RESUMO
BACKGROUND: Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS. METHODS: This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method). RESULTS: A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m2, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score. CONCLUSIONS: The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.
Assuntos
Síndrome de Turner , Humanos , Síndrome de Turner/complicações , Criança , Adolescente , Feminino , China/epidemiologia , Dilatação Patológica/etiologia , Masculino , Estudos Retrospectivos , Aorta/patologia , Aorta/diagnóstico por imagem , Coartação Aórtica , Doença da Válvula Aórtica Bicúspide/complicações , Pré-Escolar , Incidência , População do Leste AsiáticoRESUMO
BACKGROUND: There continues to be debate regarding the superiority of transcatheter (TAVR) over surgical aortic valve replacement (SAVR) in patients with bicuspid aortic valves (BAV). We aimed to compare outcomes during readmissions in elderly patients with BAV who underwent SAVR or TAVR. METHODS: Patients 65 years or older with BAV who underwent TAVR or isolated SAVR were identified using the National Readmission Database from 2012 through 2018. We compared outcomes during readmissions within 90 days after discharge from the index surgery. Propensity score matching was performed to adjust the baseline differences. RESULTS: During the study period, 8555 and 1081 elderly patients with BAV underwent SAVR and TAVR, respectively. The number of patients who underwent TAVR went up by 179 % from 2012 to 2018. Propensity score matching yielded 573 patients in each group. A total of 111 (19.4 %) in the SAVR group and 125 (21.8 %) in the TAVR group were readmitted within 90 days after the index surgery (p = .31). The mortality during the readmissions within 90 days was equivalent between the two groups (0.9 % in the SAVR group vs. 3.2 % in the TAVR group, p = .22). However, the median hospital cost was approximately doubled in the TAVR group during the readmission (18,250 dollars vs. 9310 dollars in the SAVR group, p < .001). CONCLUSIONS: Readmission within 90 days was common in both groups. While the mortality during the readmissions after the surgery was equivalent between the two groups, hospital cost was significantly more expensive in the TAVR group.
Assuntos
Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Bases de Dados Factuais , Doenças das Valvas Cardíacas , Implante de Prótese de Valva Cardíaca , Readmissão do Paciente , Substituição da Valva Aórtica Transcateter , Humanos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/mortalidade , Substituição da Valva Aórtica Transcateter/economia , Masculino , Feminino , Idoso , Resultado do Tratamento , Fatores de Tempo , Doença da Válvula Aórtica Bicúspide/cirurgia , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Doença da Válvula Aórtica Bicúspide/mortalidade , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Idoso de 80 Anos ou mais , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Fatores de Risco , Estados Unidos , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/mortalidade , Implante de Prótese de Valva Cardíaca/economia , Estudos Retrospectivos , Fatores Etários , Medição de Risco , Doenças das Valvas Cardíacas/cirurgia , Doenças das Valvas Cardíacas/mortalidade , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/fisiopatologia , Complicações Pós-Operatórias/etiologia , Estenose da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/mortalidade , Estenose da Valva Aórtica/fisiopatologiaRESUMO
Bicuspid aortic valve is the most common congenital heart defect. Transthoracic echocardiogram is the initial tool to assess and diagnose this condition, however, transesophageal echocardiogram with 3D modalities, including transillumination have a better anatomical and functional evaluation of the valve, allowing to classify the bicuspid aortic valve according to the position of the raphe and assess the main vessels for complications or exclude other cardiovascular diseases.
