A newly discovered anatomical abdominal aorta anomaly; "Arax anomaly".

BACKGROUND: Abdominal aorta (AA) is part of descending aorta. It faces with variety of malformations during embryogenic period. CASE PRESENTATION: A 35 years old woman with previous history of long term diabetes mellitus had experiment of one non-complicated successful pregnancy under surgical delivery. She referred to achieve health services due to de-novo postprandial abdominal pain and bilateral lower limbs exertional claudication. RESULTS: Following normal findings in upper esophago-gastro-duodenal study, angiographic evaluation of AA showed novel aortic variation in which celiac trunk, superior mesenteric artery, and bilateral renal arteries originated from one common arterial root. This anomaly has not been seen whether in alive or cadaveric explorations until this presentation. CONCLUSION: Precise arterial mapping is highly recommended when any attributed procedure is indicated.

A rare case of IgG4-related aortitis in the thoracic aorta mimicking an intramural hematoma: navigating the diagnostic labyrinth.

A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40-50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies.

Management of iatrogenic ascending aortic dissection after percutaneous intervention of chronic total occlusion.

BACKGROUND: This study highlights the prevalence and conservative management strategies of iatrogenic ascending aortic dissection after percutaneous intervention (PCI) of chronic total occlusion (CTO). METHODS: Retrospective analysis on patients who underwent CTO PCI from one medical center from 2020 to 2022 was performed by two operators. Data was obtained from the Prospective Global Registry for the Study of CTO database. RESULTS: Out of 318 patients, there were six reported cases of iatrogenic ascending aortic dissection. The causes of dissection included guiding catheter trauma, antegrade injection, two retrograde injection, and two cases of balloon rupture after lithotripsy. IVUS-guided stenting of the coronary back to the origin to seal the entry of the dissection flap occurred in two patients. All patients had a conservative management approach. No patient had to undergo urgent surgery. CONCLUSION: While the incidence remains rare, complications, including aortic dissection, are more frequent in CTO PCI as compared to non-CTO PCI. IVUS can be used to complete coronary intervention so long as the patient remains clinically and hemodynamically stable. Patients should have formal imaging with either transesophageal echocardiogram or computed tomography angiography. The outcomes of patients who experience iatrogenic aortic dissection during CTO PCI with stable coronary disease seem to be better than in patients with acute coronary syndrome.

Successful Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA) deployment by emergency medicine physicians for refractory non-traumatic cardiac arrest.

Aim: Cardiac arrest afflicts over 600,000 people annually in the United States. Rates of survival from cardiac arrest have remained stagnant for decades. Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA) is most commonly used in the management of severe hemorrhagic shock, primarily for non-compressible thoracoabdominal trauma. A growing body of evidence suggests it may serve a role in augmenting cardiac and cerebral perfusion in non-traumatic, refractory cardiac arrest. Typically, REBOA is deployed by interventional radiologists under real-time fluoroscopy. Limited data exist to demonstrate the feasibility or logistics of successful REBOA deployment in emergency departments by emergency medicine physicians. Methods: We describe an emergency medicine-driven training program and treatment protocol developed to deploy REBOA in the emergency department for patients experiencing refractory out-of-hospital cardiac arrest and deemed ineligible for ECPR. We detail the training, certification processes, and clinical outcomes from our first eight cases. Results: Five emergency medicine physicians underwent training for REBOA placement through a didactic curriculum and hands-on training with mannequin and live tissue porcine models. Since protocol implementation, eight patients have undergone REBOA catheterization by emergency medicine physicians: 5 males and 3 females, age range 25-79. The first pass success was 8/8 (100 %), and all 3 commercially available catheters in the United States were successfully used. ROSC was achieved in 3/8 (37.5 %) patients, although no patients survived to hospital discharge. No REBOA catheter-associated complications were identified. Conclusions: This series demonstrates feasibility of emergency physician placed REBOA for non-traumatic, refractory cardiac arrest a novel resuscitative technique. Through a combination of focused education, innovative technology use, robust large animal model-based training, and strategic procedural integration, we showcase the potential for emergency departments to spearhead the adoption of this potentially life-saving intervention.

Evaluation of the clinical features of an outpatient cohort with Marfan syndrome.

