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Congenital valve disease

Amee M Bigelow, Brandon S Arnold, Gregory C Padrutt, John M Clark
In current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein's anomaly of the tricuspid valve along with persistent supraventricular tachycardia...
October 21, 2016: Cardiology in the Young
Jagdish C Mohan, Vishwas Mohan, Madhu Shukla, Arvind Sethi
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation...
September 2016: Indian Heart Journal
Justin T Tretter, Diane E Spicer, Shumpei Mori, Sathish Chikkabyrappa, Andrew N Redington, Robert H Anderson
A comprehensive understanding of the normal and abnormal aortic root is paramount if we are to improve not only our assessment of the aortic root and its components but also the surgical approach to reconstructing this complex structure when congenitally malformed. Most anatomic and imaging-based classifications of the normal root recognize and describe the basic components, which include the shape and size of the three aortic sinuses and their three valvar leaflets, as well as the sinutubular junction and proximal ascending aorta...
October 11, 2016: Journal of the American Society of Echocardiography
Jason Misurac
Neonatal chronic kidney disease (CKD) occurs with an estimated incidence of 1 in 10,000 live births, whereas the incidence of neonatal end-stage renal disease (ESRD) is about 7.1 per million age-related population. The most frequent etiologies are renal hypoplasia/dysplasia, posterior urethral valves, and other congenital anomalies of the kidney and urinary tract. Other etiologies include polycystic kidney disease, cortical necrosis, and renal vascular thrombosis. Management of CKD focuses primarily on replacing renal functions such as erythropoietin, 1,25-hydroxylation of vitamin D, electrolyte homeostasis/excretion, and, in ESRD, waste product removal...
October 9, 2016: Seminars in Fetal & Neonatal Medicine
J Rajendra Kumar, Mamta B Kumbhare, P Goutham Kumar, T Vishal Goud
An accessory coronary cusp in aortic valve is a rare congenital cardiovascular malformation with an estimated incidence of 0.003 to 0.043% of all congenital heart disease. It is also describe as a quadricuspid aortic valve (QAV). Several different anatomical variations of quadricuspid aortic valve have been described. Current technology enables noninvasive diagnosis in most cases. Here we reported a rare an isolated congenital type 'C' QAV with aortic regurgitation; hence it was worth reporting this interesting case...
February 2016: Journal of the Association of Physicians of India
Oliver J Harrison, Narain Moorjani, Christopher Torrens, Sunil K Ohri, Felino R Cagampang
Bicuspid aortic valve (BAV) disease is the most common congenital cardiac abnormality and predisposes patients to life-threatening aortic complications including aortic aneurysm. Quantitative real-time reverse transcription PCR (qRT-PCR) is one of the most commonly used methods to investigate underlying molecular mechanisms involved in aortopathy. The accuracy of the gene expression data is dependent on normalization by appropriate housekeeping (HK) genes, whose expression should remain constant regardless of aortic valve morphology, aortic diameter and other factors associated with aortopathy...
2016: PloS One
Kyungjoong Kim, Jiwook Choi, Youngjae Doo, Yeong Seop Yun, Jongwook Kim, Jae Beom Lee
Common atrium is a rare congenital heart disease characterized by complete absence of the interatrial septum, and is commonly accompanied by malformation of the atrioventricular valve. Most patients with common atrium experience symptoms during childhood. Here, we describe a patient with common atrium who experienced his first obvious symptom at 48 years of age.
September 2016: Journal of Cardiovascular Ultrasound
Carmen Rueda-Martínez, M Carmen Fernández, María Teresa Soto-Navarrete, Manuel Jiménez-Navarro, Ana Carmen Durán, Borja Fernández
Bicuspid aortic valve (BAV) is the most frequent congenital cardiac malformation in humans, and appears frequently associated with dilatation of the ascending aorta. This association is likely the result of a common aetiology. Currently, a Syrian hamster strain with a relatively high (∼40%) incidence of BAV constitutes the only spontaneous animal model of BAV disease. The characterization of molecular alterations in the aorta of hamsters with BAV may serve to identify pathophysiological mechanisms and molecular markers of disease in humans...
2016: PloS One
Zhanguo Sun, Wenjian Xu, Shuran Huang, Yueqin Chen, Xiang Guo, Zhitao Shi
BACKGROUND: Despite dual-source computed tomography (DSCT) technology has been performed well on adults or infants with heart disease, specific knowledge about children with congenital pulmonary valve stenosis (PS) remained to be established. OBJECTIVES: This original research aimed to establish a professional approach of DSCT performing technology on children and to assess the image quality performed by DSCT to establish a diagnostic evaluation for children with PS...
April 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
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
Hoang H Nguyen, Shabana Shahanavaz, George F Van Hare, David T Balzer, Ramzi Nicolas, Jennifer N Avari Silva
BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is first-line therapy for some congenital heart disease patients with right ventricular outflow tract dysfunction. The hemodynamics improvements after PPVI are well documented, but little is known about its effects on the electrophysiologic substrate. The objective of this study is to assess the short- and medium-term electrophysiologic substrate changes and elucidate postprocedure arrhythmias. METHODS AND RESULTS: A retrospective chart review of patients undergoing PPVI from May 2010 to April 2015 was performed...
