RESUMEN
Las miocardiopatías (MC) son enfermedades del músculo cardíaco infrecuentes, con una incidencia anual de 1.1-1.2 casos por 100.000 niños. La miocardiopatía dilatada (MCD) es la principal forma, se caracteriza por dilatación ventricular y disfunción sistólica, y es causa importante de insuficiencia cardíaca congestiva (ICC). Las etiologías en niños son múltiples, siendo idiopáticas en el 50%-70%. En la evaluación de un niño con MCD es fundamental descartar causas secundarias potencialmente reversibles. El ecocardiograma es la principal herramienta diagnóstica: permite establecer el fenoti po cardíaco, grado de compromiso funcional, y la evolución y respuesta al tratamiento médico. El pronóstico es limitado, siendo mejor en pacientes menores a 1 año al momento de presentación, post miocarditis, o con menor grado de disfunción sistólica ventricular. En los primeros 2 años post presentación alrededor de 20% tienen normalización de la función ventricular; 40%-50% fallece o requiere un trasplante cardíaco (TC) en los primeros 5 años. El tratamiento médico se basa en recomendaciones de adultos, siendo la evidencia pediátrica muy limitada. El TC es la terapia definitiva en pacientes con ICC terminal, con excelentes resultados a corto y mediano plazo. Una proporción importante de pacientes requiere estabilización en lista de espera, incluyendo asistencia mecánica circulatoria como puente a trasplante. El objetivo de este artículo es actualizar la información dis ponible en etiología, mecanismos fisiopatológicos, factores pronósticos, y tratamiento de la MCD en niños.
Pediatric cardiomyopathies are infrequent diseases of the cardiac muscle, with an annual inciden ce of 1.1 to 1.2 per 100,000 children. Dilated cardiomyopathy (DCM) is the predominant form, characterized by ventricular dilatation and systolic dysfunction. Etiologies are multiple, with at least 50%-70% of cases being idiopathic. When assessing a child with DCM, secondary potentially reversible causes must be ruled out. The main diagnostic tool is the echocardiogram which allows the identification of cardiac phenotype, to establish the degree of functional compromise, and res ponse to medical therapy. Prognosis is limited but more favorable in infants younger than 1 year at the onset, post myocarditis, or with a lesser degree of ventricular dysfunction. At least 20% of patients may recover ventricular function in the first 2 years after the onset and 40%-50% may die or need heart transplant in the first 5 years. Medical therapy is mainly based on adult experience with limited scientific evidence in children. Heart transplant is the therapy of choice in patients with end-stage disease, with excellent short- and medium-term survival. A significant proportion of patients may require stabilization on the waiting list, including the use of mechanical circulatory support as a bridge to transplantation. The purpose of this revision is to update the available infor mation on etiology, physiopathological mechanisms, prognostic factors, and management of DCM in children.
Asunto(s)
Humanos , Lactante , Preescolar , Niño , Cardiomiopatía Dilatada/fisiopatología , Trasplante de Corazón , Pronóstico , Ecocardiografía , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/terapia , Listas de Espera , Factores de EdadRESUMEN
BACKGROUND: Left ventricular contraction dyssynchrony (LVCD) has been related to induced ischemia and transmural scar but the interplay of myocardial viability and dyssynchrony is unknown. The aim of the present study was to establish the role of dyssynchrony in the context of a viability study performed with nitrate augmentation gated single photon emission computed tomography (GSPECT) myocardial perfusion imaging (MPI). METHODS: Fifty-four consecutive patients with ischemic dilated cardiomyopathy (IDC) and depressed left ventricular ejection fraction (LVEF) were included. They underwent a two-day rest/nitroglycerine (NTG) study GSPECT MPI to determine the myocardial viability. Patients with a nitrate-induced uptake increase of > 10% vs baseline, in at least, two consecutive dysfunctional segments were considered viable as well as those who showed no improvement in the uptake but the uptake was > 50% on post NTG study. Patients with no nitrate-induced uptake increase of > 10% and the uptake of < 50% were considered non-viable. Perfusion, function and LVCD were compared in 25 viable patients vs 29 non-viable patients at baseline and after NTG administration. RESULTS: After NTG administration, in the viable group, the LVEF increased (36.44 ± 6.64% vs 39.84 ± 6.39%) and the end-systolic volume decreased significantly (119.28 ± 31.77 mL vs 109.08 ± 33.17 mL) (P < 0.01). These patients also experienced a significant reduction in the LVCD variables: phase standard deviation was reduced in the post NTG study (57.77° ± 19.47° vs 52.02° ± 17.09°) as well as the phase histogram bandwidth (190.20° ± 78.83° vs 178.0° ± 76.14°) (P < 0.05). Functional and LVCD variables remained similar in the non-viable patients (P > 0.05). CONCLUSION: In patients with IDC and depressed LVEF, the myocardial viability detected by rest/ NTG GSPECT MPI, might determine LVCD improvement.
