Effects of dual-chamber pacing for pediatric patients with hypertrophic obstructive cardiomyopathy.

OBJECTIVES: The effects of both temporary and permanent dual-chamber pacing (DCP) were evaluated in symptomatic pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM) unresponsive to medications. BACKGROUND: Permanent DCP pacing can reduce left ventricular outflow tract (LVOT) gradient and relieve symptoms in adult patients with HOCM. METHODS: Ten patients (mean [+/-SD] age 11.1 +/- 6 years, range 1 to 17.5) with HOCM and a Doppler LVOT gradient > or = 40 mm Hg were studied. The seven patients showing hemodynamic improvement during temporary pacing at cardiac catheterization underwent surgical implantation of a permanent DCP system. The effects of permanent pacing were evaluated using a questionnaire, Doppler evaluation, treadmill testing and repeat cardiac catheterization. RESULTS: At initial cardiac catheterization, three patients failed to respond to temporary pacing (inadequate pace capture in two; congenital mitral valve abnormality in one). The remaining seven patients (70%, 95% confidence interval 38.0% to 91.7%, mean age 13 +/- years, range 4 to 17.5) showed a significant reduction (p < 0.05) in LVOT gradient, left ventricular systolic pressure and pulmonary capillary wedge pressure. After pacemaker implantation, these seven patients reported a significant reduction in dyspnea on exertion and exercise intolerance. Serial Doppler evaluation showed a significant reduction in LVOT gradient. Follow-up catheterization at 23 +/- 4 months in six patients (one patient declined restudy) showed a persistent decrease in LVOT gradient (53 +/- 13 vs. 16 +/- 11 mm Hg), left ventricular systolic pressure (149 +/- 16 vs. 108 +/- 14 mm Hg) and pulmonary capillary wedge pressure (18 +/- 2 vs. 12 +/- 4 mm Hg) versus preimplantation values. CONCLUSIONS: Permanent DCP is an effective therapy for selected pediatric patients with HOCM. Rapid atrial rates and intrinsic atrioventricular conduction, as well as congenital mitral valve abnormalities, may preclude effective pacing in certain patients.

Catheter ablation of accessory atrioventricular pathways in young patients: use of long vascular sheaths, the transseptal approach and a retrograde left posterior parallel approach.

OBJECTIVES: This study retrospectively assesses the technical aspects of the catheter techniques used to ablate 83 accessory atrioventricular (AV) pathways during 88 procedures in 71 pediatric and adult patients (median age 14 years, range 1 month to 55 years). A number of catheter approaches and techniques evolved that may have improved success and shortened procedure times. BACKGROUND: Radiofrequency catheter ablation of accessory AV pathways can be highly successful. However, the technical difficulty of many of the procedures is masked by the success rate. METHODS: Left free wall, right free wall and septal accessory pathways were ablated with a variety of approaches. RESULTS: Left free wall pathways were ablated successfully by using a standard retrograde approach through the aortic valve in only 10 (24%) of 43 cases. The remaining 33 (76%) required an approach that was either retrograde through the mitral valve (2 of 33), transseptal (21 of 33) or retrograde where the catheter was advanced behind the posterior mitral leaflet at the point of mitral-aortic continuity, so that the catheter course was parallel rather than perpendicular to the mitral anulus (10 of 33). Nineteen of 20 septal pathways were ablated successfully by using either the parallel approach (2 of 29), a transseptal approach (2 of 19), ablation within the coronary sinus or one of its veins (8 of 19) or ablation on the atrial side of the tricuspid valve (7 of 19). Fifteen of 20 right free wall pathways were ablated successfully with a variety of approaches on both the atrial and the ventricular side of the tricuspid valve. Long vascular sheaths were judged to contribute directly to success in 33 (43%) of 77 pathways. The overall success rate has been 93% (77 of 83 pathways), with 100% success for left free wall (43 of 43), 75% for right free wall (15 of 20) and 95% for septal pathways (19 of 20). CONCLUSIONS: Thus, successful ablation of accessory AV pathways in a mixed group of pediatric and adult patients appears to benefit from a wide range of vascular and catheter approaches.

Pediatric use of intravenous amiodarone: efficacy and safety in critically ill patients from a multicenter protocol.

