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Background: Coarctation of the aorta (CoA) can either present alone as an isolated condition or in association with other aortic arch or cardiac anomalies. One percent of patients with CoA have concomitant an aberrant right subclavian artery (ARSA). Purpose: We report the case of a 35-year-old woman with uncontrolled hypertension who was found to have CoA and ARSA. Results: The patient was treated successfully using a hybrid procedure comprising ARSA ligation and subclavian to carotid transposition, followed by thoracic endovascular aortic repair. Conclusions: Patients with CoA should be carefully studied, considering the possible coexistence of other congenital aortic arch defects, such as ARSA. Hybrid repair is a safe and effective approach for this condition.

Diverticulum of Kommerell is a congenital anomaly in the development of the primitive embryonic aortic vasculature resulting in either an aberrant left subclavian artery (ALSA) from a right-sided aortic arch (RSAA) or an aberrant right subclavian artery (ARSA) from a left-sided aortic arch (LSAA). Kommerell’s diverticulum (KD), is extremely rare in the general population having prevalence of 0.7–2.0 %. KD with true aneurysmal dilatation like any vascular lesion of the thoracic aorta weakens the vasculature and increases the risk of aortic rupture. Timely surgical intervention is of paramount importance in most of these cases. Variable clinical presentations in patients with KD are either a result of compression of some mediastinal organs or strangulation by the vascular ring from the aberrant subclavian artery (ASA). We report a case of a 39 years old Chinese female with a rare co-existence of a huge KD with a microaneurysm, an aberrant right subclavian artery (ARSA), and ventricular septal defect (VSD). She underwent a successful operation and all her pre-surgical symptoms disappeared.

Objective The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). Methods This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. Results An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed amalformation: one case of hypospadias and 1 case of cleft palate. Conclusion The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers. Resumo Objetivo O objetivo do presente estudo foi determinar a frequência demalformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). Métodos Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico prénatal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Resultados Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. Conclusão A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

The objective of the present study was to determine the frequency of malformations and chromosomal abnormalities in a population of fetuses with an aberrant right subclavian artery (ARSA). This is a 6-year retrospective study of fetuses with a prenatal diagnosis of ARSA conducted during the period between September 2013 and June 2019 at a fetal medicine unit. Data were collected from ultrasound, fetal echocardiograms, genetic studies, and neonatal records. An ARSA was diagnosed in 22 fetuses. An ARSA was an isolated finding in 18 out of 22 cases (82%). Associated abnormal sonographic findings were found in 4 cases. All cases underwent invasive testing. In 1 of the cases, a chromosomal abnormality was detected (mos 45,X [13]/46,X,e(X) (p22.1q22.1)). No cases of congenital heart disease were found in any of these fetuses. There were two cases in which the postnatal evaluation revealed a malformation: one case of hypospadias and 1 case of cleft palate. The presence of an isolated ARSA is benign and is not associated with chromosomal abnormalities. The finding of ARSA, however, warrants a detailed fetal ultrasound in order to exclude major fetal abnormalities and other soft markers. O objetivo do presente estudo foi determinar a frequência de malformaçães e anomalias cromossômicas em uma população de fetos com artéria subclávia direita aberrante (ARSA). MéTODOS: Este é um estudo retrospectivo de 6 anos de fetos com diagnóstico pré-natal de ARSA realizado durante o período de setembro de 2013 a junho de 2019 em uma unidade de medicina fetal. Os dados foram coletados de ultrassom, ecocardiograma fetal, estudos genéticos e registros neonatais. Um ARSA foi diagnosticado em 22 fetos. Um ARSA foi um achado isolado em 18 dos 22 casos (82%). Achados ultrassonográficos anormais associados foram encontrados em 4 casos. Todos os casos foram submetidos a testes invasivos. Em um dos casos, foi detectada uma anormalidade cromossômica (mos 45, X [13] / 46, X, e (X) (p22.1q22.1)). Nenhum caso de doença cardíaca congênita foi encontrado em qualquer um desses fetos. Houve dois casos em que a avaliação pós-natal revelou a malformação: um caso de hipospádia e 1 caso de fenda palatina. CONCLUSãO: A presença de ARSA isolado é benigna e não está associada a anormalidades cromossômicas. O achado de ARSA, no entanto, justifica uma ultrassonografia fetal detalhada para excluir anormalidades fetais importantes e outros marcadores leves.

