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1.
J Cardiothorac Surg ; 17(1): 109, 2022 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-35526008

RESUMO

BACKGROUND: The magnitude of ascending aortic degeneration in patients with bicuspid aortic valves (BAV) is controversial. METHODS: The aim of this study was to investigate ascending aortic wall degeneration in patients with BAV as compared with tricuspid aortic valves (TAV). The ascending aortic wall of 67 consecutive patients was processed for histology and immunohistochemistry. The extent of surgery and wall degeneration were investigated. Unadjusted survival was evaluated by Kaplan-Meier analysis. Median follow-up for patients with BAV and TAV was 3.8 years (interquartile range [IQR] 3.5-4.1) and 3.7 years (IQR 3.4-3.9), respectively. RESULTS: There were 33 patients with BAV and 34 with TAV. Mid-ascending aorta diameter was 54 mm (IQR 50-60). Replacement of the aortic valve, together with an ascending aortic prosthesis, was more frequent in BAV vs TAV patients (24% vs. 3%, P = 0.013). However, medial fibrosis, elastic fiber thinning, incremental medial degeneration and smooth muscle cell nuclei loss were less prominent in BAV vs TAV patients (0.1 ± 0.4 vs. 0.8 ± 1.4, P = 0.016; 0.6 ± 1.4 vs. 1.6 ± 2.0, P = 0.027; 1.7 ± 0.7 vs. 2.2 ± 0.8, P = 0.045 and 2.3 ± 1.5 vs. 3.2 ± 1.3, P = 0.026, respectively). CONCLUSIONS: Since degeneration of the ascending aortic wall was seldom prominent, histopathology alone may not support the need for surgery of the dilated ascending aorta in BAV patients as compared with TAV patients.


Assuntos
Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Aorta/patologia , Aorta/cirurgia , Valva Aórtica/patologia , Valva Aórtica/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Humanos , Valva Tricúspide/cirurgia
2.
Physiol Rep ; 10(8): e15251, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35439345

RESUMO

Male sex and hypertension represent risk factors in the progression of an aortic aneurysm. The present study examined the morphological/cellular phenotype of the ascending aorta (AA) of male and female patients diagnosed with a bicuspid aortic valve (BAV) to test the hypothesis that hypertension-induced remodeling of male BAV patients preferentially recapitulated the expression of a panel of proteins favoring aneurysm formation. The diameter of the AA of hypertensive male (35 ± 6 mm) and female (39 ± 5 mm) BAV patients was comparable to normotensive patients reflecting an early phase of vessel expansion. Morphological/structural remodeling of the medial region of the AA of male normotensive and hypertensive BAV patients were comparable. Protein levels of non-muscle myosin IIB, the cell cycle inhibitor p27kip1, tumor suppressor p53 and matrix metalloproteinase-2 and -9 were significantly upregulated in the AA of male hypertensive BAV patients. In female hypertensive BAV patients, collagen content was significantly increased whereas elastin content and medial width of the AA were similar to normotensive BAV patients. In the AA of female hypertensive BAV patients, matrix metalloproteinase-9 and p27kip1 protein levels were unchanged whereas p53 and matrix metalloproteinase-2 protein expression was significantly reduced. Nestin protein levels were diminished in the AA of male and female hypertensive BAV patients. Thus, sexual dimorphic remodeling of the AA was prevalent in hypertensive BAV patients. Moreover, during the early phase of vessel expansion, the AA of male hypertensive BAV patients was preferentially associated with the upregulation of a panel of proteins linked to progressive dilatation and potential aneurysm formation.


