Anomalía de Ebstein en recién nacido / Ebstein's anomaly in newborn

Introducción: La anomalía de Ebstein es una rara malformación con presentación sintomática respiratoria leve a grave a causa de defectos cardiacos congénitos manifestados durante la niñez. Objetivo: Exponer el caso en el cual el diagnóstico oportuno de esta enfermedad permitió el tratamiento integral del paciente. Presentación del caso: Recién nacido a término, quien a las siete horas de su nacimiento presentó saturación de oxígeno disminuida, examen físico sin cianosis, soplo pansistólico en la auscultación y cardiomegalia detectada por rayos X de tórax. Se realizó ecocardiograma que permitió diagnosticar al bebé como portador de la enfermedad de Ebstein con manifestaciones leves. Una vez que tiene mejoría clínica, se le proporcionó el alta médica con indicación de valoración por cardiología pediátrica. Conclusiones: La anomalía de Ebstein puede comenzar en el período neonatal con sintomatología de severidad variable. La sospecha clínica permite la confirmación diagnóstica y el seguimiento estrecho, sobre todo en casos severos(AU)

Introduction: Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood. Objective: To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient. Case presentation: Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology. Conclusions: Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases(AU)

Agenesia Pericárdica Completa. Presentación de una caso y revisión de la literatura / Complete pericardial agenesis. Presentation of a case and review of the literature

Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.

Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.

Correlação entre Cardiomegalia pela Radiografia de Tórax e Diâmetro do Ventrículo Esquerdo pela Ecocardiografia em Pacientes com Doença de Chagas / Correlation between Cardiomegaly on Chest X-Ray and Left Ventricular Diameter on Echocardiography in Patients with Chagas Disease

Resumo Fundamento Cardiomegalia pela radiografia de tórax (RXT) é preditor independente de morte em indivíduos com cardiomiopatia crônica da doença de Chagas (CCDC). Contudo, a correlação entre o aumento do índice cardiotorácico (ICT) na RXT e do diâmetro telediastólico do ventrículo esquerdo (DDVE) pela ecocardiografia (ECO) nessa população não está bem definida. Objetivos Analisar a relação entre cardiomegalia pela RXT e DDVE pela ECO em pacientes com doença de Chagas (DC) e sua aplicabilidade ao escore de Rassi. Métodos Estudo retrospectivo incluiu 63 pacientes ambulatoriais com DC avaliados por RXT e ECO. Cardiomegalia na RXT foi definida como ICT > 0,5. DDVE foi avaliado como variável contínua. Curva ROC foi utilizada para avaliar o potencial do DDVE para identificação de cardiomegalia pela RXT, com ponto de corte definido pela maior somatória de sensibilidade e especificidade. Resultados Idade mediana = 61 anos [intervalo interquartil: 48-68], 56% mulheres. CCDC foi identificada em 58 pacientes; 5 tinham a forma indeterminada da DC. Cardiomegalia foi detectada em 28 indivíduos. A área sob a curva ROC do DDVE para identificação de cardiomegalia foi de 0,806 (IC 95%: 0,692-0,919). O ponto de corte ótimo para DDVE foi de 60 mm (sensibilidade = 64%, especificidade = 89%). O uso do DDVE pela ECO em substituição ao ICT pela RXT alterou o escore de Rassi em 14 pacientes, e em 10 deles houve redução do risco presumido. Conclusão DDVE pela ECO é parâmetro adequado e com alta especificidade para distinguir entre presença e ausência de cardiomegalia na RXT na DC. (Arq Bras Cardiol. 2021; 116(1):68-74)

