Neighborhood socioeconomic status and length of stay after congenital heart disease surgery.

Background and Objectives: Socioeconomic factors are associated with health outcomes and can affect postoperative length of stay after congenital heart disease (CHD) surgery. The hypothesis of this study is that patients from neighborhoods with a disadvantaged socioeconomic status (SES) have a prolonged length of hospital stay after CHD surgery. Methods: Pre- and postoperative data were collected on patients who underwent CHD surgery at the University of Maryland Medical Center between 2011 and 2019. A neighborhood SES score was calculated for each patient using data from the United States Census Bureau and patients were grouped by high vs. low SES neighborhoods. The difference of patient length of stay (LOS) from the Society for Thoracic Surgeons median LOS for that surgery was the primary outcome measure. Linear regression was performed to examine the association between the difference from the median LOS and SES, as well as other third variables. Results: The difference from the median LOS was -4.8 vs. -2.2 days in high vs. low SES groups (p = 0.003). SES category was a significant predictor of LOS in unadjusted and adjusted regression analyses. There was a significant interaction between Norwood operation and SES-patients with a low neighborhood SES who underwent Norwood operation had a longer LOS, but there was no difference in LOS by SES in patients who underwent other operations. Conclusions: Neighborhood SES is a significant predictor of the LOS after congenital heart disease surgery. This effect was seen primarily in patients undergoing Norwood operation.

Prevalence of Clinical and Subclinical Myocarditis in Competitive Athletes With Recent SARS-CoV-2 Infection: Results From the Big Ten COVID-19 Cardiac Registry.

Importance: Myocarditis is a leading cause of sudden death in competitive athletes. Myocardial inflammation is known to occur with SARS-CoV-2. Different screening approaches for detection of myocarditis have been reported. The Big Ten Conference requires comprehensive cardiac testing including cardiac magnetic resonance (CMR) imaging for all athletes with COVID-19, allowing comparison of screening approaches. Objective: To determine the prevalence of myocarditis in athletes with COVID-19 and compare screening strategies for safe return to play. Design, Setting, and Participants: Big Ten COVID-19 Cardiac Registry principal investigators were surveyed for aggregate observational data from March 1, 2020, through December 15, 2020, on athletes with COVID-19. For athletes with myocarditis, presence of cardiac symptoms and details of cardiac testing were recorded. Myocarditis was categorized as clinical or subclinical based on the presence of cardiac symptoms and CMR findings. Subclinical myocarditis classified as probable or possible myocarditis based on other testing abnormalities. Myocarditis prevalence across universities was determined. The utility of different screening strategies was evaluated. Exposures: SARS-CoV-2 by polymerase chain reaction testing. Main Outcome and Measure: Myocarditis via cardiovascular diagnostic testing. Results: Representing 13 universities, cardiovascular testing was performed in 1597 athletes (964 men [60.4%]). Thirty-seven (including 27 men) were diagnosed with COVID-19 myocarditis (overall 2.3%; range per program, 0%-7.6%); 9 had clinical myocarditis and 28 had subclinical myocarditis. If cardiac testing was based on cardiac symptoms alone, only 5 athletes would have been detected (detected prevalence, 0.31%). Cardiac magnetic resonance imaging for all athletes yielded a 7.4-fold increase in detection of myocarditis (clinical and subclinical). Follow-up CMR imaging performed in 27 (73.0%) demonstrated resolution of T2 elevation in all (100%) and late gadolinium enhancement in 11 (40.7%). Conclusions and Relevance: In this cohort study of 1597 US competitive athletes with CMR screening after COVID-19 infection, 37 athletes (2.3%) were diagnosed with clinical and subclinical myocarditis. Variability was observed in prevalence across universities, and testing protocols were closely tied to the detection of myocarditis. Variable ascertainment and unknown implications of CMR findings underscore the need for standardized timing and interpretation of cardiac testing. These unique CMR imaging data provide a more complete understanding of the prevalence of clinical and subclinical myocarditis in college athletes after COVID-19 infection. The role of CMR in routine screening for athletes safe return to play should be explored further.

