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)

Rehabilitación cardíaca integral en un caso de paciente con miocardiopatía por SARS-COV-2 / Comprehensive Cardiac Rehabilitation in a Case of a Patient with SARS-CoV-2 Cardiomyopathy

Introducción: Una complicación reportada en la infección por SARS-CoV-2 es la miocardiopatía. Se ha descrito que los pacientes pueden presentar un deterioro significativo de la clase funcional, que limita el desempeño en las actividades de la vida diaria, sociales, familiares y laborales. La rehabilitación cardíaca como componente integral es una alternativa de tratamiento no farmacológica en el manejo de secuelas cardiovasculares ocasionadas por SARS-CoV-2. Objetivo: Describir el impacto de la rehabilitación cardíaca integral en un caso de paciente con miocardiopatía por SARS-CoV-2. Caso clínico: Se presenta el caso de un hombre de 56 años de edad con miocardiopatía por SARS-CoV-2 quien realizó rehabilitación cardíaca integral. Conclusiones: La rehabilitación cardíaca con un componente de intervención integral multidisciplinar permitió al paciente mejorar la capacidad aeróbica, sintomatología, calidad de vida relacionada con la salud y reincorporarse a las actividades sociales, familiares y laborales(AU)

Introduction: A reported complication of SARS-CoV-2 infection is cardiomyopathy. It has been described that patients may present with significant impairment of functional class, limiting performance in activities of daily living, social, family and work. Cardiac rehabilitation as an integral component is a non-pharmacological treatment alternative in the management of cardiovascular sequelae caused by SARS-CoV-2. Objective: To describe the impact of comprehensive cardiac rehabilitation in a case of a patient with cardiomyopathy due to SARS-CoV-2. Case report: We present the case of a 56-year-old patient with SARS-CoV-2 cardiomyopathy who underwent comprehensive cardiac rehabilitation. Conclusions: Cardiac rehabilitation with a comprehensive multidisciplinary intervention component allowed the patient to improve aerobic capacity, symptomatology, health-related quality of life and reincorporation to social, family and work activities(AU)

Repeated stellate ganglion blockade for the treatment of ventricular tachycardia storm in patients with nonischemic cardiomyopathy: a new therapeutic option for patients with malignant arrhythmias / 中华心血管病杂志

Objectives: This study sought to describe our institutional experience of repeated percutaneous stellate ganglion blockade (R-SGB) as a treatment option for drug-refractory electrical storm in patients with nonischemic cardiomyopathy (NICM). Methods: This prospective observational study included 8 consecutive NICM patients who had drug-refractory electrical storm and underwent R-SGB between June 1, 2021 and January 31, 2022. Lidocaine (5 ml, 1%) was injected in the vicinity of the left stellate ganglion under the guidance of ultrasound, once per day for 7 days. Data including clinical characteristics, immediate and long-term outcomes, and procedure related complications were collected. Results: The mean age was (51.5±13.6) years. All patients were male. 5 patients were diagnosed as dilated cardiomyopathy, 2 patients as arrhythmogenic right ventricular cardiomyopathy and 1 patient as hypertrophic cardiomyopathy. The left ventricular ejection fraction was 37.8%±6.6%. After the treatment of R-SGB, 6 (75%) patients were free of electrical storm. 24 hours Holter monitoring showed significant reduction in ventricular tachycardia (VT) episodes from 43.0 (13.3, 276.3) to 1.0 (0.3, 34.0) on the first day following R-SGB (P<0.05) and 0.5 (0.0, 19.3) after whole R-SGB process (P<0.05). There were no procedure-related major complications. The mean follow-up was (4.8±1.1) months, and the median time of recurrent VT was 2 months. Conclusion: Minimally invasive R-SGB is a safe and effective method to treat electrical storm in patients with NICM.

Cardiac amyloidosis: pathological classification and clinical analysis of 48 cases / 中华病理学杂志

Objective: To investigate the histological features and clinical manifestations in different types of cardiac amyloidosis to improve diagnostic accuracy. Methods: The histopathological features and clinical manifestations of 48 patients diagnosed with cardiac amyloidosis by Congo red stain and electron microscopy through endomyocardial biopsy were collected in West China Hospital of Sichuan University from January 2018 to December 2021. Immunohistochemical stains for immunoglobulin light chains (κ and λ) and transthyretin protein were carried out, and a review of literature was made. Results: The patients age ranged from 42 to 79 years (mean 56 years) and the male to female ratio was 1.1 to 1.0. The positive rate of endomyocardial biopsy was 97.9% (47/48), which was significantly higher than that of the abdominal wall fat (7/17). Congo red staining and electron microscopy were positive in 97.9% (47/48) and 93.5% (43/46), respectively. Immunohistochemical stains showed 32 cases (68.1%) were light chain type (AL-CA), including 31 cases of AL-λ type and 1 case of AL-κ type; 9 cases (19.1%) were transthyretin protein type (ATTR-CA); and 6 cases (12.8%) were not classified. There was no significant difference in the deposition pattern of amyloid between different types (P>0.05). Clinical data showed that ATTR-CA patients had less involvement of 2 or more organs and lower N-terminal pro-B-type natriuretic peptide (NT-proBNP) than the other type patients (P<0.05). The left ventricular stroke volume and right ventricular ejection fraction of ATTR-CA patients were better than the other patients (P<0.05). Follow-up data of 45 patients was obtained, and the overall mean survival time was 15.6±2.0 months. Univariate survival analysis showed that ATTR-CA patients had a better prognosis, while cardiac amyloidosis patients with higher cardiac function grade, NT-proBNP >6 000 ng/L, and troponin T >70 ng/L had a worse prognosis (P<0.05). Multivariate survival analysis showed that NT-proBNP and cardiac function grade were independent prognostic factors for cardiac amyloidosis patients. Conclusions: AL-λ is the most common type of cardiac amyloidosis in this group. Congo red staining combined with electron microscopy can significantly improve the diagnosis of cardiac amyloidosis. The clinical manifestations and prognosis of each type are different and can be classified based on immunostaining profile. However, there are still a few cases that cannot be typed; hence mass spectrometry is recommended if feasible.

