Valve repair for aortic regurgitation of a bicuspid aortic valve with root dilatation late after an arterial switch operation.
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ABSTRACT:
SUBMITTER: Nakaji S
PROVIDER: S-EPMC8828926 | biostudies-literature | 2022 Feb
REPOSITORIES: biostudies-literature
Publications
Valve repair for aortic regurgitation of a bicuspid aortic valve with root dilatation late after an arterial switch operation.
Nakaji Shun S Obase Kikuko K Eishi Kiyoyuki K
JTCVS techniques 20211101
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Project description:Neoaortic root dilatation can develop during long-term follow-up after an arterial switch operation (ASO). Although few patients require surgical reintervention, significant valve regurgitation is still an important cause of late morbidity. We report on a 15-year-old boy with significant dilatation of the neoaortic root that was treated with the valve-sparing reimplantation technique. There is only one reported case of valve-preserving surgery late after the ASO. Valve preservation is believed to be superior to valve replacement in patients with aortic regurgitation due to better hemodynamic performance and avoidance of anticoagulation therapy.
| S-EPMC4360663 | biostudies-other
Project description:AimsInternational guidelines recommend screening of first-degree relatives (FDR) of people with bicuspid aortic valves (BAVs). However, the prevalence of BAV and of aortic dilatation amongst family members is uncertain.Methods and resultsA systematic review and meta-analysis of original reports of screening for BAV. Databases including MEDLINE, Embase, and Cochrane CENTRAL were searched from inception to December 2021 using relevant search terms. Data were sought on the screened prevalence of BAV and aortic dilatation. The protocol was specified prior to the searches being performed, and standard meta-analytic techniques were used. Twenty-three observational studies met inclusion criteria (n = 2297 index cases; n = 6054 screened relatives). The prevalence of BAV amongst relatives was 7.3% [95% confidence interval (CI) 6.1%-8.6%] overall and per family was 23.6% (95% CI 18.1%-29.5%). The prevalence of aortic dilatation amongst relatives was 9.4% (95% CI 5.7%-13.9%). Whilst the prevalence of aortic dilatation was particularly high in relatives with BAV (29.2%; 95% CI 15.3%-45.1%), aortic dilatation alongside tricuspid aortic valves was a more frequent finding, as there were many more family members with tricuspid valves than BAV. The prevalence estimate amongst relatives with tricuspid valves (7.0%; 95% CI 3.2%-12.0%) was higher than reported in the general population.ConclusionScreening family members of people with BAV can identify a cohort substantially enriched for the presence of bicuspid valve, aortic enlargement, or both. The implications for screening programmes are discussed, including in particular the substantial current uncertainties regarding the clinical implications of aortic findings.
| S-EPMC10702468 | biostudies-literature
Project description:BackgroundThe 3-dimensional relationship between aortic root and cusp is essential to understand the mechanism of aortic regurgitation (AR) because of aortic root dilatation (ARD). We sought to test the hypothesis that the stretched cusps in ARD enlarge to compensate for ARD.Methods and resultsComputed tomography imaged 92 patients (57 with ARD, 29 with moderate to severe AR, 28 without significant AR) and 35 normal controls. Specialized 3-dimensional software measured individual cusp surface areas relative to maximal mid-sinus cross-sectional area and minimal 3-dimensional annular area, coaptation area fraction, and asymmetry of sinus volumes and intercommissural distances. Total open cusp surface area increased (P<0.001) from 7.6±1.4 cm(2)/m(2) in normals to 12.9±2.2 cm(2)/m(2) in AR-negative and 15.2±3.3 cm(2)/m(2) in AR-positive patients. However, the ratio of closed cusp surface area to maximal mid-sinus area, reflecting cusp adaptation, decreased from normals to AR-negative to AR-positive patients (1.38±0.20, 1.15±0.15, 0.88±0.15; P<0.001), creating the lowest coaptation area fraction. Cusp distensibility (closed diastolic versus open area) decreased from 20% in controls and AR-negative patients to 5% in AR-positive patients (P<0.001). Multivariate determinants of AR and coaptation area fraction reflected both sinus size and cusp-to-annular adaptation. ARD was also progressively asymmetrical with root size, and individual cusp surface areas failed to match this asymmetry.ConclusionsAortic cusp enlargement occurs in ARD, but cusp adaptation and distensibility become limited in prominent, asymmetrical ARD, leading to AR. Optimal AR repair tailored to individual patient anatomy can benefit from appreciating valve adaptation and 3-dimensional relationships; understanding cusp adaptation mechanisms may ultimately provide therapeutic opportunities to improve such compensation.
| S-EPMC4373603 | biostudies-literature
Project description:Bicuspid aortic insufficiency (BAI) patients with root aneurysm often require aortic valve and root replacement in a composite procedure. The valve-sparing root replacement (VSARR) procedure is aimed at preserving the native valve when possible. This case highlights a successful transcatheter aortic valve replacement procedure in a BAI patient previously treated with VSARR. (Level of Difficulty: Intermediate.).
| S-EPMC8642721 | biostudies-literature
Project description:A 70-year-old male with chronic aortic regurgitation was referred with abrupt worsening heart failure. Late referral markers were pulmonary hypertension, mitral regurgitation, and tricuspid regurgitation. Evaluation revealed rupture of a raphal cord or fenestrated raphe from the conjoined cusp of a congenitally bicuspid aortic valve, a rare mechanism of aortic regurgitation. (Level of Difficulty: Intermediate.).
