Early and Late Outcomes after Arterial Switch Operation: A 40-Year Journey in a Single Low Case Volume Center.

Background and Objectives: The results of the arterial switch operation in large congenital heart centers are excellent, and the results in small and medium centers are improving. The objective of this article is to share our experience utilizing the international knowledge transfer program to improve early and late arterial switch operation outcomes in our center. Materials and Methods: A retrospective analysis of patients who underwent the arterial switch operation in Vilnius University Santaros Clinics Cardiothoracic Surgery Center between 1977-2020 was performed. Results: A total of 127 consecutive arterial switch operations were performed in our center. Surgical mortality during the entire study period was 24.6%. Surgical mortality prior to the program, during the program, and after the program was 88.24%, 41.7%, and 5.81%, respectively (p < 0.0001). The surgical mortality of patients operated on during the last 10 years was 4%. The overall survival estimate for the 97 surviving patients was 96.9%, 94.9%, 93.8%, 93.8%, 93.8%, 93.8% at 1, 3, 5, 10, 15, and 20 years, respectively. Risk factors for early mortality included longer aortic cross-clamp time and operation prior to the knowledge transfer program. The only significant risk factor for late reintervention was concomitant aortic arch obstruction treated at the time of the arterial switch. Conclusions: The surgical treatment of transposition of the great arteries by means of an arterial switch with good results can be possible in low-to-medium volume congenital heart surgery centers. International knowledge transfer programs between high-expertise high-volume congenital heart centers and low-to-medium volume congenital heart centers may help to shorten the learning curve and improve early and late outcomes after an arterial switch. The risk factors for surgical mortality and intervention-free survival in low-volume surgical centers are similar to those in high-volume centers. Late arterial switch-related complications are similar to those among different-sized congenital heart centers.

Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum.

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.

Intracoronary recombinant tissue plasminogen activator in an infant with hypoplastic left heart syndrome and complete left main coronary artery thrombosis.

An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.

Assessment of pulmonary veins after atrio-pericardial anastomosis by cardiovascular magnetic resonance.

BACKGROUND: The atrio-pericardial anastomosis (APA) uses a pericardial pouch to create a large communication between the left atrium and the pulmonary venous contributaries in order to avoid direct suturing of the pulmonary veins during the repair of congenital cardiac malformations. Post-operative imaging is routinely performed by echocardiography but cardiovascular magnetic resonance (CMR) offers excellent anatomical imaging and quantitative information about pulmonary blood flow. We sought to compare the diagnostic value of echocardiography and CMR for assessing pulmonary vein anatomy after the APA. METHODS: This retrospective study evaluated all consecutive patients between October 1998 and January 2010 after either a primary or secondary APA followed by post-repair CMR. RESULTS: Of 103 patients who had an APA, 31 patients had an analyzable CMR study. The average time to CMR was 24.6 ± 32.5 months post-repair. Echocardiographic findings were confirmed by CMR in 12 patients. There was incomplete imaging by echocardiography in 7 patients and underestimation of pulmonary vein restenosis in 12, when compared to CMR. In total, 19/31 patients (61%) from our cohort had significant stenosis following the APA as assessed by CMR. Our data suggest that at least 18% (19/103) of all patients had significant obstruction post-repair. CONCLUSIONS: Echocardiography incompletely imaged or underestimated the severity of obstruction in patients compared with CMR. Pulmonary vein stenosis remains a sizable complication after repair, even using the APA.

In vivo h-VEGF165 gene transfer improves early endothelialisation and patency in synthetic vascular grafts.

