Acute Thoracic Aortic Dissection Research Articles

Long-term outcomes after recurrent acute thoracic aortic dissection: Insights from the International Registry of Aortic Dissection

ObjectiveWith an aging population and advancements in imaging, recurrence of thoracic aortic dissection is becoming more common. MethodsAll patients enrolled in the International Registry of Aortic Dissection from 1996 to 2023 with type A and type B acute aortic dissection were identified. Among them, initial dissection and recurrent dissection were discerned. The study period was categorized into 3 eras: historic era, 1996 to 2005; middle era, 2006 to 2015; most recent era, 2016 to 2023. Propensity score matching was applied between initial dissection and recurrent dissection. Outcome of interests included long-term survival and cumulative incidence of major aortic events defined by the composite of reintervention, aortic rupture, and new dissection. ResultsThe proportion of recurrent dissection increased from 5.9% in the historic era to 8.0% in the most recent era in the entire dissection cohort. In patients with type A dissection, propensity score matching between initial dissection and recurrent dissection yielded 326 matched pairs. Kaplan–Meier curves showed similar long-term survival between the 2 groups. However, the cumulative incidence of major aortic events was significantly higher in the recurrent dissection group (40.3% ± 6.2% vs 17.8% ± 5.1% at 4 years in the initial dissection group, P = .02). For type B dissection, 316 matched pairs were observed after propensity score matching. Long-term survival and the incidence of major aortic events were equivalent between the 2 groups. ConclusionsThe case volume of recurrent dissection or the ability to detect recurrent dissection has increased over time. Acute type A recurrent dissection was associated with a higher risk of major aortic events than initial dissection. Further judicious follow-up may be crucial after type A recurrent dissection.

Immune Response Associated Gene Signatures in Aortic Dissection Compared to Aortic Aneurysm.

Thoracic aortic dissections (TAD) are life-threatening events mostly requiring immediate surgical treatment. Although dissections mainly occur independently of thoracic aortic aneurysms (TAA), both share a high comorbidity. There are several indications for an involvement of the immune system in the development of TAD, just as in TAA. Nevertheless, specific disease-relevant genes, biomolecular processes, and immune-specific phenotypes remain unknown. RNA from isolated aortic smooth muscle cells from TAD (n = 4), TAA (n = 3), and control patients were analyzed using microarray-based technologies. Additionally, three publicly available bulk RNA-seq studies of TAD (n = 23) and controls (n = 17) and one single-cell RNA-seq study of TAA (n = 8) and controls (n = 3) were analyzed. Differentially expressed genes were identified and used to identify affected pathways in TAD. Five selected genes were validated by quantitative real-time polymerase chain reaction (PCR). We identified 37 genes that were significantly dysregulated in at least three TAD studies-24 of them were not shown to be associated with TAD, yet. Gene ontology analysis showed that immune response was significantly affected. Five of the genes (CCL2, RNASE2, HAVCR2, CXCL8, and IL6R) were revealed as core genes that affect immune response in TAD. We compared the gene expression of those genes to TAA and found that CXCL8, IL6R, and potentially also CCL2 were upregulated in TAD. The identified immune-related genes showed TAD-specificity, independent of possible pre-existing comorbidities like TAA. So, these genes represent potential biomarkers and therapeutic targets linked to the immune response in acute TAD. Additionally, we identified a set of differentially expressed genes that represents a resource for further studies.

Immune-Mediated Non-Infectious Periaortitis After Aortic Graft Replacement Surgery: A Case Report Highlighting the Need for Immunosuppressive Therapy.

