Aortic Arch Obstruction Research Articles

A case of early diagnosis and surgical correction of obstructive aortic arch anomaly

A case of early diagnosis and surgical correction of obstructive aortic arch anomaly

Long-term Outcome After Repair of Transposition of the Great Arteries With Aortic Arch Obstruction

BackgroundThis study compares the long-term outcomes of patients after repair of transposition of the great arteries (TGA) with and without aortic arch obstruction (AAO). MethodsThis is a single-institution, retrospective study between October 2004 and February 2023. Patients who underwent arterial switch operation and aortic arch repair (ASO-AAR group) with patch augmentation were compared with those without AAO (ASO group). The primary end point was survival; freedom from reintervention was a secondary end point. ResultsWe identified 176 patients, 31 in the ASO-AAR group and 145 in the ASO group. The median follow-up period was 10.3 years. There were no differences between the ASO-AAR group and the ASO group in early deaths (3.2% vs 0.7%) and late deaths (3.2% vs 2.8%), or 15-year survival rates (92.6% vs 96.2%). Surgical and catheter-based reinterventions were higher in the ASO-AAR group, involving the pulmonary arteries (41.9% vs 4.8%, P < .001), aortic arch (16.1% vs 0.7%, P < .001), and residual ventricular septal defects (11.4% vs 0%, P = .05). The ASO-AAR group showed a higher prevalence of double-outlet right ventricle TGA-type (61.3% vs 4.1%, P < .001) and a lower aortopulmonary index (0.67 vs 1.01, P < .001). ConclusionsPatients undergoing surgical repair of TGA and AAO achieved excellent survival rates, comparable to patients with simple transposition. A higher rate of surgical and catheter-based reinterventions was observed in patients with arch obstruction and/or a low aortopulmonary index. AAR with patch augmentation proved to be an effective surgical technique with a low incidence of aortic reinterventions.

Outcomes of Surgical Interrupted Aortic Arch Repair in 181 Patients With Biventricular Circulation

BackgroundThis study compared the outcomes of primary and staged surgery for interrupted aortic arch (IAA) with biventricular circulation. MethodsA search of our surgical database identified 181 patients with biventricular anatomy who underwent surgical repair of IAA between 1982 and 2022. The patients were divided according to whether they underwent surgery in 1982 to 2001 or 2002 to 2022 and were categorized according to their anomalies into a simple group and a complex group. Repair was 1-stage in 130 patients (71.8%) and 2-stage in 51 (28.2%). ResultsThere were 6 early deaths (3.3%) and 9 (5.0%) late deaths. The 20-year actuarial survival rate was 91.7%. The 20-year survival rate was 94.7% in the simple group and 82.9% in the complex group (P = .007); the respective rates were 91.7% and 96.8% in 1982 to 2001 (P = .63) and 68.4% and 90.1% in 2002 to 2022 (P = .012). Multivariate analysis identified a complex anomaly and surgery performed in 1982 to 2001 to be risk factors for mortality. Aortic arch reintervention was required in 26 patients (balloon dilation, n = 15; surgical, n = 11). The 15-year freedom from reintervention rate improved from 67.8% in 1982 to 2001 to 89.8% in 2002 to 2022 (P = .041). Multivariate analysis identified type of surgical approach and emergent surgery to be risk factors for reintervention on the aortic arch. ConclusionsThe results of IAA surgery have improved in recent years, especially for complex cases. Development of surgical techniques and individualized treatment strategies could reduce postoperative mortality and morbidity. However, follow-up for recurrence of aortic arch obstruction is necessary.

