Repercusiones cardíacas en pacientes con pectus excavatum: puesta al día. Segunda parte / Cardiac repercussions in patients with pectus excavatum: update. Part two / Repercussões cardíacas em pacientes com pectus excavatum: atualização. Parte dois

El pectus excavatum (PEX) es una deformación de la pared torácica que obedece a una alteración de los cartílagos costales con el consiguiente hundimiento del esternón. Históricamente, se clasificaba como un defecto únicamente estético o cosmético, sin embargo, en los últimos años se han desarrollado nuevos métodos de estudio para la valoración de las repercusiones de esta patología. Existe cada vez más bibliografía que demuestra importantes repercusiones funcionales. Se realizó una puesta al día de las repercusiones cardíacas de la patología y un análisis de los artículos más relevantes de los últimos años. La evidencia actual permite afirmar que existe una afectación cardíaca por compresión esternal en la mayoría de los pacientes con PEX. Las afectaciones incluyen alteraciones anatomofuncionales (trastornos del ritmo, disminución del llenado ventricular), del volumen sistólico, aumento de la presión de la aurícula derecha, valvulopatías, compresión del ventrículo derecho, derrame pericárdico, entre otras. Todo lo cual permite concluir que el PEX puede presentar importantes alteraciones cardíacas que deben ser tenidas en cuenta a la hora de valorar los pacientes con esta patología.

Pectus excavatum (PEX) is a deformation of the chest wall caused by an alteration of the costal cartilages with the consequent collapse of the sternum. Historically, it had been classified as a solely aesthetic or cosmetic defect, however, in recent years new study methods have been developed to assess the repercussions of this pathology, with increasing bibliography showing important functional consequences. We updated the cardiac pathological repercussions and analyzed the most relevant articles of recent years. The current evidence suggests that there is cardiac involvement due to sternal compression in most patients with PEX. These affectations include anatomical functional alterations: rhythm disorders, decreased ventricular filling, decreased stroke volume, increased right atrial pressure, valve disease, right ventricular compression, pericardial effusion, among others. All of which enables us to conclude that PEX can present important cardiac alterations that must be taken into account when assessing patients with this pathology.

Pectus excavatum (PEX) é uma deformação da parede torácica decorrente de uma alteração das cartilagens costais com consequente colapso do esterno. Historicamente, foi classificado como um defeito exclusivamente estético ou cosmético, porém, nos últimos anos, novos métodos de estudo foram desenvolvidos para avaliar as repercussões dessa patologia, com crescente bibliografia mostrando importantes repercussões funcionais. Foi realizada uma atualização das repercussões cardíacas da patologia e análise dos artigos mais relevantes dos últimos anos. As evidências atuais permitem afirmar que há acometimento cardíaco por compressão esternal na maioria dos pacientes com PEX. As afecções incluem alterações anatomofuncionais: distúrbios do ritmo, diminuição do enchimento ventricular, diminuição do volume sistólico, aumento da pressão atrial direita, doença valvular, compressão do ventrículo direito, derrame pericárdico, entre outras. Tudo isso permite concluir que o PEX pode apresentar alterações cardíacas importantes que devem ser levadas em consideração na avaliação de pacientes com essa patologia.

Amiloidosis cardíaca: revisión de una serie de casos / Cardiac amyloidosis: a review of a series of cases

Resumen Antecedentes y objetivo: La amiloidosis cardíaca es una entidad que permanece infradiagnosticada, a pesar de los avances recientes en su diagnóstico y tratamiento. El objetivo de este estudio es revisar una serie de casos de amiloidosis cardíaca para describir los principales datos clínicos y los hallazgos en las pruebas de imagen. Materiales y métodos: Estudio retrospectivo de pacientes con diagnóstico principal o secundario de amiloidosis cardíaca en los informes de alta de pacientes hospitalizados en este centro desde 2006 hasta 2016. Se revisaron los datos clínicos de los pacientes, así como las pruebas de imagen (ECG, ecocardiograma, gammagrafía cardíaca, resonancia magnética cardíaca). Se realizó seguimiento de los pacientes hasta enero de 2018. Resultados: Se analiza a 30 pacientes (20 varones) con media de 65 años. Los principales datos ecocardiográficos fueron dilatación biauricular, disfunción diastólica e hipertrofia ventricular izquierda (HVI) en un 97%. Sólo el 6.7% cumplía criterios de HVI en el electrocardiograma. Hasta un 33% tenía disfunción sistólica. Se realizó gammagrafía y resonancia magnética cardíaca en un 33%. La supervivencia a los 12 meses fue de 61%. Conclusión: La presencia de insuficiencia cardíaca, fibrilación auricular o trastornos de conducción junto a datos ecocardiográficos indicativos debe alertar al clínico. Otros datos como disfunción sistólica o sexo femenino no deben disminuir la sospecha. El estudio debe completarse con gammagrafía y resonancia magnética cardíaca, ya que el diagnóstico temprano tiene implicaciones pronósticas y terapéuticas.

