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1.
Heart Lung ; 67: 144-151, 2024.
Article in English | MEDLINE | ID: mdl-38762962

ABSTRACT

BACKGROUND: Despite comprising almost half of all patients undergoing valvular repair, data on transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic stenosis (BAS) are limited. OBJECTIVE: We aimed to evaluate whether there are any sex differences in trends and outcomes of TAVR in this population. METHODS: We utilized the National Inpatient Sample from 2012 to 2020 to identify admissions with BAS who underwent TAVR and analyzed trends and outcomes. Our primary outcome was in-hospital mortality and secondary outcomes were in-hospital complications. We used two models to adjust for demographics (A) and interventions (B). RESULTS: Between 2012 to 2020, there were 76,540 hospitalizations for BAS patients who underwent AVR, among which 6,010 (7.9 %) underwent TAVR. There was an overall increasing trend in number of TAVR cases with a decreasing trend in mortality (2013: 8.7 %, 2020: 1.3 %). TAVR was performed more in males (61.1% vs 38.9 %). Despite the worse baseline characteristics in males, in-hospital mortality (2.4% vs. 1.5 %; OR: 1.584; 95 % CI: 0.621-4.038; p = 0.335) and secondary outcomes were similar across both sexes, even after adjusting for demographics and interventions. CONCLUSION: TAVR in BAS has grown rapidly in the last decade. Males comprised the majority and had more comorbidities, but mortality and complications were similar in both sexes. Despite the increasing number of cases, a decreasing trend in mortality was observed for both sexes ultimately approaching that of SAVR, suggesting that TAVR may be a safe alternative among eligible males and females with bicuspid AS.


Subject(s)
Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Hospital Mortality , Transcatheter Aortic Valve Replacement , Humans , Transcatheter Aortic Valve Replacement/methods , Transcatheter Aortic Valve Replacement/statistics & numerical data , Transcatheter Aortic Valve Replacement/adverse effects , Male , Female , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/epidemiology , Aortic Valve Stenosis/complications , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/complications , Hospital Mortality/trends , Aged , Sex Factors , United States/epidemiology , Aged, 80 and over , Retrospective Studies , Postoperative Complications/epidemiology , Treatment Outcome , Aortic Valve/surgery , Aortic Valve/abnormalities , Risk Factors , Heart Valve Diseases/surgery , Heart Valve Diseases/complications
2.
Int J Cardiol ; 409: 132201, 2024 Aug 15.
Article in English | MEDLINE | ID: mdl-38782071

ABSTRACT

BACKGROUND: To date, whether ascending aorta dilation (AAD) should be considered a contraindication for transcatheter aortic valve replacement (TAVR) remains a topic of debate.. OBJECTIVE: The study investigated the clinical outcome of TAVR in patients with bicuspid aortic valve stenosis (BAV-AS) complicated by AAD. METHODS: We included patients with BAV-AS who underwent TAVR between 2012 and 2019. We collected patient perioperative clinical data., tracked clinical outcomes for over four years post-TAVR, and obtained echocardiography images one year postoperatively. The Kaplan-Meier method was employed for analyzing both unadjusted and adjusted survival data, which was compared using the log-rank test. COX regression and nomograms were used to assess the impact of AAD on post-TAVR clinical outcomes in patients with aortic stenosis (AS), with all-cause mortality as the primary clinical endpoint. RESULTS: A total of 111 BAV patients were included in this study. Long-term follow-up showed an increased mortality risk in patients with BAV-AAD (adjusted Kaplan-Meier analysis: P = .02/0.001). Cox correlation analysis indicated that age (OR = 1.137; P = .034), AAD (OR = 3.51; P = .038), and postoperative left ventricular pressure (LVSP) (OR: 0.959; P = .044) were predictive factors for mortality more than four years after TAVR in patients with BAV. The area under the curve of the Nomogram predicting long-term survival for the training set of patients based on the above metrics was 0.845 (95% CI: 0.696-0.994). Short-term cardiac ultrasound follow-up showed a more rapid rate of AA expansion (0.29 [0-0.34] vs. -1 [-3.3-1] mm/month, P = .001) and a smaller proportion of AA diameter reduction (7.1% vs. 53.7%, P = .001) in patients who died. CONCLUSIONS: Patients with BAV-AAD-AS treated with TAVR have an increased risk of long-term mortality, and clinical prediction models, including AAD age and postoperative LVSP, may predict long-term patient survival. CONDENSED ABSTRACT: The study investigated the clinical outcome of transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve stenosis (BAV-AS) complicated by ascending aorta dilation (AAD). Patients with BAV-AAD-AS treated with TAVR have an increased risk of long-term mortality. AAD, age and postoperative LVSP, may predict long-term patient survival. Short-term cardiac ultrasound follow-up showed a more rapid rate of AA expansion and a smaller proportion of AA diameter reduction in patients who died. A high postoperative AAD expansion rate may indicate an adverse clinical outcome. Surgery regimens for tolerable BAV-AADs and can be considered as a treatment option.


