Role and safety of endomyocardial biopsy in the diagnosis of myocardial disease in children / 中华实用儿科临床杂志

Objective:To explore the role and safety of endomyocardial biopsy (EMB) in the diagnosis of pediatric myocardial disease.Methods:Demographic, clinical and histopathological data of all children receiving EMB in Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine between January 2016 and August 2020 were collected.The pathological results and the procedure-related complications were retrospectively analyzed.Results:A total of 22 patients with the mean age of (10.2±3.1) years underwent EMB.Among them, 13 cases (59.1%) underwent right ventricular EMB, 5 cases (22.7%) underwent left ventricular EMB, and 4 cases (18.2%) underwent biventricular EMB.Among the 12 patients with clinically suspected myocarditis or unexplained heart failure, 4 cases were diagnosed with lymphocytic myocarditis, 2 cases were dilated cardiomyopathy, and 1 case was inflammatory cardiomyopathy.Seven patients presented ventricular diastolic dysfunction, including 6 cases of restrictive cardiomyopathy and 1 of constrictive pericarditis.Three patients exhibited hypertrophic cardiomyopathy with pre-excitation syndrome, involving 2 cases were diagnosed with glycogen storage cardiomyopathy.EMB was successfully performed in all patients.No patient died, and procedure-related complications were not reported.Conclusions:EMB assists the diagnosis and treatment of pediatric patients with selected myocardial disease, which is relatively safe with less complications if performed by experienced interventionalists in qualified pediatric cardiovascular medical centers.

Role of cardiac magnetic resonance imaging in myocarditis patients with biopsy negative: a retrospective case series study / 中华心血管病杂志

Objective: To analyze the cardiac magnetic resonance (CMR) imaging feature of clinically diagnosed myocarditis patients with negative endocardial biopsy (EMB) results, and to further demonstrate the diagnostic value of CMR in these patients. Methods: This was a retrospective case series study. Fourteen patients, who were clinically diagnosed as myocarditis according to 2013 European Society of Cardiology (ESC) clinical diagnostic criteria for myocarditis, but with negative EMB results, were enrolled. All patients underwent CMR examinations. The morphological, functional and histological changes of the heart were assessed based on black blood sequence, cine sequence, T2W-STIR sequence and contrast agent late gadolinium enhancement,(LGE). Results: There were 10 males and 4 females in this cohort, the age was (25.6±13.2) years. The interval between symptom onset and CMR was 21 (13, 60) days, and the interval between symptom onset and EMB was 19 (9, 40) days. There were 13 patients with abnormal CMR results including myocardial oedema, fibrosis, decreased ejection fraction, pericardial effusion or increased cardiac chamber dimension. Nine out of 14 patients had CMR morphological and/or functional abnormalities, including 1 case of left atrium enlargement, 1 case of left ventricle enlargement, 3 cases of right ventricle enlargement, 4 cases of increased left ventricular end diastolic volume index. Left ventricular ejection fraction was<50% in three cases, right ventricular ejection fraction was<40% in 5 cases, and pericardial effusion depth>3 mm was detected in 3 cases. Of the 14 patients, 11 had histological changes, of which 6 had T2 ratio≥2. Among the 10 patients (10/14) with positive LGE, the most common patterns were subepicardial LGE of the lateral wall and/or midwall LGE of the septum (n=9); 2 cases showed extensively subendocardial LGE of the left ventricular wall. No LGE involved in the right ventricular wall in the whole cohort. Conclusion: CMR plays a complementary role in the diagnosis of myocarditis in clinically diagnosed myocarditis patients with negative EMB findings.

Feasibility and safety of right ventricular endomyocardial biopsy via the right internal jugular vein approach / 中华心血管病杂志

