Diagnosis and Therapeutic Cardiac Catheterization of Symptomatic Bicuspid Aortic Stenosis in the Pediatric Population.
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ABSTRACT:
SUBMITTER: Yassin H
PROVIDER: S-EPMC9231541 | biostudies-literature | 2022 Jan-Mar
REPOSITORIES: biostudies-literature
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Diagnosis and Therapeutic Cardiac Catheterization of Symptomatic Bicuspid Aortic Stenosis in the Pediatric Population.
Yassin Haytham H Al-Saloos Hesham H
Heart views : the official journal of the Gulf Heart Association 20220101 1
Bicuspid aortic valve (BAV) is the most common congenital heart disease with a prevalence of 0.5%-1.3% of the population. Many children with BAV are asymptomatic. Clinically relevant abnormal valve function usually occurs in adulthood. However, in rare cases, children can fail to thrive which requires valvular intervention. In this review, we will explore in more detail the anatomy of the BAV, clinical presentation of BAV, diagnosis of BAV, and its function by echocardiography, and indications f ...[more]
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Project description:Background In this study, we investigate the utility of geometric orifice area (GOA) on cardiac computed tomography (CT) and differences from effective orifice area (EOA) on Doppler echocardiography in patients with bicuspid aortic stenosis (AS). Methods A total of 163 patients (age 64 ± 10 years, 56.4% men) with symptomatic bicuspid AS who were referred for surgery and underwent both cardiac CT and echocardiography within 3 months were studied. To calculate the aortic valve area, GOACT was measured by multiplanar CT planimetry, and EOAEcho was calculated by the continuity equation with Doppler echocardiography. The relationships between GOACT and EOAEcho and patient symptom scale, biomarkers, and left ventricular (LV) functional variables were analyzed. Results There was a significant but modest correlation between EOAEcho and GOACT (r = 0.604, p < 0.001). Both EOAEcho and GOACT revealed significant correlations with mean pressure gradient and peak transaortic velocity, and the coefficients were higher in EOAEcho than in GOACT. EOAEcho of 1.05 cm2 and GOACT of 1.25 cm2 corresponds to hemodynamic cutoff values for diagnosing severe AS. EOAEcho was well correlated with the patient symptom scale and log NT-pro BNP, but GOACT was not. In addition, EOAEcho had a higher correlation coefficient with estimated LV filling pressure and LV global longitudinal strain than GOACT. Conclusion GOACT can be used to evaluate the severity of bicuspid AS. The threshold for GOACT for diagnosing severe AS should be higher than that for EOAEcho. However, EOAEcho is still the method of choice because EOAEcho showed better correlations with clinical and functional variables than GOACT.
| S-EPMC9807240 | biostudies-literature
Project description:Bicuspid aortic valve (BAV) is the most frequent congenital anomaly and has a natural evolution toward aortic regurgitation or stenosis due to the asymmetrical valve function associated with an evolutive ascending aortopathy. Several BAV classifications exist describing the presence and number of raphe, amount and location of calcium, and the symmetry of the functional cusps. The impact of BAV morphology on transcatheter aortic valve implantation (TAVI) outcomes still remains little investigated. Pivotal randomized trials comparing TAVI with surgery have excluded BAV until yet. However, data from registries and observational studies including highly selected patients have shown promising results of TAVI in BAV. With this review, we aimed at describing anatomical and pathophysiological characteristics of BAV, discussing the main aspects to assess diagnostic imaging modalities, and giving an overview of TAVI outcomes and technical considerations specific to BAV morphology.
| S-EPMC8860891 | biostudies-literature
Project description:Introduction and purposeBicuspid aortic valve (BAV) disease is associated with faster aortic valve degeneration and a high incidence of aortic stenosis (AS). In this study, we aimed to identify differences in the pathophysiology of AS between BAV and tricuspid aortic valve (TAV) patients in a multiomics study integrating metabolomics and transcriptomics as well as clinical data.MethodsEighteen patients underwent aortic valve replacement due to severe aortic stenosis: 8 of them had a TAV, while 10 of them had a BAV. RNA sequencing (RNA-seq) and proton nuclear magnetic resonance spectroscopy (1H-NMR) were performed on these tissue samples to obtain the RNA profile and lipid and low-molecular-weight metabolites. These results combined with clinical data were posteriorly compared, and a multiomic profile specific to AS in BAV disease was obtained.ResultsH-NMR results showed that BAV patients with AS had different metabolic profiles than TAV patients. RNA-seq also showed differential RNA expression between the groups. Functional analysis helped connect this RNA pattern to mitochondrial dysfunction. Integration of RNA-seq, 1H-NMR and clinical data helped create a multiomic profile that suggested that mitochondrial dysfunction and oxidative stress are key players in the pathophysiology of AS in BAV disease.ConclusionsThe pathophysiology of AS in BAV disease differs from patients with a TAV and has a specific RNA and metabolic profile. This profile was associated with mitochondrial dysfunction and increased oxidative stress.
