Device Therapy
Pacing and Defibrillators in Complex Congenital Heart Disease Henry Chubb, 1,2 Mark O’Neill 1,3 and Eric Rosenthal 2,3 1. Division of Imaging Sciences and Biomedical Engineering, King’s College London, London, UK; 2. Department of Congenital Heart Disease, Evelina Children’s Hospital, London, UK; 3. Adult Congenital Heart Disease Group, Departments of Cardiology at Guy’s and St Thomas’ NHS Foundation Trust and Evelina Children’s Hospital, London, UK
Abstract Device therapy in the complex congenital heart disease (CHD) population is a challenging field. There is a myriad of devices available, but none designed specifically for the CHD patient group, and a scarcity of prospective studies to guide best practice. Baseline cardiac anatomy, prior surgical and interventional procedures, existing tachyarrhythmias and the requirement for future intervention all play a substantial role in decision making. For both pacing systems and implantable cardioverter defibrillators, numerous factors impact on the merits of system location (endovascular versus non-endovascular), lead positioning, device selection and device programming. For those with Fontan circulation and following the atrial switch procedure there are also very specific considerations regarding access and potential complications. This review discusses the published guidelines, device indications and the best available evidence for guidance of device implantation in the complex CHD population.
Keywords Congenital heart disease, pacing, implantable cardioverter defibrillator, arrhythmia, sudden cardiac death, cardiac resynchronisation therapy Disclosure: The authors have no conflicts of interest to declare. Received: 14 January 2016 Accepted: 10 March 2016 Citation: Arrhythmia & Electrophysiology Review 2016;5(1):57–64 Access at: www.AERjournal.com DOI: 10.15420/AER.2016.2.3 Correspondence: Prof E Rosenthal, Department of Congenital Heart Disease, Evelina Children’s Hospital, Westminster Bridge Road, London, SE1 7EH, UK; E:[email protected]
Device therapy is increasingly employed in the management of complex congenital heart disease (CHD). Bradycardias, most often related to sinus nodal dysfunction (SND) or atrioventricular nodal (AVN) block, may necessitate the implantation of pacing devices, while malignant arrhythmias may be treated by appropriate use of implantable cardioverter defibrillators (ICDs). However, there is a complex interplay between these classical device indications and associated supraventricular tachyarrhythmias, failure of ventricular function and ventricular dyssynchrony. These considerations, alongside the technical demands of implantation in the context of complex anatomy, necessitate a tailored and nuanced approach in the use of device therapy in the complex CHD population. This review focuses on patients with CHD of moderate and severe complexity, including those with single ventricle circulation and transposition of the great arteries.1 The management of congenital complete heart block is not addressed directly in this review, but shares many of the management principles discussed for the paediatric group.
The incidence of sinus nodal and atrioventricular (AV) nodal dysfunction in the more common complex CHD lesions is summarised in Table 2. It is clear that a significant proportion of these patients will require pacing, in addition to those with intra-atrial conduction block and HisPurkinje disease. Sinoatrial and AV nodal dysfunction may be congenital in conditions such as left atrial isomerism (LAI) and congenitallycorrected transposition of the great arteries (ccTGA; L-transposition), but more frequently the cause is iatrogenic following corrective or palliative surgery. It is also important to note the association of indications for pacing with the increased incidence of atrial and ventricular tachycardias.3 SND, AV dyssynchrony and ventricular bradycardia predispose to the triggering of tachyarrhythmias, and the common pathogeneses of abnormal conduction, surgical scar and progressive myocardial fibrosis are evident. Therefore, device therapy should not be considered in isolation, and a multimodal approach is necessary, combining antiarrhythmic medication, catheter ablation and anti-tachycardia pacemaker (ATP) therapies.2
Pacing Devices in Complex CHD Guidelines and Prevalence of Pacing Indications
Endocardial versus Epicardial Pacing and Number of Leads
Guidelines for pacing in the complex CHD cohort are limited by a lack of prospective randomised trials, and the marked heterogeneity of the group. The indications for permanent cardiac pacing in adult patients with complex CHD are summarised in Table 1, but in children the majority of prospective data relate to patients with congenital complete heart block, and practices are generally extrapolated from those principles.