Marfan syndrome is a connective tissue disease with autosomal dominant inheritance and variable clinical presentation. The main clinical manifestations recognition could contribute to early diagnosis and cardiovascular complication prevention. We aimed to evaluate the clinical profile of a Marfan syndrome outpatient cohort. METHODS: Retrospective cross-sectional study was carried out with outpatients over 12 years of age whose electronic medical records contained the clinical information and complementary exams necessary for study inclusion. Data were analyzed using descriptive statistics and comparisons were performed using student's t-test and chi-square or Fisher's exact test. P-values<0.05 were considered statistically significant. RESULTS: 75 patients (29.5 ± 13.4 years) were included and 43(57 %) were female. Positive family history for the syndrome was observed in 55(73 %) patients and ectopia lentis in 37(49 %). Positive systemic score (≥7) was identified in 60(80 %) individuals and the most frequent score components were: skin striae in 64(85 %), scoliosis in 59(79 %), wrist and thumb sign in 45(60 %), moderate or severe myopia in 43(57 %) and plain flat foot in 40(53 %). Cardiovascular symptoms occurred in 17(23 %) patients: dyspnea in 10(13 %) and palpitations in 6(8 %). Mitral valve prolapse was observed in 32(43 %) participants and aortic root dilation (z-score ≥ 2) in 53(71 %), without significant difference between the groups with or without these alterations concerning sex, age, or symptom presence. CONCLUSION: Clinical profile of a Marfan syndrome outpatient cohort includes adolescents and young adults, most without cardiovascular symptoms and with a high incidence of skeletal, ophthalmological, and cardiovascular involvement. Recognizing these clinical signs could contribute to early disease diagnosis in the general population.

Morphological Differences in the Abdominal Aorta Between Subjects With and Without Type 2 Diabetes.

Aim Chronic hyperglycemia is a well-known risk factor for the development of many macrovascular complications, but hyperglycemia may be reportedly protective against abdominal aneurysms. Materials and methods In this study, we evaluated morphological differences in the abdominal aorta between subjects with and without type 2 diabetes mellitus (T2DM) without abdominal aortic aneurysm and evaluated the correlation between imaging findings of computed tomography (CT) and diabetes-related parameters. Results The abdominal aortic diameter was significantly smaller in subjects with T2DM compared to non-diabetes mellitus (NDM) subjects (p=0.026). Abdominal aortic wall thickness assessed by contrast-enhanced CT was significantly greater in subjects with T2DM compared to NDM subjects (p=0.011). There was a significant single correlation between abdominal aortic diameter and age, gender, Brinkman index, HbA1c, and mean/max intima-media thickness (IMT). Multiple regression analysis showed that HbA1c was an independent negative factor affecting abdominal aortic diameter (t=-3.28, p=0.0036). And Brinkmann index was an independent factor affecting aortic wall thickness (t=2.23, p=0.034). Conclusion This study revealed the imaging characteristics of smaller abdominal aortic diameter and larger wall thickness in T2DM subjects compared to NDM subjects. The abdominal aortic wall thickening was significantly correlated with cervical IMT. Therefore, close examination for other diabetes-related macrovascular complications should be aggressively considered when these findings are present.

Variation in the Branches of Arch of Aorta in Patients Undergoing Contrast Enhanced Multidetector Computed Tomography in a Tertiary Centre, Nepal.

INTRODUCTION: Variations in the branching pattern of the Arch of Aorta (AoA) are common in patients undergoing contrast-enhanced Multidetector Computed Tomography, the identification of which is crucial in managing patients undergoing cardiovascular/neck surgeries and interventions. METHODS: This prospective cross-sectional study involved 513 patients who were sent to the Department of Radiology for evaluation of various pathologies of chest and neck between August 2018 and July 2019. After approval from the Institutional Review Committee {Reference No: 11(6-11) E2/075/076}, contrast-enhanced computed tomography images were evaluated with variations in branches of the left-sided arch of the aorta and symptoms associated. RESULTS: Variations in branches of the arch of aorta were seen in 69 (13.45%; 95% CI: 10.60%-16.71%) of cases, left common carotid artery and brachiocephalic trunk having common origin or common trunk was 51(9.94%). The mean age was 52.4±20 years (Range 3 months to 92 years) with male to female ratio of 1.3:1. CONCLUSIONS: Contrast-enhanced computed tomography is the modality of choice for the detection of the variations in branches of AoA, recognition of which is crucial in vascular intervention and surgical procedures to reduce the postoperative morbidity and mortality of the patients.

