Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Ischemic preconditioning does not prevent placental dysfunction induced by fetal cardiac bypass.

BACKGROUND: Remote ischemic preconditioning (rIPC) has been applied to attenuate tissue injury. We tested the hypothesis that rIPC applied to fetal lambs undergoing cardiac bypass (CB) reduces fetal systemic inflammation and placental dysfunction. METHODS: Eighteen fetal lambs were divided into three groups: sham, CB control, and CB rIPC. CB rIPC fetuses had a hindlimb tourniquet applied to occlude blood flow for four cycles of a 5-min period, followed by a 2-min reperfusion period. Both study groups underwent 30 min of normothermic CB. Fetal inflammatory markers, gas exchange, and placental and fetal lung morphological changes were assessed. RESULTS: The CB rIPC group achieved higher bypass flow rates (p < .001). After CB start, both study groups developed significant decreases in PaO2 , mixed acidosis, and increased lactate levels (p < .0004). No significant differences in tissular edema were observed on fetal lungs and placenta (p > .391). Expression of Toll-like receptor 4 and intercellular adhesion molecule-1 in the placenta and fetal lungs did not differ among the three groups, as well as with vascular cell adhesion molecule-1 (VCAM-1) of fetal lungs (p > .225). Placental VCAM-1 expression was lower in the rIPC group (p < .05). Fetal interleukin-1 (IL-1) and thromboxane A2 (TXA2) levels were lower at 60 min post-CB in the CB rIPC group (p < .05). There were no significant differences in tumor necrosis factor-α, prostaglandin E2, IL-6, and IL-10 plasma levels of the three groups at 60-min post-bypass (p > .133). CONCLUSION: Although rIPC allowed increased blood flow during fetal CB and decreased IL-1 and TXA2 levels and placental VCAM-1, it did not prevent placental dysfunction in fetal lambs undergoing CB.

COVID-19 pandemic implications in paediatric and congenital heart surgery in Brazil.

INTRODUCTION: The Coronavirus Disease 2019 (COVID-19) pandemic negatively impacted global healthcare. Consequences in Pediatric and Congenital Heart Surgery programmes and mortality of congenital heart patients infected with severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) is still to be determined. OBJECTIVE: To study the COVID-19 pandemic implications in Brazilian Pediatric and Congenital Heart Surgery programmes. METHODS: We conducted a national online survey covering all states that perform Pediatric and Congenital Heart Surgery from 10 November to 22 November, 2020, using a Google forms questionnaire. We formulated questions related to impact on surgical volume, case-mix, and mortality. Then we asked about short-term post-operative COVID-19 infection and outcomes. RESULTS: We received responses from 46 centres representing all states where there were a Pediatric and Congenital Heart Surgery programme and all high-volume centres across the country. All but one centre experienced a significant decrease in surgical volume, and 23.9% of the responders revealed less than one-quarter of volume decrement. On the other hand, in over 70% of the centres, there was a significant surgical volume reduction. In addition to this, there was a shift in case-mix in 41 centres (89.1%) towards more complex cases. More than one-third of the responders revealed increased mortality in 2020 compared to previous years, and 43.5% of the programmes (20 centres) had at least one patient contaminated by SARS-Cov-2, accounting for 48 patients. Mortality in post-operative infected patients was 45.8% (22 patients). CONCLUSIONS: In general, Brazilian Pediatric and Congenital Heart Surgery programmes were severely affected by decreased surgical volume, unbalanced case-mix towards more complex cases, and increased mortality. Almost half of the programmes related post-operative COVID-19 contamination with high mortality.

Risk factors for death or mechanical ventilation time after bidirectional cavopulmonary anastomosis in a developing country.

