Cystic Medial Necrosis Research Articles

A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report

BackgroundLiver transplantation is the definitive therapy for patients with decompensated cirrhosis. Marfan syndrome is a systemic inheritable connective tissue disease associated with fibrillin-1 gene mutations, which cause abnormalities in connective tissue. Vascular changes due to Marfan syndrome occur mostly in the main vessels due to the high amount of connective tissue within the vessel wall and the high pressure and blood flow to which they are exposed. The incidence of changes in visceral arteries is about 0.42% and usually presents with cystic medial necrosis. This report is the first deceased-donor liver transplantation with a donor with Marfan syndrome with a history of abdominal surgery.Case presentationA patient in his 50s underwent liver transplantation for decompensated alcoholic cirrhosis. The donor, a 50s male with Marfan syndrome, was diagnosed with brain-death due to a cerebral hemorrhage caused by a cerebral aneurysm. The donor’s clinical presentation as Marfan syndrome was aortic dissection, with multiple surgical procedures performed from the aortic root to the abdominal aorta. An intraoperative biopsy of the hepatic artery showed no abnormality, so this organ was considered appropriate. The surgery was completed without any problems of the arterial anastomosis. The patient’s postoperative course was uneventful, and he was transferred to a hospital for recuperation on the 18th postoperative day. One year after the surgery, the patient is still alive without any complications from the transplantation or arterial problems.ConclusionsEven if the patient had a history of surgery for vascular anomalies extending to the abdominal aorta due to Marfan syndrome, the patient can be a donor for liver transplantation under appropriate judgment, including intraoperative biopsy.

Selenoprotein deficiency disorder predisposes to aortic aneurysm formation

Aortic aneurysms, which may dissect or rupture acutely and be lethal, can be a part of multisystem disorders that have a heritable basis. We report four patients with deficiency of selenocysteine-containing proteins due to selenocysteine Insertion Sequence Binding Protein 2 (SECISBP2) mutations who show early-onset, progressive, aneurysmal dilatation of the ascending aorta due to cystic medial necrosis. Zebrafish and male mice with global or vascular smooth muscle cell (VSMC)-targeted disruption of Secisbp2 respectively show similar aortopathy. Aortas from patients and animal models exhibit raised cellular reactive oxygen species, oxidative DNA damage and VSMC apoptosis. Antioxidant exposure or chelation of iron prevents oxidative damage in patient’s cells and aortopathy in the zebrafish model. Our observations suggest a key role for oxidative stress and cell death, including via ferroptosis, in mediating aortic degeneration.

Machine learning in assessing the association between the size and structure of the ascending aortic wall in patients with aortic dilatation of varying severity

Aim. To assess the association between pathological ascending aortic (AA) wall changes and its planimetric characteristics in non-syndromic non-familial (sporadic) aneurysm and dilation of the AA.Material and methods. The study included 174 patients with sporadic aneurysms and dilation of the AA, who underwent elective surgery between January 2010 and July 2015 and were divided into 2 groups: patients with AA aneurysm (AAA) (AA diameter >50 mm) and tricuspid aortic valve (AV) with significant aortic stenosis (AS) or regurgitation (AR) (AAA group, n=120), and persons with borderline AA dilatation (AA diameter 45-50 mm), associated with a bicuspid aortic valve (BAV) and significant AS (BD group, n=54). Standard paraclinical investigations and pathological examination of the VA wall were used. Statistical processing was carried out in the SPYDER 4.1.5 environment (Python 3.8), and included univariate correlation analysis, logistic regression analysis, as well as supervised machine learning (ML) methods (support vector machine, k-nearest neighbor method, random forest).Results. Logistic regression revealed positive associations between AA atherosclerosis and age, cystic medial necrosis (CMN) and sinus of Valsalva (SV) diameters. The support vector machine method demonstrated a tendency towards AA expansion at the SV level in individuals with CMN (accuracy, 60,5%), as well as towards expansion of the tubular AA in atherosclerosis (accuracy, 79,2%). During the random forest analysis, the first stage was to construct decision trees to predict three following outcomes: the presence of CMN, atherosclerosis, or normal aortic structure. The model accuracy was 64,2%. Next, the variables "CMN" and "atherosclerosis" were combined, and prediction was made for the outcomes "normal AA wall structure" and "pathological AA wall structure". The model accuracy was 73,5%.Conclusion. The use of ML opens up new opportunities for predicting aortopathy and a patient-centered approach to treatment. In AR, a more aggressive AA intervention is warranted. To predict aortopathies, thoracic aorta diameters indexed to body surface area should not be used. Aortic wall sampling (circular section) followed by a continuous pathological examination may be promising.