Objective: To evaluate the feasibility and safety of right ventricular endomyocardial biopsy (EMB) via the right internal jugular vein approach. Methods: It was a retrospective and descriptive study. A total of 272 patients, who underwent right ventricular EMB from December 2014 to June 2020 in Fuwai Hospital and Peking Union Medical College Hospital were enrolled. The preliminary diagnosis included suspected myocarditis, myocardiopathy, unexplained heart failure etc after exclusion of coronary heart disease. Clinical characteristics including age, sex, height, weight, NYHA functional class, NT-proBNP, chest radiography, echocardiography, and hemodynamics parameters were collected at baseline. EMB was performed via right internal jugular vein approach under the biplane fluoroscopic guidance. Success rate was calculated in this study. Complications related to operation were recorded according the following definitions. Major complications included death, urgent cardiac surgery, advanced cardiac life support, pericardiocentesis in cardiac tamponade, permanent complete atrioventricular block requiring permanent pacing etc. Minor complications included pericardial effusion without pericardiocentesis, temporary (lasting less than 24 hours) or permanent right bundle-branch block, temporary Mobitz type Ⅱ atrioventricular block (AV block) with AV conduction 2∶1 requiring medical treatment with atropine, or additive temporary pacing, non-sustained ventricular tachycardia with long runs of more than 10 ventricular complexes, and an episode of atrial fibrillation lasting less than 12 hours or cardioversion of atrial fibrillation. Other complications included tricuspid anterior chorda rupture and new onset tricuspid regurgitation after EMB. Results: In this study, right ventricular EMB were performed successfully in 270 patients, the total success rate was 99.3% (270/272), and EMB were failed in 2 (0.7%) patients. Age of the enrolled patients was (42.7±16.9) years, and there were 164 (60.3%) males. Major complication including cardiac tamponade requiring pericardiocentesis occurred in 2 (0.7%) patients. Minor complications such as small amount pericardial effusion occurred in 18 (6.6%) patients, tricuspid anterior chorda rupture occurred in 1 (0.4%) patient. No patient died, or requiring permanent pacing, or requiring emergency cardiac surgery. The complication rate was 9.3% (13/140), 7.8% (7/90), and 2.4% (1/42) in operators with 1, 2, and 3 years' experience. Conclusions: EMB via the right jugular vein approach under fluoroscopic guidance is a simple, safe and feasible procedure. The complication rates decrease significantly with increasing operator experience.

Diagnostic criteria and research progress on heart allograft pathology / 器官移植

With the improvement of surgical technique of heart transplantation and clinical application of potent immunosuppressant, the quantity of heart transplantation and the survival time of heart allograft have been significantly improved. However, a series of complications, such as right ventricular failure, ischemia-reperfusion injury, acute rejection, "Quilty lesion", infection and chronic rejection characterized by transplant coronary artery disease (TCAD) may still occur at different stages after heart transplantation. The application of endomyocardial biopsy (EMB) makes it possible to observe and understand the pathological features of multiple complications of heart allograft including rejection, which has become the most accurate diagnostic tool for postoperative complications. In this article, the brief history of heart allograft pathology, main postoperative complications and pathological diagnostic criteria, and cutting edge research progress on diagnostic criteria of rejection were illustrated, aiming to bring clinical benefits to more recipients undergoing heart transplantation.

Idiopathic systemic granulomatous pathology causing sudden death due to myocarditis: A rare case report.

Idiopathic granulomatous myocarditis is extremely rare, particularly since the introduction of drugs effective against tuberculosis (TB), viruses, fungi and the effective treatment of sarcoidosis. Here is a case of a 65-year-old female prisoner having history of sudden collapse and ultimately death. Autopsy fi ndings of various viscera on histopathological examination show granulomatous pathology, that is, in spleen, liver and in the left ventricular wall of heart. Ziehl-Neelsen staining of the sections show the absence of acid fast bacilli, negative for fungal staining as most of the granulomas are noncaseating type with presence of giant cells having no asteroid body and Schuamann body, real-time polymerase chain reaction for TB is negative. Idiopathic giant cell myocarditis is a disease of relatively young adults, that is, between 3rd and 4th decade of life. So, this case is strongly considered to be a case of sudden death due to myocarditis as a result of idiopathic systemic granulomatous pathology, a rare case in in literature.

Desmin-related restrictive cardiomyopathy in a pediatric patient: A case report.

Restrictive cardiomyopathies in the pediatric population have diverse etiologies, including storage diseases like hemosiderosis, glycogenoses and desmin with its associated proteins. Desmin-related myopathy is a rare familial disorder of the cardiac and skeletal muscle characterized by intrasarcoplasmic accumulation of desmin-reactive deposits in the muscle cells. The patients commonly present with cardiac involvement such as conduction blocks and/or restrictive cardiomyopathy. Diagnosis of desmin cardiomyopathy depends on light microscopic evaluation of endomyocardial biopsy, where abnormal deposition of desmin can be documented on immunohistochemistry and ultrastructural examination. The index report presents the clinical, light microscopic and ultrastructural fi ndings of desmin cardiomyopathy.