Analysis of blood carnitine profile and SLC22A5 gene variants in 17 neonates with Primary carnitine deficiency / 中华医学遗传学杂志

OBJECTIVE@#To analyze the blood free carnitine (C0) level and SLC22A5 gene variants in 17 neonates with Primary carnitine deficiency (PCD) and to determine its incidence in local area and explore the correlation between C0 level and genotype.@*METHODS@#148 043 newborns born in 9 counties (cities and districts) of Ningde city from September 2016 to June 2021 were selected as study subjects. Blood free carnitine and acyl carnitine of 148 043 neonates were analyzed. Variants of the SLC22A5 gene were screened in those with blood C0 < 10 µmol/L, or C0 between 10 ∼ 15 µmol/L. Correlation between the free carnitine level and genetic variants was analyzed.@*RESULTS@#In total 17 neonates were diagnosed with PCD, which yielded a prevalence of 1/8 707 in the region. Twelve variants of the SLC22A5 gene were identified, with the common ones including c.760C>T, c.1400C>G and c.51C>G. Compared with those carrying other variants of the gene, children carrying the c.760C>T variant had significantly lower C0 values (P < 0.01).@*CONCLUSION@#The prevalence of PCD is relatively high in Ningde area, and intervention measures should be taken to prevent and control the disease. The c. 760C>T variant is associated with lower level of C0, which can provide a clue for the diagnosis.

Clinical characteristics of Danon disease / 中华心血管病杂志

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.

The value of maximal rate of left ventricular pressure in evaluating cardiac function in patients with sepsis-induced cardiomyopathy / 中华危重病急救医学

OBJECTIVE@#To investigate the value of maximal rate of left ventricular pressure (dp/dtmax) in evaluating the changes of cardiac function before and after heart rate reduction in patients with sepsis-induced cardiomyopathy (SIC).@*METHODS@#A single-center, prospective randomized controlled study was conducted. Adult patients with sepsis/septic shock admitted to the department of intensive care unit (ICU) of Tianjin Third Central Hospital from April 1, 2020 to February 28, 2022 were enrolled. Speckle tracking echocardiography (STE) and pulse indication continuous cardiac output (PiCCO) monitoring were performed immediately after the completion of the 1 h-Bundle therapy. The patients with heart rate over 100 beats/minutes were selected and randomly divided into esmolol group and regular treatment group, 55 cases in each group. All patients underwent STE and PiCCO monitoring at 6, 24 and 48 hours after admission in ICU and calculated acute physiology and chronic health evaluation II (APACHE II) and sequential organ failure assessment (SOFA). Primary outcome measure: change in dp/dtmax after reducing heart rate by esmolol. Secondary outcome measures: correlation between dp/dtmax and global longitudinal strain (GLS); changes of vasoactive drug dosage, oxygen delivery (DO2), oxygen consumption (VO2) and stroke volume (SV) after the administration of esmolol; proportion of heart rate reaching the target after the administration of esmolol; 28-day and 90-day mortality in two groups.@*RESULTS@#Baseline data on age, gender, body mass index, SOFA score, APACHE II score, heart rate, mean arterial pressure, lactic acid, 24-hour fluid balance, sepsis etiology and prior comorbidities were similar between esmolol group and regular treatment group, there were no significant differences between the two groups. All SIC patients achieved the target heart rate after 24 hours of esmolol treatment. Compared with regular treatment group, parameters reflecting myocardial contraction such as GLS, global ejection fraction (GEF) and dp/dtmax were significantly increased in esmolol group [GLS: (-12.55±4.61)% vs. (-10.73±4.82)%, GEF: (27.33±4.62)% vs. (24.18±5.35)%, dp/dtmax (mmHg/s): 1 312.1±312.4 vs. 1 140.9±301.0, all P < 0.05], and N-terminal pro-brain natriuretic peptide (NT-proBNP) significantly decreased [μg/L: 1 364.52 (754.18, 2 389.17) vs. 3 508.85 (1 433.21, 6 988.12), P < 0.05], DO2 and SV were significantly increased [DO2 (mL×min-1×m-2): 647.69±100.89 vs. 610.31±78.56, SV (mL): 49.97±14.71 vs. 42.79±15.77, both P < 0.05]. The system vascular resistance index (SVRI) in esmolol group was significantly higher than that in regular treatment group (kPa×s×L-1: 287.71±66.32 vs. 251.17±78.21, P < 0.05), even when the dosage of norepinephrine was similar between the two groups. Pearson correlation analysis showed that dp/dtmax was negatively correlated with GLS in SIC patients at 24 hours and 48 hours after ICU admission (r values were -0.916 and -0.935, respectively, both P < 0.05). Although there was no significant difference in 28-day mortality between esmolol group and regular treatment group [30.9% (17/55) vs. 49.1% (27/55), χ2 = 3.788, P = 0.052], the rate of esmolol use in patients who died within 28 days was lower than that in patients who survived [38.6% (17/44) vs. 57.6% (38/66), χ2 = 3.788, P = 0.040]. In addition, esmolol has no effect on the 90-day mortality of patients. Logistic regression analysis showed that after adjusting for SOFA score and DO2 factors, patients who used esmolol had a significantly lower risk of 28-day mortality compared with patients who did not use esmolol [odds ratio (OR) = 2.700, 95% confidence interval (95%CI) was 1.038-7.023, P = 0.042].@*CONCLUSIONS@#dp/dtmax in PiCCO parameter can be used as a bedside indicator to evaluate cardiac function in SIC patients due to its simplicity and ease of operation. Esmolol control of heart rate in SIC patients can improve cardiac function and reduce short-term mortality.