Abstract Background and objective: Cardiac amyloidosis is an entity that remains underdiagnostic, despite recent advances in its diagnosis and treatment. The aim of this study is to review a series of diagnosed cases of cardiac amyloidosis to describe the main clinical data and the findings in the imaging tests. Materials and methods: Retrospective study of patients with primary or secondary diagnosis of cardiac amyloidosis in discharge reports of patients hospitalized in our center from 2006 to 2016. The clinical data of the patients were reviewed, as well as the imaging tests (ECG, echocardiogram, cardiac scintigraphy, cardiac magnetic resonance). Patients were followed until January 2018. Results: We analyze 30 patients (20 men) with an average of 65 years. The main echocardiographic data were biatrial dilatation, diastolic dysfunction and left ventricular hypertrophy (LVH) in 97%. Only 6.7% met criteria for LVH in the electrocardiogram. Up to 33% had systolic dysfunction. Scintigraphy and cardiac magnetic resonance were performed in 33%. Survival at 12 months was 61%. Conclusion: The presence of heart failure, atrial fibrillation or conduction disorders with suggestive echocardiographic data should alert the clinician. Other data such as systolic dysfunction or female sex should not decrease the suspicion. The study should be completed with gammagraphy and cardiac magnetic resonance since early diagnosis has prognostic and therapeutic implications.

Análisis de los resultados del programa de ecocardiografía funcional neonatal en un hospital pediátrico de tercer nivel / Analysis of the results of the neonatal functional echocardiography program in a third-level pediatric hospital

Resumen Introducción: Se ha demostrado la utilidad del estudio ecocardiográfico en las enfermedades que afectan a los neonatos en estado crítico, por lo que se debe capacitar al neonatólogo para la toma y la interpretación de variables hemodinámicas que apoyen la toma de decisiones. El objetivo de este estudio fue describir los resultados del programa de ecocardiografía funcional neonatal y comparar las variables clínicas y hemodinámicas entre sobrevivientes y no sobrevivientes. Métodos: Durante un periodo de 2 años se realizó un estudio observacional, transversal y comparativo en neonatos que fueron evaluados con ecocardiografía funcional neonatal por alteraciones hemodinámicas del conducto arterioso persistente, hipertensión pulmonar aguda y crónica, estado de choque y búsqueda de trombos y vegetaciones. Se evaluaron parámetros de funcionalidad de los ventrículos derecho e izquierdo, presión pulmonar y subrogados de sobrecarga pulmonar. Se realizó un análisis comparativo (U de Mann Whitney y Χ2). De acuerdo con los resultados, se emitieron recomendaciones para el inicio, el ajuste o el retiro de fármacos vasoactivos. Resultados: Se realizaron 269 estudios en 119 neonatos (64.7% prematuros, mortalidad del 15%) atendidos por hipertensión pulmonar aguda (38%), conducto arterioso persistente (27%), choque (19%), hipertensión pulmonar crónica (14%) o búsqueda de trombos (2%). Se recomendó cambiar el manejo en el 45% de los estudios. El grupo de no sobrevivientes presentó diferencias significativas en los parámetros de función sistólica del ventrículo derecho y aumento de su poscarga. Conclusiones: La causa principal de la evaluación con ecocardiografía funcional neonatal fue la hipertensión pulmonar. De forma global, se recomendó un ajuste farmacológico en el 45% de los casos.

Abstract Background: Echocardiography is useful in the group of comorbidities of critically ill newborns. The targeted neonatal echocardiography program trains neonatologists for acquiring and interpreting hemodynamic variables to support decision making. This study aimed to describe the results of the functional echocardiography program (fNE) and compare clinical and hemodynamic variables between survivors and non-survivors. Methods: Observational, cross-sectional, and comparative study of neonates that received a fNE evaluation for hemodynamic disturbances related to patent ductus arteriosus (PDA), acute and chronic pulmonary hypertension (aPH, cPH), state of shock and thrombus/vegetations surveillance for two years. Functional parameters of the right and left ventricle, pulmonary pressure, and surrogates of pulmonary over circulation were assessed. Comparative analysis with U Mann Whitney test and Χ2 was performed. Based on the results, recommendations to start, adjust, or withdraw vasoactive medications were issued. Results: Of 269 studies on 119 neonates (65% premature, 15% mortality), the reasons for consultation were aPH (38%), PDA (27%), shock (19%), cPH (14%), and thrombus surveillance (2%). A change in management was recommended on 45% of studies. Non-survivors presented significant differences in the right ventricular (RV) systolic performance and an increased right ventricular afterload. Conclusions: The main indication for fNE was pulmonary hypertension. A pharmacological adjustment was recommended on 45% of the cases.

