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1.
Heart Rhythm ; 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32497761

RESUMO

BACKGROUND: Abrupt loss of ventricular pre-excitation on non-invasive evaluation, or non-persistent pre-excitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events. OBJECTIVE: To compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted pre-excited atrial fibrillation (RC-AF) in patients with non-persistent and persistent pre-excitation. METHODS: Patients ≤21 years with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Non-persistent pre-excitation was defined as absence/sudden loss of pre-excitation on ECG, Holter, or exercise test. RC-AF was defined as clinical pre-excited atrial fibrillation with shortest pre-excited R-R interval (SPERRI) ≤250ms. AP effective refractory period (APERP), SPERRI at EPS (EPS-SPERRI), and shortest pre-excited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤250ms. RESULTS: Of 1589 patients, 244 (15%) had non-persistent pre-excitation and 1345 (85%) had persistent pre-excitation. There were no differences in sex (58 vs 60% male, p=0.49) or age (13.3±3.6 vs 13.1±3.9 years, p=0.43) between groups. Though APERP (344±76 vs 312±61ms, p<0.001), and SPPCL (394±123 vs 317±82ms, p<0.001) were longer in non-persistent versus persistent pre-excitation, there was no difference in EPS-SPERRI (331±71 vs 316±73ms, p=0.15). Non-persistent pre-excitation was associated with fewer high-risk APs (13 vs 23%, p<0.001) than persistent pre-excitation. Of 61 patients with SCA or RC-AF, 6 (10%) had non-persistent pre-excitation (3 SCA, 3 RC-AF). CONCLUSION: Non-persistent pre-excitation was associated with fewer high-risk APs, though it did not exclude risk of SCA or RC-AF in children with WPW.

2.
Heart Lung Circ ; 2020 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-32418874

RESUMO

In the context of the current global COVID-19 pandemic, this Consensus Statement provides current recommendations for patients with, or at risk of developing, genetic heart disease, and for their health care management and service provision in Australia and New Zealand. Apart from general recommendations, there are specific recommendations for the following conditions: cardiomyopathy, Brugada syndrome (including in children), long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). Other recommendations are relevant to patient self-care and primary health care.

3.
J Am Coll Cardiol ; 75(21): 2698-2707, 2020 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-32466885

RESUMO

BACKGROUND: The relative proportion of each cardiac inherited disease (CID) causing resuscitated sudden cardiac arrest (RSCA) on a population basis is unknown. OBJECTIVES: This study describes the profile of patients with CIDs presenting with RSCA; their data were collected by the national Cardiac Inherited Diseases Registry New Zealand (CIDRNZ). METHODS: Data were collated from CIDRNZ probands presenting with RSCA (2002 to 2018). RESULTS: CID was identified in 115 (51%) of 225 RSCA cases: long QT syndrome (LQTS) (n = 48 [42%]), hypertrophic cardiomyopathy (HCM) (n = 28 [24%]), Brugada syndrome (BrS) (n = 16 [14%]), catecholaminergic polymorphic ventricular tachycardia (CPVT) (n = 9 [8%]), arrhythmogenic right ventricular cardiomyopathy (ARVC) (n = 9 [8%]), and dilated cardiomyopathy (n = 5 [4%]). Seventy-one (62%) of 115 were male. Of 725 probands from the CIDRNZ with CID, the proportion presenting with RSCA was: CPVT, 9 (53%) of 17; BrS, 16 (33%) of 49; ARVC, 9 (25%) of 36; LQTS, 48 (20%) of 238; dilated cardiomyopathy, 5 (9%) of 58; and HCM, 28 (8%) of 354. Incident activity was: normal everyday activities, 44 (40%); exercising, 33 (30%); concurrent illness, 13 (12%); sleeping, 10 (9%); drugs/medication, 9 (8%); and emotion, 2 (2%). LQTS and CPVT predominated in those <24 years of age, 30 (77%) of 39; cardiomyopathies and BrS predominated in those >24 years of age, 49 (64%) of 76. For those >40 years of age, HCM was the most common (33%) CID. A genetic diagnosis in patients with CID was made in 48 (49%) of 98 tested. Diagnosis by age range was as follows: age 1 to 14 years, 78%; age 15 to 24 years, 53%; age 25 to 39 years, 54%; and age >40 years, 26%. CONCLUSIONS: The commonest CID identified after RSCA was LQTS; the most common CID cause of RSCA for those >40 years of age was HCM. CPVT was the CID most likely to present with RSCA and HCM the least. Genetic yield decreases with age. Only one-third of RSCA cases due to CID occurred while exercising.