BACKGROUND A non-infectious inflammatory reaction against replaced aortic graft for aortic dissection often manifests as fever, malaise, and peri-graft effusion. It usually lasts less than 1 month and subsides spontaneously without immunosuppressive treatment. CASE REPORT A 49-year-old man underwent ascending aorta and total arch replacement for acute thoracic aortic dissection. He had fever, malaise, nausea, and elevated serum C-reactive protein for 1 month postoperatively. Pathological examination of the aorta revealed no aortitis, and repeated blood cultures were negative. We also noted periaortic graft fluid collection, and a small amount of pleural and pericardial effusions. We suspected post-pericardiotomy syndrome. Colchicine and prednisolone were administered, with an excellent clinical response. Three weeks after discontinuation of a 7-week prednisolone treatment, the same symptoms recurred and gradually worsened. Prednisolone was restarted 6 months after the first surgery, with good clinical response. Thereafter, he developed left-sided weakness and dysarthria, being diagnosed as ischemic stroke. Contrast-enhanced computed tomography revealed fluid collection with contrast leak around the aortic grafts, suggesting peel dehiscence, and thrombus formation in anastomotic pseudoaneurysm. He underwent surgical repair. He was diagnosed with non-infectious periaortitis, likely due to an immune reaction to the grafts, based on an excellent clinical response to immunosuppressive therapy. CONCLUSIONS We report a case of non-infectious periaortitis around a thoracic aortic graft, probably with an immune-mediated mechanism, requiring immunosuppressive treatment. When fever persists after aortic graft replacement surgery, non-infectious periaortitis should be considered and immunosuppressive treatment should be considered to prevent critical complications of anastomotic pseudoaneurysm and graft dehiscence.

Causal Role for Neutrophil Elastase in Thoracic Aortic Dissection in Mice.

Thoracic aortic dissection (TAD) is a life-threatening aortic disease without effective medical treatment. Increasing evidence has suggested a role for NE (neutrophil elastase) in vascular diseases. In this study, we aimed at investigating a causal role for NE in TAD and exploring the molecular mechanisms involved. β-aminopropionitrile monofumarate was administrated in mice to induce TAD. NE deficiency mice, pharmacological inhibitor GW311616A, and adeno-associated virus-2-mediated in vivo gene transfer were applied to explore a causal role for NE and associated target gene in TAD formation. Multiple functional assays and biochemical analyses were conducted to unravel the underlying cellular and molecular mechanisms of NE in TAD. NE aortic gene expression and plasma activity was significantly increased during β-aminopropionitrile monofumarate-induced TAD and in patients with acute TAD. NE deficiency prevents β-aminopropionitrile monofumarate-induced TAD onset/development, and GW311616A administration ameliorated TAD formation/progression. Decreased levels of neutrophil extracellular traps, inflammatory cells, and MMP (matrix metalloproteinase)-2/9 were observed in NE-deficient mice. TBL1x (F-box-like/WD repeat-containing protein TBL1x) has been identified as a novel substrate and functional downstream target of NE in TAD. Loss-of-function studies revealed that NE mediated inflammatory cell transendothelial migration by modulating TBL1x-LTA4H (leukotriene A4 hydrolase) signaling and that NE regulated smooth muscle cell phenotype modulation under TAD pathological condition by regulating TBL1x-MECP2 (methyl CpG-binding protein 2) signal axis. Further mechanistic studies showed that TBL1x inhibition decreased the binding of TBL1x and HDAC3 (histone deacetylase 3) to MECP2 and LTA4H gene promoters, respectively. Finally, adeno-associated virus-2-mediated Tbl1x gene knockdown in aortic smooth muscle cells confirmed a regulatory role for TBL1x in NE-mediated TAD formation. We unravel a critical role of NE and its target TBL1x in regulating inflammatory cell migration and smooth muscle cell phenotype modulation in the context of TAD. Our findings suggest that the NE-TBL1x signal axis represents a valuable therapeutic for treating high-risk TAD patients.

Life-Threatening Aortic Dissection during Pregnancy: A Case Report of Undiagnosed FBN1-Related Marfan Syndrome at 39 Weeks Gestation.

