BackgroundSudden cardiac death (SCD) is a major global health problem, accounting for up to 20% of deaths in Western societies. Clinical quality registries have been shown in a range of disease ...conditions to improve clinical management, reduce variation in care and improve outcomes.AimTo identify existing cardiac arrest (CA) and SCD registries, characterising global coverage and methods of data capture and validation.MethodsBiomedical and public search engines were searched with the terms ‘registry cardio*’; ‘sudden cardiac death registry’ and ‘cardiac arrest registry’. Registries were categorised as either CA, SCD registries or ‘other’ according to prespecified criteria. SCD registry coordinators were contacted for contemporaneous data regarding registry details.ResultsOur search strategy identified 49 CA registries, 15 SCD registries and 9 other registries (ie, epistries). Population coverage of contemporary CA and SCD registries is highly variable with registries densely concentrated in North America and Western Europe. Existing SCD registries (n=15) cover a variety of age ranges and subpopulations, with some enrolling surviving patients (n=8) and family members (n=5). Genetic data are collected by nine registries, with the majority of these (n=7) offering indefinite storage in a biorepository.ConclusionsMany CA registries exist globally, although with inequitable population coverage. Comprehensive multisource surveillance SCD registries are fewer in number and more challenging to design and maintain. Challenges identified include maximising case identification and case verification.Trial registration numberCRD42019118910.
Background Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia syndrome, characterised by polymorphic ventricular tachycardia induced by adrenergic stress. CPVT ...can be caused by mutations the cardiac ryanodine receptor gene ( RYR2 ) or mutations in the cardiac calsequestrin gene CASQ2 . Structural heart disease is usually absent and the baseline ECG is usually normal. Patients with CPVT often present with exercise- or emotion induced syncope, the first presentation can also be sudden cardiac death. Management Besides removal of triggers treatment with beta blockers is currently a class I indication in clinically diagnosed patients. Beta blockage should be titrated up to an effective level. The addition of flecainide seems to be a promising approach in patients where arrhythmias are not completely suppressed by beta blockers. A cardioverter-defibrillator (ICD) or left cervical sympathetic denervation might be considered under special circumstances. Genetic counselling is recommended and all first degree relatives should be properly evaluated.
Objective
Pacing in a univentricular circulation has been associated with worsened outcomes. We investigated the long‐term outcomes of pacing in children with a univentricular circulation compared to ...a complex biventricular circulation. We also identified predictors of adverse outcomes.
Methods
A retrospective study of all children with major congenital heart disease who underwent pacemaker implantation under the age of 18 years between November 1994 and October 2017.
Results
Eighty‐nine patients were included; 19 with a univentricular and 70 with a complex biventricular circulation. A total of 96% of pacemaker systems were epicardial. Median follow up was 8.3 years. The incidence of adverse outcome was similar between the two groups. Five (5.6%) patients died and two (2.2%) underwent heart transplantation. Most adverse events occurred within the first 8 years after pacemaker implantation. Univariate analysis identified five predictors of adverse outcomes in the patients in the biventricular but none in the univentricular group. The predictors of adverse outcome in the biventricular circulation were a right morphologic ventricle as the systemic ventricle, age at first congenital heart disease (CHD) operation, number of CHD operations, and female gender. The nonapical lead position was associated with a much higher risk of an adverse outcome.
Conclusions
Children with a pacemaker and a complex biventricular circulation have similar survival to the ones with a pacemaker and a univentricular circulation. The only modifiable predictor was the epicardial lead position on the paced ventricle, emphasizing the importance of apical placement of the ventricular lead.
Background
Supraventricular tachycardia is a group of rhythm disturbances that affect 1 in 300‐1200 Australian children annually. The differentiation of supraventricular tachycardia (SVT) symptoms ...and age of onset according to their subtype is not well understood in the pediatric population. Most studies rely on ECG criteria only to characterize the subtype of the SVT, which is not applicable to all subtypes. The purpose of this study was to identify the symptoms and ages of onset of SVT subtypes, and to analyze whether ethnicity or severity correlated with the SVT subtype confirmed in an invasive Electrophysiology (EP) study.
Methods
A retrospective analysis and prospective survey evaluated 364 patients who underwent an EP study at The Royal Children's Hospital, Melbourne between 2009 and 2015. Age of onset, symptoms, and ethnicity were collected by phone survey or medical records in addition to EP study diagnostic data, medication status, and follow‐up information about their symptom status following EP procedure. Patients were grouped according to their SVT subtype. Data analysis was performed using chi‐squared, Fisher's exact, and ANOVA statistical tests to determine associations between SVT substrates.
