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Biomedical subjects

Naomi J Kertesz

Publications and source records attributed to Naomi J Kertesz.

17 recordsLinked to original sources
PubMed2006

Atrial flutter in infants.

OBJECTIVES: We sought to characterize the clinical nature of atrial flutter (AFL) in a large cohort of infants. BACKGROUND: There are no large studies describing the natural history of AFL in infants. Previous studies vary in the therapy used and expected prognosis. METHODS: We reviewed the records of all children younger than 1 year of age who were diagnosed with AFL at our hospital during the past 25 years, excluding those with previous cardiac surgery. RESULTS: We identified 50 infants with AFL. Most, 36 (72%), presented within the first 48 h of life. Congestive heart failure was evident in 10 infants, with 6 presenting at 1 day of age, and 4 presenting beyond 1 month of age. The remainder were asymptomatic. A large atrial septal defect was the only structural heart disease. Spontaneous conversion to sinus rhythm occurred in 13 (26%) infants. Sinus rhythm was restored in 20 of 23 (87%) attempts at direct current cardioversion and 7 of 22 (32%) attempts at transesophegeal pacing; 7 required antiarrhythmic therapy. An additional arrhythmia, all supraventricular, appeared in 11 (22%) infants. The recurrence of AFL developed in 6 infants; 5 of 6 of these incidents occurred within 24 h of the first episode. All patients with recurrence had an additional arrhythmia. CONCLUSIONS: Infants with AFL usually present within the first 2 days of life. No association was found with structural heart disease. Direct current cardioversion appears to be most effective at establishing sinus rhythm. Chronic AFL has the potential to cause cardiovascular compromise. Atrial flutter in the absence of other arrhythmias has a low risk of recurrence. Once in sinus rhythm, infants with AFL have an excellent prognosis and may not require chronic antiarrhythmic therapy.

Anti-Arrhythmia Agents↗
PubMed2006

Outpatient continuous parenteral inotropic therapy as bridge to transplantation in children with advanced heart failure.

BACKGROUND: Advanced heart failure in children is associated with high morbidity and mortality and is often refractory to standard medical therapy. The purpose of this study was to review our institutional experience with the use of outpatient parenteral inotropic therapy (PIT) for advanced chronic heart failure in children. METHODS AND RESULTS: We reviewed the medical records of all patients treated with PIT as outpatients. Seven patients received outpatient PIT from 2/99 to 1/05 (mean age was 14.6 years +/- 3.7). Median duration of therapy was 10 weeks (range 4-84 weeks). The mean number of emergency department visits per patient was greater before starting PIT than after starting PIT (2.3 +/- 1.8 versus 1.1 +/- 2.2, P < .05). The mean number of hospital admissions from exacerbation of heart failure symptoms decreased after starting PIT (2.1 +/- 1.3 versus 0.6 +/- 0.8, P < .05). Mean EF% in patients with systolic dysfunction improved while on therapy (30 +/- 14% before versus 39 +/- 16% after, P < .05). There was 1 death and 5 complications in 2 patients. Six patients were successfully bridged to transplantation. CONCLUSION: Outpatient continuous parenteral inotropic therapy may serve as a successful bridge to cardiac transplantation in selected pediatric outpatients.

Adolescent↗
PubMed2006

Innovative techniques for placement of implantable cardioverter-defibrillator leads in patients with limited venous access to the heart.

BACKGROUND: Because of venous occlusion, intracardiac shunting, previous surgery, or small size placement of implantable cardioverter-defibrillator (ICD) leads may not be possible using traditional methods. The purpose of this study was to evaluate and describe innovative methods of placing ICD leads. METHODS: The records of all patients undergoing ICD implantation at our institution were reviewed to identify patients with nontraditional lead placement. Indications for ICD, method of lead and coil placement, defibrillation thresholds, complications, and follow-up results were reviewed retrospectively. RESULTS: Eight patients (aged 11 months to 29 years) were identified. Six patients with limited venous access to the heart (four extracardiac Fontan, one bidirectional Glenn, one 8 kg 11-month-old) underwent surgical placement of an ICD coil directly into the pericardial sac. A second bipolar lead was placed on the ventricle for sensing and pacing. Two patients with difficult venous access had a standard transvenous ICD lead inserted directly into the right atrium (transatrial approach) and then positioned into the ventricle. All patients had a defibrillation threshold of <20 J, although one patient required placement of a second coil due to an elevated threshold. There have been no complications and two successful appropriate ICD discharges at follow-up (median 22 months, range 5-42 months). CONCLUSIONS: Many factors may prohibit transvenous ICD lead placement. Nontraditional surgical placement of subcutaneous ICD leads on the pericardium or the use of a transatrial approach can be effective techniques in these patients. These procedures can be performed at low risk to the patient with excellent defibrillation thresholds.