ASCENDING AORTIC ANEURYSMS: SURGERY, RMODELLING & AI

ASCENDING AORTIC ANEURYSMS: SURGERY, RMODELLING & AI

Abstract 12536: Type A Aortic Dissection in a Young Marfanoid Filipino Male Eventually Developing Fungal Mediastinitis: A Case Report

Introduction: Aortic Dissection (AD) results from a tear between the layers of the aorta. This is often seen among older males or those with heritable connective tissue disorders, and it mostly presents as chest pain. Management involves surgical repair wherein post procedure complications are to be expected. Mediastinitis, or deep sternal wound infection, however, is likewise unusual. Case Presentation: We present a case of a 29-year-old Filipino male presenting with sudden diffuse abdominal pain; he had no comorbidities and had no significant history suggestive of connective tissue disorders. Physical examination was mostly unremarkable. CT Aortogram revealed presence of dissection from the proximal ascending aorta up to the common iliac arteries, with the entry tear measuring 8.5mm at the level of T5, with extension to the proximal branches of the aortic arch, both common carotid arteries, right renal, right internal and external iliac arteries. Echocardiogram also showed a normal aortic valve but with dilated sinus of Valsalva(4.5cm, z-score 5.13). The patient underwent a Modified Bentall Procedure with Cabrol Shunt, with delayed closure of operative site due to extensive bleeding. Cystic medial necrosis was not seen on biopsy. After developing persistent post-operative fever, repeat chest CT showed a fluid collection in the mediastinum encasing the ascending aorta and main pulmonary trunk; culture of abscess fluid was positive for Candida tropicalis . He was then given prolonged parenteral followed by oral antifungal therapy and subsequently discharged improved. Discussion: Vascular causes of abdominal pain should be considered in patients with scant history and physical findings. Major vascular surgery has expected complications such as bleeding and infection; the management of such complications should incorporate a multidisciplinary approach as in such cases, a trial of medical management may be safer than repeat surgery.

Differences in Aortic Histopathology in Patients Undergoing Valve Reimplantation Surgery for Various Clinical Syndromes.

Objectives The study aims to investigate aortic histopathologic differences among patients undergoing aortic valve reimplantation, suggest different mechanisms of aortic root aneurysm pathogenesis, and identify factors associated with long-term success of reimplantation. Methods From 2006 to 2017, 568 adults who underwent reimplantation for repair of aortic root aneurysm, including patients with tricuspid aortic valves with no connective tissue disease (TAV/NoCTD, n = 314/568; 55.3%), bicuspid aortic valves (BAVs, n = 86/568; 15.1%), or connective tissue disease (CTD, n = 177/568; 31.2%), were compiled into three comparison groups. Patients with both BAV and CTD ( n = 9/568; 1.6%) were omitted to increase study power. Patient records were analyzed retrospectively, focusing on pathology reports, which were available for 98.42% of patients, and were classified based on their descriptions of aortic tissue samples, primarily from the noncoronary sinus. Mean follow-up time available for patients was 2.97 years. Results Aortitis, medial fibrosis, and smooth muscle loss were more common histopathologic findings in patients with TAV/NoCTD than in patients with BAV and CTD ( p < 0.05). Cystic medial degeneration was most often found in patients with CTD, then TAV/NoCTD, and least in BAV ( p < 0.01). Increases in mucopolysaccharides were found more often in the BAV group than in the TAV/NoCTD and CTD groups ( p < 0.01). There were no differences in the frequency of elastic laminae fragmentation/loss across these three groups. Among all patients, 1.97% ( n = 11/559) had an unplanned reintervention on the aortic valve after reimplantation, but no significant demographic or histopathologic differences were identified. Conclusion Despite some common histopathologic features among patients undergoing aortic valve reimplantation, there were enough distinguishing features among aortic tissue samples of TAV/NoCTD, BAV, and CTD patients to suggest that these groups develop root aneurysms by different mechanisms. No histopathologic features were able to predict the need for late reintervention on the aortic valve.

