Interrupted Aortic Arch Research Articles

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.

INTERRUPTED AORTIC ARCH IN A ONE DAY OLD NEWBORN: A CASE REPORT

Interrupted aortic arch (IAA) is a rare congenital heart disease (CHD) which affects approximately 1.5% of CHD patients. It is a serious congenital vascular malformation and accounts severe mortality when associated with other intracardiac defects. This report describes the case of one day old infant with atresia of aortic arch presented with rapid breathing, poor feeding, and lethargy. The diagnosis of IAA and Ventricular septal defect (VSD) was made by two-dimensional echocardiography and conrmed intraoperatively which successfully corrected by immediate surgical intervention.

Tracheobronchial Release for Left Bronchus Compression After Aortic Arch Repair

Narrowing of the retroaortic space after repair of common arterial trunk (CAT) with interrupted aortic arch (IAA) is a well-known issue. We present a newborn with CAT, IAA, and functionally univentricular heart (tricuspid atresia) who underwent repair of CAT, IAA, and left ventricle-to-pulmonary artery (LV-to-PA) conduit placement with the Lecompte maneuver. The patient suffered from left bronchus compression postoperatively, which was relieved by tracheobronchial release.

The Role of Computed Tomography in The Diagnosis of Rare Congenital Heart Disease: Interrupted Aortic Arch

Interrupted aortic arch (IAA) is a rare congenital anomaly of the aortic arch and an anatomical interruption of the lumen between the ascending and descending aorta. Computed tomography (CT) has become a reliable noninvasive diagnostic method for congenital IAA. The purpose of this study was to investigate the imaging features of IAA and improve the understanding and diagnosis of the disease. The imaging features and postoperative pathological data of 25 patients with IAA confirmed by dual-source computed tomography (DSCT) angiography were analyzed in this retrospective study. Among the 25 patients with IAA, 15 were type A, seven were type B, 0 were types C and D, and two were type E. The diameter of the pulmonary artery trunk in type A was larger than that in type B (P < 0.05). However, there were no significant differences between types A and B along the ascending aorta diameter, descending aorta, ascending aorta/descending aorta ratio, left pulmonary artery main trunk diameter, right pulmonary artery main trunk diameter, left pulmonary artery trunk/pulmonary artery trunk ratio, right pulmonary artery trunk/pulmonary artery trunk ratio, and left pulmonary artery trunk/right pulmonary artery trunk ratio. The imaging findings of IAA have typical and specific signs, and the types of IAA are not comprehensive. One type of patient identification can be added: Patients who are dissected between the left common carotid artery and left subclavian artery opening, and the descending aorta is circulated by the chest collaterals. Patients with a wide pulmonary artery in the IAA are usually type A patients. Patients with IAA after surgical repair require lifetime follow up, mainly to monitor left ventricular outflow tract obstruction and recurrent aortic coarctation. This study was approved by the Ethics Committee of Kunming Yan'an Hospital (Kunming City, Yunnan Province, China), and consent was waived because of the retrospective data collection.

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.

Outcomes of One-Stage Surgical Repair for Berry Syndrome in Neonates.

BackgroundBerry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. Here, we summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future.MethodsFrom January 2014 to December 2019, three neonates with berry syndrome underwent one-stage surgical repair in our center. We mainly used two different surgical techniques to repair it, and collected clinical data retrospectively from hospitalization history, outpatient records, and telephone follow-up.ResultsThe age at operation was 28, 8, and 8 days and the body weight was 3.65, 3.86, and 3.0 kg, respectively. The morphology of the interrupted aortic arch (IAA) was type A in two patients and type B in one patient. The aortopulmonary window (APW) morphology was type IIa, III, and IIb, respectively. The phenotype of the IAA type B combined with APW type III in our second patient was reported for the first time so far. All patients survived and were followed up to date. The second patient using intra-aortic baffle experienced twice reoperation for right pulmonary artery (RPA) stenosis. All patients grew well so far.ConclusionOnce diagnosed in the neonatal period, berry syndrome can be safely corrected by one-stage surgical repair in experienced cardiac centers. Considering the variability of the location where the RPA arises from the posterior wall of the aorta, it is difficult to find the best surgical method for each patient.

