Short-term outcome after the prenatal diagnosis of right aortic arch.

OBJECTIVES: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

Right cervical aortic arch associated with anomaly of epiaortic vessels and haemodynamically significant obstruction between ascending and descending aorta. Extra-anatomic bypass graft between ascending and descending aorta and long-term follow-up.

Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop, descends, and crosses behind the oesophagus to continue as a left descending aorta. We present a case of right cervical aortic arch associated with anomaly of epiaortic vessels and aortic obstruction, surgically treated with extra-anatomic bypass graft between ascending and descending aorta.

Left aortic arch with right descending aorta and severe coarctation: an unusual "vascular clamp" with airway compression.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

Surgical Relief of Left Ventricular Outflow Tract Obstruction in Adults With Congenital Aortic Stenosis and Associated Aortic Annulus Hypoplasia and/or Subaortic Obstruction.

BACKGROUND: In children and adolescents, a Ross/Konno operation is commonly done to both enlarge the aortic root and provide a competent aortic valve with relief of left ventricular outflow tract obstruction (LVOTO). Optimum management is not so straightforward in adults. METHODS: Between 1995 and 2014, 16 patients of mean age 39.4 years (18-57 years) with hypoplastic aortic annulus (AA) measuring 20mm and less, and mean aortic valve/LVOT gradient of 61mmHg (30-70mmHg) presented for surgery. RESULTS: Eight patients with mean LVOT/AA diameter 19.6mm (18-20mm) underwent an "inclusion-cylinder" type Ross procedure (RP). Eight patients with more severe LVOT/AA obstruction, with mean diameter of 17.4mm (16-19mm) underwent mechanical aortic valve replacement (AVR) with standard Konno-type aortoventriculoplasty. There was zero early and late mortality; with mean follow-up of 11.6 years (3-21 years) in the Ross group and 6 years (2-10 years) in the Konno-AVR group. One patient in the Konno-AVR group had reoperation after 2 years for RVOT obstruction. The postoperative echocardiograms of these patients at last follow-up show residual mean gradient across LVOT/AA of 4.4mmHg (2-6mmHg) after RP, and 11.9mmHg (8-17mmHg) after Konno-AVR. CONCLUSIONS: In adults, the "inclusion-cylinder" Ross-procedure is a good alternative for mild to moderate aortic root hypoplasia. However, for cases with severe LVOT obstruction, a Ross-Konno is not possible with the same method of autologous support used in a non-Konno RP, and this could be expected to have an impact on late durability and the need for further intervention, in a group that has already undergone multiple procedures in childhood. Both methods of RP and Konno-AVR lead to excellent early and late results.

Aortic Arch Advancement and Ascending Sliding Arch Aortoplasty for Repair of Complex Primary and Recurrent Aortic Arch Obstruction.

The optimal treatment of patients with complex coarctation of the aorta and hypoplastic aortic arch is controversial. Children undergoing repair of obstructive arch lesions are at lifelong risk of recurrence. Multiple techniques have been described to address complex primary and recurrent coarctation. Our approach at Texas Children's Hospital (Houston, TX) has been to address these lesions in an anatomic fashion via median sternotomy, under cardiopulmonary bypass using the techniques of aortic arch advancement and ascending sliding arch aortoplasty. Anatomic repair of complex primary or recurrent aortic obstruction is safe and produces a favorable repair with a low rate of recurrence. Both operations restore normal arch contour using native aortic tissue. The decision of which particular surgical strategy to use depends on the patient's age, anatomy, and the elasticity of the tissue.

Single-Stage Correction for Taussig-Bing Anomaly Associated With Aortic Arch Obstruction.