Assuntos
Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Hipertensão , Aorta/fisiologia , Valva Aórtica/metabolismo , Feminino , Doenças das Valvas Cardíacas/complicações , Humanos , Hipertensão/metabolismo , Masculino , Metaloproteinase 2 da Matriz/genética , Metaloproteinase 2 da Matriz/metabolismo , Fenótipo , Proteína Supressora de Tumor p53/genética
3.
Genes (Basel) ; 13(4)2022 04 02.
Artigo em Inglês | MEDLINE | ID: mdl-35456442

RESUMO

Coarctation of the aorta (CoA) and bicuspid aortic valve (BAV) often cooccur and are genetically linked congenital heart defects (CHD). While CoA is thought to have a hemodynamic origin from ventricular dysfunction, we provide evidence pointing to atrial hemodynamics based on investigating the genetic etiology of CoA. Previous studies have shown a rare MYH6 variant in an Icelandic cohort, and two common deletions in the protocadherin α cluster (PCDHA delCNVs) are significantly associated with CoA and BAV. Here, analysis of a non-Icelandic white CHD cohort (n = 166) recovered rare MYH6 variants in 10.9% of CoA and 32.7% of BAV/CoA patients, yielding odds ratios of 18.6 (p = 2.5 × 10-7) and 20.5 (p = 7.4 × 10-5) for the respective association of MYH6 variants with CoA and BAV/CoA. In combination with the PCHDA delCNVs, they accounted for a third of CoA cases. Gene expression datasets for the human and mouse embryonic heart showed that both genes are predominantly expressed in the atria, not the ventricle. Moreover, cis-eQTLs analysis showed the PCHDA delCNV is associated with reduced atrial expression of PCHDA10, a gene in the delCNV interval. Together, these findings showed that PCDHA/MYH6 variants account for a substantial fraction of CoA cases. An atrial rather than ventricular hemodynamic model for CoA is indicated, consistent with the known early atrial functional dominance of the human embryonic heart.


Assuntos
Coartação Aórtica , Doença da Válvula Aórtica Bicúspide , Cardiopatias Congênitas , Doenças das Valvas Cardíacas , Animais , Coartação Aórtica/complicações , Coartação Aórtica/genética , Valva Aórtica , Coenzima A , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/genética , Humanos , Camundongos
4.
JACC Cardiovasc Interv ; 15(7): 712-724, 2022 04 11.
Artigo em Inglês | MEDLINE | ID: mdl-35393104

RESUMO

OBJECTIVES: The aim of this study was to investigate the risk of coronary obstruction during redo-transcatheter aortic valve replacement (TAVR) within a previously implanted self-expanding valve in bicuspid aortic valve (BAV) versus tricuspid aortic valve (TAV) stenosis. BACKGROUND: The prevalence of BAV in TAVR patients is expected to increase as the indication expands; however, no study has investigated the risk of coronary obstruction for future redo-TAVR in these patients. METHODS: Computed tomography (CT) simulation analysis was performed in 86 type 0 BAV, 70 type 1 BAV, and 132 TAV patients who underwent TAVR with 1 VenusA-Valve (Venus Medtech) between January 2014 and December 2019. RESULTS: CT-identified risk of coronary obstruction during redo-TAVR was observed in 36.1% of patients for the left coronary ostium (LCO) and 27.8% of patients for the right coronary ostium (RCO); however, the incidences were significantly lower in the type 0 BAV group than in the type 1 BAV or TAV group (for LCO: OR: 1.00 [reference] vs OR: 2.49; 95% CI: 1.24-5.01 vs OR: 2.60; 95% CI: 1.40-4.81; for RCO: OR: 1.00 [reference] vs OR: 2.14; 95% CI: 1.02-4.48 vs OR: 1.97; 95% CI: 1.02-3.80). The leaflet laceration technique may be unfeasible to improve coronary flow in 61.5% of the threatened LCOs and 58.8% of the threatened RCOs during redo-TAVR. The percentages were significantly or numerically lower in the type 0 BAV group than other groups (for LCO: 26.3% vs 62.1% vs 73.2%; P overall = 0.001; for RCO: 43.8% vs 65.2% vs 61.0%; P overall = 0.374). CONCLUSIONS: Differences in anatomical features may impact the feasibility of future redo-TAVR. Type 0 BAV anatomy was associated with the lower incidence of CT-identified risk of coronary obstruction during redo-TAVR, and the leaflet laceration technique may be more feasible to ensure coronary flow in this population.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Oclusão Coronária , Lacerações , Substituição da Valva Aórtica Transcateter , Estenose da Valva Tricúspide , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/etiologia , Estenose da Valva Aórtica/cirurgia , Oclusão Coronária/etiologia , Humanos , Lacerações/complicações , Lacerações/cirurgia , Substituição da Valva Aórtica Transcateter/efeitos adversos , Resultado do Tratamento , Estenose da Valva Tricúspide/etiologia
5.
J Am Heart Assoc ; 11(8): e022339, 2022 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-35411791