Subject(s)
Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Transcatheter Aortic Valve Replacement , Humans , Male , Female , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/mortality , Aged , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/complications , Bicuspid Aortic Valve Disease/diagnostic imaging , Treatment Outcome , Follow-Up Studies , Retrospective Studies , Aged, 80 and over , Time Factors , Middle Aged
3.
Am Heart J ; 274: 65-74, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38701961

ABSTRACT

BACKGROUND: There has not been a consensus on the prothesis sizing strategy in type 0 bicuspid aortic stenosis (AS) patients undergoing transcatheter aortic valve replacement (TAVR). Modifications to standard annular sizing strategies might be required due to the distinct anatomical characteristics. We have devised a downsizing strategy for TAVR using a self-expanding valve specifically for patients with type 0 bicuspid AS. The primary aim of this study is to compare the safety and efficacy of downsizing strategy with the Standard Annulus Sizing Strategy in TAVR for patients with type 0 bicuspid AS. TRIAL DESIGN: It is a prospective, multi-center, superiority, single-blinded, randomized controlled trial comparing the Down Sizing and Standard Annulus Sizing Strategy in patients with type 0 bicuspid aortic stenosis undergoing transcatheter aortic valve replacement. Eligible participants will include patients with severe type 0 bicuspid AS, as defined by criteria such as mean gradient across aortic valve ≥40 mmHg, peak aortic jet velocity ≥4.0 m/s, aortic valve area (AVA) ≤1.0 cm², or AVA index ≤0.6 cm2/m2. These patients will be randomly assigned, in a 1:1 ratio, to either the Down Sizing Strategy group or the Standard Sizing Strategy group. In the Down Sizing Strategy group, a valve one size smaller will be implanted if the "waist sign" manifests along with less than mild regurgitation during balloon pre-dilatation. The primary end point of the study is a composite of VARC-3 defined device success, absence of both permanent pacemaker implantation due to high-degree atrioventricular block and new-onset complete left bundle branch block. CONCLUSION: This study will compare the safety and efficacy of Down Sizing Strategy with the Standard Annulus Sizing Strategy and provide valuable insights into the optimal approach for sizing in TAVR patients with type 0 bicuspid AS. We hypothesize that the Down Sizing Strategy will demonstrate superiority when compared to the Standard Annulus Sizing Strategy. (Down Sizing Strategy (HANGZHOU Solution) vs Standard Sizing Strategy TAVR in Bicuspid Aortic Stenosis (Type 0) (TAILOR-TAVR), NCT05511792).


Subject(s)
Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Heart Valve Prosthesis , Prosthesis Design , Transcatheter Aortic Valve Replacement , Humans , Transcatheter Aortic Valve Replacement/methods , Aortic Valve Stenosis/surgery , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/complications , Prospective Studies , Single-Blind Method , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Male , Female
5.
BMC Pediatr ; 24(1): 317, 2024 May 08.
Article in English | MEDLINE | ID: mdl-38720245

ABSTRACT

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.


