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1.
Mayo Clin Proc ; 96(7): 1874-1887, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-34218860

RESUMO

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.


Assuntos
Aorta , Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Ecocardiografia/métodos , Fatores Sexuais , Seio Aórtico , Adolescente , Fatores Etários , Aorta/diagnóstico por imagem , Aorta/crescimento & desenvolvimento , Aorta/patologia , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/etiologia , Doença da Válvula Aórtica Bicúspide/diagnóstico , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Variação Biológica da População , Criança , Progressão da Doença , Feminino , Humanos , Masculino , Tamanho do Órgão , Seio Aórtico/diagnóstico por imagem , Seio Aórtico/patologia
2.
Medicine (Baltimore) ; 100(25): e26483, 2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34160461

RESUMO

ABSTRACT: To investigate the importance of pulmonary vascular measurements on computed tomography (CT) in predicting pulmonary hypertension (PH) and worse outcomes in diffuse cystic lung diseases (DCLDs).We conducted a cross-sectional study of patients with DCLDs. Patients underwent pulmonary function tests, a six-minute walk test (6MWT), chest CT, transthoracic echocardiography, and right heart catheterization. Pulmonary artery (PA) diameter and PA-ascending aorta ratio (PA-Ao ratio) were obtained from CT. Mean pulmonary artery pressure (mPAP) from right heart catheterization was correlated with tomographic, functional, and echocardiographic variables. The association between the PA-Ao ratio with outcomes was determined by Kaplan-Meier curves.Thirty-four patients were included (18 with pulmonary Langerhans cell histiocytosis and 16 with lymphangioleiomyomatosis, mean age 46 ±â€Š9 years). Forced expiratory volume in the first second and lung diffusing capacity for carbon monoxide were 47 ±â€Š20% and 38 ±â€Š21% predicted, respectively. PA diameter and PA-Ao ratio were 29 ±â€Š6 mm and 0.95 ±â€Š0.24, respectively. PA-Ao ratio > 1 occurred in 38.2% of patients. PA-Ao ratio was a good predictor of PH. mPAP correlated best with PA-Ao ratio, PA diameter, oxygen desaturation during six-minute walk test, and echocardiographic variables. Patients with PA-Ao ratio > 1 had greater mPAP, and a higher risk of death or lung transplantation (log-rank, P < .001) than those with PA-Ao ratio ≤ 1.The PA-Ao ratio measured on CT scan has a potential role as a non-invasive tool to predict the presence of PH and as a prognostic parameter in patients with DCLDs.


Assuntos
Aorta/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico , Pneumopatias/complicações , Transplante de Pulmão/estatística & dados numéricos , Artéria Pulmonar/diagnóstico por imagem , Adulto , Aorta/patologia , Estudos Transversais , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/mortalidade , Pneumopatias/patologia , Pneumopatias/cirurgia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/patologia , Curva ROC , Medição de Risco/estatística & dados numéricos , Tomografia Computadorizada por Raios X , Teste de Caminhada
4.
Echocardiography ; 38(7): 1195-1200, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-34114255

RESUMO

A 17-year-old male sustained a blunt thoracic trauma after he had a dirt bike accident. He was admitted for the management of multiple fractures, was hemodynamically stable, and presented without any cardiac symptoms. The patient underwent transthoracic echocardiography and CT angiogram of the thorax as the workup of possible cardiac injury as he had a new aortic regurgitation murmur, troponin rise, and a new RBBB. Imaging showed aortic root rupture, type A aortic dissection involving aortic root and proximal ascending aorta, and acute severe aortic regurgitation, not typically seen with blunt thoracic trauma. The patient was immediately taken to the operating room, underwent a surgical aortic valve and root replacement with the Bentall procedure, and had a good outcome.