RESUMO

Background Intracranial aneurysms are reported in 6%-10% of patients with bicuspid aortic valve (BAV), and routine intracranial aneurysm surveillance has been advocated by some. We assessed the prevalence and features of the most important patient-outcome: aneurysmal sub-arachnoid hemorrhage (aSAH), as compared with controls without aSAH, and tricuspid aortic valve (TAV) with aSAH. Methods and Results Adult patients with accurate diagnosis of aSAH and at least one echocardiogram between 2000 and 2019 were identified from a consecutive prospectively maintained registry of aSAH admissions. Controls without a diagnosis of SAH were age- and sex-matched. BAV prevalence was confirmed echocardiographically. Severity of aSAH was categorized using modified Fisher and World Federation of Neurological Scale. Neurologic outcome was assessed using modified Rankin score. A total 488 aSAH cases and 990 controls were identified and BAV status was confirmed. Prevalence of BAV in patients with aSAH was 1.2% (6/488) versus 3.5% (35/990) in controls, P=0.01. BAV+aSAH were noted to be younger than TAV+aSAH (56±11 versus 68±14; P=0.03) with smaller aneurysms (5±2 versus 7±4; P=0.31). The severity of aSAH was lesser in BAV+aSAH than TAV (modified Fisher grade>2 50% versus 74%; P=0.19, World Federation of Neurological Scale grade>3 17% versus 36%; P=0.43). BAV+aSAH had less severe neurologic disability (modified Rankin score 3%-6 33% versus 49% in TAV; P=0.44) and comparable in-hospital mortality rates (P=0.93). BAV had lower odds for aSAH on multivariate analysis (odds ratio 0.23[CI 0.08-0.65]; P=0.01). Conclusions Prevalence of BAV was 3 times lower in the aSAH registry than in controls without aSAH. BAV+aSAH had clinically smaller aneurysms, clinically smaller bleeds, and better neurologic outcome as compared with TAV+aSAH, which needs to be confirmed in larger studies. These findings argue against routine surveillance for intracranial aneurysms in patients with BAV without aortic coarctation.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Aneurisma Intracraniano , Neurologia , Adulto , Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/diagnóstico , Hemorragia , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/epidemiologia , Prevalência , Estudos Prospectivos , Sistema de Registros
8.
J Card Surg ; 37(6): 1486-1496, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35349188

RESUMO

AIMS: This study sought to characterize the rotation of the transcatheter heart valve (THV) and evaluate the neo-commissures overlap with coronary arteries in type-0 bicuspid aortic valve (BAV). METHODS AND RESULTS: This was a single-center, 10-patient, retrospective observational cohort. Pre-TAVI computed tomography and procedural fluoroscopy were analyzed. Coplanar fluoroscopic views were coregistered to pre-TAVI computed tomography to characterize THV rotation and determine coronary overlap. The incidence of severe coronary artery overlap with one coronary artery was 90%. According to our prediction line, type-0 BAV has predicted a higher incidence of overlap with one coronary artery, but lower incidence with both coronary arteries compared to the tricuspid aortic valve (TAV). The rotational angles in two different phases were 3.8 ± 3.2° versus 11.8 ± 8.0° (p = .01) in patients with mixed cusp fusion. Commissural angles in final and initial deployment were 9.6 ± 6.6 versus 18.1 ± 11.0° (p = .021). Applying hypothetic "commissure-middle view" in 0°, ±5°, and ±10°, the incidence of overlap with one coronary artery are 20%, 40%, and 90% separately. CONCLUSIONS: The THV rotation existed and was activated in the last 1/3 deploying phase. With the observed tendency of "automatic commissural alignment," applying the "commissure-middle" view in type-0 BAV may optimize valve alignment and avoid coronary artery overlap.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Próteses Valvulares Cardíacas , Substituição da Valva Aórtica Transcateter , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/cirurgia , Humanos , Desenho de Prótese , Estudos Retrospectivos , Rotação , Substituição da Valva Aórtica Transcateter/métodos , Resultado do Tratamento
10.
Rev Cardiovasc Med ; 23(2): 47, 2022 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-35229538