Risk factors for cardiac, obstetric, and neonatal complications in patients with heart disease during pregnancy / Factores de riesgo para complicaciones cardíacas, obstétricas y neonatales en pacientes con enfermedad cardíaca durante el embarazo

Abstract Objective: Heart disease in pregnancy can cause clinical deterioration and maternal-fetal death. It is essential to evaluate risk factors related to complications. Methodology: This was a observational, analytical retrospective cohort study with a non-probabilistic convenience sample of pregnant women with congenital or acquired heart disease, corrected or not, or arrhythmias requiring urgent intervention. Patients with mild or moderate valvular regurgitation, mild valvular stenosis, patients without echocardiography or without delivery information were excluded from the study. The outcome was a composite of cardiac, obstetric, and neonatal events. Univariate and multivariate analyzes were performed with logistic regression model and discriminatory capacity with area under the curve and independent analysis of the modified World Health Organization (mWHO) risk classification (mWHO). Results: A total of 104 patients with an average age of 25 ± 6.5 years presented cardiac events in 13.5%, obstetric in 14.42%, and neonatal in 28.85%. The univariate analysis found an association with New York Heart Association functional status, hypertensive disorders of pregnancy, cesarean delivery, gestational age < 27 weeks, hypoxemia, and mWHO risk. In multivariate only cesarean delivery (odds ratio [OR], 2.68; 95% confidence interval [CI], 1.05-6.86) and gestational age at delivery (OR, 0.39; 95% CI, 0.22-0.67) maintain association with outcomes. The area under the curve for the mWHO risk is 0.75. Conclusions: There is a high rate of adverse events in patients with heart disease during pregnancy. Gestational age and cesarean delivery behaved as predictors of adverse maternal-fetal outcomes. The mWHO risk classification had an acceptable prediction of adverse outcomes.

Resumen Objetivo: La enfermedad cardíaca en el embarazo puede ocasionar deterioro clínico y muerte maternofetal. Es indispensable evaluar factores de riesgo relacionados con complicaciones. Método: Estudio observacional y analítico de cohorte retrospectivo con muestra no probabilística por conveniencia de embarazadas con cardiopatía congénita o adquirida, corregida o no, o arritmias que requerían intervención urgente. Se excluyó a pacientes con insuficiencias valvulares leves o moderadas, estenosis valvulares leves, pacientes sin ecocardiografía o sin información del parto. El desenlace fue un compuesto de episodios cardíacos, obstétricos y neonatales. Se realizó análisis univariado y multivariado con modelo de regresión logística y capacidad diferenciadora con área bajo la curva y análisis independiente de la clasificación de riesgo de la OMS modificada (OMSm). Resultados: 104 pacientes con edad promedio de 25 ± 6.5 años presentaron episodios cardíacos en 13.5%, obstétricos en 14.42% y neonatales en 28.85%. El análisis univariado encontró una relación con el estado funcional de la NYHA, trastornos hipertensivos del embarazo, parto por cesárea, edad gestacional < 27 semanas, hipoxemia y riesgo de la OMSm. En el multivariado sólo el parto por cesárea (OR, 2.68; IC 95%, 1.05-6.86) y la edad gestacional al momento del parto (OR, 0.39; IC 95%, 0.22-0.67) mantienen nexo con los desenlaces. El área bajo la curva para el riesgo de la OMSm es de 0.75. Conclusiones: Hay una elevada tasa de efectos adversos en pacientes con enfermedad cardíaca durante el embarazo. La edad gestacional y el parto por cesárea se comportaron como predictores de resultados adversos maternofetales. La clasificación de riesgo de la OMSm tuvo una predicción aceptable de desenlaces adversos.

