Subcutaneous implantable cardioverter-defibrillators: long-term results of the EFFORTLESS study.

AIMS: To report 5-year outcomes of EFFORTLESS registry patients with early generation subcutaneous implantable cardioverter-defibrillator (S-ICD) devices. METHODS AND RESULTS: Kaplan-Meier, trend and multivariable analyses were performed for mortality and late (years 2-5) complications, appropriate shock (AS) and inappropriate shock (IAS) rates. Nine hundred and eighty-four of 994 enrolled patients with diverse diagnoses (28% female, 48 ± 17 years, body mass index 27 ± 6 kg/m2, ejection fraction 43 ± 18%) underwent S-ICD implantation. Median follow-up was 5.1 years (interquartile range 4.7-5.5 years). All-cause mortality was 9.3% (95% confidence interval 7.2-11.3%) at 5 years; 703 patients remained in follow-up on study completion, 171 withdrew including 87 (8.8%) with device explanted, and 65 (6.6%) lost to follow-up. Of the explants, only 20 (2.0%) patients needed a transvenous device for pacing indications. First and final shock efficacy for discrete ventricular arrhythmias was consistent at 90% and 98%, respectively, with storm episode final shock efficacy at 95.2%. Time to therapy remained unaltered. Overall 1- and 5-year complication rates were 8.9% and 15.2%, respectively. Early complications did not predict later complications. There were no structural lead failures. Inappropriate shock rates at 1 and 5 years were 8.7% and 16.9%, respectively. Self-terminating inappropriately sensed episodes predicted late IAS. Predictors of late AS included self-terminating appropriately sensed episodes and earlier AS. CONCLUSION: In this diverse S-ICD registry population, spontaneous shock efficacy was consistently high over 5 years. Very few patients underwent S-ICD replacement with a transvenous device for pacing indications. Treated and self-terminating arrhythmic episodes predict future shock events, which should encourage more personalized device optimization.

Characterization of postoperative "fibrin web" formation after canine cataract surgery.

PURPOSE: To describe the occurrence and associated factors for "fibrin web" (FW) formation following phacoemulsification in dogs. METHODS: A retrospective review of medical records of all dogs undergoing phacoemulsification (MU-Veterinary Health Center, 2014-2018) was conducted to associate FW formation with signalment, systemic co-morbidities, cataract stage, surgeon (resident vs faculty), phacoemulsification time, IOL, and intracameral injections including viscoelastic type. Both univariate and multivariate statistical analyses were performed to evaluate associations among variables with FW formation. RESULTS: Data from 398 eyes on 201 dogs were included; 4 left eyes (4 dogs) developed presumptive endophthalmitis and were excluded from further analysis. Forty-eight eyes did not have cataract surgery. Hence, 350 eyes on 201 dogs were included in the analyses. Among these, 84 eyes (59 dogs) developed a FW. Univariate analyses showed that the odds of FW increased with age and phacoemulsification time. Additionally, FW web was associated lens type, lens brand, and viscoelastic type. Multivariate analyses showed that when comparing lens types in combination with a particular viscoelastic, viscoelastic impacted the estimated prevalence of FW formation the most. In contrast, when the data were analyzed by lens brand, lens brand impacted prevalence more than viscoelastic type. Diabetes mellitus was not associated with FW formation. CONCLUSIONS: Based on the available data, intraocular lens implantation, viscoelastic type, dog age, and phacoemulsification time were associated with FW formation. Diabetes mellitus, gender, cataract stage, surgeon, intracameral injections other than viscoeleastic, and intra- and postoperative complications were not associated with FW formation.

The learning curve associated with the introduction of the subcutaneous implantable defibrillator.

