RESUMEN
BACKGROUND AND AIMS: Atherosclerosis is the primary underlying cause of myocardial infarction and stroke, which are the major causes of death globally. Heparanase (Hpse) is a pro-inflammatory extracellular matrix degrading enzyme that has been implicated in atherogenesis. However, to date the precise roles of Hpse in atherosclerosis and its mechanisms of action are not well defined. This study aims to provide new insights into the contribution of Hpse in different stages of atherosclerosis in vivo. METHODS: We generated Hpse gene-deficient mice on the atherosclerosis-prone apolipoprotein E gene knockout (ApoE-/-) background to investigate the impact of Hpse gene deficiency on the initiation and progression of atherosclerosis after 6 and 14 weeks high-fat diet feeding, respectively. Atherosclerotic lesion development, blood serum profiles, lesion composition and aortic immune cell populations were evaluated. RESULTS: Hpse-deficient mice exhibited significantly reduced atherosclerotic lesion burden in the aortic sinus and aorta at both time-points, independent of changes in plasma cholesterol levels. A significant reduction in the necrotic core size and an increase in smooth muscle cell content were also observed in advanced atherosclerotic plaques of Hpse-deficient mice. Additionally, Hpse deficiency reduced circulating and aortic levels of VCAM-1 at the initiation and progression stages of disease and circulating MCP-1 levels in the initiation but not progression stage. Moreover, the aortic levels of total leukocytes and dendritic cells in Hpse-deficient ApoE-/- mice were significantly decreased compared to control ApoE-/-mice at both disease stages. CONCLUSIONS: This study identifies Hpse as a key pro-inflammatory enzyme driving the initiation and progression of atherosclerosis and highlighting the potential of Hpse inhibitors as novel anti-inflammatory treatments for cardiovascular disease.
Asunto(s)
Aorta , Aterosclerosis , Glucuronidasa , Ratones Noqueados para ApoE , Placa Aterosclerótica , Animales , Masculino , Ratones , Aorta/patología , Aorta/metabolismo , Aorta/enzimología , Enfermedades de la Aorta/patología , Enfermedades de la Aorta/genética , Enfermedades de la Aorta/enzimología , Enfermedades de la Aorta/metabolismo , Apolipoproteínas E/genética , Apolipoproteínas E/deficiencia , Aterosclerosis/genética , Aterosclerosis/patología , Aterosclerosis/enzimología , Aterosclerosis/metabolismo , Dieta Alta en Grasa , Modelos Animales de Enfermedad , Progresión de la Enfermedad , Glucuronidasa/deficiencia , Glucuronidasa/genética , Glucuronidasa/metabolismo , Ratones Endogámicos C57BL , Ratones Noqueados , Necrosis , Seno Aórtico/patología , Molécula 1 de Adhesión Celular Vascular/metabolismoRESUMEN
ABSTRACT: Aneurysm of sinus Valsalva is a defined as dilatation of the sinuses located between the aortic valve annulus and the sinotubular junction and mostly found in the right coronary sinus. It can be either congenital or acquired. This condition is usually asymptomatic unless it can cause intracardiac rupture or aortic valve insufficiency. Extracardiac rupture and associated fatal cases of cardiac tamponade are extremely rare. Our case is one of the rare cases in the literature that was diagnosed during autopsy. Our case is 65-year-old male patient with a history of hypertension suddenly fell ill after swimming in the sea. On gross examination of heart, there was an aneurysmatic enlargement of the right sinus Valsalva measuring 4.5 × 4 cm with a hemorrhagic appearance on the outer surface and a 0.3 cm rupture area. Histopathological examination revealed hemorrhage in and around the aneurysm wall.
Asunto(s)
Aneurisma de la Aorta , Rotura de la Aorta , Muerte Súbita , Seno Aórtico , Humanos , Masculino , Anciano , Seno Aórtico/patología , Muerte Súbita/etiología , Rotura de la Aorta/patología , Aneurisma de la Aorta/patología , Hemorragia/patología , Taponamiento Cardíaco/etiología , Taponamiento Cardíaco/patologíaRESUMEN
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.