BACKGROUND: Glenn procedure is performed for patients with cyanotic CHD and univentricular physiology and has a survival rate above 90%. AIM: To evaluate the risk factors associated with a poor outcome after Glenn procedure. METHODS: The data for this retrospective analysis were collected from a regional Brazilian registry of congenital heart surgeries (ASSIST initiative) from 2014 to 2019. Data from 97 patients who underwent the Glenn procedure were considered. The primary outcomes were prolonged mechanical ventilation (>24 hours post-operatively) or in-hospital death, alone and combined. RESULTS: The overall in-hospital mortality was 13.4% (n = 13). Prolonged mechanical ventilation occurred in 52% (n = 51) of the patients. Our analysis found that risk factors for death or prolonged post-operative mechanical ventilation were cardiopulmonary bypass duration and post-operative lactate, whereas weight-for-age z-score and age at surgery were risk factors for prolonged mechanical ventilation. Cardiopulmonary bypass duration increased (adjusted odds ratio: 1.02; 95 % CI: 1.01, 1.03) and age at surgery decreased (adjusted odds ratio: 0.96; 95 % CI: 0.93, 0.99) the odds of the combined outcome. CONCLUSIONS: Age at surgery, post-operative lactate, and low-weight-for-age z-score are associated with prolonged mechanical ventilation and death following the Glenn procedure.

Initial experience with del Nido cardioplegia solution at a Pediatric and Congenital Cardiac Surgery Program in Brazil.

OBJECTIVE: The aim of this study was to evaluate outcome measures between our standard multidose cardioplegia protocol and a del Nido cardioplegia protocol in congenital heart surgery patients. METHODS: Retrospective single-center study including 250 consecutive patients that received del Nido cardioplegia (DN group) with a mandatory reperfusion period of 30% of cross clamp time and 250 patients that received a modified St. Thomas' solution (ST group). Groups were matched by age, weight, gender, and Risk Adjustment for Congenital Heart Surgery (RACHS-1) scores. Preoperative hematocrit and oxygen saturation were also recorded. Outcomes analyzed were the vasoactive inotropic score (VIS), lactate, ventilation time, ventricular dysfunction with low cardiac output syndrome (LCOS), intensive care unit (ICU) length of stay (LOS), hospital LOS, bypass and aortic cross-clamp times, and in-hospital mortality. RESULTS: Both groups were comparable demographically. Statistically significant differences (p ⩽ 0.05) were noted for cardiac dysfunction with LCOS, hematocrit at end of surgery (p = 0.0038), VIS on ICU admission and at end of surgery (p = 0.0111), and ICU LOS (p = 0.00118) with patients in the DN group having more desirable values for those parameters. Other outcome measures did not reach statistical significance. CONCLUSION: In our congenital cardiac surgery population, del Nido cardioplegia strategy was associated with less ventricular dysfunction with LCOS, a lower VIS and decreased ICU LOS compared with patients that received our standard myocardial protection using a modified St. Thomas' solution. Despite the limitation of this study, including its retrospective nature and cohort size, these data supported our transition to incorporate del Nido cardioplegia solution with a mandatory reperfusion period as the preferred myocardial protection method in our program.

Anomalous aortic origin of the left main coronary artery associated with arterial compression-Case report.

BACKGROUND: Anomalous aortic origin of coronary artery is a rare finding, with varied presentation and symptomatology. Increasingly recognized by cardiac imaging, when found it raises questions about the appropriate approach and management. CASE PRESENTATION: We present a case of an 11-year-old female who presented with episodes of shortness of breath, angina, and syncope during exercise. Further investigation demonstrated episodes of nonsustained ventricular tachycardia on Holter and coronary angiotomography revealed that the left coronary artery had an anomalous origin from the right cusp with initial short intramural segment and significant external compression in its initial course between the aorta and the pulmonary artery. The patient was submitted to surgical correction with dissection of left coronary artery posterior to the pulmonary artery, coronary arteriotomy, roof ampliation with the autologous pericardium, and creation of neo-ostium in aorta. The patient had a satisfactory postoperative recovery, was discharged on the fifth-day post-op, and remains asymptomatic after 14 months of follow-up. Herein we present surgical video and postoperative echo and computed tomography scan.

Prognostic value of the preoperative neutrophil-lymphocyte ratio in patients undergoing the bidirectional Glenn procedure.

