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To compare aortic sac changes after endovascular aneurysm repair (EVAR) assessed by three-dimensional ultrasound (3D-US), two-dimensional ultrasound (2D-US), and traditional computed tomographic angiography (CTA).Using volume assessment with three-dimensional CTA (3D-CTA-volume) as the gold standard, this study investigated aortic sac changes at three and 12 months after EVAR with three different ultrasound methods (2D-US anterior-posterior (AP) diameter, 3D-US AP centerline diameter, and 3D-US partial volume), and traditional CT multiplanar outer-to-outer diameter (CT-MPR OTO diameter). From august 1st, 2011 to January 2014, consecutive EVAR patients (n = 113) were available for analysis in two time intervals; 1) between preoperative and three-month follow-up and 2) between three and 12 month follow-up.The risk of missing true aortic sac growth (false negative finding) at three-month postoperative visit using 3D-US partial volume, 3D-US AP centerline diameter, 2D-US AP diameter, and CT-MPR OTO diameter was 19%, 21%, 22%, and 18%, respectively. Corresponding low sensitivities (0% to 21%) and kappa-values (0.50) in detecting aortic sac changes were found. The risk of missing true growth between three and 12 months were lower (6%, 5%, 6%, and 6%, respectively), and matching sensitivities 33%, 33%, 17%, and 17%, respectively.All tested methods for aortic sac changes were as good as traditional CT-MPR OTO diameter and corresponded poorly with 3D-CTA-volume at three months postoperative visit but substantially better after 12 months where the residual sac change was more profound.

Interruption of the ascending aorta is an extremely rare anomaly defined by a point of interruption between the intrapericardial and extrapericardial aorta and can be explained by developmental errors proximal to the embryologic right aortic sac. Herein, we present a case of interruption of the ascending aorta and describe a successful biventricular surgical repair of this unique anomaly.

Our patient had undergone a previous three-fenestration Anaconda (Terumo Medical Corp, Tokyo, Japan) fenestrated endovascular aneurysm repair (EVAR) to treat a juxtarenal aortic aneurysm. At 10 years postoperatively, distal migration of the prosthesis, a proximal type I endoleak, and aortic sac enlargement of 10 mm in 6 months was observed. Because of the short length of the Anaconda's bifurcated body, we chose to use a Zenith custom-made endograft with four branches and a bifurcated body with an inverted contralateral limb. We have also described the issues that can arise during branched EVAR after fenestrated EVAR and some of the bailout techniques we performed to successfully perform the treatment.

OBJECTIVE The most common endoleak after endovascular aneurysm repair is type II. Although type II endoleaks (TIIEL) are generally considered benign, there are reports that they can lead to aortic rupture. In this study, we reviewed the effect of TIIEL on sac size change to determine if sac expansion owing to a TIIEL could result in the development of a type IA endoleak (TIAEL). METHODS After internal review board approval, all aortoiliac endovascular aneurysm repairs performed at a single institution between June 2006 and June 2012 were retrospectively reviewed. Patient demographics, comorbidities, aneurysm diameter, graft type, need for reintervention, and complications were collected. Patients with TIIEL diagnosed on follow-up imaging were categorized as those who underwent intervention for their TIIEL and those who did not. Outcomes were tabulated with attention to sac size change, development of TIAEL, rupture, and survival. RESULTS Six hundred twenty-seven patients underwent aortoiliac stent graft placement at our institution during this time period. Patients with an operative indication other than nonruptured infrarenal abdominal aortic aneurysm and those without preoperative computed tomography angiography or follow-up data available for review were excluded. The total number of patients included was 389 with an average follow-up of 58.8 months (range, 0-194 months). Follow-up imaging diagnosed 124 patients with TIIEL (32%). Patients with TIIEL were significantly older (P < .0001) and more likely to be hypertensive (P < .05) but less likely to be smokers (P = .01). They had a significantly larger sac size increase than patients without TIIEL (9.50 vs -0.78 mm; P < .0001). Those with TIIEL were significantly more likely to develop a TIAEL than patients who did not have TIIEL (14% vs 5%; P = .004), but the rate of rupture was not significantly different (4% vs 2%; P = .33). In those with a TIIEL, the average sac size increase at which TIAEL developed was 13 mm. Patients in the TIIEL group who underwent intervention for their TIIEL survived significantly longer than patients who did not undergo intervention (140 months vs 100 months; P = .004). CONCLUSIONS Our data suggest that there is an increased incidence of late TIAEL in patients with TIIEL compared with those without a TIIEL. Our study also demonstrates an increased overall survival in TIIEL patients who underwent intervention. Future studies are necessary to better define the association between TIIEL with enlarging sac and the development of TIAEL. However, it is reasonable to conclude that intervention for TIIEL should be undertaken at or before a cumulative sac size increase of 13 mm.

Aortoenteric fistula after endovascular aortic repair for an abdominal aortic aneurysm is a rare but severe complication. Particularly, a case of inflammatory abdominal aortic aneurysm is extremely rare and there are only 3 reported cases. A 70-year-old man underwent endovascular aortic repair for impending rupture of an inflammatory abdominal aortic aneurysm and was medicated steroids for approximately 2 years. Four years after endovascular aortic repair, he developed endograft infection with an aortoduodenal fistula and a left psoas abscess. He underwent total endograft excision, debridement, in situ reconstruction of the aorta using prosthetic grafts with omental coverage, and digestive tract reconstruction to prevent leakage. Pseudomonas aeruginosa was detected in the infected aortic sac. The patient has not experienced recurrence of infection in the 35 months since his operation.

