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https://www.readbyqxmd.com/read/28331759/persistent-left-superior-vena-cava-incidentally-recognized-postoperatively-after-venous-port-placement
#1
Dauren Sarsenov, Levent Onat, Vahit Özmen
Persistent left superior vena cava is the most common congenital venous anomaly of the thoracic venous system, occurring in 0.3% to 0.5% of individuals in the general population. It may remain asymptomatic throughout life and be incidentally found in healthy individuals undergoing vascular procedures such as venous access device placements and endovascular cardiac interventions. Here we present a case of persistent left superior vena cava incidentally realized during chemoport insertion in a patient with breast cancer...
October 2016: J Breast Health (2013)
https://www.readbyqxmd.com/read/28331629/congenital-diaphragmatic-hernia-a-review
#2
REVIEW
Praveen Kumar Chandrasekharan, Munmun Rawat, Rajeshwari Madappa, David H Rothstein, Satyan Lakshminrusimha
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/28329459/nineteen-years-of-adult-congenital-heart-surgery-in-a-single-center
#3
Madurra Perinpanayagam, Signe H Larsen, Kristian Emmertsen, Marianne B Møller, Vibeke E Hjortdal
BACKGROUND: Adults with congenital heart disease are a growing population. We describe surgical interventions, short- and long-term mortality and morbidity, and risk factors for adverse events in a population-based cohort. METHODS: Patients over or equal to 18 years with congenital heart disease who underwent cardiac surgery at Aarhus University Hospital, Denmark, from 1994 to 2012 were included in the study. Diagnoses, surgical procedures, postoperative complications, and survival were identified in hospital databases, medical records, and the Danish Civil Registration System...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329454/adult-congenital-cardiac-care
#4
Brian E Kogon, Kati Miller, Paula Miller, Bahaaldin Alsoufi, Joshua M Rosenblum
BACKGROUND: The Adult Congenital Heart Association (ACHA) is dedicated to supporting patients with congenital heart disease. To guide patients to qualified providers and programs, it maintains a publicly accessible directory of dedicated adult congenital cardiac programs. We analyzed the directory in 2006 and 2015, aiming to evaluate the growth of the directory as a whole and to evaluate the growth of individual programs within the directory. We also hope this raises awareness of the growing opportunities that exist in adult congenital cardiology and cardiac surgery...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329265/the-hemi-mustard-bidirectional-glenn-and-rastelli-procedures-for-anatomical-repair-of-congenitally-corrected-transposition-of-the-great-arteries-left-ventricular-outflow-tract-obstruction-with-positional-heart-anomalies%C3%A2
#5
Sen Zhang, Kai Ma, Shoujun Li, Zhongdong Hua, Hao Zhang, Jun Yan, Keming Yang, Kunjing Pang, Xu Wang, Lei Qi, Qiuming Chen
OBJECTIVES: The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies...
February 27, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28329231/cardiac-protective-effects-of-remote-ischaemic-preconditioning-in-children-undergoing-tetralogy-of-fallot-repair-surgery-a-randomized-controlled-trial
#6
Qingping Wu, Tingting Wang, Shiqiang Chen, Quanjun Zhou, Haobo Li, Na Hu, Yinglu Feng, Nianguo Dong, Shanglong Yao, Zhengyuan Xia
Aims: Remote ischaemic preconditioning (RIPC) by inducing brief ischaemia in distant tissues protects the heart against myocardial ischaemia-reperfusion injury (IRI) in children undergoing open-heart surgery, although its effectiveness in adults with comorbidities is controversial. The effectiveness and mechanism of RIPC with respect to myocardial IRI in children with tetralogy of Fallot (ToF), a severe cyanotic congenital cardiac disease, undergoing open heart surgery are unclear. We hypothesized that RIPC can confer cardioprotection in children undergoing ToF repair surgery...
February 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28329107/a-ferromagnetic-surgical-system-reduces-phrenic-nerve-injury-in-redo-congenital-cardiac-surgery
#7
Takeshi Shinkawa, Jessica Holloway, Xinyu Tang, Jeffrey M Gossett, Michiaki Imamura
A ferromagnetic surgical system (FMwand®) is a new type of dissection device expected to reduce the risk of adjacent tissue damage. We reviewed 426 congenital cardiac operations with cardiopulmonary bypass through redo sternotomy to assess if this device prevented phrenic nerve injury. The ferromagnetic surgical system was used in 203 operations (47.7%) with regular electrocautery and scissors. The preoperative and operative details were similar between the operations with or without the ferromagnetic surgical system...
January 31, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329105/medical-three-dimensional-printing-opens-up-new-opportunities-in-cardiology-and-cardiac-surgery
#8
Thomas Bartel, Andrew Rivard, Alejandro Jimenez, Carlos A Mestres, Silvana Müller
Advanced percutaneous and surgical procedures in structural and congenital heart disease require precise pre-procedural planning and continuous quality control. Although current imaging modalities and post-processing software assists with peri-procedural guidance, their capabilities for spatial conceptualization remain limited in two- and three-dimensional representations. In contrast, 3D printing offers not only improved visualization for procedural planning, but provides substantial information on the accuracy of surgical reconstruction and device implantations...