Assuntos
Doença da Válvula Aórtica Bicúspide , Humanos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/anormalidades , Transiluminação , Ecocardiografia , Ecocardiografia TransesofagianaRESUMO
BACKGROUND: The relative roles for transcatheter and surgical aortic valve replacement (SAVR) for bicuspid aortic valve (AV) stenosis are debated. This study analyzes the 5-year longitudinal outcomes of isolated SAVR in bicuspid vs tricuspid AV patients, particularly in low-risk patients. METHODS: All patients undergoing isolated index SAVR at 1146 United States hospitals in The Society of Thoracic Surgeons (STS) Adult Cardiac database between July 1, 2011, and December 31, 2018, with linkage to Medicare claims, were analyzed. RESULTS: A total of 65,687 patients were analyzed, including of 9131 bicuspid patients (13.9%). Compared with tricuspid patients, bicuspid patients were significantly younger (median 70 vs 74 years, P < .001) with lower Society of Thoracic Surgeons predicted risk of mortality scores (mean 1.6% vs 2.3%, P < .001) and lower risk profile. Risk-adjusted 30-day mortality and major morbidity were similar, but risk-adjusted 5-year mortality was significantly lower in the bicuspid patients (adjusted hazard ratio, 0.72; 95% CI, 0.66-0.77), specifically in low-risk patients (adjusted hazard ratio, 0.69; 95% CI, 0.64-0.76). Additionally, the bicuspid cohort had a lower 5-year readmission risk of heart failure, stroke, bleeding, or other cardiovascular causes (all P < .05). CONCLUSIONS: In this nationally representative study, 30-day mortality was similar, but risk-adjusted 5-year mortality was significantly lower in bicuspid patients undergoing isolated SAVR compared with tricuspid patients, specifically low-risk and normal left ventricular ejection fraction patients. This analysis provides a much-needed 5-year longitudinal national-level benchmark to better inform the discussion of transcatheter vs SAVR in bicuspid patients.
Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Implante de Prótese de Valva Cardíaca , Substituição da Valva Aórtica Transcateter , Adulto , Humanos , Idoso , Estados Unidos/epidemiologia , Valva Aórtica/cirurgia , Benchmarking , Doença da Válvula Aórtica Bicúspide/cirurgia , Substituição da Valva Aórtica Transcateter/efeitos adversos , Volume Sistólico , Resultado do Tratamento , Medicare , Função Ventricular Esquerda , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Fatores de RiscoAssuntos
Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Humanos , Função Ventricular Esquerda , Valva Aórtica/diagnóstico por imagem , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico por imagem , MiocárdioRESUMO
BACKGROUND: Decisions in the management of aortic stenosis are based on the peak pressure drop, captured by Doppler echocardiography, whereas gold standard catheterization measurements assess the net pressure drop but are limited by associated risks. The relationship between these two measurements, peak and net pressure drop, is dictated by the pressure recovery along the ascending aorta which is mainly caused by turbulence energy dissipation. Currently, pressure recovery is considered to occur within the first 40-50 mm distally from the aortic valve, albeit there is inconsistency across interventionist centers on where/how to position the catheter to capture the net pressure drop. METHODS: We developed a non-invasive method to assess the pressure recovery distance based on blood flow momentum via 4D Flow cardiovascular magnetic resonance (CMR). Multi-center acquisitions included physical flow phantoms with different stenotic valve configurations to validate this method, first against reference measurements and then against turbulent energy dissipation (respectively n = 8 and n = 28 acquisitions) and to investigate the relationship between peak and net pressure drops. Finally, we explored the potential errors of cardiac catheterisation pressure recordings as a result of neglecting the pressure recovery distance in a clinical bicuspid aortic valve (BAV) cohort of n = 32 patients. RESULTS: In-vitro assessment of pressure recovery distance based on flow momentum achieved an average error of 1.8 ± 8.4 mm when compared to reference pressure sensors in the first phantom workbench. The momentum pressure recovery distance and the turbulent energy dissipation distance showed no statistical difference (mean difference of 2.8 ± 5.4 mm, R2 = 0.93) in the second phantom workbench. A linear correlation was observed between peak and net pressure drops, however, with strong dependences on the valvular morphology. Finally, in the BAV cohort the pressure recovery distance was 78.8 ± 34.3 mm from vena contracta, which is significantly longer than currently accepted in clinical practise (40-50 mm), and 37.5% of patients displayed a pressure recovery distance beyond the end of the ascending aorta. CONCLUSION: The non-invasive assessment of the distance to pressure recovery is possible by tracking momentum via 4D Flow CMR. Recovery is not always complete at the ascending aorta, and catheterised recordings will overestimate the net pressure drop in those situations. There is a need to re-evaluate the methods that characterise the haemodynamic burden caused by aortic stenosis as currently clinically accepted pressure recovery distance is an underestimation.
Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Humanos , Valor Preditivo dos Testes , Estenose da Valva Aórtica/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Valva Aórtica/diagnóstico por imagem , Hemodinâmica , Espectroscopia de Ressonância Magnética , Velocidade do Fluxo Sanguíneo/fisiologiaRESUMO
Bicuspid aortic valve is the most common congenital cardiac malformation and the leading cause of aortopathy and aortic stenosis in younger patients. Aortic wall remodelling secondary to altered haemodynamic flow patterns, changes in peak velocity, and wall shear stress may be implicated in the development of aortopathy in the presence of bicuspid aortic valve and dysfunction. Assessment of these parameters as potential predictors of disease severity and progression is thus desirable. The anatomic and functional information acquired from 4D flow MRI can allow simultaneous visualisation and quantification of the pathological geometric and haemodynamic changes of the aorta. We review the current clinical utility of haemodynamic quantities including velocity, wall sheer stress and energy losses, as well as visual descriptors such as vorticity and helicity, and flow direction in assessing the aortic valve and associated aortopathies.
Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Humanos , Doenças das Valvas Cardíacas/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/patologia , Estenose da Valva Aórtica/diagnóstico por imagem , HemodinâmicaRESUMO
Bicuspid aortic valve (BAV), the most common congenital valvular abnormality, generates asymmetric flow patterns and increased stresses on the leaflets that expedite valvular calcification and structural degeneration. Recently adapted for use in BAV patients, TAVR demonstrates promising performance, but post-TAVR complications tend to get exacerbated due to BAV anatomical complexities. Utilizing patient-specific computational modeling, we address some of these complications. The degree and location of post-TAVR PVL was assessed, and the risk of flow-induced thrombogenicity was analyzed in 3 BAV patients - using older generation TAVR devices that were implanted in these patients, and compared them to the performance of the newest generation TAVR devices using in silico patient models. Significant decrease in PVL and thrombogenic potential was observed after implantation of the newest generation device. The current work demonstrates the potential of using simulations in pre-procedural planning to assess post-TAVR complications, and compare the performance of different devices to achieve better clinical outcomes. Patient-specific computational framework to assess post-transcatheter bicuspid aortic valve replacement paravalvular leakage and flow-induced thrombogenic complications and compare device performances.
Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Substituição da Valva Aórtica Transcateter , Humanos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Modelagem Computacional Específica para o Paciente , Simulação por Computador , Estenose da Valva Aórtica/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: Bicuspid aortic valve (BAV) is common and 7.7%-9.8% of patients with BAV have intracranial aneuryms (IAs) which might lead to a devastating subarachnoid haemorrhage (SAH). We aimed to evaluate different screening and follow-up strategies using magnetic resonance angiography for IAs among patients with BAV. METHODS: A decision-analytic model was built to evaluate the costs and effectiveness of different management strategies from the Chinese healthcare payer's perspective. The evaluated strategies included natural history without screening for possible IAs, regular screening and no follow-up for detected IAs, and regular screening with regular follow-up (Screen strategy/Follow-up strategy). Base case calculation, as well as probabilistic, one-way, and two-way sensitivity analyses, were performed. RESULTS: According to the base case calculation, natural history had the least cost and effectiveness while Every 5 years (y)/Annual gained the highest cost and effectiveness. Every 10y/Biennial was cost effective when compared with Every 10y/Every 5y under the willingness-to-pay threshold of ¥211 743 (US$30 162). Probabilistic sensitivity analysis showed that Every 10y/Biennial was superior in 88.3% of the cases when compared with Every 10y/Every 5y. One-way and two-way sensitivity analyses proved that Every 10y/Biennial was the dominant strategy under most circumstances. CONCLUSIONS: Screening for possible IAs among patients with BAV and follow-up for detected IAs would increase the effectiveness. Every 10y/Biennial was the optimal strategy from the Chinese healthcare payer's perspective.