Subject(s)
Turner Syndrome , Humans , Turner Syndrome/complications , Child , Adolescent , Female , China/epidemiology , Dilatation, Pathologic/etiology , Male , Retrospective Studies , Aorta/pathology , Aorta/diagnostic imaging , Aortic Coarctation , Bicuspid Aortic Valve Disease/complications , Child, Preschool , Incidence , East Asian People
6.
J Vet Cardiol ; 53: 13-19, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38565003

ABSTRACT

An 11-year-old male neutered American bulldog was presented for evaluation of thrombocytopenia, acute onset of ataxia, and vomiting. A new murmur was auscultated on physical examination. Transthoracic echocardiographic examination revealed a bicuspid aortic valve, vegetative lesions on the aortic valve, and continuous shunting from the aortic root to the left atrium through an aorta to left atrial fistula. The dog was euthanized due to its guarded prognosis and critical condition. Pathological examination confirmed presence of a bicuspid aortic valve, aorto-left atrial fistula, and aortic infective endocarditis. Antemortem blood culture revealed two unusual organisms: Achromobacter xylosoxidans and Fusobacterium mortiferum.


Subject(s)
Aortic Valve , Bicuspid Aortic Valve Disease , Dog Diseases , Endocarditis, Bacterial , Heart Atria , Dogs , Animals , Male , Dog Diseases/microbiology , Dog Diseases/diagnostic imaging , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Aortic Valve/pathology , Endocarditis, Bacterial/veterinary , Endocarditis, Bacterial/complications , Endocarditis, Bacterial/diagnosis , Heart Atria/pathology , Heart Atria/abnormalities , Bicuspid Aortic Valve Disease/complications , Vascular Fistula/veterinary , Vascular Fistula/complications , Vascular Fistula/diagnostic imaging , Aortic Diseases/veterinary , Aortic Diseases/complications , Aortic Diseases/diagnostic imaging , Heart Valve Diseases/veterinary , Heart Valve Diseases/complications , Echocardiography/veterinary , Heart Diseases/veterinary , Heart Diseases/complications , Fistula/veterinary , Fistula/complications , Aortic Valve Disease/veterinary , Aortic Valve Disease/complications
7.
Int J Cardiol ; 407: 132000, 2024 Jul 15.
Article in English | MEDLINE | ID: mdl-38561108

ABSTRACT

AIM: To evaluate the association between raphe in bicuspid aortic valve (BAV) patients and valve dysfunction, aortopathy and aortic valve surgery in the REBECCA registry [REgistro della valvola aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging (SIECVI)]. METHODS: Prevalence of aortic valve dysfunction and aortopathy was investigated in BAV patients with and without raphe. Aortic valve dysfunction (regurgitation or stenosis) was categorized as mild, moderate and severe. Aortopathy was defined as annulus ≥14 mm/m2; root ≥20 mm/m2; sinotubular junction ≥16 mm/m2; ascending aorta ≥17 mm/m2, and classified in Type A, isolated ascending aorta dilatation; Type B, aortic root and ascending aorta dilatation; and Type C, isolated aortic root dilatation. RESULTS: Overall, 695 patients with BAV were enrolled; 520 (74.8%) with raphe and 175 (25.2%) without raphe. BAV patients with raphe presented more frequently with moderate or severe aortic stenosis than BAV patients without raphe (183 [35.2%] vs 34 [19.4%], p < 0.001). A higher prevalence of aortopathy, particularly Type B, was observed in patients with vs without raphe. At multivariable analysis, raphe was a predictor of aortic valve surgery at three-year follow-up (odds ratio 2.19, 95% confidence interval 1.08-4.44, p < 0.001). CONCLUSIONS: Patients with BAV and raphe have a higher prevalence of significant aortic stenosis, aortopathy, especially Type B, and a higher risk of undergoing aortic valve surgery at three-year follow-up.