4.
Heart Lung Circ ; 29(6): e57-e68, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32451232

RESUMO

The COVID-19 pandemic poses a significant stress on health resources in Australia. The Heart Rhythm Council of the Cardiac Society of Australia and New Zealand aims to provide a framework for efficient resource utilisation balanced with competing risks when appropriately treating patients with cardiac arrhythmias. This document provides practical recommendations for the electrophysiology (EP) and cardiac implantable electronic devices (CIED) services in Australia. The document will be updated regularly as new evidence and knowledge is gained with time.

5.
Heart Rhythm ; 2020 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-32229296

RESUMO

BACKGROUND: New Zealand has a multiethnic population and a national cardiac inherited disease registry (Cardiac Inherited Disease Registry New Zealand [CIDRNZ]). Ancestry is reflected in the spectrum and prevalence of genetic variants in long QT syndrome (LQTS). OBJECTIVE: The purpose of this study was to study the genetic testing yield and mutation spectrum of CIDRNZ LQTS probands stratified by self-identified ethnicity. METHODS: A 15-year retrospective review of clinical CIDRNZ LQTS probands with a Schwartz score of ≥2 who had undergone genetic testing was performed. RESULTS: Of the 264 included LQTS probands, 160 (61%) reported as European, 79 (30%) NZ Maori and Pacific peoples (Polynesian), and 25 (9%) Other ethnicities, with comparable clinical characteristics across ethnic groups (cardiac events in 72%; age at presentation 28±19 years; corrected QT interval 512±55 ms). Despite comparable testing (5.3±1.4 LQTS genes), a class III-V LQTS variant was identified in 35% of Polynesian probands as compared with 63% of European and 72% of Other probands (P<.0001). Among variant-positive CIDRNZ LQTS probands (n=148), Polynesians were more likely to have non-missense variants (57% vs 39% and 25% in probands of European and Other ethnicity, respectively; P=.005) as well as LQT1-3 variants not reported elsewhere (71% vs European 22% and Other 28%; P<.0001). Variants found in multiple probands were more likely to be shared within the same ethnic group; P<.01). CONCLUSION: Genetic testing of Polynesian LQTS probands has a lower diagnostic yield, despite comparable testing and clinical disease severity. Rare LQTS variants are more common in Polynesian LQTS probands. These data emphasize the importance of increasing the knowledge of genetic variation in the Polynesian population.

7.
Heart Lung Circ ; 29(4): 512-519, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32044265

RESUMO

The genetics underlying familial long QT syndrome (LQTS) are among the best characterised of all of the inherited heart conditions. Cohort and registry studies have demonstrated important genotype-phenotype correlations that are now essential in guiding clinical practice of patients with the most common three genotypes; KCNQ1 (LQT type 1), KCNH2 (LQT type 2) and SCN5A (LQT type 3). However, the growing number of genes-now more than 16-is confusing, and there is much doubt as to whether many actually cause LQTS at all. Furthermore, changes in sequencing techniques, evolving variant classification criteria and new scientific discoveries make all genes and variants subject to a continuous process of re-classification. This review discusses the nature of variant adjudication, the important concept of pre-test probability in interpreting a genetic result and how the nomenclature of LQTS is shifting in response to this new knowledge. It further discusses the role of deep phenotyping, the inclusion of evaluation of family members in interpreting a genetic test result, or even deciding if genetic testing should occur at all, and the role of specialist multidisciplinary teams to translate this continuously evolving knowledge into the best clinical advice, in partnership with referring cardiologists.

