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Background Blood pressure variability (BPV), defined as the degree of variation between discrete blood pressure readings, is associated with poor outcomes in acute care settings. Acute kidney injury (AKI) is a common and serious postoperative complication of cardiac surgery with cardiopulmonary bypass (CPB) in children. No studies have yet assessed the association between intraoperative BPV during cardiac surgery with CPB and the development of AKI in children. Methods A retrospective chart review of children undergoing cardiac surgery with CPB was performed. Intraoperative BPV was calculated using average real variability (ARV) and standard deviation (SD). Multiple regression models were used to examine the association between BPV and outcomes of AKI, hospital and intensive care unit (PICU) length of stay, and length of mechanical ventilation. Results Among 231 patients (58% males, median age 8.6 months) reviewed, 51.5% developed AKI (47.9% Stage I, 41.2% Stage II, 10.9% Stage III). In adjusted models, systolic and diastolic ARV were associated with development of any stage AKI (OR 1.40, 95% CI 1.08-1.8 and OR 1.4, 95% CI 1.05-1.8, respectively). Greater diastolic SD was associated with longer PICU length of stay ([beta] 0.94, 95% CI 0.62-1.2). When stratified by age, greater systolic ARV and SD were associated with AKI in infants [less than or equal to] 12 months, but there was no relationship in children > 12 months. Conclusions Greater BPV during cardiac surgery with CPB was associated with development of postoperative AKI in infants, suggesting that BPV is a potentially modifiable risk factor for AKI in this high-risk population. Graphical abstract, Author(s): Jennifer E. Fishbein [sup.1] , Matthew Barone [sup.2] , James B. Schneider [sup.3] , David B. Meyer [sup.4] , John Hagen [sup.5] , Adnan Bakar [sup.3] , Kalliopi Grammatikopoulos [...]

We report a case of radiation‐induced myofibroblastoma of the right nasal cavity in a patient with a remote history of radiotherapy for pediatric retinoblastoma. The patient required maxillectomy and ethmoidectomy. To our knowledge, a rare number of cases have been reported in this location. [ABSTRACT FROM AUTHOR]

Background: Atrial fibrillation (AF) is the most common cardiac arrhythmia and is emerging in prevalence with an increasingly aging population. The complex nature of the disease and its association with significant morbidity and mortality has resulted in a call for a new integrative, multidisciplinary approach to AF management. Purpose: Determine if the use of a nurse practitioner (NP)-led AF clinic (NPAFC) can improve care for patients. Results: Overall, 1,442 patients were enrolled in the AF clinic between January 2016 and June 2018. The mean age at the first AF clinic visit was 68.7 6 12.6 years, 54% were male, and the mean body mass index was 31 6 7 kg/m2. Among the patients, 45.2% had paroxysmal AF, 43.6% persistent AF, and 5.5% permanent AF. With an average of 3 6 3 clinic visits per patient, the number of patients with $1 hospitalization decreased by 78% after clinic implementation. Similarly, the number of patients with $1 ER visit decreased by 79%, and 22.7% of patients avoided at least one ER visit. Conclusions: The number of patients with $1 hospitalization or $1ER visit decreased within two years after the implementation of an NPAFC. Implications: Implementation of an NP-led AF clinic in the United States may reduce hospitalizations and ER visits if implemented in an integrative model. [ABSTRACT FROM AUTHOR]

Control of post exercise in vivo glycogen synthesis rate in rat muscle depends equally on phosphorylation, establishing ratio of glycogen synthase I to glycogen synthase I and D and allosteric regulation of glycogen synthase by glucose 6-phosphate (G-6-P). This is evident from the application of in vivo 13C and 31P nuclear magnetic resonance (NMR) spectroscopy to time courses of the glycogen synthesis rate and of G-6-P concentration following an electrically induced exercise in the anesthetized rat gastrocnemius.