Pregnancy in teenagers with heart disease / Embarazo en pacientes adolescentes con cardiopatía

Abstract The prevalence of pregnancy in adolescent women is high in Mexico and represents a public health problem. The pregnant teenager with heart disease has a high probability of complications during pregnancy and the delivery, which carries a risk of death of both the mother and the product. In many cases the pregnancy should have been avoided, planned or interrupted, however the majority at this age is vulnerable and although certain cases must be interrupted by their high risk of maternal-fetal death, prevention and legal aspects should be considered. In some cases the woman wants a pregnancy although her health condition does not allow it, but there are options of adoption or recourse to a surrogate belly. In response to this growing social problem, the National Cardiology Institute Ignacio Chávez and National Institute of Perinatology, with the coordination of Ministry of Health in Mexico, started a pregnancy prevention module within a clinic of follow-up of cardiopathy and pregnancy. This review raises the global problem in our country that occupies the first place in pregnancies in adolescents, with more than 400,000 pregnancies a year and the form of immediate response in a multidisciplinary way.

Resumen La prevalencia de embarazo en mujeres adolescentes es muy alta en México, y representa un problema de salud pública. La adolescente embarazada con cardiopatía tiene altas posibilidades de complicaciones durante el embarazo y su resolución, lo que pone en riesgo la vida tanto de la madre como del producto. En muchos casos el embarazo debió ser evitado, planeado o interrumpido, sin embargo la mayoría a esta edad es vulnerable y si bien ciertos casos deben ser interrumpidos por su alto riesgo de muerte materno-fetal, es fundamental considerar la prevención y los aspectos legales. En algunos casos la mujer desea un embarazo aunque su condición de salud no se lo permite, pero existen opciones de adopción o recurrir a un vientre subrogado. Atendiendo este problema social cada vez más creciente, el Instituto Nacional de Cardiología Ignacio Chávez, en coordinación con la Comisión Coordinadora de la Secretaría de Salud y el Instituto Nacional de Perinatología, echaron a andar un módulo de prevención de embarazo dentro de una clínica de seguimiento de cardiopatía y embarazo. Esta revisión plantea el problema global en nuestro país, que ocupa el primer lugar en embarazos en adolescentes, con más de 400 mil embarazos al año y la forma de dar respuesta inmediata de manera multidisciplinaria.

COVID-19 y afectación cardíaca / Cardiac implications and COVID-19

The coronavirus pandemic has generated a serious global health problem. COVID-19 mainly affects the lung, but it has been seen that myocardial involvement also occurs in some patients, producing myocarditis and arrhythmias.

La pandemia por coronavirus ha generado un grave problema sanitario mundial. El COVID-19 afecta fundamentalmente el pulmón, pero se ha visto que en algunos pacientes también se produce afectación del miocardio produciendo miocarditis y arritmias.

Applicability of the Electro-Vectorcardiogram in Current Clinical Practice / Aplicabilidade do Eletrovetorcardiograma na Atual Prática Clínica - Um Retrato Preciso da Ativação Elétrica Cardíaca

Abstract The electrocardiogram (ECG) has been reinvigorated by the identification of electrical alterations that were not definitely clarified before. In this context, and mainly regarding the definition of arrhythmogenic substrates, the association of the ECG with the vectorcardiogram (VCG) has gathered much more information about the cardiac electrical phenomena, thus allowing us to differentiate potentially fatal cases from benign ones. Obtaining a VCG concomitantly with the performance of an ECG has led to a significant gain in the definition of extremely sophisticated pathologies, which function suffer some type of structural or dynamic alterations, involving either the reduction or enhancement of ionic channels and currents. The classic aspects of the ECG/VCG association in the differential diagnosis of myocardial infarctions, conduction disorders, atrial and ventricular hypertrophies, and the correlations between these electrical disorders are still valid and assertive. The association of these pathologies is further clarified when they are seen through the ECG/VCG dyad. The three-dimensional spatial orientation of both the atrial and the ventricular activity provides a far more complete observation tool than the ECG linear form. The modern analysis of the ECG and its respective VCG, simultaneously obtained by the recent technique called electro-vectorcardiogram (ECG/VCG), brought a significant gain for the differential diagnosis of some pathologies. Therefore, we illustrate how this type of analysis can elucidate some of the most important diagnoses found in our daily clinical practice as cardiologists.

