Prevalência de doença celíaca em crianças e adolescentes com miocardite e cardiomiopatia dilatada / Celiac disease prevalence in children and adolescents with myocarditis and dilated cardiomiopathy

OBJETIVO: Determinar a prevalência de doença celíaca em pacientes portadores de cardiomiopatia dilatada e miocardite. MÉTODOS: Foram avaliados 56 pacientes, com idade entre 1 e 18 anos, portadores de cardiomiopatia dilatada ou miocardite, acompanhados no Instituto Materno Infantil Professor Fernando Figueira. Foram excluídos pacientes com diagnóstico prévio de doença celíaca. A classe funcional da insuficiência cardíaca foi determinada segundo os critérios da American Heart Association, como classe funcional I, II, III e IV. O diagnóstico de miocardite foi relatado em prontuário, e o de cardiomiopatia dilatada, pelo ecocardiograma, a partir da presença de disfunção sistólica de um ou ambos os ventrículos, com fração de ejeção menor que 55% e dilatação ventricular, com diâmetro diastólico final ventricular esquerdo maior que 112%. Nos pacientes incluídos no estudo, foi aplicado um formulário com informações sobre sintomatologia gastrointestinal e cardiológica; em seguida, dosadas sorologias para anticorpos antitransglutaminase tecidual humana e antiendomísio. Aqueles com sorologia positiva foram encaminhados à biópsia intestinal para avaliação histológica para doença celíaca, segundo os critérios de Marsh. RESULTADOS: Uma das 56 crianças apresentou sorologia antitransglutaminase positiva (1,8%), porém anticorpo antiendomísio negativo. A histologia intestinal demonstrou atrofia total das vilosidades. Cerca de 30% dos pacientes apresentaram insuficiência cardíaca. Sinais e sintomas gastrointestinais foram frequentes nos pacientes, em especial dor abdominal (70%, 39/56). CONCLUSÃO: A frequência de doença celíaca em pacientes com cardiomiopatia dilatada e miocardite foi de 1,8%. É importante investigar doença celíaca nos pacientes com essas doenças cardíacas para evitar evolução das doenças e deterioração clínica do paciente.

OBJECTIVE: To determine the prevalence of celiac disease in patients with myocarditis and dilated cardiomyopathy. METHODS: Fifty-six patients between 1 and 18 years old with dilated cardiomyopathy or myocarditis were evaluated and followed up at Instituto de Medicina Integral Professor Fernando Figueira. Patients with previous diagnosis of celiac disease were excluded. The functional classification was determined according to the American Heart Association criteria (classes I, II, III and IV). Diagnosis of myocarditis was reported in the patients' medical records. Dilated cardiomyopathy was diagnosed by echocardiogram with systolic dysfunction of one or both ventricles, ejection fraction lower than 55%, ventricular dilatation, and left ventricular diastolic diameter bigger than 112%. Patients answered a questionnaire about gastrointestinal and cardiac symptoms; next, anti-tissue transglutaminase (tTG) and anti-endomysial (EMA) antibodies were dosed. Those with positive antibody results were referred to intestinal biopsy and histological evaluation to detect celiac disease according to Marsh classification. RESULTS: One of the 56 children (1.8%) had positive tTG antibody level, but negative EMA. Intestinal histological evaluation showed total villous atrophy. Approximately, 30% of patients had heart failure. Gastrointestinal symptoms and signs were frequent, especially abdominal pain (70%, 39/56). CONCLUSION: Celiac disease prevalence in pediatric patients with dilated cardiomyopathy or myocarditis was 1.8%. It is important to investigate celiac disease in patients with these conditions to avoid the progression of such diseases and patients' clinical deterioration.

Efficacy of echocardiographic criteria for diagnosis of carditis in acute rheumatic fever.

Background: There is a great need forECHOcriteria for accurate diagnosis of carditis in acute rheumatic fever. Aim: To propose and test the efficacy of ECHO criteria for accurate diagnosis of carditis. Material and Methods: The 333 cases underwent detailed clinical examination, laboratory tests and meticulous Echocardiographic study.Vijay’s ECHO criteria for the diagnosis of carditis / subclinical valvulitis was used. 220 (66.06%) cases were both Jones’ positive and ECHO positive [True +ve], 52 cases (15.61%), probably had subclinical carditis as murmur was not heard (Jones’-ve) but ECHO was positive [False - ve]. Four cases, clinically diagnosed as carditis were Jones’+ve ,but ECHO showed congenital heart disease [False +ve]. 57 cases (17.11%) were clinically , echocardiographically and Jones’ negative were taken as control (True –ve). Sensitivity is81%and specificity is 93%. Conclusions: Precise diagnosis of both carditis /subclinical valvulitis is possible with Vijay’s ECHO criteria. ECHO should be included as a major criterion in Jones’criteria.

