Arch Interruption Research Articles

Biventricular repair in low-weight patient with interrupted aortic arch and aortic atresia

BackgroundAortic atresia with ventricular septal defect is a very rare congenital cardiac anomaly, especially in combination with aortic arch interruption. It is always challenging to choose the optimal treatment tactics for such patients. One of the possible types of intervention is the Yasui procedure. There are only 19 reported cases in the literature of aortic atresia with interruption of the aortic arch type B or C, and not a single clinical case of type A.Case presentationThe proband was a 2-day-old boy with diagnosis: aortic atresia with a ventricular septal defect and interruption of the aortic arch type B. The child underwent a Yasui procedure without serious postoperative complications and with good long-term result.ConclusionsThe Yasui procedure in patients with aortic atresia and interrupted aortic arch can be performed with minimal complications, even in low-weight patients.

4D flow mapping in adult with congenital aortic arch interruption and asymptomatic bypass occlusion: competitive flow patterns.

4D flow mapping in adult with congenital aortic arch interruption and asymptomatic bypass occlusion: competitive flow patterns.

Hydrops fetalis caused by a complex congenital heart defect with concurrent hypoplasia of pulmonary blood vessels and lungs visualized by micro-CT in a French Bulldog

BackgroundHydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species, including dogs. Most of cases result from a heart defect. Exact nature of this defect is rarely clarified.Case presentationA newborn, male French bulldog puppy with severe HF underwent a full anatomopathological examination to diagnose the primary cause of HF. Based on the anatomopathological examination, fetal ultrasound, and micro-computed tomography, transposition of the great arteries with hypoplasia of the ascending aorta, aortic arch interruption, ostium secundum atrial septal defect, severe tricuspid valve dysplasia, as well as hypoplasia of pulmonary vessels and lungs were diagnosed.ConclusionsThis is the first report of HF caused by severe, complex congenital heart defects with concurrent pulmonary vessel and lung hypoplasia.

Echocardiographic features and pathological ultrastructural characteristics of fetal interruption of aortic arch.

Interruption of aortic arch (IAA) is a rare congenital heart disease. This study aims to investigate echocardiographic features and pathological ultrastructural characteristics of fetal IAA and to further analyze its pathological evolution. A retrospective analysis was conducted on prenatal echocardiographic, post-surgical, or autopsy findings of fetuses prenatally diagnosed with IAA. Prenatal echocardiographic tracking was used to observe the internal diameters and Z-scores of different segments of the aortic arch and the changes in the narrowed section. These observations were combined with autopsy and pathological findings to explore the potential intrauterine evolution of IAA and its cytological basis. The study included 34 fetuses with IAA, with 3, 3, and 28 fetuses prenatally diagnosed with aortic arch dysplasia (AAD), coarctation of aorta (CoA), and IAA, respectively. The 3 AAD and 3 CoA fetuses chose termination of pregnancy 1 to 2 weeks after prenatal ultrasound diagnosis, and autopsy confirmed IAA. Among the 28 fetuses prenatally diagnosed with IAA, 6 cases of CoA progressively worsened, eventually evolving into type A IAA as observed through echocardiographic follow-up. The remaining 22 cases were diagnosed as IAA on the first prenatal ultrasound. Postnatal surgery corrected 3 cases, while 27 cases opted for pregnancy termination, and 4 cases resulted in intrauterine death. Echocardiographic features of the fetal IAA included a significantly smaller left ventricle compared with the right or negligible difference on the four-chamber view, a significantly smaller aorta than the pulmonary artery on the three-vessel view, and a lack of connection between the aorta and the descending aorta on the three-vessel-trachea and aortic arch views. The aortic arch appears less curved and more rigid, losing the normal "V" shape between the aorta, ductus arteriosus, and descending aorta. Color Doppler ultrasound showed no continuous blood flow signal at the interruption site, with reversed blood flow visible in the ductus arteriosus. Transmission electron microscopy of 7 IAA fetuses revealed numerous disorganized smooth muscle cells between the elastic membranes near the aortic arch interruption site, significantly increased in number compared with the proximal ascending aorta. The elastic membranes were thicker and more twisted near the interruption site. The interruption area lacked normal endothelial cells and lumen, with only remnants of necrotic endothelial cells, disorganized short and thick elastic membranes, and randomly arranged smooth muscle cells. Prenatal echocardiography is the primary diagnostic tool for fetal IAA. Post-surgical follow-up and autopsy help identify complications and disease characteristics, enhancing diagnostic accuracy. Some fetal IAA may evolve from AAD or CoA, with potential pathogenesis related to ischemia, hypoxia, and migration of ductal constrictive components.

