Home monitoring of fetal heart rhythm: Lived experiences of women with anti-SSA/Ro52 autoantibodies and their co-parents.

OBJECTIVE: The aim of this study was to explore the parents' experiences of home monitoring of the fetal heart rhythm. Women with anti-SSA/Ro52 autoantibodies carry a 2%-3% risk of giving birth to a child with congenital heart block (CHB), following transplacental transfer and antibody-mediated inflammation in the fetal conduction system during 18th to 24th gestational week. Early detection and subsequent treatment have been reported to decrease morbidity and mortality. Therefore, home monitoring of the fetal heart rhythm by Doppler has been offered at our fetal cardiology center. This study was undertaken to explore the lived experience of the routine. METHODS: Participants were recruited from a single fetal cardiology center. Consecutive sampling was used. The inclusion criteria were women with SSA/Ro52 antibodies who had undergone Doppler examinations within the last two and a half years at the hospital and had monitored the fetal heartbeat at home. A semi-structured questionnaire was created, and the participants were interviewed individually. The interviews were transcribed verbatim and analyzed according to qualitative content analysis. RESULTS: The overall theme was defined as "walking on thin ice," with six underlying categories: reality, different strategies, gain and loss, healthcare providers, underlying tension, and conducting the examinations again, all with a focus on how to handle the home monitoring during the risk period. CONCLUSION: Both the mother and the co-parent expressed confidence in their own abilities and that the monitoring provided them with the advantage of growing a bond with the expected child. However, all the participants described a feeling of underlying tension during the risk period. The results show that home monitoring is not experienced as complicated or a burden for the parents-to-be and should be considered a vital part of the chain of care for mothers at risk for giving birth to a child with CHB. However, explaining the teamwork between the different caregivers, for the patients involved, their areas of expertise, and how they collaborate with the patient continues to be a pedagogic challenge and should be developed further.

Anti-SSA/Ro positivity and congenital heart block: obstetric and foetal outcome in a cohort of anti-SSA/Ro positive pregnant patients with and without autoimmune diseases.

OBJECTIVES: Neonatal lupus (NL) is an acquired disease caused by the transplacental passage of anti-SSA/Ro antibodies. The rate of congenital heart block (CHB), its most serious manifestation, ranges from 1 to 5%. The aim of this study was to retrospectively assess the prevalence of CHB in anti-SSA/Ro positive pregnant women with or without systemic autoimmune diseases from 2010 to 2020. METHODS: Patients underwent monthly visit and a shared follow-up programme of weekly (16th-24th week) foetal heart rate assessment by obstetric ultrasound. RESULTS: 322 pregnancies in 258 anti-SSA/Ro patients were included; 314 were followed from the beginning of pregnancy because of the known presence of anti-SSA/Ro autoantibodies and 1 case of CHB occurred in an anti-SSA/Ro+ asymptomatic subject (0.3%). In the same period, 8 additional patients were referred to our clinics after in utero CHB diagnosis and subsequent discovery of anti-SSA/Ro without a disease diagnosis. Globally, 9 cases of congenital CHB (2.8%) occurred: 7 complete, 1 II-III degree and 1 rst degree CHB. Anti-SSB/La positivity was associated with a higher risk of CHB (7.8% vs. 1.2%; p=0.0071). No differences in maternal or foetal outcomes were found in comparison with a large cohort of unselected pregnancies except for caesarian section. Hydroxychloroquine (HCQ) was used in 58.3% pregnancies, with a different prevalence according with maternal diagnosis. CONCLUSIONS: Our data suggest that anti-SSA/Ro positive patents with a de ned systemic autoimmune disease undergoing a strict follow-up since positive pregnancy test display a low risk of pregnancy complications, including but not limited to NL.

[Clinical characteristics of fetal cardiac disease in patients with anti-SSA antibody positive].

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.

Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period.

Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3-4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died.Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known: • Congenital complete heart block is a very rare condition associated with high morbidity and mortality. • Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect. What is New: • Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death.

Conduction delay across the cavotricuspid isthmus block line caused by the gap near the inferior vena cava: the role of conduction block in the lower lateral right atrium.