Taussig-Bing anomaly and aortic arch obstruction are two types of complex congenital cardiac malformations. Almost 50% of patients with Taussig-Bing anomaly have aortic arch obstruction. This report assesses the surgical outcomes of single-stage correction in neonates with both defects. Between November 2006 and November 2015, 39 neonates with Taussig-Bing anomaly and aortic arch obstruction (28 patients with coarctation of the aorta and 11 patients with interrupted aortic arch) underwent a one-stage arterial switch operation and aortic reconstruction. There were three in-hospital deaths and one late death (8 months after the surgery). The short-term survival rate was 92.3% (36/39), and the mid-term survival rate was 89.7% (35/39). Follow-up data were available for all patients who survived the operation (range 6-92 months). Echocardiology showed six cases of recoarctation, three cases of left ventricular outflow tract obstruction, three cases of right ventricular outflow tract obstruction, four cases of pulmonary artery stenosis, five cases of aortic regurgitation, and eight cases of pulmonary regurgitation. Eight patients required a reoperation during the follow-up period with no mortality. All survivors remained in good condition (New York Heart association functional class I or II). Single-stage correction of Taussig-Bing anomaly with aortic arch obstruction in neonates had favorable short- and mid-term outcomes in terms of mobility and reoperation rate. The optimal operative procedure should be chosen according to the position of the coronary arteries and the type of aortic anomaly.

Comparison of Total Arch and Partial Arch Transposition During Hybrid Endovascular Repair for Aortic Arch Disease.

OBJECTIVE: Total arch transposition (TAT) during hybrid endovascular repair for aortic arch disease is believed to allow a better landing zone, but also to be associated with higher peri-operative mortality than partial arch transposition (PAT). Information on this issue is limited. METHOD: This study was a retrospective analysis. All 53 consecutive patients with aortic arch disease (41 males, mean age 65.0 years) who underwent hybrid endovascular repair with TAT (zone 0, n=20) or PAT (zone 1 or 2, n=33) from 2008 to 2014 were analyzed retrospectively. The peri-operative and late outcomes of these two groups were compared. RESULTS: Baseline characteristics, including EuroSCORE II results, were similar in the two groups. After procedures, peri-operative mortalities and stroke rates were similar in the two groups (5.0% vs. 9.1%, p=1.000, and 10.0% vs. 6.1%, p=.627). Interestingly, all four strokes occurred in patients with a type III aortic arch irrespective of transposition type. Primary success rates (80.0% vs. 69.7%, p=.527) and type I endoleak incidences (20.0% vs. 27.3%, p=.744) were not significantly different. During follow up (mean duration 36.9 months), overall survival (89.7% vs. 87.4% at 1 year and 89.7% vs. 79.3% at 3 years; p=.375) and re-intervention free survival rates (78.6% vs. 92.0% at 1 year; 72.0% vs. 62.2% at 3 years, p=.872) were similar in the two groups. CONCLUSION: Morbidity and mortality were high within the first year of hybrid endovascular therapy for aortic arch disease, implying that candidates for hybrid procedures need to be selected carefully. Hybrid endovascular repair with TAT was found to have peri-operative mortality, stroke, and long-term survival rates comparable with PAT, so hybrid endovascular repair may be considered, irrespective of type of arch reconstruction, when clinically indicated.

External carotid compression: a novel technique to improve cerebral perfusion during selective antegrade cerebral perfusion for aortic arch surgery.

