Arch Obstruction Research Articles

A tale of 2 conversions: Dichotomous decision in the spectrum of Shone syndrome is still a dilemma

A tale of 2 conversions: Dichotomous decision in the spectrum of Shone syndrome is still a dilemma

Surgical Experience With Nine Cases of Obstructed Right Aortic Arch.

Right-sided aortic arch obstruction is an extremely rare congenital anomaly. A variety of surgical approaches have been described. This study reviews our institutional experience over the last 30 years. Our surgical database at the University of Alabama at Birmingham and Children's Hospital of Alabama from 1992 to 2022 was reviewed to include all patients who underwent surgical repair for right-sided aortic arch obstruction. A total of nine patients underwent surgical repair for right-sided aortic arch obstruction. Surgical approach was via thoracotomy (n = 2, 22%), sternotomy (n = 5, 56%), or combined (n = 2, 22%). Primary extended end-to-end anastomosis was utilized for patients with discrete coarctation (n = 1, 11%), reverse subclavian flap for coarctation with associated distal arch hypoplasia (n = 2, 22%), GORE-TEX® tube graft for circumflex aorta (n = 1, 11%), and aortic arch advancement (n = 5, 56%) with or without patch augmentation for those with an interrupted or severely hypoplastic aortic arch. Reintervention was required in one patient (11%) for recoarctation. All patients were discharged in good condition. There was no hospital mortality and at 10.5 years (mean) follow-up there was one late death. Right aortic arch obstruction is a rare entity. Surgical approach should be tailored to the anatomy and associated intracardiac defects. Preoperative imaging with a CT angiogram is useful for operative planning. Sternotomy with single-stage primary repair is safe, effective, and our preferred surgical approach for patients with right aortic arch obstruction and associated intracardiac pathology.

Arterial Switch Operation in Children With Aortic Arch Obstruction: Impact of Size Discrepancy Between the Great Arteries

Arterial Switch Operation in Children With Aortic Arch Obstruction: Impact of Size Discrepancy Between the Great Arteries

Outcomes of Arterial Switch Operation With Aortic Arch Reconstruction

BackgroundThis study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction. MethodsA total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS. ResultsThe median age and body weight at repair were 9 days and 3.2 kg, respectively. Forty-nine patients (96.1%) underwent 1-stage repair. A total of 28 patients (54.9%) had TBA, and 8 patients (15.7%) had interrupted aortic arch. There were 5 early deaths (9.8%) and 2 late deaths during a median follow-up duration of 59 months. The transplant-free survival rate 10 years after repair was 82.6%. A total of 21 reinterventions were required in 10 patients. The significant PS–free survival rate 10 years after repair was 68.8%. In univariable analysis, a higher ratio of the diameter of the main pulmonary artery to the ascending aorta was associated with all-cause reintervention (P = .007) and right-sided reintervention (P = .002). A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS (P = .049). ConclusionsThe rates of overall mortality and reintervention after repair were not negligible. A higher degree of size discrepancy between the 2 great arteries was associated with all-cause or right-sided reintervention. A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS.

Outcomes of Surgical Repair for Truncus Arteriosus: A 30-Year Single-Center Experience.

We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.

Hybrid First-stage Palliation and Other Strategies to Achieve Biventricular Repair in High-Risk Neonates With Complex Heart Anomalies and Aortic Arch Obstruction.

Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include aortic arch repair and pulmonary artery band with or without cardiopulmonary bypass, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.

Risk Factors for Perioperative Brain Lesions in Infants With Congenital Heart Disease: A European Collaboration.

