Clinical update on the hybrid comprehensive stage II operation.

Objective: We previously described the hybrid comprehensive stage II operation as an alternate surgical procedure for a subset of patients with single ventricle congenital heart disease with adequate native ascending aortic outflow. Here we provide a clinical update on the 4 patients who have undergone this procedure. Methods: After undergoing a hybrid approach to the stage I Norwood palliation, the hybrid comprehensive stage II procedure was performed with an incision to the main pulmonary artery (PA), dilation of the ductal stent, creation of a stented baffle between the branch PAs, and a bidirectional Glenn connection. With this approach, dissection of the distal arch and creation of a Damus-Kaye-Stansel anastomosis was avoided. A standard Fontan procedure was planned after the usual period of growth. Results: The first patient, who had trisomy 21 and elevated PA pressures, died postoperatively due to left PA thrombosis. The subsequent 3 patients survived the procedure and remain clinically well. All have required catheterizations for reintervention on their stented intrapulmonary baffles and ductal arches, and all have undergone successful completion of their Fontan procedures. Conclusions: The hybrid comprehensive stage II is a feasible, less complex alternative to the conventional comprehensive stage II operation in a subset of patients with single ventricle physiology. Early postoperative anticoagulation therapy to avoid PA thrombosis is recommended, and restenting of the ductal arch is anticipated. Although the long-term consequences of separate outflow tracts supplying the upper and lower body is unknown, the 3 surviving patients with this circulation are doing well with their Fontan circulation at midterm follow-up.

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair. METHODS: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled. Demographic, morphologic, operative, imaging, and ischemia-related data were analyzed. RESULTS: There were 395 of 682 (57%) surgical patients (45 centers, median follow-up 2.8 years). In addition to primary repair (87% unroofing, 26% commissural manipulation), 13 patients had 15 coronary-related reoperations. Of 358 patients with pre/postoperative aortic insufficiency assessment, 27 (8%) developed new mild or greater aortic insufficiency postoperatively, and 7 (2%) developed new moderate or greater aortic insufficiency. Freedom from mild aortic insufficiency differed in those with versus without commissural manipulation (85%/91% at 6 months, 83%/90% at 1 year, and 77%/88% at 3 years, respectively) (P = .05). Of 347 patients with preoperative/postoperative ejection fraction, 6 (2%) developed new abnormal ejection fraction (<50%) within 30 days of surgery which persisted. Although 64 of 395 patients (16%) had preoperative ischemia, after surgery 51 of 64 patients (80%) no longer had ischemia (13 = new postoperative ischemia, P < .0001). Four patients died postoperatively (preoperatively 2 asymptomatic, 1 symptomatic, 1 in extremis). Composite surgical adverse event rates were 7% to 13% in the entire cohort (increasing/decreasing by presentation/anatomy/repair strategy). CONCLUSIONS: Anomalous aortic origin of a coronary artery surgery may relieve ischemia with low mortality; however, it can result in a variety of important morbidities, varying by the group evaluated. Strategies avoiding commissural manipulation may decrease the risk of developing aortic insufficiency. Understanding these risks should inform surgical decision-making and support the need for standardized assessment and management.

Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease.

OBJECTIVE: To develop a risk score to predict mortality or transplant in the interstage period. BACKGROUND: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. METHODS: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ("learning cohort") to build a risk score for the composite endpoint, that was validated in the remaining patients ("validation cohort"). RESULTS: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ("NEONATE" score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. CONCLUSIONS: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.

Pulmonary artery banding in complete atrioventricular septal defect.

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.

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P < 0.0001). Aortic arch hypoplasia and coarctation were common across all four groups, while those with AA/MA and AA/MS had the smallest ascending aorta diameters. Flow in the ascending aorta was retrograde in 43% and 10% of the patients with AS/MS and iAS, respectively. The right ventricle was apex forming in 100% of patients with AA/MA and AA/MS, 96% with AS/MS and 70% with iAS (P < 0.0001). Moderate to severe tricuspid regurgitation was present in 13% of all patients. CONCLUSION: This large multi-institutional study generates insight into the distribution of the functional and morphologic spectrum in patients with critical left-sided heart disease and identifies differences in these functional and morphologic characteristics between the main anatomic subtypes of critical left heart obstruction.

Determinants of Physician, Sonographer, and Laboratory Productivity: Analysis of the Third Survey from the American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity.