Point on the Aortic Bicuspid Valve.

Background—Bicuspid aortic valve (BAV) disease is the most prevalent congenital heart disease in the world. Knowledge about its subtypes origin, development, and evolution is poor despite the frequency and the potential gravity of this condition. Its prognosis mostly depends on the risk of aortic aneurysm development with an increased risk of aortic dissection. Aims—This review aims to describe this complex pathology in way to improve the bicuspid patients’ management. Study design—We reviewed the literature with MEDLINE and EMBASE databases using MeSH terms such as “bicuspid aortic valve”, “ascending aorta”, and “bicuspid classification”. Results—There are various classifications. They depend on the criteria chosen by the authors to differentiate subtypes. Those criteria can be the number and position of the raphes, the cusps, the commissures, or their arrangements regarding coronary ostia. Sievers’ classification is the reference. The phenotypic description of embryology revealed that all subtypes of BAV are the results of different embryological pathogenesis, and therefore, should be considered as distinct conditions. Their common development towards aortic dilatation is explained by the aortic media’s pathological histology with cystic medial necrosis. At the opposite, BAV seems to display a profound genetic heterogeneity with both sporadic and familial forms. BAV can be even isolated or combined with other congenital malformations. Conclusions—All those characteristics make this pathology a highly complex condition that needs further genetic, embryological, and hemodynamic explorations to complete its well described anatomy.

Complicated Intramural Hematoma with Medionecrosis of the Aortic Wall.

Medionecrosis and medial degeneration are rare complications associated with intramural hematomas (IMHs). We present a case of a 69-year-old Asian female with an IMH with medionecrosis and medial degeneration of the aortic wall. The patient underwent successful surgical intervention, and pathological findings were significant for cystic medial degeneration of the aortic wall.

Isolated Dissection of the Ductus Arteriosus Associated with Sudden Unexpected Intrauterine Death.

We report five cases of sudden intrauterine death due to premature closure of the ductus arteriosus. In four cases, this was caused by dissecting the hematoma of the ductus arteriosus with intimal flap and obliteration of the lumen. In one case, the ductus arteriosus was aneurysmatic, with lumen occlusion caused by thrombus stratification. No drug therapy or free medication consumption were reported during pregnancy. The time of stillbirth ranged between 26 and 33 gestational weeks. We performed TUNEL analysis for apoptosis quantification. The dissecting features were intimal tears with flap formation in four of the cases, just above the origin of the ductus arteriosus from the pulmonary artery. The dissecting hematoma of the ductus arteriosus extended downward to the descending aorta and backward to the aortic arch with involvement of the left carotid and left subclavian arteries. TUNEL analysis showed a high number of apoptotic smooth muscle cells in the media in two cases. Abnormal ductal remodeling with absence of subintimal cushions, lacunar spaces rich in glycosaminoglycans (cystic medial necrosis), and smooth muscle cell apoptosis were the pathological substrates accounting for failure of remodeling process and dissection.

Bicuspid Aortic Valve in Infants, Children, and Adolescents: A Review for Primary Care Providers.