| S-EPMC10887224 | biostudies-literature
Project description:BackgroundBicuspid aortic valve (BAV) is associated with a faster progression of aortic stenosis (AS). Whether the determinants of AS progression are the same or different in patients with BAV vs tricuspid aortic valve (TAV) is unknown. The aim of this study was to identify the factors associated with the progression of AS in patients with BAV vs patients with TAV.MethodsPatients with AS were prospectively recruited in the Metabolic Determinants of the Progression of Aortic Stenosis (PROGRESSA) study (ClinicalTrials.gov Identifier: NCT01679431). The haemodynamic progression rate of AS was assessed by the annualized progression rate of peak aortic jet velocity (Vpeak). Univariable and multivariable linear regression analyses were used to identify the factors associated with a faster progression of AS in patients with BAV vs patients with TAV.ResultsThere were 79 patients with BAV and 208 patients with TAV. The baseline severity of AS was similar between the 2 groups of patients as well as the annualized progression rate of AS. In patients with BAV, obesity (β = 0.25, P = 0.04), diabetes (β = 0.26, P = 0.02), and BAV with right-noncoronary cusp fusion (β = 0.29, P = 0.01) were found to be independently associated with a faster progression of AS, whereas in patients with TAV, AS baseline severity (baseline Vpeak, β = 0.14, P = 0.04) and chronic kidney disease (β = 0.16, P = 0.02) were significantly associated with AS progression.ConclusionFactors associated with progression rate of AS are different in BAV and TAV. The main factors associated with a faster progression of AS appear to be obesity, diabetes, right-noncoronary cusp fusion in patients with BAV vs chronic kidney disease in patients with TAV.
| S-EPMC10642113 | biostudies-literature
Project description:AimsTo compare the progression of aortic stenosis (AS) in patients with bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV).Methods and resultsOne hundred and forty-one patients with mild-to-moderate AS, recruited prospectively in the PROGRESSA study, were included in this sub-analysis. Baseline clinical, Doppler echocardiography and multidetector computed tomography characteristics were compared between BAV (n = 32) and TAV (n = 109) patients. The 2-year haemodynamic [i.e. peak aortic jet velocity (Vpeak) and mean transvalvular gradient (MG)] and anatomic [i.e. aortic valve calcification density (AVCd) and aortic valve calcification density ratio (AVCd ratio)] progression of AS were compared between the two valve phenotypes. The 2-year progression rate of Vpeak was: 16 (-0 to 40) vs. 17 (3-35) cm/s, P = 0.95; of MG was: 1.8 (-0.7 to 5.8) vs. 2.6 (0.4-4.8) mmHg, P = 0.56; of AVCd was 32 (2-109) vs. 52 (25-85) AU/cm2, P = 0.15; and of AVCd ratio was: 0.08 (0.01-0.23) vs. 0.12 (0.06-0.18), P = 0.16 in patients with BAV vs. TAV. In univariable analyses, BAV was not associated with AS progression (all, P ≥ 0.26). However, with further adjustment for age, AS baseline severity, and several risk factors (i.e. sex, history of hypertension, creatinine level, diabetes, metabolic syndrome), BAV was independently associated with faster haemodynamic (Vpeak: β = 0.31, P = 0.02) and anatomic (AVCd: β = 0.26, P = 0.03 and AVCd ratio: β = 0.26, P = 0.03) progression of AS.ConclusionIn patients with mild-to-moderate AS, patients with BAV have faster haemodynamic and anatomic progression of AS when compared to TAV patients with similar age and risk profile. This study highlights the importance and necessity to closely monitor patients with BAV and to adequately control and treat their risk factors.Clinical trial registrationhttps://clinicaltrials.gov Unique identifier: NCT01679431.