Resumo O eletrocardiograma (ECG) foi revigorado com o reconhecimento de alterações elétricas que ainda não haviam sido observadas de maneira definitiva. Nesse contexto, principalmente na definição dos substratos arritmogênicos, a associação do ECG com o vetorcardiograma (VCG) propiciou mais informações sobre os fenômenos elétricos cardíacos, possibilitando distinguir os casos potencialmente fatais dos benignos. A obtenção de um VCG ao mesmo tempo da realização do ECG trouxe um ganho muito importante para definir patologias extremamente sofisticadas, com alterações estruturais ou dinâmicas que envolvem canais e correntes iônicas, cujas funções são diminuídas ou exageradas em razão de mutações genéticas. Os aspectos clássicos da relação ECG/VCG no diagnóstico diferencial das áreas inativas, dos distúrbios de condução, das sobrecargas atriais e ventriculares e das associações entre esses distúrbios elétricos continuam vigentes e assertivos, e ficam mais claros quando vistos pelo binômio ECG/VCG. Além disso, a orientação espacial tridimensional das atividades atrial e ventricular cria uma ferramenta de observação muito mais completa do que o modo linear do ECG. A análise moderna do ECG e do respectivo VCG, obtidos simultaneamente pela técnica recente chamada de eletrovetorcardiograma (ECG/VCG), trouxe um ganho importante no diagnóstico diferencial de algumas patologias. Desse modo, são abordadas aqui as principais utilidades dessa análise na elucidação de diagnósticos importantes da atividade diária na clínica cardiológica.

Low levels of dehydroepiandrosterone sulfate are associated with the risk of developing cardiac autonomic dysfunction in elderly subjects

ABSTRACT Objective: To assess the relationships between serum dehydroepiandrosterone sulfate (DHEA-S) levels and heart rate variability (HRV) among different age groups. Subjects and methods: Forty-five healthy men were divided into 3 groups: young age (YA; 20-39 yrs; n = 15), middle age (MA; 40-59 yrs; n = 15) and old age (OA; ≥ 60 yrs; n = 15). Hemodynamic parameters, linear analyses of HRV and concentrations of cortisol and DHEA-S were measured at rest. Results: The OA group presented a higher resting heart rate (84.3 ± 4.6 bpm) than the YA group (72.0 ± 4.4 bpm; p < 0.05). The YA group showed an attenuated variance of HRV (2235.1 ± 417.9 ms2) compared to the MA (1014.3 ± 265.2 ms2; p < 0.05) and OA (896.3 ± 274.1 ms2; p < 0.05) groups, respectively. The parasympathetic modulation of HRV was lower in both the MA (244.2 ± 58.0 ms2) and OA (172.8 ± 37.9 ms2) groups in comparison with the YA group (996.0 ± 255.4 ms2; p < 0.05), while serum DHEA-S levels were significantly lower in both the MA (91.2 ± 19.6 mg/dL) and OA (54.2 ± 17.7 mg/dL) groups compared to the YA group (240.0 ± 50.8 mg/dL; p < 0.05). A positive correlation between lower serum concentrations of DHEA-S and attenuated variance of HRV (r = 0.47, p = 0.031), as well as lower serum concentrations of DHEA-S and decreased parasympathetic modulation of HRV (r = 0.54, p = 0.010), were found. Conclusion: The present study demonstrated that the decline of plasma DHEA-S is associated with reduced cardiac autonomic modulation during the aging process.

Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult

SUMMARY Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. In the first visit, he showed signs and symptoms of overt congestive heart failure and was referred to the emergency department. The electrocardiogram showed sinus tachycardia and low voltage criteria. Echocardiography showed biventricular hypertrophy with preserved ejection fraction, restrictive physiology with elevated filling pressures, thickened interatrial septum and atrioventricular valves, small pericardial effusion and relative "apical sparing" on 2D longitudinal strain. Cardiac MRI showed diffuse subendocardial late enhancement. Serum protein electrophoresis was inconclusive, however urine analysis revealed nephrotic range proteinuria, positive Bence Jones protein and an immunofixation test with a monoclonal lambda protein band. Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS complexes and typical echocardiography features should raise the suspicion for cardiac amyloidosis. Prognosis is dictated by the level of cardiac involvement; therefore, early diagnosis and treatment are crucial.

