Complete Atrioventricular Block Research Articles

BRAНH syndrome: clinical case

The article describes a clinical case of BRAHH syndrome in a patient with arterial hypertension and permanent atrial fibrillation (AF). The patient took perindopril 10 mg, indapamide 2.5 mg, amlodipine 10 mg, bisoprolol 2.5-5 mg daily. She was admitted to a hospital complaining of severe weakness, a heart rate decreases to 38 beats per minute against the background of high blood pressure. During the examination, she was diagnosed with complete atrioventricular block against the background of AF, stage 4 chronic kidney disease and severe hyperkalemia (potassium 8.7 mmol/l). The patient was prescribed treatment aimed at eliminating hyperkalemia, and temporary pacing was established. Against this background, her condition improved, and the complete atrioventricular blockade was resolved. This clinical example meets the criteria of BRAHH syndrome, since against the background of taking an atrioventricular node blocker in a small dose, a patient with chronic kidney disease and severe hyperkalemia developed complete atrioventricular block against the background of AF, accompanied by high blood pressure.

A national analysis of systemic adverse events of beta-blockers used for glaucoma therapy

Purpose To evaluate systemic complications for timolol, carteolol, levobunolol, and/or betaxalol by using an FDA Federal Adverse Event Reporting System (FAERS) Methods We evaluated FAERS for adverse events associated with β-blocker use for glaucoma. All reported symptoms were reviewed to identify systemic adverse events and to detect safety signals, defined as information on a new or known side effect that may be caused by a medicine. We used the proportional reporting ratio (PRR), reporting odds ratio (ROR), empirical Bayes geometric mean (EBGM), and information component (IC) as a part of a disproportionality analysis comparing the frequency of β-blocker symptoms with all other adverse event reports. We considered a signal to be detected when all four disproportionality analysis metrics were positive. Results We found 10,500,309 total adverse event reports from the FAERS database 2004-2022Q3, which included 8,793 case reports with a primary suspect of a β-blocker use for glaucoma. 1,838 unique adverse symptoms were reported were associated with β-blocker. Regarding outcomes, there were 165 (1.88%) reports of disability, 671 (7.63%) reports of hospitalisation, and 1,934 (21.99%) reports of some other unspecified complication. Regarding adverse events, the most reported general, cardiac, and respiratory symptoms were respectively dizziness (n = 281), bradycardia (n = 145), and dyspnoea (n = 195). 256 (2.91%) cases of death were reported. We found significant signals on bradycardia (n = 145), complete atrioventricular block (n = 38), and bronchospasm (n = 23). No allergic, endocrine, constitutional, or gastrointestinal symptoms generated positive signals. Conclusion β-blocker use in glaucoma therapy can be rarely associated with serious systemic and life-threatening complications.

Incidence and Outcomes of Iatrogenic Complete Atrioventricular Block After Congenital Heart Surgery

BackgroundIatrogenic complete atrioventricular block (ICAVB) has long been noted as a major complication after congenital heart surgery (CHS), and it contributes to complex postoperative care and potentially affects patients’ outcomes. MethodsThis study is a retrospective review of the Pediatric Health Information System database from January 1, 2004 to September 30, 2023. All patients who underwent The Society of Thoracic Surgeons benchmark procedures were included. International Classification of Diseases (ICD) 9th and 10th editions were used to identify diagnoses and procedures. All patients with a diagnosis of complete atrioventricular block and placement of a permanent pacemaker after CHS but in the same hospitalization were identified as having ICAVB. ResultsA total of 42,332 patients were identified, with 17,106 (41%) female and 23,042 (55%) non-Hispanic White and with a median age of 5.4 months [interquartile range, 0.4-25.8 months]. Of those patients, 246 (0.6%) had ICAVB. The procedure with the highest incidence of ICAVB was the arterial switch operation with ventricular septal defect (VSD) repair (74 of 1552; 4.5%). On multivariable analysis, the arterial switch operation with VSD repair had the highest adjusted odds of ICAVB (odds ratio, 5.41; 95% CI, 3.57-8.19; P < .001) when compared with isolated VSD repair. A diagnosis of endocarditis was significantly associated with ICAVB. Center volume was not associated with ICAVB. ICAVB was associated with a 121% (95% CI, 98.5%-146.8%) increase in length of stay (P < .001) and increased in-hospital mortality (odds ratio, 2.26; 95% CI, 1.34-3.82; P < .001). ConclusionsThe overall incidence of ICAVB after CHS is low. However, certain procedures have incidences as high as 4.5%. ICAVB is associated with increased postoperative mortality and length of stay. Further work is needed to identify drivers of variation among centers to improve overall outcomes.

