Mapeamento do Potencial da Superfície Corporal durante a Despolarização Ventricular em Atletas com Intervalo PQ Prolongado após Exercício / Body Surface Potential Mapping during Ventricular Depolarization in Athletes with Prolonged PQ Interval after Exercise

Resumo Fundamento: O prolongamento do intervalo PQ, geralmente associado a um atraso na condução atrioventricular, pode estar relacionado a alterações na propagação do impulso intraventricular. Objetivo: Avaliar, por meio do mapeamento do potencial de superfície corporal (BSPM), o processo de despolarização ventricular em atletas com intervalos PQ prolongados em repouso e após o exercício. Métodos: O estudo incluiu 7 esquiadores cross-country com intervalo PQ superior a 200 ms (grupo PQ Prolongado) e 7 com intervalo PQ inferior a 200 ms (grupo PQ Normal). O BSPM de 64 derivações unipolares do tronco foi realizado antes (Pré-Ex) e após o teste ergométrico de bicicleta (Pós-Ex). Mapas equipotenciais da superfície corporal foram analisados durante a despolarização ventricular. O nível de significância foi de 5%. Resultados: Comparado com atletas com PQ Normal, o primeiro e o segundo períodos de posição estável dos potenciais cardíacos na superfície do tronco foram mais longos, e a formação da distribuição de potencial "sela" ocorreu mais tarde, no Pré-Ex, nos atletas com PQ Prolongado. No Pós-Ex, o grupo PQ Prolongado apresentou um encurtamento do primeiro e segundo períodos de distribuições de potencial estáveis e uma diminuição no tempo de aparecimento do fenômeno "sela" em relação ao Pré-Ex (para valores próximos aos do Normal -Grupo PQ). Além disso, no Pós-Ex, a primeira inversão das distribuições de potencial e a duração total da despolarização ventricular em atletas com PQ Prolongado diminuíram em comparação com o Pré-Ex e com valores semelhantes em atletas com PQ Normal. Em comparação com atletas com PQ Normal, a segunda inversão foi mais longa no Pré-Ex e Pós-Ex em atletas com PQ Prolongado. Conclusão: Atletas com PQ prolongado apresentaram diferenças significativas nas características temporais do BSPM durante a despolarização ventricular, tanto em repouso quanto após o exercício, em comparação com atletas com PQ normal.

Abstract Background: Prolongation of the PQ interval, generally associated with an atrioventricular conduction delay, may be related to changes in intraventricular impulse spreading. Objective: To assess, using body surface potential mapping (BSPM), the process of ventricular depolarization in athletes with prolonged PQ intervals at rest and after exercise. Methods: The study included 7 cross-country skiers with a PQ interval of more than 200 ms (Prolonged-PQ group) and 7 with a PQ interval of less than 200 ms (Normal-PQ group). The BSPM from 64 unipolar torso leads was performed before (Pre-Ex) and after the bicycle exercise test (Post-Ex). Body surface equipotential maps were analyzed during ventricular depolarization. The significance level was 5%. Results: Compared to Normal-PQ athletes, the first and second periods of the stable position of cardiac potentials on the torso surface were longer, and the formation of the "saddle" potential distribution occurred later, at Pre-Ex, in Prolonged-PQ athletes. At Post-Ex, the Prolonged-PQ group showed a shortening of the first and second periods of stable potential distributions and a decrease in appearance time of the "saddle" phenomenon relative to Pre-Ex (to the values near to those of the Normal-PQ group). Additionally, at Post-Ex, the first inversion of potential distributions and the total duration of ventricular depolarization in Prolonged-PQ athletes decreased compared to Pre-Ex and with similar values in Normal-PQ athletes. Compared to Normal-PQ athletes, the second inversion was longer at Pre-Ex and Post-Ex in Prolonged-PQ athletes. Conclusion: Prolonged-PQ athletes had significant differences in the temporal characteristics of BSPM during ventricular depolarization both at rest and after exercise as compared to Normal-PQ athletes.

