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Bicuspid aortic valve

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https://www.readbyqxmd.com/read/29346529/echocardiographic-criteria-to-detect-unicuspid-aortic-valve-morphology
#1
Sebastian Ewen, Irem Karliova, Petra Weber, Stephan H Schirmer, Hashim Abdul-Khaliq, Jakob Schöpe, Felix Mahfoud, Hans-Joachim Schäfers
Aims: Unicuspid aortic valve (UAV) is a rare congenital malformation associated with severe aortic stenosis or regurgitation. This study aimed to systematically determine echocardiographic criteria to identify UAV. Methods and results: All patients underwent a preoperative baseline examination, including echocardiography. A total of 69 patients with intraoperatively confirmed UAV underwent an aortic valve repair procedure between August 2001 and May 2011. To compare the findings of UAV cases with those of other valve morphologies, we examined 99 consecutive patients with a bicuspid aortic valve (BAV) and 103 consecutive patients with a tricuspid aortic valve (TAV) undergoing isolated aortic valve surgery before May 2016...
January 15, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29340729/pulmonary-valve-morphology-in-patients-with-bicuspid-aortic-valves
#2
Wilke M C Koenraadt, Margot M Bartelings, Adriana C Gittenberger-de Groot, Regina Bökenkamp, Marco C DeRuiter, Martin J Schalij, Monique R M Jongbloed
The aortic and pulmonary valve share a common developmental origin from the embryonic arterial trunk. Bicuspid aortic valve is the most common congenital anomaly and can occur isolated as well as in association with other congenital heart disease (CHD). Data on pulmonary valve morphology in these cases are scarce. In this study, we aimed to determine pulmonary valve morphology in hearts with BAV associated with CHD. In 83 post-mortem heart specimens with BAV and associated CHD, pulmonary valve morphology was studied and related to BAV morphology...
January 16, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29339711/unusual-combination-of-mitral-valve-prolapse-bicuspid-aortic-valve-and-ventricular-septal-defect-restricted-by-the-tricuspid-septal-leaflet
#3
Umut Kocabaş, Esra Kaya, Cahide Soydaş Çınar
No abstract text is available yet for this article.
January 2018: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/29339678/endarteritis-of-coarctation-of-the-aorta-diagnosed-with-pet-ct
#4
Ümit Yaşar Sinan, Burcu Dirlik Serim, Rukiye Yıldırım, Özge Çetinarslan, Mehmet Serdar Küçükoğlu
Infective endocarditis (IE) is an infectious disease that affects the endothelium of the large intrathoracic vessels, heart valves, and intra-cardiac foreign body material. A 20-year-old woman was admitted to the cardiology department with complaints of fever and palpitations. Transthoracic echocardiography revealed a bicuspid aortic valve, aortic root enlargement, and aortic coarctation. Transesophageal echocardiography revealed a bicuspid aortic valve, but there was no vegetation. Methicillin-sensitive Staphylococcus aureus was identified on a blood culture...
January 2018: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29339108/phenotype-in-girls-and-women-with-turner-syndrome-association-between-dysmorphic-features-karyotype-and-cardio-aortic-malformations
#5
Iris Noordman, Anthonie Duijnhouwer, Livia Kapusta, Marlies Kempers, Nel Roeleveld, Michiel Schokking, Dominique Smeets, Kim Freriks, Henri Timmers, Janiëlle van Alfen-van der Velden
INTRODUCTION: Turner syndrome (TS) is a genetic disorder characterized by the (partial) absence or a structural aberration of the second sex chromosome and is associated with a variety of phenotypes with specific physical features and cardio-aortic malformations. The objective of this study was to gain a better insight into the differences in dysmorphic features between girls and women with TS and to explore the association between these features, karyotype and cardio-aortic malformations...