1. Talha Niaz, MBBS* 2. Jonathan N. Johnson, MD*,† 3. Frank Cetta, MD*,† 4. Timothy M. Olson, MD*,† 5. Donald J. Hagler, MD*,† 1. *Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, and 2. †Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN * Abbreviations: AHA : : American Heart Association BAV : : bicuspid aortic valve TTE : : transthoracic echocardiography Bicuspid aortic valve is the most common congenital heart defect in children, adolescents, and adults. Primary care providers play an important role in screening, referral, and follow-up of these patients and should be aware of the family screening guidelines, sports participation recommendations, and periodic follow-up requirements for adequate surveillance of the complications that arise from bicuspid aortic valve. After reading this article, readers should be able to: 1. Describe the epidemiology and anatomy of bicuspid aortic valve (BAV). 2. Understand the clinical presentation and diagnosis of BAV in infants, children, and adolescents. 3. Identify the various complications of BAV disease. 4. Discuss the management and follow-up requirements for BAV. 5. Analyze the family screening and sports participation guidelines for patients with BAV. Bicuspid aortic valve (BAV) is the most common congenital heart defect in children, adolescents, and adults. (1) It is a heterogeneous disease that affects both the aortic valve and the aorta. It can lead to many complications, including aortic valve stenosis, regurgitation, or endocarditis. (2)(3) It also can lead to dilation of the aorta, predisposing individuals to a significantly higher risk of aortic aneurysm and dissection. (4) Although most individuals with BAV present with these long-term complications during adulthood, a considerable number of patients may also present during childhood and adolescence with early-onset disease; that may require interventions in up to 12% to 15% of the patients. (5)(6) Therefore, patients with BAV require lifelong follow-up and surveillance. BAV has multiple implications in terms of sports participation and family screening, making it an important subject for primary care providers. This article reviews the anatomy, genetics, presentation, diagnosis, …

Causes and mechanisms of sudden cardiac death in children

The paper presents basic morphological criteria of the diagnosis of sudden cardiac death related to different diseases in young people. Attention is drawn to cardiomyopathies, channelopathies, connective tissue dysplasias, Marfan syndrome with cystic medial necrosis, sudden arrhythmic death. Initiating events in sudden cardiac death are discussed. Besides morphological manifestations, the paper dwells upon major genetic events and clinical presentation of the diseases. It is based on both existing publications and own data.

A death due to acute aortic dissection

Acute aortic syndrome (AAS) is a term used to describe a collection of life-threatening aortic disease conditions that have similar presentations, but differ in their demographic, pathological, clinical and other characteristics. The exact number of deaths due to AAS is unknown. However studies suggest that the majority of deaths in AAS are due to acute aortic dissection. A 51-year-old male developed sudden onset chest pain while he was taking a bath in the evening. It was a sharp, tearing type chest pain, which was radiating to the left side of his neck. On admission to hospital his low blood pressure was found to be low. He suffered cardiac arrest a few minutes later and died without regaining consciousness. Post-mortem examination revealed a massive haemopericardium (600 ml). Aortic dissection was identified extending from the aortic root to the lower part of the descending abdominal aorta. The thickest point of the dissection was 1.5 cm. There was an intimal tear inferiorly. The dissection extended through the aortic arch to the descending aorta ending 1 cm above the renal artery branching point. Sections of the aorta showed cystic medial degeneration with prominent pools of mucin. Mild atheroma was seen in some sections. The presence of aortic dissection was confirmed. The cause of death was given as haemopericardium due to ruptured aortic dissection. Acute aortic dissection may mimic other cardiovascular conditions, especially ischemic cardiac events. Therefore, the clinical suspicion and a comprehensive history-taking are the key for an early diagnosis. Careful dissection of the aorta and its branches is essential when making a post-mortem diagnosis of aortic dissection. Proper histopathology examination of the aorta assisted immensely in confirming the diagnosis.

Age-Related Changes in the Canine Aorta.

Degenerative changes in the aorta are commonly observed in both dogs and humans, and those changes that occur with age morphologically overlap with those observed in genetic or degenerative diseases. Therefore, recognition of age-related aortic changes is important for diagnosticians, as such histologic findings should be distinguished from lesions of specific diseases. The aortas from 37 dogs without clinical cardiovascular disease ranging in age from 2 months to 15 years were divided into 3 cohorts to assess age-relatedness, and evaluated histologically using standardized nomenclature and diagnostic criteria adapted and modified from the human literature. We found that the histopathologic severity scores for intimal thickening, translamellar medial fibrosis, loss of smooth muscle cell nuclei, and medial microcalcification were higher in older dogs, whereas the scores for both intralamellar and translamellar mucoid extracellular matrix accumulation ("cystic medial necrosis") were not different among age groups. Dogs with translamellar medial fibrosis and aortic medial microcalcification were significantly older compared with dogs without these findings, while the presence of aortic medial chondro-osseous metaplasia was not related to age. Taken together, we demonstrate a range of age-related aortic histologic changes in dogs without clinical cardiovascular disease and suggest that integration of signalment and clinical data can aid in the differentiation of such findings from non-age-related disease processes.