OBJECTIVES: Small-diameter synthetic vascular graft performance is inferior to autologous vein grafts. This study tested the hypotheses that local in vivo administration of plasmids encoding for human vascular endothelial growth factor (VEGF), or co-administration of plasmids encoding for human vascular endothelial growth factor/plasmids encoding for fibroblast growth factor-2 in the tissues surrounding a porous synthetic vascular graft would enhance graft endothelialisation and, consecutively, graft patency. METHODS: First, optimal gene for small-diameter synthetic graft endothelialisation was studied in rat abdominal aorta model (n=132): plasmids encoding for human vascular endothelial growth factor; co-administration of plasmids encoding for human vascular endothelial growth factor/plasmids encoding for fibroblast growth factor-2; or control plasmids were injected around 60 microm ePTFE graft. Second, optimal small-diameter synthetic graft design for endothelialisation was explored in rabbit abdominal aorta model (n=90). Various ePTFE grafts or pre-clotted polyester grafts were used with/without plasmids encoding for human vascular endothelial growth factor. Third, clinically used medium-size synthetic grafts were investigated with/without plasmids encoding for human vascular endothelial growth factor in dog carotid (n=20) and femoral arteries (n=15). Endothelialisation was assessed in midgraft area with scanning electron microscopy. RESULTS: In rats, plasmids encoding for human vascular endothelial growth factor enhanced endothelialisation; whereas co-administration of plasmids encoding for human vascular endothelial growth factor/plasmids encoding for fibroblast growth factor-2 had worst outcome at 1 week (NS), 2 weeks (P=0.01) and 4 weeks (P=0.02). In rabbits, pre-clotted polyester grafts had a trend for faster endothelialisation than ePTFE grafts (P=0.08); whereas plasmids encoding for human vascular endothelial growth factor enhanced endothelialisation compared to controls at 2 weeks (P=0.06), however, the effect reversed at 4 weeks (P=0.03). In dogs, synthetic graft patency was improved by plasmids encoding for human vascular endothelial growth factor in femoral position (P=0.103); whereas all carotid grafts were patent at 6 weeks. CONCLUSIONS: Thus, these data suggested that endothelialisation was fastest in pre-clotted polyester grafts; and that local application of plasmids encoding for human vascular endothelial growth factor had a potential to improve early endothelialisation and patency in synthetic vascular grafts.

Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot.

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.

Medium term outcome for infant repair in tetralogy of Fallot: Indicators for timing of surgery.

OBJECTIVE: To assess the impact of early corrective surgery on the short and medium term outcome in tetralogy of Fallot (TOF). MATERIALS AND METHODS: All patients under 12 months of age undergoing correction of isolated TOF between February 1997 and July 2003 were reviewed retrospectively. Outcome data for mortality, post-operative care management, major morbidity and clinical follow-up were analysed. RESULTS: Fifty-two operations were performed. The mean age at surgery was 5 months (range 1-12) of whom 16 (30.8%) were less than 3 months old, including 2 neonates, 22 (42.3%) were 3-6 months old and 14 (26.9%) were 7-12 months old. There was 1 (1.9%) early death caused by a cerebro-vascular accident and 1 (1.9%) late death secondary to acute infective endocarditis. There were no differences in post-operative morbidities attributable to age. Patients under 3 months old required greater duration of post-operative ventilation, ITU stay and in-hospital stay. At a mean follow-up of 4.0 years (range 1.5-8.0), 33 (63.5%) patients had well-tolerated pulmonary regurgitation (PR) and 3 (5.8%) patients required re-operation for right ventricular outflow tract obstruction (RVOTO). All patients had right bundle-branch-block but with QRS < 150 ms. CONCLUSION: Early definitive repair of TOF can be performed safely on patients under 6 months old. Age at surgery does not appear to affect the medium term haemodynamic outcome. However, early surgery does escalate the need for ICU care. This data suggests repair in asymptomatic patients be delayed until 3-6 months of age.

Valve-sparing aortic root replacement in adult patients previously operated for congenital heart defects: an initial experience.