Arch intervention following stage 1 palliation in hypoplastic left heart syndrome is associated with slower feed advancement: a report from the National Pediatric Quality Cardiology Improvement Collaborative.

INTRODUCTION: Infants undergoing stage 1 palliation for hypoplastic left heart syndrome may have post-operative feeding difficulties. Although the cause of feeding difficulties in these patients is multi-factorial, residual arch obstruction may affect gut perfusion, contributing to feeding intolerance. We hypothesised that undergoing arch reintervention following stage 1 palliation would be associated with post-operative feeding difficulties. METHODS: This was a retrospective cohort study. We analysed data from the National Pediatric Cardiology Quality Improvement Collaborative, which maintains a multicentre registry for infants with hypoplastic left heart syndrome discharged home following stage 1 palliation. Patients who underwent arch reintervention (percutaneous or surgical) prior to discharge following stage 1 palliation were compared with those who underwent non-aortic arch interventions after stage 1 palliation and those who underwent no intervention. Median post-operative days to full enteral feeds and weight for age z-scores were compared. Predictors of post-operative days to full feeds were identified. RESULTS: Among patients who underwent arch reintervention, post-operative days to full enteral feeds were greater than for those who underwent non-aortic arch interventions (25 versus 16, p = 0.003) or no intervention (median days 25 versus 12, p < 0.001). Arch intervention, multiple interventions, gestational age, and the presence of a gastrointestinal anomaly were predictors of days to full feeds. CONCLUSIONS: Repeat arch intervention is associated with a longer time to achieve full enteral feeding in patients with hypoplastic left heart syndrome after stage 1 palliation. Further investigation of this association is needed to understand the role of arch obstruction in feeding problems in these patients.

Comparison of cumulative dispersed energy (CDE) in femtosecond laser-assisted cataract surgery (FLACS) and conventional phacoemulsification.

PURPOSE: To compare the amount of phacoemulsification ultrasound energy used between eyes undergoing femtosecond laser-assisted cataract surgery (FLACS) and conventional phacoemulsification. METHODS: One eye of consecutive patients undergoing routine non-complicated phacoemulsification from January 2014 to December of 2015 was included in the analysis. FLACS was performed using the Alcon LenSx. Linear regression was used for analysis with type of surgery (FLACS versus conventional phacoemulsification) as the exposure and cumulative dispersed energy (CDE) as the outcome variable. Age, surgeon, eye side, and eye sequence (first versus second eye) were covariates. RESULTS: A total of 1159 surgeries met inclusion criteria. The average age of the cohort was 70.6 (SD 8.6) years, 590 cases (51%) were performed by surgeon 1, and 582 cases (50%) were right eyes. Overall, FLACS resulted in significantly lower CDE as compared to conventional phacoemulsification (ß = 0.89, 95% CI 0.83, 0.95). When stratified by eye side and surgeon, FLACS performed on left eyes operated on by surgeon 1 resulted in lower CDE as compared to conventional phacoemulsification (ß = 0.76, 95% CI 0.66, 0.87), but not for right eyes operated on by surgeon 1 (ß = 0.92, 95% CI 0.79, 1.07) or for eyes operated on by surgeons 2 or 3. CONCLUSIONS: The use of FLACS on the Alcon LenSx platform results in a small decrease in phacoemulsification energy as compared to conventional phacoemulsification in certain cases. Further study assessing optimal laser settings and surgical technique is necessary.

Angiotensin converting enzyme inhibitors and interstage failure in infants with hypoplastic left heart syndrome.

