Emanuele Micaglio, Unit of Clinical Arrhythmology and Electrophysiology, IRCCS San Donato Polyclinic, Italy
This is an open-access article distributed under the terms of the
Data on the use of the wearable cardioverter defibrillator in patients suffering from inherited and congenital heart disease are limited. Consequently, evidence for guideline recommendations in this patient population is lacking.
In total 1,675 patients were included in a multicenter registry of eight European centers. In the present cohort, we included 18 patients suffering from congenital and inherited heart disease.
Nine patients (50%) were male with a mean age of 41.3 ±
This retrospective study of patients with inherited and congenital heart disease shows that WCD use is not beneficial in the majority of patients with inherited and congenital heart disease.
Implantable Cardioverter-Defibrillators (ICD) have been used for a long time in primary or secondary prevention of sudden cardiac death (SCD). However, ICDs might be related to several complications e.g., infection, lead failure or dysfunction in patients suffering from inherited cardiovascular diseases (
Studies regarding the use of the WCD in inherited and congenital heart disease are sparce and therefore currently not recommended by any international guideline. The 2022 ESC guideline for the management of patients with ventricular arrhythmias and the prevention of sudden death suggest WCD use in patients with a secondary prevention ICD indication, who are temporarily not candidates for ICD implantation and for patients in the early phase after myocardial infarction (
In this multicenter, retrospective study, patients were recruited between April 2012 and December 2021. 1,675 patients with an indication and prescription of a WCD (LifeVest; ZOLL; Pittsburgh, PA) from nine hospitals (Bergmannsheil University Hospital, University Hospital Mannheim, Helios Clinic Krefeld, University Hospital St. Josef-Hospital Bochum, Klinikum St. Georg Leipzig, University Hospital Bonn, Clinic Saarbrücken, University Hospital Zurich, Frankfurt University Hospital) were included. The study was approved by the local ethics committee. All procedures, analyses and statistical evaluations were performed in accordance with guidelines and regulations of the institutional research committee and conform to the 1975 declaration of Helsinki.
Data from the WCD (wear time, wear days, arrhythmic events) was extracted from ZOLL LifeVest NetworkTM and hospital patient records. The WCD was programmed as recently described (
The events were classified and reviewed by at least two independent physicians. Ventricular tachycardia events were divided in sustained ventricular tachycardia (VT, lasting ≥
All data was collected retrospectively using the electronic patient records and reports. For follow-up data, treating hospital archives were screened and treating physicians or patients were contacted.
Beside baseline characteristics at the initial hospital stay such as baseline left ventricular ejection fraction (LVEF), the New York Heart Association (NYHA) classification, ECG parameters (PR, QRS and QTc- interval) and medication at discharge, follow-up data was collected at the times of three-month (short-term) and six to twelve-month (long-term) follow-up. LVEF was measured by the biplane Simpson's method using echocardiography and/or cardiac magnetic resonance imaging (MRI). An improvement of LVEF was defined as ≥
Continuous variables were analyzed for normal distribution using the Shapiro-Wilk normality test. Data are presented as mean ±
In the present cohort, we included 18 patients suffering from congenital and inherited heart disease. Nine patients (50%) were male with a mean age of 41.3 ±
Baseline characteristics of the cohort.
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SD, Standard deviation; CABG, coronary artery bypass grafting; TIA, transient ischaemic attack; LVEF, left ventricular ejection fraction; BMI, body-mass-index; COPD, Chronic obstructive pulmonary disease; ACE, angiotensin-converting-enzyme; ARNI, angiotensin-converting enzyme inhibitors.
At the initial hospital stay, the mean LVEF of the whole cohort was 48.7 ±
The average wear hours of the WCD in this cohort were 20 ±
WCD data.