Assuntos
Doença da Válvula Aórtica Bicúspide , Aneurisma Intracraniano , Análise Custo-Benefício , Humanos , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/diagnóstico , Angiografia por Ressonância Magnética , Cadeias de Markov , Programas de Rastreamento , Anos de Vida Ajustados por Qualidade de VidaRESUMO
AIMS: Bicuspid aortic valve (BAV) is the commonest congenital heart valve malformation, and is associated with life-threatening complications. Given the high heritability index of BAV, many experts recommend echocardiography screening for first-degree relatives (FDRs) of an index case. Here, we aim to evaluate the cost-effectiveness of such cascade screening for BAV. METHODS AND RESULTS: Using a decision-analytic model, we performed a cost-effectiveness analysis of echocardiographic screening for FDRs of a BAV index case. Data on BAV probabilities and complications among FDRs were derived from our institution's BAV familial cohort and from the literature on population-based BAV cohorts with long-term follow-up. Health gain was measured as quality-adjusted life years (QALYs). Cost inputs were based on list prices and literature data. One-way and probabilistic sensitivity analyses were performed to account for uncertainty in the model's variables. Screening of FDRs was found to be the dominant strategy, being more effective and less costly than no screening, with savings of 644 and gains of 0.3 QALY. Results were sensitive throughout the range of the main model's variables, including the full range of reported BAV rates among FDRs across the literature. A gradual decrease of the incremental effect was found with the increase in screening age. CONCLUSION: This economic evaluation model found that echocardiographic screening of FDRs of a BAV index case is not only clinically important but also cost-effective and cost-saving. Sensitivity analysis supported the model's robustness, suggesting its generalization.
Assuntos
Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Análise Custo-Benefício , Ecocardiografia , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/genética , HumanosRESUMO
OBJECTIVE: To investigate prenatal changes in cardiac biometric and flow parameters in fetuses with bicuspid aortic valve (BAV) diagnosed neonatally compared with controls with normal cardiac anatomy. METHODS: This analysis was conducted as part of the Copenhagen Baby Heart Study, a multicenter cohort study of 25 556 neonates that underwent second-trimester anomaly scan at 18 + 0 to 22 + 6 weeks' gestation and neonatal echocardiography within 4 weeks after birth, in Copenhagen University Hospital Herlev, Hvidovre Hospital and Rigshospitalet in greater Copenhagen, between April 2016 and October 2018. From February 2017 (Rigshospitalet) and September 2017 (Herlev and Hvidovre hospitals), the protocol for second-trimester screening of the heart was extended to include evaluation of the four-chamber view, with assessment of flow across the atrioventricular valves, sagittal view of the aortic arch and midumbilical artery and ductus venosus pulsatility indices. All images were evaluated by two investigators, and cardiac biometric and flow parameters were measured and compared between cases with BAV and controls. All cases with neonatal BAV were assessed by a specialist. Maternal characteristics and first- and second-trimester biomarkers were also compared between the two groups. RESULTS: Fifty-five infants with BAV and 8316 controls with normal cardiac anatomy were identified during the study period and assessed using the extended prenatal cardiac imaging protocol. There were three times as many mothers who smoked before pregnancy in the group with BAV as in the control group (9.1% vs 2.7%; P = 0.003). All other baseline characteristics were similar between the two groups. Fetuses with BAV, compared with controls, had a significantly larger diameter of the aorta at the level of the aortic valve (3.1 mm vs 3.0 mm (mean difference, 0.12 mm (95% CI, 0.03-0.21 mm))) and the pulmonary artery at the level of the pulmonary valve (4.1 mm vs 3.9 mm (mean difference, 0.15 mm (95% CI, 0.03-0.28 mm))). Following conversion of the diameter measurements of the aorta and pulmonary artery to Z-scores and Bonferroni correction, the differences between the two groups were no longer statistically significant. Pregnancy-associated plasma protein-A (PAPP-A) multiples of the median (MoM) was significantly lower in the BAV group than in the control group (0.85 vs 1.03; P = 0.04). CONCLUSIONS: Our findings suggest that fetuses with BAV may have a larger aortic diameter at the level of the aortic valve, measured in the left-ventricular-outflow-tract view, and a larger pulmonary artery diameter at the level of the pulmonary valve, measured in the three-vessel view, at 20 weeks' gestation. Moreover, we found an association of maternal smoking and low PAPP-A MoM with BAV. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.