Asunto(s)
Tomografía Computarizada por Emisión de Fotón Único Sincronizada Cardíaca/métodos , Cardiomiopatía Dilatada/fisiopatología , Contracción Miocárdica/fisiología , Isquemia Miocárdica/fisiopatología , Imagen de Perfusión Miocárdica/métodos , Función Ventricular Izquierda/fisiología , Anciano , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
A Cardiomiopatia Dilatada Canina (CMD), enfermidade que afeta principalmente cães de porte grande macho e adulto, se caracteriza por uma dilatação miocárdica das câmaras cardíacas e diminuição de sua capacidade de contração, com consequentes alterações hemodinâmicas e posterior evolução para um quadro de insuficiência cardíaca congestiva. A ecocardiográfica tem sido cada vez mais considerada como diagnóstico definitivo para a CMD canina por ser um método dinâmico e não invasivo de avaliação do coração, que permite identificar a dilatação da parede ventricular. Esta revisão bibliográfica tem objetivo de revisar aspectos relacionados à fisiopatologia da doença, manifestações clínicas e métodos de diagnósticos mais atuais e possíveis interversões terapêuticas para proporcionar melhor estabilidade ao quadro.
Canine dilated Cardiomyopathy (CMD), a disease that affects mainly male and adult large-sized dogs, is characterized by myocardial dilation of the cardiac chambers and decreased contraction capacity, with consequent alterations Hemodynamic and posterior progression to a congestive heart failure condition. The echocardiographic has been increasingly considered as a definitive diagnosis for canine CMD because it is a dynamic and non-invasive method of assessing the heart, which allows to identify the dilation of the ventricular wall. This bibliographic review aims to review aspects related to the pathophysiology of the disease, clinical manifestations and more current diagnostic methods and possible therapeutic interversions to provide better stability to the picture.
Asunto(s)
Animales , Perros , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/veterinaria , Miocardio , Ecocardiografía/veterinariaRESUMEN
A Cardiomiopatia Dilatada Canina (CMD), enfermidade que afeta principalmente cães de porte grande macho e adulto, se caracteriza por uma dilatação miocárdica das câmaras cardíacas e diminuição de sua capacidade de contração, com consequentes alterações hemodinâmicas e posterior evolução para um quadro de insuficiência cardíaca congestiva. A ecocardiográfica tem sido cada vez mais considerada como diagnóstico definitivo para a CMD canina por ser um método dinâmico e não invasivo de avaliação do coração, que permite identificar a dilatação da parede ventricular. Esta revisão bibliográfica tem objetivo de revisar aspectos relacionados à fisiopatologia da doença, manifestações clínicas e métodos de diagnósticos mais atuais e possíveis interversões terapêuticas para proporcionar melhor estabilidade ao quadro.(AU)
Canine dilated Cardiomyopathy (CMD), a disease that affects mainly male and adult large-sized dogs, is characterized by myocardial dilation of the cardiac chambers and decreased contraction capacity, with consequent alterations Hemodynamic and posterior progression to a congestive heart failure condition. The echocardiographic has been increasingly considered as a definitive diagnosis for canine CMD because it is a dynamic and non-invasive method of assessing the heart, which allows to identify the dilation of the ventricular wall. This bibliographic review aims to review aspects related to the pathophysiology of the disease, clinical manifestations and more current diagnostic methods and possible therapeutic interversions to provide better stability to the picture.(AU)
Asunto(s)
Animales , Perros , Cardiomiopatía Dilatada/veterinaria , Miocardio , Cardiomiopatía Dilatada/fisiopatología , Ecocardiografía/veterinariaRESUMEN
Pediatric cardiomyopathies are infrequent diseases of the cardiac muscle, with an annual inciden ce of 1.1 to 1.2 per 100,000 children. Dilated cardiomyopathy (DCM) is the predominant form, characterized by ventricular dilatation and systolic dysfunction. Etiologies are multiple, with at least 50%-70% of cases being idiopathic. When assessing a child with DCM, secondary potentially reversible causes must be ruled out. The main diagnostic tool is the echocardiogram which allows the identification of cardiac phenotype, to establish the degree of functional compromise, and res ponse to medical therapy. Prognosis is limited but more favorable in infants younger than 1 year at the onset, post myocarditis, or with a lesser degree of ventricular dysfunction. At least 20% of patients may recover ventricular function in the first 2 years after the onset and 40%-50% may die or need heart transplant in the first 5 years. Medical therapy is mainly based on adult experience with limited scientific evidence in children. Heart transplant is the therapy of choice in patients with end-stage disease, with excellent short- and medium-term survival. A significant proportion of patients may require stabilization on the waiting list, including the use of mechanical circulatory support as a bridge to transplantation. The purpose of this revision is to update the available infor mation on etiology, physiopathological mechanisms, prognostic factors, and management of DCM in children.
