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Congenital aortic stenosis

Neeraj Awasthy, Ria Garg, S Radhakrishnan, Savitri Shrivastava
: Balloon aortic valvuloplasty (BAV) is a well accepted modality of treatment in congenital aortic stenosis in all age groups. Although in infants and children it is the modality of choice, in adolescents and young adults, it is of debatable efficacy. AIM: To evaluate long-term results of aortic valvuloplasty particularly in adolescent and adults (>12 years) and compare the outcome in other age groups that are <1 year and between 1 are 11 years. SETTING: Tertiary referral center...
September 2016: Indian Heart Journal
Deepak Ameta, Rajiv Bharat Kharwar, Pallavi Aga, Rishi Sethi, Sharad Chandra, Sudhanshu Kumar Dwivedi, Varun Shankar Narain, Ram Kirti Saran
Bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, and rarely, it is associated with sinus of Valsalva aneurysms (SOVAs). And very infrequently, these SOVAs rupture into left side of heart. We hereby report a case of 12-year-old male with BAV with severe aortic stenosis with a large SOVA that ruptured into the left side of the heart. The anatomy was delineated with multimodality imaging; initially with two-dimensional trans-thoracic echocardiography (TTE), and later on with three-dimensional TTE and with multi detector computed tomography...
September 2016: Indian Heart Journal
Takaaki Saito, Hiroki Tanaka, Naoto Yamamoto, Kazunori Inuzuka, Masaki Sano, Naoki Unno
We report the rare case of a 54-year-old man with uncontrolled renovascular hypertension, who was found to have an abdominal aortic aneurysm with congenital solitary pelvic kidney and superior mesenteric artery stenosis. A single renal artery branched from aneurysmal aortic bifurcation, and both the renal artery and the superior mesenteric artery (SMA) had severe stenosis at their origins. The aneurysm was repaired with a bifurcated Dacron graft, to which the renal artery was anastomosed. SMA bypass was created between the graft's left limb and the SMA using another Dacron graft...
2016: Annals of Vascular Diseases
Afshan B Hameed, Shahbudin H Rahimtoola
No abstract text is available yet for this article.
October 18, 2016: Journal of the American College of Cardiology
Hee-Jin Kwon, Jae-Hyeong Park, Song Soo Kim, Byung Joo Sun, Sun Ah Jin, Jun-Hyung Kim, Jae-Hwan Lee, Siwan Choi, Jin-Ok Jeong, In-Whan Seong
Unicuspid aortic valve (UAV) is an extremely rare form of congenital aortic valvular abnormality. Although UAV shows similar clinical characteristics to bicuspid aortic valve, the clinical symptoms develop at earlier age and progress at a faster pace in UAV. In this report, we are presenting a 42-year-old male with severe aortic stenosis associated with unicommissural UAV. The patients underwent a successful Bentall operation.
September 2016: Journal of Cardiovascular Ultrasound
Kavita Sharma, Poonam Thankavel
Congenital stenosis/atresia of a coronary artery is an exquisitely rare anomaly (Congenit Heart Dis, 2, 2007, 347) with increased risk of sudden death. Bilateral coronary obstruction is even more unusual but has been reported in conjunction with aortic valve disease, syphilis, and Takayasu's arteritis. To the best of our knowledge, obstruction of both coronaries in a pediatric patient has only been reported once (Ann Thorac Surg, 55, 1993, 1564). We present a patient with an intramural, anomalous aortic origin of the right coronary artery from the contralateral sinus (AAORCA) with near atresia of the left main coronary ostium...
October 4, 2016: Echocardiography
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
Patrick M Sullivan, Agustin E Rubio, Troy A Johnston, Thomas K Jones
OBJECTIVES: To describe long-term risk of mortality, aortic insufficiency (AI), and re-intervention following balloon aortic valvuloplasty (BAV) in pediatric patients and to identify risk factors for re-intervention. BACKGROUND: Few studies report long-term outcomes following BAV in infants and children. METHODS: Kaplan-Meier estimates and proportional hazards regression were used in a retrospective study of 154 patients undergoing BAV from 1993 to 2013...