The electrophysiological properties of the gap associated with the cavotricuspid isthmus (CTI) block line near the inferior vena cava (IVC) are not fully elucidated. Of 143 patients who underwent CTI block line ablation between September 2020 and April 2021, high-resolution CTI gap mapping was performed for 15 patients. Four patients were identified as having a gap near the IVC (IVC-side gap) despite wide double potentials (DPs) with > 90 ms intervals at the block line. Detailed gap mapping during coronary sinus ostial pacing was performed before and after touch-up ablation. CTI conduction delays caused by an IVC-side gap were classified into 3 patterns: (1) conduction delay at the IVC-side gap without detouring gap conduction, (2) detouring gap conduction due to intrinsic lower lateral right atrium (LLRA)-IVC functional block, and (3) detouring gap conduction due to LLRA-IVC conduction block created by lateral deviation of the CTI ablation line. In Pattern 2, IVC-side gap conduction traveled backward toward the crista terminalis below the LLRA-IVC junction and came back forward again above the border. One patient presented with a head-to-bottom activation pattern of the lateral right atrium (pseudo-CTI block). Pattern 3 was caused by lateral deviation of initial RF deliveries and presented with the same course as intrinsic LLRA-IVC functional block. All patients had wide DP intervals near the tricuspid annulus (mean, 112 ms) and just above the gap site (mean, 109 ms). An IVC-side gap associated with the CTI block line can present with various conduction delay patterns.

Histological finding of maternal antibody-associated congenital heart block accompanied by large atrial septal defect and severe cardiac dysfunction.

Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.

Interferons and innate immune activation in autoimmune congenital heart block.

Autoimmune congenital heart block (CHB) may develop in foetuses of women carrying anti-Ro/SSA and La/SSB autoantibodies and is characterized by disruption of signal conduction at the atrioventricular (AV) node, resulting in partial or complete AV block. If not fatal in utero, complete CHB typically requires lifelong cardiac pacing. No treatment has so far been unequivocally demonstrated to prevent or treat autoimmune CHB, and the relatively low incidence (1%-5%) and recurrence (12%-16%) rates of second/third-degree AV block add to the complexity of managing pregnancies in women with anti-Ro/La antibodies. Altogether, a better understanding of events leading to development of autoimmune CHB is needed to improve surveillance and treatment strategies. In the past decade, studies have started to look beyond the role of maternal autoantibodies in disease pathogenesis to assess other contributing factors such as foetal genetics and, more recently, immune responses in foetuses and neonates of anti-Ro/La antibody-positive women. In this review, we provide an update on the epidemiology, clinical presentation and current treatment approaches of autoimmune CHB, summarize the previously proposed pathogenic mechanisms implicating maternal autoantibodies, and discuss the recent findings of type I interferon (IFN) and innate immune activation in foetuses with autoimmune CHB and in neonates of anti-Ro/La antibody-positive mothers, and how these may contribute to autoimmune CHB pathogenesis.

Reversible complete heart block in a patient with coronavirus disease 2019.

Patients infected with novel coronavirus (SARS-CoV-2) can present with a variety of arrhythmias. We report an unusual case of reversible complete heart block (CHB) in the setting of acute coronavirus disease 2019 (COVID-19). A 23-year-old male with a history of Hodgkin's Lymphoma presented with dizziness and syncope. He was found to be in CHB associated with hypotension requiring a transvenous pacemaker. Methylprednisolone and remdesivir were started with rapid resolution of the CHB. Further study is needed to determine the mechanism of CHB in COVID-19. This case underscores the importance of including COVID-19 in one's differential diagnosis for acute CHB.

Case Report Detailing an Inferior Myocardial Infarction, Third-Degree Heart Block, and Cardiogenic Shock.