Tactics of Surgical Repair of Single Ventricle with Excessive Pulmonary Blood Flow and Obstruction of the Distal Arch of the Aorta in Newborns

Pulmonary artery stenosis with distal aortic arch reconstruction and coarctation of the aorta in newborns is an effective palliative procedure for single ventricle and high pulmonary hypertension on the way to total cavo-pulmonary anastomosis. The aim. To present a case of complex correction of a single ventricle of the heart with tricuspid valve atresia and high pulmonary hypertension, obstruction of the distal aortic arch and coarctation of the aorta, and a final effective Fontan procedure with a good long-term outcome. Case report. On December 15, 2011, a 9-day-old newborn patient M. was admitted for treatment at the Department of Cardiovascular Surgery of Odesa Regional Children’s Clinical Hospital with a diagnosis of: a single ventricle of the heart, transposition of the great arteries, tricuspid atresia, distal arch hypoplasia, coarctation of the aorta, patent ductus arteriosus, high pulmonary hypertension. The first stage of surgical treatment was carried out on December 19, 2011: main pulmonary artery banding, reconstruction of the distal arch of the aorta through modified Amato technique, closure of the patent ductus arteriosus, resection of the coarctation of the aorta and extended end-to-end aortoplasty. At the age of 1 year (December 27, 2012), the second stage was performed: bidirectional Glenn procedure with plastic surgery of bifurcation and right pulmonary artery branch. The third stage was performed at the age of 3 years 11 months (November 19, 2015): Fontan procedure with extracardiac conduit. Conclusions. Early elimination of pulmonary hypertension by pulmonary artery banding ensures the preservation of the pulmonary vascular bed with low resistance, which is a crucial condition for the effective final hemodynamic correction of the single ventricle of the heart – the Fontan procedure. In case of hypoplasia of the distal aortic arch with coarctation in newborns, one of the alternative approaches is the use of modified Amato technique avoiding artificial circulation.

Percutaneous Balloon-Expandable Stent Implantation to Treat Transverse Aortic Arch Obstruction: Medium- to Long-Term Outcomes of a Retrospective Multicenter Study.

Transverse aortic arch obstruction is a challenging lesion for which stent implantation provides a potentially important alternate therapy. The objectives were to evaluate the technical, procedural, and medium-to-long-term clinical outcomes of percutaneous stent implantation of transverse aortic arch obstruction. This is a retrospective, multicenter study of transverse aortic arch stent implantation. Univariable and multivariable analyses were performed. Index catheterization included 187 stent implants in 146 patients. The median age is 14.3 years (interquartile range, 9.3-19), weight is 53 kg (30-69), and follow-up is 53 months (12-120). The most common stent design was open cell (n=90, 48%). Stents overlapped 142 arch vessels (37 carotid arteries) in 118 (81%) cases. Technical and procedural success rates were 100% and 88%, respectively. Lower weight (P=0.018), body surface area (P=0.013), and minimum-to-descending aortic diameter ratio (P<0.001) were associated with higher baseline aortic gradient. The residual gradient was inversely associated with implant and final dilation diameters (P<0.001). The combined incidence of aortic injury and stent-related complications was 14%. There were no reports of abnormal brain scans or stroke. Blood pressure cuff gradient, echocardiographic arch velocity, and hypertension rates improved within 1-year follow-up with increased antihypertensive medication use. Reintervention was reported in 60 (41%) patients at a median of 84 (22-148) months to first reintervention. On multivariable logistic regression, residual aortic gradient >10 mm Hg was associated with increased odds of reintervention at all time points when controlling for each final dilation diameter, weight, and minimum-to-descending aortic diameter ratio. Transverse aortic arch stent implantation has high rates of technical, procedural, and medium-to-long-term clinical success. Aortic gradient >10 mm Hg is associated with increased odds of reintervention at 1-year and most recent follow-ups. Open cell stent design was frequently used for its advantages in conformability, perfusion of arch vessels, low fracture rate, and the ability to perform effective angioplasty of side cells.