AIMS: The subcutaneous implantable cardioverter defibrillator (S-ICD) was introduced to overcome complications related to transvenous leads. Adoption of the S-ICD requires implanters to learn a new implantation technique. The aim of this study was to assess the learning curve for S-ICD implanters with respect to implant-related complications, procedure time, and inappropriate shocks (IASs). METHODS AND RESULTS: In a pooled cohort from two clinical S-ICD databases, the IDE Trial and the EFFORTLESS Registry, complications, IASs at 180 days follow-up and implant procedure duration were assessed. Patients were grouped in quartiles based on experience of the implanter and Kaplan-Meier estimates of complication and IAS rates were calculated. A total of 882 patients implanted in 61 centres by 107 implanters with a median of 4 implants (IQR 1,8) were analysed. There were a total of 59 patients with complications and 48 patients with IAS. The complication rate decreased significantly from 9.8% in Quartile 1 (least experience) to 5.4% in Quartile 4 (most experience) (P = 0.02) and non-significantly for IAS from 7.9 to 4.8% (P = 0.10). Multivariable analysis demonstrated a hazard ratio of 0.78 (P = 0.045) for complications and 1.01 (P = 0.958) for IAS. Dual-zone programming increased with experience of the individual implanter (P < 0.001), which reduced IAS significantly in the multivariable model (HR 0.44, P = 0.01). Procedure time decreased from 75 to 65 min (P < 0.001). The complication rate and procedure time stabilized after Quartile 2 (>13 implants). CONCLUSION: There is a short and significant learning curve associated with physicians adopting the S-ICD. Performance stabilizes after 13 implants.

Worldwide experience with a totally subcutaneous implantable defibrillator: early results from the EFFORTLESS S-ICD Registry.

AIMS: The totally subcutaneous implantable-defibrillator (S-ICD) is a new alternative to the conventional transvenous ICD system to minimize intravascular lead complications. There are limited data describing the long-term performance of the S-ICD. This paper presents the first large international patient population collected as part of the EFFORTLESS S-ICD Registry. METHODS AND RESULTS: The EFFORTLESS S-ICD Registry is a non-randomized, standard of care, multicentre Registry designed to collect long-term, system-related, clinical, and patient reported outcome data from S-ICD implanted patients since June 2009. Follow-up data are systematically collected over 60-month post-implant including Quality of Life. The study population of 472 patients of which 241 (51%) were enrolled prospectively has a mean follow-up duration of 558 days (range 13-1342 days, median 498 days), 72% male, mean age of 49 ± 18 years (range 9-88 years), 42% mean left ventricular ejection fraction. Complication-free rates were 97 and 94%, at 30 and 360 days, respectively. Three hundred and seventeen spontaneous episodes were recorded in 85 patients during the follow-up period. Of these episodes, 169 (53%) received therapy, 93 being for Ventricular Tachycardia/Fibrillation (VT/VF). One patient died of recurrent VF and severe bradycardia. Regarding discrete VT/VF episodes, first shock conversion efficacy was 88% with 100% overall successful clinical conversion after a maximum of five shocks. The 360-day inappropriate shock rate was 7% with the vast majority occurring for oversensing (62/73 episodes), primarily of cardiac signals (94% of oversensed episodes). CONCLUSION: The first large cohort of real-world data from an International patient S-ICD population demonstrates appropriate system performance with clinical event rates and inappropriate shock rates comparable with those reported for conventional ICDs. Clinical trial registration URL: http://www.clinicaltrials.gov. Unique identifier NCT01085435.

Safety and efficacy of a totally subcutaneous implantable-cardioverter defibrillator.

BACKGROUND: The most frequent complications associated with implantable cardioverter-defibrillators (ICDs) involve the transvenous leads. A subcutaneous implantable cardioverter-defibrillator (S-ICD) has been developed as an alternative system. This study evaluated the safety and effectiveness of the S-ICD System (Cameron Health/Boston Scientific) for the treatment of life-threatening ventricular arrhythmias (ventricular tachycardia/ventricular fibrillation). METHODS AND RESULTS: This prospective, nonrandomized, multicenter trial included adult patients with a standard indication for an ICD, who neither required pacing nor had documented pace-terminable ventricular tachycardia. The primary safety end point was the 180-day S-ICD System complication-free rate compared with a prespecified performance goal of 79%. The primary effectiveness end point was the induced ventricular fibrillation conversion rate compared with a prespecified performance goal of 88%, with success defined as 2 consecutive ventricular fibrillation conversions of 4 attempts. Detection and conversion of spontaneous episodes were also evaluated. Device implantation was attempted in 321 of 330 enrolled patients, and 314 patients underwent successful implantation. The cohort was followed for a mean duration of 11 months. The study population was 74% male with a mean age of 52±16 years and mean left ventricular ejection fraction of 36±16%. A previous transvenous ICD had been implanted in 13%. Both primary end points were met: The 180-day system complication-free rate was 99%, and sensitivity analysis of the acute ventricular fibrillation conversion rate was >90% in the entire cohort. There were 38 discrete spontaneous episodes of ventricular tachycardia/ventricular fibrillation recorded in 21 patients (6.7%), all of which successfully converted. Forty-one patients (13.1%) received an inappropriate shock. CONCLUSIONS: The findings support the efficacy and safety of the S-ICD System for the treatment of life-threatening ventricular arrhythmias.