Mad honey (wild honey) poisoning: clinical case series from Nepal.

Mad honey is commonly used for hypertension, and coronary artery disease, and as a sexual stimulant. Patients with mad honey poisoning present with dizziness, nausea, syncope, blurred vision, bradycardia, and hypotension with ECG findings of sinus bradycardia, complete AV block, and ST elevation. Here, the authors report five cases admitted to our tertiary care center following the consumption of mad honey. The amount of ingestion of honey varies from 1 to 2 teaspoons (~10-20ml). Most of the cases presented with chief complaints of nausea, dizziness, and vomiting, and all the cases had hypotension and bradycardia. Two cases were admitted to the ward and three of them were admitted to the ICU for further management. They were managed with intravenous fluid, injection atropine along with adjunctive vasopressor and oxygen whenever necessary. Mad honey contains grayanotoxin extracted from the nectar of Rhododendron species. This honey contains grayanotoxin, which binds to sodium channels in its open state causing hyperpolarization of the sodium channel predominantly causing gastrointestinal, neurological, and respiratory symptoms. Intravenous fluids and injection atropine are the mainstays of management in an ICU setup. Some also may require vasopressors. Mad honey poisoning is rare, and limited cases have been reported in Nepal. Physicians should consider mad honey poisoning in cases with ingestion history and clinical symptoms, as it may be a clinical diagnosis due to limited lab tests for grayanotoxin intoxication. Supportive management still forms the cornerstone for its management after diagnosis.

A proposed algorithm for management of patients with left bundle branch block post-TAVR: 1-year follow-up

BackgroundTAVR has revolutionized the management of aortic stenosis and has become the standard of care across a broad spectrum of patients with aortic stenosis. However, it is still associated with high incidence of conduction abnormalities, particularly new LBBB. Management of these patients remains a challenge. ObjectiveTo assess the clinical outcomes of patients with post-TAVR conduction disorders managed according to a pre-specified institutionally developed algorithm. MethodsA retrospective analysis including all patients undergoing TAVR in our institute between October-2018 to December-2022 was performed. Patients with new LBBB were managed according to the algorithm comprising QRS width and electrophysiology study. In-hospital and one-year clinical outcomes were assessed. ResultsA total of 230 patients were included in the present analysis. Seventy patients (30.4%) developed new LBBB after TAVR. Overall, 44 patients (19.1%) required permanent pacemaker implantation (PPM): 20 patients (8.7%) with Mobitz II, complete AV block or alternating bundle branch block, 21 patients (9.1%) with persistent new LBBB, and 3 others (1.3%) per physician discretion. During 1-year follow-up, only 3 patients required late PPM, of whom only 1 patient with new LBBB. There was no difference in mortality or heart failure hospitalizations between the PPM and No PPM groups. Multivariable analysis identified atrial fibrillation, chronic kidney disease, and pre-TAVR RBBB as independent predictors for PPM following TAVR. ConclusionOur findings suggest that the presented algorithm may serve as a safe and efficacious strategy for management of patient with post-TAVR LBBB, although the PPM rate may be further reduced.

Dual-chamber vs. single-chamber pacemaker in patients in sinus rhythm with an atrioventricular block: a nationwide cohort study.

