5.5 +/- 1.1) mm] were notably decreased (P < 0.01) after treatment with carvedilol.</p><p><b>CONCLUSION</b>These data indicated that plasma concentration of BNP significantly increased in children with EFE, carvedilol can decrease plasma concentration of BNP, inhibit the remodeling of ventricle, significantly improve the cardiac function in children with EFE. Carvedilol is effective and safe in treatment of children with EFE.</p>

Estudo de 11 casos de fibroelastose endocárdica primária / Primary endocardial fibroelastosis: study of 11 cases

INTRODUÇÃO: A fibroelastose endocárdica (FEE) é definida como espessamento difuso do endocárdio ventricular em função da proliferação de fibras colágenas e elásticas. Pode ser classificada como primária ou secundária. A FEE primária apresenta-se na forma dilatada, mais comum, ou na forma restritiva. Geralmente manifesta-se como insuficiência cardíaca inexplicada, em crianças com menos de 12 meses de idade. OBJEIVOS: Avaliar aspectos epidemiológicos, clínicos e anatomopatológicos da FEE primária. MATERIAL E MÉTODO: Foram pesquisadas, entre 1960 e 2000, 6.211 necropsias perinatais e pediátricas do Hospital de Clínicas da Universidade Federal do Paraná (UFPR), tendo sido verificados 11 casos de FEE. RESULTADOS: Em nove casos (81,8%), o surgimento dos sintomas ocorreu antes de 1 ano de idade. Em oito (72,7%), o óbito ocorreu antes de 1 ano de idade. Dentre os sinais clínicos, destacaram-se taquipnéia (54,5%), hepatomegalia (45,5%), tosse (36,4%), cianose (36,4%) e febre (36,4%). Todas as radiografias (n = 6) apresentavam cardiomegalia. O espessamento endocárdico predominou no coração esquerdo (90,9%). A causa de óbito foi insuficiência respiratória aguda em cinco casos (45,5%) e choque cardiogênico em quatro (36,4%). DISCUSSÃO E CONCLUSÕES: Os resultados mostram acometimento predominante de crianças com menos de 1 ano de idade e rápida progressão para óbito. Sinais clínicos freqüentes, como taquipnéia, tosse e febre, sugeriram infecção respiratória, que foi confirmada por necropsia em três casos (27,3%). Os exames complementares mostraram apenas achados sugestivos de insuficiência cardíaca. Os dados analisados neste estudo mostram que, em nosso meio, a FEE primária não difere significativamente do que é apresentado na literatura internacional em relação a características epidemiológicas, clínicas e anatomopatológicas.

BACKGROUND: Endocardial fibroelastosis (EFE) is a diffuse thickening of the ventricular endocardium resulting from proliferation of fibrous and elastic tissue. Primary and secondary forms have been described. Primary EFE occurs in the dilated and contracted forms. EFE usually manifests as unexplained congestive heart failure in infants younger than 1 year old. OBJECTIVES: To analyze the epidemiologic, clinical and anatomopathological aspects of primary EFE. METHODS: Eleven cases of histologically confirmed EFE were found in 6211 necropsies performed between 1960 and 2000 in the Hospital of the University of Paraná. Results: In nine cases (81.8%) the initial clinical presentation occurred before the age of 1 year. In eight cases (72.7%) patients died before the age of 1 year. The most common clinical findings were tachypnea (54.5%), hepatomegaly (45.5%), cough (36.4%) and fever (36.4%). Cardiomegaly was the main radiographic finding, present in all cases (n = 6). The endocardial thickening was more frequent at the left heart (90.9%). The death cause was acute respiratory failure in five cases (45.5%) and cardiogenic chock in four cases (36.4%). DISCUSSION AND CONCLUSIONS: The results showed that EFE affects usually infants younger than 1 year old that rapidly progress to death. Some common clinical findings like tachypnea, cough and fever suggested respiratory infection which was confirmed by necropsy in three cases (27.3%). The complementary tests showed only non-specific findings of congestive heart failure. The information analyzed here demonstrated no significant difference of the epidemiological, clinical and anatomopathological characteristics of primary EFE between Brazil and the international literature.