Desarrollo histórico de la protección miocárdica: el gran salto adelante de la cirugía cardiovascular / Historical development of myocardial protection: the big jump ahead of cardiovascular surgery

La cirugía a "corazón abierto" con circulación extracorpórea se apronta a cumplir 70 años. Desde un comienzo se hizo necesario buscar la forma de tener un campo operatorio quieto y exangüe, sin riesgo de embolia aérea, lo que llevó al desarrollo de la primera "cardioplegía" por Melrose, a fines de los años 1950's. Sin embargo, esta cayó en descrédito rápidamente y fue abandonada en años 1960's. Se necesitó que transcurrieran casi 15 años para el retorno de la cardioplegía, ahora como forma de proteger al corazón de la isquemia. Durante este periodo se volvió a la fibrilación ventricular a la perfusión coronaria, al paro isquémico y a la hipotermia tópica. Sin embargo, algunos investigadores mantuvieron su interés en la cardioplegía, facilitando el retorno clínico de esta con Hearse en 1976, con la solución cardioplégica cristaloide del Hospital St. Thomas, la que se asentó como el principal método de protección miocárdica, hasta la irrupción de Buckberg con su cardioplegía sanguínea en multidosis, la que se convirtió, a comienzo de los años 1990's, en el procedimiento preferido para proteger al corazón durante el periodo de isquemia requerido para operar en él, infundida ahora no solo por vía anterógrada, sino que también por vía retrógrada. Esto, hasta Pedro Del Nido y su vuelta a la dosis única, solo por vía anterógrada.

Open heart surgery with extracorporeal circulation is approaching its 70th anniversary. From the beginning it was necessary to find a way to have a still and bloodless operative field, without the risk of air embolism, which led to the development of the first "cardioplegía" by Melrose, at the end of the 1950's. However, it quickly fell into disrepute and was abandoned in the 1960's. It took almost 15 years for cardioplegía to return, now as a way of protecting the heart from ischemia. During this period, ventricular fibrillation, coronary perfusion, ischemic arrest and topical hypothermia returned. However, some investigators maintained their interest in cardioplegía, facilitating the clinical return of cardioplegía with Hearse in 1976, with the crystalloid cardioplegic solution of St. Thomas's Hospital. This became the main method of myocardial protection until the irruption of Buckberg with his multidose blood cardioplegía, which became, at the beginning of the 90's, the preferred method to protect the heart during the ischemic period required to operate on it, now infused not only by anterograde route but also by retrograde route. This, until Pedro Del Nido and his return to the single dose, only via the antegrade route.

Miocardiopatía periparto: revisión de la literatura / Peripartum Cardiomyopathy

Resumen: La Miocardiopatía Periparto es una patología que se presenta como una insuficiencia cardíaca aguda que aparece en el último mes del embarazo o los primeros 5 meses post parto, en ausencia de otra causa identificable. A pesar de ser más frecuente en países Afrodescendientes, el efecto migratorio ha provocado un aumento en su incidencia en los países de América latina, influyendo en la morbimortalidad materna. Si bien su etiología aún no está claramente definida, se han propuesto algunos mecanismos como el aumento del estrés oxidativo, el desequilibrio de la angiogénesis y las reacciones inflamatorias que, en un organismo genéticamente predispuesto, podrían ser los desencadenantes de esta enfermedad. Su manejo aún se considera de soporte, pero se sigue investigando en alternativas terapéuticas que puedan mejorar los resultados a largo plazo. Así, el motivo de esta revisión es evaluar la evidencia disponible hasta el momento, para el enfrentamiento del equipo tratante de estas pacientes.

Abstract: Peripartum Cardiomyopathy is a diseae presenting as acute heart failure that appears in the last month of pregnancy or within 5 months postpartum, in the absence of other identifiable cause. Despite being more frequent in Afro-descendant populations, the migratory effect has caused an increase in its incidence in Latin American countries, influencing maternal morbidity and mortality. Although its etiology is not yet defined, some mechanisms have been proposed such as increased oxidative stress, angiogénesis imbalance and inflammatory reactions that in a genetically predisposed organism, could be the triggers of this disease. Supportive therapy is still the initial management. Therapeutical alternatives that are still being investigated. The main purpose of this review is to evaluate the evidence available to improve the prognosis of the disease.

Primer uso de tafamidis en amiloidosis cardíaca TTR en Uruguay / First use of tafamidis in cardiac amyloidosis TTR in Uruguay / Primeiro uso de tafamidis em amiloidose cardíaca TTR no Uruguai

La amiloidosis cardíaca es una entidad con creciente reconocimiento, la variedad por transtiretina es la que más se diagnostica en la tercera edad de la vida. Hay reciente disponibilidad de fármacos que mejoran el pronóstico y la calidad de vida de los pacientes. Presentamos un caso de amiloidosis por transtiretina donde se usó por primera vez en nuestro país el fármaco tafamidis aprobado para el tratamiento de esta enfermedad.