Arteria lusoria is a rare cause of dysphagia in which dysphagia due to esophageal compression. The upper GI endoscopy does not bring significant element that can orient the diagnosis. The injected thoracic CT scan remains the key examination for the diagnosis of dysphagia lusoria and to characterize the defective artery., Arteria lusoria is a rare cause of dysphagia, in which dysphagia due to esophageal compression. The upper GI endoscopy does not bring significant element that can orient the diagnosis. The injected thoracic CT scan remains the key examination for the diagnosis of dysphagia lusoria and to characterize the defective artery.

An aberrant right subclavian artery (ARSA) is a rare arterial anomaly. Although a few cases of total endovascular repair for the ARSA aneurysm have been previously reported, anatomical limitations and the possibility of endoleaks remained. In this case, we created 4 holes on the stent graft for each cervical branch, with reference to the preoperative computed tomography findings. This approach might enable us to repair all types of thoracic aortic aneurysms with ARSA with each anatomical feature.

We report an emergent complex hybrid repair of a type A intramural hematoma with a tear of the aortic arch at the site of Kommerell's diverticulum and an aberrant right subclavian artery. We identified a type IA endoleak intraoperatively, which was managed immediately with proximal extension. Performing this operation in the hybrid operating room facilitated optimal surgical management.

Se presenta una paciente femenina de 54 años con hallazgo incidental de arteria subclavia derecha aberrante, también conocida en la literatura como arteria lusoria. Se puede considerar a la arteria lusoria como la anomalía embriológica del arco aórtico más común; descrita desde 1735 por Hunauld. La incidencia se estima entre 0.5-2% en la población general.1 La anatomía se describe como 4 arterias originandose del cayado aórtico: arteria carótida común derecha, carótida común izquierda, subclavia izquierda y subclavia derecha (la cual cruza la línea media en su recorrido). La prevalencia de esta anomalía varía según la región geográfica, en Estados Unidos se reporta una prevalencia de 0.5%.1 La falta de protocolos estandarizados de tratamiento hacen que el mismo sea basado en criterio médico, la sintomatología del paciente y su capacidad para ser sometido a procedimientos invasivos. La paciente descrita no presentaba sintomatología por lo que no recibió tratamiento.

Background An anomalous origin of the right vertebral artery (VA) from the right internal carotid artery (ICA) occurs only rarely. Case Description A 70-year-old man underwent carotid artery stenting for right internal carotid artery stenosis. In this patient, the right VA arose from the right ICA associated with an aberrant right subclavian artery. Conclusion Embryologically, failure of involution of 1 of the first 6 intersegmental arteries causes various abnormal origins of the VA. The embryonic development of this anomaly is also reviewed.

Aberrant right subclavian artery (ARSA) with associated Kommerell diverticulum (KD) is a rare congenital aortic arch anomaly. Patients with KD have a high risk of rupture, dissection, compression of adjacent structures, as well as distal embolization symptoms. Several treatment options have been proposed (surgical, hybrid, endovascular), however, a consensus regarding optimal surgical management has not been established yet. We present a successful single-stage hybrid repair of distal aortic arch dissection aneurysm with dissecting KD and ARSA with debranching of innominate and left common carotid artery, bilateral carotid-subclavian bypass, and stent grafting.

