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2.
J Vet Cardiol ; 52: 61-67, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38430823

ABSTRACT

A dog was presented for lameness, fever, and extreme lethargy. On physical exam, a new heart murmur, arrhythmia, and joint effusion were detected. These findings were not detected two months prior. A diagnostic work-up confirmed septic suppurative inflammation in multiple joints. Echocardiogram revealed aortic valvular endocarditis along with a communication, as a consequence of a fistula, that extended from just below the aortic sinotubular junction to the left atrial lumen. Due to a poor prognosis, humane euthanasia was elected. Necropsy and histopathology confirmed infective endocarditis of the aortic valve and an aorto-left atrial fistulous tract extending from the left coronary sinus of the aortic valve to the lumen of left atrium.


Subject(s)
Dog Diseases , Echocardiography , Heart Atria , Animals , Dogs , Dog Diseases/pathology , Dog Diseases/diagnostic imaging , Heart Atria/pathology , Heart Atria/diagnostic imaging , Echocardiography/veterinary , Fistula/veterinary , Fistula/diagnostic imaging , Endocarditis, Bacterial/veterinary , Endocarditis, Bacterial/complications , Endocarditis, Bacterial/diagnostic imaging , Endocarditis, Bacterial/pathology , Vascular Fistula/veterinary , Vascular Fistula/diagnostic imaging , Vascular Fistula/complications , Male , Aortic Diseases/veterinary , Aortic Diseases/diagnostic imaging , Aortic Diseases/pathology , Aortic Diseases/complications , Endocarditis/veterinary , Endocarditis/complications , Endocarditis/diagnostic imaging , Endocarditis/pathology , Heart Diseases/veterinary , Heart Diseases/diagnostic imaging , Heart Diseases/pathology , Heart Diseases/etiology , Heart Diseases/complications , Female
3.
Nefrologia (Engl Ed) ; 44(2): 256-267, 2024.
Article in English | MEDLINE | ID: mdl-38555207

ABSTRACT

RATIONALE AND OBJECTIVES: Increased central (aortic) arterial stiffness has hemodynamic repercussions that affect the incidence of cardiovascular and renal disease. In chronic kidney disease (CKD) there may be an increase in aortic stiffness secondary to multiple metabolic alterations including calcification of the vascular wall (VC). The objective of this study was to analyze the association of central aortic pressures and aortic stiffness with the presence of VC in abdominal aorta (AAC) and coronary arteries(CAC). MATERIALS AND METHODS: We included 87 pacientes with CKD stage 3 and 4. Using applanation tonometry, central aortic pressures and aortic stiffness were studied. We investigated the association of aortic pulse wave velocity (Pvc-f) and Pvc-f adjusted for age, blood pressure, sex and heart rate (Pvc-f index) with AAC obtained on lumbar lateral radiography and CAC assessed by multidetector computed tomography. AAC and CAC were scored according to Kauppila and Agatston methods, respecti-vely. For the study of the association between Pvc-f index, Kauppila score, Agatston score, central aortic pressures, clinical parameters and laboratory data, multiple and logistic regression were used. We investigated the diagnosis performance of the Pvc-f index for prediction of VC using receiver-operating characteristic (ROC). RESULTS: Pvc-f and Pvc-f index were 11.3 ± 2.6 and 10.6 m/s, respectively. The Pvc-f index was higher when CKD coexisted with diabetes mellitus (DM). AAC and CAC were detected in 77% and 87%, respectively. Albuminuria (ß = 0.13, p = 0.005) and Kauppila score (ß = 0.36, p = 0.001) were independently associated with Pvc-f index. In turn, Pvc-f index (ß = 0.39, p = 0.001), DM (ß = 0.46, p = 0.01), and smoking (ß = 0.53; p = 0.006) were associated with Kauppila score, but only Pvc-f index predicted AAC [OR: 3.33 (95% CI: 1.6-6.9; p = 0.001)]. The Kauppila score was independently associated with the Agatston score (ß = 1.53, p = 0.001). The presence of AAC identified patients with CAC with a sensitivity of 73%, a specificity of 100%, a positive predictive value of 100% and a negative predictive value of 38%. The Vpc-f index predicted the presence of CAC [OR: 3.35 (95% CI: 1.04-10.2, p = 0.04)]. In the ROC curves, using the Vpc-f index, the AUC for AAC and CAC was 0.82 (95%CI: 0.71-0.93, p = 0.001) and 0.81 (95% CI: 0.67-0.96, p = 0.02), respectively. CONCLUSIONS: When stage 3-4 CKD coexists with DM there is an increase in aortic stiffness determined by the Vpc-f index. In stage 3-4 CKD, AAC and CAC are very prevalent and both often coexist. The Vpc-f index is independently associated with AAC and CAC and may be useful in identifying patients with VC in these territories.