Assuntos
Doença da Válvula Aórtica Bicúspide/diagnóstico , Biometria , Ecocardiografia , Coração Fetal/fisiopatologia , Ultrassonografia Pré-Natal , Adulto , Aorta/diagnóstico por imagem , Aorta/embriologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/embriologia , Doença da Válvula Aórtica Bicúspide/embriologia , Circulação Sanguínea , Estudos de Casos e Controles , Feminino , Coração Fetal/diagnóstico por imagem , Coração Fetal/embriologia , Feto/irrigação sanguínea , Feto/diagnóstico por imagem , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Gravidez , Segundo Trimestre da Gravidez , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/embriologia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/embriologiaRESUMO
PURPOSE: Bicuspid aortic valve (BAV) is a complex malformation affecting not merely the aortic valve. However, little is known regarding the dynamic physiology of the aortic annulus in these patients and whether it is similar to tricuspid aortic valves (TAV). Determining the BAV annular plane is more challenging than for TAV. Our aim was to present a standardized methodology to determine BAV annulus and investigate its changes in shape and dimensions during the cardiac cycle. METHODS: BAV patients were prospectively included and underwent an ECG-gated cardiac CTA. The annulus plane was manually identified on reconstructions at 5% intervals of the cardiac cycle with a new standardized method for different BAV types. Based on semi-automatically defined contours, maximum and minimum diameter, area, area-derived diameter, perimeter, asymmetry ratio (AR), and relative area were calculated. Differences of dynamic annular parameters were assessed also per BAV type. RESULTS: Of the 55 patients included (38.4 ± 13.3 years; 58% males), 38 had BAV Sievers type 1, 10 type 0, and 7 type 2. The minimum diameter, perimeter, area, and area-derived diameter were significantly higher in systole than in diastole with a relative change of 13.7%, 4.8%, 13.7%, and 7.2% respectively (all p < 0.001). The AR was ≥ 1.1 in all phases, indicating an elliptic shape, with more pronounced flattening in diastole (p < 0.001). Different BAV types showed comparable dynamic changes. CONCLUSIONS: BAV annulus undergo significant changes in shape during the cardiac cycle with a wider area in systole and a more elliptic conformation in diastole regardless of valve type. KEY POINTS: ⢠A refined method for the identification of the annulus plane on CT scans of patients with bicuspid aortic valves, tailored for the specific anatomy of each valve type, is proposed. ⢠The annulus of patients with bicuspid aortic valves undergoes significant changes during the cardiac cycle with a wider area and more circular shape in systole regardless of valve type. ⢠As compared to previously published data, the bicuspid aortic valve annulus has physiological dynamics similar to that encountered in tricuspid valves but with overall larger dimensions.
Assuntos
Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Substituição da Valva Aórtica Transcateter , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Feminino , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Humanos , MasculinoRESUMO
BACKGROUND: Aortic root evaluation is conventionally based on 2-dimensional measurements at a single phase of the cardiac cycle. This work presents an image analysis method for assessing dynamic 3-dimensional changes in the aortic root of minimally calcified bicuspid aortic valves (BAVs) with and without moderate to severe aortic regurgitation. METHODS: The aortic root was segmented over the full cardiac cycle in 3-dimensional transesophageal echocardiographic images acquired from 19 patients with minimally calcified BAVs and from 16 patients with physiologically normal tricuspid aortic valves (TAVs). The size and dynamics of the aortic root were assessed using the following image-derived measurements: absolute mean root volume and mean area at the level of the ventriculoaortic junction, sinuses of Valsalva, and sinotubular junction, as well as normalized root volume change and normalized area change of the ventriculoaortic junction, sinuses of Valsalva, and sinotubular junction over the cardiac cycle. RESULTS: Normalized volume change over the cardiac cycle was significantly greater in BAV roots with moderate to severe regurgitation than in normal TAV roots and in BAV roots with no or mild regurgitation. Aortic root dynamics were most significantly different at the mid-level of the sinuses of Valsalva in BAVs with moderate to severe regurgitation than in competent TAVs and BAVs. CONCLUSIONS: Echocardiographic reconstruction of the aortic root demonstrates significant differences in dynamics of BAV roots with moderate to severe regurgitation relative to physiologically normal TAVs and competent BAVs. This finding may have implications for risk of future dilatation, dissection, or rupture, which warrant further investigation.