Cardiac amyloidosis is an entity on increasing recognition, transthyretin variety is the most diagnosed in the third age. There is a recent availability of drugs that can improve the prognosis and quality of life of these patients. We present a case of transthyretin amyloidosis and the first use of tafamidis in our country.

A amiloidose cardíaca é uma entidade em crescente reconhecimento, a variedade transtiretina é a mais diagnosticada em idosos. Há disponibilidade recente de medicamentos que melhoram o prognóstico e a qualidade de vida dos pacientes. Apresentamos um caso de amiloidosis transteretina onde o medicamento tafamidis aprovado para esta doença foi utilizado pela primeira vez em nosso país.

Aporte de la centellografía con trazadores tecneciados fosfatados al diagnóstico de amiloidosis cardíaca / Contribution of scintigraphy with technetium phosphated tracers to the diagnosis of cardiac amyloidosis / Contribuição da cintilografia com traçadores fosfatados com tecnécio para o diagnóstico de amiloidose cardíaca

Las amiloidosis son enfermedades causadas por el depósito patológico extracelular de un material proteico fibrilar e insoluble denominado amiloide, que puede estar vinculado a cadenas livianas (AL) o transtirretina (TTR). La amiloidosis cardíaca provoca una cardiomiopatía restrictiva de carácter progresivo caracterizada por falla cardíaca con función sistólica relativamente preservada, que se asocia a elevada mortalidad. Aunque el diagnóstico definitivo tradicionalmente se basa en la biopsia endomiocárdica, los avances en imagenología han mejorado su abordaje y la reciente introducción de terapias especificas permiten augurar cambios significativos en el pronóstico. El tratamiento difiere según el tipo de amiloide involucrado y su resultado depende de la instauración precoz de este, por lo cual resulta esencial un diagnóstico preciso y temprano. El centellograma cardíaco con fosfatos marcados (99mTc-PYP u otros), ampliamente disponible y de relativo bajo costo, se considera en la actualidad como una "biopsia molecular no invasiva" para el diagnóstico de la amiloidosis tipo ATTR, que debe ser usado en conjunto con la investigación de proteínas monoclonales en pacientes con sospecha clínica de la enfermedad.

Amyloidoses are diseases caused by the extracellular deposition of a fibrillar and insoluble protein material called amyloid, which can be linked either to light chains (AL) or transthyretin (TTR). Cardiac amyloidosis causes a progressive restrictive cardiomyopathy characterized by heart failure with relatively preserved systolic function, which is associated with high mortality. Although a definitive diagnosis is traditionally based on endomyocardial biopsy, advances in cardiac imaging have improved its approach, and the recent introduction of specific therapies predicts significant changes in prognosis. Since treatment differs according to the type of amyloid involved and the results depend on a prompt implementation, an accurate and early diagnosis is essential. Cardiac scintigraphy with labeled phosphates (99mTc-PYP or others), widely available and relatively inexpensive, is currently considered a "noninvasive molecular biopsy" for the diagnosis of ATTR type amyloidosis, which should be used in conjunction with investigation of monoclonal proteins in patients with clinical suspicion of the disease.

As amiloidoses são doenças causadas pela deposição patológica extracelular de um material proteico fibrilar e insolúvel, denominado amiloide, que pode estar ligado a cadeias leves (AL) ou transtirretina (TTR). A amiloidose cardíaca causa cardiomiopatia restritiva progressiva caracterizada por insuficiência cardíaca com função sistólica relativamente preservada, que está associada a alta mortalidade. Embora o diagnóstico definitivo seja tradicionalmente baseado na biópsia endomiocárdica, os avanços nos exames de imagem aprimoraram sua abordagem e a recente introdução de terapias específicas pode predizer mudanças significativas no prognóstico. O tratamento varia de acordo com o tipo de amiloide envolvida e seu resultado depende do início precoce, por isso um diagnóstico preciso e precoce é essencial. A cintilografia cardíaca com fosfatos marcados (99mTc-PYP ou outros), amplamente disponível e relativamente econômico, é atualmente considerada uma "biópsia molecular não invasiva" para o diagnóstico de amiloidose do tipo ATTR, que deve ser usada em conjunto com a investigação de proteínas monoclonais em pacientes com suspeita clínica da doença.

Estudio de la amiloidosis cardíaca por cardiorresonancia / Cardiac amyloidosis study by cardioresonance / Estudo da amiloidose cardíaca por cardiorressonância

Dada su epidemiología, la amiloidosis cardíaca se ha vuelto un desafío diagnóstico del siglo XXI. La resonancia cardíaca se ha transformado en una herramienta diagnóstica y pronóstica fundamental en la práctica clínica diaria. En este artículo de revisión resumimos el conocimiento, primero abordando la técnica en sí, desde protocolos generales a específicos para su diagnóstico. Posteriormente, y tomando estas herramientas, sistematizamos la utilidad diagnóstica, pronóstica y terapéutica, con base en la practicidad y evidencia científica más recientes, otorgando la posibilidad de utilizar y abordar desde un correcto diagnóstico por imagen diferentes aspectos de esta enfermedad prevalente.

Given its epidemiology, cardiac amyloidosis has become a diagnostic challenge of the twenty-first century. Cardiac resonance has become a fundamental diagnostic and prognostic tool in daily clinical practice. In this review article we summarize the knowledge, first addressing the technique itself, from general to specific protocols for its diagnosis. Subsequently, and taking this tool we systematize the diagnostic, prognostic, and therapeutic utility, based on the most recent practicality and scientific evidence, granting the possibility of using and addressing from a correct diagnostic imaging different aspect of this prevalent disease.

