Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block / Prevalencia de lupus neonatal en un pequeño grupo de madres portadoras de anticuerpos y frecuencia de bloqueo auriculoventricular completo

Abstract Background: Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. Objective: To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. Methods: From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. Results: Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. Conclusions: CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

Resumen Antecedentes: El lupus neonatal (LN) es extremadamente raro y es ocasionado por el paso transplacentario de auto-anticuerpos maternos IgG contra las proteínas Ro, La y/o RNP a la circulación fetal que puede ocasionar bloqueo aurículo-ventricular completo congénito (BAVCC) permanente, lesiones dérmicas y afectación hepática. Objetivo: Conocer la prevalencia de LN en paciente con BAVCC y la evolución clínica en un seguimiento a largo plazo. Métodos: De enero de 1992 a diciembre 2017 se incluyeron paciente con BAVCC. La presencia de anticuerpos antinucleares anti-SSA/Ro y anti-SSB/La en suero materno confirmó LN. Resultados: Ocho pacientes fueron incluidos con seguimiento de 10 ± 6 años, el 62.5 % con LN; dos fueron del sexo masculino. Uno diagnosticado in útero, dos al nacimiento, en ellos se implantó marcapaso; uno a los 12 años de edad y otro a los 15. Los otros dos casos fueron diagnosticados a los 18 y 26 años, se implantó marcapaso definitivo en ellos 8 y 5 años después respectivamente. En un caso no se implantó marcapaso definitivo; un recién nacido con solo un año de seguimiento. Al dar a luz, el 60 % de las madres estaban libres de enfermedad reumática y en conjunto todas tuvieron 19 hijos, ninguno de ellos presentó manifestaciones de LN. Conclusiones: El BAVCC es raro y frecuentemente está asociado a una enfermedad autoinmune materna, prácticamente todos requerirán de marcapaso definitivo en alguna época de su vida.

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.

A Case of Infective Endocarditis Treated with Ventricular Septal Defect Closure with the Sandwich Method, Aortic Valve Replacement, and Tricuspid Valve Replacement / 日本心臓血管外科学会雑誌

A 74-year-old man was diagnosed with infective endocarditis (IE) involving the aortic and tricuspid valves, ventricular septal defect (VSD), and complete atrioventricular block. He was admitted to a previous hospital with complaints of fever and neck pain, and he developed complete atrioventricular block during the course of his illness. An echocardiogram revealed severe aortic regurgitation, aortic valve vegetations, and a ventricular septal defect. He was then transferred to our hospital, and he underwent emergent surgery. The aortic valve cusps were calcified and thick, with significant cusp destruction. The vegetations partly extended to the subvalvular area of the right and non-coronary cusp. The vegetations also extended from the atrial septum to the tricuspid valve septal leaflet and perimembranous VSD. Ventricular septal reconstruction using the sandwich technique with two bovine pericardial patches, aortic valve replacement, and tricuspid valve replacement were performed. Postoperatively, he received antibiotic therapy for six weeks and was discharged from our hospital after the implantation of a cardiac resynchronization therapy pacemaker. Echocardiography showed no residual shunts. Our case suggests that the sandwich technique can be a useful method of septal reconstruction for IE with extensive destruction of the ventricular septum.

The impact of programming optimization for atrioventricular synchrony after Micra AV leadless pacemakers implantation / 中国介入心脏病学杂志

Objective To analyze the atrioventricular synchronization rate after implantation of Micra AV leadless pacemaker,and the impact of postoperative programming optimization on atrioventricular synchronization rate.Methods A prospective cohort study was conducted to select patients with complete atrioventricular block who underwent Micra AV leadless pacemaker implantation at Beijing Anzhen Hospital from August 2022 to June 2023.Programming optimization were performed at 1 week,1 month,and 3 months postoperatively,and atrioventricular synchronization rate,electrical parameters,and echocardiography were recorded.Results A total of 68 patients with complete atrioventricular block implanted with Micra AV were selected,with an average age of(68.2±9.7)years,including 47 males(69.1％).All patients were successfully implanted with Micra AV,and there were no serious postoperative complications;The average threshold,sense,and impedance parameters were stable during 1 week,1 month,and 3 months after the procedure;There was no significant difference in the EF value of postoperative echocardiography(P=0.162);The average atrioventricular synchronization rates at 1 week,1 month,and 3 months postoperatively were(75.2％vs.83.8％vs.91.6％,P=0.001).Conclusions As an mechanical atrial sensing,Micra AV requires personalized adjustment of relevant parameters;Postoperative follow-up programming optimization plays an important role in the atrioventricular synchronization and comfort level in patients with complete atrioventricular block after implantation of Micra AV leadless pacemaker.

