The 2017 Seventh World Congress of Paediatric Cardiology and Cardiac Surgery: "The Olympics of our Profession".

The 1st World Congress of Paediatric Cardiology was held in London, United Kingdom, in 1980, organised by Dr Jane Somerville and Prof. Fergus Macartney. The idea was that of Jane Somerville, who worked with enormous energy and enthusiasm to bring together paediatric cardiologists and surgeons from around the world. The 2nd World Congress of Paediatric Cardiology took place in New York in 1985, organised by Bill Rashkind, Mary Ellen Engle, and Eugene Doyle. The 3rd World Congress of Paediatric Cardiology was held in Bangkok, Thailand, in 1989, organised by Chompol Vongraprateep. Although cardiac surgeons were heavily involved in these early meetings, a separate World Congress of Paediatric Cardiac Surgery was held in Bergamo, Italy, in 1988, organised by Lucio Parenzan. Thereafter, it was recognised that surgeons and cardiologists working on the same problems and driven by a desire to help children would really rather meet together. A momentous decision was taken to initiate a Joint World Congress of Paediatric Cardiology and Cardiac Surgery. A steering committee was established with membership comprising the main organisers of the four separate previous Congresses and additional members were recruited in an effort to achieve numerical equality of cardiologists and surgeons and a broad geographical representation. The historic 1st "World Congress of Paediatric Cardiology and Cardiac Surgery" took place in Paris in June, 1993, organised by Jean Kachaner. The next was to be held in Japan, but the catastrophic Kobe earthquake in 1995 forced relocation to Hawaii in 1997. Then followed Toronto, Canada, 2001, organised by Bill Williams and Lee Benson; Buenos Aires, Argentina, 2005, organised by Horatio Capelli and Guillermo Kreutzer; Cairns, Australia, 2009, organised by Jim Wilkinson; Cape Town, South Africa, 2013, organised by Christopher Hugo-Hamman; and Barcelona, Spain, 2017, organised by Sertac Cicek. With stops in Europe (1993), Asia-Pacific (1997), North America (2001), South America (2005), Australia (2009), Africa (2013), and Europe again (2017), in 2021, The World Congress of Paediatric Cardiology and Cardiac Surgery will be held for the first time in the continental United States. 1 The 8th World Congress of Paediatric Cardiology and Cardiac Surgery will be held in Washington DC, United States of America, 19-24 September, 2021, and will be organised by Jeffrey P. Jacobs and Gil Wernovsky. Mitchell I. Cohen served as the Scientific Program Co-Chair for the 2017 World Congress of Paediatric Cardiology and Cardiac Surgery, and he will again serve as the Scientific Program Co-Chair for the 2021 World Congress of Paediatric Cardiology and Cardiac Surgery along with Kathyrn Dodds RN, MSN, CRNP. Information about the upcoming 8th World Congress of Paediatric Cardiology and Cardiac Surgery can be found at www.WCPCCS2021.org.

Carotid endarterectomy using a "home-constructed" shunt for patients intolerant to cross-clamping.

PURPOSES: There is a small minority of patients with occlusive carotid artery disease, who are at high-risk for general anesthesia because of their intolerance to carotid flow blockage, even if only for seconds, without neurologic deficit. Even <30 s of temporary clamping of the carotid arteries to deploy a shunt may prove eventful in this patient group. We define safe carotid endarterectomy after the insertion of a novel shunt that we made from simple medical equipment in this patient population. METHODS: Among 65 patients who underwent carotid endarterectomy between March 2010 and December 2012, 5 (7.7 %; 3 men and 2 women; age range 56-77 years) could not tolerate carotid clamping. We used an alternative carotid shunt, made by us from simple equipment in our clinic, during surgery for these patients. RESULTS: Two patients had bilateral lesions and the remainder had unilateral disease. The degree of stenosis ranged from 70 to 95 %. Temporary carotid clamping resulted in neurologic events, such as loss of consciousness in all and tremor in one, in <10 s (range, from immediately to 8 s after clamping). Full neurologic function was regained 15-30 s after releasing the clamps. All of the patients tolerated the procedures well with the support of our novel shunt. Shunt flow was adequate in all patients and no neurologic deterioration occurred after carotid clamping. The mean carotid clamp time was 28.11 ± 14.19 min. There was no mortality and all patients were followed up for a mean period of 9.3 ± 3.6 months, uneventfully. CONCLUSIONS: An alternative, simple shunt, which is easily constructed in the operating room or clinic, using an angiocatheter, a three-way stopcock, and a serum line can provide adequate cerebral flow and permit safe carotid endarterectomy for those rare patients with carotid artery stenosis, who cannot tolerate even seconds of carotid occlusion.

