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1.
Cesk Patol ; 60(2): 124-128, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39138015

RESUMO

In developed countries, calcific aortic stenosis (CAS) has become the most common acquired valvular disease and cause for valve replacement. The prevalence of the disease increases with age, reaching over 5 % in adults over 75 years of age. The cases of CAS are classified as either of a previously normal (tricuspid) aortic valve (senile, syn. age - related, "sclerotic" type), or based on a congenitally malformed, usually bicuspid aortic valve. This paper is a brief summary of our 5 previous publications from the years 2007 - 2021, devoted to histopathology of CAS, namely to vascularization, inflammatory infiltrate and metaplastic ossification of the valve, and also to topography of these lesions in individual valve cusps. We conclude that calcification of the aortic valve is not a passive degenerative lesion, but an active multifactorial inflammatory process driven by cells native to the aortic valve. Pathogenesis of CAS is similar to that of atherosclerosis.


Assuntos
Estenose da Valva Aórtica , Valva Aórtica , Calcinose , Humanos , Estenose da Valva Aórtica/patologia , Estenose da Valva Aórtica/etiologia , Calcinose/patologia , Valva Aórtica/patologia , Valva Aórtica/anormalidades , Inflamação/patologia
3.
JACC Cardiovasc Interv ; 17(14): 1667-1675, 2024 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-39048253

RESUMO

BACKGROUND: Outcomes from transcatheter aortic valve replacement (TAVR) in low-surgical risk patients with bicuspid aortic stenosis beyond 2 years are limited. OBJECTIVES: This study aimed to evaluate 3-year clinical and echocardiographic outcomes from the Evolut Low Risk Bicuspid Study. METHODS: The Evolut Low Risk Bicuspid Study is a prospective, multicenter, single-arm study conducted in 25 U.S. CENTERS: Patients with severe aortic stenosis at low surgical risk with bicuspid aortic valve anatomy (all subtypes) underwent TAVR with a self-expanding, supra-annular Evolut R or PRO (Medtronic) bioprosthesis. An independent clinical events committee adjudicated all deaths and endpoint-related adverse events, and a central echocardiographic core laboratory assessed hemodynamic endpoints. RESULTS: An attempted implant was performed in 150 patients from December 2018 to October 2019. The mean age was 70.3 ± 5.5 years, 48% (72/150) of the patients were women, and the mean Society of Thoracic Surgeons Predicted Risk of Mortality score was 1.3% (Q1-Q3: 0.9%-1.7%). Sievers type 1 was the dominant bicuspid morphology (90.7%, 136/150). The Kaplan-Meier rates of all-cause mortality or disabling stroke were 1.3% (95% CI: 0.3%-5.3%) at 1 year, 3.4% (95% CI: 1.4%-8.1%) at 2 years, and 4.1% (95% CI: 1.6%-10.7%) at 3 years. The incidence of new permanent pacemaker implantation was 19.4% (95% CI: 12.4%-29.6%) at 3 years. There were no instances of moderate or severe paravalvular aortic regurgitation at 2 and 3 years after TAVR. CONCLUSIONS: The 3-year results from the Evolut Low Risk Bicuspid Study demonstrate low rates of all-cause mortality or disabling stroke and favorable hemodynamic performance.


Assuntos
Estenose da Valva Aórtica , Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Bioprótese , Próteses Valvulares Cardíacas , Hemodinâmica , Desenho de Prótese , Substituição da Valva Aórtica Transcateter , Humanos , Feminino , Masculino , Estenose da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/mortalidade , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/mortalidade , Substituição da Valva Aórtica Transcateter/instrumentação , Idoso , Fatores de Risco , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Aórtica/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Valva Aórtica/anormalidades , Medição de Risco , Doença da Válvula Aórtica Bicúspide/cirurgia , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Estados Unidos/epidemiologia , Índice de Gravidade de Doença , Complicações Pós-Operatórias/mortalidade , Recuperação de Função Fisiológica , Idoso de 80 Anos ou mais , Pessoa de Meia-Idade , Doenças das Valvas Cardíacas/fisiopatologia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Doenças das Valvas Cardíacas/mortalidade
5.
BMC Cardiovasc Disord ; 24(1): 367, 2024 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-39014301