Abstract Background Cardiomegaly on chest X-ray is an independent predictor of death in individuals with chronic Chagas cardiomyopathy (CCC). However, the correlation between increased cardiothoracic ratio (CTR) on chest X-ray and left ventricular end-diastolic diameter (LVEDD) on echocardiography is not well established in this population. Objectives To assess the relationship between chest X-ray and LVEDD on echocardiography in patients with Chagas disease and its applicability to the Rassi score. Methods Retrospective study on 63 Chagas disease outpatients who underwent chest X-ray and echocardiography. Cardiomegaly on chest X-ray was defined as a CTR>0.5. LVEDD was analyzed as a continuous variable. ROC curve was used to evaluate the ability of LVEDD in detecting cardiomegaly by chest X-ray, with a cut-off point defined by the highest sum of sensitivity and specificity. Results Median age 61 years [interquartile range 48-68], 56% were women. CCC was detected in 58 patients, five patients had the indeterminate form of Chagas disease. Cardiomegaly was detected in 28 patients. The area under the ROC curve for LVEDD was 0.806 (95%CI: 0.692-0.919). The optimal cut-off for LVEDD was 60 mm (sensitivity = 64%, specificity = 89%). The use of LVEDD on echocardiography as a surrogate for CTR on chest X-ray changed the Rassi score values of 14 patients, with a reduction in the presumed risk in 10 of them. Conclusion LVEDD on echocardiography is an appropriate, highly specific parameter to distinguish between the presence and absence of cardiomegaly on chest X-ray in Chagas disease. (Arq Bras Cardiol. 2021; 116(1):68-74)

The role of left atrial enlargement and age in the prediction of recurrence in embolic strokes of undetermined source / El papel del crecimiento auricular izquierdo y la edad como predictores de recurrencia en infarto cerebral embólicos de origen indeterminado

Abstract Objectives: Left atrial disease is an independent risk factor for ischemic stroke and can be used to predict atrial fibrillation (AF). We examine whether left atrial enlargement (LAE) could predict stroke recurrence in patients with embolic stroke of undetermined source (ESUS). Materials and methods: Sixty-four patients with a confirmed diagnosis of ESUS were followed for a median of 22 months. Clinical data and echocardiogram findings were recorded. The echocardiogram interpretation was performed centrally and blindly. The Brown ESUS – AF score was used to categorize patients into high (human resource planning [HRP]: score > 2) and low-risk patients (non-HRP score 0-1). Stroke recurrence was the primary outcome. Results: The median age was 62 years (range: 22-85 years); and 33 (51.6%) were men. The median initial NIHSS score was three points (range: 0-27). Twelve (18.8%) patients were categorized as HRP. We found a significant tendency toward recurrence among HRP versus non-HRP patients. Three (25%) HRP versus 2 (3.8%) non-HRP experienced recurrence (OR: 8.3 95% CI 1.2-57; p=0.042); this association was related to severe atrial dilatation (OR: 14.5 95% CI 0.78-277, p = 0.02) and age > 75 years (OR: 12.7 95% CI 1.7-92.2, p = 0.03). We found no differences in recurrence in a univariate analysis. Conclusions: Patients with severe LAE who are 75 years old or older have a significant tendency to experience stroke recurrence.

Resumen Objetivos: La patología atrial izquierda es factor de riesgo independiente para infarto cerebral y puede utilizarse para predecir fibrilación auricular. Examinamos si el crecimiento aurícular izquierdo puede predecir recurrencia en pacientes con infarto embolico de origen indeterminado (ESUS). Materiales y métodos: Sesenta y cuatro pacientes con diagnóstico confirmado de ESUS fueron seguidos por una mediana de seguimiento de 22 meses. Registramos los datos clínicos y ecocardiográficos. La interpretación ecocardiográfica fue centralizada y cegada. La escala de Brown ESUS – AF fue utilizada para categorizar a los pacientes en riesgo alto (HRP puntaje > 2) y bajo riesgo (no-HRP: puntaje 0-1). El descenlace primario fue recurrencia de infarto cerebral. Resultados: Mediana de edad fue de 62 años (rango: 22-85 años); 33 (51.6%) fueron hombres. La mediana inicial de la escala de NIHSS fue de 3 putnos (rango de 0 a 27). 12 (18.8%) pacientes fueron de alto riesgo (HRP) y 52 (81.3%) de bajo riesgo (non- HRP). El grupo HRP mostró tendencia significatica hacia mayor recurrencia. Tres (25%) HRP versus 2 (3.8%) no-HRP experimentaron recurrencia (OR: 8.3 IC 95% 1.2-57; p = 0.042); esta asociación se relacionó con dilatación auricular severa (OR: 14.5 IC 95% 0.78-277, p = 0.02) y edad > 75 años (OR: 12.7 IC 95% 1.7-92.2, p = 0.03). En el análisis multivarioado, no encontramos significativas. Conclusiones: El crecimiento auricular izquierdo severo y la edad mayor de 75 años mostraron tendencia significativa a recurrencia de infarto cerebral.

