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Cardiac Electrophysiology Clinics Jun 2024The following case series presents three different pediatric patients with SCN5A-related disease. In addition, family members are presented to demonstrate the variable... (Review)
Review
The following case series presents three different pediatric patients with SCN5A-related disease. In addition, family members are presented to demonstrate the variable penetrance that is commonly seen. Identifying features of this disease is important, because even in the very young, SCN5A disorders can cause lethal arrhythmias and sudden death.
Topics: Humans; NAV1.5 Voltage-Gated Sodium Channel; Long QT Syndrome; Male; Female; Arrhythmias, Cardiac; Child; Electrocardiography; Child, Preschool; Adolescent; Infant
PubMed: 38749643
DOI: 10.1016/j.ccep.2023.10.007 -
Journal of Veterinary Cardiology : the... Apr 2024A seven-year-old male castrated Labrador Retriever presented emergently due to concern for pacemaker malfunction five years after successful transvenous pacemaker...
A seven-year-old male castrated Labrador Retriever presented emergently due to concern for pacemaker malfunction five years after successful transvenous pacemaker implantation to treat partial atrial standstill. On presentation, the dog's pulse rate was 30-50 beats per minute. An electrocardiogram showed no spontaneous atrial activity or paced ventricular activity. Pacemaker interrogation revealed an increased impedance of 7557 ohms, indicating a lead malfunction. Thoracic radiographs confirmed the lead was fractured and had excessive coiling. The transvenous pacing system was turned off, left in place, and an epicardial pacing system was implanted the following day. The dog was discharged with no perioperative complications. The dog eventually required escalated medical therapy for progressive cardiac disease and was euthanized two years after implantation of the replacement pacemaker. This manuscript illustrates a complete lead fracture and excessive lead coiling, which has not previously been detailed in veterinary medicine.
Topics: Dogs; Animals; Male; Pacemaker, Artificial; Dog Diseases; Equipment Failure; Electrocardiography
PubMed: 38432142
DOI: 10.1016/j.jvc.2024.02.004 -
Medicine Feb 2024Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and...
RATIONALE
Previous studies have found that the main treatment of sinus arrest is pacemaker treatment. It is rare to have 12 s of sinus arrest after radiofrequency ablation, and whether a permanent pacemaker is implanted immediately in this case is not described in the guidelines.
PATIENT CONCERNS
A 76-year-old male patient with persistent atrial fibrillation (AF) developed sinus arrest lasting 12 s in the early morning of the fourth day after using radiofrequency ablation for pulmonary vein isolation.
DIAGNOSIS
The patient was diagnosed with AF and sinus arrest.
INTERVENTIONS
The patient received cardiopulmonary resuscitation, intravenous injection of atropine 1 mg, and intravenous infusion of isoproterenol 1mg and immediately recovered consciousness thereafter. Approximately, 1.5 h later, the patient underwent surgery to install a temporary pacemaker in the right femoral vein.
OUTCOMES
The patient had repeated episodes of sinus arrest after the implantation of a temporary pacemaker. After 3 weeks, the patient stabilized and was discharged. The patient was followed up for 1 year and did not experience any recurrence of sinus arrest or AF.
LESSONS
We consider the potential for postoperative myocardial edema, injury to the sinoatrial node during the procedure, propafenone poisoning, and autonomic dysfunction as contributors to the occurrence of sinus arrest after radiofrequency ablation. When sinus arrest occurs after radiofrequency ablation, we can choose the appropriate treatment according to the patient's condition.
Topics: Male; Humans; Aged; Treatment Outcome; Catheter Ablation; Atrial Fibrillation; Radiofrequency Ablation; Heart Arrest; Heart Atria; Heart Block; Cardiomyopathies; Genetic Diseases, Inborn
PubMed: 38394505
DOI: 10.1097/MD.0000000000037191 -
The Journal of Innovations in Cardiac... Jan 2024
PubMed: 38304085
DOI: 10.19102/icrm.2024.15016 -
Journal of Veterinary Cardiology : the... Feb 2024To investigate the clinical and echocardiographic presentation of dogs with persistent atrial standstill (PAS), identify variables measured at first presentation that...
OBJECTIVES
To investigate the clinical and echocardiographic presentation of dogs with persistent atrial standstill (PAS), identify variables measured at first presentation that could predict their survival, and document the progression of the disease after pacing.
MATERIALS AND METHODS
Retrospective study of medical records of dogs diagnosed with PAS at three referral hospitals of the United Kingdom over seven years.
RESULTS
Twenty-six dogs were diagnosed with PAS during the study period. Median age of the population was three years (range: seven months-12.5 years). The most common clinical sign was syncope (14/26). Twenty-four dogs received artificial pacemakers (PM). Major complications after PM implantation were observed in four dogs (four/24). Serial echocardiographic examinations showed that cardiac dimensions of PAS dogs with left atrial or left ventricular dilation at first presentation did not return to reference range after pacing. Further dilation of the cardiac chambers, recurrence of congestive heart failure (CHF), or development of new episodes of CHF were documented in seven, four, and 10 PAS dogs, respectively, despite pacing. Median survival time for cardiac-related deaths after PM implantation was 1512 days (18-3207). Neither CHF nor echocardiographic variables at presentation predicted survival after PM implantation in PAS dogs.
CONCLUSIONS
Persistent atrial standstill (PAS) is an uncommon bradyarrhythmia, occurring in young adult dogs. Affected dogs were often presented with syncope. Whilst syncope resolved, cardiac remodeling persisted after PM implantation. Long-term survival was favorable after PM implantation and was not predicted by congestive status or cardiac chamber size at first presentation.