An infarction in the right coronary artery affects the inferior wall of the heart and can also cause impedance to the cardiac conduction system. The right coronary artery perfuses the sinoatrial and atrioventricular nodes, and a loss of blood flow contributes to a breakdown in the communication system within the heart, causing associated bradycardias, heart blocks, and arrhythmias. This case report details the prehospital and emergency care of a middle-aged man who experienced an inferior myocardial infarction, concomitant third-degree heart block, and subsequent cardiogenic shock, with successful revascularization. This case is informative for emergency clinicians to review symptoms of acute coronary syndrome, rapid lifesaving diagnostics and intervention, and the unique treatment and monitoring considerations associated with right ventricular involvement and third-degree heart block.

Intraprocedural high-degree atrioventricular block or complete heart block in transcatheter aortic valve replacement recipients with no prior intraventricular conduction disturbances.

BACKGROUND: Conduction disturbances are the most frequent complication of transcatheter aortic valve replacement (TAVR). However, no data exists regarding the outcomes of intraprocedural high-degree atrioventricular block (HAVB) or complete heart block (CHB) in patients without previous conduction disturbances. OBJECTIVES: The aim of this study was to evaluate the outcomes of intraprocedural-HAVB/CHB in patients without previous intraventricular conduction disturbances. METHODS: The occurrence of intraprocedural-HAVB/CHB was assessed in 676 consecutive patients undergoing TAVR, and two groups were established according to its duration: persistent-HAVB/CHB (PHAVB/CHB) and transient-HAVB/CHB (THAVB/CHB), not present at the end of the procedure. RESULTS: Intraprocedural-HAVB/CHB occurred in 50 patients (7.4%), being persistent in 32 (64.0%), and transient in 18 (36.0%). The use of Medtronic Corevalve Revalving System (MCRS) and a greater oversizing of the valve increased the risk of intraprocedural-HAVB/CHB (p < 0.001). Permanent pacemaker implantation (PPI) was more frequent in the PHAVB/CHB than in the THAVB/CHB group (96.9% vs. 33.3%; p < 0.001). At 1-month follow-up, the PHAVB/CHB group showed a 98% ventricular pacing rate (VPR) compared to 16% in the THAVB/CHB group (p < 0.001), and similar VPR were observed at 1-year follow-up (98% vs. 37%, p < 0.001). Left ventricular ejection fraction (LVEF) decreased at 1-year follow-up in patients with PHAVB/CHB (-3.9 ± 1.8%, p = 0.003). CONCLUSIONS: In TAVR recipients with no prior intraventricular conduction disturbances, intraprocedural-HAVB/CHB occurred in 7.4% of cases. HAVB/CHB was persistent in most cases and determined a high rate of PPI post-TAVR. Very high VPR at 1- and 12-month follow-up were observed, which in turn was associated with a negative effect on LVEF. These results support early PPI and close follow-up in patients developing intraprocedural-PHAVB/CHB.

Chest palpitations in a teenager as an unusual presentation of Lyme disease: case report.

BACKGROUND: The incidence of Lyme disease (LD) in North America has increased substantially in the past two decades. Concomitant with the increased incidence of infection has been an enhancement in the recognition of LD complications. Here, we report a case of Lyme carditis complicated by heart block in a pediatric patient admitted to our children's hospital. What is unique about this case is that the complaint of chest palpitations is an infrequent presentation of LD, and what it adds to the scientific literature is an improved understanding of LD in the pediatric population. CASE PRESENTATION: The patient was a 16-year-old male who presented with the main concerns of acute onset of palpitations and chest pain. An important clinical finding was Erythema migrans (EM) on physical exam. The primary diagnoses were LD with associated Lyme carditis, based on the finding of 1st degree atrioventricular heart block (AVB) and positive IgM and IgG antibodies to Borrelia burgdorferi. Interventions included echocardiography, electrocardiography (EKG), and intravenous antibiotics. The hospital course was further remarkable for transition to 2nd degree heart block and transient episodes of complete heart block. A normal sinus rhythm and PR interval were restored after antibiotic therapy and the primary outcome was that of an uneventful recovery. CONCLUSIONS: Lyme carditis occurs in < 5% of LD cases, but the "take-away" lesson of this case is that carditis can be the presenting manifestation of B. burgdorferi infection in pediatric patients. Any patient with suspected Lyme carditis manifesting cardiac symptoms such as syncope, chest pain, or EKG changes should be admitted for parenteral antibiotic therapy and cardiac monitoring. The most common manifestation of Lyme carditis is AVB. AVB may manifest as first-degree block, or may present as high-grade second or third-degree block. Other manifestations of Lyme carditis may include myopericarditis, left ventricular dysfunction, and cardiomegaly. Resolution of carditis is typically achieved through antibiotic administration, although pacemaker placement should be considered if the PR interval fails to normalize or if higher degrees of heart block, with accompanying symptoms, are encountered. With the rising incidence of LD, providers must maintain a high level of suspicion in order to promptly diagnose and treat Lyme carditis.