PURPOSE: Selective antegrade cerebral perfusion (SACP) involving cannulation of either the axillary or innominate artery is a commonly used technique for maintaining cerebral blood flow (CBF) during the use of hypothermic cardiac arrest (HCA) for operations on the aortic arch. Nevertheless, asymmetrical CBF with hypoperfusion of the left cerebral hemisphere is a common occurrence during SACP. The purpose of this report is to describe an adjunctive maneuver to improve left hemispheric CBF during SACP by applying extrinsic compression to the left carotid artery. CLINICAL FEATURES: A 77-yr-old male patient with a history of aortic valve replacement presented for emergent surgical repair of an acute type A aortic dissection of a previously known ascending aortic aneurysm. His intraoperative course included cannulation of the right axillary artery, which was used as the aortic inflow during cardiopulmonary bypass and also allowed for subsequent SACP during HCA. After the onset of HCA, the innominate artery was clamped at its origin to allow for SACP. Shortly thereafter, however, the left-sided cerebral oxygen saturation (SrO2) began to decrease. Augmenting the PaO2, PaCO2 and both SACP pressure and flow failed to increase left hemispheric SrO2. Following the use of ultrasound guidance to confirm the absence of atherosclerotic disease in the carotid artery, external pressure was applied partially compressing the artery. With the carotid compression, the left cerebral saturation abruptly increased, suggesting pressurization of the left cerebral hemispheric circulation and augmentation of CBF. CONCLUSIONS: Direct ultrasound visualization and cautious partial compression of the left carotid artery may address asymmetrical CBF that occurs with SACP during HCA for aortic arch surgery. This strategy may lead to improved symmetry of CBF and corresponding cerebral oximetry measurements during aortic arch surgery.

RéSUMé: OBJECTIF: La perfusion cérébrale antérograde sélective réalisée via la canulation de l'artère axillaire ou du tronc artériel brachiocéphalique est une technique répandue pour maintenir la circulation sanguine cérébrale pendant un arrêt circulatoire hypothermique pour les opérations de la crosse aortique. Toutefois, on observe fréquemment une circulation sanguine cérébrale asymétrique accompagnée d'une hypoperfusion de l'hémisphère cérébrale gauche pendant la perfusion cérébrale antérograde sélective. L'objectif de ce compte rendu est de décrire une manœuvre d'appoint destinée à améliorer la circulation sanguine cérébrale pendant la perfusion cérébrale antérograde sélective en appliquant une compression extrinsèque à l'artère carotide gauche. ÉLéMENTS CLINIQUES: Un homme de 77 ans avec des antécédents de remplacement valvulaire aortique s'est présenté pour une réparation chirurgicale urgente d'une dissection aortique aiguë de type A d'un anévrisme de l'aorte ascendante connu. Son parcours peropératoire a inclus la canulation de l'artère axillaire droite, qui a été utilisée comme canule artérielle pendant la circulation extracorporelle et a également permis la perfusion cérébrale antérograde sélective subséquente pendant l'arrêt circulatoire hypothermique. Après l'amorce de l'arrêt circulatoire hypothermique, le tronc artériel brachiocéphalique a été clampé à sa base afin de permettre la perfusion cérébrale antérograde sélective. Peu après toutefois, la saturation en oxygène cérébral (SrO2) gauche a commencé à baisser. L'augmentation de la PaO2, de la PaCO2 et de la pression et du débit de perfusion cérébrale antérograde sélective n'a pas réussi à augmenter la SrO2 dans l'hémisphère gauche. Après avoir utilisé l'échographie pour confirmer l'absence d'athérosclérose dans l'artère carotide, une pression externe a été appliquée en comprimant partiellement l'artère. En comprimant la carotide, la saturation cérébrale gauche a subitement augmenté, suggérant une pressurisation de la circulation hémisphérique cérébrale gauche et une augmentation de la circulation sanguine cérébrale. CONCLUSION: Une visualisation directe par échographie et une compression partielle prudente de l'artère carotide gauche pourraient régler le problème de circulation sanguine cérébrale asymétrique qui survient en cas de perfusion cérébrale antérograde sélective pendant l'arrêt circulatoire hypothermique pour la chirurgie de la crosse aortique. Cette stratégie pourrait améliorer la symétrie de la circulation sanguine cérébrale et les mesures de l'oxymétrie cérébrale correspondantes pendant les chirurgies de la crosse aortique.

Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring.

Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies.

Transposition Complex with Aortic Arch Obstruction: Outcomes of One-Stage Repair Over 10 Years.