Dada a sua epidemiologia, a amiloidose cardíaca tornou-se um desafio do século XXI. A ressonância cardíaca tornou-se uma ferramenta diagnóstica e prognóstico fundamental na prática clínica diária. Neste artigo de revisão resumimos primeiro o conhecimento abordando a própria técnica, desde protocolos gerais até específicos para seu diagnóstico. Posteriormente, e tomando essa ferramenta sistematizamos a utilidade diagnóstica, prognóstica e terapêutica, com base na praticidade e evidência científica mais recentes, concedendo a possibilidade de utilização e abordagem de uma imagem diagnóstica correta de diferentes aspectos desta doença prevalente.

Síndrome inflamatorio multisistémico post COVID-19 con afectación miocárdica: reporte de caso / Clinical case: post-COVID multisystem inflammatory syndrome with myocardial involvement / Síndrome inflamatória multissistêmica pós-COVID com acometimento do miocárdio (SIMC): relato de caso

En abril de 2020, durante el pico de la pandemia COVID-19 producida por el coronavirus emergente SARS-CoV-2, en el Reino Unido se comunicaron casos de shock hiperinflamatorio de características similares a la enfermedad de Kawasaki y el síndrome de shock tóxico en un grupo de ocho niños. El Royal College of Pediatrics and Child Health lo denominó síndrome inflamatorio multisistémico pediátrico temporalmente asociado con COVID-19 (SIM-C). Actualmente, el SIM-C es una enfermedad infrecuente, solapada con otras entidades, que requiere una alta sospecha clínica para identificarlo oportunamente. El síndrome inflamatorio multisistémico temporal asociado con SARS-CoV-2 pediátrico (PIMST) es una nueva entidad clínica con un amplio espectro de presentación postexposición al virus, inmunomediado con hiperinflamación y activación de una tormenta de citoquinas. Ocurre típicamente entre la segunda y cuarta semana de evolución. Se describen marcadores de inflamación característicamente elevados, como son la ferritina, proteína C reactiva (PCR), velocidad de eritrosedimentación (VES), lactato deshidrogenasa y dímero-D, asociados a neutropenia, linfopenia y anemia. La Organización Mundial de la Salud (OMS) define: caso a menores de 19 años con fiebre ≥3 días, marcadores inflamatorios elevados, evidencia de infección por SARS-CoV-2 y ninguna otra etiología microbiana; con afectación de al menos dos sistemas: dermatológico (rash, conjuntivitis no exudativa, inflamación mucocutánea), hemodinámico (hipotensión, shock), cardíaco (disfunción de miocardio, pericardio, valvular o coronario), hematológico (coagulopatía), digestivo (vómitos, diarrea, dolor abdominal). Considerando la gravedad de esta nueva entidad, es necesario el reconocimiento oportuno y referencia temprana para atención especiaizada y tratamiento oportuno.

Summary: In April 2020, during the peak of the COVID-19 pandemic caused by the emerging coronavirus SARS-CoV-2, 8 children reported cases of hyperinflammatory toxic shock with characteristics similar to Kawasaki disease and syndrome in the United Kingdom. The Royal College of Pediatrics and Child Health has called it pediatric Multisystem Inflammatory Syndrome (MIS) temporally associated with COVID-19. Currently, MIS-C is a rare disease, overlapping with other conditions, which requires a high clinical suspicion for its timely identification. Pediatric SARS-CoV-2-associated temporary multisystem inflammatory syndrome (TMIS-C) is a new clinical entity with a broad spectrum of presentation after exposure to the virus, immune-mediated with hyperinflammation and activation of a cytokine storm. It typically occurs between the 2nd to 4th week of evolution. Characteristically elevated markers of inflammation are described, such as ferritin, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), lactate dehydrogenase and D-dimer, associated with neutropenia, lymphopenia and anemia. The World Health Organization (WHO) defines it as: a case under 19 years of age with fever ≥ 3 days, elevated inflammatory markers, evidence of SARS-CoV-2 infection and no other microbial etiology; with involvement of at least 2 systems: dermatological (rash, non-exudative conjunctivitis, mucocutaneous inflammation), hemodynamic (hypotension, shock), cardiac (myocardial, pericardial, valvular, or coronary dysfunction), hematologic (coagulopathy), digestive (vomiting, diarrhea, abdominal pain) Considering the seriousness of this new entity, timely recognition and early referral for specialized care and timely treatment are key.

No mês de abril de 2020, durante o pico da pandemia de COVID-19 causada pelo emergente coronavírus SARS-CoV-2, 8 casos de crianças com choque hiperinflamatório com características semelhantes à doença e síndrome de Kawasaki foram relatados no Reino Unido. O Royal College of Pediatrics and Child Health nomeou-o como síndrome inflamatória multissistêmica pediátrica (MIS) temporariamente associada ao COVID-19. Atualmente, o SIM-C é uma doença rara, sobrepondo-se a outras entidades, o que requer alta suspeição clínica para sua identificação oportuna. A síndrome inflamatória multissistêmica temporária associada ao SARS-CoV-2 pediátrico (SIMT) é uma nova entidade clínica com amplo espectro de apresentação após exposição ao vírus, imunomediada com hiperinflamação e ativação de uma tempestade de citocinas. Geralmente ocorre entre a 2ª a 4ª semana de evolução. São descritos marcadores de inflamação caracteristicamente elevados, como ferritina, proteína C reativa (PCR), velocidade de hemossedimentação (VHS), lactato desidrogenase e D-dímero, associados a neutropenia, linfopenia e anemia. A Organização Mundial da Saúde (OMS) a define como: caso de menor de 19 anos com febre ≥ 3 dias, marcadores inflamatórios elevados, evidência de infecção por SARS-CoV-2 e nenhuma outra etiologia microbiana; com envolvimento de pelo menos 2 sistemas: dermatológico (erupção cutânea, conjuntivite não exsudativa, inflamação mucocutânea), hemodinâmica (hipotensão, choque), cardíaca (disfunção miocárdica, pericárdica, valvar ou coronariana), hematológica (coagulopatia), digestiva (vômitos, diarreia, dor abdominal) Considerando a gravidade dessa nova entidade, é necessário o reconhecimento oportuno e encaminhamento precoce para atendimento especializado e tratamento oportuno.