Adolescent↗
PubMed2006

Therapeutic levels of intravenous procainamide in neonates: a retrospective assessment.

STUDY OBJECTIVES: To evaluate dosing and pharmacokinetic parameters of intravenous continuous-infusion procainamide in neonates, and to identify dosage regimens and factors leading to therapeutic procainamide levels and minimal adverse events. DESIGN: Retrospective, observational study. SETTING: Pediatric hospital. PATIENTS: . Twenty-one patients (seven preterm, 14 full term) younger than 30 days who received continuous-infusion procainamide therapy for more than 15 hours or had two consecutive therapeutic procainamide levels obtained while receiving therapy between June 1, 2002, and December 31, 2005. MEASUREMENTS AND MAIN RESULTS: Data on demographics, dosing, drug levels, and adverse effects were collected. Doses that achieved therapeutic levels were documented, and procainamide clearance was calculated and evaluated with regard to renal function and gestational age in patients who were at steady state. Mean clearance and mean N-acetylprocainamide (NAPA):procainamide ratios were compared between preterm and term neonates. No patients experienced hemodynamic instability or other adverse effects due to procainamide. Procainamide was given as a mean +/- SD 9.6 +/- 1.5-mg/kg bolus in 20 of 21 patients before continuous infusion. The mean +/- SD dose at which two therapeutic levels were achieved was 37.56 +/- 13.52 microg/kg/minute. Procainamide clearance was 6.36 +/- 8.85 ml/kg/minute and correlated with creatinine clearance (r=0.78, p<0.00001) and age at day of sampling (r=0.49, p<0.00001). The NAPA:procainamide ratio at steady state was 0.84 +/- 0.53; two patients were determined to be fast acetylators (ratio > 1). Preterm infants had lower mean clearance rates (p<0.001) but higher NAPA:procainamide ratios (p<0.01) than those of term infants. Five patients experienced seven supratherapeutic levels while receiving therapy; four of these patients were preterm, and all had creatinine clearances less than 30 ml/minute/1.73 m(2). Three patients had four pairs of levels obtained after discontinuation of procainamide, and elimination rate constant and half-life were calculated. CONCLUSION: Procainamide can be safely used in neonates, with no short-term adverse effects. The dosage regimen for intravenous procainamide required to achieve therapeutic levels in neonates is similar to that of older infants and children. Doses may need to be reduced in premature infants and in those with renal dysfunction.

Acecainide↗
PubMed2005

Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study.

OBJECTIVES: Our objective was to evaluate the short-term safety and efficacy of cardiac resynchronization therapy (CRT) in children. BACKGROUND: Cardiac resynchronization therapy has been beneficial for adult patients with poor left ventricular function and intraventricular conduction delay. The efficacy of this therapy in the young and in those with congenital heart disease (CHD) has not yet been established. METHODS: This is a multi-center, retrospective evaluation of CRT in 103 patients from 22 institutions. RESULTS: Median age at time of implantation was 12.8 years (3 months to 55.4 years). Median duration of follow-up was four months (22 days to 1 year). The diagnosis was CHD in 73 patients (71%), cardiomyopathy in 16 (16%), and congenital complete atrioventricular block in 14 (13%). The QRS duration before pacing was 166.1 +/- 33.3 ms, which decreased after CRT by 37.7 +/- 30.7 ms (p < 0.01). Pre-CRT systemic ventricular ejection fraction (EF) was 26.2 +/- 11.6%. The EF increased by 12.8 +/- 12.7 EF units with a mean EF after CRT of 39.9 +/- 14.8% (p < 0.05). Of 18 patients who underwent CRT while listed for heart transplantation, 3 improved sufficiently to allow removal from the transplant waiting list, 5 underwent transplant, 2 died, and 8 others are currently awaiting transplant. CONCLUSIONS: Cardiac resynchronization therapy appears to offer benefit in pediatric and CHD patients who differ substantially from the adult populations in whom this therapy has been most thoroughly evaluated to date. Further studies looking at the long-term benefit of this therapy in this population are needed.