The anatomic repair of recurrent aortic arch obstruction in children and adolescents

ObjectiveSurgery for recurrent aortic arch obstruction is highly challenging and publications are rare. The aim of this retrospective, single-center study was to evaluate mortality, complications, and reintervention rate after an anatomic repair. MethodsBetween 1999 and 2022, in total 946 operations on the aortic arch were performed at the Children's Heart Center Linz. In 39 cases, the indication was a recurrent or residual aortic arch obstruction or coarctation in a patient aged 18 years or younger. This is our study cohort. The aorta was reconstructed by a direct anastomosis/autograft in 20 patients, patch in 17 patients, and interposition graft in 2 adolescents. In 32 procedures, cardiopulmonary bypass with whole body perfusion was employed, in 4, antegrade cerebral perfusion was employed, in 2, a left heart bypass was employed, and in 1 no cardiopulmonary bypass was used. ResultsMedian (Q1, Q3) age at operation was 253 days (100, 2198 days), weight 7.5 kg (4.5, 17.8 kg). Median cardiopulmonary bypass time was 177 minutes (115, 219 minutes), crossclamp time 73 minutes (49, 102 minutes). Three infants died during the hospital stay: 1 with Williams syndrome, 1 with hypoplastic left heart syndrome, and 1 with heterotaxia. There was no death due to an arch complication. The main complications were 1 neurologic injury after postoperative resuscitation (Williams syndrome) and 1 permanent recurrent laryngeal nerve paralysis. During the follow-up period of median 8.1 years (2.6, 12 years) 1 re-reintervention on the aortic arch was necessary. ConclusionsSophisticated reoperations on the aortic arch could be performed safely. In children, the growth potential of all segments of the aorta could be sustainably preserved by avoiding interposition or extra-anatomic bypass grafts.

Effects of hemodynamic alterations and oxygen saturation on cerebral perfusion in congenital heart disease

BackgroundPatients with severe congenital heart disease (CHD) are at risk for neurodevelopmental impairment. An abnormal cerebral blood supply caused by the altered cardiac physiology may limit optimal brain development. The aim of this study was to evaluate the effect of a systemic-to-pulmonary shunt, aortic arch obstruction and arterial oxygen saturation on cerebral perfusion in patients with severe CHD.MethodsPatients with severe CHD requiring cardiac surgery within the first six weeks of life, who underwent pre- and/or postoperative brain magnetic resonance imaging (MRI), and healthy controls with one postnatal scan were included. Cerebral perfusion in deep and cortical gray matter was assessed by pseudocontinuous arterial spin labeling MRI.ResultsWe included 59 CHD and 23 healthy control scans. The presence of a systemic-to-pulmonary shunt was associated with decreased perfusion in cortical (p = 0.003), but not in deep gray matter (p = 0.031). No evidence for an effect of aortic arch obstruction and arterial oxygen saturation on cerebral perfusion was found. After adjusting for hemodynamic and oxygen saturation parameters, deep (p = 0.018) and cortical (p = 0.012) gray matter perfusion was increased in patients with CHD compared to controls.ConclusionWe detected regional differences in compensation to the cerebral steal effect in patients with severe CHD.ImpactPatients with severe congenital heart disease (CHD) have altered postnatal brain hemodynamics.A systemic-to-pulmonary shunt was associated with decreased perfusion in cortical gray matter but preserved perfusion in deep gray matter, pointing towards regional differences in compensation to the cerebral steal effect.No effects of aortic arch obstruction and arterial oxygenation on cerebral perfusion were seen.Cerebral perfusion was increased in patients with CHD compared to healthy controls after adjusting for hemodynamic alterations and oxygen saturation.To improve neuroprotection and neurodevelopmental outcomes, it is important to increase our understanding of the factors influencing cerebral perfusion in neonates with severe CHD.

Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.

Foramen ovale flap aneurysm in fetuses associated with and without heart defects: Prenatal diagnosis, imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes.