Assuntos
Aneurisma Dissecante , Ruptura Aórtica , Insuficiência da Valva Aórtica , Adolescente , Aneurisma Dissecante/complicações , Aneurisma Dissecante/diagnóstico por imagem , Aneurisma Dissecante/cirurgia , Aorta/diagnóstico por imagem , Aorta/cirurgia , Valva Aórtica , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/cirurgia , Humanos , Masculino
5.
Medicine (Baltimore) ; 100(26): e26518, 2021 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-34190185

RESUMO

ABSTRACT: Bicuspid aortic valve (BAV) disease has significant gaps in its clinical management practices. To highlight the potential utility of advanced hemodynamic biomarkers in strengthening BAV assessment, we used 4-dimentional flow magnetic resonance imaging to investigate altered hemodynamics in the ascending aorta (AAo).A total of 32 healthy controls and 53 age-matched BAV patients underwent cardiac magnetic resonance imaging at 3T, with cine imaging and 4D-flow. Analysis planes were placed along 3D-segmented aortas at the left ventricular outflow tract (LVOT), sinuses of Valsalva, mid-ascending aorta (MAA), and proximal to the first aortic branch. Locations were analyzed for aortic diameter (normalized to body surface area), pressure drop (PD), viscous energy loss (EL), and wall shear stress (WSS) sub-vectors (axial wall shear stress, circumferential wall shear stress [WSSC], magnitude wall shear stress). Student's t tests, or non-parametric equivalents, compared parameters between cohorts. Univariable and multivariable analyses explored the associations of AAo diameter with hemodynamics within the BAV cohort.Compared to control cohort, BAV patients showed significantly greater PD (MAA: 9.5 ±â€Š8.0 vs 2.8 ±â€Š2.4 mm Hg; P < .01), EL (from LVOT-AA1: 7.39 ±â€Š4.57 mW vs 2.90 ±â€Š1.07 mW; P < .01), and WSSC (MAA: 0.3 ±â€Š0.1 vs 0.2 ±â€Š0.06 Pa; P ≤ .01) throughout the AAo. Correlational analyses revealed an inverse association between AAo diameter and both magnitude wall shear stress and axial wall shear stress.BAV patients exhibited increased PD, EL, and WSSC in the AAo, and an inverse association between AAo diameter and WSS sub-vectors. This demonstrated the impact of PD, EL, and WSS in BAV disease and the importance of altered hemodynamics in aortic remodelling.


Assuntos
Aorta , Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Remodelação Vascular , Adulto , Aorta/diagnóstico por imagem , Aorta/patologia , Aorta/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Pressão Arterial , Doença da Válvula Aórtica Bicúspide/diagnóstico , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Velocidade do Fluxo Sanguíneo , Correlação de Dados , Feminino , Hemodinâmica , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Tamanho do Órgão , Resistência ao Cisalhamento
6.
Bratisl Lek Listy ; 122(7): 513-518, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34161120

RESUMO

AIM: A useful tool for determining the presence of systemic inflammation is the neutrophil-to-lymphocyte ratio (NLR). Chronic inflammation causes more microvascular resistance, which is known to be involved with coronary slow flow (CSF). Aortic flow propagation velocity (APV) can be used to evaluate the aorta's rigidity. We hypothesized that NLR and APV might be related to CSF. Therefore, we aimed to evaluate the NLR and APV in CSF patients. METHOD: Eighty-six CSF patients and 43 subjects with normal coronary flow were enrolled in this study. We utilized the Thrombolysis in Myocardial Infarction (TIMI) frame count (TFC) method for determining each enrolled patient's coronary flow rate. APV values were determined via color M-mode Doppler echocardiography. Differences in NLR and APV values were determined in CSF and control groups. RESULTS: Patients with CSF had lower APV values and higher NLR values than the normal controls (39.9±11.4 vs 48.0±10.6, p<0.01; 3.3±2.8 vs 2.3±1.2, p=0.03). TFC was significantly higher in CSF patients compared to controls (29.8±5.0 vs 12.9±2.6; p<0.01). TFC and APV were negatively correlated in CSF patients (r=-0.338 p=0.001). APV was independently associated with CSF (OR: 1.164, CI: 1.078-1.257, p=0.001). CONCLUSIONS: APV was significantly associated with TFC, and it is independently associated with CSF (Tab. 4, Fig. 3, Ref. 30). Text in PDF www.elis.sk Keywords: coronary slow flow, neutrophil-to-lymphocyte ratio, aortic flow propagation velocity, atherosclerosis, inflammation.