Left bundle branch area. A new site for physiological pacing: a pilot study.

Chronic RV pacing may lead to pacing induced cardiomyopathy in some patients and results in a higher risk of development of LV systolic dysfunction, heart failure, mitral regurgitation and atrial fibrillation. His bundle pacing emerged as the most physiologic form of ventricular pacing. However, wide adoption of this technique in routine clinical practice is limited by higher capture thresholds at implant sometimes, lower R wave amplitudes, atrial over sensing and increased risk for late rise in pacing thresholds (resulting in the need for lead revisions). Some recent studies have focused on left bundle branch area pacing as a solution to these problems. In our study, we have compared left bundle branch area pacing (in 22 patients) with conventional right ventricular apical pacing (in 28 patients) who presented to us with conventional indications for pacemaker implantations in term of procedure and fluoroscopy time and short-term lead performance and left ventricular function. The results of our study showed that left bundle branch area pacing is associated with shortened QRS duration (22.36 ± 9.36 ms) and better LV function (higher left ventricular ejection fraction 64.00 ± 3.03 vs. 59.73 ± 6.73 with a p value of 0.013 and lower left ventricular diastolic internal diameter 4.58 ± 0.32 vs. 5.23 ± 0.40 cm with a p value of < 0.001) in comparison to right ventricular apical pacing. The total procedure time and fluoroscopy time was similar (63.15 ± 7.02 vs. 55.15 ± 6.16 min, p value 0.142 and 6.08 ± 1.42 vs. 5.06 ± 1.30 min, p value 0.332 respectively) in left bundle branch area pacing group. The results of this study indicate that left bundle branch area pacing may be an option for physiological pacing in patients requiring a high percentage of ventricular pacing.

Risk factors for permanent pacemaker implantation in patients receiving a balloon-expandable transcatheter aortic valve prosthesis.

Permanent pacemaker implantation (PPI) is a widely recognized complication associated with TAVI (incidence up to 20%). Smaller registries have identified several variables associated with PPI. The objective was to validate patient- and transcatheter aortic valve implantation (TAVI)-related procedural variables associated with PPI. We performed a retrospective analysis of patients from six European centers undergoing TAVI with the Edwards SAPIEN 3 prosthesis. Baseline variables and pre-procedural ECG characteristics and CT-scans were taken into account. Data for 1745 patients were collected; 191 (10.9%) required PPI after TAVI. The baseline variables pulmonary hypertension (OR 1.64; 95% CI 1.01-2.59), QRS duration > 117 ms (OR 2.58; 95% CI 1.73-3.84), right bundle branch block (RBBB; OR 5.14; 95% CI 3.39-7.72), left anterior hemi block (OR 1.92; 95% CI 1.19-3.02) and first-degree atrioventricular block (AVB, OR 1.63; 95%CI 1.05-2.46) were significantly associated with PPI. RBBB (OR 8.11; 95% CI 3.19-21.86) and first-degree AVB (OR 2.39; 95% CI 1.18-4.66) remained significantly associated in a multivariate analysis. Procedure-related variables included access site (TF; OR 1.97; 95% CI 1.07-4.05), implanted valve size (29 mm; OR 1.88; 95% CI 1.35-2.59), mean TAVI valve implantation depth below the annulus > 30% (OR 3.75; 95% CI 2.01-6.98). Patients receiving PPI had longer ICU stays and later discharges. Acute kidney injury stage 2/3 was more common in patients with PPI until discharge (15.2 vs. 3.1%; p = 0.007), but was not statistically significant thereafter. Further differences in outcomes at 30 days did not reach significance. The data will aid pre- and post-procedural patient management and prevent adverse long-term outcomes.Clinical Trial: NCT03497611.

