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Cyanotic heart disease

Marine Flechet, Fabian Güiza, Dirk Vlasselaers, Lars Desmet, Stoffel Lamote, Heidi Delrue, Marc Beckers, Michaël P Casaer, Pieter Wouters, Greet van den Berghe, Geert Meyfroidt
OBJECTIVES: To assess whether near-infrared cerebral tissue oxygen saturation, measured with the FORESIGHT cerebral oximeter (CAS Medical Systems, Branford, CT) predicts PICU length of stay, duration of invasive mechanical ventilation, and mortality in critically ill children after pediatric cardiac surgery. DESIGN: Single-center prospective, observational study. SETTING: Twelve-bed PICU of a tertiary academic hospital. PATIENTS: Critically ill children and infants with congenital heart disease, younger than 12 years old, admitted to the PICU between October 2012 and November 2015...
February 19, 2018: Pediatric Critical Care Medicine
Giancarlo Suffredini, Natalia Diaz-Rodriguez, Krishnan Chakravarthy, Aarti Mathur, Heather K Hayanga, Steve M Frank, Richard E Ringel, Stephen Freiberg, Viachaslau M Barodka, Jochen Steppan
Survival rates for patients with palliated congenital heart disease are increasing, and an increasing number of adults with cyanotic congenital heart disease (CCHD) might require surgical resection of pheochromocytoma-paraganglioma (PHEO-PGL). A recent study supports the idea that patients with a history of CCHD and current or historical cyanosis might be at increased risk for developing PHEO-PGL. We review the anesthetic management of two adults with single-ventricle physiology following Fontan palliation presenting for PHEO-PGL resection and review prior published case reports...
December 8, 2017: Curēus
Gil Shechter-Maor, Nicholas Czuzoj-Shulman, Andrea R Spence, Haim Arie Abenhaim
PURPOSE: Our study objective is to examine the association between births conceived with assisted reproductive technology (ART) and birth defects using a large database from the United States. METHODS: Using the Centers for Disease Control and Prevention's Period-linked birth-infant death data files and fetal death database for 2011-2013, we conducted a retrospective cohort study comprised of live births that occurred in the USA during that time. Multivariate logistic regression was used to estimate the association between ART and birth defects, both overall and by specific defects...
February 15, 2018: Archives of Gynecology and Obstetrics
Sai-Hou Fan, Zhen-Ya Shen, Yi-Min Xiao
Tetralogy of Fallot (TOF) is one of the most severe forms of cyanotic congenital heart disease (CHD) and is also the most common. Previous genome-wide association study (GWAS) and replication studies have suggested that a polymorphism in the neuropilin 1 (NRP1) gene is significantly associated with the risk of TOF. To further confirm the association between the NRP1 polymorphism and the risk of TOF and to identify additional positive functional single-nucleotide polymorphisms (SNPs) for TOF risk, we systematically screened for functional polymorphisms throughout the regulatory and coding regions of the NRP1 gene...
February 9, 2018: Gene
Sathish M Chikkabyrappa, Rohit S Loomba, Justin T Tretter
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy...
February 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
Shuenn-Nan Chiu, Jieh-Neng Wang, Yun-Ching Fu, Hung-Tao Chung, Ling-Yin Chang, Mei-Hwan Wu, Yu-Chuan Hua, Ming-Tai Lin, Chun-Wei Lu, Chun-An Chen, Jing-Ming Wu, Jou-Kou Wang
OBJECTIVE: To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenital heart disease (hsCHD) in subtropical areas without clear respiratory syncytial virus seasonality. STUDY DESIGN: Since July 2013, the National Health Insurance program has provided reimbursement for palivizumab prophylaxis with a novel monthly protocol in selected patients with hsCHD under 1 year of age. We performed a multicenter study to assess the trend of respiratory syncytial virus hospitalizations in patients with hsCHD from 2010 to 2016 during the prepalivizumab, transition, and postpalivizumab periods, and compared treatment and propensity-matched control groups...
January 29, 2018: Journal of Pediatrics
Nabil E Hassan, Diann E Reischman, Robert K Fitzgerald, Edward Vincent S Faustino
OBJECTIVES: To determine the prevailing hemoglobin levels in PICU patients, and any potential correlates. DESIGN: Post hoc analysis of prospective multicenter observational data. SETTINGS: Fifty-nine PICUs in seven countries. PATIENTS: PICU patients on four specific days in 2012. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients' hemoglobin and other clinical and institutional data...