Asunto(s)
Cardiomiopatía Dilatada/fisiopatología , Trasplante de Corazón , Factores de Edad , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/terapia , Niño , Preescolar , Ecocardiografía , Humanos , Lactante , Pronóstico , Listas de EsperaRESUMEN
BACKGROUND: Chagas cardiomyopathy (CDC) is associated with a poor prognosis compared to other cardiomyopathies. Speckle tracking echocardiography (STE), which provides direct assessment of myocardial fiber deformation, may be useful in predicting prognosis. OBJECTIVE: This study assessed STE in CDC and compared with idiopathic cardiomyopathy (IDC), and also examined the incremental prognostic information of STE over left ventricular ejection fraction (LVEF) in these patients. METHODS: We enrolled 112 patients, age of 56.7 ± 11.8 years, 81 with CDC and 31 with IDC. STE indices were obtained at baseline in all patients. The endpoint was a composite of death, hospitalization for heart failure, or need for heart transplantation. RESULTS: Patients with IDC had worse LV systolic function compared to CDC, with LVEF of 34.5% vs 41.3%, p = 0.004, respectively. After adjustment for LVEF, there were no differences in STE values between CDC and IDC. During a median follow-up of 18.2 months (range, 11 to 22), 26 patients met the composite end point (24%). LV longitudinal strain was a strong predictor of adverse events, incremental to LVEF and E/e' ratio (HR 1.463, 95% CI 1.130-1.894; p = 0.004). The risk of cardiac events increased significantly in patients with GLS > - 12% (log-rank p = 0.035). CONCLUSIONS: STE indices were abnormal in patients with dilated cardiomyopathy, without differences between CDC and IDC. LV longitudinal strain was a powerful predictor of outcome, adding prognostic information beyond that provided by LVEF and E/e' ratio.
Asunto(s)
Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Chagásica/diagnóstico por imagen , Insuficiencia Cardíaca/diagnóstico por imagen , Adulto , Anciano , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Chagásica/complicaciones , Cardiomiopatía Chagásica/mortalidad , Cardiomiopatía Chagásica/fisiopatología , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Trasplante de Corazón/estadística & datos numéricos , Hospitalización/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Volumen Sistólico/fisiología , Análisis de Supervivencia , Función Ventricular Izquierda/fisiologíaRESUMEN
The mechanism of sudden cardiac death (SCD) in patients with nonischemic dilated cardiomyopathy (NIDCM) is mostly due to sustained ventricular tachycardia and ventricular fibrillation. The clinical guidelines for the therapeutic management of this set of patients are mostly based on left ventricular ejection fraction value which has a low specificity to differentiate the risk of SCD from the risk of mortality associated with heart failure or other comorbidities. Moreover, since SCD can occur in patients with normal or mildly depressed ejection fraction, it is necessary to identify new markers to improve the prognostic stratification of SCD. Several studies that analyzed the ventricular arrhythmia substrate found that myocardial fibrosis plays an important role in the genesis of ventricular arrhythmias in patients with NIDCM. The surrounding zone of the area of fibrosis is a heterogeneous medium, where tissue with different levels of fibrosis coexists, resulting in both viable and nonviable myocardium. This myocardial fibrosis may constitute a substrate for ventricular arrhythmias, where slow and heterogeneous conduction may favor the genesis of reentry mechanism increasing the chance to develop sustained ventricular tachycardia or ventricular fibrillation. Therefore, the evaluation of ventricular fibrosis by late gadolinium enhancement (LGE) cardiac magnetic resonance imaging has been suggested as an indicator for SCD risk stratification. Indeed, LGE in patients with NIDCM is associated with increased risk of all-cause mortality, heart failure hospitalization, and SCD. Detection of myocardial fibrosis as LGE by cardiac magnetic resonance imaging can be considered as a useful pathway of prediction of malignant ventricular arrhythmias since it has excellent prognostic characteristics and may help guide risk stratification and management in patients with NIDCM.
