Teachable moments in ECG: The physiology behind the pattern

@#The electrocardiographic analysis of heart blocks provides great opportunities for the discussion of mechanisms of electrical cardiac conduction, serving as “teachable moments” in medicine. Recognition of heart blocks can sometimes be a challenge as they can present in many forms, different severities and levels of blocks that present as varied patterns on electrocardiographic tracing. The ultimate key to correct diagnosis rests on adequate understanding of normal electrophysiology of the electrical system of the heart. While it is vital to recognize the pattern, we should always know and understand the physiology behind the pattern. This article presents a detailed analysis of a case of heart block which can easily be misinterpreted on first look. The case is featured not for its rarity but for the interesting concepts in cardiac electrophysiology that are highlighted. Navigation of the different elements of tracing can be an adventure and a great learning experience enjoyed by both students and experts.

Clinical characteristics of fetal cardiac disease in patients with anti-SSA antibody positive / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical manifestations and laboratory indicators of anti-Sjögren's-syndrome-related antigen A (SSA) antibody associated fetal cardiac disease.@*METHODS@#Pregnant women hospitalized at Peking University People's Hospital from January 2013 to July 2023 were included. Eleven patients with anti-SSA antibody positive were eventually diagnosed with fetal cardiac di-sease. And patients with anti-SSA antibody positive without fetal cardiac disease were selected as controls. Clinical manifestations, laboratory indications and drug usage were compared between the two groups.@*RESULTS@#Among these 11 patients, congenital heart block was confirmed in seven, which was the most common manifestations of fetal cardiac malformation. The proportion of the patients diagnosed with autoimmune disease before pregnancy in fetal cardiac malformation group was significantly lower than that in the control group (P=0.032), while most of the patients in the fetal cardiac malformation group received immune-related examinations for the first time because of this time's fetal cardiac diagnosis. While most of the patients in the control group received routine examinations because of autoimmune diseases diagnosed before pregnancy. During pregnancy, the white blood cell level [(9.29±2.58)×109/L vs. (7.10±1.90×109/L, t=3.052, P=0.004], erythrocyte sedimentation rate [(49.50 (48.00, 51.00) mm/h vs. 23.00 (15.00, 30.25) mm/h, Z=-2.251, P=0.024], IgA level [3.46 (2.30, 5.06) g/L vs. 2.13 (1.77, 2.77) g/L, Z=-2.181, P=0.029], and antinuclear antibody (ANA) titers [1∶320 (1∶160, 1∶320) vs. 1∶80 (1∶40, 1∶160), Z=-3.022, P=0.003] were significantly higher in fetal cardiac malformation group than in the control group. The proportion of positive anti-SSB antibody during pregnancy did not show a statistically significant difference between the two groups (37.5% vs. 7.7%, P=0.053). There was no significant difference in hydroxychloroquine dosage and initiation time between the two groups. The dosage of prednisone in the second and third trimesters was significantly higher in the cardiac malformation group than that in the control group, but there was no significant difference in the first trimester.@*CONCLUSION@#Fetal cardiac disease is rare in pregnant women with anti-SSA antibody. White blood cell, erythrocyte sedimentation rate, IgA, the titer of ANA positivity were higher in the fetal heart disease group during pregnancy. Since congenital heart block is difficult to reverse, its prevention and monitoring are more important than remedial treatment.

Síndrome de marcapasos: cuál es el mecanismo? / Pacemaker syndrome: what is the mechanism?

Resumen Presentamos un caso de síndrome de marcapasos asociado a la ausencia de sincronía aurículo-ventricular, debido a la colocación invertida e inadvertida de los electrodos, en una paciente portadora de bloqueo aurículo-ventricular completo; para su diagnóstico, es fundamental un cuidadoso análisis del electrocardiograma, las imágenes con rayos X y los electrogramas intracavitarios.

Abstract Pacemaker syndrome: what is the mechanism? We present the case of pacemaker syndrome related to the absence of atrioventricular synchrony, due to inverted and inadvertent placement of the electrodes, in a patient with complete atrioventricular block. A careful analysis of the electrocardiogram, X-ray images and intracavitary electrograms are essential for its diagnosis.