INTRODUCTION: The neutrophil-lymphocyte ratio (NLR) has been associated with worse outcomes in patients undergoing coronary artery bypass graft surgery. Little is known about this association in the pediatric population who require surgery for congenital heart defects, especially in patients with a single ventricle (SV). OBJECTIVE: To analyze the association of the preoperative NLR with outcomes in patients undergoing the bidirectional Glenn procedure. METHODS: This study involved a retrospective cohort analysis of 141 consecutive patients with SV undergoing the bidirectional Glenn procedure between January 2011 and December 2017 in two centers. The preoperative NLR was included in the last hemogram test before surgery. According to the NLR level, the patients were divided into group I (NLR < 1), group II (NLR between 1 and 2), and group III (NLR > 2). The primary endpoint was total hospital length of stay (LOS), and secondary endpoints were mechanical ventilation (MV) time, intensive care unit (ICU) LOS, ventricular dysfunction, complications, and middle-term mortality. RESULTS: The average follow-up duration was 48 months. There were 61, 47, and 33 patients in groups I, II, and III, respectively. Patients in group III exhibited an increased risk of prolonged total hospital LOS (P = .00). An increase in MV time (P = .03) and ICU LOS (P = .02) was also observed in this group, and these patients experienced greater mortality in 24 months following the surgery (P = .03). There was no association between the NLR and ventricular dysfunction (P = .26) and complications (P = .46). CONCLUSION: A high preoperative NLR was associated with worse outcomes in patients with SV physiology undergoing the bidirectional Glenn procedure.

In Vitro Evaluation of Pediatric Hollow-Fiber Membrane Oxygenators on Hemodynamic Performance and Gaseous Microemboli Handling: An International Multicenter/Multidisciplinary Approach.

The objective of this study was to compare the hemodynamic performances and gaseous microemboli (GME) handling ability of two pediatric oxygenators in a simulated pediatric cardiopulmonary bypass (CPB) model and the importance of adding an arterial filter in the circuit. The circuit consisted of a Braile Infant oxygenator or a Maquet Quadrox-I Pediatric oxygenator without integrated arterial filter (parallel arrangement), 1/4 in. ID tubing A-V loop, and a 12-Fr arterial cannula, primed with lactated Ringer's solution and packed red blood cells. Trials were conducted at flow rates ranging from 500 to 2000 mL/min (500 mL/min increment) at 35°C and 28°C. Real-time pressure and flow data were recorded using a custom-based data acquisition system. For GME testing, 5 cc of air was manually injected into the venous line. GME were recorded using the Emboli Detection and Classification Quantifier (EDAC) System. An additional experiment using a separate arterial filter was conducted. There was no difference in the mean circuit pressure, pressure drop, total hemodynamic energy level, and energy loss between the two oxygenators. The venous line pressures were higher in the Braile than in the Quadrox group during all trials (P <0.01). GME count and volume at pre-/post oxygenator and pre-cannula sites in the Quadrox were lower than the Braile group at high flow rates (P < 0.05). In the additional experiment, an arterial filter captured a significant number of microemboli at all flow rates. The Braile Infant oxygenator has a matched hemodynamic characteristic with the Quadrox-i Pediatric oxygenator. The Quadrox-i has a better GME handling ability compared with the Braile Infant oxygenator. Regardless of type of oxygenator an additional arterial filter decreases the number of GME.

Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients.

Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO2 expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO2 Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02-97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO2 [FVC (r = 0.86, p < 0.001); FEV1 (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [-79(±28) vs -109(±44) cmH2O (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO2 with the FVC, FEV1, TLC, and DLCO measured by complete pulmonary test.

Fifth "Jatene Lecture on Surgical Innovation": Innovation in Congenital Heart Surgery: Contributions From South America.

We present the fifth "Jatene Lecture on Surgical Innovation" on Innovation in Congenital Heart Surgery, given at the Eighth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery and Eighth World Congress of Pediatric Cardiology and Cardiac Surgery in Washington DC in 2023. We highlight what surgical innovation is and how innovation was accomplished in cardiac surgery and particularly in congenital heart surgery. A brief history of the development of congenital heart surgery across the world is summarized and we finally illustrate the South American contributions to congenital heart surgery, acknowledging the great innovations of Adib Jatene and Guillermo Kreutzer to our field.

Neutrophil-Lymphocyte Ratio in Congenital Heart Surgery: What Is Known and What Is New?