In complete atrioventricular block (AVB) with underlying sinus rhythm, it is recommended to implant a dual-chamber pacemaker rather than a single-chamber pacemaker. However, no large-scale study has been able to demonstrate the superiority of this choice on hard clinical criteria such as morbimortality. This retrospective observational study included all patients who received a primary pacemaker implantation in the indication of complete AVB with underlying sinus rhythm in France, based on the national administrative database between January 2013 and December 2022. After propensity score matching, we obtained two groups containing 19 219 patients each. The incidence of all-cause mortality was 9.22%/year for the dual-chamber pacemaker group, compared with 11.48%/year for the single-chamber pacemaker group (hazard ratio (HR) 0.807, P < 0.0001]. Similarly, there was a lower incidence of cardiovascular mortality (HR 0.766, P < 0.0001), heart failure (HR 0.908, P < 0.0001), atrial fibrillation (HR 0.778, P < 0.0001), and ischaemic stroke (HR 0.873, P = 0.008) in the dual-chamber pacemaker group than in the single-chamber pacemaker group. Regarding re-interventions and complications, there were fewer upgrades (addition of atrial lead or left ventricular lead) in the dual-chamber group (HR 0.210, P < 0.0001), but more haematomas (HR 1.179, P = 0.006) and lead repositioning (HR 1.123, P = 0.04). In the indication of complete AVB with underlying sinus rhythm, our results are consistent with current recommendations to prefer implantation of a dual-chamber pacemaker rather than a single-chamber pacemaker for these patients. Implantation of a dual-chamber pacemaker is associated with a lower risk of mortality, heart failure, atrial fibrillation, and stroke during follow-up.

A clinical case of surgical treatment of coronary heart disease against the background of concomitant pathology: infective endocarditis of the aortic valve, Gerbode defect and complete atrioventricular block

A clinical case of surgical treatment of coronary heart disease against the background of concomitant pathology: infective endocarditis of the aortic valve, Gerbode defect and complete atrioventricular block

Accelerated idioventricular rhythm after left bundle branch pacing lead implantation

Premature ventricular contractions with right bundle branch block morphology induced during left bundle branch (LBB) pacing (LBBP) lead implantation serve as a marker indicating that the lead is close to or has reached the LBB region. However, no reports to date have described accelerated idioventricular rhythm (AIVR) induced by LBBP lead deployment.We herein describe a patient who underwent LBBP for complete atrioventricular block. The patient's intrinsic escape rhythm was overtaken by AIVR induced immediately after LBBP lead deployment.AIVR is another marker indicating that the lead has reached the LBB region.

Temporal Trends in Patient Characteristics and Clinical Outcomes of TAVR: Over a Decade of Practice.

Background/Objective: Transcatheter aortic valve replacement (TAVR) is indicated for severe aortic stenosis patients with a prohibitive surgical risk. However, its use has been expanding in recent years to include intermediate- and low-risk patients. Thus, registry data describing changes in patient characteristics and outcomes are needed. The aim of this study was to analyse the temporal changes in patient profiles and clinical outcomes of all-comer TAVR. Methods: Baseline characteristics and VARC-3 outcomes of 1632 consecutive patients undergoing TAVR between 2008 and 2021 were analysed. Results: The annual rate of TAVR increased from 30 procedures in 2008-2009 to 398 in 2020-2021. Over the follow-up period, patient age decreased from 85 ± 4 to 80 ± 6.8 (p < 0.001) and the STS score decreased from 5.9% to 2.8% (p < 0.001). Procedural characteristics significantly changed, representing a shift into a minimally invasive approach: adoption of local anaesthesia (none to 48%, p < 0.001) and preference of transfemoral access (74% in 2011-2012 vs. 94.5% in 2020-2021, p < 0.001). The rates of almost all procedural complications decreased, including major vascular and bleeding complications, acute kidney injury (AKI) and in-hospital heart failure. There was a striking decline in rates of complete atrioventricular block (CAVB) and the need for a permanent pacemaker (PPM). PPM rates, however, remain high (17.8%). Thirty-day and one-year mortality significantly declined to 1.8% and 8.3%, respectively. Multivariable analysis shows that AKI, bleeding and stroke are strong predictors of one-year mortality (p < 0.001). Conclusions: The TAVR procedure has changed dramatically during the last 14 years in terms of patient characteristics, procedural aspects and device maturity. These shifts have led to improved procedural safety, contributing to improved short- and long-term patient outcomes.

Extraction of lumenless pacing leads from the His bundle and left bundle branch area: outcomes of the high-volume centre.

