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Congenital valve disease

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https://www.readbyqxmd.com/read/29659801/long-term-performance-of-homografts-versus-stented-bioprosthetic-valves-in-the-pulmonary-position-in-patients-aged-10-20-years
#1
Douglas Bell, Sudesh Prabhu, Kim S Betts, Yilin Chen, Dorothy Radford, Chris Whight, Cameron Ward, Homayoun Jalali, Prem Venugopal, Nelson Alphonso
OBJECTIVES: We aimed to compare the long-term performance of pulmonary homografts and stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years. METHODS: Between January 1995 and December 2015, 188 patients aged 10-20 years undergoing pulmonary valve replacement were identified retrospectively from hospital databases in both congenital cardiac centres in Brisbane. Valve performance was evaluated using previously described standard criteria...
April 11, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29658866/mechanical-valve-replacement-for-congenital-heart-disease-complicated-by-native-pulmonary-valve-endocarditis-a-case-report-and-literature-review
#2
Qing Wang, Yan Gao, Wang Xi, Hua Shen, Jing Wang, Jian Xiao, Zhinong Wang
Congenital heart disease (CHD) is one of the most common risk factors for infective endocarditis. However, it is rare to find a CHD patient complicated by isolated pulmonary valve endocarditis. Here, we report an adult patient with congenital heart disease complicated by native pulmonary valve endocarditis who underwent a mechanical valve replacement. We also review previous literature to examine key points in the treatment of such patients.
March 7, 2018: Heart Surgery Forum
https://www.readbyqxmd.com/read/29653232/gata6-loss-of-function-mutation-contributes-to-congenital-bicuspid-aortic-valve
#3
Ying-Jia Xu, Ruo-Min Di, Qi Qiao, Xiu-Mei Li, Ri-Tai Huang, Song Xue, Xing-Yuan Liu, Juan Wang, Yi-Qing Yang
Congenital bicuspid aortic valve (BAV), the most common form of birth defect in humans, is associated with substantial morbidity and mortality. Increasing evidence demonstrates that genetic risk factors play a key role in the pathogenesis of BAV. However, BAV is a genetically heterogeneous disease and the genetic determinants underpinning BAV in an overwhelming majority of patients remain unknown. In the present study, the coding exons and flanking introns of the GATA6 gene, which encodes a zinc-finger transcription factor essential for the normal development of the aortic valves, were sequenced in 152 unrelated patients with congenital BAV...
April 10, 2018: Gene
https://www.readbyqxmd.com/read/29622096/management-of-patients-with-aortic-valve-stenosis
#4
REVIEW
Amrit Kanwar, Jeremy J Thaden, Vuyisile T Nkomo
With increased life expectancy and aging of the population, aortic stenosis is now one of the most common valvular heart diseases. Early recognition and management of aortic stenosis are of paramount importance because untreated symptomatic severe disease is universally fatal. The advent of transcather aortic valve replacement technologies provides exciting avenues of care to patients with this disease in whom traditional surgical procedures could not be performed or were associated with high risk. This review for clinicians offers an overview of aortic stenosis and updated information on the current status of various treatment strategies...
April 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29615572/the-role-of-hedgehog-signalling-in-the-formation-of-the-ventricular-septum
#5
REVIEW
Antonia Wiegering, Ulrich Rüther, Christoph Gerhardt
An incomplete septation of the ventricles in the vertebrate heart that disturbes the strict separation between the contents of the two ventricles is termed a ventricular septal defect (VSD). Together with bicuspid aortic valves, it is the most frequent congenital heart disease in humans. Until now, life-threatening VSDs are usually treated surgically. To avoid surgery and to develop an alternative therapy (e.g., a small molecule therapy), it is necessary to understand the molecular mechanisms underlying ventricular septum (VS) development...
December 12, 2017: Journal of Developmental Biology
https://www.readbyqxmd.com/read/29615331/a-novel-wall-water-system-for-cardiopulmonary-bypass-may-reduce-the-risk-of-aerosolized-infection
#6
Gregory S Matte, Thomas J Sandora, Robert J Howe, William L Regan, Gail Potter-Bynoe, James R Neal, Pedro J Del Nido
OBJECTIVE: Oxygenators for cardiopulmonary bypass require water flow for their integral heat exchanger. Heater-cooler units are nearly universally used for this requirement. Heater-cooler units pose the risk of aerosolized infection. The Centers for Disease Control and Prevention recommended discontinuing use of Stöckert 3T heater-cooler units (LivaNova PLC, London, United Kingdom) in October 2016 because of this risk. We aimed to reduce the risk of aerosolized infection posed by heater-cooler units by eliminating those devices from our operating rooms...
March 7, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29602423/valve-sparing-root-surgery-in-congenital-heart-disease-shoulda-coulda%C3%A2
#7
EDITORIAL
Joseph A Dearani
No abstract text is available yet for this article.