Although the use of a right ventricle to pulmonary artery shunt as a source of pulmonary blood flow in stage 1 Norwood palliation is common, there is no uniform agreement on the optimal surgical technique. We present a technique for creating the distal connection aimed at minimizing procedural bleeding, promoting distal patency, and facilitating second-stage palliation. [ABSTRACT FROM AUTHOR]

Background: Avoidance of cardiopulmonary bypass (CPB) and aortic cross-clamping during the Fontan procedure has been advocated to improve outcomes. We continue to use CPB with aortic cross-clamping for the Fontan procedure. Methods: We performed a review of patients undergoing the Fontan procedure between January 1, 2000 and December 31, 2004. Results: The Fontan procedure was performed in 160 patients. The median age was 2.2 years (range, 1.0 to 29.1 years). Hypoplastic left heart syndrome or a variant was present in 114 patients (71%), and heterotaxy was present in 19 (12%). CPB and modified ultrafiltration were used in all patients. Aortic cross-clamping was used in 154 (96%) of 160 patients and deep hypothermic circulatory arrest (DHCA) in 132 (83%). A lateral tunnel Fontan was performed in 69 patients (43%) and an extracardiac Fontan in 91 (57%). A fenestration was created in 144 patients (90%). Two patients died. Freedom from death or takedown was 98% (157/160). Median duration of pleural drainage was 2 days (range, 1 to 44 days) and was more than 14 days in 16 patients. Median duration of hospitalization was 6 days (range, 3 to 55 days). The small number of deaths precluded assessment of risk factors for mortality. By multivariable analysis, risk factors for pleural drainage longer than 3 days were extracardiac connection (p < 0.001) and increasing mean pulmonary artery pressure before the Fontan procedure (p = 0.033). By multivariable analysis, risk factors for hospitalization for more than 7 days were extracardiac connection (p = 0.003), increasing duration of total support (CPB and DHCA, p = 0.027), and decreasing systemic oxygen saturation before the Fontan procedure (p = 0.048). Conclusions: The Fontan procedure can be performed using CPB and aortic cross-clamping with low morbidity and mortality. [Copyright &y& Elsevier]

In July 1997, a dual chamber pacemaker combined with a tiered therapy implantable cardioverter defibrillator (ICD) first become available in the United States. We report the first-year experience of one center in the United States with this dual chamber ICD. Of a total of 174 ICDs, 95 (55%) were dual chamber devices and 79 (45%) were single chamber. New dual chamber ICD insertions averaged 57.4 ± 8.9 minutes, though there was a learning curve as the last 30 implants averaged 45.1 ± 6.1 minutes with a negative slope to the regression line of procedure duration (-0.52, P < 0.05). New single chamber ICD implants were 18.5 minutes quicker (38.9 ± 7.2 minutes). The most challenging implants were dual chamber upgrades (mean procedure duration 64.9 ± 15.8 minutes), especially if there was a previously implanted pacemaker and ICD at separate sites. Indications for a new dual chamber device were grouped into classic pacemaker indications (52.5%), which comprised the Class IACC/AHA guidelines, ICD-specific indications (24.6%), and other (23.0%). In the 34 patients undergoing dual chamber upgrade, the classic and ICD-specific groups were equal (47.0% each). Complications were rare (2.8%), though 3 (8.8%) of 34 undergoing a dual chamber upgrade developed late infections requiring explantation. In its first year, the dual chamber ICD has become a common device at our institution comprising 55% of new implants. As experience grows, we anticipate similar usage at most institutions. [ABSTRACT FROM AUTHOR]