INTRODUCTION: Angiotensin converting enzyme inhibitors are commonly prescribed medications after the Norwood procedure. There are little data that can be used to determine if angiotensin converting enzyme inhibitors improve interstage outcomes in children with single ventricle defects. The objective of this study was to investigate the relationship between angiotensin converting enzyme inhibitors and interstage failure among infants born with hypoplastic left heart syndrome. METHODS: We conducted a retrospective cohort study using data from the National Pediatric Cardiology Quality Improvement Collaborative database (collected between 2008 and 2015). We used logistic regression models to assess the exposure-outcome associations and propensity score matching to account for differences in baseline patient characteristics associated with use of angiotensin converting enzyme inhibitors. RESULTS: A total of 1 487 neonates participated in the study. Thirty-nine percent of patients were prescribed angiotensin converting enzyme inhibitors after the Norwood procedure; 11% experienced interstage failure (death, heart transplantation, and not being a candidate for the second-stage surgery). Before propensity score matching, patients receiving angiotensin converting enzyme inhibitors were significantly more likely to experience interstage failure, compared to patients not on angiotensin converting enzyme inhibitors (OR = 1.44; 95% CI: 1.04, 1.99; P = 0.03). Although there was an increased odds of interstage failure among patients receiving angiotensin converting enzyme inhibitors compared to patients not receiving angiotensin converting enzyme inhibitors in the propensity score-matched cohort, this association was not significantly different (adjusted OR = 1.29; 95% CI: 0.88, 1.95; P = 0.18). CONCLUSION: Angiotensin converting enzyme inhibitor therapy did not demonstrate a beneficial effect on interstage failure among infants with hypoplastic left heart syndrome, even when patient characteristics associated with the use of angiotensin converting enzyme inhibitors were considered.

Interstage Weight Gain Is Associated With Survival After First-Stage Single-Ventricle Palliation.

BACKGROUND: Low birth and operative weight have been identified as risk factors for death after first-stage single-ventricle palliation. We hypothesize that weight gain after the first-stage operation is associated with transplant-free interstage survival to admission for the second-stage operation. METHODS: We used historical data from the National Pediatric Cardiology Quality Improvement Collaborative database to conduct a longitudinal study to assess the association between weight gain and transplant-free interstage survival. The primary predictor was weight gain. The primary outcome was transplant-free survival. We constructed a repeated-measures logistic regression model using the general estimating equation method to examine the association between weight gain and transplant-free interstage survival. RESULTS: The study population included 1,501 infants who were discharged alive from the first-stage single-ventricle palliation between June 2008 and January 2015. Patients who underwent a hybrid operation (n = 132) or were lost to follow-up (n = 11) were excluded. Transplant-free interstage survival was 90% (1,228 of 1,358). The mean weight gain was 2.5 (SD, 1.0) kg. Adjusted for age at the time of each measurement, the number of measurements, age at discharge from the first-stage operation, sex, diagnosis, postoperative arrhythmia, postoperative complications, and discharge antibiotic therapy, each 100-g increase in weight was associated with an odds ratio of transplant-free interstage survival of 1.03 (95% confidence limit, 1.01, 1.05). CONCLUSIONS: After first-stage single-ventricle palliation, interstage weight gain is significantly associated with transplant-free interstage survival.

Predictors of Prolonged Hospital Length of Stay Following Stage II Palliation of Hypoplastic Left Heart Syndrome (and Variants): Analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) Database.