OBJECTIVE: The purpose of this study was to analyze the efficacy and safety of intravenous amiodarone in young patients with critical, drug-resistant arrhythmias. BACKGROUND: Intravenous amiodarone has been investigated in adults since the early 1980s. Experience with the drug in young patients is limited. A larger pediatric study group was necessary to provide responsible guidelines for the drug's use before its market release. METHODS: Eight centers obtained institutional approval of a standardized protocol. Other centers were approved on a compassionate use basis after contacting the primary investigator (J.C.P). RESULTS: Forty patients were enrolled. Standard management in all failed. Many patients had early postoperative tachyarrhythmias (25 of 40), with early successful treatment in 21 (84%) of 25. Twelve patients had ventricular tachyarrhythmias: seven had successful therapy, and six died, none related to the drug. Eleven patients had atrial tachyarrhythmias: 10 of 11 had immediate success, but 3 later died. Fourteen patients had junctional ectopic tachycardia, which was treated with success (sinus rhythm or slowing, allowing pacing) in 13 of 14, with no deaths. Three other patients had supraventricular tachycardias, with success in two and no deaths. The average loading dose was 6.3 mg/kg body weight, and 50% of patients required a continuous infusion. Four patients had mild hypotension during the amiodarone bolus. One postoperative patient experienced bradycardia requiring temporary pacing. There were no proarrhythmic effects. Deaths (9 [23%] of 40) were not attributed to amiodarone. CONCLUSIONS: Intravenous amiodarone is safe and effective in most young patients with critical tachyarrhythmia. Intravenous amiodarone can be lifesaving, particularly for postoperative junctional ectopic tachycardia, when standard therapy is ineffective.

Evaluation of recurrent pediatric syncope: role of tilt table testing.

OBJECTIVE: To determine the current practice and effectiveness of evaluating recurrent syncope in pediatric patients, and to establish the role of tilt table testing in the evaluation. DESIGN: Retrospective analysis of 54 pediatric patients with the history of syncope referred to cardiologists. Group I consisted of 27 patients examined without tilt table testing; group II consisted of 27 patients whose examination included tilt table testing. RESULTS: Group I had an average of 5.4 studies and group II, 6.6 studies performed per patient. Studies included chest radiograph (16 vs 13), electrocardiogram (24 vs 27), echocardiography (21 vs 27), 24-hour electrocardiogram (14 vs 16), transtelephonic monitor (7 vs 8), electrophysiology study (1 vs 3), complete blood cell counts (11 vs 12), chemistries (10 vs 11), thyroid function test (3 vs 3), neurology consult (12 vs 6), electroencephalogram (12 vs 5), and head computed tomographic scan (5 vs 3). Of the 298 non-tilt studies, the results of only 5 (1.6%) were abnormal. Diagnoses were made in 5 (18.5%) of 27 group I patients (Wolff-Parkinson-White syndrome, 1; conversion reaction, 2; hyperventilation, 1; migraines, 1), whereas diagnosis was made in 27 (100%) of 27 group II patients (neurocardiogenic syncope, 25; conversion reaction, 2). CONCLUSION: An extensive workup is not routinely indicated in syncopal patients with a history consistent with neurocardiogenic syncope. Tilt table testing performed early in the evaluation will increase the probability of a diagnosis, and will often prevent the need for further extensive, expensive anxiety-producing tests.

Variable location of accessory pathways associated with the permanent form of junctional reciprocating tachycardia and confirmation with radiofrequency ablation.

Permanent junctional reciprocating tachycardia (PJRT) occurs primarily in young patients and causes nearly incessant tachycardia that is frequently refractory to pharmacologic treatment. Previous nonpharmacologic therapy has included surgical or direct-current catheter ablation of either the His bundle or the accessory pathway. The accessory pathway in PJRT has been described as having retrograde and anterograde decremental conduction properties, and is typically identified in the posteroseptal location. This report describes radiofrequency catheter ablation of accessory pathways in 8 patients with PJRT. All ablations were successful and without adverse effects. Accessory pathway potentials were detected just before atrial activation in 6 of 8 patients. A new finding was that 5 of the 8 pathway locations, as identified by the site of successful ablation, were not in the typical posteroseptal region. In 1 patient it was located in the right posteroseptal region, 2 were in the right atrial freewall, 1 was in the right anterior septum and 1 was in the left posterior region just outside of the septal region. In conclusion, radiofrequency catheter ablation can be a highly effective and safe method for treatment of young patients with PJRT. Because the accessory pathways can be located outside of the posteroseptal region, careful mapping of both the right and left atrioventricular groove may be necessary for successful ablation.