RESUMO

BACKGROUND: The limitation of aortic size-based criteria is gradually recognized in the prediction of aortic events especially in bicuspid aortic valve (BAV) cohorts, while most aortic events happen in patients with proximal aortic diameters <50 mm. Circulating microRNAs (miRs) have been addressed as a novel tool to improve risk stratification in patients with different aortopathies. We aimed to elucidate the correlation between peripheral whole blood and aortic tissue miRs in order to prove the potential availability as a biomarker in the clinical routine. METHODS: All patients who received elective aortic valve repair/replacement ± proximal aortic replacement to BAV disease (n = 65, 2013-2018) were prospectively included. The expression of 10 miRs (miR-1, miR-17, miR-18a, miR-19a, miR-20a, miR-21, miR-106a, miR-133a, miR-143 and miR-145) was analyzed in the intraoperatively acquired aortic tissue as well as in the peripheral blood before the surgery. RESULTS: We found a significant correlation between circulating miRs in the peripheral blood and aortic tissue levels of miR-21 (r = 0.293, p = 0.02), miR-133a (r = 0.43, p = 0.02), miR-143 (r = 0.68, p < 0.001), and miR-145 (r = 0.68, p < 0.001). Further, the multivariate logistic regression analysis revealed an association between blood and aortic tissue miR-143 levels each other (Odds Ratio [OR] 1.29, 95% Confidence Interval [CI] 1.11-1.67, p = 0.02; OR 1.36, 95% CI 1.19-2.01, p = 0.03, respectively) and a blood/aortic miR-143 level to dilated aorta (OR 3.61, 95% CI 1.62-9.02, p = 0.01; OR 2.92, 95% CI 1.81-7.05, p = 0.02, respectively). CONCLUSIONS: Our study demonstrates a significant correlation between peripheral whole blood and aortic tissue miRs, confirming the hypothesis that circulating miRs may reflect remodeling processes in the proximal aorta in bicuspid aortopathy patients.


Assuntos
Doença da Válvula Aórtica Bicúspide , MicroRNA Circulante , Doenças das Valvas Cardíacas , MicroRNAs , Valva Aórtica/cirurgia , MicroRNA Circulante/genética , MicroRNA Circulante/metabolismo , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/genética , Doenças das Valvas Cardíacas/cirurgia , Humanos , MicroRNAs/genética , MicroRNAs/metabolismo
11.
Comput Biol Med ; 144: 105326, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35245697

RESUMO

Studying anatomical shape progression over time is of utmost importance to refine our understanding of clinically relevant processes. These include vascular remodeling, such as aortic dilation, which is particularly important in some congenital heart defects (CHD). A novel methodological framework for three-dimensional shape analysis has been applied for the first time in a CHD scenario, i.e., bicuspid aortic valve (BAV) disease, the most common CHD. Three-dimensional aortic shapes (n = 94) reconstructed from cardiovascular magnetic resonance imaging (MRI) data as surface meshes represented the input for a longitudinal atlas model, using multiple scans over time (n = 2-4 per patient). This model relies on diffeomorphism transformations in the absence of point-to-point correspondence, and on the right combination of initialization, estimation and registration parameters. We computed the shape trajectory of an average disease progression in our cohort, as well as time-dependent parameters, geometric variations and the average shape of the population. Results cover a spatiotemporal spectrum of visual and numerical information that can be further used to run clinical associations. This proof-of-concept study demonstrates the feasibility of applying advanced statistical shape models to track disease progression and stratify patients with CHD.