The objective of this study is to identify predictors of prolonged hospital length of stay (LOS) for single ventricle patients following stage 2 palliation (S2P), excluding patients who underwent a hybrid procedure. We explore the impact of demographic features, stage 1 palliation (S1P), interstage I (IS1) management, S2P, and post-surgical care on hospital LOS following S2P. We conducted a retrospective analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database. The NPC-QIC database is an established registry of patients with hypoplastic left heart syndrome (HLHS) and its variants. It contains detailed information regarding the demographic features, S1P, IS1, S2P, and interstage 2 (IS2) management of children with HLHS and related single ventricle cardiac malformations. Between 2008 and 2012, there were 477 participants with recorded LOS data in the NPC-QIC registry. Excluding the 29 patients who underwent hybrid procedure, there were 448 participants who underwent a Norwood (or Norwood-variant procedure) as S1P. In order to be included in the NPC-QIC database, participants were discharged to home following S1P and prior to S2P. We found that postoperative LOS among the 448 S2P procedure recipients is most strongly influenced by the need for reoperation following S2P, the need for an additional cardiac catheterization procedure following S2P, the use of non-oral methods of nutrition (e.g., nasogastric tube, total parental nutrition, gastrostomy tube), and the development of postoperative complications. Factors such as age at the time of S2P, the presence of a major non-cardiac anomaly, site participant volume, IS1 course, the type and number of vasoactive agents used following S2P, and the need for more than 1 intensive care unit (ICU) hospitalization (following discharge to the ward but prior to discharge to home) were significant predictors by univariate analysis but not by multivariate analysis. We excluded participants undergoing the hybrid procedure as S1P from this analysis given that the S2P following the initial hybrid is typically a more complicated procedure. Hospital LOS following S2P among children undergoing the Norwood or Norwood-variant procedure as S1P is most strongly influenced by events following S2P and not demographic or S1P factors. Factors most predictive of prolonged LOS include the need for reoperation, the need for an additional cardiac catheterization procedure following S2P, the need for non-oral methods of nutrition, and the development of postoperative complications.

Impact of prenatal diagnosis in survivors of initial palliation of single ventricle heart disease: analysis of the National Pediatric Cardiology Quality Improvement Collaborative database.

Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31-46), and 66% had variants of hypoplastic left heart syndrome. PD was made in 445 (75%), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p < 0.001), there were no differences in birth weight, the presence of major syndromes or other organ system anomalies. Those without PD were more likely to have atrioventricular valve regurgitation (p = .002), ventricular dysfunction (p = 0.06), and pre-operative risk factors including acidosis (p < 0.001), renal insufficiency (p = 0.007), and shock (p = 0.05). Post-operative ventilation was shorter in the PD group (9 vs. 12 d, p = 0.002). Other early post-operative outcomes, interstage course, and outcomes at BDG were similar between groups. In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD showed significant inter-site variation and was associated with improved pre-operative status and shorter duration of mechanical ventilation. The significance of such associations merits further study.

Predictors of prolonged length of intensive care unit stay after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

The objective of this study is to identify predictors of prolonged intensive care unit (ICU) length of stay (LOS) for single ventricle patients following Stage I palliation. We hypothesize that peri-operative factors contribute to prolonged ICU stay among children with hypoplastic left heart syndrome (HLHS) and its variants. In 2008, as a part of the Joint Council on Congenital Heart Disease initiative, the National Pediatric Cardiology-Quality Improvement Collaborative established a data registry for patients with HLHS and its variants undergoing staged palliation. Between July 2008 and August 2011, 33 sites across the United States submitted discharge data essential to this analysis. Data describing the patients, their procedures, and their hospital experience were entered. LOS estimates were generated. Prolonged LOS in the ICU was defined as stay greater than or equal to 26 days (i.e., 75th percentile). Statistical analyses were carried out to identify pre-operative, operative, and post-operative predictors of prolonged LOS in the ICU. The number of patients with complete discharge data was 303, and these subjects were included in the analysis. Univariate and multivariate analyses were performed. Multivariate analysis revealed that lower number of enrolled participants (e.g., 1-10) per site, the presence of pre-operative acidosis, increased circulatory arrest time, the occurrence of a central line infection, and the development of respiratory insufficiency requiring re-intubation were associated with prolonged LOS in the ICU. Prolonged LOS in the ICU following Stage I palliation in patients with HLHS and HLHS variant anatomy is associated with site enrollment, circulatory arrest time, pre-operative acidosis, and some post-operative complications, including central line infection and re-intubation. Further study of these associations may reveal strategies for reducing LOS in the ICU following the Norwood and Norwood-variant surgeries.

