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.

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).

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.

Surgical Experience With Crossed and Anomalous Origin of the Pulmonary Arteries From the Pulmonary Trunk: A Single Center Report of 24 Cases.

BACKGROUND: Anomalies of the pulmonary origin of the pulmonary arteries are uncommon; however, the true incidence is unknown and may be underestimated. We sought to review our experience with this unusual pathology and examine its surgical implications. METHODS: We reviewed medical records between 2011 and 2022. RESULTS: A total of 24 patients were identified. Genetic syndromes were present in 12 patients (50%). The patients were divided into two main groups. Those with septal defects (14 patients,58.3%), and those without (10 patients, 41.7%). Aortic arch hypoplasia with/without coarctation was present in nine patients (37.5%). Pulmonary arterial branch hypoplasia and/or obstruction was present in six patients (25%) with resultant preoperative systemic or suprasystemic right ventricular pressure. Four patients (16.7%) underwent pulmonary artery branch and/or right ventricular outflow tract interventions prior to surgery. All patients underwent surgical interventions for their associated cardiac defects. The crossed pulmonary arteries were uncrossed for those four patients (16.7%) who required concomitant pulmonary arterioplasties and pulmonary arterial branch rehabilitation and who had elevated right ventricular pressures preoperatively. No early or late mortalities. One Alagille's syndrome underwent liver transplant prior to discharge. Late transcatheter interventions were needed in four (16.7%) during the follow-up period, while four patients required repeat aortic arch augmentation for recurrent arch obstruction. CONCLUSIONS: Anomalous origin of the pulmonary arteries from the pulmonary trunk including crossed pulmonary arteries is an uncommon anomaly that may be underrecognized. It can occur in isolation or in association with other heart defects. Recognition of this malpositional anomaly is important as it has specific surgical and/or transcatheter implications.

Towards Zero Phrenic Nerve Injury in Reoperative Pediatric Cardiac Surgery: The Value of Intraoperative Phrenic Nerve Stimulation.

BACKGROUND: Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. METHODS: Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). RESULTS: There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). CONCLUSION: Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

Bilateral branch pulmonary arterial reconstruction in the Alagille syndrome: The technique and its pitfalls.

This tutorial describes in detail the surgical technique of bilateral branch pulmonary arterial reconstruction in a 10-month-old boy with Alagille syndrome and advanced liver disease. The procedure was performed via a standard median sternotomy and moderate hypothermia and involves bilateral pulmonary branch arterioplasties combined with relief of valvular and supravalvular pulmonary stenosis and subtotal closure of secundum atrial septal defect. The patient presented with systemic/suprasystemic right ventricular pressure.

Cor triatriatum sinister: Long-term surgical outcomes in children and a proposal for a new classification.

BACKGROUND: Cor Triatriatum Sinister (CTS) is a rare congenital anomaly with an estimated incidence of 0.4%, resulting from abnormal left atrial septation. It may present in isolation or in association with other heart defects. High percentage of patients require surgery with low mortality and recurrence rates. CTS has been classified in the past however we aim to describe a case series with more comprehensive and inclusive classification. METHODS: This was a single-center retrospective cohort study of 16 children with the diagnosis of CTS between 2000 and 2020. Medical records were reviewed for clinical presentations, hospital, and postoperative courses. RESULTS: Sixteen patients (63% female), with a median age at diagnosis of 4.3 months, five (31%) were neonates. Six (38%) had isolated CTS, two (13%) with functional single ventricle (SV), and the remaining eight patients (50%) had other associated heart defects (septal defects in three, coarctation of the aorta in another three, and anomalous pulmonary venous connections in three). Eight patients (50%) presented with obstructed CTS. Twelve patients (75%) underwent surgical intervention. Mortality occurred in three patients (19%) with two surgical (one with total anomalous pulmonary venous connection and another with SV) and one nonsurgical (septal defect with Fanconi anemia). The surgical median follow up was 4.7 years. Recurrence of the membrane occurred in two patients (17%). CONCLUSIONS: This study showed good long-term outcomes for patients with isolated and complex CTS. Complete and proper classification of CTS ensures appropriate diagnosis, surgical planning, and better family counseling which may correlate with outcomes.

Descending thoracic aortic homograft for superior vena caval translocation in the Warden procedure: Report of six cases.

BACKGROUND: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. METHODS: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. RESULTS: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. CONCLUSIONS: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.

Successful rapid staged hybrid approach for obstructed total anomalous pulmonary venous connection.

Obstructed total anomalous pulmonary venous connection (TAPVC) represents a true pediatric cardiac emergency. The patient may present in extremis secondary to severe pulmonary hypertension and cardiogenic shock which increases perioperative mortality. We present a neonate who underwent a successful staged hybrid approach for an Infradiaphragmatic obstructed TAPVC.