Uncontrolled Diabetes with Underlying Structural Changes in the Atrioventricular Node / Diabetes no Controlada con Cambios Estructurales Subyacentes en el Nodo Atrioventricular

SUMMARY: Diabetic cardiomyopathy, characterized by diabetes mellitus (DM) -induced cardiac muscular abnormalities, is a strong inducer of impaired cardiac contraction and arrhythmia. Atrioventricular block, a serious type of arrhythmia resulting from interruption of cardiac impulse conduction via the atrioventricular node (AVN), frequently occurs among diabetic patients. However, details of structural changes in AVN in DM remain poorly explained. Here, this study defined the effects of DM on the morphological remodeling of the AVN in male Sprague Dawley rats induced by intraperitoneal injection of streptozotocin (60 mg/kg body weight). At 24 weeks, the pathological changes in the AVN were assessed by light microscopy (LM) and transmission electron microscopy (TEM). Under LM, the AVN in diabetic rats became a less compact mass and exhibited the intracellular vacuolation. The nodal cells were more varied in sizes with the absence or shrinkage of nuclei and clear cytoplasm compared to the control. The collagen content significantly increased in relation to the presence of myofibroblasts. Consistent with LM, TEM images of the diabetic nodal cells revealed several signs of cell damage, such as mitochondrial changes, deterioration of cell organelles, gap junction internalization, and cell separation. Furthermore, changes in AVN innervation, evidenced by damaged Schwann cells and axons, were also found. These results indicated alterations in important components in the AVN during diabetic condition, which may lead to the impairment of electrical conduction, causing abnormal cardiac functions in diabetic patients.

La miocardiopatía diabética, caracterizada por anomalías musculares cardíacas inducidas por diabetes mellitus (DM), es un fuerte inductor de alteración de la contracción cardíaca y arritmia. El bloqueo atrioventricular, un tipo grave de arritmia resultante de la interrupción de la conducción del impulso cardíaco a través del nodo atrioventricular (NAV), se produce con frecuencia entre los pacientes diabéticos. Sin embargo, los detalles de los cambios estructurales en NAV en DM siguen estando pobremente explicados. Aquí, este estudio definió los efectos de la DM en la remodelación morfológica del NAV en ratas macho Sprague Dawley inducidas por inyección intraperitoneal de estreptozotocina (60 mg/kg de peso corporal). A las 24 semanas, los cambios patológicos en el NAV se evaluaron mediante microscopía óptica (MO) y microscopía electrónica de transmisión (MET). Bajo MO, el NAV en ratas diabéticas se convirtió en una masa menos compacta y exhibió la vacuolización intracelular. Las células nodales tenían tamaños más variados con ausencia o contracción de núcleos y citoplasma claro en comparación con el control. El contenido de colágeno aumentó significativamente en relación con la presencia de miofibroblastos. De acuerdo con MO, las imágenes MET de las células nodales diabéticas revelaron varios signos de daño celular, como cambios mitocondriales, deterioro de los orgánulos celulares, internalización de uniones comunicantes y separación celular. Además, también se encontraron cambios en la inervación del NAV, evidenciados por schwannocitos y axones dañados. Estos resultados indicaron alteraciones en componentes importantes en el NAV durante la condición diabética, lo que puede conducir al deterioro de la conducción eléctrica, causando funciones cardíacas anormales en estos pacientes.

Comportamiento del tiempo de conducción retrógrada al momento de la inducción de las taquicardias supraventriculares y su rol en el diagnóstico diferencial / Behavior of Retrograde Conduction Time at Supraventricular Tachycardia Induction and its Role in Differential Diagnosis

RESUMEN Antecedentes : El diagnóstico diferencial entre la taquicardia reentrante ortodrómica (TRO) y la taquicardia por reentrada nodal atípica (TRNa) puede ser dificultoso. Nuestra hipótesis es que las TRNa tienen más variabilidad en el tiempo de con ducción retrógrada al comienzo de la taquicardia que las TRO. Nuestros objetivos fueron evaluar la variabilidad en el tiempo de conducción retrógrada al inicio de la taquicardia en TRNa y TRO, y proponer una nueva herramienta diagnóstica para diferenciar estas dos arritmias. Métodos : Se midió el intervalo ventrículo-auricular (VA) de los primeros latidos tras la inducción de la taquicardia, hasta su estabilización. La diferencia entre el intervalo VA máximo y el mínimo se definió como delta VA (ΔVA). También contamos el número de latidos necesarios para que se estabilice el intervalo VA. Se excluyeron las taquicardias auriculares. Resultados : Se incluyeron 101 pacientes. Se diagnosticó TRO en 64 pacientes y TRNa en 37. El ΔVA fue 0 (rango intercuartílico, RIC, 0-5) milisegundos (ms) en la TRO frente a 40 (21-55) ms en la TRNa (p < 0,001). El intervalo VA se estabilizó significativamente antes en la TRO (1,5 [1-3] latidos) que en la TRNa (5 [4-7] latidos; p < 0,001). Un ΔVA < 10 ms diagnosticó TRO con 100% de sensibilidad, especificidad y valores predictivos positivo y negativo. La estabilización del intervalo VA en menos de 3 latidos predijo TRO con buena precisión diagnóstica. Los resultados fueron similares considerando sólo vías accesorias septales. Las TRN típicas tuvieron una variación intermedia. Conclusión : Un ΔVA < 10 ms es un criterio simple, que distingue con precisión la TRO de la TRNa, independientemente de la localización de la vía accesoria.