Subject(s)
Aorta, Abdominal , Renal Insufficiency, Chronic , Vascular Calcification , Vascular Stiffness , Humans , Male , Female , Middle Aged , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/physiopathology , Vascular Calcification/diagnostic imaging , Vascular Calcification/physiopathology , Vascular Calcification/etiology , Aorta, Abdominal/diagnostic imaging , Aorta, Abdominal/physiopathology , Aged , Severity of Illness Index , Cross-Sectional Studies , Pulse Wave Analysis , Coronary Artery Disease/physiopathology , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/complications , Aortic Diseases/diagnostic imaging , Aortic Diseases/physiopathology , Aortic Diseases/complications , Aortic Diseases/etiology
4.
J Cardiothorac Surg ; 19(1): 112, 2024 Mar 09.
Article in English | MEDLINE | ID: mdl-38461352

ABSTRACT

BACKGROUND: Aortoesophageal fistula (AEF) is a rare condition characterized by communication between the aorta and esophagus. AEF caused by an esophageal foreign body is even rare, and there is currently no recommended standard treatment protocol. We report a case of delayed aortic rupture after the endoscopic removal of a fish bone, which was successfully treated with a combined approach of vascular stenting and thoracic surgery. CASE PRESENTATION: A 33-year-old man presented to the hospital after experiencing chest discomfort for 3 days following the accidental ingestion of a fish bone. Under endoscopic guidance, the fish bone was successfully removed, and the patient was subsequently admitted for medical therapy. On the fourth postoperative day, the patient suddenly developed hematemesis, and chest computed tomography angiography revealed the presence of an AEF. This necessitated urgent intervention; hence, thoracic surgery was performed and a vascular-covered stent was placed. Following the surgical procedure, the patient received active medical treatment, recovered well, and was successfully discharged from the hospital. CONCLUSIONS: In patients with esophageal perforation caused by foreign bodies, hospitalization for observation, computed tomography angiography examination, early use of antibiotics, and careful assessment of aortic damage are advised. Thoracic endovascular aortic repair and esophageal rupture repair may have benefits for the treatment of AEF.


Subject(s)
Aortic Diseases , Esophageal Fistula , Vascular Fistula , Male , Animals , Humans , Adult , Thoracic Surgery, Video-Assisted/adverse effects , Aortic Diseases/surgery , Aortic Diseases/complications , Esophageal Fistula/surgery , Esophageal Fistula/complications , Gastrointestinal Hemorrhage , Stents/adverse effects , Vascular Fistula/diagnostic imaging , Vascular Fistula/etiology , Vascular Fistula/surgery
5.
J Cardiothorac Surg ; 19(1): 152, 2024 Mar 23.
Article in English | MEDLINE | ID: mdl-38521956