January 30, 2018: Pediatric Critical Care Medicine
Kriti Puri, Peter Kazembe, Treasure Mkaliainga, Msandeni Chiume, Antonio G Cabrera, Amy Sims Sanyahumbi
Malawi is one of the poorest nations in the world, ranked 151st among 195 countries by the World Bank, with an under-5-year mortality rate of 63 per 1000 live births. There are no previous studies describing the spectrum of inpatient pediatric cardiology consultations in sub-Saharan Africa. A descriptive cohort study was performed at Kamuzu Central Hospital (KCH), a tertiary care hospital in Lilongwe, Malawi. Demographic, anthropometric, and clinical information for all cardiology consults patients aged 0-18 years admitted to the children's wards over a period of 1 month was reviewed...
January 25, 2018: Congenital Heart Disease
Ivan Duran, Jessica Tenney, Carmen M Warren, Anna Sarukhanov, Fabiana Csukasi, Mark Skalansky, Maria L Iruela-Arispe, Deborah Krakow
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. It involves anatomical abnormalities that change the normal flow of blood through the heart resulting in low oxygenation. Although not all of the underlying causes of TOF are completely understood, the disease has been associated with varying genetic etiologies including chromosomal abnormalities and Mendelian disorders, but can also occur as an isolated defect. In this report, we describe a familial case of TOF associated with a 1...
January 24, 2018: American Journal of Medical Genetics. Part A
Neeti Makhija, Rohan Magoon, Minati Choudhury, Sivasubramanian Ramakrishnan
Cyanotic congenital heart disease presents an increased tendency to bleed in view of subtle coagulation defects. Airway bleeding can be particularly difficult to manage while maintaining an adequate ventilation. An isolated lung bleed with the exclusion of possible traumatic, medical and surgical causes of bleeding, should alert the attending anesthesiologist to the possibility of the collateral-related bleeding. Preoperative coil embolization remains an important initial management step in a case of tetralogy of Fallot (TOF) with major aortopulmonary collaterals...
January 2018: Annals of Cardiac Anaesthesia
Barakat Adeola Animasahun, Aminat Titilayo Ogunlana, Henry Olusegun Gbelee
Background: The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally. There is a lack of data on the profile and outcome of patients with persistent truncus arteriosus (PTA) in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center...
October 2017: Heart Views: the Official Journal of the Gulf Heart Association
Lin Liu, Hong-Dan Wang, Cun-Ying Cui, Yun-Yun Qin, Tai-Bing Fan, Bang-Tian Peng, Lian-Zhong Zhang, Cheng-Zeng Wang
Background: Tetralogy of Fallot is the most common cyanotic congenital heart disease. However, its pathogenesis remains to be clarified. The purpose of this study was to identify the genetic variants in Tetralogy of Fallot by whole exome sequencing. Methods: Whole exome sequencing was performed among eight small families with Tetralogy of Fallot. Differential single nucleotide polymorphisms and small InDels were found by alignment within families and between families and then were verified by Sanger sequencing...
December 5, 2017: Oncotarget
Nobuhiro Mukai, Yoshinobu Nakayama, Satoshi Murakami, Toshihito Tanahashi, Daniel I Sessler, Sachiyo Ishii, Satoru Ogawa, Natsuko Tokuhira, Toshiki Mizobe, Teiji Sawa, Yasufumi Nakajima
BACKGROUND: Children with cyanotic heart disease develop secondary erythrocytosis and thrombocytopenia via unknown mechanisms. Mature erythrocyte microRNAs may reflect clinical pathologies and cell differentiation processes pre-enucleation. This study evaluated erythrocyte microRNAs in children with cyanotic heart disease. METHODS: Erythrocyte microRNAs from children with cyanotic and acyanotic heart disease and without cardiac disease were quantified with Ion PGM System (n=10 per group)...
