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Longitudinal follow-up, resource utilization, and health disparities are top congenital heart research and care priorities. Medicaid claims include longitudinal data on inpatient, outpatient, emergency, pharmacy, rehabilitation, home health utilization, and social determinants of health-including mother-infant pairs.The New York Congenital Heart Surgeons Collaborative for Longitudinal Outcomes and Utilization of Resources linked robust clinical details from locally held state and national registries from 10 of 11 New York congenital heart centers to Medicaid claims, building a novel, statewide mechanism for longitudinal assessment of outcomes, expenditures, and health inequities.The authors included all children 18 years of age undergoing cardiac surgery in The Society of Thoracic Surgeons Congenital Heart Surgery Database or the New York State Pediatric Congenital Cardiac Surgery Registry from 10 of 11 New York centers, 2006 to 2019. Data were linked via iterative, ranked deterministic matching on direct identifiers. Match rates were calculated and compared. Proportions of the linked cohort trackable over 3, 5, and 10 years were described.Of 14,097 registry cases, 59% (n = 8,322) reported Medicaid use. Of these, 7,414 were linked to New York claims, at an 89% match rate. Of matched cases, the authors tracked 79%, 74%, and 65% of children over 3, 5, and 10 years when requiring near-continuous Medicaid enrollment. Allowing more lenient enrollment criteria, the authors tracked 86%, 82%, and 76%, respectively. Mortality over this time was 7.7%, 8.4%, and 10.0%, respectively. Manual validation revealed ∼100% true matches.This establishes a novel statewide data resource for assessment of longitudinal outcome, health expenditure, and disparities for children with congenital heart disease.

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. 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. 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 (β 0.94, 95% CI 0.62–1.2). When stratified by age, greater systolic ARV and SD were associated with AKI in infants ≤ 12 months, but there was no relationship in children > 12 months. 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.

Interrupted aortic arch (IAA) often presents with a wide gap between the proximal and distal portions of the arch which challenge a tension-free anastomosis, potentially increasing the risk for residual or recurrent arch obstruction, and left main bronchial compression. When possible, to preserve potential for growth of the repair, native tissue-to-tissue reconstruction is always preferred. Accordingly, rather than forcing a direct anastomosis with native tissue under tension or needing an interposition graft, the ascending aortic slide is an alternative useful technique to repair interrupted aortic arch in neonates and infants with difficult anatomy. Using a bridging flap of native tissue from the split ascending aorta and completion with patch material to fill in the lesser arch curvature, it has successfully been used in nine babies for bi-ventricular repair and uni-ventricular palliation with satisfactory results, and potential for growth.

Isolated aortic regurgitation and myocardial infarction are a rare congenital defect among neonatal patients. We present a case of a neonate with an unusual aortic valve morphology causing both regurgitation and obstruction of the left coronary artery ostium. Despite both non-invasive and invasive imaging modalities, accurate diagnosis of the valve morphology was only determined by direct visualisation at the time of surgical repair. To the knowledge of authors, this particular aortic valve morphology in neonatal population has not been previously reported in the literature.

Despite patients having increased access to their own electronic health record (EHR) in recent times, patients are often still not considered a primary audience of pathology reports. An alternative to in-person patient education is the use of multimedia programming to enhance health literacy. Curated video presentations designed to explain diagnosis-specific pathology terms were reviewed by a board-certified pathologist and oncologist team and then shown to patients with a primary diagnosis of either pancreatic, colorectal, or prostate cancer in-clinic; these patients then completed a secure electronic survey immediately afterwards. Seventy patients were surveyed, with 91% agreeing or strongly agreeing that the video they watched increased their understanding of the medical terms used in their pathology reports, with a corresponding average Likert score (ALS) of 4.21 (SD = 0.77, CI = ± 0.18). Furthermore, 95% agreed or strongly agreed that the video they watched both enhanced their understanding of the role of the pathologist in diagnosing cancer (ALS = 4.27; SD = 0.65, CI = ± 0.15) and reported they found the video useful (ALS = 4.27; SD = 0.53, CI = ± 0.13). Curated videos such as those utilized in this study have the potential to increase patient health literacy and inform patients of the multidisciplinary nature of cancer diagnosis.

We hypothesize that there are post-operative, non-surgical risk factors that could be modified to prevent the occurrence of chylothorax, and we seek to determine those factors. Retrospective chart review of 285 consecutive patients

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.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.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 (β 0.94, 95% CI 0.62-1.2). When stratified by age, greater systolic ARV and SD were associated with AKI in infants ≤ 12 months, but there was no relationship in children 12 months.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.