Subject(s)
Aortic Valve , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Registries , Humans , Male , Female , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/diagnostic imaging , Bicuspid Aortic Valve Disease/complications , Middle Aged , Aortic Valve/abnormalities , Aortic Valve/surgery , Aortic Valve/diagnostic imaging , Aged , Heart Valve Diseases/surgery , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/complications , Aortic Diseases/surgery , Aortic Diseases/diagnostic imaging , Aortic Diseases/epidemiology , Adult , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/diagnostic imaging , Follow-Up Studies , Italy/epidemiology
9.
J Cardiovasc Electrophysiol ; 35(6): 1069-1077, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38509335

ABSTRACT

INTRODUCTION: Bicuspid aortic valves (BAV) are the most common congenital heart defects and the extent of ventricular arrhythmias (VA) in patients with BAV is unclear. The objective of this study is to describe VAs and late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR) in patients with BAV. METHODS: A total of 19 patients with BAV (18 males, age: 58 ± 13 years) were referred for VA ablation procedures. Ten patients had BAVs at the time of ablation, nine patients had prior aortic valve replacement for a BAV. All but one patient had LGE-CMR and all patients underwent programmed ventricular stimulation at the time of the ablation. RESULTS: Frequent PVCs were the targeted VAs in 17/19 patients and VT in 2/19 patients. Monomorphic ventricular tachycardia (VT) was inducible in 6 patients. A total of 15 VTs were inducible (2.5 ± 1.0 VTs per patient with a mean cycle length of 322 ± 83 msec). LGE was present in 13 patients. Patients with inducible VT had larger borderzone and core scar compared to non-inducible patients (7.8 ± 2.1 cm3 vs. 2.5 ± 3.1 cm3 and 5.1 ± 2.6 cm3 vs. 1.9 ± 3.0 cm3, p-value < .05 for both). PVCs and VTs were mapped to the periaortic valve area in 12 patients and 4 patients, respectively. The PVC burden was reduced from 27 ± 13 to 3 ± 6 (p < .001) and the ejection fraction improved from 49 ± 13% to 55 ± 9% (p = .005). CONCLUSIONS: VAs in patients with BAV often originate from the perivalvular area and patients often have LGE and inducible VT. LGE may be due to ventricular remodeling secondary to the presence of BAV and harbors the arrhythmogenic substrate for VT.


Subject(s)
Bicuspid Aortic Valve Disease , Tachycardia, Ventricular , Humans , Male , Female , Middle Aged , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/physiopathology , Bicuspid Aortic Valve Disease/complications , Bicuspid Aortic Valve Disease/diagnostic imaging , Tachycardia, Ventricular/physiopathology , Tachycardia, Ventricular/diagnosis , Tachycardia, Ventricular/etiology , Aged , Adult , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Catheter Ablation , Action Potentials , Heart Valve Diseases/physiopathology , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/complications , Heart Valve Diseases/surgery , Heart Rate , Treatment Outcome , Risk Factors , Predictive Value of Tests , Retrospective Studies , Electrophysiologic Techniques, Cardiac , Contrast Media/administration & dosage , Magnetic Resonance Imaging, Cine
11.
BMC Cardiovasc Disord ; 24(1): 51, 2024 Jan 15.
Article in English | MEDLINE | ID: mdl-38221637

ABSTRACT

Mitral valve aneurysm (MVA) is characterized by a saccular outpouching of the mitral leaflet, and it represents a rare condition typically associated with aortic valve endocarditis. Three-Dimensional Transesophageal Echocardiography (3D-TEE) serves as an effective tool for detecting the presence of MVA and its potential complications. In this report, we present a case involving a young man with striking images of bicuspid aortic valve endocarditis complicated by an aortic root abscess and multiple perforated mitral valve aneurysms, diagnosed using 3D TEE. This case suggests the uncommon coexistence of Marfan like morphotype, bicuspid aortic valve, and infective endocarditis as a triple mechanism in the occurrence of MVA. It underscores the significance of early and accurate imaging diagnosis for facilitating prompt surgical intervention.


