scholarly journals Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study

EP Europace ◽  
2020 ◽  
Author(s):  
Gabrielle Norrish ◽  
Henry Chubb ◽  
Ella Field ◽  
Karen McLeod ◽  
Maria Ilina ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Implantable Cardioverter Defibrillator ◽  
Incidence Rate ◽  
Clinical Outcomes ◽  
Cardioverter Defibrillator ◽  
National Cohort ◽  
Icd Shock ◽  
The Uk ◽  
Device Complications

Abstract Aims Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. Methods and results Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM from the UK. Seventy-six (84%) had an endovascular system [14 (18%) dual coil], 3 (3%) epicardial, and 11 (12%) subcutaneous system. Defibrillation threshold (DFT) testing was performed at implant in 68 (76%). Inadequate DFT in four led to implant adjustment in three patients. Over a median follow-up of 54 months (interquartile range 28–111), 25 (28%) patients had 53 appropriate therapies [ICD shock n = 45, anti-tachycardia pacing (ATP) n = 8], incidence rate 4.7 per 100 patient years (95% CI 2.9–7.6). Eight inappropriate therapies occurred in 7 (8%) patients (ICD shock n = 4, ATP n = 4), incidence rate 1.1/100 patient years (95% CI 0.4–2.5). Three patients (3%) died following arrhythmic events, despite a functioning device. Other device complications were seen in 28 patients (31%), including lead-related complications (n = 15) and infection (n = 10). No clinical, device, or programming characteristics predicted time to inappropriate therapy or lead complication. Conclusion In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.

scholarly journals Clinical outcomes and programming strategies of implantable cardioverter defibrillator (ICD) devices during childhood in hypertrophic cardiomyopathy: a UK national cohort study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Norrish ◽  
H Chubb ◽  
E Field ◽  
K McCleod ◽  
J Till ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Incidence Rate ◽  
Clinical Outcomes ◽  
P Value ◽  
Funding Source ◽  
Complication Rates ◽  
Antitachycardia Pacing ◽  
Icd Implantation ◽  
National Cohort ◽  
Icd Shock

Abstract Background Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM). ICDs have been shown to be effective at terminating malignant ventricular arrhythmias but at the expense of a high incidence of complications. The optimal device and programming strategies to reduce complications in this patient group are unknown. Purpose To describe the programming strategies and clinical outcomes of ICD implantation in childhood HCM. Methods Anonymised, non-invasive clinical data were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM (n=687) and an ICD in-situ from the United Kingdom. Results 96 patients (61 male (64%), 6 non-sarcomeric (6%)) underwent ICD implantation at a median age 14yr (IQR 11–16, range 3–16) and weight 52.3 kg (IQR 34.8–63.1). Indication for ICD was primary prevention in 72 (75%). 82 (85%) had an endovascular system, 3 (3%) epicardial and 11 (12%) subcutaneous system. 61 patients (74%) were receiving one or more cardioactive medications at implantation [B blockers n=66, 70%, disopyramide n=14, 15%, amiodarone n=7, 7%, calcium channel blocker n=7, 9%, other n=5, 6%]. Programming practices varied: all had VF therapies activated (median 220bpm, IQR 212–230); 70 (73%) had a VT zone programmed (median rate 187 bpm, SD 20.9), of which 26 (27%) had therapies activated. 50 patients (61%) had antitachycardia pacing (ATP) activated. Over a median follow up of 53.6 months (IQR 27.3,108.4), 4 patients (4.2%) died following arrhythmic events despite a functioning device. 25 patients had 53 appropriate therapies (ICD shock n=47, ATP n=8), incidence rate 5.22 (95% CI 3.5–7.8). On univariable analysis, secondary prevention indication for ICD implantation was the only predictor of therapy [16 (64%) vs 8 (11.3%), p value <0.001]. 8 (8.3%) patients had 9 inappropriate therapies (ICD shock n=4, ATP n=5), incidence rate 1.37 (95% CI 0.65–2.8), caused by T wave oversensing (n=2), lead migration (n=1), supraventricular tachycardia (n=1). Device complications were seen in 30 patients (31%), including lead complications (n=16) and infection (n=10). No clinical characteristics predicted time to inappropriate therapy or lead complication. Conclusions In a contemporary cohort of children with HCM, the incidence of inappropriate therapies is lower than previously reported, yet complication rates remain higher than reported in adult patients. No clinical, device or programming strategies were associated with inappropriate therapies or lead complications. Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation

scholarly journals Risk of subsequent ventricular arrhythmia is higher in primary prevention patients with implantable cardioverter defibrillator than in secondary prevention patients

