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Cyanotic heart disease

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https://www.readbyqxmd.com/read/28100970/sickle-cell-disease-with-cyanotic-congenital-heart-disease-long-term-outcomes-in-5-children
#1
Glen J Iannucci, Olufolake A Adisa, Matthew E Oster, Michael McConnell, William T Mahle
Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28100569/multiple-pregnancy-in-a-primigravida-with-uncorrected-pentalogy-of-fallot
#2
Pamela Partana, Jarrod Kah Hwee Tan, Ju Le Tan, Lay Kok Tan
Pentalogy of Fallot is a cyanotic congenital heart disease that has guarded prognosis without surgical intervention in infancy. Women with uncorrected defects rarely survive into childbearing age and pregnancy in this group is associated with a high rate of perinatal loss. Physiological cardiovascular changes in pregnancy can lead to maternal haemodynamic instability with subsequent adverse cardiac sequelae with or without fetal decompensation. Optimum management and pregnancy outcomes in mother with uncorrected Pentalogy of Fallot and twin pregnancy have not been described in the literature...
January 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28094126/prevalence-of-neuroendocrine-tumors-in-patients-with-cyanotic-congenital-heart-disease
#3
Inés Ponz de Antonio, José Ruiz Cantador, Ana E González García, José María Oliver Ruiz, Ángel Sánchez-Recalde, José Luis López-Sendón
No abstract text is available yet for this article.
January 13, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28077182/functional-tricuspid-stenosis-a-rare-presentation-of-suspected-rhabdomyoma-as-congenital-cyanotic-heart-disease
#4
Anishkumar Nair, Gopalan Nair Rajesh, Chakanalil Govindan Sajeev
Cardiac tumours in newborns are often asymptomatic and can be sporadically detected on routine screening unless they result in intractable arrhythmias or haemodynamically significant obstructions causing heart failure. Their presentation as a cause of congenital cyanosis is never anticipated. We report a rare case of a newborn presenting with congenital cyanosis consequent to suspected cardiac rhabdomyoma causing tricuspid inflow obstruction. Our experience with this patient with two large cardiac masses illustrates the significance of its inclusion in the differential diagnosis of perinatal cyanosis, as early detection and surgical management might be the only lifesaving options, if performed well in time...
January 12, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#5
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28068786/topical-and-low-dose-intravenous-tranexamic-acid-in-cyanotic-cardiac-surgery
#6
Jigar Patel, Mrugesh Prajapati, Hardik Patel, Hemang Gandhi, Shilpa Deodhar, Himani Pandya
Background Coagulopathy is a major problem in surgery for cyanotic congenital heart disease. Tranexamic acid has been used both topically and systemically and plays a vital role in pediatric cardiac surgery by reducing blood loss and blood product requirement. We aimed to determine the anti-fibrinolytic effectiveness of low-dose systemic or topical tranexamic acid or a combination of both. Methods Seventy-five patients were divided in 3 groups of 25. Group A patients were given tranexamic acid 20 mg kg(-1) intravenously after sternotomy and 20 mg kg(-1) after heparin reversal...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28033078/the-accuracy-of-noninvasive-peripheral-pulse-oximetry-after-palliative-cardiac-surgery-in-patients-with-cyanotic-congenital-heart-disease
#7
Gemma E Scrimgeour, Michael J Griksaitis, John V Pappachan, Andrew J Baldock
BACKGROUND: Children with cyanotic congenital heart disease (CCHD) live with oxyhemoglobin saturations that are typically expressed as percentages in the range of 70s and 80s. Peripheral pulse oximetry (measurement of SpO2) performs poorly in this range and yet is widely used to inform clinical decisions in these patients. The reference standard is co-oximetry of arterial samples (SaO2). METHODS: In this study, 515 paired measurements of SpO2 and SaO2 were taken from 19 children who had undergone palliative cardiac surgery...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28032672/hippocampal-damage-and-memory-impairment-in-congenital-cyanotic-heart-disease
#8
Mónica Muñoz-López, Aparna Hoskote, Martin J Chadwick, Anna M Dzieciol, David G Gadian, Kling Chong, Tina Banks, Michelle de Haan, Torsten Baldeweg, Mortimer Mishkin, Faraneh Vargha-Khadem
Neonatal hypoxia can lead to hippocampal atrophy, which can lead, in turn, to memory impairment. To test the generalisability of this causal sequence, we examined a cohort of 41 children aged 8-16, who, having received the arterial switch operation to correct for transposition of the great arteries, had sustained significant neonatal cyanosis but were otherwise neurodevelopmentally normal. As predicted, the cohort had significant bilateral reduction of hippocampal volumes relative to the volumes of 64 normal controls...