Assuntos
Circulação Coronária , Neutrófilos , Aorta/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Angiografia Coronária , Humanos , Linfócitos
7.
Medicine (Baltimore) ; 100(22): e26001, 2021 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-34087845

RESUMO

ABSTRACT: To identify and evaluate the spectrum and prevalence of variations in bronchial artery (BA) origin by multidetector computed tomography (MDCT) and digital subtraction angiography (DSA) in a large population with hemoptysis.From July 2008 to June 2015, data from 600 individuals with hemoptysis who underwent MDCT and DSA were retrospectively analyzed. The pattern of BA origin was investigated and classified according to distribution.A total of 1674 BAs were evaluated, 866 were right BA and 808 were left BA. Most BAs originated from the upper descending thoracic aorta, classified as orthotopic origin (n = 1464, 87.5%). Among ectopic origin BAs (n = 210, 12.5%), concavity of the aortic arch was the most common (n = 107). The most common distribution pattern was a single artery in each side (n = 262). According to our classification, Type I was most common (n = 457), including BAs originating in orthotopic fashion from the descending thoracic aorta. Type II (n = 2) was defined as BAs originating from the aortic arch or ascending aorta. Type III (not found) was defined as BAs originating from subclavian arteries, common carotid arteries, and their branch vessels. Type IV (n = 92) was Type I and II combined, Type V (n = 41) was Type I and III combined, Type VI (not found) was Type II and III combined, and Type VII (n = 8) was Type I, II, and III combined.Variations of BA origin could be systematically described in detail.


Assuntos
Angiografia Digital/métodos , Artérias Brônquicas/anatomia & histologia , Artérias Brônquicas/diagnóstico por imagem , Tomografia Computadorizada Multidetectores/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aorta/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
8.
Kyobu Geka ; 74(6): 413-417, 2021 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-34059582

RESUMO

Aortic valve-sparing surgery is a delicate procedure that requires specialized skills to control aortic valve regurgitation. Therefore, simulating the surgery before performing it on a patient is good practice for inexperienced surgeons. Herein, we present our experience of a simulation surgery using a three-dimensionally printed aortic root model of a 60-year-old man with severe aortic regurgitation and aortic root enlargement. We fabricated the model using multi-slice computed tomography data. The model revealed the two pitfalls:unbalanced commissure position and lower coaptation height of the right coronary cusp. We completed the simulation surgery from the proximal suture and valve reimplantation to coronary ostial reconstructions during approximately three hours with medical staffs. In the actual operation, the aortic valve regurgitation completely disappeared by accurately reconstructing the commissure in the Valsalva graft and adjusting the height of the right coronary cusp using central plication procedure. We believe that carrying out simulations before the actual surgery improves the surgeon's confidence and the patient's outcomes.


Assuntos
Insuficiência da Valva Aórtica , Procedimentos Cirúrgicos Cardíacos , Aorta/diagnóstico por imagem , Aorta/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Reimplante
9.
J Cardiothorac Surg ; 16(1): 152, 2021 May 29.
Artigo em Inglês | MEDLINE | ID: mdl-34051842

RESUMO

BACKGROUND: Intra-aortic foreign body (IAFB) is uncommon, which is usually caused by a rupture of the catheter or guidewire. IAFB can cause catastrophic complications, including arrhythmia, embolization of guidewire fragments, intravascular clipping of the guidewire and vascular perforation. However, there are still no guidelines on removal and management of IAFB. Here, we present a rare case of fractured cerebral angiographic guidewires in the aorta that resulted in multiple cerebral infarctions. CASE PRESENTATION: A 50-year-old man experienced new cerebral infarction after cerebral angiography. Computed tomography and echocardiography demonstrated foreign bodies in his ascending aorta and aortic arch. Open surgery was successfully performed to retrieve the guidewires. The postoperation and follow-up was uneventful. CONCLUSION: It is very important for interventional radiologists to check the catheter and guidewire after operation and perform ultrasound or radiograph to prevent IAFB. Additionally, the effective management of IAFB requires the early detection and the selection of appropriate treatment options, as well as long-time follow up.


Assuntos
Aorta , Angiografia Cerebral/instrumentação , Infarto Cerebral/etiologia , Corpos Estranhos/cirurgia , Aorta/diagnóstico por imagem , Infarto Cerebral/diagnóstico por imagem , Ecocardiografia , Corpos Estranhos/complicações , Corpos Estranhos/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia , Tomografia Computadorizada por Raios X , Lesões do Sistema Vascular
10.
J Card Surg ; 36(8): 2890-2900, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34047395

RESUMO

BACKGROUND: Anomalous origin of one pulmonary artery from the aorta is a rare congenital anomaly affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. Early surgical treatment has been proven safe with excellent results. The surgical approach and technique is challenging and should be decided ahead before the patient to surgery. Different techniques were described including direct reimplantation, conduit interposition, aortic ring flap. AIM: We present a neonate with anomalous origin of the right pulmonary artery from the aorta and discuss the surgical technique and complications in the literature.