| S-EPMC10107042 | biostudies-literature
Project description: Not available
| S-EPMC6110703 | biostudies-literature
Project description:PurposeAbsolute local normalized helicity (LNH) can differentiate flow alterations in the aorta between healthy controls and bicuspid aortic valve (BAV) patients.MethodsA total of 65 controls and 50 subjects with BAV underwent in vivo four-dimensional (4D) flow MRI. Data analysis included the three-dimensional (3D) segmentation of the thoracic aorta (ascending aorta, aortic arch, and descending aorta) and calculation of absolute LNH. The mean velocity in the entire aorta was used to identify peak systole, systolic deceleration, and middiastole. A sensitivity analysis was performed to identify the optimal absolute LNH threshold, comparing control and BAV groups. A reproducibility test was performed for 3D segmentation and absolute LNH.ResultsAbsolute LNH above 0.6 was significantly higher (P < 0.001) in BAV in comparison to controls for all aortic segments and cardiac time frames. Absolute LNH in the ascending aorta correlated with maximal aortic diameter (R = 0.83, P < 0.001, at peak systole; r = 0.84, P < 0.001, at systolic deceleration; R = 0.88, P < 0.001, at middiastole) and significantly increased (P < 0.001) with aortic stenosis severity. Intra- and interobserver errors were 5 ± 2% and 12 ± 6% for 3D segmentation and 7 ± 6% and 12 ± 7% for absolute LNH.ConclusionAbsolute LNH can differentiate between controls and subjects with aortic dilatation, and was associated with maximal aortic diameter and aortic stenosis severity. Magn Reson Med 78:689-701, 2017. © 2016 International Society for Magnetic Resonance in Medicine.
| S-EPMC5316512 | biostudies-literature
Project description:BackgroundLeft atrial reservoir strain (LARS) is a novel imaging biomarker of left ventricular diastolic dysfunction. This study aimed to examine the prognostic implications of LARS in patients with bicuspid aortic valve and significant (moderate-severe to severe) aortic regurgitation.Methods and resultsA total of 220 patients with bicuspid aortic valve and significant aortic regurgitation were prospectively enrolled in our study. LARS and left ventricular global longitudinal strain were derived from speckle-tracking echocardiography. The end point was a composite of all-cause mortality, heart failure hospitalization, and aortic valve repair or replacement. The threshold value of LARS <24% was used to identify impaired left atrial mechanics based on prior results. During a median follow-up of 364 (interquartile range, 294-752) days, 46 patients (20.9%) reached the composite end points. On multivariable Cox analysis, impaired LARS (adjusted hazard ratio, 2.08 [95% CI, 1.05-4.11]; P=0.036) was a statistically significant predictor of composite end points after adjustment for other statistically significant predictors. Finally, adding impaired LARS to other statistically significant predictors (New York Heart Association functional class and left ventricular global longitudinal strain) significantly improved the global χ2 (from 32.19 to 36.56; P=0.037) and reclassification (continuous net reclassification index=0.55; P<0.001) of the prediction model.ConclusionsIn patients with bicuspid aortic valve and significant aortic regurgitation, the impairment of LARS is a strong independent prognostic predictor and confers incremental prognostic utility over clinical and other echocardiographic parameters. These findings suggest that LARS could be considered in risk stratification for such populations.
| S-EPMC11009999 | biostudies-literature
Project description:After an arterial switch operation for complete transposition of the great arteries, neo-aortic root dilatation occurs, with unclear hemodynamic effects. This study analyzes three groups (severe dilation, mild dilation, and normal) using computational fluid dynamics (CFD) on cardiac CT scans. Aortic arch angles in severe (median 72.3, range: 68.5-77.2) and mild dilation (76.6, 71.1-85.2) groups are significantly smaller than the normal group (97.3, 87.4-99.0). In the normal and mild dilatation groups, Wall Shear Stress (WSS) exhibits a consistent pattern: it is lowest at the aortic root, gradually increases until just before the bend in the aortic arch, peaks, and then subsequently decreases. However, severe dilation shows disrupted WSS patterns, notably lower in the distal ascending aorta, attributed to local recirculation. This unique WSS pattern observed in severely dilated patients, especially in the transverse aorta. CFD plays an essential role in comprehensively studying the pathophysiology underlying aortic dilation in this population.
| S-EPMC11885326 | biostudies-literature
Project description:BackgroundThe bicuspid aortic valve (BAV) is prone to ascending aortic dilatation (AAD) involving both the tubular segment and the aortic root. The genetic factor was proposed as one of the most important mechanisms for AAD. We hypothesized that the rare genetic variants mainly contribute to the pathogenesis of aortic roots in affected individuals.MethodsThe diameter of aortic root or ascending aorta ≥ 40 mm was counted as AAD. The targeted next-generation sequencing of 13 BAV-associated genes were performed on a continuous cohort of 96 unrelated BAV patients. The rare variants with allele frequency < 0.05% were selected and analyzed. Variants frequency was compared against the Exome aggregation consortium database. The pathogenicity of the genetic variants was evaluated according to the American College of Medical Genetics and Genomics guidelines.ResultsA total of 27 rare nonsynonymous coding variants involving 9 genes were identified in 25 individuals. The burden analysis revealed that variants in GATA5, GATA6, and NOTCH1 were significantly associated with BAV. Eighty percent of the pathogenic variants were detected in root group. The detection rate of rare variants was higher in root dilatation group (71.4%) compared with normal aorta (29.0%) and tubular dilatation groups (29.6%) (P = 0.018). The rare variant was identified as the independent risk factor of root dilatation [P = 0.014, hazard ratio = 23.9, 95% confidence interval (1.9-302.9)].ConclusionsOur results presented a broad genetic spectrum in BAV patients. The rare variants of BAV genes contribute the most to the root phenotype among BAV patients.
| S-EPMC8404252 | biostudies-literature