ABSTRACT

BACKGROUND: The presence of the severe thoracic aortic calcification (TAC) in cardiac surgery patients is associated with adverse post-operative outcome. However, the relationship between cardiovascular risk factors and aortic plaque burden remains unknown. The objective of this study was to determine the predictive factors of TAC in patients candidate for cardiac surgery. METHODS: Patients who underwent thoracic CT scan prior to cardiac surgery between August 2020 to April 2021 were included. Of 556 patients, 209 (36.7%) had a thoracic aortic calcium score (TACS) ≥ 400 mm [3] and were compare with the remaining patients. Predictors of severe TAC were assessed through stepwise multivariable logistic regression analysis. RESULTS: The patients with TACS ≥ 400 had a higher mean age (67.3 ± 7.1 vs. 55.7 ± 10.6; p < 0.001) with a higher frequency of diabetes mellitus (40.7% vs. 30.8%; p = 0.018), dyslipidemia (49.8% vs. 38.6%; p = 0.010), hypertension (60.8% vs. 44.7%; p < 0.001), opium addiction (18.2% vs. 11.2%; p = 0.023), peripheral vascular disease (PVD) (7.7% vs. 2.3%; p = 0.005) as compared with TACS < 400. The multiple determinants of TAC were PVD (OR = 2.86) followed by opium addiction, diabetes and age. CONCLUSIONS: Thoracic CT scan prior to cardiac surgery for patients with older age, diabetes, opium addiction and PVD is recommended. Our study could serve as a foundation for future research endeavors aimed at establishing a risk score for TAC.


Subject(s)
Aortic Diseases , Cardiac Surgical Procedures , Diabetes Mellitus , Opium Dependence , Humans , Opium Dependence/complications , Risk Factors , Tomography, X-Ray Computed , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Aortic Diseases/diagnostic imaging , Aortic Diseases/surgery , Aortic Diseases/complications
6.
BMJ Open ; 14(3): e067977, 2024 Mar 20.
Article in English | MEDLINE | ID: mdl-38508639

ABSTRACT

OBJECTIVES: The objective of this study was to develop clinical classifiers aiming to identify prevalent ascending aortic dilatation in patients with bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV). DESIGN AND SETTING: A prospective, single-centre and observational cohort. PARTICIPANTS: The study involved 543 BAV and 491 TAV patients with aortic valve disease and/or ascending aortic dilatation, excluding those with coronary artery disease, undergoing cardiothoracic surgery at the Karolinska University Hospital (Sweden). MAIN OUTCOME MEASURES: Predictors of high risk of ascending aortic dilatation (defined as ascending aorta with a diameter above 40 mm) were identified through the application of machine learning algorithms and classic logistic regression models. EXPOSURES: Comprehensive multidimensional data, including valve morphology, clinical information, family history of cardiovascular diseases, prevalent diseases, demographic details, lifestyle factors, and medication. RESULTS: BAV patients, with an average age of 60.4±12.4 years, showed a higher frequency of aortic dilatation (45.3%) compared with TAV patients, who had an average age of 70.4±9.1 years (28.9% dilatation, p <0.001). Aneurysm prediction models for TAV patients exhibited mean area under the receiver-operating-characteristic curve (AUC) values above 0.8, with the absence of aortic stenosis being the primary predictor, followed by diabetes and high-sensitivity C reactive protein. Conversely, prediction models for BAV patients resulted in AUC values between 0.5 and 0.55, indicating low usefulness for predicting aortic dilatation. Classification results remained consistent across all machine learning algorithms and classic logistic regression models. CONCLUSION AND RECOMMENDATION: Cardiovascular risk profiles appear to be more predictive of aortopathy in TAV patients than in patients with BAV. This adds evidence to the fact that BAV-associated and TAV-associated aortopathy involves different pathways to aneurysm formation and highlights the need for specific aneurysm preventions in these patients. Further, our results highlight that machine learning approaches do not outperform classical prediction methods in addressing complex interactions and non-linear relations between variables.


Subject(s)
Aneurysm , Aortic Diseases , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Humans , Middle Aged , Aged , Aortic Valve/surgery , Heart Valve Diseases/complications , Heart Valve Diseases/surgery , Prospective Studies , Dilatation , Aortic Diseases/complications
8.
Echocardiography ; 41(3): e15799, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38516862