Asunto(s)
Aneurisma de la Aorta , Enfermedades de la Aorta , Disección Aórtica , Rotura de la Aorta , Seno Coronario , Seno Aórtico , Tabique Interventricular , Masculino , Humanos , Adulto , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/cirugía , Seno Aórtico/patología , Tabique Interventricular/diagnóstico por imagen , Tabique Interventricular/cirugía , Aneurisma de la Aorta/complicaciones , Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/cirugía , Enfermedades de la Aorta/complicaciones , Rotura de la Aorta/diagnóstico , Rotura de la Aorta/diagnóstico por imagenRESUMEN
PURPOSE: The coronary arterial tree has a wide range of possible benign anatomical variations. It is important to differentiate them from coronary arterial anomalies, which can remain asymptomatic or in some cases lead to sudden death if undiagnosed. METHODS: A 42-year-old female patient with a transient ST depression in right precordial leads performed an ECG-gated computed tomography angiography with dual layer spectral CT (IQon Elite Spectral CT, Philips, Amsterdam, The Netherlands) at Circolo Hospital of Varese. RESULTS: A rare variant was observed and studied: a single common trunk arising from the right sinus of Valsalva which branches into a right coronary artery, a left anterior descending artery with malignant course and a left main with a retroaortic course; the left main gives origin to a dual anterior interventricular artery ("Dual LAD") and a left circumflex artery. CONCLUSIONS: This type of variation was never described in the English literature. Identifying this variant is crucial for potential ischemic complications during sports activities or with the onset of atherosclerotic disease.
Asunto(s)
Enfermedad de la Arteria Coronaria , Anomalías de los Vasos Coronarios , Seno Aórtico , Humanos , Adulto , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/anomalías , Seno Aórtico/patología , Anomalías de los Vasos Coronarios/diagnóstico , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Angiografía Coronaria/métodos , Aorta/patologíaRESUMEN
As widely discussed in recent literature, coronary artery anomalies only occasionally lead to potentially serious myocardial ischemic events. The most important group of coronary anomalies has been called anomalous coronary artery origin from an abnormal sinus or a site in the ascending aorta (ACAOS). Only some cases of right- or left-sided intramural-course ACAOS (R-ACAOS-IM or L-ACAOS-IM) can potentially cause significant symptoms or sudden cardiac death, typically during exertion in athletes. After an ACAOS-IM case is qualitatively identified, it is necessary to establish the severity of associated stenosis (which is always present to some degree in ACAOS-IM). The 3 stages of a comprehensive diagnostic process are: 1. initial screening of high-risk populations (young elite athletes, optimally by use of magnetic resonance imaging [MRI]) to identify the prevalence of similar cases in large populations (the denominator of any risk calculation); 2. evaluating symptoms (chest pain, syncope, or sudden death) and performing stress testing; 3. in patients found to carry ACAOS-IM, evaluating the severity of coronary obstruction by intravascular ultrasonography, which is an objective, definitive, and quantifying imaging modality for this condition, essential in selected carriers of such anomalies. The possible treatment alternatives are discussed and updated.
Asunto(s)
Enfermedad de la Arteria Coronaria , Anomalías de los Vasos Coronarios , Seno Aórtico , Humanos , Prevalencia , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/epidemiología , Anomalías de los Vasos Coronarios/terapia , Seno Aórtico/anomalías , Seno Aórtico/patología , Angiografía Coronaria/métodos , Enfermedad de la Arteria Coronaria/patologíaRESUMEN
OBJECTIVE: To elucidate sex differences in valve morphology, disease phenotype, progression, and outcomes among children and young adults with bicuspid aortic valve (BAV). PATIENTS AND METHODS: This is a retrospective cohort study examining all children and young adults (aged ≤22 years) with isolated BAV diagnosed, by excluding patients with concomitant congenital heart defects or genetic syndromes, from January 1, 1990, through December 1, 2016, at Mayo Clinic in Rochester, Minnesota. RESULTS: Of 1010 patients with BAV, 558 had isolated BAV. Distributions of morphology were right-left in 65.8% (n=367), right-noncoronary in 34% (n=190), and left-noncoronary cusp fusion in 0.2% (n=1) of patients; with no sex differences. Male to female ratio was 3:1. At the first echocardiographic evaluation in the study, there were no sex differences in terms of frequency of aortic valve stenosis or regurgitation. However, males had significantly higher grades of aortic valve regurgitation at 17 years of age onward (P<.0001). Males had significantly larger mid-ascending aorta (P=.01) and sinus of Valsalva dimensions (z score; P=.0001) as compared with females, with a novel finding of peak aortic dimensions around 8 years of age. Males also had more than 2-fold higher risk for sinus of Valsalva dilation (z score >2) as compared with females (odds ratio, 2.3; 95% CI, 1.2 to 4.2; P=.01). There were no significant sex differences in the primary cardiac outcomes of interventions on aortic valve and/or aorta, aortic dissection, or death. CONCLUSION: In children and young adults with BAV, males have a higher grade of aortic regurgitation in late adolescence, significantly larger aortic dimensions, different patterns of aortic growth, and more frequent sinus of Valsalva dilation as compared with females. Overall, the rate of primary cardiac events is lower in young patients, with no significant sex differences.