January 12, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29338868/survival-and-reoperation-pattern-after-20%C3%A2-years-of-experience-with-aortic-valve-sparing-root-replacement-in-patients-with-tricuspid-and-bicuspid-valves
#6
Stefan Klotz, Sina Stock, Hans-Hinrich Sievers, Michael Diwoky, Michael Petersen, Ulrich Stierle, Doreen Richardt
OBJECTIVE: Remodeling or reimplantation are established operative techniques of aortic valve-sparing root replacement. Long-term follow-up is necessary comparing tricuspid and bicuspid aortic valves. METHODS: A total of 315 patients (tricuspid, n = 225, bicuspid, n = 89, quadricuspid, n = 1; remodeling, n = 101, reimplantation, n = 214) were evaluated. Mean follow-up was 10.1 ± 5.6 and 6.4 ± 4.2 years for the remodeling and reimplantation group, respectively...
December 13, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29332374/transcatheter-aortic-valve-replacement-in-bicuspid-aortic-valve-stenosis-where-do-we-stand
#7
Sung-Han Yoon, Rahul Sharma, Tarun Chakravarty, Masaki Miyasaka, Tomoki Ochiai, Takahiro Nomura, Norman Gellada, Shadi Nemanpour, Mamoo Nakamura, Wen Chen, Raj Makkar
Bicuspid aortic valve is the most common congenital cardiac defect in adults, and symptom typically develops in adulthood. In the majority of cases, bicuspid aortic valve disease progress with ages and surgical aortic valve replacement is performed with excellent operative outcomes. However, with the relatively slow progression of disease, surgical aortic valve replacement is required in elderly patients but the surgical risk often deemed extremely high due to old age and multiple comorbidities. Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for intermediate- and high-risk patients with symptomatic severe aortic valve stenosis (AS)...
January 11, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29329770/yield-of-family-screening-in-patients-with-isolated-bicuspid-aortic-valve-in-a-general-hospital
#8
Luc Cozijnsen, Hester J van der Zaag-Loonen, Richard L Braam, Mirjam Bakker-de Boo, Jan G Post, Berto J Bouma, Barbara J M Mulder
AIM: To determine the prevalence of unidentified bicuspid aortic valve (BAV) or aortic dilatation (>40mm) in first degree relatives (FDR) of patients with isolated BAV in a general hospital. METHODS AND RESULTS: Patients with isolated BAV received information advising cardiac screening of their FDR. Referred and screened were 134 FDR of 54 adult index patients with isolated BAV (median 2 per index patient). FDR's mean age was 49years (range 16-83years) and 41% were male...
December 22, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29325903/gata4-loss-of-function-mutation-and-the-congenitally-bicuspid-aortic-valve
#9
Ruo-Gu Li, Ying-Jia Xu, Juan Wang, Xing-Yuan Liu, Fang Yuan, Ri-Tai Huang, Song Xue, Li Li, Hua Liu, Yan-Jie Li, Xin-Kai Qu, Hong-Yu Shi, Min Zhang, Xing-Biao Qiu, Yi-Qing Yang
Aggregating evidence suggests that genetic determinants play a pivotal role in the pathogenesis of the congenitally bicuspid aortic valve (BAV). BAV is of pronounced genetic heterogeneity, and the genetic components underlying BAV in an overwhelming majority of patients remain elusive. In the current study, the whole coding exons and adjacent introns, as well as 5' and 3' untranslated regions of the GATA4 gene, which codes for a zinc-finger transcription factor crucial for the normal development of the aortic valve, were screened by direct sequencing in 150 index patients with congenital BAV...
November 23, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29322698/timing-and-indications-for-aortic-valve-surgery-in-korean-bicuspid-aortic-valve-patients
#10
EDITORIAL
Chi Young Shim, Geu Ru Hong
No abstract text is available yet for this article.