September 21, 2016: Catheterization and Cardiovascular Interventions
Tetsuo Yamanaka, Toru Fukatsu, Yoshimaro Ichinohe, Hirotaka Komatsu, Masahiro Seki, Kenichi Sasaki, Hideaki Takai, Takashi Kunihara, Yasunobu Hirata
We herein report an adult case of unicommissural unicuspid aortic valve (UAV). A 59-year-old man, who was noted to have a cardiac murmur at 31 years of age, was admitted to our hospital due to acute heart failure. Severe calcification in the aortic valve with severe low-flow/low-gradient aortic stenosis and moderate aortic regurgitation was observed and thought to be the cause of heart failure, however, the etiology of aortic valve dysfunction was not clear. Aortic valve replacement was subsequently performed, and unicommissural UAV was diagnosed according to the intraoperative findings...
2016: Internal Medicine
Roland Fiszer, Małgorzata Szkutnik, Linda Litwin, Sebastian Smerdziński, Beata Chodór, Jacek Białkowski
INTRODUCTION: Balloon angioplasty (BAP) and aortic or pulmonary balloon valvuloplasty (BAV, BPV) are well-established treatment options in congenital heart defects. Recently, significant technological progress has been made and new catheters have been implemented in clinical practice. AIM: To analyze the results of BAP, BAV and BPV with the new balloon catheter Valver and its second generation Valver II, which the company Balton (Poland) launched and developed. These catheters have not been clinically evaluated yet...
2016: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
Khalil Ahmad, Christian Juhl Terkelsen, Kim Allan Terp, Ole Norling Mathiassen, Bjarne Linde Nørgaard, Henning Rud Andersen, Steen Hvitfeldt Poulsen
Transcatheter aortic valve implantation (TAVI) is an established therapeutic alternative to surgical aortic valve replacement (SAVR) in high-risk or inoperable patients with symptomatic aortic valve stenosis. Hitherto, TAVI is not recommended in young and low-intermediate risk patients. However, TAVI may also serve as an alternative to SAVR in selected young patients, e.g., patients who have previously undergone multiple cardiac surgery procedures. We report a case of trans-femoral TAVI in a 25-year-old heart transplant (HTx) recipient with prior surgery for congenital heart disease...
August 2016: Journal of Thoracic Disease
Elham Abbas, Devin M Cox, Teri Smith, Merlin G Butler
We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11...
September 2016: Journal of Pediatric Genetics
Marco Morosin, Valeria Leonelli, Rita Piazza, Matteo Cassin, Luigi Neglia, Elisa Leiballi, Eugenio Cervesato, Giulia Barbati, Gianfranco Sinagra, Gian Luigi Nicolosi
BACKGROUND AND OBJECTIVES: Bicuspid aortic valve (BAV) disease is the most common congenital cardiac malformation. The aim of the present article is to determine clinical and echocardiographic prognostic factors and provide a predictive model of outcome of a large cohort of patients with BAV. METHODS: We retrospectively enrolled 337 patients consecutively assessed for echocardiography at our Cardiology Department from 1993 to 2014. We considered aortic valve replacement, aortic surgery and cardiovascular death as a clinical combined end-point...
September 7, 2016: Journal of Cardiovascular Medicine
Kevin Gao, Ritu Sachdeva, Bryan H Goldstein, Sean Lang, Christopher J Petit
OBJECTIVES: We sought to determine the relationship between aortic valve morphology and left ventricular (LV) systolic function in children with aortic stenosis (AS) prior to balloon aortic valvuloplasty (BAV). BACKGROUND: Both aortic valve morphology and LV systolic function have been linked with outcomes in children with congenital AS undergoing BAV. The relationship between aortic valve morphology and LV function is poorly defined despite their importance in regard to outcomes...