Evaluation of ICD function can now be performed noninvasively with intravenous sedation. To determine the value of follow-up electrophysiological studies for ICD implants, we performed a retrospective review of predischarge and 2-month ICD restudies, identifying critical problems uncovered. Of the 123 patients implanted, 122 had a predischarge study, 105 had both predischarge and elective 2-month follow-up studies, and 1 patient expired prior to restudy. Patients who underwent 2-month studies for nonelective indications (e.g., frequent shocks) were excluded from analysis. Programming changes were made in 62% of the predischarge studies (n = 122) and 70% of the elective 2-month studies (n = 105). The average number of programming changes per study was 1.3 for predischarge testing and 1.1 for 2-month testing. The most common changes at predischarge testing were adjustment of the tachyarrhythmia rate cutoff (35%) and at 2-month study, reprogramming of bradycardia pacing parameters (41%). Of the patients who underwent both predischarge and 2-month testing, 91% had programming changes in at least one of their restudies. Of 227 restudies performed, 18 studies in 14 patients yielded 24 critical findings which included: DFT increases to ≥ 25 J (n = 13); sensing abnormalities of induced ventricular arrhythmia (n = 6); dislodged lead (n = 2); and serious pacemaker interactions (n = 3). Six of these critical cases (5% of total patients) required reoperation. The data suggests that routine ICD restudy is a valuable tool for management of the ICD patient. Additionally, ICD restudy is likely to increase the diagnostic yield of clinically silent critical system problems that could result in device failure. [ABSTRACT FROM AUTHOR]

Twiddler's syndrom is a highly recognized yet rare complication of pacemaker and cardioverter defibrillator (ICD) implantation. We present a case in which persistent generator rotation resulted in lead dislodgment and inappropriate shocks in an initial ICD and recurrent lead fracture in a second ICD system. This case is unusual in that even with extensive surgical precautions including use of a Dacron pouch, generator rotation could not be prevented. Submuscular implantation and use of a smaller generator may prevent Twiddler's syndrome. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor in response to the article "A Technique for Intraoperative Arrhythmia Induction During Automatic Implantable Defibrillator Placement," by B. P. Grubb and M. Mancini in the 1990 issue.

Circumflex aortic arch with coarctation and anomalous origin of the left pulmonary artery from the aorta are rare cardiovascular anomalies. These conditions can lead to early pulmonary hypertension and challenging management. Early diagnosis and surgical intervention are beneficial for optimal outcome. We present a case where both anomalies coexisted and were repaired with aortic uncrossing, arch augmentation, and reimplantation of the left pulmonary artery. To our knowledge, this is the first documented instance of these anomalies coexisting and being repaired in the neonatal period., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Background: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation., Methods: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index., Results: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm 2 /m 2 (range 15-144 mm 2 /m 2 ) and at time of septation 184 mm 2 /m 2 (range 56-510 mm 2 /m 2 ; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair., Conclusion: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

Background: Regional cerebral perfusion (RCP) is used as an adjunct or alternative to deep hypothermic circulatory arrest (DHCA) for neonates and infants undergoing aortic arch repair. Clinical studies have not demonstrated clear superiority of either strategy, and multicenter data regarding current use of these strategies are lacking. We sought to describe the variability in contemporary practice patterns for use of these techniques., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2013) was queried to identify neonates and infants whose index operation involved aortic arch repair with cardiopulmonary bypass. Perfusion strategy was classified as isolated DHCA, RCP (with less than or equal to ten minutes of DHCA), or mixed (RCP with more than ten minutes of DHCA). Data were analyzed for the entire cohort and stratified by operation subgroups., Results: Overall, 4,523 patients (105 centers) were identified; median age seven days (interquartile range: 5.0-13.0). The most prevalent perfusion strategy was RCP (43%). Deep hypothermic circulatory arrest and mixed perfusion accounted for 32% and 16% of cases, respectively. In all, 59% of operations involved some period of RCP. Regional cerebral perfusion was the most prevalent perfusion strategy for each operation subgroup. Neither age nor weight was associated with perfusion strategy, but reoperations were less likely to use RCP (31% vs 45%, P < .001). The combined duration of RCP and DHCA in the RCP group was longer than the DHCA time in the DHCA group (45 vs 36 minutes, P < .001)., Conclusion: There is considerable variability in practice regarding perfusion strategies for arch repair in neonates and infants. In contemporary practice, RCP is the most prevalent perfusion strategy for these procedures. Use of DHCA is also common. Further investigation is warranted to ascertain possible relative merits of the various perfusion techniques., (© The Author(s) 2016.)