Assuntos
Cardiopatias Congênitas , Malformações Vasculares , Aorta/diagnóstico por imagem , Aorta/cirurgia , Humanos , Recém-Nascido , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Retalhos Cirúrgicos
11.
Int J Cardiol ; 337: 119-126, 2021 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-33933510

RESUMO

BACKGROUND: Reproducible aortic diameter measurements are crucial for assessment of aortic growth and aneurysm formation in patients with Marfan syndrome. The objective of this study was to perform an intraindividual comparison of aortic measurements at 1.5 T and 3 T using non-contrast magnetic resonance angiography (MRA) in pre-surgical and post-surgical Marfan patients. METHODS: Forty consecutive Marfan patients were retrospectively evaluated by ECG-gated 2D balanced steady-state free precession (bSSFP) MRA at 1.5 T and 3 T after 363 ± 58 days. 24 patients were before and 16 patients after aortic root surgery. Two readers independently measured aortic diameters at seven aortic levels and rated the image quality/image artifacts (1 = poor/severe, 4 = excellent/none). Contrast-to-noise ratio (CNR) and signal intensity slopes between aortic lumen and vessel walls were semiautomatically determined. RESULTS: In pre-surgical Marfan patients, interobserver agreement of aortic root diameter measurements was significantly higher at 3 T compared to 1.5 T (p < 0.05). In post-surgical Marfan patients, image quality and artifacts were significantly worse at 3 T compared to 1.5 T (p < 0.05). CNR was higher at 3 T compared to 1.5 T at all aortic levels. Significantly steeper slopes of signal intensity curves were observed at 3 T at all aortic levels (p < 0.001). CONCLUSIONS: In pre-surgical Marfan patients, non-contrast MRA provides higher reproducibility of aortic diameter measurements at 3 T compared to 1.5 T. In post-surgical Marfan patients, metallic implants result in significantly worse imaging artifacts and reduced image quality at 3 T compared to 1.5 T. Therefore, we propose to monitor the thoracic aorta with non-contrast MRA at 3 T in pre-surgical Marfan patients and at 1.5 T in post-surgical Marfan patients.


Assuntos
Síndrome de Marfan , Aorta/diagnóstico por imagem , Meios de Contraste , Humanos , Angiografia por Ressonância Magnética , Síndrome de Marfan/diagnóstico por imagem , Síndrome de Marfan/cirurgia , Reprodutibilidade dos Testes , Estudos Retrospectivos
12.
Am J Physiol Heart Circ Physiol ; 320(6): H2313-H2323, 2021 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-33961507

RESUMO

Vascular calcification is associated with a higher incidence of cardiovascular events, but its prevalence in different vascular zones and the influence of demographics, risk factors, and morphometry remain insufficiently understood. Computerized tomography angiography scans from 211 subjects 5-93 yr old (mean age 47 ± 24 yr, 127 M/84 F) were used to build 3D vascular reconstructions and measure arterial diameters, tortuosity, and calcification volumes in six vascular zones spanning from the ascending thoracic aorta to the pelvic arteries. A machine learning random forest algorithm was used to determine the associations between calcification in each zone with demographics, risk factors, and vascular morphometry. Calcification appeared during the fourth decade of life and was present in all subjects after 65 yr. The abdominal aorta and the iliofemoral segment were the first to develop calcification, whereas the ascending thoracic aorta was the last. Demographics and risk factors explained 33-59% of the variation in calcification. Age, creatinine level, body mass index, coronary artery disease, and hypertension were the strongest contributors, whereas the effects of sex, race, tobacco use, diabetes, dyslipidemia, and alcohol and substance use disorders on calcification were small. Vascular morphometry did not directly and independently affect calcium burden. Vascular zones develop calcification asynchronously, with distal segments calcifying first. Understanding the influence of demographics and risk factors on calcium prevalence can help better understand the disease pathophysiology and may help with the early identification of patients that are at higher risk of cardiovascular events.NEW & NOTEWORTHY We investigated the prevalence of vascular calcification in different zones of the aorta and pelvic arteries using computerized tomography angiography reconstructions and have applied machine learning to determine how calcification is affected by demographics, risk factors, and morphometry. The presented data can help identify patients at higher risk of developing vascular calcification that may lead to cardiovascular events.