Adolescent↗
PubMed2005

Danon disease as an underrecognized cause of hypertrophic cardiomyopathy in children.

BACKGROUND: Some patients with hypertrophic cardiomyopathy (HCM) or left ventricular hypertrophy also present with skeletal myopathy and Wolff-Parkinson-White (WPW) syndrome; mutations in the gene encoding the lysosome-associated protein-2 (LAMP-2) have been identified in these patients, suggesting that some of these patients have Danon disease. In this study we investigated the frequency of LAMP2 mutations in an unselected pediatric HCM population. METHODS AND RESULTS: LAMP2 was amplified from genomic DNA isolated from peripheral lymphocytes of 50 patients diagnosed with HCM and analyzed by direct DNA sequencing. In 2 of the 50 probands (4%), nonsense mutations were identified. In 1 family the proband initially presented with HCM as a teenager, which progressed to dilated cardiomyopathy (DCM) and heart failure. Skeletal myopathy and WPW were also noted. The teenage sister of the proband is a carrier of the same LAMP2 mutation and has HCM without skeletal myopathy or WPW. The other proband presented with HCM, WPW, and skeletal myopathy as a teenager, whereas his carrier mother developed DCM during her 40s. Skeletal and cardiac muscle sections revealed the absence of LAMP-2 on immunohistochemical staining. CONCLUSIONS: LAMP2 mutations may account for a significant proportion of cases of HCM in children, especially when skeletal myopathy and/or WPW is present, suggesting that Danon disease is an underrecognized entity in the pediatric cardiology community.

Adolescent↗
PubMed2005

Radio-frequency catheter ablation of accessory pathway mediated tachycardia in a child after orthotopic heart transplantation.

Successful radio-frequency catheter ablation of accessory pathway mediated supraventricular tachycardia in the transplanted heart has been reported sporadically in adults, but has not been reported in children. This is the first report of a child having received a donor heart with manifest pre-excitation and who subsequently underwent successful radio-frequency ablation of the accessory pathway.

Adolescent↗
PubMed2005

Atrioventricular junction ablation and pacemaker therapy in patients with drug-resistant atrial tachyarrhythmias after the Fontan operation.

INTRODUCTION: Drug-resistant intraatrial reentrant tachycardia (IART) occurs frequently after the Fontan operation and is a major cause of morbidity and rarely mortality. We describe our experience with AV junction ablation after pacemaker implantation in postoperative Fontan patients with drug-resistant IART. METHODS AND RESULTS: We performed retrospective analysis of Fontan patients with IART and attempted radiofrequency ablation (RFA) of the AV junction. Seven patients (6 male) were identified, with a mean age at Fontan of 9.3 years (range 5.8-13.3) and a median age at RFA of 18 years (range 14.5-23.3). Mean follow-up prior to RFA was 764 +/- 235 days and after RFA 1,541 +/- 1,235 days. IART was refractory to antiarrhythmic drugs in all patients, and all had undergone pacemaker placement. Mean onset of IART was 44.1 months (range 0-142) after Fontan. Mean duration of atrial arrhythmia prior to RFA was 72 +/- 48 m (range 16-148). Ablation of the AV junction was successful or partially successful in all patients. Complete AV block occurred in 6 patients. Normal AV conduction was not seen during a mean follow-up of 1,541 days. The mean number of antiarrhythmic medications decreased from 2.8 +/- 1.5 to 0.7 +/- 0.8 (P <0.05). CONCLUSION: In Fontan patients with drug-resistant IART, RFA of the AV junction with prior pacemaker implant is an effective therapeutic option. Despite the introduction of pacemaker dependence, this option should be considered in patients who did not respond to RFA of IART or who are at high operative risk for Fontan conversion.