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.

Arc aortic întrerupt asociat cu alte malformaţii cardiace congenitale

The interrupted aortic arch is a rare congenital mal­for­ma­tion (about 1.5% of cardiac malformations) with several anatomical variants and is often associated with other con­ge­nital cardiac anomalies. The authors present the case of a pediatric patient with aortic arch interruption and other congenital heart defects, along with the diagnostic features and indications for treatment.

Interrupted aortic arch in children: An in-depth look at etiology, embryological mechanisms, and morphology (a literature review)

Relevance: Congenital heart defects (CHD) account for one-third of all congenital malformations and occur in 0.7-1.7% of newborns. Interrupted aortic arch (IAA) is quite rare, accounting for 1% of CHD. IAA is an unusual shape of critical neonatal heart disease in which there is a lack of continuity between the ascending aorta and the descending thoracic aorta. IAA is almost always severe in the neonatal period and requires urgent corrective surgery.This study aimed to identify gaps in knowledge, suggest directions for in-depth research through critical analysis, and improve our understanding of risk factors and mechanisms of embryology that contribute to aortic arch interruption to improve the approach to diagnosis and treatment.Materials and Methods: A literature search was conducted in electronic databases PubMed, MEDLINE, Web of Science, Google Scholar, and e-library. The review covered 20 sources meeting the inclusion criteria.Results: We systematize the available literature data on embryology and risk factors to improve the IAA diagnosis and treatment approach. We found that aorta development begins at Week 3 of pregnancy, and the main factor in further IAA etiology is the chromosomal deletion 22q11.2.Conclusion: Understanding the timeline during early embryonic development plays a critical role in aortic arch formation. Abnormalities occurring in this period, such as chromosomal deletions, may contribute to IAA. This finding highlights the importance of genetic factors in the etiology of this condition and the potential for genetic screening and counseling for individuals with chromosomal abnormalities, especially those in the 22q11.2 region.

Damus-Kaye-Stansel surgery in a patient with tricuspid atresia, transposition of the great arteries, and type A aortic arch interruption.

Damus-Kaye-Stansel surgery in a patient with tricuspid atresia, transposition of the great arteries, and type A aortic arch interruption.

Abstract 9409: Risk Factors and Outcomes Associated With Loss of Follow-Up in Children With Congenital Heart Disease

Introduction: Adults with congenital heart disease (CHD) benefit from cardiology follow-up. However, benefit for children is less clear as studies of follow-up patterns in children with CHD are limited. Hypothesis: There are identifiable risks for loss to cardiology follow-up in children with CHD, and loss to follow-up changes healthcare utilization and outcomes. Methods: Our cohort included children <10 years old with CHD and a healthcare encounter from 2008 to 2013 at one of four North Carolina hospitals. Using a Cox proportional hazards model, we assessed associations between demographics, presence of a severe lesion (conotruncal, endocardial cushion, and single ventricle lesions, aortic arch interruption, and total anomalous pulmonary venous return), and healthcare access, including educational isolation (EI) via geo-coded neighborhood of residence, and at least one cardiology follow-up visit within two years of initial encounter. We analyzed healthcare utilization by follow-up using a negative binomial landmark analysis. We described incidence of death and cardiac transplantation. Results: Of the 6,969 children, 24.8% were non-Hispanic Black and 14.1% were Hispanic. Overall, 60.4% received follow-up within 2 years, including 53.1%, 58.1%, and 79.0% of those with valve, shunt, and severe lesions respectively. Factors associated with loss to follow-up were increased drive time to the nearest cardiology clinic (Hazard Ratio 0.92 per 15-minute increase), EI (HR 0.94 per 0.2-unit increase), lower lesion severity (HR 0.48 for shunt/valve vs severe), and older age at initial encounter (HR 0.95 per month if <1 year old and 0.94 per year if >1 year old; p<0.05 ). Children lost to follow-up had more emergency department (ED) visits (Rate Ratio 1.59) and fewer inpatient encounters and procedures (RR 0.51, 0.35; p<0.05 ). Only 0.8% of children died or received a cardiac transplant after the 2-year landmark with no significant difference between follow-up groups. Conclusions: We found novel factors associated with loss to cardiology follow-up in children with CHD and worse utilization with loss to follow-up. Our findings demonstrate a need to mitigate healthcare barriers and generate clear cardiology follow-up guidelines for children.