Asunto(s)
Muerte Súbita Cardíaca/prevención & control , Imagen por Resonancia Cinemagnética/métodos , Miocardio/patología , Medición de Riesgo/métodos , Fibrilación Ventricular , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/fisiopatología , Muerte Súbita Cardíaca/etiología , Fibrosis , Humanos , Valor Predictivo de las Pruebas , Pronóstico , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/etiología , Fibrilación Ventricular/prevención & controlAsunto(s)
Arritmias Cardíacas/etiología , Arritmias Cardíacas/patología , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/patología , Adulto , Válvula Aórtica/anomalías , Válvula Aórtica/patología , Válvula Aórtica/fisiopatología , Arritmias Cardíacas/fisiopatología , Enfermedad de la Válvula Aórtica Bicúspide , Cardiomiopatía Dilatada/etiología , Cardiomiopatía Dilatada/patología , Cardiomiopatía Dilatada/fisiopatología , Progresión de la Enfermedad , Insuficiencia Cardíaca/fisiopatología , Trasplante de Corazón , Enfermedades de las Válvulas Cardíacas/patología , Enfermedades de las Válvulas Cardíacas/fisiopatología , Humanos , Masculino , Miocardio/patologíaRESUMEN
Yes-associated protein (YAP) is an important regulator of cellular proliferation and transdifferentiation. However, little is known about the mechanisms underlying myofibroblast transdifferentiation in dilated cardiomyopathy (DCM). We investigated the role of YAP in the pathological process of cardiac matrix remodeling. A classic model of DCM was established in BALB/c mice by immunization with porcine cardiac myosin. Cardiac fibroblasts were isolated from neonatal Sprague-Dawley rats by density gradient centrifugation. The expression levels of α-smooth muscle actin (α-SMA) and collagen volume fraction (CVF) were significantly increased in DCM mice. Angiotensin II (Ang II)-mediated YAP activation promoted the proliferation and transdifferentiation of neonatal rat cardiac fibroblasts, and this effect was significantly suppressed in the shRNA YAP + Ang II group compared with the shRNA Control + Ang II group in vitro (2.98±0.34 ×105 vs 5.52±0.82 ×105, P<0.01). Inhibition of endogenous Ang II-stimulated YAP improved the cardiac function by targeting myofibroblast transdifferentiation to attenuate matrix remodeling in vivo. In the valsartan group, left ventricular ejection fraction and fractional shortening were significantly increased compared with the DCM group (52.72±5.51% vs 44.46±3.01%, P<0.05; 34.84±3.85% vs 26.65±3.12%, P<0.01). Our study demonstrated that YAP was a regulator of cardiac myofibroblast differentiation, and regulation of YAP signaling pathway contributed to improve cardiac function of DCM mice, possibly in part by decreasing myofibroblast transdifferentiation to inhibit matrix remodeling.
