José Fragata

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.nnMethods 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.nnConclusions— 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.nn# Clinical Perspective {#article-title-44}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

OBJECTIVEnThis 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).nnnMETHODSnThe 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.nnnRESULTSnA 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).nnnCONCLUSIONSnTrans-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.

Late cardiac tamponade after transcatheter closure of atrial septal defect with Cardioseal® device

Cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.

Pediatric Coronary Artery Revascularization: A European Multicenter Study

BACKGROUNDnWe sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association.nnnMETHODSnFrom 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%).nnnRESULTSnTwelve 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).nnnCONCLUSIONSnBoth 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.

Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation.

An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy.

Surgical options after Fontan failure

Objective The 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 and (re-)HTX. Methods A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). Results The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30u2005days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9u2005years (range 0–23.7u2005years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). Conclusions Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

Characterization of non-technical skills in paediatric cardiac surgery: communication patterns †

OBJECTIVESnThe management of non-technical skills in complex surgical domains, such as paediatric cardiac surgery, is being recognized as a major factor for both performance and safety. Communication patterns are very relevant for safety and were analysed in this human-factor observational study.nnnMETHODSnOne behaviour observer taped and video-recorded the communication patterns for 10 consecutive paediatric open-heart cases, at a university institution, performed by variable, informal teams. Records were analysed for communication frequency, direction, type, content and pattern on the one hand, and on the other hand for factors influencing communication.nnnRESULTSnA total of 10xa0167 communication flows were read, with an average of 1017xa0±xa0170.9 per procedure over an average duration of 136.15xa0±xa019.52xa0min. The frequency of communication was maximal between the main surgeon and the scrub nurse (16% of all communications), followed by the main surgeon to the first surgical assistant (13.8%) and the main surgeon to the perfusionist (12.4%). Communication between the main surgeon and the anaesthetist was not more than 5%. Types of communication varied from requests, questions, answers, statements, informations and explanations, and being different for distinct staff roles: the main surgeon to the scrub nurse involved 84.2% requests, the main surgeon to the first surgical assistant 59.9% statements and the perfusionist to the main surgeon 65.4% answers. Communication patterns varied, being closed-loop (with feedback and double-check) only between the main surgeon and the perfusionist, and mostly open among other team members. Communication-disturbing factors such as noise, technology-related events, resource-based problems and supervisory and training-related issues were also found.nnnCONCLUSIONSnCommunication is very frequent in paediatric cardiac surgery and shows a complex pattern. There is room for improvement, namely by a more formal and standardized communication flow structure that can be achieved with the help of behavioural, technological and organizational initiatives.

Partial Anomalous Pulmonary Venous Connections Surgical Management

Partial anomalous pulmonary venous connections (PAPVCs) are a heterogeneous group of congenital heart lesions in which at least one pulmonary vein will drain into the systemic venous system. The consequences are a variable left-to-right hemodynamic shunt and more rarely pulmonary artery hypertension. Often, PAPVC occurs in association with other congenital cardiac malformations. Surgical correction is most often advisable and is generally straightforward and simple to achieve. Historically, some repairs have included incision across the junction of the superior vena cava with the right atrium, which can lead to late arrhythmias. The Warden technique avoids incision across the atriocaval junction. Neonates and infants with Scimitar syndrome represent the most challenging subset of patients with PAPVC.

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

BACKGROUNDnWe 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.nnnMETHODSnSeventeen 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 (nxa0= 99) and DORV TGA-type (nxa0= 12). Main indications for LSR were neoaortic valve insufficiency (nxa0= 52 [47%]) and coronary artery problems (CAPs) (nxa0= 21 [19%]).nnnRESULTSnMedian 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%) (pxa0= 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%]).nnnCONCLUSIONSnReoperations 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.

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.