BACKGROUND: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity was formed in 2011 to study institutional factors that could influence the clinical productivity of physicians and sonographers in academic pediatric echocardiography laboratories. In the previous two surveys, staff clinical productivity remained stable while total echocardiography volumes increased. This third survey was designed to assess how clinical productivity is associated with laboratory infrastructure elements such as training, administrative tasks, quality improvement, research, and use of focused cardiac ultrasound (FCU). METHODS: Survey questions were sent by e-mail to North American laboratories. The aims were to assess (1) educational and training obligations, (2) academic productivity and research, (3) laboratory medical director satisfaction, (4) quality improvement, (5) laboratory leadership roles, and (6) impact and use of FCU. Survey responses were compared with clinical productivity metrics defined in the first two surveys. RESULTS: There were 38 responses. Academic productivity was higher at institutions with more dedicated imaging personnel, personnel with dedicated protected academic time, and advanced imaging fellows. Academic productivity did not correlate with clinical productivity and was not significantly affected by the presence of dedicated research sonographers. The satisfaction level of laboratory medical directors was related to dedicated administrative time and an administrative stipend. The majority of administrative roles were tasked to the laboratory medical director with support of the technical director. FCU was listed as a hospital privilege at four institutions (13%). Twenty-two (58%) were training FCU providers in one or more subspecialties. FCU was not associated with clinical or academic productivity. CONCLUSIONS: This third survey gathered supplemental data to complement the clinical productivity data collected from the first two surveys. Together, the results of these surveys further describe the range of factors that can affect North American academic pediatric echocardiography laboratories.

Optimizing patient care and outcomes through the congenital heart center of the 21st century.

Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a state-of-the-art congenital heart center of the 21st century. Care center collaboration helps overcome the barrier of relative small volumes at any given program. Patients and families have become active collaborative partners with care centers in the definition of acute and longitudinal outcomes and our quality metrics. Understanding programmatic metrics that create an environment to provide outstanding congenital heart care will allow centers to improve their structure, processes and ultimately outcomes, leading to an increasing number of centers that provide excellent care. This manuscript provides background, as well listing of proposed specialty domain quality metrics for centers, and thus serves as an updated baseline for the ongoing dynamic process of optimizing care and realizing patient value.

Surgical Palliation of Right Aortic Arch With an Isolated Innominate Artery From the Left Ductus Arteriosus.

A right aortic arch with an isolated left innominate artery from the left patent ductus arteriosus is a rare arch anomaly, and establishing continuity between the innominate artery and aorta can be challenging. We describe repair of this lesion in a three-week-old male using an autologous pedicle flap of ascending aorta as well as a homograft patch as the roof to recreate continuity between the aorta and left innominate artery.

Determinants of Pediatric Echocardiography Laboratory Productivity: Analysis from the Second Survey of the American Society of Echocardiography Committee on Echocardiography Laboratory Productivity.

BACKGROUND: The American Society of Echocardiography Committee on Pediatric Echocardiography Laboratory Productivity aimed to study factors that could influence the clinical productivity of physicians and sonographers and assess longitudinal trends for the same. The first survey results indicated that productivity correlated with the total volume of echocardiograms. METHODS: Survey questions were designed to assess productivity for (1) physician full-time equivalent (FTE) allocated to echocardiography reading (echocardiograms per physician FTE per day), (2) sonographer FTE (echocardiograms per sonographer FTE per year), and (3) machine utilization (echocardiograms per machine per year). Questions were also posed to assess work flow and workforce. RESULTS: For fiscal year 2013 or academic year 2012-2013, the mean number of total echocardiograms-including outreach, transthoracic, fetal, and transesophageal echocardiograms-per physician FTE per day was 14.3 ± 5.9, the mean number of echocardiograms per sonographer FTE per year was 1,056 ± 441, and the mean number of echocardiograms per machine per year was 778 ± 303. Both physician and sonographer productivity was higher at high-volume surgical centers and with echocardiography slots scheduled concordantly with clinic visits. Having an advanced imaging fellow and outpatient sedation correlated negatively with clinical laboratory productivity. Machine utilization was greater in laboratories with higher sonographer and physician productivity and lower for machines obtained before 2009. CONCLUSION: Measures of pediatric echocardiography laboratory staff productivity and machine utilization were shown to correlate positively with surgical volume, total echocardiography volumes, and concordant echocardiography scheduling; the same measures correlated negatively with having an advanced imaging fellow and outpatient sedation. There has been no significant change in staff productivity noted over two Committee on Pediatric Echocardiography Laboratory Productivity survey cycles, suggesting that hiring practices have matched laboratory volume increases.

Hybrid approach to the comprehensive stage II operation in a subset of single-ventricle variants.

OBJECTIVE: The objective of a hybrid approach to staged palliation of single-ventricle anomalies is designed to minimize the trauma of the first stage. However, the second stage is a complex procedure that may negate the advantages of the first stage. We sought to devise a "hybrid" approach to the second stage when aortic outflow is expected to remain unobstructed. METHODS: The procedure involves a simple incision into the main pulmonary artery, dilation/stenting of the ductal continuation, formation of a stented baffle between the branch pulmonary arteries' orifices, and a bidirectional Glenn connection. It avoids dissection of the distal arch and ductal continuation and obviates the need for a Damus-Kaye-Stansel connection. We carried out this procedure in 2 patients, one with unbalanced atrioventricular canal and the other with mitral atresia. RESULTS: Both patients underwent an uncomplicated operative procedure. Both patients were successfully weaned from the ventilator, with no clinically evident neurologic injury. The first patient died of complications related to thrombosis of the left pulmonary artery before initiation of anticoagulation. The second patient is alive and well 1 year postoperation with no obstruction to either systemic or pulmonary flow and no baffle leak and good right ventricle function. CONCLUSIONS: This hybrid comprehensive stage II operation appears feasible and technically simpler than the conventional comprehensive stage II procedure. It is applicable to a subset of single-ventricle cases in which aortic outflow is anticipated to remain unobstructed. We recommend early postoperative anticoagulation to avoid early left pulmonary artery thrombosis.

