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Congenital aortic stenosis

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https://www.readbyqxmd.com/read/29202750/assessment-of-tetralogy-of-fallot-associated-congenital-extracardiac-vascular-anomalies-in-pediatric-patients-using-low-dose-dual-source-computed-tomography
#1
Bi-Yue Hu, Ke Shi, Yu-Ping Deng, Kai-Yue Diao, Hua-Yan Xu, Rui Li, Zhi-Gang Yang, Ying-Kun Guo
BACKGROUND: To investigate the diagnostic value of dual-source computed tomography (DSCT) in the evaluation of tetralogy of Fallot (TOF)-associated extracardiac vascular abnormalities in pediatric patients compared with transthoracic echocardiography (TTE). METHODS: One hundred and twenty-three pediatric patients diagnosed with TOF were included in this retrospective study. All patients underwent DSCT and TTE preoperatively. All associated extracardiac vascular abnormalities and their percentages were recorded...
December 4, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29199861/stenting-the-complex-patent-ductus-arteriosus-in-tetralogy-of-fallot-with-pulmonary-atresia-challenges-and-outcomes
#2
Rizwan Rehman, Mood Che Marhisham, Mazeni Alwi
Patent ductus arteriosus (PDA) stenting has gained acceptance for palliation in cyanotic congenital heart disease. The PDA in tetralogy of Fallot with pulmonary atresia (ToF-PA) arises, in the left aortic arch, from underneath the arch and connects to the proximal left pulmonary artery, often resulting in stenosis. The PDA is usually elongated and tortuous, making stent implantation challenging. Shorter duration of palliation, aggravation of branch pulmonary artery stenosis resulting in poor growth and difficulty at surgery makes ductal stenting controversial...
December 4, 2017: Future Cardiology
https://www.readbyqxmd.com/read/29191731/characteristic-morphologies-of-the-bicuspid-aortic-valve-in-patients-with-genetic-syndromes
#3
Talha Niaz, Joseph T Poterucha, Timothy M Olson, Jonathan N Johnson, Cecilia Craviari, Thomas Nienaber, Jared Palfreeman, Frank Cetta, Donald J Hagler
BACKGROUND: In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. METHODS: The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016...
November 28, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29188316/impact-of-bicuspid-aortic-valve-morphology-on-aortic-valve-disease-and-aortic-dilation-in-pediatric-patients
#4
Rebekah M Ward, Jordan M Marsh, Jeffrey M Gossett, Mallikarjuna R Rettiganti, R Thomas Collins
Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is associated with aortic stenosis and insufficiency, and aortic dilation in adult groups, but data in pediatric groups are limited. We sought to assess the impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients. We performed a retrospective review of all echocardiograms in patients with isolated BAV who were followed at our institution from July 2002 to July 2012. BAV morphology, aortic valve stenosis and/or insufficiency, and aortic dimensions were measured manually...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29174590/double-outlet-right-ventricle-with-subpulmonary-ventricular-septal-defect-taussig-bing-anomaly-and-other-complex-congenital-cardiac-malformations-in-an-american-quarter-horse-foal
#5
Rebecca Kohnken, Karsten Schober, Jennifer Godman, Alison Gardner, Tiffany Jenkins, Eric Schroeder, Peter Baker, Laura Dunbar
A 4-week-old American Quarter Horse colt presented with a recent history of diarrhea and decreased activity level. On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. While in the hospital, the clinical condition of the foal warranted further imaging to determine the cause and extent of cardiac disease. A variety of congenital cardiac malformations were identified during echocardiographic examination and autopsy, including a double outlet right ventricle and a subpulmonary interventricular septal defect (Taussig-Bing anomaly), ventricular inversion with atrioventricular discordance, tricuspid valve atresia, a septum primum interatrial septal defect, mitral valve dysplasia with a cleft in the septal mitral valve cusp, aortic, and subaortic stenosis, tubular hypoplasia of the ascending aorta and the aortic arch, a patent ductus arteriosus, an aberrant circumflex coronary artery, and aberrant left and right subclavian arteries...
