OBJECTIVE: To explore the clinical and genetic characteristics of five children with Catecholaminergic polymorphic ventricular tachycardia (CPVT). METHODS: Five children with clinical manifestations consistent with CPVT admitted to the Department of Cardiology of Children's Hospital Affiliated to Zhengzhou University from November 2019 to November 2021 were selected as the study subjects. Their clinical data were collected. Potential variants were detected by whole exome sequencing, and Sanger sequencing was used to verify the candidate variants. All patients were treated with β-blocker propranolol and followed up. RESULTS: All patients had developed the disease during exercise and presented with syncope as the initial clinical manifestation. Electrocardiogram showed sinus bradycardia. The first onset age of the 5 patients were (10.4 ± 2.19) years, and the time of delayed diagnosis was (1.6 ± 2.19) years. All of the children were found to harbor de novo heterozygous missense variants of the RYR2 gene, including c.6916G>A (p.V2306I), c.527G>C (p.R176P), c.12271G>A (p.A4091T), c.506G>T (p.R169L) and c.6817G>A (p.G2273R). Among these, c.527G>C (p.R176P) and c.6817G>A (p.G2273R) were unreported previously. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.527G>C (p.R176P) was classified as a pathogenic variant (PS2+PM1+PM2_Supporting+PM5+PP3+PP4), and the c.6817G>A (p.G2273R) was classified as a likely pathogenic variant (PS2+PM2_Supporting+PP3+PP4). The symptoms of all children were significantly improved with the propranolol treatment, and none has developed syncope during the follow up. CONCLUSION Discovery of the c.527G>C (p.R176P) and c.6817G>A (p.G2273R) variants has expanded the mutational spectrum of the RYR2 gene. Genetic testing of CPVT patients can clarify the cause of the disease and provide a reference for their genetic counseling.
Child ; Humans ; Mutation ; Propranolol ; Ryanodine Receptor Calcium Release Channel/genetics* ; Syncope ; Tachycardia, Ventricular/diagnosis* ; United States

Diagnosis, Differential ; Electrocardiography ; Humans ; Tachycardia, Ventricular ; Torsades de Pointes

To investigate the value of ventricular tachycardia (VT) score in diagnosing pre-excited tachycardia.
 Methods: Twelve-lead electrocardiograph results were obtained from 30 patients at pre-excited tachycardia attacking stage who were diagnosed by electrophysiology. We scored pre-excitation tachycardia based on the VT score. To analyze the electrocardiogram of pre-excited tachycardia using 7 diagnostic indicators of the VT score and calculate the specificity of 7 diagnostic indicators and right superior axis (-90º to ±180º), the differences were compared among VT score of 2 points and brugada, Wellens, and Vereckei algorithms in diagnosing pre-excited tachycardia. According to the specificity of Vereckei, Wellens, and Brugada algorithms, and VT scores from low to high, their prediction value and differences were analyzed.
 Results: Single indicator such as atrioventricular (AV) dissociation or right superior axis (-90º to ±180º) showed the highest specificity (100%) for identifying pre-excited tachycardia. No patient with VT score was ≥3 points, and the specificity was 100%. The specificity of VT score of 2 point was higher than that of Brugada, Wellens, or Vereckei algorithms in the diagnosing pre-excited tachycardia (76.7% vs 50.0%, 23.3% or 20.0%, P<0.05). The specificity of Vereckei, Wellens, and Brugada algorithms and VT score were gradually increased after each of stepwise individually eliminated VT (20.0%, 40.0%, 66.7%, 83.3%, P<0.05). However, there was no significant difference in the specificity in the remaining false positive cases between the 4 methods and VT score.
 Conclusion: VT score ≥3 points can identify pre-excited tachycardia and VT with 100% specificity. VT score of 2 points cannot completely distinguish pre-excited tachycardia from VT, but specificity of VT score with 2 points is obviously higher than that of Brugada, Wellens, and Vereckei algorithms.
Algorithms ; Diagnosis, Differential ; Electrocardiography ; Humans ; Sensitivity and Specificity ; Tachycardia, Ventricular ; diagnosis