February 17, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29589379/results-and-factors-associated-with-adverse-outcome-after-tricuspid-valve-replacement
#8
Paweł Litwiński, Piotr Kołsut, Tadeusz Sitko, Tomasz Zieliński, Piotr Hoffman, Tomasz Hryniewiecki, Jacek Różański, Mariusz Kuśmierczyk
BACKGROUND: This retrospective analysis of patients with severe tricuspid valve disease, who underwent tricuspid valve replacement (TVR) for either tricuspid regurgitation or stenosis, represents an attempt to determine the factors that predict poor hospital and long-term survival. METHODS: The study population consisted of 86 patients, 65 female and 21 male, who underwent TVR with or without concomitant surgical procedures between 2000 and 2010 at our institution...
March 28, 2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29588148/spontaneous-chordae-tendineae-rupture-during-peripartum
#9
Feng-Han Chiu, Chih-Jen Yang, Chih-Kang Huang, Chih-Yuan Lin, Shih-Hung Tsai
Acute cardiopulmonary distress in pregnancy always carries exceptionally arduous challenge for physicians. Here we report a patient who sustained spontaneous chordae tendineae rupture complicated with severe mitral regurgitation and acute pulmonary edema during peripartum period. Probable causes of chordae tendineae rupture include mitral valve prolapse, infectious endocarditis, congenital heart disease, rheumatic heart disease, ischemic heart disease, connective tissue diseases, previous mitral valve surgery or pregnancy itself...
March 19, 2018: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29576787/management-of-cardiovascular-disorders-in-patients-with-noonan-syndrome-a-case-report
#10
Mohammad Rafie Khorgami, Maryam Moradian, Negar Omidi, Mohammad Yousef Aarabi Moghadam
The Noonan syndrome is a rare disorder, one of whose major complications is cardiovascular involvement. A wide spectrum of congenital heart diseases has been observed in this syndrome. The most common cardiac disorder is pulmonary valve stenosis, which has a progressive nature. Hypertrophic cardiomyopathy is less common, but its morbidity and mortality rates are high. We herein introduce a 12-year-old boy with the typical findings of the Noonan syndrome. His symptoms began from infancy, and there was a gradual exacerbation in his respiratory and cardiac manifestations with age...
October 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/29571348/first-surgical-melody-valve-in-valve-implantation-for-early-degeneration-in-mitral-position
#11
Maria Grandinetti, Alessandro Varrica, Alessandro Giamberti, Mario Carminati, Alessandro Frigiola
Congenital mitral valve disease is a real challenge in infants and small children. The implantation of expandable stented valves in mitral position has become one of the options of choice in consideration of their acceptable short-term expandable durability and subsequent feasibility of balloon expansion of these devices. We report the first case of a surgical Melody valve-in-valve procedure for early Melody valve degeneration in the mitral position. The result was good, and the procedure safe, rapid, and without an increased risk when a mechanical valve implantation will be necessary...
April 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29569266/a-late-presentation-of-congenital-cardiac-anomaly-accessory-chordae-from-the-left-atrium-causing-severe-mitral-regurgitation
#12
Giovanni D'Onghia, Marta Martin, Maria Teresa Mancini, Silvia Quintarelli, Adele Cozza, Fabrizio Guarracini, Roberto Bonmassari
Mitral regurgitation secondary to accessory mitral valve (MV) chordae of the left atrium is an extremely rare congenital disease. A 85-year-old female (NYHA I-II) was hospitalized for investigations. An echocardiogram showed calcification of the MV with mild stenosis and moderate regurgitation. Transesophageal three-dimensional echocardiogram revealed a band-like structure extending from the distal third of the anterior wall of the left atrium to the MV. This accessory chordae determined severe systolic regurgitation and mild mitral stenosis...
March 22, 2018: Echocardiography
https://www.readbyqxmd.com/read/29568912/clinical-study-of-prenatal-ultrasonography-combined-with-t%C3%A2-box-transcription-factor-1-as-a-biomarker-for-the-diagnosis-of-congenital-heart-disease
#13
Bingcheng Guo, Jing Xiao, Long Li, Shuanglong Wang, Lijuan Wang, Shuyong Liu
Congenital heart disease (CHD) seriously threatens fetal health. Therefore, prenatal examination to detect deformity is extremely important. The present study aimed to investigate the clinical application value of prenatal ultrasonography combined with molecular biology methods in the diagnosis of fetal CHD. A total of 1,000 pregnant women who had received fetal ultrasonography to examine fetal CHD were enrolled. Ultrasounds were performed for fetal heart examination and diagnosis, mainly on fetal heart position, size, structure and function, and heart valve morphology and function...