Assuntos
Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Insuficiência da Valva Aórtica/fisiopatologia , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Ecocardiografia Tridimensional , Ecocardiografia Transesofagiana , Calcificação Vascular/fisiopatologia , Adulto , Idoso , Insuficiência da Valva Aórtica/complicações , Doença da Válvula Aórtica Bicúspide/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Calcificação Vascular/complicaçõesRESUMO
BACKGROUND: We aimed to provide a comprehensive aortic stiffness description using magnetic resonance imaging (MRI) in patients with ascending thoracic aorta aneurysm and tricuspid (TAV-ATAA) or bicuspid (BAV) aortic valve. METHODS: This case-control study included 18 TAV-ATAA and 19 BAV patients, with no aortic valve stenosis/severe regurgitation, who were 1:1 age-, gender- and central blood pressures (BP)-matched to healthy volunteers. Each underwent simultaneous aortic MRI and BP measurements. 3D anatomical MRI provided aortic diameters. Stiffness indices included: regional ascending (AA) and descending (DA) aorta pulse wave velocity (PWV) from 4D flow MRI; local AA and DA strain, distensibility and theoretical Bramwell-Hill (BH) model-based PWV, as well as regional arch PWV from 2D flow MRI. RESULTS: Patient groups had significantly higher maximal AA diameter (median[interquartile range], TAV-ATAA: 47.5[42.0-51.3]mm, BAV: 45.0[41.0-47.0]mm) than their respective controls (29.1[26.8-31.8] and 28.1[26.0-32.0]mm, p < 0.0001), while BP were similar (p ≥ 0.25). Stiffness indices were significantly associated with age (ρ ≥ 0.33), mean BP (arch PWV: ρ = 0.25, p = 0.05; DA distensibility: ρ = -0.30, p = 0.02) or AA diameter (arch PWV: ρ = 0.28, p = 0.03; DA PWV: ρ = 0.32, p = 0.009). None of them, however, was significantly different between TAV-ATAA or BAV patients and their matched controls. Finally, while direct PWV measures were significantly correlated to BH-PWV estimates in controls (ρ ≥ 0.40), associations were non-significant in TAV-ATAA and BAV groups (p ≥ 0.18). CONCLUSIONS: The overlap of MRI-derived aortic stiffness indices between patients with TAV or BAV aortopathy and matched controls highlights another heterogeneous feature of aortopathy, and suggests the urgent need for more sensitive indices which might help better discriminate such diseases.
Assuntos
Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Rigidez Vascular , Valva Aórtica/diagnóstico por imagem , Estudos de Casos e Controles , Doenças das Valvas Cardíacas/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Análise de Onda de PulsoRESUMO
Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as "the scimitar sign", is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.
Assuntos
Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Imageamento por Ressonância Magnética , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/fisiopatologia , Tomografia Computadorizada por Raios X , Adulto , Doença da Válvula Aórtica Bicúspide/complicações , Técnicas de Imagem Cardíaca , Humanos , Masculino , Imagem Multimodal , Síndrome de Cimitarra/complicaçõesRESUMO
PURPOSE: Bicuspid aortic valve (BAV) is a congenital heart malformation with phenotypic heterogeneity. There is no prior computational study that assesses the haemodynamic and valve mechanics associated with BAV type 2 against a healthy tricuspid aortic valve (TAV) and other BAV categories. METHODS: A proof-of-concept study incorporating three-dimensional fluid-structure interaction (FSI) models with idealised geometries (one TAV and six BAVs, namely type 0 with lateral and anterior-posterior orientations, type 1 with R-L, N-R and N-L leaflet fusion and type 2) has been developed. Transient physiological boundary conditions have been applied and simulations were run using an Arbitrary Lagrangian-Eulerian formulation. RESULTS: Our results showed the presence of abnormal haemodynamics in the aorta and abnormal valve mechanics: type 0 BAVs yielded the best haemodynamical and mechanical outcomes, but cusp stress distribution varied with valve orifice orientation, which can be linked to different cusp calcification location onset; type 1 BAVs gave rise to similar haemodynamics and valve mechanics, regardless of raphe position, but this position altered the location of abnormal haemodynamic features; finally, type 2 BAV constricted the majority of blood flow, exhibiting the most damaging haemodynamic and mechanical repercussions when compared to other BAV phenotypes. CONCLUSION: The findings of this proof-of-concept work suggest that there are specific differences across haemodynamics and valve mechanics associated with BAV phenotypes, which may be critical to subsequent processes associated with their pathophysiology processes.
