Minimally invasive valve-sparing aortic root replacement via an upper mini-sternotomy in a young adult with Marfan syndrome.

We describe an upper mini-sternotomy for valve-sparing aortic root replacement in a teenager.

Use of low titer O whole blood in infants and young children undergoing cardiac surgery with cardiopulmonary bypass.

BACKGROUND: Low titer group O whole blood (LTOWB) is commonly used for severe bleeding in trauma patients. LTOWB may also benefit young children requiring cardiac surgery with cardiopulmonary bypass (CPB) at risk of severe bleeding. STUDY DESIGN AND METHODS: In this retrospective study, children <2 years old who underwent cardiac surgery with CPB were included. Comparisons were performed between those receiving component therapy (CT) versus those receiving LTOWB plus CT (LTOWB+CT). Outcomes included drainage tube (DT) output and total transfusion volumes. Optimization-based weighting was used for adjusted analyses between groups. RESULTS: There were 117 patients transfused with only CT and 127 patients transfused with LTOWB+CT. In the LTOWB+CT group, 66 were Group non-O and 61 were Group O. Total transfusion volumes given from the start of the operation until the first 24 h in the cardiac intensive care unit was a median (IQR) 41 (10, 93) mL/kg in the CT group and 48 (28, 77) mL/kg in the LTOWB+CT group, (p = .28). Median (IQR) DT output was 22 (15-32) in CT versus 22 (16-28) in LTOWB+CT groups, (p = .27). There were no differences in death, renal failure and a composite of death and renal failure between the two groups, but there were statistically fewer re-explorations for bleeding in the LTOWB+CT group (p < .001). CONCLUSIONS: The use of LTOWB appears to be safe in <2 years old undergoing cardiac surgery and may reduce re-explorations for severe bleeding. Large trials are needed to determine the efficacy and safety of LTOWB in this population with severe bleeding.

Hybrid stage 1 Norwood combined with reverse Blalock-Taussig shunt and sutureless repair of total anomalous pulmonary venous connections.

The patient is a 35-week-old, 2.6-kg neonate who was diagnosed prenatally with hypoplastic left heart syndrome and a supracardiac variant of total anomalous pulmonary venous connection. We present the technique of stage 1 hybrid palliation combined with repair of total anomalous pulmonary venous connection. Due to the risk of retrograde aortic arch obstruction from the ductal stents, a reverse Blalock-Taussig shunt was performed as well.

Third sternotomy with modified Bentall/Konno procedure for recurrent multilevel left ventricular outflow tract obstruction in a 15-year-old.

This is a 15-year-old, 73.5 kg male who was born with congenital aortic valve stenosis. He underwent a transcatheter balloon aortic valvuloplasty in the neonatal period, followed by an open aortic valvotomy at 2 months of age. When he was 3 years old, he underwent aortic root replacement with a 15 mm aortic homograft. Recently, he presented with recurrent left ventricular outflow tract obstruction that appeared valvular and subvalvular in nature. We present our technique of repeat aortic root replacement and left ventricular outflow tract enlargement using modified Bentall combined with Konno-Rastan aortoventriculoplasty.

Complex Pulmonary Artery Rehabilitation in Children with Alagille Syndrome: An Early Single-Center Experience of a Successful Collaborative Work.

Objective: In this paper, we share our single-center experience of successful multidisciplinary management of patients with Alagille syndrome. In addition, we aim to highlight the need for an Alagille program for effectively managing these patients, in general, and particularly peripheral pulmonary artery stenosis associated with this syndrome. Study Design: This is a retrospective review of six children with Alagille syndrome and advanced liver involvement who underwent pulmonary artery reconstruction between 2021 and 2022. Cardiac diagnosis, co-existing liver disease burdens, management approach, and short-term outcomes were analyzed. Results: All the patients underwent one-stage extensive bilateral branch pulmonary rehabilitation. Concomitant procedures included repair of tetralogy of Fallot in one patient and repair of supravalvar pulmonary artery stenosis in two. One patient had balloon pulmonary branch angioplasty before surgery. In all patients, there was a decrease in right ventricular systolic pressure post-operatively. Three patients underwent liver transplantation for pre-existing liver dysfunction. At a median 3-year follow-up, all the patients were alive with their right ventricular systolic pressure less than half of their systemic systolic pressure. One patient underwent balloon angioplasty due to new and recurrent left pulmonary artery stenosis 13 months after surgery. Conclusion: Pulmonary arteries can be successfully rehabilitated surgically in the presence of complex branch disease. Patients with advanced liver disease can undergo successful complex pulmonary artery reconstruction, which can facilitate their future liver transplantation course. A multidisciplinary team approach is a key for successful management of Alagille patients.