IVUS-Guided Intravascular Lithotripsy of the Juxtarenal and Visceral Coral Reef Aorta.

A patient with coral reef aorta at the level of the renal and visceral arteries was treated endovascularly. We used intravascular lithotripsy for improving stent expansion and intravascular ultrasound as an intraoperative diagnostic tool. The renal artery periscope technique maintained renal perfusion after placement of the aortic endograft.

Multiple Variations in Abdominal Aorta Branching and Their Clinical Significance: A Case Report.

The abdominal aorta extends from the T12 vertebra and terminates at the L4 vertebra. It gives off anterior, lateral, and posterior branches that supply the abdominal viscera and vertebrae at this level, eventually dividing into the common iliac arteries. Dissection of the abdominal aorta and its branches from a female cadaver revealed several variations: the right inferior phrenic artery arose from the celiac trunk, the left middle suprarenal artery originated at the L1 vertebra, while the right middle suprarenal artery arose at the L2 vertebra, and the left and right renal arteries emerged from the L2 and L1 vertebrae, respectively. The gonadal arteries did not originate from the abdominal aorta. Inferior phrenic arteries may arise from the abdominal aorta, celiac trunk, or occasionally form renal arteries and are linked with extrahepatic supply in hepatocellular carcinoma. Middle suprarenal arteries typically originate from the abdominal aorta at L1, but may occasionally arise from L2 or be absent. Variations in the middle suprarenal arteries often correspond with variations in the inferior phrenic and gonadal arteries. Renal arteries may arise at the L1 vertebra, the L1/L2 intervertebral disc, or the L2 vertebra, with additional variations reported. The gonadal arteries may not originate from the abdominal aorta in some cases. These branching variations of the abdominal aorta are important for clinical, diagnostic, and therapeutic procedures and should be documented accordingly.

Resuscitative endovascular balloon occlusion of the aorta (REBOA) for non-trauma patients in an urban hospital: a series of two cases.

Background: Resuscitative endovascular balloon occlusion of the aorta (REBOA) is increasingly being used for temporary bleeding control in patients with trauma with non-compressible truncal hemorrhage (NCTH). In recent years, the technique is gaining popularity in postpartum hemorrhage and non-traumatic cardiac arrest, although still underutilized. In other surgical fields, however, there is not yet much awareness for the possible advantages of this technique. Consequently, for non-trauma indications, limited data are available. Methods: Description of the use of REBOA in two patients with hemorrhagic shock due to exsanguinating non-traumatic NCTH. Results: In the first case, REBOA was deployed at the emergency department in a patient in their 80s presenting with hemorrhagic shock due to a ruptured abdominal aortic aneurysm. Hemodynamic stability was obtained and a CT scan was subsequently performed for planning of endovascular aneurysm repair. After successful placement of the endograft, the REBOA catheter was deflated and removed. In the second case, REBOA was performed in a patient with shock due to iatrogenic epigastric artery bleeding after an umbilical hernia repair to prevent hemodynamic collapse and facilitate induction of anesthesia for definitive surgery. During laparotomy, blood pressure-guided intermittent aortic balloon occlusion was used to preserve perfusion of the abdominal organs. Patient made a full recovery. Conclusion: REBOA deployment was successful in achieving temporary hemorrhage control and hemodynamic stability in patients with non-traumatic NCTH. REBOA facilitated diagnostic work-up, transportation to the operating room and prevented hemodynamic collapse during definitive surgical repair. In the right patient and skilled hands, this relatively simple endovascular procedure could buy precious time and prove lifesaving in a variety of non-compressible hemorrhage.

Comprehensive analysis of traumatic rupture of the aorta in road traffic crashes: incorporating epidemiological insights and K-prototype clustering.