Cardiac conduction defects and complete heart block in myotonic dystrophy / Defectos de conduccion cardiaca y bloqueo cardiaco completo en la distrofìa miotonica

Classic myotonic dystrophy is a multisystem disorder that results from RNA toxicity and is one of the commonest adult onset muscular dystrophies. Patients often present with muscle stiffness from myotonia and dysphagia or dysarthria from laryngopharyngoesophageal muscle weakness. Benign electrocardiogram changes such as first degree atrioventricular block are commonly present and rarely merit further work up. Occasionally, patients develop advanced conduction defects which can unexpectedly progress to complete heart block perioperatively

La distrofia miotonica clásica es un trastorno multi-sistémico que resulta de la toxicidad del RNA y es una de las distrofias musculares más comunes en adultos. Los pacientes suelen presentar rigidez muscular por la miotonía, así como disfagia o disartria por debilidad muscular laringo-faríngea-esofágica. Los cambios benignos en el electrocardiograma, como el bloqueo auriculoventricular de primer grado, suelen estar presentes y rara vez merecen un análisis más profundo. Ocasionalmente, los pacientes desarrollan defectos de conduccion avanzados que pueden progresar inespera-damente para completar el bloqueo cardiaco perioperatorio.

Congenital complete atrioventricular heart block in a pregnant woman with sjögren syndrome: prenatal care follow-up and the challenge of intrauterine treatment / Bloqueio atrioventricular completo congênito em uma mulher grávida com síndrome de Sjögren: seguimento pré-natal e desafio do tratamento intrauterino

Abstract The present report describes a case of complete atrioventricular block (CAVB) diagnosed at 25 weeks of gestation in a pregnant woman with Sjögren's syndrome and positive anti-Ro/SSA antibodies. Fluorinated steroids (dexamethasone and betamethasone) and terbuline were used to increase the fetal heart rate, but the fetal heart block was not reversible, and the administration of drugs was discontinued due to maternal collateral effects. Follow-up fetal echocardiograms were performed, and the fetus evolved with pericardial effusion, presence of fibroelastosis in the right ventricle, and ventricular dysfunction. Interruption of pregnancy by cesarean section was indicated at 34 weeks of gestation, and a cardiac pacemaker was implanted in the male newborn immediately after birth. Therapy for fetuses with CAVB is controversial mainly regarding the use or not of corticosteroids; however, monitoring of the atrioventricular interval by fetal echocardiography should be performed in fetuses from pregnant women with positive autoantibodies anti-Ro/SSA and/or anti-La/SSB to prevent the progression to CAVB.

Resumo Este relato descreve um caso de bloqueio atrioventricular completo (BAVC) diagnosticado com 25 semanas de gestação em uma mulher com síndrome de Sjögren e anticorpos anti-Ro/SSA positivos. Esteroides fluoretados (dexametasona e betametasona) e terbulina foram utilizados para aumentar a frequência cardíaca fetal, mas o bloqueio cardíaco fetal não foi reversível, e a administração dos medicamentos foi interrompida devido a efeitos colaterais maternos. Ecocardiogramas fetais de acompanhamento foram realizados, e o feto evoluiu com derrame pericárdico, presença de fibroelastose no ventrículo direito, e disfunção ventricular. A interrupção da gravidez por cesariana foi indicada com 34 semanas, e um marca-passo cardíaco foi implantado no recém-nascido do sexo masculino imediatamente após o nascimento. A terapia para fetos com BAVC é controversa, principalmente no que diz respeito ao uso ou não de corticosteroides; no entanto, o monitoramento do intervalo atrioventricular pela ecocardiografia fetal deve ser feito em fetos de mulheres grávidas com autoanticorpos positivos anti-Ro/SSA e/ou anti-La/SSB para impedir a progressão para o BAVC.