Diagnostic work-up and current management strategies for infective endocarditis in the pediatric population.

Infective endocarditis (IE) remains a serious disease that is associated with significant morbidity and mortality, and despite the significant advances that have been made in understanding the disease process in past decades, its incidence appears to be on the rise recently. Endocarditis in children is no longer a rare occurrence. This appeared to be related to a combination of the improved survival of children with congenital heart diseases (CHDs), increase use of intracardiac protheses, and catheter-related interventions. The American Heart Association (AHA) 2007 guidelines reduced the recommendations for use of prophylactic antibiotics in those with CHDs which occurred despite the noticeable increase in endocarditis incidence around that time. In general, the recommendations for managing children with IE are derived from the adults' guidelines, and the evidence-base is lacking in many clinical scenarios. Understanding the epidemiology, clinical presentations, microbiology, and outcomes of different management strategies for endocarditis is needed to have a clear and optimal plan for these children. In the current narrative review, we discuss IE in the pediatric population in terms of etiology, predisposing factors, and different treatment strategies for this unique population.

Aortic root anatomy: insights into annular and root enlargement techniques.

The introduction of the Y(ang)-technique for aortic root enlargement has sparked a renewed interest in annular and root enlargement procedures world-wide. In order to execute these procedures proficiently however, it's important to understand the complex three-dimensional structure of the aortic root and left ventricular outflow tract, and also be familiar with the different enlargement techniques. Herein, we are providing a description of the aortic root anatomy and the most commonly utilized root enlargement procedures. This should facilitate clinical decision making and guidance of patients towards the most appropriate procedure, which should not only treat the patients' acute symptoms, but should also set the patient up for potentially needed future procedures and respective life-time management of aortic valve disease.

Improved Early Outcomes With Off-Pump Coronary Artery Bypass Grafting in Patients With Left Ventricular Dysfunction: A Systematic Review and Meta-Analysis.

The ongoing debate surrounding coronary artery bypass grafting (CABG) with or without cardiopulmonary bypass persists, particularly in individuals with left ventricular dysfunction. The objective of this study was to evaluate the safety and efficacy of these 2 strategies through a comprehensive meta-analysis of existing studies. A systematic search of PubMed, EMBASE, Web of Science, and the Cochrane Central Registry was conducted from inception to July 2023. The primary focus was on studies comparing on-pump versus off-pump CABG as the primary treatment for multivessel coronary artery disease in patients with left ventricular dysfunction (ejection fraction ≤40%), with mortality as the primary outcome. The meta-analysis included 26 studies with a total of 35,863 patients. The results revealed a significant reduction in mortality risk [risk ratio (RR), 0.75; 95% confidence interval (CI), 0.60-0.93; P = 0.009] and other perioperative morbidities associated with off-pump CABG. These included stroke (RR, 0.67; 95% CI, 0.54-0.82; P = 0.0002), myocardial infarction (RR, 0.74; 95% CI, 0.56-0.97; P = 0.03), pulmonary complications (RR, 0.71; 95% CI, 0.55-0.92; P = 0.010), postoperative transfusion (RR, 0.70; 95% CI, 0.55-0.88; P = 0.002), neurological dysfunction (RR, 0.80; 95% CI, 0.64-1.00; P = 0.05), infection (RR, 0.74; 95% CI, 0.56-0.97; P = 0.03), renal failure (RR, 0.79; 95% CI, 0.67-0.95; P = 0.010), and reoperation for bleeding (RR, 0.66; 95% CI, 0.52-0.84; P = 0.0006). However, no significant difference was observed between the 2 groups regarding postoperative atrial fibrillation (RR, 0.97; 95% CI, 0.84-1.12; P = 0.69). In conclusion, off-pump CABG demonstrates a lower perioperative mortality risk and improved overall early outcomes compared with on-pump techniques in individuals with reduced left ventricular function.