Miocarditis en niños con fiebre por dengue hemorrágico en un hospital universitario de Colombia / Myocarditis in children affected by dengue hemorrhagic fever in a teaching hospital in Colombia

Objetivo Determinar la frecuencia de miocarditis en niños con fiebre por Dengue hemorrágico-FDH, en el Huila, Colombia. Materiales y Métodos Se realizó un estudio descriptivo de observación prospectiva, entre Marzo del 2005 y Octubre de 2006. Se observaron 102 pacientes con diagnóstico de FDH, basado en los criterios de la OMS, para identificar la frecuencia de miocarditis. Resultados Un 10,7 por ciento de los niños presentaron miocarditis. Solo un caso con miocarditis fue clasificado como dengue II; los otros diez casos fueron dengue III y IV. La diferencia en la presentación de miocarditis en los estadíos más severos de dengue fue estadísticamente significativa, con un p=0.0004. Diez de los pacientes tuvieron miocarditis que respondió al soporte inotrópico temprano. En el electrocardiograma (EKG), dos pacientes presentaron taquiarritmias, uno tipo supraventricular y otro ventricular, el resto mostró bradicardia sinusal con trastornos de repolarización y bajos voltajes. El ecocardiograma se realizó en 50 por ciento de los niños, encontrándose derrame pericárdico grado I-II en dos pacientes y uno con la fracción de eyección muy disminuida. La estancia promedio fue de 7 días. Un paciente presentó desenlace fatal. Conclusión La presentación de miocarditis alerta sobre la presencia de disfunción miocárdica como una patología a considerarse en pacientes con FDH.

Objective To determining myocarditis frequency in children suffering from DHF in the Huila region of Colombia. Methods This was a descriptive and prospective study which was carried out between March 2005 and October 2005 involving 102 patients suffering from DHF, based on WHO criteria. These children were followed-up to identify signs of myocarditis. Results 10.7 percent of the children had signs of myocarditis; one of them was classified as having dengue II and the other ten were classified as suffering form dengue III and IV. This difference was statistically significant (p=0.0004). 10 patients had fulminant myocarditis and required early inotropic support. Two patients had tachyarrythmias in the EKG (one SV and one ventricular); the others had sinusal bradichardia and low voltages. Echocardiograms were perfomed on 50 percent of the children, revealing pericardial leakage grade I and II in two patients, one with very diminished EF and AF. Average hospital stay was 7 days. 10 had satisfactory outcome whereas one died. Conclusion This series of cases in children reinforces the association between myocarditis and dengue viral infection.

Kawasaki disease – An indian perspective.

Kawasaki disease (KD) was first reported from Japan in 1967 by a young pediatrician, Tomisaku Kawasaki, while working at the Red Cross Hospital in Tokyo. Soon therafter, Marian Melish independently reported children with a similar clinical profile from Hawaii in the United States. KD has now been reported from all parts of the world, including several centers in India. Based on the epidemiology and clinical features, an infectious etiology has been suspected for long but no definitive causative agent has been implicated so far. Like many other vasculitides, the diagnosis of this condition is based on the recognition of a temporal sequence of clinical features, none of which is pathognomonic in isolation. KD is believed to be the commonest vasculitic disorder of children. Incidence rates as high as 60-150 per 100,000 children below 5 years of age have been reported from several countries. In India (as also perhaps in many other developing countries), however, majority of children with KD continue to remain undiagnosed probably because of the lack of awareness amongst pediatricians. The clinical features of KD can be confused with other common conditions like scarlet fever and the Stevens Johnson syndrome, if the clinician is not careful. Development of coronary artery abnormalities (CAA) is the hallmark of KD and accounts for most of the morbidity and mortality associated with the disease. Prompt recognition of the disease and early initiation of treatment with intravenous immunoglobulin (IVIG) results in significant reduction in the occurrence of CAA. It is, therefore, imperative for the pediatrician to diagnose and treat KD expeditiously. KD should be considered in the differential diagnosis of all febrile illnesses in young children where the fever persists for more than 5-7 days.

