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Congenital valve disease

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https://www.readbyqxmd.com/read/29912937/comparison-of-three-non-invasive-hemodynamic-monitoring-methods-in-critically-ill-children
#1
Chanapai Chaiyakulsil, Marut Chantra, Poomiporn Katanyuwong, Anant Khositseth, Nattachai Anantasit
INTRODUCTION: Hemodynamic parameters measurements were widely conducted using pulmonary artery catheter (PAC) with thermodilution as a reference standard. Due to its technical difficulties in children, transthoracic echocardiography (TTE) has been widely employed instead. Nonetheless, TTE requires expertise and is time-consuming. Noninvasive cardiac output monitoring such as ultrasonic cardiac output monitor (USCOM) and electrical velocimetry (EV) can be performed rapidly with less expertise requirement...
2018: PloS One
https://www.readbyqxmd.com/read/29911003/bicuspid-aortic-valve-registry-of-the-italian-society-of-echocardiography-and-cardiovascular-imaging-registro-della-valvola-aortica-bicuspide-della-societ%C3%A3-italiana-di-ecocardiografia-e-cardiovascular-imaging-rationale-and-study-design
#2
Rodolfo Citro, Moreno Cecconi, Salvatore La Carrubba, Eduardo Bossone, Francesco Antonini-Canterin, Stefano Nistri, Fabio Chirillo, Ilaria Dentamaro, Michele Bellino, Alfredo Posteraro, Mauro Giorgi, Licia Petrella, Ines Monte, Vincenzo Manuppelli, Antonio Mantero, Scipione Carerj, Frank Benedetto, Paolo Colonna
Background: Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%-2% of the general population. It is associated not only with notable valvular risk (aortic stenosis and/or regurgitation, endocarditis) but also with aortopathy with a wide spectrum of unpredictable clinical presentations, including aneurysmal dilation of the aortic root and/or ascending thoracic aorta, isthmic coarctation, aortic dissection, or wall rupture. Methods: The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective (from January 1, 2010)/prospective, multicenter, observational registry, expected to enroll 3000 patients with definitive diagnosis of BAV made by transthoracic and/or transesophageal echocardiography, computed tomography, cardiovascular magnetic resonance, or at surgery...
April 2018: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/29908036/acquired-von-willebrand-syndrome-in-congenital-heart-disease-surgery-results-from-an-observational-case-series
#3
Vanya Icheva, Martina Nowak-Machen, Ulrich Budde, Karl Jaschonek, Felix Neunhoeffer, Matthias Kumpf, Michael Hofbeck, Christian Schlensak, Gesa Wiegand
BACKGROUND: Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications. CHD-related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in blood stream and thus cause acquired von Willebrand syndrome (aVWS). OBJECTIVE: To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD...
June 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29884292/role-of-genetic-heart-disease-in-sentinel-sudden-cardiac-arrest-survivors-across-the-age-spectrum
#4
John R Giudicessi, Michael J Ackerman
BACKGROUND: Sudden cardiac arrest (SCA) may be the sentinel expression of a sudden cardiac death-predisposing genetic heart disease (GHD). Although shown to underlie many unexplained SCAs in the young, the contribution of GHDs to sentinel SCA has never been quantified across the age spectrum. Thus, we sought to determine the contribution of GHDs in single-center referral cohort of non-ischemic SCA survivors. METHODS AND RESULTS: Retrospective analysis of 3037 patients was used to identify all individuals who experienced a sentinel event of SCA...
May 30, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29877302/combined-surgical-and-medical-therapy-for-candida-prosthetic-endocarditis-in-a-patient-with-repaired-tetralogy-of-fallot
#5
Kana Kubota, Katsura Soma, Masae Uehara, Toshiro Inaba, Akihito Saito, Norihiko Takeda, Masaru Hatano, Hiroyuki Morita, Ryo Inuzuka, Yasutaka Hirata, Atsushi Yao, Issei Komuro
Candida prosthetic endocarditis (CPE) is an uncommon and fatal complication in adults with congenital heart disease. The current guidelines for the management of fungal endocarditis recommend a combination of surgical and medical therapy. However, it still remains uncertain when surgical management in CPE patients should be performed. Therefore, the prognosis of CPE patients is very poor. Here we report a case of CPE in a 31-year-old woman who had undergone surgical repair for tetralogy of Fallot during childhood and pulmonary valve replacement at the age of 21 years...
June 6, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29877299/ascending-aorta-and-myocardial-mechanics-in-patients-with-clinically-normal-bicuspid-aortic-valve
#6
Gaetano Nucifora, Jamie Miller, Carl Gillebert, Ranjit Shah, Rebecca Perry, Cherie Raven, Majo X Joseph, Joseph B Selvanayagam
Aortic valve dysfunction and aortic wall changes are well-known complications of bicuspid aortic valve (BAV) disease. The aim of the present study was to investigate whether a remodeling process of the left ventricle (LV) is present in patients with isolated BAV. Twenty-two consecutive patients (39 ± 15 years, 9 males) with clinically normal BAV and 18 age- and gender-matched control subjects (37 ± 10 years, 9 males) were included. Cardiovascular magnetic resonance (CMR) imaging was performed to evaluate LV function, aortic valve morphology, aortic orifice area, and ascending aorta (AA) dimensions...