ABSTRACT Background : Differential diagnosis between orthodromic reentrant tachycardia (ORT) and atypical nodal reentrant tachy cardia (ANRT) can be challenging. Our hypothesis was that ANRT presents more variability in retrograde conduction time at tachycardia onset than ORT. Objectives : The objectives of this study were to assess retrograde conduction time variability at the start of tachycardia in ANRT and ORT, and postulate a new diagnostic tool to differentiate these two types of arrhythmias. Methods : The ventriculoatrial (VA) interval of the first beats after tachycardia induction was measured until stabilization. The difference between the maximum and minimum VA interval was defined as delta VA (ΔVA), and the number of beats needed for VA interval stabilization was also assessed. Atrial tachycardias were excluded. Results : In a total of 101 patients included in the study, ORT was diagnosed in 64 patients and ANRT in 37. ΔVA interval was 0 (interquartile range [IQR] 0-5) milliseconds (ms) in ORT vs. 40 (21-55) ms in ANRT (p <0.001). The VA interval significantly stabilized earlier in ORT (1.5 [1-3] beats) than in ANRT (5 [4-7] beats) (p<0.001). A ΔVA <10 ms diagnosed ORT with 100% sensitivity, specificity, and positive and negative predictive values. Ventriculoatrial interval stabilization in less than 3 beats predicted ORT with good diagnostic accuracy. The results were similar considering only accessory septal pathways. Typical NRTs presented an intermediate variation. Conclusion : Presence of DVA <10 ms is a simple criterion that accurately differentiates ORT from ANRT, independently of the accessory pathway localization.

Partial Atrioventricular Septal Defect with Left Atrioventricular Valve Aneurysm Mimicking Valve Perforation

ABSTRACT Left atrioventricular valve aneurysm is a rare condition. Here we present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. Preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the "cleft" and leaflet perforation. But we discovered a left sided atrioventricular valve aneurysm instead of a valve perforation. The "cleft" edge and the aneurysm were closed.

Cisto Hidático Cardíaco: Uma Causa Incomum de Bloqueio Atrioventricular Total / Cardiac Hydatid Cyst: An Uncommon Cause of Complete Atrioventricular Block

Resumo A hidatidose é uma zoonose causada pelo Echinococcus granulosus, levando à formação de cistos nos órgãos acometidos. O envolvimento cardíaco é raro e pode causar diversas complicações secundárias à ruptura, embolização ou compressão. Seu diagnóstico é desafiador, sendo confirmado por meio de dados relacionados a manifestações clínicas, exposição ambiental, exames laboratoriais e de imagem. A ressecção cirúrgica é necessária na maioria dos casos, sendo indicada a associação com terapia antiparasitária. No presente artigo, descreve-se um caso de cisto hidático cardíaco associado a bloqueio atrioventricular total em paciente jovem, com necessidade de implante de marcapasso, uma apresentação atípica e pouco relatada na literatura.

Abstract Hydatidosis is a zoonosis caused by Echinococcus granulosus, leading to the formation of cysts on involved organs. Cardiac involvement is rare and can cause a wide range of complications secondary to rupture, embolization, or compression. Its diagnosis is challenging, and is generally confirmed through data related to clinical manifestations, environmental exposure, and laboratory and imaging exams. Surgical removal is necessary in most cases, in which an association with antiparasite therapy is recommended. The present article describes a case of a cardiac hydatid cyst associated with a complete atrioventricular block (AVB) in a young adult patient, with the need for a pacemaker implant, an atypical presentation, and scarce reports in the literature.