Aortik Ark Kesintisi Olan Erişkin Hastada Perkütan Aort Stent İmplantasyonu

An interrupted aortic arch (IAA) is an infrequent congenital or acquired malformation defined as a complete absence or discontinuation of a portion of the aortic arch. In most cases especially in neonates, IAA is accompanied by a cardiac malformation or genetic syndromes. IAA has a very poor prognosis in patients without surgical treatment, so it is rarely detected in adulthood. Nowadays the definitive treatment option is still surgery. However, percutaneous aortic stent implantation may be a life-saving alternative treatment method, especially in patients with high surgical risk. Herein we present a very rare case of Type A IAA in a 55-year-old female patient which was successfully treated with percutaneous aortic stent implantation.

Truncus Arteriosus with Interrupted Aortic Arch: Aortic Arch Reconstruction Without Circulatory Arrest

AbstractTruncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare congenital cardiac anomaly. Traditionally, IAA would require period of deep hypothermic circulatory arrest (DHCA) for aortic arch reconstruction. DHCA may cause myocardial depression and neuro-developmental dysfunction. IAA repair is presently being performed with hypothermic low-flow selective cerebral perfusion and distal body circulatory arrest. Even though this prevents cerebral adverse effects of DHCA, there is still risk to the spinal cord and kidneys.Here, we describe the aortic arch reconstruction by maintaining the total body perfusion without any periods of circulatory arrest in a neonate with TA with IAA.

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.

Incidence of Aberrant Right Subclavian Artery in General Population

Objective: The existence of an anomalous right subclavian artery is the most significant aortic arch defect. Dysphagia, wheezing, stridor, and other symptoms may occur if this vessel pressures the neighbouring structures. The goal of this study is to find out how common ARSA is based on CT scans of the thorax and neck (plain and contrast).&#x0D; Method: Between January 1st and December 31st, 2020, 1122 patients had CT Thorax/neck scans, and the data were evaluated. The origin of the ARSA, the vessel's path, and the Kommerell diverticulum were all assessed. We looked at the literature to see how important ARSA is in clinical practise.&#x0D; Results: ARSA was found in eight of 1122 patients. All of the ARSAs in these eight individuals began at the posterior part of the aortic arch and travelled via the retroesophageal path to the thoracic outlet. All eight ARSA were found in the front part of the thoracic vertebral bodies, from the first to the fourth.&#x0D; Conclusion: Other clinical symptoms such as dysphagia, dyspnea, retrosternal discomfort, cough, and weight loss must be differentiated from compression of surrounding tissues by an abnormal right subclavian artery.

Hospital Outcomes Among Infants With Interrupted Aortic Arch With Simple and Complex Associated Heart Defects

There is a lack of current, multi-institutional data regarding hospital outcomes of infants with interrupted aortic arch (IAA). We analyzed the Pediatric Health Information System database to identify infants with IAA who underwent repair during 2004 to 2019. We classified patients as simple or complex based on associated heart defects. We evaluated factors associated with hospital mortality and complications related to 22q11.2 deletion syndrome (22q11.2del) using mixed logistic regression, accounting for hospital clustering. In 1,283 infants included (904 simple, 379 complex), mortality was higher in the complex group (11.7% vs 4.4%, p <0.001). Factors associated with mortality in the simple group were low birth weight (adjusted risk ratio [aRR] 3.77, 95% confidence interval [95% CI] 1.83 to 7.77), non-22q11.2del genetic conditions (aRR 6.44, 95% CI 1.73 to 23.96), and gastrointestinal anomalies (aRR 8.47, 95% CI 3.12 to 22.95), whereas surgery between 2012 and 2015 (aRR 0.36, 95% CI 0.13 to 0.99) was protective. In the complex group, factors associated with mortality were male (aRR 2.32, 95% CI 1.10 to 4.24) and central nervous system anomalies (aRR 3.73, 95% CI 1.62 to 8.59). Compared with their nonsyndromic counterparts, infants with simple IAA and 22q11.2del were at higher risk of sepsis (aRR 1.63, 95% CI 1.02 to 2.39) and gastrostomy tube placement (aRR 3.18, 95% CI 2.13 to 4.74), and infants with complex IAA and 22q11.2del were at higher risk of gastrostomy tube placement (aRR 2.42, 95% CI 1.20 to 4.88). In conclusion, presence of complex cardiac lesions is associated with increased mortality after IAA repair. The co-occurrence of extracardiac congenital anomalies and non-22q11.2del genetic conditions elevates mortality risk. Presence of 22q11.2del is associated with hospital complications.