8.
Circ Arrhythm Electrophysiol ; 13(3): e007471, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32063070

RESUMO

BACKGROUND: Risk stratification in catecholaminergic polymorphic ventricular tachycardia remains ill defined. Heart rate recovery (HRR) immediately after exercise is regulated by autonomic reflexes, particularly vagal tone, and may be associated with symptoms and ventricular arrhythmias in patients with catecholaminergic polymorphic ventricular tachycardia. Our objective was to evaluate whether HRR after maximal exercise on the exercise stress test (EST) is associated with symptoms and ventricular arrhythmias. METHODS: In this retrospective observational study, we included patients ≤65 years of age with an EST without antiarrhythmic drugs who attained at least 80% of their age- and sex-predicted maximal HR. HRR in the recovery phase was calculated as the difference in heart rate (HR) at maximal exercise and at 1 minute in the recovery phase (ΔHRR1'). RESULTS: We included 187 patients (median age, 36 years; 68 [36%] symptomatic before diagnosis). Pre-EST HR and maximal HR were equal among symptomatic and asymptomatic patients. Patients who were symptomatic before diagnosis had a greater ΔHRR1' after maximal exercise (43 [interquartile range, 25-58] versus 25 [interquartile range, 19-34] beats/min; P<0.001). Corrected for age, sex, and relatedness, patients in the upper tertile for ΔHRR1' had an odds ratio of 3.4 (95% CI, 1.6-7.4) of being symptomatic before diagnosis (P<0.001). In addition, ΔHRR1' was higher in patients with complex ventricular arrhythmias at EST off antiarrhythmic drugs (33 [interquartile range, 22-48] versus 27 [interquartile range, 20-36] beats/min; P=0.01). After diagnosis, patients with a ΔHRR1' in the upper tertile of its distribution had significantly more arrhythmic events as compared with patients in the other tertiles (P=0.045). CONCLUSIONS: Catecholaminergic polymorphic ventricular tachycardia patients with a larger HRR following exercise are more likely to be symptomatic and have complex ventricular arrhythmias during the first EST off antiarrhythmic drug.

9.
Heart Lung Circ ; 29(4): 641-652, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31974024

RESUMO

At least one-third of adults living with an inherited cardiac condition report clinically-significant levels of psychological distress. Poorer health-related quality of life compared with population norms is also consistently reported. These outcomes are associated with younger patient age, having an implantable cardioverter defibrillator, and receipt of uncertain clinical test results, and can influence self-management behaviours, such as adherence to potentially critical life-preserving medications. According to the Common Sense Model of Illness, people use information from multiple sources to 'make sense' of their health condition, and how they conceptualise the condition can strongly influence adaptation and coping responses. Previous studies with people with inherited cardiac conditions show that illness perceptions, such as greater perceived consequences and a poorer understanding of the condition, are associated with greater psychological distress and poorer adherence to medication. The Common Sense Model provides one potential framework for identifying patients who may be more vulnerable to adverse health outcomes, and for developing early interventions to reduce the physical and psychosocial burden of these conditions. Interventions based on the Common Sense Model have successfully improved physical and psychosocial outcomes associated with other cardiac conditions, and could be tailored for use with patients with an inherited cardiac condition (ICC).