Early and mid-term results of surgical treatment for complete atrioventricular septal defect / 中华胸心血管外科杂志

Objective:To summarize the results of surgical treatment for complete atrioventricular septal defect(CAVSD) in early and middle stages.Methods:147 children with CAVSD in Guangzhou Women and Children’s Medical Center from January 2010 to December 2019 were selected, Males 85, females 62, median age of surgery 5 months(1 months-10 years old), median body mass 5.5 kg(2.4-20.9 kg). Complete atrioventricular septal defect was diagnosed by ultrasonic cardiogram before surgery. All the children underwent atrial ventricular valve formation and underwent simultaneous repair.Outpatient follow-up was planned.Ultrasonic cardiogram and electrocardiogram were performed. SPSS 22 statistical software was used for data analysis.Results:All 147 CAVSD patients underwent one-time surgical correction.Early postoperative death occurred in 7 cases(4.76%). The causes of death were: 3 cases of pulmonary hypertension crisis, 3 cases of severe mitral insufficiency(MI), 1 case of postoperative malignant arrhythmia, and the rest of the children were cured and discharged. Permanent pacemaker was installed in 3 patients due to atrioventricular block(AVB). The follow-up time was 1-10 years old, and 2 patients died in late stage: 1 patient did not seek medical treatment in time due to infection, and 1 patient had unknown cause. Five patients underwent secondary surgery: 4 due to severe mitral/tricuspid insufficiency(MI/TI) and 1 due to delayed AVB. The mid-term follow-up showed 9 cases of severe MI and 4 cases of severe TI. Compared with children with surgical age<3 months and ≥3 months, there were statistically significant differences in postoperative ventilator-assisted ventilation time, severe MI before postoperative discharge and total mortality between the two groups( P<0.05). Mid-term follow-up results showed no difference between the two groups. There were statistically significant differences in surgical age, postoperative CICU stay time and total hospital stay between the children with trisomy 21-syndrome and those without trisomy 21-syndrome( P<0.05), and there was no difference between the two groups in mid-term follow-up results. Residual shunt of 1-3 mm VSD was found in 29 cases, 26 cases were closed during follow-up, and 3 cases had smaller residual shunt. Conclusion:Modified single patch technique treatment of CAVSD has good effect, low mortality and low re-operation rate. But age <3 months group, infant mortality was significantly increased, the duration of postoperative mechanical assisted ventilation was prolonged, and the proportion of early postoperative severe MI was high.Severe MI and TI is easy to occur after CAVSD, which requires long-term follow-up and timely treatment. The children with trisomy 21-syndrome were similar to those with normal chromosome except for longer stay in ICU and total hospital stay.

Clinical analysis of 17 cases of neonatal lupus erythematosus / 中国小儿急救医学

Objective:To summarize the clinical features of neonatal lupus erythematosus(NLE) and improve the understanding of the disease.Methods:The clinical data of 17 NLE neonates admitted to the First Affiliated Hospital of Zhengzhou University from February 2015 to September 2021 were retrospectively analyzed, and relevant literatures were reviewed.Results:Of the 17 patients, nine were boys and eight were girls.There were 13 cases of skin damage and eight cases of heart damage, including four cases of atrioventricular block, one case of QT interval prolongation complicated with atrial premature beat, three cases of atrial septal defect, one case of ventricular septal defect, two cases of patent ductus arteriosus, one case of pericardial effusion, one case of pulmonary hypertension, and two cases of cardiac insufficiency.One case developed skin damage and heart block at the same time.There were 15 patients with blood system damage and 11 patients with hepatobiliary system damage.Among the serological indicators, 17 cases were positive for anti-ANA antibody, 12 cases were positive for anti-SSA-60, 13 cases were positive for anti-SSA-52, seven cases were positive for SSB, one case was positive for Sm, and three cases were positive for RNP.Among them, four neonates with atrioventricular block were positive for anti-SSA-60 and anti-SSA-52.Most of the patients with skin lesions had good prognosis, and only one patient had brown scars, one neonate with second-degree type Ⅱ atrioventricular block and one neonate with QT interval prolongation combined with premature atrial contractions had normal electrocardiograms.Additionally, two patients still had third-degree atrioventricular block, and the other one patient was installed with a pacemaker at one year and two months, and left spastic cerebral palsy.A total of 16 mothers had serological tests, among which ten cases had abnormal connective tissue antibodies before delivery and six cases had abnormal connective tissue antibodies after delivery.Conclusion:There is no significant gender difference in NLE, third-degree atrioventricular block is difficult to reverse, and most patients with extracardiac damage have a good prognosis.