Management of Interrupted Aortic Arch With Associated Anomalies: A Single-Center Experience.

Interrupted aortic arch (IAA) includes a broad spectrum of associated anomalies. In this study, we present our surgical management and patient-specific decisions regarding IAA anomalies with early- and mid-term outcomes. The medical records of 25 patients undergoing IAA repair between 2014 and 2019 were retrospectively reviewed. Sixteen patients had type B (64%) interruptions, 7 had type A (28%) interruptions, and 2 had type C (8%) interruptions. Fourteen patients had an isolated ventricular septal defect, and 3 of them had associated left ventricular outflow tract obstruction. Other associated anomalies were functional single ventricle (n = 5), Taussig-Bing anomaly (n = 3), aortopulmonary window (n = 1), multiple ventricular septal defects (n = 1), and truncus arteriosus with dextrocardia (n = 1). The initial operation age was 17.2 ± 14 (range: 1 - 60) days. Single-stage total repair was performed for 15 patients. Six patients underwent aortic arch repair and pulmonary artery banding. Four patients with left ventricular outflow tract obstruction or who were premature underwent the hybrid procedure. The aortic arch repair was performed in 16 cases (64%) by the anterior patch augmentation technique, in 3 cases (12%) by the reverse left subclavian artery flap technique, and in 3 cases (12%) by direct end-to-end anastomosis. Postoperative early mortality occurred in 4 (16%) patients, and sternal closure was delayed in 13 (52%) patients. Three patients who underwent a hybrid procedure due to left ventricular outflow tract obstruction underwent biventricular repair 8 to 13 months later. Eight patients (38%) required reintervention due to arch restenosis during the follow-up period. The mean follow-up was 37.1 ± 21.7 months. Planning surgical treatment according to the characteristics of the patients and accompanying anomalies may improve the results.

Msx1 haploinsufficiency modifies the Pax9-deficient cardiovascular phenotype

BackgroundSuccessful embryogenesis relies on the coordinated interaction between genes and tissues. The transcription factors Pax9 and Msx1 genetically interact during mouse craniofacial morphogenesis, and mice deficient for either gene display abnormal tooth and palate development. Pax9 is expressed specifically in the pharyngeal endoderm at mid-embryogenesis, and mice deficient for Pax9 on a C57Bl/6 genetic background also have cardiovascular defects affecting the outflow tract and aortic arch arteries giving double-outlet right ventricle, absent common carotid arteries and interruption of the aortic arch.ResultsIn this study we have investigated both the effect of a different genetic background and Msx1 haploinsufficiency on the presentation of the Pax9-deficient cardiovascular phenotype. Compared to mice on a C57Bl/6 background, congenic CD1-Pax9–/– mice displayed a significantly reduced incidence of outflow tract defects but aortic arch defects were unchanged. Pax9–/– mice with Msx1 haploinsufficiency, however, have a reduced incidence of interrupted aortic arch, but more cases with cervical origins of the right subclavian artery and aortic arch, than seen in Pax9–/– mice. This alteration in arch artery defects was accompanied by a rescue in third pharyngeal arch neural crest cell migration and smooth muscle cell coverage of the third pharyngeal arch arteries. Although this change in phenotype could theoretically be compatible with post-natal survival, using tissue-specific inactivation of Pax9 to maintain correct palate development whilst inducing the cardiovascular defects was unable to prevent postnatal death in the mutant mice. Hyoid bone and thyroid cartilage formation were abnormal in Pax9–/– mice.ConclusionsMsx1 haploinsufficiency mitigates the arch artery defects in Pax9–/– mice, potentially by maintaining the survival of the 3rd arch artery through unimpaired migration of neural crest cells to the third pharyngeal arches. With the neural crest cell derived hyoid bone and thyroid cartilage also being defective in Pax9–/– mice, we speculate that the pharyngeal endoderm is a key signalling centre that impacts on neural crest cell behaviour highlighting the ability of cells in different tissues to act synergistically or antagonistically during embryo development.