An entirely subcutaneous implantable cardioverter-defibrillator.

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) prevent sudden death from cardiac causes in selected patients but require the use of transvenous lead systems. To eliminate the need for venous access, we designed and tested an entirely subcutaneous ICD system. METHODS: First, we conducted two short-term clinical trials to identify a suitable device configuration and assess energy requirements. We evaluated four subcutaneous ICD configurations in 78 patients who were candidates for ICD implantation and subsequently tested the best configuration in 49 additional patients to determine the subcutaneous defibrillation threshold in comparison with that of the standard transvenous ICD. Then we evaluated the long-term use of subcutaneous ICDs in a pilot study, involving 6 patients, which was followed by a trial involving 55 patients. RESULTS: The best device configuration consisted of a parasternal electrode and a left lateral thoracic pulse generator. This configuration was as effective as a transvenous ICD for terminating induced ventricular fibrillation, albeit with a significantly higher mean (+/-SD) energy requirement (36.6+/-19.8 J vs. 11.1+/-8.5 J). Among patients who received a permanent subcutaneous ICD, ventricular fibrillation was successfully detected in 100% of 137 induced episodes. Induced ventricular fibrillation was converted twice in 58 of 59 patients (98%) with the delivery of 65-J shocks in two consecutive tests. Clinically significant adverse events included two pocket infections and four lead revisions. After a mean of 10+/-1 months, the device had successfully detected and treated all 12 episodes of spontaneous, sustained ventricular tachyarrhythmia. CONCLUSIONS: In small, nonrandomized studies, an entirely subcutaneous ICD consistently detected and converted ventricular fibrillation induced during electrophysiological testing. The device also successfully detected and treated all 12 episodes of spontaneous, sustained ventricular tachyarrhythmia. (ClinicalTrials.gov numbers, NCT00399217 and NCT00853645.)

Implant and Midterm Outcomes of the Subcutaneous Implantable Cardioverter-Defibrillator Registry: The EFFORTLESS Study.

BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (S-ICD) was developed to defibrillate ventricular arrhythmias, avoiding drawbacks of transvenous leads. The global EFFORTLESS S-ICD (Evaluation oF FactORs ImpacTing CLinical Outcome and Cost EffectiveneSS of the S-ICD) registry is collecting outcomes in 985 patients during a 5-year follow-up. OBJECTIVES: The primary goal of the EFFORTLESS registry is to determine the safety of the S-ICD by evaluating complications and inappropriate shock rate. METHODS: This is the first report on the full patient cohort and study endpoints with follow-up ≥1 year. The predefined endpoints are 30- and 360-day complications, and shocks for atrial fibrillation or supraventricular tachycardia. RESULTS: Patients were followed for 3.1 ± 1.5 years and 82 completed the study protocol 5-year visit. Average age was 48 years, 28% were women, ejection fraction was 43 ± 18%, and 65% had a primary prevention indication. The S-ICD system and procedure complication rate was 4.1% at 30 days and 8.4% at 360 days. The 1-year complication rate trended toward improvement from the first to last quartile of enrollment (11.3% [quartile 1]) to 7.8% [quartile 2], 6.6% [quartile 3], and 7.4% [quartile 4]; quartile 1 vs. quartiles 2 to 4; p = 0.06). Few device extractions occurred due to need for antitachycardia (n = 5), or biventricular (n = 4) or bradycardia pacing (n = 1). Inappropriate shocks occurred in 8.1% at 1 year and 11.7% after 3.1 years. At implant, 99.5% of patients had a successful conversion of induced ventricular tachycardia or ventricular fibrillation. The 1- and 5-year rates of appropriate shock were 5.8% and 13.5%, respectively. Conversion success for discrete spontaneous episodes was 97.4% overall. CONCLUSIONS: This registry demonstrates that the S-ICD fulfills predefined endpoints for safety and efficacy. Midterm performance rates on complications, inappropriate shocks, and conversion efficacy were comparable to rates observed in transvenous implantable cardioverter-defibrillator studies. (Evaluation oF Factors ImpacTing CLinical Outcome and Cost EffectiveneSS of the S-ICD [The EFFORTLESS S-ICD Registry]; NCT01085435).