BACKGROUND Inherited deficiencies in the FBN1 gene, which encodes fibrillin-1, result in Marfan syndrome, an autosomal dominant connective tissue disorder that is associated with aortic root dilatation and predisposes to aortic dissection. This report is of a 37-year-old woman presenting at 39 weeks of pregnancy with acute thoracic aortic dissection due to previously undiagnosed FBN1-related Marfan syndrome. This case report aims to illustrate the challenges in the diagnosis and in the peri-operative management of acute aortic dissection during pregnancy. CASE REPORT A healthy 37-year-old woman at 39 weeks of gestation presented to our hospital with dyspnea and chest pain. Initial evaluation for pulmonary embolism with chest computed tomography was unrevealing. The patient was admitted to the intensive care unit for further management. Overnight, her clinical conditions deteriorated, and a transthoracic echocardiography was obtained, demonstrating an acute ascending aortic dissection. She emergently underwent a successful combined cesarean section and ascending aortic dissection repair, with no immediate complications. On postoperative day 4 she developed cardiac tamponade, for which she underwent emergent mediastinal exploration. She was discharged home on postoperative day 10. A month later she completed genetic testing, which revealed a pathogenic mutation in the FBN1 gene, consistent with a molecular diagnosis of Marfan syndrome. CONCLUSIONS This report has shown that FBN1-related Marfan's syndrome has a variable clinical presentation that can include life-threatening aortic dissection during pregnancy. Successful diagnosis and management of these patients is challenging and requires multidisciplinary expertise, including confirmation of the diagnosis by a clinical geneticist.

Successful resuscitation from acute coronary syndrome during mediastinoscope-assisted transhiatal esophagectomy: a case report

BackgroundAlthough unexpected cardiac arrest is a very rare intraoperative complication, strategies regarding preoperative screening and procedures to be taken in the event of an emergency need to be well established.Case presentationA man in his late 70 s diagnosed with thoracic esophageal cancer, cT3N1M0, and cStage III was admitted. His metabolic equivalents were 4 or more. Electrocardiogram (ECG), ultrasound cardiography, and hematological examinations revealed no severe abnormalities. Computed tomography (CT) showed highly calcified coronary arteries. We performed mediastinoscope-assisted transhiatal esophagectomy. Procedures in the mediastinum involving access from the neck and abdomen were completed uneventfully. Middle mediastinal lymph node dissection and gastrointestinal reconstruction with the patient in the prone position were scheduled for later. However, before the change in position, pulseless nonsustained ventricular tachycardia (VT) suddenly occurred and caused blood pressure to drop sharply to below 30 mmHg. The VT disappeared in approximately 20 s, and there was a return to sinus rhythm after cardiac resuscitation; however, the ECG showed a decrease in the ST segments of leads II, III, and aVF. Immediately thereafter, the patient was transferred to the cardiac catheterization laboratory for percutaneous coronary intervention. Cardiac catheterization revealed diffuse stenosis of 90% in the left anterior descending branch of the coronary artery (segment no. 6). Plain old balloon angioplasty and stent placement were performed. Dual antiplatelet therapy was needed. On the next day, thoracoscopic esophagectomy was performed in the left lateral decubitus position, followed by cervical esophagostomy in the supine position. He developed acute respiratory distress syndrome and thoracic aortic dissection on the 5th postoperative day (POD) and intraperitoneal bleeding on the 16th POD. On the 105th POD, laparoscopic-assisted cervical esophagogastric anastomosis was performed. Parkinson’s disease was diagnosed on the 126th POD. On the 313th POD, the patient was discharged.ConclusionSurgeons should be familiar with the guidelines for patient screening and management of intraoperative cardiac arrest. In patients with severe coronary artery calcification, further investigation such as coronary angiography CT may be necessary before esophagectomy. Furthermore, highly invasive surgery should be performed in well-equipped hospitals.

Detection of acute thoracic aortic dissection based on plain chest radiography and a residual neural network (Resnet)

Acute thoracic aortic dissection is a life-threatening disease, in which blood leaking from the damaged inner layer of the aorta causes dissection between the intimal and adventitial layers. The diagnosis of this disease is challenging. Chest x-rays are usually performed for initial screening or diagnosis, but the diagnostic accuracy of this method is not high. Recently, deep learning has been successfully applied in multiple medical image analysis tasks. In this paper, we attempt to increase the accuracy of diagnosis of acute thoracic aortic dissection based on chest x-rays by applying deep learning techniques. In aggregate, 3,331 images, comprising 716 positive images and 2615 negative images, were collected from 3,331 patients. Residual neural network 18 was used to detect acute thoracic aortic dissection. The diagnostic accuracy of the ResNet18 was observed to be 90.20% with a precision of 75.00%, recall of 94.44%, and F1-score of 83.61%. Further research is required to improve diagnostic accuracy based on aorta segmentation.