The application of conduction system pacing (CSP) in clinical practice is growing, and the need for lead extraction will also increase. The data on outcomes and safety of CSP lead extraction are limited. The aim of this study was to assess procedural outcomes and safety of CSP lead removal. Forty-seven patients from the EXTRACT Registry with the indication for CSP lead removal were enrolled in the study conducted at the Department of Electrocardiology in Katowice, Poland. Extraction technique, outcomes, safety, and complication were evaluated. Forty-three (91.5%) leads were successfully removed, and 41 (87.2%) were removed with traction only. The dwelling time of 28 extracted leads was longer than 1 year, and the oldest extracted lead was implanted for 89 months. Seven (14.9%) leads were removed from the left bundle branch (LBB) area and 36 from the His bundle (HB). Transient complete atrioventricular block occurred during the procedure in two patients. In 27 out of 31 attempts (87.1%), new CSP leads were implanted: nine (33.3%) HB pacing leads and 18 (66.7%) LBB area pacing leads. The CSP lead extraction is safe and feasible with a low complication rate and high rate of CSP lead reimplantation.

Repeated complete atrioventricular block during remifentanil administration in a pediatric patient with brain tumor and acute hydrocephalus: a case report

BackgroundRemifentanil, an ultra-short-acting µ-opioid receptor agonist, is commonly used for anesthetic management due to excellent adjustability. Remifentanil is known to cause sinus bradycardia, however, because it has a direct negative chronotropic effect on the cardiac conduction system and there is an indirect negative chronotropic effect via the parasympathetic nervous system.Case presentationAn 8-year-old Japanese boy was diagnosed with acute hydrocephalus due to a brain tumor in the fourth ventricle and underwent emergency surgery. Imaging examination showed brainstem compression. Endoscopic third ventriculostomy and ventriculoperitoneal shunt surgery were scheduled. Remifentanil was started during induction of general anesthesia, but electrocardiogram showed sinus bradycardia, then Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was immediately discontinued, and we administered atropine sulfate. Complete atrioventricular block was restored to sinus rhythm. When remifentanil was restarted, however, the electrocardiogram again showed sinus bradycardia, Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was again immediately discontinued, we administered adrenaline, and then complete atrioventricular block was restored to sinus rhythm. Fentanyl was used instead of remifentanil with continuous infusion of dopamine. There has since been no further occurrence of complete atrioventricular block.ConclusionsThis is the first known case of complete atrioventricular block in a pediatric patient with increased intracranial pressure seemingly caused by administration of remifentanil.

Late onset complete atrioventricular block following surgical closure of atrioventricular defect: case report

Late onset complete atrioventricular block following surgical closure of atrioventricular defect: case report

Abstract Or118: Transcription Factor Gene Therapy for Treatment of Junctional Bradycardia in a Porcine Model of Complete Atrioventricular Block Following His Bundle Ablation

Background: Pacemaker implantation is standard treatment for patients with complete atrioventricular (AV) block. However, contraindications for device implantations create a need for alternative non-hardware cardiac pacing solutions. We developed a porcine model of complete heart block with junctional bradycardia to test a novel transcription factor-based gene therapy consisting of Tbx18, Shox2 and Tbx3 (TST). Somatic reprogramming of ventricular cardiomyocytes by TST induces pacemaker-like activity and provides biological pacing in vitro . Methods: Eight domestic swine (Yorkshire/Landrace) received catheter ablation of the AV node/His Bundle resulting in complete AV block, confirmed by dissociation of P and R waves on ECG. An implanted device pacemaker (VVI mode) maintained cardiac output during occurrences of junctional bradycardia below 50 bpm. Telemetric 3-lead ECG was recorded continuously. Subjects received TST gene therapy or control gene (GFP) via catheter-based intramuscular injection into the high right ventricular septum. Pacemaker usage and ECG were continuously monitored for 8 weeks. Results: Following AV node ablation, junctional escape rhythm decelerated into junctional bradycardia. After 3 weeks of AV block, 49.9 ± 13.1% of heartbeats were initiated by the device pacemaker. Upon reaching pacemaker dependence of 95% in an average of 27.9 ± 5.5 days, subjects received TST gene therapy or control injection. In subjects receiving TST therapy, daily pacemaker reliance decreased to 50% dependency in an average of 14.7 ± 2.3 days, while in GFP controls, daily pacemaker dependency at 50 bpm remained unchanged at &gt;98% throughout follow-up. In TST-treated subjects, reductions in pacemaker dependence were associated with increases in ventricular rates exceeding 70 bpm. Efficacy of gene therapy waned after about one month; an improvement of &gt;10% pacemaker dependency over two consecutive days was still observed on average 29.0 ± 4.1 days after TST injection. Conclusion: In a porcine model of complete AV block, ventricular junctional escape rhythm decelerated into bradycardia in less than one month. Subsequent gene therapy by TST injection into the high ventricular septum alleviated pacemaker dependency and enhanced heart rate for 29.0 ± 4.1 days. Somatic reprogramming of cardiomyocytes by TST gene delivery may provide a biological pacemaker alternative to hardware device implantation for therapeutic cardiac pacing.