Asunto(s)
Aorta , Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Ecocardiografía/métodos , Factores Sexuales , Seno Aórtico , Adolescente , Factores de Edad , Aorta/diagnóstico por imagen , Aorta/crecimiento & desarrollo , Aorta/patología , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/fisiopatología , Insuficiencia de la Válvula Aórtica/diagnóstico , Insuficiencia de la Válvula Aórtica/etiología , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico , Enfermedad de la Válvula Aórtica Bicúspide/fisiopatología , Variación Biológica Poblacional , Niño , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Tamaño de los Órganos , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/patologíaAsunto(s)
Aneurisma de la Aorta/diagnóstico por imagen , COVID-19/complicaciones , Seno Aórtico/patología , Anciano , COVID-19/diagnóstico , Dolor en el Pecho/etiología , Angiografía por Tomografía Computarizada , Ecocardiografía , Humanos , Masculino , Reacción en Cadena de la Polimerasa , Tomografía Computarizada por Rayos XRESUMEN
This study aimed to investigate the impact of weight loss after laparoscopic sleeve gastrectomy (LSG) on cardiac structural and functional remodeling in obese Chinese. A total of 44 obese participants were enrolled consecutively. The physical, laboratory, electrocardiographic, and echocardiographic parameters of pre-and postoperative were recorded. The average follow-up time was 12.28 ± 5.80 months. The body mass index (BMI) of the patients with obesity was decreased from 41.6 ± 7.44 to 30.3 ± 5.73kg/m2 (P<0.001) after LSG. The systolic and diastolic blood pressure of the subjects was significantly reduced from 137.9 ± 15.7mmHg to 123.0 ± 16.0 and 83.4 ± 10.8 to 71.3 ± 11.7mmHg (P<0.001), respectively. The levels of fasting insulin and fasting blood glucose were significantly decreased (38.8 ± 32.1 to 8.43 ± 4.16 mU/L, P<0.001; 6.95 ± 2.59 to 4.64 ± 0.50mmol/L, P<0.001). Total cholesterol (TC, 4.66 ± 0.84 to 4.23 ± 0.75mmol/L, P<0.001) and triglyceride (TG, 1.92 ± 1.21 to 0.85 ± 0.30mmol/L, P<0.001) decreased significantly. Cardiovascular geometric parameters including aortic sinus diameter (ASD, 32.9 ± 2.83mm to 32.0 ± 3.10mm, P<0.05), left atrial diameter (LAD, 38.8 ± 4.03 to 36.2 ± 4.12mm, P<0.001), and interventricular septum thickness(IVS, 10.2 ± 0.93 to 9.64 ± 0.89mm, P<0.001) were significantly reduced. The ratio of weight loss (RWL) was positively correlated with the changes of LAD. The change of IVS was negatively correlated with the change of fasting blood glucose (GLU). Weight loss after LSG could effectively improve cardiac structural, but not functional, abnormality in obese Chinese.
