a sword threatening the heart the scimitar syndrome

Abstract In this review, we discuss the natural and surgical history of patients with scimitar syndrome. We describe the clinical presentation, the different forms and symptoms that can be identified and the possible surgical approaches used. A discussion is also provided on the outcomes of patients undergoing surgery compared to those undergoing medical treatment. The choice of appropriate treatment is still challenging today, but new data and large multicenter studies have shown interesting new results

repair of congenital mitral valve dysplasia in infants and children is it always possible

Objectives: Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. Methods: Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days‐18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). Results: Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months‐12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. Conclusions: Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses. q 2000 Elsevier Science B.V. All rights reserved

Right atrium and superior vena cava pressure measurements in a novel animal model to study one and a half ventricle repair as compared to Fontan type procedure

Background & Objectives: To evaluate the advantages of the one and a half ventricle repair on maintaining a low pressure in the inferior vena cava district. Also evaluate the competition of flows at the superior vena cava – right pulmonary artery anastomosis site, in order to understand the hemodynamic interaction of a pulsatile flow in combination to a laminar one. Materials & Methods: Adult rabbits (n=30) in terminal anaesthesia with a follow up of 8 h were used, randomly distributed in three experimental groups: Group 1: animals with an anastomosis between superior vena cava and right pulmonary artery, as a model of one and one half ventricle repair; Group 2: animals with the cavopulmonary anastomosis followed by clamping of the right pulmonary artery proximal to the anastomosis; and Group 3: sham animals. Pressures of superior vena cava and pulmonary arteries were afterwards measured, in a resting condition as well as after induced pharmacological stress test.Results: In Group 1, superior vena cava pressure was significantly higher, while venous pressure in the inferior vena cava – right atrium district was constant or lower in comparison with the other groups. After stress test, the pressure in the superior vena cava and the heart rate both increased further, but the right ventricular, right atrial and pulmonary artery pressures remained similar to the values in a resting condition. This proved that the inferior vena cava return was well-preserved, and no venous hypertension was present in the inferior vena cava district even after stress test (good exercise tolerance).Conclusion: One and one half ventricle repair can be considered a good surgical strategy for maintaining a low pressure in the inferior vena cava district with potential for right ventricle growth, restoring the more physiological circulation in borderline or failing right ventricle conditions. The experiment presented a positive finding in favour of one and one half ventricle repair, as compared to Fontan type procedure

Surgical Versus Percutaneous Occlusion of Ostium Secundum Atrial Septal Defects Results and Cost-Effective Considerations in a Low-Income Country

ObjectivesWe compared the effectiveness and cost of percutaneous occlusion using an Amplatzer septal occluder (ASO) (AGA Medical Corp., Golden Valley, Minnesota) device compared with surgical closure of an ostium secundum atrial septal defect (ASD II) in Guatemala.BackgroundThe percutaneous occlusion of ASD II in first-world nations seems to offer better clinical results and lower cost compared with surgical closure.MethodsWe reviewed the clinical course of 111 patients referred to our institution for closure of isolated ASD II. Successful closure was assessed immediately after the procedures and at 12 months. Actual hospital costs were calculated for every patient who underwent either of the two procedures.ResultsEighty-three patients with ASD II (75%) were selected for percutaneous occlusion with the ASO device, and the remaining 28 patients (25%) underwent surgical closure. In the device group, in 72 patients (86.7%) devices were successfully deployed. At immediate and 12-month follow-up, the complete closure rate was 87.5% (63 of 72 patients) and 97.2% (70 of 71 patients), respectively. In the surgical group, all patients had successful closure immediately after the procedure and at 12 months. Surgical closure offered a 27% cost savings in comparison with percutaneous occlusion (U.S. $3,329.50 ±$411.30 and U.S. $4,521.03 ±$429.71; p < 0.001, respectively). Cost of the device (U.S. $2,930.00) proved to be the main cause for this difference.ConclusionsWe confirmed the clinical advantages of percutaneous occlusion over surgical closure of ASD II. However, percutaneous occlusion costs were higher compared with surgical closure. In Guatemala, where health care resources are limited, ASD II closure with the ASO device did not prove to be cost-effective