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.

Bloqueo aurículoventricular en la granulomatosis con poliangeítis (Granulomatosis de Wegener). Caso clínico y revisión de la literatura / Atrioventricular block in granulomatosis with polyangiitis (Wegener's granulomatosis). Case report and review of literature

La Granulomatosis con Poliangeítis (GPA), o Granulomatosis de Wegener, es una vasculitis sistémica de pequeño y mediano vaso inmunológicamente mediada, que preferentemente compromete la vía aérea superior, pulmones y riñones, y es poco frecuente que se asocie a manifestaciones cardíacas. El compromiso del sistema éxcitoconductor (SEC) es muy raro y se han descrito casos aislados de distintos grados de bloqueo aurículoventricular (BAV). Describimos el caso de un paciente con GPA que durante una recidiva de su enfermedad consultó por disnea, documentándose signos de bajo débito, bradicardia de 30/ min, BAV de 2° grado avanzado y bloqueo completo de rama izquierda (BCRI) en el electrocardiograma (ECG). Se presenta una revisión de la literatura y discutimos sus causas, la evolución y manejo de estos pacientes.

Granulomatosis with Polyangiitis (GPA), or Wegener's Granulomatosis, is an immunologically mediated systemic vasculitis of small and medium vessels, which commonly compromises the upper airway, lungs, and kidneys and is rarely associated with cardiac manifestations. Compromise of the cardiac conduction system is rare, and isolated cases of different degrees of atrioventricular block (AVB) have been described. We report a case of a 49-year-old male patient previously diagnosed with GPA 3 years ago, who presented to the emergency department with dyspnea, clinical signs of low output, bradycardia of 30/min, advanced second-degree AVB and complete left bundle branch block (LBBB) on the ECG. A literature review is presented, and we discuss the causes, evolution, and management of this GPA complication.

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.

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.

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.

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.

Predictors of delayed high atrioventricular block after transcatheter aortic valve replacement and risk analysis of pacemaker implantation / 中华胸心血管外科杂志

Objective:To investigate the predictive factors of delayed high atrioventricular block(DH-AVB) after transcatheter aortic valve replacement(TAVR) and the risk of pacemaker implantation.Methods:Patients who underwent TAVR in the heart center of Qingdao Municipal Hospital from January 2019 to October 2022 were retrospectively analyzed. A total of 85 patients who met the criteria of transcatheter aortic valve replacement were included in this study. They were divided into DH-AVB group after TAVR and non-DH-AVB group after TAVR. The data were analyzed by univariate analysis and binary logistic regression analysis. Results:The results showed that the over-rate of valve implantation( OR=3.582, 95% CI: 0.923-13.902, P=0.048), the depth of valve implantation( OR=3.727, 95% CI: 1.138-12.204, P=0.030), the new postoperative CLBBB( OR=5.958, 95% CI: 1.258-28.220, P=0.025)and the prolonged PR time limit( OR=1.036, 95% CI: 1.008-1.065, P=0.011) were independent risk factors for DH-AVB after TAVR. With the progress of conduction block, patients in DH-AVB group had a higher pacemaker implantation rate(81.82 % vs.18.18 %, P<0.001). Conclusion:The excessive rate of valve implantation, the depth of valve implantation, the new complete left bundle branch block(CLBBB) and the prolonged PR time after operation are independent predictors of delayed complete atrioventricular block after TAVR. The incidence of pacemaker implantation in patients with delayed complete atrioventricular block after operation is higher.

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.

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.

Unexplained Syncope With the λ Pattern in Electrocardiogram:a Case Report / 中国循环杂志

The λ pattern is a rare electrocardiogram feature in clinical practice,which often indicates the occurrence of impending fatal ventricular tachyarrhythmias or sudden cardiac death.In this case,Holter monitoring recorded a progressive evolution of the λ pattern followed by advanced atrioventricular blocks in a patient with chest pain accompanied by syncope.λ sign occurred at the time of chest pain,and advanced atrioventricular blocks were recorded at the time of subsequent syncope.Coronary stenosis was detected in the left circumflex by coronary angiography.The patient recovered after stent implantation in the culprit vessel and related medications to prevent coronary spasm.Our case indicate that the λ pattern could occur prior advanced atrioventricular blocks-induced syncope in patient with ischemic heart disease.

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.

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.