Cardiac Alterations in Patients with Familial Lipodystrophy / Alterações Cardiovasculares em Pacientes Portadores de Lipodistrofia Familiar

Abstract Familial lipodystrophy is a rare genetic condition in which individuals have, besides metabolic changes and body fat deposits, a type of cardiomyopathy that has not been well studied. Many of the patients develop cardiovascular changes, the most commonly reported in the literature being the expression of a type of hypertrophic cardiomyopathy. This article, presented as a bibliographic review, reviews the clinical and cardiovascular imaging aspects in this scenario of cardiomyopathy in a rare metabolic disease, based on the latest scientific evidence published in the area. Despite the frequent association of congenital lipodystrophy and ventricular hypertrophy described in the literature, the pathophysiological mechanisms of this cardiomyopathy have not yet been definitively elucidated, and new information on cardiac morphological aspects is emerging in the aegis of recent and advanced imaging methods, such as cardiac magnetic resonance.

Resumo A lipodistrofia familiar é uma condição genética rara na qual indivíduos apresentam, além das alterações metabólicas e de depósitos de gordura físicos, um tipo de cardiomiopatia pouco estudada. Muitos dos pacientes desenvolvem alterações cardiovasculares, sendo a mais comumente reportada em literatura, a expressão de um tipo de cardiomiopatia hipertrófica. Este artigo, apresentado como uma revisão bibliográfica, revisa os aspectos clínicos e de imagem cardiovascular neste cenário de cardiomiopatia em doença metabólica rara, com base nas últimas evidências científicas publicadas na área. Apesar da frequente associação de lipodistrofia congênita e hipertrofia ventricular descrita em literatura, os mecanismos fisiopatológicos desta cardiomiopatia ainda não estão definitivamente elucidados, e novas informações do aspecto morfológico cardíaco surgem à égide de recentes e avançados métodos de imagem como a ressonância cardíaca magnética.

Correlação entre os achados radiográficos e ecocardiográficos sugestivos de aumento cardíaco em cães: 104 casos / Correlation between radiographic and ecocardiographic findings suggestive of cardiomegaly in 104 dogs

Radiografias torácicas e ecocardiogramas de 104 caninos foram avaliados e correlacionados quanto ao aumento das câmaras cardíacas. Os achados radiográficos foram correlacionados estatisticamente a fim de se estabelecer a acurácia do exame radiográfico na detecção do aumento cardíaco em comparação ao ecocardiográfico - padrão-ouro não invasivo. A correlação entre os achados radiográficos indicativos de aumento cardíaco e os índices ecocardiográficos mostrou-se fraca, significativa somente para VHS versus relação átrio esquerdo/aorta (r=0,3136), eixo curto versus relação átrio esquerdo/aorta (r=0,3813) e eixo curto versus velocidade da onda E (r=0,2021). A acurácia da radiografia na determinação subjetiva de aumento das câmaras cardíacas foi razoável, variando entre 72,1% e 80,8%. Em contrapartida, o VHS apresentou baixa acurácia (50,9%) na detecção de cardiomegalia.(AU)

Thoracic radiographs and echocardiograms of 104 canines were evaluated and correlated regarding cardiac chambers enlargement. The radiographic findings were statistically correlated in order to establish the accuracy of the radiographic examination in the detection of cardiac enlargement in comparison with the echocardiogram - non-invasive gold standard. The correlation between the radiographic findings indicative of cardiac enlargement and echocardiographic indexes was weak, significant only for VHS versus left atrium to aorta ratio (r= 0.3136), short axis versus left atrium to aorta ratio (r= 0, 3813) and short axis versus E wave velocity (r= 0.2021). The radiographic accuracy in the subjective determination of cardiac chamber enlargement was reasonable, ranging from 72.1% to 80.8%. On the other hand, VHS presented low accuracy (50.9%) in the detection of cardiomegaly.(AU)