The surgical management of transposition complex with aortic arch obstruction remains technically demanding due to anatomic complexity. Even in the recent surgical era, there are centers that address this anomaly with a staged strategy. This report presents our experiences with a one-stage repair of transposition complexes with aortic arch obstructions more than the last 10 years. Since 2003, 19 patients with a transposition of the great arteries (TGA, 2 patients) or a double outlet of the right ventricle (DORV, 17 patients) and aortic arch obstruction have undergone one-stage repair of their anomalies. The mean age was 6.7 ± 2.3 days, and the mean body weight was 3.4 ± 0.3 kg. The 2 patients with TGA exhibited coarctation of the aorta. The 17 patients with DORV all exhibited the Taussig-Bing type. The great artery relationships were anteroposterior in 4 patients (21.1%). The coronary artery anatomies were usual (1LCx; 2R) in 8 patients (42.1%). There were 2 early deaths (10.5%). Seven patients (36.8%) required percutaneous interventions. One patient required re-operation for pulmonary valvar stenosis and left pulmonary artery patch angioplasty. The overall survival was 84.2%. The freedom from mortality was 83.5% at 5 years, and the freedom from intervention was 54.4% at 5 years. The one-stage repair of transposition complexes with aortic arch obstructions resulted in an acceptable survival rate and a relatively high incidence of postoperative catheter interventions. Postoperative catheter interventions are highly effective. Transposition complexes combined with aortic arch obstructions can be managed by one-stage repair with good early and midterm results.

Prenatal echocardiographic diagnosis of double aortic arch.

A double aortic arch is a congenital anomaly associated with the formation of a vascular ring, which can cause varying degrees of airway compression. This can be subclinical or clinical, manifesting as acute stridor, severe respiratory compromise or symptoms of chronic airway compression. An accurate diagnosis of double aortic arch can be made prenatally by fetal echocardiography. In this report, we describe two infants with normal karyotypes in whom a prenatal diagnosis of double aortic arch was made at our fetal cardiology center, between July 2012 and August 2013. The diagnosis was confirmed postnatally by two-dimensional echocardiography and cardiac magnetic resonance imaging (MRI). The presence of a right aortic arch should prompt more careful evaluation to exclude a double aortic arch. Postnatal assessment should comprise echocardiography and cross-sectional imaging in the form of cardiac MRI/computed tomography. Bronchoscopy may be considered to exclude subclinical airway compression and reduce potential long-term respiratory sequelae.

The Presence of a Right Aortic Arch Associated with Severe Stenosis of the Right Common Carotid Artery and Steal Phenomenon.

The aim of this article is to describe the clinical presentation and imaging findings of the right-sided aortic arch, an uncommon anatomical variant. We present a case of associated steal syndrome, to our knowledge the first such case in the literature. Diagnostic imaging of a 49-year-old woman with symptoms of cerebrovascular insufficiency revealed the presence of a right aortic arch, with an aberrant left subclavian artery (lusorian artery). This arrangement placed pressure on the right common carotid artery at the level of its departure with the steal phenomenon dependent on the respiratory phase.

Pulmonary artery rupture after bilateral pulmonary artery banding in a neonate with Loeys-Dietz syndrome and an interrupted aortic arch complex: report of a case.

Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation.

Crossed pulmonary arteries with hypoplasia of the transverse aortic arch.

BACKGROUND: The entity of crossed pulmonary arteries was first described by Jue, Lockman, and Edwards in 1966, in a patient with trisomy 18. Since then, several series have been described, both in terms of the isolated anatomic variant, or its association with other intracardiac or extracardiac anomalies. We describe a rare association that has previously not been reported. Methods and results Institutional Review Board approval for a retrospective chart review was obtained. Over the period 2011 through 2013, we have encountered six patients in whom the crossed origins of the pulmonary arteries from the pulmonary trunk were associated with hypoplasia of the transverse aortic arch, an association that, to the best of our knowledge, has previously not been reported. In all of the patients, the isthmic component of the aortic arch was inserted in an end-to-side manner into the ductal arch, with additional discrete coarctation in half of the patients. CONCLUSION: To the best of our knowledge, no cases of crossed pulmonary arteries have been described in association with hypoplasia of the transverse aortic arch. We draw comparisons between the cases with exclusively tubular hypoplasia, and those with the added problem of the more typical isthmic variant of aortic coarctation. In all cases, the ability to reconstruct cross-sectional images added significantly to the diagnosis and understanding of these complex lesions. These findings have specific surgical implications, which are discussed.