Fulminant Myocarditis with Combination Immune Checkpoint Blockade.

Immune checkpoint inhibitors have improved clinical outcomes associated with numerous cancers, but high-grade, immune-related adverse events can occur, particularly with combination immunotherapy. We report the cases of two patients with melanoma in whom fatal myocarditis developed after treatment with ipilimumab and nivolumab. In both patients, there was development of myositis with rhabdomyolysis, early progressive and refractory cardiac electrical instability, and myocarditis with a robust presence of T-cell and macrophage infiltrates. Selective clonal T-cell populations infiltrating the myocardium were identical to those present in tumors and skeletal muscle. Pharmacovigilance studies show that myocarditis occurred in 0.27% of patients treated with a combination of ipilimumab and nivolumab, which suggests that our patients were having a rare, potentially fatal, T-cell-driven drug reaction. (Funded by Vanderbilt-Ingram Cancer Center Ambassadors and others.).

Permanent pacing and conduction recovery in patients undergoing cardiac surgery for active infective endocarditis in an Australian Tertiary Center.

BACKGROUND: Postoperative heart block is common among patients undergoing surgery for infective endocarditis (IE). Limited data exists allowing cardiologists to predict who will require permanent pacemaker (PPM) implantation postoperatively. We aimed to determine the rate of postoperative PPM insertion, predictors for postoperative PPM, and describe PPM utilization and rates of device-related infection during follow-up. MATERIALS AND METHODS: A retrospective analysis was performed of 191 consecutive patients from a single institution who underwent cardiac surgery for IE between 2001 and 2017. Preoperative and operative predictors for postoperative PPM were evaluated using univariate and multivariate logistic regression. RESULTS: The rate of postoperative PPM implantation was 11% (17/154). The PPM group had more preoperative prolonged PR interval alone (33% vs 12%; P = .03), coexistent prolonged PR and QRS durations (13% vs 2%; P = .01), infection beyond the valve leaflets (82% vs 41%; P = .001), aortic root debridement (65% vs 23%; P = <.001), patch repair (47% vs 20%; P = .01), postoperative prolonged PR interval (50% vs 24%; P = .01), and prolonged QRS duration (47% vs 15%; P = .001). On multivariate analysis, infection beyond the valve leaflets emerged as an independent predictor for postoperative PPM (odds ratio, 1.94, 95% confidence interval, 1.14-3.28; P = .014). A reduction in PPM utilization was observed in five patients while eight patients continued to show significant ventricular pacing with no underlying rhythm at 12 months. There were no device-related infections. CONCLUSION: Postoperative PPM was required in 11% of patients undergoing surgery for IE over a 16-year period. Infection beyond the valve leaflet was an independent predictor for postoperative PPM insertion.

Benefits of fetal echocardiographic surveillance in pregnancies at risk of congenital heart block: single-center study of 212 anti-Ro52-positive pregnancies.