Hypoplastic left heart syndrome: current considerations and expectations.

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

Variation in preoperative and intraoperative care for first-stage palliation of single-ventricle heart disease: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

BACKGROUND AND METHODS: As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. RESULTS: From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. CONCLUSIONS: In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.

Variation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.

BACKGROUND AND METHODS: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. RESULTS: Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common. CONCLUSIONS: Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.

Development of a pediatric cardiology quality improvement collaborative: from inception to implementation. From the Joint Council on Congenital Heart Disease Quality Improvement Task Force.

While clinical outcomes in pediatric cardiac disease have improved in recent years, marked institutional and individual cardiology practice variability exists. Quality improvement science has demonstrated that reducing process variation leads to more favorable outcomes, safer practices, cost savings, and improved operating efficiency. This report describes the process undertaken to develop the first collaborative quality improvement project of the Joint Council on Congenital Heart Disease. The project chosen aims to reduce mortality and improve the quality of life of infants with hypoplastic left heart syndrome during the interstage period between discharge from the Norwood procedure and admission for the bidirectional Glenn procedure. The objective of this special article is to inform the pediatric cardiology and cardiac surgery communities of the project to help ensure that the early work by the project pilot participants will spread to clinicians caring for children with cardiovascular disease. It is anticipated that this project will add to our understanding of care for this challenging group of children with hypoplastic left heart syndrome, identifying clinical care changes with the potential to lead to improvements in outcome. It will also introduce the field of pediatric cardiology to the science of collaborative quality improvement and assist in reducing clinical process variation and improving patient outcomes across centers. Finally, it will establish an ongoing network of pediatric cardiologists and their teams linked through a longitudinal data set and collaboration for improvement and research.

Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum.

OBJECTIVES: Right ventricular sinus myectomy has been proposed for pulmonary atresia with intact ventricular septum for morphology falling within the uncertain area for eventual biventricular repair. Our objective was to evaluate right ventricular sinus myectomy by characterizing the morphologic spectrum of these patients, determining whether biventricular repair was achieved, ascertaining growth of right-sided structures, and assessing survival. METHODS: We evaluated medical records, all imaging studies, and follow-up data (complete in all but 1 patient) from 43 patients with pulmonary atresia with intact ventricular septum treated from October 1993 to July 2005, 16 of whom underwent right ventricular sinus myectomy. Serial echocardiographic measurements of right-sided cardiac structures were converted to Z values to estimate their growth relative to somatic growth. RESULTS: Patients undergoing right ventricular sinus myectomy had mild-to-moderate right ventricular size diminution (grade -1.2 +/- 3.2) and a tricuspid valve Z value of -4.9 +/- 1.9. Thirteen (87%) of the 16 patients achieved biventricular repair. After right ventricular sinus myectomy, mean right ventricular cavity size grade increased to 1.4 +/- 0.66, but the tricuspid valve Z value did not change appreciably over time. Five-year survival after sinus myectomy was 85%; late deaths were in patients with the smallest tricuspid valves at presentation (Z value < -7). CONCLUSIONS: Right ventricular sinus myectomy in the uncertain area for biventricular repair of pulmonary atresia with intact ventricular septum leads to immediate increase in right ventricular volume. It, in combination with establishing right ventricle-pulmonary trunk continuity, allowed early biventricular repair in 87% of patients. However, tricuspid valve growth in relation to somatic growth was minimal. Thus, small tricuspid valve size might limit the long-term success of biventricular repair achieved by means of right ventricular sinus myectomy.

Can pediatric electrophysiologists safely perform electrophysiology studies on adults with congenital heart disease?