November 21, 2017: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/29157538/mast-cells-in-calcific-aortic-stenosis
#6
Ivo Šteiner, Václav Stejskal, Pavel Žáček
In developed countries, calcific aortic stenosis (CAS) has become the most common acquired valvular disease and reason for aortic valve replacement. It is considered a form of atherosclerosis and, like the latter, of inflammatory origin, with presence in the calcified aortic valves of blood vessels, lymphatics, lymphocytes, plasma cells, histiocytes, and sometimes also of metaplastic bone tissue. This study is aimed at examining the presence of CD117 - positive mast cells in CAS. In 56 examined calcified aortic valves excised by cardiac surgery, mast cells were constantly present as a part of the polymorphous cellular infiltrate; in individual cases, their numbers were 1-90 (median 24)...
July 23, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29156017/early-and-late-outcomes-after-surgical-repair-of-congenital-supravalvular-aortic-stenosis-a-european-congenital-heart-surgeons-association-multicentric-study
#7
Massimo A Padalino, Anna Chiara Frigo, Marina Comisso, Martin Kostolny, Ikenna Omeje, Christian Schreiber, Jelena Pabst von Ohain, Julie Cleuziou, David J Barron, Bart Meyns, Viktor Hraska, Bohdan Maruszewski, Michal Kozlowski, Luca A Vricella, Narutoshi Hibino, Sarah Collica, Hakan Berggren, Mats Synnergren, Stojan Lazarov, David Kalfa, Emile Bacha, Christian Pizarro, Mark Hazekamp, Vlado Sojak, Jeffrey P Jacobs, Matej Nosal, Jose Fragata, Sertac Cicek, George E Sarris, Panayotis Zografos, Vladimiro L Vida, Giovanni Stellin
OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events...
October 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29128868/association-of-lpa-variants-with-aortic-stenosis-a-large-scale-study-using-diagnostic-and-procedural-codes-from-electronic-health-records
#8
Hao Yu Chen, Line Dufresne, Hannah Burr, Athithan Ambikkumar, Niko Yasui, Kevin Luk, Dilrini K Ranatunga, Rachel A Whitmer, Mark Lathrop, James C Engert, George Thanassoulis
Importance: Elevated lipoprotein(a) levels are a risk factor for aortic stenosis (AS). However, a large-scale replication of associations between LPA variants and AS, their interactions with risk factors, and the effect of multiple risk alleles is not well established. Objective: To replicate the association between LPA variants with AS and identify subgroups who are at higher risk of developing AS. Design, Setting, and Participants: This case-control study of AS included 44 703 individuals (3469 cases) 55 years or older who were enrolled in the Genetic Epidemiology Research on Aging cohort and who were members of the Kaiser Permanente Northern California health care delivery system...
November 12, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/29121837/triple-outlet-right-ventricle-with-duplication-of-the-aortic-root-and-intrapericardial-ascending-aorta
#9
Ashish Katewa, Balswaroop Sahu, Vishal Jain, Robert H Anderson
We present a case of a highly unusual congenital cardiac malformation, namely, triple outlet right ventricle with duplication of the aortic root and the intrapericardial component of the ascending aorta. A girl, aged five, presented with complaints of cyanosis and effort intolerance and was diagnosed with double-outlet right ventricle and subpulmonary infundibular stenosis. Intraoperatively, we noted that the aortic root was guarded by two separate aortic valves, oriented anteroposteriorly relative to each other, and separated within the right ventricle by a muscle bar...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29098290/fluid-structure-interaction-models-of-bicuspid-aortic-valves-the-effects-of-non-fused-cusp-angles
#10
Karin Lavon, Rotem Halevi, Gil Marom, Sagit Ben Zekry, Ashraf Hamdan, Hans Joachim Schäfers, Ehud Raanani, Rami Haj-Ali
Bicuspid aortic valve (BAV) is the most common type of congenital heart disease, occurring in 0.5-2% of the population, where the valve has only two rather than the three normal cusps. Valvular pathologies, such as aortic regurgitation and aortic stenosis, are associated with BAVs, thereby increasing the need for a better understanding of BAV kinematics and geometrical characteristics. The aim of this study is to investigate the influence of the non-fused cusp (NFC) angle in BAV type-1 configuration on the valve's structural and hemodynamic performance...