Miocardiopatía dilatada secundaria a hipotiroidismo primario. Reporte de un caso / Dilated cardiomyopathy secondary to primary hypothyroidism. A case report

Resumen El vínculo entre el hipotiroidismo y varias enfermedades cardiovasculares va más allá de la asociación con los factores de riesgo vascular clásicos. Sin embargo, la miocardiopatía dilatada es una forma rara de presentación del hipotiroidismo. Se reporta el caso de una paciente femenina de 52 años que acude a urgencias por presentar tos húmeda, edema en miembros inferiores y disnea de esfuerzo, así como otros síntomas y signos típicos de hipotiroidismo. La radiografía de tórax revela cardiomegalia y el ecocardiograma informa ventrículo izquierdo dilatado, fracción de eyección disminuida y derrame pericárdico. La TSH estaba aumentada y T4 libre disminuida. Luego de comenzar tratamiento hormonal con levotiroxina se alcanzó la resolución de los síntomas y de las alteraciones radiológicas y ecocardiográficas. El presente caso resalta la importancia de realizar un interrogatorio exhaustivo y examen físico completo que aporten las pistas diagnósticas para sospechar el hipotiroidismo en pacientes con miocardiopatía dilatada.

ABSTRACT The link between hypothyroidism and several cardiovascular diseases goes beyond the association with classical vascular risk factors. However, dilated cardiomyopathy is a rare form of presentation of hypothyroidism. We report the case of a 52-year-old female patient who comes to the emergency room for having wet cough, lower limb edema and dyspnea on exertion, as well as other typical symptoms and signs of hypothyroidism. Chest x-ray reveals cardiomegaly and the echocardiogram reports dilated left ventricle, decreased ejection fraction and pericardial effusion. TSH was increased and free T4 was decreased. After starting hormonal treatment with levothyroxine, resolution of symptoms and radiological and echocardiographic alterations was achieved. The present case highlights the importance of a thorough interrogatory and complete physical examination that provide the diagnostic clues to suspect hypothyroidism in patients with dilated cardiomyopathy.

Fluid Collection in the Right Lateral Portion of the Superior Aortic Recess Mimicking a Right Mediastinal Mass: Assessment with Chest Posterior Anterior and MDCT

OBJECTIVE: We observed patients in whom the fluid collection in the right lateral portion of the superior aortic recess on computed tomography (CT) scans mimicked a right anterior mediastinal mass on chest PA radiographs. The purpose of this study was to assess chest PA and CT features of these patients. MATERIALS AND METHODS: All chest PA radiographs and CT scans in 9 patients were reviewed by two radiologists on a consensus basis; for the presence of pleural effusion, pulmonary edema and heart size on chest PA radiographs. For the portion of the fluid collection in the superior aortic recess (SAR), a connection between the right lateral portion of the SAR (rSAR) and posterior portion of the SAR (pSAR) on CT scans, and the distance between the right lateral margin of the rSAR and the right lateral margin of the superior vena cava. RESULTS: Fluid collection in the rSAR on CT scans caused a right anterior mediastinal mass or a bulging contour on chest PA radiographs in all women patients. All patients showed cardiomegaly, five patients had pleural effusion, and two patients had mild pulmonary edema. Further, eight patients showed a connection between the rSAR and the pSAR. CONCLUSION: The characteristic features of these patients are the right anterior mediastinal mass-like opacity due to fluid collection in the rSAR, are bulging contour with a smooth margin and cardiomegaly regardless of pulmonary edema on the chest PA radiographs, and fluid connection between the rSAR and the pSAR on CT scans.

