Gesmar Volga Haddad Herdy

Surgical mitral valve repair in children with rheumatic fever

FUNDAMENTO: La reconstruccion mitral tiene buena aceptacion en ninos con fiebre reumatica. OBJETIVO: Analizar los resultados de la reconstruccion quirurgica mitral en ninos con lesiones reumaticas despues de cuatro anos de evolucion. METODOS: Estudio retrospectivo de 40 pacientes menores de 18 anos, operados en el Instituto Nacional de Cardiologia (RJ), entre enero de 1998 y enero de 2003. Se analizo el grado de regurgitacion mitral por ecocardiograma, la tecnica quirurgica, la clase funcional pre y post operatoria, la evolucion de los casos, la necesidad de cambio valvular y obitos. RESULTADOS: Veintiun pacientes (52,5%) eran del sexo femenino. La insuficiencia mitral era grave en 32 pacientes (80%) y moderada en ocho (20%). Ocurrieron tres obitos inmediatos (7,5%). Despues de tres meses de cirugia, el ecocardiograma mostro que en 35/37 (94,6%) no habia regurgitacion valvular o era leve, y en dos pacientes (5,2%) era grave. La clase funcional en el preoperatorio era III y IV en 33 casos (82,5%), y tres meses despues de la cirugia los 37 casos (100%) estaban en clase funcional I y II. La diferencia entre los datos del grados de regurgitacion mitral y clase funcional en el pre y post operatorio fueron estadisticamente significativos (p<0,01). El cambio valvular antes de cuatro de evolucion ocurrio en siete (19%) de los casos. CONCLUSION: La reconstruccion de la valvula mitral mostro resultado favorable en la mayoria de los casos al considerar el grado de regurgitacion mitral y la clase funcional pre y post quirurgica, y solo el 19% de los pacientes necesitaron cirugia para cambio valvular antes de cuatro anos de evolucion.The accumulation of chyle in the pericardial space, or chylopericardium, is a condition occurring most frequently after trauma, cardiac and thoracic surgery, or in association with tumors, tuberculosis or lymphangiomatosis. When its precise cause cannot be identified, it is called primary or idiopathic chylopericardium. This is a rare clinical entity. We report the case of a surgically treated 20-year-old female patient. A brief review of the literature and comments on the clinical presentation, etiopathogenesis, ancillary diagnostic tests and treatment options are also presented.BACKGROUND Mitral repair is well accepted in children with rheumatic fever. OBJECTIVE To analyze the outcomes of surgical mitral repair in children with rheumatic lesions after four years of follow-up. METHODS Retrospective study of 40 patients younger than 18 years, who underwent surgery in the National Institute of Cardiology (Rio de Janeiro) between January 1998 and January 2003. The echocardiographic degree of mitral regurgitation; surgical technique used; pre and postoperative functional class; patient outcome; need for valve replacement; and deaths were analyzed. RESULTS Twenty one patients (52.5%) were females. Severe mitral regurgitation was observed in 32 patients (80%) and moderate in eight (20%). Three immediate deaths occurred (7.5%). Three months after surgery, echocardiography showed no valve regurgitation or mild regurgitation in 35 of 37 cases (94.6%) patients, and severe regurgitation in two (5.2%). Thirty three cases (82.5%) were in functional class III or IV in the preoperative period, and three months after surgery all the 37 cases (100%) were in functional class I or II. The differences between the degree of mitral regurgitation and functional class in pre and postoperative periods were statistically significant (p<0.01). Seven (19%) patients underwent heart valve replacement before four years of follow-up. CONCLUSION Mitral valve repair showed favorable results in most of the cases as regards the degree of mitral regurgitation and the pre and postoperative functional class. Only 19% of the patients required surgical valve replacement before four years of follow-up.