Anticoagulants ; therapeutic use ; Cardiomyopathy, Hypertrophic ; complications ; diagnostic imaging ; therapy ; Coronary Angiography ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Echocardiography ; Electric Countershock ; Electrocardiography ; Heart Aneurysm ; complications ; diagnostic imaging ; therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tachycardia, Ventricular ; diagnosis ; etiology ; therapy

Anaphylaxis during anesthesia is rare, but often fatal. Rocuronium is a neuromuscular relaxant used for induction of anesthesia. We experienced a case of anaphylaxis after rocuronium administration during induction of anesthesia. A 64-year-old female patient was scheduled for bilateral radius fracture fixation. The history of patient showed no specific findings other than hypersensitivity to mackerel. She had no previous experience of anesthesia. Anesthesia was induced by intravenous injection of propofol 100 mg and continuous infusion was begun with remifentanil 0.25 µg/kg/min, followed by rocuronium 40 mg. Immediately after intravenous administration of rocuronium, the manual ventilation became difficult, and the patient developed erythema and severe hypotension. The patient was diagnosed with anaphylaxis based on clinical features and started treatment. First, we performed endotracheal intubation promptly. Then, immediate intravenous administration of epinephrine and fluid followed. Despite adequate treatment, hypotension was not corrected and intravenous epinephrine was administered. However, ventricular tachycardia occurred which was successfully treated with a defibrillator. Later, the patient uneventfully recovered in the intensive care unit. The patient was not tested for skin prick test, but rocuronium was the most likely cause of anaphylaxis at that time. The authors unexpectedly experienced drug-induced anaphylaxis, which is life-threatening to the patient. Clinicians should be aware of the diagnosis, treatment, and prevention as anaphylaxis can be hazardous to the patient.
Administration, Intravenous ; Anaphylaxis* ; Anesthesia* ; Defibrillators ; Diagnosis ; Epinephrine ; Erythema ; Female ; Humans ; Hypersensitivity ; Hypotension ; Injections, Intravenous ; Intensive Care Units ; Intubation, Intratracheal ; Middle Aged ; Perciformes ; Propofol ; Radius Fractures ; Skin ; Tachycardia, Ventricular ; Ventilation

Adolescent ; Calsequestrin ; genetics ; Child ; Child, Preschool ; China ; Diagnostic Errors ; Female ; Humans ; Male ; Tachycardia, Ventricular ; diagnosis

BACKGROUND AND OBJECTIVES: Multifocal atrial tachycardia (MAT), in general, has a favorable outcome. However, there are insufficient data regarding MAT in a pediatric population. This study sought to determine the clinical course of MAT and identify potential prognostic factors. METHODS: The medical records of MAT patients from 1997–2015 were reviewed. The arrhythmia control rate and factors for unfavorable outcomes were assessed and compared to those in the literature. RESULTS: Of the 33 included patients (19 boys and 14 girls), 27 were infants less than 1 year of age. The median age at diagnosis was 1.7 months (range, 0 day to 14 years). Fourteen (42%) patients had structural heart disease. Eight (24%) patients had lung disease and 6 (18%) had a syndromic diagnosis belonging to RASopathy. Two patients developed polymorphic ventricular tachycardia, in whom genetic analysis confirmed the presence of the RyR2 mutation several years later. MAT was controlled in 26 patients (84%) within 3.9 months (median; range, 16 days–18.4 years) using an average of 2.4 medications. There were 3 cases of cardiopulmonary mortality. The arrhythmia control rate was higher in the infant group (85%) than in the non-infant group (67%), although this trend was not statistically significant. There was a significantly lower rate of unfavorable outcomes in the idiopathic infant group (n=11) than in the other groups (p=0.008). Considering the findings of previous studies, the mortality rate was significantly higher in patients with structural heart disease than in patients without (21% vs. 5%, p=0.01). CONCLUSIONS: MAT usually affects infants and has a favorable prognosis, particularly in the idiopathic infant group. However, in the presence of other comorbidities, MAT may have a variable clinical course.
Arrhythmias, Cardiac ; Atrial Fibrillation ; Atrial Flutter ; Comorbidity ; Diagnosis ; Heart Diseases ; Humans ; Infant ; Lung Diseases ; Medical Records ; Mortality ; Prognosis ; Ryanodine Receptor Calcium Release Channel ; Tachycardia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular

INTRODUCTION: Atrial fibrillation (AF) with rapid ventricular rate (RVR) is a common diagnosis in the Emergency Department (ED) requiring evaluation and treatment. We present the characteristics and outcomes of patients presenting with primary or secondary AF in a tertiary hospital ED. MATERIALS AND METHODS: This retrospective cohort study included consecutive patients ≥21 years old, with a primary or secondary diagnosis of AF with RVR in the ED over a 1-year period from 1 January 2016 to 31 December 2016. Primary AF is defined as AF with no precipitating cause and secondary AF as AF secondary to a precipitating cause. RESULTS: A total of 464 patients presented to the ED from 1 January to 31 December 2016 with primary and secondary diagnosis of AF with RVR; 44.8% had primary diagnosis of AF whereas 55.2% had secondary AF. Overall admission rate from ED was high at 91.8% (primary 84.6% vs secondary 97.7%). Patients with primary AF were younger (68 vs 74 years, <0.001), had lower rates of cardiovascular risk factors, and shorter length of stay (median 4 vs 5 days). Within 30 days of discharge, they had lower ED reattendance (16.3% vs 25.8%, <0.001) and lower readmission (16.3% vs 25.8%, <0.001). There was no mortality in the primary AF group (0% vs 9.8%, <0.001). CONCLUSION Currently, majority of patients with AF with RVR are admitted from the ED. Other study suggests patients with uncomplicated primary AF have lower adverse outcomes and some could potentially be treated as outpatients.
Aged ; Atrial Fibrillation ; diagnosis ; epidemiology ; therapy ; Emergency Service, Hospital ; statistics & numerical data ; Female ; Humans ; Male ; Middle Aged ; Outcome and Process Assessment (Health Care) ; Patient Care Management ; methods ; statistics & numerical data ; Patient Readmission ; statistics & numerical data ; Retrospective Studies ; Risk Factors ; Singapore ; epidemiology ; Tachycardia, Ventricular ; diagnosis ; epidemiology ; therapy ; Tertiary Care Centers ; statistics & numerical data

Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias. Although most neonatal arrhythmias are asymptomatic and rarely life-threatening, the prognosis depends on the early recognition and proper management of the condition in some serious cases. Precise diagnosis with risk stratification of patients with nonbenign neonatal arrhythmia is needed to reduce morbidity and mortality. In this article, I review the current understanding of the common clinical presentation, etiology, natural history, and management of neonatal arrhythmias in the absence of an underlying congenital heart disease.
Arrhythmia, Sinus ; Arrhythmias, Cardiac* ; Atrial Premature Complexes ; Diagnosis* ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Mortality ; Natural History ; Prognosis ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Ventricular Premature Complexes

Ventricular extrasystole originating from the right ventricular outflow tract or the left ventricular outflow tract are the most commonly encountered ventricular arrhythmias recorded in ostensibly healthy individuals with no evidence of heart disease. These ventricular arrhythmias have a distinctive electrocardiographic morphology. The morphology is so distinctive that it is common practice to accept the diagnosis of “idiopathic benign ventricular arrhythmias from the outflow tract” based on this unique morphology when the electrocardiogram during sinus rhythm and the echocardiogram are normal, sometimes removing the need to perform invasive tests in patients. Even if the outflow ventricular extrasystole ultimately triggers sustained ventricular arrhythmia, the resulting ventricular tachycardia (VT) will be a monomorphic VT originating from the outflow tract, which is known to be hemodynamically well tolerated. Thus, idiopathic ventricular arrhythmias originating from outflow tracts are universally considered benign. In 2005, we described a rare form of malignant polymorphic VT resulting in syncope or cardiac arrest. Here, we review the literature on this topic since the emergence of initial descriptions of this intriguing phenomenon.
Arrhythmias, Cardiac ; Death, Sudden, Cardiac ; Diagnosis ; Electrocardiography ; Heart Arrest ; Heart Diseases ; Humans ; Syncope ; Tachycardia, Ventricular* ; Ventricular Premature Complexes