ABSTRACT

Atherosclerosis is the most common cause of heart disease and stroke. Plaque thickness ≥4 mm in the ascending aorta or aortic arch is strongly correlated with cerebral embolic events and ischemic stroke. However, despite imaging workup, the cause of embolic stroke remains unidentified in many patients. Transesophageal echocardiography (TEE) is the preferred echocardiographic method for the evaluation of cardiac source of emboli. 2D TEE imaging evaluates aortic root and aortic arch in a single plane or two planes with biplane imaging. However, 2D TEE often fails to detect mobile or complex components in the ascending aorta and aortic arch plaques. The routine availability of 3D TEE in current ultrasound systems may significantly improve the assessment of aortic plaques as a potential embolic source. In this case series, we present four consecutive patients with stroke who underwent TEE by a single cardiologist for possible cardioembolic source. Some of these patients may have been labelled as "cryptogenic stroke" or "embolic stroke of undetermined source" (ESUS) due to the presence of insignificant or nonmobile ascending aortic or aortic arch plaques on 2D TEE imaging. In our four consecutive patients with ESUS who underwent TEE by a single operator, 3D TEE showed complex aortic arch plaques with ulceration with mobile components and established these plaques as the likely source of embolic stroke.


Subject(s)
Aortic Diseases , Atherosclerosis , Embolic Stroke , Embolism , Plaque, Atherosclerotic , Stroke , Humans , Plaque, Atherosclerotic/complications , Plaque, Atherosclerotic/diagnostic imaging , Echocardiography, Transesophageal/methods , Embolic Stroke/complications , Atherosclerosis/complications , Atherosclerosis/diagnostic imaging , Stroke/complications , Stroke/diagnostic imaging , Aorta, Thoracic/diagnostic imaging , Embolism/complications , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging
9.
BMJ Case Rep ; 17(2)2024 Feb 20.
Article in English | MEDLINE | ID: mdl-38378584

ABSTRACT

A man in his 60s attended emergency for acute-onset abdominal pain and haematemesis. Requiring resuscitation, a CT abdomen/pelvis revealed a primary aortoenteric fistula actively bleeding into the duodenum. His background included a previous severe Q-fever infection and a heavy smoking history. Despite attempts at resuscitation and an emergent surgical attempt at haemostasis, the patient did not survive the massive gastrointestinal haemorrhage.Even in less severe cases, management of aortoenteric fistulas is tricky. Blood cultures and angiographic imaging are important investigations in guiding surgical approach. The pathology tends to have a significant rate of mortality even at tertiary-level vascular surgical centres.


Subject(s)
Aortic Diseases , Intestinal Fistula , Vascular Fistula , Male , Humans , Vascular Fistula/complications , Vascular Fistula/diagnostic imaging , Intestinal Fistula/complications , Intestinal Fistula/diagnostic imaging , Intestinal Fistula/surgery , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging , Tomography, X-Ray Computed , Gastrointestinal Hemorrhage/therapy , Gastrointestinal Hemorrhage/surgery
10.
J Cardiothorac Surg ; 19(1): 70, 2024 Feb 07.
Article in English | MEDLINE | ID: mdl-38326831

ABSTRACT

BACKGROUND: Aortoesophageal fistula (AEF) is an extremely rare and highly fatal complication leading to a high risk of morbidity and mortality. Successful management of AEF after esophagectomy for esophageal carcinoma has rarely been reported in the literature. CASE PRESENTATION: Here we present a rare case of a 44-year-old female with complications of AEF after esophagectomy for esophageal carcinoma, mainly presented as vomiting of blood. Both computed tomographic and computed tomography angiography of the chest showed bilateral pleural effusion and atelectasis, while gastroscopy showed large gastrointestinal bleeding. Emergency surgery was performed that included the removal of the mediastinal abscess, left lower pulmonary wedge resection, and thoracic endovascular aortic repair (TEVAR), followed by supportive treatment. The surgery went successful, and the patient was followed up for 1 year after discharge and showed good recovery. We also reviewed previous literature on the history, causes, pathophysiology, clinical presentation, diagnosis, and treatment of AEF after esophagectomy for esophageal adenocarcinoma. CONCLUSIONS: In our case, thoracotomy combined with TEVAR was effective in treating AEF after esophagectomy for esophageal adenocarcinoma. This case provides successful experiences for clinical diagnosis and treatment of AEF after esophagectomy for esophageal carcinoma.