Bentall Operation in a Patient with a Unicommissural Unicuspid Aortic Valve / 대한흉부외과학회지

A unicuspid aortic valve is a rare congenital malformation that frequently presents with valvular dysfunction and dilatation or aortic aneurysm, requiring combined aortic valve surgery and aortic repair. Some patients show severe valve calcification extending into the interventricular septum, possibly resulting in damage to the conduction system during debridement for valve replacement. We present a rare case of severe aortic stenosis with a unicommissural unicuspid aortic valve diagnosed by preoperative transesophageal echocardiography in a 36-year-old man. After composite graft replacement of the aortic valve, aortic root, and ascending aorta, a permanent pacemaker was placed because of postoperative complete heart block.

Complete heart block: A rare central venous catheter placement complication / 中南大学学报(医学版)

Central venous catheter (CVC) placement is an operation which can establish a fast, safe, and effective deep venous access to rescue patients under critical conditions, especially for those receiving hemodialysis. It is a simple operation with almost no complications, but different complications have been still reported, such as bleeding, infection, embolism, low blood flow, and cardiac arrhythmias. In this case, the patient with preexisting left bundle branch block suffered right bundle branch block, leading to complete heart block during CVC placement. When the patient developed complete heart block, we immediately treated him with isoproterenol, and the surgery was terminated as soon as possible. The patient gradually recovered the sinus rhythm after the treatment. This complication is rare but severe, and clinicians should recognize the risks and take strategies as early as possible. We think the cause of complete heart block in this patient may be related to mechanical trauma to the right ventricle by the guide wire or catheter insertion. Therefore, CVC placement should be performed with more caution, and the guide wire and catheter tip should be inserted less than 18 cm deep.

A retrospective survey comparing suture techniques regarding the risk of permanent epicardial pacemaker implantation after ventricular septal defect closure

Abstract Objective: The aim of this study is to compare the continuous and combined suturing techniques in regards to the needing epicardial pacing at the time of weaning from cardiopulmonary bypass (EP-CPB) and to evaluate permanent epicardial pacemaker (PEP) implantation in patients who had undergone surgical ventricular septal defect (VSD) closure. Methods: This single-centre retrospective survey includes 365 patients who had consecutively undergone VSD closure between January 2006 and October 2015. Results: The median age and weight of the patients were 15 months (range 27 days - 56.9 years) and 10 kg (range 3.5 - 100 kg), respectively. Continuous and combined suturing techniques were utilised in 302 (82.7%) and 63 (17.3%) patients, respectively. While 25 (6.8%) patients required EP-CPB, PEP was implanted in eight (2.2%) patients. Comparison of the continuous and combined suturing techniques regarding the need for EP-CPB (72% vs. 28%, P=0.231) and PEP implantation (87.5% vs. 12.5%, P=1.0) were not statistically significant. The rate of PEP implantation in patients with perimembraneous VSD without extension and perimembraneous VSD with inlet extension did not reveal significant difference between the suture techniques (P=1.0 and P=0.16, respectively). In both univariate and multivariate analyses, large VSD (P=0.001; OR 8.63; P=0.011) and perimembraneous VSD with inlet extension (P<0.001; OR 9.02; P=0.005) had a significant influence on PEP implantation. Conclusion: Both suturing techniques were comparable regarding the need for EP-CPB or PEP implantation. Caution should be exercised when closing a large perimembraneous VSD with inlet extension.

Permanent pacemaker implantation in complete congenital fetal atrioventricular (AV) block: a case report / Implantación de marcapasos permanente en bloqueo atrioventricular (AV) fetal congénito completo: reporte de caso

Abstract: One of the most common pathologies attributed to lupus neonatal refers to atrioventricular (AV) congenital block, which diagnosis can be made between 16 and 30 weeks of gestation due to persistent fetal bradycardia. The development of this disease is mostly related to maternal anti-Ro/SSA and anti-Smith autoantibodies. Currently, there are a number of alternatives for prenatal and postnatal treatment, with some controversy about their viability. The placement of a permanent pacemaker is presented as one of the most appropriate procedures currently, even with the risks awarded. This case report describes the placement of a permanent pacemaker to a two-month-old newborn with high maternal contents of anti-Ro/SSA and anti-Smith nuclear autoantibodies, with a favorable outcome.(AU)