A Systematic Review of Acute Thoracic Aortic Dissections in Africa-The Need for a Registry.

In this systematic review, the available literature on the presentation and management of acute thoracic aortic dissections in Africa is examined. Though Africa has 17% of the world population, it accounts for approximately 1% of the available literature with much of our understanding coming from registries arising from the developed world, such as the International Registry of Acute Aortic Dissection. The literature from the African continent consists mainly of case reports, small case series, and few original studies. Case reports make an important contribution to our understanding of uncommon conditions but can skew our understanding of aortic dissections in this region by describing unusual presentations and management. In this review, we describe the available studies retrieved from large medical databases (Medline and Health Management Information Consortium) and motivate the need for national registries to provide a more accurate appreciation of the scope of the problem on this continent.

Aggressive Treatment of Acute Thoracic Aortic Dissection, Ulcers, and Intramural Hematomas Analysis

Acute aortic syndrome consists of dissection (TBAD), penetrating ulcer (PAU), and intramural hematoma (IMH), and thoracic endograft treatment (TEVAR) has become more aggressive to prevent aortic degeneration and rupture. The purpose of this study was to compare outcomes of TEVAR for acute phase TBAD to PAU/IMH.

Aortic root management in acute type A aortic dissection: A nationwide analysis.

Acute type A aortic dissection (ATAAD) is a life-threatening condition and surgical repair often includes aortic valve replacement (AVR). Aortic valve repair (AVr) is increasingly being reported with favorable outcomes from single-center experiences. This study examined national trends and outcomes of AVr in patients with ATAAD. Adults with a primary diagnosis of acute thoracic aortic dissection who underwent proximal aortic surgery from January 2016 to December 2017 were obtained from the National Inpatient Sample. Patients were stratified into an isolated aortic surgery group (no aortic valve procedure), concomitant AVR, or concomitant AVr groups. The primary outcome was in-hospital mortality and secondary outcomes included stroke, acute kidney injury, heart block, and bleeding. Propensity score matching was used to address patient and hospital-level confounders between AVR and AVr groups. In total, 5115 patients underwent surgery for ATAAD and were included. Overall, 3220 (63%) underwent isolated ATAAD repair, while 1120 (22%) had concomitant AVR, and 775 (15%) had concomitant AVr. In 455 propensity-matched pairs, there was no difference in mortality or stroke between AVr and AVR groups, however, heart block (1.1% vs. 7.5%, p < .001) and bleeding (65.9% vs. 81.3%, p < .001) were significantly less common among those who underwent AVr. Patients who underwent AVr had shortest LOS (11.9 vs. 13.5 days, p < .001). There were no differences in outcomes of AVr in ATAAD based on hospital size or teaching status. In selected patients, AVr is being performed safely in the setting of ATAAD with mortality and composite outcomes comparable to AVR.

CT angiogram field of view for suspected thoracic aortic dissection

Background: Computed tomography (CT) angiograms performed to look for acute thoracic aortic dissection routinely include the abdomen. Data collected over a two-year period (2018–2020) at Oxford University Hospitals found a 2.9% positive detection rate for thoracic aortic dissection and 1.7% of scans showed a thoracic aortic dissection extending below the diaphragm. Detecting potential abdominal extension is the primary reason for including the abdomen in the examination. The need to routinely scan the abdomen and pelvis for cases of suspected thoracic aortic dissection alone was therefore questioned.

Insulin Resistance Promotes the Formation of Aortic Dissection by Inducing the Phenotypic Switch of Vascular Smooth Muscle Cells.