10.
Circulation ; 141(6): 429-439, 2020 Feb 11.
Artigo em Inglês | MEDLINE | ID: mdl-31941373

RESUMO

BACKGROUND: Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare KCNE1 variants implicated in LQT5 was sought through an international multicenter collaboration. METHODS: Patients with either presumed autosomal dominant LQT5 (N = 229) or the recessive Type 2 Jervell and Lange-Nielsen syndrome (N = 19) were enrolled from 22 genetic arrhythmia clinics and 4 registries from 9 countries. KCNE1 variants were evaluated for ECG penetrance (defined as QTc >460 ms on presenting ECG) and genotype-phenotype segregation. Multivariable Cox regression was used to compare the associations between clinical and genetic variables with a composite primary outcome of definite arrhythmic events, including appropriate implantable cardioverter-defibrillator shocks, aborted cardiac arrest, and sudden cardiac death. RESULTS: A total of 32 distinct KCNE1 rare variants were identified in 89 probands and 140 genotype positive family members with presumed LQT5 and an additional 19 Type 2 Jervell and Lange-Nielsen syndrome patients. Among presumed LQT5 patients, the mean QTc on presenting ECG was significantly longer in probands (476.9±38.6 ms) compared with genotype positive family members (441.8±30.9 ms, P<0.001). ECG penetrance for heterozygous genotype positive family members was 20.7% (29/140). A definite arrhythmic event was experienced in 16.9% (15/89) of heterozygous probands in comparison with 1.4% (2/140) of family members (adjusted hazard ratio [HR] 11.6 [95% CI, 2.6-52.2]; P=0.001). Event incidence did not differ significantly for Type 2 Jervell and Lange-Nielsen syndrome patients relative to the overall heterozygous cohort (10.5% [2/19]; HR 1.7 [95% CI, 0.3-10.8], P=0.590). The cumulative prevalence of the 32 KCNE1 variants in the Genome Aggregation Database, which is a human database of exome and genome sequencing data from now over 140 000 individuals, was 238-fold greater than the anticipated prevalence of all LQT5 combined (0.238% vs 0.001%). CONCLUSIONS: The present study suggests that putative/confirmed loss-of-function KCNE1 variants predispose to QT prolongation, however, the low ECG penetrance observed suggests they do not manifest clinically in the majority of individuals, aligning with the mild phenotype observed for Type 2 Jervell and Lange-Nielsen syndrome patients.

11.
Heart Lung Circ ; 29(1): 5-39, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31735685

RESUMO

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

12.
Eur J Hum Genet ; 28(1): 17-22, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31534214

RESUMO

Sudden cardiac death (SCD) is often associated with structural abnormalities of the heart during autopsy. This study sought to compare the diagnostic yield of postmortem genetic testing in (1) cases with structural findings of uncertain significance at autopsy to (2) cases with autopsy findings diagnostic of cardiomyopathy. We evaluated 57 SCD cases with structural findings at cardiac autopsy. Next-generation sequencing using a panel of 77 primary electrical disorder and cardiomyopathy genes was performed. Pathogenic and likely pathogenic variants were classified using American College of Medical Genetics (ACMG) consensus guidelines. In 29 cases (51%) autopsy findings of uncertain significance were identified whereas in 28 cases (49%) a diagnosis of cardiomyopathy was established. We identified a pathogenic or likely pathogenic variant in 10 cases (18%); in 1 (3%) case with non-specific autopsy findings compared with 9 (32%) cases with autopsy findings diagnostic of cardiomyopathy (p = 0.0054). The yield of genetic testing in SCD cases with autopsy findings consistent with cardiomyopathy is comparable with the yield in cardiomyopathy patients that are alive. Genetic testing in cases with findings of uncertain significance offers lower clinical utility than in cardiomyopathy, with lower yields than detected previously. This highlights the need for stringent evaluation of variant pathogenicity.

14.
Heart Lung Circ ; 2019 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-31501048

RESUMO

BACKGROUND: Risk perceptions influence patient engagement with treatment recommendations, yet it is unknown whether patients with a cardiac inherited disease (CID) hold accurate risk perceptions. The study aimed to examine whether CID patients' and clinician's risk perceptions correlate and factors associated with patient perceptions. METHODS: 202 CID patients (of 618 [36%]) participated in a postal survey assessing perceived risk of aborted cardiac arrest or sudden cardiac death (ACA/SCD). Median age was 53 (16 to 83 years); 86 had Long QT Syndrome (LQTS), 69 had hypertrophic cardiomyopathy, 12 had dilated cardiomyopathy, and 27 had 'other'. Clinical and genetic characteristics were collected from the CID registry; clinical estimate of 5-year risk was determined for LQTS participants (n = 77) using a combination of cardiac arrest or syncope history, maximal QTc length, age, sex and genotype. RESULTS: Patients' risk perceptions of ACA/SCD ranged from 0 to 100%, (median 20%). Greater risk perceptions were associated with: non-New Zealand (NZ) Europeans (p < 0.01), probands (p < 0.05), reporting more physical symptoms (including those unrelated to CID) (p < 0.01), and more symptoms of anxiety (p < 0.05). Median risk assessment by LQTS patients was 15%, and by the clinician was 4.5%. No association was found between patient and clinician assessments of risk (rs = 0.13, ns), 56% of LQTS patients overestimated their risk, 14% underestimated and 30% were accurate. CONCLUSION: Cardiac inherited disease patients' risk perceptions correlate poorly with those of the clinician. Patients overestimating risk tend to have physical symptoms usually unrelated to their CID, and underlying anxiety. Techniques to better communicate risk are needed.