Remote delivery of congenital cardiac magnetic resonance imaging services: a unique telemedicine model.

Cardiac magnetic resonance imaging (CMRI) is increasingly utilized in the management of patients with congenital heart disease. Unfortunately, the expertise to perform and interpret these studies is not universally available, despite an increasing population of congenital heart survivors. This retrospective analysis describes our experience providing on-site CMRI services compared with providing the same services over a geographic distance of 250 miles. There were 83 local scans with both physician and patient on-site compared with 91 scans controlled by a physician geographically remote from the patients. The patients were well-matched for age, sex, study duration, scan type, and history of prior cardiac intervention. There was no difference in use of deep sedation or diazepam for anxiolysis, or use of atropine for arrhythmia suppression. There were no patient safety issues and there was satisfaction on the part of the referring physicians who were able to obtain more timely studies, as well as the remote-scanning physicians who had a workflow comparable with the local scans, but no lost travel time. This experience suggests that remote delivery of cardiac MRI services for the congenital heart population is feasible and can be done with comparable success and safety to a traditional "local" model. We also suggest the configuration to provide such remote CMRI services with commercially available hardware and software.

Results of the U.S. Food and Drug Administration continued access clinical trial of the GORE HELEX septal occluder for secundum atrial septal defect.

OBJECTIVES: This report describes the immediate, 1-, and 5-year follow-up results of the U.S. Food and Drug Administration Continued Access clinical trial of the GORE HELEX Septal Occluder (W. L. Gore & Associates, Inc., Flagstaff, Arizona) for closure of secundum atrial septal defect. BACKGROUND: The trial was conducted between May 2003 and August 2006 to allow continued enrollment in a trial of the investigational device during review of data from the Pivotal Trial. Devices with hydrophilic coating on the expanded polytetrafluoroethylene to improve echocardiographic visualization were first used in this trial. METHODS: A total of 137 devices were implanted in 137 patients at 13 US institutions. Evaluations were scheduled at 1, 6, and 12 months for the initial trial and at 36 and 60 months for a later extension of the trial in those who consented to longer term evaluations. RESULTS: Twelve-month follow-up was completed on 122 of 126 patients with implantations, and 5-year follow-up on 83 of 95 patients who agreed to the trial extension. The overall clinical success rate was 96.7%, and the major adverse event rate 3.6%. Wire frame fractures were seen in 11.7% of patients with no clinical symptoms. A trivial, clinically insignificant leak was seen, or could not be ruled out, in 26.6% of patients at the 5-year evaluation, but no clinically significant leaks were seen. No patient experienced an erosion or sudden catastrophic event. CONCLUSIONS: The immediate, 1-, and 5-year follow-up outcomes of the Continued Access clinical trial continue to demonstrate that the GORE HELEX Septal Occluder is a safe and effective transcatheter occluder for repair of ostium secundum atrial septal defect.

Results of the U.S. multicenter pivotal study of the HELEX septal occluder for percutaneous closure of secundum atrial septal defects.

OBJECTIVES: This study sought to compare the safety and efficacy of the HELEX septal occluder (HSO) with surgical repair of atrial septal defect (ASD). BACKGROUND: The HSO is a low-profile, double-disk occluder device for percutaneous closure of secundum ASD. METHODS: Patients were enrolled (HSO arm prospectively, surgery arm prospectively/retrospectively) from 14 U.S. sites and followed up for 12 months postprocedure. Investigator-reported outcomes were evaluated, including closure success (no or clinically insignificant residual shunt) and the incidence of adverse events. The first 3 HSO patients at each site were considered training cases and were excluded from analysis. RESULTS: Between March 2001 and April 2003, 119 nontraining cases received an HSO and 128 had surgical repair. The groups were similar with statistical but clinically unimportant differences in median age, weight, and preprocedural echocardiographic defect size. Anesthesia time and hospital stay were significantly shorter in the HSO group. Closure success, defined as complete closure or a clinically insignificant residual shunt, was similar in both groups. Major and minor adverse events rates were not statistically different. The most common major adverse events for the HSO group was device embolization requiring catheter retreival (1.7%), and in the surgery group was postpericardiotomy syndrome (6.3%), including one death because of tamponade. The primary end point, clinical success, a composite of closure success and no major adverse events at 12 months, satisfied the noninferiority hypothesis comparing device closure with surgery. CONCLUSIONS: Closure of ASD with the HELEX septal occluder is safe and effective when compared with surgical repair, with reduced anesthesia time and hospital stay. (U.S. Multicenter Pivotal Study of the HELEX Septal Occluder for Percutaneous Closure of Secundum Atrial Septal Defects; this study was approved by the Food and Drug Administration before the National Institutes of Health website was active, so there is not a URL or registration number.).