European Congenital Heart Surgeons Association Research Articles

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.

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.

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

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.

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

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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.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study

We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n= 99) and DORV TGA-type (n= 12). Main indications for LSR were neoaortic valve insufficiency (n= 52 [47%]) and coronary artery problems (CAPs) (n= 21 [19%]). Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p= 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

Current Practices in the Timing of Stage 2 Palliation.

Mortality through single-ventricle palliation remains high and the effect of the timing of stage 2 palliation (S2P) is not well understood. We investigated current practice patterns in the timing of S2P across two professional societies and compared them to actual practice patterns from two databases of patients who underwent S2P. A ten-question survey was distributed to the members of the Congenital Heart Surgeons' Society (CHSS) and the European Congenital Heart Surgeons' Association (ECHSA). Results were summarized using descriptive statistics. Surgeon-reported preferences were compared to clinical data from the CHSS Critical Left Ventricular Outflow Tract Obstruction (LVOTO) Registry and the Pediatric Heart Network Single Ventricle Reconstruction (SVR) database. Overall, 38% (88 of 232) of surgeons from 74 institutions responded, of which 70% (62 of 88) were CHSS members and 30% (26 of 88) were ECHSA members. Surgeons reported performing S2P at a median of five months after stage 1 (interquartile range [IQR]: 4.5-6), with no difference between CHSS and ECHSA surgeons. Surgeons reported performing nonelective S2P at a median of 4.5 months after stage 1 (IQR: 3.5-5.5), again with no difference by society. No difference existed between the surgeon-reported preferences and patient data in the Critical LVOTO and SVR databases for the timing of elective (5 vs 5.1 vs 5.3 months, P = .19) or nonelective S2P (4.5 vs 4.6 vs 4.2 months, P = .06). There was a remarkable lack of variation in surgeon preferences regarding the timing of S2P. This may represent a natural standardization of practice across congenital heart surgery, which is notable, given the current lack of guidelines regarding the timing of S2P.

Abstracts of the Fourth Joint Meeting of the European Congenital Heart Surgeons Association and the Congenital Heart Surgeons' Society, Venice, Italy, June 24–26, 2016

Abstracts of the Fourth Joint Meeting of the European Congenital Heart Surgeons Association and the Congenital Heart Surgeons' Society, Venice, Italy, June 24–26, 2016

Paediatric cardiac surgery in a peripheral European region: is a joint programme a safe alternative to regionalisation?

In 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands. An operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated. In total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients). A joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

Surgical options after Fontan failure

ObjectiveThe objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality...

Paediatric coronary revascularization: a European multi-center study

Purpose: To evaluate the hospital and long term results of different surgical revascularization techniques in paediatric patients within the European Congenital Heart Surgeons Association (ECHSA). Methods: From 1969 to 2011, 80 patients from 13 ECHSA centers underwent 65 Paediatric Coronary Artery Bypass Grafting (PCABG) and 27 Other Coronary Artery Procedures (OCAP) (12 patients combined PCABG and OCAP). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range 2 days–16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). Results: Twelve patients (15%) died in hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range 0.9–23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range 9 months-8.8 years) from PCABG. Fourteen of 64 patients (21.8%) presented with symptoms, including congestive heart failure (n=10) and angina (n=5) that were related to a low ejection fraction (p<0.001) and the presence of moderate/severe mitral valve regurgitation (p=0.0003). Six patients underwent a re-intervention for impaired myocardial perfusion; all of them had a stenotic/atretic PCABG (p=0.001) and the majority were symptomatic (5/6 pts, 83.3%, p=0.001). Conclusions: PCABG and OCAP are suitable surgical options in paediatric patients with impaired myocardial perfusion which increases operative and mid-term survival.

Pediatric Coronary Artery Revascularization: A European Multicenter Study

We sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association. From 1973 to 2011, 80 patients from 13 European Congenital Heart Surgeons Association centers underwent 65 pediatric coronary artery bypass grafting (PCABG) and 27 other coronary artery procedures (OCAP; 12 patients had combined PCABG and other coronary artery procedures). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range, 2 days to 16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). Twelve patients (15%) died in the hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range, 0.9 to 23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range, 9 months to 8.8 years) after PCABG. Fourteen patients (20.5%) presented with symptoms, including congestive heart failure (n=10) and angina (n=4), that were significantly associated with a low ejection fraction (p<0.001) and the presence of moderate or severe mitral valve regurgitation (p=0.0003). Six patients underwent a reintervention for impaired myocardial perfusion; all of them had a stenotic or atretic PCABG (p=0.001), and the majority were symptomatic (5 of 6 patients; 83.3%; p=0.001). Both PCABG and other coronary artery procedures are suitable surgical options in pediatric patients with impaired myocardial perfusion, which increases operative and midterm survival. Such population of patients needs to be followed for life to prevent and treat any possible cause of further myocardial ischemia.