15.
Patient Educ Couns ; 102(12): 2296-2301, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31262672

RESUMO

OBJECTIVE: Patients and clinicians need to have similar understandings of cardiac risk, so patients can make informed decisions. The aim of this study was to assess the concordance of risk estimates between Long-QT-Syndrome (LQTS) patients and an experienced clinician. METHODS: This cross-sectional study included 86 LQTS patients recruited from a clinical registry. Participants completed two questions on their risk of cardiac arrest; likelihood (1=very-unlikely to 5=very-likely), and chance (%), and an experienced clinician computed the same based on risk factors. RESULTS: 30% and 55% of patients had concordant perceptions with the clinician estimate on the chance and likelihood questions respectively. The patients who overestimated their risk (%) had significantly greater emotional responses and concerns about their LQTS. 22 (29%) patients reported a risk of 50% or greater, in contrast to the clinician's risk estimates not exceeding 30%. CONCLUSION: Many LQTS patients had discordant risk perceptions to the clinician's. Patients and clinicians may have different frames of reference, and patients' estimates are linked with emotions. PRACTICAL IMPLICATIONS: Clinicians need to take into account LQTS patients' different frame of reference when discussing risk information. This will support shared decision making.

16.
BMC Cardiovasc Disord ; 19(1): 174, 2019 07 23.
Artigo em Inglês | MEDLINE | ID: mdl-31337358

RESUMO

BACKGROUND: We aimed to determine the mutation yield and clinical applicability of "molecular autopsy" following sudden arrhythmic death syndrome (SADS) by validating and utilizing low-cost high-throughput technologies: Fluidigm Access Array PCR-enrichment with Illumina HiSeq 2000 next generation sequencing (NGS). METHODS: We validated and optimized the NGS platform with a subset of 46 patients by comparison with Sanger sequencing of coding exons of major arrhythmia risk-genes (KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, RYR2). A combined large multi-ethnic international SADS cohort was sequenced utilizing the NGS platform to determine overall molecular yield; rare variants identified by NGS were subsequently reconfirmed by Sanger sequencing. RESULTS: The NGS platform demonstrated 100% sensitivity for pathogenic variants as well as 87.20% sensitivity and 99.99% specificity for all substitutions (optimization subset, n = 46). The positive predictive value (PPV) for NGS for rare substitutions was 16.0% (27 confirmed rare variants of 169 positive NGS calls in 151 additional cases). The overall molecular yield in 197 multi-ethnic SADS cases (mean age 22.6 ± 14.4 years, 68% male) was 5.1% (95% confidence interval 2.0-8.1%), representing 10 cases carrying pathogenic or likely pathogenic risk-mutations. CONCLUSIONS: Molecular autopsy with Fluidigm Access Array and Illumina HiSeq NGS utilizing a selected panel of LQTS/BrS and CPVT risk-genes offers moderate diagnostic yield, albeit requiring confirmatory Sanger-sequencing of mutational variants.


Assuntos
Arritmias Cardíacas/genética , Autopsia/métodos , Análise Mutacional de DNA , Morte Súbita Cardíaca/etiologia , Sequenciamento de Nucleotídeos em Larga Escala , Técnicas Analíticas Microfluídicas , Mutação , Patologia Molecular , Reação em Cadeia da Polimerase , Adolescente , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/mortalidade , Austrália , Causas de Morte , Criança , Pré-Escolar , Europa (Continente) , Feminino , Predisposição Genética para Doença , Hereditariedade , Humanos , Lactente , Masculino , Nova Zelândia , Linhagem , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Fatores de Risco , Adulto Jovem
17.
Eur Heart J ; 40(35): 2953-2961, 2019 Sep 14.
Artigo em Inglês | MEDLINE | ID: mdl-31145795