Infants with congenital heart disease are at risk of brain injury and impaired neurodevelopment. The aim was to investigate risk factors for perioperative brain lesions in infants with congenital heart disease. Infants with transposition of the great arteries, single ventricle physiology, and left ventricular outflow tract and/or aortic arch obstruction undergoing cardiac surgery <6 weeks after birth from 3 European cohorts (Utrecht, Zurich, and London) were combined. Brain lesions were scored on preoperative (transposition of the great arteries N=104; single ventricle physiology N=35; and left ventricular outflow tract and/or aortic arch obstruction N=41) and postoperative (transposition of the great arteries N=88; single ventricle physiology N=28; and left ventricular outflow tract and/or aortic arch obstruction N=30) magnetic resonance imaging for risk factor analysis of arterial ischemic stroke, cerebral sinus venous thrombosis, and white matter injury. Preoperatively, induced vaginal delivery (odds ratio [OR], 2.23 [95% CI, 1.06-4.70]) was associated with white matter injury and balloon atrial septostomy increased the risk of white matter injury (OR, 2.51 [95% CI, 1.23-5.20]) and arterial ischemic stroke (OR, 4.49 [95% CI, 1.20-21.49]). Postoperatively, younger postnatal age at surgery (OR, 1.18 [95% CI, 1.05-1.33]) and selective cerebral perfusion, particularly at ≤20 °C (OR, 13.46 [95% CI, 3.58-67.10]), were associated with new arterial ischemic stroke. Single ventricle physiology was associated with new white matter injury (OR, 2.88 [95% CI, 1.20-6.95]) and transposition of the great arteries with new cerebral sinus venous thrombosis (OR, 13.47 [95% CI, 2.28-95.66]). Delayed sternal closure (OR, 3.47 [95% CI, 1.08-13.06]) and lower intraoperative temperatures (OR, 1.22 [95% CI, 1.07-1.36]) also increased the risk of new cerebral sinus venous thrombosis. Delivery planning and surgery timing may be modifiable risk factors that allow personalized treatment to minimize the risk of perioperative brain injury in severe congenital heart disease. Further research is needed to optimize cerebral perfusion techniques for neonatal surgery and to confirm the relationship between cerebral sinus venous thrombosis and perioperative risk factors.

Intensive care follow-up of an infant who underwent Yasui procedure due to interrupted aortic arch and left ventricular outflow tract obstruction

Kesintili aortik ark (KAA) neonatal dönemde bulgu veren oldukça karmaşık ve yönetimi zor bir konjenital kalp hastalığıdır (KKH). Bu vakalarda, çıkan ve inen aorta arasında anatomik ve luminal kesinti vardır. KAA, geçmişte mortalitesi yüksek olan ancak günümüzde yaşam beklentisi giderek artan bir kardiyak anomalidir. KAA’nın tedavisi cerrahi olarak arkus tamiri yapılmasıdır. Sol ventrikül çıkım yolu (LVOT) darlığının eşlik etmesi daha komplike cerrahi teknikleri uygulanmasını gerektirir. Bu yazıda tip B KAA ve LVOT darlığı nedeniyle Yasui prosedürü uygulanan ve postoperatif 12. günden sonra yenidoğan yoğun bakım ünitesinde izlenen bir bebeğin yoğun bakım sürecinden yola çıkarak kardiyak hasta izlem ilkeleri tartışılmaktadır. Açık kalp cerrahisi yapılan bebek hastaların yönetimi konusundaki deneyimlerin kardiyak bakım ile ilgilenen yenidoğan hemşire ve doktor ekipleri için yararlı olması amaçlanmıştır.

Hybrid Approach for Correction of Recurrent Aortic Arch Obstruction After Repair of Interrupted Aortic Arch—A Case Report

Recurrent aortic arch obstruction (RAAO) following repair of interrupted aortic arch (IAA) occurs in up to 50% of patients. Treatment options are surgical correction or percutaneous catheter intervention. Balloon angioplasty has become more popular over the last 3 ​decades, but current studies suggest that it may be less effective than surgical treatment.1,2

Journey Toward Improved Long-Term Outcomes After Norwood-Sano Procedure: Focus on the Aortic Arch Reconstruction.

The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta. Artificial materials decrease the elastic properties, increase wall stiffness, and decrease the distensibility of the aorta; and as a result, RV function gradually deteriorates. This inelastic reconstructed aorta may be one of the reasons why long-term outcomes after the Fontan procedure are worse in hypoplastic left heart syndrome (HLHS) patients, in comparison to non-HLHS. Reconstruction of the neoaorta without any patch materials, or at least techniques that largely minimize the use of non-autologous materials, will offer a further refinement of our ability to optimize ventriculoarterial coupling and thereby long-term RV function.

Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience

We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures. We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years. We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04). Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations.