Minimal requirements for a congenital heart surgery programme: contribution of the Aristotle complexity score

Aims: The European Congenital Heart Surgeons Association stated that an optimal structure in Europe should operate on over 250 patients per year. However, not only the number of cases should be considered, but also related surgical performance should be involved.

Scimitar Syndrome

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association

This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.

Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study

Optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow obstruction (LVOTO) remains controversial. Although the Rastelli operation has been the most widely performed surgical procedure during the past decades, several studies have shown its suboptimal long-term prognosis. Other operations have been developed to improve results. This study was performed to compare the outcomes of the different surgical approaches for patients with TGA, VSD and LVOTO, as well as to determine risk factors for mortality and re-intervention. Records from 146 patients undergoing surgery from 1980 to 2008 from eight European hospitals were reviewed. Median age at operation was 21.5 months (range 0.2-165.1 months), and median weight was 10.0 kg (range 2.0-41.0 kg). Surgical procedures involved were the Rastelli procedure (82), arterial (24) and atrial (5) switch operation with relief of LVOTO, Réparation à l'Etage ventriculaire (REV) procedure (7) and the Metras modification (24), as well as the Nikaidoh procedure (4). The overall survival was 88%, 88% and 58% at 1, 10 and 20 years, respectively. The overall event-free survival was 80%, 45% and 26% at 1, 10 and 20 years, respectively. The REV procedure and the Metras modification were found to have the best long-term results in both survival and event-free survival rates. Multivariate analysis revealed year of operation, non-commitment of the VSD and prolonged cardiopulmonary bypass (CPB) time as risk factors for mortality while age at surgery, year of operation and type of corrective surgery were risk factors for re-intervention. Different surgical approaches have been developed for patients with TGA, VSD and LVOTO. The REV procedure and the Metras modification were observed to have favourable long-term results in survival and event-free survival rates. Aortic translocation techniques such as the Nikaidoh procedure seem promising, but further studies will be needed to confirm this in the long term.

Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease – the perspective of anaesthesia

The Congenital Cardiac Anesthesia Society was formed in 2005 by representatives from many of the busiest congenital cardiac surgical programs in North America and is now in the process of partnering with The Society of Thoracic Surgeons to create a joint congenital cardiac surgery and congenital cardiac anaesthesia database. Even the busiest of congenital cardiac programs have a low frequency of anaesthesia-related cardiac complications and deaths. One of the only mechanisms for accurately determining the incidence and outcomes of low frequency events is to aggregate large amounts of data from multiple sources. To that end, the Congenital Cardiac Anesthesia Society has joined with the Society of Thoracic Surgeons Congenital Database Task Force to incorporate anaesthesia-specific data points into their surgical registry, which is now the largest single reporting site for children and adults undergoing surgical repair of congenital cardiac malformations in North America. The Joint Congenital Cardiac Anesthesia Society--Society of Thoracic Surgeons Database will therefore become an optional module of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Initial data fields have been selected and are presented in this article. Efforts are ongoing to make this initiative a global project. Initial collaborative discussions have taken place about the possibility of linking this initiative with the European Association of Cardiothoracic Anesthesiologists. It is certainly possible and desirable that the planned anaesthesia module of The Society of Thoracic Surgeons Congenital Heart Database has an identical module in the congenital heart database of The European Association for Cardio-Thoracic Surgery and The European Congenital Heart Surgeons Association. This project should also ideally spread beyond North America and Europe. Efforts to involve Africa, Asia, Australia, and South America are necessary and already underway. The creation of a joint cardiac surgery and anaesthesia database is another step towards the ultimate goal of creating a database for congenital heart disease that spans both geographical and subspecialty boundaries.

The arterial switch operation in Europe for transposition of the great arteries: A multi-institutional study from the European Congenital Heart Surgeons Association

This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.

Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association

Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.