English

Congenital heart disease (CHD) often poses a great diagnostic challenge for physicians. Despite antenatal diagnostic tests advancing to a great level, accurate diagnosis and treatment of congenital heart diseases is mandatory. These diseases range from mild to severe life-threatening scenarios sometimes having vague presentations making diagnosis even more difficult. Early diagnosis and treatment are usually lifesaving.1 Congenital heart diseases can often be classified as cyanotic and acyanotic based on clinical presentation. After birth, fetal structures like foramen ovale, ductus venosus and ductus arteriosus are no longer required for survival and they begin to close.2 Persistence of such structures after birth is a sign of congenital heart diseases. High mortality contributing diseases which require prompt intervention include hypoplastic left heart syndrome (HLHS), coarctation of aorta (COA), interrupted aortic arch (IAA), transposition of the great arteries (TGA), total anomalous pulmonary venous return (TAPVR), critical aortic stenosis (AS) pulmonary atresia (PA) and tricuspid atresia (TA).3 They contribute to significant mortality amongst neonatal age groups. Recognition of congenital heart diseases based on clinical fractures like cyanosis, tachycardia, tachypnoea, irritability, refusal to feed stabilisation and prompt referral to tertiary cardiac centre are critical to improve outcomes in neonates with CHDs, seizures, murmur etc is diagnostically challenging but lifesaving. Life-threatening CHDs may perhaps present with cyanosis, respiratory distress, shock or collapse; all of these are also frequent clinical presentation of various respiratory problems or sepsis in newborn. Early diagnosis and prompt treatment are the only life saving measures.

Protective role of melatonin and spirulina in aortic occlusion-reperfusion model in rats.

The aim of this experiment was to investigate the role of melatonin and spirulina on multiorgan damage induced by ischemia/reperfusion injury (IR) in a rat model. A total of 32 male rats weighing 200-220 g were allocated into 4 groups (n = 8/group) (Sham, Control-IR [CIR], Melatonin-IR [MIR], and Spirulina-IR [SIR]). Sham group underwent midline laparotomy and dissection of the aorta without injury. In other groups, an IR model was established by clamping (ischemia) and releasing (reperfusion) the abdominal aorta at the supraceliac level for 20 min. All rats were given 3 ml/kg of distilled water by gavage for 14 days before and 7 days after the experiment. The treatment groups received either melatonin (50 mg/kg) or spirulina (50 mg/kg) by the same route. On the 21st day of the experiment, the rats were sacrificed. We found that melatonin and spirulina ameliorated the effects of IR at different levels of significance (ranging from p = .01 to p < .001), increasing total antioxidant capacity (TAC) and superoxide dismutase levels, and decreasing total oxidant status, oxidative stress index (OSI), myeloperoxidase, tumor necrosis factor-alfa and malondialdehyde levels. When compared MIR and SIR groups, only TAC and OSI levels did differ in favor of melatonin between the groups (p < .05). Histopathological and immunohistochemical examinations showed that melatonin and spirulina similarly reduced IR-related tissue damage and apoptosis. We concluded that melatonin and spirulina may have a protective role against oxidative tissue damage and apoptosis in the abdominal aortic IR animal model. PRACTICAL APPLICATIONS: Coarctation of aorta (CoA) and interrupted aortic arch (IAA) are serious cardiac defects with high morbidity and mortality if not diagnosed and treated early in life. Restoration of blood flow in CoA or IAA through prostaglandin E1 infusion, angioplasty or surgery can cause ischemia/reperfusion (IR) injury. This reperfusion period may be complicated IR injury at remote organs. It may be beneficial to increase antioxidant capacity in preventing stress-induced tissue damage. Melatonin and spirulina are agents with strong antioxidant properties. In this animal research, protective role of these products on multiorgan damage induced by IR was investigated for the first time. We found that both melatonin and spirulina ameliorate the effects of IR to varying degrees. This study provides evidence that melatonin and spirulina may have preventive effects on oxidative tissue damage and apoptosis in the abdominal aortic IR animal model.