Asunto(s)
Remodelación Atrial , Cirugía Bariátrica , Gastrectomía , Obesidad/cirugía , Remodelación Ventricular , Pérdida de Peso , Adulto , Glucemia/metabolismo , Presión Sanguínea , China , Colesterol/metabolismo , Ecocardiografía , Electrocardiografía , Femenino , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/patología , Humanos , Insulina/metabolismo , Laparoscopía , Masculino , Obesidad/metabolismo , Obesidad/fisiopatología , Tamaño de los Órganos , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/patología , Resultado del Tratamiento , Triglicéridos/metabolismo , Tabique Interventricular/diagnóstico por imagen , Tabique Interventricular/patología , Adulto JovenRESUMEN
BACKGROUND: The aim of this study was to evaluate the fate of the preserved aortic root after supracoronary aortic replacement for acute type A aortic dissection. METHODS: Between October 1999 and March 2018, 339 patients underwent supracoronary aortic replacement for acute type A aortic dissection at our institution. Late outcomes were evaluated, including overall survival, aortic-related death, and aortic root-related reoperation. The median follow-up was 3.7 years (1.4-8.4 years). RESULTS: Operative mortality was 46 patients (13.6%). The cumulative incidences at 5 years for aortic root-related reoperation, aortic-related death, and non-aortic related death were 2.5%, 14.5% and 12.4%, respectively. Multivariable Cox hazard regression analysis demonstrated greater sinus of Valsalva diameter and number of commissural detachments to be significant risk factors for a composite outcome consisting of aortic-related death or aortic root-related reoperation. Mixed-effects regression demonstrated that sinus of Valsalva diameter significantly increased with time (P < .001), and aortic regurgitation significantly worsened (P < .001). CONCLUSIONS: Sinus of Valsalva diameter and commissural detachment were independent predictors of unfavorable outcomes after supracoronary aortic replacement. Close follow-up is particularly necessary for these patients, and aortic root replacement at the time of initial operation may lead to more favorable late outcomes.
Asunto(s)
Aorta/cirugía , Aneurisma de la Aorta/cirugía , Disección Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/etiología , Injerto Vascular/métodos , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Seno Aórtico/patología , Análisis de Supervivencia , Resultado del Tratamiento , Injerto Vascular/efectos adversosRESUMEN
Importance: Women with aortopathy conditions are at risk for pregnancy-related aortic dissection, and these conditions may not be recognized until after the aortic dissection occurs. Objective: To examine the clinical characteristics, imaging features, and outcomes in women with pregnancy-related acute aortic dissection. Design, Setting, and Participants: A cohort study, comprising data from the International Registry of Acute Aortic Dissection (IRAD) (February 1, 1998, to February 28, 2018). The multicenter referral center study included 29 women with aortic dissection during pregnancy or less than 12 weeks post partum in IRAD from 1998 to 2018. Main Outcomes and Measures: Clinical features of pregnancy-related aortic dissection to be studied included underlying aortopathy, aortic size, type of aortic dissection, timing of dissection, hypertension, and previous aortic surgery. Results: A total of 29 women (mean [SD] age, 32 [6] years) had pregnancy-related aortic dissection, representing 0.3% of all aortic dissections and 1% of aortic dissection in women in the IRAD. Among women younger than 35 years, aortic dissection was related to pregnancy in 20 of 105 women (19%). Thirteen women (45%) had type A aortic dissection, and 16 women (55%) had type B. Aortic dissection onset was known in 27 women (93%): 15 during pregnancy, 4 in the first trimester, and 11 in the third trimester; 12 were post partum, occurring a mean (SD) of 12.5 (14) days post partum. At type A aortic dissection diagnosis, the mean (SD) aortic diameters were sinus of Valsalva, 54.5 (5) mm and ascending aorta, 54.7 (6) mm. At type B aortic dissection diagnosis, the mean (SD) descending aortic diameter was 32.5 (5) mm. Twenty women (69%) had an aortopathy condition or a positive family history: 13 women (65%) with Marfan syndrome, 2 women (10%) with Loeys-Dietz syndrome, 2 women (10%) with bicuspid aortic valves, 2 women (10%) with a family history of aortic disease, and 1 woman (5%) with familial thoracic aortic aneurysm. Aortopathy was not recognized until after aortic dissection in 47% of the women. Twenty-eight women (97%) survived aortic dissection hospitalization. Conclusions and Relevance: Aortic dissection complicating pregnancy is rare. Most pregnancy-related aortic dissection is due to an aortopathy often not diagnosed until after aortic dissection. In this study, type A aortic dissections were associated with a dilated aorta, and type B aortic dissections often were not. Recognition of underlying conditions and risks for aortic dissection may improve management of pregnancy in women with aortopathy.