RESUMO

AIMS: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), implantable cardioverter-defibrillator (ICD) shocks are sometimes ineffective and may even trigger fatal electrical storms. We assessed the efficacy and complications of ICDs placed in patients with CPVT who presented with a sentinel event of sudden cardiac arrest (SCA) while undiagnosed and therefore untreated. METHODS AND RESULTS: We analysed 136 patients who presented with SCA and in whom CPVT was diagnosed subsequently, leading to the initiation of guideline-directed therapy, including ß-blockers, flecainide, and/or left cardiac sympathetic denervation. An ICD was implanted in 79 patients (58.1%). The primary outcome of the study was sudden cardiac death (SCD). The secondary outcomes were composite outcomes of SCD, SCA, appropriate ICD shocks, and syncope. After a median follow-up of 4.8 years, SCD had occurred in three patients (3.8%) with an ICD and none of the patients without an ICD (P = 0.1). SCD, SCA, or appropriate ICD shocks occurred in 37 patients (46.8%) with an ICD and 9 patients (15.8%) without an ICD (P < 0.0001). Inappropriate ICD shocks occurred in 19 patients (24.7%) and other device-related complications in 22 patients (28.9%). CONCLUSION: In previously undiagnosed patients with CPVT who presented with SCA, an ICD was not associated with improved survival. Instead, the ICD was associated with both a high rate of appropriate ICD shocks and inappropriate ICD shocks along with other device-related complications. Strict adherence to guideline-directed therapy without an ICD may provide adequate protection in these patients without all the potential disadvantages of an ICD.

18.
Arch Dis Child ; 104(8): 789-792, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31005896

RESUMO

OBJECTIVE: Guidelines state that verapamil is contraindicated in infants. This is based on reports of cardiovascular collapse and even death after rapid intravenous administration of verapamil in infants with supraventricular tachycardia (SVT). We wish to challenge this contraindication for the specific indication of verapamil sensitive ventricular tachycardia (VSVT) in infants. DESIGN: Retrospective case series and critical literature review. SETTING: Hospitals within New Zealand. PATIENTS: We present a series of three infants/young children with VSVT or 'fascicular VT'. RESULTS: Three children aged between 8 days and 2 years presented with tachycardia 200-220 beats per minute with right bundle brunch block and superior axis. Adenosine failed to cardiovert and specialist review diagnosed VSVT. There were no features of cardiovascular shock. Verapamil was given as a slow infusion over 10-30 min (rather than as a push) and each successfully cardioverted without incident. Critical review of the literature reveals that cardiovascular collapses were associated with a rapid intravenous push in cardiovascularly compromised infants and/or infants given other long-acting antiarrhythmics prior to verapamil. CONCLUSIONS: Verapamil is specifically indicated for the treatment of fascicular VT, and for this indication should be used in infancy, as well as in older children, as first-line treatment or after failure of adenosine raises suspicion of the diagnosis. We outline how to distinguish this tachycardia from SVT and propose a strategy for the safe intravenous slow infusion of verapamil in children, noting that extreme caution is necessary with pre-existing ventricular dysfunction.


Assuntos
Antiarrítmicos/administração & dosagem , Taquicardia Supraventricular/diagnóstico , Verapamil/administração & dosagem , Serviços de Saúde da Criança , Diagnóstico Diferencial , Esquema de Medicação , Eletrocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Infusões Intravenosas , Masculino , Nova Zelândia , Estudos Retrospectivos , Taquicardia Supraventricular/tratamento farmacológico
19.
Resuscitation ; 138: 53-58, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30802556

