Editorial: Coronary Artery Anomalies: A 2020 Review.

No abstract availabl

Extracorporeal membrane oxygenation: The simplified weaning bridge

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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

Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing.

Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are a challenge because of their various anatomic and clinical presentation. Although the prevalence is low, the absolute numbers of detected ACAOS are increasing because of the growing use of noninvasive anatomical imaging for ruling out coronary artery disease. As evidence-based guidelines are lacking, treating physicians are left in uncertainty for the optimal management of such patients. The sole presence of ACAOS does not justify surgical correction, and therefore a thorough anatomic and hemodynamic assessment is warranted. Invasive and noninvasive multimodality imaging provides information to the clinical question whether the presence of ACAOS is an innocent coincidental finding, is responsible for the patient's symptoms, or even might be a risk for sudden cardiac death. Based on recent clinical data, focusing on the pathophysiology of patients with ACAOS, myocardial ischemia is dependent on both the extent of fixed and dynamic components, represented by anatomic high-risk features. These varying combinations should be considered individually in the decision making for the different therapeutic options. This state-of-the-art review focuses on the advantages and limitations of the common contemporary surgical, interventional, and medical therapy with regard to the anatomy and pathophysiology of ACAOS. Further, we propose a therapeutic management algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge

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

610 Fontan associated kidney and liver disease: can we predict organ involvement with echocardiographic assessment of systolic function and atrioventricular valve insufficiency?

Abstract Aims Fontan operation represents the surgical palliative option for congenital heart disease with single ventricle physiology. With the improvement of surgical and percutaneous technique, we are facing a growing population of patients with an unique pathophysiology and potential complications. Methods and results Patients that underwent Fontan palliation in our centre between 1993 and 2016 were included in this prospective study. We excluded patients with major congenital renal anomalies, those that underwent cardiac transplantation, and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old, median age 15 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Regarding to cardiac anatomy, 10 patients had functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); 4 patients had undetermined single ventricle (11.5%). Total cavo-pulmonary connection (TCPC) with intracardiac lateral tunnel was performed in 7 patients (20%, N = 7), whereas 28 patients had TCPC with external conduit (80%). Data from echocardiographic evaluation showed a medium EF established with Simpson's method of 60% in patients with FSLV; patients with a FSRV or undetermined single ventricle had a medium FAC of 41.1%, with 15.1% having a reduced FAC < 35%. No FSLV patients had an EF < 50%. When using creatinine-based formula, data about renal function in our population showed a stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq) in 11% of total population (N = 4), that became 26% when using cystatin C-based equation (N = 9), with one patient showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min 1.73 mq). Urinalysis showed 29% (N = 10) of patients having microalbuminuria (microalbumin/creatinine ratio between 30 and 300 mg/g). Statistical analysis demonstrated a negative correlation between systolic function (TAPSE for FSRV) and cystatin C blood levels (Pearson's R −0.428, P = 0.053), and between systolic function (FAC and Simpson) and microalbuminuria (Pearson's R −0.414 with P = 0.049 and Pearson's R −0.754 with P = 0.019, respectively). Transient elastography reported 10 patients (29.4%) with abnormal hepatic stiffness for Fontan patients. That condition appeared to be more frequent in patients with higher grade of AV valve insufficiency (P < 0.05). Conclusions Our population showed an higher prevalence of FSRV Fontan patients, with an expected lower systolic function compared with FSLV. 2D evaluation of systolic function showed a linear inverse correlation with renal function, suggesting that Fontan patients need a closer renal monitoring. Hepatic stiffness, which is a warning sign of potential hepatic cirrhosis need to be monitored in all Fontan patients, especially those with a worse AV valve insufficiency

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

Correlations between the alpha-Gal antigen, antibody response and calcification of cardiac valve bioprostheses: experimental evidence obtained using an alpha-Gal knockout mouse animal model

IntroductionPreformed antibodies against αGal in the human and the presence of αGal antigens on the tissue constituting the commercial bioprosthetic heart valves (BHVs, mainly bovine or porcine pericardium), lead to opsonization of the implanted BHV, leading to deterioration and calcification. Murine subcutaneous implantation of BHVs leaflets has been widely used for testing the efficacy of anti-calcification treatments. Unfortunately, commercial BHVs leaflets implanted into a murine model will not be able to elicit an αGal immune response because such antigen is expressed in the recipient and therefore immunologically tolerated.MethodsThis study evaluates the calcium deposition on commercial BHV using a new humanized murine αGal knockout (KO) animal model. Furtherly, the anti-calcification efficacy of a polyphenol-based treatment was deeply investigated. By using CRISPR/Cas9 approach an αGal KO mouse was created and adopted for the evaluation of the calcific propensity of original and polyphenols treated BHV by subcutaneous implantation. The calcium quantification was carried out by plasma analysis; the immune response evaluation was performed by histology and immunological assays. Anti-αGal antibodies level in KO mice increases at least double after 2 months of implantation of original commercial BHV compared to WT mice, conversely, the polyphenols-based treatment seems to effectively mask the antigen to the KO mice’s immune system.ResultsCommercial leaflets explanted after 1 month from KO mice showed a four-time increased calcium deposition than what was observed on that explanted from WT. Polyphenol treatment prevents calcium deposition by over 99% in both KO and WT animals. The implantation of commercial BHV leaflets significantly stimulates the KO mouse immune system resulting in massive production of anti-Gal antibodies and the exacerbation of the αGal-related calcific effect if compared with the WT mouse. DiscussionThe polyphenol-based treatment applied in this investigation showed an unexpected ability to inhibit the recognition of BHV xenoantigens by circulating antibodies almost completely preventing calcific depositions compared to the untreated counterpart