Results
Two hundred and thirty‐three suitable cases of SVT were identified (131 men, 102 women) aged between 0 and 18 years. Atrioventricular Reentrant Tachycardia (AVRT) (n = 153) was the most common SVT subtype, followed by Atrioventricular Nodal Reentrant Tachycardia (AVNRT) (n = 55), Atrial Tachycardia (AT) (n = 17), and other SVT subtypes (n = 8) which included Atrial Fibrillation, Atrial Flutter, and Junctional Tachycardia. There was a male predominance in all subtypes, except for AVNRT. AVNRT patients had palpitations, dyspnoea, dizziness, and anxiety more than any other group, AVRT patients complained of vomiting most and patients with AT had the most fatigue. The mean age of symptom onset varied among groups, being earlier in AVRT, later in AVNRT with a significant difference between AVRT with unidirectional retrograde accessory pathway (URAP) and AVNRT subtypes (P < 0.01).
Conclusion
Some specific symptoms were strong discriminators between different SVT subtypes. Ethnicity did not have strong correlations with SVT subtype incidence. This study was able to show clinical differences among children with SVT due to AVRT (URAP) compared to AVNRT, allowing the prognosis and intended management of pediatric SVT to be anticipated by less invasive means.
Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct translocation of the left coronary artery could be ...difficult due to its intramural course and proximity to the aortic valve. We described the surgical management of a child after sudden hemodynamic collapse and the coronary translocation technique that prevented distortion of the aortic root and allowed successful translocation of the abnormal coronary artery despite its intramural course immediately behind the aortic valve commissure.
Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern.
The purpose of ...this study was to determine the time course and predictors of myocardial recovery in pediatric TIC.
An international multicenter study of pediatric TIC was conducted. Children ≤18 years with incessant tachyarrhythmia, cardiac dysfunction (left ventricular ejection fraction LVEF <50%), and left ventricular (LV) dilation (left ventricular end-diastolic dimension LVEDD z-score ≥2) were included. Children with congenital heart disease or suspected primary cardiomyopathy were excluded. Primary end-points were time to LV systolic functional recovery (LVEF ≥55%) and normal LV size (LVEDD z-score <2).
Eighty-one children from 17 centers met inclusion criteria: median age 4.0 years (range 0.0-17.5 years) and baseline LVEF 28% (interquartile range 19-39). The most common arrhythmias were ectopic atrial tachycardia (59%), permanent junctional reciprocating tachycardia (23%), and ventricular tachycardia (7%). Thirteen required extracorporeal membrane oxygenation (n = 11) or ventricular assist device (n = 2) support. Median time to recovery was 51 days for LVEF and 71 days for LVEDD. Two (4%) underwent heart transplantation, and 1 died (1%). Multivariate predictors of LV systolic functional recovery were age (hazard ratio HR 0.61, P = .040), standardized tachycardia rate (HR 1.16, P = .015), mechanical circulatory support (HR 2.61, P = .044), and LVEF (HR 1.33 per 10% increase, p=0.005). For normalization of LV size, only baseline LVEDD (HR 0.86, P = .008) was predictive.
Pediatric TIC resolves in a predictable fashion. Factors associated with faster recovery include younger age, higher presenting heart rate, use of mechanical circulatory support, and higher LVEF, whereas only smaller baseline LV size predicts reverse remodeling. This knowledge may be useful for clinical evaluation and follow-up of affected children.
Background To review the long-term outcomes of patients born with single-ventricle physiology and aortic arch obstruction. Methods Follow-up of 70 consecutive neonates undergoing single-ventricle ...palliation and arch repair, excluding hypoplastic left heart syndrome, between 1983 and 2008, was reviewed. Dominant arch anomalies were coarctation (n = 48), interrupted arch (n = 10), and hypoplastic arch alone (n = 12). Neonatal Damus procedure with arch repair and shunt became the dominant approach, being performed in 1 (10%) of 10 in 1983 to 1989, 9 (32%) of 28 in 1990 to 1999, and 23 (72%) of 32 in 2000 to 2008. Results All patients underwent an initial procedure at a median of 6 days (range, 4-12 days): pulmonary artery banding and arch repair (n = 35); Damus, arch repair, and shunt (n = 33); and other (n = 2). Twenty-six patients died before Fontan completion. Of the 34 survivors of initial banding, 17 (50%) later required a Damus and 4 (12%) required subaortic stenosis relief. Forty patients underwent Fontan completion at a median age of 5 years (range, 4-7 years). After a mean of 5 ± 6 years after Fontan, there was 1 hospital death and 1 Fontan takedown. Overall survival was similar if patients initially underwent a Damus or pulmonary artery banding ( P = .3). Overall survival at 10 years was 53% (95% confidence interval, 42%-67%). Conclusions Patients born with single-ventricle physiology and arch obstruction have a high risk of mortality in the first years of life. Their outcomes seem excellent once they reach Fontan status. It is likely that, in patients with single-ventricle and arch obstruction, strategies to avoid systemic outflow tract obstruction should be implemented in early life, and regular monitoring of blood pressure is warranted.
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