Purpose of review To address the incidental versus pathogenic nature of dysphagia lusoria and to provide a review of the cause, clinical presentation, diagnosis, and treatment of this condition with respect to recent literature. Recent findings Case reports comprise the majority of recent literature concerning dysphagia lusoria. Many patients with an aberrant right subclavian artery (ARSA) have additional coexisting vascular anomalies. While most individuals present around age 50, some present as children and neonates. Of note, this population may present with dysphagia as opposed to respiratory findings alone, as previously described. In the diagnostic workup, most patients receive a battery of radiologic tests, which may not be necessary. Significantly, dietary modifications and medical management alone may resolve symptoms. Nonetheless, a wide range of operative techniques are available for the treatment of dysphagia lusoria. Summary Clinicians should have a higher suspicion for dysphagia lusoria in patients with known vascular, heart, or chromosomal anomalies. Diagnosis should begin with a barium esophagram followed by a computed tomography angiogram or magnetic resonance angiogram. Avoid unnecessary studies. In many cases, an ARSA may be an incidental finding with comorbid gastroesophageal reflux disease or another medical condition responsible for the symptoms. Medical versus surgical management should be considered on a case-by-case basis.

BACKGROUND We report a rare case of numbness in the right hand, finally diagnosed as bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome and explain the cause of these diseases. CASE SUMMARY The patient was a 65-year-old woman. She complained of dizziness, numbness and weakness of the right hand for 6 mo. She was diagnosed with bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome by ultrasound, enhanced computed tomography, computed tomography angiography and other examinations. Considering the surgical risks, the patient refused the aberrant right subclavian artery stent implantation and was discharged. We hypothesize that these two kinds of deformity and right subclavian steal syndrome may not occur by accident and result from multiple malformations. CONCLUSION Bilateral common carotid artery common trunk with aberrant right subclavian artery combined with right subclavian steal syndrome is rare. This case reminds interventional radiologists of the possibility of these abnormalities before surgery.

p.p1 {margin: 0.0px 0.0px 2.9px 0.0px; text-align: justify; font: 9.0px 'Helvetica Neue Light'} Sag aberrant subclavian arter (ARSA), nadir gorulen ve genellikle asemptomatik seyreden konjenital bir anomalidir (populasyonun %0.5-1.8’i). Bu calismada supraklavikular insizyon ile basarili bir sekilde tedavi edilen uc kiz hastanin sunulmasi amaclandi. Ameliyat sirasinda ortanca yas 15’di (aralik: 10-18 yas). Ameliyat sirasinda ve sonrasinda komplikasyon izlenmedi. Bilateral ulnar ve radyal pulsasyonlari ameliyat sonrasi normaldi. Ameliyat sonrasinda beslenme ile ilgili problem gozlenmedi. Supraklavikuler insizyon ile ARSA’ nin uc yan anastomozu, disfaji lusoriasi olan cocuklar icin uygulanabilir ve minimal invaziv bir yontem olarak dusunulmelidir.

内頚動脈低形成と異所性右鎖骨下動脈に合併した未破裂内頚動脈瘤に対し，コイル塞栓術を施行した症例を経験したので報告する．症例は56歳女性．めまいの精査目的に他院で施行したmagnetic resonance (MR) imagingで左内頚動脈瘤を指摘されたため当院へ紹介となった．初診時，神経学的な異常は認めなかった．脳血管撮影とCT angiographyで右内頚動脈低形成と異所性右鎖骨下動脈を認めた．左内頚動脈瘤の大きさは7.5×5.8 mm，neckは3.6 mmであり，ブレブを伴っていた．破裂予防目的に左内頚動脈瘤に対してバルーン併用下コイル塞栓術を施行した．術中，左内頚動脈の血流遮断を数回要した．左内頚動脈瘤は完全に塞栓され，周術期に有害事象は認めなかった．内頚動脈低形成と異所性右鎖骨下動脈の合併は稀であり，これらの破格を有する症例では血管内手術を行う際，アクセスルートの制限や脳虚血耐性に注意が必要である．