OBJECTIVES: To review our experience with valve-sparing aortic root replacement (VSARR) in adult patients after the previous surgery for congenital heart disease. METHODS: From August 2008 to February 2014, 11 patients (mean age: 30.5 ± 7.7 years), previously operated for various congenital cardiac defects, mostly conotruncal lesions, underwent VSARR for progressive aortic root dilatation. Five patients had also developed moderate, and 1 patient severe aortic valve (AoV) insufficiency. All aortic root procedures were performed by the same operating surgeon in two institutions. The mean interval between the initial procedure and aortic root surgery was 25.7 ± 7.7 years. At the time of reoperation, the mean aortic root diameter was 53.4 ± 6.2 mm. The reimplantation technique (David procedure) was used in all patients. Seven patients underwent concomitant procedures. The median follow-up was 32 months (range 12-78 months). RESULTS: No mortality occurred in hospital or during the follow-up period. Ten patients remain in NYHA functional class I; 1 patient with a single-ventricle circulation is in NYHA functional class II. During the immediate postoperative period, 2 patients underwent drainage of a pericardial collection and 1 patient required renal replacement therapy with eventual full recovery of renal function. No other reoperations or reinterventions were needed during the follow-up period. The most recent echocardiogram in 10 patients have demonstrated no or trivial AoV insufficiency. One patient, however, with severe aortic insufficiency preoperatively, developed mild AoV regurgitation shortly after the operation, which has progressed to moderate regurgitation 1 year following the operation. CONCLUSIONS: Analysis of our experience in a small group of patients confirms that valve-sparing aortic root surgery can be safely performed in adult congenital patients presenting with progressive aortic root dilatation following their previous surgery. Although we have analysed patients with different original congenital cardiac lesions and the follow-up period is not long, we can conclude that the use of the reimplantation technique (David procedure) have resulted in overall satisfactory AoV function at follow-up. Longer follow-up with larger series will be needed, however, before firm conclusions can be drawn.

[Revision surgery after closure of isolated ventricular septal defects]. / Pakartotines operacijos po izoliuoto skilveliu pertvaros defekto korekcijos.

During the period from 1964 10 15 to 2002 06 01 428 patients underwent defect closure for isolated ventricular septal defect at Vilnius University Heart Surgery Clinic. Seventeen (4.2%) patients necessitated revision surgery in the postoperative period to repair haemodinamically significant postoperative complications. The interval between definitive defect correction and revision surgery ranged from 1 month to 24 years. Repeated procedures were contemplated because of large (Q(p)/Q(s) >1.5) interventricular residual shunts, aortic and pulmonary valve insufficiency, complete persistent heart block. Hospital mortality was 5.88%. No late postoperative deaths were observed.

Contemporary management of pulmonary and systemic circulations after the Norwood procedure.

Hypoplastic left heart syndrome remains one of the most challenging pathologies in pediatric cardiac surgery. The surgical techniques, and anesthetic and intensive care management, have evolved over the last decades, which has resulted in improved outcomes. A central component in the postoperative management of hypoplastic left heart syndrome patients is to achieve an optimal balance between the pulmonary and systemic circulations. This article discusses the contemporary postoperative management of pulmonary and systemic circulations in detail.

Sustainable knowledge transfer in pediatric cardiac surgery: a team approach to shared learning.

The study assesses the impact of a program using a model of knowledge transfer on the long-term development of a pediatric cardiac service in Lithuania. A team from the United Kingdom evaluated Lithuanian pediatric cardiac services and provided support in areas targeted for improvement. The total number of infant operations performed in Lithuania from 1990 to 2008, together with in-hospital mortality rates, was broken down into 3 time periods and analyzed to estimate the efficacy of the program: (1) 1990-1998, before the program; (2) 1999-2002, during the program; (3) 2003-2008, following the end of the program. Lithuanian results in children older than 1 year were comparable with other European centers. However, only 315 infant and neonatal cardiac procedures were performed between 1990 and 1998, and there was an in-hospital mortality of 34.2%. Between 1999 and 2002, the UK team performed 23 highly complex demonstration procedures (in-hospital mortality, 13%). During the same period the Lithuanian team performed 305 additional operations in neonates and infants, and in-hospital mortality decreased to 18.7%. From 2003 to 2008 results continued to improve-559 infant operations were performed, with in-hospital mortality of 11.3% (P < .0001). Knowledge transfer has led to substantial and sustainable long-term improvement in the results of infant cardiac surgery in Lithuania. Demonstrating techniques and care on a limited number of more complex cases is an efficient way of transferring knowledge and skills to the developing pediatric cardiac centers.