INTRODUCTION: Adults with congenital heart disease (ACHD) are known to have arrhythmias. The purpose of this study was to review the safety and efficacy of a pediatric electrophysiologist performing electrophysiology studies (EPS) on ACHD. METHODS: All ACHD >18 years of age who underwent an EPS performed by a pediatric electrophysiologist between 1995 and 2004 were included. Patient records were reviewed for demographics, cardiac diagnosis, surgery, arrhythmia, and catheterization issues such as vascular access, fluoroscopy time, complications, procedure performed, and success rates. RESULTS: The study identified 70 patients who underwent 93 EPS: 42 with ventricular tachycardia (VT) and 28 with supraventricular tachycardia (SVT). With respect to safety, vascular access was obtained in 100% of patients, fluoroscopy time averaged 34.2 minutes, and complications occurred in 3 patients (4%) including permanent (1) or transient (1) atrio-ventricular node injury and coagulum on the catheter (1), but no deaths. With respect to efficacy, 30 patients had an attempted radiofrequency ablation that was successful in 21 of 28 (75%) with SVT and 1 of 2 (50%) with VT. CONCLUSIONS: (1) ACHD can safely undergo an electrophysiology study performed by pediatric electrophysiologists, with low fluoroscopy times and few complications. (2) Success rates for ablation in this patient population approach 75%.

Aortic valve repair for congenital abnormalities of the aortic valve.

BACKGROUND: Due to shortcomings of any valve replacement, repairing and retaining the native valve may be beneficial for congenital aortic valve disease. METHODS: Retrospective review of data and follow-up of aortic valve repair from a single institution. RESULTS: From 1993 to 2001, 56 patients underwent aortic valve repair [median age 13.4 years (range 1 day to 45 years)]. The predominant aortic valve lesion was mixed aortic stenosis/aortic insufficiency 25 (45%), aortic insufficiency 24 (43%) and aortic stenosis 7 (13%). Repair techniques included sub-commissural plication 36 (64%), commissurotomy 24 (43%), cusp plication 15 (27%), pericardial patch cusp extension 8 (14%) and resuspension of commissures 4 (7%). Most patients (88%) required a combination of techniques; 61% required additional procedures. Hospital survival was 55/56 [98%; (95% CI 91-100%)] no patient was discharged on anticoagulation for aortic valve pathology. Fifty-three patients [95%; (95% CI 85-98%)] remain alive after a median follow-up of 37 months; four survivors required aortic valve replacement and two required repeat aortic valve repair [84%; (95% CI 72-91%) reintervention-free survival]. CONCLUSIONS: (1) In this study, aortic valve repair for congenital abnormalities avoided reoperation in the majority of patients, avoided anticoagulation and retained growth potential of the valve. (2) Repeat aortic valve repair or replacement was used to treat subsequent valve deterioration.

Apical right ventriculotomy for closure of apical ventricular septal defects.

BACKGROUND: Apical ventricular septal defects (VSDs) are difficult to visualize through a transatrial approach, while the use of a left ventriculotomy may result in progressive ventricular dysfunction. Transcatheter closure has not been reliable, especially in small infants. Apical right ventriculotomy provides good exposure, preserves left ventricular function, and allows secure closure of apical VSDs. METHODS: From November 1994 through April 2002, nine children, median age 8 months (range, 74 days to 2.5 years) underwent 10 operations for VSD closure via apical right ventriculotomy (one patient with a residual defect underwent successful VSD closure via a second apical right ventriculotomy). Two patients were status post pulmonary artery banding and two patients had previous unsuccessful attempts at closure via a transatrial approach. RESULTS: There was no hospital mortality; there were 2 late deaths (78% survival; 95% confidence interval [CI], 45% to 94%), 3 months and 4 years postoperatively. Postoperative echocardiography demonstrated no residual VSDs in 3 and insignificant residual VSDs in 4 of the survivors. All survivors currently exhibit normal biventricular function during a median follow-up of 25 months (range, 11 to 104 months). CONCLUSIONS: Apical right ventriculotomy provides excellent exposure allowing safe and effective closure of apical VSDs. The observed late morbidity and mortality reflects the complexity that often exists in these cases due to additional irreparable lesions.

Risk factors for surgical site infections after pediatric cardiovascular surgery.