Resumen: Una de las patologías más comunes atribuidas al lupus neonatal se refiere al bloqueo congénito atrioventricular (AV), diagnóstico que se puede realizar entre 16 y 30 semanas de gestación debido a bradicardia fetal persistente. El desarrollo de esta enfermedad se relaciona principalmente con los anticuerpos anti-Ro/SSA materno y anti-Smith. Actualmente, existen varias alternativas para el tratamiento prenatal y postnatal, con cierta controversia sobre su viabilidad. La colocación de un marcapasos permanente se presenta como uno de los procedimientos más adecuados actualmente, incluso con los riesgos adjudicados. Este relato de caso describe la colocación de un marcapasos permanente en un recién nacido de dos meses con alto contenido materno de autoanticuerpos anti-Ro/SSA y anti-Smith, con un resultado favorable.(AU)

Minitoracotomia direita para implante de marcapasso epicárdico definitivo dupla-câmara / Dual chamber epicardial pacing through right minithoracotomy

A cirurgia minimamente invasiva é segura e eficaz no tratamento de diversas afecções cardíacas, com evolução intra-hospitalar bastante positiva. Descrevemos aqui o caso de paciente do sexo masculino, com 72 anos de idade, portador de comunicação interatrial com shunt bidirecional e bloqueio atrioventricular avançado com síncope. Foi contraindicado o fechamento da comunicação interatrial e indicado marcapasso dupla-câmara com cabos-eletrodos epicárdicos em decorrência da presença do shunt. O procedimento foi realizado por meio de minitoracotomia direita, com implante de cabos-eletrodos atrial e ventricular direitos bipolares, com gerador implantado em loja subcutânea na região infraclavicular direita. O paciente apresentou boa evolução, recebendo alta no quarto dia de pós-operatório em boas condições

Minimally invasive cardiac surgery is safe and effective in the treatment of a wide range of cardiac diseases, with very positive in-hospital outcomes. We describe the case of a 72-year-old male patient, with atrial septal defect, bidirectional shunt and advanced atrioventricular block with syncope. The atrial septal defect closure was contraindicated and he was referred for a dual-chamber pacemaker and epicardial leads implantation due to the presence of shunt. The patient underwent a right minithoracotomy with the implantation of bipolar atrial and ventricular leads and placement of a pacemaker generator in a subcutaneous envelope in the right infraclavicular region. The patient evolved well and was discharged on the fourth postoperative day in good conditions

Two Cases of Neonatal Lupus and Literature Review

Neonatal lupus is a rare rheumatic disease. Clinical manifestations include characteristic annular or macular rashes, congenital heart block, cytopenias, and hepatitis. Neonatal lupus is caused by transmission of maternal immunoglobulin G autoantibodies such as anti-SSA/Ro antibody or anti-SSB/La antibody to the fetus through the placenta. We report two cases of neonatal lupus. The first case refers to an 18-day-old male with annular rashes on both cheeks, neutropenia, positive tests for antinuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody. His mother was diagnosed with systemic lupus erythematosus characterized by positive tests for antinuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody. The second case represents a 32-day-old female with annular rash on both hands, soles, and the genital area, neutropenia, hepatitis, positive tests for antinuclear antibody, and anti-SSA/Ro antibody. Skin punch biopsy was conducted. Her mother did not have history of connective tissue diseases. We referred her mother to the division of rheumatology of the department of internal medicine. The mother was suspected with primary Sjögren's syndrome because of arthralgia and dry eye symptoms with positive tests for antinuclear antibody, anti-SSA/Ro antibody, anti-SSB/La antibody, and rheumatoid factor. It is necessary to suspect neonatal lupus in neonates or infants with characteristic annular rash with or without maternal history of connective tissue disorders.