A Case of Third-degree Atrioventricular Block after Cardiac Radiofrequency Ablation Treated with the Method of Boosting Qi， Warming Yang and Unblocking Collaterals / 中医杂志

Third-degree atrioventricular block is a severe bradyarrhythmia， for which there is no proven effective drugs currently. Permanent pacemaker implantation recommended by the guideline， however， is not suitable for most patients. This paper reported on a case of third-degree atrioventricular block after cardiac radiofrequency ablation who has been treated with the method of boosting qi， warming yang and unblocking collaterals. The TCM syndrome of this case was diagnosed as yang qi depletion and phlegm-stasis blocking the collaterals， for which Baoyuan Decoction and Mahuang Fuzi Xixin Decoction （保元汤合麻黄附子细辛汤） in modification has been used to boost qi， warm yang and raise the sunken， dissolve phlegm， invigorate blood and unblock collaterals. After nearly 7-month treatment， the symptoms such as palpitations， shortness of breath and fatigue were basically cured， and the electrocardiogram returned to the normal.

A Case of Intermediate Type Atrioventricular Septal Defect Associated with a Membranous Ventricular Septal Aneurysm in a 70-Year-Old Female Patient / 日本心臓血管外科学会雑誌

The case was a 70-year-old female. Atrioventricular septal defect was diagnosed in her childhood, however, surgical treatment had not been performed. The patient had suffered from heart failure at the age of 69, and she was referred to our hospital for treatment. Her diagnosis was intermediate type atrioventricular septal defect, moderate left atrioventricular valve regurgitation, membranous ventricular septal aneurysm and atrial flutter. An autologous pericardial patch was used to close the ostium primum type atrial septal defect associated with simultaneous covering of membranous ventricular septal aneurysmal wall. Concomitant left and right atrioventricular valvuloplasty and arrhythmia surgery were performed. Her postoperative course was uneventful and the patient was discharged from our department on the 16th postoperative day. To our knowledge, there are few reports of surgery for incomplete type atrioventricular septal defect in the elderly and no report for intermediate type atrioventricular septal defect in Japan. In incomplete type atrioventricular septal defect, symptoms such as supraventricular arrhythmia and heart failure develop according to aging. Reported surgical results in the elderly are quite good, and improvement of excise tolerance is expected. Precise evaluation and proper indication of surgical treatment is mandatory even in older patients.

A Case of Neonatal Marfan Syndrome Requiring Double Atrioventricular Valve Replacement due to Rapid Exacerbation of the Repaired Valves / 日本心臓血管外科学会雑誌

Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.

Implante de marcapasos a través de la vena cava superior izquierda persistente por ausencia de vena cava superior derecha. Reporte de un caso / Pacemaker implantation through the persistent left superior vena cava due to the absence of the right superior vena cava. Report of a case / Implante de marcapasso pela veia cava superior esquerda persistente devido à ausência da veia cava superior direita. Relato de um caso

Resumen: Introducción: la persistencia de la vena cava superior izquierda (PVCSI) tiene una prevalencia de 0.21% en la población general. Presentación del caso: masculino de 67 años de edad diagnosticado con bloqueo auriculoventricular (AV) de tercer grado y que requirió colocación de marcapasos; sin embargo, debido a la ausencia de la vena cava superior derecha, corroborado por angiotomografía, se colocó marcapasos definitivo a través de la vena cava superior izquierda persistente. Conclusión: la persistencia de la vena cava superior izquierda (PVCSI) es la anomalía venosa torácica más común. Puede causar una serie de síntomas clínicamente significativos, incluso en un corazón con anatomía normal. La colocación de marcapasos dificulta la fijación del electrodo debido al recorrido tortuoso que genera.

Abstract: Introduction: the persistence of the left superior vena cava (PLSVC) has a prevalence of 0.21% in the general population. Case presentation: 67-year-old male diagnosed with third-degree AV block, requiring pacemaker placement. However, due to the absence of a right superior vena cava, corroborated by CT angiography, a definitive pacemaker was placed through a persistent left superior vena cava. Conclusion: the persistence of the left superior vena cava (PLSVC) is the most common thoracic venous anomaly. It can cause a number of clinically significant symptoms, even in a heart with normal anatomy. Pacemaker placement makes electrode fixation difficult due to tortuous travel.

Resumo: Introdução: a persistência da veia cava superior esquerda (PVCSI) tem uma prevalência de 0.21% na população geral. Apresentação do caso: Homem de 67 anos com diagnóstico de bloqueio AV de terceiro grau, necessitando de colocação de marcapasso. No entanto, devido à ausência da veia cava superior direita, corroborada pela angiotomografia, colocou-se marcapasso definitivo através da veia cava superior esquerda persistente. Conclusão: a persistência da veia cava superior esquerda (PVCSI) é a anomalia venosa torácica mais comum. Pode causar uma série de sintomas clinicamente significativos, mesmo em um coração com anatomia normal. A colocação do marcapasso dificulta a fixação do eletrodo devido ao trajeto tortuoso.

