Congenital Heart Surgeons Research Articles

Epicardial Echocardiography in Pediatric and Congenital Heart Surgery.

Epicardial echocardiography (e-echo) is a useful approach to intraoperative imaging for the smallest patients and in those with contraindications to transesophageal echocardiography (TEE). The e-echo has additional advantages that include improved visualization of anterior and vascular structures and three-dimensional capabilities. In this review, we describe the advantages and disadvantages of e-echo versus TEE for pediatric and congenital heart surgery with the goal of enhancing the utility and feasibility of the former among congenital heart surgeons. We also review the technique for performance of an e-echo and provide demonstrations of the images obtained for select lesions.

Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons' Society study

ObjectivesWe sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery. MethodsWe enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports. ResultsThere were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features. ConclusionsAnatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

The Congenital Heart Technical Skill Study: Rationale and Design.

We report the rationale and design for a peer-evaluation protocol of attending congenital heart surgeon technical skill using direct video observation. All surgeons contributing data to The Society of Thoracic Surgeons-Congenital Heart Surgery Database (STS-CHSD) are invited to submit videos of themselves operating, to rate peers, or both. Surgeons may submit Norwood procedures, complete atrioventricular canal repairs, and/or arterial switch operations. A HIPPA-compliant website allows secure transmission/evaluation. Videos are anonymously rated using a modified Objective Structured Assessment of Technical Skills score. Ratings are linked to five years of contemporaneous outcome data from the STS-CHSD and surgeon questionnaires. The primary outcome is a composite for major morbidity/mortality. Two hundred seventy-six surgeons from 113 centers are eligible for participation: 83 (30%) surgeons from 53 (45%) centers have agreed to participate, with recruitment ongoing. These surgeons vary considerably in years of experience and outcomes. Participants, both early and late in their careers, describe the process as "very rewarding" and "less time consuming than anticipated." An initial subset of 10 videos demonstrated excellent interrater reliability (interclass correlation = 0.85). This study proposes to evaluate the technical skills of attending pediatric cardiothoracic surgeons by video observation and peer-review. It is notable that over a quarter of congenital heart surgeons, across a range of experiences, from almost half of United States centers have already agreed to participate. This study also creates a mechanism for peer feedback; we hypothesize that feedback could yield broad and meaningful quality improvement.

Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study

ObjectivesImproved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. MethodsFollow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. ResultsDomain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. ConclusionsMorbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability

ObjectiveArch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. MethodsFrom 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. ResultsOf the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). ConclusionsInterdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.

OC53 OPERATIVE RISK OF CARDIAC SURGERY FOR ADULT PATIENTS WITH CONGENITAL HEART DISEASE IN THE ADULT

Background and aim: Advances in paediatric cardiac surgical and medical care have led to vastly increased survival of patients with congenital heart disease (CHD). We would like to explore and analyse data from the European Congenital Heart Surgeons Association (ECHSA) database with the aim of evaluating the risk of surgery for treating adult patients with CHD. Methods: We included in our study data from 19589 surgical procedures. We excluded 1389 procedured including hemodynamic procedures, anesthesiologist procedures). Outcome included early and late mortality according to the main surgical procedure. Results: fx1 Conclusions: Surgical risk for adult patients with CHD is low, either considering early and late mortality. Mortality continue to be high for particular categories of patients.

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

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. 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. 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.

Anatomy of the Functionally Univentricular Heart.

The world of pediatric cardiac surgery and cardiac surgery as a whole lost one of the great pioneers with the passing, at the beginning of 2018, of Francis Fontan. Hence to add to the recognition of his achievements, the European Congenital Heart Surgeons Association (ECHSA) has established a lecture to be given in his memory at their annual meetings. It was a significant honor and privilege to be invited to present the initial lecture. In this report, we describe the essence of the presentation. Many patients are now palliated by construction of the Fontan circulation. Very few of those put forward for this operative procedure have anatomically univentricular hearts. It remains frequent, nonetheless, to find accounts of many patients allegedly having "single" ventricles. We discuss the background to this illogical approach to description of hearts having one big and one small ventricle, showing that those with normal hearts have a single left ventricle, albeit co-existing with a single right ventricle. We show that analysis of the ventricular mass in tripartite fashion produces much needed clarity in the appropriate description of the ventricular mass in those increasingly submitted for construction of the Fontan circulation. We emphasize that although it was patients with univentricular atrioventricular connections who were the first to benefit from the procedure, the majority of patients now have biventricular atrioventricular connections, although the hypoplastic ventricle possesses all three of its normal components. We show that description of the ventricular arrangement as being functionally, or physiologically, univentricular provides logic in what had previously been an illogical environment.