Supplemental Digital Content is available in the text., Background: De novo genic and copy number variants are enriched in patients with congenital heart disease, particularly those with extra-cardiac anomalies. The impact of de novo damaging variants on outcomes following cardiac repair is unknown. Methods: We studied 2517 patients with congenital heart disease who had undergone whole-exome sequencing as part of the CHD GENES study (Congenital Heart Disease Genetic Network). Results: Two hundred ninety-four patients (11.7%) had clinically significant de novo variants. Patients with de novo damaging variants were 2.4 times more likely to have extra-cardiac anomalies (P=5.63×10−12). In 1268 patients (50.4%) who had surgical data available and underwent open-heart surgery exclusive of heart transplantation as their first operation, we analyzed transplant-free survival following the first operation. Median follow-up was 2.65 years. De novo variants were associated with worse transplant-free survival (hazard ratio, 3.51; P=5.33×10−04) and longer times to final extubation (hazard ratio, 0.74; P=0.005). As de novo variants had a significant interaction with extra-cardiac anomalies for transplant-free survival (P=0.003), de novo variants conveyed no additional risk for transplant-free survival for patients with these anomalies (adjusted hazard ratio, 1.96; P=0.06). By contrast, de novo variants in patients without extra-cardiac anomalies were associated with worse transplant-free survival during follow-up (hazard ratio, 11.21; P=1.61×10−05) than that of patients with no de novo variants. Using agnostic machine-learning algorithms, we identified de novo copy number variants at 15q25.2 and 15q11.2 as being associated with worse transplant-free survival and 15q25.2, 22q11.21, and 3p25.2 as being associated with prolonged time to final extubation. Conclusions: In patients with congenital heart disease undergoing open-heart surgery, de novo variants were associated with worse transplant-free survival and longer times on the ventilator. De novo variants were most strongly associated with adverse outcomes among patients without extra-cardiac anomalies, suggesting a benefit for preoperative genetic testing even when genetic abnormalities are not suspected during routine clinical practice. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT01196182.

OBJECTIVE Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA. METHODS A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan. RESULTS CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P

We aimed to correlate photoplethysmographic parameters with stroke volume in infants with PDA. Photoplethysmography constitutes the optical signal in pulse oximetry. Stroke volume was determined echocardiographically. Pulse transit time, right hand to foot arrival time difference, and relative amplitude were measured from pulse oximeter and ECG waveforms. Photoplethysmographic parameters before and after PDA closure were compared with stroke volume. After PDA closure, pulse transit time to the hand and to the foot were prolonged (54.7 ± 6.7 vs 65.5 ± 9.8 ms, p

Post-pericardiotomy syndrome (PPS) is an inflammatory process involving the pleura, pericardium, or both and occurs after cardiothoracic surgery. Surgical atrial septal defect (ASD) closure is associated with higher incidence of PPS post-operatively as compared to other operations. Reported incidence of PPS varies from 1 to 40%. NSAIDs are often used to treat PPS and in our center, some practitioners have prescribed ibuprofen prophylactically. This study sought to investigate the impact of prophylactic treatment with ibuprofen on the development and severity of PPS following surgical ASD closure, with particular attention to secundum-type ASDs. We retrospectively reviewed clinical and operative data of all surgical ASD repairs in our center from 1/2007 to 7/2017. ASDs were grouped by subtype. PPS was considered positive if the primary cardiologist diagnosed and documented clinical signs of PPS on post-operative outpatient follow-up. Records were reviewed to confirm documented diagnosis of PPS. A total of 245 cases were reviewed with 207 having sufficient data. Median age was 2 years (range 4 months-27 years), female 57%. Overall incidence of PPS was 10%. There was no difference in incidence of PPS in those prescribed ibuprofen as compared to those who were not. This was true for both the entire cohort and the subgroup analysis (P = 1.0). Four patients overall required pericardiocentesis, none of whom received prophylactic ibuprofen. Prophylactic ibuprofen prescription following surgical ASD repair did not reduce the rate of PPS in our cohort.

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 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; 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.

Objectives To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. Methods PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. Results At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). Conclusions PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

Isolated aortic regurgitation and myocardial infarction are a rare congenital defect among neonatal patients. We present a case of a neonate with an unusual aortic valve morphology causing both regurgitation and obstruction of the left coronary artery ostium. Despite both non-invasive and invasive imaging modalities, accurate diagnosis of the valve morphology was only determined by direct visualisation at the time of surgical repair. To the knowledge of authors, this particular aortic valve morphology in neonatal population has not been previously reported in the literature.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

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.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9–24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

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.