Miocarditis aguda primaria. Experiencia de diez años en el Instituto Nacional de Cardiología "Ignacio Chávez" / Primary Acute Myocarditis. A 10- years institutional experience at the National Institute of Cardiology "I. Chavez"

La miocarditis aguda (MA) se asocia a infecciones virales: Coxsackie B, ECHOvirus y otros. Mecanismos autoinmunes se suponen patogénicos. La clínica es variable. El beneficio de inmunosupresión clásica (prednisona-azatioprina) o inmunomodulación (IgG-monomérica) no ha sido confirmado. Objetivo: Revisar la incidencia y enfoque de estudio de la miocarditis. Material y métodos: De 1992-2003, aplicamos un cuestionario estándar a 49 pacientes consecutivos con diagnóstico establecido en forma independiente. Se excluyeron MA con causa identificable. Resultados: La MA ocurrió en 17 mujeres y 32 hombres, edad (mediana) 24 y 28 años. Predominaron disnea/ortopnea (70-47%), edema/plétora yugular (41-37%), dolor precordial y taquicardia (50%). En 22 (45%) la clase funcional era III-IV. El electrocardiograma mostró taquicardia sinusal (35%) y bloqueos de rama (24%). En 47 pacientes el ecocardiograma mostró FEVI 41% (promedio), DDVI 53 mm (promedio) y discinesia 89%. En 11 (22%) casos investigamos anticuerpos contra Coxsackie/ECHOvirus, 6 (54%) tenían anticuerpo reconocido. Veintinueve casos (61%) progresaron a miocardiopatía dilatada (MD), hubo tres defunciones (6%). No fue posible valorar tratamiento inmunomodulador, se estudió sólo a 12 casos. Conclusiones: La incidencia de MAes 1/1,000 ingresos/año. Es necesario estandarizar manejo diagnóstico y tratamiento, pues la progresión a MD y/o muerte en etapa aguda ocurre en 2/3 de los pacientes.

Acute myocarditis (AM) is associated with viral infections: Coxsackie and ECHOviruses among others. Autoimmunity has been proposed as a pathogenic mechanism. Benefit of classic immunosuppression (prednisone-azathioprine) or immunomodulation (monomeric-human IgG) is still uncertain. Objective: To review incidence and clinical approach to AM at a Cardiology referral center. Material and methods: A 10-yeard period (1992-2003) is reviewed. A standard ques-tionary was applied to 49 consecutive patients referred by clinicians with a diagnosis of AM. Results: AM was found in 17 women and 32 men, median age 24 and 28 years, respectively. They presented heart failure with dyspnea/ortop-nea (70-47%), peripheral edema/jugular vein plethora (41-37%), chest pain, and tachycardia (50%), NYHA functional class was III-IV in 22. The EKG showed sinus tachycardia or conduction defects. Transthoracic echocardiograms in 47 cases showed EF (mean) of 41% with enlarged left ventricle diameter. Antivirus antibodies were present in 54% of those cases studied, Coxsackie or ECHOvirus were identified through a serologic assay. Twenty-nine (61%) of our cases developed dilated cardiomyopathy, three patients died. It is not possible to reach a conclusion regard to immunomodulation therapy, because it was applied to only 12 patients. Conclusions: At the Instituto Nacional de Cardiología "I. Chávez", AM depicts an incidence of 1/1,000 patients a year. It is necessary to standardize the clinical approach for diagnosis and treatment, progression to dilated cardiomyopathy and deadth during acute stage occurs in two-thirds of our patients.

Atualização em miocardites / Myocarditides update

As miocardites (inflamações do músculo cardíaco, podendo envolver os miócitos, o interstício, estruturas vasculares e/ou o pericárdio) são importantes causas de morte súbita e inesperada em adultos com menos de 40 anos de idade e atletas jovens. Têm etiologia diversa, incluindo vírus, bactérias, protozoários, drogas e doenças sistêmicas. Sua fisiopatologia envolve a ativação prolongada das imunidades celular e humoral. Seu curso geralmente é assintomático ou insidioso, havendo considerável dificuldade no estabelecimento do diagnóstico, devido à inespecificidade de seu quadro clínico e à baixa sensibilidade dos exames complementares. O tratamento é basicamente suportivo e direcionado às possíveis complicações ou às causas de base. Possuem prognóstico variável, dependendo da causa-base, sendo pior nas doenças sistêmicas e na infecção pelo HIV.