Tilt table evaluation for control pediatric patients: comparison with symptomatic patients.

This study was designed to evaluate pediatric control patients during head-up tilt in comparison with symptomatic neurocardiogenic syncope patient head-up tilt responses. Twenty-three pediatric control (c) patients (13 females, 10 males; 11.9 +/- 3.1 years) were tested with head-up tilt (HUT) and compared with 66 symptomatic (s) patients. Baseline drug-free HUT (cHUT-1), a second drug-free HUT (cHUT-2), and a final HUT with isoproterenol infusion (cHUT-3) were each performed at 80 degrees tilt angle for 30 min or until positive. For comparison, 66 symptomatic patients (41 females, 25 males; 13.6 +/- 2.5 years) underwent drug-free HUT (sHUT-1); negative responders during sHUT-1 underwent follow-up HUT with isoproterenol (sHUT-2). HUT data were compared for both groups at both 30 and 20 min tilt duration. Twelve control patients (52%) had a symptomatic response during cHUT-1 at 18 +/- 8 min. During cHUT-2, 5 of 23 patients were positive at 13 +/- 5 min; each had previously tested positive during cHUT-1. Two patients, each positive in cHUT-1 and cHUT-2, refused cHUT-3. The only patient testing positive during cHUT-3 was test positive in cHUT-1 but negative for cHUT-2. In comparison, 43 of 66 (65%) symptomatic patients tested positive during drug-free sHUT-1 at 11 +/- 6 min. Subsequently, 20 of the 23 negative patients underwent HUT with isoproterenol (sHUT-2), with 8 of 20 testing positive. Thus, 51 of 66 symptomatic patients (77%) were called "true positives."(ABSTRACT TRUNCATED AT 250 WORDS)

Factors influencing impedance during radiofrequency ablation in humans.

Impedance during radiofrequency (RF) catheter ablation procedures is dependent on a variety of parameters related to the catheter, cabling, reference patch, body size, and temperature. To examine the influence of body size, impedance was measured during clinical ablation procedures in 93 patients (Group I) with a wide range of body sizes. In 14 other patients (Group II), impedance was measured during variations in catheter tip size (5, 6 and 7 Fr), reference patch size (120 and 60 cm2), patch location (chest vs. thigh), and catheter tip tissue contact. The average impedance was also compared to average tip temperature in Group II patients. Impedance decreased with increasing catheter tip size, reference patch size and proximity of the patch to the heart. However, the effects of body geometry were complex. For example, using a chest patch, impedance increased with body surface area, but using a thigh patch it decreased, suggesting that lung volume may increase impedance, but body width may actually decrease it. An increase in tip tissue contact, relative to blood contact, increased the impedance, suggesting that impedance may be a useful measure of tip tissue contact. Finally impedance decreased with increasing tip temperature, suggesting that impedance may be useful as a real time measure of tissue and blood heating. The results are interpreted in terms of an electrical analog which suggests further that despite the lower total power when the same voltage is applied to a higher impedance, less voltage should be applied to achieve the same tissue effect when the measured impedance is higher.

Late enlargement of radiofrequency lesions in infant lambs. Implications for ablation procedures in small children.

