Multimodality imaging of isolated bicuspid pulmonary valve leading to pulmonary stenosis.
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ABSTRACT:
SUBMITTER: Ozturk C
PROVIDER: S-EPMC5337025 | biostudies-literature | 2015 Jan
REPOSITORIES: biostudies-literature
Publications
Multimodality imaging of isolated bicuspid pulmonary valve leading to pulmonary stenosis.
Öztürk Cengiz C Demirkol Sait S Aparcı Mustafa M Sarı Sebahattin S Bozlar Uğur U Çelik Turgay T İyisoy Atila A
Anatolian journal of cardiology 20150121 2
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Project description:BackgroundTricuspid valve (TV) stenosis is a very rare condition and the aetiology is primarily due to rheumatic disease, carcinoid disease, congenital heart disease, endocarditis, and following degeneration of biological valve prosthesis.Case summaryWe present a 45-year-old man with a rare case of symptomatic TV stenosis (TS) in a previously isolated TV repair. A meticulous multimodality diagnostic approach is presented in order to determine the severity of the TS and to evaluate the right ventricular function.DiscussionThis case report presents an integrated multimodality imaging and haemodynamic approach to evaluate and document the suspicion of development of a symptomatic significant stenosis in a previous TV repair. The initial TV repair was done without ring annuloplasty, because only the anterior leaflet was affected and bicuspidalization of the valve made it patent. In addition, minimizing the amount of implanted material was intended to minimize the risk of reinfection. The final treatment was performed as a TV replacement with insertion of a bioprosthesis.
| S-EPMC7501896 | 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: Not available
| S-EPMC8114650 | biostudies-literature
Project description:Aortic stenosis (AS) is a disease of the valve and the myocardium. A correct assessment of the valve disease severity is key to define the need for aortic valve replacement (AVR), but a better understanding of the myocardial consequences of the increased afterload is paramount to optimize the timing of the intervention. Transthoracic echocardiography remains the cornerstone of AS assessment, as it is universally available, and it allows a comprehensive structural and hemodynamic evaluation of both the aortic valve and the rest of the heart. However, it may not be sufficient as a significant proportion of patients with severe AS presents with discordant grading (i.e., an AVA ≤ 1 cm2 and a mean gradient <40 mmHg) which raises uncertainty about the true severity of AS and the need for AVR. Several imaging modalities (transesophageal or stress echocardiography, computed tomography, cardiovascular magnetic resonance, positron emission tomography) exist that allow a detailed assessment of the stenotic aortic valve and the myocardial remodeling response. This review aims to provide an updated overview of these multimodality imaging techniques and seeks to highlight a practical approach to help clinical decision making in the challenging group of patients with discordant low-gradient AS.
| S-EPMC7744378 | 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:BackgroundSex-specific thresholds of computed tomography (CT)-derived aortic valve calcification (AVC) or AVC density (AVCd) to identify severe aortic stenosis (AS) have been established in populations that consisted mainly of Caucasians with a tricuspid aortic valve. The objective of this study was to evaluate the accuracy (i.e., sensitivity and specificity) of previously established thresholds to identify severe AS in patients with bicuspid aortic valve (BAV) and according to ethnicity: Caucasian vs. Asian.MethodsWe built a multicenter registry of echocardiographic and CT data collected in BAV patients with at least mild AS and preserved left ventricular ejection fraction from 7 different centers. Anatomic severity of AS obtained by CT-derived AVC and AVCd was compared to hemodynamic severity of AS obtained by echocardiography.ResultsAmong 485 BAV patients (60% men, 73% Asians), the best thresholds of AVC and AVCd to identify severe AS in BAV patients were 2315 arbitrary units (AU) (sensitivity [Se]/specificity [Spe] = 82/78%) in men, 1103 AU (Se/Spe = 80/82%) in women, and 561 AU/cm2 (Se/Spe = 86/91%) in men, and 301 AU/cm2 (Se/Spe = 83/82%) in women, respectively. According to ethnicity, thresholds for severe AS in Caucasian patients were, respectively, in men and women: 2208 AU (Se/Spe = 83/83%) and 1230 AU (Se/Spe = 87/82%) for AVC and 474 AU/cm2 (Se/Spe = 88/83%) and 358 AU/cm2 (Se/Spe = 80/82%) for AVCd. In Asian patients, they were 2582 AU (Se/Spe = 76/78%) and 924 AU (Se/Spe = 84/80%) for AVC and 640 AU/cm2 (Se/Spe = 82/89%) and 255 AU/cm2 (Se/Spe = 86/80%) for AVCd.ConclusionsThe optimal thresholds to identify hemodynamically severe AS in BAV patients are similar in Caucasians but appear to be higher in Asian men, compared with thresholds previously reported in tricuspid aortic valve patients. Nonetheless, the thresholds currently proposed in the guidelines have good accuracy and can be applied in BAV patients to confirm AS severity.
| S-EPMC10236792 | biostudies-literature
Project description:Bicuspid pulmonary valves and pulmonary artery aneurysms are two rare entities, reported in association, and usually attributed to hemodynamic alterations caused by the bicuspid pulmonary valve. We present magnetic resonance images of a patient with a bicuspid pulmonary valve and pulmonary artery aneurysm, and propose an alternative mechanism for this association, based on recent embryologic studies that link anomalies of the semilunar valves and great vessels with derangement of the cardiac neural crest cell development.
| S-EPMC4505617 | 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