Evaluation of subcutaneous ICD early performance in hypertrophic cardiomyopathy from the pooled EFFORTLESS and IDE cohorts.

BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (S-ICD) is a potential alternative to transvenous systems in hypertrophic cardiomyopathy (HCM) where lead complications are a significant issue. OBJECTIVES: To compare the S-ICD efficacy of defibrillation threshold (DFT) testing, arrhythmia therapy, and complications in HCM versus non-HCM patients. METHODS: Outcomes of patients with HCM implanted with S-ICD were compared to non-HCM S-ICD recipients using pooled data from a total of 872 subjects enrolled in the EFFORTLESS Registry and US IDE study. RESULTS: The cohort included 99 HCM (75% male) and 773 non-HCM (72% male) patients with a median follow-up of 637 days. The HCM cohort was younger and more likely to receive a primary-prevention S-ICD (88.5% vs 67.5%, P < .0001). During implant testing, successful defibrillation at ≤80 J was achieved in 98.9% of HCM and 98.5% of non-HCM patients. One year postoperative complication-free rates were similar: 92.7% in HCM (with no lead complications) versus 89.5% in non-HCM. There were 3 appropriate shocks for ventricular tachycardia in 3 HCM patients that were all converted by the first shock. Overall final shock conversion efficacy was 100% in HCM versus 98% in non-HCM (P = ns). Inappropriate shocks occurred in 12.5% of HCM patients and 10.3% of non-HCM patients (P = ns), being reduced by 47% using dual-zone programming. CONCLUSION: These initial data indicate the S-ICD is safe and effective in patients with HCM who are at high risk of ventricular arrhythmias and pass preimplantation electrocardiogram screening. Inappropriate shocks were mainly due to T-wave oversensing, but there were no lead complications requiring reintervention.

Longevity of the Subcutaneous Implantable Defibrillator: Long-Term Follow-Up of the European Regulatory Trial Cohort.

BACKGROUND: The recent advent of subcutaneous implantable cardioverter defibrillators (S-ICDs) has provided investigators with a safe and effective new therapy in patients at risk of sudden cardiac death. At present, no data are available with regard to the longevity of these new devices. This study evaluated the longevity of the S-ICD system. METHODS AND RESULTS: All patients enrolled in the European Regulatory Trial were included in the analysis. During follow-up, time and causes of device replacement or explantation were assessed and categorized. Device longevity was estimated using Kaplan-Meier analysis. Fifty-five patients were followed for a median of 5.8 years. During follow-up, 26 (47%) patients underwent device replacement and 5 (9%) underwent device explantation. Median time to replacement was 5.0 years (Q1-Q3, 4.4-5.6 years). Replacement was caused by battery depletion in 25 patients (92%), of which 5 within 1.5 years because of premature battery depletion, and by infection in 1 patient (2%). Replacement for a transvenous ICD system was required in 4 patients (7%) because of ineffective defibrillation in 1 (0.003 per patient-year), need for resynchronization therapy in 2 (0.01 per patient-year), and for antibradycardia pacing in 1 (0.003 per patient-year). At 5 years follow-up, 71% of devices were still in service. CONCLUSIONS: This study provides the first estimate of S-ICD system longevity since its introduction in clinical practice. Median longevity of the first generation S-ICD system was 5.0 years. The majority of devices were replaced because of battery depletion. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01117792.

Pediatric medical complexity algorithm: a new method to stratify children by medical complexity.

OBJECTIVES: The goal of this study was to develop an algorithm based on International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), codes for classifying children with chronic disease (CD) according to level of medical complexity and to assess the algorithm's sensitivity and specificity. METHODS: A retrospective observational study was conducted among 700 children insured by Washington State Medicaid with ≥1 Seattle Children's Hospital emergency department and/or inpatient encounter in 2010. The gold standard population included 350 children with complex chronic disease (C-CD), 100 with noncomplex chronic disease (NC-CD), and 250 without CD. An existing ICD-9-CM-based algorithm called the Chronic Disability Payment System was modified to develop a new algorithm called the Pediatric Medical Complexity Algorithm (PMCA). The sensitivity and specificity of PMCA were assessed. RESULTS: Using hospital discharge data, PMCA's sensitivity for correctly classifying children was 84% for C-CD, 41% for NC-CD, and 96% for those without CD. Using Medicaid claims data, PMCA's sensitivity was 89% for C-CD, 45% for NC-CD, and 80% for those without CD. Specificity was 90% to 92% in hospital discharge data and 85% to 91% in Medicaid claims data for all 3 groups. CONCLUSIONS: PMCA identified children with C-CD (who have accessed tertiary hospital care) with good sensitivity and good to excellent specificity when applied to hospital discharge or Medicaid claims data. PMCA may be useful for targeting resources such as care coordination to children with C-CD.