| S-EPMC7306858 | biostudies-literature
Project description:IntroductionAlthough staging of the extent of aortic stenosis (AS)-related cardiac damages is usually performed via echocardiography, this technique has considerable limitations in assessing pulmonary artery and right chamber pressures. The present hypothesis-generating study sought to explore the efficacy of a staging system of cardiac damage based on echocardiographic and invasive [right heart catheterization (RHC)] hemodynamic parameters in patients undergoing transcatheter aortic valve implantation (TAVI).MethodsWe studied 90 symptomatic patients with severe AS in whom echocardiographic and invasive evaluation by RHC was obtained prior to TAVI. Cardiac damage stages were defined as follows: no cardiac damage (stage 0), left ventricular (LV) damage (stage 1), left atrial or mitral valve damage (stage 2), pulmonary vasculature or tricuspid valve damage (stage 3), and right ventricular (RV) dysfunction or low-flow state (stage 4). With the integrative approach using RHC, pulmonary hypertension (PH) was defined as an mPAP ≥25 mmHg and the low-flow state corresponded to a cardiac index of <1.8 L/min/m2 and a right atrial pressure of >10 mmHg.ResultsDuring follow-up (median: 2.9 years), 43 patients (47.8%) died. The integrative cardiac damage staging was associated with a significant increase in all-cause and cardiovascular mortality per each increase of cardiac damage stage, whereas the outcome was similar according to the echocardiographic staging.ConclusionsA staging system of cardiac lesion based on echocardiographic and invasive hemodynamic parameters in patients with severe AS undergoing TAVI predicts mortality. Patients with pre-existing PH, ≥ moderate tricuspid regurgitation and/or RV dysfunction, and a low-flow state had a markedly increased risk of death. Further larger studies are needed to validate our findings.
| S-EPMC10436206 | biostudies-literature
Project description:The bicuspid aortic valve (BAV) represents a complex anatomic scenario for transcatheter aortic valve replacement (TAVR) because of its unique technical challenges. As TAVR is moving towards younger and lower-risk populations, the proportion of BAV patients undergoing TAVR is expected to rise. Initial experiences of TAVR with first-generation transcatheter heart valves in high surgical risk patients with BAV stenosis showed higher rates of device failure and periprocedural complications as compared to tricuspid anatomy. The subsequent advances in imaging techniques and understanding of BAV anatomy, new iterations of transcatheter heart valves, and growing operators' experience yielded better outcomes. However, in the lack of randomized trials and rigorous evidence, the field of TAVR in BAV has been driven by empirical observations, with wide variability in transcatheter heart valve sizing and implantation techniques across different centers and operators. Thus, in this review article, we provide a fully illustrated overview of operative periprocedural steps for TAVR in BAV stenosis, though recognizing that it still remains anecdotal.
| S-EPMC10332651 | biostudies-literature
Project description:ImportanceThe outcomes of transcatheter aortic valve replacement (TAVR) in low-risk patients with bicuspid aortic valve stenosis have not been studied in a large scale, multicentered, prospective fashion.ObjectiveTo evaluate the procedural safety, efficacy, and 30-day outcomes of TAVR in patients with bicuspid aortic stenosis at low surgical risk.Design, setting, and participantsThe Low Risk Bicuspid Study is a prospective, single-arm trial study with inclusion/exclusion criteria developed from the Evolut Low Risk Randomized Trial. Follow-up is planned for 10 years. Patients underwent TAVR at 25 centers in the United States who were also participating in the Evolut Low Risk Randomized Trial from December 2018 to October 2019. Eligible patients had severe bicuspid aortic valve stenosis and met American Heart Association/American College of Cardiology guideline indications for aortic valve replacement.InterventionsPatients underwent attempted implant of an Evolut or Evolut PRO transcatheter aortic valve, with valve size based on annular measurements.Main outcomes and measuresThe prespecified primary end point was the incidence of all-cause mortality or disabling stroke at 30 days. The prespecified primary efficacy end point was device success defined as the absence of procedural mortality, the correct position of 1 bioprosthetic heart valve in the proper anatomical location, and the absence of more than mild aortic regurgitation postprocedure.ResultsA total of 150 patients underwent an attempted implant. Baseline characteristics include mean age of 70.3 (5.5) years, 48.0% female (n = 72), and a mean Society of Thoracic Surgeons score of 1.4 (0.6%). Most patients (136; 90.7%) had Sievers type I valve morphology. The incidence of all-cause mortality or disabling stroke was 1.3% (95% CI, 0.3%-5.3%) at 30 days. The device success rate was 95.3% (95% CI, 90.5%-98.1%). At 30 days, the mean (SD) AV gradient was 7.6 (3.7) mm Hg and effective orifice area was 2.3 (0.7) cm2. A new permanent pacemaker was implanted in 22 patients (15.1%). No patients had greater than mild paravalvular leak.Conclusions and relevanceTranscatheter aortic valve replacement in low-surgical risk patients with bicuspid aortic valve stenosis achieved favorable 30-day results, with low rates of death and stroke and high device success rate.Trial registrationClinicalTrials.gov Identifier: NCT03635424.