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

DiGeorge’s syndrome is a 22q11.2 deletion leading to abnormal embryogenesis of pharyngeal arches and it is manifesting in a variety of clinical signs and symptoms. The spectrum of anomalies varies from minor facial dysmorphism and cleft palate to a broad spectrum of cardiovascular anomalies, thymic disfunction and immune deficiencies, hypocalcemia due tohypoparathyroidism,growth and developmental delay and speech disturbances. Cardiovascular anomalies might include right sided aortic arch, aberrant vesiclesand vascular ring. Here we present an atypical case of partial DiGeorge’s syndrome with feeding and swallowing difficulties and laryngeal stridor in the neonatal period. Early presentation in this period is usually due to severe hypocalcemia and cardiac disease. Feeding difficulties in a preterm baby needed clinical assessment skills in order to establish the diagnosis and delineate it from feeding difficulties usually seen in preterm babies. Esophagogram (barium X Ray) showed antero-posterior oblique impression towards the right side, the latero- lateral view showed impression on the rare side, suspected to be esophageal sub stenosis due to vascular anomaly, aberrant right subclavian arteryand suspectedthymic hypoplasia. We report a 9-year follow up periodbya team of subspecialists. The child had two surgeries due to aberrant vessel and velopharyngeal deficiency. Optimal management of patients with DiGeorge’s syndrome requires a multidisciplinary teamwhichshould include a cardiologist, immunologist, geneticist, speech/language therapist, endocrinologist and other subspecialists depending on patient`'s phenotype., DiGeorge Синдромот е резултат на делеција на 22q11.2 која води до пореметена ембриогенеза на фарингеалните лаци и се манифестира со различни знаци и симптоми. Спектарот на аномалии во овој синдром е варијабилен и може да се презентира само со минорна лицева дизморфија и расцеп на непцето но и со широк спектар на кардиоваскуларни аномалии, дисфункција на тимус и имун дефицит, хипокалцемија поради хипопаратироидизам, пореметување во растот и развојот и проблеми во говорот. Кардиоваскуларните аномалии може да се од типот на десен аортен лак, аберантни крвни садови и васкуларен ринг. Во овој приказ на случај прикажуваме атипичен случај на парцијален синдром на DiGeorge кој се презентираше со потешкотии во голтање и ларингеален стридор во неонаталниот период. Раната презентација на овој синдром во неонаталната возраст најчесто се дијагностицира со хипокалцемија и конгенитална кардиопатија. Потребна е добра клиничка проценка за да се разликува проблем при хранење на прематурно новородено од другите проблеми при голтање. Езофагограмот покажа коса импресија на антеро-постериорниот правец со ориентација кон десно суспектна за субстеноза на езофагусот на опишаното ниво од васкуларна етиологија, аберантна десна артерија субклавија и суспектна хипоплазија на тимус. Пациентот беше следен во период од 9 години од тим на субспецијалисти. Во тој период имаше две хируршки интервенции (корекција на аберантен крвен сад и велофарингеален дефицит). Пациентите со синдром на DiGeorge треба да бидат следени од мултидисциплинарен тим кој вклучува кардиолог, имунолог, генетичар, ендокринолог, логопед и други субспецијалисти според фенотипот на пациентот.

Managing type B aortic dissection (TBAD) involving Kommerell’s diverticulum (KD), aberrant right subclavian artery (ARSA), and isolated left vertebral artery (ILVA), is extremely challenging. As treatment, we described a one-stage hybrid technique combined with thoracic endovascular aortic repair (TEVAR) with open surgery through a supraclavicular incision. A 57-year-old man with TBAD and the three anomalies successfully underwent hybrid TEVAR. A side-to-side artificial bypass between the ARSA and the right common carotid artery was established through a right supraclavicular incision before TEVAR. The release of the stent-graft was designed from the distal aortic ostium to the left common carotid artery (LCCA) to cover the ILVA, ARSA, and left subclavian artery (LSA). Then, the ILVA and LSA were transposed to the LCCA through a left supraclavicular incision. Intraoperative angiography confirmed complete false lumen exclusion and KD, with all branches patent and without endoleaks. Computed tomography angiography 7 days and 1 year postoperatively demonstrated well-perfused ARSA, LSA, and ILVA, and a fully expanded stent-graft with no endoleaks, migration, disconnection, or stenosis. TBAD involving KD, ARSA, and ILVA in one case is rare. This is the first report to treat this pathology with a one-stage supraclavicular hybrid procedure.