Subject(s)
Bicuspid Aortic Valve Disease , Echocardiography, Three-Dimensional , Endocarditis, Bacterial , Endocarditis , Heart Aneurysm , Marfan Syndrome , Humans , Male , Abscess/diagnostic imaging , Abscess/etiology , Aorta, Thoracic , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Bicuspid Aortic Valve Disease/complications , Echocardiography, Transesophageal/methods , Endocarditis/complications , Endocarditis/diagnostic imaging , Endocarditis, Bacterial/complications , Endocarditis, Bacterial/diagnostic imaging , Endocarditis, Bacterial/surgery , Heart Aneurysm/etiology , Heart Aneurysm/complications , Marfan Syndrome/complications , Marfan Syndrome/diagnosis , Mitral Valve/diagnostic imaging , Mitral Valve/surgery
13.
Eur Heart J Cardiovasc Imaging ; 25(3): 425-435, 2024 Feb 22.
Article in English | MEDLINE | ID: mdl-37966276

ABSTRACT

AIMS: Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. METHODS AND RESULTS: A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). CONCLUSION: BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.


Subject(s)
Aortic Valve Insufficiency , Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Humans , Female , Adult , Bicuspid Aortic Valve Disease/complications , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/abnormalities , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/complications , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/complications , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/surgery , Aortic Valve Insufficiency/complications , Retrospective Studies
14.
Curr Probl Cardiol ; 49(2): 102223, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38042228

ABSTRACT

Aortic stenosis is one of the most prevalent cardiac valvular diseases throughout the world and has a significant impact on quality of life. While there are several etiologies, we will be discussing the case of a male in his mid-thirties of southeast Asian descent with a bicuspid aortic valve which was found to be functionally unicuspid and complicated by aortic dilation. Following a comprehensive review of literature, it appears this subset of aortic stenosis is not commonly encountered. In addition to presenting this fascinating case, we will review the epidemiology, classification and management of aortic stenosis. Furthermore, we will examine the latest evidence-based literature on bicuspid aortic valve and unicuspid aortic valve and discuss interventions and diagnostic tools that may improve clinical prognosis.


Subject(s)
Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Adult , Male , Humans , Bicuspid Aortic Valve Disease/complications , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Quality of Life , Heart Valve Diseases/diagnosis , Aortic Valve Stenosis/diagnosis , Aortic Valve Stenosis/etiology , Aortic Valve Stenosis/surgery
15.
Ann Thorac Surg ; 118(1): 173-179, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38135262

ABSTRACT

BACKGROUND: This study investigated the safety and effectiveness of surgical aortic valve replacement with RESILIA tissue (Edwards Lifesciences) through 5 years in patients with native bicuspid aortic valves. Outcomes were compared with those for patients with tricuspid aortic valves. METHODS: Of 689 patients from the COMMENCE (ProspeCtive, nOn-randoMized, MulticENter) trial who received the study valve, 645 had documented native valve morphology and core laboratory-evaluable echocardiograms from any postoperative visit, which were used to model hemodynamic outcomes over 5 years. Linear mixed-effects models were used to estimate longitudinal changes in mean gradient and effective orifice area. RESULTS: Patients with native bicuspid aortic valves (n = 214) were more than a decade younger than those with tricuspid aortic valves (n = 458; 59.8 ± 12.4 years vs 70.2 ± 9.5 years; P < .001). The bicuspid aortic valve cohort exhibited no structural valve deterioration over 5 years, and rates of paravalvular leak and transvalvular regurgitation were low (0.7% and 2.9%, respectively [all mild] at 5 years). These outcomes mirrored those in patients with native tricuspid aortic valves. The model-estimated postoperative mean gradient and effective orifice area, as well as the rate of change of these outcomes, adjusted for age, body surface area, and bioprosthesis size, did not differ between the 2 cohorts. CONCLUSIONS: Among patients with bicuspid aortic valves, RESILIA tissue valves demonstrated excellent outcomes to 5 years, including no structural valve deterioration and very low rates of paravalvular and transvalvular regurgitation. These results are encouraging for RESILIA tissue durability in young patients.