BACKGROUND: Although risk factors for surgical site infection (SSI) after cardiovascular (CV) surgery have been well-documented among adults, few studies have been conducted in children. We performed a case-control study to identify risk factors for hospitalized SSI in children undergoing CV surgery. METHODS: National Nosocomial Infections Surveillance System criteria were used prospectively to identify cases of hospitalized SSI in patients who underwent CV surgery. Seventy-nine patients who underwent CV surgery without hospitalized SSI were randomly selected as controls. Cases were compared with controls to determine preoperative, intraoperative and postoperative risk factors for hospitalized SSI. Multivariable logistic regression was performed. RESULTS: An SSI developed in 19 of the 826 patients who underwent CV surgery (2.3 cases per 100 surgeries) during the study period. Infection was limited to soft tissue in 12, whereas deeper infection occurred in 7. Causative agents included Staphylococcus aureus (11), coagulase-negative Staphylococcus (5) and Escherichia coli (2). One patient did not have a pathogen isolated. In a multivariable analysis, duration of surgery (odds ratio, 1.4; 95% confidence interval, 1.2 to 1.8) and age <1 month (odds ratio, 14; 95% confidence interval, 3.3 to 58.4) were independently associated with SSI. CONCLUSIONS: Age <1 month and longer duration of surgery were independently associated with hospitalized SSI after CV surgery in children.

Surgical treatment of congenital mitral stenosis: medium-term results

OBJETIVO: Analisar a evolução de pacientes portadores de estenose mitral congênita (EmiC) submetidos a tratamento cirúrgico, buscando estudar os fatores desfavoráveis da plastia valvar e os seus marcadores de durabilidade. MÉTODO: De 1989 a 2002, 23 pacientes foram submetidos a tratamento cirúrgico da EmiC, excluindo aqueles com defeito do septo atrioventricular, e formas univentriculares. A idade mediana foi de 15,5 meses (variação de 2 a 204), e o peso mediano foi de 11 kg (variação 4,5 a 51,6 kg). Anomalias associadas estiveram presentes em 17 (73,9 por cento), complexo de Shone em nove (39,1 por cento) e hipertensão pulmonar em 14 (60,9 por cento). A estenose mitral era grave em 14 (60,9 por cento) pacientes, e moderada no restante (gradiente transmitral mediano de 16 mmHg, variação 8,5 a 32). A plastia valvar foi realizada em 18 (78,3 por cento) pacientes e a troca em cinco (21,7 por cento). As técnicas de plastia incluiram a papilarotomia (n=10), ressecção de membrana supravalvar (n=9) e comissurotomia (n=8). Doze (52,2 por cento) pacientes requereram procedimentos associados. RESULTADOS: Não houve óbitos precoces ou tardios num seguimento médio foi de 58,5 ñ 46,7 meses (variação 1 a 156 meses). O tempo médio de hospitalização foi de 12,7 ñ 8,2 dias. Não foram encontrados fatores de risco associados a insucesso da plastia valvar. A curva atuarial livre de reoperaçäes em cinco anos foi de 67,1 por cento (IC 95 por cento: 56,8 por cento a 77,4 por cento). O grupo submetido a plastia valvar necessitou de reoperação em oito (44,4 por cento) pacientes, sendo dois precoces e seis tardios, comparados com uma (20 por cento) reoperação no grupo submetido à troca valvar. A presença de hipertensão pulmonar no pré-operatório esteve relacionada significativamente (p<0,005) a maiores taxas de reoperação. Todos os pacientes, exceto dois, encontram-se em classe funcional I da NYHA e o ecocardiograma mostra no máximo insuficiência e/ou estenose mitral leve. CONCLUSåES: As reoperaçäes foram o maior fator de morbidade no seguimento a médio prazo e a hipertensão pulmonar pôde ser considerada o fator adverso de durabilidade da plastia mitral, a qual foi o tratamento de escolha para a estenose mitral congênita.