Operative mortality after repair of congenital heart disease has improved dramatically over the past few decades. Nevertheless, there is always room for the additional mitigation of complications and mortality. Being able to anticipate adverse outcomes is clearly important, especially when using low-cost and easily accessible resources. The neutrophil-lymphocyte ratio (NLR) is defined as the ratio of the absolute neutrophil to lymphocyte count, which can be easily measured using a regular white blood cell count. Recently, preoperative NLR has been shown to be a predictor of outcomes in patients undergoing congenital heart surgery. Although it presented promising results, there are still many gaps to be filled like the normal value for children, the ideal cutoff value to predict adverse outcomes, the wide variation and its correlation with other biomarkers, and if it is a modifiable risk factor. The aim of this review is to understand the prognostic value of preoperative NLR as a biomarker predictor of outcomes in patients undergoing congenital heart surgery based on previous clinical studies and to propose future directions in order to solve the above-mentioned questions.

Holt-Oram syndrome: novel TBX5 mutation and associated anomalous right coronary artery.

The Holt-Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected the septal defect and the initial segment of the anomalous vessel was unroofed and enlarged. Anomalous coronary arteries were not previously described in the Holt-Oram syndrome patients and should be added to the list of possible associated cardiac defects.

Anemia in Cardiac Surgery - Can Something Bad Get Worse?

INTRODUCTION: Anemia and blood transfusion are risk factors for morbidity/mortality in patients undergoing cardiac surgery with cardiopulmonary bypass (CPB). The objective of this study is to analyze the association of blood transfusion with morbidity/ mortality in patients undergoing coronary artery bypass grafting (CABG) under CPB in the state of São Paulo, Brazil. METHODS: This is a retrospective analysis using the State of São Paulo Registry of Cardiovascular Surgery from November 2013 to August 2014. Blood transfusion was only considered during surgery or within six hours after surgery. Anemia was defined as hematocrit ≤ 37.5%. Patients < 18 years old were excluded. The sample was divided in four groups - Group I (851, no anemia), Group II (200, anemia without blood transfusion), Group III (181, no anemia and transfusion), and Group IV (258, anemia and transfusion). RESULTS: A total of 1,490 patients were included; 639 (42.9%) were anemic and 439 (29.5%) underwent blood transfusion. Group II showed lower composite morbidity (odds ratio [OR] -0.05; confidence interval [CI] -0.27-0.17; P=0.81) than Group III (OR 0.41; CI 0.23-0.59; P=0.018) or Group IV (OR 0.54; CI 0.31- 0.77; P=0.016). Group III was at greater risk of mortality (OR 0.73; CI 0.43-1.03; P=0.02) than Group II, which was exposed only to anemia (OR -0.13; CI -0.55-0.29; P=0.75), or Group IV (OR 0.29; CI -0.13-0.71; P=0.539). CONCLUSION: Anemia in patients undergoing CABG with CPB is bad, but blood transfusion can be worse, increasing at least 50% the risk for mortality and/or morbidity.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Reversible pulmonary trunk banding: Myocardial vascular endothelial growth factor expression in young goats submitted to ventricular retraining.

BACKGROUND: Ventricle retraining has been extensively studied by our laboratory. Previous studies have demonstrated that intermittent overload causes a more efficient ventricular hypertrophy. The adaptive mechanisms involved in the ventricle retraining are not completely established. This study assessed vascular endothelial growth factor (VEGF) expression in the ventricles of goats submitted to systolic overload. METHODS: Twenty-one young goats were divided into 3 groups (7 animals each): control, 96-hour continuous systolic overload, and intermittent systolic overload (four 12-hour periods of systolic overload paired with 12-hour resting period). During the 96-hour protocol, systolic overload was adjusted to achieve a right ventricular (RV) / aortic pressure ratio of 0.7. Hemodynamic evaluations were performed daily before and after systolic overload. Echocardiograms were obtained preoperatively and at protocol end to measure cardiac masses thickness. At study end, the animals were killed for morphologic evaluation and immunohistochemical assessment of VEGF expression. RESULTS: RV-trained groups developed hypertrophy of RV and septal masses, confirmed by increased weight and thickness, as expected. In the study groups, there was a small but significantly increased water content of the RV and septum compared with those in the control group (p<0.002). VEGF expression in the RV myocardium was greater in the intermittent group (2.89% ± 0.41%) than in the continuous (1.80% ± 0.19%) and control (1.43% ± 0.18%) groups (p<0.023). CONCLUSIONS: Intermittent systolic overload promotes greater upregulation of VEGF expression in the subpulmonary ventricle, an adaptation that provides a mechanism for increased myocardial perfusion during the rapid myocardial hypertrophy of young goats.