Aortopulmonary window, aortic arch interruption, and anomalous origin of the right pulmonary artery in a neonate with Goldenhar syndrome.

The combination of aortopulmonary window, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the ascending aorta is a rare and complex congenital cardiac malformation. Despite good prenatal care in our case, this cardiac anomaly was not detected prior to birth. Untreated infants who do not undergo surgical correction have a mortality rate of 70% in their first year, and 30% will die within the first 3 months of life.

Aortic Atresia or Complex Left Outflow Tract Obstruction in the Presence of a Ventricular Septal Defect.

Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

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.

22q11.2 Deletion Syndrome: Impact of Genetics in the Treatment of Conotruncal Heart Defects.

Congenital heart diseases represent one of the hallmarks of 22q11.2 deletion syndrome. In particular, conotruncal heart defects are the most frequent cardiac malformations and are often associated with other specific additional cardiovascular anomalies. These findings, together with extracardiac manifestations, may affect perioperative management and influence clinical and surgical outcome. Over the past decades, advances in genetic and clinical diagnosis and surgical treatment have led to increased survival of these patients and to progressive improvements in postoperative outcome. Several studies have investigated long-term follow-up and results of cardiac surgery in this syndrome. The aim of our review is to examine the current literature data regarding cardiac outcome and surgical prognosis of patients with 22q11.2 deletion syndrome. We thoroughly evaluate the most frequent conotruncal heart defects associated with this syndrome, such as tetralogy of Fallot, pulmonary atresia with major aortopulmonary collateral arteries, aortic arch interruption, and truncus arteriosus, highlighting the impact of genetic aspects, comorbidities, and anatomical features on cardiac surgical treatment.

Outcomes Following Aortic Valve Replacement in Children With Conotruncal Anomalies.

Conotruncal anomalies can develop aortopathy and/or aortic valve (AV) disease and AV replacement (AVR) is occasionally needed. We report long-term results and examine factors affecting survival following AVR in this group. We queried the Pediatric Cardiac Care Consortium (PCCC, US database for interventions for congenital heart diseases) to identify patients with repaired conotruncal anomalies and AVR. Long-term outcomes were provided by the PCCC, the US National Death Index, and Organ Procurement and Transplantation Network. Competing risks analysis examined outcomes following AVR (death/transplantation, reoperation) and multivariable regression analysis assessed significant factors. One hundred six children with repaired conotruncal anomalies underwent AVR (1982-2003). Underlying anomaly was truncus (n = 40), d-transposition (n = 22), type-B interrupted arch (n = 16), double-outlet right ventricle (n = 12), pulmonary atresia with ventricular septal defect (n = 9), tetralogy of Fallot (n = 6), corrected transposition (n = 1). 18 (17%) had prior aortic valvuloplasty (surgical = 12, percutaneous = 6). Median age at AVR was 6.9 years (interquartile range = 2.5-12.4). AV pathophysiology was regurgitation (n = 83, 78%), stenosis (n = 9, 9%), and mixed (n = 14, 15%). AVR type was mechanical (n = 72, 68%), homograft (n = 21, 20%), and Ross (n = 13, 12%). Operative mortality was 13(12%). Infant age at AVR was risk factor (odds ratio = 55, 95% confidence interval [CI] = 6-539, P = .0006). On competing risks analysis, five years after AVR, 6% died or received transplantation, 20% had reoperation. Twenty-five years transplant-free survival was 53%. Factors associated with death after hospital discharge included mitral surgery (hazards ratio [HR] = 11, 95% CI = 3-39, P = .0002), underlying defect (HR = 2, 95% CI = 1-5, P = .446). Twenty years transplant-free survival in conotruncal anomalies group was inferior to matched children undergoing AVR for congenital non-conotruncal disease (61% vs 82%, P = .0012). Long-term survival following AVR in children with conotruncal anomalies is inferior to that of isolated congenital AV disease and is linked to an underlying cardiac defect. Although valve type was not associated with survival, infant age was a risk factor for operative mortality. Continuous attrition and high reoperation warrant vigilant monitoring.