Complication Rate of Transfemoral Endomyocardial Biopsy with Fluoroscopic and Two-dimensional Echocardiographic Guidance: A 10-Year Experience of 228 Consecutive Procedures

Endomyocardial biopsy (EMB) is one of the reliable methods for the diagnosis of various cardiac diseases. However, EMB can cause various complications. The purpose of this study is to evaluate the complication of transfemoral EMB with both fluoroscopic and two-dimensional (2-D) echocardiographic guidance. A total of 228 patients (148 men; 46.0+/-14.6 yr-old) who underwent EMB at Kyungpook National University Hospital from January 2002 to June 2012 were included. EMB was performed via the right femoral approach with the guidance of both echocardiography and fluoroscopy. Overall, EMB-related complications occurred in 21 patients (9.2%) including one case (0.4%) with cardiac tamponade requiring emergent pericardiocentesis, four cases (1.8%) with small pericardial effusion without pericardiocentesis, two cases (0.9%) with hemodynamically unstable ventricular tachycardia (VT), one case (0.4%) with nonsustained VT, one case (0.4%) with tricuspid regurgitation, twelve cases (5.3%) with right bundle branch block. There was no occurrence of either EMB-related death or cardiac surgery. Left ventricular ejection fraction was significantly lower (32.0+/-18.7% vs 42.0+/-19.1%, P=0.023) and left ventricular end-diastolic dimension was larger (60.0+/-10.0 mm vs 54.2+/-10.2 mm, P=0.013) in patients with EMB related complications than in those without. It is concluded that transfemoral EMB with fluoroscopic and 2-D echocardiographic guidance is a safe procedure with low complication rate.

The value of cardiovascular magnetic resonance for diagnosis of viral myocarditis / 国际儿科学杂志

Endocardial myocardial biopsy is the gold standard in the diagnosis of viral myocarditis.However,the limitations of sampling to the low sensitivity,and invasive inspection methods limit its clinical application.The traditional diagnostic tool electrocardiography,laboratory values and echocardiography are lack of specificity.Thus,a non-invasive diagnostic approach with high sensitivity is in need.Cardiovascular magnetic resonance(CMR) can evaluate the function of the heart parameters,such as left ventricular function and regional wall motion,and various CMR imagings including T2-weighted imaging,and early- and late-contrast enhanced T1-weighted imaging can evaluate the hyperemia,oedema and necrosis of myocardial inflammation histological character.CMR provides a scientific basis for the diagnosis of viral myocarditis in clinical.

Amiloidosis cardíaca con obstrucción dinámica subaórtica / Cardiac Amyloidosis with Dynamic Subaortic Obstruction

La amiloidosis es una enfermedad infiltrativa sistémica que compromete el corazón y representa una causa importante de miocardiopatía restrictiva. En esta presentación se describe el caso de una paciente con insuficiencia cardíaca (IC) secundaria a miocardiopatía infiltrativa por depósito amiloide y obstrucción dinámica del tracto de salida del ventrículo izquierdo. El diagnóstico hematológico fue de mieloma múltiple por cadenas livianas y se demostró amiloidosis en dos tejidos extracardíacos. El ecocardiograma reveló aumento de los espesores parietales con obstrucción dinámica subaórtica significativa y la resonancia cardíaca mostró un patrón compatible con infiltración amiloide. La biopsia endomiocárdica confirmó la amiloidosis cardíaca. La publicación de este caso constituye la primera comunicación en nuestro país de esta forma de presentación atípica de amiloidosis cardíaca.

Primary amyloidosis is a systemic infiltrative disease that compromises the heart and represents an important cause of restrictive cardiomyopathy. We describe the case of a patient with heart failure secondary to an infiltrative cardiomyopathy with amyloid deposition and dynamic left ventricular outflow tract obstruction. The hematological diagnosis was light chain multiple myeloma with presence of amyloidosis in two extracardiac tissues. The echocardiogram revealed substantial wall thickening with significant dynamic subaortic obstruction; the magnetic resonance imaging showed a pattern suggestive of amyloid infiltration. An endomyocardial biopsy confirmed the diagnosis of cardiac amyloidosis. This is the first case of this atypical presentation of cardiac amyloidosis reported in our country.