January 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29317805/compression-of-the-right-coronary-artery-by-an-aortic-pseudoaneurysm-after-infective-endocarditis-an-unusual-case-of-myocardial-ischemia
#11
Juan Lacalzada-Almeida, Alejandro De la Rosa-Hernández, María Manuela Izquierdo-Gómez, Javier García-Niebla, Iván Hernández-Betancor, Juan Alfonso Bonilla-Arjona, Antonio Barragán-Acea, Ignacio Laynez-Cerdeña
A 61-year-old male with a prosthetic St Jude aortic valve size 24 presented with heart failure symptoms and minimal-effort angina. Eleven months earlier, the patient had undergone cardiac surgery because of an aortic root dilatation and bicuspid aortic valve with severe regurgitation secondary to infectious endocarditis by Coxiela burnetii and coronary artery disease in the left circumflex coronary artery. Then, a prosthesis valve and a saphenous bypass graft to the left circumflex coronary artery were placed...
2018: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/29315371/computed-tomography-characteristics-of-the-aortic-valve-and-the-geometry-of-sapien-3-transcatheter-heart-valve-in-patients-with-bicuspid-aortic-valve-disease
#12
Hiroyuki Kawamori, Sung-Han Yoon, Tarun Chakravarty, Yoshio Maeno, Mohammad Kashif, Sharjeel Israr, Yigal Abramowitz, Geeteshwar Mangat, Masaki Miyasaka, Tanya Rami, Yoshio Kazuno, Nobuyuki Takahashi, Hasan Jilaihawi, Mamoo Nakamura, Wen Cheng, John Friedman, Daniel Berman, Rahul Sharma, Raj R Makkar
Aims: We assessed the geometry of transcatheter heart valve (THV) and valve function associated with SAPIEN 3 implantation in patients with bicuspid aortic valve (BAV) stenosis. Methods and results: We included 280 consecutive patients who had a contrast computed tomography (CT) before and after transcatheter aortic valve implantation (TAVI) in our institution. Each THV was assessed by CT at five cross-sectional levels: inflow, annulus, mid, sinus, and outflow. The geometry of THV was assessed for eccentricity (1 - minimum diameter/maximum diameter) and expansion (CT derived external valve area/nominal external valve area)...
January 5, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29305187/cardiac-arrest-and-ventricular-arrhythmia-in-adults-with-ebstein-anomaly-and-left-ventricular-non-compaction
#13
Magdalena Kumor, Magdalena Lipczyńska, Elzbieta Katarzyna Biernacka, Anna Klisiewicz, Anna Wójcik, Marek Konka, Katarzyna Kożuch, Piotr Szymański, Piotr Hoffman
BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features)...
January 2, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29302949/simultaneous-transcatheter-intervention-for-coarctation-of-the-aorta-and-bicuspid-aortic-valve
#14
Yukiko Mizutani, Norio Tada, Takahiko Masuda, Masaki Hata
Coarctation of the aorta (CoA) is a relatively common congenital heart anomaly, and bicuspid aortic valve (BAV) is a common congenital heart disease that coexists with CoA. In larger children and adults with CoA, transcatheter intervention has gained acceptance, but for surgical high-risk patients with aortic stenosis, the use of transcatheter aortic valve implantation (TAVI) has been established. Recently, although favorable data have been reported for TAVI when treating BAV, simultaneous transcatheter intervention for CoA and BAV will prove to be a challenge because of the unique anatomy involved requires multiple procedural steps and also has problems of site access...
July 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/29302462/valve-sparing-aortic-root-replacement-midterm-outcomes-and-quality-of-life
#15
Abdel-Kémal Bori Bata, Nicolas D'Ostrevy, Bruno Pereira, Etienne Geoffroy, Nicolas Dauphin, Vedat Eljezi, Kasra Azarnoush, Lucie Ulman, Lionel Camilleri
Background: This study evaluated the midterm outcomes, the valve related events and quality of life of patients treated by valve-sparing aortic root replacement (VSRR). Methods: From January 2003 to December 2014, 88 consecutive patients diagnosed with an aortic root aneurysm or ascending aortic aneurysms underwent VSRR surgery. The mean age was 55±14 (range 19-77) years. A total of 84% of the patients were male; 89% were in New York Heart Association functional class I or II, 55% had aortic regurgitation (AR) grade III or IV...