Interrupted aortic arch: Is it always a neonatal emergency?

Interrupted aortic arch (IAA) is a rare congenital ductal-dependent heart disease. It commonly presents in infancy following spontaneous closure of the patent ductus arteriosus (PDA) [1] , [2] . Few cases have been reported in pediatric age [3] . We present the case of 13 years old boy with IAA and a large ventricular septal defect (VSD), a restrictive PDA and an Eisenmenger syndrome. A thirteen years old Tunisian boy presented to our consultation with malaise, headache and weakness of his legs. He reported reduced exercise tolerance since two years. There was no family history of congenital heart disease or sudden unexpected death ( Fig. 1 , Fig. 2 ). Physical examinations noted a generalized cyanosis. Radial pulses were normal but his femoral pulses were weak. The systolic pressure difference between the upper and lower limbs was 40 mmHg. Transthoracic echocardiography noted a conal VSD with a posterior malalignment of the conal septum with right to left shunt. The short axis examination showed a large pulmonary tract and a restrictive PDA with an increased velocity of 3.3 m/s and right to left shunt. The high left parasternal examination with a slightly rightward angulation of the transducer demonstrated a complete interruption of aortic arch. Computed tomography revealed an IAA between the left common carotid artery and the left subclavian artery (type B) and a PDA (3.3 mm) between the left pulmonary artery (LPA) and the descending aorta. Pulmonary arterial hypertension was confirmed in cathlab with mean pulmonary arterial pressure (mPAP) at 100 mmHg. Following multidisciplinary team discussion, on regard of patient health state, Eisenmenger syndrome, the restrictive PDA and the contraindication to performing surgery, we discuss the percutaneous stenting of the PDA. Interrupted aortic arch is a rare congenital abnormality that presents in infancy. We presented the case of 13 years old boy with IAA, Eisenmenger syndrome and a restrictive PDA. Following multidisciplinary team discussion, giving the restrictive pattern of the PDA and the Eisenmenger syndrome, we discuss a percutaneous of the PDA.

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

Is surgery necessary for adults with isolated interrupted aortic arch?: Case series with literature review.

Interrupted aortic arch (IAA) is defined as a complete interruption of aortic lumen between the ascending and descending aorta. It is an uncommon and complicated congenital heart disease. It is rare for patients with isolated IAA to survive to adulthood without operation. Here we present three rare cases with isolated IAA together with a review of the literature of IAA. Besides, we reviewed reported adult cases with isolated IAA in the last 20 years and summarized the relevant data of the isolated type. We retrospectively searched the hospital databases for adult patients with isolated IAA diagnosed at the Wuhan Union Hospital over the past 10 years. Cases related to adult isolated IAA published in last 20 years were identified by searching Pubmed. Three adult patients with isolated IAA were identified. Two were referred to us for hypertension management and were diagnosed with IAA. They declined surgical treatments and took antihypertensive medications. One patient was referred to our hospital for further treatment options after diagnosed with IAA at another hospital. He received an extra-anatomic bypass surgery. But his hypertension did not well resolve after surgery, and was subsequently managed by anti-hypertensives medications. 25 published adult patients with isolated IAA were identified in Pubmed and relevant details were summarized. Adult patients with isolated IAA usually have extensive collateral vessels joining the descending aorta. Anti-hypertensives medical management with long-term follow-up appears to be a reasonable treatment option for these patients, although surgical intervention is a good choice.

Hospital Survival After Surgical Repair of Truncus Arteriosus with Interrupted Aortic Arch: Results from a Multi-institutional Database.

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7-18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.