Isolated Dilation of Right Heart Chambers in Fetal Echocardiography: Correlation with Postnatal Findings in a Quarterly Hospital

Background: Despite technological advances in fetal echocardiography, the assessment of the aortic arch is challenging due to the particularities of fetal anatomy and physiology. Both isolated dilatation of right heart chambers, direct visualization and measurements of the aortic arch are related to left obstructive heart disease, such as Coarctation of the Aorta (CoAo). So far, there is still no consensus in the published literature about which echocardiographic finding is of greater value for prenatal diagnosis of these changes. Objectives: To determine possible correlation between right cardiac chambers dilatation and direct aortic arch measurements with the presence of postnatal CoAo. Methods: A retrospective study of fetal and postnatal echocardiograms was carried out at Hospital Israelita Albert Einstein (HIAE) between 2011 and 2017. Morphological and functional parameters of the fetal circulation and the transitional circulation period in the newborn were analyzed. Fetal echocardiogram findings were compared with postnatal echocardiograms (presence or absence of congenital heart disease). Results: Of 2,869 exams performed, 22 fetuses with right chamber dilatation were born at our institution; 20 (90%) had postnatal echocardiogram with normal findings, and only 2 newborns (10%) presented aortic arch obstruction, 1 newborn had critical obstruction and required neonatal intervention. Among all fetal echocardiographic parameters evaluated, only the direct measurement of the aortic isthmus showed correlation with postnatal changes. Conclusion: In our series, isolated dilation of right heart chambers on fetal echocardiography was not associated with CoAo, but the association of right heart chamber dilatation with aortic arch diameter reduction was, however, more studies with larger numbers of patients are still needed.

Methodology and immediate results of selective controlled cerebral and myocardial perfusion for aortic arch obstruction in children

Highlights. Congenital heart defects in combination with aortic arch hypoplasia or coarctation of aorta are quite common in cardiac surgeon’s practice. The protection of internal organs is an important point in the treatment of these defects. Several methods have been proposed from hypothermic arrest to selective perfusion of various vascular regions. Given the variety of techniques, the effectiveness and advantages of each one are still a topic of debates. The article reflects the experience of using cerebro-myocardial perfusion in children with surgical correction of the aortic arch pathology.Aim. To analyze the prospects of regional selective controlled cerebral and myocardial perfusion method in surgical correction of aortic arch obstruction in children.Methods. A retrospective single-center analysis of the treatment in 29 pediatric patients was carried out and immediate results of cerebral-myocardial perfusion used in children with surgical correction of aortic arch obstruction in the period from 2016 to 2020 are presented. Median age was 6 days (1 day – 15 years old). Median weight was 3.6 kg (1.8–47 kg). 15 patients were girls, 14 patients were boys. The inclusion criteria for all the patients were cerebro-myocardial perfusion for aortic arch reconstruction.Results.Cardiopulmonary bypass time median was 78 minutes (43–206 minutes). The average time of cerebro-myocardial perfusion was 22.4 + –6.4 min (12–35 min). The median duration of the surgery was 3.1 hours (2.25–5.5 hours). The median time for mechanical ventilation was 101 hours (6–744 hours), in the ICU – 8.5 days (1–31 days). The average time of hospital stay was 18.2 days + –6.7 days (7–31 days). Early postoperative mortality was 3.4%. Renal replacement therapy was required for 2 newborns (7%). Operational risks, according to the Aristotle Score, were significantly higher in newborns (χ2 = 3.9277, df = 1, p-value = 0.0475). There were no cardiac events associated with myocardial perfusion. The type of cardioplegia (blood or “Kustadiol”) did not significantly affect the pumping function of the heart, assessed by transthoracic echocardiography on day 1 after the surgery (χ2 = 0.27273, df = 1, p-value = 0.6015). Acute ischemic cerebrovascular accident occurred in 1 patient (3.5%).Conclusion. The technique of controlled selective cerebro-myocardial perfusion is reproducible without any additional resource costs. We can confidently say that the time of surgery and cardiopulmonary bypass is much shorter than in case of using deep hypothermic arrest or isolated selective cerebral perfusion, as soon as there is less time needed for cooling and re-warming the body. Also, the time of cardiac arrest was reduced or completely eliminated (with isolated reconstruction of the aortic arch). The technique is effective and safely reproducible in both newborns and older children. The results for mortality and complications are encouraging but must be further evaluated and compared.