Aneurysm sac regression following endovascular repair (EVAR) of an abdominal aortic aneurysm (AAA) is an established indicator of surgical success. However, even with a completely excluded aneurysm, the degree of aortic sac regression may vary. This study evaluates the relationship between aneurysm sac regression after EVAR and the presence of morphological features in the thoracic aorta that can be associated with a subclinical aortic dissection, termed dissection morphology in this study.Patients who underwent EVAR to repair an infrarenal aortic aneurysm at Mount Sinai Hospital between 1996 and 2017 with a postoperative CT scan and a 3-year follow-up scan available for analysis were included in the study. Patients with a type I or type III endoleak were not included. The thoracic aorta was evaluated for dissection morphology on CT scan, which included the presence of aortic dissection, penetrating aortic ulcers, and intramural hematomas. AAA sac regression after EVAR was compared between patients with dissection morphology (n = 157) and patients without those characteristics (n = 141). An independent investigator performed the CT analysis and was blinded to the degree of sac regression.Demographics and comorbid clinical conditions were compared between patients with and without dissection morphology. There were no significant differences in age, gender, smoking habits, or cardiovascular conditions. The median AAA diameter after EVAR, over the course of the study, in patients with dissection morphology decreased by 11.30 mm (-17.20, -3.60) compared to a median change of 0.30 mm (-8.60, 8.60) in patients without dissection morphology features (p 0.001). Patients with dissection morphology also had fewer type II endoleaks in postoperative follow-up scans (22.9% vs. 53.9%, p 0.001). Additionally, patients with dissection morphology had longer EVAR operative times (192.00 min [167.25, 230.00] vs.174.00 min [150.00, 215.00], p = 0.004). AAA-related mortality after 3 years was not significantly different between the 2 groups (p = 1.0).The presence of imaging features consistent with dissection morphology in the thoracic aorta correlated with greater AAA sac regression and fewer type II endoleaks after EVAR. Assessing these imaging features in patients undergoing EVAR may be useful in understanding aneurysm behavior in terms of aneurysm growth, risk of rupture, and outcomes following endovascular surgery. Identifying differential rates of aneurysm sac regression may have implications regarding the role of subclinical dissections in the etiology of AAA development.

Aneurysm shrinkage is clinically observed after successful endovascular aortic aneurysm repair (EVAR). However, global understanding of post-operative aneurysm evolutions remains weak. In this work, we propose to study these effects using numerical simulation. We set up a 3D finite-element model of post-EVAR vascular adaptation within an open-source finite-element code, which was initially developed for growth and remodeling (G&R). We modeled the endograft with a set of uniaxial prestrained springs that apply radial forces on the inner surface of the artery. Constitutive equations, momentum balance equations, and equations related to the mechanobiology of the artery were formulated based on the homogenized constrained mixture theory. We performed a sensitivity analysis by varying different selected parameters, namely oversizing and compliance of the stent-graft, gain parameters related to collagen G&R, and the residual pressure in the aneurysm sac. This permitted us to evaluate how each factor influences post-EVAR vascular adaptation. It was found that oversizing, compliance or gain parameters have a limited influence compared to that of the residual pressure in the aneurysm sac, which was found to play a critical role in the stability of aneurysm after stent-graft implantation. An excessive residual pressure larger than 50 mmHg can induce a continuous expansion of the aneurysm while a moderate residual pressure below this critical threshold yields continuous shrinkage of the aneurysm. Moreover, it was found that elderly patients, with relatively lower amounts of remnant elastin in the arterial wall, are more sensitive to the effect of residual pressure. Therefore, these results show that elderly patients may present a higher potential risk of aortic sac expansion due to intra-aneurysm sac pressure after EVAR than younger patients.

Pre-emptive, nonselective perigraft embolization of abdominal aortic aneurysm sac to reduce the risk of type II endoleak has been previously reported with a percutaneous technique using contralateral access with resheathing for coiling. The approach has been modified to simplify the procedure and to eliminate unnecessary sheath exchanges. Keywords: Endovascular aneurysm repair, Endoleak, Embolization

We present a case of a 66-year-old woman who developed perigraft seroma after having undergone elective, open abdominal aortic aneurysm repair with a polytetrafluoroethylene graft 5 years previously. One year after graft implantation, she started to suffer from abdominal discomfort and was therefore offered surgical intervention, which she declined owing to the fear of postoperative complications. Instead, an observational strategy was used. Five years later, the patient presented with sudden severe abdominal pain. Subsequent laparotomy revealed that the pseudomembrane lining the perigraft seroma had penetrated through the native aortic wall and into the intraperitoneal cavity, where it had ruptured. The patient was successfully treated by replacing the polytetrafluoroethylene graft with a polyethylene terephthalate (Dacron) graft. Keywords: Perigraft seroma, Abdominal aortic aneurysm, Infection

Background Second heart field cells and neural crest cells have been reported to participate in the morphogenesis of the pharyngeal arch arteries (PAAs); however, how the PAAs grow out and are separated from the aortic sac into left and right sections is unknown. Results An Isl-1 positive pharyngeal mesenchyme protrusion in the aortic sac ventrally extends and fuses with the aortic sac wall to form a midsagittal septum that divides the aortic sac. The aortic sac division separates the left and right PAAs to form independent arteries. The midsagittal septum dividing the aortic sac has a different expression pattern from the aortic-pulmonary (AP) septum in which Isl-1 positive cells are absent. At 11 days post-conception (dpc) in a mouse embryo, the Isl-1 positive mesenchyme protrusion appears as a heart-shaped structure, in which subpopulations with Isl-1+ Tbx3+ and Isl-1+ Nkx2.5+ cells are included. Conclusions The aortic sac is a dynamic structure that is continuously divided during the migration from the pharyngeal mesenchyme to the pericardial cavity. The separation of the aortic sac is not complete until the AP septum divides the aortic sac into the ascending aorta and pulmonary trunk. Moreover, the midsagittal septum and the AP septum are distinct structures.

Objective Most type II endoleaks have a benign natural history, but 6% to 8% are associated with sac enlargement and respond poorly to treatment. Our aim was to evaluate whether these enlargements are associated with delayed or occult type I and III endoleaks. Methods Patients with interventions for endoleak after endovascular aortic repair from 2000 to 2016 were reviewed retrospectively. Patient demographics, comorbidities, endoleak type, secondary procedures, aortic sac growth (≥5 mm), and mortality were collected. Successful treatment was defined as endoleak resolution with no further aortic sac growth. Secondary procedures, ruptures, endograft explant, and death were captured. Results There were 130 patients diagnosed with a primary type II endoleak after endovascular aortic repair at a median of 1.3 months (interquartile range, 1.0-13.3 months). One hundred eighteen had their initial treatment for a primary type II. Twelve of the 130 were initially stable and observed, but were treated for a delayed type I or III endoleak. The 130 patients underwent 279 procedures for endoleaks (mean of 2.2 ± 1.3) over 6.9 ± 3.8 years of follow-up. Of the 118 patients treated for primary type II endoleaks, 26 (22.0%) later required interventions for delayed type I and III endoleaks. The mean time to intervention for a delayed type I or III endoleak was 5.4 ± 2.8 years. Overall, there were 16 type IA, 11 type IB, 2 type III, 7 combined type IA/IB, and 2 type IA/III delayed endoleaks. The odds of harboring a delayed type I or III endoleak was 22.0% before the first attempt at type II endoleak treatment, 35.1% before the second, 44.8% before the third, and 66.6% before the fourth attempts. Rapid aortic sac growth of ≥5 mm/y before initial endoleak treatment was associated with increased risk for delayed type I or III endoleak (47.8 vs 14.1%; P = .003). Patients with delayed type I or III endoleaks had a lower successful treatment rate (8.3% vs 52.3%; P = .001) than those with only type II endoleaks. Late rupture was increased with delayed type I or III endoleak (P = .002), whereas mortality (P = .96) and aortic-related mortality (P = .46) were similar. Graft explant (P = .06) trended toward an increase with a delayed type I or III endoleak, but was not statistically significant. Conclusions Failed attempts treating type II endoleaks and/or a rapid aortic sac growth of 5 mm/y or greater should raise the suspicion of a delayed or occult type I or III endoleak. Occult endoleaks are associated with decreased chance of endoleak resolution.