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27.8% of the cohort actively inhibited shocks due to false alarms,
In our cohort, four patients suffered from hypertrophic cardiomyopathy (HCM), one of them with an obstructed left ventricular outflow tract (patient nr. 2) and three patients without (patients nr. 1, 3 and 4). Three out of four patients used the WCD as secondary prevention due to prior sustained VT/VF, their mean LVEF was 59.1%. Patient nr. 1 had an infection of her ICD and was bridged during antibiotic therapy with the WCD. Patient nr. 2 received an event recorder two years prior, due to recurrent presyncope. He was then admitted to the hospital in 2015 due to a tumor excision and the event recorder showed multiple nsVTs, which led to an ICD indication. The tumor excision delayed ICD implantation by a few weeks, which was bridged by the WCD.
Patient nr. 3 and 4 were newly diagnosed with HCM and received the WCD after survived SCD in times of further diagnostic measures. Individual patient data regarding definite ICD implantation and follow-up is provided in
Patient information.
| Patient number (gender/age) | Disease | Indication for WCD | VT/VF in WCD | ICD-implantation | ICD shock | VT/VF prior to WCD use | EF baseline (%) |
|---|---|---|---|---|---|---|---|
| 1 (F/16) | HNOCM | After ICD infection WCD as bridge | No | Yes | No | No | 55 |
| 2 (F/60) | HOCM | S/P implanted event recorder following multiple syncopes: detection of nsVTs and ICD-indication. | No | Yes | No | Yes | 60 |
| Now: tumor excision → temporarily ICD-implantation not possible | |||||||
| 3 (M/49) | HNOCM | CPR (VF) | No | Patient denied | Yes | 56.4 | |
| 4 (F/13) | HNOCM (no genetic mutation found) | Secondary prevention | No | Yes | No | Yes | 61 |
| 5 (F/49) | ARVC | Diagnostic | No | Yes | No | No | 61.5 |
| 6 (F/46) | ARVC | CPR (VF) | No | Yes | Yes (inappropriate shock) | Yes | 74.4 |
| 7 (M/76) | Brugada syndrome (variant of the SCN5A gene) | WCD as bridge for patient decision about ICD; S/P syncope and inducible VT and VF in EPS in 2007 | No | Yes | No | No | Unknown |
| 8 (M/61) | Brugada syndrome | Syncope; WCD as bridge until ICD | No | Yes | No | No | 60 |
| 9 (M/32) | Brugada syndrome | Inducible VF in EPS | No | Yes | Yes (inappropriate shock) | No | 56.9 |
| 10 (F/40) | Long-QT-syndrome (point mutation in the KCNQ1 gene) | New ADHD medication | No | No | No | 60 | |
| 11 (M/36) | Non-compaction CM | EF ≤35% | No | No | No | 15.1 | |
| 12 (M/50) | Non-compaction CM | Family history of SCD | No | No | No | 39.4 | |
| 13 (F/17) | Non-compaction CM | EF ≤35% | No | Yes | Yes (VT/VF) | No | 20 |
| 14 (M/51) | Non-compaction CM | EF ≤35% & secondary prevention | No | Yes | No | Yes | 27 |
| 15 (F/42) | Muscular dystrophy of the limb-girdle type with cardiac involvement | VF after ICD-explantation & EF ≤35% | Yes | Died before implantation | Yes | 13 | |
| 16 (M/38) | Transposition of the great arteries | Suspected shunt defect and reprogramming of the CRT-D | No | N/A | No | No | 60 |
| 17 (F/28) | Pulmonary atresia with intact ventricular septum | Premature ventricular contractions and short ventricular salvo | No | No | Yes | 60 | |
| 18 (M/39) | Cardiac sarcoidosis | unknown | No | Unknown | No | Unknown |
Another four patients suffered from non-compaction cardiomyopathy (NCCM), patients nr. 11, 12, 13 and 14. All four patients had severe LV dysfunction, with an LVEF≤
Two patients (nr. 5 and 6) were diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) and subsequently wore the WCD. One patient (patient nr. 5) had further diagnostic measures and wore the WCD as a bridging option, and one patient (patient nr. 6), had cardiac arrest and was incompliant, but declined the use of the WCD after only two hours due to discomfort.