Epicardial pacing systems possess obvious advantages for patients with complex CHD. They do not rely upon venous access, may be placed during concurrent surgery, avoid endovascular leads and associated thromboembolic events19 and present fewer concerns regarding lead extraction. However, these factors need to be weighed up against increased lead failure rates and pacing thresholds, with a
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Device Therapy Table 1: Recommendations for Permanent Pacing in Adults with Complex CHD Class
Clinical Indication
Level of
Class I
Symptomatic SND, including documented sinus
C
Table 2: Prevalence of Indications for Pacing Therapy and ICD Therapy in Selected Congenital Heart Disease of Moderate and Severe Complexity
Evidence bradycardia or chronotropic incompetence that is intrinsic or secondary to required drug therapy Symptomatic bradycardia in conjunction with any
Intervention ToF
Surgical repair
1–2 %4,5
D-TGA
Post-Mustard
60–82 %6–8
Post-Senning
%7–9
B
C
Ventricular
5 %5
2–15 %4,5
arrhythmias
2 %6
4 %10
7 %4
1 %11
1–2 %10,11
Pacing and Defibrillators in Complex Congenital Heart Disease Henry Chubb, 1,2 Mark O’Neill 1,3 and Eric Rosenthal 2,3 1. Division of Imaging Sciences and Biomedical Engineering, King’s College London, London, UK; 2. Department of Congenital Heart Disease, Evelina Children’s Hospital, London, UK; 3. Adult Congenital Heart Disease Group, Departments of Cardiology at Guy’s and St Thomas’ NHS Foundation Trust and Evelina Children’s Hospital, London, UK
Abstract Device therapy in the complex congenital heart disease (CHD) population is a challenging field. There is a myriad of devices available, but none designed specifically for the CHD patient group, and a scarcity of prospective studies to guide best practice. Baseline cardiac anatomy, prior surgical and interventional procedures, existing tachyarrhythmias and the requirement for future intervention all play a substantial role in decision making. For both pacing systems and implantable cardioverter defibrillators, numerous factors impact on the merits of system location (endovascular versus non-endovascular), lead positioning, device selection and device programming. For those with Fontan circulation and following the atrial switch procedure there are also very specific considerations regarding access and potential complications. This review discusses the published guidelines, device indications and the best available evidence for guidance of device implantation in the complex CHD population.
Keywords Congenital heart disease, pacing, implantable cardioverter defibrillator, arrhythmia, sudden cardiac death, cardiac resynchronisation therapy Disclosure: The authors have no conflicts of interest to declare. Received: 14 January 2016 Accepted: 10 March 2016 Citation: Arrhythmia & Electrophysiology Review 2016;5(1):57–64 Access at: www.AERjournal.com DOI: 10.15420/AER.2016.2.3 Correspondence: Prof E Rosenthal, Department of Congenital Heart Disease, Evelina Children’s Hospital, Westminster Bridge Road, London, SE1 7EH, UK; E:[email protected]
Device therapy is increasingly employed in the management of complex congenital heart disease (CHD). Bradycardias, most often related to sinus nodal dysfunction (SND) or atrioventricular nodal (AVN) block, may necessitate the implantation of pacing devices, while malignant arrhythmias may be treated by appropriate use of implantable cardioverter defibrillators (ICDs). However, there is a complex interplay between these classical device indications and associated supraventricular tachyarrhythmias, failure of ventricular function and ventricular dyssynchrony. These considerations, alongside the technical demands of implantation in the context of complex anatomy, necessitate a tailored and nuanced approach in the use of device therapy in the complex CHD population. This review focuses on patients with CHD of moderate and severe complexity, including those with single ventricle circulation and transposition of the great arteries.1 The management of congenital complete heart block is not addressed directly in this review, but shares many of the management principles discussed for the paediatric group.
The incidence of sinus nodal and atrioventricular (AV) nodal dysfunction in the more common complex CHD lesions is summarised in Table 2. It is clear that a significant proportion of these patients will require pacing, in addition to those with intra-atrial conduction block and HisPurkinje disease. Sinoatrial and AV nodal dysfunction may be congenital in conditions such as left atrial isomerism (LAI) and congenitallycorrected transposition of the great arteries (ccTGA; L-transposition), but more frequently the cause is iatrogenic following corrective or palliative surgery. It is also important to note the association of indications for pacing with the increased incidence of atrial and ventricular tachycardias.3 SND, AV dyssynchrony and ventricular bradycardia predispose to the triggering of tachyarrhythmias, and the common pathogeneses of abnormal conduction, surgical scar and progressive myocardial fibrosis are evident. Therefore, device therapy should not be considered in isolation, and a multimodal approach is necessary, combining antiarrhythmic medication, catheter ablation and anti-tachycardia pacemaker (ATP) therapies.2
Pacing Devices in Complex CHD Guidelines and Prevalence of Pacing Indications
Endocardial versus Epicardial Pacing and Number of Leads
Guidelines for pacing in the complex CHD cohort are limited by a lack of prospective randomised trials, and the marked heterogeneity of the group. The indications for permanent cardiac pacing in adult patients with complex CHD are summarised in Table 1, but in children the majority of prospective data relate to patients with congenital complete heart block, and practices are generally extrapolated from those principles.
Epicardial pacing systems possess obvious advantages for patients with complex CHD. They do not rely upon venous access, may be placed during concurrent surgery, avoid endovascular leads and associated thromboembolic events19 and present fewer concerns regarding lead extraction. However, these factors need to be weighed up against increased lead failure rates and pacing thresholds, with a
© RADCLIFFE CARDIOLOGY 2015
AER5.1_Rosenthal_FINAL.indd 57
57
18/05/2016 01:54
Device Therapy Table 1: Recommendations for Permanent Pacing in Adults with Complex CHD Class
Clinical Indication
Level of
Class I
Symptomatic SND, including documented sinus
C
Table 2: Prevalence of Indications for Pacing Therapy and ICD Therapy in Selected Congenital Heart Disease of Moderate and Severe Complexity
Evidence bradycardia or chronotropic incompetence that is intrinsic or secondary to required drug therapy Symptomatic bradycardia in conjunction with any
Intervention ToF
Surgical repair
1–2 %4,5
D-TGA
Post-Mustard
60–82 %6–8
Post-Senning
%7–9
B
C
Ventricular
5 %5
2–15 %4,5
arrhythmias
2 %6
4 %10
7 %4
1 %11
1–2 %10,11