Minimizing the risk of preoperative brain injury in neonates with aortic arch obstruction.

OBJECTIVE: To determine whether prenatal diagnosis lowers the risk of preoperative brain injury by assessing differences in the incidence of preoperative brain injury across centers. STUDY DESIGN: From 2 prospective cohorts of newborns with complex congenital heart disease studied by preoperative cerebral magnetic resonance imaging, one cohort from the University Medical Center Utrecht (UMCU) and a combined cohort from the University of California San Francisco (UCSF) and University of British Columbia (UBC), patients with aortic arch obstruction were selected and their imaging and clinical course reviewed. RESULTS: Birth characteristics were comparable between UMCU (n = 33) and UCSF/UBC (n = 54). Patients had a hypoplastic aortic arch with either coarctation/interruption or hypoplastic left heart syndrome. In subjects with prenatal diagnosis, there was a significant difference in the prevalence of white matter injury (WMI) between centers (11 of 22 [50%] at UMCU vs 4 of 30 [13%] at UCSF/UBC; P < .01). Prenatal diagnosis was protective for WMI at UCSF/UBC (13% prenatal diagnoses vs 50% postnatal diagnoses; P < .01), but not at UMCU (50% vs 46%, respectively; P > .99). Differences in clinical practice between prenatally diagnosed subjects at UMCU vs UCSF/UBC included older age at surgery, less time spent in the intensive care unit, greater use of diuretics, less use of total parenteral nutrition (P < .01), and a greater incidence of infections (P = .01). In patients diagnosed postnatally, the prevalence of WMI was similar in the 2 centers (46% at UMCU vs 50% at UCSF/UBC; P > .99). Stroke prevalence was similar in the 2 centers regardless of prenatal diagnosis (prenatal diagnosis: 4.5% at Utrecht vs 6.7% at UCSF/UBC, P = .75; postnatal diagnosis: 9.1% vs 13%, respectively, P > .99). CONCLUSION: Prenatal diagnosis can be protective for WMI, but this protection may be dependent on specific clinical management practices that differ across centers.

Double aortic arch: postnatal obliteration of the left aortic arch. Is arterial duct closure responsible?

We present a case of double aortic arch with a predominant right and a double arterial duct detected by echocardiogram in a 28-week gestation foetus. The first evaluation revealed that both arches were perfused; the 1-month postnatal echocardiogram showed the closure of both arterial ducts and the partial obliteration of the left aortic arch between the left subclavian artery and the dorsal aorta. In our case, the postnatal obliteration of the left arch in a double aortic arch was probably due to the closure of the left-sided arterial duct.

Spontaneous resolution of postoperative chylothorax following surgery for persistent right aortic arch in two dogs.

Two young dogs underwent surgical management of a persistent right aortic arch (PRAA) and developed chylothorax postoperatively. In both cases, the surgical procedure and anesthetic recovery were uncomplicated and routine. Following surgery, both patients appeared bright, alert, responsive, and previous signs of regurgitation had resolved. Dyspnea and tachypnea developed 1-2 days postoperatively in each patient, and chylous effusion was detected on thoracocentesis. For each case, a diagnosis of chylothorax was based on cytology and triglyceride concentrations of the aspirated pleural fluid. Similar protocols for monitoring were used in the treatment of each patient's chylothorax. The duration and volume of chylous effusion production were closely monitored via routine thoracostomy tube aspiration. Both dogs rapidly progressed to recovery with no additional complications. With diligent monitoring, chylothorax secondary to surgical trauma can resolve in a rapid, uncomplicated manner.

