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Cyanotic heart disease

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https://www.readbyqxmd.com/read/28923474/beyond-the-inhaled-nitric-oxide-in-persistent-pulmonary-hypertension-of-the-newborn
#1
REVIEW
Mei-Yin Lai, Shih-Ming Chu, Satyan Lakshminrusimha, Hung-Chih Lin
Persistent pulmonary hypertension (PPHN) is a consequence of failed pulmonary vascular transition at birth and leads to pulmonary hypertension with shunting of deoxygenated blood across the ductus arteriosus (DA) and foramen ovale (FO) resulting in severe hypoxemia, and it may eventually lead to life-threatening circulatory failure. PPHN is a serious event affecting both term and preterm infants in the neonatal intensive care unit. It is often associated with diseases such as congenital diaphragmatic hernia, meconium aspiration, sepsis, congenital pneumonia, birth asphyxia and respiratory distress syndrome...
August 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28920017/esophageal-atresia-with-tracheo-esophageal-fistula-presenting-beyond-7-days
#2
Nilesh Nagdeve, Mohini Sukhdeve, Tushar Thakre, Suresh Morey
AIM: To describe our experience of neonates with esophageal atresia with tracheo-esophageal fistula (EA with TEF) who presented after a week. DESIGN: Retrospective study of the patients of EA with TEF who presented after a week. STUDY SETTING: Department of Pediatric Surgery, Government Medical College Nagpur. Study Duration: Eight years. MATERIALS AND METHODS: Demographic information, hematological, biochemical and radiological data were obtained from the patients' medical records...
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28903840/burr-hole-aspiration-of-brain-abscess-in-children-with-cyanotic-heart-disease
#3
Muhammad Ashraf, Shakeel Ahmed, Shafiq Ahmad, Mukhtar Hussain
OBJECTIVE: To determine the efficacy of burr hole aspiration of brain abscess in children with cyanotic heart disease in terms of number of aspirations and residual abscess. STUDY DESIGN: Experimental study. PLACE AND DURATION OF STUDY: Department of Pediatric Neurosurgery at The Children's Hospital and The Institute of Child Health, Multan, from July 2010 to June 2014. METHODOLOGY: Pediatric patients of cyanotic heart disease with brain abscess were admitted...
August 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28890875/cyanotic-congenital-heart-diseases-among-nigerian-children
#4
Barakat Adeola Animasahun, Akpoembele Deborah Madise-Wobo, Olusola Yejide Kusimo
BACKGROUND: There are only few reports on cyanotic congenital heart diseases (CCHD) among Nigerian and African Children. The current report aim to provide the most recent hospital based data on the distribution of CCHD in children less than 14 years of age, the demographic characteristics and risk factors identified. METHODS: Prospective and cross-sectional involving consecutive cases of CCHD diagnosed with echocardiography at the Lagos State University Teaching Hospital between January 2007 and June 2016...
August 2017: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/28880458/determinants-of-platelet-count-in-pediatric-patients-with-congenital-cyanotic-heart-disease-role-of-immature-platelet-fraction
#5
Randa M Matter, Iman A Ragab, Alaa M Roushdy, Ahmed G Ahmed, Hanan H Aly, Eman A Ismail
OBJECTIVES: Congenital heart defects are common noninfectious causes of mortality in children. Bleeding and thrombosis are both limiting factors in the management of such patients. We assessed the frequency of thrombocytopenia in pediatric patients with congenital cyanotic heart disease (CCHD) and evaluated determinants of platelet count including immature platelet fraction (IPF) and their role in the pathogenesis of thrombocytopenia. METHODS: Forty-six children and adolescents with CCHD during pre-catheter visits were studied; median age was 20...
September 7, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28878122/wnt11-regulates-cardiac-chamber-development-and-disease-during-perinatal-maturation
#6
Marlin Touma, Xuedong Kang, Fuying Gao, Yan Zhao, Ashley A Cass, Reshma Biniwale, Xinshu Xiao, Mansuoreh Eghbali, Giovanni Coppola, Brian Reemtsen, Yibin Wang
Ventricular chamber growth and development during perinatal circulatory transition is critical for functional adaptation of the heart. However, the chamber-specific programs of neonatal heart growth are poorly understood. We used integrated systems genomic and functional biology analyses of the perinatal chamber specific transcriptome and we identified Wnt11 as a prominent regulator of chamber-specific proliferation. Importantly, downregulation of Wnt11 expression was associated with cyanotic congenital heart defect (CHD) phenotypes and correlated with O2 saturation levels in hypoxemic infants with Tetralogy of Fallot (TOF)...
September 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/28867175/thrombo-hemorrhagic-liability-in-children-with-congenital-heart-diseases
#7
REVIEW
Shebl Said Shebl, Walid Ahmed Naguib El-Shehaby, Amira Hamed Darwish, Yasmin Shebl Said, Nabeh Helal Elfadaly, Eman Amer
BACKGROUND: The precise mechanisms of the increased incidence of hemostatic abnormalities in congenital heart disease (CHD) have not been determined. The aim of the study was to evaluate some indicators of activation of platelets and vascular endothelial cells in patients with CHD, evaluation of bleeding liability of these patients, and correlation with the clinical presentation of these patients. METHODS: This work was carried out on 20 patients with cyanotic congenital heart disease (CCHD), 20 patients with cyanotic congenital heart disease (ACHD), and 20 healthy children who served as the control group, aged between 1 and 10years...