Clinical and Post-Surgical Features and Outcomes in Post-Surgical Patients with Aortic arch Interruption at a Specialty Hospital in Bogotá Between 2006 and 2019. Retrospective Multivariable Analysis

Introduction. Interruption of the aortic arch due to absence of tissue between the ascending and descending portions of the aorta is a pathology of low incidence, yet it has a mortality rate of nearly 90% within the first year of life. Surgical intervention is aimed at re-establishing continuity of the vessel, as well as correcting associated lesions through closure of IVC or performing a pulmonary artery cerclage correction. The mortality is 18% to 42%. The survival rate is close to 73% at one month, 65% at one year and 63% at 3 years after surgery. Materials and Methods. Review of Cardiovascular Surgery database between 2006 and 2019, identifying patients who underwent surgery due to aortic arch interruption. Results. In the last 4 years, an institutional technique was introduced, using pericardium tissue for reconstruction and partial junction of the descending aorta with the arch through advancement of the arterial cannulae towards the brachiocephalic trunk and has been positively associated to brain protection. Since, results have improved: lower tissue traction, greater amplitude of the neoarch and better neurological outcomes have been achieved. The objective of this paper is to identify the most important factors that ultimately determine prognosis and survival of patients with these pathologies, as well as to describe results of the implemented technique. Discussion. Aortic arch surgery is considered to be highly complex. Results yielded in this research showed a high mortality within the first years, which decreased into a similar rate to that of medical literature. The main factors associated with complications identified were a late diagnosis and malnutrition. In addition, morbidity and mortality rates were similar to those reported worldwide.

Repairing Facial Fractures with Interrupted Maxillary-mandibular Arches by Computer-assisted Reverse Planning Model Surgery.

Background:Management of comminuted facial fractures with maxillary-mandibular arch interruption is difficult, resulting in inadequate bone reduction and malocclusion. Traditionally, a good quality dental splint is helpful, but difficult to obtain in acute trauma. We apply a computer-assisted design and three-dimensional printing technology to improve splint fabrication and utilization, thus facilitating restoration of dental occlusion and facial fracture.Methods:We retrospectively reviewed patients who suffered from facial fractures with interruption of the maxillary-mandibular arches. We developed the “computer-assisted reverse planning and three-dimensional printing model surgery” algorithm and applied it in selected patients. An occlusal splint was created as a surgical guide to enhance the maxilla-mandibular unit repair by taking care of the bone reduction and occlusion. All included patients were followed up to assess the functional outcome and patients suitable for this method.Results:From Jan 2015 to Aug 2020, 10 patients (eight men and two women) with comminuted facial fractures were included. The average time of surgery was 9.2 days. The average follow-up time was 8.6 months. There was no patient who needed major revision to correct malocclusion or facial asymmetry.Conclusions:A computer-assisted design splint decreases intraoperative inaccuracies and difficulty in comminuted maxillo-mandibular fractures. It is a useful and reliable alternative. Collaboration with an experienced engineer and patient selection are indispensable in delivering successful outcomes. Patients who have more than three bone fragments in a single dental arch or more than four bone fragments in the entire maxillary-mandibular unit appear to be excellent candidates for this method.

Common Arterial Trunk with Interrupted Aortic Arch.

Clinical data: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. Diagnosis: Association of aortic arch interruption type A is uncommon and should be considered. Operation: Debanding of pulmonary arteries allowing for possible future complete repair.