Minimally Invasive Approach in Surgery for Congenital Heart Disease

Surgery for congenital heart disease (CHD) has changed considerably during the last decade. Improved surgical results in patients with simple CHD and new interventional cardiology procedures have stimulated the surgeon to adopt minimally invasive techniques to reduce the patient’s surgical insult and obtain good functional and cosmetic results. As a consequence, new surgical techniques and specialized equipment for minimally invasive cardiac surgery (MICS) procedures have been developed and refined in recent years. The improving surgical outcomes in patients with CHD, the significant advances in surgical instrumentation and perfusion technology, and the broad utilization of new catheter-based interventional procedures to repair simple CHD have triggered surgeons’ interest to adopt and innovate minimally invasive approaches for CHD repair, so as to reduce patient’s surgical trauma and improve functional and cosmetic results while maintaining a high standard of clinical outcomes and possibly shortening hospitalization times. This article reports on our updated full experience and institutional protocols with MICS in children and adults with CHD

Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Europe and China:An Analysis of the European Congenital Heart Surgeons Association Congenital Heart Surgery Database

Background: The European Congenital Heart Surgeons Association (ECHSA) Congenital Heart Surgery Database (CHSD) was founded in 1999 and is open for worldwide participation. The current dataset includes a large amount of surgical data from both Europe and China. Thepurposeofthisanalysisistocomparepatternsof practice and outcomes among pediatric congenital heart defect surgeries in Europe and China using the ECHSA-CHSD. Methods: We examined all European (125 centers, 58,261 operations) and Chinese (13 centers, 23,920 operations) data in the ECHSA-CHSD from 2006-2018. Operative mortality, postoperative length of stay, median patient age and weight were calculated for the ten benchmark operations for China and Europe, respectively. Results: Benchmark procedure distribution frequencies differed between Europe and China. In China, ventricular septal defect repair comprised approximately 70% of procedures, while Norwood operations comprised less than one percent of all procedures. Neonatal cardiac procedures were rare in China overall. For procedures in STAT mortality category 1, Chinese centers had lower operative mortality rates, while procedures in categories 3 and 5 mortality is lower in European centers. Operative mortality over the time period decreased from 3.89% to 1.64% for the whole cohort, with a sharper decline in China. This drop coincides with an increase of submitted procedures over this 13-year-period. Conclusion: Chinese centers had higher program-matic volume of congenital heart surgeries, while European centers have a more complex case mix. Palliation for patients with functionally univentricular heart was performed less commonly in China. These comparison of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement

Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Japan and Europe

Objectives: The Japan Cardiovascular Surgery Database-Congenital section (JCVSD-Congenital) and the European Congenital Heart Surgeons Association (ECHSA) Congenital Heart Surgery Database (CHSD) share the same nomenclature. We aimed at comparing congenital cardiac surgical patterns of practice and outcomes in Japan and Europe using the JCVSD-Congenital and ECHSA-CHSD. Methods and Results: We examined Japanese (120 units, 63,365 operations) and European (96 units, 90,098 operations) data in JCVSD-Congenital and ECHSA-CHSD from 2011 to 2017. Patients' age and weight, periprocedural times, mortality at hospital discharge, and postoperative length of stay were calculated for ten benchmark operations. There was a significantly higher proportion of ventricular septal defect closures and Glenn operations and a significantly lower proportion of coarctation repairs, tetralogy of Fallot repairs, atrioventricular septal defect repairs, arterial switch operations, truncus repairs, Norwood operations, and Fontan operations in JCVSD-Congenital compared to ECHSA-CHSD. Postoperative length of stay was significantly longer following all benchmark operations in JCVSD-Congenital compared to ECHSA-CHSD. Mean STAT mortality score (Society of Thoracic Surgeons European Association for Cardio-Thoracic Surgery mortality score) was significantly higher in JCVSD-Congenital (0.78) compared to ECHSA-CHSD (0.71). Mortality at hospital discharge was significantly lower in JCVSD-Congenital (4.2%) compared to ECHSA-CHSD (6.0%, P < .001). Conclusions: The distribution of the benchmark procedures and age at the time of surgery differ between Japan and Europe. Postoperative length of stay is longer, and the mean complexity is higher in Japan compared to European data. These comparisons of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement

Right atrium and superior vena cava pressure measurements in a novel animal model to study one and a half ventricle repair as compared to Fontan type procedure

Background &amp; Objectives: To evaluate the advantages of the one and a half ventricle repair on maintaining a low pressure in the inferior vena cava district. Also evaluate the competition of flows at the superior vena cava – right pulmonary artery anastomosis site, in order to understand the hemodynamic interaction of a pulsatile flow in combination to a laminar one. Materials &amp; Methods: Adult rabbits (n=30) in terminal anaesthesia with a follow up of 8 h were used, randomly distributed in three experimental groups: Group 1: animals with an anastomosis between superior vena cava and right pulmonary artery, as a model of one and one half ventricle repair; Group 2: animals with the cavopulmonary anastomosis followed by clamping of the right pulmonary artery proximal to the anastomosis; and Group 3: sham animals. Pressures of superior vena cava and pulmonary arteries were afterwards measured, in a resting condition as well as after induced pharmacological stress test.Results: In Group 1, superior vena cava pressure was significantly higher, while venous pressure in the inferior vena cava – right atrium district was constant or lower in comparison with the other groups. After stress test, the pressure in the superior vena cava and the heart rate both increased further, but the right ventricular, right atrial and pulmonary artery pressures remained similar to the values in a resting condition. This proved that the inferior vena cava return was well-preserved, and no venous hypertension was present in the inferior vena cava district even after stress test (good exercise tolerance).Conclusion: One and one half ventricle repair can be considered a good surgical strategy for maintaining a low pressure in the inferior vena cava district with potential for right ventricle growth, restoring the more physiological circulation in borderline or failing right ventricle conditions. The experiment presented a positive finding in favour of one and one half ventricle repair, as compared to Fontan type procedure.</p

Survival and Clinical Course at Fontan After Stage One Palliation With Either a Modified Blalock-Taussig Shunt or a Right Ventricle to Pulmonary Artery Conduit

ObjectivesWe sought to determine whether the type of shunt used at stage one palliation (S1P) affected the survival and the perioperative course through Fontan completion.BackgroundAlthough improved surgical and interstage survival have been demonstrated with the use of the right ventricle to pulmonary artery (RV-PA) conduit compared with a modified Blalock-Taussig shunt (BTS) at S1P, it is unknown whether this effect will be observed in long-term follow-up.MethodsAll patients who underwent a S1P during 2002 and 2003 (n = 80) at our institution were included for analysis. Patients were followed until death or June 1, 2007. Perioperative variables at Fontan completion were recorded.ResultsFor the entire cohort, cumulative survival for those who underwent a RV-PA conduit (n = 34) was 79.4% at 3 years compared with 65.8% in the modified BTS group (n = 46) (log-rank = 0.31). At Fontan (n = 44), when compared with those who had received a modified BTS, those who had a RV-PA conduit placed at S1P had no difference in the median duration of ventilation (21 h [range 10 to 96 h] vs. 26.5 h [range 7 to 204 h], p = 0.09) or hospital stay (9 days [range 5 to 29 days] vs. 10 days [range 6 to 48 days], p = 0.89), although length of stay in the intensive care unit was shorter (2 days [range 0 to 6 days] vs. 4 days [range 1 to 25 days], p = 0.01). Sixty-seven percent of the RV-PA conduit group had at least one PA intervention 3 years after S1P compared with 42.8% in the modified BTS group (log-rank = 0.11).ConclusionsNonstatistically significant trends toward improved cumulative survival and increased PA interventions were demonstrated in patients who had a RV-PA conduit placed at S1P. Longitudinal follow-up of larger groups of randomized patients is required to determine the influence of the RV-PA conduit on long-term outcomes