RESUMEN
Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. Endocardial fibroelastosis has been associated with poor prognosis and outcomes. In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. This article aims to review the recent development in pathophysiology, diagnostic workup, and management, and to discuss possible differential diagnoses.
Asunto(s)
Fibroelastosis Endocárdica , Humanos , Lactante , Niño , Preescolar , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico , Endocardio , Diagnóstico DiferencialRESUMEN
Heart failure due to dilated cardiomyopathy is a major indication for paediatric cardiac transplantation. Endocardial fibroelastosis is a recognised pathological finding of unknown prognostic significance in paediatric dilated cardiomyopathy. To evaluate the nature of the association between left ventricular endocardial fibroelastosis and paediatric dilated cardiomyopathy, we reviewed surgical pathology reports of dilated cardiomyopathy explants (1986-2016) in order to characterise the pathological findings and to compare and contrast their frequency among four age groups: less than 1 year; 1-5 years; 6-10 years; and greater than 11 years. The 89 explants (47 males and 42 females) were all characterised by increased weight and left ventricular chamber dilatation without increased wall thickness. Ninety-five per cent of the specimens in the two youngest subsets had left ventricular endocardial fibroelastosis. Compared to the oldest age group, recipients aged 1-5 years had a 6-fold increase and those younger than 1 year a 19-fold increase in the odds of observing left ventricular endocardial fibroelastosis. Explants with and without endocardial fibroelastosis were otherwise phenotypically similar. In paediatric dilated cardiomyopathy endocardial fibroelastosis is a very common pathological finding, especially in infants and young children. We propose that the descriptive, clinico-pathological designation "Dilated Cardiomyopathy with Endocardial Fibroelastosis" should be adopted to facilitate future investigation into the potential prognostic/therapeutic significance of left ventricular endocardial fibroelastosis.
Asunto(s)
Cardiomiopatía Dilatada , Fibroelastosis Endocárdica , Trasplante de Corazón , Cardiomegalia , Niño , Preescolar , Fibroelastosis Endocárdica/complicaciones , Endocardio/patología , Femenino , Ventrículos Cardíacos , Humanos , Lactante , MasculinoRESUMEN
In a 6-month-old, intact female, Japanese spitz presenting with severe dyspnea, lung ultrasonography revealed confluent B lines associated with severe echocardiographic left sided volume overload and systolic dysfunction. A congenital shunt or valvular dysplasia was not demonstrable. On electrocardiogram, there was a constant sinus rhythm, respectively sinus tachycardia. Cardiac troponin I was normal. Within 2 days of admission, the dog died of heart failure. On macroscopic postmortem examination, the left ventricle and atrium were markedly dilated, and the left ventricular endocardium had a mild diffuse whitish appearance. Histopathology revealed moderate to severe thickening of the left ventricular endocardium, composed mostly of abundant elastic fibers and fewer collagen fibers, diagnostic for endocardial fibroelastosis. In addition, there were mild degenerative changes of the atrioventricular valves. Endocardial fibroelastosis is a rare congenital disease and should be considered in a young dog if more common causes of echocardiographic dilated cardiomyopathy phenotype are ruled out.
Asunto(s)
Enfermedades de los Perros/diagnóstico , Fibroelastosis Endocárdica/veterinaria , Insuficiencia Cardíaca/veterinaria , Animales , Diagnóstico Diferencial , Enfermedades de los Perros/diagnóstico por imagen , Enfermedades de los Perros/fisiopatología , Perros , Disnea/etiología , Disnea/veterinaria , Ecocardiografía/veterinaria , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico , Femenino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , LinajeRESUMEN
The purpose of the study was to investigate the morphogenesis of the left ventricle in the hypoplastic left heart syndrome (HLHS). There are five types of hypoplastic left ventricles were identified: with a slit-like shape and hypoplasia of LV wall, with a slit-like cavity shape and wall hypertrophy and types with endocardial fibroelastosis (with a cylindrical cavity shape, with lacunar cavities and lacunar-cylindrical cavity of the left ventricle), as a result of differences in the wall structure, cavity shape, presence or absence of endocardial fibroelastosis. The analysis of morphometric data of pathomorphological types of the left ventricle in the HLHS revealed the possible ways of their morphogenesis. Left displacement of interventricular septum in embryogenesis at 4-5 weeks of intrauterine development is associated with the occurrence of atresia of the left atrioventricular orifice and aortic valve and the appearance of a slit-like shape and hypoplasia of LV wall in the HLHS. The displacement of only the conotruncus septum leads to the appearance of a slit-like shape of cavity and hypertrophy of LV wall in the HLHS. The pathomorphological types with endocardial fibroelastosis in the HLHS depends on the stage of embryogenesis of myocardium at which fibroelastosis appears: before the myocardial compaction (up to 4th week of gestation) - the lacunar shape of LV cavity with thin compact layer of myocardium; during the compaction of myocardium (5-6th week of gestation) - the lacunar-cylindrical shape of LV cavity and after compaction (after 7-8th week of fetal development) - a cylindrical shape of LV cavity.