RESUMO

OBJECTIVE: To investigate the accuracy and consistency of MDCT and TEE in the preoperative assessment of aortic root surgery. METHODS: From January 2021 to September 2022, 118 patients who underwent aortic root surgery were included in this study. All patients underwent high-quality preoperative MDCT and TEE examinations, and the examination results were independently measured and assessed by two senior radiologists or ultrasound specialists. Bland-Altman analysis and Pearson correlation testing were employed to assess the correlation and consistency between MDCT and TEE. These analyses were then compared with actual intraoperative measurement data. RESULTS: Among all the patients, 73 (61.86%) had tricuspid aortic valve (TAV), and 45 (38.14%) had bicuspid aortic valve (BAV). A comparison between the TEE and MDCT measurements showed that for the annulus diameter, the area-derived diameter had the best correlation and agreement. For the sinus of Valsalva diameter, the circumference-derived diameter was optimal. However, for the STJ diameter, the minimum cross-sectional diameter showed the best agreement with TEE. In contrast, measurements of geometric height showed a weaker correlation and agreement. CONCLUSION: Contrast-enhanced MDCT can be a valuable tool for perioperative evaluation in aortic root surgery, with good correlation, consistency, and feasibility when compared to TEE. The choice of MDCT measurement methodology, specifically area-derived and circumference-derived diameter, proved to be more accurate than other methods. Further research is required to enhance the understanding of aortic valve repair and associated imaging techniques.


Assuntos
Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Ecocardiografia Transesofagiana , Tomografia Computadorizada Multidetectores , Valor Preditivo dos Testes , Humanos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Reprodutibilidade dos Testes , Doença da Válvula Aórtica Bicúspide/cirurgia , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Adulto , Estudos Retrospectivos , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Cuidados Pré-Operatórios
6.
PLoS One ; 19(7): e0306515, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38954721

RESUMO

BACKGROUND: Bicuspid aortic valves (BAV) are frequently associated with ascending aortic aneurysms. The etiology is incompletely understood, but genetic factors, in addition to flow perturbations, are likely involved. Since loss of contractility and elaboration of extracellular matrix in the vessel wall are features of BAV-associated aortopathy, phenotypic modulation of smooth muscle cells (SMCs) may play a role. METHODS: Ascending aortic tissue was collected intra-operatively from 25 individuals with normal (i.e., tricuspid) aortic valves (TAV) and from 25 individuals with BAVs. For both TAV and BAV, 10 patients had non-dilated (ND) and 15 patients had dilated (D) aortas. SMCs were isolated and cultured from a subset of patients from each group. Aortic tissue and SMCs were fluorescently immunolabeled for SMC phenotypic markers (i.e., alpha-smooth muscle actin (ASMA, contractile), vimentin (synthetic) and p16INK4a and p21Cip1 (senescence). SMCs were also analyzed for replicative senescence in culture. RESULTS: In normal-sized and dilated BAV aortas, SMCs switched from the contractile state to either synthetic or senescent phenotypes, as observed by loss of ASMA (ND: P = 0.001, D: P = 0.002) and associated increases in vimentin (ND: P = 0.03, D: P = 0.004) or p16/p21 (ND: P = 0.03, D: P<0.0001) compared to TAV. Dilatation of the aorta exacerbated SMC phenotypic switching in both BAV and TAV aortas (all P<0.05). In SMCs cultured from normal and dilated aortas, those isolated from BAV reached replicative senescence faster than those from TAV aortas (all P = 0.02). Furthermore, there was a stark inverse correlation between ASMA and cell passage number in BAV SMCs (ND: P = 0.0006, D: P = 0.01), but not in TAV SMCs (ND: P = 0.93, D: P = 0.20). CONCLUSIONS: The findings of this study provide direct evidence from cell culture studies implying that SMCs switch from the contractile state to either synthetic or senescent phenotypes in the non-dilated BAV aorta. In cultured SMCs from both non-dilated and dilated aortas, we found that this process may precede dilatation and accompany aneurysm development in BAV. Our findings suggest that therapeutically targeting SMC phenotypic modulation in BAV patients may be a viable option to prevent or delay ascending aortic aneurysm formation.