Subject(s)
Aortic Valve , Bicuspid Aortic Valve Disease , Bioprosthesis , Heart Valve Diseases , Heart Valve Prosthesis , Prosthesis Design , Humans , Male , Female , Middle Aged , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Valve/diagnostic imaging , Bicuspid Aortic Valve Disease/surgery , Bicuspid Aortic Valve Disease/complications , Heart Valve Diseases/surgery , Aged , Prospective Studies , Treatment Outcome , Heart Valve Prosthesis Implantation/methods , Time Factors , Follow-Up Studies
16.
Cytogenet Genome Res ; 163(5-6): 290-294, 2023.
Article in English | MEDLINE | ID: mdl-38128492

ABSTRACT

INTRODUCTION: Bicuspid aortic valve is the most common congenital cardiac malformation (CCM) in adults and is 30-50 times more frequent in Turner syndrome (TS). We hypothesize that both X and Y chromosome dosages contribute to the prevalence of CCM in TS. The recognition of genotype-phenotype correlations may improve risk stratification of patients with 45,X karyotypes who have cryptic Y chromosome mosaicism. METHODS: Utilizing data and samples from the UTHealth Turner Syndrome Research Registry, we correlated Y chromosome DNA identified by multiplex quantitative PCR and SNP microarrays with the presence of congenital heart lesions. RESULTS: We identified Y chromosome DNA in more than 10% of registry participants, including 2 participants who had no detectable Y DNA by karyotype or SNP microarray. CONCLUSIONS: There were no significant correlations between the presence of Y DNA and CCM.


Subject(s)
Chromosomes, Human, Y , Turner Syndrome , Humans , Turner Syndrome/genetics , Turner Syndrome/complications , Female , Chromosomes, Human, Y/genetics , Adult , Polymorphism, Single Nucleotide , Chromosomes, Human, X/genetics , Mosaicism , Adolescent , Heart Defects, Congenital/genetics , Karyotype , Bicuspid Aortic Valve Disease/genetics , Bicuspid Aortic Valve Disease/complications , Karyotyping , Child , Cohort Studies , Genetic Association Studies , Young Adult , Aortic Valve/abnormalities
17.
Monaldi Arch Chest Dis ; 94(1)2023 Sep 15.
Article in English | MEDLINE | ID: mdl-37721026

ABSTRACT

The bicuspid aortic valve (BAV) presents a multifaceted clinical challenge due to its diverse morphologies and associated complications. This review aims to elucidate the critical role of cardiac imaging in guiding optimal management strategies for BAV patients. BAV, with a prevalence of 1-2%, has genetic underpinnings linked to the NOTCH1 gene mutation. Variability in BAV morphology necessitates tailored surgical approaches. The three primary types of BAV morphology - right-left cusp fusion, right-noncoronary cusp fusion, and left-noncoronary cusp fusion - demand nuanced considerations due to their distinct implications. Valvular dysfunction results in aortic stenosis or regurgitation, attributed to altered valve structure and turbulent hemodynamics. Cardiac imaging modalities, including echocardiography, magnetic resonance imaging, and computerized tomography, are instrumental in assessing valve function, aortic dimensions, and associated complications. Imaging helps predict potential complications, enabling informed treatment decisions. Regular follow-up is crucial to detecting alterations early and intervening promptly. Surgical management options encompass aortic valve repair or replacement, with patient-specific factors guiding the choice. Post-surgical surveillance plays a vital role in preventing complications and optimizing patient outcomes. The review underscores the significance of advanced cardiac imaging techniques in understanding BAV's complexities, facilitating personalized management strategies, and improving patient care. By harnessing the power of multimodal imaging, clinicians can tailor interventions, monitor disease progression, and ultimately enhance the prognosis and quality of life for individuals with BAV.