Asunto(s)
Fibroelastosis Endocárdica/patología , Válvulas Cardíacas/embriología , Ventrículos Cardíacos/embriología , Síndrome del Corazón Izquierdo Hipoplásico/patología , Morfogénesis , Válvula Aórtica , Fibroelastosis Endocárdica/complicaciones , Válvulas Cardíacas/patología , Ventrículos Cardíacos/anomalías , HumanosAsunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/etiología , Obstrucción del Flujo Ventricular Externo/cirugía , Biopsia con Aguja , Dolor en el Pecho/diagnóstico , Dolor en el Pecho/etiología , Ecocardiografía Transesofágica/métodos , Fibroelastosis Endocárdica/cirugía , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagenRESUMEN
Hypertrabeculation/noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/noncompaction on echocardiography. This supports the hypothesis that this condition is highly arrhythmogenic and is associated with high mortality similarly to the left ventricular hypertrabeculation/noncompaction cardiomyopathy.
Asunto(s)
Muerte Súbita Cardíaca/etiología , Electrocardiografía/métodos , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico por imagen , Disfunción Ventricular Derecha/complicaciones , Disfunción Ventricular Derecha/diagnóstico por imagen , Adulto , Fibroelastosis Endocárdica/fisiopatología , Resultado Fatal , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Disfunción Ventricular Derecha/fisiopatologíaAsunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Fibroelastosis Endocárdica , Anuloplastia de la Válvula Mitral/métodos , Insuficiencia de la Válvula Mitral , Adulto , Cardiomiopatía Restrictiva/etiología , Cardiomiopatía Restrictiva/fisiopatología , Cardiomiopatía Restrictiva/cirugía , Disección/métodos , Ecocardiografía Transesofágica/métodos , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico , Fibroelastosis Endocárdica/fisiopatología , Humanos , Imagen por Resonancia Cinemagnética/métodos , Masculino , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/etiología , Insuficiencia de la Válvula Mitral/fisiopatología , Insuficiencia de la Válvula Mitral/cirugía , Resultado del Tratamiento , Disfunción Ventricular/etiología , Disfunción Ventricular/fisiopatologíaRESUMEN
A 62-year-old woman had a prior ischemic stroke in the right temporal lobe with dysarthria and dysesthesia of the left hand. Embolic stroke of undetermined source (ESUS) was diagnosed and warfarin was administered. However, transient ischemic attack recurred upon admission to our hospital. Paroxysmal atrial fibrillation and cerebral arterial stenotic lesions were absent. Transesophageal echocardiography revealed a mobile hyperechoic structure on the aortic valve indicating papillary fibroelastoma. She was diagnosed with a brain embolism due to the intracardiac tumor which was surgically excised and pathologically confirmed as papillary fibroelastoma. This type of tumor is relatively rare but it is important as an embolic source especially in ESUS. Transesophageal echocardiography was indispensable for detecting the embolic source in this patient with ESUS.