Assuntos
Doença da Válvula Aórtica Bicúspide , Cardiopatias Congênitas , Doenças das Valvas Cardíacas , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Progressão da Doença , Estudos de Viabilidade , Cardiopatias Congênitas/diagnóstico por imagem , Doenças das Valvas Cardíacas/diagnóstico por imagem , Humanos
12.
Circ Genom Precis Med ; 15(2): e003523, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35133174

RESUMO

BACKGROUND: Whole-genome sequencing in families enables deciphering of congenital heart disease causes. A shared genetic basis for familial bicuspid aortic valve (BAV) and hypoplastic left heart syndrome (HLHS) was postulated. METHODS: Whole-genome sequencing was performed in affected members of 6 multiplex BAV families, an HLHS cohort of 197 probands and 546 relatives, and 813 controls. Data were filtered for rare, predicted-damaging variants that cosegregated with familial BAV and disrupted genes associated with congenital heart disease in humans and mice. Candidate genes were further prioritized by rare variant burden testing in HLHS cases versus controls. Modifier variants in HLHS proband-parent trios were sought to account for the severe developmental phenotype. RESULTS: In 5 BAV families, missense variants in 6 ontologically diverse genes for structural (SPTBN1, PAXIP1, and FBLN1) and signaling (CELSR1, PLXND1, and NOS3) proteins fulfilled filtering metrics. CELSR1, encoding cadherin epidermal growth factor laminin G seven-pass G-type receptor, was identified as a candidate gene in 2 families and was the only gene demonstrating rare variant enrichment in HLHS probands (P=0.003575). HLHS-associated CELSR1 variants included 16 missense, one splice site, and 3 noncoding variants predicted to disrupt canonical transcription factor binding sites, most of which were inherited from a parent without congenital heart disease. Filtering whole-genome sequencing data for rare, predicted-damaging variants inherited from the other parent revealed 2 cases of CELSR1 compound heterozygosity, one case of CELSR1-CELSR3 synergistic heterozygosity, and 4 cases of CELSR1-MYO15A digenic heterozygosity. CONCLUSIONS: CELSR1 is a susceptibility gene for familial BAV and HLHS, further implicating planar cell polarity pathway perturbation in congenital heart disease.


Assuntos
Doença da Válvula Aórtica Bicúspide , Caderinas , Cardiopatias Congênitas , Síndrome do Coração Esquerdo Hipoplásico , Receptores Acoplados a Proteínas G , Alelos , Animais , Valva Aórtica/anormalidades , Doença da Válvula Aórtica Bicúspide/genética , Caderinas/genética , Cardiopatias Congênitas/genética , Humanos , Síndrome do Coração Esquerdo Hipoplásico/genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Glicoproteínas de Membrana/genética , Camundongos , Receptores Acoplados a Proteínas G/genética
13.
Arq Bras Cardiol ; 118(3): 588-596, 2022 Mar.
Artigo em Português, Inglês | MEDLINE | ID: mdl-35137777

RESUMO

BACKGROUND: The bicuspid aortic valve (BAV) affects 0.5 to 2% of the population and is associated with valve and aortic alterations. There is a lack of studies on the profile of these patients in the Brazilian population. OBJECTIVE: To describe the profile of patients with BAV undergoing valve and/or aortic surgery in a tertiary cardiology center, in addition to the outcomes related to the intervention. METHODS: Retrospective cohort including 195 patients (mean age 54±14 years, 73.8% male) diagnosed with BAV who underwent surgical approach (valvular and/or aorta) from 2014 to 2019. Clinical data, echocardiographic and tomographic studies were evaluated, as well as characteristics of the intervention and events in 30 days. A value of p<0.05 was considered statistically significant. RESULTS: We found a high prevalence of aortic aneurysm (56.5%), with a mean diameter of 46.9±10.2 mm. Major aortic regurgitation was found in 25.1% and major aortic stenosis in 54.9%. Isolated aortic valve surgery was performed in 48.2%, isolated aortic surgery in 6.7% and combined surgery in 45.1%. The 30-day mortality was 8.2%. In the multivariate analysis, the predictors of the combined outcome at 30 days (death, atrial fibrillation and reoperation) were age (OR 1.044, 95% CI 1.009-1.081, p=0.014) and left ventricular mass index (OR 1.009, 95% CI 1.000-1.018, p=0.044). CONCLUSION: Patients with BAV approached in our service have a higher incidence of aortopathy, with the additional need to evaluate the aorta with computed tomography or magnetic resonance imaging.