December 29, 2016: Hippocampus
https://www.readbyqxmd.com/read/28031653/complications-after-a-bidirectional-cavopulmonary-anastomosis-with-accessory-sources-of-pulmonary-blood-flow
#9
Efrén Martínez-Quintana, Fayna Rodríguez-González
Creating an accessory source of pulsatile pulmonary blood flow in a patient with a bidirectional cavopulmonary anastomosis may have advantages and disadvantages. In relation to the latter, we report the complications seen in a cyanotic congenital heart disease patient who developed a superior vena cava syndrome plus severe swelling of his right hand that evolved satisfactorily after percutaneous and medical treatment.
December 2016: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/27978862/serum-c-reactive-protein-levels-and-body-mass-index-in-children-and-adolescents-with-chd
#10
Maíra Ribas Goulart, Daniela Schneid Schuh, David W Moraes, Sandra Mari Barbiero, Lucia Campos Pellanda
BACKGROUND: The prevalence of overweight in children with CHD is about 26.9%. Increase in adipose tissue is related to the secretion of proinflammatory markers such as C-reactive protein. Assuming that children with CHD are exposed to other inherent risk factors for heart disease, our objective was to evaluate the correlation between levels of C-reactive protein and body mass index in children and adolescents with CHD. METHODS: A cross-sectional study with 377 children and adolescents with CHD in a clinical setting of a reference hospital was carried out...
December 16, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27977539/delirium-in-children-after-cardiac-bypass-surgery
#11
Anita K Patel, Katherine V Biagas, Eunice C Clarke, Linda M Gerber, Elizabeth Mauer, Gabrielle Silver, Paul Chai, Rozelle Corda, Chani Traube
OBJECTIVES: To describe the incidence of delirium in pediatric patients after cardiac bypass surgery and explore associated risk factors and effect of delirium on in-hospital outcomes. DESIGN: Prospective observational single-center study. SETTING: Fourteen-bed pediatric cardiothoracic ICU. PATIENTS: One hundred ninety-four consecutive admissions following cardiac bypass surgery, 1 day to 21 years old. INTERVENTIONS: Subjects were screened for delirium daily using the Cornell Assessment of Pediatric Delirium...
December 13, 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27957813/neurocognitive-functioning-in-adults-with-congenital-heart-disease
#12
Dawn Ilardi, Kim E Ono, Rebecca McCartney, Wendy Book, Anthony Y Stringer
OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD...
December 13, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27935164/kaolin-activated-thromboelastography-and-standard-coagulation-assays-in-cyanotic-and-acyanotic-infants-undergoing-complex-cardiac-surgery-a-prospective-cohort-study
#13
Alessandra Rizza, Zaccaria Ricci, Chiara Pezzella, Isabella Favia, Giovina Di Felice, Marco Ranucci, Paola Cogo
BACKGROUND AND AIMS: Several studies report the use of thromboelatography (TEG) to monitor coagulation in pediatric cardiac surgery. The aim of this study was to compare baseline and intraoperative TEG, TEG-functional fibrinogen, and standard coagulation assays in children with cyanotic and acyanotic congenital heart disease (CHD) undergoing cardiac surgery. METHODS: This is a prospective observational study of 63 children aged <24 months undergoing cardiac surgery with cardiopulmonary bypass (CPB)...
February 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27928498/case-report-emergency-awake-craniotomy-for-cerebral-abscess-in-a-patient-with-unrepaired-cyanotic-congenital-heart-disease
#14
Corinne D'Antico, André Hofer, Jens Fassl, Daniel Tobler, Daniel Zumofen, Nicolai Goettel
We report the case of a 39-year-old male with complex cyanotic congenital heart disease undergoing emergency craniotomy for a cerebral abscess. Maintenance of intraoperative hemodynamic stability and adequate tissue oxygenation during anesthesia may be challenging in patients with cyanotic congenital heart disease. In this case, we decided to perform the surgery as an awake craniotomy after interdisciplinary consensus. We discuss general aspects of anesthetic management during awake craniotomy and specific concerns in the perioperative care of patients with congenital heart disease...