February 16, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28328585/mechanical-support-with-impella-during-malignant-arrhythmia-ablation-a-case-report-on-the-growing-trend-in-the-electrophysiology-laboratory
#9
Adam C Adler, Ramesh Kodavatiganti
Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease in which the persistence of the right ventricle as the systemic ventricle leads to heart failure, tricuspid valve insufficiency, and arrhythmia. Supraventricular arrhythmias are especially common in these patients. We discuss the anesthetic management of a 33-year-old patient with congenitally corrected transposition of the great arteries who required a ventricular assist device to maintain cardiac output during ablation of supraventricular tachyarrythmia...
March 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28328555/association-of-pediatric-cardiac-surgical-volume-and-mortality-after-cardiac-ecmo
#10
Cindy S Barrett, Titus T Chan, Jacob Wilkes, Susan L Bratton, Ravi R Thiagarajan
Centers with higher surgical and ECMO volume have improved survival for children undergoing pediatric cardiac surgery and ECMO respectively. We examined the relationship between both cardiac surgical and cardiac ECMO volumes, with survival. Using data from the Pediatric Health Information System, we reviewed patients who underwent ECMO during the hospitalization for cardiac surgery or heart transplantation from January 2003- June 2014. Among 106,967 patients in 43 centers undergoing a Risk Adjustment for Congenital Heart Surgery-1 (RACHS) 1-6 procedure (n =104,951) and/or cardiac transplantation (n=2016), 2...
March 20, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28328125/two-patients-with-the-heterozygous-r189h-mutation-in-acta2-and-complex-congenital-heart-defects-expands-the-cardiac-phenotype-of-multisystemic-smooth-muscle-dysfunction-syndrome
#11
Thushiha Logeswaran, Christoph Friedburg, Karoline Hofmann, Hakan Akintuerk, Saskia Biskup, Michael Graef, Ali Rad, Axel Weber, Bernd A Neubauer, Dietmar Schranz, Patrice Bouvagnet, Birgit Lorenz, Andreas Hahn
De novo heterozygous mutations changing R179 to histidine, leucine, or cysteine in the ACTA2 gene are associated with Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS). Characteristic hallmarks of this condition, caused only by these specific ACTA2 mutations, are congenital mydriasis (mid-dilated, non-reactive pupils), a large persistent ductus arteriosus (PDA), aortic aneurysms evolving during childhood, and cerebrovascular anomalies. We describe two patients, a 3-day-old newborn and a 26-year-old woman, with this unique mutation in association with a huge PDA and an aorto-pulmonary window...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28327570/puf60-variants-cause-a-syndrome-of-id-short-stature-microcephaly-coloboma-craniofacial-cardiac-renal-and-spinal-features
#12
Karen J Low, Morad Ansari, Rami Abou Jamra, Angus Clarke, Salima El Chehadeh, David R FitzPatrick, Mark Greenslade, Alex Henderson, Jane Hurst, Kory Keller, Paul Kuentz, Trine Prescott, Franziska Roessler, Kaja K Selmer, Michael C Schneider, Fiona Stewart, Katrina Tatton-Brown, Julien Thevenon, Magnus D Vigeland, Julie Vogt, Marjolaine Willems, Jonathan Zonana, D D D Study, Sarah F Smithson
PUF60 encodes a nucleic acid-binding protein, a component of multimeric complexes regulating RNA splicing and transcription. In 2013, patients with microdeletions of chromosome 8q24.3 including PUF60 were found to have developmental delay, microcephaly, craniofacial, renal and cardiac defects. Very similar phenotypes have been described in six patients with variants in PUF60, suggesting that it underlies the syndrome. We report 12 additional patients with PUF60 variants who were ascertained using exome sequencing: six through the Deciphering Developmental Disorders Study and six through similar projects...
March 22, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28326958/persistent-left-superior-vena-cava
#13
Vrinda Nair, Kamran Yusuf, Weiming Yu, Hafez AlAwad, Kathy Paul, Essa Al Awad
Persistent left superior vena cava (PLSVC) is a common cardiac anomaly associated with congenital heart diseases. A diagnosis of PLSVC usually warrants a detailed fetal echocardiography. Lesser known associations are the extra cardiac anomalies notably the upper airway and the gastrointestinal tract anomalies. We highlight here the importance of detailed fetal assessment for extra cardiac anomalies in addition to fetal echocardiography in fetuses diagnosed with PLSVC. We hereby present a preterm infant who presented with a triad of PLSVC, laryngeal atresia, and esophageal atresia...