Short- and long-term outcome of dogs following surgical correction of a persistent right aortic arch.

This study reports the survival to discharge, postdischarge survival, and long-term outcome of dogs following surgical correction of a persistent right aortic arch (PRAA). Information for 52 dogs, 28 dogs, and 23 dogs was available for analysis of survival to discharge, postdischarge survival, and long-term outcome, respectively. Ninety-two percent of dogs survived to the time of discharge and 18% of dogs surviving to discharge were euthanized within 2 mo of surgery. Breed, age at the time of presentation, and gender were not correlated with increased odds of death prior to discharge from the hospital. Long-term outcome for dogs was excellent in 30%, good in 57%, and poor in 13% of dogs. Although this study shows a relatively high mortality rate prior to discharge from the hospital and within 2 mo of surgical treatment, the long-term outcome is good or excellent in 87% of survivors. Dogs frequently display either residual clinical signs or require dietary modification long-term, but owner satisfaction is high.

Applicability and mid-term results of endovascular treatment for descending thoracic acute aortic syndromes.

BACKGROUND: Descending thoracic acute aortic syndromes (DTAASs) are life-threatening pathologies in which thoracic endovascular aortic repair (TEVAR) is an attractive therapeutic option. There are few data in the literature on the survival and morbidity rates of TEVAR in such indications. We provide the results of TEVAR from our single-center cohort of DTAASs: ruptured aneurysms (RAs), acute symptomatic dissections (ADs), and traumatic ruptures (TRs). METHODS: Between 2004 and 2011, data from all patients treated by TEVAR for DTAAS in our university center were collected prospectively. Primary end points were rates of 30-day mortality and morbidity. Secondary end points were mid-term outcomes and reinterventions. RESULTS: Forty-eight patients underwent TEVAR for DTAASs: 19 RAs (39.6%), 12 ADs (25.0%), and 17 TRs (35.4%). The cumulative 30-day mortality and morbidity rate was 33%. There were 10 deaths (6 RAs, 1 AD, and 3 TRs), 2 cases of paraplegia (0 postoperative and 2 preoperative cases (2 TRs), and no major reinterventions and 4 minor reinterventions (embolization for endoleak exclusion). Long-term outcomes were known in 33 patients (5 patients were lost to follow-up), with a mean duration follow-up of 27 months. The cumulative mortality and morbidity rate was 9%: no deaths were reported, and 2 major reinterventions (open aneurysm repair) and 1 minor reintervention (endoleak exclusion) were required. CONCLUSIONS: Our results confirm that TEVAR, as an emergency therapeutic option, is suited to DTAASs. The 21% perioperative mortality rate for such dramatic lesions remains high but is lower than medical or open repair therapeutic options previously reported. No additional deaths occurred during the 2-year follow-up period, and a low incidence of endograft-related reinterventions was observed. Nevertheless, scheduled follow-up visits with computed tomodensitometry scans on a regular basis are mandatory.

Intracranial aneurysm in childhood and interrupted aortic arch.

BACKGROUND: Intracranial aneurysms are very rare in children. Headache and nausea/vomiting are the most prominent clinical findings. The only effective treatment is obliteration of aneurysm by surgical or endovascular techniques. Interrupted aortic arch is also a rare, congenital cardiovascular malformation characterized by the lack of continuity between the ascending and descending thoracic aorta. Aortic interruption is an uncommon cause of intracranial aneurysm. The course of disease is lethal unless effective collateral flow develops. Long-term survival may be possible with surgical repair. METHOD: We report on a 17-year-old boy affected with interrupted aortic arch disease and associated multiple intracranial aneurysms. Both aneurysms clipped successfully. After patient had recovered, he referred to cardiovascular surgery for further treatment. CONCLUSION: Surgical or endovascular obliteration remains the main therapy for intracranial aneurysms. Accompanying systemic diseases such as interrupted aortic arch challenge the management of both diseases.