Plastia mitral cirúrgica em crianças com febre reumática

FUNDAMENTO: La reconstruccion mitral tiene buena aceptacion en ninos con fiebre reumatica. OBJETIVO: Analizar los resultados de la reconstruccion quirurgica mitral en ninos con lesiones reumaticas despues de cuatro anos de evolucion. METODOS: Estudio retrospectivo de 40 pacientes menores de 18 anos, operados en el Instituto Nacional de Cardiologia (RJ), entre enero de 1998 y enero de 2003. Se analizo el grado de regurgitacion mitral por ecocardiograma, la tecnica quirurgica, la clase funcional pre y post operatoria, la evolucion de los casos, la necesidad de cambio valvular y obitos. RESULTADOS: Veintiun pacientes (52,5%) eran del sexo femenino. La insuficiencia mitral era grave en 32 pacientes (80%) y moderada en ocho (20%). Ocurrieron tres obitos inmediatos (7,5%). Despues de tres meses de cirugia, el ecocardiograma mostro que en 35/37 (94,6%) no habia regurgitacion valvular o era leve, y en dos pacientes (5,2%) era grave. La clase funcional en el preoperatorio era III y IV en 33 casos (82,5%), y tres meses despues de la cirugia los 37 casos (100%) estaban en clase funcional I y II. La diferencia entre los datos del grados de regurgitacion mitral y clase funcional en el pre y post operatorio fueron estadisticamente significativos (p<0,01). El cambio valvular antes de cuatro de evolucion ocurrio en siete (19%) de los casos. CONCLUSION: La reconstruccion de la valvula mitral mostro resultado favorable en la mayoria de los casos al considerar el grado de regurgitacion mitral y la clase funcional pre y post quirurgica, y solo el 19% de los pacientes necesitaron cirugia para cambio valvular antes de cuatro anos de evolucion.The accumulation of chyle in the pericardial space, or chylopericardium, is a condition occurring most frequently after trauma, cardiac and thoracic surgery, or in association with tumors, tuberculosis or lymphangiomatosis. When its precise cause cannot be identified, it is called primary or idiopathic chylopericardium. This is a rare clinical entity. We report the case of a surgically treated 20-year-old female patient. A brief review of the literature and comments on the clinical presentation, etiopathogenesis, ancillary diagnostic tests and treatment options are also presented.BACKGROUND Mitral repair is well accepted in children with rheumatic fever. OBJECTIVE To analyze the outcomes of surgical mitral repair in children with rheumatic lesions after four years of follow-up. METHODS Retrospective study of 40 patients younger than 18 years, who underwent surgery in the National Institute of Cardiology (Rio de Janeiro) between January 1998 and January 2003. The echocardiographic degree of mitral regurgitation; surgical technique used; pre and postoperative functional class; patient outcome; need for valve replacement; and deaths were analyzed. RESULTS Twenty one patients (52.5%) were females. Severe mitral regurgitation was observed in 32 patients (80%) and moderate in eight (20%). Three immediate deaths occurred (7.5%). Three months after surgery, echocardiography showed no valve regurgitation or mild regurgitation in 35 of 37 cases (94.6%) patients, and severe regurgitation in two (5.2%). Thirty three cases (82.5%) were in functional class III or IV in the preoperative period, and three months after surgery all the 37 cases (100%) were in functional class I or II. The differences between the degree of mitral regurgitation and functional class in pre and postoperative periods were statistically significant (p<0.01). Seven (19%) patients underwent heart valve replacement before four years of follow-up. CONCLUSION Mitral valve repair showed favorable results in most of the cases as regards the degree of mitral regurgitation and the pre and postoperative functional class. Only 19% of the patients required surgical valve replacement before four years of follow-up.

Study of the cardiac alterations in HIV-infected children consequent to the antiretroviral therapy: prospective study of 47 cases

OBJECTIVE Detect of cardiac alterations in children with AIDS and compare their evolution with the administration of only one anti-retroviral and the recent cases who received drugs in combination. METHODS We prospectively studied 47 children in 3 groups: group 1, 20 cases treated only with zidovudine; group 2, 10 patients treated initially with zidovudine and later with a combination of drugs and in group 3, 17 patients, who receiced two or three since the beginning. In all patients it was done chest X-ray, EKG and echocardiography every 6 months and after death complete pathological study. RESULTS Among the 45 patients cases 26 (57%) were index cases. Malnutrition, diarrhea tachycardia, signs of congestive heart failure, pericardial effusion, abnormal ventricular repolarization and arrhythmias were more frequent in group 1. Echocardiographic abnormalities were present in 10 (50%) children of group 1. They were less frequent in the others two groups. In regard to the outcome in group 1, two patients had worsening of sings of cardiomyopaty and 4 died. Cardiac dysfunction in all cases of group 2 and 3 improved with the medication. CONCLUSION The children who received combination and their cardiac alterations had more favorable outcome than those who received only one drug.