Manifestations cardiovasculaires au cours de l'infection à VIH à Libreville: étude comparative de patients séropositifs et séronégatifs / Cardiovascular Manifestations during HIV infection in Libreville: Comparative study of seropositive and seronegative patients

Introduction. Le spectre des atteintes cardiovasculaires au cours de l'infection à VIH a été modifi é par la trithérapie antirétrovirale. L'objectif de ce travail était de décrire le profi l des manifestations cardiovasculaires chez les patients vivants avec le VIH en le comparant à celui de patients séronégatifs. Méthodes. Il s'est agi d'une étude cas-témoins des dossiers de patients respectivement séropositifs et séronégatifs hospitalisés pour une pathologie cardiovasculaire au service de cardiologie du Centre Hospitalier Universitaire de Libreville de janvier 2015 à décembre 2018. L'analyse statistique a été réalisée à l'aide du logiciel Statview 5.0. Lestests de Chi-2 de Pearson ou Exact de Ficher ont été utilisés pour la comparaison des proportions. Résultats. L'étude a porté sur sur l'analyse de 82 et 150 dossiers de patients respectivement séropositifs et séronégatifs. Un âge inférieur à 50 ans était retrouvé chez 70,7% des séropositifs et 43,3% des séronégatifs (p<0,01). Le taux de CD4 moyen des séropositifs était de 189±170/mm3 et 45,1% d'entre eux étaient sous trithérapie antiretrovirale.La cardiomyopathie dilatée était l'atteinte cardiaque la plus fréquente chez les séropositifs (42,7%) et chez les séronégatifs (52,7%) (p=0,14). La maladie thromboembolique veineuse était relevée chez 7(8,5%) séropositifs et 14 (8,8%) séronégatifs (p=0,93). Une péricardite était diagnostiquée chez 25,6% des séropositifs avec une étiologie tuberculeuse dans 85,7% des cas. Les pathologies vasculaires athéromateuses étaient plus fréquentes chez les séronégatifs (23,1%) comparés aux séropositifs (6,1%) (p<0,01). La mortalité des séropositifs était principalement due aux péricardites (71,4%). Conclusion. les manifestations cardiovasculaires liées à l'immunodépression persistent chez les personnes vivant avec le VIH à Libreville. Un dépistage précoce de ces atteintes permettrait de réduire la mortalité.

Introduction. The spectrum of cardiovascular damage during HIV infection has been modified by triple antiretroviral therapy. The objective of this study was to describe the profile of cardiovascular manifestations in patients living with HIV by comparing it to the one of seronegative patients. Methods. This was a case-control study which focused on the files of patients hospitalized for a cardiovascular pathology in the cardiology department of the Center Hospitalier Universitaire de Libreville from january 2015 to december. 2018. Results. In total, there was on the analysis of the files of 82 seropositive patients and 150 seronegative patients. The age found was less than 50 years old in 70.7% of seropositives and 43.3% of seronegatives (p <0.01). The mean CD4 count in seropositives was 189 ± 170 /mm3 and 45.1% of them were on triple antiretroviral therapy. Dilated cardiomyopathy was the most common cardiac disease in HIVpositive (42.7%) and HIV-negative (52.7%) (p = 0.14). Venous thromboembolic disease was noted in 7 (8.5%) seropositives and 14 (8.8%) seronegatives (p=0.93).Pericarditis was diagnosed in 25.6% of seropositives patients with a tuberculous etiology in 85.7% of cases. Atheromatous vascular pathologies were more frequent in seronegative (23.1%) compared to seropositive (6.1%) (p <0.01). Mortality among seropositive was mainly due to pericarditis (71.4%)

Advances in extracardiac mechanisms for heart failure with preserved ejection fraction / 中南大学学报(医学版)

Heart failure with preserved ejection fraction (HFpEF) is a syndrome with highly heterogeneous clinical symptoms, and its incidence has been increasing in recent years. Compared with heart failure with reduced ejection fraction (HFrEF), HFpEF has a worse prognosis. Traditional therapies targeting the internal mechanisms of the heart show limited or inefficacy on HFpEF, and new therapeutic targets for HFpEF are expected to be found by focusing on the extracardiac mechanisms. Recent studies have shown that cardiopulmonary pathophysiological interaction exacerbates the progression of HFpEF. Hypertension, systemic vascular injury, and inflammatory response lead to coronary microvascular dysfunction, myocardial hypertrophy, and coronary microvascular remodeling. Acute kidney injury affects myocardial energy production, induces oxidative stress and catabolism of myocardial protein, which leads to myocardial dysfunction. Liver fibrosis mediates heart injury by abnormal protein deposition and inflammatory factors production. Skeletal muscle interacts with the sympathetic nervous system by metabolic signals. It also produces muscle factors, jointly affecting cardiac function. Metabolic syndrome, gut microbiota dysbiosis, immune system diseases, and iron deficiency promote the occurrence and development of HFpEF through metabolic changes, oxidative stress, and inflammatory responses. Therefore, the research on the extracardiac mechanisms of HFpEF has certain implications for model construction, mechanism research, and treatment strategy formulation.