Bloqueo auriculoventricular completo y asistolia en un niño con ataxia-telangiectasia / Complete heart block and asystole in a child with ataxia-telangiectasia

La ataxia-telangiectasia es una entidad caracterizada por un cuadro de ataxia cerebelosa progresiva, telangiectasias, defectos inmunológicos y una mayor tendencia al desarrollo de tumores malignos. La mutación genética responsable (ataxia-telangiectasia mutada) parece jugar un papel importante en la función celular normal y el remodelado cardiovascular. Se describe la aparición de una arritmia maligna en un paciente de 14 años con un diagnóstico de ataxia-telangiectasia, en remisión completa de linfoma no Hodgkin B de alto grado. Consultó en el Servicio de Urgencias Pediátricas por episodios de presíncope, y se observó, al ingresar, bloqueo auriculoventricular completo que evolucionó hacia asistolia, por lo que requirió la colocación de un marcapasos definitivo. Las dosis acumuladas de fármacos cardiotóxicos recibidos fueron de bajo riesgo. Sin embargo, es posible que esta enfermedad degenerativa crónica afecte con el tiempo al tejido de citoconducción. En la bibliografía revisada, no existen o se desconocen reportes previos de arritmias malignas en pacientes con ataxia-telangiectasia.

Ataxia-telangiectasia is a disorder characterized by cerebellar ataxia, telangiectasia, immunodeficiency, and increased predisposition to cancer susceptibility. Mutations in the ataxia telangiectasia mutated gene seem to play an important role in normal cell function and in cardiovascular remodeling. We report a case of a 14-year-old boy with ataxia-telangiectasia and high-grade B-non-Hodgkin lymphoma who remained in continuous complete remission after chemotherapy and who was admitted into our Emergency Room presenting with episodes of presyncope. At admission he presented a complete atrioventricular block that evolved into asystole and required placement of a pacemaker. Cumulative cardiotoxic drugs received were at low risk. However, it is possible that this chronic degenerative disease may affect the cardiac conduction system over time. In the reviewed literature there are no or unknown reports of ataxia-telangiectasia with malignant cardiac arrhythmias.

Arritmias ventriculares como manifestación de / sarcoidosis cardiaca primaria Ventricular arrhythmias as a first sign of primary cardiac sarcoidosis

Resumen La afectación cardíaca en pacientes con sarcoidosis está siendo cada vez más reconocida y se asocia con mal pronóstico. Aunque en su patogénesis están implicados los factores ambientales y genéticos, la etiología de la sarcoidosis cardíaca no es clara. Las manifestaciones clínicas incluyen alteraciones de la conducción aurículo-ventricular, arritmias e insuficiencia cardíaca congestiva. Es una entidad extremadamente difícil de diagnosticar debido a que las manifestaciones clínicas son inespecíficas, y la sensibilidad y la especificidad de las modalidades de diagnóstico son limitadas. El tratamiento cardíaco óptimo no ha sido bien definido, y aunque los corticoides siguen siendo el pilar del manejo, hay poca evidencia de la dosis o la duración de la terapia. Se expone el caso de una paciente con sarcoidosis cardíaca aislada, que debutó con extrasístoles ventriculares y progresó a falla cardiaca y arritmias ventriculares sostenidas.

Abstract Cardiac involvement is increasingly being observed in patients with sarcoidosis and is associated with a poor prognosis. Although environmental and genetic factors play a part in its pathogenesis, the aetiology of cardiac sarcoidosis is still not clear. The clinical signs include anomalies in atrial-ventricular conduction, arrhythmias, and congestive cardiac failure. It is an extremely difficult condition to diagnose as the clinical signs are vague, and the sensitivity and specificity of the diagnostic models are limited. There is no well-defined optimum cardiac treatment, and although corticosteroids continue to be the mainstay of its management, there is little evidence on the dose or duration of the treatment. It is presented a case of a patient with isolated cardiac sarcoidosis that debuted with ventricular extrasystoles and progressed to cardiac failure and sustained ventricular arrhythmias.

Minimally Invasive Epicardial Pacemaker Implantation in Neonates with Congenital Heart Block / Implante de Marca-Passo Epicárdico por Acesso Minimamente Invasivo em Neonatos com Bloqueio Atrioventricular Congênito

Abstract Background: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). Objective: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Methods: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. Results: All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Conclusion: Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.

