Transvenous Versus Epicardial Pacing in Fontan Patients.

Up to 10% of Fontan patients require pacemakers; an epicardial approach has historically been used. A transvenous approach can be used but carries risk of Fontan obstruction, thromboembolism, and can be technically challenging. The safety and efficacy of these approaches is not well described. The aim of this study was to compare epicardial and transvenous pacemaker outcomes in Fontan patients, specifically, device performance and adverse event rate. A retrospective review was performed on Fontan pacemaker patients followed at a single institution. Thirty-one Fontan pacemaker patients were identified between 1985 and 2017. Twenty-six had an epicardial system, five transvenous, and three converted from epicardial to transvenous. Average atrial lead sensing at placement was 3.23 versus 2.35 mV (p = 0.52) for epicardial and transvenous leads, respectively. Median atrial and ventricular lead longevity was 86.4 versus 98.8 months (p = 0.56) and 73.2 versus 140 months (p = 0.3) with generator longevity of 65.5 versus 73.9 (p = 0.16) months for epicardial versus transvenous systems, respectively. One major complication occurred in a transvenous patient, and two minor complications occurred in epicardial patients. All transvenous patients received warfarin except one, who converted to dabigatran. Epicardial patients received aspirin (n = 20), warfarin (n = 3) or a warfarin/aspirin combination (n = 3). No thromboembolic events occurred. System revision was required in 13 epicardial and 5 transvenous patients. There were two deaths, none related to the pacemaker system. Transvenous pacemakers can be utilized with equal efficacy compared to epicardial pacemakers with trends toward longer lead longevity in transvenous pacemaker systems.

The clinical course of multifocal atrial tachycardia in infants and children.

OBJECTIVES: This study outlines the clinical course, treatment and the late outcome of infants and children with multifocal atrial tachycardia (MAT). BACKGROUND: Multifocal atrial tachycardia is defined by three distinct P-waveforms, irregular P-P intervals, isoelectric baseline between P-waves and rapid rate on an electrocardiogram. Several smaller prior reports have described pediatric patients with MAT, but their long-term outcome has not been fully assessed. METHODS: The clinical records, echocardiograms and long-term follow-up of patients with MAT were reviewed and compared to previous reports of MAT. RESULTS: Fourteen boys and seven girls (median age 1.8 months) presented with MAT. At diagnosis, six patients had respiratory illness, of whom two were critical. Ten were asymptomatic. Seven patients had structural heart disease (SHD), one of whom died. Four of 15 patients (27%) with echocardiograms had diminished ventricular function. Ventricular rates were 111 to 253 beats/min (mean 181 beats/min). Median duration of the arrhythmia was 4.9 months (mean 6.7 months). Electrical cardioversion was attempted in 4 patients without success and 15 patients received antiarrhythmic medication. Seventeen patients were followed for a mean of 60 months. Four patients were lost to follow-up. There were no late arrhythmias. CONCLUSIONS: The majority of children with MAT are healthy infants under one year of age; a few may exhibit mild to life-threatening cardiorespiratory disease. Less often, MAT accompanies SHD. Mild ventricular dysfunction may be observed in the presence of MAT, but symptoms are few and resolution is generally complete. Response to antiarrhythmic agents is mixed, and cardioversion is of no avail. Finally, long-term cardiovascular and developmental outcome depends principally on underlying condition; for otherwise healthy children, it is excellent.

Inducibility of intra-atrial reentrant tachycardia after the first two stages of the Fontan sequence.

OBJECTIVES: We sought to examine the incidence and possible factors for inducible intra-atrial reentrant tachycardia (IART) in a group of patients after two stages of the Fontan sequence but before the operation. BACKGROUND: Intra-atrial reentrant tachycardia occurs in 10% to 40% of patients after the Fontan operation. No data are available regarding the potential for IART after the first two stages of the Fontan sequence but before the operation. METHODS: The IART induction protocol included programmed extrastimulation and rapid atrial pacing, with and without isoproterenol. RESULTS: The median age of the study group (n = 44, 27 males) was 1.7 years (range 1.2 to 5.2). Forty patients were in sinus rhythm. Twelve patients (27%) had inducible, sustained (>1 min) IART. Three patients (8%) had inducible, nonsustained IART. Bivariate analysis revealed that patients with sustained IART were significantly older at their second operation (median 0.54 vs. 0.40 years, p = 0.05). Multivariate logistic modeling revealed that older age (> or =0.55 years) at the second palliative operation (p = 0.04), older age (> or =1.95 years) at evaluation before the Fontan sequence (p = 0.04) and female gender (p = 0.03) were independently associated with sustained IART. A trend toward a greater frequency of sustained IART was seen in those patients with moderate or severe atrioventricular valve regurgitation (p = 0.07) and in those with resection of the atrial septum (p = 0.06). CONCLUSIONS: The rate of inducible, sustained IART in a group of patients before the Fontan operation is 27% and is associated with older age at the time of second-stage palliation, older age at pre-Fontan evaluation and female gender.

Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan.

BACKGROUND: Following the Norwood procedure for hypoplastic left heart syndrome (HLHS), pulmonary artery distortion and hypoplasia are common and may negatively impact late outcome. The hemi-Fontan procedure (HFP) augments the central pulmonary arteries and establishes a connection between the right atrial/superior vena cava junction and the pulmonary arteries, while excluding the inferior vena cava. METHODS: The hospital records of all 114 patients undergoing a HFP for HLHS between August 1993 and April 1998 were reviewed to assess patient, procedural, and morphologic determinations of outcome. The results of cardiac catheterization, Doppler/echocardiography, 12 lead electrocardiograms, hospital and subsequent course, as well as suitability and outcome for the Fontan procedure were analyzed. RESULTS: Mean age was 5.4 months (range 1.5 to 15 months). Right ventricular function was normal in 95 patients, moderately depressed in 14, and severely depressed in five. Tricuspid regurgitation was absent or mild in 91 patients, moderate in 13, and severe in 10. Concomitant procedures included left superior vena cava to pulmonary artery anastomosis (12), tricuspid valve repair (10), pulmonary artery stent placement (3), coarctation repair (2), and aortic pseudoaneurysm repair (1). Hospital survival was 112/114, 98% (95% confidence interval [CI]: 95% to 100%). There were two late deaths, one noncardiac. Sinus rhythm is present in 105 patients (92%, 95% CI: 87% to 97%). To date, 79 of these patients have undergone the Fontan procedure with 74 survivors (94%, 95% CI: 89% to 99%). CONCLUSIONS: The HFP may be performed with excellent results for HLHS. It effectively augments the central pulmonary arteries while preserving sinus rhythm in the majority. In addition, the HFP facilitates the subsequent Fontan procedure and has significantly improved the overall outcome.

The G-algorithm for extraction of robust decision rules--children's postoperative intra-atrial arrhythmia case study.

Clinical medicine is facing a challenge of knowledge discovery from the growing volume of data. In this paper, a data mining algorithm (G-algorithm) is proposed for extraction of robust rules that can be used in clinical practice for better understanding and prevention of unwanted medical events. The G-algorithm is applied to the data set obtained for children born with a malformation of the heart (univentricular heart). As the result of the Fontan surgical procedure, designed to palliate the children, 10%-35% of patients postoperatively develop an arrhythmia known as the intra-atrial reentrant tachycardia. There is an obvious need to identify the children that may develop the tachycardia before the surgery is performed. Prior attempts to identify such children with statistical techniques have been unrewarding. The G-algorithm discussed in this paper shows that there exists an unambiguous relationship between measurable features and the tachycardia. The data set used in this study shows that, for 78.08% of infants, the occurrence of tachycardia can be accurately predicted. The authors' prior computational experience with diverse medical data sets indicates that the percentage of accurate predictions may become even higher if data on additional features is collected for a larger data set.

Use of the His/RVA electrode catheter in children.