FUNDAMENTO: A válvula aórtica bicúspide (VAB) atinge de 0,5 a 2% da população e está associada a alterações valvares e de aorta. Há carência de estudos sobre o perfil desses pacientes na população brasileira. OBJETIVO: Descrever o perfil de pacientes com VAB submetidos à cirurgia valvar e/ou de aorta em um centro cardiológico terciário, assim como os desfechos relacionados à intervenção. MÉTODOS: Coorte retrospectiva incluindo 195 pacientes (idade média 54±14 anos, 73,8% do sexo masculino) com diagnóstico de VAB submetidos à abordagem cirúrgica (valvar e/ou de aorta) no período de 2014 a 2019. Foram avaliados dados clínicos, ecocardiográficos e tomográficos, além das características da intervenção e eventos em 30 dias. O valor de p<0,05 foi considerado estatisticamente significante. RESULTADOS: Encontramos alta prevalência de aneurisma de aorta (56,5%), com diâmetro médio de 46,9±10,2 mm. Insuficiência aórtica importante foi encontrada em 25,1% e estenose aórtica importante em 54,9%. Cirurgia isolada em valva aórtica foi realizada em 48,2%, cirurgia isolada de aorta em 6,7% e cirurgia combinada em 45,1%. A mortalidade em 30 dias foi de 8,2%. Na análise multivariada, os fatores preditores de desfecho combinado em 30 dias (morte, fibrilação atrial e reoperação) foram idade (OR 1,044, IC 95% 1,009-1,081, p=0,014) e o índice de massa do ventrículo esquerdo (OR 1,009, IC 95% 1,000-1,018, p=0,044). CONCLUSÃO: Pacientes com VAB abordados no nosso serviço apresentam uma maior incidência de aortopatia, com a necessidade adicional de avaliação da aorta com tomografia computadorizada ou ressonância magnética.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Adulto , Idoso , Aorta , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Feminino , Doenças das Valvas Cardíacas/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
14.
Int Heart J ; 63(2): 319-326, 2022 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-35185086

RESUMO

Bicuspid aortic valve (BAV) patients with aortic stenosis (AS) are known to develop dilatation of the ascending aorta at a younger age, but the morphology of the aorta in these patients is yet to be investigated. Thus, in this study, we aim to evaluate the aortic morphology of BAV patients with severe AS using thin-slice electrocardiogram (ECG) -gated computed tomography (CT) and identify the possible contributing effect of age.In this retrospective study, 122 BAV and 154 tricuspid aortic valve (TAV) patients who received aortic valve replacement for severe AS were assessed by thin-slice ECG-gated CT and three-dimensional reconstruction. The morphology of the ascending aorta was also evaluated among BAV patients aged < 70 (n = 72) and ≥ 70 (n = 50) years old. As per our findings, BAV patients with severe AS had significantly greater diameter (P < 0.01), elongation (P < 0.01), and tortuosity (P = 0.03) of the ascending aorta; minimum aortic arch angle (P < 0.01); and significantly lower calcified plaque (P < 0.01) compared with those of TAV patients even after adjusting for background. Multiple regression analysis showed that standardized partial regression coefficients (ß) of dilatation (0.5) and elongation (0.35) were higher among other measurements of aortic morphology for BAV patients. BAV patients with severe AS aged ≥ 70 years had significantly greater diameter (42.0 [37.2-46.1] mm versus 40.4 [35.2-44.2] mm, P = 0.049) and elongation (133.8 [123.5-147.3] mm versus 127.0 [111.0-140.0] mm, P = 0.01) of the ascending aorta than those aged < 70 years.BAV patients with severe AS were determined to have greater dilatation and elongation of the ascending aorta. Moreover, BAV patients older than 70 years had greater diameter and elongation of the ascending aorta.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Idoso , Aorta/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/diagnóstico por imagem , Eletrocardiografia , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
15.
Echocardiography ; 39(3): 538-542, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35118713

RESUMO

A 61-year-old male presented with symptoms of decompensated heart failure and cardiogenic shock. Transthoracic and transesophageal echocardiography showed severely impaired left ventricular (LV) systolic function (LVEF of 20-25%), bicuspid aortic valve with moderate aortic insufficiency and no significant stenosis, dilated coronary sinus and a tortuous vascular structure in the left-sided atrioventricular groove. Cardiac computed tomography confirmed the diagnosis of persistent left superior vena cava and a giant coronary artery fistula to the coronary sinus. Cardiac magnetic resonance illustrated non-specific late gadolinium enhancement in the mid-wall of the septum. The patient was treated medically and with cardiac re-synchronization therapy.