BACKGROUND: Despite the current clinical use of radiofrequency (RF) catheter ablation in infants, the acute and late effects of RF lesion production in immature myocardium remain unknown. This study was specifically designed to investigate the pathology of RF lesions in developing sheep myocardium. METHODS AND RESULTS: In study 1, RF lesions were made on the epicardial left ventricular surface of the beating heart in 15 sheep, 5 approximately 4 weeks of age (11.0 +/- 1.0 kg) and 10 approximately 8 weeks of age (23.8 +/- 3.4 kg), to assess the effects of RF application duration (10 to 90 seconds) and electrode tip temperature (45 degrees to 90 degrees C) on lesion size in immature myocardium. Lesion width and depth increased asymptotically with RF duration, to 7.0 +/- 0.7 and 4.8 +/- 1.0 mm at 90 seconds, respectively. The time to reach one-half lesion size was 6.5 seconds for width and 12.0 seconds for depth. Lesion width increased nearly linearly with tip temperature above 50 degrees C, but depth followed a sigmoid relation, with no increase above 80 degrees C. In study 2, RF lesions were made in all four cardiac chambers under fluoroscopic guidance in 19 infant sheep (10.9 +/- 1.4 kg). Lesion sizes and histological characteristics were assessed acutely (acute, n = 5), at 1.07 +/- 0.02 months (1 month, n = 5), and at 8.5 +/- 0.5 months (late, n = 9). Atrial and ventricular lesions but not atrioventricular groove lesions apparently increased in size during the follow-up period. Atrial lesions width increased from 5.3 +/- 0.5 to 8.7 +/- 0.7 mm at 1 month (164%) but did not increase further at late follow-up, while ventricular lesion width increased from 5.9 +/- 0.8 to 10.1 +/- 0.7 mm (171%) at late follow-up but was not significantly changed at 1 month. Histological evaluation revealed replacement of normal myocytes with fibrous and elastic tissue at 1 month and late follow-up in all locations but also demonstrated a poorly delineated border with multiple extensions of fibrous and elastic tissue into surrounding normal myocardium in late ventricular lesions. CONCLUSIONS: RF lesion formation in immature sheep myocardium is similar to that in adult myocardium acutely but is associated with late lesion enlargement and fibrous tissue invasion of normal myocardium. These findings may have implications for clinical RF ablation procedures in infants.

Cardiovascular effects of cocaine in neonates exposed prenatally.

This blinded cross-sectional study was to determine whether chronic cocaine exposure in utero produces abnormalities in left ventricular function (shortening fraction), heart rate, rhythm, and conduction in term neonates. Three groups of neonates were evaluated by two-dimensional echo Doppler and 24 hour Holter monitor, with studies initiated in the first 24 hours of life. Group A (n = 32) neonates had a positive history of chronic maternal cocaine use in pregnancy (MCU+) and a positive neonatal urine cocaine test (NUC+). Group B (n = 23) neonates were MCU+ but NUC-. Group C (n = 32) neonates were MCU- and NUC-. Measured parameters were compared statistically by analysis of variance. p < 0.05 was regarded as significant. Echocardiography showed no significant difference between groups A, B, and C for left ventricular shortening fraction. Holter monitor likewise revealed no significant difference between groups in minimal, maximal, and average heart rate, or in the incidence of supraventricular and ventricular arrhythmias greater than 20 beats/h in the 24-hour period. None of the patients had atrioventricular or bundle branch block. It is possible that the developmental state of the newborn heart makes it less responsive to the adverse effects of cocaine.

Transcatheter ablation of ectopic atrial tachycardia in young patients using radiofrequency current.

BACKGROUND: Ectopic atrial tachycardia (EAT) is a reversible cause of cardiomyopathy but may be quite difficult to control with conventional therapy. Transcatheter ablation with radiofrequency current was tested as an alternative to medical or surgical treatment of this condition. METHODS AND RESULTS: Twelve young patients (aged 10 months to 19 years) with drug-resistant EAT were treated with direct transcatheter ablation of the ectopic focus using radiofrequency (RF) energy. All had depressed left ventricular contractility by echocardiographic criteria, involving shortening fractions of 10-26% (median, 20%; normal, 28-35%). The EAT was mapped to the left atrium in seven cases and to the right atrium in five. Local atrial activation at the ectopic site preceded the onset of the surface P wave by 20-60 msec (median, 42 msec). Tachycardia terminated 0.5-13.0 seconds (median, 2.0 seconds) into a successful RF application. The ablation effectively eliminated EAT in 11 of 12 patients (92%), all of whom were discharged in sinus rhythm without medications after a median hospital stay of 48 hours. Ablation was unsuccessful in one patient with diffuse dysplasia of the anterior right atrium, who eventually did well after surgical resection of abnormal atrial tissue. Transient depression of sinus node function was noted in one patient who had successful ablation of an EAT focus in close proximity to the sinus node, although normal sinus node function returned within 72 hours. No other complications were encountered. During follow-up (3-21 months; median, 13 months), one patient had recurrence of a slower and less-sustained EAT that was successfully eliminated at a second ablation session. All others remained in sinus rhythm, and all 12 subjects recovered normal ventricular function. CONCLUSIONS: RF ablation appears to be a safe and effective therapeutic option for drug-resistant ectopic atrial tachycardia and may be the preferred first-line therapy for those patients with depressed ventricular function.