El electrocardiograma en la amiloidosis cardíaca: un arma para la sospecha diagnóstica de esta enfermedad / The electrocardiogram in cardiac amyloidosis: a weapon for the diagnosis suspect of this disease / O electrocardiograma na amiloidose cardíaca: uma arma para a suspeita diagnóstica desta doença

La amiloidosis cardíaca (AC) es una enfermedad con mal pronóstico si el tratamiento no se inicia de forma temprana, por lo que una de las asignaturas pendientes en esta enfermedad consiste en realizar un diagnóstico precoz. El electrocardiograma (ECG) es una prueba diagnóstica de bajo costo y amplia disponibilidad que nos permite sospechar esta enfermedad, dado que resulta normal en < 5% de los pacientes. El hallazgo clásico es la presencia de bajos voltajes en relación con la gran hipertrofia que se observa en las pruebas de imagen, así como el conocido patrón de pseudoinfarto. Ambos hallazgos son más frecuentes en el subtipo de amiloidosis por cadenas ligeras, que era el más frecuentemente diagnosticado en el pasado. Sin embargo, con la expansión del diagnóstico no invasivo del subtipo a transtiretina, su identificación ha crecido de forma exponencial y se convirtió en el más diagnosticado con más frecuencia en nuestro medio. Se debe prestar especial atención a todos estos hallazgos electrocardiográficos, con el fin de que esta prueba diagnóstica de sencilla obtención pueda contribuir de forma importante a la sospecha y al diagnóstico precoz de la AC.

Cardiac amyloidosis (CA) is a serious disease with a poor prognosis if treatment is not started early, so one of the pending issues in this condition is to make an early diagnosis. The electrocardiogram (EKG) is an inexpensive and widely available diagnostic test that can offer differential data when suspecting this disease, being normal in < 5% of these patients. The classic EKG finding is the presence of low voltages in relation to the large hypertrophy seen on imaging tests, as well as the well-known pseudoinfarct pattern. Both findings are more frequent in the light chain subtype of CA, which was the most frequently diagnosed in the past. However, with the growth of noninvasive diagnostic tests, the identification of the transtyretin subtype has grown exponentially, becoming the most frequently diagnosed in our setting. Special attention should be paid to all these electrocardiographic findings, so that this simple diagnostic test can make an important contribution to the early suspicion and diagnosis of CA.

A amiloidose cardíaca (AC) é uma doença grave com um mau prognóstico no caso de não se iniciar tratamento de forma precoce, pelo que a necessidade de um pronto diagnóstico é imperiosa. Quando se suspeita desta doença, o eletrocardiograma (ECG) é um teste de diagnóstico pouco dispendioso e disponível em todo o mundo, que pode fornecer dados discriminativos importantes, sendo normal em menos de 5% dos casos. O achado clássico do ECG é a presença de baixas voltagens em relação à grande hipertrofia vista na imagem, bem como o conhecido padrão de pseudoinfarte. Ambos os resultados são mais frequentes no subtipo a cadenas ligeras, o mais frequentemente diagnosticado no passado. No entanto, com o aumento dos testes de diagnóstico não-invasivos, o diagnóstico ddo subtipo a transtirretina, o mais cresceu de forma exponencial, tornando-se o mais frequentemente diagnosticado no nosso meio. Deve ser dada especial atenção a todos estes achados eletrocardiográficos já que esta prova de diagnóstico de fácil obtenção pode contribuir de forma importante para a suspeição de diagnóstico precoce de AC.

Diagnóstico prenatal de enfermedad de canal atrio-ventricular, análisis epidemiológico y experiencia CERPO entre 2003 y 2021 / Prenatal diagnosis of atrio-ventricular canal disease, epidemiological analysis and CERPO experience between 2003 and 2021

