Inducibility of atrioventricular nodal reentrant tachycardia and ectopic atrial tachycardia in children under general anesthesia.

BACKGROUND: In children, invasive electrophysiological studies (EPS) and radiofrequency catheter ablations (RFA) of supraventricular tachycardia (SVT) are often performed under general anesthesia. Atrioventricular nodal reentrant tachycardia (AVNRT) and ectopic atrial tachycardia (EAT) must be inducible during EPS as reliable diagnosis and subsequent therapy are not possible in sinus rhythm. This study aims to assess the problem of noninducible AVNRT and EAT under general anesthesia. METHODS AND RESULTS: Anesthesia protocols of 166 patients undergoing EPS were retrospectively analyzed. 122 AVNRT patients were compared to 22 whose tachycardia was not inducible but probably due to an AVNRT mechanism. Another 16 patients with inducible EAT were compared to 6 whose EAT appeared on surface ECG but not during EPS. Demographic characteristics were similar among all groups. Inducibility did not differ (p = .42) between AVNRT patients with inhalational anesthesia (sevoflurane and/or nitrous oxide) and patients with intravenous anesthesia (propofol with/without remifentanil). The EAT group exhibited lower inducibility under intravenous anesthesia (64%) than under inhalational (88%), however without significance (p = .35). CONCLUSION: Tachycardia induction succeeds with similar frequency under both inhalational and intravenous general anesthesia in children with AVNRT. In children with EAT, inhalational anesthesia is associated with a trend towards better inducibility.

Severe congenital RYR1-associated myopathy complicated with atrial tachycardia and sinus node dysfunction: a case report.

BACKGROUND: Cardiac arrhythmias are sometimes encountered in patients with hereditary myopathies and muscular dystrophies. Description of arrhythmias in myopathies and muscular dystrophies is very important, because arrhythmias have a strong impact on the outcomes for these patients and are potentially treatable. CASE PRESENTATION: A girl with severe congenital RYR1-related myopathy exhibited atrial tachycardia and sinus node dysfunction during infancy. She was born after uncomplicated caesarian delivery. She showed no breathing, complete ophthalmoplegia, complete bulbar paralysis, complete facial muscle paralysis, and extreme floppiness. At 5 months old, she developed persistent tachycardia around 200-210 beats per minutes. Holter monitoring revealed ectopic atrial tachycardia during tachyarrhythmia and occasional sinus pauses with junctional escape beats. Propranolol effectively alleviated tachyarrhythmia but was discontinued due to increased frequency and duration of the sinus pauses that led to bradyarrhythmia. There was no evidence of structural heart diseases or heart failure. The arrhythmia gradually resolved spontaneously and at 11 months old, she showed complete sinus rhythm. CONCLUSIONS: Although supraventricular arrhythmia is sometimes encountered in congenital myopathies, this is the first report of cardiac arrhythmia requiring drug intervention in RYR1-associated myopathy.

A challenging ECG: a case of a lengthening PR interval.

A 62-year-old man presented to the emergency department with shortness of breath and chest tightness. His initial ECG appeared to have an ectopic P-wave with a lengthening PR interval consistent with second degree AV block - Mobitz Type I. But closer inspection showed a faster, independent atrial rate. The patient was diagnosed with ectopic atrial bradycardia with complete AV block and junctional escape, and was ultimately referred for pacemaker.

Risk of Stroke in Patients With Short-Run Atrial Tachyarrhythmia.

BACKGROUND AND PURPOSE: The risk of stroke in patients with short-run atrial tachyarrhythmia (AT) remains unclear. This study aimed to investigate the relationship between short-run AT and the stroke and the use of the CHA2DS2-VASc score for the risk stratification. METHODS: From the registry of 24-hour Holter monitoring, 5342 subjects without known atrial fibrillation or stroke were enrolled. Short-run AT was defined as episodes of supraventricular ectopic beats <5 seconds. RESULTS: There were 1595 subjects (29.8%) with short-run AT. During the median follow-up period of 9.0 years, 494 subjects developed new-onset stroke. Patients with short-run AT had significantly higher stroke rates compared with patients without short-run AT (11.4% versus 8.3%; P<0.001). In patients with short-run AT, the number of strokes per 100 person-years for patients with CHA2DS2-VASc score of 0 and 1 were 0.23 and 0.67, respectively. However, the number of them for patients with CHA2DS2-VASc score of 2, 3, 4, and ≥5 were 1.62, 1.89, 1.30, and 2.91, respectively. In patients with CHA2DS2-VASc score of 0 or 1, age (>61 years old) and burden of premature atrial contractions (>25 beats/d) independently predicted the risk of stroke. In subgroup analyses, short-run AT patients were divided into 3 groups based on their CHA2DS2-VASc scores: low score (score of 0 [men] or 1 [women]; n=324), intermediate score (score of 1 [men] or 2 [women]; n=275), and high score (score of ≥2 [men] or ≥3 [women]; n=996). When compared with low score, intermediate and high scores were independent predictors for stroke (hazard ratio, 6.165; P<0.001 and hazard ratio, 8.577; P<0.001, respectively). CONCLUSIONS: Short-run AT increases the risk of stroke. Therefore, the CHA2DS2-VASc score could be used for the risk stratification. Age and burden of premature atrial contractions were independent predictors for stroke in patients with CHA2DS2-VASc score of 0 or 1.