Purpose: Abdominal aortic aneurysm (AAA) growth after endovascular aneurysm repair (EVAR) is still unpredictable. The issue of optimal frequency of computed tomography angiography for surveillance and its measurement method accuracy remain unclear. We aimed to assess the value of abdominal aneurysm sac volume measurement for detecting expansions and the association of preprocedural intraluminal thrombus (ILT) volume with aneurysm sac growth following EVAR. Material and methods: A total of 107 patients underwent elective EVAR. Inclusion criteria provided a cohort of 39 patients. Changes of postoperative maximum aneurysm sac diameter and AAA volume were calculated. Volumetric AAA changes and demographic data of the cases with clinically irrelevant AAA diameter enlargement were evaluated. Preoperative ILT volumes were collected. ILT and AAA sac volume ratio was calculated. Statistical data analysis was performed using standard methods. Results: The mean changes of maximum AAA diameter and volume in percentage after EVAR were -5.08 ± 8.20 mm and -13.39 ± 23.32%, respectively. A moderate positive linear correlation between those changes was found (R2 = 0.731; p < 0.0001). The mean relative AAA volume increase in cases without clinically relevant diameter enlargement was 11.50 ± 8.27%. The means of ILT and AAA sac ratios were 0.59 ± 0.17 and 0.52 ± 1.8 in growing AAA sac and in stable or shrinking AAA sac groups, respectively (p = 0.308). Conclusions: Volumetric AAA measurement may be useful as an additional method to diameter measurement after EVAR to identify clinically relevant sac growth. Preoperative volume of ILT may not significantly affect the growth rate of AAA after EVAR.

It is customary, at the current time, to consider many, if not most, of the lesions involving the ventricular outflow tract in terms of conotruncal malformations. This reflects the introduction, in the early 1940s, of the terms conus and truncus to describe the components of the developing outflow tract. The definitive outflow tracts in the postnatal heart, however, possess three, rather than two, components. These are the intrapericardial arterial trunks, the arterial roots, and the subvalvar ventricular outflow tracts. Congenital lesions afflicting the arterial roots, however, are not currently considered to be conotruncal malformations. This suggests a lack of logic in the description of cardiac development and its use as a means of categorizing congenital malformations. It is our belief that the developing outflow tract, like the postnatal outflow tracts, can readily be described in tripartite fashion, with its distal, intermediate, and proximal components forming the primordiums of the postnatal parts. In this review, we present evidence obtained from developing mice and human hearts to substantiate this notion. We show that the outflow tract, initially with a common lumen, is divided into its aortic and pulmonary components by a combination of an aortopulmonary septum derived from the dorsal wall of the aortic sac and outflow tract cushions that spiral through its intermediate and proximal components. These embryonic septal structures, however, subsequently lose their septal functions as the outflow tracts develop their own discrete walls. We then compare the developmental findings with the anatomic arrangements seen postnatally in the normal human heart. We show how correlations with the embryologic findings permit logical analysis of the congenital lesions involving the outflow tracts.

The cardiovascular system is the first system in the body to reach a functional state, at around the 21st and 22nd day of gestation. As a result, any aortic arch anatomical variant results from the aberrant embryologic migration and/or differentiation of undifferentiated cardiac cell precursors. Initially, six paired aortic channels encircle the embryonic pharynx. They supply the developing pharyngeal arches and arise from the aortic sac. These channels run dorsally, embedded in the mesenchyme of the pharyngeal arches, and terminate in the right and left dorsal aortae.

In the accompanying article appearing in this issue of the Journal, Prabhu and his colleagues, from Bengalaru in India, describe their experience with patients having a right aortic arch. They discuss the fact that the anomalous arrangements they encountered can all be interpreted on the basis of the hypothetical double arch proposed by Edwards. They point to the fact that interpretation of the developmental changes underscoring the production of the double arch is currently confused by reference to the so-called Rathke diagram, in which six sets of arteries are shown extending through the mesenchyme of the pharyngeal arches. As the authors point out, Graham and his associates have now shown that the alleged fifth set of pharyngeal arches do not exist. Based on our own observations, we endorse this statement. It means that new explanations must now be provided for the lesions previously described on the basis of persistence of the alleged artery of the fifth pharyngeal arch. We have previously claimed to have observed such an artery in a human fetus. We now believe, on the basis of our latest findings, that our earlier observation is better explained on the basis of presence of a collateral channel. We suggest that the so-called “fifth arch arteries” are themselves then best explained either on the basis of existence of such collateral channels, or remodelling of the aortic sac, which is the manifold, during development, that gives rise to the pharyngeal arch arteries.

Background Aortic sac hygroma and concealed endoleaks (EL) after endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm needs particular attention with aggressive management as they are associated with rapid sac expansion and rupture risk. However, they can be erroneously reported as type IV or V EL with supine computed tomography (CT) scans, leading to delay in management. Therefore, we describe a novel diagnostic technique, ‘Prone contrASt enHanced computed tomography Angiography’ (PASHA), to document concealed EL Methods We present eight case descriptions with continuous sac expansion after primary EVAR. Management began with diagnosis using the PASHA imaging technique. PASHA is a multiphase CTA positional technique for increasing the accuracy of detecting EL after EVAR. Furthermore, the PASHA imaging technique also guides whether the open or endovascular intervention could be used effectively to manage the sac expansion. In synchrony with the PASHA technique, “EVAR GORE SalvAge FAbric Technique” (ARAFAT) was to salvage previous EVAR. Results The PASHA technique diagnosed all cases of type IIIb EL, as it enhanced the degree of contrast infiltration into the aortic sac when microleaks were present. ARAFAT was effectively used in five elderly patients. Another three had an open conversion; two with double breasting of the aortic sac and one EVAR explantation. Conclusions The PASHA protocol helped classify and localize the concealed EL (type IV, V), which were not appropriately diagnosed by supine CT protocols. PASHA and ARAFAT were used as a fully functioning protocol to overcome apparent challenges in accurate diagnosis and subsequent concealed EL management in high-risk patients.