Subject(s)
Aortic Valve Stenosis , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Adult , Humans , Bicuspid Aortic Valve Disease/complications , Bicuspid Aortic Valve Disease/pathology , Heart Valve Diseases/complications , Quality of Life , Aortic Valve/diagnostic imaging , Echocardiography/methods , Retrospective Studies
18.
Ultrasound Med Biol ; 49(11): 2354-2360, 2023 11.
Article in English | MEDLINE | ID: mdl-37573177

ABSTRACT

OBJECTIVE: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Although the etiology is complex, altered flow dynamics is thought to play an important role. Blood speckle tracking (BST) allows for visualization and quantification of complex flow, which could be useful in identifying patients at risk of root dilation and could aid in surgical planning. The aims of this study were to assess and quantify flow in the aortic root and left ventricle using BST in children with bicuspid aortic valves. METHODS AND RESULTS: A total of 38 children <10 y of age were included (24 controls, 14 with BAV). Flow dynamics were examined using BST in the aortic root and left ventricle. Children with BAV had altered systolic flow patterns in the aortic root and higher aortic root average vorticity (25.9 [23.4-29.2] Hz vs. 17.8 [9.0-26.2] Hz, p < 0.05), vector complexity (0.17 [0.14-0.31] vs. 0.05 [0.02-0.13], p < 0.01) and rate of energy loss (7.9 [4.9-12.1] mW/m vs. 2.7 [1.2-7.4] mW/m, p = 0.01). Left ventricular average diastolic vorticity (20.9 ± 5.8 Hz vs. 11.4 ± 5.2 Hz, p < 0.01), kinetic energy (0.11 ± 0.05 J/m vs. 0.04 ± 0.02 J/m, p < 0.01), vector complexity (0.38 ± 0.1 vs. 0.23 ± 0.1, p < 0.01) and rate of energy loss (11.1 ± 4.8 mW/m vs. 2.7 ± 1.9 mW/m, p < 0.01) were higher in children with BAV. CONCLUSION: Children with BAV exhibit altered flow dynamics in the aortic root and left ventricle in the absence of significant aortic root dilation. This may represent a substrate and potential predictor for future dilation and diastolic dysfunction.


Subject(s)
Bicuspid Aortic Valve Disease , Heart Valve Diseases , Humans , Child , Bicuspid Aortic Valve Disease/complications , Aortic Valve/diagnostic imaging , Heart Valve Diseases/diagnostic imaging , Aorta , Thorax
19.
Eur Heart J ; 44(43): 4549-4562, 2023 11 14.
Article in English | MEDLINE | ID: mdl-37611071