Subject(s)
Adenocarcinoma , Aortic Diseases , Carcinoma , Esophageal Fistula , Vascular Fistula , Adult , Female , Humans , Adenocarcinoma/surgery , Adenocarcinoma/complications , Aortic Diseases/surgery , Aortic Diseases/complications , Carcinoma/surgery , Endovascular Aneurysm Repair , Esophageal Fistula/etiology , Esophageal Fistula/surgery , Esophagectomy/adverse effects , Gastrointestinal Hemorrhage , Vascular Fistula/etiology , Vascular Fistula/surgery
11.
J Cardiothorac Surg ; 19(1): 53, 2024 Feb 04.
Article in English | MEDLINE | ID: mdl-38311759

ABSTRACT

BACKGROUND: Sinus of Valsalva aneurysm (SVA) is an extremely rare condition, and its rupture causes acute symptoms such as chest pain and dyspnea. Ruptured SVA is frequently associated with other congenital defects. CASE PRESENTATION: A 37-year-old male presented with SVA originating from the left coronary sinus that ruptured into the interventricular septum. SVA was diagnosed by echocardiography, cardiac computed tomography and magnetic resonance imaging, and confirmed during the operation. CONCLUSIONS: SVA is a rare cardiac abnormality which can lead to severe clinical symptoms upon rupture. Immediate surgery is necessary to repair the ruptured SVA.


Subject(s)
Aortic Aneurysm , Aortic Diseases , Aortic Dissection , Aortic Rupture , Coronary Sinus , Sinus of Valsalva , Ventricular Septum , Male , Humans , Adult , Sinus of Valsalva/diagnostic imaging , Sinus of Valsalva/surgery , Sinus of Valsalva/pathology , Ventricular Septum/diagnostic imaging , Ventricular Septum/surgery , Aortic Aneurysm/complications , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/surgery , Aortic Diseases/complications , Aortic Rupture/diagnosis , Aortic Rupture/diagnostic imaging
12.
J Cardiothorac Surg ; 19(1): 71, 2024 Feb 07.
Article in English | MEDLINE | ID: mdl-38326839

ABSTRACT

BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children. CASE PRESENTATION: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome. CONCLUSION: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.


Subject(s)
Aortic Coarctation , Aortic Diseases , Adolescent , Humans , Male , Aorta, Abdominal/surgery , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Aorta, Thoracic/pathology , Aortic Coarctation/complications , Aortic Coarctation/diagnosis , Aortic Coarctation/surgery , Aortic Diseases/complications , Aortic Diseases/diagnosis , Aortic Diseases/surgery , Coral Reefs
13.
Clin Endocrinol (Oxf) ; 100(3): 269-276, 2024 03.
Article in English | MEDLINE | ID: mdl-38214123

ABSTRACT

OBJECTIVE: The risk of aortic dissection (AoD) is increased in Turner syndrome (TS) but predicting those at risk is difficult. Based on scarce evidence, preventive aortic surgery is recommended when aortic diameter increases >5 mm/year. To investigate the aortic growth rate in TS and TS-related conditions associated with aortic growth. We also reported our experience of women who suffered aortic dissection (AoD), and who had preventive aortic replacement. METHODS: 151 adult TS were retrospectively identified. Women who had more than one transthoracic echocardiogram (TTE) after age 16 years were included in the aortic growth study. Aortic diameters at sinuses of Valsalva (SoV) and ascending aorta (AA) were analysed by two experts. RESULTS: 70/151 women had more than one TTE (interscan interval 4.7 years). Mean aortic growth was 0.13 ± 0.59 mm/year at SoV and 0.23 ± 0.82 mm/year at AA. Known risk factors for aortic dilatation and TS-related conditions were not associated with aortic growth. 4/151 women experienced AoD (age 25±8 years): two had paired scans for aortic growth, which was 0.67 mm/year at both SoV and AA in the first woman, and 11 mm/year (SoV) and 4 mm/year (AA) in the second. Only 1/4 of women with AoD survived; she used a TS cardiac-alert card to inform emergency personnel about her risk of AoD. 5/151 had a preventive aortic replacement, but one died post-operatively. CONCLUSIONS: Mean aortic growth in our TS population was increased compared to non-TS women and was not associated with currently known risk factors for AoD, suggesting that aortic growth rate itself could be a useful variable to stratify who is at risk for AoD.