RESUMO A amiloidose cardíaca corresponde a uma miocardiopatia infiltrativa, resultante do depósito da proteína amiloide no miocárdio. Na amiloidose sistêmica primária (tipo AL), a proteína amiloide é composta por cadeias leves que resultam de discrasia dos plasmócitos, havendo envolvimento cardíaco em até 50% dos doentes. Apresentamos o caso de um homem de 43 anos, com queixas de edema periódico da língua e xerostomia, hemorragia gengival e hematúria há dois meses. Analiticamente havia a destacar anemia normocítica, trombocitopenia e um INR alto espontâneo, pelo que foi referenciado à consulta de Medicina Interna. Na primeira consulta, apresentou-se com sinais de insuficiência cardíaca congestiva franca, pelo que foi referenciado ao Serviço de Urgência. O eletrocardiograma demonstrou taquicardia sinusal e critérios de baixa voltagem. O ecocardiograma revelou hipertrofia biventricular com fração de ejeção preservada, fisiologia restritiva com elevação das pressões de enchimento, espessamento do septo interauricular e das válvulas auriculoventriculares, derrame pericárdico ligeiro e padrão de apical sparing no strain longitudinal 2D. Realizou ainda ressonância magnética cardíaca, que mostrou realce tardio subendocárdico difuso. A eletroforese das proteínas foi inconclusiva, contudo a análise da urina revelou proteinúria no espectro nefrótico, presença de proteína de Bence Jones e um teste de imunofixação com uma banda monoclonal de cadeias lambda. A biópsia da gordura abdominal foi negativa. Não obstante, foi realizada uma biópsia da medula óssea, verificando-se plasmocitose monoclonal lambda, o que confirmou o diagnóstico de amiloidose primária sistêmica. Este caso representa uma causa rara de insuficiência cardíaca no jovem adulto. A baixa voltagem no eletrocardiograma e os achados ecocardiográficos típicos devem fazer suspeitar de amiloidose cardíaca. O prognóstico é ditado pelo nível de envolvimento cardíaco, motivo pelo qual o diagnóstico e o tratamento precoces são essenciais.

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study / Análise da Deformação Atrial Direita na Amiloidose Cardíaca - Resultados de Ecocardiografia Tridimensional de Speckle-Tracking do Estudo MAGYAR-Path

Abstract Background: Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis. Objectives: To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE). Methods: A total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant. Results: Significant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients. Conclusion: Significantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

Resumo Fundamento: A amiloidose cardíaca (AC) de cadeias leves (AL) é caracterizada por depósitos fibrilares, que são compostos por cadeias leves de imunoglobulina monoclonal. O ventrículo direito é mais afetado pela AC-AL, e o comprometimento da sua função é preditor de um prognóstico pior. Objetivos: Caracterizar as propriedades volumétricas e funcionais do átrio direito (AD) na AC-AL por ecocardiografia tridimensional de speckle-tracking (3DSTE). Métodos: Um total de 16 pacientes (idade média: 64,5 ± 10,1 anos, 11 homens) com AC-AL foram examinados. Seus resultados foram comparados aos de 15 controles saudáveis pareados por idade e gênero (média de idade: 58,9 ± 6,9 anos, 8 homens). Todos os casos foram submetidos a Doppler bidimensional completo e 3DSTE. Um valor p bicaudal inferior a 0,05 foi considerado estatisticamente significativo. Resultados: Diferenças significativas foram demonstradas em volumes do AD com respeito ao ciclo cardíaco. O total (19,2 ± 9,3% vs. 27,9 ± 10,7%, p = 0,02) e as frações ativas de esvaziamento atrial (12,1 ± 8,1 vs. 18,6 ± 9,8%, p = 0,05) foram significativamente menores nos pacientes com AC-AL. Picos de strain no AD em áreas globais (16,7 ± 10,3% vs. 31,2 ± 19,4%, p = 0,01) e segmentares médias (24,3 ± 11,1% vs. 38,6 ± 17,6%, p = 0,01), juntamente com alguns parâmetros de strain por áreas circunferenciais, longitudinais e segmentares, mostraram-se menores em pacientes com AC-AL. Strains globais longitudinais (4,0 ± 5,2% vs. 8,2 ± 5,5%, p = 0,02) e por área (7,8 ± 8,1 vs. 15,9 ± 10,3%, p = 0,03) na contração atrial e alguns parâmetros de circunferência e de strain por área na contração atrial foram menores em pacientes com AC-AL. Conclusão: Foi possível demonstrar o aumento significativo dos volumes do AD e a deterioração de suas funções na AC-AL.

Global Longitudinal Strain or Left Ventricular Twist and Torsion? Which Correlates Best with Ejection Fraction? / Strain Longitudinal Global ou Twist do Ventrículo Esquerdo e Torção? Qual se Correlaciona Melhor com a Fração de Ejeção?