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
You Zhou ◽  
Shuang Zhao ◽  
Keping Chen ◽  
Wei Hua ◽  
Yangang Su ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Ventricular Arrhythmia ◽  
Secondary Prevention ◽  
Implantable Cardioverter Defibrillator ◽  
Incidence Rate ◽  
Home Monitoring ◽  
Icd Therapy ◽  
Cardioverter Defibrillator ◽  
All Cause Mortality

Abstract Background Because of previous ventricular arrhythmia (VA) episodes, patients with implantable cardioverter-defibrillator (ICD) for secondary prevention (SP) are generally considered to have a higher burden of VAs than primary prevention (PP) patients. However, when PP patients experienced VA, the difference in the prognosis of these two patient groups was unknown. Methods The clinical characteristics and follow-up data of 835 ICD patients (364 SP patients and 471 PP patients) with home monitoring feature were retrospectively analysed. The incidence rate and risk of subsequent VA and all-cause mortality were compared between PP patients after the first appropriate ICD therapy and SP patients. Results During a mean follow-up of 44.72 ± 20.87 months, 210 (44.59%) PP patients underwent appropriate ICD therapy. In the Kaplan-Meier survival analysis, the PP patients after appropriate ICD therapy were more prone to VA recurrence and all-cause mortality than SP patients (P<0.001 for both endpoints). The rate of appropriate ICD therapy and all-cause mortality in PP patients after the first appropriate ICD therapy was significantly higher than that in SP patients (for device therapy, 59.46 vs 20.64 patients per 100 patient-years; incidence rate ratio [IRR] 2.880, 95% confidence interval [CI]: 2.305–3.599; P<0.001; for all-cause mortality, 14.08 vs 5.40 deaths per 100 patient-years; IRR 2.607, 95% CI: 1.884–3.606; P<0.001). After propensity score matching for baseline characteristics, the risk of VA recurrence in PP patients with appropriate ICD therapy was still higher than that in SP patients (41.80 vs 19.10 patients per 100 patient-years; IRR 2.491, 95% CI: 1.889–3.287; P<0.001), but all-cause mortality rates were similar between the two groups (12.61 vs 9.33 deaths per 100 patient-years; IRR 1.352, 95% CI: 0.927–1.972; P = 0.117). Conclusions Once PP patients undergo appropriate ICD therapy, they will be more prone to VA recurrence and death than SP patients.

P986Future risk of bradyarrhythmias in patients with hypertrophic cardiomyopathy and implantable cardioverter defibrillator indication

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Nakasuka ◽  
S Kitada ◽  
Y Kawada ◽  
M Kato ◽  
N Ohte
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Implantable Cardioverter Defibrillator ◽  
Demographic Information ◽  
Cardioverter Defibrillator ◽  
Significant Difference ◽  
Pr Interval ◽  
Qrs Duration ◽  
Baseline Characteristics ◽  
Japanese Guideline