Arritmia como única forma de presentación de la miocarditis / Arrhythmia as the only presentation form of myocarditis / Arritmia como a única forma de apresentação de miocardite

Introducción. Las arritmias ventriculares se presentan, en algunos pacientes, como único signo clínico de miocarditis, siendo ésta no incluida como probabilidad etiológica. La dificultad de la caracterización clínica de esta forma de presentación en esta patología no permite iniciar el tratamiento oportuno. Objetivo. Analizar la evolución clínica y el tratamiento de pacientes con arritmias, como única manifestación clínica de miocarditis. Materiales y método. Estudio descriptivo, observacional, transversal, retrospectivo de diecinueve pacientes con diagnóstico de miocarditis, asistidos en nuestra institución, entre los años 2000 y 2009. Se seleccionaron siete pacientes (35%), que presentaron arritmias ventriculares como única forma de manifestación clínica. La edad media de estos pacientes fue de 18 ± 7 años, cinco hombres y dos mujeres. Todos tuvieron biopsia endomiocárdica positiva para miocarditis. Se examinaron las historias clínicas a fin de observar el tipo de arritmia, la función ventricular, la respuesta al tratamiento recibido y la evolución clínica a corto y largo plazo. Para el análisis estadístico, se empleó el programa estadístico InfoStat/Profesional versión 2009p. Resultados. Todos los pacientes presentaron extrasístoles ventriculares frecuentes, 3 (43%) taquicardia ventricular no sostenida y 4 (57%) taquicardia ventricular sostenida, de éstos, 1 paciente padeció además fibrilación ventricular. La función ventricular fue normal en todos los pacientes (fracción de eyección: 62 ± 8,08%). Seis pacientes (86%) fueron tratados con inmunosupresores (corticoides y azatioprina), de los cuales 5 (72%) evolucionaron con extrasístoles ventriculares aisladas. Sólo un paciente continuó con taquicardia ventricular sostenida, que requirió implante de cardiodesfibrilador y ablación por radiofrecuencia. No hubo óbitos, en un seguimiento de 6 ± 3 años. Conclusiones. De los pacientes tratados con inmunosupresores se logró reducir la inflamación y controlar las arritmias. Se evidenció una buena evolución de los pacientes a corto y largo plazo.

Background. Ventricular arrhythmias occur in some patients as the only clinical sign of myocarditis; still it is not included as an etiological probability. The complexity of its clinical characterization does not allow an appropriate treatment. Aim.To analyze clinical evolution and treatment in those patients with arrhythmia as the only clinical manifestation of myocarditis. Materials and method. Descriptive, observational, transversal, retrospective study, enrolling nineteen patients diagnosed with myocarditis, attended in our institution within the period 2000-2009. Seven patients (35%) who presented ventricular arrhythmia as the only clinical manifestation were selected. Their mean age was 18 ± 7 years old, five men and two women. All of them underwent endomyocardial biopsy, resulting positive for myocarditis. Clinical histories were analyzed in order to examine arrhythmia type, ventricular function, response to received treatment, and clinical long and short-term evolution. Statistical analysis was performed with statistical program InfoStat/Profesional version 2009p. Results. All patients presented frequent ventricular extrasystoles; 3 (43%) non sustained ventricular tachycardia; 4 (57%) sustained ventricular tachycardia, and 1 of these patients had ventricular fibrillation. Ventricular function was normal in all patients (ejection fraction 62 ± 8,08%). Six patients (86%) received immunosupressors (corticoids y azatioprin), of which 5 (72%) evolved with isolated ventricular extrasystoles. Only one patient continued with sustained ventricular tachycardia, requiring defibrillator implantation and radiofrequency ablation. There were no deaths in a follow up of 6 ± 3 years. Conclusions. Immunosuppressive therapy allowed reducing inflammation and controlling arrhythmias. Patients showed a good short and long term evolution.