Prevalence of Critical Congenital Heart Disease During Surgical Mission Trips to Low-Middle Income Countries. What to Expect

Background&#x0D; The incidence of congenital heart disease (CHD) is about 8 per 1000 live births. About 20% of those patients diagnosed with CHD will present early after birth with Critical Congenital Heart Disease (CCHD) necessitating a catheter or surgical intervention during the neonatal period. This is based on Western literature and Western countries. Little is known about the actual prevalence of CCHD at the time of clinical assessment and treatment during surgical missions in low and middle income countries (LMICs).&#x0D; Methods&#x0D; This is a retrospective study and it compares prevalence of CCHD at time of presentation for surgery in LMICs&#x0D; to published data for similar cohorts in the USA. We discuss potential causes for epidemiological discrepancies&#x0D; Proportions were compared using OR and 95% CI. Significance was set at p&lt;0.01. We chose to classify our patients considering the number of ventricles (1or 2) combined with the existence of Aortic Arch obstruction (Yes/No) resulting in a CCHD classification with 4 categories&#x0D; Results&#x0D; Between Feb 2008 - June 2019 we operated 6115 patients in 27 countries. Of those, 243 Neonates were presented to our cardiologists&#x0D; after excluding simple PDA ligations in Preterm newborns and 239 neonates were surgically treated for their CCHD&#x0D; (4% of the total). overall surgical mortality among the 243 neonates operated in LMIC for their CCHD was 19.7% (n=48). Main discrepancies were in Classes 1 represented by 63% of our treated CCHD (p&lt;.0001) while Class 4 neonates represented only 4.2% of our total (p&lt;.0001). When compared to Western prevalence, Transposition of the Great Arteries was over-represented while some cyanotic (ToF) and non-cyanotic diagnosis (Critical Aortic Stenosis) were under-represented.&#x0D; Conclusions&#x0D; Patients diagnosed and treated for CCHD in LMICs during humanitarian missions represent but a very small fraction of the total number of patients born with CCHD in those countries. &#x0D; Discrepancies in specific diagnoses and prevalence between LMIC and their high-income counterparts are likely multifactorial, and management should be adapted to the conditions of each country and health system.&#x0D; Proper prenatal diagnosis, specialized critical neonatal transportation systems, countrywide availability of Prostaglandins and at least one highly functioning heart center are some of the most basic needs required in order to revert the current natural history of CCHD in countries with limited resources settings.

Single-Stage Surgical Management of Atrioventricular Septal Defects with Coarctation of the Aorta.

Surgical options for coarctation of aorta (CoA) with atrioventricular septal defect (AVSD) include single-stage repair vs. staged approach with neonatal CoA repair and delayed AVSD repair. The durability of left atrioventricular valve (LAVV) function after neonatal repair is questioned, and the optimal approach remains controversial. Eighteen CoA-AVSD patients who underwent single-stage repair 2005–2015 by a single surgeon were retrospectively analyzed. Fifteen patients had complete and three had partial AVSD. Birth weight was 3.19 kg (2.17–4.08). Age at surgery was 16 days (6–127). One- and ten-year survival were 80% and 69%. Freedom from reintervention was 60% and 40% at one and ten-year respectively. Reinterventions included relief of left ventricular outflow tract obstruction (LVOTO) (n = 4), repair of cleft LAVV (n = 3), and LAVV and aortic valve replacement (n = 1). Freedom from LAVV reintervention was 85.6% and 66% at 1 and 10 years respectively. There were four deaths: two post-operative and two following hospital discharge. Mortality was due to sepsis in three patients, and heart failure related to LVOTO and LAVV insufficiency in one. At 68-month (0.6–144) follow-up the majority had mild or less LAVV regurgitation, and all had normal LV dimension and systolic function. There was no recurrent arch obstruction. Single-stage surgical repair of CoA-AVSD is feasible and reasonable. Survival and freedom from reintervention in our cohort approximate those outcomes of two-stage repair with durable left AV valve function and no recurrent arch obstruction. These patients are frequently syndromic and demonstrate mortality risk from non-cardiac causes. Consideration of a single-staged approach is warranted for appropriate patients with CoA-AVSD.

A single-centre, retrospective study of mid-term outcomes of aortic arch repair using a standardized resection and patch augmentation technique.