Sudden Unexpected Death in Infancy: Analysis of 34 Cases Including 13 Autopsies

PURPOSE: The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. METHODS: We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical history, death scene examination, autopsy findings, acylcarnitine and organic acid analysis. RESULTS: Among the total 34 infants, 18 were male(52.9%) and 16 were female(47.1%). Thirty infants(88%) were below the six months of age. Winter was the most affected season(38.2%). Eighteen infants(52.9%) died between 6 and 12AM. The prone sleeping position was observed more frequently than the supine position at death; nine cases in the prone position, six cases in the supine position. The cause of death of 23 cases could not be found by only history and death scene examination. Autopsy was done in 13 cases. Seven cases of them were thought to be SIDS. In six cases, we explained the cause of death with autosy findings. They were an endocardial fibroelastosis, a nesidioblastosis, a subdural hematoma, a bronchopneumonia and two fatty changes of liver. Metabolic screening tests performed in three cases to rule out metabolic disorder since 2000 were all normal. CONCLUSION: We concluded that autopsy and metabolic screening test should be performed to find out the cause of death in sudden unexpected death in infancy.

Contraced endocardial fibroelastosis in childhood: about one case

Endocardial fibroelastosis is un uncommon diseases and it has a very bad prognosis since fatal evolution is usual before 2 years old. We report the case of a 20 years old woman who is affected with the contracted form of this disease associated with atrial septal defect [ASD] and mitral regurgitation. This disease was discovered by endocardial biopsy when she was 4 years old and underwent surgical resection of endocardial fibrosis, a path on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy

Establecimiento del virus de la parotiditis epidémica como agente etiológico de la fibroelastosis endocárdica / Establishment of the epidemic mumps virus as etiologic agent endocardial fibroelastosis

La fibroelastosis endocárdica primaria es una miocardiopatía primaria caracterizada por dilatación e hipertrofia del ventrículo izquierdo, debido a un engrosamiento difuso del endocardio producido por la proliferación del tejido fibroso y elástico, que disminuye la flexibilidad ventricular y conduce a una marcada disfunción diastólica y sistólica del ventrículo izquierdo. Es fundamentalmente, una enfermedad del lactante menor, junto con las miocarditis activas, la fibroelastosis endocárdica constituye la inmensa mayoría de las cardiopatías del lactente y el niño pequeño. El diagnóstico diferencial entre ambas entidades es importante, pues las miocarditis activas responden al tratamiento inmunosupresor, el cual no está indicado en la fibroelastosis endocárdica. El ecocardiograma permite reconocer el endocardio engrosado e hiperrefringente, característico de esta enfermedad. Su etiología ha sido motivo de controversia, Frühling, en 1962, describió casos de fibroelastosis endocárdica durante una epidemia causada por el virus Coxsackie B. En el intervalo de 1963 y 1964, Norem, Adams y Anderson, por un lado y Shone, Muñoz Armas, Manning y Keith, por el otro, sugirieron un papel preponderante del virus de la parotiditis epidémica en su etiología, al encontrar reacción positiva a la prueba intradérmica con el antígeno de ese virus en más del 90 por ciento de casos estudiados. Nuestro estudio, con el mayor número de casos demostrados (50 pacientes), encontró negatividad de la prueba en 180 de 202 casos del grupo control (89 por ciento). En cambio, Gersony y Col, en un estudio publicado simultáneamente con el nuestro en la revista Pediatrics, en un grupo de sólo 16 pacientes con fibroelastosis endocárdica, excluyeron el virus de la parotiditis epidémica como agente etiológico. Este criterio había prevalecido hasta 1997, cuando Jiyuan Ni y Col; en la revista Circulation de enero de 1997, mediante una de las más avanzadas técnicas de la virología molecular, la reacción...