Assuntos
Doença da Válvula Aórtica Bicúspide , Seio Coronário , Fístula , Veia Cava Superior Esquerda Persistente , Meios de Contraste , Seio Coronário/diagnóstico por imagem , Vasos Coronários/diagnóstico por imagem , Gadolínio , Humanos , Masculino , Pessoa de Meia-Idade , Veia Cava Superior/diagnóstico por imagem
16.
Ann Biomed Eng ; 50(1): 1-15, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34993699

RESUMO

The clinical benefit of patient-specific modeling of heart valve disease remains an unrealized goal, often a result of our limited understanding of the in vivo milieu. This is particularly true in assessing bicuspid aortic valve (BAV) disease, the most common cardiac congenital defect in humans, which leads to premature and severe aortic stenosis or insufficiency (AS/AI). However, assessment of BAV risk for AS/AI on a patient-specific basis is hampered by the substantial degree of anatomic and functional variations that remain largely unknown. The present study was undertaken to utilize a noninvasive computational pipeline ( https://doi.org/10.1002/cnm.3142 ) that directly yields local heart valve leaflet deformation information using patient-specific real-time three-dimensional echocardiographic imaging (rt-3DE) data. Imaging data was collected for patients with normal tricuspid aortic valve (TAV, [Formula: see text]) and those with BAV ([Formula: see text] with fused left and right coronary leaflets and [Formula: see text] with fused right and non-coronary leaflets), from which the medial surface of each leaflet was extracted. The resulting deformation analysis resulted in, for the first time, quantified differences between the in vivo functional deformations of the TAV and BAV leaflets. Our approach was able to capture the complex, heterogeneous surface deformation fields in both TAV and BAV leaflets. We were able to identify and quantify differences in stretch patterns between leaflet types, and found in particular that stretches experienced by BAV leaflets during closure differ from those of TAV leaflets in terms of both heterogeneity as well as overall magnitude. Deformation is a key parameter in the clinical assessment of valvular function, and serves as a direct means to determine regional variations in structure and function. This study is an essential step toward patient-specific assessment of BAV based on correlating leaflet deformation and AS/AI progression, as it provides a means for assessing patient-specific stretch patterns.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Aorta , Valva Aórtica/diagnóstico por imagem , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Doenças das Valvas Cardíacas/diagnóstico por imagem , Humanos , Valva Tricúspide
17.
Can J Cardiol ; 38(5): 688-694, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35093466

RESUMO

BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital heart disease, often associated with valve dysfunction, coarctation of the aorta, and ascending aorta dilatation. Aortic dilatation might result from abnormal regional hemodynamics or inherent vascular disease. Vascular function in pediatric BAV remains poorly characterized. METHODS: A cross-sectional study was performed to evaluate vascular function in 142 children with BAV aged 7-18 years compared with healthy control children. Echocardiography was performed to assess aortic dimensions, BAV function, and vascular function (aortic arch pulse wave velocity [PWV]), carotid intima media thickness, and aortic stiffness and distensibility). Carotid-femoral and carotid-radial PWV were assessed using tonometry. Vascular function was compared for 4 patient groups stratified according to aortic dilatation and a history of coarctation of the aorta. Multivariate regression analysis was performed to determine predictors of aortic dilatation. RESULTS: Children with BAV had stiffer and less distensible ascending aortas with higher aortic arch PWV compared with control children. Carotid-femoral and carotid-radial PWV were not increased in patients with BAV, and the vascular assessment of the abdominal aorta was unremarkable. Multivariate regression revealed that aortic arch PWV was the only vascular function parameter that was associated with aortic dilatation. CONCLUSIONS: Children with BAV have differences in vascular function that are confined to their proximal aorta, even in normal functioning BAV. The observed differences in vascular function are likely multifactorial, with contributions from abnormal regional flow and a potential localized primary aortopathy.


