There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary ...artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation. We divided the patients into two groups according to the change in the pulmonary artery index before and after the Fontan operation: the increased pulmonary artery index group (
n
= 51) and decreased pulmonary artery index group (
n
= 43). The pulmonary artery index after the Fontan operation was significantly higher in the increased pulmonary artery index group than decreased pulmonary artery index group (
p
< 0.0001). The central venous pressure (
p
< 0.0001) and pulmonary vascular resistance (
p
= 0.007) were significantly lower in the increased pulmonary artery index group. The mixed venous oxygen saturation was significantly higher in the increased pulmonary artery index group (
p
< 0.0001). The pulmonary artery index was more increased in the patients who were administered a phosphodiesterase type 5 inhibitor than in those who were not (
p
= 0.03). The change in the pulmonary artery index reflected the hemodynamics after a Fontan operation during the short term. In that respect, phosphodiesterase type 5 inhibitors might be involved in the process.
The Senning operation used to be widely performed for an intracardiac repair in a complete transposition of the great arteries. During the long-term follow-up, supraventricular tachycardia (SVT) is ...often observed because of the complex suture lines. The typical mechanism of a Senning-related SVT is cavo-tricuspid isthmus-dependent atrial flutter. On rare occasions, complex SVTs (e.g., biatrial tachycardia (BiAT)) whose diagnosis and treatment are challenging, may occur. We report a rare case of a BiAT following a Senning operation that was successfully ablated from the superior vena cava, and the local activation time histogram module (CARTO3 V7 module Biosense Webster, Irvine, CA, USA) was crucial for analyzing the complex circuit.
...of the improvement in surgical treatment over the past few decades, the life expectancy of patients with an atrial septal defect (ASD) has been substantially extended. ...as they age, the ...incidence of atrial arrhythmias including atrial fibrillation (AF) increases. A TSP after a repaired ASD is reported to be feasible, safe, and efficacious. 2 As discussed earlier, the TSP is performed through the repaired septum, through foreign patches, occluding devices, or the surrounding native interatrial septum. 1 In contrast, the TSP through the repaired atrial septum in the present case was challenging due to severe calcifications. Because the calcified trans-patch puncture precluded the possibility of a direct puncture and had a risk of an embolic event, the only viable option was to target the narrow native tissue next to the patch.
An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization ...against a pleural effusion before a Fontan operation is still controversial.This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first. Factors associated with the duration of pleural drainage (median: 6 (2-41) days) and that of postoperative hospital stay (median: 25 (14-91) days) were analyzed using a multiple regression analysis. The pulmonary artery index (PAI; Nakata index) was associated with both the pleural drainage duration (P < 0.05, r2 = 0.17) and postoperative hospital stay (P < 0.05, r2 = 0.10).Thereafter, all the 227 patients were classified into the following three groups: Group A (12 patients in whom the embolization was performed within 30 days before the Fontan surgery), Group B (131 patients in whom the embolization was performed more than 30 days before the Fontan surgery), and Group C (84 patients in whom the embolization was not performed). Patients in Group A were found to be associated with the shortest length of both periods (P < 0.05).Lower PAI values were related to a prolonged pleural drainage duration and postoperative hospital stay. APC coil embolizations may reduce the risk if they are performed shortly (less than 30 days) before the operation.
An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the ...inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD). This report describes the efficacy of S-ICDs in a child with an ER pattern after aborted sudden cardiac death.
Abstract
Average beat interval (BI) and beat interval variability (BIV) are primarily determined by mutual entrainment between the autonomic-nervous system (ANS) and intrinsic mechanisms that govern ...sinoatrial node (SAN) cell function. While basal heart rate is not affected by age in humans, age-dependent reductions in intrinsic heart rate have been documented even in so-called healthy individuals. The relative contributions of the ANS and intrinsic mechanisms to age-dependent deterioration of SAN function in humans are not clear. We recorded ECG on patients (n = 16 < 21 years and n = 23 41–78 years) in the basal state and after ANS blockade (propranolol and atropine) in the presence of propofol and dexmedetomidine anesthesia. Average BI and BIV were analyzed. A set of BIV features were tested to designated the “signatures” of the ANS and intrinsic mechanisms and also the anesthesia “signature”. In young patients, the intrinsic mechanisms and ANS mainly contributed to long- and short-term BIV, respectively. In adults, both ANS and intrinsic mechanisms contributed to short-term BIV, while the latter also contributed to long-term BIV. Furthermore, anesthesia affected ANS function in young patients and both mechanisms in adult. The work also showed that intrinsic mechanism features can be calculated from BIs, without intervention.
In adult patients, subcutaneous implantable cardioverter defibrillators (S-ICDs) have been reported to be non-inferior to transvenous ICDs with respect to the incidence of device-related ...complications and inappropriate shocks. Only a few reports have investigated the efficacy of S-ICDs in the pediatric field. This study aimed to investigate the utility and safety of S-ICDs in patients ≤18 years old.
This study was a multicenter, observational, retrospective study on S-ICD implantations. Patients <18 years old who underwent S-ICD implantations were enrolled. The detailed data on the device implantations and eligibility tests, incidence of appropriate- and inappropriate shocks, and follow-up data were assessed.
A total of 62 patients were enrolled from 30 centers. The patients ranged in age from 3 to 18 (median 14 years old IQR 11.0-16.0 years). During a median follow up of 27 months (13.3-35.8), a total of 16 patients (26.2%) received appropriate shocks and 13 (21.3%) received inappropriate shocks. The common causes of the inappropriate shocks were sinus tachycardia (n = 4, 30.8%) and T-wave oversensing (n = 4, 30.8%). In spite of the physical growth, the number of suitable sensing vectors did not change during the follow up. No one had any lead fractures or device infections in the chronic phase.
Our study suggested that S-ICDs can prevent sudden cardiac death in the pediatric population with a low incidence of lead complications or device infections. The number of suitable sensing vectors did not change during the patients' growth.
Background:Genetic testing for long QT syndrome (LQTS) is now in clinical practice. We conducted molecular genetic analyses to definitively diagnose LQTS and to determine its subtypes for ...gene-specific treatment. We conducted a retrospective study to determine the characteristics of schoolchildren with LQTS living on the Okinawa Islands.Methods and Results:The study population included children identified in a school-based electrocardiographic (ECG) screening program for cardiovascular diseases who were referred to Okinawa Children’s Medical Center between 2007 and 2012; 23 children met the diagnostic criteria for LQTS. Of them, 17 were genotype-positive and 14 were found to harbor theSCN5AE1784K mutation exclusively among the LQTS genotype-positive children. The children were divided into genotype-positive and -negative groups. Clinical characteristics and ECG data were analyzed and compared. The median Schwartz score was 3. The median QT interval was 521 ms.Conclusions:The major finding is that the prevalent subtype of LQTS in Okinawa is discordant with other cohorts living in other regions of Japan or overseas. We cannot exclude the possibility of the presence of a specific founder mutation in this geographically clustered population, particularly considering that the hospital is the only tertiary heart center for children in Okinawa. However, this uniquely high prevalence of theSCN5AE1784K mutation serves as a compelling justification to conduct a larger study. (Circ J 2014; 78: 1974–1979)
PDF
