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Aortic valvar stenosi

Michelle Carr, Stephanie Curtis, Jan Marek
Congenital obstruction of the left ventricular outflow tract remains a significant problem and multilevel obstruction can often coexist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation...
June 2018: Echo Research and Practice
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
Michelle Carr, Stephanie Curtis, Jan Marek
Congenital obstruction of the left ventricular outflow tract remains a significant problem and multi-level obstruction can often co-exist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation...
March 16, 2018: Echo Research and Practice
Loreta Casquel De Tomasi, Dijon Henrique Salomé Campos, Paula Grippa Sant'Ana, Katashi Okoshi, Carlos Roberto Padovani, Gilson Masahiro Murata, Son Nguyen, Stephen C Kolwicz, Antonio Carlos Cicogna
Pathological cardiac hypertrophy leads to derangements in lipid metabolism that may contribute to the development of cardiac dysfunction. Since previous studies, using high saturated fat diets, have yielded inconclusive results, we investigated whether provision of a high-unsaturated fatty acid (HUFA) diet was sufficient to restore impaired lipid metabolism and normalize diastolic dysfunction in the pathologically hypertrophied heart. Male, Wistar rats were subjected to supra-valvar aortic stenosis (SVAS) or sham surgery...
2018: PloS One
A Crucean, A Alqahtani, D J Barron, W J Brawn, R V Richardson, J O'Sullivan, R H Anderson, D J Henderson, B Chaudhry
BACKGROUND: Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the mitral and aortic valves. Despite many studies, the causes of HLHS remain unclear and there are conflicting views regarding the role of flow, valvar or myocardial abnormalities in its pathogenesis, all of which were proposed prior to the description of the second heart field. Our aim was to re-evaluate the patterns of malformation in HLHS in relation to recognised cardiac progenitor populations, with a view to providing aetiologically useful sub-groupings for genomic studies...
August 10, 2017: Orphanet Journal of Rare Diseases
Merja Kallio, Otto Rahkonen, Ilkka Mattila, Jaana Pihkala
OBJECTIVE: To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. DESIGN: Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000-2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient  ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR...
October 2017: Scandinavian Cardiovascular Journal: SCJ
Thiago Marinho Florentino, David Le Bihan, Alexandre Antonio Cunha Abizaid, Alexandre Vianna Cedro, Amably Pessoa Corrêa, Alexandre Roginski Mendes Dos Santos, Alexandre Costa Souza, Tiago Costa Bignoto, José Eduardo Moraes Rego Sousa, Amanda Guerra de Moraes Rego Sousa
Background: Mitral valve regurgitation (MR), present in up to 74% of the patients with severe aortic stenosis (AS), can be a negative prognostic factor when moderate or severe. The outcome of MR after percutaneous transcatheter aortic valve implantation (TAVI) and predictors associated with that outcome have not been well established in the literature. Objective: To assess the outcome of primary MR in patients submitted to TAVI and to identify associated factors...
July 10, 2017: Arquivos Brasileiros de Cardiologia
Nikunj Vaidhya, Amit Mishra, Sumbul Siddiqui, Harshil Joshi, Hardik Patel
Ventricular septal defect (VSD) with valvar pulmonary stenosis (PS) is a combination of cardiac defects for which treatment by means of percutaneous catheter-directed intervention is sometimes considered. Septal occluder device embolization is a rare but potentially dreadful complication. Adequate precautions are of great importance, as operator-related and anatomical factors can contribute to the risk of device embolization. In this report, we present a case of a five-year-old patient with a perimembranous VSD and valvar PS with infundibular muscle hypertrophy...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
Viktor Hraška
Neonates with critical aortic stenosis represent a challenging group of patients with severe obstruction at a valvar level and with symptoms of heart failure. If biventricular repair is chosen, open valvotomy (OV) has been firmly established as the most effective initial treatment. In comparison with blind ballooning, OV, with exact splitting of fused commissures and shaving of obstructing nodules, can produce a better valve with a maximum valve orifice, without causing regurgitation. Thus, predictable and consistent early and longer-lasting results in any type of valve morphology are provided...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
Yidong Wang, Bingruo Wu, Emily Farrar, Wendy Lui, Pengfei Lu, Donghong Zhang, Christina M Alfieri, Kai Mao, Ming Chu, Di Yang, Di Xu, Michael Rauchman, Verdon Taylor, Simon J Conway, Katherine E Yutzey, Jonathan T Butcher, Bin Zhou
Aims: Congenital anomalies of arterial valves are common birth defects, leading to valvar stenosis. With no pharmaceutical treatment that can prevent the disease progression, prosthetic replacement is the only choice of treatment, incurring considerable morbidity and mortality. Animal models presenting localized anomalies and stenosis of congenital arterial valves similar to that of humans are critically needed research tools to uncover developmental molecular mechanisms underlying this devastating human condition...
March 1, 2017: European Heart Journal
Kwang Ho Choi, Si Chan Sung, Hyungtae Kim, Hyung Doo Lee, Gil Ho Ban, Geena Kim, Hee Young Kim
The surgical management of transposition complex with aortic arch obstruction remains technically demanding due to anatomic complexity. Even in the recent surgical era, there are centers that address this anomaly with a staged strategy. This report presents our experiences with a one-stage repair of transposition complexes with aortic arch obstructions more than the last 10 years. Since 2003, 19 patients with a transposition of the great arteries (TGA, 2 patients) or a double outlet of the right ventricle (DORV, 17 patients) and aortic arch obstruction have undergone one-stage repair of their anomalies...