Assuntos
Aorta , Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Miócitos de Músculo Liso , Fenótipo , Humanos , Valva Aórtica/patologia , Valva Aórtica/metabolismo , Valva Aórtica/anormalidades , Doença da Válvula Aórtica Bicúspide/patologia , Doença da Válvula Aórtica Bicúspide/metabolismo , Miócitos de Músculo Liso/metabolismo , Miócitos de Músculo Liso/patologia , Doenças das Valvas Cardíacas/metabolismo , Doenças das Valvas Cardíacas/patologia , Aorta/patologia , Aorta/metabolismo , Masculino , Pessoa de Meia-Idade , Feminino , Dilatação Patológica , Adulto , Senescência Celular , Células Cultivadas , Idoso , Actinas/metabolismo , Aneurisma Aórtico/metabolismo , Aneurisma Aórtico/patologia , Vimentina/metabolismo
7.
Braz J Cardiovasc Surg ; 39(4): e20230438, 2024 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-39038201

RESUMO

OBJECTIVES: The aims of the present study were to compare the long-term outcomes for ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement between patients with bicuspid aortic valve (BAV) and tricuspid aortic valve ( TAV). METHODS: This retrospective study included 310 patients who had undergone isolated aortic valve replacement with an ascending aorta diameter ≤ 45 mm between January 2010 and September 2021. The patients were divided into BAV group (n=90) and TAV group (n=220). The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. RESULTS: Overall survival was 89 ± 4% in the BAV group vs. 75 ± 6% in the TAV group at 10 years postoperatively (P=0.007), yet this difference disappeared after adjusting exclusively for age (P=0.343). The mean annual growth rate of the ascending aorta was similar between the two groups during follow-up (0.5 ± 0.6 mm/year vs. 0.4 ± 0.5 mm/year; P=0.498). Ten-year freedom from adverse aortic events was 98.1% in the BAV group vs. 95.0% in the TAV group (P=0.636). Multivariable analysis revealed preoperative ascending aorta diameter to be a significant predictor of adverse aortic events (hazard ratio: 1.76; 95% confidence interval: 1.33 to 2.38; P<0.001). CONCLUSION: Our study revealed that the long-term survival and the risks of adverse aortic events between BAV and TAV patients were similar after isolated aortic valve replacement. BAV was not a risk factor of adverse aortic events.


Assuntos
Aorta , Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Feminino , Estudos Retrospectivos , Doença da Válvula Aórtica Bicúspide/cirurgia , Doença da Válvula Aórtica Bicúspide/complicações , Pessoa de Meia-Idade , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Idoso , Implante de Prótese de Valva Cardíaca/mortalidade , Aorta/cirurgia , Doenças das Valvas Cardíacas/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/mortalidade , Valva Tricúspide/cirurgia , Progressão da Doença , Fatores de Risco , Resultado do Tratamento , Complicações Pós-Operatórias
9.
Adv Exp Med Biol ; 1441: 777-796, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38884748

RESUMO

The great arteries of the vertebrate carry blood from the heart to the systemic circulation and are derived from the pharyngeal arch arteries. In higher vertebrates, the pharyngeal arch arteries are a symmetrical series of blood vessels that rapidly remodel during development to become the asymmetric aortic arch arteries carrying oxygenated blood from the left ventricle via the outflow tract. At the base of the aorta, as well as the pulmonary trunk, are the semilunar valves. These valves each have three leaflets and prevent the backflow of blood into the heart. During development, the process of aortic arch and valve formation may go wrong, resulting in cardiovascular defects, and these may, at least in part, be caused by genetic mutations. In this chapter, we will review models harboring genetic mutations that result in cardiovascular defects affecting the great arteries and the semilunar valves.


Assuntos
Aorta Torácica , Animais , Aorta Torácica/anormalidades , Humanos , Mutação , Modelos Animais de Doenças , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia
10.
Adv Exp Med Biol ; 1441: 741-759, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38884746

RESUMO

The following semilunar valve defects and aortic arch anomalies are called simple defects because there is a single problem that can be well described. Based on the degree of malformation and hemodynamic consequence, these simple lesions can however be life threatening immediately after birth. They all affect either the left or right outflow tract or the aortic arch.