Community detection of long QT syndrome with a clinical registry: an alternative to ECG screening programs?

BACKGROUND: Long QT syndrome (LQTS) prevalence is estimated at 4 of 10,000 based on community electrocardiogram (ECG) screening, about which there is disagreement regarding efficacy, accuracy, cost-effectiveness, and practicality. Family studies of autosomal dominant conditions such as LQTS have revealed 8-9 gene-positive family members per proband. OBJECTIVE: To evaluate a cardiac/genetic registry and family screening program as a tool to identify LQTS in the community. METHODS: Possible LQTS probands were referred to the New Zealand Cardiac Inherited Disease service. The registry was first established in the northern region (population 2.03 million), including central Auckland (population 0.46 million). After clinical evaluation, genetic testing and family cascade screening were initiated. Genotype-positive individuals were classified as definite LQTS, and others were classified as definite or probable LQTS by clinical and ECG criteria. RESULTS: One hundred twelve probands were identified (presentation: 7 sudden death, 82 cardiac event, 16 ECG abnormality, and 7 sudden death of a family member). Following cascade screening, 309 patients with LQTS were identified (248 definite and 61 probable). Two hundred twenty patients had LQTS-causing mutations identified (120 [55%] LQT1, 78 [35%] LQT2, 19 [9%] LQT3, 1 [0.5%] LQT 5, and 2 [1%] LQT7). Thus far, an average of 2.1 definitely or probably affected family members have been identified per proband. The community detection rate is 1.5 of 10,000 for the whole region and 2.2 of 10,000 in Auckland. CONCLUSIONS: A high level of community detection of LQTS is possible using a clinical registry. With adequate resourcing, this has the potential to be an effective alternative to community ECG screening.

Subcutaneous chronic implantable defibrillation systems in humans.

The recent introduction of subcutaneous implantable cardioverter defibrillator (S-ICD) has raised attention about the potential of this technology for clinical use in daily clinical practice. We review the methods and results of the four studies conducted in humans for approval of this innovative technology for daily practice. Two studies using a temporary S-ICD system (acute human studies) were conducted to search for an appropriate lead configuration and energy requirements. For this purpose, 4 S-ICD configurations were tested in 78 patients at the time of transvenous (TV)-ICD implantation. The optimal configuration was tested in 49 more patients to comparatively assess the subcutaneous defibrillation threshold (S-DFT) versus the standard TV-ICD. Long-term implants were evaluated in 55 patients using an implanted system (chronic human study). The acute humans studies led to an optimal S-ICD configuration comprising a parasternal electrode and left anterolateral thoracic pulse generator. Both configurations successfully terminated 98% of induced ventricular fibrillation (VF), but significantly higher energy levels were required with S-ICD than with TV-ICD systems (36.6 ± 19.8 J vs. 11.1 ± 8.5 J). In the chronic study, all 137 VF episodes induced at time of implant were detected with a 98% conversion rate. Two pocket infections and four lead revisions were required during 10 ± 1 months of follow-up. During this period, survival was 98%, and 12 spontaneous ventricular tachyarrhythmias were detected and treated by the device. These data show that the S-ICD systems here consistently detected and converted VF induced at time of implant as well as sustained ventricular tachyarrhythmias occurring during follow-up (248).

First human demonstration of cardiac stimulation with transcutaneous ultrasound energy delivery: implications for wireless pacing with implantable devices.