Scimitar syndrome: the curved Turkish sabre.

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. Patients are either diagnosed early with severe symptoms (infantile type) or late with minimal symptoms (childhood/adult type). In this chapter we reviewed the history, pathophysiology, presentation, diagnosis, and treatment methods of Scimitar syndrome in the current era.

Anomalous origin of the left subclavian artery from the pulmonary artery.

Isolated left subclavian artery from the pulmonary trunk is a rare congenital cardiovascular malformation. In this report, we present the images of ascending aortic aneurysm and left subclavian artery originating from the pulmonary artery in a 4-year-old girl in addition to her congenital cardiac pathology.

The Seventh World Congress of Pediatric Cardiology and Cardiac Surgery, Istanbul, Turkey, June 19-24, 2017: "Bridge together".

The Seventh World Congress of Pediatric Cardiology and Cardiac Surgery will held in Istanbul, the only city in the world that is located on two continents, June 19-24, 2017. This World Congress promises academic excellence in a culturally stimulating environment. All those interested in the care of patients with pediatric and congenital cardiac disease should attend this meeting, which represents "The Olympics of our Profession".

Double outlet right ventricle with unilateral absence of left pulmonary artery.

Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.

Tetralogy of Fallot with unilateral absent pulmonary artery.

BACKGROUND: Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity. PATIENTS AND METHODS: There were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy. RESULTS: Urgent modified Blalock-Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively. CONCLUSION: Our modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.

Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.

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.

Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES).

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

A Case Series of Long-Term Surgical Outcomes of Primary Pulmonary Artery Sarcomas With Opportunities for 3D-Printed Models in Surgical Planning.

There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution's long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.

Comparative Effectiveness of Mechanical Valves and Homografts in Complex Aortic Endocarditis.

BACKGROUND: The ideal surgical reconstruction of the aortic root in patients with complex endocarditis is controversial. We compared the short- and long-term outcomes between mechanical valves, bioprostheses, and homografts. METHODS: We identified all patients undergoing an operation for active complex aortic endocarditis at our institution between 2003 and 2017. We grouped patients according to those who received a mechanical valve, bioprosthesis, or homograft. We used multiple logistic regression and proportional hazards models. To minimize confounding by indication, we used marginal risk adjustment to simulate that every patient would undergo (contrary to fact) all 3 operations. RESULTS: Of 159 patients with complex active endocarditis, 48 (30.2%) had a valve plus patch reconstruction, and 85 (53.4%) had a root replacement. Of all, 50 (31.5%) had a mechanical valve, 56 (35.2%) had a bioprosthesis, and 53 (33.3%) had a homograft. The groups were similar in age, sex, body mass index, comorbid conditions, organism, abscess location, and mitral involvement (all P > .05). However, patients receiving mechanical reconstructions were more likely to have native valve endocarditis (46% vs 37.5% vs 17%; P = .005) and less likely to undergo root replacement (32% vs 28.6% vs 100%; P < .001). Marginal risk-adjusted operative mortality was lowest for mechanical valves (4.8%) and highest for homografts (16.9%; P = .041). Long-term survival after root replacement was worse with homografts than with mechanical valve conduits (adjusted hazard ratio, 2.9; P = .045). CONCLUSIONS: In patients with complex endocarditis, mechanical valves are associated with similar, if not better, short- and long-term outcomes compared with homografts, even after adjusting for important baseline characteristics and limiting the analysis to root replacements only.