Efficacy of automated fasteners versus hand-tied knots in cardiac surgery: a systematic review and meta-analysis.

Valve surgery is common in cardiac procedures, with fasteners like COR-KNOT® and hand-tied knots used for knot securing. This study compares their efficacy in valve surgery patients. We searched PubMed, SCOPUS, and Cochrane Central until August 2023. Outcomes assessed included aortic cross-clamp time (AXT), cardiopulmonary bypass (CPB) time, valvular regurgitation, mortality, prolonged ventilatory support, atrial fibrillation, postoperative left ventricular ejection fraction (LVEF), and renal failure. Subgroup analysis was performed for minimally invasive and open cardiac surgery. We used a random effects model for analysis. We included eight observational studies and two randomized controlled trials (RCTs) with a total of 1.411 participants. COR-KNOT significantly reduced AXT [MD -15.14, 95 % CI (-18.57, -11.70), P<0.00001] and CPB time [MD -12.38, 95 % CI (-14.99, -9.77), P<0.00001]. Valvular regurgitation [RR 0.40, 95 % CI (0.26, 0.61), P<0.0001] and need for prolonged ventilatory support [RR 0.29, 95 % CI (0.13, 0.65), P=0.003] were significantly lower with COR-KNOT. There were no significant differences in mortality [RR 0.39, 95 % CI (0.09, 1.69), P=0.44], atrial fibrillation [RR 1.03, 95 % CI (0.83, 1.27), P=0.81], LVEF changes [MD 0.05, 95 % CI (-1.37, 1.47), P = 0.95], or renal failure [RR 0.87, 95 % CI (0.16, 4.80), P = 0.87]. COR-KNOT devices reduce operative time and valvular regurgitation without increasing mortality or adverse outcomes. This supports their use in enhancing surgical efficiency and patient outcomes. However, ongoing discussions about suturing techniques, especially in minimally invasive procedures, highlight the need for further research and consensus among practitioners. See also the graphical abstract(Fig. 1).

Combined transaortic/transapical left ventricular septal myectomy for subaortic and midventricular obstruction in a paediatric patient.

The patient is a 15-year-old girl who was diagnosed with hypertrophic cardiomyopathy and has been symptomatic due to severe left ventricular outflow tract obstruction. Combined transaortic and transapical left ventricular septal myectomy was performed to relieve the left ventricular outflow tract obstruction and address both subaortic and midventricular gradients.

Partial anomalous venous connection with intrapulmonary dual drainage: Transcatheter treatment of a rare entity.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital condition, and dual-drainage connection PAPVR to the left atrium has been reported in a few cases in the literature; in which cases, percutaneous catheterization was successfully used in lieu of surgery. We, hereby, describe a 7-month-old boy with a functional single-ventricle physiology with dual drainage of the left upper pulmonary vein to the left atrium and the innominate vein. Appropriate recognition of this entity allowed safe occlusion of the anomalous draining vein.

Late complications after Norwood stage 1 palliation: surgical strategies and a word of caution.

The current case represents one of the complications that may occur when inserting a stent in the left pulmonary artery after a previous Norwood procedure and in the presence of an aneurysmal neo-aorta and large Damus-Kaye-Stansel connection. We demonstrate the technique of a fourth sternotomy with reconstruction of the left pulmonary artery and the neo-aorta in a 12-year-old boy with a functional single ventricle who underwent all three previous stages of palliation of hypoplastic left heart syndrome.

Repair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Safety and Efficacy of Right Axillary Thoracotomy for Repair of Congenital Heart Defects in Children.