OBJECTIVESThe aim of this study was to evaluate the mid-term outcomes after the repair of aortic arch using a standard patch augmentation technique.METHODSThe study included all patients who underwent repair of a hypoplastic/interrupted aortic arch (IAA) in a single institute from June 2012 to December 2019 by a standardized patch augmentation (irrespective of concomitant intra-cardiac lesions). End points evaluated were reintervention for arch obstruction and persistent/new-onset hypertension.RESULTSThe study included 149 patients [hypoplastic aortic arch, n = 92 (62%), IAA, n = 9 (6%), Norwood procedure, n = 48 (32%)]. The patch material used for augmentation of the aortic arch included pulmonary homograft (n = 120, 81%), homograft pericardium (n = 18, 12%), CardioCel® (n = 9, 6%) and glutaraldehyde-treated autologous pericardium (n = 2, 1%). The median age and weight at surgery were 7 days [interquartile range (IQR) 5–17 days] and 3.5 kg (IQR 3–3.9 kg), respectively. The median follow-up was 3.27 years (IQR 1.28, 5.08), range (0.02, 8.76). Freedom from reintervention at 1, 3 and 5 years was 95% [95% confidence interval (CI) = 89%, 98%], 93% (95% CI = 86%, 96%) and 93% (95% CI = 86%, 96%) respectively. One patient (0.6%) had persistent hypertension 8 years after correction for interrupted arch with truncus arteriosus.CONCLUSIONSRepair of hypoplastic/IAA by transection and excision of all ductal tissue and standardized patch augmentation provide good mid-term durability. The freedom from reintervention at 5 years is >90%. The incidence of persistent systemic hypertension following arch reconstruction is low. The technique is reproducible and applicable irrespective of underlying arch anatomy.

Surgical Repair of Supravalvar Aortic Stenosis in Association With Transverse and Proximal Descending Aortic Abnormalities.

Supravalvar aortic stenosis (SVAS) may be an isolated defect of the proximal ascending aorta. However, more severe cases have extension of the arteriopathy into the transverse and proximal descending aorta. The purpose of this study was to review our experience with SVAS with and without aortic arch arteriopathy. This was a retrospective review of 58 patients who underwent surgical repair of SVAS. The median age at repair was 18 months. A total of 37 patients had Williams syndrome. A total of 31 (53%) patients had associated peripheral pulmonary artery stenosis and 23 (39%) had coronary artery ostial stenosis (CAOS). A total of 37 of 58 (64%) patients had surgical repair of SVAS without the need for arch intervention while 21 (36%) patients had repair of the distal aortic arch. There were 3 (5.2%) operative deaths, 2 of whom had aortic arch involvement and one without arch involvement. There were 2 deaths after discharge from the hospital. Patients who needed arch surgery were more likely to have severe arch gradients compared to those without arch involvement (71% vs 30%, P < .05), were more likely to undergo concomitant procedures for peripheral pulmonary artery stenosis or CAOS (90% vs 62%, P < .05), and to have Williams syndrome (86% vs 51%, P < .05). More than one-third of patients who had SVAS repair at our institution had procedures directed at the transverse or proximal descending aorta. Patients with arch involvement had more severe arch obstruction, required more concomitant procedures, and were more likely to have Williams syndrome.

Staged left ventricular recruitment facilitated by Sano conduit upsizing

Staged left ventricular recruitment facilitated by Sano conduit upsizing

The association of bicuspid aortic valve on long-term outcome following one-stage repair of aortic arch obstruction associated with ventricular septal defect.

The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect. Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients' median age was 7 ± 29 days (range, 2-150); median weight was 3.3 ± 0.7 kg (range, 1.5-6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA). The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up. Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.

Antegrade cerebral perfusion during arterial switch procedure in patients with aortic arch obstruction

<h3>ЦЕЛЬ ИССЛЕДОВАНИЯ</h3> Представить опыт одномоментной коррекции транспозиции магистральных сосудов (ТМС) и обструкции дуги аорты с антеградной перфузией головного мозга. <h3>МАТЕРИАЛ И МЕТОДЫ</h3> За период с 2014 г. по декабрь 2021 г. оперировано 10 больных с ТМС в сочетании с обструкцией дуги аорты. Из них 8 больных были с ТМС и межжелудочковым дефектом, 2 пациента — с двойным отхождением магистральных сосудов от правого желудочка и подлегочным дефектом межжелудочковой перегородки (аномалия Тауссиг—Бинга). Аномалии дуги аорты включали коарктацию аорты с гипоплазией дуги аорты (80%) и прерванную дугу аорты (20%). <h3>РЕЗУЛЬТАТЫ</h3> В ближайшем послеоперационном периоде умер 1 пациент, отдаленных летальных исходов не было. При использовании антеградной перфузии головного мозга мы не получили неврологических осложнений, а умеренная гипотермия позволила избежать капиллярной утечки и развития синдрома полиорганной недостаточности. <h3>ВЫВОД</h3> У больных с комбинацией ТМС и обструкции дуги аорты показана одноэтапная коррекция в период новорожденности. Сохранение перфузии головного мозга позволяет снизить риск когнитивного и психомоторного дефицита, а умеренная гипотермия — избежать синдрома системного воспалительного ответа, капиллярной утечки и синдрома полиорганной недостаточности.