Open hemiarch versus clamped ascending aorta replacement for aortopathy during initial bicuspid aortic valve replacement.

BACKGROUND: There is controversy regarding the extent of aortic resection necessary in patients with aortopathy related to bicuspid aortic valve disease. To address this issue, we reviewed our experience in patients undergoing ascending aorta replacement during bicuspid aortic valve replacement. METHODS: We reviewed 702 patients who underwent ascending aorta replacement at the time of initial nonemergent native bicuspid aortic valve replacement at our institution between January 2000 and June 2017. Treatment cohorts included an open hemiarch replacement group (n = 225; 32%) and a clamped ascending aorta replacement group (n = 477; 68%). RESULTS: Median patient age was 60 years (interquartile range [IQR], 51-67 years), female sex was present in 113 patients (16%), ejection fraction was 62% (IQR, 56%-66%), and aortic arch diameter was 33 mm (IQR, 29-36 mm). Cardiopulmonary bypass time was longer in the hemiarch replacement group (188 minutes vs 97 minutes; P < .001). Procedure-related complications (36%) and mortality (<1%) were similar in the 2 groups; however, the hemiarch group had an increased odds of blood transfusion (odds ratio, 1.62; 95% confidence interval [CI], 1.15-2.28; P = .006). The median duration of follow-up was 6.0 years (95% CI, 5.3-6.8 years). Overall survival was 94 ± 1% at 5 years and 80 ± 2% at 10 years. Multivariable analysis demonstrated similar survival in the 2 groups (hazard ratio, 0.83; 95% CI, 0.51-1.33; P = .439). No repeat aortic arch operations were done for aortopathy over the duration of clinical follow-up. CONCLUSIONS: Compared with patients in the clamped ascending aorta replacement group, patients in the hemi-arch replacement group had longer cardiopulmonary bypass and aortic cross-clamp times, along with an increased risk of blood transfusion, but similar freedom from repeat aortic arch operation and survival. We identified no advantage of performing hemiarch replacement in the absence of aortic arch dilation.

The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

The impact of coronary artery bypass grafting surgical technique on stroke in young and elderly patients.

BACKGROUND: Today, the elderly represent a large and rapidly growing segment of society and are being referred in increasing numbers for coronary artery bypass grafting (CABG) surgery. Stroke is a major complication of CABG surgery. The risk of stroke after CABG can be managed successfully, especially in high-risk patients, by choosing an adequate and appropriate surgical technique. METHODS: We evaluated 890 consecutive patients who underwent isolated CABG surgery by the same team between June 2006 and July 2008. The patients were divided by age into 2 groups. Group I consisted of 480 patients <65 years of age, and group II comprised 410 patients >65 years of age. Each group was then divided into 4 subgroups according to the surgical technique used: double-clamp technique (DCT), single-clamp technique (SCT), off-pump, and on-pump cross-clamp off. Preoperative risk factors for stroke and all clinical data were collected for the patients. RESULTS: In group I, 192 (40%) of the patients were female, and 288 (60%) were male. In group II, 170 (41.5%) were female, and 240 (58.5%) were male. Five patients in each group experienced stroke, with an incidence of 1.04% in group I (4 men and 1 woman) and 1.21% in group II (3 men and 2 women). The stroke rates of the 2 age groups were not significantly different (P= .802). Three of the 480 patients in group I died, with only 1 (33.3%) of the deaths related to stroke. In group II, however, 2 (50%) of the 4 deaths were related to stroke. The incidences of stroke-associated mortality in the 2 age groups were not significantly different (P=1.0). CONCLUSION: Although off-pump and on-pump cross-clamp off techniques were performed for some of the patients, DCT and SCT were used for the majority of the patients. We detected no statistically significant difference between these 2 groups of patients in the impact of applying DCT and SCT on the stroke rate. We conclude that this result was due to the different surgical techniques performed on a minority of the patients (82 patients).