September 2016: Journal of Invasive Cardiology
Molly Starkovich, Seema R Lalani, Catherine L Mercer, Daryl A Scott
Congenital heart defects (CHD) are present in over 1% of all newborns and are the leading cause of birth-defect-related deaths in the United States. We describe two male subjects with CHD, one with an atrial septal defect, a ventricular septal defect, and pulmonary artery stenosis; and the other with tetralogy of Fallot and a right aortic arch, who carry partially overlapping, de novo deletions of chromosome 5q33. The maximum region of overlap between these deletions encompasses HAND1 and SAP30L, two genes that have previously been shown to play a role in cardiac development...
September 2, 2016: American Journal of Medical Genetics. Part A
Samantha L Freeze, Benjamin J Landis, Stephanie M Ware, Benjamin M Helm
Bicuspid aortic valve (BAV) is the most common congenital heart defect and falls in the spectrum of left-sided heart defects, also known as left ventricular outflow tract obstructive (LVOTO) defects. BAV is often identified in otherwise healthy, asymptomatic individuals, but it is associated with serious long term health risks including progressive aortic valve disease (stenosis or regurgitation) and thoracic aortic aneurysm and dissection. BAV and other LVOTO defects have high heritability. Although recommendations for cardiac screening of BAV in at-risk relatives exist, there are no standard guidelines for providing genetic counseling to patients and families with BAV...
August 22, 2016: Journal of Genetic Counseling
Stéphane Kajingu Enciso, Maxime Elens, Jean Rubay
We report a case of successful heterograft aortic valve replacement following an impede Ross procedure in a 48-year-old man presenting with a congenital bicuspid pulmonary valve. The patient was admitted for aortic valve stenosis that required an aortic valve replacement (AVR). Owing to his young age and reluctance to long-term anticoagulation therapy, it was decided to do an AVR by pulmonary autograft. During surgery, the anatomical unsuitability of the graft was discovered leading to the procedure's readjustment...
August 22, 2016: Acta Chirurgica Belgica
José G Díez, Michael Schechter, Kathryn G Dougherty, Ourania Preventza, Joseph S Coselli
Transcatheter aortic valve replacement (TAVR) is a well-established method for replacing native aortic valves; however, it was conceived for elderly patients with aortic valve stenosis, and the lack of data on long-term durability has led practitioners to restrict the use of TAVR to patients who have short life expectancies. Here, we describe the case of a 21-year-old woman who had undergone 3 previous open aortic valve replacements and who presented with symptoms of recurrent valvular failure. Transthoracic echocardiograms and computed tomographic angiograms revealed a degenerating aortic root homograft with substantial calcification, moderate-to-severe aortic valve stenosis, and severe aortic valve regurgitation...
August 2016: Texas Heart Institute Journal
Garick D Hill, Salil Ginde, Rodrigo Rios, Peter C Frommelt, Kevin D Hill
BACKGROUND: Optimal initial treatment for congenital aortic valve stenosis in children remains unclear between balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). METHODS AND RESULTS: We performed a contemporary systematic review and meta-analysis to compare survival in children with congenital aortic valve stenosis. Secondary outcomes included frequency of at least moderate regurgitation at hospital discharge as well as rates of aortic valve replacement and reintervention...
August 2016: Journal of the American Heart Association
Yasuhiro Hamatani, Hatsue Ishibashi-Ueda, Toshiyuki Nagai, Yasuo Sugano, Hideaki Kanzaki, Satoshi Yasuda, Tomoyuki Fujita, Junjiro Kobayashi, Toshihisa Anzai
BACKGROUND: Congenital bicuspid aortic valve (CBAV) is the main cause of aortic stenosis (AS) in young adults. However, the histopathological features of AS in patients with CBAV have not been fully investigated. METHODS AND RESULTS: We examined specimens of aortic valve leaflets obtained from patients who had undergone aortic valve re/placement at our institution for severe AS with CBAV (n = 24, CBAV-AS group), severe AS with tricuspid aortic valve (n = 24, TAV-AS group), and severe aortic regurgitation (AR) with CBAV (n = 24, CBAV-AR group)...
2016: PloS One
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