OBJECTIVES: Assuming that autoimmune congenital heart block (CHB) is a progressive disease amenable to therapeutic modulation, we introduced a surveillance program for at-risk pregnancies with the dual aim of investigating if fetal atrioventricular block (AVB) could be detected and treated before becoming complete and irreversible, and to establish the incidence of AVB I, II and III in a large prospective cohort. METHODS: This was a prospective study of 212 anti-Ro52 antibody-exposed pregnancies at risk of fetal AVB that were followed weekly between 18 and 24 weeks' gestation at our tertiary fetal cardiology center from 2000 to 2015. A 12-lead electrocardiogram (ECG) was recorded within 1 week after birth. Fetal Doppler atrioventricular (AV) intervals were converted to Z-scores using reference standard values derived from normal pregnancies. Each fetus was represented by the average value of the two recordings, obtained at two consecutive visits, which resulted in the longest AV interval. AV interval values were classified into normal AV conduction (Z-score ≤ 2.0) and three levels of delayed AV conduction: Z-score > 2.0 and ≤ 3.0, Z-score > 3.0 and ≤ 4.0, and Z-score > 4.0. RESULTS: AVB II or III developed in 6/204 (2.9%) pregnancies without a CHB history and 1/8 (12.5%) of those with a CHB history. AV intervals > 2 and ≤ 3, > 3 and ≤ 4, and > 4 were detected in 16.0%, 7.5% and 2.8% of cases, respectively, and were related to the PR interval on 185 available ECGs. Three of the five cases with AVB III and one of two cases with 2:1 AVB II developed within 1 week of AV interval Z-score of 1.0, 1.9, 2.8 and 1.9, respectively. Transplacental treatment with betamethasone was associated with restoration of 1:1 AV conduction in the two fetuses with AVB II, with a better long-term result (normal ECG vs AVB I or II) observed in the case in which treatment was started within 1 week after AVB developed. Betamethasone treatment did not reverse AVB III, although a temporary effect on AV conduction was observed in 1/5 cases. Notably, the three cases in which treatment was started within 1 week after AVB III development responded with a higher ventricular rate than the other two cases and did not require pacemaker implantation until a later age (2-5 years vs 1.5-2 months). CONCLUSION: Fetal AV interval is a poor predictor of CHB progression, but CHB surveillance still allows detection of fetuses with AVB II or III shortly after its development, allowing for timely treatment initiation and potentially better outcome. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Interesting case of narrow QRS tachycardia. What is the mechanism?

A 67-year-old female presented with history of recurrent palpitations. During one of the episodes of palpitation, a narrow QRS tachycardia was recorded and it was reported to be terminated with intravenous adenosine. The 12-lead electrocardiogram (ECG) showed no manifest ventricular preexcitation. ECG was within normal limits. The patient underwent an electrophysiology study after informed consent. She was taking calcium channel blockers and that was stopped five half-lives prior to the procedure. As catheters were being placed, a narrow QRS tachycardia got induced. During the study, there was development of right bundle branch block during the tachycardia. What is the mechanism of the tachycardia?

Electro-vectorcardiographic demonstration of rate-independent transient left posterior fascicular block.

Left posterior fascicular block (LPFB) is a rare intraventricular conduction disorder of rare occurrence, especially as an isolated entity. Its transient form is even rarer and maybe rate-independent or rate-dependent intermittent LPFB (phase 3 block, tachycardia-dependent and phase 4 block or bradycardia-dependent). We present a case of a young adult male whose baseline ECG/VCG showed the typical LPFB pattern. A treadmill stress test revealed rate-independent intermittent LPFB with random occurrence. Imaging exams ruled out structural heart disease. To our knowledge, this is the first case in the literature of a rate-independent intermittent LPFB with no underlying structural heart disease.

Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis - a case report.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block. CASE PRESENTATION: We report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy. CONCLUSIONS: A limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.

Lev's Syndrome: A rare case of progressive cardiac conduction disorder presenting to the emergency department.

Lev's Syndrome is a rare, progressive cardiac conduction defect (PCCD) due to myocardial fibrosis first described by Maurice Lev in 1964. This condition, proposed to start in the fourth decade of life, involves a sclerotic fibro-fatty degeneration of the Bundle of His and Purkinje fibers, which Lev proposed caused increasing AV delay with age. With the prevalence of electrocardiogram (ECG) use in the emergency department (ED) for cardiac- and non-cardiac complaints, dysrhythmias can be incidentally found and confuse diagnosis and disposition. We highlight the case of an 84-year-old male who presented to the ED for acute onset of diffuse facial paresthesias with elevated blood pressure at home and was found to be significantly bradycardic on initial evaluation. On serial ECGs, the conduction rhythm changed from an initial new first-degree atrioventricular (AV) block with left bundle branch block (LBBB), to a later first-degree AV block without LBBB. Cardiology was consulted. Serial ECGs demonstrated an evolving conduction block arrhythmia consistent with Lev's Syndrome. Here we describe a case of symptomatic bradycardia found to be consistent with Lev's Syndrome.