Successful use of sirolimus for refractory atrial ectopic tachycardia in a child with cardiac rhabdomyoma.

Cardiac rhabdomyomas are common in tuberous sclerosis. We report a child who developed rhabdomyoma related arrhythmia refractory to antiarrhythmic drug therapy. Reversion of the atrial ectopic tachycardia was achieved with mammalian target of rapamycin pathway (mTOR) inhibitor sirolimus. As per our knowledge, this is the first time that sirolimus has been successfully used in this setting.

Predictors of Pharmacological Therapy of Ectopic Atrial Tachycardia in Children.

Ectopic atrial tachycardia (EAT) is a relatively common type of supraventricular tachycardia in the pediatric population, and it can be resistant to antiarrhythmic drugs and lead to tachycardia-induced cardiomyopathy (TIC) if not properly managed. The purpose of this study was to determine the predictors of the response to pharmacological therapy in children with EAT. From January 2009 to April 2014, 115 children were admitted to our hospital with a diagnosis of EAT and placed on antiarrhythmic drugs. We examined the clinical history, response to therapy, and follow-up of the children. The incidence of TIC secondary to EAT was 22.6% (n = 26) in children. Incessant EAT accounted for 44.3% of all patients. Control of EAT with antiarrhythmic therapy was achieved in 73.9% (n = 85) of the children. The combination of sotalol and propafenone performed well in controlling EAT in children [complete control in 35 (49.3%) of 71]. The mean time of conversion to sinus rhythm was 24 days, and the mean duration of therapy was 11 months in children with resolution. Multivariate predictors of the control of EAT were age at presentation (OR 0.289, P = 0.038) and tachycardia type (OR 0.276, P = 0.006). TIC occurs in 22.6% of children with EAT. Incessant EAT is more frequently complicated by TIC. Independent factors associated with a good response to pharmacological therapy include a younger age at presentation and non-incessant tachycardia in children with EAT.

Prevalence and distribution of focal triggers in persistent and long-standing persistent atrial fibrillation.

BACKGROUND: The relevance of focal triggers in persistent atrial fibrillation (PerAF) and long-standing persistent atrial fibrillation (LSPAF) has not been previously investigated. OBJECTIVE: We prospectively evaluated the prevalence and distribution of AF triggers in patients referred for catheter ablation of PerAF and LSPAF. METHODS: We analyzed consecutive patients undergoing first time AF ablation who underwent a standardized trigger protocol including cardioversion of induced or spontaneous AF and infusion of up to 20 µg of isoproterenol for 15-20 minutes either before or after pulmonary vein (PV) isolation accomplished. Triggers were defined as AF/sustained atrial tachyarrhythmia or repetitive atrial premature depolarizations. RESULTS: A total of 2168 patients were included (mean age 57 ± 11 years; 1636 [75%] men), with 1531 patients having paroxysmal AF (PAF) (71%), 496 having PerAF (23%), and 141 having LSPAF (7%). PV triggers were found in 1398 patients with PAF (91%), 449 patients with PerAF (91%), and 129 patients with LSPAF (91%) (P = .856 for comparison across groups). Non-PV triggers were elicited in a total of 234 patients (11%), and the prevalence was similar across the different types of AF (PAF, 165 [11%]; PerAF, 54 [11%]; LSPAF, 15 [11%]; P = .996 for comparison across groups). CONCLUSION: PVs are the main AF trigger site in patients with PerAF and LSPAF, with an overall prevalence similar to that found in patients with PAF. These results support the current recommendations for PV isolation as the cornerstone of catheter ablation to eliminate AF triggers in PerAF and LSPAF.

Utility of Routine Exercise Testing to Detect Rate-Related QRS Widening in Patients Without Structural Heart Disease on Class Ic Antiarrhythmic Agents (Flecainide and Propafenone).