Three patients presented with Brugada syndrome (BrS) (patients nr. 7, 8 and 9):
All three patients used the WCD as primary prevention for SCD. Patient nr. 7 had a previous dysfunction of the ICD but was not sure whether he wanted another ICD and the WCD was used for bridging during this decision period. This patient previously had a syncope, and VF was induced during the electrophysiological study in 2007. Patients nr. 8 and 9 both received their WCD, as diagnostics for BrS and differential diagnosis were still going on. Patient nr. 8 presented with a syncope and patient nr. 9 with an inducible VF in EPS. Besides 19 false alarms in patient nr. 8, there were no conspicuities during the WCD wear time.
Patient nr. 10 suffered from long-QT syndrome type 1 (LQTS1). The patient received the WCD as a primary prevention method for SCD and monitoring option while being dosed up on methylphenidate for her previously diagnosed “Attention Deficit Hyperactivity Disorder” (ADHD). This patient did not have an ICD indication and did not receive an ICD after the WCD wear time, but physicians worried about possible QT-time prolongation due to the new drug, which can prolong the QTc interval, and prescribed the WCD as a monitoring device. After titration of methylphenidate the QTc interval was 470 ms at maximum with no clinical symptoms. The WCD and monitoring were terminated without any further ECG abnormalities after a wear time of 60 days.
Two patients (patients nr. 16 and 17) had congenital heart defects. Patient nr. 16 had a corrected transposition of the great arteries, had a suspected shunt defect and was at the hospital for reprogramming of his CRT-D and deactivating his tachyarrhythmia absoluta. Patient nr. 17 suffered from pulmonary atresia with an intact ventricular septum and used the WCD as secondary prevention due to prior sustained VT/VF.
Patient nr. 18 was diagnosed with cardiac sarcoidosis (CS) but unfortunately stopped the WCD treatment after only five hours due to incompliance and concerns regarding the device.
Lastly, patient nr. 15 suffered from muscular dystrophy of the limb-girdle type with cardiac involvement, secondary cardiomyopathy,
The patient received the WCD as a bridging therapy after ICD-system explantation due to device malfunction and received a temporary CRT-P due to no intrinsic rhythm. After explantation of the ICD, the patient suffered from an episode of ventricular fibrillation in the hospital, whose termination was unsuccessful by multiple defibrillations and amiodarone administrations and was ultimately achieved by lowering the heart rate due to overstimulation with the temporary pacemaker. This arrhythmia was followed by episodes of sustained VTs. The WCD was used as a bridging therapy. There were no shocks delivered by the WCD. Unfortunately, this patient was admitted one month later with recurrent self-limiting sustained VTs, also documented by the WCD. The patient died shortly after admission due to cardiogenic shock, despite extensive attempts of resuscitation. Data regarding definite ICD implantation and follow-up is provided in
ICD-implantation and follow up.
| Variables | All patients ( |
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The main reason for stopping WCD use was definite ICD implantation in 10 out of 18 patients (55.6%). Patient nr. 12 had an LVEF improvement large enough to discontinue WCD use. Two patients (patient nr. 3 and 18) stopped WCD treatment due to incompliance and two patients stopped WCD use due to a normal LVEF from the start and no arrhythmias during the wear time (patient nr. 10 and 16),
The mean follow-up time of the cohort was 570 ±
Six patients were hospitalized, three out of six due to a cardiovascular cause. Two of these patients were hospitalized due to sustained VT/VF and one patient suffered from congestive heart failure. The remaining three patients had a non-cardiovascular cause of hospitalization,
The European Society of Cardiology (ESC) recommends WCD use in adult patients with a secondary prevention ICD indication, who temporarily cannot be implanted with an ICD (class IIa recommendation) (
In our multicenter WCD-registry with 1,675 patients, we identified 18 patients with an inherited or congenital heart disease. 61.1% received the WCD as primary prevention and in 38.9% the WCD was recommended for secondary prevention due to sustained ventricular tachyarrhythmia prior to WCD use.
None of these patients received an appropriate WCD shock. Only one patient (patient nr. 15) had self-limiting sustained VTs which were correctly identified by the WCD but a shock was avoided by the patient. In five patients there were warning signals due to artefacts, but WCD shock delivery was stopped with the response button by the patients.