Resumo Fundamento: Há poucos estudos caracterizando os desfechos cirúrgicos após implante de marca-passo em neonatos com bloqueio atrioventricular total congênito (BAVTC). Objetivos: Este estudo procurou avaliar os resultados a longo prazo de uma abordagem epicárdica minimamente invasiva com utilização de acesso subxifoide para implante de marca-passo em neonatos. Métodos: Entre julho de 2002 a fevereiro de 2015, 16 neonatos consecutivos foram submetidos a implante de marca-passo epicárdico devido a BAVTC. Entre eles, 12 (75,0%) apresentavam defeitos cardíacos congênitos associados ao BAVTC. Os pacientes tinham uma média de idade de 4,5 ± 5,3 dias e nove (56,3%) eram do sexo feminino. Cabo-eletrodo bipolar revestido com esteroide foi implantado em todos os pacientes através de uma abordagem subxifoide minimamente invasiva e fixado na superfície ventricular diafragmática. O gerador de pulsos foi alojado em uma posição epigástrica submuscular. Resultados: Todos os procedimentos foram realizados com sucesso, sem complicações intraoperatórias ou mortes. O tempo médio de duração das operações foi de 90,2 ± 16,8 minutos. Após seguimento médio de 4,1 ± 3,9 anos e máximo de 12,2 anos, não foram observadas complicações de loja do gerador de pulsos, aumento crônico do limiar de comando, fratura de cabo-eletrodo ou outros problemas relacionados ao sistema de estimulação cardíaca. Três crianças foram submetidas à troca do gerador de pulsos por depleção normal de bateria aos 4,0, 7,2 e 9,0 anos de idade, sem necessidade de troca do cabo-eletrodo ventricular. Houve duas mortes aos 12 e 325 dias após o implante do marca-passo devido a sangramento decorrente do uso de trombolítico e insuficiência cardíaca refratária progressiva, respectivamente. Conclusões: O implante de marca-passo epicárdico através de abordagem subxifoide em neonatos com BAVTC com alojamento epigástrico do gerador de pulsos é tecnicamente viável e associado a excelentes desfechos cirúrgicos e longevidade do cabo-eletrodo de estimulação.

Avaliação de dessincronia atrioventricular em portadores de marca-passo bicameral devido à doença do nó sinusal e bloqueio atrioventricular de primeiro grau / Evaluation of atrioventricular dyssynchrony in patients with dual-chamber pacemaker implanted due to sinus node disease and first degree atrioventricular bloc

INTRODUÇÃO: O intervalo PR longo, em conjunto com a duração e a morfologia do QRS gerado pela estimulação cardíaca artificial, associam-se com dessincronia e disfunção cardíaca em diferentes níveis. Na doença do nó sinusal, durante a programação do marca-passo, podemos optar por duas estratégias: PR longo com QRS estreito (quando se busca evitar a ativação ventricular em detrimento do sincronismo atrioventricular) ou PR otimizado e QRS largo estimulado pelo marca-passo (quando se busca corrigir o intervalo atrioventricular em detrimento da sincronia ventricular). Neste estudo, buscamos comparar a evolução clínica e estrutural cardíaca destas estratégias. MÉTODOS: Acompanhou-se por 1 ano uma coorte com doença do nó sinusal, bloqueio atrioventricular de 1º grau (doença binodal) e marca-passo DDD. Através da ecocardiografia Doppler de fluxo transmitral avaliou-se a duração do enchimento diastólico ventricular e sincronia atrioventricular (? ondas E+A>=40% do ciclo cardíaco). Os pacientes dessincrônicos (DAV) tiveram o intervalo atrioventricular otimizado (intervenção) ao melhor rendimento hemodinâmico, porém com QRS estimulado. Estes retornavam ao PR basal após 6 meses (cross-over). Os sincrônicos (SAV) foram mantidos sob PR longo e QRS intrínseco durante todo o seguimento (controles)...

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block / 고신대학교의과대학학술지

Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. KSS can lead to a risk of sudden death because of the potential progression of conduction abnormalities such as right or left bundle branch block or complete atrioventricular (AV) block. Here we describe the case of a KSS patient with type I diabetes who experienced syncope in the presence of complete AV block, confirmed by muscular biopsy.

