Diferenças entre Duas Condições Cardíacas Hipertróficas Distintas: Doença de Fabry Versus Cardiomiopatia Hipertrófica / Differences Between Two Distinct Hypertrophic Cardiac Conditions: Fabry Disease versus Hypertrophic Cardiomyopathy

Resumo Fundamento A cardiomiopatia hipertrófica (CMH) e a doença de Fabry (DF) são doenças herdadas geneticamente com características fenotípicas de hipertrofia ventricular esquerda (HVE) que causam resultados cardíacos adversos. Objetivos Investigar as diferenças demográficas, clínicas, bioquímicas, eletrocardiográficas (ECG) e ecocardiográficas (ECO) entre CMH e DF. Métodos 60 pacientes com CMH e 40 pacientes com DF foram analisados retrospectivamente como uma subanálise do "estudo LVH-TR" após exclusão de pacientes com fibrilação atrial, ritmo de estimulação, bloqueios de ramo e bloqueios atrioventriculares (AV) de segundo e terceiro graus. O nível de significância foi aceito como <0,05. Resultados O sexo masculino (p=0,048) e a creatinina (p=0,010) são significativamente maiores a favor da DF; entretanto, infradesnivelamento do segmento ST (p=0,028), duração do QT (p=0,041), espessura do septo interventricular (SIVd) (p=0,003), espessura da parede posterior (PWd) (p=0,009), insuficiência mitral moderada a grave (IM) (p=0,013) e o índice de massa ventricular esquerda (IMVE) (p=0,041) são significativamente maiores a favor da CMH nas análises univariadas. Na análise multivariada, a significância estatística apenas permanece na creatinina (p=0,018) e na duração do intervalo QT (0,045). A DF foi positivamente correlacionada com a creatinina (rho=0,287, p=0,004) e a CMH foi positivamente correlacionada com o PWd (rho=0,306, p=0,002), IVSd (rho=0,395, p<0,001), IM moderada-grave (rho= 0,276, p<0,005), IMVE (rho=0,300, p=0,002), espessura relativa da parede (ERP) (rho=0,271, p=0,006), duração do QT (rho=0,213, p=0,034) e depressão do segmento ST (rho =0,222, p=0,026). Conclusão Características bioquímicas, ECG e ECO específicas podem auxiliar na diferenciação e no diagnóstico precoce da CMH e da DF.

Abstract Background Hypertrophic cardiomyopathy (HCM) and Fabry disease (FD) are genetically inherited diseases with left ventricular hypertrophy (LVH) phenotype characteristics that cause adverse cardiac outcomes. Objectives To investigate the demographic, clinical, biochemical, electrocardiographic (ECG), and echocardiographic (ECHO) differences between HCM and FD. Methods 60 HCM and 40 FD patients were analyzed retrospectively as a subanalysis of the 'LVH-TR study' after excluding patients with atrial fibrillation, pace rhythm, bundle branch blocks, and second and third-degree atrioventricular (AV) blocks. The significance level was accepted as <0.05. Results Male gender (p=0.048) and creatinine (p=0.010) are significantly higher in favor of FD; however, ST depression (p=0.028), QT duration (p=0.041), interventricular septum thickness (IVSd) (p=0.003), posterior wall thickness (PWd) (p=0.009), moderate-severe mitral regurgitation (MR) (p=0.013), and LV mass index (LVMI) (p=0.041) are significantly higher in favor of HCM in the univariate analyses. In multivariate analysis, statistical significance only continues in creatinine (p=0.018) and QT duration (0.045). FD was positively correlated with creatinine (rho=0.287, p=0.004) and HCM was positively correlated with PWd (rho=0.306, p=0.002), IVSd (rho=0.395, p<0.001), moderate-severe MR (rho=0.276, p<0.005), LVMI (rho=0.300, p=0.002), relative wall thickness (RWT) (rho=0.271, p=0.006), QT duration (rho=0.213, p=0.034) and ST depression (rho=0.222, p=0.026). Conclusion Specific biochemical, ECG, and ECHO characteristics can aid in the differentiation and early diagnosis of HCM and FD.