Abstract 9023: Post-Operative Ectopic Atrial Tachycardia in Congenital Heart Disease

Introduction: Ectopic atrial tachycardia (EAT) is relatively common in infants following surgery for congenital heart disease (CHD). There is limited data regarding this arrhythmia. Hypothesis: Operative factors, including CHD diagnosis, can identify risk factors for post-operative EAT. Methods: This was a retrospective analysis of patients undergoing CHD surgery in the first year of life between January 2007 and July 2020. Patients and surgeries with EAT were compared to those without EAT. Results: Out of 5,792 infant CHD surgeries, 129 (2.2%) developed postoperative EAT. The onset of EAT occurred at a median of 9 days (IQR 5-14 days) after CHD surgery. Compared to controls, the EAT cohort was younger (median 7 vs 85 days, p &lt;0.01) and had lower weight (3.3 vs 4.3 kg, p &lt;0.01) with no gender difference. EAT occurred following 24/265 (9%) total anomalous pulmonary venous connection (TAPVC) repairs (relative risk of 4.7, p&lt;0.001), 22/351 (6%) arterial switch operations (RR 3.2, p&lt;0.001), 6/90 (7%) interrupted arch repair (RR 3.1, p=0.004), and 23/460 (5%) Norwood operations (RR 2.5, p&lt;0.001). EAT patients had longer cardiopulmonary bypass (median 64 vs 58 min, p=0.008) and deep hypothermic circulatory arrest times (15 vs 0 min, p&lt;0.001) but no statistically significant difference in cross clamp times (25 vs 31 min). Antiarrhythmics were given to 109 patients (84%), but treated patients were more likely to be identified for study inclusion potentially biasing this percent-treated value. Propranolol (60%), amiodarone (22%), and flecainide (4%) were the most frequently used antiarrhythmics during hospitalization. On discharge, 60% of EAT patients were on antiarrhythmics, most commonly propranolol (55%), amiodarone (4%), and flecainide (4%). Although 15 patients did not survive to hospital discharge, EAT was not directly implicated in any deaths. Conclusions: In this study of a large cohort of postoperative EAT patients, the arrhythmia occurred in 2.2% of cases with a median onset of 9 days after surgery. TAPVC repair carried the highest risk of postoperative EAT, followed by arterial switch, interrupted aortic arch repair, and Norwood operations. Patients with EAT were younger, smaller, and had longer bypass and circulatory arrest times than controls.

Long-term evolution of aortic arch interruption repair with pulmonary artery autograft

Surgical correction of the aortic arch interruption (IAA) is regularly complicated by anastomosis stenosis or the long-term occurrence of arterial hypertension. In order to prevent these complications, a new surgical technique has been developed in our center consisting of the interposition of a segment of the main pulmonary artery to enlarge the aortic anastomosis. The purpose of this study was to assess the 10 years outcome of this intervention. Four children undergoing this surgery with a follow-up over 10 years were retrospectively included. Data collected focused on the last measure of blood pressure (BP), the last echocardiographic evaluation, and the aortic arch dimension on the last CT. The whole patients had IAA type B and 22q11 microdeletion. The median follow-up duration was 13.9 years (range: 9.3 to 15.3). None of them had a new surgery of the aortic arch, but one need enlargement of ascending aorta. Only one patient developed a mild restenosis at the isthmic insertion of the pulmonary autograft [Z score −2.7 at CT ( Fig. 1 C)], responsible of mild arterial hypertension (systolic BP 75th percentile, diastolic BP 98th percentile), and a left ventricle hypertrophy (Z score IVSd 2.5, Z Score LVPWd 2.1). The three others patients displayed normal BP, no left ventricular hypertrophy, no significant aortic arch obstruction on Doppler evaluation and minimum Z score of aortic arch at CT from −1.8 to −0.4 ( Fig. 1 A, B, D). None of them did experience a main pulmonary artery stenosis. Reconstruction of the aortic arch with a pulmonary artery autograft for the correction of IAA provides a good long-term result.

Unusual presentation of aortopulmonary septal defect and aortic arch interruption

Unusual presentation of aortopulmonary septal defect and aortic arch interruption