Comprehensive electrophysiologic study with radiofrequency ablation requires a number of intracardiac catheters. To reduce the number of catheters placed in children, the authors evaluated a series of customized catheters that combined the functions of two catheters. The customized 6F catheter contains eight electrodes placed in pairs at 4, 5, 6, 7, or 8 cm from the tip for recording the His electrogram and at the tip for right ventricular pacing. The amplitude of the bundle of His potential recorded through the His right ventricular apex (RVA) catheter (n = 63) and the ventricular pacing threshold (in mA) (n = 48) were measured and compared to the maximal bundle of His potential recorded with a 6F hexapolar catheter in 24 and 13 other patients, respectively. The relationship between the distance from the distal electrode pair at the tip and the third electrode from the tip (the His/RVA distance) and patient size was analyzed in 42 patients. Following the initial study in the 90 patients, the selection of the optimal His/RVA catheter for 19 patients was determined by examining the regression plots derived from the first group of 90 patients. The measured His/RVA distance was then determined by noting the His/RVA distance of the catheter used. Regression analysis was then used to evaluate the fit between the predicted His/RVA distance based on weight, height, or body surface area (BSA) and the observed His/RVA distance. The maximal bundle of His electrogram measured in the two groups using the His/RVA catheter was compared. To evaluate catheter stability during the study, the amplitude of the maximal His potential was measured in the 19 patients at the onset, midpoint, and end of the study. The maximal His potential recorded through the octapolar catheter (0.21 mV) was significantly (P < .04) greater than that recorded through the hexapolar catheter (0.10 mV). The mean ventricular threshold measured through the octapolar catheter (0.44 mA) was significantly (P < .001) less than that measured through the hexapolar catheter (1.13 mA). There was a significant (P < .0001) correlation between BSA, weight, and height and the His/RVA distance. There was no significant difference in the mean maximal amplitude of the His potential (0.21 +/- 0.31 mV vs 0.15 +/- 0.12 mV) recorded through the His/RVA catheter between the two groups. The His/RVA distance estimated by weight, when plotted against the measured distance, demonstrated a good correlation (r = .84) between the expected His/RVA distance based on the subject's weight and that actually observed. In 18 of 19 subjects, the first catheter based on the patient's weight (in kilograms) predicted the appropriate and only catheter used. There was no significant difference in the mean maximal bundle of His electrogram recorded at the beginning of the study (0.15 +/- 0.12 mV), midway into the study (0.15 +/- 0.11 mV), and at the end (0.13 +/- 0.13 mV); however, there was extensive variation within individuals and over time. These data support the recording of a stable, high-quality bundle of His electrogram and RVA pacing through a single catheter system and, hence, have important, practical implications for invasive electrophysiologic studies in children.

Use of a single coil transvenous electrode with an abdominally placed implantable cardioverter defibrillator in children.

While transvenous defibrillator electrode placement avoiding a thoracotomy is preferable, electrode size, a large intercoil spacing, and the need for subclavicular device placement preclude this approach in most children. We investigated a single RV coil to an abdominally placed active can ICD device. Five children ages 8-16 years (weight 21-50 kg, mean 35 kg) underwent ICD placement. Placement of a single coil Medtronic model 6932 or 6943 electrode was performed via the left subclavian vein approach and the electrode positioned in the RV apex with the coil lying along the RV diaphragmatic surface. The ICD (Medtronic Micro Jewel II model 7223 Cx) was implanted in a left abdominal pocket with the lead tunneled from the infraclavicular region to the pocket. Implant DFTs were < or = 15 J using a biphasic waveform. DFTs rechecked within 3-month postimplant were unchanged. Lead impedance at implant ranged from 38 to 56 omega, mean 51 omega. Follow-up was 3-21 months (total 82 months) with no electrode dislodgment, lead fractures, or inappropriate discharges. Two of the five patients have had successful appropriate ICD discharges. Transvenous ICD electrode placement can be performed in children as small as 20 kg with the device implanted in a cosmetically acceptable abdominal pocket that is well tolerated. Excellent DFTs can be achieved. This approach avoids a thoracotomy in all but the smallest child, does not require subclavicular placement of the device, and avoids use of a second intravascular coil.

Access to the left atrium for delivery of radiofrequency ablation in young patients: retrograde aortic vs transseptal approach.

Reported experience with the transseptal approach to the left atrium for delivery of radiofrequency energy in the young patient is limited. To compare two approaches for radiofrequency ablation (RFA) in the left atrium we reviewed our experience from January 1, 1991, through February 1, 1999, in 154 procedures performed on 136 patients (mean age 12.2 years). The patients were grouped by either the retrograde aortic route (R, n = 30) or the transseptal atrial route (T, n = 106). No significant differences were found in age, weight, height, supraventricular tachycardia cycle length, or electrocardiograph characteristics (manifest vs concealed accessory pathway) between the two approaches. Comparison of the transseptal group to the retrograde aortic group revealed a significant difference in the number of catheters (mean = 4 R vs 3 T, p < 0.0001), total fluoroscopic time (71.3 min R vs 43.0 min T, p = 0.0007), diagnostic fluoroscopic time (40.2 min R vs 16.6 min T, p < 0.0001), ablation fluoroscopic time (44.7 min R vs 25.3 min T, p = 0.019), and procedure time (5.0 hours R vs 4.1 hours T, p < 0.0001). No significant difference was found in success rate, number of radiofrequency applications, or major complication rate. These data suggest that although outcomes and major complication rates are similar for the two groups, the use of fewer catheters and shorter fluoroscopic times warrant consideration of the transseptal atrial approach in young patients.