Proton magnetic resonance spectroscopy in children with fetal alcohol spectrum disorders

OBJECTIVE To analyze the metabolic constitution of brain areas through proton magnetic resonance spectroscopy in children affected with fetal alcohol spectrum disorder compared with normal children. METHOD The sample of this case-control study included eight boys with epidemiologic history of in utero exposure to alcohol (median age 13.6+/-3.8 years) who were diagnosed with fetal alcohol spectrum disorder, and eight controls (median age 12.1+/-3,4 years). An 8 cm(3) single voxel approach was used, with echo time 30 ms, repetition time 1500 ms, and 128 acquisitions in a 1.5T scanner, and four brain areas were analyzed: anterior cingulate, left frontal lobe, left striatum, and left cerebellar hemisphere. Peaks and ratios of metabolites N-acetylaspartate, choline, creatine, and myo-inositol were measured. RESULTS Children with fetal alcohol spectrum disorder showed a decrease in choline/creatine ratio (p=0.020) in left striatum and an increase in myo-inositol/creatine ratio (p=0.048) in left cerebellum compared with controls. There was no statistically significant difference in all peaks and ratios from the anterior cingulate and frontal lobe between the two groups. CONCLUSION This study found evidence that the left striatum and left cerebellum are affected by intrauterine exposure to alcohol. Additional studies with larger samples are necessary to expand our knowledge of the effects of fetal exposure to alcohol.

Asfixia perinatal e problemas cardíacos

Objetivo - Avaliar a gravidade das complicacoes cardiacas na asfixia neonatal, sua evolucao e correlacionalas com o grau e duracao do processo hipoxico. Metodos - Foram estudados 90 bebes nos ultimos 7 anos com grau de Apgar <6 no 5°min de vida. Pelo protocolo, apos o exame fisico e os cuidados intensivos, eram submetidos a dosagem do pH arterial, CPK-MB, DHL, glicemia, alem da realizacao de radiografia de torax, eletrocardiograma (ECG), ecocardiograma, seriados e repetidos a cada semana. Aqueles que faleceram eram levados a necropsia. Resultados - Dos 90, 73 (81%) eram prematuros, 30 (41%) eram apropriados para a idade gestacional (AIG) e 43 (59%) eram pequenos(PIG). Em 21 (23%) casos havia pH arterial <7,2. Os quadros clinicos mais observados foram: pneumonia em 28 (31%), anemia 24 (26%) e ictericia moderada 12 (5%), entre outros. Ao exame fisico observaram-se: sopro sistolico em 46 (50%), ictus propulsivo 18 (20%) e ICC em 8 (9%). No ECG, os sinais mais frequentes foram alteracoes de repolarizacao (ST e T) em 44 (49%). No ecocardiograma, observou-se persistencia do canal arterial (PCA) em 20 (22%), regurgitacao tricuspide em 6 (7%), hipertensao pulmonar em 6 (8%), hipocontratilidade de VE e dilatacao de VD em 4 (5%). Dos 23 obitos, 14 foram estudados e as alteracoes mais frequentes foram necrose de fibras em 8 (68%) casos e em 4 (29%) congestao, vacuolizacao e perda de estriacao. Conclusao - A maioria teve evolucao favoravel mesmo naqueles que tiveram acidemia importante. Muitas alteracoes de ECG e ecocardiograma se normalizaram. Daqueles que evoluiram para o obito, as lesoes mais graves ocorreram nos que sofreram, por mais tempo, processo anoxico.

Follow-up of rheumatic carditis treated with steroids.

OBJECTIVE To present the long-term follow-up of children hospitalised for severe rheumatic carditis who were treated with corticosteroids. METHODS This is a retrospective analysis of the outcome of 242 patients with severe rheumatic carditis after discharge from two public hospitals in Niteroi, Brazil. We followed up 118 patients for 4 years or more, with an average of 7.7 years. They were treated with antibiotics to accomplish bacterial eradication and either intravenous methylprednisolone - 40 cases - or oral prednisone - 78 patients - to treat carditis. They were followed up in outpatient clinic. RESULTS Cardiac failure was categorised as classes III and IV according to the New York Heart Association classification. In the intravenous corticosteroid group, 21 cases (52.5%) had isolated mitral valve regurgitation, 12 (30%) had mitral plus aortic involvement, and seven (17.5%) had aortic lesion only. In the oral prednisone group, 45 (58%) had mitral valve regurgitation only, 27 (34%) had mitral plus aortic involvement, and six (8%) had aortic lesion only. A total of 28 children were in their first disease attack, of whom 19 (68%) had a rupture of chordae tendineae. A total of 58 patients (49%) sustained recurrence of carditis because of neglected secondary prophylaxis. In all, 19 cases (16%) underwent cardiac surgery - valve replacement or valvuloplasty. In 33% of the cases, the outcome was favourable - asymptomatic at follow-up. The overall mortality rate was 6.8%. CONCLUSION Many critically ill patients who complied with secondary prophylaxis were left with minor injuries, whereas those who neglected it or abandoned it had serious sequelae. The rate of abandonment and loss to follow-up was very high. Many cases (49%) were re-hospitalised because of carditis recurrence.