August 26, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28866869/total-anomalous-pulmonary-venous-connection-tapvc-a%C3%A2-familial-cluster-of-3-siblings
#8
Jennifer M Acevedo, Simon Lee, Nina Gotteiner, Amy S Lay, Angira Patel
Total anomalous pulmonary venous connection (TAPVC) is a rare form of cyanotic congenital heart disease (CHD) that occurs when the pulmonary veins drain into a site other than the morphologic left atrium. As with other forms of CHD, TAPVC has been shown to cluster in families and is known to have genetic association. We report on a case series of familial TAPVC in three consecutive siblings. A combination of fetal echocardiography, transthoracic echocardiography, as well as cross-sectional imaging was utilized in the diagnosis as well as management of each sibling...
September 3, 2017: Echocardiography
https://www.readbyqxmd.com/read/28828743/pregnancy-in-women-with-congenital-heart-disease
#9
REVIEW
Evin Yucel, Doreen DeFaria Yeh
Advances in cardiac surgical interventions in infancy and childhood have led to an increased number of women with congenital heart disease of childbearing age. For these women, individualized preconception counseling and pregnancy planning should be a vital component of their medical management, and presentation for obstetric care may even be an opportunity to re-establish cardiovascular care for patients who have been lost to follow-up. These patients have unique cardiovascular anatomy and physiology, which is dependent upon the surgical intervention they may have undergone during childhood or adolescence...
August 22, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28811093/risk-factors-for-infective-endocarditis-in-children-with-congenital-heart-diseases-a-nationwide-population-based-case-control-study
#10
Li-Chuan Sun, Chih-Cheng Lai, Cheng-Yi Wang, Ya-Hui Wang, Jen-Yu Wang, Yo-Ling Hsu, Yin-Lan Hu, En-Ting Wu, Ming-Tai Lin, Leticia B Sy, Likwang Chen
BACKGROUND: Infective endocarditis (IE) is uncommon in childhood. Its associated epidemiological characteristics in patients with congenital heart disease (CHD) remain unclear. METHODS: The study population included children born in Taiwan during the years 1997 to 2005 who were diagnosed as having CHD before 3years of age. All children were followed up until the end year of 2010, the diagnosis of infective endocarditis, or death. The demographic characteristics of patients with and without IE, the invasive procedures performed during 6months before the index date, the prophylactic antibiotics related to dental procedures, and in-hospital mortality were collected...
August 12, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28794526/effect-of-ketamine-on-pro-and-anti-inflammatory-cytokine-response-in-paediatric-cardiac-surgery-a-prospective-randomised-controlled-study
#11
Tamer Hamed Ibrahim, Hassan Saad Abdelrahman, Mohammed A Alharbi, Ibrahim A Zabani, Mohamed Fouad Ismail, Heba Kary
BACKGROUND AND AIMS: Paediatric cardiac surgery with cardiopulmonary bypass (CPB) is associated with a marked inflammatory response and triggers release of inflammatory cytokines. The aim of this study was to study the effect of ketamine on the inflammatory response during correction of congenital cyanotic heart diseases. METHODS: Sixty-six patients with congenital cyanotic heart diseases scheduled for cardiac surgery were randomised into three groups. Group A patients did not receive ketamine (control group), Group B patients received 2 mg/kg ketamine intravenous (IV) and Group C patients received ketamine 2 mg/kg IV and an IV infusion of ketamine (50 μg/kg/min)...
July 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28780403/forgotten-not-yet-cardiogenic-brain-abscess-in-children-a-case-series-based-review
#12
Suhas Udayakumaran, Chiazor U Onyia, R Krishnakumar
INTRODUCTION: Brain abscess is a significant cause of morbidity in patients with uncorrected or partially palliated congenital cyanotic heart disease (CCHD). Unfortunately, in the developing world, the majority of the patients with CCHD remain either uncorrected or partially palliated. Furthermore, a risk of this feared complication also exists even among those undergoing staged corrective operations in the interval in between operations. There have been no recent articles in the literature on surgical outcomes of management of cardiogenic brain abscess in children...
August 2, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28713984/downregulation-of-microrna%C3%A2-199a%C3%A2-5p-protects-cardiomyocytes-in-cyanotic-congenital-heart-disease-by-attenuating-endoplasmic-reticulum-stress
#13
Yang Zhou, Wei-Kun Jia, Zhao Jian, Liang Zhao, Chen-Cheng Liu, Yong Wang, Ying-Bin Xiao
Chronic hypoxia is a key pathological change in patients with cyanotic congenital heart defect (CCHD). It has been demonstrated that enhanced myocardial unfolded protein response (UPR) increases the capacity to buffer endoplasmic reticulum (ER) stress and to avoid subsequent apoptosis caused by the hypoxia that underlies CCHD. The present study was performed to determine the regulatory role of microRNAs (miRNAs) in this cytoprotective UPR process. The results revealed that miR‑199a‑5p was markedly downregulated in the cardiac tissue of patients with CCHD and in human myocardial cells cultured in hypoxic conditions...