BACKGROUND: Minimally invasive repair of congenital heart defects in children has not gained wide popularity yet compared to minimally invasive approaches in adults. We sought to review our experience with this approach in children. METHODS: This study included a total of 37 children (24 girls, 64.9%) with a mean age of 6.5 ± 5.1 years, who underwent vertical axillary right minithoracotomy for repair of a variety of congenital heart defects between May 2020 and June 2022. RESULTS: The mean weight of these children was 25.66 ± 18.3 kg. Trisomy 21 syndrome was present in 3 patients (8.1%). The most common congenital heart defects that were repaired via this approach were atrial septal defects (secundum in 11 patients, 29.7%; primum in 5, 13.5%; and unroofed coronary sinus in 1, 2.7%). Twelve patients (32.4%) underwent repair of partial anomalous pulmonary venous connections with or without sinus venosus defects, while 4 patients (10.8%) underwent closure of membranous ventricular septal defects. Mitral valve repair, resection of cor triatriatum dexter, epicardial pacemaker placement, and myxoma resection occurred in 1 patient (2.7%) each. No early mortality or reoperations. All patients were extubated in the operating room, and the mean length of hospital stay was 3.3 ± 2.04 days. Follow-up was complete (mean 7 ± 5 months). No late mortality or reoperations. One patient required epicardial pacemaker placement due to sinus node dysfunction 5 months after surgery. CONCLUSIONS: Vertical axillary right thoracotomy is a cosmetically superior approach that is safe and effective for repair of a variety of congenital heart defects in children.

Single-incision, off-pump repair of a right aortic arch with an aberrant left subclavian artery and diverticulum of Kommerell in an adult.

Repairing a complete vascular ring in adults can be challenging. A right aortic arch with an aberrant retro-oesophageal left subclavian artery and persistent diverticulum of Kommerell represents one of the most common variants seen in adults, and the ring is completed by the left-sided ligamentum arteriosum. Most presentations in adults occur secondary to oesophageal compression, resulting in varying degrees of dysphagia. Owing to the difficulty and challenges associated with exposure in adults, it is not unusual for surgeons to offer a two-incision approach or to stage the procedure. We present a detailed surgical technique for a single-incision repair of a right aortic arch with an aberrant retro-oesophageal left subclavian artery via a left posterolateral thoracotomy.

Early Concerning Outcomes for the Edwards Inspiris Resilia Bioprosthesis in the Pulmonary Position.

BACKGROUND: The Inspiris Resilia (Edwards Lifesciences LLC) bioprosthesis has gained widespread use in the aortic position; however no robust data are available about its use in the pulmonary position. METHODS: We reviewed our outcomes for the Inspiris Resilia in the pulmonary position between August 2019 and October 2021. RESULTS: Twenty-seven patients (15 female patients [56%]; mean age, 22.26 ± 14.99 years) were included. The most common original pathology was tetralogy of Fallot (13 patients, 48%). Five patients (18.5%) had prior transcatheter interventions. The mean right ventricular end-diastolic volume index was 164.25 ± 45.1 mL/m2. Sternotomy or repeat sternotomy was the most common approach (21 patients, 77.8%). The standard technique for pulmonary valve replacement was used in 22 patients (81.5%), whereas the prosthesis was implanted as a conduit in the remaining 5 (18.5%). Trivial to mild prosthetic regurgitation was present in 6 patients (22.2%) at the time of discharge. There was no early mortality. Follow-up was complete in all patients (mean, 16 ± 8 months), with no late mortality or late reoperations. New prosthetic regurgitation developed in 13 patients (48%), all of whom underwent replacement with the standard surgical technique. No regurgitation occurred in the conduit cases. This progressed to moderate regurgitation in 6 patients (22%) and severe in 3 (11%). Three patients (11%) underwent transcatheter valve-in-valve after their surgical pulmonary valve replacement. CONCLUSIONS: The early data regarding the Inspiris Resilia bioprosthesis use in the pulmonary position is concerning. The prosthesis design may not be suitable for low-pressure circulation, or modification of its implantation technique may be needed.