September 30, 2016: Journal of the American Heart Association
Hadyl Asfari, Julien Souvignet, Agnès Lillo-Le Louët, Béatrice Trombert, Marie-Christine Jaulent, Cédric Bousquet
AIM: To propose an alternative approach for building custom groupings of terms that complements the usual approach based on both hierarchical method (selection of reference groupings in medical dictionary for regulatory activities [MedDRA]) and/or textual method (string search), for case reports extraction from a pharmacovigilance database in response to a safety problem. Here we take cardiac valve fibrosis as an example. METHODS: The list of terms obtained by an automated approach, based on querying ontology of adverse drug reactions (OntoADR), a knowledge base defining MedDRA terms through relationships with systematized nomenclature of medicine-clinical terms (SNOMED CT) concepts, was compared with the reference list consisting of 53 preferred terms obtained by hierarchical and textual method...
July 21, 2016: Thérapie
K Li, F E Zhang, A P Wang, G F Zhu
Objective: To investigate the clinical features, pathogenic distribution and drug susceptibility of patients with infective endocarditis (IE). Methods: Clinical data of IE patients were collected, who were admitted to Capital Medical University Affiliated Beijing Anzhen Hospital from January 2012 to March 2015. Results: Three hundred and three IE patients were enrolled with age of (43±16) years old. Fever (85.5%)and cardiac murmur (62.4%)were the most common clinical presentations. Congenital heart diseases was the leading underlying diseases in IE patients...
October 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Qinghai Peng, Qichang Zhou, Ming Zang, Jiawei Zhou, Ran Xu, Tao Wang, Shi Zeng
OBJECTIVE: To screen and detect cortex gyration in fetuses with congenital heart disease (CHD) using Ultrasonography (US) during routine obstetric scans. METHODS: The depth of the Sylvian fissure (SF), parieto-occipital fissure (POF) and calcarine (CF) fissure were prospectively serially measured and compared in 45 fetuses with CHD and 45 normal fetuses. Cardiac hemodynamic parameters, including aortic valve diameter (AV), pulmonary valve diameter (PV), velocity time integral of the aortic valve (VTIav), and velocity time integral of the pulmonary valve (VTIpv), were recorded...
September 28, 2016: Prenatal Diagnosis
Lillian M Lai, Joseph Y Cheng, Marcus T Alley, Tao Zhang, Michael Lustig, Shreyas S Vasanawala
PURPOSE: To assess the feasibility of ferumoxytol-enhanced anesthesia-free cardiac MRI in neonates and young infants for complex congenital heart disease (CHD). MATERIALS AND METHODS: With Institutional Review Board approval, 21 consecutive neonates and young infants (1 day to 11 weeks old; median age of 3 days) who underwent a rapid two-sequence (MR angiography [MRA] and four-dimensional [4D] flow) MRI protocol with intravenous ferumoxytol without sedation (n = 17) or light sedation (n = 4) at 3 Tesla (T) (except one case at 1...
September 28, 2016: Journal of Magnetic Resonance Imaging: JMRI
Robert W Loar, Athar M Qureshi, Christina Y Miyake, Santiago O Valdes, Jeffrey J Kim, Caridad M De la Uz
BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is utilized for dysfunctional right ventricular outflow tracts (RVOT) to relieve obstruction, regurgitation, and RV dysfunction. PPVI has not been reported to induce arrhythmias. This study is the first to report the incidence of ventricular tachycardia (VT) after PPVI. METHODS: This was a retrospective study of all patients who had PPVI at a single institution. All patients were admitted after PPVI for overnight telemetry monitoring...
September 26, 2016: Journal of Interventional Cardiology
Natalie Behrle, Peter Dyke, Abdallah Dalabih
Motor vehicle accident is the most common cause of blunt cardiac injury (BCI) in children (85.3%) due to the height of the child in relation to proper restraints and the compliant pediatric rib cage (J Trauma. 1996;40:200-202). Trauma to the chest wall may lead to injury of the myocardium, resulting in myocardial contusion, ventricular septal defect (VSD), ventricular free wall rupture, or valve compromise (J Trauma. 1996;40; 200-202; Heart Lung. 2012;41:200-202; J Inj Violence Res. 2012;4:98-100). There are several proposed mechanisms for the formation of VSD after blunt chest trauma including rupture of ischemic myocardium related to the initial trauma and reopening of a spontaneously closed congenital VSD...
September 23, 2016: Pediatric Emergency Care
Veysel Tosun, Necmettin Korucuk, Mustafa Mustafa Karakaş, Ünal Güntekin
Congenitally corrected transposition of the great arteries is a rare abnormality accounting for approximately 1% of clinically apparent congenital heart disease. Age at time of diagnosis and survival rate vary and depend on associated anomalies, including pulmonary stenosis, ventricular septal defect, atrial septal defect, atrioventricular block, and atrioventricular valve regurgitation. Reported cases of corrected transposition of the great arteries with single coronary ostium anomaly and atrial septal defect are very rare...
September 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Rouven Kubicki, Brigitte Stiller, Jochen Grohmann
Pseudoaneurysm formation is a rare but potentially life-threatening complication after surgical repair of congenital heart disease. We present a boy with truncus arteriosus communis 14 years after homograft placement in pulmonary position. On follow-up, he presented progressive chronic homograft degeneration. Moreover, a large pseudoaneurysm in the right ventricular outflow tract was surprisingly depicted. We opted for a two-stage interventional approach.
2016: SpringerPlus
Max B Mitchell
No abstract text is available yet for this article.
November 2016: Journal of Thoracic and Cardiovascular Surgery
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