Asunto(s)
Animales , Masculino , Ratas , Angiotensina II/farmacología , Cardiomiopatía Dilatada/fisiopatología , Proteínas Adaptadoras Transductoras de Señales/efectos de los fármacos , Transdiferenciación Celular/efectos de los fármacos , Miofibroblastos/efectos de los fármacos , Fosfoproteínas/antagonistas & inhibidores , Fosfoproteínas/fisiología , Porcinos , Ecocardiografía , Cardiomiopatía Dilatada/patología , Diferenciación Celular , Western Blotting , Ratas Sprague-Dawley , Proteínas de Ciclo Celular , Proteínas Adaptadoras Transductoras de Señales/antagonistas & inhibidores , Proteínas Adaptadoras Transductoras de Señales/fisiología , Modelos Animales de Enfermedad , Miofibroblastos/fisiología , Ratones Endogámicos BALB C , Microscopía FluorescenteRESUMEN
Yes-associated protein (YAP) is an important regulator of cellular proliferation and transdifferentiation. However, little is known about the mechanisms underlying myofibroblast transdifferentiation in dilated cardiomyopathy (DCM). We investigated the role of YAP in the pathological process of cardiac matrix remodeling. A classic model of DCM was established in BALB/c mice by immunization with porcine cardiac myosin. Cardiac fibroblasts were isolated from neonatal Sprague-Dawley rats by density gradient centrifugation. The expression levels of α-smooth muscle actin (α-SMA) and collagen volume fraction (CVF) were significantly increased in DCM mice. Angiotensin II (Ang II)-mediated YAP activation promoted the proliferation and transdifferentiation of neonatal rat cardiac fibroblasts, and this effect was significantly suppressed in the shRNA YAP + Ang II group compared with the shRNA Control + Ang II group in vitro (2.98±0.34 ×105 vs 5.52±0.82 ×105, P<0.01). Inhibition of endogenous Ang II-stimulated YAP improved the cardiac function by targeting myofibroblast transdifferentiation to attenuate matrix remodeling in vivo. In the valsartan group, left ventricular ejection fraction and fractional shortening were significantly increased compared with the DCM group (52.72±5.51% vs 44.46±3.01%, P<0.05; 34.84±3.85% vs 26.65±3.12%, P<0.01). Our study demonstrated that YAP was a regulator of cardiac myofibroblast differentiation, and regulation of YAP signaling pathway contributed to improve cardiac function of DCM mice, possibly in part by decreasing myofibroblast transdifferentiation to inhibit matrix remodeling.
Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/efectos de los fármacos , Angiotensina II/farmacología , Cardiomiopatía Dilatada/fisiopatología , Transdiferenciación Celular/efectos de los fármacos , Miofibroblastos/efectos de los fármacos , Fosfoproteínas/efectos de los fármacos , Proteínas Adaptadoras Transductoras de Señales/antagonistas & inhibidores , Proteínas Adaptadoras Transductoras de Señales/fisiología , Animales , Western Blotting , Cardiomiopatía Dilatada/patología , Proteínas de Ciclo Celular , Diferenciación Celular , Modelos Animales de Enfermedad , Ecocardiografía , Masculino , Ratones , Ratones Endogámicos BALB C , Microscopía Fluorescente , Miofibroblastos/fisiología , Fosfoproteínas/antagonistas & inhibidores , Fosfoproteínas/fisiología , Ratas , Ratas Sprague-Dawley , Porcinos , Proteínas Señalizadoras YAPRESUMEN
La miocardiopatía dilatada es la principal causa de insuficiencia cardíaca que lleva a trasplante cardíaco. Su pronóstico es variable y depende de la etiología, la edad de presentación y el grado de insuficiencia cardíaca. El manejo está orientado a minimizar los síntomas y evitar la progresión de la enfermedad; se requiere de una evaluación integral en la pesquisa de comorbilidades y prevención de complicaciones que permitan mejorar la condición general de estos niños y atenuar su pronóstico. A continuación, se realiza una revisión orientada al manejo multidisciplinario que el pediatra debería considerar a la hora de enfrentarse a este tipo de pacientes.
Dilated cardiomyopathy is the main cause of heart failure leading to heart transplant. Its prognosis is variable and depends on the etiology, the patient's age at onset, and the severity. The management of dilated cardiomyopathy is aimed at minimizing symptoms and preventing disease progression; it requires a comprehensive screening for comorbidities and the prevention of complications to improve the overall status of these children and mitigate their prognosis. Here we present a review oriented at the multidisciplinary management that pediatricians should consider when seeing these patients.
Asunto(s)
Humanos , Niño , Cardiomiopatía Dilatada/complicaciones , Insuficiencia Cardíaca/etiología , Pronóstico , Índice de Severidad de la Enfermedad , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/terapia , Tamizaje Masivo/métodos , Trasplante de Corazón/métodos , Edad de Inicio , Progresión de la Enfermedad , Pediatras , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/terapiaRESUMEN
Dilated cardiomyopathy is the main cause of heart failure leading to heart transplant. Its prognosis is variable and depends on the etiology, the patient's age at onset, and the severity. The management of dilated cardiomyopathy is aimed at minimizing symptoms and preventing disease progression; it requires a comprehensive screening for comorbidities and the prevention of complications to improve the overall status of these children and mitigate their prognosis. Here we present a review oriented at the multidisciplinary management that pediatricians should consider when seeing these patients.