Subject(s)
Aortic Diseases , Aortic Dissection , Turner Syndrome , Adult , Female , Humans , Adolescent , Young Adult , Turner Syndrome/complications , Turner Syndrome/epidemiology , Retrospective Studies , Aortic Diseases/complications , Aortic Diseases/epidemiology , Risk Assessment
15.
Sci Rep ; 14(1): 25, 2024 01 02.
Article in English | MEDLINE | ID: mdl-38167947

ABSTRACT

Chronic risk factors for pseudoaneurysm (PSA) or penetrating aortic ulcer (PAU) have not been fully clarified. This study aims to evaluate the association of aortic calcification with PSA or PAU of different etiologies. Totally 77 pseudoaneurysms, 80 PAU, and 160 healthy controls (HCs) were retrospectively included, of which 30 were infected, 34 were immunological, and 93 were atherosclerotic etiologies. The aortic calcification status, position of aortic tears/ulcers, and risk factors for disease or acute aortic syndrome (AAS) were identified. Atherosclerotic patients aged more than 65 and infective patients aged more than 60 had significantly higher calcification scores. The immunological group had a lower level of calcification in the infrarenal aorta. For patients of infective or atherosclerotic etiology, 60% (18/30) and 60.22% (56/93) of the tears/ulcers occurred at the aortic parts with the highest level of calcification. Patients with longitudinal calcification exceeding 1/3 of the aortic arch had an increased risk of acquiring diseases (OR = 13.231). The presence of longitudinal calcification of the descending aorta or cross-sectional calcification of the infrarenal aorta increased the risks of acquiring diseases (OR = 8.484 and 8.804). After adjusting for age, longitudinal calcification of the descending aorta exceeding 1/3 length was found to be associated with AAS (OR = 4.662). Tears/ulcers of pseudoaneurysm and PAU were both generally found at the part of the aorta with most calcification. Distinct aorta calcification characteristics were observed for lesions of different etiologies. Longitudinal thoracic and cross-sectional infrarenal abdominal aortic calcification increased the risk of acquiring diseases, and descending aortic calcification was associated with symptomatic patients.


Subject(s)
Aneurysm, False , Aortic Diseases , Atherosclerosis , Penetrating Atherosclerotic Ulcer , Humans , Aneurysm, False/etiology , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging , Ulcer/pathology , Retrospective Studies , Cross-Sectional Studies , Aorta, Thoracic/pathology , Atherosclerosis/pathology , Aorta, Abdominal/diagnostic imaging , Aorta, Abdominal/pathology
16.
EMBO Mol Med ; 16(1): 132-157, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38177536

ABSTRACT

Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening condition associated with Marfan syndrome (MFS), a disease caused by fibrillin-1 gene mutations. While various conditions causing TAAD exhibit aortic accumulation of the proteoglycans versican (Vcan) and aggrecan (Acan), it is unclear whether these ECM proteins are involved in aortic disease. Here, we find that Vcan, but not Acan, accumulated in Fbn1C1041G/+ aortas, a mouse model of MFS. Vcan haploinsufficiency protected MFS mice against aortic dilation, and its silencing reverted aortic disease by reducing Nos2 protein expression. Our results suggest that Acan is not an essential contributor to MFS aortopathy. We further demonstrate that Vcan triggers Akt activation and that pharmacological Akt pathway inhibition rapidly regresses aortic dilation and Nos2 expression in MFS mice. Analysis of aortic tissue from MFS human patients revealed accumulation of VCAN and elevated pAKT-S473 staining. Together, these findings reveal that Vcan plays a causative role in MFS aortic disease in vivo by inducing Nos2 via Akt activation and identify Akt signaling pathway components as candidate therapeutic targets.