Assuntos
Aorta Torácica , Humanos , Aorta Torácica/anormalidades , Aorta Torácica/fisiopatologia , Aorta Torácica/diagnóstico por imagem , Valva Aórtica/anormalidades , Cardiopatias Congênitas/terapia , Cardiopatias Congênitas/fisiopatologia
11.
Adv Exp Med Biol ; 1441: 761-775, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38884747

RESUMO

Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic valve disease will be discussed including the key role of NOTCH1 mutations. In addition, the complex trait of bicuspid aortic valve disease will be outlined, both in sporadic/familial cases and in the context of associated syndromes, such as Alagille, Williams, and Kabuki syndromes. Aortic arch abnormalities particularly coarctation of the aorta and interrupted aortic arch, including their association with syndromes such as Turner and 22q11 deletion, respectively, are also discussed. Finally, the genetic basis of congenital pulmonary valve stenosis is summarized, with particular note to Ras-/mitogen-activated protein kinase (Ras/MAPK) pathway syndromes and other less common associations, such as Holt-Oram syndrome.


Assuntos
Aorta Torácica , Valva Aórtica , Humanos , Aorta Torácica/anormalidades , Aorta Torácica/patologia , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Anormalidades Múltiplas/genética , Anormalidades Múltiplas/patologia , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia , Doença da Válvula Aórtica Bicúspide/genética , Estenose da Valva Pulmonar/genética , Mutação , Receptor Notch1/genética , Valvopatia Aórtica/genética , Doenças das Valvas Cardíacas/genética , Doenças das Valvas Cardíacas/patologia , Calcinose/genética , Calcinose/patologia , Doenças Hematológicas/genética , Doenças Hematológicas/patologia , Doenças Vestibulares/genética , Doenças Vestibulares/patologia
12.
Clin Interv Aging ; 19: 695-703, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38711477

RESUMO

Purpose: In mixed aortic valve disease (MAVD), the results of transcatheter aortic valve replacement (TAVR) are conflicting. There is limited data on the outcomes of TAVR in patients with bicuspid aortic valve (BAV) and MAVD. The objective of this study is to compare outcomes after TAVR in BAV patients with MAVD and predominant aortic stenosis (PAS). Patients and Methods: Patients with BAV who underwent TAVR between January 2016 and April 2023 were included. The primary outcome was device success. The secondary endpoints were periprocedural mortality and other complications as defined by the Valve Academic Research Consortium-3 (VARC-3). Propensity score matching was used to minimize potential confounding. Results: A total of 262 patients were included in this study, 83 of whom had MAVD. The median age was 72 years, and 55.7% were male. The baseline comorbidity risk files were comparable between the two groups. Patients with MAVD had more mitral regurgitation, tricuspid regurgitation and pulmonary hypertension, larger annular and left ventricular outflow tract dimensions, and more severe calcification than PAS. In the unmatched population, MAVD patients had similar device success rate (69.9% vs 79.9%, P=0.075) and 30-day mortality (3.6% vs 3.4%, P=1) compared to PAS. Propensity score matching resulted in 66 patient pairs. Device success rate were still comparable in the matched population. Other clinical outcomes, including stroke, bleeding (type 2-4), major vascular complications, acute kidney injury (stage 2-4) and permanent pacemaker implantation, were comparable between the two groups. Multivariable logistic regression analysis did not show MAVD to be an independent negative predictor of device success. At one year, survival was similar between patients with MAVD and those with PAS. Conclusion: For the bicuspid valve, patients with MAVD had a more challenging anatomy. MAVD patients associated with comparable 30-day clinical outcomes after TAVR compared to PAS patients in patients with BAV.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Complicações Pós-Operatórias , Pontuação de Propensão , Substituição da Valva Aórtica Transcateter , Humanos , Masculino , Feminino , Estenose da Valva Aórtica/cirurgia , Idoso , Doença da Válvula Aórtica Bicúspide/cirurgia , Idoso de 80 Anos ou mais , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Pessoa de Meia-Idade , Fatores de Risco , Doenças das Valvas Cardíacas/cirurgia
14.
Ann Card Anaesth ; 27(1): 51-52, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-38722121

RESUMO

ABSTRACT: The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography.