Comparison of Early and Mid-Term Outcomes After Classic and Modified Morrow Septal Myectomy in Patients with Hypertrophic Obstructive Cardiomyopathy

ABSTRACT Introduction: The aim of our study is to compare the early and mid-term outcomes of patients with hypertrophic obstructive cardiomyopathy who underwent classic and modified Morrow septal myectomy. Methods: Between 2014 and 2019, 48 patients (24 males; mean age 49.27±16.41 years) who underwent septal myectomy were evaluated. The patients were divided into two groups - those who underwent classic septal myectomy (n=28) and those who underwent modified septal myectomy (n=20). Results: Mitral valve intervention was higher in the classic Morrow group than in the modified Morrow group, but there was no significant difference (P=0.42). Mortality was found to be lower in the modified Morrow group than in the classic Morrow group (P=0.01). In both groups, the mean immediate postoperative gradient was significantly higher than the mean of the 3rd and 12th postoperative months. The preoperative and postoperative gradient difference of the modified Morrow group was significantly higher than of the classic Morrow group (P<0.001). Conclusion: Classic Morrow and modified Morrow procedures are effective methods for reducing left ventricular outflow tract obstruction. The modified Morrow procedure was found to be superior to the classic Morrow procedure in terms of reducing the incidence of mitral valve intervention with the reduction of the left ventricular outflow tract gradient.

Cardiomyopathie du péripartum: aspects cliniques, thérapeutiques et évolutifs à propos de 24 cas / Clinical features, management and outcome of peri-partum cardiomyopathy: a study of 24 patients

Introduction. La fréquence, la présentation et le devenir de cardiomyopathie du peripartum (CMPP) varient considérablement dans le monde. Au Niger, les données sont inexistantes d'où le but de notre étude qui était de préciser les aspects épidémiologiques, diagnostiques, thérapeutiques et évolutifs de la cardiomyopathie du peripartum (CMPP). Méthodologie. Il s'agit d'une étude rétrospective concernant les patientes hospitalisées pour CMPPau pôle de Cardiologie de l'Hôpital National de Niamey du 01/01/2019 au 31/12/2019. Les facteurs de risque et les aspects épidémiologiques, cliniques, paracliniques, diagnostiques, thérapeutiques et évolutifs ont été étudiés. Résultats. La prévalence hospitalière de la CMPP était de 3,83%. L'âge moyen était de 27,4 ans (extrêmes de 16 et 45 ans). La multiparité a été rapportée chez 79 % des patientes contre 21% qui étaient primipares. La dyspnée était le signe le signe fonctionnel rapporté chez toutes les patientes. Les anomalies échocardiographiques les plus fréquentes étaient l'HVG (75%) et L'HAG (45,83%). L'hypocinésie était diffuse dans 100% des cas. L'altération de la fraction d'éjection du ventricule du ventricule gauche (FEVG) était moyenne dans 50%. Toutes les patientes ont ont eu comme traitement des bétabloquants, des diurétiques et des IEC. L'évolution a été favorable dans 87,5 % des cas. Les principaux facteurs pronostiques péjoratifs étaient la multiparité (79%) une fraction d'éjection du ventricule inférieure à (FEVG)

Introduction. The frequency, presentation and outcome of peripartum cardiomyopathy (PCM) vary considerably throughout the world. In Niger, data are non-existent, hence the aim of our study, which was to clarify the epidemiological, diagnostic, therapeutic and evolutionary aspects of peripartum cardiomyopathy (PPCM). Methodology. This is a retrospective study concerning patients hospitalized for CMPPat the Cardiology pole of the National Hospital of Niamey from 01/01/2019 to 31/12/2019. Risk factors and epidemiological, clinical, paraclinical, diagnostic, therapeutic and evolutionary aspects were studied. Results. The hospital prevalence of CMPP was 3.83%. The mean age was 27.4 years (extremes 16 and 45 years). Multiparity was reported in 79% of patients versus 21% who were primiparous. Dyspnea was the functional sign reported in all patients. The most frequent echocardiographic abnormalities were LVH (75%) and GAH (45.83%). Hypokinesia was diffuse in 100% of cases. Impaired left ventricular ejection fraction (LVEF) was moderate in 50%. All patients were treated with beta-blockers, diuretics and ACE inhibitors. Progression was favorable in 87.5% of cases. The main pejorative prognostic factors were multiparity (79%) lower ventricular ejection fraction (LVEF)

Signaling Pathways Related to Dilated Cardiomyopathy and Their Regulation by Traditional Chinese Medicine： A Review / 中国实验方剂学杂志