Background In a normal neonatal heart the ductus arteriosus forms the direct continuity of the pulmonary trunk to the descending aorta being the main channel for the prenatal blood flow. The pulmonary arteries originate separately from the pulmonary trunk. In congenital heart malformations with pulmonary stenosis to atresia there is often an abnormal lateral DA to left pulmonary artery connection combined with a localised narrowing (pulmonary ductal coarctation / PDC) or even interruption. We investigated embryonic remodelling and pathogenesis of this area Material and methods Normal development was studied in WntCre reporter mice (E10.0-12.5) for neural crest cells and Nkx2.5 immunostaining for second heart field cells. Data were compared to stage matched human embryos including histopathology of neonatal specimen, and a VEGF120/120 mutant mouse strain developing pulmonary atresia. Results Normal mouse and human embryos showed that, in the early embryo, the mid-pharyngeal endothelial plexus, partly covered by second heart field, connected side-ways to the 6 th pharyngeal arch artery (PAA). The ventral segment of this PAA effectively formed the proximal pulmonary artery. The origin of the cells encasing the endothelial tubes differed significantly. The dorsal 6 th PAA segment (future DA on the left side) was solely surrounded by neural crest cells. The ventral segment had a dual outer lining with neural crest cells laterally and second heart field cells medially, while the distal pulmonary artery was covered by neither of these cells. The asymmetric contribution of second heart field to the future pulmonary trunk on the left side of the aortic sac (so-called pulmonary push) was evident. During remodelling the ventral segment of the 6 th PAA became incorporated into the pulmonary trunk leading to a separate connection of the left and right pulmonary arteries to the latter. The VEGF120/120 embryos showed a severely stunted pulmonary push and later on a variety of vascular anomalies in the DA to pulmonary artery connections. Conclusion Side-way connection of the DA to the left pulmonary artery is a congenital anomaly. The primary problem is a stunted development of the second heart field-derived pulmonary push leading to pulmonary stenosis/atresia and a subsequent lack of proper incorporation of the ventral segment of the 6 th PAA into the aortic sac. Clinically, the aberrant smooth muscle tissue of the DA should be addressed to prohibit development of severe PDC or even interruption of the left pulmonary artery.

Little evidence is available supporting the optimal treatment of type II endoleaks associated with aortic sac growth. Previous studies have lacked comparisons between treatment methods and long-term follow-up. The purpose of the present study was to review our center's experience with the treatment of type II endoleaks comparing Onyx (a liquid embolization agent consisting of ethylene vinyl alcohol; Medtronic, Minneapolis, Minn) embolization and coil embolization.A retrospective review of prospectively collected data from a vascular surgery database was performed to identify all patients who had undergone embolization of a type II endoleak for aortic sac growth after endovascular aneurysm repair from 2005 to 2018. The Onyx and coil embolization groups were compared using univariate statistics.A total of 58 patients had undergone 77 embolization procedures for type II endoleaks with either Onyx (27 patients; 37 procedures) or coils (31 patients; 40 procedures). The average aneurysm size at embolization was larger in the Onyx group (77.9 ± 15.1 mm) compared with coil embolization (73.4 ± 11.9 mm). The mean follow-up was 57 months for the Onyx group and 74 months for the coil embolization group. Of the 27 patients who had undergone Onyx embolization, 2 (7.4%) had required graft explantation compared with 5 of the 31 patients (16.1%) who had undergone coil embolization (P = .33). The results of the per-patient analysis showed that the coil embolization group had a significantly greater rate of the need for further reintervention compared with the Onyx group (55% vs 19%; P .01). Clinical success was observed in 13 patients (48%) in the Onyx embolization group compared with 10 patients (32%) in the coil embolization group (P = .04). Two patients in each group had presented with secondary rupture of the aneurysm sac after attempted embolization.Type II endoleaks associated with sac growth treated with Onyx were less likely to require further reinterventions than were those treated with coil embolization. A trend was found toward a greater need for endovascular aneurysm repair explant after coil embolization. With a high rate of further reintervention and potential for sac rupture, diligent follow-up is required after attempted type II embolization, regardless of the technique used.

The present study evaluates aneurysmal sac remodeling and the loss of the intercostal arteries after the first step of staged treatment of thoracoabdominal aortic aneurysms (TAAAs). The purpose of this approach is to keep the aneurysmal sac temporarily perfused to induce progressive thrombosis of the aneurysm while simultaneously allowing the spinal cord to establish adequate perfusion thereby promoting the development of collateral circulation.All patients with type II or type III TAAAs, having undergone 2-step endovascular treatment with at least a 2-branch endoprosthesis at our institution between April 2017 and May 2019, were retrospectively evaluated. Thirty-day mortality and spinal cord ischemia was assessed. The mean number of the intercostal and lumbar arteries, coverage length between the left subclavian artery and the stent graft proximal landing zone, total volume of the aneurysmal sac, lumen volume, and thrombosis volume were measured by preoperative and first-step postoperative computed tomography angiography. Patients were also grouped based on the chosen endoprosthesis (group A: double-branch aneurysmal sac reperfusion; group B: single-branch aneurysmal sac reperfusion).Eleven patients (mean age: 76.5 years; range: 61-86) were considered. No 30-day mortality was observed after the first-step procedure, and 1 patient died after second-step treatment. No permanent paraplegia was observed after either the first or second endovascular steps. The lumen volume significantly decreased (27%; P 0.001) after first-step endovascular treatment although there was a significant increase in aneurysm thrombosis (34% to 54%). The mean number of the intercostal arteries decreased from 19.7 to 9.3 (P 0.001) after first-step endovascular treatment. Volume variations and percentage of intercostal loss did not significantly differ between the 2 groups.Although aneurysm volume continued to increase after first-step treatment, two-step endovascular treatment is a feasible alternative to reduce the risk of severe ischemia in patients with extended TAAAs.