| S-EPMC7542521 | biostudies-literature
Project description:ObjectivesBicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta.MethodsFour-dimensional flow magnetic resonance imaging was performed in 50 subjects with right-left bicuspid aortic valve and 15 age- and aortic size-matched controls with tricuspid aortic valve. Patients with bicuspid aortic valve were categorized into 3 groups according to their aortic valve function as follows: bicuspid aortic valve with no more than mild aortic valve dysfunction (bicuspid aortic valve control, n = 20), bicuspid aortic valve with severe aortic insufficiency (n = 15), and bicuspid aortic valve with severe aortic stenosis (n = 15).ResultsAll patients with right-left bicuspid aortic valve exhibited peak wall shear stress at the right-anterior position of the ascending aorta (bicuspid aortic valve vs trileaflet aortic valve at the right-anterior position: 0.91 ± 0.23 N/m2 vs 0.43 ± 0.12 N/m2, P < .001) with no distinct alteration between bicuspid aortic valve with severe aortic insufficiency and bicuspid aortic valve with severe aortic stenosis. The predominance of dilatation involving the tubular ascending aorta (82%, type 2 aortopathy) persisted, with or without valve dysfunction. Compared with bicuspid aortic valve control subjects, the bicuspid aortic valve with severe aortic insufficiency group displayed universally elevated wall shear stress (0.75 ± 0.12 N/m2 vs 0.57 ± 0.09 N/m2, P < .01) in the ascending aorta, which was associated with elevated cardiac stroke volume (P < .05). The bicuspid aortic valve with severe aortic stenosis group showed elevated flow eccentricity in the form of significantly increased standard deviation of circumferential wall shear stress, which correlated with markedly increased peak aortic valve velocity (P < .01).ConclusionsThe location of peak aortic wall shear stress and type of aortopathy remained homogeneous among patients with right-left bicuspid aortic valve irrespective of valve dysfunction. Severe aortic insufficiency or stenosis resulted in further elevated aortic wall shear stress and exaggerated flow eccentricity.
| S-EPMC5438758 | biostudies-literature
Project description:BackgroundIt is unknown whether there are differences in coronary access after transcatheter aortic valve replacement (TAVR) between bicuspid and tricuspid anatomy.AimsOur aim was to investigate coronary access after TAVR using a self-expanding transcatheter heart valve (THV) in bicuspid versus tricuspid aortic valves (BAV vs TAV), based on CT simulation.MethodsA total of 86 type 0 BAV, 70 type 1 BAV, and 132 TAV patients were included. If the coronary ostium faced the sealed parts of the THV or the tilted-up native leaflet (NL), this was defined as THV- or NL-related challenging coronary access, respectively. If coaxial engagement was not allowed due to interference from the unwrapped frame, THV-related complex coronary access was defined.ResultsThe incidence of THV-related challenging coronary access was 21.2% for the left coronary artery (LCA) and 17.7% for the right coronary artery (RCA), and type 0 BAV patients encountered fewer THV-related challenging LCA access than their TAV counterparts (OR 0.42, 95% CI: 0.20-0.89). NL-related challenging coronary access was observed in 3.1% for LCA and 1.4% for RCA, and THV-related complex coronary access was identified in 5.9% for LCA and 17.0% for RCA; however, no significant differences were found among groups. The proportion of optimal fluoroscopic viewing angles suitable for guiding LCA engagement was similar among groups (64.0% vs 70.0% vs 62.1%), but those suitable for guiding RCA engagement were significantly higher in the type 0 BAV group (31.4% vs 4.3% vs 9.1%).ConclusionsCoronary access may be challenging or complex in a significant proportion of both BAV and TAV patients after TAVR. Type 0 BAV anatomy may be more favourable for post-TAVR coronary access.
| S-EPMC9912966 | biostudies-literature