Asunto(s)
Aneurisma de la Aorta/epidemiología , Disección Aórtica/epidemiología , Complicaciones Cardiovasculares del Embarazo/epidemiología , Trastornos Puerperales/epidemiología , Adulto , Disección Aórtica/complicaciones , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/terapia , Aorta/patología , Aorta Torácica/patología , Aneurisma de la Aorta/complicaciones , Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/terapia , Enfermedades de la Aorta/complicaciones , Enfermedad de la Válvula Aórtica Bicúspide/complicaciones , Femenino , Mortalidad Hospitalaria , Humanos , Síndrome de Loeys-Dietz/complicaciones , Síndrome de Marfan/complicaciones , Tamaño de los Órganos , Embarazo , Complicaciones Cardiovasculares del Embarazo/diagnóstico por imagen , Complicaciones Cardiovasculares del Embarazo/terapia , Trastornos Puerperales/diagnóstico por imagen , Trastornos Puerperales/terapia , Sistema de Registros , Seno Aórtico/patología , Enfermedades no Diagnosticadas/complicaciones , Adulto JovenRESUMEN
The aim of the present study was to examine whether inhibition of Interleukin (IL)-6 signaling by MR16-1, an IL-6 receptor antibody, attenuates aortitis, cardiac hypertrophy, and arthritis in IL-1 receptor antagonist deficient (IL-1RA KO) mice. Four weeks old mice were intraperitoneally administered with either MR16-1 or non-immune IgG at dosages that were adjusted over time for 5 weeks. These mice were stratified into four groups: MR16-1 treatment groups, KO/MR low group (first 2.0 mg, following 0.5 mg/week, n=14) and KO/MR high group (first 4.0 mg, following 2.0 mg/week, n=19) in IL-1RA KO mice, and IgG treatment groups, KO/IgG group (first 2.0 mg, following 1.0 mg/week, n=22) in IL-1RA KO mice, and wild/IgG group (first 2.0 mg, following 1.0 mg/week, n=17) in wild mice. Aortitis, cardiac hypertrophy and arthropathy were histologically analyzed. Sixty-eight percent of the KO/IgG group developed aortitis (53% developed severe aortitis). In contrast, only 21% of the KO/MR high group developed mild aortitis, without severe aortitis (P<0.01, vs KO/IgG group). Infiltration of inflammatory cells, such as neutrophils, T cells, and macrophages, was frequently observed around aortic sinus of the KO/IgG group. Left ventricle and cardiomyocyte hypertrophy were observed in IL-1RA KO mice. Administration of high dosage of MR16-1 significantly suppressed cardiomyocyte hypertrophy. MR16-1 attenuated the incidence and severity of arthritis in IL-1RA KO mice in a dose-dependent manner. In conclusion, blockade of IL-6 signaling may exert a beneficial effect to attenuate severe aortitis, left ventricle hypertrophy, and arthritis.
Asunto(s)
Aortitis/metabolismo , Artritis/metabolismo , Hipertrofia Ventricular Izquierda/metabolismo , Proteína Antagonista del Receptor de Interleucina 1/deficiencia , Interleucina-6/metabolismo , Transducción de Señal , Animales , Anticuerpos/farmacología , Aortitis/patología , Arterias/patología , Peso Corporal , Femenino , Hemodinámica , Inmunidad Innata , Inflamación/patología , Proteína Antagonista del Receptor de Interleucina 1/metabolismo , Masculino , Ratones Noqueados , Tamaño de los Órganos , Seno Aórtico/patologíaRESUMEN
Anti-apolipoprotein A-1 (anti-apoA-1 IgG) and anti-double stranded DNA (anti-dsDNA IgG) autoantibodies have been described as mediators of atherogenesis in mice and humans. In the present study, we aim to investigate the association between atherosclerotic parameters, autoantibodies and plaque vulnerability in the context of systemic lupus erythematosus (SLE). We therefore bred a lupus prone-mouse model (Nba2.Yaa mice) with Apoe-/- mice resulting in Apoe-/-Nba2.Yaa mice spontaneously producing anti-apoA-1 IgG antibodies. Although Apoe-/-Nba2.Yaa and Apoe-/- mice subject to a high cholesterol diet displayed similar atherosclerosis lesions size in aortic roots and abdominal aorta, the levels of macrophage and neutrophil infiltration, collagen, MMP-8 and MMP-9 and pro-MMP-9 expression in Apoe-/-Nba2.Yaa mice indicated features of atherosclerotic plaque vulnerability. Even though Apoe-/-Nba2.Yaa mice and Apoe-/- mice had similar lipid levels, Apoe-/-Nba2.Yaa mice showed higher anti-apoA-1 and anti-dsDNA IgG levels. Apoe-/-Nba2.Yaa mice displayed a reduction of the size of the kidney, splenomegaly and lymph nodes (LN) hypertrophy. In addition, anti-apoA-1 and anti-dsDNA IgG increased also in relation with mRNA levels of GATA3, IL-4, Bcl-6 and CD20 in the spleen and aortic arch of Apoe-/-Nba2.Yaa mice. Our data show that although atherosclerosis-lupus-prone Apoe-/-Nba2.Yaa mice did not exhibit exacerbated atherosclerotic lesion size, they did show features of atherosclerotic plaque destabilization in correlation with the increase of pro-atherogenic autoantibodies.