Isolated redundant foramen ovale flap aneurysm (RFOA) in the absence of restrictive foramen ovale is believed to be a cause for pseudocoarctation of aorta since the impediment of blood flow to the left heart can be severe, resembling the picture of left ventricular hypoplasia with retrograde aortic flow. The primary objective of the study is to find whether RFOA is always a benign lesion. The main focus of the study is to share my experience in particular on fetuses having redundant foramen ovale flap aneurysm developing into coarctation of aorta and to study the associated factors. Retrospective study (January 2020 to June 2023). All fetuses with RFOA associated with and without congenital heart defects were included. Fetuses with restrictive foramen ovale and RFOA with single ventricle hearts were excluded. The imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes with at least 3 months follow-up were presented. During the study period, a total of 1499 fetal echocardiography were performed. Twenty-two fetuses with RFOA were included. Fourteen fetuses had isolated RFOA and eight had associated abnormalities [extracardiac (n=5); intracardiac (n=2), Both (n=1)]. Genetic evaluation were performed only in fetuses with associated defects were normal. Postnatally all isolated RFOA fetuses had no aortic arch obstruction. Fetuses with associated aberrant right subclavian artery, isolated left superior vena cava, absent ductus venosus and ventricular septal defects developed aortic arch obstruction after birth. RFOA causes smallish left ventricle in fetuses with tetralogy of Fallot which recovered to normal size postnatally. Isolated RFOA can be benign, however, if it is associated with cardiac or extracardiac anomalies predominantly resulted in aortic arch obstruction. Though it is a cause for pseudocoarctation of aorta, through postnatal reassessment of aortic arch is mandatory. Careful search for intracardiac and systemic venous anomalies is recommended. It created confusion regarding adequacy of left ventricle when associated with congenital heart defects.

Эффективность неонатального скрининга на критические врожденные пороки сердца путем двузонного измерения систолического артериального давления и сатурации

Aim. To evaluate screening methods for critical congenital heart defects (CHD), in particular obstructive aortic arch defects (OАADs), using dual-zone systolic blood pressure (SBP) and oxygen saturation measurements in healthy newborns. Design. A prospective observational study. Material and methods. The study included 12 098 healthy newborns aged 48 (36–50) hours of life, who were screened for critical CHDs using dual-zone measurements of SBP and saturation, as well as using physical methods. Screening for critical CHDs was considered normal if the saturation difference was 2% or less, the saturation was more than 95%, the difference in SBP was 9 mm Hg and less. If the screening was abnormal, echocardiography (ECHO-CG) was performed. The end point of the study was to determine the sensitivity and specificity of screening for OPAADs in healthy newborns using dual-zone measurement of SBP and saturation. Results. 12 037 children had normal screening results. In these children, the difference in pre- and postductal SBP was 0 (standard deviation (σ) = 4.26 mm Hg, and the difference (Me) in saturation was 0 (σ = 0.93%). Dual-zone SBP screening was abnormal in 34 newborns. The difference in their SBP was 11 (10–34) mm Hg. In 16 of these children ECHO-CG did not reveal CHD, and in 4 — critical OAADs — coarctation of aorta was diagnosed. A decrease in peripheral pulsation in the femoral arteries was noted only in children with critical OAADs. Pulse oximetry screening was normal in these children with critical OAADs. Dual-zone saturation screening was abnormal in 16 children. The difference in saturation between them was 3 (3–5)%. In 13 of these children, ECHO-CG did not reveal CHDs, and in 1 child critical CHD was diagnosed — transposition of the great arteries. Also, this child (and another who was healthy) had a decrease in saturation of less than 95%. In 11 newborns, screening was abnormal only due to saturation of 95% or less, but ECHO-CG did not reveal CHDs. The sensitivity and specificity of screening for OAADs using dual-zone SBP measurement was 100 and 80%, and using dual-zone pulse oximetry was 0 and 73.6%, respectively. Conclusions. In newborns with OAADs, a difference in SBP of 10 mmHg is recorded after 36 hours of their life. Dual-zone measurement of SBP is the most highly sensitive and specific method for detecting OAADs in comparison with dual-zone pulse oximetry. Dual-zone measurement of SBP and determination of peripheral pulsation are recommended for inclusion in the neonatal screening protocol for critical CHDs. Keywords: congenital heart disease, neonatal screening for congenital heart defects, aortic coarctation, aortic arch interruption, blood pressure.