This shock rate of 0% was previously described in other studies about inherited and congenital heart diseases. E.g., Sarubbi et al. described eight patients with congenital heart disease, also without WCD shocks (
Nevertheless, Rao et al. described a cohort of 43 patients with congenital and 119 patients with inherited heart disease from the USA with two appropriate shocks and four inappropriate shocks, resulting in an event rate of 27 appropriate shocks per 100 patient-years. All shocks were received by patients with an inherited heart disease (
Likewise, Owen et al. described a cohort of nine LQTS patients with one appropriate shock after two days of wear time in a patient after multiple syncopal episodes and seizures (
Spar et al. described a cohort in pediatric patients with 167 patients with cardiomyopathy, 90 patients with congenital heart disease, 47 patients with channelopathy and 36 patients with cardiac arrest without diagnosis, where two appropriate shocks were documented, both in the congenital heart disease group (
Therefore, we may argue that the WCD is not beneficial for all patient groups with inherited and congenital heart disease. Even though there still is a lack of data for the use of WCD in these patients, in the published studies there are often no shocks or a low shock rate, similar to our study (
However, as Rao et al. (
The LQTS1 patient (patient nr. 10), received the WCD as a monitoring option while being dosed up on methylphenidate for their previously diagnosed ADHD. The 2022 ESC guidelines for management of patients with ventricular arrhythmias and prevention of SCD (
Long-QT syndrome has an absolute SCD risk of 4.9%–13% (
Another example of individual indications and benefits from the WCD are the two patients (nr. 5 and 6) suffering from ARVC. One patient (patient nr. 5) had further diagnostic tests for ARVC and wore the WCD as a bridging option, and the other patient (patient nr. 6) had cardiac arrest but declined the use of the WCD after only two hours due to discomfort. This patient was implanted with an ICD, with whom he experienced an inappropriate shock during follow-up. Risk factors described in the literature for SCD in ARVC patients, were not met in patient nr. 5 (
Consequently, these were two patients with very different presentations of ARVC. On the one hand, patient nr. 5 had no prior arrhythmic events and was implanted with a primary prevention ICD without any ICD-related complications, the WCD solely being the bridge until the diagnostic tests were completed and the diagnosis of ARVC confirmed. She wore the WCD as a safety measure until ICD implantation but had no apparent benefit from it.
Patient nr. 6 on the other hand, was at a very high risk for SCD, because of the previous cardiac arrest. She declined WCD use after two hours and was implanted with an ICD but had an inappropriate shock shortly after. This patient might have benefited from the WCD. Moreover, with the WCD she could have manually aborted the inappropriate shock with the response button, which may have a led to appropriate measures to avoid an inappropriate shock with the definite ICD.
A pooled annualized incidence rate of both aborted SCD and SCD without ICD treatment by Agbaedeng et al., describes a rate of 12.66 per 1,000 patients with ARVC. Appropriate ICD shocks are described at an annual rate of 84.7 per 1,000 patients (
Kutyifa et al. describes a congenital/inherited subgroup of their prospective study with WCD use which also included ARVC patients. Patients with ischemic and congenital/inherited heart disease were shown to have significantly higher probabilities of VT/VF than those with nonischemic cardiomyopathy with a rate of sustained VT of 3% after 3 months (
In this study, we also looked at patients with non-compaction cardiomyopathy (NCCM) which presents itself with an alteration of trabeculation (
Patient nr. 13 did not have multiple risk factors for ventricular tachyarrhythmias, except a low EF of 20% which improved to 32% over the first year. This patient received an appropriate ICD shock 126 days after ICD-implantation, due to recorded VT and VF. This may speculate, that the risk for SCD might also be high in some patients with NCCM, with their only risk factor being a low LVEF, where an ICD implantation should be considered early.