Abstract Mo072: Sustained Biological Pacemaker Activity Induced by AAV9-hTBX18

Background: Sinoatrial node (SAN) dysfunction is a clinical disorder characterized by the inability of the SAN to maintain an appropriate heart rate (HR). Patients with this condition often need an electronic pacemaker. Although it is the current standard of care, devices can have complications. A biological pacemaker has been created by adenovirus expressing TBX18 as an alternative. Hypothesis: However, adenoviruses are immunogenic, limiting the duration of expression. We hypothesized that an AAV9 would promote prolonged TBX18 expression, induce somatic reprogramming in vivo, and sustained pacemaker activity. Methods: AAV9 containing GFP or TBX18 was directly injected into the myocardium of eight-week-old male Sprague Dawley rats (n=70) at low (1x10 9 GC), mid (1x10 10 GC) and high (1x10 11 GC) doses. Transient AV block was induced by tail vein injection of adenosine. A permanent complete AV block was induced by ablation of the his bundle. Hearts were harvested for optical mapping and mRNA analysis. Results: Six weeks after injection, all doses of AAV9-TBX18 had increased HR (low: 104±4, p=0.04; mid: 106±3.4, p=0.008; high: 103±4.1, p=0.04; vs GFP: 89±3 bpm) compared to AAV9-GFP when we induced AV block using adenosine. AAV9-GFP and -TBX18 mid dose injected rats underwent surgery to induce complete AV block. TBX18 group presented superior HR over the 24 hours monitored. 6-lead EKG analysis of the QRS axis in the frontal plane revealed that the ventricular ectopic beats from the AAV9-TBX18-injected group originated from the injection site. Activation maps further confirmed that the injection site originated those beats in TBX18-injected rats only. Successful reprogramming of adult ventricular myocytes into SAN-like cells was confirmed by transcriptional profiles with increased expression of TBX18 (2.2±0.5 FC, p&lt;0.0001) and Hcn2 (25.2±1.1 FC, p=0.02), and reduced Gja1 (-10.8±0.7 FC, p=0.005), Scn5a (-1.1±0.8 FC, p=0.01) and Nkx2.5 (-2.0±0.6 FC, p=0.004) relative to GFP. Conclusion: Collectively, these data indicate that the AAV-based somatic reprogramming induced by TBX18 leads to sustained biological pacemaker activity in rats with AV block. Long-term studies are needed to characterize the long-term effects of sustained TBX18 expression.

Impact of left atrial strain on clinical outcomes in patients with permanent pacemaker implantation.

This study aimed to investigate the clinical impact of pre-procedural left atrial strain (LAS) in patients undergoing permanent pacemaker implantation (PPI). This single-centre retrospective study enrolled 434 patients who were admitted for transvenous PPI between 2010 and 2020. After excluding patients with persistent atrial fibrillation, PPI for complete atrioventricular block, severe valvular disease, history of open-heart surgery and those without LAS data, 172 patients were analysed. The LAS was measured using commercially available software to calculate the average strain value of the apical four- and two-chamber views before PPI. The primary composite endpoint was hospitalization due to heart failure or cardiovascular death. Cox proportional hazard models were used to evaluate risk factors for the primary composite endpoint. The mean patient age was 78±8years, and 42% of the patients were men. PPI was performed for sick sinus syndrome in 64% and second-degree atrioventricular block in 36% of the patients. The pre-procedure left atrial reservoir strain (LASr) was 28±11%. The median follow-up period was 4.7years, and the primary endpoint was observed in 23 (13%) patients. In multivariate Cox proportional risk analysis, LASr was independently associated with the primary composite endpoint (hazard ratio, 1.08 per 1% decrease; 95% confidence interval, 1.02-1.15; P=0.007). The receiver operating characteristic curve of the LASr for the primary composite endpoint showed a cutoff value of 21% (area under the curve 0.657, P=0.004). The prognostic impact of LASr was consistent with that of sick sinus syndrome and atrioventricular block. A decreased pre-procedure LASr was associated with long-term adverse outcomes after PPI use.