Assuntos
Valva Aórtica , Ecocardiografia Transesofagiana , Humanos , Feminino , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Pessoa de Meia-Idade , Ecocardiografia Transesofagiana/métodos , Insuficiência da Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia
16.
Heart Lung ; 67: 144-151, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38762962

RESUMO

BACKGROUND: Despite comprising almost half of all patients undergoing valvular repair, data on transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic stenosis (BAS) are limited. OBJECTIVE: We aimed to evaluate whether there are any sex differences in trends and outcomes of TAVR in this population. METHODS: We utilized the National Inpatient Sample from 2012 to 2020 to identify admissions with BAS who underwent TAVR and analyzed trends and outcomes. Our primary outcome was in-hospital mortality and secondary outcomes were in-hospital complications. We used two models to adjust for demographics (A) and interventions (B). RESULTS: Between 2012 to 2020, there were 76,540 hospitalizations for BAS patients who underwent AVR, among which 6,010 (7.9 %) underwent TAVR. There was an overall increasing trend in number of TAVR cases with a decreasing trend in mortality (2013: 8.7 %, 2020: 1.3 %). TAVR was performed more in males (61.1% vs 38.9 %). Despite the worse baseline characteristics in males, in-hospital mortality (2.4% vs. 1.5 %; OR: 1.584; 95 % CI: 0.621-4.038; p = 0.335) and secondary outcomes were similar across both sexes, even after adjusting for demographics and interventions. CONCLUSION: TAVR in BAS has grown rapidly in the last decade. Males comprised the majority and had more comorbidities, but mortality and complications were similar in both sexes. Despite the increasing number of cases, a decreasing trend in mortality was observed for both sexes ultimately approaching that of SAVR, suggesting that TAVR may be a safe alternative among eligible males and females with bicuspid AS.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Mortalidade Hospitalar , Substituição da Valva Aórtica Transcateter , Humanos , Substituição da Valva Aórtica Transcateter/métodos , Substituição da Valva Aórtica Transcateter/estatística & dados numéricos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Masculino , Feminino , Estenose da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/epidemiologia , Estenose da Valva Aórtica/complicações , Doença da Válvula Aórtica Bicúspide/cirurgia , Doença da Válvula Aórtica Bicúspide/complicações , Mortalidade Hospitalar/tendências , Idoso , Fatores Sexuais , Estados Unidos/epidemiologia , Idoso de 80 Anos ou mais , Estudos Retrospectivos , Complicações Pós-Operatórias/epidemiologia , Resultado do Tratamento , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Fatores de Risco , Doenças das Valvas Cardíacas/cirurgia , Doenças das Valvas Cardíacas/complicações
17.
J Cardiothorac Surg ; 19(1): 281, 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38715080

RESUMO

Injury to coronary arteries during mitral surgery is a rare but life-threatening procedural complication, an anomalous origin and course of the left circumflex artery (LCx) increase this risk. Recognizing the anomaly by the characteristic angiographic pattern and identifying its relationship with the surrounding anatomical structure using imaging techniques, mainly transesophageal echocardiography (TOE) or coronary computed tomography angiography (CCTA), is of crucial importance in setting up the best surgical strategy. We report a case of anomalous origin of a circumflex artery (LCx) from the proximal portion of the right coronary artery (RCA) with a pathway running retroaortically through the mitro-aortic space. An integrated diagnostic approach using a multidisciplinary team with a cardiologist and an imaging radiologist allowed us to decide the surgical strategy. We successfully performed a mitral valvular repair using a minimally invasive minithoracotomic approach and implanting a complete semirigid ring.


Assuntos
Valva Aórtica , Anomalias dos Vasos Coronários , Ecocardiografia Transesofagiana , Valva Mitral , Humanos , Valva Aórtica/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/anormalidades , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Anomalias dos Vasos Coronários/cirurgia , Anomalias dos Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Vasos Coronários/diagnóstico por imagem , Valva Mitral/cirurgia , Valva Mitral/diagnóstico por imagem , Valva Mitral/anormalidades , Insuficiência da Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem
18.
Am J Physiol Heart Circ Physiol ; 327(1): H56-H66, 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38758128