Introdução. As arritmias ventriculares ocorrem em alguns pacientes, como o único sinal clínico de miocardite, ainda não incluída como de probabilidade de diagnóstico. A dificuldade da caracterização clínica da doença não permite o tratamento adequado para controlá-lo. Objetivo. Analisar a evolução clínica e tratamento de pacientes com arritmias, como a única manifestação clínica da miocardite. Materiais e métodos. Estúdio descritivo, observacional, transversal, retrospectivo, com dezenove pacientes com diagnóstico de miocardite, atendidos em nossa instituição entre os anos 2000 e 2009. Foram selecionados sete pacientes (35%) com arritmia ventricular como única forma de manifestação clínica. A idade média desses pacientes foi de 18± 7 anos, cinco homens e duas mulheres. Todos foram submetidos à biópsia endomiocárdica, resultando positivo para miocardite. As histórias clínicas foram analisadas a fim de avaliar o tipo de arritmia, função ventricular, a resposta ao tratamento recebido e evolução clínica a longo e curto prazo. A análise estatística foi realizada com o programa estatístico InfoStat/Profesional versão 2009p. Resultados. Todos os pacientes apresentaram extra-sístoles ventriculares freqüentes, 3 (43%) taquicardia ventricular não sustentada; 4 (57%) taquicardia ventricular sustentada, e um destes pacientes também sofreu fibrilação ventricular. A função ventricular era normal em todos os pacientes (fração de ejeção de 62 ± 8,08%). Seis pacientes (86%) receberam imunossupressores (corticóides e azatioprina), dos quais 5 (72%) evoluíram com extra-sístoles ventriculares isoladas. Apenas um paciente continuou com taquicardia ventricular sustentada, necessitando de implante de desfibrilador e ablação por radiofreqüência. Não houve mortes em um seguimento de 6 ± 3 anos. Conclusões. A terapia imunossupressora permitiu reduzir a inflamação e controlar arritmias. Os pacientes apresentaram boa evolução a curto e longo prazo.

Propuesta de protocolo de biopsia cardíaca. Servicio de Anatomía Patológica y Departamento de Cardiología / Proposal of cardiac biopsy protocol. Service of Pathology and Department of Cardiology / Proposta de protocolo de biópsia cardíaca. Serviços de anatomia patológica e departamento de cardiologia

Introducción: Las biopsias endomiocárdicas se utilizan habitualmente para el diagnóstico de muchas patologías que han sido agrupadas por Billigham y Tazelaar en inflamatorias, metabólicas, endocrinas, neuromusculares, tóxicas, procesos linfoproliferativos y el diagnóstico de rechazo a trasplante cardíaco para evaluar la cardiotoxicidad por drogas (antraciclinas, dexoxirubina, cocaína, alcohol, entre otras); también como análisis de miocardio isquémico y su zona limítrofe. Objetivo: Trataremos de evaluar desde un punto de vista cuantitativo, la amplitud de observaciones que se han hecho de la biopsia cardíaca, en un innumerable listado de patologías, desde la MO a la ME (Microscopía óptica a microscopía electrónica). Se intentará graduar los hallazgos morfológicos de la misma, enlazándolos con la topografía y función, teniendo en cuenta, además, los factores bioquímicos y genéticos, organismos vivos, drogas, agentes físicos y procedimientos diagnósticos. Material y Métodos: Para el presente estudio se realizó una recopilación bibliográfica teniendo en cuenta los siguientes criterios: 1) cambios morfológicos sub-celulares-matriz tisular; 2) score de necrosis; 3) score inflamatorio, 4) score de fibrosis; 5) depósitos intracelulares y de pigmentos. Procesamiento de las muestran fijadas en formol buffer (tiempo 2-12 hs.) con coloración de rutina: H/E, Pas, Masson, Zihel Neelsen prolongada, Perls, Azul de Toluidina, Rojo Congo, Orceina. Panel con técnicas de I.H.Q.(BIO SB®).Conclusión: Creemos importante establecer un método que permita hacer una adecuada correlación clínico patológica aplicable a mejorar la interpretación de la injuria tisular, celular y facilitar de este modo, una correcta elección terapéutica.