Dilated cardiomyopathy （DCM） is characterized by the main pathological changes of global cardiac enlargement， especially left ventricular enlargement. Clinical manifestations include decreased heart function， arrhythmia， thromboembolism， and even sudden death. It is one of the refractory cardiovascular diseases. Conservative drug treatment is still the main approach in clinical practice， but due to its unavoidable side effects such as low blood pressure， it is often difficult to achieve a satisfactory prognosis. The combination of traditional Chinese medicine and Western medicine can effectively improve side effects and enhance efficacy. The research has found that nuclear transcription factors-κB （NF-κB）， adenylate activated protein kinase （AMPK）/mammalian rapamycin target protein （mTOR）， transforming growth factor-β （TGF-β）/Smads， Toll like receptors （TLR） 4/c-Jun amino terminal kinase （JNK）， mitogen activated protein kinase （MAPK）， phosphatidylinositol 3-kinase （PI3K）/protein kinase （Akt）， and other signaling pathways play a crucial regulatory role in the occurrence and development of DCM. Traditional Chinese medicine can improve myocardial fibrosis， reverse ventricular remodeling， alleviate oxidative stress， and achieve anti-inflammatory and other effects by regulating the above signaling pathways， thus improving DCM. Due to its multi-target and multi-mechanism characteristics， it has the advantages of high safety and good tolerance and has become an important part of current clinical treatment.

Heart transplantation in patients with anthracycline-induced cardiomyopathy / Trasplante cardiaco en pacientes con cardiotoxicidad inducida por antraciclinas

Abstract Objective: The objective of this study was to describe the clinical and imaging characteristics and the evolution of heart transplantation patients due to anthracycline-induced cardiomyopathy Methods: Patients with a diagnosis of ACM who received a heart transplantation in our institution in the period of November 2009-April 2021 were included. Clinical characteristics, pre-transplant studies, and clinical outcomes after transplantation were collected retrospectively from the electronic medical record. Results: A total of 11 patients were included in the study. The median age at the time of cancer diagnosis was 15 years (IQR 10-37 years), while the median age at the time of heart transplant was 56 years (IQR 39-62 years). Regarding post-transplant outcomes, three patients died in the post-operative period. One died 4 years after the intervention due to chronic rejection, while the other seven had a favorable evolution. No oncological relapse was observed with a median follow-up of 2.5 years (IQR 1.86-3.85 years). Conclusion: End-stage anthracycline-induced cardiomyopathy can occur many years after chemotherapy treatment, so close cardiovascular follow-up is extremely important. Heart transplantation is a treatment option after an exhaustive multidisciplinary evaluation, to minimize the risk of oncological relapse.

Resumen Objetivo: Describir las características clínicas, imagenológicas y la evolución de los pacientes trasplantados cardiacos por cardiotoxicidad inducida por antraciclinas. Métodos: Serie de casos descriptiva de pacientes consecutivos trasplantados cardiacos debido a cardiotoxicidad mediada por antraciclinas en el periodo de Noviembre de 2009 a Abril de 2021.Las características clínicas, los estudios complementarios pretrasplante y la información sobre la evolución posterior al trasplante fue recolectada de la historia clínica electrónica de forma retrospectiva. Resultados: Se incluyeron un total de 11 pacientes. La mediana de edad al diagnóstico de la patología oncológica fue de 15 años (RIC 10-37 años), mientras que la mediana de edad en la que recibieron el trasplante cardiaco fue de 56 años (RIC 39-62 años). Con respecto a la evolución posterior al trasplante, 3 pacientes murieron en el periodo del post operatorio inmediato. 1 paciente falleció a los 4 años del trasplante y los otros 7 pacientes tuvieron una evolución favorable. No se observó recaída oncológica en ningún paciente durante una mediana de seguimiento o de 2,5 años (RIC 1.86-3.85 años). Conclusión: La etapa final de la miocardiopatía inducida por antraciclinas puede ocurrir muchos años después del tratamiento con quimioterapia, por lo que es extremadamente importante un seguimiento cardiológico estricto. El trasplante cardiaco es una opción en este grupo de pacientes luego de una exhaustiva evaluación multidisciplinaria, con el fin de minimizar el riesgo de recaída oncológica.