Antecedentes: En Chile, la mitad de los casos de mortalidad perinatal son atribuibles a anomalías congénitas, y un tercio de estas corresponde a cardiopatías congénitas. Aproximadamente un 35% de estos últimos requerirán cirugía antes del año de vida, por lo que la pesquisa prenatal impacta profundamente en el pronóstico. Objetivo: Dar a conocer los resultados perinatales de pacientes con diagnóstico prenatal de canal atrio-ventricular controlados en el Centro de Referencia Perinatal Oriente (CERPO) entre los años 2003 y 2021, su asociación a otras anomalías, características demográficas y pronóstico a un año. Métodos: En este estudio se puede apreciar que tanto el pronóstico como el plan terapéutico posnatal dependerán de la presencia de otras alteraciones morfológicas y del estudio genético. De los factores estudiados, se puede concluir que tanto la presencia de anomalías cardiacas asociadas, como el grado de insuficiencia valvular y el tipo de canal no son predictores de la sobrevida perinatal. Conclusiones: Finalmente, en relación con la sobrevida posnatal, en este estudio, la sobrevida a un año de los recién nacidos vivos fue de un 52%, pero al desglosarlo en los niños con cariotipo euploide y trisomía 21, estos valores se tornan muy distintos, 44 y 81% respectivamente.

Background: In Chile, half of the perinatal mortality cases are attributable to congenital anomalies, and one third of these correspond to congenital heart disease. Approximately 35% of the later will require surgery before one year of life, so prenatal screening has a profound impact on the prognosis. Objective: To present the perinatal results of patients with a prenatal diagnosis of atrio-ventricular canal controlled at Centro de Referencia Perinatal Oriente CERPO) between 2003 and 2021, its association with other anomalies, demographic characteristics, and 1-year prognosis. Methods: In this study it can be seen that both the prognosis and the postnatal therapeutic plan will depend on the presence of other morphological alterations and the genetic study. From the factors studied, it can be concluded that the presence of associated cardiac anomalies, the degree of valvular insufficiency, and the type of canal are not predictors of perinatal survival. Conclusions: Finally, in relation to postnatal survival, in this study, the 1-year survival of live newborns was 52%, but when broken down into children with euploid karyotype and trisomy 21, these values become very different, 44 and 81% respectively.

Flutter Atrial na Síndrome do PRKAG2: Características Clínicas e Eletrofisiológicas / Atrial Flutter in PRKAG2 Syndrome: Clinical and Electrophysiological Characteristics

Resumo Fundamento A síndrome do PRKAG2 é uma rara doença genética autossômico dominante, fenocópia da miocardiopatia hipertrófica, caracterizada pelo acúmulo intracelular de glicogênio. Manifestações clínicas incluem pré-excitação ventricular, hipertrofia ventricular, distúrbio de condução cardíaca e arritmias atriais. Objetivo Comparar características clínicas e eletrofisiológicas observadas em pacientes com flutter atrial, com e sem síndrome do PRKAG2. Métodos Estudo observacional, comparativo de pacientes com flutter atrial: grupo A, cinco pacientes de família com síndrome do PRKAG2; e grupo B, 25 pacientes sem fenótipo da síndrome. O nível de significância foi de 5%. Resultados Todos os pacientes do grupo A apresentaram pré-excitação ventricular e bloqueio de ramo direito; quatro tinham marca-passo (80%). Pacientes do grupo A tinham menor idade (39±5,4 vs. 58,6±17,6 anos, p=0,021), e maior espessura de septo interventricular (mediana=18 vs. 10 mm; p<0,001) e parede posterior (mediana=14 vs. 10 mm; p=0,001). Quatro do grupo A foram submetidos a estudo eletrofisiológico, sendo observada via acessória fascículo-ventricular; em três foi realizada ablação do flutter atrial. Todos os do grupo B foram submetidos à ablação do flutter atrial, sem evidência de via acessória. Observado maior prevalência no grupo B de hipertensão arterial, diabetes mellitus, doença coronariana e apneia do sono, sem diferença estatisticamente significante. Conclusão Portadores da síndrome do PRKAG2 apresentaram flutter atrial em idade mais precoce, e menos comorbidades, quando comparados a pacientes com flutter atrial sem fenótipo da mutação. Importante suspeitar de miocardiopatia geneticamente determinada, como síndrome do PRKAG2, em jovens com flutter atrial, especialmente na presença de pré-excitação ventricular e hipertrofia ventricular familiar.