Introdução: As biópsias endomiocárdicas são utilizadas habitualmente para o diagnóstico de muitas patologias, que foram agrupadas por Billigham e Tazelaar em inflamatórias, metabólicas, endócrinas, neuromusculares, tóxicas, processos linfoproliferativos e o diagnóstico de rejeição a trasplante cardíaco, para avaliar a cardiotoxicidade por drogas (antraciclinas, doxorrubicina, cocaína, álcool, entre outras). Também como análise de miocárdio isquêmico e sua zona limítrofe. Objetivo: Trataremos de avaliar sob o ponto de vista quantitativo, a amplidão de observações feitas da biópsia cardíaca, em uma inumerável lista de patologias, de MO a ME.Tentaremos graduar os descobrimentos morfológicos da mesma, enlaçando-os com a topografia e função, levando em consideração também os fatores bioquímicos e genéticos, organismos vivos, drogas, agentes físicos, procedimentos e diagnósticos.Material e Métodos: para o presente estudo realizou-se uma recopilação bibliográfica considerando os seguintes critérios, 1) alterações morfológicas subcelulares-matriz tissular; 2) escore de necrose; 3) escore inflamatório, 4) escore de fibrose; 5) depósitos intracelulares e de pigmentos. Processamento das amostras fixadas em formol buffer (tempo 2 -12 h.) com coloração de rotina: H/E, Pas, Masson, Zihel Neelsen prolongada, Perls, Azul de Toluidina, Vermelho Congo, Orceína. Painel com técnicas de I.H.Q. (BIO SB®). Conclusão: Achamos importante estabelecer um método que permita fazer uma adequada correlação clínico patológica aplicável a melhorar a interpretação da injúria tisular celular e, deste modo, facilitar uma correta decisão terapêutica.

Introduction: The endomiocárdica biopsies is habitually used for the diagnosis of many pathologies, grouped by Billigham and Tazelaar in inflammatory, metabolic, endocrine, neuromuscular, toxic, processes linfoproliferative and in diagnosis of rejection to heart transplant or to evaluate the cardiotoxicity for drugs (antracicline, dexoxirubine, cocaine, alcohol among other). Also as analysis of cardiac ischemia and their bordering area. Objective: We will try to evaluate from a quantitative point of view, the width of observations that you/they have been made of the heart biopsy in a countless listing of pathologies, from the MO to ME. We will try to graduate the morphologic discoveries of the same one, connecting them with the topography and function, also keeping in mind the biochemical and genetic factors, alive organisms, you drug, physical agents, procedures diagnoses. (Sherman Bloom, pag 329, I Diagnose of Cardiovasc. Pathology).Material and Methods: for the present study was carried out a bibliographical summary keeping in mind the following approaches, 1) changes morphologic subcellular-main tissue; 2) necrosis score; 3) inflammatory score 4) fibrosis score; 5) deposits intracellular and of pigments. Prosecution of they show them fixed in formol buffer (time 2 -12 hs.) with routine coloration: H/E, Pas, Masson, Zihel lingering Neelsen, Perls, Blue of Toluidine, Red Congo, Orceine. Panel with technical of I.H.Q. (BIO SB®). Conclusion: We believe as very important to establish a methodology eich allow an applicable pathological clinical appropriate correlation to improve the interpretation of the tissue insult and to facilitate in this way a correct therapeutic election.

Miocarditis viral en el adulto / Viral myocarditis in the adult

La miocarditis viral es resultado de una inflamación del miocardio provocada por diferentes virus. Esta enfermedad cardíaca constituye sin duda una urgencia cardiovascular en el adulto por las complicaciones que ocasiona. El cuadro clínico se caracteriza por arritmias e insuficiencia cardíaca que pueden conducir a la muerte. La secuela más frecuente de la miocarditis viral a largo plazo es la miocardiopatía dilatada. En el presente trabajo se revisa etiología, fisiopatología, sintomatología, diagnóstico y tratamiento de la miocarditis viral en el adulto

Viral myocarditis is the result of a myocardial inflammation provoked by different viruses. This cardiac disease is doubtless a cardiovascular urgency in the adult due to its complications. The clinical picture is characterized by arrhythmias and cardiac failure that may to lead to death. The more frequent long-term myocarditis sequela is the dilated myocardiopathy. In present paper etiology, pathophysiology, symptomatology, diagnosis and treatment of viral myocarditis in the adult are reviewed