Impact of Vacuum-Assisted Venous Drainage on Forward Flow in Simulated Pediatric Cardiopulmonary Bypass Circuits Utilizing a Centrifugal Arterial Pump Head.

OBJECTIVE: To analyze the impact of vacuum-assisted venous drainage (VAVD) on arterial pump flow in a simulated pediatric cardiopulmonary bypass circuit utilizing a centrifugal pump (CP) with an external arterial filter. METHODS: The simulation circuit consisted of a Quadrox-I Pediatric oxygenator, a Rotaflow CP (Maquet Cardiopulmonary AG, Rastatt, Germany), and a custom pediatric tubing set primed with Lactated Ringer's solution and packed red blood cells. Venous line pressure, reservoir pressure, and arterial flow were measured with VAVD turned off to record baseline values. Four other conditions were tested with progressively higher vacuum pressures (-20, -40, -60, and -80 mmHg) applied to the baseline cardiotomy pressure. An arterial filter was placed into the circuit and arterial flow was measured with the purge line in both open and closed positions. These trials were repeated at set arterial flow rates of 1500, 2000, and 2500 mL/min. RESULTS: The use of progressively higher vacuum caused a reduction in effective arterial flow from 1490±0.00 to 590±0.00, from 2020±0.01 to 1220±0.00, and from 2490±0.0 to 1830±0.01 mL/min. Effective forward flow decreased with increased levels of VAVD. CONCLUSION: The use of VAVD reduces arterial flow when a CP is used as the main arterial pump. The reduction in the forward arterial flow increases as the vacuum level increases. The loss of forward flow is further reduced when the arterial filter purge line is kept in the recommended open position.An independent flow probe is essential to monitor pump flow during cardiopulmonary bypass.

Early outcomes of transcatheter tricuspid valve-in-valve implantation: a case series.

OBJECTIVES: In this study, we sought to evaluate early outcomes of transcatheter valve-in-valve implantation in patients with a degenerated bioprosthesis in the tricuspid position. METHODS: We used a prospective study, which included patients with a degenerated bioprosthesis in the tricuspid position who were considered high-risk by our heart team and who underwent a valve-in-valve implantation. The procedures were performed via transjugular venous access and were done with the patient under general anaesthesia with transoesophageal echocardiographic and fluoroscopic guidance. Perioperative data were analysed, and the patients were followed prospectively. RESULTS: Seven patients were included. The baseline diagnoses were 3 patients with Ebstein's anomaly, 1 patient with tetralogy of Fallot, 1 patient with neonatal endocarditis, 1 patient with ventricular septal defect with a double tricuspid lesion and 1 patient with rheumatic mitral and tricuspid valve who underwent a combined transapical mitral and transjugular tricuspid valve-in-valve implantation. The mean age was 33 ± 10.8 years and 57.1% of the patients were men. The mean number of previous thoracotomies was 3 ± 2 (range 1-5) procedures. The mean follow-up was 1.24 years. The implant was successful in all cases, and there was no need for conversion to open surgery. There were no deaths during the study period, and all patients are currently in the New York Heart Association functional class I/II. There was a statistically significant difference when the mean transvalvular gradients preimplantation and postimplantation were compared (P < 0.001). CONCLUSIONS: Percutaneous tricuspid valve implantation should be considered a safe and effective therapy and stands as a viable, reliable alternative for the treatment of a degenerated bioprosthesis in high-surgical-risk/inoperable patients.

Early and Long-Term Outcomes of Surgical Treatment of Ebstein's Anomaly.

OBJECTIVE: This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes. METHODS: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. RESULTS: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. CONCLUSION: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.