Cardiac Arrhythmias In Congenital Heart Diseases

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Abstract

Arrhythmias figure prominently among the complications encountered in the varied and diverse population of patients with congenital heart disease, and are the leading cause of morbidity and mortality. The incidence generally increases as the patient ages, with multifactorial predisposing features that may include congenitally malformed or displaced conduction systems, altered hemodynamics, mechanical or hypoxic stress, and residual or postoperative sequelae. The safe and effective management of arrhythmias in congenital heart disease requires a thorough appreciation for conduction system variants, arrhythmia mechanisms, underlying anatomy, and associated physiology. We, therefore, begin this review by presenting the scope of the problem, outlining therapeutic options, and summarizing congenital heart disease-related conduction system anomalies associated with disorders of the sinus node and AV conduction system. Arrhythmias encountered in common forms of congenital heart disease are subsequently discussed. In so doing, we touch upon issues related to risk stratification for sudden death, implantable cardiac devices, catheter ablation, and adjuvant surgical therapy.

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Most cited references 88

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The incidence of congenital heart disease.

Julien Hoffman, Samuel Kaplan (2002)

This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times.

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Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.

Georg Nollert, Teddy Fischlein, Stefan Bouterwek … (1997)

We sought to analyze risk factors for long-term survival (up to 36 years) after surgical repair of tetralogy of Fallot (TOF). Survival after repair is excellent, but data >20 years are rare. From 1958 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 +/- 8.6 years [mean +/- SD], range 2 to 67), 39.7% had a previous palliation. Operative (n = 139) and 1-year (n = 29) deaths were excluded for long-term calculations, resulting in a study group of 490 patients. Actuarial 10-, 20-, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively. Mortality increased 25 years postoperatively from 0.24%/year to 0.94%/year (p = 0.003). The most common cause of death was sudden death (n = 13), followed by congestive heart failure (n = 6). Multivariate correlates of impaired long-term survival were date of operation (before 1970, p = 0.0104), preoperative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079). Postoperative systolic RV/left ventricular pressure ratio and age showed no influence. Patients without preoperative polycythemia and an RV outflow patch (n = 164) had a 36-year actuarial survival rate of 96% and normal life expectancy. Cyanosis, operative experience of the surgeon and an RV outflow tract patch influence long-term outcome after repair of TOF in older children. Early repair by experienced surgeons to avoid polycythemia and excessive RV hypertrophy is supported by this study. However, mortality risk increases 25 years postoperatively, and thus heart monitoring should be intensified.

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Implantable cardioverter-defibrillators in tetralogy of Fallot.

L Beauchesne, Janelle Louise Harris, Hari S. Viswanathan … (2008)

Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population. We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD discharges, identify risk factors, and characterize ICD-related complications. A total of 121 patients (median age 33.3 years; 59.5% male) were enrolled from 11 sites and followed up for a median of 3.7 years. ICDs were implanted for primary prevention in 68 patients (56.2%) and for secondary prevention in 53 (43.8%), defined by clinical sustained ventricular tachyarrhythmia or resuscitated sudden death. Overall, 37 patients (30.6%) received at least 1 appropriate and effective ICD discharge, with a median ventricular tachyarrhythmia rate of 213 bpm. Annual actuarial rates of appropriate ICD shocks were 7.7% and 9.8% in primary and secondary prevention, respectively (P=0.11). A higher left ventricular end-diastolic pressure (hazard ratio 1.3 per mm Hg, P=0.004) and nonsustained ventricular tachycardia (hazard ratio 3.7, P=0.023) independently predicted appropriate ICD shocks in primary prevention. Inappropriate shocks occurred in 5.8% of patients yearly. Additionally, 36 patients (29.8%) experienced complications, of which 6 (5.0%) were acute, 25 (20.7%) were late lead-related, and 7 (5.8%) were late generator-related complications. Nine patients died during follow-up, which corresponds to an actuarial annual mortality rate of 2.2%, which did not differ between the primary and secondary prevention groups. Patients with tetralogy of Fallot and ICDs for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common.

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Author and article information

Journal

Journal ID (nlm-ta): Indian Pacing Electrophysiol J

Journal ID (publisher-id): Indian Pacing Electrophysiol J

Title: Indian Pacing and Electrophysiology Journal

Publisher: Indian Heart Rhythm Society

ISSN (Electronic): 0972-6292

Publication date Collection: Nov-Dec 2009

Publication date (Electronic): 01 November 2009

Volume: 9

Issue: 6

Pages: 299-317

Affiliations

[1 ]Adult Congenital Heart Center and Electrophysiology Service, Montreal Heart Institute, University of Montreal, Montreal, QC, Canada

[2 ]Division of Pediatric Cardiology, Department of Pediatrics, Oregon Health & Science University, Portland, OR, USA

Author notes

Address for correspondence: Dr. Paul Khairy, Adult Congenital Heart Center, Montreal Heart Institute, 5000 Belanger St. E., Montreal, QC, Canada, H1T 1C8. E-mail: paul.khairy@ 123456umontreal.ca

Funding and conflict of interest disclosure Khairy reports having received speaker honoraria from St. Jude Medical, Inc. This work was supported in part by the Canada Research Chair in Electrophysiology and Adult Congenital Heart Disease.

Article

Publisher ID: ipej090299-00

PMC ID: 2766579

PubMed ID: 19898654

SO-VID: f5588ebc-f05c-4bf1-ad5d-d7b9a1d1c16b

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This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cardiac Arrhythmias In Congenital Heart Diseases

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Cardiovascular Innovations and Applications

Most cited references 88

The incidence of congenital heart disease.

Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.

Implantable cardioverter-defibrillators in tetralogy of Fallot.

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