Studies regarding long-term results after repair of anomalous left main coronary artery from the pulmonary artery (ALCAPA) have mainly focused on survival rates, left ventricular function, and mitral valve function. The development of left main coronary stenosis following repair has been infrequently reported and its incidence unknown. Optimal therapy is also not known. Here we report the use of surgical angioplasty to achieve revascularization of a stenotic left main coronary artery in a patient with ALCAPA who had undergone coronary transfer 15 years earlier.

The article Non‑surgical Risk Factors for the Development of Chylothorax in Children after Cardiac Surgery‑Does Fluid Matter?, written by Tanya Perry, Kelly Bora, Adnan Bakar, David B. Meyer and Todd Sweberg, was originally published electronically on the publisher's internet portal (currently SpringerLink) on November 2019 with open access. With the author(s)' decision to step back from Open Choice, the copyright of the article changed on November 2019 to © Springer Science+Business Media, LLC, part of Springer Nature 2019 and the article is forthwith distributed under the terms of copyright.

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.

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.We performed a review of patients undergoing the Fontan procedure between January 1, 2000 and December 31, 2004.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 (p0.001) 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).The Fontan procedure can be performed using CPB and aortic cross-clamping with low morbidity and mortality.

OBJECTIVES We sought to test the postulate that biventricular pacing diminishes the need for appropriate tachycardia therapy. We reviewed the frequency of therapy in patients, serving as their own controls, who were enrolled in the Ventak CHF (congestive heart failure) biventricular pacing study. BACKGROUND It is well established that both acute and chronic CHF contribute to the need for tachyarrhythmia therapy in recipients of an automatic implantable cardioverter defibrillator (ICD). Synchronized biventricular (BV) pacing is a new and promising therapy for symptomatic improvement of CHF in selected patients (low ejection fraction, intraventricular conduction delay). We postulate that this pacing therapy will diminish the need for tachyarrhythmia therapy. METHODS Participants in the Ventak CHF trial received a triple-chamber biventricular ICD with a transvenous right ventricular lead and a left ventricular (LV) lead placed via thoracotomy. Of 54 patients enrolled in the Ventak CHF trial, 32 could be analyzed, with each completing three blinded months programmed to BV VDD pacing and a second randomly assigned three-month period of no pacing. RESULTS Of the 32 patients, 13 (41%) received appropriate therapy for a ventricular tachyarrhythmia at least once in the six-month monitoring period postimplant. Five patients (16%) had at least one tachyarrhythmic episode while programmed to BV pacing, whereas 11 (34%) had at least one episode while programmed to no pacing. Three patients (9%) received therapy in both pacing periods, two with BV pacing only. The decrease in necessary tachycardia therapy during the BV pacing period was statistically significant (p = 0.035). CONCLUSIONS In patients with standard ICD indications who also have CHF, LV dysfunction, and an intraventricular conduction delay, ICD therapy is less common with BV pacing. The mechanism for this improvement is unclear but may be related to hemodynamic improvement in CHF. Although BV pacing does not obviate the need for an ICD, it does diminish the need for appropriate tachyarrhythmia therapy in selected patients.

In July 1997, a dual chamber pacemaker combined with a tiered therapy implantable cardioverter defibrillator (ICD) first became 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 I ACC/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.

Ductal arteriosus aneurysm (DAA) is a well-recognized condition, especially in infancy, and is usually asymptomatic. We report the first case of a newborn who presented with significant inspiratory stridor and, using multiple imaging investigations, was subsequently diagnosed with the rare constellation of a congenital DAA, a right aortic arch and an isolated left subclavian artery with normal intra-cardiac anatomy. The patient underwent surgical resection of the DAA with significant improvement in symptoms.

Time courses of the glycogen synthesis rate and of the glucose 6-phosphate (G-6-P) concentration after an electrically induced exercise were followed in the anesthetized rat gastrocnemius by in vivo 13C and 31P nuclear magnetic resonance (NMR) spectroscopy, respectively. The ratio of glycogen synthase I to glycogen synthase I and D (I/I+D) and allosteric activation by G-6-P were also studied in vitro on muscles sampled at rest and 10 min (early recovery) and 100 min (late recovery) after exercise. From early recovery to late recovery, the in vivo glycogen synthesis rate dropped from 0.46 +/- 0.06 to 0.11 +/- 0.04 mmol.kg wet tissue-1.min-1, the G-6-P concentration from 0.83 +/- 0.08 to 0.32 +/- 0.05 mmol/kg wet tissue, and I/I+D from 83 +/- 4 to 47 +/- 1%. The combination of the changes in G-6-P concentration and in I/I+D quantitatively describes the fourfold decrease in glycogen synthesis rate from early to late recovery. These results demonstrate that phosphorylation, determining glycogen synthase I/I+D, and allosteric control of glycogen synthase by G-6-P contribute approximately equally to the regulation of the postexercise in vivo glycogen synthesis rate.