Asunto(s)
Susceptibilidad a Enfermedades/etiología , Lupus Eritematoso Sistémico/complicaciones , Placa Aterosclerótica/etiología , Animales , Autoanticuerpos/sangre , Modelos Animales de Enfermedad , Ensayo de Inmunoadsorción Enzimática , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Placa Aterosclerótica/patología , Reacción en Cadena en Tiempo Real de la Polimerasa , Seno Aórtico/patologíaRESUMEN
OBJECTIVE: To investigate the aneurysmal of the left sinus of Valsalva, and to improve the understanding of the disease and the level of diagnosis and treatment. METHOD: This article mainly reports a case of a huge aneurysmal of the left sinus of Valsalva patients treated with surgical treatment. RESULT: After surgery, the prognosis of the case was good. CONCLUSION: Aneurysmal of the left sinus of Valsalva has a low incidence, which is rare in clinical with no clinical specific symptoms leading to difficulty in early detection. The appropriate surgical method should be considered to the patient's condition, to prevent the tumor rupture and the death of patients.
Asunto(s)
Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/cirugía , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/cirugía , Aneurisma de la Aorta/patología , Ecocardiografía , Humanos , Imagenología Tridimensional , Periodo Intraoperatorio , Masculino , Persona de Mediana Edad , Radiografía Torácica , Seno Aórtico/patología , Tomografía Computarizada por Rayos X , Procedimientos Quirúrgicos Vasculares/métodosAsunto(s)
Rotura de la Aorta/diagnóstico , Cardiopatías Congénitas/diagnóstico , Defectos del Tabique Interventricular/diagnóstico , Seno Aórtico/patología , Adulto , Anomalías Cardiovasculares/complicaciones , Diagnóstico Diferencial , Disnea/diagnóstico , Disnea/etiología , Ecocardiografía/métodos , Electrocardiografía/métodos , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/patología , Defectos del Tabique Interventricular/cirugía , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico , Masculino , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/cirugíaRESUMEN
The aim of this study was to compare the size and geometry of the aorta in patients with 7q11.23 duplication (Dup7) to healthy controls. We retrospectively reviewed all echocardiograms in all patients with Dup7 evaluated at our institutions from June 2017 through September 2019. All standard aortic diameter measurements were made and recorded. Z-scores for the measurements were calculated. For comparison, a set of control echocardiograms was developed by randomly selecting 24 normal echocardiograms in age-matched patients who had undergone echocardiograms for an indication of either chest pain or syncope. In 58 echocardiograms from 21 Dup7 patients, all aortic measurements were increased compared to controls (p < 0.0001). Effacement of the sinotubular junction (STJ) of the aorta was present in all Dup7 patients. Our novel STJ-to-aortic annulus ratio of ≥ 1.15 had a 98.28% sensitivity (95% CI 90.76-99.96) and 100% specificity (95% CI 85.75-100) for distinguishing Dup7 from controls with a positive predictive value of 100% and a negative predictive value of 96.00% (95% CI 77.47-99.41). All patients in our study with Dup7 had echocardiographic evidence of aortopathy. Effacement of the STJ was present in all Dup7 patients. The STJ-to-annulus ratio is a better indicator of aortopathy in Dup7 than the aortic Z-score.
Asunto(s)
Aorta/patología , Síndrome de Williams/patología , Adolescente , Aorta/diagnóstico por imagen , Niño , Preescolar , Ecocardiografía , Femenino , Humanos , Masculino , Estudios Retrospectivos , Sensibilidad y Especificidad , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/patología , Síndrome de Williams/diagnóstico por imagenRESUMEN
BACKGROUND: Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity. METHODS: A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical-instrumental-genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve. RESULTS: Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004). CONCLUSIONS: In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis.