Topics: Dogs; Animals; Retrospective Studies; Heart Atria; Heart Block; Heart Failure; Syncope; Cardiac Pacing, Artificial; Dog Diseases; Cardiomyopathies; Genetic Diseases, Inborn
PubMed: 38150959
DOI: 10.1016/j.jvc.2023.11.016 -
Frontiers in Cardiovascular Medicine 2023A pregnant patient had symptomatic atrial standstill and indications for pacing therapy with an expected high ventricular pacing ratio. With the consideration of...
A pregnant patient had symptomatic atrial standstill and indications for pacing therapy with an expected high ventricular pacing ratio. With the consideration of potential pacing-induced cardiomyopathy in the future we conducted zero-fluoro left bundle branch pacing (zLBBP) implantation for heart failure prevention. An ex vivo 3D cardiac model (Medtronic, USA) was used preoperatively to simulate the zLBBP implantation to improve procedure safety and efficiency. Intraoperatively, the simulation steps were followed, and a combination of electroanatomic navigation systems (EANS) and intracardiac echocariography (ICE) were used to ensure that the procedure was performed efficiently and safely.
PubMed: 38130689
DOI: 10.3389/fcvm.2023.1325442 -
Journal of Cardiovascular... Feb 2024Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience...
INTRODUCTION
Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node.
OBJECTIVE
Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP.
METHODS
This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods.
RESULTS
Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA.
CONCLUSION
CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB.
Topics: Humans; Female; Child; Adolescent; Young Adult; Adult; Male; Atrioventricular Block; Sick Sinus Syndrome; Atrioventricular Node; Syncope; Heart Atria; Heart Block; Cardiomyopathies; Genetic Diseases, Inborn
PubMed: 38038245
DOI: 10.1111/jce.16145 -
Anales de Pediatria Dec 2023
Topics: Humans; Cardiomyopathies; Heart Block; Heart Atria; Muscular Dystrophies
PubMed: 37949737
DOI: 10.1016/j.anpede.2023.11.001 -
BMC Oral Health Sep 2023Intraoperative sinus arrest is rarely seen during zygomatic fracture treatment. The patient was diagnosed with sick sinus syndrome based on repeated postoperative sinus...
BACKGROUND
Intraoperative sinus arrest is rarely seen during zygomatic fracture treatment. The patient was diagnosed with sick sinus syndrome based on repeated postoperative sinus arrest, which could have resulted in death if diagnosed late, making this case very significant to report.
CASE PRESENTATION
Sick sinus syndrome is an arrhythmia associated with reduced automaticity of the sinoatrial node or impaired sinoatrial node conduction. We report the case of a 67-year-old man diagnosed with the syndrome after a sinus arrest that occurred during a zygomatic fracture treatment. The patient had cheek pain and mouth opening disorder, dizziness after fainting and sustaining a facial injury. Preoperative examination determined that the syncope was due to drug-induced arrhythmia, and surgery was authorized after drug withdrawal. During the operation, sinus arrest was observed due to trigeminal vagal reflex, and heart rate was restarted by stopping the operation and chest compressions. After the surgery, the patient showed symptoms of dizziness and palpitations, and sinus arrest following atrial fibrillation and supraventricular tachycardia, which was diagnosed as sick sinus syndrome, and a pacemaker was implanted. Currently, 8 years have passed since the surgery, and there are no symptoms of mouth opening disorder, dizziness, or palpitations.
CONCLUSIONS
In the case of maxillofacial injuries due to syncope, cardiogenic syncope is a possibility, and repeated syncope is a risk for death due to delayed diagnosis. There are no reports of maxillofacial trauma leading to a diagnosis of sick sinus syndrome. The purpose of this case report is to disseminate the importance of diagnosing the cause of syncope as well as injury treatment.
Topics: Male; Humans; Aged; Zygomatic Fractures; Sick Sinus Syndrome; Dizziness; Syncope; Atrial Fibrillation
PubMed: 37726766
DOI: 10.1186/s12903-023-03413-0 -
European Heart Journal. Case Reports Jun 2023Juvenile onset of extensive atrial electromechanical failure, including atrial standstill, is a rare disease entity that may precede ventricular cardiomyopathy. Genetic...
Juvenile-onset multifocal atrial arrhythmias, atrial standstill and compound heterozygosity of genetic variants in : sentinel event for evolving dilated cardiomyopathy-a case report.
BACKGROUND
Juvenile onset of extensive atrial electromechanical failure, including atrial standstill, is a rare disease entity that may precede ventricular cardiomyopathy. Genetic variants associated with early-onset atrioventricular (AV) cardiomyopathy are increasingly recognized.
CASE SUMMARY
A 16-year-old patient presented with atrial brady- and tachyarrhythmias and concomitant impaired atrial electromechanical function (atrial standstill). The atrial phenotype preceded the development of a predominantly right-sided AV dilated cardiomyopathy with pronounced myocardial fibrosis. A His-bundle pacemaker was installed for high-degree AV conduction block and sinus arrest. Using familial-based whole-exome sequencing, a missense mutation and a copy number variant deletion (compound heterozygosity) of the gene (involved in ribosomal RNA synthesis) were identified.
DISCUSSION
Juvenile onset of severe atrial electromechanical failure with atrial arrhythmias should prompt deep pheno- and genotyping and calls for vigilance for downstream cardiomyopathic deterioration.
PubMed: 37501913
DOI: 10.1093/ehjcr/ytad255