Abstract Background: Estimative of left ventricular ejection fraction (LVEF) is a major indication for echocardiography. Speckle tracking echocardiography (STE) allows analysis of LV contraction mechanics which includes global longitudinal strain (GLS) and twist/torsion, both the most widely used. Direct comparison of correlations between these novel parameters and LVEF has never been done before. Objective: This study aims to check which one has the highest correlation with LVEF. Methods: Patients with normal LVEF (> 0,55) and systolic dysfunction (LVEF <0,55) were prospectively enrolled, and underwent echocardiogram with STE analysis. Correlation of variables was performed by linear regression analysis. In addition, correlation among levels of LV systolic impairment was also tested. Results: A total of 131 patients were included (mean age, 46 ± 14y; 43%, men). LVEF and GLS showed a strong correlation (r = 0.95; r2 = 0.89; p < 0.001), more evident in groups with LV systolic dysfunction than those with preserved LVEF. Good correlation was also found with global longitudinal strain rate (r = 0.85; r2 = 0.73; p < 0.001). Comparing to GLS, correlation of LVEF and torsional mechanics was weaker: twist (r = 0.78; r2 = 0.60; p < 0.001); torsion (r = 0.75; r2 = 0.56; p < 0.001). Conclusion: GLS of the left ventricle have highly strong positive correlation with the classical parameter of ejection fraction, especially in cases with LV systolic impairment. Longitudinal strain rate also demonstrated a good correlation. GLS increments analysis of LV systolic function. On the other hand, although being a cornerstone of LV mechanics, twist and torsion have a weaker correlation with LV ejection, comparing to GLS.

Resumo Fundamento: A estimativa da fração de ejeção do ventrículo esquerdo (FEVE) é uma das pincipais indicações para a ecocardiografia. Speckle tracking (ST) permite a análise da mecânica de contração do VE que inclui a deformação (strain) longitudinal global (SLG) e o twist / torção, sendo ambos os mais utilizados. A comparação direta de correlações entre esses novos parâmetros e a FEVE nunca foi feita antes. Objetivo: Verificar qual delas tem a maior correlação com a FEVE. Métodos: Pacientes com FEVE normal (> 0,55) e disfunção sistólica (FEVE < 0,55) foram prospectivamente inscritos e submetidos a ecocardiograma com análise do ST. A correlação das variáveis foi realizada por análise de regressão linear. Além disso, também foi testada a correlação entre os níveis de comprometimento sistólico do VE. Resultados: Foram incluídos 131 pacientes (média de idade de 46 ± 14 anos e 43% de homens). A FEVE e SLG apresentaram forte correlação (r = 0,95; r2 = 0,89; p < 0,001), mais evidente em grupos com disfunção sistólica do VE do que naqueles com FEVE preservada. Também foi encontrada boa correlação com a taxa de deformação (strain rate) longitudinal (SRL) global (r = 0,85; r2 = 0,73; p < 0,001). Comparando-a à SLG, a correlação entre FEVE e mecânica de torção foi mais fraca: twist (r = 0,78; r2 = 0,60; p < 0,001); Torção (r = 0,75, r2 = 0,56, p < 0,001). Conclusão: A SLG do ventrículo esquerdo apresenta alta correlação positiva com o parâmetro clássico de fração de ejeção, principalmente nos casos de comprometimento sistólico do VE. O SRL também demonstrou uma boa correlação. A SLG incrementa a análise da função sistólica do VE. Por outro lado, apesar de ser a pedra angular da mecânica do VE, o twist e a torção têm uma correlação mais fraca com a ejeção do VE, quando comparados ao SLG.

Assessment of fatigue using the Identity-Consequence Fatigue Scale in patients with lung cancer / Avaliação da fadiga utilizando a Escala de Identificação e Consequências da Fadiga em pacientes com câncer de pulmão

ABSTRACT Objective: To evaluate the properties of the Identity-Consequence Fatigue Scale (ICFS) in patients with lung cancer (LC), assessing the intensity of fatigue and associated factors. Methods: This was a cross-sectional study involving LC patients, treated at a teaching hospital in Brazil, who completed the ICFS. Patients with chronic heart disease (CHD) and healthy controls, matched for age and gender, also completed the scale. Initially, a Brazilian Portuguese-language version of the ICFS was administered to 50 LC patients by two independent interviewers; to test for reproducibility, it was readministered to those same patients. At baseline, the LC patients were submitted to spirometry and the six-minute walk test, as well as completing the Epworth Sleepiness Scale (ESS), Hospital Anxiety and Depression Scale (HADS), Medical Outcomes Study 36-item Short-Form Health Survey (SF-36), and Fatigue Severity Scale (FSS). Inflammatory status was assessed by blood C-reactive protein (CRP) levels. To validate the ICFS, we assessed the correlations of its scores with those variables. Results: The sample comprised 50 patients in each group (LC, CHD, and control). In the LC group, the intraclass correlation coefficients for intra-rater and inter-rater reliability regarding ICFS summary variables ranged from 0.94 to 0.76 and from 0.94 to 0.79, respectively. The ICFS presented excellent internal consistency, and Bland-Altman plots showed good test-retest reliability. The ICFS correlated significantly with FSS, HADS, and SF-36 scores, as well as with CRP levels. Mean ICFS scores in the LC group differed significantly from those in the CHD and control groups. Conclusions: The ICFS is a valid, reliable instrument for evaluating LC patients, in whom depression, quality of life, and CRP levels seem to be significantly associated with fatigue.