Association of Digoxin Use With Transplant-Free Interstage Survival in Infants Palliated With a Stage 1 Hybrid Procedure.

Background Digoxin prescription in patients with single-ventricle physiology after stage 1 palliation is associated with reduced interstage death. Prior literature has primarily included patients having undergone the Norwood procedure. We sought to determine if digoxin prescription at discharge in infants following hybrid stage 1 palliation was associated with improved transplant-free interstage survival. Methods and Results A retrospective multicenter cohort analysis was conducted using data from the National Pediatric Cardiology Quality Improvement Collaborative registry data from 2008 to 2021. Infants with functional single ventricles and aortic arch obstruction discharged home after the hybrid stage 1 palliation hospitalization were included. Patients were excluded if they had supraventricular tachycardia or conversion to Norwood operation. The primary outcome was transplant-free survival. Multivariable logistic regression analysis including a propensity score for digoxin use identified associations between digoxin use and interstage death or transplant. Of 259 included infants from 45 sites, 158 (61%) had hypoplastic left heart syndrome. Forty-nine percent had a gestational age ≤38 weeks, 18% had a birth weight <2.5 kg, and 58% had a preoperative risk factor. Of the 259 subjects, 129 (50%) were discharged on digoxin. Interstage death or transplant occurred in 30 (23%) patients in the no-digoxin group compared with 18 (14%) in the digoxin group (P=0.06). With multivariate analysis, discharge digoxin prescription was associated with a lower risk of interstage death or transplant (adjusted odds ratio, 0.48 [95% CI, 0.24-0.93]; P=0.03). Conclusions In infants with single-ventricle physiology who underwent hybrid stage 1 palliation, digoxin prescription at hospital discharge was associated with improved interstage transplant-free survival.

Augmented Reality Visualization of 3D Rotational Angiography in Congenital Heart Disease: A Comparative Study to Standard Computer Visualization.

Augmented reality (AR) visualization of 3D rotational angiography (3DRA) provides 3D representations of cardiac structures with full visualization of the procedural environment. The purpose of this study was to evaluate the feasibility of converting 3DRAs of congenital heart disease patients to AR models, highlight the workflow for 3DRA optimization for AR visualization, and assess physicians' perceptions of their use. This single-center study prospectively evaluated 30 retrospectively-acquired 3DRAs that were converted to AR, compared to Computer Models (CM). Median patient age 6.5years (0.24-38.8) and weight 20.6kg (3.4-107.0). AR and CM quality were graded highly. RV pacing was associated with higher quality of both model types (p = 0.02). Visualization and identification of structures were graded as "very easy" in 81.1% (n = 73) and 67.8% (n = 61) of AR and CM, respectively. Fifty-nine (66%) grades 'Agreed' or 'Strongly Agreed' that AR models provided superior appreciation of 3D relationships; AR was found to be least beneficial in visualization of aortic arch obstruction. AR models were thought to be helpful in identifying pathology and assisting in interventional planning in 85 assessments (94.4%). There was significant potential seen in the opportunity for patient/family counseling and trainee/staff education with AR models. It is feasible to convert 3D models of 3DRAs into AR models, which are of similar image quality as compared to CM. AR models provided additional benefits to visualization of 3D relationships in most anatomies. Future directions include integration of interventional simulation, peri-procedural counseling of patients and families, and education of trainees and staff with AR models.

Aortic Arch Reconstruction Using Nonvalved Femoral Vein Homograft in High-Risk Neonates.

Aortic arch obstruction is often present with complex concomitant congenital heart defects (CHDs). The use of nonvalved femoral vein homograft (FVH) to reconstruct the aortic arch has distinct surgical advantages, including simplified reconstruction. We present an intraoperative video of a Yasui procedure utilizing FVH for aortic reconstruction in a 12-day-old (2.2 kg) neonate with right ventricular outflow tract obstruction, malalignment ventricular septal defect, aortic valve atresia, aortic arch hypoplasia, atrial septal defect, and ductal dependent systemic circulation. Further, we report outcomes for a series of three additional neonatal patients with complex CHD and aortic arch obstruction who underwent FVH arch reconstruction.