A case of cardiac involvement in Churg-Strauss syndrome presenting without cardiomegaly in an asymptomatic patient / 대한내과학회지

We report a case of Churg-Strauss syndrome with cardiac involvement presenting without cardiomegaly or cardiopulmonary symptoms. A 47-year-old woman was referred to our institution for myalgia, peripheral numbness, and eosinophilia. She had been diagnosed with bronchial asthma and allergic rhinitis four years ago. The patient exhibited eosinophilia (71%) and elevated cardiac enzymes (cTnI, 2.977 ng/mL). Cardiomegaly was not observed on chest radiography, but nonspecific ST segment changes were observed on electrocardiography. A transthoracic echocardiography revealed a dilated left ventricular cavity, a decreased left ventricle (42%), and diastolic dysfunction. Contrast-enhanced cardiac magnetic resonance imaging revealed delayed hyperenhancement 10 minutes after injecting gadolinium. An endomyocardial biopsy showed eosinophilic myocarditis associated with vasculitis. The patient was diagnosed with Churg-Strauss syndrome and received combination therapy with steroid and cyclophosphamide. After the second treatment cycle, the blood eosinophilia disappeared and the vasculitis and infiltration of eosinophils into the endomyocardial tissue had completely resolved.

Miocardiopatías en población infantil y adolescente: estudio en autopsias y biopsias endomiocárdicas / Diseases of the myocardium in a infantile and adolescent population: study en autopsy and endomyocardial biopsy

El principal objetivo de este trabajo es contribuir al conocimiento de la anatomía patológica especialmente del aspecto histopatológico de las enfermedades del miocardio en menores o igual de 18 años de edad en Venezuela. Se estudiaron desde el punto anatomopatológico 19 casos de enfermedades del miocardio en una población infantil y adolescente de ambos sexos, (8 autopsias y 11 biopsias endomiocárdicas), según los criterios de la OMS. En 7 casos se hizo el estudio del miocardio mediante microscopio electrónico. Para el diagnóstico de las miocarditis se aplicaron los criterios de Dallas. Las miocardiopatías fueron más frecuentes que las miocardiopatías específicas(11/9), siendo las miocardiopatías dilatadas y las miocardiopatías inflamatorias las que ocuparon el primer lugar en ambos grupos respectivamente. Las alteraciones histológicas y ultraestructurales fueron inespecíficas caracterizadas por hipertrofia celular y degeneración celular, fibrosis en las miocardiopatías dilatadas y proceso inflamatorio en las miocarditis. Se señalaron algunos aspectos característicos histopatológicos de la biopsia endomiocárdica en la fibrosis endomiocárdica, (dos casos), de la distrofia muscular (un caso de autopsia)y de la miocardiopatía hipertrófica (un caso de autopsia). No se encontró un solo caso de miocarditis chagásica ni de displasia arritmogénica del ventrículo derecho. Se concluyó que el perfil de las miocardiopatías en las dos primeras décadas de la vida es el mismo que en el adulto. Igualmente, las miocarditis ocuparon el primer lugar de las miocardiopatías inflamatorias.

The main objective of this work is to contribute specially to the knowledge of the pathological anatomy of the histopathologic aspect of the disease of the myocardium of young in or smaller of 18 years of age in Venezuela. 19 cases of disease of the myocardium in an infantile and adolescent population of both sexes studied (8 endomyocardial biopsy and 11 autopsy), according to the criteria of the WHO. In 7 cases the study became of the myocardium by means of electron microscope. For the identification of the myocardditis the criteria of Dallas were applied. The cardiomyopathies were more frequent than the specific cardiomyopathy (11/9), and the inflammatory cardiomyopathy those that respectively occupied the first place in both groups. The histological and ultrastructural alterations were unspecific characterized by hypertrophied cellular and callular degeneration, fibrosis in the cardiomyopathy and inflammatory process in the myocarditis. Some histopathologic aspects were indicated characteristic of the endomyocardial biopsy in the endomyocardial fibrosis. (two cases), of the muscular dystrophy (a case of autopsy) and of the hypertrophic cardiomyopathy (a case of autopsy). Was not a single case of cardiomyopathy arritmogenic of the right ventricle and chagastic myocarditis. One concluded that the profile of the cardiomyopathies in the two first decades of the life, is he himself who in the adult. Also, the myocarditis occupied the first place of the inflammatory cardiomyopathy.