La miocardiopatía dilatada es la principal causa de insuficiencia cardíaca que lleva a trasplante cardíaco. Su pronóstico es variable y depende de la etiología, la edad de presentación y el grado de insuficiencia cardíaca. El manejo está orientado a minimizar los síntomas y evitar la progresión de la enfermedad; se requiere de una evaluación integral en la pesquisa de comorbilidades y prevención de complicaciones que permitan mejorar la condición general de estos niños y atenuar su pronóstico. A continuación, se realiza una revisión orientada al manejo multidisciplinario que el pediatra debería considerar a la hora de enfrentarse a este tipo de pacientes.
Asunto(s)
Cardiomiopatía Dilatada/complicaciones , Insuficiencia Cardíaca/etiología , Pediatras/organización & administración , Edad de Inicio , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/terapia , Niño , Progresión de la Enfermedad , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/terapia , Trasplante de Corazón/métodos , Humanos , Tamizaje Masivo/métodos , Pronóstico , Índice de Severidad de la EnfermedadRESUMEN
Aims: Cardiac resynchronization therapy (CRT) is an established procedure for patients with heart failure. However, trials evaluating its efficacy did not include patients with chronic Chagas cardiomyopathy (CCC). We aimed to assess the role of CRT in a cohort of patients with CCC. Methods and results: This retrospective study compared the outcomes of CCC patients who underwent CRT with those of dilated (DCM) and ischaemic cardiomyopathies (ICM). The primary endpoint was all-cause mortality and the secondary endpoints were the rate of non-advanced New York Heart Association (NYHA) class 12 months after CRT and echocardiographic changes evaluated at least 6 months after CRT. There were 115 patients in the CCC group, 177 with DCM, and 134 with ICM. The annual mortality rates were 25.4%, 10.4%, and 11.3%, respectively (P < 0.001). Multivariate analysis adjusted for potential confounders showed that the CCC group had a two-fold [hazard ratio 2.34 (1.47-3.71), P < 0.001] higher risk of death compared to the DCM group. The rate of non-advanced NYHA class 12 months after CRT was significantly higher in non-CCC groups than in the CCC group (DCM 74.0% vs. ICM 73.9% vs. 56.5%, P < 0.001). Chronic Chagas cardiomyopathy and ICM patients had no improvement in the echocardiographic evaluation, but patients in the DCM group had an increase in left ventricular ejection fraction and a decrease in left ventricular end-diastolic diameter. Conclusion: This study showed that CCC patients submitted to CRT have worse prognosis compared to patients with DCM and ICM who undergo CRT. Studies comparing CCC patients with and without CRT are warranted.
Asunto(s)
Terapia de Resincronización Cardíaca , Cardiomiopatía Chagásica , Brasil/epidemiología , Terapia de Resincronización Cardíaca/efectos adversos , Terapia de Resincronización Cardíaca/métodos , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Chagásica/diagnóstico , Cardiomiopatía Chagásica/mortalidad , Cardiomiopatía Chagásica/fisiopatología , Cardiomiopatía Chagásica/terapia , Desfibriladores Implantables , Ecocardiografía/métodos , Femenino , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/terapia , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Volumen SistólicoRESUMEN
Objetivo: Avaliar o estado nutricional de pacientes com cardiomiopatia dilatada internados em Unidade de Terapia Intensiva (UTI) clínica de adultos de hospital especializado. Método: Pesquisa prospectiva e observacional, com avaliação subjetiva global (ASG), antropométrica (índice de massa corporal -IMC, prega cutânea tricipital -PCT, circunferência do braço -CB- e circunferência muscular do braço -CMB), bioquímica (proteínas totais, albumina, linfócitos, hemoglobina (Hb), lipídeos e BNP), impedância bioelétrica (IB) e desfechos obtidos. Foram aplicados testes t de Student, correlação de Pearson e Análise de Variância, adotando-se como significante p≤0,05. Resultados: Foram acompanhados 24 pacientes com miocardiopatia dilatada internados na UTI clínica de adultos, com idade média de 48,25±18,05 anos, sendo 62,5% (n=15) homens e 37,5% (n=9) idosos. Os valores de ângulo de fase (AF) variaram de 2,2 a 9,2º, com média de 5,33±1,66º, e verificou-se correlação estatística entre os valores reduzidos de AF e albumina (p=0,018). Em 41,7% da amostra (n=10), o valor do AF foi ≤ ao ponto de corte estipulado de 4,5º, esses com idade média de 46,3±19,26 anos, 50% homens e 40% idosos. Com base na ASG, 60% estavam desnutridos, por meio do IMC, 40% apresentavam baixo peso, e em relação a CB, CMB e PCT, 90%, 70% e 60%, respectivamente, estavam desnutridos. Com base nas dosagens, 80% estavam anêmicos, 100% apresentam hipoalbuminemia e 90% diminuição de colesterol total, sendo que desses, 60% foram a óbito. Conclusão: Pacientes estudados apresentaram desnutrição quando avaliados por ASG e medidas antropométricas, exceto IMC, principalmente naqueles com AF menor. Foi possível associarmos os valores reduzidos de AF ao pior estado nutricional, e não houve significância estatística entre AF e mortalidade e entre AF e demais variáveis, provavelmente devido ao número reduzido de pacientes que compunham a amostra.(AU)
Objective: To evaluate the nutritional status of patients with dilated cardiomyopathy admitted to an Intensive Care Unit (ICU) of a specialized hospital. Methods: Prospective and observational research, with global subjective assessment (SGA), anthropometric (body mass index - BMI, tricipital cutaneous fold - PCT, arm circumference - CB and arm muscle circumference - CMB), biochemistry (total proteins, Albumin, lymphocytes, hemoglobin (Hb), lipids and BNP), Bioelectrical Impedance (BI) and outcomes. Student's t-tests, Pearson's correlation and Variance Analysis were applied, with a significance level of p≤0.05. Results: Twenty-four patients with dilated cardiomyopathy were hospitalized in the adult clinical ICU, with a mean age of 48.25±18.05 years, 62.5% (n=15) men and 37.5% (n=9). Phase angle (PA) values ranged from 2.2 to 9.2º, with an average of 5.33±1.66º, and a statistical correlation was observed between the reduced values of PA and albumin (p=0.018). In 41.7% of the sample (n=10), the PA value was ≤ 4.5 s, with a mean age of 46.3±19.26 years, 50% men and 40% elderly. On the basis of SGA, 60% were malnourished, 40% were under weight, and 90%, 70% and 60%, respectively, weremalnourished. Based on the dosages, 80% wereanemic, 100% presented hypoalbuminemia and 90% decreased total cholesterol, of which, 60% died. Conclusion: Patients studied presented malnutrition when evaluated by ASG and anthropometric measurements, except for BMI, especially in those with lower PA. It was possible to associate the reduced values of PA to the worst nutritional status, and there was no statistical significance between PA and mortality and between PA and other variables, probably due to the small number of patients that composed the sample.(AU)
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Humanos , Cardiomiopatía Dilatada/fisiopatología , Estado Nutricional , Pacientes Internos , Unidades de Cuidados Intensivos , Antropometría/instrumentación , Estudios Prospectivos , Impedancia Eléctrica , Estudio ObservacionalRESUMEN
Cardiac ß-myosin variants cause hypertrophic (HCM) or dilated (DCM) cardiomyopathy by disrupting sarcomere contraction and relaxation. The locations of variants on isolated myosin head structures predict contractility effects but not the prominent relaxation and energetic deficits that characterize HCM. During relaxation, pairs of myosins form interacting-heads motif (IHM) structures that with other sarcomere proteins establish an energy-saving, super-relaxed (SRX) state. Using a human ß-cardiac myosin IHM quasi-atomic model, we defined interactions sites between adjacent myosin heads and associated protein partners, and then analyzed rare variants from 6112 HCM and 1315 DCM patients and 33,370 ExAC controls. HCM variants, 72% that changed electrostatic charges, disproportionately altered IHM interaction residues (expected 23%; HCM 54%, p=2.6×10-19; DCM 26%, p=0.66; controls 20%, p=0.23). HCM variant locations predict impaired IHM formation and stability, and attenuation of the SRX state - accounting for altered contractility, reduced diastolic relaxation, and increased energy consumption, that fully characterizes HCM pathogenesis.