Asunto(s)
Ecocardiografía Transesofágica , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico por imagen , Infarto del Miocardio/diagnóstico por imagen , Infarto del Miocardio/etiología , Infarto Cerebral/diagnóstico por imagen , Infarto Cerebral/etiología , Diagnóstico Diferencial , Fibroelastosis Endocárdica/patología , Fibroelastosis Endocárdica/cirugía , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
BACKGROUND The aim of this study was to investigate the clinical presentation, operative data, and early and late outcomes of a large patient cohort undergoing surgical treatment for cardiac tumors in our institution. MATERIAL AND METHODS A total of 181 patients underwent surgery because of suspected cardiac tumor in our institution between 1998 and 2016. In 162 cases, the diagnosis was confirmed postoperatively and these patients were included in this study. Preoperative baseline characteristics, operative data, and postoperative early and long-term outcomes were analyzed. RESULTS Mean age at presentation was 56.6±17.6 years, and 95 (58.6%) patients were female. There were 126 (77.8%) patients with benign cardiac tumors, while the remaining patients had malignant tumors (primary and metastasized). The mean follow-up time was 5.2±4.7 years. The most frequent histologically verified tumor type was myxoma (63%, n=102). In terms of malignant tumors, various types of sarcomas presented most primary malignant cardiac tumors (7.4%, n=12). The mean ICU length of stay was 1.7±2.2 days and overall in-hospital mortality was 3.1% (n=5). Frequent postoperative complications included mediastinal bleeding (5.8%, n=9), wound infection (1.3%, n=2), acute renal failure (5.6%, n=9), and major cerebrovascular events (n=7, 4.6%). The overall cumulative survival after cardiac tumor resection was 94% at 30 days, 85% at 1 year, 72% at 5 years, and 59% at 15 years. CONCLUSIONS Surgical treatment of cardiac tumors is a safe and highly effective strategy associated with good early and long-term outcomes.
Asunto(s)
Neoplasias Cardíacas/patología , Neoplasias Cardíacas/cirugía , Adulto , Anciano , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Estudios de Cohortes , Fibroelastosis Endocárdica/complicaciones , Femenino , Neoplasias Cardíacas/mortalidad , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Mixoma/complicaciones , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Factores de Tiempo , Resultado del TratamientoAsunto(s)
Apéndice Atrial/patología , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/cirugía , Infarto del Miocardio/diagnóstico por imagen , Angina de Pecho/diagnóstico por imagen , Angina de Pecho/fisiopatología , Apéndice Atrial/diagnóstico por imagen , Cateterismo Cardíaco/métodos , Dolor en el Pecho/diagnóstico , Dolor en el Pecho/etiología , Angiografía Coronaria/métodos , Diagnóstico Diferencial , Ecocardiografía Transesofágica/métodos , Electrocardiografía/métodos , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico por imagen , Fibroelastosis Endocárdica/patología , Femenino , Neoplasias Cardíacas/diagnóstico por imagen , Humanos , Imagen por Resonancia Cinemagnética/métodos , Persona de Mediana Edad , Infarto del Miocardio/etiología , Infarto del Miocardio/patología , Recurrencia , Medición de Riesgo , Resultado del TratamientoRESUMEN
No disponible
Asunto(s)
Humanos , Femenino , Adulto , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/etiología , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/cirugía , Insuficiencia Cardíaca , Ecocardiografía , Calcinosis/complicaciones , Calcinosis , Trasplante de Corazón-Pulmón/métodos , Disnea/complicaciones , Disnea , Radiografía Torácica/instrumentación , Radiografía Torácica/métodos , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar , Fibrosis Endomiocárdica/complicaciones , Fibrosis EndomiocárdicaAsunto(s)
Fibroelastosis Endocárdica/complicaciones , Contracción Miocárdica/fisiología , Obstrucción del Flujo Ventricular Externo/etiología , Angiografía Coronaria , Diagnóstico Diferencial , Fibroelastosis Endocárdica/diagnóstico , Humanos , Imagen por Resonancia Cinemagnética , Masculino , Persona de Mediana Edad , Sístole , Obstrucción del Flujo Ventricular Externo/diagnóstico , Obstrucción del Flujo Ventricular Externo/fisiopatologíaAsunto(s)
Muerte Súbita Cardíaca/patología , Fibroelastosis Endocárdica/patología , Cardiopatías/patología , Mixedema/patología , Adulto , Autopsia , Muerte Súbita Cardíaca/etiología , Diagnóstico Diferencial , Fibroelastosis Endocárdica/complicaciones , Endocardio/patología , Resultado Fatal , Femenino , Humanos , Miocardio/patología , Mixedema/complicacionesRESUMEN
Endocardial fibroelastosis is an important cause of congestive heart failure and death in infancy and early childhood. When present, it is commonly associated with non immune hydrops fetalis. The aim of this study is to draw attention for possible cardiac abnormalities in cases of fetal hydrops, and report a case of premature death by primary endocardial fibroelastosis with autopsy.
