Results of a Multicenter Retrospective Implantable Cardioverter-Defibrillator Registry of Pediatric and Congenital Heart Disease Patients
Charles I. Berul, MD*,*,
George F. Van Hare, MD , ,
Naomi J. Kertesz, MD ,
Anne M. Dubin, MD,
Frank Cecchin, MD*,
Kathryn K. Collins, MD,
Bryan C. Cannon, MD,
Mark E. Alexander, MD*,
John K. Triedman, MD*,
Edward P. Walsh, MD* and
Richard A. Friedman, MD
* Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
Stanford University, Palo Alto, California
University of California, San Francisco, California
Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
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Figure 1 Anatomic Diagnoses of Pediatric and Congenital Implantable Cardioverter-Defibrillator Recipients
Congenital heart disease (CHD) accounts for 46% of total, cardiomyopathies (CM) 23%, and structurally normal hearts (NL) with primary electrical diseases accounting for 31% of all patients. Among CHD patients, diagnoses included tetralogy of Fallot (TOF), transposition of great arteries (TGA), atrial and/or ventricular septal defects, valve abnormalities, single ventricle, Shone's complex, coronary artery congenital anomalies, Ebstein anomaly of tricuspid valve, and others. Cardiomyopathies included hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC), left ventricular noncompaction (LVNC), and restrictive cardiomyopathy (RCM).
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Figure 2 Electrical Diagnoses of Pediatric ICD Recipients With Structurally Normal Hearts
The majority of patients with primary electrical diseases had long-QT syndrome (LQTS). Smaller subgroups included idiopathic (idio) ventricular fibrillation (VF), catecholaminergic polymorphic ventricular tachycardia (CPVT), ventricular tachycardia (VT) not otherwise specified (NOS), conduction system disease (cond sys dz), and Wolff-Parkinson-White (WPW) syndrome with atrioventricular block (AVB). ICD = implantable cardioverter-defibrillator.
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Figure 3 Indications for Implantable Cardioverter-Defibrillator Implantation, Overall and by Era
There is a significant change in the ratio of primary to secondary prevention indications over time between eras (*p = 0.002 for prevention type by era).
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Figure 4 Shock Frequency by Center and Era
The 2 California centers were combined and analyzed as 1 center because they merged electrophysiology programs with overlapping implanting physicians, and also to allow adequate sample size. Appropriate shock frequency by center (top panel) and by era of initial device implantation (middle panel). Overall, secondary indications patients are more likely to have received an appropriate shock than those implanted for primary indications (32% vs. 17%, p < 0.001); however, there were no significant differences between centers. Appropriate shocks were more common in patients implanted in the earlier era (p < 0.05), regardless of indication type. Inappropriate shock frequency by center and overall (bottom panel). The proportion of inappropriate shocks does not differ for primary and secondary indications, and there is no significant difference (p > 0.05) between centers. Center A had a trend toward less inappropriate shocks in secondary indications, but this was also not statistically significant.
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