The muscular ductus arteriosus (DA) has many unique characteristics setting it apart from the adjoining elastic arteries. Preparation for neonatal closure takes place in utero with the development of intimal thickening. Ductus-specific gene and protein expression patterns were demonstrated during this process. We postulated that the closing process, with increased expression of progerin, might reflect aspects of premature ageing. Persistent patency of the DA, can be congenitally or immaturity based. During embryonic development the sixth pharyngeal arch arteries are the last to develop. In avian embryos this arch is divided into a proximal (part of the future pulmonary artery) and a distal part (on the left side the origin of the DA). The consequence is that the pulmonary arteries have a double vascular contribution being a proximal sixth arch artery component and a distal true pulmonary artery segment. We have conclusive evidence that this is not encountered in the human embryo. Both sixth arch arteries and the right and left pulmonary arteries connect separately and at distinct locations to the pulmonary trunk side of the aortic sac. The insertion of the wall of the DA between the proximal and distal part of the left pulmonary artery, referred to as pulmonary coarctation, constitutes a congenital malformation and not a remnant of what is encountered during normal development. This configuration is found almost exclusively in combination with pulmonary atresia leading to neonatal interruption of the proximal left pulmonary artery with consequences for surgical or intervention repair.

We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a "five-embryonic aortic arches" concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the "five-embryonic-aortic-arches" concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.

Background Pancreatitis rarely complicates aortic repair. The aim of the study was to examine the role of imaging in identifying this complication and to characterize pancreatitis occuring in this setting. Methods The radiology information system queried reports for terms pancreatitis, fluid collection, peripancreatic fluid, and aortic/aneurysm/endovascular/open repair from January 2010 through May 2018 and yielded 243 unique patients. Aortic repair and pancreatitis did not occur in temporal proximity (within 30 days) in 227 patients, and three patients had invalid medical record numbers. The final population was 13 patients. Surgical data included indication for repair and surgical approach. Clinical/imaging data points included method of diagnosis, type of pancreatitis, location, management of collections, and patient outcome. Results Thirteen patients (n = 9 male, age 58–76 years) met inclusion criteria. All patients underwent open repair, 9 electively. Acute pancreatitis was first identified by computed tomography in 10 (77%) patients and by serum lipase levels in 3 patients (23%). Necrotizing pancreatitis was present in 10 patients (77%), eight with infected collections (one not sampled). Four patients (31%) had collections fistulizing to the aortic sac, and there was one case of aortic anastomotic pseudoaneurysm. There were 7 deaths (4 septic shock, 1 hemorrhagic pancreatitis, 1 pulmonary embolism, and 1 multiorgan failure) despite 5 of these patients undergoing drainage of collections. In surviving patients, 4 underwent drainage of collections, 1 necrosectomy, and 1 no intervention. Conclusions Pancreatitis complicating aortic repair occurs after open repair and is often necrotizing. Pancreatitis is more often first detected by imaging rather than serum lipase levels. High mortality is more attributable to complications of pancreatitis rather than failure of the aortic repair.

Background “Chimney” techniques with parallel grafts used in order to extend the landing zones in endovascular aneurysm repair (ch-EVAR) are increasingly being reported. Conflicting data has been reported regarding the success and durability of the repair. We report a single center experience using ch-EVAR in treating complex aortic pathologies. Methods We performed a retrospective review of all patients treated with ch-EVAR in our institution between 2013 and 2017. Data collected included patients demographics, indications for surgery, configuration of parallel grafts, technical success, and perioperative morbidity and mortality. Follow-up data included aortic sac size, reintervention rate, and overall mortality. Results Thirty-five patients underwent treatment of their aneurysms with parallel grafts. Sixty parallel grafts were placed. Mean age was 75 years (range 59–93) and 30 (85%) were male. Technical success was achieved in 32 (91%) patients. Mean follow up was 12 months. Sac size decreased in diameter or remained unchanged in 26 of the 30 (86.6%) patients. Four patients were found to have enlarging aneurysms due to gutter endoleaks. All were treated successfully with endovascular methods. The estimated primary patency was 95% at 12 months. Probability of freedom from intervention was 75% at 12 months. No late aneurysm related mortality occurred. Conclusion The use of ch-EVAR in treating complex aortic aneurysms is technically feasible and safe. Gutter endoleaks are encountered only in a minority of the cases, and can be treated with minimally invasive techniques. Longer term follow up is required to evaluate the patency of these parallel grafts and the durability of the aneurysm exclusion.

Background: Outflow tract (OFT) septation defects are a common cause of congenital heart disease. Numerous studies have focused on the septation mechanism of the OFT, but have reported inconsistent conclusions. This study, therefore, aimed to investigate the septation of the aortic sac and the OFT in the early embryonic human heart. Methods: Serial sections of 27 human embryonic hearts from Carnegie stage (CS) 10 to CS19 were immunohistochemically stained with antibodies against c-smooth muscle actin (α-SMA) and myosin heavy chain. Results: At CS10–CS11, the OFT wall was an exclusively myocardial structure that was continuous with the aortic sac at the margin of the pericardial cavity. From CS13 onward, the OFT was divided into nonmyocardial and myocardial portions. The cushion formed gradually, and its distal border with the OFT myocardium was consistently maintained. The aortic sac between the fourth and sixth aortic arch arteries was degenerated. At CS16, the α-SMA-positive aortopulmonary septum formed and fused with the two OFT cushions, thus septating the nonmyocardial portion of the OFT into two arteries. At this stage, the cushions were not fused. At CS19, the bilateral cushions were fused to septate the myocardial portion of the OFT. Conclusions: Data suggest that the OFT cushion is formed before the aortopulmonary septum is formed. Thus, the OFT cushion is not derived from the aortopulmonary septum. In addition, the nonmyocardial part of the OFT is septated into the aorta and pulmonary trunk by the aortopulmonary septum, while the main part of the cushion fuses and septates the myocardial portion of the OFT. Key words: Aortopulmonary Septum; Human Embryonic Heart; Immunohistochemistry; Outflow Tract; Outflow Tract Cushion

We present the case of delayed migration of a thrombosed aortic endograft within a thrombosed aneurysm sac that expanded and ruptured. Dilation of the aortic neck likely led to endograft migration and exposure of the occluded endograft and aneurysm sac to systemic pressure. Although no endoleak was identified, a key finding on ultrasound showed mobility of the sac thrombus. This may be an indicator of flow within the sac that may predict potential for rupture. Despite thrombosis of the aortic sac and endograft, the risk of rupture still lingers, and thus continued surveillance of occluded endografts may be prudent.