OBJECTIVES: The identification of crash characteristics associated with traumatic rupture of the aorta (TRA) can significantly enhance countermeasures against TRA. Conventional epidemiological approaches struggle to adequately handle the substantial variability of traffic crash data. Consequently, this study aims to integrate conventional epidemiological analysis with data-driven cluster analysis to more comprehensively analyze TRA-related crash characteristics. METHODS: A total of 350 unweighted TRA crashes were extracted from traffic crash databases including comprehensive crash details and injury descriptions. Initially, a selection was made of 11 continuous variables and 9 categorical variables, describing crash characteristics. After correlation analysis and principal component analysis were applied to the dataset, K-prototype clustering was finally conducted using 6retained categorical variables and 6 principal components derived from the continuous variables. RESULTS: This study found significant age and gender disparities among TRA victims, with 50% falling within the age range of 25-59 years and an overwhelming majority (62.2%) being males. Side impacts emerged as the primary cause of TRA-related crashes (37.2%), followed by collisions with off-road objects (28.6%) and head-on collisions (24.8%). Cluster analyses revealed 6 distinct clusters within the TRA-related crash dataset. These clusters were characterized by factors such as vehicle model year, curb weight, collision dynamics, and seatbelt usage, providing a deeper understanding of the heterogeneity in TRA incidents and their associated factors. CONCLUSIONS: Although limitations related to available data sources and factors such as accompanying injuries and vehicle weight warrant further comprehensive investigations in the future, this study contributes valuable insights into TRA analysis to enhance understanding and prevention strategies.

Physician-modified funnel-shaped covered stent for selective false lumen exclusion in chronic type B aortic dissection.

PURPOSE: To describe the technique of off-centering a balloon-expandable covered stent for selective occlusion of a distal entry tear (ET) in a patient, conservatively treated for chronic type B aortic dissection (cTBAD), presenting FL expansion. TECHNIQUE: A 63-year-old male, with conservatively managed cTBAD, presented at follow-up with FL partial thrombosis and expansion (thoracic aorta FL from 21 mm to 27 mm and abdominal aorta FL from 11 mm to 15 mm in 6 months). No proximal ET was identifiable. Distal FL perfusion was caused by an ET in the abdominal aorta feeding a 2 mm accessory renal artery (ARA). As the aortic diameter was below the threshold for endovascular repair, a selective occlusion of the distal ET and ARA was planned. A balloon-expandable covered stent was modified by off-centering the covered stent proximally and resulting in a funnel-shape occluder after deployment across the ET into the ARA. To prevent type Ic endoleak due to possible FL expansion caused by an intra-operatively detected phrenic artery (PA), coils were deployed into the lumen of the modified stent and the ARA. The pre-discharge computed tomography angiography showed exclusion of both the ARA and ET and a type 2 endoleak from the PA. CONCLUSION: A balloon-expandable covered stent can be modified by off-centering the covered stent resulting in a funnel shape to adapt to different diameter requirements.

Unraveling aortic hemodynamics using fluid structure interaction: biomechanical insights into bicuspid aortic valve dynamics with multiple aortic lesions.

Aortic lesions, exemplified by bicuspid aortic valves (BAVs), can complicate congenital heart defects, particularly in Turner syndrome patients. The combination of BAV, dilated ascending aorta, and an elongated aortic arch presents complex hemodynamics, requiring detailed analysis for tailored treatment strategies. While current clinical decision-making relies on imaging modalities offering limited biomechanical insights, integrating high-performance computing and fluid-structure interaction algorithms with patient data enables comprehensive evaluation of diseased anatomy and planned intervention. In this study, a patient-specific workflow was utilized to biomechanically assess a Turner syndrome patient's BAV, dilated ascending aorta, and elongated arch. Results showed significant improvements in valve function (effective orifice area, EOA increased approximately twofold) and reduction in valve stress (~ 1.8-fold) following virtual commissurotomy, leading to enhanced flow dynamics and decreased viscous dissipation (~ twofold) particularly in the ascending aorta. However, increased viscous dissipation in the distal transverse aortic arch offset its local reduction in the AAo post-intervention, emphasizing the elongated arch's role in aortic hemodynamics. Our findings highlight the importance of comprehensive biomechanical evaluation and integrating patient-specific modeling with conventional imaging techniques for improved disease assessment, risk stratification, and treatment planning, ultimately enhancing patient outcomes.

What is pentalogy of Cantrell?

We present the unusual case of an 8-month-old female with tetralogy of Fallot, coarctation of aorta, and complete presentation of pentalogy of Cantrell. A meta-analysis of 236 cases of Cantrell's syndrome reported in the literature was performed to compare intracardiac findings.

The Effect of Stenting on Post-Stenotic Dilatation in Coarctation of The Aorta.