Patient nr. 15 with muscular dystrophy of the limb-girdle type with cardiac involvement and secondary cardiomyopathy, received their WCD as a bridge after ICD-system explantation. This patient had a history of VT/VF and suffered from ventricular fibrillation after explantation and from self-limiting VTs while wearing the WCD. After hospitalization, the patient sadly died shortly after with cardiogenic shock. To our knowledge, there is no data available on the rate of annual SCD in patients with muscular dystrophy of the limb-girdle type with cardiac involvement. These cases are associated with an alteration of different ion channel currents, which may enhance the risk of SCD (
Overall compliance in our cohort was 77.8%, with an average wear time of 20 ±
Our compliance rate can be compared to previously published studies, with Spar et al. (
Olic et al. also reports patients with inherited and congenital heart disease stopping WCD use due to incompliance and discomfort, but that may be influenced by the fact that their patients were pregnant at the time and consequently of younger age (
Due to the limited size of this cohort, the retrospective data collection and the heterogeneity of the underlying disease, this study has limitations regarding direct applicability to clinical scenarios. Nevertheless, the aim of this study was to present more data on these rare indications and diseases for WCD in a systematic fashion.
This retrospective study of patients with inherited and congenital heart disease shows, that WCD use is not beneficial in the majority of patients with inherited and congenital heart disease. The appropriate WCD shock rate seems to be relatively low, especially in patients with congenital heart disease. Nevertheless, indications and reasons in this patient group varied individually and the WCD might be a good way to bridge some patients at high risk for SCD until definite ICD implantation, completion of further work-up and optimal drug therapy.
The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.
The studies involving humans were approved by the ethics commission of the medical faculty of the Ruhr University Bochum, head of commission: Prof. Dr. rer. nat. Hans-Joachim Trampisch. The studies were conducted in accordance with the local legislation and institutional requirements. Written informed consent for participation was not required from the participants or the participants’ legal guardians/next of kin in accordance with the national legislation and institutional requirements.
KK: Data curation, Formal Analysis, Writing – original draft, Writing – review & editing. TD: Data curation, Writing – review & editing. CB: Data curation, Writing – review & editing. MG: Data curation, Writing – review & editing. NK: Data curation, Writing – review & editing. TK: Data curation, Writing – review & editing. D-IS: Data curation, Writing – review & editing. HL: Data curation, Writing – review & editing. FS: Data curation, Writing – review & editing. MA: Data curation, Writing – review & editing. TB: Data curation, Writing – review & editing. CW: Data curation, Writing – review & editing. BK: Data curation, Writing – review & editing. SR: Data curation, Writing – review & editing. JK: Data curation, Writing – review & editing. AS: Data curation, Writing – review & editing. JE: Data curation, Writing – review & editing. FD: Data curation, Writing – review & editing. AM: Writing – review & editing. IA: Data curation, Writing – review & editing. AA: Data curation, Writing – review & editing. NH: Data curation, Writing – review & editing. IE-B: Data curation, Methodology, Project administration, Supervision, Validation, Writing – review & editing.
The author(s) declare that financial support was received for the research, authorship, and/or publication of this article.
We acknowledge support by the Open Access Publication Funds of the Ruhr-Universität Bochum. We thank the financial support from the Else-Kröner-foundation (2022 EKES.48) for this research to IEl, the German-Heart-Foundation/German Foundation of Heart Research for this research to IEl, the Hector-Stiftung for this Research to IEL, IA and NH. This research was funded by EU's Horizon 2020 research and innovation program under grant agreement No. 739593 to NH; DFG (Deutsche Forschungsgemeinschaft) HA 7512/2-4 and HA 7512/2-1 to NH. Funders had no role in paper design, data collection, data analysis, interpretation, writing of the paper.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
The author(s) declared that they were not an editorial board member of Frontiers, at the time of submission. AS received educational grants through his institution from Abbott, Bayer Healthcare, Biosense Webster, Biotronik, Boston Scientific, BMS/Pfizer, and Medtronic; and speaker/advisory board/consulting fees from Bayer Healthcare, Biotronik, Daiichi-Sankyo, Medtronic, Novartis, Pfizer, Stride Bio Inc. and Zoll.
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