Assuntos
Aorta Abdominal/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Calcificação Vascular/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Aorta/diagnóstico por imagem , Criança , Pré-Escolar , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Imageamento Tridimensional , Aprendizado de Máquina , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de Risco , Calcificação Vascular/diagnóstico por imagem , Adulto Jovem
13.
Biomed Res Int ; 2021: 9995749, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33997053

RESUMO

Aortic injury, particularly traumatic aortic dissection caused by thoracic and abdominal injuries, is extremely rare. The diagnosis rate of blunt aortic injury caused by chest and abdominal injuries is often low, and its clinical manifestations are atypical. Once missed or misdiagnosed, the consequences are serious. Early diagnosis of traumatic aortic injury in complex thoracic and abdominal injuries is a key factor in reducing the mortality of trauma patients. Among all trauma patients treated in our department from December 2018 to December 2020, we diagnosed four cases of aortic injury, including three cases of aortic dissection and one case of intramural hematoma. Successful surgical treatment and clinical outcome were achieved in all four patients. We found that early diagnosis and surgical treatment can help to reduce the mortality of patients with traumatic aortic injury and improve the prognosis.


Assuntos
Aorta , Traumatismos Torácicos , Lesões do Sistema Vascular , Traumatismos Abdominais/complicações , Traumatismos Abdominais/diagnóstico , Traumatismos Abdominais/cirurgia , Adulto , Aneurisma Dissecante , Aorta/diagnóstico por imagem , Aorta/lesões , Diagnóstico Precoce , Feminino , Hematoma , Humanos , Masculino , Pessoa de Meia-Idade , Traumatismos Torácicos/complicações , Traumatismos Torácicos/diagnóstico , Traumatismos Torácicos/cirurgia , Lesões do Sistema Vascular/diagnóstico , Lesões do Sistema Vascular/etiologia , Lesões do Sistema Vascular/cirurgia
14.
Khirurgiia (Mosk) ; (5): 125-128, 2021.
Artigo em Russo | MEDLINE | ID: mdl-33977707

RESUMO

Heart or great outflow vessel injury followed by cardiac tamponade is an extremely rare event. However, it is the most dangerous complication of sternal puncture requiring urgent surgical intervention. The effectiveness of specialized care directly depends on its availability and timely diagnosis. Echocardiography is the simplest and informative method for objective diagnosis. We report successful surgical treatment of a 21-year-old patient with intrapericardial injury of the ascending aorta following sternal puncture.


Assuntos
Tamponamento Cardíaco , Adulto , Aorta/diagnóstico por imagem , Tamponamento Cardíaco/diagnóstico , Tamponamento Cardíaco/etiologia , Tamponamento Cardíaco/cirurgia , Ecocardiografia , Humanos , Punções , Esterno/diagnóstico por imagem , Esterno/cirurgia , Adulto Jovem
15.
Nat Commun ; 12(1): 2628, 2021 05 11.
Artigo em Inglês | MEDLINE | ID: mdl-33976159

RESUMO

Thoracic aortic aneurysm, as occurs in Marfan syndrome, is generally asymptomatic until dissection or rupture, requiring surgical intervention as the only available treatment. Here, we show that nitric oxide (NO) signaling dysregulates actin cytoskeleton dynamics in Marfan Syndrome smooth muscle cells and that NO-donors induce Marfan-like aortopathy in wild-type mice, indicating that a marked increase in NO suffices to induce aortopathy. Levels of nitrated proteins are higher in plasma from Marfan patients and mice and in aortic tissue from Marfan mice than in control samples, indicating elevated circulating and tissue NO. Soluble guanylate cyclase and cGMP-dependent protein kinase are both activated in Marfan patients and mice and in wild-type mice treated with NO-donors, as shown by increased plasma cGMP and pVASP-S239 staining in aortic tissue. Marfan aortopathy in mice is reverted by pharmacological inhibition of soluble guanylate cyclase and cGMP-dependent protein kinase and lentiviral-mediated Prkg1 silencing. These findings identify potential biomarkers for monitoring Marfan Syndrome in patients and urge evaluation of cGMP-dependent protein kinase and soluble guanylate cyclase as therapeutic targets.


