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congenital critical aortic stenosis

Brian A Boe, Jeffrey D Zampi, Kevin F Kennedy, Natalie Jayaram, Diego Porras, Susan R Foerster, Aimee K Armstrong
OBJECTIVES: The aim of this study was to evaluate practice patterns and outcomes of a contemporary group of patients undergoing balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS). BACKGROUND: BAV is the most common treatment for isolated congenital AS. METHODS: Within the IMPACT (Improving Pediatric and Adult Congenital Treatments) Registry, all BAV procedures performed between January 2011 and March 2015 were identified...
September 11, 2017: JACC. Cardiovascular Interventions
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
Q M Zhao, F Liu, L Wu, M Ye, B Jia, X J Ma, G Y Huang
Objective: Undiagnosed critical congenital heart disease (CCHD) was assessed before discharge from maternity hospital.Basic information was provided for screening CCHD in the early neonatal stage.Chi-squared test was used for comparison of categorical variables(detection rate of different types of CCHD). Method: A retrospective cohort study was conducted in neonates with CCHD who were admitted to Children's Hospital of Fudan University between 1 January 2012 and 31 December 2015. For comparing with the previously reported undiagnosed rate of CCHD at discharge, CCHD was defined as all duct dependent congenital heart disease (DDCHD) and any cyanotic CHD that required early surgery...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Elles J Dijkema, Tim Leiner, Heynric B Grotenhuis
Coarctation of the aorta (CoA ) is a well-known congenital heart disease (CHD) , which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of C o A remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (> 20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV)...
August 2017: Heart: Official Journal of the British Cardiac Society
Ting-Ying Lei, Ru Li, Fang Fu, Jun-Hui Wan, Yong-Ling Zhang, Xiang-Yi Jing, Can Liao
OBJECTIVE: Smith-Magenis syndrome (SMS) is a multiple congenital anomalies/mental retardation disorder characterized by an interstitial deletion involving chromosome 17p11.2 containing the retinoic acid-induced 1 (RAI1) gene or due to mutation of RAI1. Few cases have been reported in the medical literature regarding prenatal diagnosis of SMS. We report on the prenatal diagnosis of SMS in two fetuses with increased nuchal translucency (NT), mild lateral ventriculomegaly, and congenital heart defects by whole-genome and high-resolution chromosome microarray analysis (CMA)...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
Kemal Nişli, Serra Karaca, Aygün Dindar
Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates of morbidity and mortality...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Jaeger P Ackerman, John A Smestad, David J Tester, Muhammad Y Qureshi, Beau A Crabb, Nancy J Mendelsohn, Michael J Ackerman
OBJECTIVE: To use whole exome sequencing (WES) of a family trio to identify a genetic cause for polyvalvular syndrome. METHODS AND RESULTS: A male child was born with mild pulmonary valve stenosis and mild aortic root dilatation, and an atrial septal defect, ventricular septal defect, and patent ductus arteriosus that were closed surgically. Subsequently, the phenotype of polyvalvular syndrome with involvement of both semilunar and both atrioventricular valves emerged...
September 2016: Congenital Heart Disease
Shi-Min Yuan, Gulimila Humuruola
Fetal cardiac interventions for congenital heart diseases may alleviate heart dysfunction, prevent them evolving into hypoplastic left heart syndrome, achieve biventricular outcome and improve fetal survival. Candidates for clinical fetal cardiac interventions are now restricted to cases of critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block...
2016: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
Ramush Bejiqi, Hana Bejiqi, Ragip Retkoceri
BACKGROUND: Obstruction to the left ventricular outflow of the heart may be above the aortic valve (5%), at the valve (74%), or in the subvalvar region (23%). These anomalies represent 3 to 6% of all patients with congenital heart defects (CHD), and it occurs more often in males (male-female ratio of 4:1). AIM: The purpose of this study was to determine the sensitivity and specificity of transthoracic echocardiography in diagnosis of discrete subaortic membrane, to determine convenient time for surgical intervention, and for identifying involvement of the aortic valve by subaortic shelf...
March 15, 2016: Open Access Macedonian Journal of Medical Sciences
Lindsay R Freud, Wayne Tworetzky
PURPOSE OF REVIEW: This article discusses the rationale, patient selection, technical aspects, and outcomes of percutaneous, ultrasound-guided fetal cardiac intervention (FCI) for structural congenital heart disease. RECENT FINDINGS: FCI is most commonly performed for three forms of congenital heart disease: severe aortic stenosis with evolving hypoplastic left heart syndrome (HLHS), pulmonary atresia with intact ventricular septum and evolving hypoplastic right heart syndrome, and HLHS with intact or highly restrictive atrial septum...
April 2016: Current Opinion in Pediatrics
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
Michael O Murphy, Hannah Bellsham-Revell, Gareth J Morgan, Thomas Krasemann, Eric Rosenthal, Shakeel A Qureshi, Caner Salih, Conal B Austin, David R Anderson
BACKGROUND: Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure. METHODS: Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2...