Trasplante cardíaco y autólogo de células progenitoras hematopoyéticas en pacientes con amiloidosis AL / Cardiac and autologous stem cell transplantation hematopoietic in patients with AL amyloidosis

Resumen Objetivo: Describir la evolución de las cadenas livianas libres séricas (CLL) en el período comprendido entre el trasplante cardíaco ortotópico (TCO) y el trasplante de células progenitoras hematopoyéticas (TCPH), la respuesta hematológica al año tras el TCPH y el tratamiento quimioterápico e inmunosupresor en pacientes con amiloidosis AL. Método: Serie de casos de pacientes consecutivos con diagnóstico de amiloidosis AL que recibieron TCO seguido de TCPH del Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, entre enero de 2010 y noviembre de 2021. Se reportaron los valores de CLL entre trasplantes y al año del TCPH. Las variables cuantitativas se describieron como mediana e intervalo intercuartil, y las variables categóricas como frecuencias absolutas y relativas. Resultados: De 106 pacientes con amiloidosis AL, seis tuvieron TCO seguido de TCPH. La mediana de edad fue de 55 años. La mayoría eran hombres (n = 5). En el período entre trasplantes, la CLL involucrada disminuyó en dos pacientes y se mantuvo estable en tres. Todos lograron la remisión hematológica completa al año del TCPH. Un solo paciente presentó recaída en el órgano sólido trasplantado. Tacrolimus, micofenolato de mofetilo y corticoides fue el esquema inmunosupresor utilizado después del TCO. Conclusiones: El TCO representa una opción de tratamiento en pacientes con falla cardíaca grave por amiloidosis, permitiendo luego un tratamiento intensivo con quimioterapia de inducción y TCPH. Si bien faltan estudios, la terapia inmunosupresora después del TCO podría tener algún efecto sobre las células plasmáticas clonales.

Abstract Objective: To describe the evolution of serum free light chains (FLC) in the period between orthotopic heart transplantation (OHT) and autologous stem cell transplantation (ASCT), the hematological response one year after ASCT and chemotherapy and immunosuppressive treatment in patients with AL amyloidosis. Method: Case series of consecutive patients diagnosed with AL amyloidosis who received OHT followed by ASCT from the Institutional Registry of Amyloidosis of the Italian Hospital of Buenos Aires, between January 2010 and November 2021. FLC values between transplants and at year post ASCT. Quantitative variables were described with their median and interquartile range. Categorical variables as absolute and relative frequencies. Results: Of 106 patients with AL amyloidosis, 6 had an OHT followed by ASCT. The median age was 55 years. Most were men (n = 5). In the period between transplants, the involved CLL decreased in two patients and remained stable in three. All achieved complete hematologic remission 1 year after ASCT. A single patient presented relapse in the transplanted solid organ. Tacrolimus, mycophenolate mofetil, and corticosteroids were the immunosuppressive regimen used after OHT. Conclusions: OHT represents a treatment option in patients with severe heart failure due to amyloidosis, allowing later intensive treatment with induction chemotherapy and ASCT. Although studies are lacking, immunosuppressive therapy after OHT might have some effect on clonal plasma cells.

Desfibrilador subcutáneo. Primeros casos en el Instituto Nacional de Cardiología, incluyendo dos pacientes pediátricos / Use of subcutaneous cardioverter-defibrillator. First cases reported from National Institute of Cardiology Ignacio Chávez-Mexico

Resumen Introducción: El desfibrilador automático implantable (DAI) transvenoso es el tratamiento de elección para la prevención de muerte súbita (MS) cardiaca por arritmias ventriculares malignas. Su uso se puede ver limitado cuando existe infección del sistema de estimulación o en población pediátrica donde representa un reto por diversas razones, incluyendo: las mínimas dimensiones del sistema venoso de los niños, la longitud de los electrodos, el tamaño del generador, así como por la complejidad anatómica en casos de cardiopatía congénita asociada. Objetivo: El presente artículo tiene por objetivo presentar la primera serie de casos de pacientes mexicanos a los cuales se les implantó un DAI subcutáneo (DAI-SC) como terapia para la prevención de MS. Métodos: Se presentan los cuatro primeros casos que fueron implantados en el Instituto Nacional de Cardiología Ignacio Chávez con un DAI-SC (Emblem, Boston Scientific, EE.UU.), tres de ellos eran pacientes pediátricos, incluyendo el primer implante de este tipo de dispositivo en un paciente pediátrico en América Latina. Las técnicas de tres y dos incisiones fueron empleadas bajo anestesia general. Resultados: Se realizó el implante exitoso con técnica de tres incisiones en los dos primeros casos y con técnica de dos incisiones en los dos últimos casos. Se corroboró el adecuado funcionamiento del dispositivo en sala, con la verificación de terapia apropiada (65 J) de la fibrilación ventricular inducida mediante estimulación a 50 Hz. No hubo complicaciones inmediatas. Un paciente presentó descargas apropiadas a los dos meses del implante. En el seguimiento, un niño desarrolló erosión de la piel a nivel de la curva del electrodo en el esternón, sin datos de infección. En quirófano se resecó la piel dañada, se retiró el barril y la seda de fijación, se realizó lavado quirúrgico y se volvió a cerrar la piel, logrando así evitar el retiro del sistema. Conclusiones: El DAI-SC es una terapia alternativa al DAI endovenoso y puede ser considerado de primera elección en aquellos casos que no requieran de estimulación ventricular, incluyendo pacientes pediátricos. Pueden ocurrir complicaciones cutáneas, pero no representan una amenaza como las complicaciones venosas de los DAI convencionales.