June 6, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29875858/a-very-rare-case-of-cor-triatriatum-with-severe-mitral-regurgitation
#7
Selman Dumani, Ermal Likaj, Edlira Ruci, Ervin Bejko, Ali Refatllari
BACKGROUND: Cor triatriatum sinister is rare congenital heart disease. It is mainly presented in childhood and often accompanied with other congenital anomalies. The cases with cor triatriatum treated surgically in adults and accompanied with severe mitral regurgitation are very rare. CASE REPORT: We present a case with diagnosed cor triatriatum and severe mitral regurgitation. The diagnose was made by echocardiography. She was a female 25 years that was hospitalised with signs of heart failure NYHA II-III...
May 20, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29860487/atrial-flow-regulator-for-failing-fontan-circulation-an-initial-european-experience
#8
Lucia Manuri, Rita Emanuela Calaciura, Andrea De Zorzi, Lilia Oreto, Massimiliano Raponi, Anja Lehner, Nikolaus Haas, Salvatore Agati
Transcatheter creation or enlargement of an atrial septal defect has been used to promote adequate blood flow and mixing in some forms of congenital heart defects or as a relief valve in right or left atrial hypertension, resulting in better cardiac output and/or systemic saturation. We report a case of a 4-year-old male affected by complex congenital heart disease who was admitted for management of severe cyanosis following a staged pericardial fenestrated Fontan procedure. Transoesophageal echocardiogram showed a wide fenestration of 9 mm in size with a severely dilated pericardial Fontan system...
May 30, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29848449/biological-versus-mechanical-heart-valve-prosthesis-during-pregnancy-in-women-with-congenital-heart-disease
#9
Heleen Lameijer, Ymkje J van Slooten, Monique R M Jongbloed, Martijn A Oudijk, Marlies A M Kampman, Arie P van Dijk, Marco C Post, Barbara J Mulder, Krystyna M Sollie, Dirk J van Veldhuisen, Tjark Ebels, Joost P van Melle, Petronella G Pieper
BACKGROUND: We evaluate pregnancy outcome and anticoagulation regimes in women with mechanical and biological prosthetic heart valves (PHV) for congenital heart disease. METHODS: Retrospective multicenter cohort studying pregnancy outcomes in an existing cohort of patients with PHV. RESULTS: 52 women had 102 pregnancies of which 78 pregnancies (46 women) ≥20 weeks duration (59 biological, 19 mechanical PHV). Miscarriages (n = 19, ≤20 weeks) occurred more frequently in women using anticoagulation (P < ...
May 23, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29808135/uncommon-infections-in-children-suggest-underlying-immunodeficiency-a-case-of-infective-endocarditis-in-a-3-year-old-male
#10
Aisha Shakoor, Ahmed El-Isa, Elizabeth Kinsella, Ryan Halas, Andrey Leonov
Infective endocarditis (IE) results from bacterial or fungal infection and is associated with significant morbidity and mortality. Several known risk factors exist for endocarditis, and 90% of pediatric cases have an underlying structural or congenital heart disease or prosthetic heart valve. Literature on IE in previously healthy children is relatively sparse, and the pathogenesis and underlying risk factors remain mostly unknown. Our patient was a 3-year-old male with a unique presentation of IE. His lack of structural and congenital risk factors for endocarditis prompted further workup, and labs were consistent with insufficient immunoglobulin, suggesting a primary immunodeficiency (PAD)...
2018: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/29789917/comparison-of-clinical-profiles-in-patients-with-protein-losing-enteropathy-with-and-without-fontan-circulation
#11
Shin Ono, Hideo Ohuchi, Aya Miyazaki, Osamu Yamada
Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs...
May 22, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29786408/impact-of-additional-tricuspid-valve-annuloplasty-in-tof-patients-undergoing-pulmonary-valve-replacement
#12
Sabrina Lueck, Eike Bormann, Kathrin Rellensmann, Sven Martens, Andreas Rukosujew
BACKGROUND: Many patients with tetralogy of Fallot (TOF) who underwent surgical correction of their congenital cardiac malformation during infancy develop right ventricular dysfunction and exercise intolerance in the long term. The right ventricle (RV) dilates due to the development of severe pulmonary regurgitation (and secondary tricuspid insufficiency). To reduce RV dilation and improve exercise tolerance pulmonary valve replacement (PVR) is the common therapeutic strategy. Whether concomitant tricuspid valve repair (TVR) is beneficial in these pure volume-overload conditions is still unknown...