Calculation of the Cardiothoracic Ratio from Portable Anteroposterior Chest Radiography

Cardiothoracic ratio (CTR), the ratio of cardiac diameter (CD) to thoracic diameter (TD), is a useful screening method to detect cardiomegaly, but is reliable only on posteroanterior chest radiography (chest PA). We performed this cross-sectional 3-phase study to establish reliable CTR from anteroposterior chest radiography (chest AP). First, CDChest PA/CDChest AP ratios were determined at different radiation distances by manipulating chest computed tomography to simulate chest PA and AP. CDChest PA was inferred from multiplying CDChest AP by this ratio. Incorporating this CD and substituting the most recent TDChest PA, we calculated the 'corrected' CTR and compared it with the conventional one in patients who took both the chest radiographies. Finally, its validity was investigated among the critically ill patients who performed portable chest AP. CDChest PA/CDChest AP ratio was {0.00099 x (radiation distance [cm])} + 0.79 (n = 61, r = 1.00, P < 0.001). The corrected CTR was highly correlated with the conventional one (n = 34, difference: 0.00016 +/- 0.029; r = 0.92, P < 0.001). It was higher in congestive than non-congestive patients (0.53 +/- 0.085; n = 38 vs 0.49 +/- 0.061; n = 46, P = 0.006). Its sensitivity and specificity was 61% and 54%. In summary, reliable CTR can be calculated from chest AP with an available previous chest PA. This might help physicians detect congestive cardiomegaly for patients undergoing portable chest AP.

Real-time three-dimensional echocardiographic assessment of left ventricular remodeling index in patients with hypertensive heart disease and coronary artery disease / 华中科技大学学报（医学）（英德文版）

Left ventricular remodeling index (LVRI) was assessed in patients with hypertensive heart disease (HHD) and coronary artery disease (CAD) by real-time three-dimensional echocardiography (RT3DE). RT3DE data of 18 patients with HHD, 20 patients with CAD and 22 normal controls (NC) were acquired. Left ventricular end-diastolic volume (EDV) and left ventricular end-diastolic epicardial volume (EDVepi ) were detected by RT3DE and two-dimensional echocardiography Simpson biplane method (2DE). LVRI (left ventricular mass /EDV) was calculated and compared. The results showed that LVRI measurements detected by RT3DE and 2DE showed significant differences inter-groups (P0.05), but significant difference in HHD and CAD intra-group (P0.05). It was concluded that LVRI derived from RT3DE as a new index for evaluating left ventricular remodeling can provide more superiority to LVRI derived from 2DE.

An increase of the cardiothoracic ratio leads to a diagnosis of Bart's hydrops.

BACKGROUND: Thalassemia is a common single gene disorder in Southeast Asia. a-thalassemia is a group of syndrome characterized by deficient production of the alpha-globin chain. Individuals with heterozygous alpha-thalassemia-1 are at risk of having a fetus that has Hemoglobin Bart's hydrops fetalis (Hb Bart's). Usually, when the hemoglobin electrophoresis in heterozygous alpha-thalassemia-1 is normal, the Mean Corpuscular Volume (MCV) is lowered. We report a case of increased cardiothoracic ratio that led to a diagnosis of Hb Bart's in a couple who had normal hemoglobin electrophoresis and low MCV. CASE REPORT: A 23-year-old woman, gravida 2, Para 0-0-1-0, initially presented for antenatal care at 13 weeks pregnancy. Her MCV was 67 fentolitre, DiChlorophenol-IndolPhenol (DCIP) test was negative and hemoglobin electrophoresis was normal. Her husbands MCV was 67 fentolitre, and hemoglobin electrophoresis was normal. Cardiomegaly (an increased of the cardiothoracic ratio) was detected by ultrasonogram at 25 weeks of gestation. She and her husband were comprehensively counseled after an Hb Bart's was suspected. A cordocentesis was performed and the fetal blood was tested for hemoglobin electrophoresis. The result was later known and confirmed as Hb Bart's. The couple decided to terminate the pregnancy. The induced abortion was successful and the patient was discharged on the second day after the abortion. She was well at the 4-week follow-up. CONCLUSION: A prenatal ultrasonographic screening should be conducted in couples who are suspected of being alpha-thalassemia-1 carriers when DNA study of alpha-globin gene cannot be performed. The increase of cardiothoracic ratio will help detect an early stage of Hb Bart's.

Left ventricular muscle mass by echocardiography: methodology.

The limitations of electrocardiography for diagnosing left ventricular hypertrophy (LVH), due to unacceptable accuracy and lack of serial quantifications, are well known. The use of angiocardiography for LVH assessment is invasive, hazardous and costly. Echocardiography provides an excellent method of estimation of left ventricular muscle mass, which is simple, non-hazardous, accurate and reproducible.