Abstract Introduction: The transvenous implantable cardioverter defibrillator (ICD) is the treatment of choice for the prevention of sudden cardiac death (SCD). Its use could be restricted when device-related infections occurs or in the pediatric population. In the later, an ICD represents a challenge, due to the minimal dimensions of the venous system in children, the length of the electrodes, the size of the generator, as well as the anatomical complexity in cases with associated congenital heart disease. Objective: This article presents the first Mexican patients with a subcutaneous ICD (SC-ICD) implant as a therapy for the prevention of SCD. Methods: The first four cases were implanted at the Ignacio Chávez National Institute of Cardiology with a SC-ICD (Emblem, Boston Scientific, USA), three of them were pediatric patients, including the first implant of this type of device in a pediatric patient in Latin America. The 3-incision and 2-incision techniques were used under general anesthesia. Results: A successful implantation was obtained with the 3-incision technique in the first 2 cases and the last 2 with the 2-incision technique. Proper functioning of the device was corroborated in the operating room with proof of appropriate therapy (65 J) for ventricular fibrillation induced with 50 Hz stimulation. No immediate complications were observed. One patient had appropriate shocks two months after the implant. During follow-up, one child developed skin erosion at the level of the curve of the electrode on the sternum, with no signs of infection. In the operating room, the damaged skin was resected, the barrel and the fixation silk were removed, surgical lavage was performed, and the skin was closed again, thus avoiding removal of the system. Conclusions: The SC-ICD is an alternative therapy to the transvenous ICD. It can be considered first choice in subjects who do not require ventricular pacing, including pediatric patients. Skin complications can occur but do not pose a threat as venous complications of conventional ICDs.

Infección por dengue sin signos de alarma en un niño con cardiopatía chagásica crónica

Se presenta el caso de un preescolar en que se diagnosticó una cardiomiopatía chagásica crónica posterior a una infección aguda por virus dengue. El niño, residente de un área endémica para enfermedad de Chagas, debutó con insuficiencia cardíaca que mejoró con el tratamiento de soporte y tras la resolución completa de la arbovirosis, persistieron los cambios de cardiomiopatía crónica. Esta evidencia de alteración estructural miocárdica se atribuyó a la cardiomiopatía chagásica de base previamente subclínica. La coexistencia de enfermedades infecciosas en el trópico representa un reto diagnóstico, situación a la que se agregan problemas socioeconómicos como la pobreza y falta de acceso a servicios sanitarios básicos.

A case is reported of a chronic chagasic cardiomyopathy in a two-years old child who was diagnosed after an acute dengue virus infection. The patient residing in an endemic area for Chagas disease, debuted with heart failure that improved with support treatment and after the complete resolution of the arbovirus, the changes of chronic cardiomyopathy persisted. The myocardial structural alteration was attributed to subclinical chagasic cardiomyopathy. The coexistence of infectious diseases in the tropics represents a diagnostic challenge, a situation to which are added problems such as poverty and lack of access to basic health services.

Trasplante cardíaco por hemocromatosis. Caso pediátrico / Cardiac transplantation due to hemochromatosis. A pediatric case report

La hemocromatosis es una enfermedad caracterizada por el excesivo depósito de hierro en múltiples órganos, entre ellos hígado, páncreas, piel y corazón. La infiltración de este último es un importante factor en morbilidad y mortalidad. Presentamos un caso de un paciente pediátrico con insuficiencia cardíaca terminal que ameritó trasplante cardíaco, que resultó sin complicaciones. Posterior a la cirugía, mostró mejoría bioquímica y clínica, lo que influyó positivamente en su calidad de vida y prolongó su supervivencia.

Hemochromatosis is a disease characterized by excess iron stores in multiple organs, including the liver, pancreas, skin, and heart. The infiltration of the heart is an important factor in morbidity and mortality. Here we describe the case of a pediatric patient with end-stage heart failure who required a heart transplantation, with no complications. After the surgery, she showed biochemical and clinical improvement, with a positive impact on her quality of life and a prolonged survival.