Incremental History of the Congenital Heart Surgeons' Society (2014-2018).

The history of the first 41 years of the Congenital Heart Surgeons Society (CHSS) was recorded in 2015 which chronicled the metamorphosis of a small informal meeting into a mature organization with bylaws, officers, committees, funded research, the Kirklin-Ashburn Fellowship, and a vision to become the premier organization of congenital heart surgery in North America. Chief among these transformations was the implementation and development of the CHSS Data Center. Member participation, fellowship education, and significant outcomes research have been the hallmark of the CHSS. This incremental historical review highlights continued CHSS sentinel advances. Fifty-three CHSS Data Center manuscripts have been published. Citation scores (number of literature citations that each manuscript has accrued) have been collated and analyzed by cohort study. The average citation score for all manuscripts was 75.4 ± 76.3 (range: 1-333). The Kirklin/Ashburn Fellowship continues to thrive with academic achievements and generous contributions to the endowment. The World Journal of Pediatric and Congenital Heart Surgery has been adopted as the official organ of the CHSS. A Past President's Dinner has been inaugurated serving as a senior advisory committee to the Executive Council. Toronto Work Weekends continue. Congenital Heart Surgeons Society growth has accrued to 159 active members and 82 institutional members. Future considerations include the size, content, and duration of the annual meeting; the potential for increased membership; and political penetrance into national cardiothoracic governing organizations regarding committee appointments, executive council representation, and education initiatives. Congenital Heart Surgeons Society has achieved numerous advances during this incremental period.

Development of a Congenital Heart Surgery Composite Quality Metric: Part 1—Conceptual Framework

Current pediatric and congenital heart surgery quality measures focus on operative mortality, and numerous stakeholders are interested in more comprehensive measures. This report describes the background, rationale, and conceptual framework related to the development of the first composite quality metric in the field. A multidisciplinary panel reviewed methodology and framework related to quality measurement and several composite quality measures across adult cardiac surgery and other fields. The panel subsequently developed methodology and selected measures for a congenital heart surgery composite measure and reviewed potential advantages and limitations. Individual measures considered for potential inclusion in the composite were reviewed within the context of Donabedian's triad and the Institute of Medicine quality domains. Decisions were made through group consensus. The final composite measure selected is comprised of two domains: (1) a mortality domain (operative mortality) and (2) a morbidity domain (the 6 major complications endorsed by The Society of Thoracic Surgeons and Congenital Heart Surgeons Society plus cardiac arrest, and postoperative length of stay). Potential advantages include the more comprehensive view of quality compared with mortality alone and improvements in discrimination of hospital performance through increasing the number of end points. Potential limitations include the lack of longer term outcomes and challenges related to case-mix adjustment. We have applied and adapted conceptual framework and methodology related to composite quality measures across other fields to congenital heart surgery. The composite quality metric created is inclusive of both morbidity and mortality, and expands our view of quality in this patient population.

Abstracts of the 2018 Meeting of the European Congenital Heart Surgeons Association, Cascais, Portugal, May 31 to June 2, 2018.

Restricted accessAbstractFirst published online August 29, 2018Abstracts of the 2018 Meeting of the European Congenital Heart Surgeons Association, Cascais, Portugal, May 31 to June 2, 2018Volume 9, Issue 5https://doi.org/10.1177/2150135118793089

Strengthening International Collaboration in Congenital Heart Surgery.

This paper was presented by the author at the 2016 biannual scientific meeting in Abu dhabi of the World Society for Pediatric and Congenital Heart Surgery as his incoming presidential address. In addition to listing the accomplishments of the World Society including several successful international scientific meetings and regional meetings, the Society has established the only journal devoted to congenital heart surgery and is in the process of establishing the new World Society database. The mission statement of the World Society emphasizes equitable global management of patients with congenital heart disease and includes an emphasis on excellence in education, research and community service. The Society will promote international exchange of trainees in congenital heart surgery, the facilitation of international teaching and treatment missions as well as a system for global certification of congenital heart surgeons to facilitate international movement of surgeons to areas of greatest need.