We report a case of infolding of a fenestrated stent graft involving the visceral vessel segment after a juxtarenal abdominal aorta aneurysm repair. The patient remains free of any significant endoleak, and the aortic sac has shown regression. The patient remains asymptomatic, with no abdominal pain, with normal renal function, and without ischemic limb complications. We hypothesize that significant graft oversizing (20%-30%) with asymmetric engineering of the diameter-reducing ties may have contributed to the infolding. Because of the patient's asymptomatic nature and general medical comorbidities, further intervention was deemed inappropriate as the aneurysmal sac is regressing despite the infolding.

It is now established that the entity often described as an “aortopulmonary septal complex„ is better considered as an “outflow tract septal complex„. This change is crucial for appropriate understanding of not only malformations of the outflow tract, but also ventricular septal defects. Thus, the embryonic outflow tract, as it develops, is separated into its two components by fusion of a protrusion from the dorsal wall of the aortic sac with the distal end of the outflow cushions. The key point with regard to morphogenesis is that, with ongoing development, these structures lose their septal integrity, although they can still be identified as septal structures when the ventricular septum itself is deficient. In the normal postnatal heart, however, the aortic and pulmonary components have their own walls throughout the length of the outflow tracts. All of this is of clinical significance, since some current concepts of categorisation of the ventricular septal defects are based on the existence in the normal heart of a “conal septum„, along with a “septum of the atrioventricular canal„. In this review, we show how analysis of postnatal hearts reveals the definitive ventricular septum to possess only muscular and fibrous components in the absence of either discrete outflow or inlet components. We also show that this information regarding development, in turn, is of major significance in determining whether categorisation of ventricular septal defects is best approached, in the first instance, on the basis of the borders of the defects or the fashion in which they open to the right ventricle.

An enlarging aneurysm after endovascular aneurysm repair (EVAR) without clear endoleak is a clinical challenge. Management of this problem is guided by the current evidence for adequate EVAR follow up and recommended thresholds for re-intervention. In a frail patient, careful risk assessment of aneurysm related mortality against the risks associated with examinations and interventions is required.The literature was reviewed for imaging modalities for EVAR follow up and their advantages and disadvantages. The current evidence and guideline recommendations regarding follow up and re-intervention after EVAR were assessed in relation to the presented case.To detect sac expansion after EVAR, repeated examinations with the same imaging modality are needed. Verified expansion must be above the inter-observer variation of the method used. Although duplex ultrasound is an excellent modality for EVAR follow up, the finding of a significant expansion on duplex requires further examination, primarily with computed tomography angiography to assess sealing, stent graft integrity, and presence of endoleak. A frail patient should be assessed thoroughly before any kind of surgical intervention, the extent of which is related to the identified or suspected cause of expansion.Failure to totally exclude the aneurysm from continuing circulation, pressure and endoleak remains a potential shortcoming of EVAR. Significant sac expansion is an indication of EVAR failure. Decisions regarding further examinations or intervention are guided by the stability of the initial EVAR performed, the cause and extent of expansion, and the patient's comorbidities.

The abdominal aorta is the most common site of an aortic aneurysm. The visceral and most proximal infrarenal segment (aneurysm neck) are usually spared and considered more resistant to aneurysmal degeneration. However, if an abdominal aortic aneurysm (AAA) is left untreated, the natural history of the aortic neck is progressive dilatation and shortening. This may have significant implications for patients undergoing endovascular repair of AAAs (EVAR) as endograft stability and integrity of the repair are dependent on an intact proximal seal zone. Compromised seal zones, caused by progressive diameter enlargement and foreshortening of the aortic neck, may lead to distal endograft migration, type Ia endoleak, aortic sac repressurization, and, ultimately, aortic rupture.

Pre-emptive selective embolization of inferior mesenteric artery (IMA), lumbar arteries (LAs), and perigraft sac for prevention of type II endoleak (T2EL) has not been widely adopted. We perform pre-emptive nonselective perigraft aortic sac embolization with coils (PNPASEC) in patients at high risk for development of T2EL (four or more patent LAs, patent IMA ≥3 mm, and ≥30-mm aortic flow lumen). The goal of this study was to see whether PNPASEC decreases T2ELs requiring reinterventions.All 266 patients undergoing elective endovascular aneurysm repair between September 1, 2007, and October 31, 2015, were retrospectively evaluated from a prospectively maintained database. Patients (N = 212; 211 men) with preoperative and postoperative contrast-enhanced computed tomography scans were included. Our PNPASEC technique involves leaving a wire in the sac after cannulation of the contralateral gate and inserting large (0.035-inch) coils into the sac after bifurcated graft deployment. T2EL and reintervention rates were compared between patients who underwent PNPASEC (group I) and those who met the criteria but did not have PNPASEC (group II) and those who did not meet the criteria (Group III).Forty-seven (22.2%) patients were PNPASEC candidates and 165 (77.8%) patients (group III) were not. Among PNPASEC candidates, 16 (7.5%) underwent PNPASEC (group I) and 31 (14.6%) did not (group II). There were no significant differences between groups in terms of comorbidities, aneurysm size, and anatomic and neck characteristics. Mean number of patent LAs was similar between group I (4.5 ± 0.8) and group II (4.5 ± 0.9), which was significantly greater than in group III (1.9 ± 1.3; P .001); 43.6% of group III patients had patent IMA. Mean follow-up was 44 ± 25 months. T2EL at 6 months was observed in 48.4% in group II, 3.0% in group III, and 6.3% in group I (P .001). Sac diameter increase was seen in 38.7% in group II vs 6.1% in group III and 6.3% in group I (P .001), with complete sac shrinkage in 23.3% in group II vs 23.8% in group III and 50.0% in group I (P = .09). T2EL-related interventions were performed in 29.0% in group II vs 1.2% in group III and 6.3% in group I (P .001). Any endoleak at last follow-up was seen in 25.8% in group II vs 2.4% in group III and none in group I (P .001).Nonselective perigraft sac coil embolization in patients at high risk for T2EL (20% of patients undergoing endovascular aneurysm repair) is effective in preventing development of T2EL and is associated with decrease in sac size and reintervention rates.