Abstract Background Subcutaneous-implantable cardioverter defibrillator (S-ICD) has been shown to be effective for prevention of sudden cardiac death. Patients with hypertrophic cardiomyopathy (HCM) having ICD indication are considered to introduce either S-ICD or transvenous-ICD, however it is uncertain which is better since S-ICD is not suitable for patients who need pacing and data on the necessity for pacing in HCM patients are limited. Purpose The purpose of this study was to investigate the risk factors associated with the future risk of bradyarrhythmias needed to be paced in patients with HCM and ICD indication. Methods This is a retrospective, single-center cohort study. Of 169 HCM patients diagnosed in our institution, 80 with ICD indication in accordance with the Japanese guideline in 2011 were enrolled as study subjects (31 females, mean age 63±15 years). They were divided into two groups – patients who progressed to bradyarrhythmias which needed pacing during the follow-up period (Brady group) and those who not (Non-brady group). Baseline characteristics at the time of diagnosis of HCM including demographic information, the results of clinical examination such as blood test, echocardiography and electrocardiography (ECG) were compared between the Brady and Non-brady group. Results During a mean follow-up period of 6.8±5.4 years, 9 patients (11%) progressed bradyarrhtyhmias which needed pacing. Symptomatic sick sinus syndrome (SSS) was the primary cause (7 SSS, 1 atrial fibrillation [AF] with bradycardia, 1 after atrioventricular nodal ablation). Comparing between the Brady and Non-brady group, there was no significant difference in clinical variables such as age, heart rate, PR interval and QRS duration in ECG, EF levels, BNP levels, beta-blocker usage rate, prevalence of hypertension or diabetes mellitus. On the other hand, women were more in Brady group than in Non-brady group (7/9, 78% vs. 24/71, 34%; p=0.01). Furthermore, more patients in Brady group had documented AF at the beginning and during follow-up period (7/9, 78% vs. 25/71, 36%; p=0.02) and took anti-arrhythmic drugs (AAD) (8/9, 89% vs. 19/71, 27%; p<0.001) including amiodarone than those in Non-brady group. Conclusion In HCM patients with ICD indication, around 10% of patients have a potential risk of bradyarrhythmias needed to be paced, especially in female, those with AF and/or AAD usage.

scholarly journals Long-term follow-up of paediatric patients with a subcutaneous implantable cardioverter-defibrillator

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
W Van Der Stuijt ◽  
K.C De Wilde ◽  
S.W.E Baalman ◽  
A.B.E Quast ◽  
N.A Blom ◽  
...  
Keyword(s):  
Implantable Cardioverter Defibrillator ◽  
Clinical Outcomes ◽  
Paediatric Population ◽  
Physical Growth ◽  
Cardioverter Defibrillator ◽  
Inappropriate Shocks ◽  
Paediatric Patients ◽  
Long Term Follow Up

Abstract Background Inherited channelopathies, cardiomyopathies and structural heart diseases are known to increase the risk of sudden cardiac death in children and are occasionally treated with an implantable cardioverter-defibrillator (ICD). Due to their physical growth and higher level of activity, children experience more adverse events than adults, such as lead fractures, vascular problems and infections. The subcutaneous ICD (S-ICD) was introduced to avoid these lead-related complications and is expected to be especially beneficial for children who require ICD therapy. However, long-term follow-up data of this population are lacking. Purpose To evaluate long-term clinical outcomes of the S-ICD in the paediatric population. Methods Follow-up data were prospectively collected of all paediatric patients (age &lt;18 years) who received their S-ICD in our tertiary centre between November 2009 and June 2019. Clinical outcomes that were assessed were appropriate or inappropriate shocks and complications that required surgical intervention. Results A total of 21 paediatric patients received an S-ICD, with a median age of 15 years (ranged 8–17) and a median body mass index of 18.8 kg/m2 (ranged 15.5–28.8, lowest weight 30kg). The indication for ICD implantation was mostly idiopathic ventricular fibrillation (38.1%) or genetic arrhythmic disease (28.6%). The subcutaneous lead was implanted in an S-shape to accommodate for growth in small patients, and straightening of the lead could be observed on successive chest X-rays (see figure). There was no need for lead or device revisions in this cohort. After a median follow-up of 4.3 years (IQR 2.7–6.5), five patients (23.8%) received an appropriate shock for ventricular arrhythmias and six patients (28.6%) received an inappropriate shock for supraventricular tachycardia (n=4) or double-counting (n=2). Complications that required device extraction occurred in three patients after 0.8±1.1 years and was the result of inappropriate shocks (n=1) and pocket infection (n=2). In two patients the S-ICD was extracted due to progression of their cardiac disease, of whom one needed biventricular pacing and one underwent heart transplantation. Conclusions The S-ICD appears efficacious in this heterogeneous paediatric population. Although higher than reported in adults, the long-term complication rate in paediatric S-ICD recipients is similar to the paediatric transvenous ICD population. Funding Acknowledgement Type of funding source: None

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