Asunto(s)
Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Hipertrófica/fisiopatología , Miosinas Ventriculares/química , Miosinas Ventriculares/metabolismo , Humanos , Modelos Moleculares , Contracción Miocárdica , Unión ProteicaAsunto(s)
Cardiomiopatía Dilatada/patología , Cardiomiopatía Dilatada/fisiopatología , Insuficiencia Cardíaca/patología , Insuficiencia Cardíaca/fisiopatología , Adulto , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/etiología , Progresión de la Enfermedad , Ecocardiografía Transesofágica , Electrocardiografía , Resultado Fatal , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/etiología , Humanos , Masculino , Trastornos Relacionados con Sustancias/complicacionesAsunto(s)
Humanos , Masculino , Adulto , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/patología , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/patología , Cardiomiopatía Dilatada/etiología , Cardiomiopatía Dilatada/diagnóstico por imagen , Resultado Fatal , Ecocardiografía Transesofágica , Progresión de la Enfermedad , Trastornos Relacionados con Sustancias/complicaciones , Electrocardiografía , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/diagnóstico por imagenRESUMEN
Abstract Background: Amyloidosis is a disease caused by deposits of insoluble fibrils in extracellular spaces. The most common type of familial amyloidosis is mediated by mutation of transthyretin, especially Val30Met. Symptoms and ejection fraction decrease may occur in cardiac amyloidosis only in case of poor prognosis. Myocardial strain detected by two-dimensional speckle tracking echocardiography can indicate changes in myocardial function at early stages of the disease. Objective: To determine the accuracy of left ventricular longitudinal strain by two-dimensional speckle tracking echocardiography in patients with familial amyloidosis caused by Val30Met transthyretin mutation. Methods: Eighteen consecutive patients, carriers of transthyretin mutation, were evaluated by two-dimensional speckle tracking echocardiography, by which myocardial strain curves were obtained, following the American Society of Echocardiography recommendations. Results: Patients were divided into three groups: 1- Val30Met with cardiac amyloidosis; 2-Val30Met with extracardiac amyloidosis; 3 - Val30Met without evidence of disease. As the three groups were compared by the Mann-Whitney test, we found a statistically significant difference between groups 1 and 2 in the mean longitudinal tension (p=0.01), mean basal longitudinal strain (p=0.014); in mean longitudinal tension and mean longitudinal strain between groups 1 and 3 (p=0.005); and in the ratio of longitudinal strain of apical septum segment to longitudinal strain of basal septum (p=0.041) between groups 2 and 3. Conclusion: Left ventricular longitudinal strain detected by two-dimensional speckle tracking echocardiography is able to diagnose left ventricular dysfunction in early stages of familial amyloidosis caused by transthyretin Val30Met mutation.
Resumo Fundamento: A amiloidose é uma doença de depósito de fibrilas insolúveis nos espaços intercelulares. A forma mais comum de amiloidose familiar é mediada por mutação da transtirretina, sendo a Val30Met a mutação mais frequente. A amiloidose cardíaca só causa sintomas e queda da fração de ejeção em fases tardias quando o prognóstico é pobre. A deformação miocárdica obtida com speckle tracking bidimensional pode detectar alterações da função miocárdica em estágios precoces da doença. Objetivos: Determinar a acurácia da deformação longitudinal do ventrículo esquerdo obtida com speckle tracking bidimensional em um grupo de pacientes com amiloidose familial por mutação da transtirretina Val30Met. Métodos: Foram examinados 18 pacientes consecutivos com a mutação da transtirretina com speckle tracking bidimensional obtendo curvas de deformação miocárdica segundo normas da American Society of Echocardiography. Resultados: Os pacientes foram divididos em três grupos: 1- Val30Met com amiloidose cardíaca; 2- Val30Met com amiloidose extra-cardíaca; 3- Val30Met sem doença aparente. Ao compararmos os três grupos com o teste de Mann-Whitney encontramos diferença estatística significativa entre os grupos 1 e 2 na tensão longitudinal média (p=0,01), deformação longitudinal basal média (p=0,014); entre os grupos 1 e 3 na tensão longitudinal média (p=0,005), deformação longitudinal média (p=0,002); entre os grupos 2 e 3 na relação de deformação longitudinal do septo apical/deformação longitudinal do septo basal (p=0,041). Conclusão: A deformação longitudinal do ventrículo esquerdo obtida com speckle tracking bidimensional é capaz de diagnosticar disfunção do ventrículo esquerdo em fases precoces da amiloidose familial por mutação Val30Met da transtirretina.