December 2017: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/29290898/first-in-man-implantation-of-the-retrievable-and-repositionable-venusa-plus-valve
#16
Xian-Bao Liu, Yu-Xin He, Chun-Hui Liu, Li-Han Wang, Feng Gao, Lei Yu, Ai-Qiang Dong, Min-Jian Kong, Ji-Fang Chen, Yong Xu, Qi-Jing Zhou, Min Yan, Jian-An Wang
BACKGROUND: No retrievable and repositionable second generation transcatheter aortic valve is available in China. Here, we report the first-in-man implantation of the retrievable and repositionable VenusA-Plus valve. METHODS: A 76-year-old patient with symptomatic severe aortic stenosis and high surgical risk (STS 13.8%) was recommended for transcatheter aortic valve replacement (TAVR) by heart valve team. Type 0 bicuspid aortic valve with asymmetric calcification was identified by dual source computed tomography, and the unfavorable anatomies increased the possibility of malposition and paravalvular leakage during TAVR...
2018: World Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29286269/landmark-lecture-perloff-lecture-tribute-to-professor-joseph-kayle-perloff-and-lessons-learned-from-him-aortopathy-in-adults-with-chd
#17
Koichiro Niwa
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery...
December 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29281610/aortic-replacement-in-cardiac-surgery
#18
REVIEW
Eric E Roselli
The number of aorta procedures performed annually in the United States has grown substantially during the past decade. Cleveland Clinic is a leader in research on the risk of aortic dissection in patients with a bicuspid or tricuspid aortic valve and associated aneurysm, which has led to changes in the recommendations of when to operate. Safety and efficacy data support more proactive treatment for most patients with thoracic aortic aneurysm and/or dissection with a growing emphasis on the need to provide life-long care to patients with aortic conditions...
December 2017: Cleveland Clinic Journal of Medicine
https://www.readbyqxmd.com/read/29276909/-management-of-patients-with-isolated-aortic-stenosis-considering-negative-prognostic-factors
#19
I G Nikitin, G E Gendlin, E I Emelina, A I Kovaleva
BACKGROUND: Aortic stenosis (AS) is the most common valve defect in developed countries; its prevalence increases with age of the population. The most frequent cause for isolated AS in adults is calcification of normal tricuspid or congenital bicuspid aortic valve (AV). The only effective treatment of AS is aortic valve replacement (AVR). Major negative prognostic factors include low LV ejection fraction (EF), age older than 70, female gender, and comorbidities such as renal dysfunction, chronic obstructive pulmonary disease, and neural disorders...
2017: Kardiologiia
https://www.readbyqxmd.com/read/29273600/deficiency-of-natriuretic-peptide-receptor-2-promotes-bicuspid-aortic-valves-aortic-valve-disease-left-ventricular-dysfunction-and-ascending-aortic-dilatations-in-mice
#20
Mark C Blaser, Kuiru Wei, Rachel L Adams, Yu-Qing Zhou, Laura-Lee Caruso, Zahra Mirzaei, Alan Lam, Richard K Tam, Hangjun Zhang, Scott P Heximer, Mark Henkelman, Craig A Simmons
Rationale: Aortic valve disease is a cell-mediated process without effective pharmacotherapy. C-type natriuretic peptide (CNP) inhibits myofibrogenesis and osteogenesis of cultured valve interstitial cells (VICs), and is downregulated in stenotic aortic valves. However, it is unknown whether CNP signaling regulates aortic valve health in vivo. Objective: To determine whether a deficient CNP signaling axis in mice causes accelerated progression of aortic valve disease. Methods and Results: In cultured porcine VICs, CNP inhibited pathological differentiation via the guanylate cyclase natriuretic peptide receptor 2 (NPR2) and not the G-protein-coupled clearance receptor NPR3...
December 22, 2017: Circulation Research
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