Pleural changes in patients with pneumothoraces and Marfan syndrome.

BackgroundPatients with Marfan syndrome (MFS) often develop pneumothorax, but the features of pneumothorax in the context of MFS have not been well described in the literature. We clarified the clinical and histopathological characteristics of this condition in these patients.MethodsPatients with MFS were selected from among all patients who underwent surgery for pneumothorax, between December 1991 and January 2015, in our hospital. We studied the histopathological characteristics of the resected lungs as well as the clinical features of the selected patients, including surgical findings and postoperative recurrence status.ResultsThere were 966 operations underwent pneumothorax-related surgeries in our hospital. A total of 16 operations (1.66%) were performed on patients with MFS in 11 cases. In this study, 9 patients (6 men, 3 women) were included. Clinically, 7 patients (77.8%) had bilateral pneumothoraces and 4 (44.4%) exhibited postoperative recurrent pneumothoraces. Pathologically, the resected pulmonary bullae exhibited blood vessel cystic medial degeneration (55.6% of cases), calcification (55.6% of cases), and demonstrated elastic fiber fragmentation and degeneration (all cases).ConclusionsAs in few previous reports, many patients with MFS develop bilateral or postoperative recurrent pneumothoraces. In many patients, characteristic changes in the pulmonary bullae, possibly caused by degenerated elastic fibers, were observed.

Comparison of microstructural alterations in the proximal aorta between aortic stenosis and regurgitation

ObjectiveWe aimed to analyze the association among flow patterns, gene expression, and histologic alterations of the proximal aorta in patients with aortic valve disease. MethodsA total of 131 patients referred for aortic valve replacement were grouped by valve dysfunction (aortic stenosis vs aortic regurgitation) and valve morphology (bicuspid vs tricuspid). On the basis of magnetic resonance imaging, aortic tissue from outer and inner curvature was collected for gene expression and histologic analysis. To identify differences in aortic remodeling, age- and sex-adjusted data for inflammation (CCL2, VCAM1, inflammation and atherosclerosis) and medial degeneration (COL1A1, ELN, fibrosis, elastin fragmentation, and cystic medial necrosis) were compared. ResultsFirst, we compared all patients with aortic regurgitation (n = 64) and patients with aortic stenosis (n = 67). In patients with aortic regurgitation, COL1A1 expression and all histologic markers were significantly increased. With respect to aortic diameter, all subsequent analyses were refined by considering only individuals with aortic diameter 40 mm or greater. Second, patients with bicuspid aortic valve were compared, resulting in a similar aortic diameter. Although patients with aortic regurgitation were younger, no differences were found in gene expression or histologic level. Third, valve morphology was compared in patients with aortic regurgitation. Although aortic diameter was similar, patients with regurgitant bicuspid aortic valve were younger than patients with regurgitant tricuspid aortic valve. Inflammatory markers were similar, whereas markers for medial degeneration were increased in patients with regurgitant tricuspid aortic valve. ConclusionsOur results indicate that the proximal aorta in patients with aortic regurgitation showed an increased inflammation and medial degeneration compared with patients with aortic stenosis. Refining both groups by valve morphology, in patients with bicuspid aortic valve, no difference except age was detected between aortic regurgitation and aortic stenosis. In patients with aortic regurgitation, tricuspid aortic valve revealed increased markers for medial degeneration but no differences regarding inflammatory markers.

Pathologic properties of SOD3 variant R213G in the cardiovascular system through the altered neutrophils function.

The SOD3 variant, SOD3R213G, results from substitution of arginine to glycine at amino acid 213 (R213G) in its heparin binding domain (HBD) and is a common genetic variant, reported to be associated with ischemic heart disease. However, little is understood about the role of SOD3R213G in innate immune function, and how it leads to dysfunction of the cardiovascular system. We observed pathologic changes in SOD3R213G transgenic (Tg) mice, including cystic medial degeneration of the aorta, heart inflammation, and increased circulating and organ infiltrating neutrophils. Interestingly, SOD3R213G altered the profile of SOD3 interacting proteins in neutrophils in response to G-CSF. Unexpectedly, we found that G-CSF mediated tyrosine phosphatase, SH-PTP1 was down-regulated in the neutrophils of SOD3R213G overexpressing mice. These effects were recovered by reconstitution with Wt SOD3 expressing bone marrow cells. Overall, our study reveals that SOD3R213G plays a crucial role in the function of the cardiovascular system by controlling innate immune response and signaling. These results suggest that reconstitution with SOD3 expressing bone marrow cells may be a therapeutic strategy to treat SOD3R213G mediated diseases.