September 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28701598/the-blalock-and-taussig-shunt-revisited
#14
REVIEW
Usha Kiran, Shivani Aggarwal, Arin Choudhary, B Uma, Poonam Malhotra Kapoor
The systemic to pulmonary artery shunts are done as palliative procedures for cyanotic congenital heart diseases ranging from simple tetralogy of Fallots (TOFs)/pulmonary atresia (PA) to complex univentricular hearts. They allow growth of pulmonary arteries and maintain regulated blood flow to the lungs till a proper age and body weight suitable for definitive corrective repair is reached. We have reviewed the BT shunt with its anaesthtic considerations and management of associated complications.
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28685697/does-pharmacological-therapy-still-play-a-role-in-preventing-sudden-death-in-surgically-treated-tetralogy-of-fallot
#15
Gabriele Bronzetti, Maurizio Brighenti, Marco Bonvicini
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, with a familial recurrence risk of 3%. Despite the performing of an optimal surgical repair, TOF patients may feature a poor medium and long-term survival rate: atrial re-entrant tachycardia will develop in more than 30% of patients and high-grade ventricular arrhythmias will be seen in about 10% of patients. These life-threatening arrhythmias and consequent sudden death continue to represent serious complications following TOF repair...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28680470/a-case-of-successfully-managed-pregnancy-in-a-patient-with-complex-cyanotic-congenital-heart-disease
#16
J Y Liu, W K Tan, E L Tan, J L Tan, L K Tan
Medical advances have increased survival of patients with congenital heart disease. However, cardiac disease in pregnancy carries significant maternal and fetal risks, posing enormous challenges to obstetricians. Cyanotic congenital heart disease is associated with maternal complications such as arrhythmias, thromboembolic events and death. Fetal complications include small for gestational age, miscarriage and prematurity. Cyanotic congenital heart disease patients who continue their pregnancies require holistic multidisciplinary team care with early and coordinated planning for delivery...
June 2017: Obstetric Medicine
https://www.readbyqxmd.com/read/28674625/gender-related-differences-in-self-reported-dental-care-in-adults-with-congenital-heart-disease-at-increased-risk-of-infective-endocarditis
#17
Susann Schmidt, Marlies Ramseier-Hadorn, Corina Thomet, Kerstin Wustmann, Markus Schwerzmann
OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of infective endocarditis (IE). Women with CHD have a lower IE risk, potentially due to gender-related differences in dental care. We aimed to assess self-reported dental hygiene measures in adults with CHD, and to identify factors associated with good oral hygiene. METHODS AND RESULTS: Descriptive study includes 187 adults with CHD at increased risk of IE. The patients' IE knowledge was assessed using an adapted version of the Leuven Knowledge Questionnaire for CHD...
2017: Open Heart
https://www.readbyqxmd.com/read/28663364/platelet-count-and-mean-platelet-volume-predict-outcome-in-adults-with-eisenmenger-syndrome
#18
Agustin C Martin-Garcia, Deepa Rj Arachchillage, Aleksander Kempny, Rafael Alonso-Gonzalez, Ana Martin-Garcia, Anselm Uebing, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Pedro Luis Sanchez, Konstantinos Dimopoulos, Michael A Gatzoulis
OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years...
June 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28634172/identifying-an-oxygenation-index-threshold-for-increased-mortality-in-acute-respiratory-failure
#19
Brandon G Hammond, Pamela Garcia-Filion, Paul Kang, Mounica Y Rao, Brigham C Willis, Heidi J Dalton
BACKGROUND: The objective of this work was to examine current oxygenation index (OI) data and outcomes using electronic medical record data to identify a specific OI value associated with mortality. METHODS: This study was a retrospective electronic medical record data review from the pediatric ICU of Phoenix Children's Hospital, with data mining for variables to calculate OIs on subjects age 1 month to 20 y mechanically ventilated >24 h, excluding those with known intracardiac shunts or cyanotic heart disease...
June 20, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28632506/adenotonsillectomy-for-the-management-of-pulmonary-hypertension-in-a-patient-with-complex-congenital-heart-disease-a-case-report
#20
Adam C Adler, Yuan-Jiun Nicole Chao
Pulmonary hypertension is a feared complication in congenital heart disease patients. Patients with pulmonary hypertension are at risk for major perioperative cardiopulmonary complications when undergoing any surgical procedure, especially airway and laparoscopic procedures. We present the anesthetic management for a 2-year old with Down syndrome and complex cyanotic congenital heart disease undergoing tonsillectomy and adenoidectomy for severe obstructive sleep apnea.
June 19, 2017: A & A Case Reports
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