The Congenital Heart Surgeon's Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics

Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies age < 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions. A standardized echo review protocol of 111 quantitative and qualitative measures was used. Descriptive statistics were computed and Pearson correlation coefficients were calculated to assess correlation among unbalance indices with valvar and ventricular dimensions. Two-hundred fifty-seven baseline echocardiograms of infants with complete AVSD were included. Median age at baseline echocardiogram was 11 days (interquartile range 1-79) and mean atrioventricular valve index was 0.45 ± 0.1. Mean right ventricle/left ventricle inflow angle was 90.2 ± 15.6° and median left ventricular inflow index was 0.46 (interquartile range 0.4-0.5). There are weak or moderate correlations between the measures of unbalance. Correlations between the measures of unbalance with common AVV leaflet or ventricular sizes are also weak to moderate, when statistically significant. Measures of unbalance in common clinical use correlate poorly, or not at all, with one another, common AVV, and ventricular dimensions. The concept of "unbalance" is difficult to define using baseline echocardiographic indices. These findings suggest that the indices may describe different morphologic and functional characteristics. Further analysis is necessary to quantify the contributions of unbalance indices to patient outcome.

3D Printing in Surgical Management of Double Outlet Right Ventricle.

Double outlet right ventricle (DORV) is a heterogeneous group of congenital heart diseases that require individualized surgical approach based on precise understanding of the complex cardiovascular anatomy. Physical 3-dimensional (3D) print models not only allow fast and unequivocal perception of the complex anatomy but also eliminate misunderstanding or miscommunication among imagers and surgeons. Except for those cases showing well-recognized classic surgical anatomy of DORV such as in cases with a typical subaortic or subpulmonary ventricular septal defect, 3D print models are of enormous value in surgical decision and planning. Furthermore, 3D print models can also be used for rehearsal of the intended procedure before the actual surgery on the patient so that the outcome of the procedure is precisely predicted and the procedure can be optimally tailored for the patient’s specific anatomy. 3D print models are invaluable resource for hands-on surgical training of congenital heart surgeons.

Higher Programmatic Volume in Neonatal Heart Surgery Is Associated With Lower Early Mortality

The early results of congenital heart surgery in neonates remain a challenge. We sought to determine the nature of the association between annual center volume of neonatal cardiac surgery and operative mortality using a multicenter cohort. The dataset consists of 27,556 neonatal procedures performed between 1999 and 2015 in 90 centers participating in the European Congenital Heart Surgeons Association database. Centers with mean annual volume load of six or more that submitted data for at least 3 consecutive years were included. World Bank annual gross national index per capita was utilized as an indicator of temporal national affluence. Multilevel logistic regression was used to create a model including the significant risk factors and to calculate odds ratios for operative mortality. Iterative modeling of the dataset incrementally excluding centers with lower annual caseload was used to identify the relationship between annual volume and mortality. In the model thus calculated including The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score, operative weight and age, noncardiac genetic anomalies, and annual volume of operations were independent risk factors for operative mortality in the analysis of the entire cohort. In the model containing these variables, annual gross national index and year of surgery were not significantly associated with mortality. In the iterative process, annual volume ceased to be a risk factor when units operating on fewer than 60 neonates annually were excluded. In neonatal congenital heart surgery, the risk of operative death decreased with the increase of volume load. The cutoff point in this cohort was a mean annual volume of 60 neonatal operations per year.

Landmark lecture on surgery: paediatric cardiothoracic surgery - training the next generation of congenital heart surgeons.

We used a qualitative thematic analysis approach and performed phone interviews with the top five congenital experts in the United States of America. Experts unanimously felt that duty-hour restrictions have negatively affected congenital training programmes in the following ways: current fellows do not seem as conditioned as fellows in the past, patient handoffs are not consistent with excellent performance, the mentor-mentee relationship has been affected by duty-hour restrictions, and fellows may be less prepared for real-world practice. Three positive themes emerged in response to duty-hour restrictions: fellows appear to be doing less menial task work, fellows are now better rested for learning, and we are attracting more individuals into the speciality. Experts agreed that congenital fellowships should be increased to 2 years. There was support for both the traditional and integrated residency pathways. Discussion We are in a new era of education and must work together to overcome the challenges that have arisen in recent years.

Abstracts of the Annual Spring Meeting 2017 of the European Congenital Heart Surgeons Association, Leicester, UK, 19-20th May, 2017

An abstract is not available for this content so a preview has been provided. As you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

The Optimal Timing of Stage-2-Palliation After the Norwood Operation

The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival. The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factorsfor death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P. A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months. Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.

Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.

Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.