Effectiveness of Repair of Aortic Coarctation in Neonates: A Long-Term experience

To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in neonates. This is a retrospective clinical review of neonates with CoAo, who underwent surgery between 1995 and 2019. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by means of relative risk ratio and Cox and logistic multivariate analysis. 218 consecutive neonates (M/F: 129/89, median age 11 days, IQR 7–17 days) were included; 202 (92.7%) had a left thoracotomy; 178 underwent extended end-to-end anastomosis (EEEA, 81.6%). Hypoplastic aortic arch (HAA) was present in 102 patients (46.8%); complex cardiac anomalies in 85 (39%). Significant postoperative complications occurred in 20 (9.2%). Thirty-day mortality was 2.3% (most in complex types). At a median follow-up of 10.4 years (IQR 5.6–15.0 years; FU completeness 95.9%), there were 8 late deaths (3.7%), all associated to complex CoAo. Among 196 survivors, 177 (93.2%) were in NYHA class I; re-interventions on aortic arch occurred in 9.2% (2.0% were surgical). Freedom from mortality and re-intervention on aorta at 10 years were 94.3% and 96.7%, respectively. Surgical repair of CoAo in newborns without CPB in our series was safe and low-risk, with excellent early and late outcomes.

Off-pump hypoplastic aortic arch ± aortic coarctation repair via median sternotomy using a temporary shunt

An off-pump technique was developed to surgically manage the problem of aortic arch hypoplasia frequently associated with coarctation of the aorta and borderline size left-sided structures. The technique was successfully utilized in 5 patients adequately repairing the arch without any side effects.

Bentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection

Neoaortic root dilation is a common phenomenon after the Norwood procedure, but the real incidence and its natural history are unclear. Regular surveillance in these patients after the operation is necessary. Herein, we present an 11-year-old boy born with tricuspid atresia, a discordant ventriculo-arterial connection and a hypoplastic aortic arch, who was palliated initially with a hybrid stage I procedure involving a reversed Blalock–Taussig shunt, followed by comprehensive stage II and then, an extra-cardiac fenestrated Fontan operation. The patient developed an aortic root aneurysm and severe aortic regurgitation. He was electively taken into the operating room, where cardiopulmonary bypass was established through a peripheral cannulation of the femoral vessels due to the high risk nature of the reoperation. A mechanical Bentall procedure was performed without residual lesions and the native ascending aorta was anastomosed as a single coronary button to the anterior wall of the graft.

A rare complication of myocardial ischaemia following single-stage repair in a case of Berry syndrome

Berry syndrome is a rare congenital cardiac lesion consisting of a distal aortopulmonary window, the aortic origin of the right pulmonary artery (PA), intact ventricular septum and an interrupted or hypoplastic aortic arch. Different repair techniques have been described in the literature. We report a case of Berry syndrome, in whom myocardial ischaemia developed following direct implantation of the right PA to the main PA, which was resolved using an interposition tube graft.

The Features of Pregnancy, Early Neonatal Period and Tactics of Surgical Treatment in Newborn with Hypoplastic Aortic Arch (First-Hand Experience)

Aortic pathology occurs in 10% of all newborn with congenital heart diseases (CHD). According to the data of various authors, in 50–80% of cases coarctation of aorta is accompanied by hypoplastic aortic arch. The newborn with such diagnosis represent a unique cohort of children who remain in severe condition and require immediate surgical intervention. Despite the significant development of diagnostic opportunities of radiographic methods, aortic arch patho­ logy remains one of the most difficult diagnoses in prenatal diagnostics. The objective of the study is to provide rationale for perinatal prognosis based on the analysis of pregnancy course, condition of the fetus and newborn in order to provide timely diagnosis and perform cardiosurgical interventions in patients with aortic arch pathology. Materials and methods. In the period from 2011 to 2019 at the State Institution “Institute of Pediatrics, Obstetrics and Gynecology of NAMS of Ukraine” and State Institution “National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine” 121 pregnant women with fetal aortic arch hypoplasia were observed, followed and gave birth according to the algorithm developed by our team. The most optimal terms for fetus examination were weeks 20–22 and 30–32 of pregnancy. Patients with hypoplastic aortic arch as part of hypoplastic left heart syndrome were excluded from the analysis of tactics and results of surgical treatment since this category of patients requires separated study. Among 53 patients with hypoplastic aortic arch (36 patients with isolated hypoplastic aortic arch, and 17 patients with that combined with other CHD), 43 patients required surgical treatment after additional in-patient examination. Results and discussion. Analysis of the pregnancy course in women with fetal aortic arch hypoplasia showed that the number of multifetal pregnancies (including those having occurred after the use of extracorporeal technologies) was higher in pregnant women of the main group. Also, the percentage of threat of termination of pregnancy in the early term was high threatened miscarriage in the early stages of pregnancy was higher, and, vice versa, the threat of premature birth was lower. It is worth noting that 7 (5.7%) children had the appearance of “completely healthy child” in the first day of life, which confirms the importance of prenatal echocardiography in the timely diagnosis of critical CHD. To analyze the effectiveness of prenatal diagnosis and timely referral of patients to a specialized cardiac surgery center, it was found that the number of complications at the preoperative stage was statistically significantly higher in the group of patients without prenatal diagnosis. Conclusion. In case of diagnosed or suspected fetal aortic hypoplasia (isolated or combined with other abnormalities) a multidisciplinary case management team should be involved to determine further perinatal tactics and timing of cardiac surgery which will significantly reduce pre- and postoperative complications.