The management of type II endoleaks that develop after endovascular repair of aortic aneurysms now includes the transcaval approach. We reviewed the safety and efficacy of this technique in 10 consecutive patients (82 ± 7 years old; 80% male) who presented with a mean sac enlargement of 1.2 ± 0.7 cm and documented flow within the aortic sac. Patients presented a mean of 5.5 ± 3.1 years after endovascular aneurysm repair, and five (50%) patients had prior attempts at endovascular repair of the documented endoleak. Patients underwent a percutaneous transcaval approach to the aorta with use of the Rosch-Uchida Transjugular Liver Access Kit (Cook Medical, Bloomington, Ind) through an 8F sheath in a biplane angiography room. Coil embolization of the sac and lumbar arteries and occasional use of gelatin granules and human thrombin slurry allowed obliteration of the endoleak. No complications developed. Nine patients (90%) were noted to have a decreased sac diameter on the postprocedure study, and only one (10%) had persistent (but markedly diminished) flow noted on the follow-up ultrasound examination. Follow-up is early and extends to 6 months without recurrence of the endoleaks. The ability to safely access the aortic sac through the vena cava is a potentially efficient, safe, and useful technique to treat aortic endoleaks.

Objective Endovascular aneurysm sealing (EVAS) with the Nellix stent graft system is a novel concept in the management of abdominal aortic aneurysm (AAA) that aims to reduce the prevalence of all endoleaks following endovascular repair. There are few data describing the longer-term durability of this approach. The aim was to report the longer-term outcomes following EVAS in a single centre. Methods This is a retrospective review of all patients that underwent Nellix at Cambridge University Hospitals Foundation Trust. Factors that are described as device failure include secondary sac rupture, graft explantation, further surgical procedures for Type 1 endoleak, or major migration of the stent grafts with pressurisation of the aortic sac. Results A total of 161 patients have been treated with Nellix. The indications included primary AAA ( n = 115), ruptured AAA ( n = 4), salvage of other aortic grafts ( n = 18), primary iliac aneurysm ( n = 6), and chimney EVAS (ChEVAS) for pararenal AAA ( n = 18). In total there have been 42 graft failures in patients treated with EVAS for primary AAA. The 4 year freedom from graft failure was 42% in patients treated for primary AAA. Failures mostly occurred more than 2 years post-Nellix implant. There were eight secondary sac ruptures (incidence 2.4 per 100 person years) and there have been 14 graft explants. Conclusions Failure of aneurysm sealing following treatment with Nellix has been more common than anticipated and can cause aortic rupture. Post-operative surveillance of Nellix stent grafts is crucial to identify features of failure.

Stent-graft migration and type I endoleaks are associated with a higher rate of reintervention and increased mortality and morbidity. This article describes a patient presented with an infrarenal aortic stent-graft which had migrated into the aortic sac with loss of all aortic neck attachment. The acutely expanding abdominal aortic aneurysm was treated by placing a second modular endograft within and above the migrated stentgraft. The patient returned 36 months later, with features of an acute myocardial infarction, severe bilateral lower limb ischemia, and renal failure. He was too ill for intervention and demised within 48 hours.

Address for Correspondence: Dr. Deepa Somanath, Assistant Professor, Dept. of Anatomy, Sri Manakula Vinayagar Medical College, Madagadipet, Puducherry605 107, India. E-Mail: deepa.somanath@gmail.com *1 Assistant Professor, Dept. of Anatomy, Sri Manakula Vinayagar Medical College, Puducherry, India. 2 Post Graduate Student, Dept. of Anatomy, Sri Manakula Vinayagar Medical College, Puducherry, India. Arterial variations in the branching pattern of arch of aorta are uncommon and may occur as a result of developmental changes in the fusion and absorption of various pharyngeal arch arteries into the aortic sac. The present case report describes a finding in a male cadaver aged between 6065 years about the origin of left vertebral artery directly from the arch of aorta. Such variations should be kept in mind during surgical procedures in the superior mediastinum.

Introduction Aorto-enteric fistula after endovascular aortic repair is an exceedingly rare but serious condition. Report A rare case of a fistula between the excluded aortic sac and the transverse colon 15 years after endovascular aortic repair is described. Onset was endocarditis without gastrointestinal haemorrhage, migration, endoleak, or aortic sac dilatation. The patient was successfully treated by fistula excision, debridement and broad spectrum antibiotic treatment without endograft explantation. Discussion Aorto-enteric fistula can emerge after endovascular repair with an unforeseen onset such as endocarditis, which in this case probably occurred as metastatic sepsis from endograft infection., Highlights • Uneventful endovascular aneurysm repair can generate an aorto-enteric fistula to a successful excluded and stable aneurysm sac. • This case underlines the need for awareness of unusual symptoms that could represent potential late EVAR related complications. • A conservative approach without graft removal seems feasible in this case with severe comorbidity.

An extensive search of the medical literature examining the development of the thoracic aortic tree reveals that the thoracic aorta does not develop as one unit or in one stage: the oldest part of the thoracic aorta is the descending aorta with the aortic arch being the second oldest, developing under influence from the neural crest cell. Following in chronological order are the proximal ascending aorta and aortic root, which develop from a conotruncal origin. Different areas of the thoracic aorta develop under the influence of different gene sets. These parts develop from different cell lineages: the aortic root (the conotruncus), developing from the mesoderm; the ascending aorta and aortic arch, developing from the neural crest cells; and the descending aorta from the mesoderm. Findings illustrate that the thoracic aorta is not a single entity, in developmental terms. It develops from three or four distinct areas, at different stages of embryonic life, and under different sets of genes and signaling pathways. Genetically triggered thoracic aortic aneurysms are not a monolithic group but rather share a multi-genetic origin. Identification of therapeutic targets should be based on the predilection of certain genes to cause aneurysmal disease in specific aortic segments.

Background The primary goal of endovascular aneurysm repair (EVAR) is to prevent death from aneurysm rupture. Regression of aortic sac size is believed to be a marker for success after EVAR. This study analyzes the changes in aneurysm sac size and the factors affecting sac regression after EVAR. Patients and Methods We retrospectively reviewed 121 patients with abdominal aortic aneurysm (AAA) who underwent elective treatment with EVAR at our institution from January 2005 to December 2011. In this study, 17 of the 121 patients were excluded due to loss during follow-up or for not having undergone a postoperative computed tomographic (CT) scan, and 3 patients were excluded due to an isolated iliac artery aneurysm. CT scans were scheduled at months 1, 6, and 12, and annually thereafter. Aneurysm size was defined by the minor axis on the largest axial cut of the aneurysm on a two-dimensional CT scan. Sac regression was defined as a reduction in the diameter of more than 5 mm. Results Sac regression was observed during follow-up in 39 of the 101 patients. There was 1 regression in 87 patients (1%) at 1 month, 18 in 62 patients at 6 months (29%), 26 regressions in 44 patients (59%) at 12 months, and 18 regressions in 34 patients (53%) at 24 months. After multivariate analysis, the absence of endoleaks was the only factor associated with sac regression (hazard ratio, 3.620; confidence interval, 1.692–7.747; p = 0.001). Conclusion Sac regression over 5 mm is associated with current or previous endoleaks after EVAR. Continued surveillance is necessary in all patients after EVAR to prevent late complications.