March 14, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29567669/gata6-regulates-aortic-valve-remodeling-and-its-haploinsufficiency-leads-to-rl-type-bicuspid-aortic-valve
#14
Lara Gharibeh, Hiba Komati, Yohan Bossé, Munir Boodhwani, Mahyar Heydarpour, Megan Fortier, Romina Hassandazeh, Janet Ngu, Patrick Mathieu, Simon Body, Mona Nemer
Background -Bicuspid aortic valve (BAV), the most common congenital heart defect affecting 1-2% of the population, is a major risk factor for premature aortic valve disease and accounts for the majority of valve replacement. The genetic basis and the mechanisms of BAV etiology and pathogenesis remain largely undefined. Methods -Cardiac structure and function was assessed in mice lacking a Gata6 allele. Human GATA6 gene variants were analyzed in 452 BAV cases from the BAV consortium and 1849 controls from the Framingham GWAS study...
March 22, 2018: Circulation
https://www.readbyqxmd.com/read/29555832/longitudinal-assessment-of-the-doppler-estimated-maximum-gradient-in-patients-with-congenital-valvar-aortic-stenosis-pre-and-post-balloon-valvuloplasty
#15
Joseph D Kuebler, Jill Shivapour, Selcen Yaroglu Kazanci, Kimberlee Gauvreau, Steven D Colan, Doff B McElhinney, David W Brown
BACKGROUND: Aortic stenosis has been reported to manifest a slow rate of progression in mild disease, with a greater likelihood of progression in patients with moderate-severe disease. The natural history of the Doppler-estimated maximum gradient (DEMG) in patients after balloon aortic valvuloplasty (BAVP) has not previously been studied on a large scale. METHODS AND RESULTS: A retrospective review was performed of 360 patients from 1984 to 2012 with aortic stenosis, providing a total of 2059 echocardiograms both before and after BAVP...
March 2018: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29548280/comprehensive-microrna-profiling-reveals-potential-augmentation-of-the-il1-pathway-in-rheumatic-heart-valve-disease
#16
Qiyu Lu, Yi Sun, Yuyin Duan, Bin Li, Jianming Xia, Songhua Yu, Guimin Zhang
BACKGROUND: Valvular heart disease is a leading cause of cardiovascular mortality, especially in China. More than a half of valvular heart diseases are caused by acute rheumatic fever. microRNA is involved in many physiological and pathological processes. However, the miRNA profile of the rheumatic valvular heart disease is unknown. This research is to discuss microRNAs and their target gene pathways involved in rheumatic heart valve disease. METHODS: Serum miRNA from one healthy individual and four rheumatic heart disease patients were sequenced...
March 16, 2018: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29547941/re-repair-of-the-failed-mitral-valve-insights-into-aetiology-and-surgical-management
#17
Gaby Aphram, Laurent De Kerchove, Stefano Mastrobuoni, Emiliano Navarra, Silvia Solari, Saadallah Tamer, Jerome Baert, Alain Poncelet, Jean Rubay, Parla Astarci, Philippe Noirhomme, Gebrine El Khoury
OBJECTIVES: Mitral valve (MV) repair is the gold standard for treatment of degenerative mitral regurgitation. A variety of surgical techniques allow surgeons to achieve a high rate of MV repair even with MV diseases of other aetiologies. However, a certain number of repairs fail over time. The aim of this study was to review our single-centre experience of MV re-repair and analyse the mode of repair failure, re-repair safety and efficiency in relation to the initial aetiology. METHODS: Between 1997 and 2015, 91 patients underwent redo MV re-repair...
March 14, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29546472/transcatheter-pulmonary-valve-replacement-current-state-of-art
#18
REVIEW
Wail Alkashkari, Amani Alsubei, Ziyad M Hijazi
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction...
March 15, 2018: Current Cardiology Reports
https://www.readbyqxmd.com/read/29543640/anesthesia-for-percutaneous-pulmonary-valve-implantation-a-case-series
#19
Rafael Arboleda Salazar, Jane Heggie, Piotr Wolski, Eric Horlick, Mark Osten, Massimiliano Meineri
BACKGROUND: Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center...
March 14, 2018: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/29538649/slit-robo-signalling-in-heart-development
#20
Juanjuan Zhao, Mathilda T M Mommersteeg
The Slit ligands and their Robo receptors are well-known for their roles during axon guidance in the central nervous system, but are still relatively unknown in the cardiac field. However, data from different animal models suggest a broad involvement of the pathway in many aspects of heart development, from cardiac cell migration and alignment, lumen formation, chamber formation, to the formation of the ventricular septum, semilunar and atrioventricular valves, caval veins and pericardium. Absence of one or more of the genes in the pathway results in defects ranging from bicuspid aortic valves to ventricular septal defects and abnormal venous connections to the heart...
March 10, 2018: Cardiovascular Research
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