ABSTRACT

BACKGROUND AND AIMS: Bicuspid aortic valve (BAV) is the most common congenital heart anomaly. Lifetime morbidity and whether long-term survival varies according to BAV patient-sub-groups are unknown. This study aimed to assess lifetime morbidity and long-term survival in BAV patients in the community. METHODS: The authors retrospectively identified all Olmsted County (Minnesota) residents with an echocardiographic diagnosis of BAV from 1 January 1980 to 31 December 2009, including patients with typical valvulo-aortopathy (BAV without accelerated valvulo-aortopathy or associated disorders), and those with complex valvulo-aortopathy (BAV with accelerated valvulo-aortopathy or associated disorders). RESULTS: 652 consecutive diagnosed BAV patients [median (IQR) age 37 (22-53) years; 525 (81%) adult and 127 (19%) paediatric] were followed for a median (IQR) of 19.1 (12.9-25.8) years. The total cumulative lifetime morbidity burden (from birth to age 90) was 86% (95% CI 82.5-89.7); cumulative lifetime progression to ≥ moderate aortic stenosis or regurgitation, aortic valve surgery, aortic aneurysm ≥45 mm or z-score ≥3, aorta surgery, infective endocarditis and aortic dissection was 80.3%, 68.5%, 75.4%, 27%, 6% and 1.6%, respectively. Survival of patients with typical valvulo-aortopathy [562 (86%), age 40 (28-55) years, 86% adults] was similar to age-sex-matched Minnesota population (P = .12). Conversely, survival of patients with complex valvulo-aortopathy [90 (14%), age 14 (3-26) years, 57% paediatric] was lower than expected, with a relative excess mortality risk of 2.25 (95% CI 1.21-4.19) (P = .01). CONCLUSION: The BAV condition exhibits a high lifetime morbidity burden where valvulo-aortopathy is close to unavoidable by age 90. The lifetime incidence of infective endocarditis is higher than that of aortic dissection. The most common BAV clinical presentation is the typical valvulo-aortopathy with preserved expected long-term survival, while the complex valvulo-aortopathy presentation incurs higher mortality.


Subject(s)
Aortic Dissection , Bicuspid Aortic Valve Disease , Endocarditis , Heart Valve Diseases , Adult , Humans , Child , Aged, 80 and over , Adolescent , Bicuspid Aortic Valve Disease/complications , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/abnormalities , Heart Valve Diseases/complications , Retrospective Studies , Morbidity , Endocarditis/complications
20.
BMC Cardiovasc Disord ; 23(1): 363, 2023 07 19.
Article in English | MEDLINE | ID: mdl-37468858

ABSTRACT

INTRODUCTION: Bicuspid aortic valve (BAV) patients have an increased risk to develop thoracic aortic complications. Little is known about the prevalence and severity of atherosclerosis in the BAV ascending aortic wall. This study evaluates and compares the prevalence of thoracic aortic atherosclerosis in BAV and tricuspid aortic valve (TAV) patients. METHODS: Atherosclerosis was objectified using three diagnostic modalities in two separate BAV patient cohorts (with and without an aortic dilatation). Within the first group, atherosclerosis was graded histopathologically according to the modified AHA classification scheme proposed by Virmani et al. In the second group, the calcific load of the ascending aorta and coronary arteries, coronary angiographies and cardiovascular risk factors were studied. Patients were selected from a surgical database (treated between 2006-2020), resulting in a total of 128 inclusions. RESULTS: Histopathology showed atherosclerotic lesions to be more prevalent and severe in all TAV as compared to all BAV patients (OR 1.49 (95%CI 1.14 - 1.94); p = 0.003). Computed tomography showed no significant differences in ascending aortic wall calcification between all BAV and all TAV patients, although a tendency of lower calcific load in favor of BAV was seen. Coronary calcification was higher in all TAV as compared to all BAV (OR 1.30 (95%CI 1.06 - 1.61); p = 0.014). CONCLUSION: Ascending aortic atherosclerotic plaques were histologically more pronounced in TAV as compared to the BAV patients, while CT scans revealed equal amounts of calcific depositions within the ascending aortic wall. This study confirms less atherosclerosis in the ascending aortic wall and coronary arteries of BAV patients as compared to TAV patients. These results were not affected by the presence of a thoracic aortic aneurysm.


Subject(s)
Aortic Diseases , Atherosclerosis , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Humans , Bicuspid Aortic Valve Disease/complications , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/epidemiology , Heart Valve Diseases/complications , Case-Control Studies , Aortic Valve/surgery , Aortic Diseases/diagnostic imaging , Aortic Diseases/epidemiology , Aortic Diseases/complications , Atherosclerosis/diagnostic imaging , Atherosclerosis/epidemiology
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