Assuntos
Doença da Válvula Aórtica Bicúspide/fisiopatologia , Hemodinâmica , Modelos Cardiovasculares , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Fenômenos Biomecânicos , Velocidade do Fluxo Sanguíneo , Simulação por Computador , Módulo de Elasticidade , Humanos , Fenótipo , Estudo de Prova de Conceito , Estresse MecânicoRESUMO
Background In calcific aortic valve disease on tricuspid aortic valves (TAVs), men have higher aortic valve calcification and less fibrosis than women. However, little is known in bicuspid aortic valves (BAV). We thus aimed to investigate the impact of age, sex, and valve phenotype (TAVs versus BAVs) on fibro-calcific remodeling in calcific aortic valve disease. Methods and Results We included 2 cohorts: 411 patients who underwent multidetector computed tomography (37% women) for aortic valve calcification density assessment and 138 explanted aortic valves (histological cohort; 50% women). The cohorts were divided in younger (<60 years old) or older patients with BAV (≥60 years old), and TAV patients. In each group, women and men were matched. Women presented less aortic valve calcification density than men in each group of the multidetector computed tomography cohort (all P≤0.01). Moreover, in women, younger patients with BAV had the lowest aortic valve calcification density (both P=0.02). In multivariate analysis, aortic valve calcification density correlated with age (ß estimate±standard error: 6.5±1.8; P=0.0004) and male sex (109.2±18.4; P<0.0001), and there was a trend with TAVs (41.5±23.0; P=0.07). Women presented a higher collagen content than men (77.8±10.8 versus 69.9±12.9%; P<0.001) in the entire cohort. In women, younger patients with BAV had denser connective tissue than TAV and older patients with BAV (both P≤0.05), while no difference was observed between men. Conclusions In calcific aortic valve disease, women had less calcification and more fibrotic remodeling than men, regardless of the phenotype of the valve or age of the patient. Moreover, younger women with BAVs had less valve calcification. Thus, mineralization/fibrosis of the aortic valve is likely to have sex/age-specific mechanisms and be influenced by the valve morphology.
Assuntos
Estenose da Valva Aórtica/patologia , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Doença da Válvula Aórtica Bicúspide/patologia , Calcinose/patologia , Disparidades nos Níveis de Saúde , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/química , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/metabolismo , Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/metabolismo , Estenose da Valva Aórtica/fisiopatologia , Doença da Válvula Aórtica Bicúspide/diagnóstico por imagem , Doença da Válvula Aórtica Bicúspide/metabolismo , Doença da Válvula Aórtica Bicúspide/fisiopatologia , Calcinose/diagnóstico por imagem , Calcinose/metabolismo , Calcinose/fisiopatologia , Colágeno/análise , Feminino , Fibrose , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Medição de Risco , Fatores de Risco , Fatores SexuaisRESUMO
BACKGROUND: Measuring and improving outcomes is a central element of value-based health care. However, selecting improvement interventions based on outcome measures is complex and tools to support the selection process are lacking. The goal was to present strategies for the systematic identification and selection of improvement interventions applied to the case of aortic valve disease and to combine various methods of process and outcome assessment into one integrated approach for quality improvement. METHODS: For this case study a concept-driven mixed-method approach was applied for the identification of improvement intervention clusters including: (1) benchmarking outcomes, (2) data exploration, (3) care delivery process analysis, and (4) monitoring of ongoing improvements. The main outcome measures were long-term survival and 30-day mortality. For the selection of an improvement intervention, the causal relations between the potential improvement interventions and outcome measures were quantified followed by a team selection based on consensus from a multidisciplinary team of professionals. RESULTS: The study resulted in a toolbox: the Intervention Selection Toolbox (IST). The toolbox comprises two phases: (a) identifying potential for improvement, and (b) selecting an effective intervention from the four clusters expected to lead to the desired improvement in outcomes. The improvements identified for the case of aortic valve disease with impact on long-term survival in the context of the studied hospital in 2015 include: anticoagulation policy, increased attention to nutritional status of patients and determining frailty of patients before the treatment decision. CONCLUSIONS: Identifying potential for improvement and carefully selecting improvement interventions based on (clinical) outcome data demands a multifaceted approach. Our toolbox integrates both care delivery process analyses and outcome analyses. The toolbox is recommended for use in hospital care for the selection of high-impact improvement interventions.