Adolescent↗
PubMed2005

Mechanism of induction of atrioventricular node reentry by simultaneous anterograde conduction over the fast and slow pathways.

INTRODUCTION: AV node reentry (AVNRT) is typically induced with anterograde (Ant) block over the fast pathway (FP) and conduction over the slow pathway (SP), with subsequent retrograde (Ret) conduction over the FP. Rarely, a premature atrial complex (PAC) conducts simultaneously over the FP and SP to induce AVNRT (2 for 1). This study investigates the mechanism of 2 for 1 induction. METHODS AND RESULTS: Of 192 consecutive patients (pts) undergoing posteroseptal radiofrequency ablation to treat AVNRT, 4 pts (2%) had 2 for 1 AVNRT induction. All needed isoproterenol for AVNRT initiation, and Ant conduction was over the SP during AVNRT. Controls (n = 15) were randomly selected from the remaining 188 pts and required isoproterenol to induce AVNRT with Ant block over the FP. For 2 for 1 versus control, respectively, there was no difference in mean age (55 vs. 46 yr), AVNRT cycle length (420 vs. 320 ms), or the Ant effective refractory period of the FP (320 vs. 344 ms). Of note, the PAC that induced AVNRT had a significantly longer AH interval over the SP in pts with 2 for 1 versus control (470 vs. 320 ms, P = 0.016), even though the A1A2 interval for induction was longer for 2 for 1 (315 vs. 260 ms, P = 0.003). Ret conduction over the SP was relatively poor in the 2 for 1 group as evidenced by 4/4 pts with induction of AVNRT during incremental ventricular pacing versus only 1/15 control pts (P < 0.001). CONCLUSION: The unique induction of AVNRT by a PAC with simultaneous conduction over the FP and SP is best explained by minimal to no retrograde invasion of the SP from the anterogradely conducted fast pathway impulse, and consistent with this observation is the initiation of slow/fast AVN reentry during incremental RV pacing.

Adult↗
PubMed2004

Clinical course of atrial ectopic tachycardia is age-dependent: results and treatment in children < 3 or > or =3 years of age.

OBJECTIVES: We assessed the clinical presentation, natural history, and treatment response of atrial ectopic tachycardia (AET) in children <3 years of age (group 1) compared with those > or =3 years of age (group 2). BACKGROUND: Atrial ectopic tachycardia is a common cause of chronic supraventricular tachycardia in children and can be resistant to pharmacologic therapy. Radiofrequency ablation (RFA) can eliminate AET arising from a single focus. METHODS: A retrospective review identified all children at Texas Children's Hospital diagnosed with AET from March 1991 to November 2000. Data obtained included clinical presentation, echocardiographic evaluation, response to antiarrhythmic therapy, spontaneous resolution, and outcomes of radiofrequency and surgical ablation. RESULTS: Sixty-eight children were identified (22 children <3 years and 46 children > or =3 years of age). Control of AET with antiarrhythmic therapy was achieved in 91% of the younger children but only 37% of the older children (p < 0.001). There was a higher rate of spontaneous resolution in the younger group (78%) compared with the older group (16%) (p < 0.001). Radiofrequency ablation was performed in 35 of the older children, with ultimate success in 74%. Surgical intervention was required for six children. CONCLUSIONS: Younger children respond to antiarrhythmic therapy and have a high incidence of AET resolution, thus warranting a trial of antiarrhythmic therapy. In children > or =3 years, AET is unlikely to resolve spontaneously, and antiarrhythmic medications are frequently ineffective. Thus, RFA should be considered early in the course of treatment for these children; however, surgical intervention may be necessary.

Adolescent↗
PubMed2004

Late pacemaker requirement after pediatric orthotopic heart transplantation may predict the presence of transplant coronary artery disease.