A 1-year-old female Akita dog was referred for intermittent regurgitation. Computed tomographic angiography (CTA) showed an aberrant right subclavian artery (ARSA), resulting in constriction of the esophagus. After surgical ligation of the ARSA, CTA showed that the ARSA was not enhanced by contrast medium, and that sufficient collateral circulation of the right forelimb was supplied through the vertebral artery. Furthermore, the right and left vertebral arteries merged into the basilar artery at the level of the atlas, and no abnormal expansion of the ventral spinal artery was observed. Overall, we demonstrated the importance of post-surgical CTA for identification of surgical complications, including the formation of abnormal vessel alterations.

Objectives We present a case in which a 44-year-old female smoker with a complex medical and surgical history presented with acute upper limb ischemia. Methods The patient was diagnosed with thoracic outlet syndrome causing symptomatic thrombosis of an aberrant right subclavian artery associated with diverticulum of Kommerell. The patient had history of extensive surgeries and radiation in the right chest with fibrotic changes, so surgical dissection in the area was associated with significant risk of nerve injury. Therefore, revascularization of the arm was performed via carotid to brachial artery bypass with reversed saphenous vein and the thoracic outlet was decompressed with segmental clavicular resection. Results Patient has been symptom free for 3 years with patent bypass. Conclusions This case demonstrates a unique presentation of distal thrombosis of a symptomatic aberrant right subclavian artery related to thoracic outlet syndrome. The combined claviculectomy–vein bypass is a safe and efficacious surgical approach that provides durable relief.

Aberrant right subclavian is a rare anomaly presenting in 0.3–3.0% of the population. Kommerell’s diverticulum is an aneurysm associated with this phenomen; data relating to its incidence is sparse. Currently there are no well-established guidelines for the treatment of Kommerell’s diverticulum, limitation being the rare occurrence of disease and heterogenous population with disease presentation. This case report illustrates a novel approach to the repair of a symptomatic Kommerrel’s diverticulum with severe coronary disease. Management should be tailored on a case by case basis to the individual patient.

Purpose: Aberrant right subclavian artery (ARSA) associated with Kommerell’s diverticulum (KD) is a common congenital arch anomaly. It can be complicated by type B aortic dissection (TBAD) or aneurysmal formation at its ostium. Recently, hybrid repair with thoracic endovascular aortic repair (TEVAR) has appeared to be more favorable. Due to the normal anatomic proximity of the ARSA to the left subclavian artery (LSA) orifice in KD, coverage of the bilateral subclavian arteries (SCAs) to obtain an adequate proximal landing zone (PLZ) is usually required, and double cervicotomy for SCA revascularization potentially increases the risk of complications. Technique: This technique was demonstrated on a 50-year-old man presenting with progressive aneurysmal formation of KD with ARSA after chronic TBAD. A 3-step technique, namely left cervical debranching with a left common carotid artery to LSA bypass graft, TEVAR, and an LSA-to-ARSA endovascular debranching with a self-expanding covered stent by a through-and-through wire from the right brachial artery to the bypass graft, was performed in a 1-stage repair to cover the primary tear of TBAD and preserve the bilateral SCAs. The postoperative course was uneventful. Conclusion: This technique can prevent complications from double cervicotomy and achieve an adequate PLZ with preservation of the bilateral SCAs for TEVAR.

Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortic arch. The presence of ARSA has been previously associated with an increased risk of Down syndrome. ARSA at birth may be associated with dysphagia, respiratory distress and stridor and there is no clear evidence‐based management. The aim of this study was to describe the associations with chromosomal abnormalities and the postnatal outcome of fetuses diagnosed with ARSA. We analysed fetuses diagnosed antenatally with ARSA between January 2013 and September 2019 in the fetal echocardiography unit of the Hospital Monaldi, University ‘Vanvitelli’ of Naples, Italy. The results showed fifty fetuses diagnosed with ARSA, all confirmed after birth. The ARSA was an isolated finding in 46 fetuses (92%), while in 4 fetuses the ARSA was associated with other cardiac and/or extra-cardiac anomalies. Only one fetus was diagnosed with trisomy 21 (2%). In this fetus the ARSA was the only ultrasound anomaly identified. There were no cases necessitating referral due to the presence of compression symptoms at birth. The presence of ARSA was associated with trisomy 21 in the 2% of cases in our series and there were no neonatal complications due to airway compression at birth.IMPACT STATEMENTWhat is already known on this subject? Aberrant right subclavian artery (ARSA) is the most common embryologic abnormality of the aortich arch. ARSA at birth could be associated with dysphagia, respiratory distress and stridor and no evidence-based management of these fetuses has been described yet. The presence of ARSA has been previously associated with an increased risk of Down syndrome. What do the results of this study add? This study confirms known data on association with chromosomal defects and provides some original data on the absence of symptomatology due to tracheal compression with a postnatal follow-up up to three years of age. What are the implications of these findings for clinical practice and/or further research? Our findings suggest that in cases with adequate prenatal assessment performed by experienced clinicians, delivery can safely take place at local hospitals, with no need of referral soon after birth. The use of transthoracic echocardiography to confirm the diagnoses of ARSA after birth and to plan the next follow-up appointments can be supported.

Abnormal right subclavian artery (ARSA) is the most common anomaly of the aortic arch with a rate of 0.5-1.4%. Normally, three vessels arises from the aortic arch, while four vessels arise in ARSA. ARSA leaves the distal of the aortic arch and passes behind the esophagus and trachea. It is also called the abnormal retroesophageal right subclavian artery. It is generally an asymptomatic benign finding, but it can cause esophageal compression causing dysphagia. In this article, an isolated ARSA and trisomy 21 case referred to our clinic in the second trimester of pregnancy and detected ultrasonographically is presented.

Koolen de Vries syndrome is a rare genetic disorder with an estimated prevalence 1:16000 in the general population but it is considered to be an underdiagnosed syndrome. We report a 17q21.31 microdeletion which was diagnosed prenatally in a fetus with aberrant right subclavian artery (ARSA). Koolen de Vries syndrome is a rare chromosomal abnormality and according to the literature this is the first case in which the detection of ARSA led to this syndrome.

La arteria subclavia derecha aberrante (ARSA) es la anomalía del arco aórtico más común. La probabilidad de hallar un ARSA durante un estudio ecográfico es de alrededor de 1%. En aquellos fetos con ARSA, la probabilidad de tener otros hallazgos cardiacos y/o extracardiacos o una anomalía cromosómica es elevada. La prevalencia de la relación del ARSA con el síndrome de Down es de aproximadamente 20%, por lo que este marcador puede contribuir al asesoramiento del síndrome de Down en el segundo trimestre y probablemente en el primer trimestre. La recomendación ante el hallazgo de ARSA es realizar un estudio detallado de la anatomía fetal en busca de otros marcadores de aneuploidías y realizar ecocardiografía fetal. La realización de estudios invasivos quedará limitado a aquellas situaciones en donde además del ARSA se encuentren otros marcadores u otras condiciones que aumenten el riesgo de síndrome de Down. Sin embargo, ante el hallazgo de ARSA aislado, el incremento en el riesgo es igual a cero, pudiendo considerarse una variante de la normalidad.