Tetralogy of Fallot repair in patients presenting after Infancy: a single surgeon experience

Objective: To determine the early surgical outcomes of Tetralogy of Fallot [TOP] repair in children and young adults operated after the age of one year

Methods: In this retrospective study, 307 cases of primary repair of Tetralogy of Fallot were done between September 2012 to February 2017, at CPE Institute of cardiology, Multan. Out of 307 operated patients, 4 [1.3%] patients had previous modified Blalock Taussig shunts, 2 [0.6%] associated ASD with TOF, 3 [0.9%] co-association of TOF with PDA, 2 [0.6%] had large conal arterial branch crossing the annulus, 3 [0.9%] had dextrocardia with situs inversus, 12 [3.9%] TOF with double outlet right ventricle [DORV], 2 [0.6%] were associated with complete AV canal defect, 8 [2.60%] with absent pulmonary valve syndrome, 15 [5.5%] I with left pulmonary artery stenosis. Data of post-operative complications and operative parameters was recorded for all patients

Results: Mean age of operated patients was 9.56+/-4.89 years. Post-operative complications occurred in 7.8% of patients. Most common post-operative complications were pleural effusion with a frequency of 12[3.9%] patients, and complete heart block in one patient. Insignificant small residual VSD was diagnosed in 8 [2.6%] patients. One moderately large VSD was closed surgically after one year of 1st surgery. Moderate to severe pulmonary valve regurgitation was diagnosed in 114 [37.1%] patients. Mild to moderate tricuspid regurgitation in 15 [4.8%] patients and moderate right ventricular outflow tract obstruction [RVOT] in 16 [5.2%] patients. Thirty-day mortality was only four [1.3%]

Conclusion: Surgical correction of Tetralogy of Fallot [TOF] in children after one year carries good operative outcomes with minimum morbidity and mortality

Manifestaciones cardiovasculares en pacientes con dengue durante dos brotes epidémicos en Colombia / Cardiovascular manifestations in dengue fever patients during two epidemic breakouts in Colombia

la afección cardiovascular es una presentación atípica del dengue, que abarca desde bradicardia sinusal asintomática hasta miocarditis grave, aunque no hay datos locales de esta presentación. Objetivo: describir las características clínicas de una serie de pacientes con dengue y evidencia de afección cardiovascular. Materiales y métodos: se realizó un estudio observacional, de corte trasversal, de pacientes admitidos con dengue a un centro de referencia cardiovascular en Medellín, Colombia, durante dos epidemias del virus entre junio de 2010 y diciembre de 2016. Se evaluaron variables clínicas y paraclínicas. Resultados: se registraron un total de 60 pacientes, de los cuales el 30% (18/60) tuvieron compromiso cardiovascular y fueron incluidos en este estudio. La edad media de estos pacientes fue de 63 años y el 56% (10/18) eran hombres. La comorbilidad más frecuente fue la hipertensión arterial esencial (61%, 11/18). La alteración del ritmo cardíaco afectó al 72% (13/18) de los pacientes, con presencia de bloqueo auriculoventricular de primer grado (46%), bradicardia sinusal (38%), fibrilación/aleteo/taquicardia atriales (23%) y ritmo de la unión (8%). La miocarditis y la pericarditis se presentaron en el 17% (3/18) y 11% (2/18) de los pacientes, respectivamente. Otros hallazgos menos comunes fueron la disfunción ventricular izquierda y la angina de pecho (11%, 2/18). No hubo muertes durante la realización del estudio. Conclusiones: las alteraciones asintomáticas del ritmo cardiaco fueron las manifestaciones cardiovasculares más frecuentes del dengue y las afecciones cardiovasculares graves fueron infrecuentes. Se recomienda atención a los síntomas leves de disfunción cardiovascular en los pacientes con dengue