BACKGROUND: Few data are available regarding pacemaker implantation after pediatric orthotopic heart transplantation. The purpose of this study was to assess the incidence, indications and associations with regard to pacemaker placement in children who have undergone orthotopic heart transplantation. METHODS: We performed a retrospective study of all patients undergoing orthotopic heart transplantation at our institution from October 1984 to March 2001. Data obtained included demographics, indications for pacemaker, presence of transplant coronary artery disease and long-term follow-up. Patients were divided into: Group 1, patients requiring a pacemaker within 3 months of transplantation; and Group 2, patients requiring a pacemaker beyond 3 months. RESULTS: Pacemakers were required in 7 of 106 (6.6%) transplant recipients. Pacing indications for patients in Group 1 (n = 2) were persistent bradycardia with pause-related ventricular arrhythmia and atrial flutter with resultant sinus pauses of up to 4 seconds. In Group 2 patients (n = 5), indications for pacing were high-grade atrioventricular (AV) block in 1 patient and episodic sinus pauses up to 3.3 seconds associated with syncope/dizziness in the remaining 4 patients. All patients in Group 2 had transplant coronary disease diagnosed within 1 year of pacemaker implantation. All had resolution of symptoms and no complications after implantation. CONCLUSIONS: Pacemakers are infrequently required after cardiac transplantation in children. Despite not meeting classic symptomatic sinus bradycardia criteria, pacemaker placement should be considered post-transplantation in patients with episodic sinus pauses and dizziness or syncope. Patients who present with the aforementioned symptoms or high-grade AV block should be evaluated closely for the presence or development of transplant coronary artery disease, as it may be their first symptom.

Bradycardia↗
PubMed2003

Frequency of recurrence among infants with supraventricular tachycardia and comparison of recurrence rates among those with and without preexcitation and among those with and without response to digoxin and/or propranolol therapy.

Approximately 60% of children with supraventricular tachycardia (SVT) develop their initial episode by 1 year of age. Despite resolution in most of these patients by 1 year, approximately 30% of the SVT will recur. We performed a retrospective review of all patients <1 year of age with SVT between January 1984 and December 2000. Recurrence was defined as documented SVT at >1 year of age. Patients were divided into: (1) a first line (FL) group (controlled with digoxin and/or propranolol) and (2) a second line (SL) group (requiring additional antiarrhythmics). The groups were divided based on the presence of preexcitation. The FL group included 116 patients, 20 of whom (17%) had Wolff-Parkinson-White (WPW) syndrome. The SL group included 34 patients, 21 of whom (62%) had WPW (p <0.001). Recurrence of SVT occurred in 32 patients (28%) in the FL group and in 23 patients (68%) in the SL group (p <0.001). SVT recurred in 36 of 41 patients (88%) with WPW compared with 19 of 109 patients (17%) without WPW syndrome (p <0.001). Logistic regression analysis demonstrated that the presence of WPW syndrome was associated with a 29-fold higher odds of SVT recurrence (p <0.001), and that patients with WPW syndrome were more likely to require additional antiarrhythmic therapy (p <0.001). Thus, patients with WPW syndrome who had SVT at <1 year of age have 29-fold higher odds of recurrence at >1 year of age versus those patients with preexcitation. These patients are also more likely to require additional antiarrhythmic therapy to control SVT. Furthermore, children with WPW syndrome who are refractory to treatment with digoxin and/or propranolol are at increased risk of SVT recurrence.

Age of Onset↗
PubMed2003

The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation.

BACKGROUND: This multicenter study evaluated experience with implantable cardioverter defibrillators (ICD) as a bridge to orthotopic heart transplantation (OHT) in children. METHODS: The application of ICD therapy continues to expand in pediatric populations, due in part to improved technology and new indications, including the prevention of sudden death while awaiting OHT. METHODS: We performed a retrospective review of ICD databases at 9 pediatric transplant centers. RESULTS: Twenty-eight patients (16 males) underwent implantation or had a preexisting ICD while awaiting OHT between 1990 and 2002. The median age at implant was 14.3 years (11 months to 21 years) with a median weight of 49 kg (11.7-88 kg). Diagnoses included cardiomyopathy (n=22), and congenital heart disease (n=6). Indications for ICD implantation included ventricular tachycardia/fibrillation (n=23), syncope (n=5), aborted sudden death with no documentation of rhythm disturbance (n=5), ventricular ectopy (n=1), and poor function (n=5). Of the 28 ICDs, 23 were implanted by a transvenous approach and 5 by epicardial route. There were 55 defibrillator discharges in 17 patients, 47 (85%) of which (in 13 patients) were appropriate. The 8 inappropriate discharges (in 6 patients) were triggered by sinus tachycardia, inappropriate sensing, and atrial flutter. The mean time from implantation to first appropriate shock was 6.9 months (1 day to 2.6 years). Twenty-one patients underwent transplantation during the study period, whereas 2 died while awaiting a donor. Morbidity included a lead fracture, 3 episodes of electromechanical dissociation, and 1 episode of electrical storm. CONCLUSIONS: ICD implantation represents an effective bridge to transplantation in pediatric patients. The complication rate is low, with inappropriate device discharge due primarily to sinus tachycardia or atrial flutter. There is a high incidence of appropriate ICD therapy for malignant ventricular arrhythmias in this highly selected group of patients.