Assuntos
Atenção à Saúde/normas , Cardiopatias Congênitas , Doenças das Valvas Cardíacas , Melhoria de Qualidade , Resultado do Tratamento , Valva Aórtica/fisiopatologia , Benchmarking , Doença da Válvula Aórtica Bicúspide , Serviços de Saúde , Cardiopatias Congênitas/mortalidade , Doenças das Valvas Cardíacas/mortalidade , Hospitais , Humanos , Avaliação de Resultados em Cuidados de SaúdeRESUMO
Background Four-dimensional (4D) flow MRI enables the evaluation of blood flow alterations in patients with congenital bicuspid aortic valve (BAV). However, current analysis methods are cumbersome and lack the use of the volumetric data from 4D MRI. Purpose To investigate the feasibility and reproducibility of a technique that uses a catheter-like mathematical model (virtual catheter) to assess volumetric intra-aortic hemodynamics from 4D flow MRI in patients with BAV. Materials and Methods In this retrospective study, data were collected from adult patients with BAV and healthy participants who underwent aortic 4D flow MRI from November 2011 through August 2014. Reproducibility was tested in healthy study participants who underwent test-retest examinations within 2 weeks. Patients were grouped on the basis of the severity of aortic valve regurgitation (AVR) and aortic valve stenosis (AVS). A 4D virtual catheter mathematical model for probing intra-aortic hemodynamic flow was constructed as a tube with an automatically derived radius along the entire thoracic aorta centerline. Volumetric intra-aortic hemodynamics were computed from 4D flow MRI only within the virtual catheter, and the following volume-normalized systolic peaks were derived: kinetic energy (KE), viscous energy loss rate (VELR), and vorticity. Hemodynamic data were presented as medians with interquartile ranges and compared by using Mann-Whitney U test and Kruskal-Wallis test. Results The study included 91 participants (57 patients [mean age, 46 years ± 12], 18 women; 34 healthy participants [mean age: 44 years ± 14], 12 women; 15 healthy participants underwent test-retest examinations). Patients showed higher VELR values compared with healthy participants (median, 31 W/m3 [interquartile range, 21-72] vs 23 W/m3 [interquartile range, 17-30], respectively; P < .001) and vorticity (69 sec-1 [interquartile range, 59-87] vs 60 sec-1 [interquartile range, 50-67], respectively; P < .001). Four-dimensional virtual catheter showed differences among different AVS and AVR grades with the highest VELR (120 W/m3; interquartile range, 99-166; P < .001) and vorticity (108 sec-1; interquartile range, 84-151; P < .001) found in severe AVS. High test-retest reproducibility was found for all virtual catheter-derived metrics (intraclass correlation, 0.80 ± 0.07; coefficient of variation, 9% ± 3). Conclusion The proposed four-dimensional (4D) virtual catheter technique enabled reproducible automated evaluation of volumetric intra-aortic hemodynamics alterations from 4D flow MRI in patients with bicuspid aortic valve. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Mitsouras and Hope in this issue.