Rates, predictors, and causes of readmission after transcatheter aortic valve replacement in patients with chronic kidney disease.

Transcatheter aortic valve replacement (TAVR) is a revolutionary procedure for severe aortic stenosis. The coexistence of chronic kidney disease (CKD) and TAVR introduces a challenge that significantly impacts patient outcomes. To define readmission rates, predictors, and causes after TAVR procedure in CKD stage 1-4 patients. We used the national readmission database 2018 and 2020 to look into readmission rates, causes and predictors after TAVR procedure in patients with CKD stage 1-4. Out of 24758 who underwent TAVR and had CKD, 7892 (32.4%) patients were readmitted within 90 days, and had higher adjusted odds of being females (adjusted odds ratio: 1.17, 95%CI: 1.02-1.31, P = 0.02) with longer length of hospital stay > 6 days, and more comorbidities including but not limited to diabetes mellitus, anemia, and congestive heart failure (CHF). Most common causes of readmission included CHF (18.0%), sepsis, and complete atrioventricular block. Controlling readmission predictors with very close follow-up is warranted to prevent such high rate of readmission.

Incidental Findings of Congenital Unileaflet Mitral Valves in Young Patient Presenting Complete Atrioventricular Block.

The singular leaflet mitral valve is a rare and complex entity that has been described in only a few case reports. It is commonly observed in infancy and combined with symptomatic mitral regurgitation (MR). In most asymptomatic individuals, this complication is attributed mainly to a hypoplastic posterior mitral leaflet and is usually revealed in adulthood. We present the case of a 30-year-old man who presented with chest pain and dyspnea for two days. The electrocardiogram showed complete atrioventricular block with a beat rate of 30 bpm.Echocardiography revealed an elongated anterior mitral valve leaflet and an almost completely absent posterior mitral valve leaflet with no MR. Our case is unusual presentation of a unileaflet mitral valve that should be investigated even in adulthood as these patients are likely to be at risk of developing or worsening MR later in life; hence, performing periodic echocardiograms is important for detecting the progression of MR early.

Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy

BackgroundNonsteroidal immunosuppressive therapy is a potential therapeutic strategy for cardiac sarcoidosis. However, it is not recommended as an established treatment option. This study aimed to demonstrate the clinical outcomes of patients with cardiac sarcoidosis using nonsteroidal immunosuppressants through the ILLUstration of the Management and PrognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis multicenter retrospective registry. MethodsFrom a cohort of 512 patients, 426 who received corticosteroid therapy and 26 who received other immunosuppressive therapy were included for analysis. Clinical outcomes included all-cause death, fatal ventricular arrhythmic events (FVAE), and worsening heart failure with hospitalization. ResultsNonsteroidal immunosuppressants were used for retained fluorodeoxyglucose uptake in the heart (n = 14), corticosteroid side effects (n = 7), ventricular arrhythmia (n = 4), complete atrioventricular block (n = 2), worsened extracardiac sarcoidosis (n = 2), and other reasons (n = 2). They comprised of methotrexate (n = 20), cyclosporine (n = 2), cyclophosphamide (n = 2), and azathioprine (n = 3). After the addition of a nonsteroidal immunosuppressant, corticosteroids were reduced in 14 of 26 patients (5 [5–17] mg), although no patient discontinued corticosteroids. Of the 14 patients, decreased fluorodeoxyglucose uptake was observed in seven at follow-up. Clinical outcomes were observed in 11 patients (42.3 %). Detected events included all-cause death in five patients (19.2 %), FVAE in four (15.4 %), and worsening heart failure with hospitalization in five (19.2 %), with some overlap. ConclusionsNonsteroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized with corticosteroids alone or develop corticosteroid side effects.

An unusual cause of cardiac arrest in a young infant

BackgroundAnomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age.Case presentationWe describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm.ConclusionsBy timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.

A patient with Duchenne muscular dystrophy and complete AV block undergoing a successful left bundle area pacing implantation

A patient with Duchenne muscular dystrophy and complete AV block undergoing a successful left bundle area pacing implantation