December 2015: Annals of Thoracic Surgery
Rohit S Loomba, Jessica L Bowman, Yumei Cao, James Tweddell, Joseph A Dearani, Pippa M Simpson, Frank Cetta, Andrew N Pelech
BACKGROUND: Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy. METHODS: A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions...
November 2015: Congenital Heart Disease
Saeid Hosseini, Fahimeh Kashfi, Niloufar Samiei, Amirjamshid Khamoushi, Alireza Alizadeh Ghavidel, Forouzan Yazdanian, Yalda Mirmesdagh, Carlos A Mestres
BACKGROUND AND AIM OF THE STUDY: Cardiac surgery during pregnancy is rarely required and potentially increases feto-maternal mortality. The study aim was to evaluate pregnancy outcomes in females who underwent open-heart surgery with cardiopulmonary bypass (CPB) during pregnancy. METHODS: Between 1999 and 2014, a total of 16 pregnant women (mean age 27 ± 7 years; mean gestational age 13 ± 7.7 weeks) underwent urgent cardiac surgery using CPB. The preoperative diagnosis included prosthetic valve dysfunction in 12 women (five aortic, seven mitral), native valve endocarditis and critical aortic stenosis each in one woman, and intracardiac masses in two women...
March 2015: Journal of Heart Valve Disease
Laura Ciocca, M Cristina Digilio, Antonietta Lombardo, Gemma D'Elia, Anwar Baban, Rossella Capolino, Stefano Petrocchi, Serena Russo, Pietro Sirleto, M Cristina Roberti, Bruno Marino, Adriano Angioni, Bruno Dallapiccola
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect (CHD), associated with extracardiac anomalies in the 15-28% of cases, in the setting of chromosomal anomalies, mendelian disorders, and organ defects. We report on a syndromic female newborn with HLHS and terminal 21q22.3 deletion (del 21q22.3), investigated by Fluorescence In Situ Hybridization (FISH) using a panel of 26 contiguous BAC probes. Although rare, del 21q22.3 has been described in two additional patients with HLHS. In order to investigate the frequency and role of this chromosomal imbalance in the pathogenesis of left-sided obstructive heart defects, we screened for del 21q22...
March 2015: American Journal of Medical Genetics. Part A
Hujun Cui, Xinxin Chen, Jianbin Li, Yanqin Cui, Li Ma, Yuansheng Xia, Shengchun Yang
OBJECTIVE: To discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors. METHODS: The clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case...
October 2014: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
Yusuke Misumi, Takaya Hoashi, Koji Kagisaki, Satoshi Yazaki, Masataka Kitano, Kenichi Kurosaki, Isao Shiraishi, Hajime Ichikawa
The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment...
April 2015: Pediatric Cardiology
Maythem Saeed, Steven W Hetts, Robert Jablonowski, Mark W Wilson
Myocardial pathologies are major causes of morbidity and mortality worldwide. Early detection of loss of cellular integrity and expansion in extracellular volume (ECV) in myocardium is critical to initiate effective treatment. The three compartments in healthy myocardium are: intravascular (approximately 10% of tissue volume), interstitium (approximately 15%) and intracellular (approximately 75%). Myocardial cells, fibroblasts and vascular endothelial/smooth muscle cells represent intracellular compartment and the main proteins in the interstitium are types I/III collagens...
November 26, 2014: World Journal of Cardiology
Nuria Iglesias-Román, Teresa Alvarez, Coral Bravol, Ricardo Pérez, Francisco Gámez, Juan De León Luis
This is a report about a case of prenatal diagnosis of critical fetal aortic stenosis with severe mitral valve insufficiency in a 35+6 weeks fetus. Aortic stenosis represents 3% of congenital heart diseases, but its association with mitral regurgitation is quite unusual. Thanks to the latest advances in fetal ultrasonography we can now achieve a more precise diagnosis and we have been able to improve the understanding of its physiopathology. Based on this case we have reviewed the most recent literature about fetal aortic stenosis and mitral valve insufficiency, with the aim of summarizing its main physiopathological features, highlighting the clues and key points for its intrauterine diagnosis, describing its principal complications and summarizing its current treatment options...
September 2014: Ginecología y Obstetricia de México
Sergej M Prijic, Vladislav A Vukomanovic, Mila S Stajevic, Bojko B Bjelakovic, Marija D Zdravkovic, Igor N Sehic, Jovan Lj Kosutic
Percutaneous balloon aortic valvoplasty (BAV) and surgical aortic valvotomy (SAV) are palliative procedures in patients with non-critical congenital valve stenosis. The aim of the study was to evaluate long-term BAV and SAV results after up to 24 years of follow-up. From 1987 to 2013, 74 consecutive interventions were performed in patients with aortic stenosis, and 62 were included in the study (39 BAVs and 23 SAVs). Age of BAV patients was 1.3 months to 17 years, and of SAV patients 1.2 months to 15 years...
March 2015: Pediatric Cardiology
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