Pathologic Analysis of Endomyocardial Biopsies in Heart Transplantation

Endomyocardial biopsy (EMB) is a valuable diagnostic procedure for the surveillance of cardiac allograft rejection. Interpretation of individual cases is still problematic due to variations of findings for grading of rejection and other associated lesions. We reevaluated an experience on endomyocardial biopsies to develop better diagnostic criteria for rejection and other complications. Immunohistochemical studies against cytokines were performed to assess the usefulness of the method for the diagnosis or researches. A total of 249 EMBs taken from 33 cardiac allograft recipients were reviewed. There were 25 males and 8 females. Dilated cardiomyopathy was present (24 cases) and valvular heart disease (4 cases), restrictive cardiomyopathy (3 cases) were also common conditions. We applied the grading system of the International Society for Heart Transplantation (ISHT) for the assessment of acute cellular rejection. Grades of 0, 1A, 1B, 2, 3A and 3B were 39.0%, 28.1%, 11.2%, 11.5%, 12.4% and 1.6% respectively, but 3.2% were inadequate. Thirty five episodes of grade 3A or 3B were present in 17 patients. The response to therapy was assessed using a next follow up biopsy, which revealed resolving or resolved rejection in 85% of patients. The intensity of immunohistochemical stains for IL-6 and TNF-alpha was increased in proportion to the histologic grade but Quilty lesion and cardiomyopathy also showed a positive reaction. The other pathologic findings were ischemic change, previous biopsy site, interstitial edema and fibrosis, and Quilty lesion. These findings showed usefulness of endomyocardial biopsy not only for the evaluation of cardiac allograft rejection but also for the diagnosis of associated cardiac lesions. Immunohistochemical study of the cytokines was related to the degree of inflammation rather than degree of rejection.

A Case of Eosinophilic Myocarditis associated with Hypereosinophilia showing Myocardial Edema

Chusid et al proposed diagnostic criteria of hypereosinophilic syndrome (HES) that remain valid today. These were, (1) a sustained peripheral blood eosinophil count of more than 1500/L present for longer than 6 months ; (2) no evidence of other apparent causes for eosinophilia, and (3) presumptive signs of parenchymal organ involvement. Any organ system may be affected in HES, but the most severe clinicopathological involvements are of the heart and nervous system. Although multiple organ systems may be involved, the most common cause of morbidity and mortality is cardiac involvement with extensive fibrous thickening of the endomyocardium and overlying thrombus. We report a case of acute peri-myocarditis with eosinophilia, which was confirmed as eosinophilic myocarditis by endomyocardial biopsy, with literature review.

A Case of Eosinophilic Myocarditis associated with Hypereosinophilia showing Myocardial Edema

Chusid et al proposed diagnostic criteria of hypereosinophilic syndrome (HES) that remain valid today. These were, (1) a sustained peripheral blood eosinophil count of more than 1500/L present for longer than 6 months ; (2) no evidence of other apparent causes for eosinophilia, and (3) presumptive signs of parenchymal organ involvement. Any organ system may be affected in HES, but the most severe clinicopathological involvements are of the heart and nervous system. Although multiple organ systems may be involved, the most common cause of morbidity and mortality is cardiac involvement with extensive fibrous thickening of the endomyocardium and overlying thrombus. We report a case of acute peri-myocarditis with eosinophilia, which was confirmed as eosinophilic myocarditis by endomyocardial biopsy, with literature review.

A Case of Incessant Ventricular Tachycardia Abolished after Endomyocardial Biopsy

Ventricular tachycardia is an important tachyarrhythmia which is encountered commonly in clinical field. The accompanying manifestations could be variable just from palpitation to sudden cardiac death. The classification of this arrhythmia has not completly settled yet, but in a broad way this arrhythmia is classified according to the prescence or abscence or underlying heart disease, especially coronary artery disease. Recently, therapeutic modalities for this arrhythmia have been changed a lot from classical antiarrhythmic drugs to radiofrequency ablation or ICD implantation although there are still some problems to overcome. We experienced a case of 34-year-old female having an incessant ventricular tacycardia which was abolished after endomyocardial biopsy performed to differentiate underlying myocardial pathology.