Assuntos
Aneurisma da Aorta Torácica/patologia , Proteína Quinase Dependente de GMP Cíclico Tipo I/metabolismo , Síndrome de Marfan/complicações , Guanilil Ciclase Solúvel/metabolismo , Animais , Aorta/citologia , Aorta/diagnóstico por imagem , Aorta/efeitos dos fármacos , Aorta/patologia , Aneurisma da Aorta Torácica/diagnóstico , Aneurisma da Aorta Torácica/etiologia , Aneurisma da Aorta Torácica/prevenção & controle , Biomarcadores/sangue , Biomarcadores/metabolismo , Carbazóis/administração & dosagem , GMP Cíclico/sangue , GMP Cíclico/metabolismo , Modelos Animais de Doenças , Feminino , Fibrilina-1/genética , Técnicas de Silenciamento de Genes , Humanos , Masculino , Síndrome de Marfan/sangue , Síndrome de Marfan/genética , Síndrome de Marfan/patologia , Camundongos , Músculo Liso Vascular/citologia , Mutação , Miócitos de Músculo Liso , Óxido Nítrico/metabolismo , Doadores de Óxido Nítrico/administração & dosagem , Cultura Primária de Células , Guanilil Ciclase Solúvel/antagonistas & inibidores , Ultrassonografia
16.
J Cardiothorac Surg ; 16(1): 108, 2021 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-33892751

RESUMO

BACKGROUND: Abdominal aortic aneurysm (AAA) is an asymptomatic condition characterized by progressive dilatation of the aorta. The purpose of this study is to identify important 2D-TTE aortic indices associated with AAA as predictive tools for undiagnosed AAA. METHODS: In this retrospective study, we evaluated the size of the ascending aorta in patients without known valvular diseases or hemodynamic compromise as predictive tool for undiagnosed AAA. We studied the tubular ascending aorta of 170 patients by 2-dimensional transthoracic echocardiography (2D-TTE). Patients were further divided into two groups, 70 patients with AAA and 100 patients without AAA with normal imaging results. RESULTS: Dilatation of tubular ascending aorta was measured in patients with AAA compared to the group with absent AAA (37.5 ± 4.8 mm vs. 31.2 ± 3.6 mm, p < 0.001, respectively) and confirmed by computed tomographic (CT) (35.6 ± 5.1 mm vs. 30.8 ± 3.7 mm, p < 0.001, respectively). An increase in tubular ascending aorta size was associated with the presence of AAA by both 2D-TTE and CT (r = 0.40, p < 0.001 and r = 0.37, p < 0.001, respectively). The tubular ascending aorta (D diameter) size of ≥33 mm or ≥ 19 mm/m2 presented with 2-4 times more risk of AAA presence (OR 4.68, CI 2.18-10.25, p = 0.001 or OR 2.63, CI 1.21-5.62, p = 0.02, respectively). In addition, multiple logistic regression analysis identified tubular ascending aorta (OR 1.46, p < 0.001), age (OR 1.09, p = 0.013), gender (OR 0.12, p = 0.002), and LVESD (OR 1.24, p = 0.009) as independent risk factors of AAA presence. CONCLUSIONS: An increased tubular ascending aortic diameter, measured by 2D-TTE, is associated with the presence of AAA. Routine 2D-TTE screening for silent AAA by means of ascending aorta analysis, may appear useful especially in older patients with a dilated tubular ascending aorta (≥33 mm).


Assuntos
Aneurisma da Aorta Abdominal/complicações , Dilatação Patológica/complicações , Ecocardiografia/métodos , Idoso , Idoso de 80 Anos ou mais , Aorta/diagnóstico por imagem , Dilatação , Feminino , Humanos , Masculino , Programas de Rastreamento/métodos , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X
18.
Pediatr Cardiol ; 42(5): 1157-1161, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33847800