cardiovascular involvement is an atypical presentation of dengue infection. There range from asymptomatic sinus bradycardia to severe life-threatening myocarditis. Data are lacking regarding epidemiology on this topic. Objective: To describe clinical characteristics of patient's series with dengue and evidence of cardiovascular involvement. Materials and methods: It was performed a cross-section, observational study of patients admitted with dengue fever in a cardiovascular reference center in Medellín, Colombia during two epidemic viral outbreaks from June 2010 to December 2016. Results: A total of 60 patients were registred, 30% of them with evidence of cardiovascular involvement. Mean age of patients was 63 years old and 55% were male. Most common comorbidity was hypertension (61%, 11/18). Rhythm disturbances affected 72% (13/18) of patients: first grade AV block (46%), sinus bradycardia (38%), atrial fibrillation/flutter/tachycardia (23%) and junctional rhythm (8%). Myocarditis and pericarditis were present in 17% (3/18) and 11% (2/18) of patients, respectively. Other less common findings were left ventricular dysfunction and angor pectoris (11%, 2/18). There were no deaths during the study period. Conclusions: Asymptomatic rhythm disturbances were the most common cardiovascular manifestations of dengue fever and severe forms of cardiovascular dengue were rare. It is recommended to be attentive to the mild symptoms of cardiovascular dysfunction in patients with dengue

Terapia de la resincronización cardiaca transvenosa en la población pediátrica / Transvenous cardiac resynchronization therapy in pediatric population

Resumen La disfunción ventricular izquierda y la cardiopatía dilatada (CD), debidas a la estimulación crónica ventricular derecha son complicaciones reconocidas en adultos y niños. La terapia de la resincronización cardiaca (TRC), ha demostrado beneficios en los adultos con disfunción ventricular izquierda (FEVI < 35%), deterioro de la clase funcional y bloqueo de la rama izquierda (intrínseco o inducido por estimulación). En los niños la incidencia de cardiomiopatía dilatada es desconocida y no existen guías o grandes estudios sobre la resincronización cardiaca, por lo que no es posible extrapolar las recomendaciones de los pacientes adultos. Se presenta la experiencia en una paciente con bloqueo A-V completo postquirúrgico tras corrección de cardiopatía congénita, portadora de un marcapaso quien desarrolló la falla cardiaca, esta fue tratada exitosamente mediante la resincronización biventricular en dos oportunidades, con recuperación de la clase funcional y los parámetros hemodinámicos.

Abstract Left ventricular dysfunction and dilated cardiomyopathy (DC) caused by right ventricular chronic stimulation are recognised complications in adults and children. Cardiac resynchronization therapy (CRT) has shown benefits in adults with left ventricular dysfunction (LVEF < 35%), deterioration of functional class and left bundle-branch block (instrinsic or induced by stimulation). Incidence of dilated cardiomyopathy in children is unknown, and there are no guides or big studies about cardiac resynchronization therapy, so recommendations for adult patients cannot be extrapolated. The experience of a female patient with a pacemaker and postoperative complete AV block after correction of a congenital heart disease is presented; she developed heart failure that was successfully treated with biventricular resynchronization in two opportunities, with recovery of functional class and hemodynamic parametres.

Bloqueo auriculoventricular completo como manifestación de amiloidosis cardiaca / Complete atrioventricular block as an evidence of amyloidosis

La amiloidosis cardiaca es una manifestación de un grupo de enfermedades sistémicas que se caracteriza por el mal plegamiento de proteínas que causa un depósito extracelular de la amiloide, conocido como amiloidosis. Es una causa importante de las enfermedades infiltrativas, que pueden ser responsables tanto del compromiso cardiaco como de otros órganos. Es importante el reconocimiento de su etiología primaria o secundaria para dar el tratamiento adecuado según su causa. Describimos un caso de un paciente de 69 años de edad, con amiloidosis que ingresa al servicio de urgencias con un bloqueo auriculoventricular completo.

Cardiac amyloidosis is a manifestation of a systemic diseases group characterized by protein misfolding caused by extracellular deposition of amyloid, known as amyloidosis. It is the main cause of infiltrative diseases that can be responsible of cardiac involvement as to other organs. It is important to recognize its primary or secondary etiology to give the appropriate treatment depending of the cause. We describe a case of a 69 year old patient with cardiac amyloidosis debuting in the emergency room with complete atrioventricular block.