Adolescent↗
PubMed2002

Flecainide and sotalol: a new combination therapy for refractory supraventricular tachycardia in children <1 year of age.

OBJECTIVES: The goal of this study was to assess the efficacy and safety of the combination therapy of flecainide and sotalol for the treatment of refractory supraventricular tachycardia (SVT) in children <1 year of age. BACKGROUND: Supraventricular tachycardia in infants can be refractory to single-drug as well as standard combination medical therapy. Radiofrequency ablation (RFA) is the definitive treatment of refractory SVT; however, interventional therapy poses a high risk of morbidity and mortality in this age group. METHODS: A retrospective review was performed identifying infants who required flecainide and sotalol to control refractory SVT. Patient age, previous drug therapy, duration of treatment, flecainide levels and corrected QT intervals were recorded; 24 h Holter monitoring was utilized to gauge efficacy of treatment. Efficacy was defined as suppression of SVT to no more than rare nonsustained episodes or slowing of SVT to a clinically tolerable rate. RESULTS: Ten patients (median age: 29 days, range: 1 to 241 days) failed at least two antiarrhythmic agents including either flecainide or sotalol as single agents before initiating combination therapy. Efficacy was achieved in all patients. The failure rate for therapy was reduced from 100% to 0% (95% confidence interval: 0% to 26%). The median doses used were: flecainide 100 mg/m(2)/day (range: 40 to 150 mg/m(2)/day) and sotalol 175 mg/m(2)/day (range: 100 to 250 mg/m(2)/day). Median duration of therapy was 16 months (range: 5 to 35 months). No proarrhythmia occurred. CONCLUSIONS: The combination of flecainide and sotalol can safely and effectively control refractory SVT and may obviate the need for RFA in children <1 year.

Anti-Arrhythmia Agents↗
PubMed2002

Circadian and seasonal variation of malignant arrhythmias in a pediatric and congenital heart disease population.

INTRODUCTION: Recent studies in adult populations have revealed seasonal variation in the frequency of acute cardiovascular events, including life-threatening arrhythmias, demonstrating increased events during winter and early spring. Trends in the time of day that arrhythmias occur also were noted. We sought to establish whether pediatric and young adult congenital heart disease implantable cardioverter defibrillator (ICD) recipients have circadian or seasonal variability in shock frequency, similar to adult populations. METHODS AND RESULTS: Data from ICD patients at six pediatric centers in North America were analyzed to assess the timing of life-threatening arrhythmias. The populations consisted of children and adults with congenital heart disease and ICDs placed for malignant arrhythmias. Data were considered in 46 patients who received appropriate therapy (total 139 episodes) for ventricular tachycardia or ventricular fibrillation. Multiple variables were analyzed, including time of day, day of week, and month of year. In contrast to previously studied adult patients, fewer events occurred in the early morning (7.5%), with the most therapies occurring between 6 P.M. and midnight (35%). An increased frequency of therapies was observed in the fall and winter (September-January), representing 60% of all appropriate shocks. Unlike adult populations, Mondays did not have an increased frequency of malignant arrhythmias. CONCLUSION: Pediatric and adult congenital heart disease populations have moderate seasonal and 24-hour variation in ICD event rate, with some distinctly different peaks than those seen in typical adult ICD populations. These findings suggest circadian variation in arrhythmia vulnerability that may differ from conventional occupational, physical, or emotional stressors. (J Cardiovasc Electrophysiol, Vol. 13, pp.

Adolescent↗