The effect of stenting of native aortic coarctation (CoA) on post-stenotic dilatation (PSD) has not been previously described. We hypothesized that CoA stenting may lead to positive remodeling of PSD. Retrospective analysis of patients who underwent stent implantation for native CoA from 1999 to 2021 was performed. Primary outcome was incremental change PSD diameter and the PSD/DescAo (Descending Aorta) following stent implantation and comparison between covered and bare-metal stents. 90 consecutive patients, (26 female, average age at first intervention 12.0 years) were included. 35 patients (38.9%) underwent dilatation with bare-metal stents and 55 patients (61.1%) with covered stents. The covered stent subgroup was older (14.0 vs. 9.2 years old, p < 0.001) and PSD was larger (17.0 vs 14.0 mm, p < 0.001). Over a mean of 3.2 years, mean inter-catheterization growth of the PSD was blunted [- 0.05 mm, 95% CI (- 1.5 to 1.4)]. The covered stent subgroup demonstrated a negative inter-catheterization growth compared to the bare-metal stent subgroup (- 0.7 vs 1.6 mm, p < 0.001). When controlled for somatic growth, the PDS/DescAo decreased more significantly among those with covered stent vs bare metal (- 0.12 vs - 0.058, p = 0.004). Stenting of native CoA blunts the growth of PSD; covered stents were significantly associated with regression of the diameter of the PSD over time compared to bare-metal stents.

Clinical effects of hybrid debranching technique for acute Stanford type A aortic dissection.

BACKGROUND: To investigate the clinical effects and safety of the hybrid debranching technique for patients with acute Stanford type A aortic dissection (AD). METHODS: One hundred nine patients with acute Stanford type a AD were selected and divided into observation group and control group according to the different surgical methods. Fifty-five patients in the observation group were treated with hybrid debranching, and 54 patients in the control group were treated with Sun's operation. The operation duration, clamp time, cardiopulmonary bypass duration, volume of blood transfusion, ventilator application duration, duration of stay in the intensive care unit, aortic rupture, second thoracotomy due to hemorrhage, gastrointestinal hemorrhage, stroke, paraplegia, renal failure, and all-cause mortality were recorded. Postoperative follow-up was conducted. The number of cases that underwent follow-up and the number of cases with complete thrombosis of the false aneurysm cavity detected by computed tomography angiography (CTA) was recorded. RESULTS: The surgical success rate was 100% in both groups, and there were no cases with unplanned secondary surgery. Compared with the control group, only the difference in the volume of blood transfusion was not significantly significant between the two groups (P = 0.052), while the rest of the observation indicators were significantly lower in the observation group than in the control group (P < 0.001 for all). The proportion of cases with complete thrombosis of the false aneurysm cavity was significantly higher in the observation group than in the control group at 3 and 6 months after surgery (P < 0.05). CONCLUSION: In patients with acute Stanford type A AD involving the arch, the hybrid debranching technique was safe and effective. It was recommended for patients with advanced age and a high risk of intolerance to deep hypothermic circulatory arrest.

Maffucci Syndrome May Be a Heritable Thoracic Aortic Disease and a Cause of Aortic Dissection.

A woman with Maffucci syndrome (MS) presented post partum with type B aortic dissection leading to rupture of a thoracoabdominal aneurysm. Results of multiple-gene testing for heritable thoracic aortic disease were negative. Although conjectural, this patient's aortic disease may be related to MS, and surveillance for aortic disease in patients with MS may be appropriate.

Atypical Cardiac Anatomy Leading to Subvalvar Aortic Stenosis in a 19-Month-Old Boy With a Murmur.

A 19-month-old boy presented with a murmur and was found to have an unusual etiology of subvalvar aortic stenosis with discrete subaortic membrane and anomalous attachment of the anterior mitral valve papillary muscle to the interventricular septum. Preoperative suspicion for mitral valve involvement impacted surgical planning.

A rare case of Erdheim Chester disease.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. There are few documented cases in the medical literature. Here, we present an infrequent case of a 53-year-old patient who presented with cutaneous xanthelasma and a gradual decline in general health characterized by asthenia, anorexia, and chronic dyspnea over the last 5 years. Chest, abdominal, and pelvic CT scans revealed distinct findings suggestive of ECD, including peri-renal fat infiltration resulting in the "hairy kidney" sign, hepatosplenomegaly, renal artery ostial stenosis, pneumopericardium thickening, interstitial lung parenchymal involvement, metaphyseal-diaphyseal osteosclerosis affecting long bones, and sinus osteosclerosis. A biopsy confirmed the diagnosis. This case highlights the importance of radiologists being familiar with the characteristic radiologic signs of ECD to avoid unnecessary repeat examinations, delays in diagnosis, or misdiagnosis.