BackgroundInsulin resistance (IR) plays a key role in the development of type 2 diabetes mellitus (T2DM) and is one of its most important characteristics. Previous studies have shown that IR and T2DM were independent risk factors for a variety of cardiovascular and cerebrovascular diseases. However, there are few studies on the relationship between IR and aortic dissection (AD). The goal of this research was to find evidence that IR promotes the occurrence of AD.MethodsThrough the statistical analysis, we determined the proportion of glycosylated hemoglobin (HbA1c) abnormalities (HbA1c > 5.7) in people with acute thoracic aortic dissection (ATAD) and compared the difference of messenger RNA (mRNA) and protein expression of GluT1 in the thoracic aorta of normal people and those with ATAD to find evidence that IR is a causative factor in AD. The mouse model of IR and AD and the IR model of human aortic vascular smooth muscle cells (HA-VSMC) were established. Real time-PCR (RT-PCR) and Western blotting were used to study the mRNA and protein expression. Hematoxylin and eosin (H&E), Masson, and elastic fiber staining, and immunofluorescence were used to study the morphological structure.ResultsThe proportion of HbA1c abnormalities in patients with ATAD was 59.37%, and the mRNA and protein expression of GluT1 were significantly lower than that in normal people. Fasting glucose concentration (FGC), serum insulin concentration (SIC), and the homeostasis model assessment of insulin resistance (HOMA-IR) of mice was obviously increased in the high-fat diet group and the protein expressions of Glut1 and GluT4 were reduced, indicating that the mouse IR model was successfully established. The incidence of AD was different between the two groups (IR: 13/14, Ctrl: 6/14), and the protein expression of MMP2, MMP9, and OPN were upregulated and SM22 and α-SMA were downregulated in mice. The expressions of mRNA and protein of GluT1 and SM22 in HA-VSMCs with IR were reduced and OPN was increased.ConclusionCombined results of clinical findings, mouse models, and cell experiments show that IR induced the phenotypic switching of vascular smooth muscle cells (VSMCs) from contractile to synthetic, which contributes to the occurrence of AD. It provides a basis for further research on the specific mechanism of how IR results in AD and a new approach for the prevention and treatment of AD.

Deep Learning Algorithms for Detecting Acute Thoracic Aortic Dissection on Plain Chest Radiography: A Retrospective Multicenter Study

Deep Learning Algorithms for Detecting Acute Thoracic Aortic Dissection on Plain Chest Radiography: A Retrospective Multicenter Study

Male-female differences in acute thoracic aortic dissection: a systematic review and meta-analysis.

OBJECTIVESThis study aims to systematically review published literature on male–female differences in presentation, management and outcomes in patients diagnosed with acute thoracic aortic dissection (AD).METHODSA systematic literature search was conducted for studies published between 1 January 1999 and 19 October 2020 investigating mortality and morbidity in adult patients diagnosed with AD. Patient and treatment characteristics were compared with odds ratios (ORs) and standardized mean differences and a meta-analysis using a random-effects model was performed for early mortality. Overall survival and reoperation were visualized by pooled Kaplan–Meier curves.RESULTSNine studies investigating type A dissections (AD-A), 1 investigating type B dissections (AD-B) and 3 investigating both AD-A and AD-B were included encompassing 18 659 patients. Males were younger in both AD-A (P < 0.001) and AD-B (P < 0.001), and in AD-A patients males had more distally extended dissections [OR 0.57, 95% confidence interval (CI) 0.46–0.70; P < 0.001]. Longer operation times were observed for males in AD-A (standardized mean difference 0.29, 95% CI 0.17–0.41; P < 0.001) while male patients were less often treated conservatively in AD-B (OR 0.65, 95% CI 0.58–0.72; P < 0.001). The pooled early mortality risk ratio for males versus females was 0.94 (95% CI 0.84–1.06, P = 0.308) in AD-A and 0.92 (95% CI 0.83–1.03, P = 0.143) in AD-B. Pooled overall mortality in AD-A showed no male–female difference, whereas male patients had more reinterventions during follow-up.CONCLUSIONSThis systematic review shows male–female differences in AD patient and treatment characteristics, comparable early and overall mortality and inconsistent outcome reporting. As published literature is scarce and heterogeneous, large prospective studies with standardized reporting of male–female characteristics and outcomes are clearly warranted. Improved knowledge of male–female differences in AD will help shape optimal individualized care for both males and females.Clinical registration numberPROSPERO, ID number: CRD42020155926.