Abstract Background PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias. Objective To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome. Methods An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients without phenotype of PRKAG2 syndrome. The level of significance was 5%. Results All patients in group A had ventricular preexcitation and right branch block, and four had pacemakers (80%). Patients in group A were younger (39±5.4 vs 58.6±17.6 years, p=0.021), had greater interventricular septum (median=18 vs 10 mm; p<0.001) and posterior wall thickness (median=14 vs 10 mm; p=0.001). In group A, four patients were submitted to an electrophysiological study, showing a fasciculoventricular pathway, and atrial flutter ablation was performed in tree. All patients in group B were submitted to ablation of atrial flutter, with no evidence of accessory pathway. Group B had a higher prevalence of hypertension, diabetes mellitus, coronary artery disease and sleep apnea, with no statistically significant difference. Conclusion Patients with PRKAG2 syndrome presented atrial flutter at an earlier age and had fewer comorbidities when compared to patients with atrial flutter without mutation phenotype. The occurrence of atrial flutter in young individuals, especially in the presence of ventricular preexcitation and familial ventricular hypertrophy, should raise the suspicion of PRKAG2 syndrome.

Associação entre Bloqueio Atrioventricular e Mortalidade em Pacientes de Atenção Primária: O Estudo CODE / Association between Atrioventricular Block and Mortality in Primary Care Patients: The CODE Study

Resumo Fundamento O bloqueio atrioventricular (BAV) descreve um comprometimento na condução dos átrios para os ventrículos. Embora o curso clínico do BAV tenha sido avaliado, os achados são de países de alta renda e, portanto, não podem ser extrapolados para a população latina. Objetivo Avaliar a associação entre BAV e mortalidade. Métodos Foram incluídos pacientes do estudo CODE (Clinical Outcomes in Digital Electrocardiology), maiores de 16 anos que realizaram eletrocardiograma (ECG) digital de 2010 a 2017. Os ECGs foram relatados por cardiologistas e por software automatizado. Para avaliar a relação entre BAV e mortalidade, foram utilizados o modelo log-normal e as curvas de Kaplan-Meier com valores de p bicaudais < 0,05 considerados estatisticamente significativos. Resultados O estudo incluiu 1.557.901 pacientes; 40,23% eram homens e a média de idade foi de 51,7 (DP ± 17,6) anos. Durante um seguimento médio de 3,7 anos, a mortalidade foi de 3,35%. A prevalência de BAV foi de 1,38% (21.538). Os pacientes com BAV de primeiro, segundo e terceiro graus foram associados a uma taxa de sobrevida 24% (taxa de sobrevida relativa [RS] = 0,76; intervalo de confiança [IC] de 95%: 0,71 a 0,81; p < 0,001), 55% (RS = 0,45; IC de 95%: 0,27 a 0,77; p = 0,01) e 64% (RS = 0,36; IC de 95%: 0,26 a 0,49; p < 0,001) menor quando comparados ao grupo controle, respectivamente. Os pacientes com BAV 2:1 tiveram 79% (RS = 0,21; IC de 95%: 0,08 a 0,52; p = 0,005) menor taxa de sobrevida do que o grupo controle. Apenas Mobitz tipo I não foi associado a maior mortalidade (p = 0,27). Conclusão BAV foi um fator de risco independente para mortalidade geral, com exceção do BAV Mobitz tipo I.

Abstract Background Atrioventricular block (AVB) describes an impairment of conduction from the atria to the ventricles. Although the clinical course of AVB has been evaluated, the findings are from high-income countries and, therefore, cannot be extrapolated to the Latinx population. Objective Evaluate the association between AVB and mortality. Methods Patients from the CODE (Clinical Outcomes in Digital Electrocardiology) study, older than 16 years who underwent digital electrocardiogram (ECG) from 2010 to 2017 were included. ECGs were reported by cardiologists and by automated software. To assess the relationship between AVB and mortality, the log-normal model and the Kaplan-Meier curves were used with two-tailed p-values < 0.05 considered statistically significant. Results The study included 1,557,901 patients; 40.2% were men, and mean age was 51.7 (standard deviation ± 17.6) years. In a mean follow-up of 3.7 years, the mortality rate was 3.35%. The AVB prevalence was 1.38% (21,538). Patients with first-, second-, and third-degree AVB were associated with 24% (relative survival rate [RS] = 0.76; 95% confidence interval [CI]: 0.71-0.81; p < 0.001), 55% (RS = 0.45; 95% CI: 0.27-0.77; p = 0.01), and 64% (RS = 0.36; 95% CI: 0.26-0.49; p < 0.001) lower survival rate when compared to the control group, respectively. Patients with 2:1 AVB had 79% (RS = 0.21; 95% CI: 0.08-0.52; p = 0.005) lower survival rate than the control group. Only Mobitz type I was not associated with higher mortality (p = 0.27). Conclusion AVB was an independent risk factor for overall mortality, with the exception of Mobitz type I.