Asunto(s)
Fibroelastosis Endocárdica/patología , Insuficiencia Cardíaca/patología , Hidropesía Fetal/patología , Miocardio/patología , Adulto , Autopsia , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/patología , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Humanos , Hidropesía Fetal/diagnóstico , MasculinoRESUMEN
Neonatal aortic valvar stenosis can be challenging to treat because of the varied morphology of the valve, the association with hypoplasia of other left heart structures, and the presence of left ventricular systolic dysfunction or endomyocardial fibroelastosis. Balloon valvuloplasty and surgical valvotomy have been well described in the literature for the treatment of neonatal aortic stenosis. Transcatheter therapy for neonatal aortic stenosis is the preferred method at many centres; however, some centres prefer a surgical approach. Balloon valvuloplasty for neonatal aortic stenosis is reviewed in this manuscript, including the history of the procedure, technical aspects, and acute and long-term outcomes.
Asunto(s)
Estenosis de la Válvula Aórtica/cirugía , Valvuloplastia con Balón/métodos , Estenosis de la Válvula Aórtica/complicaciones , Estenosis de la Válvula Aórtica/congénito , Fibroelastosis Endocárdica/complicaciones , Humanos , Recién Nacido , Disfunción Ventricular Izquierda/complicacionesRESUMEN
La fibroelastosis endocárdica es una causa reconocida, aunque poco frecuente, de muerte súbita infantil. Se trata de una enfermedad rara con un índice de mortalidad muy alto. Su presentación típica es la de una insuficiencia cardiaca global de rápida evolución o arritmias, que requiere un trasplante cardiaco. Se presenta el caso de una niña de 19 meses de edad que, tras un proceso afebril compatible con un cuadro de vías respiratorias altas de 24 horas de evolución, presenta un paro cardiorrespiratorio y es trasladada a un área básica de salud, falleciendo a las pocas horas de su ingreso en la unidad de cuidados intensivos a la que fue derivada posteriormente. En el estudio post mortem se hallaron cambios compatibles con una fibrosis laxa endocárdica(AU)
Endocardial fibroelastosis is a known but rare cause for sudden death in children. It is a rare disease with a high rate of mortality. Its usual presentation is similar to global cardiac insufficiency or arrhythmia leading to heart transplantation. We report the case of a 19-month old female who, after having 24 hours of a non feverish upper respiratory infection, went into cardiac arrest and was taken to the outpatient's department, but died a few hours later in the intensive care unit. In post-mortem studies evidence of endocardial fibrosis laxa was found(AU)
Asunto(s)
Humanos , Femenino , Lactante , Fibroelastosis Endocárdica/complicaciones , Fibroelastosis Endocárdica/diagnóstico , Fibroelastosis Endocárdica/mortalidad , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Muerte Súbita/epidemiología , Muerte Súbita/patología , Muerte Súbita del Lactante/diagnóstico , Muerte Súbita del Lactante/epidemiología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca , Arritmias Cardíacas/complicaciones , Arritmias Cardíacas/mortalidadRESUMEN
Endocardial fibroelastosis is a cardiomyopathy not commonly seen in the present age. We describe the case of a 4-year-old girl who presented with sustained incessant ventricular tachycardia, a severely dilated left ventricle, and cardiac dysfunction refractory to all medical management and even to ablation; she eventually underwent cardiac transplantation. The diagnosis was made only after histopathologic examination of the explanted heart showed clear evidence of endocardial fibroelastosis. Through this report, we would like to highlight the fact that primary endocardial fibroelastosis can masquerade as idiopathic dilated cardiomyopathy and that associated frequent premature ventricular contractions and nonsustained ventricular tachycardia require close monitoring. Progressive ventricular dilation and ventricular dysfunction can convey a poor prognosis. Sustained recalcitrant ventricular tachycardia in these patients can be a life-threatening event that requires emergent mechanical support and heart transplantation.
Asunto(s)
Cardiomiopatía Dilatada/etiología , Fibroelastosis Endocárdica/complicaciones , Taquicardia Ventricular/etiología , Antiarrítmicos/uso terapéutico , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/terapia , Ablación por Catéter , Preescolar , Ecocardiografía , Electrocardiografía , Fibroelastosis Endocárdica/diagnóstico , Fibroelastosis Endocárdica/terapia , Oxigenación por Membrana Extracorpórea , Femenino , Trasplante de Corazón , Humanos , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/terapia , Resultado del TratamientoRESUMEN
The authors report a case of cerebral infarction in a young woman with no obvious risk factors for stroke. The use of effective cardiac imaging enabled a diagnosis of mitral valve papillary fibroelastoma to be made and aided effective surgical management.