RESUMO

Mouse models of congenital aortic valve malformations are useful for studying disease pathobiology, but most models have incomplete penetrance [e.g., ∼2 to 77% prevalence of bicuspid aortic valves (BAVs) across multiple models]. For longitudinal studies of pathologies associated with BAVs and other congenital valve malformations, which manifest over months in mice, it is operationally inefficient, economically burdensome, and ethically challenging to enroll large numbers of mice in studies without first identifying those with valvular abnormalities. To address this need, we established and validated a novel in vivo high-frequency (30 MHz) ultrasound imaging protocol capable of detecting aortic valvular malformations in juvenile mice. Fifty natriuretic peptide receptor 2 heterozygous mice on a low-density lipoprotein receptor-deficient background (Npr2+/-;Ldlr-/-; 32 males and 18 females) were imaged at 4 and 8 wk of age. Fourteen percent of the Npr2+/-;Ldlr-/- mice exhibited features associated with aortic valve malformations, including 1) abnormal transaortic flow patterns on color Doppler (recirculation and regurgitation), 2) peak systolic flow velocities distal to the aortic valves reaching or surpassing ∼1,250 mm/s by pulsed-wave Doppler, and 3) putative fusion of cusps along commissures and abnormal movement elucidated by two-dimensional (2-D) imaging with ultrahigh temporal resolution. Valves with these features were confirmed by ex vivo gross anatomy and histological visualization to have thickened cusps, partial fusions, or Sievers type-0 bicuspid valves. This ultrasound imaging protocol will enable efficient, cost effective, and humane implementation of studies of congenital aortic valvular abnormalities and associated pathologies in a wide range of mouse models.NEW & NOTEWORTHY We developed a high-frequency ultrasound imaging protocol for diagnosing congenital aortic valve structural abnormalities in 4-wk-old mice. Our protocol defines specific criteria to distinguish mice with abnormal aortic valves from those with normal tricuspid valves using color Doppler, pulsed-wave Doppler, and two-dimensional (2-D) imaging with ultrahigh temporal resolution. This approach enables early identification of valvular abnormalities for efficient and ethical experimental design of longitudinal studies of congenital valve diseases and associated pathologies in mice.


Assuntos
Valva Aórtica , Modelos Animais de Doenças , Receptores do Fator Natriurético Atrial , Animais , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/patologia , Feminino , Masculino , Receptores do Fator Natriurético Atrial/genética , Receptores do Fator Natriurético Atrial/deficiência , Receptores do Fator Natriurético Atrial/metabolismo , Camundongos , Camundongos Knockout , Receptores de LDL/genética , Receptores de LDL/deficiência , Camundongos Endogâmicos C57BL , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem
19.
Anat Sci Int ; 99(3): 305-319, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38758496

RESUMO

Variations in the arch of the aorta and aortic valves among fetal, cadaveric, and post-mortem specimens present a spectrum of anatomical configurations, posing challenges in establishing a standard norm. While some variations hold surgical significance, many bear little functional consequence but provide insights into embryological origins. The aortic arch exhibits diverse branching patterns, including common trunks and different orders, relevant for endovascular surgeries. Meanwhile, malformations in the aortic valve, affecting the aorta, may lead to ischemia and cerebral infarction, warranting understanding of coexisting arch and valve anomalies to predict complications like aortic dissection. Studies in the Indian population mirror global variations, underscoring the need to explore embryological, clinical, and surgical implications for safer vascular surgeries involving the aortic arch and valves. The study's objectives included examining branching patterns, diameters, and distances between arch branches and exploring aortic valve variations. Employing a cross-sectional design, the study was conducted across Anatomy, Forensic Medicine, and Obstetrics and Gynecology departments. A sample of 100, comprising cadavers, fetuses, and postmortem specimens, were gathered. Specimens ranged from 14 weeks of intrauterine life to 85 years, with intact thoracic cages as inclusion criteria. Methodology involved dissection, specimen fixation, and macroscopic examination for variations and morphological parameters. Results showed aortic diameter increase with age, with significant gender differences. A statistically significant association between arch variations and anomalous valves was observed, suggesting mutual predictability. Individuals with valve anomalies should undergo comprehensive cardiology evaluation to avert complications like aortic dissection during endovascular surgeries. While atheromatous plaques were prevalent in younger groups, their frequency rose with age, necessitating vigilant vascular monitoring. Careful handling during surgeries is paramount, given potential adverse outcomes resulting from variations. Overall, the study underscores the importance of comprehensive anatomical understanding in clinical contexts, guiding effective management strategies and ensuring patient safety in vascular surgeries.


Assuntos
Variação Anatômica , Aorta Torácica , Valva Aórtica , Cadáver , Humanos , Feminino , Masculino , Valva Aórtica/anatomia & histologia , Valva Aórtica/anormalidades , Aorta Torácica/anatomia & histologia , Aorta Torácica/embriologia , Idoso , Adulto , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Adolescente , Adulto Jovem , Criança , Lactente , Pré-Escolar , Feto/anatomia & histologia , Recém-Nascido , Estudos Transversais
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