Long-term outcomes of patients undergoing tricuspid valve surgery†.

OBJECTIVES: Limited literature is available on the best management strategy for tricuspid valve (TV) disease in adults. We sought to review our long-term outcomes of TV surgery with regard to survival and reoperation. METHODS: A retrospective analysis of all patients ≥18 years of age [n = 2541, aged 67 ± 13years, 1433 (56%) females] who underwent first-time TV surgery between January 1993 and December 2013 was done. There were 1735 patients who received TV repair and 806 patients underwent replacement. A gradient boosting machine model was used to derive a propensity score for predicting replacement using 27 preoperative characteristics. Four hundred and eighteen propensity-matched pairs of TV repair and replacement were identified. Cox proportional hazard regression was used on the matched subset to determine the effect of replacement. RESULTS: Functional TV regurgitation was present in 54% (n = 1369). A bioprosthesis was used in 84% (n = 680) of replacements, while 54% (n = 934) of TV repairs were ring annuloplasties. Operative mortality was 8% (n = 212). Overall survival was 54%, 29% and 13% at 5, 10 and 15 years, respectively. After propensity score matching, replacement was significantly associated with increased mortality [hazard ratio (HR) 1.54, 95% confidence interval (CI) 1.18-2.00; P = 0.001]. The cumulative incidence of TV reoperation was similar between the 2 groups when accounting for the competing risk of death [Fine-Gray HR 1.56, 95% CI 0.9-2.8; P = 0.144]. CONCLUSIONS: TV surgery is associated with poor outcomes due to multiple patient comorbidities. TV repair results in better survival compared to replacement in patients with similar comorbidities with no increased risk of getting a reoperation.

Does Mitral Valve Calcium in Patients Undergoing Mitral Valve Replacement Portend Worse Survival?

BACKGROUND: Mitral annular calcification (MAC) is associated with worse outcomes after mitral valve replacement (MVR). With limited data available on long-term outcomes, we reviewed our experience of MVR in presence of MAC. METHODS: A retrospective review of 1,710 consecutive patients who underwent MVR between January 2000 and December 2015 was performed. Patients with isolated primary MVR (n = 496) were included, whereas patients with concomitant cardiac surgery (n = 1,068), previous MVR (n = 110), and mitral valve (MV) endocarditis (n = 36) were excluded. MV calcification was classified as MAC present in anterior/posterior annulus and vertically at the level of leaflets/subvalvular apparatus. A conservative approach towards annular debridement was followed. RESULTS: Our sample's mean age was 64.4 ± 14.1 years, and included 279 (56%) women. MV calcification was observed in 169 (34%) patients with MAC in 115 (23%). Older age, higher ejection fraction, peripheral vascular disease, diabetes, dialysis, and previous aortic valve surgery were associated with increased prevalence of MAC. Patients with MV calcification had higher stroke rate (p = 0.040), patients with anterior leaflet and commissural calcification had higher pacemaker implantation (p = 0.010, p = 0.001, respectively), and patients with circumferential MAC had higher postoperative dialysis (p = 0.006). Operative mortality was not significantly different (p = 0.466) between MAC (n = 1, 1%) and non-MAC (n = 9, 2%) patients. MAC was associated with late mortality (unadjusted hazard ratio, 1.62; 95% confidence interval, 1.20 to 2.18), though on multivariable analysis age, diabetes, dialysis, hypertension, previous aortic valve surgery, previous coronary artery bypass grafting, and MVR with a bioprosthetic valve were found to be independent risk factors for mortality whereas MAC was not. CONCLUSIONS: A conservative approach to treat MAC achieves satisfactory results. Patients with MAC have significant comorbidities contributing to a worse survival, though MAC in itself is not a risk factor for mortality.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.

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

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.