2016: F1000Research
https://www.readbyqxmd.com/read/27928258/evaluation-of-pulse-oximetry-in-the-early-detection-of-cyanotic-congenital-heart-disease-in-newborns
#15
Amir Hosein Movahedian, Ziba Mosayebi, Setareh Sagheb
Background: Delayed or missed diagnosis of critical and cyanotic congenital heart disease (CHD) in asymptomatic newborns may result in significant morbidity and mortality. The aim of this study was to determine the accuracy of pulse oximetry screening performed on the first day of life for the early detection of critical and cyanotic CHD in apparently normal newborns. Methods: This cross-sectional study used postductal pulse oximetry to evaluate term neonates born between 2008 and 2011 with normal physical examinations...
April 13, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/27913766/myocardial-cytochrome-oxidase-activity-increases-with-age-and-hypoxemia-in-patients-with-congenital-heart-disease
#16
Michael Onwugbufor, Richard J Levy, David Zurakowski, Richard A Jonas, Pranava Sinha
BACKGROUND: Myocardial tolerance to ischemia is influenced by age and preoperative cyanosis through unknown mechanisms and significantly affects postoperative outcomes. Cytochrome c oxidase (CcOx), the terminal enzyme of the mitochondrial electron transport chain, may play a role in the susceptibility to ischemic-reperfusion (IR) injury. Our study aimed at investigating changes in human myocardial CcOx activity based on age and preoperative oxygen saturation to understand its role in transition from neonatal to mature myocardium and hypoxic conditions...
December 1, 2016: Perfusion
https://www.readbyqxmd.com/read/27904570/existence-of-mutations-in-the-homeodomain-encoding-region-of-nkx2-5-gene-in-iranian-patients-with-tetralogy-of-fallot
#17
Majid Kheirollahi, Fereshteh Khosravi, Saeideh Ashouri, Alireza Ahmadi
BACKGROUND: Tetralogy of Fallot (TOF), the most common cyanotic heart defect and one of the most common congenital heart diseases, occurs mostly sporadically and nonsyndromically. The underlying molecular genetic mechanism is not known. Therefore, the existence of mutations in the homeodomain-encoding region of NKX2.5 gene in Iranian patients with tetralogy of Fallot is evaluated. MATERIALS AND METHODS: In the present study, we analyzed the peripheral blood samples of27 patients in order to find any mutation in the 180 bp homeodomain-encoding region of NKX2...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/27899861/echocardiographic-follow-up-of-patent-foramen-ovale-and-the-factors-affecting-spontaneous-closure
#18
Ali Yildirim, Alperen Aydin, Tevfik Demir, Fatma Aydin, Birsen Ucar, Zubeyir Kilic
BACKGROUND: The aim of the present study was to evaluate the echocardiographic follow-up of patent foramen ovale, which is considered a potential etiological factor in various diseases, and to determine the factors affecting spontaneous closure. METHODS: Between January 2000 and June 2012, records of 918 patients with patent foramen ovale were retrospectively reviewed. Patency of less than 3 mm around the fossa ovalis is called patent foramen ovale. Patients with cyanotic congenital heart diseases, severe heart valve disorders and severe hemodynamic left to right shunts were excluded from the study...
November 2016: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27878778/elevated-serum-levels-of-ghrelin-and-tnf-%C3%AE-in-patients-with-cyanotic-and-acyanotic-congenital-heart-disease
#19
Sai Zhang, Gong-Liang Guo, Li-Li Yang, Li-Qun Sun
BACKGROUND: The levels of ghrelin and tumor necrosis factor alpha (TNF-α) are considered biological markers of congenital heart diseases (CHD). The present meta-analysis was conducted to investigate the clinical significance of serum levels of ghrelin and TNF-α in children with (CHD). METHODS: Chinese and English scientific literature databases were searched to retrieve published studies relevant to ghrelin, TNF-α and CHD. Manual search was additionally employed to identify other relevant studies from cross-references...
November 23, 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27861982/following-up-adult-patients-with-tetralogy-of-fallot-the-role-of-echocardiography
#20
REVIEW
Ana Maria Daraban, Ruxandra Jurcuţ, Elisabeta Bădilă, Daniela Bartoş, Gheorghe Andrei Dan
Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases. With surgical repair and the advances in postoperative care, contemporary mortality has dramatically improved and an increasing number of patients survive into adulthood, leading to a growing number of adult TOF. However, residual anatomic and hemodynamic abnormalities are encountered in nearly all patients, making follow-up mandatory. Furthermore, mortality starts to increase 25 years after surgery, emphasizing that, in adult TOF, closer monitoring is necessary...
November 10, 2016: Journal of Clinical Ultrasound: JCU
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