Subject(s)
Aortic Aneurysm, Thoracic , Aortic Diseases , Aortic Dissection , Azides , Deoxyglucose , Marfan Syndrome , Animals , Humans , Mice , Aortic Aneurysm, Thoracic/complications , Aortic Aneurysm, Thoracic/genetics , Aortic Aneurysm, Thoracic/metabolism , Aortic Diseases/complications , Deoxyglucose/analogs & derivatives , Marfan Syndrome/complications , Marfan Syndrome/genetics , Marfan Syndrome/metabolism , Nitric Oxide Synthase Type II/metabolism , Proto-Oncogene Proteins c-akt/metabolism , Versicans/metabolism
17.
Eur J Cardiothorac Surg ; 65(1)2024 Jan 02.
Article in English | MEDLINE | ID: mdl-38212990

ABSTRACT

OBJECTIVES: This study aimed to investigate changes in haematoma thickness during the early period and their association with disease progression in patients who received initial medical treatment for type A intramural haematoma (IMH). METHODS: Medical records and serial computed tomography angiography (CTA) images of patients who did not undergo emergency aortic repair for type A IMH upon presentation were retrospectively reviewed. The haematoma remodelling rate was determined using the following equation: thickness of the haematoma on the first CTA (mm) - thickness of the haematoma on the second CTA (mm)time between the first and second CTAs (h). RESULTS: Among the 40 patients included in this study, 38 were indicated for initial watch-and-wait strategy, whereas 2 were indicated for emergency aortic repair but declined it. During hospitalization, 10 patients developed disease progression, with 2 in-hospital mortality cases. Analysis of the haematoma remodelling rate in 39 patients revealed that such a rate was significantly associated with the reciprocal of the time from onset. Analysis of all 70 CTA examinations performed within 24 h after the onset of IMH showed that haematoma thickness was significantly associated with the logarithm of the time from onset. Initial regression of the haematoma was not necessarily associated with avoidance of disease progression. CONCLUSIONS: In type A IMH, the thickness of the haematoma in the ascending aorta tended to decrease in the very early period; however, prompt regression of the haematoma was not necessarily associated with avoidance of disease progression.


Subject(s)
Aortic Diseases , Humans , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging , Retrospective Studies , Hematoma/diagnostic imaging , Hematoma/etiology , Disease Progression , Tomography, X-Ray Computed
19.
Ann Thorac Surg ; 117(2): 387-394, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37414382

ABSTRACT

BACKGROUND: The incidence of reintervention for aortic arch obstruction is 5% to 14% after coarctation or hypoplastic aortic arch repair and 25% after the Norwood procedure. Institutional practice review indicated higher than reported reintervention rates. Our aim was to assess the impact of an interdigitating reconstruction technique on reintervention rates for recurrent aortic arch obstruction. METHODS: Children (<18 years) were included if they had undergone aortic arch reconstruction by sternotomy or the Norwood procedure. Three surgeons participated in the intervention with staggered rollout dates between June 2017 and January 2019, with the study ending December 2020 and review for reinterventions ending February 2022. Preintervention cohorts represented patients who underwent aortic arch reconstructions with patch augmentation, and postintervention cohorts represented patients who underwent an interdigitating reconstruction technique. Reinterventions by cardiac catheterization or operation were measured within 1 year of initial operation. Wilcoxon rank sum and χ2 tests were used to compare preintervention and postintervention cohorts. RESULTS: Overall, 237 patients were included for participation in this study, with 84 patients in the preintervention cohort and 153 in the postintervention cohort. Patients undergoing the Norwood procedure represented 30% (n = 25) of the retrospective cohort and 35% (n = 53) of the intervention cohort. Overall reinterventions were significantly decreased after the study intervention from 31% (n = 26/84) to 13% (n = 20/153; P < .001). Reintervention rates were decreased for each intervention cohort: aortic arch hypoplasia (24% [n = 14/59] vs 10% [n = 10/100]; P = .019) and Norwood procedure (48% [n = 12/25] vs 19% [n = 10/53]; P = .008). CONCLUSIONS: The interdigitating reconstruction technique for obstructive aortic arch lesions was successfully implemented and is associated with a decrease in reinterventions.


Subject(s)
Aortic Coarctation , Aortic Diseases , Norwood Procedures , Child , Humans , Infant , Aorta, Thoracic/surgery , Aorta, Thoracic/abnormalities , Retrospective Studies , Aortic Coarctation/surgery , Aortic Coarctation/etiology , Aortic Diseases/complications , Norwood Procedures/adverse effects , Treatment Outcome
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