Aortic dissection and ruptures in adult congenital heart disease in Texas from 2009 to 2019.

Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5-7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR. This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used. A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P < 0.001) and TADR2 (0.3% vs 0.04%; P < 0.001). Adjusting for known TADR risk factors, CHD had an odds ratio of 1.69 (95% confidence interval: 1.09-2.63; P = 0.020) for TADR1 and an odds ratio of 1.69 (95% confidence interval: 0.99-2.88; P = 0.056) for TADR2. No in-hospital deaths were found in patients with CHD with TADR. ACHD discharges had a higher frequency of TADR versus the general population (0.9-1.2 vs 0.3-0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk.

UNUSUAL PRESENTATION OF AORTIC DISSECTION WITH RIGHT CALF PAIN

TOPIC: Cardiothoracic Surgery TYPE: Medical Student/Resident Case Reports INTRODUCTION: The incidence rate of acute type A aortic dissection is between 2.1 and 16.3 per 100,000 persons per year. Over 90% of patients with acute aortic dissection present with chest or back pain. In the case of an acute dissection, mortality can be almost 50% within the first 48 hours of symptom onset, 21 to 49% of the patients with aortic dissection die before reaching the hospital. Therefore, prompt diagnosis and treatment is imperative for survival. Aortic dissection can be easily missed in the settings of atypical presentation. We are presenting a case of a 48-year-old male who presented with right calf pain as a symptom of acute aortic dissection. CASE PRESENTATION: The patient is a 48 year old male with no known medical history presented with chief complain of right calf pain. He woke up from his sleep due to severe throbbing pain in his right calf that radiated to the calf with associated paresthesia. He denied recent trauma, prolonged period of immobilization, recent travel, known history of Baker's cyst, insect bites or exertion. Upon further questioning, the patient did report chronic back pain. Vitally, his blood pressure was elevated at 216/126 with no significant difference between both arms, no tachycardic, and not hypoxic. Clinically, his right lower calf is swollen with tortuous vein, not tense. Distal pulses are palpable with a sensory deficit at the dorsum of the right foot. Laboratory studies were only significant of a D-dimer of 32,279. Due to the elevated D-dimer, CTA chest and abdominal CT Aorta/Iliofemoral angiography was obtained that revealed an acute Stanford type A dissection of the thoracoabdominal aorta, large fusiform dissecting aneurysm of the distal abdominal aorta extending into bilateral common iliac arteries with absent distal runoff enhancement to the right foot, indicative of critical limb ischemia. An esmolol drip was quickly started and transferred to high-level care facility for an emergent surgical procedure. DISCUSSION: Our patient presented with an acute right leg pain that prompted us to get a D-dimer to further evaluate for an acute deep venous thrombosis. An elevated D-dimer has been found to have 96.7% sensitivity for aortic dissection, the significantly elevated d-dimer levels prompted an emergent CTA of the chest and abdomen for evaluation of dissection. This case was not considered "text book," since patient did not complain of the typical "tearing" chest pain radiating to the back to be concerned aortic dissection. CONCLUSIONS: Aortic dissection is a life-threatening disease, immediate evaluation can save a patient's life. Since many of these cases are often missed due to atypical presentation, it is imperative to develop awareness regarding the various ways an acute aortic dissection can disguise itself as. This case elucidates the importance of critical limb ischemia and a significantly elevated D-dimer due to an acute aortic dissection. REFERENCE #1: Inga H. Melvinsdottir, Sigrun H. Lund, Bjarni A. Agnarsson, Kristinn Sigvaldason, Tomas Gudbjartsson, Arnar Geirsson, The incidence and mortality of acute thoracic aortic dissection: results from a whole nation study, European Journal of Cardio-Thoracic Surgery, Volume 50, Issue 6, December 2016, Pages 1111–1117, https://doi.org/10.1093/ejcts/ezw235 REFERENCE #2: Wundram, M., Falk, V., Eulert-Grehn, JJ. et al. Incidence of acute type A aortic dissection in emergency departments. Sci Rep 10, 7434 (2020). https://doi.org/10.1038/s41598-020-64299-4 DISCLOSURES: No relevant relationships by Mary Dickow, source=Web Response No relevant relationships by Padmini Giri, source=Web Response No relevant relationships by Verisha Khanam, source=Web Response No relevant relationships by Sarwan Kumar, source=Web Response