Malignant primary cardiac tumors are very rare.Desmoplastic small round cell tumors are also rare tumors and,when present, are generally found in the abdomen. We report a case of an adolescent male who presented with chest pain,abdominal pain, and difficulty in breathing, who was found to have a primary cardiac sarcoma with several characteristic features of a desmoplastic small round cell tumor.

An unusual case of total anomalous pulmonary venous connection surviving to adulthood without surgical correction is presented. Transthoracic echocardiography first led to this diagnosis and magnetic resonance imaging refined the anatomic diagnosis leading to successful surgical correction.

Of 122 patients with single-chamber implantable cardioverter-defibrillators (ICDs) reviewed retrospectively, 35 had traditional indications, 14 had other indications, and 18 had ICD-specific indications for dual-chamber pacing therapy. Thus, 67 patients (55%) were potential candidates for dual-chamber pacing, which has only recently become available combined with ICD therapy.

A 32-year-old female presented with dysphagia. Radiographic studies revealed external compression of esophagus by a vascular ring. The anatomy was a right-sided aortic arch with aberrant retroesophageal left subclavian artery, emanating from a large Kommerell diverticulum (KD). Traditional repair with ligamentum division and adhesiolysis leaves a large KD still adjacent to the esophagus with the potential for persistent or recurrent symptoms. The objective of this study was the modification of operative technique to minimize the potential for persistent or recurrent symptoms. The operative repair included resection of KD with transposition of the left subclavian artery into the left carotid artery, in addition to the division of the ligamentum arteriosum and mobilization of the esophagus. The patient's dysphagia resolved and postoperative barium studies showed no residual compression. There were no significant perioperative complications. Resection of KD is a potential adjunct to traditional repair of vascular rings and might offer better long-term palliation by minimizing residual vascular compression of the esophagus.

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 re studies, 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 re studies. Of 227 re studies performed, 18 studies in 14 patients yielded 24 critical findings which included: DFT increases to > or = 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.

Twiddler's syndrome is a highly recognized yet rare complication of pacemaker and cardioverter defibrillator (i.c.d.) 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.

A fine structural investigation of the transport of intravenously-injected ferritin (110 A) through the external vascular barrier (choriocapillaris and Bruch's membrane) of the Japanese quail retina has been sequentially detailed. One minute after injection ferritin is found mainly within the vascular lumina of the choriocapillaris but also is seen in pinocytic vesicles within the capillary endothelium and dispersed throughout the various components of Bruch's membrane. Ferritin transport through the capillary endothelium appears to occur via pinocytic vesicles in the thickened portions and diaphragmatic pores (single and double) in the attenuated areas. Within 5–10 min post-injection ferritin is observed within the basal infoldings of the pigment epithelium.

Landolt's club process has been detailed in the adult Japanese quail retina. In Golgiimpregnated specimens, the process has been shown to arise either as a direct continuation of the dendritic trunk of some cells of bipolar nature or from a branch of their dendritic tuft. It courses through the outer plexiform layer to end at the level of the external limiting membrane (ELM) in a terminal expansion. In several cases, a thin cilium-like structure arises from the expansion and extends toward the pigment epithelium. Additionally the electron microscope reveals the following associations: club-club, club-photoreceptor cell and club-Muller cell. Electron-dense thickenings in the region of the cell boundaries were observed in club-photoreceptor cell associations, but no synaptic vesicles were found. Ultrastructurally, the terminal expansion narrows to a slender neckpiece which in turn expands into a bulbous swelling external to the ELM. A cilium is found in the terminal expansion (9+0 arrangement of microtubules) and its companion centriole is present in the bulbous swelling.

The development of the cornea in the chick, based on the microscopical examination of serial sections of 108 morphologically staged chick embryos between stage 26 and hatching, is described. A description of the adult cornea is also provided. Correlations between the internal histogenesis of the cornea and the external morphology of the chick embryo have been established from the use of staged embryos, so that the probable state of development of the cornea can be assessed from surface appearances only. The cornea of the chick, at stage 26, consists of an anterior epithelium, an acellular postepithelial layer, and a mesothelium. Mesenchymal cells (corneal ‘corpuscles’) begin to infiltrate the postepithelial layer at stage 27. By stages 29–31 these cells occupy a continuous layer, the substantia propria, which comprises the entire extent of the postepithelial layer except for an anterior, acellular band subjacent to the anterior epithelium. The anterior band becomes progressively thinner and disappears at stage 36, the substantia propria then extending across the entire distance between the anterior epithelium and the mesothelium. The anterior limiting lamina first appears at stage 39. It closely resembles and is often confused with the temporary anterior band of the postepithelial layer. The posterior limiting lamina was not identified with certainty in any of the embryonic corneae examined. It has been stressed that embryological studies in which external staging and internal development are correlated provide a more precise interpretation of developmental processes, and are an essential prelude to experimental, physiological, biochemical, and histochemical investigations of embryonic development.