Background Complete thrombosis of an aortic endograft after an endovascular aortic aneurysm repair is a rare complication. The majority of thrombotic events occur in the iliac limbs. Case presentation We present the case of a patient who presented with acute limb ischemia as the result of a thrombosed infra-renal aortic endograft. After restoration of blood flow to the lower extremities with an axillary to bi-femoral artery bypass, the patient was lost to follow-up. The patient returned two years later with a ruptured abdominal aortic aneurysm due to a type 1A endoleak. Discussion We propose that all patients after endovascular aortic aneurysm repair, including those with a thrombosed aortic endograft, continue to undergo regular graft surveillance. This case report highlights the importance of continued surveillance of the aortic sac, even after total thrombosis of the endovascular aortic aneurysm repair.

Conservative management of an aortic graft infection is defined as “the nonresectional treatment of an aortic graft that has an established infection.” Incidence of aortic graft infections is 0.5 to 5% and the estimated mortality rate from aortic graft infections ranges from 8 to 27%. We present the case of a 73-year-old male patient with an infected abdominal aortic graft following an emergency ruptured abdominal aortic aneurysm repair. Postemergency repair, he developed ischemic colitis with sigmoid colon perforation leading to fecal peritonitis and secondary sepsis. He developed a large infective collection within the aortic sac growing vancomycin-resistant enterococcus sensitive to linezolid. A percutaneous drain was placed in the aortic sac and this was irrigated with linezolid for a total of 28 days. The patient clinically improved. Overall, 7 months later, follow-up scan shows complete resolution of infection and the patient remains clinically stable. Conventional treatment of aortic graft infections involves an extra-anatomical bypass. Percutaneous drainage and antibiotic use may be used as bridging therapy for surgery or as definitive therapy when surgical treatment is impractical. Most aortic graft infections grow gram-positive cocci, the organisms form a biofilm which is protected from the external environment. Percutaneous drainage and antibiotic irrigation could possibly penetrate the biofilm and eradicate infection. Morris et al conducted a study on 10 patients having irrigation therapy and systemic antibiotic treatment and found a 1-year survival rate of 80%. In conclusion, conservative aortic graft treatment may be an effective alternative where surgical intervention is not suitable.

Objectives Volume estimation is more sensitive than diameter measurement for detection of aneurysm growth after endovascular aneurysm repair (EVAR), but this has only been confirmed on three-dimensional, reconstructed computer tomography (3-D CT). The potential of 3-D ultrasound (3-D US) for volume estimation in EVAR surveillance is unknown. Design Prospective validation study comparing 3-D US with 3-D CT, using 3-D CT as the gold standard. Materials and methods From August 2011 to March 2012, 93 consecutive EVAR patients were enrolled and examined with both 3-D US and CT angiography (CTA). Image data were analysed in a mutual blinded setup using a 3-D interactive segmentation technique. Results The technical success rate of 3D-US was 98% (91/93). In 91 EVAR patients (F/M; 10/81) eligible for further analysis, the mean maximum volume (SD) was 126 (58) ml using 3-D US and 128 (58) ml using 3-D CT. The mean difference was 1 ml (0.4%) and the limits of agreement were −14 to 16 ml (−11; 12%). Conclusion Volume estimation of the aortic sac after EVAR using 3-D US is a feasible and accurate method using 3-D CT as the gold standard.

Variations and mutations in the human genome, such as 22q11.2 microdeletion, can increase the risk for congenital defects, including aortic arch malformations. Animal models are increasingly expanding our molecular and genetic insights into aortic arch development. However, in order to justify animal-to-human extrapolations, a human morphological, and molecular reference model would be of great value, but is currently lacking. Here, we present interactive three-dimensional reconstructions of the developing human aortic arch system, supplemented with the protein distribution of developmental markers for patterning and growth, including T-box transcription factor TBX1, a major candidate for the phenotypes found in patients with the 22q11.2 microdeletion. These reconstructions and expression data facilitate unbiased interpretations, and reveal previously unappreciated aspects of human aortic arch development. Based on our reconstructions and on reported congenital anomalies of the pulmonary trunk and tributaries, we postulate that the pulmonary arteries originate from the aortic sac, rather than from the sixth pharyngeal arch arteries. Similar to mouse, TBX1 is expressed in pharyngeal mesenchyme and epithelia. The endothelium of the pharyngeal arch arteries is largely negative for TBX1 and family member TBX2 but expresses neural crest marker AP2α, which gradually decreases with ongoing development of vascular smooth muscle. At early stages, the pharyngeal arch arteries, aortic sac, and the dorsal aortae in particular were largely negative for proliferation marker Ki67, potentially an important parameter during aortic arch system remodeling. Together, our data support current animal-to-human extrapolations and future genetic and molecular analyses using animal models of congenital heart disease. © 2013 Wiley Periodicals, Inc.

Congenital anomalies of the aortic arch (AoA) compose a very important group of cardiovascular malformations. Some of them are isolated, but the other ones associate with congenital heart defects (CHD) and genetic syndromes. During embryonic development 6 pairs of primitive aortic arches are formed. The normal formation of AoA, pulmonary trunk and ductus arteriosus depends on transformation of the aortic sac and left 4 th and 6 th aortic arches. The vascular rings are common AoA malformations. An example of them is aberrant subclavian artery, which courses behind oesophagus and trachea, and may result in airway and alimentary tract obstruction. Double AoA is formed by persistent both right and left 4 th aortic arches and may provide similar symptoms. Regression in the left 4 th aortic arch results in a right arch of aorta, which is rare in normal individuals and usually associates with CHD, e. g. tetralogy of Fallot. Interrupted AoA is defined as loss of continuity between ascending and descending aorta and is classified into 3 types depending on site of interruption. The narrowing of AoA can be observed in its different parts. The most common is coarctation of the aorta defined as congenital narrowing of the descending aorta distally to the left subclavian artery. The article is a review of embryology and morphology of AoA malformations.