THORACOABDOMINAL AORTIC DISSECTION: ATYPICAL PRESENTATION AND MULTIORGAN DAMAGE

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Acute Aortic Dissection (AoD) is the disruption of the tunica intima and tunica media of the aortic wall forming a blood-filled dissection plane dividing into "true" and "false" lumen. We categorize AoD as Type A and Type B (Stanford classification) based on the part of the aorta involved. However, an accurate diagnosis is difficult due to inconsistent clinical presentations (typically present with tearing chest pain radiating to the back). This is a unique clinical case of an atypical presentation with a complicated clinical course. CASE PRESENTATION: A 53-year-old man with untreated hypertension presented to the emergency room (ER) with worsening right lower extremity (RLE) pain and numbness for 6 days, aggravated with ambulation along with right lower quadrant (RLQ) abdominal pain. He endorses extensive tobacco, alcohol, marijuana, and cocaine use. In the ER, the blood pressure was 275/142 mmHg, biventricular hypertrophy on the EKG. He also had RLQ tenderness and diminished pulses in the RLE. The abdominal computed tomography (CT) discovered a distal abdominal aortic division. The larger false lumen dissected the left renal artery, left common iliac artery, with thrombosis of the left internal iliac artery. The narrow true lumen minimizes perfusion to the RLE and the right kidney. CT angiography of the chest confirmed a Stanford Type B aortic dissection with the intimal tear distal to the left subclavian artery. Intravenous Nicardipine and Esmolol drip controlled the blood pressure, followed by a femorofemoral bypass to improve RLE perfusion. Post-operative contrast nephropathy improved on day 10. Subsequently, a persistent ileus due to poor intestinal perfusion was corrected by transverse loop colostomy on day 15. On day 20, the patient was discharged with oral antihypertensive medications. DISCUSSION: The incidence of AoD is 5-30 per million people per year. 38% of ER presentations are missed; 28% are diagnosed at autopsy. Hypertension, atherosclerosis, cocaine abuse, cardiac surgeries, and systemic inflammatory syndromes can increase risk. Sullivan et al. suggest that a physician suspects AoD in 86% of patients with chest and back pain, 45% with only chest pain, and only 8% with abdominal pain. Our case is a unique combination of an atypical presentation (abdominal pain, leg numbness) with significant malperfusion of RLE, small bowel, and the right kidney (IRAD registry). For Type B dissection, medical management is preferred followed by surgical techniques to improve perfusion. Estimated inpatient mortality is 30% in proximal dissection and 10% in distal dissection. After the acute phase, the 10-year survival rate for AoD is <50%. CONCLUSIONS: Even with atypical symptoms, AoD and associated complications were successfully diagnosed and treated. Even with current surgical and medical advancements, controversies regarding diagnosis, the timing of repair, and type of repair still exist. REFERENCE #1: Cebicci, Huseyin & Salt, Omer & Gurbuz, Sukru & Sahin, Taner & Cumaoglu, Mustafa & Koyuncu, Serhat. (2014). Atypical presentations of aortic dissections: A case series. Acta Medica Mediterranea. 30. 85-90. REFERENCE #2: Sullivan PR, Wolfson AB, Leckey RD, Burke JL. Diagnosis of acute thoracic aortic dissection in the emergency department. Am J Emerg Med. 2000 Jan;18(1):46-50. doi: 10.1016/s0735- 6757(00)90047-0. PMID: 10674531 REFERENCE #3: Crawford, Todd C, et al. "Malperfusion Syndromes in Aortic Dissections." Vascular Medicine, vol. 21, no. 3, 2016, pp. 264–273., doi:10.1177/1358863x15625371 DISCLOSURES: No relevant relationships by Saurabh Chokshi, source=Web Response No relevant relationships by David Goldgrab, source=Web Response No relevant relationships by Devansh Patel, source=Web Response no disclosure on file for Jorge Perez