Changes in Cerebral Regional Oxygen Saturation Variability in Neonates Undergoing Cardiac Surgery: A Prospective Cohort Study.

Near-infrared spectroscopy is routinely used in the monitoring of cerebral regional oxygen saturation (crSO2) in neonates following congenital heart surgery. Decreased postoperative crSO2 variability in these patients is associated with worse clinical outcomes, including neurodevelopmental outcomes. We sought to explore changes in crSO2 variability between the preoperative and postoperative periods and associations with short-term clinical outcomes in neonates undergoing cardiac surgery. We performed a prospective cohort study of neonates undergoing cardiac surgery with cardiopulmonary bypass between November 2019 and May 2021. We calculated crSO2 variability using averaged 1min of crSO2 values for a minimum of 12h before, and the first 48h following surgery. 37 neonates (median age at start of monitoring 4days (interquartile range 2-5days)) were included in our study. We observed a 30% decrease in crSO2 variability between the preoperative and postoperative monitoring periods (p < 0.001). Preoperative crSO2 variability increased by 9% (p = 0.009) for each additional postnatal day. There were no associations between the degree of decrease in crSO2 variability postoperatively and class of cardiac lesion (e.g., aortic arch obstruction, single ventricle physiology) or short-term postoperative clinical outcomes. There was a significant decrease in postoperative crSO2 variability following neonatal cardiac surgery as compared to the preoperative period, likely influenced by several factors. The impact of interventions on crSO2 variability and resultant influence on long-term outcomes, such as neurodevelopmental outcomes, requires further exploration.

Interdigitating Technique for Repair of Aortic Arch Obstruction to Reduce Reintervention Rates

BackgroundThe incidence of reintervention for aortic arch obstruction is 5% to 14% after coarctation or hypoplastic aortic arch repair and 25% after the Norwood procedure. Institutional practice review indicated higher than reported reintervention rates. Our aim was to assess the impact of an interdigitating reconstruction technique on reintervention rates for recurrent aortic arch obstruction. MethodsChildren (<18 years) were included if they had undergone aortic arch reconstruction by sternotomy or the Norwood procedure. Three surgeons participated in the intervention with staggered rollout dates between June 2017 and January 2019, with the study ending December 2020 and review for reinterventions ending February 2022. Preintervention cohorts represented patients who underwent aortic arch reconstructions with patch augmentation, and postintervention cohorts represented patients who underwent an interdigitating reconstruction technique. Reinterventions by cardiac catheterization or operation were measured within 1 year of initial operation. Wilcoxon rank sum and χ2 tests were used to compare preintervention and postintervention cohorts. ResultsOverall, 237 patients were included for participation in this study, with 84 patients in the preintervention cohort and 153 in the postintervention cohort. Patients undergoing the Norwood procedure represented 30% (n = 25) of the retrospective cohort and 35% (n = 53) of the intervention cohort. Overall reinterventions were significantly decreased after the study intervention from 31% (n = 26/84) to 13% (n = 20/153; P < .001). Reintervention rates were decreased for each intervention cohort: aortic arch hypoplasia (24% [n = 14/59] vs 10% [n = 10/100]; P = .019) and Norwood procedure (48% [n = 12/25] vs 19% [n = 10/53]; P = .008). ConclusionsThe interdigitating reconstruction technique for obstructive aortic arch lesions was successfully implemented and is associated with a decrease in reinterventions.

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.

Long-term follow-up after simultaneous arterial switch operation and aortic arch repair.

Taussig-Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5-9] days, weight 3.38 (IQR 2.9-3.8) kg and follow-up 9.3 (IQR 3.1-14.5) years. The AA reconstruction was performed without patch material in 49 cases. There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate.

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.