March 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28326155/dental-plaque-microbial-profiles-of-children-from-khartoum-sudan-with-congenital-heart-defects
#14
Hiba Mohamed Ali, Ellen Berggreen, Daniel Nguyen, Raouf Wahab Ali, Thomas E Van Dyke, Hatice Hasturk, Manal Mustafa
Few studies have focused on the bacterial species associated with the deterioration of the dental and gingival health of children with congenital heart defects (CHD). The aims of this study were (1) to examine the dental plaque of children with CHD in order to quantify bacterial load and altered bacterial composition compared with children without CHD; and (2) to investigate the correlation between the level of caries and gingivitis and dental biofilm bacteria among those children. In this cross-sectional study, participants were children (3-12 years) recruited in Khartoum State, Sudan...
2017: Journal of Oral Microbiology
https://www.readbyqxmd.com/read/28325567/microvascular-surgery-in-the-congenital-cardiac-patient-a-case-series-exploring-feasibility-and-practical-applications
#15
John A LoGiudice, Karri Adamson, Nancy Ghanayem, Ronald K Woods, Michael E Mitchell
BACKGROUND: Pediatric congenital heart disease patients are at risk for vascular injuries during surgical procedures or when the arterial system is accessed for monitoring or diagnostic studies. Our treatment of emergent situations in this patient population using microvascular techniques shows the feasibility of such techniques. METHODS: A retrospective chart review of patients aged 0-18 years with congenital heart disease identified six patients who underwent microvascular surgery by the senior surgeon from June 2007 to May 2015...
February 17, 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/28325177/equine-cardiovascular-therapeutics
#16
REVIEW
Meg M Sleeper
Heart disease can be defined as any abnormality of the heart whether it is a cardiac dysrhythmia or structural heart disease, either congenital or acquired. Heart failure occurs when a cardiac abnormality results in the inability of the heart to pump enough blood to meet the body's needs. Heart disease can be present without leading to heart failure. Heart failure, however, is a consequence of heart disease. There are 4 main areas where the clinician can intervene to improve cardiac output with heart failure: preload, afterload, myocardial contractility, and heart rate...
April 2017: Veterinary Clinics of North America. Equine Practice
https://www.readbyqxmd.com/read/28322432/bilateral-congenital-deafness-what-investigations-should-be-performed
#17
Nicolas Gürtler, Claudine Gysin, Nevenka Schmid, Claudia Pieren, Mattheus Vischer, Stefan Schumacher, Peter Oppermann, Daniel Leuba, Dorothée Veraguth
BACKGROUND: The introduction of newborn hearing screening has led to earlier identification of children with congenital sensorineural hearing loss (SNHL). Aetiological clarification offers several benefits. There is currently a lack of agreement on which examinations should be recommended. OBJECTIVE: Descriptive review of the literature reporting investigations performed to establish the aetiology of congenital SNHL and comparison of the management policy in Swiss referral centres...
March 21, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28322033/prenatal-diagnosis-of-aberrant-right-subclavian-artery-in-an-unselected-population
#18
Mi Jin Song, Byoung Hee Han, Young-Hwa Kim, So Young Yoon, Yoo Mi Lee, Hye Su Jeon, Bo Kyung Park
Purpose: The purpose of this study was to determine the frequency of aberrant right subclavian artery (ARSA) among unselected fetuses and to evaluate its association with chromosomal abnormalities and other congenital anomalies. Methods: In all, 7,547 fetuses (gestational age, 20 to 34 weeks) were examined using routine antenatal sonography at our institution between April 2014 and September 2015. The right subclavian artery was assessed using grayscale and color Doppler ultrasonography in the transverse 3-vessel and tracheal view, and confirmed in the coronal plane...
February 20, 2017: Ultrasonography
https://www.readbyqxmd.com/read/28321396/popular-hybrid-congenital-heart-procedures-without-cardiopulmonary-bypass
#19
REVIEW
Aamisha Gupta, Zahid Amin
As surgical and catheter interventions advance, patients with congenital heart disease are now offered alternative treatment options that cater to their individual needs. Furthermore, collaboration between interventional cardiologists and cardiac surgeons have led to the development of hybrid procedures, using the best techniques of each respective field to treat these complex cardiac entities from initial treatment in the pediatric patient to repeat intervention in the adult. We present a review of the increased popularity and trend in hybrid procedures in congenital heart disease without the use of cardiopulmonary bypass...
2017: Frontiers in Surgery
https://www.readbyqxmd.com/read/28321308/late-presentation-of-an-anomalous-left-coronary-artery-from-the-pulmonary-artery-treated-with-conservative-surgical-management-with-long-term-cardiac-magnetic-resonance-imaging-follow-up
#20
Pishoy Gouda, John Gouda, Craig Butler, Robert C Welsh
Anomalous origin of the left coronary artery from the pulmonary artery is rare congenital abnormality that most commonly presents in childhood and is associated with a high mortality. In the elderly, patients may present acutely with arrhythmias or signs of ischemia or with vague chronic presentations of shortness of breath and fatigue. In the high-risk elderly population, it is unclear as to whether conservative surgical management by means of suture ligation of the left coronary artery is associated with positive long-term outcomes...
2017: SAGE open medical case reports
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