RESUMO

BACKGROUND: Survival from out-of-hospital cardiac arrest (OHCA) is improved when public access defibrillators are used. Areas of socioeconomic deprivation may have higher rates of OHCA and thus a greater demand for public access defibrillators. We aimed to determine if there was a relationship between socioeconomic factors, the geographic distribution of public access defibrillators (PADs) and incidence of OHCA. METHOD: Socioeconomic deprivation data was obtained from the Census-based 2013 Index of Deprivation. Spatial information for PADs was obtained from a New Zealand PAD database (AED Locations) in 2016 and 2018. Location data for OHCA was obtained from the St John New Zealand OHCA registry for the period 1 October 2013 to 30 June 2016. Relationships between these variables were analysed using a Poisson regression analysis. RESULTS: Cardiac arrest incidence increased with increasing deprivation. The incidence in the most deprived areas of 156.5 events per 100,000 person years (135.4-180.9, 95% CI) is double the incidence in the least deprived areas at 78.0 events per 100,000 person years (66.4-91.7, 95% CI). Significant increases in the rates of OHCA were observed with every 1% increase in proportions of Maori (1.0%, 0.61-1.4%, 95% CI, p = 0.001), Pacific Peoples (0.6%, 0.21-0.9%, p = 0.005), >65 year olds (3.7%, 3.0-4.3%, p < 0.001), and males (3.7%, 1.8-5.6%, p < 0.001). In 2018, the decile 10 areas had the lowest coverage of PADs (65% of these areas contained a PAD) compared with less deprived areas (68-84%, median 81%). CONCLUSIONS: The most socioeconomically deprived communities had the highest incidence of OHCA and the least availability of PADs. This provides impetus for targeted PAD placement in areas of higher deprivation.

20.
Pediatr Crit Care Med ; 20(3): 262-268, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30640888

RESUMO

OBJECTIVES: To raise awareness among pediatric intensive care specialists of catecholaminergic polymorphic ventricular tachycardia; an uncommon cause of polymorphic ventricular tachycardia and ventricular fibrillation arrest in children and young adults where epinephrine (adrenaline), even when given according to international protocols, can be counter-productive and life-threatening. We review three cases of cardiac arrest in children, later proven to be catecholaminergic polymorphic ventricular tachycardia related, where delay in recognition of this condition resulted in significantly longer resuscitation efforts, more interventions, and a longer time to return of spontaneous circulation. DESIGN: Retrospective case series. SETTING: Tertiary children's hospital. PATIENTS AND RESULTS: Three previously well children 4, 5, and 10 years old presented with cardiac arrest triggered by light activity, partial water immersion, and running, respectively. Initial resuscitation was bystander cardiopulmonary resuscitation and community defibrillation in all three cases. Electrocardiograms revealed multifocal ventricular ectopy, and in two (4 and 10 yr old), this correlated with repeated administration of epinephrine during repeated ventricular tachycardia and ventricular fibrillation cardiac arrest resuscitation cycles. This ultimately resolved immediately (at 78 and 140 min, respectively) with IV opiates once catecholaminergic polymorphic ventricular tachycardia was suspected. During recovery, on extracorporeal membrane oxygenation, epinephrine challenge in two children induced polymorphic ventricular tachycardia, bidirectional ventricular tachycardia, and ventricular fibrillation, which was cardioverted with flecainide in the 4-year-old. The third case was recognized early as catecholaminergic polymorphic ventricular tachycardia and was managed by avoiding epinephrine and using opiates and general anesthesia after the initial (single) cardioversion, and had a much better clinical course, without recourse to extracorporeal membrane oxygenation. All three carried de novo RyR2 (cardiac ryanodine) mutations. CONCLUSIONS: Those involved in resuscitation of young people should be aware of catecholaminergic polymorphic ventricular tachycardia and be suspicious of persistent ventricular ectopy, polymorphic, or bidirectional ventricular tachycardia during resuscitation. Appropriate management is avoidance of epinephrine, administration of general anesthesia, IV opiates, and consideration of flecainide.


Assuntos
Epinefrina/administração & dosagem , Parada Cardíaca Extra-Hospitalar/terapia , Taquicardia Ventricular/terapia , Criança , Pré-Escolar , Feminino , Humanos , Unidades de Terapia Intensiva Pediátrica , Masculino , Parada Cardíaca Extra-Hospitalar/genética , Parada Cardíaca Extra-Hospitalar/fisiopatologia , Estudos Retrospectivos , Canal de Liberação de Cálcio do Receptor de Rianodina/genética , Taquicardia Ventricular/genética , Taquicardia Ventricular/fisiopatologia
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