Synchrotron Radiation-based X-ray phase-contrast imaging of the aortic walls in acute aortic dissection

ObjectiveSynchrotron radiation-based X-ray phase-contrast tomography (XPCT) imaging is an innovative modality for the quantitative analysis of three-dimensional morphology. XPCT has been used in this study to evaluate ascending aorta specimens from patients with acute type A aortic dissection (ATAAD) and to analyze the morphologic structure of the aortic wall in patients with this condition. MethodsAortic specimens from 12 patients were obtained during repairs for ATAAD and were fixed with formalin. Five patients had Marfan syndrome (MFS), and seven did not. In addition, six normal aortas were obtained from autopsies. Using XPCT (effective pixel size, 12.5 μm; density resolution, 1 mg/cm3), the density of the tunica media (TM) in each sample was measured at eight points. The specimens were subsequently analyzed pathologically. ResultsThe density of the TM was almost constant within each normal aorta (mean, 1.081 ± 0.001 g/cm3). The mean density was significantly lower in the ATAAD aortas without MFS (1.066 ± 0.003 g/cm3; P < .0001) and differed significantly between the intimal and adventitial sides (1.063 ± 0.003 vs 1.074 ± 0.002 g/cm3, respectively; P < .0001). The overall density of the TM was significantly higher in the ATAAD aortas with MFS than those without MFS (1.079 ± 0.008 g/cm3; P = .0003), and greater variation and markedly different distributions were observed in comparison with the normal aortas. These density variations were consistent with the pathologic findings, including the presence of cystic medial necrosis and malalignment of the elastic lamina in the ATAAD aortas with and without MFS. ConclusionsXPCT exhibited differences in the structure of the aortic wall in aortic dissection specimens with and without MFS and in normal aortas. Medial density was homogeneous in the normal aortas, markedly varied in those with MFS, and was significantly lower and different among those without MFS. These changes may be present in the TM before the onset of aortic dissection.

Chronic dissection aneurysm of the aorta: clinical and pathologic study

An analysis of the clinical case of chronic dissection aortic aneurysm (DAA) in a 47-year-old man was conducted. Diagnosis of chronic DAA type A for Stanford is established at CT angiography of the thoracic and abdominal aorta with proven contrast enhancement. The operation of supracoronary prosthetics of the ascending department of the aorta was performed with full root method. During surgery, a chronic RAA of the ascending aorta with a diameter of about 5.5 cm was detected. The aorta was enlarged in the area of the root, whereas at the point of transition into the arc was of ordinary diameter. The aortic valve was three-lobed with signs of slight dilation of the fibrous ring. The ascending aorta was circularly mobilized and excised 1.0 cm above the coronary artery cells. The organized thrombus from the false lumen from the lateral wall of the ascending aorta (from the side of the superior vena cava) was removed. A proximal anastomosis was applied between the vascular prosthesis and the ascending aorta with a telescopic double continuous suture. Subsequently, a distal ascending aorta 1.5 cm below the shoulder-main trunk was dissected. Therefore, a distal anastomosis was applied between the distal ascending aorta and the vascular prosthesis with a telescopic double continuous suture. The postoperative period was satisfactory. On the 8th day of the postoperative period the patient was in a satisfactory condition discharged home.&#x0D; Pathomorphological examination of the operating material revealed segmental hyperplasia of the media with focal mucoid swelling, multiple cystic cavities, diffuse-focal fibrosis. The cavity of the dissection looked like a “two-stemmed” (two-channel) lumen, located on the border of adventitia and media, was filled with blood clots with the spread of hemorrhagic infiltration into the peri-adventitious fat. The new pseudocanal contained blood clots and fresh blood clots, and the old one was completely covered with endothelium with neointima formation. The aortic architecture is completely broken, the division into layers was absent. In the section of the irregular canal, the formation of a two-layer structure was observed – neointima, which differed from the intima of the true lumen of the aorta by the number of smooth muscle cells and heterogeneous accumulation of components of the extracellular matrix. In the middle layers, connective tissue remodeling of the aorta was detected in the form of chaotically located immature mesenchymal cells in combination with muscular and connective tissue hyperplasia, complete loss of elastic fibers, massive sclerosis of adventitia, and periadventitial layer. In sections of the aorta, out of stratification, its structure was preserved, there are signs of cystic medial necrosis.&#x0D; The revealed pathomorphological changes in the described case point to the processes of vascular remodeling, which explain the relative stability of DAA.