November 2, 2017: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/29082593/a-rare-mechanism-of-aortic-regurgitation-in-a-young-patient
#11
Ahmet Guner, Sabahattin Gunduz, Cagatay Onal, Taylan Akgun, Emrah Bayam, Ozkan Candan, Mehmet Ozkan
A 19-year-old male patient was admitted to our institute with dyspnea. His medical history had no rheumatic fever or infective endocarditis. Physical examination revealed a diastolic murmur over the aortic area, rales of bilateral lungs. Bedside transthoracic echocardiography (TTE) revealed a severe aortic regurgitation (AR) without aortic valve stenosis and a moderately dilated left ventricle accompanied by an ejection fraction of 55%. The aortic valve could not be clearly demonstrated as either bicuspid or tricuspid...
October 29, 2017: Echocardiography
https://www.readbyqxmd.com/read/29075526/supravalvar-mitral-ring-with-a-parachute-mitral-valve-and-subcoarctation-of-the-aorta-in-a-child-with-hemodynamically-significant-vsd-a-study-of-the-morphology-echocardiographic-diagnostics-and-surgical-therapy
#12
Wojciech Mądry, Maciej A Karolczak, Krzysztof Grabowski
The authors present a case of echocardiographic diagnosis of supravalvar mitral ring (a fibromembranous structure that arose from the atrial surface of the mitral leaflets) in a child with a parachute mitral valve, a ventricular septal defect, and mild narrowing of the aortic isthmus. The supravalvar mitral stenosis is a typical but very infrequently detected element of the complex of anatomical abnormalities located within the left heart and the proximal aorta, called the Shone's complex (syndrome). Diagnosing an additional, hemodynamically significant anatomic defect during echocardiography was possible thanks to the detection of marked mobility limitation of the ring-adjacent part of the mitral valve mural leaflet as well as of an atypical image of turbulence occurring during the inflow from the left atrium to the left ventricle...
September 2017: Journal of Ultrasonography
https://www.readbyqxmd.com/read/29024719/calcification-or-not-this-is-the-question-a-one-year-study-of-bovine-pericardial-vascular-patches-cardiocel%C3%A2-in-minipigs
#13
Aida Salameh, Wiebke Greimann, David Vondrys, Martin Kostelka
Objectives A main problem with bioprosthesis used for surgical correction of congenital cardiac malformation is their tendency to shrink and to calcify. Recently, a new material i.e. de-cellularised bovine pericardium (CardioCel(®)) was introduced in clinics. It was proposed that this new patch material should not calcify and should be particularly suitable for the correction of vascular defects in inborn cardiac diseases. The aim of our chronic minipig study was to evaluate the performance of CardioCel(®) patches implanted in aortic and pulmonary artery position, respectively...
October 9, 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28983175/isolated-supravalvular-aortic-stenosis-with-left-ventricular-diverticulum-and-cleft-mitral-valve-surgical-repair-in-adulthood
#14
U M Nagamalesh, Ravi Shankar Shetty, Ravi Narayan, Anil Kumar, Ravi Naik, Sadath Uzma, K C Karthik Naidu
Supravalvular aortic stenosis is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or can be associated with Williams syndrome. We are reporting a very rare presentation of supravalvular aortic stenosis with associated left ventricular diverticulum and cleft mitral valve. Repair consisted of resection of the ascending aorta, patch augmentation of the aortic root, and mitral valve repair...
October 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28967590/a-case-of-intrathoracic-carotid-bifurcation-without-klippel-feil-syndrome
#15
Ryo Itabashi, Yukako Yazawa, Yuya Shigehatake, Eisuke Furui
BACKGROUND: Intrathoracic carotid bifurcation is a rare vascular anomaly, with only 8 cases reported. This vascular anomaly was recently correlated with Klippel-Feil syndrome, a rare congenital disorder involving fusion of the cervical vertebrae. METHODS: A 70-year-old deaf mute man was admitted to our department because of right hemiparesis and right sensory disturbance. He displayed no abnormalities associated with Klippel-Feil syndrome. Diffusion-weighted imaging revealed acute multiple infarcts in bilateral hemispheres...