Class Ic antiarrhythmic agents are effective in the treatment of various atrial tachyarrhythmias. They are known to cause rate-related QRS widening in the presence of structural heart disease, which can lead to life-threatening arrhythmias. The role of routine exercise electrocardiography in patients without structural heart disease is unknown. All patients initiated on class Ic antiarrhythmic agents and who had exercise electrocardiography performed from June 2009 to June 2013 were included. Symptom-limited treadmill electrocardiography was performed to detect significant QRS widening at peak exercise (defined as an increase of >25% of baseline QRS). Fifty-six patients were included in the study. All patients were screened for structural heart disease before initiation of the medication. Significant QRS widening and atrial tachycardia occurred in a single patient, which terminated with cessation of exercise. This patient had a history of tachycardia-mediated cardiomyopathy with normalization of ejection fraction 3 years before being placed on flecainide. In conclusion, routine exercise testing to detect QRS widening is not warranted in patients with no structural heart disease.

Optimal noninvasive assessment of initial left ventricular dysfunction in children with ectopic atrial tachycardia.

UNLABELLED: Tissue Doppler imaging (TDI) can identify cardiac dysfunction in adults. This study is aimed to improve early identification of initial left ventricular (LV) dysfunction secondary to ectopic atrial tachycardia (EAT) in children by TDI. A total of 70 children with EAT were included in the present study. Cardiac function was evaluated by conventional echocardiography, TDI, and plasma N-terminal pro-brain natriuretic peptide (NT-proBNP). Doppler signals obtained from the mitral inflow and TDI of the mitral annulus were the average values of three consecutive heartbeats. Left ventricular ejection fraction (LVEF), peak early diastolic transmitral velocity (E), peak systolic mitral annulus velocity (S'), early diastolic mitral annular velocity (E'), the ratio E/E', and TDI-derived myocardial performance index (TDI-MPI) were compared between two groups of children with normal or elevated plasma NT-proBNP concentrations. Of the children, 18.6% demonstrated tachycardia-induced cardiomyopathy (TIC). Compared with LVEF, the TDI-MPI and E/E' showed better correlations with elevated plasma NT-proBNP. Addition of TDI-MPI and E/E' to LVEF provided increased information to detect elevated plasma NT-proBNP (91.67% sensitivity). CONCLUSIONS: TIC occurred in 18.6% of children with EAT. Initial LV dysfunction assessed by the TDI-MPI and E/E' is associated with elevated plasma NT-proBNP, even the LVEF is normal.

The presence of extensive atrial scars hinders the differential diagnosis of focal or macroreentrant atrial tachycardias in patients with complex congenital heart disease.

AIMS: Atrial tachycardias (ATs) frequently develop in patients with congenital heart defects (CHDs). This study aimed to evaluate the effects of extensive atrial scar formation on the total atrial activation time (TAAT) and its relation to the tachycardia cycle length (CL) to classify AT. METHODS AND RESULTS: Seventy-one patients were included and divided into two groups: patients without CHD (Group I, 35 patients) and with CHD (Group II, 36 patients). All patients underwent CARTO electroanatomical activation mapping. Two subgroups were created: centrifugal (CAT) or macroreentrant AT (MRAT). Total atrial activation time, CL, and mean bipolar signal amplitude (BiSA) were analysed. In Group I, 18 patients (51.4%) had CAT and 17 (48.6%) MRAT. The mean BiSA for Group I was 1.30 ± 0.32 mV. Total atrial activation time/CL ratios were different between CAT and MRAT (28.4 ± 16.9 vs. 66.6 ± 14.3%, P < 0.001). In Group II, 18 patients (50%) had CAT and 18 patients (50%) MRAT. The mean BiSA was 0.94 ± 0.50 mV and was not different for CAT and MRAT subgroups (1.04 ± 0.64 vs. 0.85 ± 0.29, P = 0.243). Total atrial activation time/CL ratios were comparable between CAT and MRAT patients (69.0 ± 40.4 vs. 83.6 ± 8.3%, P = 0.243). A significant lower BiSA was found for CAT with TAAT/CL ratios above 40% (0.62 ± 0.11 vs. 1.90 ± 0.18 mV, P < 0.001). A strong negative correlation was identified between the BiSA and the TAAT/CL ratio in patients with CAT in Group II (-0.742; P < 0.001). CONCLUSION: Low mean BiSA values in CHD patients are associated with altered impulse propagation, making TAAT- and CL-based diagnostic tools inaccurate. Further diagnostic tests are needed to determine the correct mechanism of ATs.