Effects of Qilong Capsules on myocardial fibrosis and insufficient blood circulation in ischemic cardiomyopathy with Qi deficiency and blood stasis / 中国中药杂志

Protective effect of Qilong Capsules(QL) on the myocardial fibrosis and blood circulation of rats with coronary heart disease of Qi deficiency and blood stasis type was investigated. Sleep deprivation and coronary artery ligation were used to construct a disease-symptom combination model, and 60 SD rats were divided into sham operation(sham) group, syndrome(S) group, disease and syndrome(M) group and QL group randomly. The treatment group received administration of QL 0.4 g·kg~(-1)·d~(-1). Other groups were given the same amount of normal saline. The disease indexes of each group [left ventricular end diastolic diameter(LVESD), left ventricular end systolic diameter(LVEDD), left ventricular ejection fraction(LVEF), left ventricular axis shortening rate(LVFS), myocardial histopathology, platelet morphology, peripheral blood flow] and syndrome indexes(tongue color, pulse, grip power) were detected. In sham group, cardiomyocytes and myocardial fibers were arranged neatly and densely with clear structures. The tongues' color in sham were light red, and the pulse shape were regular. RGB is a parameter reflected the brightness of the image of the tongue. In the S group, the amplitude and frequency of the animal's pulse increased accompanied by decreasing R,G,B, however, the decreased R,G,B was accompanied by reduced pulse amplitude in M group. And in M group, we observed fuzzy cell morphology, hypertrophied myocytes, disordered arrangement of cardiomyocytes and myocardial fibers, reduced peripheral blood flow and increased collagen volume fraction(CVF). Increased LVESD and LVEDD, and decreased LVEF and LVFS represented cardiac function in S group was significantly lower than that in sham. In QL group, the tongue's color was red and the pulse was smooth. The myocardial fibers of the QL group were arranged neatly and secreted less collagen. It improved the blood circulation in the sole and tail, and reversed the increasing of LVEDD, LVESD and the decreasing of LVEF and LVFS of M group. Platelets in M and S group showed high reactivity, and QL could decrease aggregation risk. In conclusion, Qilong Capsules has an obvious myocardial protective effect on ischemic cardiomyopathy, which may inhibit the degree of myocardial fibrosis and reduce platelet reactivity.

Clinical characteristics and long-term follow-up results of radiofrequency ablation for the treatment of ventricular tachycardia in patients with arrhythmogenic left ventricular cardiomyopathy / 中华心血管病杂志

Objective: To investigate the acute and long-term outcome of catheter ablation for the treatment of ventricular tachycardia (VT) in patients with arrhythmogenic left ventricular cardiomyopathy (ALVC). Methods: This retrospective, cross-sectional study enrolled ALVC patients undergoing radiofrequency ablation for the treatment of VT at the First Affiliated Hospital of Nanjing Medical University from January 2011 to December 2018 and collected their clinical characteristics and intraoperative electrophysiological examination. Patients were followed up every 6 months after radiofrequency ablation until August 2021. Echocardiographic results and VT recurrence post radiofrequency ablation were analysed. Results: Totally 12 patients were enrolled (mean age: (42±15) years, 11 males(11/12)). The mean of left ventricular end diastolic diameter (LVDd) and left ventricular ejection fraction (LVEF) were (51±5)mm and (65±5)%, respectively. Twelve VTs were induced in 10 patients during the electrophysiological study, and the mean tachycardia cycle length was (293±65) ms. Three-dimensional substrate mapping revealed the diseased area at endocardial site in one patient, at epicardial sites in the other 11 patients (involved endocardial sites in 2 cases) with the basal part near the mitral annulus being the predilection for the substrate (10/11). After the catheter ablation at the endocardial and epicardial sites respectively, the complete procedure endpoint was achieved in all patients (VT cannot be induced post ablation). The median follow-up time was 65 (25, 123) months. One patient was lost to follow-up, and the other 11 patients survived without VT. No significant cardiac function deterioration was detected by the echocardiographic examination ((51±5)mm vs. (52±5)mm, P>0.05 for LVDd, (65±5)% vs. (60±6)%, P>0.05 for LVEF) at the end of follow-up. Conclusion: After radiofrequency ablation, the complete procedure endpoint is achieved in ALVC patients, and the catheter ablation provides long-term ventricular tachycardia control during the long-term follow-up.

Perfil Clínico, Laboratorial e de Métodos de Imagem na Amiloidose Sistêmica em um Centro de Referência Cardiológico Brasileiro / Clinical, Laboratory, and Imaging Profile in Patients with Systemic Amyloidosis in a Brazilian Cardiology Referral Center