Assuntos
Coartação Aórtica , Doenças da Aorta , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Rigidez Vascular , Coartação Aórtica/complicações , Doenças da Aorta/complicações , Doenças da Aorta/etiologia , Valva Aórtica/anormalidades , Espessura Intima-Media Carotídea , Criança , Estudos Transversais , Dilatação , Dilatação Patológica , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico , Humanos , Análise de Onda de Pulso
19.
Pediatr Cardiol ; 43(4): 837-843, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34999921

RESUMO

Echocardiography is often used to assess for significant heart disease in newborns, but there is little information on how to best manage non-emergent echocardiographic findings in asymptomatic babies. We reviewed the literature regarding the natural history of a patent foramen ovale (PFO), atrial septal defect (ASD), ventricular septal defect (PFO), and patent ductus arteriosus (PDA). We surveyed pediatric cardiologists to determine their recommendations for ten echocardiographic findings (PFO, 3 mm ASD, 6 mm ASD, small muscular VSD, small perimembranous VSD, small PDA with left to right shunting, small PDA with bidirectional shunting, trivial mitral insufficiency, trivial aortic insufficiency, and a normally functioning bicuspid aortic valve) in an asymptomatic one-day old with a heart murmur. These ten findings were set in three clinical contexts (an otherwise normal term baby, a baby born at 34 weeks gestation, and a term baby with trisomy 21). 149 survey responses were evaluated. Follow-up was universally recommended for those babies with a 6 mm ASD, a perimembranous VSD and a bicuspid aortic valve and frequently recommended for newborns with a 3 mm ASD, a small muscular VSD and any PDA. Depending on the context, between 17.5 and 23% of respondents recommended follow-up for an isolated PFO. Follow-up typically included repeat echocardiography. Some form of follow-up, typically with repeat echocardiography was recommended for many asymptomatic day-old newborns who had echocardiographic findings which were unlikely to be clinically significant. Given the wide range of recommendations, a consensus guideline could prove useful to clinicians.


Assuntos
Doença da Válvula Aórtica Bicúspide , Cardiologistas , Permeabilidade do Canal Arterial , Forame Oval Patente , Comunicação Interatrial , Comunicação Interventricular , Criança , Ecocardiografia , Forame Oval Patente/diagnóstico por imagem , Humanos , Recém-Nascido , Inquéritos e Questionários
20.
Curr Cardiol Rep ; 24(1): 33-41, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-35099762

RESUMO

PURPOSE OF REVIEW: Bicuspid aortic valve (BAV) disease is the most common congenital heart defect worldwide. When severe, symptomatic aortic stenosis ensues, the treatment has increasingly become transcatheter aortic valve replacement (TAVR). The purpose of this review is to identify BAV classification and imaging methods, outline TAVR outcomes in BAV anatomy, and discuss how computational modeling can enhance TAVR treatment in BAV patients. RECENT FINDINGS: TAVR use in BAV patients, when compared to use in tricuspid aortic valves, showed lower device success rate, and there remains no long-term randomized trial data. It has been reported that BAV patients with severe calcification increase the rate of complications. Additionally, the asymmetrical morphology of BAVs often results in asymmetric stent geometries which have implications for increased thrombosis risk and decreased durability. These adverse outcomes are currently very difficult to predict from routine pre-procedural imaging alone. Recently developed patient specific experimental and computational techniques have the potential to assist in filling knowledge gaps in the mechanisms of these complications and provide more information during preclinical planning for better TAVR selection in low surgical risk BAV patients. Efficacy of TAVR for irregular BAV anatomies remains concerning due to the lack of a long-term randomized trial data, their increased rate of short-term complications, and signs that long-term durability could be an issue. More knowledge on identifying which BAV anatomies are at greater risk for these adverse outcomes can potentially improve patient selection for TAVR versus SAVR in low surgical risk BAV patients.


Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Estenose da Valva Mitral , Substituição da Valva Aórtica Transcateter , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/etiologia , Estenose da Valva Aórtica/cirurgia , Constrição Patológica , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Humanos , Estenose da Valva Mitral/cirurgia , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/métodos , Resultado do Tratamento
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