May 22, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29777670/the-impact-of-mitral-disease-etiology-on-operative-mortality-following-mitral-valve-operations
#13
J Scott Rankin, Maria Grau-Sepulveda, David M Shahian, A Marc Gillinov, Rakesh Suri, James S Gammie, Steven F Bolling, Patrick M McCarthy, Vinod H Thourani, Niv Ad, Sean M O'Brien, Jeffrey P Jacobs, Vinay Badhwar
BACKGROUND: The patho-etiology of mitral regurgitation (MR) has been suggested as a mediator of outcomes following mitral valve (MV) operations, particularly in ischemic functional mitral regurgitation (IMR). This study examined the independent association of MV etiology with mortality. METHODS: Using the Society of Thoracic Surgeons Database, all patients undergoing mitral valve replacement or repair from 2011-2014 were assessed. Patients who underwent concomitant surgical ablation, septal defect closure, tricuspid valve repair, or coronary artery bypass grafting were included...
May 16, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29777556/electrophysiology-and-structural-interventions-in-adults-with-congenital-heart-disease-comparison-of-combined-versus-separate-procedures
#14
Ian Lindsay, Farnoosh Nik-Ahd, Jamil A Aboulhosn, Jeremy P Moore
BACKGROUND: Electrophysiologic (EP) and structural interventions in adult congenital heart disease (ACHD) are typically completed during separate hospital encounters. With planning/coordination, these cases can be combined. OBJECTIVES: We hypothesized that this integrated approach would yield patient and health system benefits. METHODS: Consecutive ACHD patients undergoing combined interventions were matched to controls with identical but separate procedures...
May 18, 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29776810/congenital-heart-disease-in-adults-assessmentof-functional-capacity-using-cardiopulmonary-exercise-testing
#15
Sílvia Aguiar Rosa, Ana Agapito, Rui M Soares, Lídia Sousa, José Alberto Oliveira, Ana Abreu, Ana Sofia Silva, Sandra Alves, Helena Aidos, Fátima F Pinto, Rui Cruz Ferreira
AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction...
May 15, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29775406/phrase-mining-of-textual-data-to-analyze-extracellular-matrix-protein-patterns-across-cardiovascular-disease
#16
David Alexandre Liem, Sanjana Murali, Dibakar Sigdel, Yu Shi, Xuan Wang, Jiaming Shen, Howard Choi, J Harry Caufield, Wei Wang, Peipei Ping, Jiawei Han
Extracellular matrix (ECM) proteins have been shown to play important roles regulating multiple biological processes in an array of organ systems, including the cardiovascular system. By using a novel bioinformatics text-mining tool, we studied six categories of cardiovascular disease (CVD), namely ischemic heart disease (IHD), cardiomyopathies (CM), cerebrovascular accident (CVA), congenital heart disease (CHD), arrhythmias (ARR), and valve disease (VD), anticipating novel ECM protein-disease and protein-protein relationships hidden within vast quantities of textual data...
May 18, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#17
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770292/innovative-interventional-catheterization-techniques-for-congenital-heart-disease
#18
REVIEW
Jeffrey D Zampi, Wendy Whiteside
Since 1929, when the first cardiac catheterization was safely performed in a human by Dr. Werner Forssmann (on himself), there has been a rapid progression of cardiac catheterization techniques and technologies. Today, these advances allow us to treat a wide variety of patients with congenital heart disease using minimally invasive techniques; from fetus to infants to adults, and from simple to complex congenital cardiac lesions. In this article, we will explore some of the exciting advances in cardiac catheterization for the treatment of congenital heart disease, including transcatheter valve implantation, hybrid procedures, biodegradable technologies, and magnetic resonance imaging (MRI)-guided catheterization...
April 2018: Translational pediatrics
https://www.readbyqxmd.com/read/29767458/overlapping-but-distinct-roles-for-notch-receptors-in-human-cardiovascular-disease
#19
REVIEW
J A N Meester, A Verstraeten, M Alaerts, D Schepers, L Van Laer, B L Loeys
The NOTCH signalling pathway is an essential pathway, involved in many cellular processes, including cell fate decision, cell proliferation, and cell death and important in the development of most organs. Mutations in genes encoding components of the NOTCH signalling pathway lead to a spectrum of congenital disorders. Over the past decades mutations in human NOTCH signalling genes have been identified in several diseases with cardiovascular involvement. NOTCH1 mutations have been described in bicuspid aortic valve disease, left-sided congenital heart disease, and Adams-Oliver syndrome...
May 16, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29756160/fetal-and-postnatal-echocardiographic-diagnosis-of-ebstein-anomaly-of-the-mitral-valve
#20
Safwat Aly, John Bokowski, Karim Diab, Brie Ann Muller
Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement into LV cavity. Postnatal transthoracic and transesophageal echocardiograms confirmed the diagnosis with apical displacement of the level of coaptation MV into the LV cavity. To the best of our knowledge, fetal diagnosis of Ebstein anomaly of MV has not yet been reported in the medical literature...
May 14, 2018: Pediatric Cardiology
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