December 27, 2017: Pediatric Research
Peter Bak, Cristel S Hjortshøj, Peter Gaede, Lars Idorn, Lars Søndergaard, Annette S Jensen
OBJECTIVE: Cyanotic congenital heart disease is a systemic disease, with effects on multiple organ systems. A high prevalence of subclinical hypothyroidism (SCH) has been reported in a small cohort of cyanotic congenital heart disease patients. Subclinical hypothyroidism has been associated with various adverse cardiovascular effects, as well as an increased risk of progression to overt hypothyroidism. The aim of this study was to examine the prevalence of SCH in cyanotic congenital heart disease patients, consider possible etiologies, and evaluate thyroid function over time...
December 27, 2017: Congenital Heart Disease
Tabitha G Moe, Victor A Abrich, Edward K Rhee
Advances in surgical techniques have led to the survival of most patients with congenital heart disease (CHD) up to their adulthood. During their lifetime, many of them develop atrial tachyarrhythmias due to atrial dilatation and scarring from surgical procedures. More complex defects and palliative repairs are linked to a higher incidence and earlier occurrence of arrhythmias. Atrial fibrillation (AF) is common in patients who have atrial septal defects repaired after age 55 and in patients with tetralogy of Fallot repaired after age 45...
June 2017: Journal of Atrial Fibrillation
Lauge Østergaard, Nana Valeur, Nikolaj Ihlemann, Henning Bundgaard, Gunnar Gislason, Christian Torp-Pedersen, Niels Eske Bruun, Lars Søndergaard, Lars Køber, Emil Loldrup Fosbøl
Aims: Patients with prior infective endocarditis (IE), a prosthetic heart valve, or a cyanotic congenital heart disease (CHD) are considered to be at high risk of IE by guidelines. However, knowledge is sparse on the relative risk of IE between these three groups and compared controls. Methods and results: Using Danish nationwide registries (1996-2015), we identified all patients with prior IE, a prosthetic heart valve, or a complex CHD (defined as tetralogy of Fallot, truncus arteriosus, and transposition of great arteries) as well as matched control populations...
December 12, 2017: European Heart Journal
Brijesh Takkar, Archita Singh, Prateek Kakkar, Shorya Azad, Rajvardhan Azad
Retinal vasculature is frequently altered in systemic vascular disorders such as Diabetes and Hypertension. We present a rare case in which vitreous hemorrhage was the presenting sign of an underlying congenital cyanotic heart disease.
October 2017: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
Emrah Can, Şahin Hamilcikan, Ceren Can
OBJECTIVE: The purpose of this study was to investigate the relationship between neonate early-onset sepsis (EOS) and the neutrophil to lymphocyte ratio (NLR) and the platelet to lymphocyte ratio (PLR) of term neonates. MATERIALS AND METHODS: This prospective observational study was conducted with term neonates diagnosed with EOS compared with 44 healthy controls. Exclusion criteria were prematurity, postmaturity, small or large for gestational age according to week of pregnancy, preeclampsia, gestational diabetes mellitus, chorioamnionitis, congenital major anomalies, and cyanotic congenital heart disease...
December 6, 2017: Journal of Pediatric Hematology/oncology
Yupeng Wen, Zongxiao Li, Cheng Chang, Peng Zhang, Yang Lyu
OBJECTIVE: To investigate the diagnostic value of urinary neutrophil gelatin enzyme-related lipid delivery protein (NGAL) and kidney injury molecule-1 (KIM-1) in the acute kidney injury (AKI) after cardiopulmonary bypass (CPB) operation in children with non-cyanotic congenital heart disease (CHD). METHODS: A retrospective analysis was conducted. 200 CPB undergoing cardiac surgery in children with non-cyanotic CHD admitted to Tianjin Children's Hospital from June 2015 to May 2017 were enrolled...
December 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
Rizwan Rehman, Mood Che Marhisham, Mazeni Alwi
Patent ductus arteriosus (PDA) stenting has gained acceptance for palliation in cyanotic congenital heart disease. The PDA in tetralogy of Fallot with pulmonary atresia (ToF-PA) arises, in the left aortic arch, from underneath the arch and connects to the proximal left pulmonary artery, often resulting in stenosis. The PDA is usually elongated and tortuous, making stent implantation challenging. Shorter duration of palliation, aggravation of branch pulmonary artery stenosis resulting in poor growth and difficulty at surgery makes ductal stenting controversial...
January 2018: Future Cardiology
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