Miocardiopatía hipertrófica en la edad pediátrica. Revisión a propósito de una serie de casos

Introducción: La miocardiopatía hipertrófica (MCH) constituye una enfermedad compleja y variable en cuanto a morfología, fisiopatología, pronóstico y sobrevida. Se caracteriza por una hipertrofia del ventrículo izquierdo, aunque en ocasiones puede ser biventricular o ventricular derecha aislada. En la edad pediátrica predominan las causas no sarcoméricas. La ecocardiografía es el método diagnóstico más utilizado para caracterizar la morfología y repercusión hemodinámica, sin embargo, la resonancia magnética cardíaca (RMC) es de elección por aportar mayores ventajas morfológicas y funcionales. Las pruebas genéticas son de gran importancia en la determinación del diagnóstico etiológico, manejo terapéutico y pronóstico. El tratamiento tiene como objetivo principal mejorar la capacidad funcional y aliviar los síntomas. Los betabloqueantes no vasodilatadores son considerados de primera líneay en algunos casos se puede recurrir a terapias avanzadas como disopiramida o terapia de reducción septal (TRS), uso del cardiodesfibrilador implantable (CDI), y en ocasiones se debe considerar trasplante cardiaco en pacientes con síntomas refractarios al tratamiento médico y en MCH del tipo obstructivo. Materiales y métodos: Estudio observacional, descriptivo, corte transversal. Resultados: Se estudiaron 12 pacientes, desde enero de 2020 a diciembre de 2022, con edades comprendidas desde 5 meses hasta 16 años, diagnosticados con MCH de presentación clínica variable y de distintas etiologías. Conclusión: La MCH de causas no sarcoméricas como los síndromes malformativos y errores innatos del metabolismo son las que tienen peor pronóstico y sobrevida. La finalidad de detectar precozmente la MCH es el inicio temprano de la terapia específica, para así retrasar el compromiso cardiovascular y mejorar la sobrevida.

Introduction: Hypertrophic cardiomyopathy (HCM) is a complex and variable disease in terms of morphology, pathophysiology, prognosis and survival. It is characterized by left ventricular hypertrophy, although it can sometimes be biventricular or isolated right ventricular. In the pediatric population, non-sarcomeric causes predominate. Echocardiography is the most widely used diagnostic method to characterize morphology and hemodynamic repercussions; however, cardiac magnetic resonance imaging (CMR) is preferred because it provides greater morphological and functional advantages. Genetic tests are of great importance in determining the etiological diagnosis, therapeutic management and prognosis. The main objective of the treatment is to improve functional capacity and alleviate symptoms. Non-vasodilator beta-blockers are considered first-line treatment and in some cases advanced therapies such as disopyramide or septal reduction therapy (SRT), use of implantable cardioverter-defibrillator (ICD) can be used; heart transplantation should be considered in patients with symptoms refractory to medical treatment and in HCM of the obstructive type. Materials and methods: This was an observational, descriptive, cross-sectional study. Results: 12 patients were studied, from January 2020 to December 2022, with ages ranging from 5 months to 16 years, diagnosed with HCM of variable clinical presentation and of different etiologies. Conclusion: HCM from non-sarcomeric causes such as malformation syndromes and inborn errors of metabolism have the worst prognosis and survival. The purpose of early detection of HCM is the early initiation of specific therapy, in order to delay cardiovascular compromise and improve survival.

Métodos diagnósticos cardiovasculares en pacientes con diabetes mellitus. Revisión / Cardiovascular diagnostic methods in patients with diabetes mellitus. A review

Resumen Las enfermedades cardiovasculares constituyen la causa más común de mortalidad en el mundo. Actualmente, la diabetes mellitus tipo 2 (DM2) representa uno de los principales factores de riesgo de eventos adversos cardiovasculares mayores. Los pacientes que las padecen tienen un riesgo cuatro veces mayor de desarrollar insuficiencia cardíaca y una mortalidad de 10 a 12 veces mayor. La ecocardiografía en todas sus modalidades es la mejor herramienta clínica para el diagnóstico de la insuficiencia cardíaca, ya que proporciona imágenes estáticas y dinámicas del corazón que permiten identificar cambios estructurales y funcionales, como alteraciones en las presiones, cambios de flujo, fracción de expulsión del ventrículo izquierdo y remodelación anatómica de las superficies miocárdicas.