Resection of Kommerell\u2019s diverticulum in an infant with prenatal diagnosis of right aortic arch

BackgroundA right aortic arch is a congenital vascular anomaly that is present in up to 0.1% of pregnancies. The anomaly observed by fetal ultrasonography was recently reported to indicate vascular and chromosomal abnormalities that may complicate postnatal management.Case presentationWe report the successful resection of a Kommerell’s diverticulum with left subclavian artery transfer to the left carotid artery in a 5-month-old Japanese boy. The patient was prenatally diagnosed as having a right aortic arch, and a vascular ring was confirmed at 4 months of age with enhanced computed tomography. The pathology of the resected aortic wall revealed severe disruption and fragmentation of elastic fibers associated with a disarray of smooth muscle cells in the tunica media, and cystic medial necrosis with mucoid extracellular matrix deposition.ConclusionThese abnormal pathological findings supported the resection of Kommerell’s diverticulum at this point of time, and division of the ligamentum arteriosus alone was not recommended. Early intervention in this condition once the diagnosis is made may thus be advocated. The fetal diagnosis of a right aortic arch may provide a clue to the possibility of a vascular ring.

Immediate of Impact Prosthetic Graft Replacement of the Ascending Aorta on Circumferential Strain in the Descending Aorta

Prosthetic replacement of the ascending aorta (AA) can potentially modify energy propagation to the distal aorta and contribute to adverse aortic remodelling. This preliminary study employed intra-operative transoesophageal echocardiography (TOE) to assess the immediate impact of prosthetic graft replacement of the AA on circumferential strain in the descending aorta. Intra-operative TOEs in patients undergoing AA graft replacement were analysed for circumferential strain, fractional area change (FAC), dimensions (end diastolic area [EDA], and end systolic area [ESA]) in the descending aorta immediately before and after graft replacement. Deformation was assessed via global peak circumferential aortic strain (CAS), together with pulse pressure corrected strain, time to peak strain (TTP), and aortic distensibility. Forty-five patients undergoing AA replacement with prosthetic graft (91% elective) were studied. Following grafting, descending thoracic aortic circumferential strain increased (6.3 ± 2.8% vs. 8.9 ± 3.4%, p = .001) paralleling distensibility (5.7 [3.7–8.6] 10−3 mmHg vs. 8.5 [6.4–12.4] 10−3 mmHg, p < .001). Despite slight increments in post graft left ventricular ejection fraction (LVEF) (52.3 ± 10.8% vs. 55.0 ± 11.9, p < .001), stroke volume was similar (p = .41), and magnitude of increased strain did not correlate with change in stroke volume (r = −.03, p = .86), LVEF (r = .18, p = .28), or pulse pressure (r = .28, p = .06). Descending aortic size (EDA 4 [2.7–4.6] cm2vs. 3.7 [2.5–5] cm2, p = .89; ESA 4.3 [3.2–5.3] cm2vs. 4.5 [3.3–5.8] cm2, p = .14) was similar pre- and post graft. In subgroup analysis, patients with cystic medial necrosis had a significantly higher post procedure CAS than patients with atherosclerotic aneurysms (9.7 ± 3.5% vs. 7.0 ± 2.3%, p = .03). Prosthetic graft replacement of the AA increases immediate aortic circumferential strain of the descending aorta, particularly in patients with cystic medial necrosis. Our findings suggest that grafts augment energy transfer to the distal aorta, a potential mechanism for progressive distal aortic dilation and/or dissection.