The practicability of applying histochemical reactions to bulk staining has been explored by subjecting whole chick embryos at early stages to the periodic acid-Schiff (PAS) reaction. A comparison of the microscopic distribution of PAS positive substances revealed by this procedure with that obtained by the standard routine, i.e., staining of deparaffinized sections on slides, has shown similar localizations of PAS positive material and, in addition, finer morphological detail and more intensive reactions by staining the specimens in toto. The following method is recommended for chick embryos between stages 11-17 (Hamburger and Hamilton): Fixation in Gendre's fluid at 4°C; oxidation with alcoholic buffered periodic acid, 15 min; rinsing in distilled water, 10 min; Schift's reagent, 30 min; 3 sulfite rinses, 5 min each; running tap water, 10 min; dehydration, clearing and double-embedding in celloidin and paraffin.

The intraembryonic distribution of selectively stained (periodic acid-Schiff positive) primordial germ cells (PGCs) was investigated in morphologically staged chick embryos from the stage when they first arrive in the embryo proper until they have become firmly established in the gonadal anlage. The number and precise localization of the PGCs at each developmental stage have been recorded and have provided significant data on the mechanisms involved in the transport and disposition of the PGCs, particularly their colonization of the gonadal primordium. In general, the PGCs of the chick measure between 12 and 14 μ in diameter and possess abundant intracytoplasmic deposits of PAS-positive glycogen. They originate extraembryonically and are transported passively by the circulating blood to all vascularized parts of the developing embryo. The time of their first appearance within the embryo proper (stage 12) coincides with the onset of cardiac propulsion and blood circulation. Thereafter, the number of PGCs increases steadily from an average of 30 at stage 13 to an average of 894 at stage 17. In all stages examined the PGCs are evenly distributed on the right and left sides of the body. Initially, the PGCs are observed throughout the existing vascular channels, particularly in the heart, great vessels, and the small vessels of the cephalic mesenchyme. Many are found in such incongruous sites as the notochord, neural and surface ectoderm, and endoderm. By stage 15, however, the majority of PGCs are concentrated in the bilateral future gonadal region, i.e., an extensive longitudinal area of mesodermal tissue surrounding the medial portion of the intraembryonic coelom caudal to the place of exit of the omphalomesenteric arteries. The pattern of distribution within the gonadal territory varies with the developmental stage and appears to be determined by the morphological and concomitant vascular changes which occur there. At stage 15, for example, the dorsal aorta is situated directly medial to the medial angle of the gonadal territory and gives off splanchnopleuric branches which form a capillary network below the floor of the coelom. Because this is the major blood supply of the gonadal region at this stage, the vast majority (98%) of the intragonadal PGCs are confined to the capillary stroma and the epithelium of this zone. The small caliber of the capillaries compared to the large size of the PGCs serves to impede circulation here so that the PGCs become halted and then are able to begin their active amoeboid movement and to invade the overlying coelomic epithelium. Subsequent medial displacement of the coelomic cavity and aorta permits a gradual overlapping of the roof of the coelom by the dorsal aorta and concomitant alterations in the vascular relationships so that by stage 18 the majority of PGCs come to occupy the medial angle and roof zones, either by migrating from the floor region or by actively penetrating the aortic wall.

The avian eye is considered to be “supreme amongst all living creatures” and capable of attaining “an order of excellence unmatched in any other species not excepting man” (Duke-Elder, 1958). Many structural and functional adaptations have become involved in the perfection of visual processes in birds which, according to Pumphrey (1948a) represent the culmination of phylogenetic development toward diurnal vision. This chapter is concerned with several of these adaptations, the most obvious of which are the oil droplet inclusions and glycogen deposits within their photoreceptors, the well-developed areae and foveae within their retina and the highly vascularized pecten within their vitreous body. A detailed description of the avian eye is not intended; excellent sources are available on this subject: Wood (1914), Slonaker (1918), Franz (1934), Walls (1942), Rochon-Duvigneaud (1943), Polyak (1957), Duke-Elder (1958), and Hodges (1974)