Miocardiopatias na infância / Infancy's myocardiopathies

Este artigo descreve a relaçäo entre as várias causas de acometimento miocárdico e comenta brevemente as características de cada doença, com ênfase no quadro clínico e nos achados de exame físico e laboratorial do envolvimento cardíaco. Levando-se em conta a faixa etária pediátrica, o assunto foi mais aprofundado em relaçäo ao vírus Coxsackie tipo B e à difteria.

Fibroelastosis endocárdica congénita: aspectos anatomopatológicos / Congenital endocardial fibroelastosis: anatomopathological aspects

Se estudiaron morfológicamente quince casos de Fibroelastosis endocárdiaca (FEE) del material de 64 autopsias de enfermedades del músculo cardíaco infantiles, practicadas en el Instituto Anatomopatológico de la Universidad Central de Venezuela. Diez casos resultaron de FEE primaria y cinco de secundaria. Las edades oscilaron entre 3 y 36 meses. Todos los casos fallecían con insuficiencia cardíaca refractaria. Se hizo énfasis en los hallazgos patológicos miocárdicos de las FEE primarias. Las alteraciones de las fibras miocárdicas fueron: Hipertrofia y atrofia focal con alteraciones nucleares, desarreglo celular, fibrosis interticial y degeneración hidiopática celular subendocárdica. Todas estas lesiones fueron discretas y no explican claramente la disfunción ventricular severa en estos casos. La posibilidad de que los cambios endocárdicos sean secundarios a alteracioes metabólicas es sugestiva dado los buenos resultados obtenidos con el tratamiento con L-carnitina en series estudiadas. La importancia de la biopsia endomiocárdica en casos sospechosos de FEE radica en la mejor comprensión de la etiopatogenia y tratamiento de esta enfermedad

Aspectos clínicos e evolutivos da miocardiopatia dilatada nos lactentes e na infância / Clinical and evolutive aspects of dilated cardiomyopathy in infants and in childhood

Avaliar os aspectos clínicos, evolutivos e de prognóstico, em lactentes e crianças com miocardiopatia dilatada (MCPD). Trinta e oito pacientes divididos em grupos: A) lactentes até 23 (11,60 ñ 6,50) meses, 15 femininos, e B) 15 crianças de 2 a 12 (5,23 ñ 3,12) anos, a maioria (10) masculinos. O estudo foi retrospectivo, através de avaliaçäo clínica, eletrocardiográfica, radiológica e ecocardiográfica. A dispnéia aos esforços (inclusive às mamadas) foi sintoma predominante, observada em 15 (65,22%) pacientes do grupo A e 10 (66,6%) do B, seguida da cianose perilabial, em 7 (30,43%) e 6 (40%) pacientes respectivamente. No grupo A, a hipótese clínica foi de miocardite inespecífica (MI) em 8 (34,79%), e de MCPD "idiopática" em 3 (13,04%). No grupo B diagnosticou-se miocardite em 10 (66,67%) - sendo 5 inespecíficos - FEM em 3 (20%) e MCPD "idiopática" em 2 (13,33%). O tempo médio de evoluçäo foi de 5,48 meses no grupo A e 18,56 no B. No grupo A a evloluçäo foi ótima em 3 (13,04%), boa em 10 (43,46%), estável em 2 (8,70%) e má em 1 (4,35%), e no B: ótima em 8 (53,33%), boa em 3 (20%) e estável em 1 (6,67%), em nenhum má evoluçäo. Houve um óbito do grupo A (4,34%) e 6 pacientes deste grupo (26,09%) e 3 (20%) do B interromperam o controle evolutivo. 1) o prognóstico dos lactentes com MCPD, inclusive com hipótese diagnóstica de FEM parece ser menos sombrio do que habitualmente se supöe; 2) o prognóstico de crianças nas quais a MCPD é diagnosticada acima de 2 anos parece ser boa; ...