Abstract Cardiovascular diseases are the most common cause of mortality in the world. Currently, type 2 diabetes mellitus (T2DM) is one of the main risk factors for major adverse cardiovascular events. T2DM patients have a four-fold higher risk of developing heart failure and 10 to 12 times higher mortality. Echocardiography in all its modalities is the best clinical tool for heart failure diagnosis, since it provides static and dynamic images of the heart that allow to identify structural and functional changes, such as pressure variations, flow changes, left ventricular ejection fraction and myocardial surfaces anatomical remodeling.

Mecanismos y dianas terapéuticas del inflamasoma NLRP3 en la miocardiopatía diabética / NLRP3 inflammasome mechanism and therapeutic targets in diabetic cardiomyopathy

Resumen La cardiomiopatía diabética es una complicación grave de la diabetes causada por estrés oxidativo, inflamación, resistencia a la insulina, fibrosis miocárdica y lipotoxicidad. Se trata de un padecimiento insidioso, complejo y difícil de tratar. El inflamasoma NLRP3 desencadena la maduración y liberación de citoquinas proinflamatorias, participa en procesos fisiopatológicos como la resistencia a la insulina y la fibrosis miocárdica, además de estar estrechamente relacionado con la aparición y progresión de la cardiomiopatía diabética. El desarrollo de inhibidores dirigidos a aspectos específicos de la inflamación sugiere que el inflamasoma NLRP3 puede utilizarse para tratar la cardiomiopatía diabética. Este artículo pretende resumir el mecanismo y las dianas terapéuticas del inflamasoma NLRP3 en la cardiomiopatía diabética, así como aportar nuevas sugerencias para el tratamiento de esta enfermedad.

Abstract Diabetic cardiomyopathy (DCM) is a serious complication of diabetes caused by oxidative stress, inflammation, insulin resistance, myocardial fibrosis, and lipotoxicity; its nature is insidious, complex and difficult to treat. NLRP3 inflammasome triggers the maturation and release of pro-inflammatory cytokines, participates in pathophysiological processes such as insulin resistance and myocardial fibrosis, in addition to being closely related to the development and progression of diabetic cardiomyopathy. The development of inhibitors targeting specific aspects of inflammation suggests that NLRP3 inflammasome can be used to treat diabetic cardiomyopathy. This paper aims to summarize NLRP3 inflammasome mechanism and therapeutic targets in diabetic cardiomyopathy, and to provide new suggestions for the treatment of this disease.

Síndrome de tako-tsubo en una adolescente. Presentación de caso

El síndrome de tako-tsubo es una miocardiopatía aguda por estrés, caracterizada por una anormalidad regional del movimiento de la pared del ventrículo izquierdo, con un patrón circunferencial peculiar que resulta en un abultamiento notorio del ventrículo izquierdo durante la sístole. En Cuba, el síndrome de tako-tsubo ha sido publicado en pacientes adultos, pero se considera una entidad rara en niños, razón por la cual se decidió hacer este reporte. Se presenta el caso de una adolescente de 16 años, con antecedentes de haber dado a luz hacía 44 días al momento del ingreso, que fue recibida en el Servicio de Emergencias en estado grave, con disnea, dolor precordial y manifestaciones de choque, por lo que fue derivada inmediatamente a la Unidad de Cuidados Intensivos Pediátricos. Los antecedentes de haber tenido un factor desencadenante de estrés como el parto por cesárea, el electrocardiograma con signos de isquemia miocárdica y el ecocardiograma típico, con discinesia de segmentos apicales del ventrículo izquierdo, permitieron hacer el diagnóstico de síndrome de tako-tsubo y dirigir el tratamiento en ese sentido. Este síndrome es infrecuente en la edad pediátrica, por tanto, puede presentarse como una forma grave y no ser reconocido, trayendo implicaciones pronósticas.

Takotsubo syndrome is an acute stress cardiomyopathy, characterized by a regional abnormality of left ventricular wall motion, with a peculiar circumferential pattern that results in marked left ventricular bulging during systole. In Cuba, takotsubo syndrome has been published in adult patients, but it is considered a rare entity in children, so it was decided to make this report. The case of a 16-years-old adolescent, with a history of having given birth 44 days before at the time of admission, who was received at the Emergency Department in serious condition, with dyspnea, precordial pain, and manifestations of shock, for what she was immediately referred to the Pediatric Intensive Care Unit it is presented. The history of having had a stress trigger such as delivery by cesarean section, the electrocardiogram with signs of myocardial ischemia, and the typical echocardiogram, with dyskinesia of the apical segments of the left ventricle, allowed the diagnosis of takotsubo syndrome and direct treatment in that sense. This syndrome is rare in the pediatric age, therefore, it can present as a severe form and not be recognized, bringing prognostic implications.