Resumo Fundamento Amiloidose sistêmica é uma doença com manifestações clínicas diversas. O diagnóstico envolve suspeita clínica, aliada a métodos complementares. Objetivo Descrever o perfil clínico, laboratorial, eletrocardiográfico e de imagem no acometimento cardíaco da amiloidose sistêmica. Métodos Estudo de uma amostra de conveniência, analisando dados clínicos, laboratoriais, eletrocardiográficos, ecocardiográficos, medicina nuclear e ressonância magnética. Considerou-se significância estatística quando p < 0,05. Resultados Avaliaram-se 105 pacientes (com mediana de idade de 66 anos), sendo 62 homens, dos quais 83 indivíduos apresentavam amiloidose por transtirretina (ATTR) e 22 amiloidose por cadeia leve (AL). Na ATTR, 68,7% eram de caráter hereditário (ATTRh) e 31,3% do tipo selvagem (ATTRw). As mutações mais prevalentes foram Val142Ile (45,6%) e Val50Met (40,3%). O tempo de início dos sintomas ao diagnóstico foi 0,54 e 2,15 anos nas formas AL e ATTR (p < 0,001), respectivamente. O acometimento cardíaco foi observado em 77,9% dos ATTR e 90,9% dos AL. Observaram-se alterações de condução atrioventricular em 20% e intraventricular em 27,6% dos pacientes, sendo 33,7 % na ATTR e 4,5% das AL (p = 0,006). A forma ATTRw apresentou mais arritmias atriais que os ATTRh (61,5% x 22,8%; p = 0,001). Ao ecocardiograma a mediana da espessura do septo na ATTRw x ATTRh x AL foi de 15 mm x 12 mm x 11 mm (p = 0,193). Observou-se BNP elevado em 89,5% dos indivíduos (mediana 249 ng/mL, IQR 597,7) e elevação da troponina em 43,2%. Conclusão Foi possível caracterizar, em nosso meio, o acometimento cardíaco na amiloidose sistêmica, em seus diferentes subtipos, através da história clínica e dos métodos diagnósticos descritos.

Abstract Background Systemic amyloidosis is a disease with heterogeneous clinical manifestations. Diagnosis depends on clinical suspicion combined with specific complementary methods. Objective To describe the clinical, laboratory, electrocardiographic, and imaging profile in patients with systemic amyloidosis with cardiac involvement. Methods This study was conducted with a convenience sample, analyzing clinical, laboratory, electrocardiographic, echocardiographic, nuclear medicine, and magnetic resonance data. Statistical significance was set at p < 0.05. Results A total of 105 patients were evaluated (median age of 66 years), 62 of whom were male. Of all patients, 83 had transthyretin (ATTR) amyloidosis, and 22 had light chain (AL) amyloidosis. With respect to ATTR cases, 68.7% were the hereditary form (ATTRh), and 31.3% were wild type (ATTRw). The most prevalent mutations were Val142Ile (45.6%) and Val50Met (40.3%). Time from onset of symptoms to diagnosis was 0.54 and 2.15 years, in the AL and ATTR forms, respectively (p < 0.001). Cardiac involvement was observed in 77.9% of patients with ATTR and in 90.9% of those with AL. Alterations were observed in atrioventricular and intraventricular conduction in 20% and 27.6% of patients, respectively, with 33.7% in ATTR and 4.5% in AL (p = 0.006). In the ATTRw form, there were more atrial arrhythmias than in ATTRh (61.5% versus 22.8%; p = 0.001). On echocardiogram, median septum thickness in ATTRw, ATTRh, and AL was 15 mm, 12 mm, and 11 mm, respectively (p = 0.193). Elevated BNP was observed in 89.5% of patients (median 249, ICR 597.7), and elevated troponin was observed in 43.2%. Conclusion In this setting, it was possible to characterize cardiac involvement in systemic amyloidosis in its different subtypes by means of clinical history and the diagnostic methods described.

Cardiomiopatía en el paciente con cirrosis hepática: artículo de revisión / Cardiomyopathy in patients with liver cirrhosis: review article

La cardiomiopatía cirrótica se trata de una complicación en el paciente cirrótico, con una pre- valencia superior al 40%. Es una entidad subclínica, pero ante el ejercicio o estrés circulatorio, desencadena su sintomatología. Su fisiopatología se explica por la hipertensión portal que lleva a vasodilatación esplácnica, con posterior liberación de vasodilatadores y factores cardiosupresores, lo cual conduce a una circulación hiperdinámica y disfunción circulatoria, con elevación del gasto cardíaco, disminución de la resistencia vascular y presión arterial baja. Sus características principales son disfunción cardiaca sistólica y diastólica, circulación hiperdinámica y alteraciones electrofisiológicas, especialmente prolongación del intervalo QT. Para su diagnóstico se usa la ecocardiografía y pruebas de estrés físico o farmacológico. No existe un protocolo de tratamiento estandarizado, sin embargo, el trasplante hepático puede ser un procedimiento efectivo para revertir la disfunción cardiaca en algunos pacientes. En el presente artículo se describen tanto las características de la cardiomiopatía cirrótica, como su papel en la morbilidad y mortalidad del paciente con cirrosis.

Cirrhotic cardiomyopathy is a complication in cirrhotic patients, with a prevalence of more than 40%. During the resting state, it is a subclinical entity, which triggers symptoms upon exercise or circulatorystress. Its pathophysiology is explained by portal hypertension leading to splanchnic vasodilatation, with subsequent release of vasodilators and cardio-suppressive factors, which leads to hyperdynamic circulation and circulatory dysfunction, with elevated cardiac output, decreased vascular resistance and low blood pressure. The main characteristics are systolic and diastolic cardiac dysfunction, hyperdynamic circulation and electrophysiological alterations, especially prolongation of the QT interval. Echocardiography and physical or pharmacological stress tests are used in diagnosis. There is not an standardized treatment protocol. Liver transplantation can be an effective procedure to reverse cardiac dysfunction in selected patients. In this review, we describe the characteristics of cirrhotic cardiomyopathy and its role in the morbidity and mortality of the patient with cirrhosis.