OBJECTIVES: The purpose of this study was to evaluate the feasibility and safety of a novel technology that uses energy transfer from an ultrasound transmitter to achieve cardiac stimulation without the use of a pacing lead in humans. BACKGROUND: To overcome the limitations of pacemaker leads, a new technology enabling stimulation without the use of a lead is desirable. METHODS: A steerable bipolar electrophysiology catheter incorporating a receiver electrode into the tip and circuitry to convert ultrasound energy to electrical energy was inserted transvenously into the heart. An ultrasound transmitting transducer was placed on the chest wall with ultrasound gel. Ultrasound energy was amplitude-adjusted and transmitted at 313 to 385 kHz. The output waveform of the receiver electrode was monitored while the transmitter was moved on the chest wall to target the receiver. The ultrasound transmission amplitude was limited to a mechanical index of 1.9, the maximum allowed for ultrasound imaging systems. Ultrasound-mediated pacing with minimum voltage but consistent capture was obtained for 12 s. RESULTS: Twenty-four patients (48 +/- 12 years) were tested during or after completion of clinical electrophysiology procedures. A total of 80 pacing sites were tested (mean 3.3 sites/patient): 12 right atrial, 35 right ventricular, and 33 left ventricular (31 endocardial) sites. The transmit-to-receive distance was 11.3 +/- 3.2 cm (range 5.3 to 22.5 cm). Ultrasound-mediated pacing was achieved at all 80 test sites, with consistent capture at 77 sites. The mechanical index during pacing was 0.5 +/- 0.3 (range 0.1 to 1.5). The mean ultrasound-mediated capture threshold was 1.01 +/- 0.64 V. There was no adverse event related to ultrasound pacing. No patient experienced discomfort during pacing. CONCLUSIONS: The feasibility and safety of pacing usng ultrasound energy has been shown acutely.

Atrial pacing should be used more frequently in sinus node disease.

BACKGROUND: Single lead atrial (AAI(R)) pacing for sick sinus syndrome provides physiological pacing and is less expensive than a dual chamber system. Compared with ventricular-based pacing, it maintains the normal cardiac depolarization sequence, is associated with less atrial fibrillation and heart failure, and avoids pacemaker syndrome. We sought whether it is possible to select patients for AAI(R) pacing with a low likelihood of subsequent ventricular pacing, and whether this approach was cost-effective. METHODS: A retrospective review was conducted of AAI(R) pacemaker implantations. Patients requiring a further procedure for insertion of a ventricular lead (for DDD(R) or VVI(R) pacing) were compared with those who remained atrially paced. RESULTS: 2.7% (117 of 4,366) of implants were AAI(R), compared with less than 1% overall for New Zealand and Australia. During follow-up of 3.5 (2.3, 7.7) years, insertion of a ventricular lead was required in 14 (12%), and was more likely in those with pre-existing PR interval >0.20 seconds (odds ratio 7.8, P = 0.003) or left bundle branch block (LBBB, odds ratio 17, P = 0.037). Right bundle branch block, left anterior fascicular block, or history of paroxysmal atrial fibrillation were not more frequent in the group requiring ventricular pacing, and preimplantation Wenckebach point was not different. The most efficient strategy was initial AAIR implantation in all except those with LBBB or PR interval >0.20 seconds. Compared with routine DDDR implantation, cost was reduced by 20%, with a 1.4% annual incidence of further procedures. CONCLUSION: AAIR is the appropriate pacing choice for sick sinus syndrome without LBBB or PR interval >0.20 seconds.

Trimethoprim-induced aseptic meningitis in an adolescent male.

Drug-induced aseptic meningitis attributable to trimethoprim alone has only rarely been documented in the literature. A previously healthy adolescent male presented to our hospital with recurrent headaches, photophobia, and meningismus after serially starting and stopping trimethoprim. Cerebrospinal fluid studies revealed elevated white blood cell counts with a polymorphonuclear predominance. This case is the second documented report of trimethoprim-induced aseptic meningitis in a pediatric patient.

Cerebral embolism from atrial myxoma in pediatric patients.

We describe 2 children with cerebrovascular events caused by emboli from left atrial myxomas and review 7 other pediatric cases from the literature. Transient cutaneous eruptions involving the extremities preceded the cerebrovascular events and were most likely attributable to fragmentation of the atrial tumor with peripheral embolization. Our first case demonstrates the more common presentation with acute hemiplegia caused by cerebral infarction and the second case a transient ischemic attack manifested by more subtle features as a result of involvement of the vertebrobasilar circulation. Neither child had a history or other signs of cardiac disease. Atrial myxoma should be considered in the differential diagnosis when children present with neurologic symptoms or with signs of embolization, because surgical removal of the tumor is critical and may be curative.