P254 Congenitally corrected transposition of the great arteries with an ebstenoid tricuspid valve, aortic atresia, hypoplastic aortic arch and a dysplastic pulmonary valve

Funding Acknowledgements No financial support received Introduction Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation accounting for around 0.5% of congenital heart disease. Common associations are ventricular septal defects (VSD), pulmonary stenosis and heart block. CCTGA associated with aortic atresia is very rarely described in the literature. Description We present a case of a term neonate weighing 2.5kg. Fetal echo had initially made the diagnosis at 21/40. The baby was born in good condition and the postnatal echo confirmed the diagnosis of CCTGA. There were 2 secundum atrial septal defects, the sub-systemic tricuspid valve was ebstenoid with remarkably trivial regurgitation, there were multiple muscular ventricular septal defects with predominantly left to right flow, although flow appeared bi-directional in some views. The atretic aortic valve was anterior and to the left of the dysplastic, bicuspid pulmonary valve. The pulmonary valve leaflet motion was restricted with peak velocity of 2.4m/s and mild incompetence. The hypoplastic aortic arch filled retrogradely via the arterial duct. There was discrete coarctation with peak velocity of 2.2m/s down the descending aorta. The persistent left SVC drained to the coronary sinus. Following several discussions there was agreement that the most appropriate management for him would be to receive palliative care. He was discharged home at a week of age and continued to have intensive support from his local hospice and the cardiac team, until he passed away peacefully at home at 16 days of age. Discussion The multidisciplinary team decided surgery was extremely high risk due to the anatomy described above particularly in the context of his dysplastic, bicuspid pulmonary valve, which would have provided systemic outflow if intervention had been undertaken. Figure 1: 4 chamber view showing reverse offsetting of the atrioventricular valves, arch view showing a hypoplastic aortic arch with restrograde flow from the arterial duct, short axis view showing the semilunar valves and their relative positions, long axis view showing aortic atresia with retrograde flow into the ascending aorta Abstract P254 Figure 1

Pseudoaneurysm of the aortic arch early after coarctation repair in the neonatal period

Aortic arch aneurysm or pseudoaneurysm is a rare complication early after coarctation repair in the neonatal period. We report the case of a newborn with a ventricular septal defect and aortic coarctation with a hypoplastic aortic arch that developed a large aortic arch pseudoaneurysm following a radically extended end-to-end coarctation repair. Successful surgical correction of the pseudoaneurysm was performed.

P2789A new technique to restore shape and pattern of flow in hypoplastic aortic arch

Background There is no agreement about the best method for correcting hypoplastic aortic arch in infancy. Residual abnormalities in shape can cause late complications. Patients and methods We have developed and applied a new method of direct anastomosis of the descending to the ascending aorta to allow for restoration of shape, function and growth. 12 infants undergoing this technique at the age of 61±3months (range 6–28 months) underwent repeat CT 4–15 months after operation. Pre- and post-operative arch width, height and height to width ratio were recorded for all cases. Computerized shape analysis using an in-house tool (developed in MATLAB) was used for processing the 3D segmented shapes of the aortic arch, and assessing changes in the cross-sectional area at different levels. Computational Fluid Dynamics (CFD) was used to simulate aortic flow propagation and pattern after surgery, using patient specific geometry and flow boundary conditions. Results The pre- and post-operative cross-sectional area along the centerline of the aorta and arch was almost normalized, Figure 1. The pattern of flow during the cardiac cycle with a retrograde helix in the aortic arch, similar to normal, was observed in all cases with peak velocities of 1.1±0.2m/s. Aortic Shape and flow after surgery Conclusion We here describe a new technique, which at least in the short term appears to normalize the size, shape and pattern of flow in the arch. Long term studies are required to establish the value of this technique.