Dieta cetogênica para epilepsia intratável em crianças e adolescentes: relato de seis casos

OBJETIVO: Descrever a introducao e o manejo da dieta cetogenica em um grupo de seis criancas e adolescentes com epilepsia refrataria. METODOS: Os autores reviram o prontuario medico de cada paciente menor de 15 anos submetido a dieta cetogenica entre abril de 1999 e julho de 2003 e compararam os resultados terapeuticos e efeitos adversos e beneficos com a literatura pertinente. RESULTADOS: A dieta cetogenica foi introduzida para seis pacientes, com idade mediana de sete anos (faixa: 1,8-12,2). A duracao media da aplicacao da dieta foi 9,7 meses (faixa: 7 dias-4 anos). Observou-se uma reducao igual ou maior que 50% da frequencia das crises epilepticas em metade dos casos. As complicacoes observadas foram leucopenia, constipacao, desidratacao, priapismo e recorrencia das crises epilepticas. CONCLUSOES: A dieta cetogenica foi eficaz e segura em tres pacientes de uma serie de seis casos com epilepsia intratavel. A complicacao mais comum foi leucopenia.

Validation study of a scale of life quality evaluation in a group of pediatric patients infected by HIV

With the advent of potent antiretroviral therapy and the increase in life expectancy of pediatric patients infected with HIV, the quest for the promotion of enhanced quality of life should currently be the main focus in care of children with HIV/Aids. The scope of this study was to validate the Scale of Childrens Quality of Life in a group of children infected with HIV receiving clinical care in Aids Service Units in Rio de Janeiro, Brazil. This scale consists of 26 questions and was tested on 100 children, with ages varying between 4 and 12, and their respective parents or guardians. Statistical analysis was conducted using canonical correlation and confidence interval analysis and the X² test. The results showed that the cut-off point obtained was 49; the internal consistency with Cronbachs alpha was 0.73 for the children and 0.67 for parents or guardians. The response profile revealed marked satisfaction with aspects such as vacations and birthdays, though less satisfaction with items including hospitalization and playing alone. The conclusion was that the scale revealed satisfactory psychometric measurements, proving to be a reliable, consistent, valid and recommended instrument for measuring the quality of life of children infected with HIV.

Pseudotumor cerebral secundário a trombose venosa dural: relato de caso pediátrico

Pseudotumor cerebri is a relatively common neurologic syndrome in adolescence. In most cases, etiology is idiopathic, but it may have serious complications, such as blindness, that are related to increased intracranial pressure. The aim of this article is to emphasize the differential diagnosis of pseudotumor cerebri, with special attention to treatable etiologies. We report a case of an 12 year-old adolescent who presented with diplopia and headache 9 days after right-sided otitis media and mastoiditis. Head computerized tomography was normal, but brain magnetic resonance imaging demonstrated thrombosis of ipsilateral transverse and sigmoid sinuses, which responded promptly to early anticoagulation. The conclusion is that magnetic resonance imaging is essential for patients with a clinical diagnosis of pseudotumor cerebri in order to exclude treatable causes, such as dural sinus thrombosis.Pseudotumor cerebri is a relatively common neurologic syndrome in adolescence. In most cases, etiology is idiopathic, but it may have serious complications, such as blindness, that are related to increased intracranial pressure. The aim of this article is to emphasize the differential diagnosis of pseudotumor cerebri, with special attention to treatable etiologies. We report a case of an 12 year-old adolescent who presented with diplopia and headache 9 days after right-sided otitis media and mastoiditis. Head computerized tomography was normal, but brain magnetic resonance imaging demonstrated thrombosis of ipsilateral transverse and sigmoid sinuses, which responded promptly to early anticoagulation. The conclusion is that magnetic resonance imaging is essential for patients with a clinical diagnosis of pseudotumor cerebri in order to exclude treatable causes, such as dural sinus thrombosis.

Complicações cardiovasculares em criança com insuficiência renal crônica

This is the report of an 11-year-old boy with chronic renal disease and secondary hyperparathyroidism. The child had been on dialysis, calcitriol, calcium carbonate, and presented dyslipidemia and calcified thrombi in various vessels and organs in the course of his condition. Pathological examination showed ischemic cerebral necrosis, calcification in coronary arteries, and myocardial infarction.