Myocarditis are defined as inflammation of the cardiac muscle, which may involve the myocite, the interstitium, the vascular structures and/or the pericardium. They are important causes of sudden and unexpected death in adults and in young athletes. There are many known etiologies for myocarditis, including virus, bacteria, protozoan, drugs and systemic diseases. Its pathophysiology involves the continuum activation of humoral and cellular immunity. The course of the disease usually is assymptomatic or insidious, clinical manifestations are quite non-specific and complementary, exams show poor sensibility and specificity, making diagnosis difficult. Treatment is basically supportive and addressed to the possible complications or its causes. The prognosis is somewhat variable, depending on the underlying cause, being worse in systemic diseases and in HIV infections.

Miocardite virótica / Viral myocarditis

A miocardite é definida como um processo inflamatório do músculo cardíaco. Uma grande variedade de doenças infecciosas, sistêmicas, drogas e toxinas tem-se associado ao desenvolvimento da doença. Os vírus são os principais agentes envolvidos. Baseado em estudos experimentais e pelas técnicas de biologia molecular, a miocardite viral pode ser dividida em uma doença trifásica. O conhecimento dessas fases cronológicas facilita a compreensão tanto do diagnóstico como das possibilidades terapêuticas. A fase aguda caracteriza-se pela agressão viral direta; a subaguda é decorrente da resposta imune inadequada; e a crônica, pelo desenvolvimento de miocardiopatia dilatada. Métodos complementares diagnósticos, como ressonância nuclear magnética, técnicas de biologia molecular e imuno-histoquímica, têm contribuído para o diagnóstico das miocardites. O tratamento imunossupressor é controverso; entretanto, novos estudos estão sendo desenvolvidos para elucidar o efeito da imunossupressão e quais grupos de pacientes se beneficiarão desse tratamento.

Coxsackie B virus infection and myopericarditis in Thailand, 1987-1989.

Serum specimens of 363 myopericarditis patients from the hospital all over the country were examined for coxsackie B virus antibody during 1987-1989 by means of microneutralization test in order to assess association between myopericarditis and coxsackie B virus infection. The data established that certain virus infection rates were 24.3%, 19.4% and 23.6% respectively, no differences in incidence were found between sex (p > 0.05) and the incidence rate between age groups below 15 years and 15 years and older was significantly different (p < 0.05). It was found that the epidemic happened throughout the year and the most common serotype in 1987 and 1989 was B4 whereas in 1988 it was B3.

Miocarditis viral asociada a bronquiolitis por virus sincitial respiratorio

La bronquiolitis por virus sincitial respiratorio (USR) es una enfermedad de las vías aéreas pequeñas, caracterizada por inflamación y obstrucción de los bronquiolos. El VSR fue aislado por primera vez en 1995, pertenece a la familia de los paramixovirus. Es clara la presentación epidérmica anual que compromete especialmente a lactantes pequeños. La tasa de infección por VSR durante el primer año de vida alcanza el 69 por ciento y el 83 por ciento durante el segundo. La mortalidad en niños previamente sanos es del 0.005 por ciento al 0.002 por ciento; en pacientes hospitalizados es del 1 al 3 por ciento. Aunque se ha descrito que la infección por VSR no altera la función cardíaca en pacientes con corazón sano, se encuentra en la literatura informes aislados al respecto; a continuación presentamos un caso de un paciente con bronquiolitis por VSR, en el que se documentó miocarditis por este mismo germen.

Estado actual de los conocimientos sobre la enfermedad de Chagas en la República Mexicana / Current status of knowledges on Chagas disease in Mexican Republic

Se presenta una minuciosa revisión de la literatura médica nacional e internacional acerca de la enfermedad de Chagas en la República Mexicana, desde 1939, cuando el Trypanosoma cruzi, agente etiológico de la enfermedad de Chagas, fue reportado por primera vez, la cual ha hecho posible presentar el panorama general sobre esta parasitosis en el país, desde 1939 hasta 1991. Mediante una serie de tablas y figuras se presentan las localidades del país en donde se han encontrado casos humanos, reservorios y vectores de T. cruzi. Se hacen comentarios en relación a los resultados reportados y se señala la importancia de incrementar los estudios sobre la enfermedad de Chagas en México