RESUMO Objetivo: Avaliar as propriedades da Escala de Identificação e Consequências da Fadiga (EICF) em pacientes com câncer de pulmão (CP), analisando a intensidade da fadiga e fatores associados. Métodos: Estudo transversal com pacientes com CP, atendidos em um hospital-escola no Brasil, que preencheram a EICF. Pacientes com doenças cardíacas crônicas (DCC) e controles saudáveis, pareados por idade e sexo, também preencheram a escala. Inicialmente, uma versão brasileira da escala foi aplicada a 50 pacientes com CP por dois entrevistadores independentes; para testar a reprodutibilidade, ela foi reaplicada aos mesmos pacientes. No momento basal, os pacientes com CP realizaram espirometria e teste de caminhada de seis minutos, bem como preencheram a Epworth Sleepiness Scale (ESS), Hospital Anxiety and Depression Scale (HADS), Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) e Fatigue Severity Scale (FSS). O estado inflamatório foi avaliado pelos níveis de proteína C reativa (PCR) no sangue. Para validar a EICF, avaliamos as correlações entre as pontuações na mesma e essas variáveis. Resultados: A amostra foi composta por 50 pacientes em cada grupo (CP, DCC e controle). No grupo CP, os coeficientes de correlação intraclasse para confiabilidade intra e interobservador para as variáveis resumidas da EICF variaram de 0,94 a 0,76 e de 0,94 a 0,79, respectivamente. A EICF apresentou excelente consistência interna, e as disposições gráficas de Bland-Altman demonstraram boa confiabilidade teste-reteste. A EICF apresentou correlações significativas com as pontuações na FSS, HADS e SF-36, bem como com os níveis de PCR. As médias das pontuações na EICF do grupo CP diferiram significativamente das dos grupos DCC e controle. Conclusões: A EICF é um instrumento válido e confiável para a avaliação de pacientes com CP, nos quais depressão, qualidade de vida e níveis de PCR parecem estar significativamente associados à fadiga.

Dysfunctional hyperpolarization-activated cyclic nucleotide-gated ion channels in cardiac diseases

Abstract Hyperpolarization-activated cyclic nucleotide-gated (HCN) channels are reverse voltage-dependent, and their activation depends on the hyperpolarization of the membrane and may be directly or indirectly regulated by the cyclic adenosine monophosphate (cAMP) or other signal-transduction cascades. The distribution, quantity and activation states of HCN channels differ in tissues throughout the body. Evidence exhibits that HCN channels play critical roles in the generation and conduction of the electrical impulse and the physiopathological process of some cardiac diseases. They may constitute promising drug targets in the treatment of these cardiac diseases. Pharmacological treatment targeting HCN channels is of benefit to these cardiac conditions.

Overexpression of myeloid differentiation protein 88 in mice induces mild cardiac dysfunction, but no deficit in heart morphology

Cardiac remodeling involves changes in heart shape, size, structure, and function after injury to the myocardium. The proinflammatory adaptor protein myeloid differentiation protein 88 (MyD88) contributes to cardiac remodeling. To investigate whether excessive MyD88 levels initiate spontaneous cardiac remodeling at the whole-organism level, we generated a transgenic MyD88 mouse model with a cardiac-specific promoter. MyD88 mice (male, 20-30 g, n=∼80) were born at the expected Mendelian ratio and demonstrated similar morphology of the heart and cardiomyocytes with that of wild-type controls. Although heart weight was unaffected, cardiac contractility of MyD88 hearts was mildly reduced, as shown by echocardiographic examination, compared with wild-type controls. Moreover, the cardiac dysfunction phenotype was associated with elevation of ANF and BNP expression. Collectively, our data provide novel evidence of the critical role of balanced MyD88 signaling in maintaining physiological function in the adult heart.