January 2016: Pediatric Cardiology
Marcos Danillo Peixoto Oliveira, Fernando Roberto de Fazzio, José Mariani Junior, Carlos M Campos, Luiz Junya Kajita, Expedito E Ribeiro, Pedro Alves Lemos
Coronary artery anomalies are congenital changes in their origin, course, and/or structure. Most of them are discovered as incidental findings during coronary angiographic studies or at autopsies. We present herein the case of a 70-year-old man with symptomatic severe aortic valvar stenosis whose preoperative coronary angiogram revealed a so far unreported coronary anomaly circulation pattern.
2015: Case Reports in Cardiology
Kevin G Friedman, Lindsay Freud, Maria Escobar-Diaz, Puja Banka, Sitaram Emani, Wayne Tworetzky
Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function...
October 2015: Pediatric Cardiology
Laura Gellis, Puja Banka, Audrey Marshall, Sitaram Emani, Diego Porras
BACKGROUND: Valve-sparing repair in patients with tetralogy of Fallot (TOF) carries the risk of residual or recurrent right ventricular outflow tract (RVOT) obstruction, which is often treated with transcatheter balloon dilation (BD). The outcomes and associated complications of BD of the RVOT in this scenario remain unknown. METHODS: Retrospective review of the records of the Department of Cardiology at Boston Children's Hospital from 2000 to 2013 was performed...
October 2015: Catheterization and Cardiovascular Interventions
Constantine Mavroudis, Constantine D Mavroudis, Jeffrey P Jacobs
Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous "membrane" resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis...
December 2014: Cardiology in the Young
Gary E Stapleton
Neonatal aortic valvar stenosis can be challenging to treat because of the varied morphology of the valve, the association with hypoplasia of other left heart structures, and the presence of left ventricular systolic dysfunction or endomyocardial fibroelastosis. Balloon valvuloplasty and surgical valvotomy have been well described in the literature for the treatment of neonatal aortic stenosis. Transcatheter therapy for neonatal aortic stenosis is the preferred method at many centres; however, some centres prefer a surgical approach...
December 2014: Cardiology in the Young
Jeremy T Affolter, Nancy S Ghanayem
Neonatal critical aortic stenosis is a rare form of CHD that often presents with cardiogenic shock. Although surgical and cardiac catheterisation-based interventions have been successful in alleviating left ventricular outflow tract obstruction, it remains associated with high morbidity and mortality. Critical aortic stenosis results in elevated left ventricular wall stress, which ultimately increases myocardial oxygen consumption and disrupts coronary artery perfusion during diastole, leading to ventricular dysfunction and cardiogenic shock...
December 2014: Cardiology in the Young
Hasan Jilaihawi, Raj R Makkar, Mohammad Kashif, Kazuaki Okuyama, Tarun Chakravarty, Takahiro Shiota, Gerald Friede, Mamoo Nakamura, Niraj Doctor, Asim Rafique, Kentaro Shibayama, Hirotsugu Mihara, Alfredo Trento, Wen Cheng, John Friedman, Daniel Berman, Gregory P Fontana
AIMS: We sought to optimize a method for quantification of the calcium in the aortic-valvar complex for the prediction of significant paravalvular leak (PVL) after transcatheter aortic valve implantation (TAVI). METHODS AND RESULTS: All patients had severe symptomatic aortic stenosis and were treated with balloon-expandable TAVI (Sapien/Sapien-XT, Edwards Lifesciences LLC, Irvine, CA, USA). In order to correct for precise annular sizing, only patients with available contrast computed tomography (CT) data for measurements were included (n = 198)...
December 2014: European Heart Journal Cardiovascular Imaging
Jasminkumar Patel, Fernanda Kupferman, Susana Rapaport, Jeffrey H Kern
This study aimed to determine current practices regarding prophylaxis against infective endocarditis among pediatric cardiologists in the United States 5 years after publication of the most recent American Heart Association (AHA) recommendations. A descriptive, analytical, cross-sectional study was conducted from June 2012 to November 2012 in the format of an anonymous self-administered e-mailed questionnaire among pediatric cardiologists across the United States. The questionnaire inquired about demographic information of cardiologists and their current practices of prescribing preprocedure antibiotic prophylaxis against endocarditis to patients with specific preexisting cardiac conditions...
October 2014: Pediatric Cardiology
Lars G Svensson, Adil H Al Kindi, Alessandro Vivacqua, Gösta B Pettersson, A Marc Gillinov, Tomislav Mihaljevic, Eric E Roselli, Joseph F Sabik, Brian Griffin, Donald F Hammer, Leonardo Rodriguez, Sarah J Williams, Eugene H Blackstone, Bruce W Lytle
BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1% to 2% of the population. Eventually, 20% develop clinically important valvar regurgitation requiring surgical intervention. Aortic valve repair avoids anticoagulation and prosthetic valve-related complications. This study evaluated long-term durability of BAV repair. METHODS: From 1985 to 2011, 728 patients, mean age 42±12 years, underwent BAV repair at Cleveland Clinic...
May 2014: Annals of Thoracic Surgery
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