September 26, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28967205/surgical-strategies-and-outcomes-of-congenital-supravalvular-aortic-stenosis
#16
Hongli Liu, Botao Gao, Qi Sun, Xinwei Du, Yanjun Pan, Zhongqun Zhu, Xiaomin He, Jinghao Zheng
BACKGROUND: Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus upon the optimal approach. This study reviewed our institutional experience in the management of SVAS. METHODS: Ninety patients undergoing surgery for SVAS were identified between 2009 and 2016. Based on surgical techniques, patients were divided into three groups: McGoon repair (n = 63), Doty repair (n = 24), and Brom repair (n = 3)...
October 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28966455/combined-mitral-and-aortic-valve-stenosis-caused-by-two-different-etiologies-rheumatic-and-congenital
#17
William C Roberts, Joshua K Dodderer
Described herein are five patients who had double left-sided cardiac valve replacement for mitral and aortic valve stenosis resulting from two different etiologies: rheumatic heart disease, the cause of the mitral stenosis, and congenital heart disease (bicuspid valve), the underlying cause of the aortic stenosis.
October 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28964424/hybrid-balloon-valvuloplasty-for-the-treatment-of-severe-congenital-aortic-valve-stenosis-in-infants
#18
Wen-Bin Ou-Yang, Shou-Jun Li, Yong-Quan Xie, Sheng-Shou Hu, Shou-Zheng Wang, Feng-Wen Zhang, Gai-Li Guo, Yao Liu, Kun-Jing Pang, Xiang-Bin Pan
BACKGROUND: Surgical or percutaneous interventional treatment of severe congenital aortic valve stenosis (CAS) in early infancy remains challenging. This single-center, retrospective study analyzed midterm outcomes of a hybrid balloon valvuloplasty procedure through the ascending aorta by way of median sternotomy, including cases with improved technique. METHODS: Included were 45 consecutive infants (aged <90 days) with CAS and selected for biventricular repair who underwent hybrid balloon valvuloplasty in a hybrid or ordinary operating room from October 2010 to March 2016...
September 27, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28947226/outcomes-of-thoracic-endovascular-aortic-repair-in-adult-coarctation-patients
#19
Salim Lala, Salvatore T Scali, Robert J Feezor, Satish Chandrekashar, Kristina A Giles, Javairiah Fatima, Scott A Berceli, Martin R Back, Thomas S Huber, Thomas M Beaver, Adam W Beck
BACKGROUND: Aortic coarctation (AC) is most commonly identified in pediatric patients; however, adults can present with late sequelae of untreated coarctation or complications of prior open repair. To date, there are limited data about the role of thoracic endovascular aortic repair (TEVAR) in this group of patients. The purpose of this analysis was to describe our experience with management of adult coarctation patients using TEVAR. METHODS: All TEVAR patients treated for primary coarctation or late sequelae of previous open repair (eg, pseudoaneurysm, recurrent coarctation or anastomotic stenosis related to index open coarctation repair) were reviewed...
September 22, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28940406/congenital-aortic-valve-repair-using-cormatrix-%C3%A2-a-histologic-evaluation
#20
Michael Hofmann, Martin O Schmiady, Barbara E Burkhardt, Hitendu H Dave, Michael Hübler, Oliver Kretschmar, Peter K Bode
BACKGROUND: The reconstruction of heart valves provides substantial benefits, particularly in the pediatric population. We present our experience using decellularized extracellular matrix (dECM, CorMatrix(®) ) for aortic valve procedures. METHODS: We retrospectively reviewed the case histories of 6 patients (aged from 2 months - 14 years) who underwent surgery for severe aortic valve stenosis (n = 4) or regurgitation (n = 2). Aortic valve repair was performed on all patients using dECM as a leaflet replacement or leaflet extension...
September 22, 2017: Xenotransplantation
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