Asunto(s)
Enfermedad de la Válvula Aórtica Bicúspide/epidemiología , Síndrome de Marfan/complicaciones , Anamnesis , Seno Aórtico/patología , Adolescente , Enfermedad de la Válvula Aórtica Bicúspide/etiología , Niño , Preescolar , Estudios de Cohortes , Dilatación Patológica/epidemiología , Dilatación Patológica/etiología , Ecocardiografía , Femenino , Humanos , Masculino , Adulto JovenRESUMEN
Aneurysms in the sinuses of Valsalva (SVA) are the least frequent and occur due to a weakness in the aortic wall that forms part of the sinus. This causes dilatation and the formation of a blind pocket in one of the aortic sinuses (usually he right sinus and less frequently the posterior one). It may be congenital or acquired: in a congenital SVA, the condition is frequently associated with Marfan's syndrome or other connective tissue disorders; instead, acquired forms of sinus of Valsalva aneurysm are associated with infections (syphilis, bacterial endocarditis, and tuberculosis), atherosclerosis and medial cystic necrosis, traumatic and degenerative diseases, abuse of drugs or alcoholism. Despite SVA is a well-known anomaly, autopsy images or reviews of the condition are very uncommon. Indeed we report here a fatal case of SVA in a 58-year-old homeless man found dead on the street. The autopsy, performed to determine the cause of death, releaved a massive aneurysm (in excess of 4 cm) involving the right coronary sinus of the aorta. In this case, the aneurysm may be an accidental finding: in effect we found no tromboses inside the aneurysm and the ostium was not obstructed, therefore the cause of death could be attribuited to fatal arrhythmia.
Asunto(s)
Aneurisma de la Aorta/patología , Seno Aórtico/patología , Dilatación Patológica , Resultado Fatal , Humanos , Masculino , Persona de Mediana EdadRESUMEN
In this paper, 64-slice spiral computed tomography (CT) retrospective electrocardiographic (ECG) gating technology was used to perform coronary CT angiography scans. The aorta and aortic pulmonary artery diameter were measured quantitatively in healthy people and patients with hypertension and cerebrovascular disease. Corresponding aortic pulmonary artery ratios were obtained through calculation, and the changes of aortic diameter, aortic pulmonary artery ratio, and aortic diameter difference between different cardiac phases, ages, sexes, and levels of hypertension were discussed. Through research, it can be concluded that 64-row spiral CT scan combined with ECG gating technology can accurately measure the dynamic changes of the aortic diameter with the cardiac cycle. At the same time, the aortic diameter measured by multidetector CT scan combined with ECG gating technology and the phase difference between different phases can objectively reflect the degree of arterial damage in patients with hypertension; therefore, early screening of aortic diseases in patients with hypertension can be performed. Diagnosis to detect abnormalities as early as possible and start treatment as early as possible to prevent the disease from progressing and even affecting other tissues and organs can also be obtained.
Asunto(s)
Aorta/diagnóstico por imagen , Técnicas de Imagen Sincronizada Cardíacas/métodos , Angiografía por Tomografía Computarizada/métodos , Hipertensión/diagnóstico por imagen , Arteria Pulmonar/diagnóstico por imagen , Tomografía Computarizada Espiral/métodos , Adulto , Anciano , Anciano de 80 o más Años , Aorta/patología , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/patología , Estudios de Casos y Controles , Angiografía Coronaria/métodos , Diástole , Electrocardiografía , Femenino , Humanos , Hipertensión/patología , Masculino , Persona de Mediana Edad , Tomografía Computarizada Multidetector , Tamaño de los Órganos , Arteria Pulmonar/patología , Seno Aórtico/diagnóstico por imagen , Seno Aórtico/patología , Sístole , Tomografía Computarizada por Rayos XRESUMEN
A 72-year-old woman was referred to our institution with decompensated congestive heart failure owing to subacute severe aortic regurgitation and mitral regurgitation. Her blood sample tested positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). Cardiac computed tomography revealed abnormal thickening and shortening of the aortic valvar leaflets as well as wall thickening of the sinuses of Valsalva. Based on the diagnosis of ANCA-associated vasculitis, predominantly involving the aortic root, prednisolone administration was initiated, which failed to improve the valvar dysfunction. The patient underwent aortic root replacement and mitral annuloplasty. Histopathology confirmed severe inflammation involving both the aortic valvar sinuses and leaflets.