Human SMAD4 Genomic Variants Identified in Individuals with Heritable and Early-Onset Thoracic Aortic Disease.

Thoracic aortic aneurysms (TAAs) that progress to acute thoracic aortic dissections (TADs) are life threatening vascular events that have been associated with altered transforming growth factor (TGF) β signaling. In addition to TAA, multiple genetic vascular disorders, including hereditary hemorrhagic telangiectasia (HHT), involve altered TGFβ signaling and vascular malformations. Due to the importance of TGFβ, genomic variant databases have been curated for activin receptor-like kinase 1 (ALK1) and endoglin (ENG). This case report details seven variants in SMAD4 that are associated with either heritable or early onset aortic dissections and compares them to pathogenic exon variants in gnomAD v2.1.1. The TAA and TAD variants were identified through whole exome sequencing of 346 unrelated heritable thoracic aortic disease (HTAD) and 355 individuals of early onset (age ≤ 56 years old) of thoracic aortic dissection (ESTAD). An allele frequency filter of less than 0.05% was applied in the Genome Aggregation Database (gnomAD exome v2.1.1) with a combined annotation dependent depletion score (CADD) greater than 20. These seven variants also have a higher REVEL score (>0.2), indicating pathogenic potential. Further in vivo and in vitro analysis is needed to evaluate how these variants affect mRNA stability and SMAD4 protein activity in association with thoracic aortic disease.

Ultrasound diagnosis of dissecting thoracic aortic aneurysms: procedure with a handheld device and a video-illustrated case.

Acute thoracic aortic dissection is an uncommon, although not rare, life-threatening condition. With protean signs and symptoms that often suggest more common cardiac or pulmonary conditions, it can be difficult to diagnose. Ultrasound has proven useful in making the correct diagnosis. This case demonstrates that training gained using standard ultrasound machines can be easily and successfully adapted to newer handheld ultrasound devices. The examination technique using the handheld device is illustrated with photos and a video.

Acute thoracic aortic dissection: a case report

Introduction: Aortic dissection (AD) is the most common aortic emergency, result in a catastrophic outcome. Patients usually asymptomatic, some of them present with sudden severe, tearing or sharp back or anterior chest pain and haemodynamic disturbance. Early and accurate diagnosis and treatment determine the patient outcome.Case Report: A case of 40 years old man complained of acute, sharp, chest pain since 4 days before admission which worsen instead of medical treatment, with a history of smoking since a few years ago. A vital sign was within normal limit. Laboratory result showed high CKMB, troponin T, LDH result. Occasional ventricular extrasystoles and inferior ischaemic was found in the ECG examination. From echocardiography, the patient suspected to have aortic dissection Stanford type A since an intimal flap on ascending aorta was found, with left ventricle hypertrophy, dilatation of right atrium and right ventricle, severe aortic regurgitation, moderate mitral regurgitation, severe tricuspid regurgitation, moderate pulmonary regurgitation and moderate pulmonary hypertension. From plain radiography, the patient showed mediastinum widening and cardiomegaly, CT angiography showed Stanford type A aortic dissection (DeBakey type I). The patient refused to seek further treatment for operation as suggested.Conclusion: It is still challenging to differentiate aortic dissection (AD) and another acute chest emergency. Since clinical presentation and laboratory result may be similar between those illnesses. Therefore careful history taking and further imaging examination should be considered to increase the patient outcome.