Echocardiographic and Etiopathogenic Features of Hypertrophic Cardiomyopathy: Casablanca University Hospital Experience

Hypertrophic cardiomyopathy (HCM) is the most common non-ischemic cardiomyopathy with a prevalence of 1:500 in the general population, based on the recognition of the phenotype. HCM is defined by the presence of increased left ventricular (LV) wall thickness that is not solely explained by abnormal loading conditions and the phenotype also includes disorganized myocyte arrangement, fibrosis, small-vessel disease, and abnormalities of the mitral valve apparatus. In particular to this pathology, we have conducted a one-year prospective study to determine clinical, echocardiographic features and etiopathogenic aspects of hypertrophic cardiomyopathy in the Casablanca university hospital. The results concluded that 50% of the causes was due to amyloidosis 35%, sarcomeric HCM and 15% Fabry disease in which 2 cases were related with pregnancy. Transthoracic echocardiography and cardia MRI plays an important role in HCM diagnosis and prognosis.

Left Ventricular Non-Compaction Cardiomyopathy: Case Report and Review of Literature

Non-compaction cardiomyopathy (NCC) is characterized by trabeculations in either one or both ventricles. Clinical presentation is highly variable: dyspnea, palpitation, thromboembolic events, arrhythmia, or sudden cardiac death. There are currently no universally-accepted criteria for classifying and diagnosing left ventricular non-compaction (LVNC) cardiomyopathy. Transthoracic echocardiography (TTE) is the diagnostic exam of choice. The diagnosis is often missed or delayed because of a lack of knowledge about this uncommon disease. Progression of LVNC is highly variable and prognosis is very difficult to predict. We report a case of a 50-year-old female patient with a history of total thyroidectomy under hormonal supplementation who consults for dyspnea and paroxysmal palpitations revealing an isolated LVNC. This case emphasizes the importance of imaging techniques, which are, TTE and cardiac magnetic resonance imaging (MRI) in early diagnosis, management, and follow-up.

Challenges In The Anesthetic Management Of Peripartum Cardiomyopathy Undergoing Emergency Ceserean Section

Peripartum cardiomyopathy (PPCM) is an unusual form of dilated cardiomyopathy which manifests as acute heart failure in the last trimester of pregnancy or early postpartum period. Choice of anaesthesia is based on the urgency of lower segment caesarean section and severity of PPCM. Here we report a 31year old female G3P2L2 36weeks + 2days diagnosed as Peripartum Cardiomyopathy came with complaints of leaking per vagina was taken up for emergency caesarean section under general anaesthesia. In this case report, we discuss the anaesthetic management of a case of PPCM posted for emergency caesarean section with intra-operative event of desaturation. Anaesthetic management was directed towards optimization of desaturation, myocardial contractility, preload and after load. No adverse events or complications were observed.

Como eu faço no Coração de Atleta: avaliação dos diferentes tipos de adaptação ao exercício / My approach to "Athlete'S Heart": evaluation of the different types of adaptation to exercise

A prática regular de esportes pode induzir adaptações no coração, sendo essa condição comumente chamada de "coração de atleta". As alterações observadas incluem dilatação das câmaras cardíacas, aumento da espessura miocárdica, melhora do enchimento ventricular, aumento da trabeculação do ventrículo esquerdo (VE), dilatação da veia cava inferior, entre outras. Essas alterações também podem ser observadas em algumas doenças cardíacas, como cardiomiopatia (CMP) dilatada, hipertrófica e outras. Dessa forma, os exames de imagem cardíaca são fundamentais na identificação dessas alterações e na diferenciação entre o "coração de atleta" e uma possível cardiopatia.(AU)

Exercise-induced adaptation may occur in amateur and professional athletes. This condition is commonly named "athlete's heart". The alterations observed include dilation of the heart chambers, increased myocardial thickness, improved ventricular filling, increased left ventricular trabeculation, dilation of the inferior vena cava, among others. These changes can also be observed in some heart diseases, such as dilated, hypertrophic and other cardiomyopathies (CMP). Thus, cardiac imaging tests are fundamental in identifying these alterations and in differentiating between "athlete's heart" and possible heart disease. (AU)