RESUMO

Progressive aortic dilation is common in Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS). Risk factors for progression are poorly understood. Normal variation in the aortic root (AoR) rotational position relative to the left ventricular base may impact this risk. We aimed to assess the relationship between the rotational position of the AoR and aortic dimensions in this population. Patients with a genetic diagnosis of MFS or LDS were included. AoR and ascending aorta (AAo) dimensions were measured from the first and most recent transthoracic echocardiogram. The AoR rotational angle was measured in the parasternal short-axis plane in diastole. Linear regression was used to study the correlation between AoR rotation angle and aortic dimensions. 53 MFS and 14 LDS patients were included (age 11.5 ± 5.8 years at first TTE and 21.2 ± 7.2 years at most recent, 68% male). The mean indexed AoR and AAo values were 2.26 ± 0.58 cm/m2 and 1.64 ± 0.35 cm/m2 at the first TTE and 1.98 ± 0.39 cm/m2 and 1.45 ± 0.25 cm/m2 at the most recent TTE, respectively. The mean AoR rotational angle was 8 ± 14°. AoR rotational angle was central (- 9 to + 14°) in 42, clockwise (≥ + 15°) in 19, and counterclockwise (≤ -10°) in 6. The six outliers with counterclockwise position were excluded. There was a positive association between the AoR rotation angle and most recent TTE indexed AoR (r2 = 0.08, p = 0.02) and AAo sizes (r2 = 0.08, p = 0.02). There was no association between AoR rotational angle and rate of change in indexed AoR size (p = 0.8). There was a positive association between AoR rotation angle and rate of change in indexed AAo size (r2 = 0.10, p = 0.01). There is an association between clockwise rotational position of the AoR and increased AoR and AAo dimensions in children and young adults with MFS and LDS patients. The rotational position of the AoR may guide follow-up in these patient populations. However, this potential risk factor for dilation warrants further investigation.


Assuntos
Aorta/patologia , Doenças da Aorta/etiologia , Dilatação Patológica/etiologia , Síndrome de Loeys-Dietz/complicações , Síndrome de Marfan/complicações , Adolescente , Adulto , Aorta/diagnóstico por imagem , Criança , Pré-Escolar , Ecocardiografia , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
19.
J Cardiothorac Surg ; 16(1): 85, 2021 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-33858452

RESUMO

BACKGROUND: Behçet's disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet's disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. CASE PRESENTATION: Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special "skirted" conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. CONCLUSIONS: The surgical treatment of cardiovascular manifestations of Behçet's disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.


Assuntos
Falso Aneurisma/cirurgia , Aneurisma Aórtico/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Síndrome de Behçet , Implante de Prótese de Valva Cardíaca , Adulto , Falso Aneurisma/diagnóstico por imagem , Falso Aneurisma/etiologia , Aorta/diagnóstico por imagem , Aorta/cirurgia , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/etiologia , Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Síndrome de Behçet/complicações , Ecocardiografia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Masculino , Falha de Prótese , Reoperação
20.
J Cardiothorac Surg ; 16(1): 83, 2021 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-33858466

RESUMO

BACKGROUND: Retroaortic innominate vein (RIV) is a rare vascular abnormality. Although RIV itself is asymptomatic, its presence in patients with partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) is surgically challenging because a simple Warden procedure is impossible. CASE PRESENTATION: A 16-year-old girl was diagnosed with tetralogy of Fallot, secundum, and sinus venosus atrial septal defect (ASD) at birth. She underwent total correction of tetralogy of Fallot and ASD closure at the age of 14-months. However, the diagnosis of PAPVR was missed. At the age of 16, she developed dyspnea on exercise. Echocardiography demonstrated severe pulmonary regurgitation, mild tricuspid regurgitation, and D-shaped left ventricle with paradoxical septal motion along with RIV and sinus venous ASD. Computed tomography confirmed RIV and PAPVR. Systemic and pulmonary venous blood pathways were separated by bovine pericardial patch, and pulmonary valve replacement was performed. Postoperative echocardiography demonstrated improvement of D-shaped left ventricle and laminar flow through the SVC and pulmonary veins. Postoperative computed tomography showed a well-reconstructed SVC and pulmonary venous pathway without stenosis. After an uneventful postoperative course, patient was discharged. CONCLUSIONS: PAPVR in patients with RIV may be surgically challenging to repair. We report the first case of successfully repaired PAPVR associated with RIV.


Assuntos
Veias Braquiocefálicas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Diagnóstico Ausente/efeitos adversos , Veias Pulmonares/cirurgia , Síndrome de Cimitarra/cirurgia , Adolescente , Animais , Aorta/anormalidades , Aorta/diagnóstico por imagem , Veias Braquiocefálicas/anormalidades , Veias Braquiocefálicas/diagnóstico por imagem , Bovinos , Dispneia/etiologia , Dispneia/cirurgia , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Humanos , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Reoperação , Síndrome de Cimitarra/diagnóstico , Tomografia Computadorizada por Raios X , Veia Cava Superior/anormalidades , Veia Cava Superior/cirurgia
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