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https://www.readbyqxmd.com/read/28342684/persistent-pulmonary-hypertension-of-the-newborn
#1
REVIEW
Mamta Fuloria, Judy L Aschner
Failure of the normal circulatory adaptation to extrauterine life results in persistent pulmonary hypertension of the newborn (PPHN). Although this condition is most often secondary to parenchymal lung disease or lung hypoplasia, it may also be idiopathic. PPHN is characterized by elevated pulmonary vascular resistance with resultant right-to-left shunting of blood and hypoxemia. Although the preliminary diagnosis of PPHN is often based on differential cyanosis and labile hypoxemia, the diagnosis is confirmed by echocardiography...
March 23, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28333672/case-report-pediatric-scar-management-after-open-heart-surgery
#2
Ha Phan, Beau Harger, Nick Estrada
Tetralogy of Fallot is a congenital disease caused by structural defects within the heart that can lead to cyanosis. The purpose of this case report is to discuss the use of PracaSil-Plus, a proprietary topical anhydrous silicone base containing pracaxi oil, in scar-management therapy, following open-heart surgery on a pediatric patient with tetralogy of Fallot. The Patient and Observer Scar Assessment Scale was the research instrument used to evaluate for efficacy of the scar therapy. Following 8 weeks of treatment with PracaSil-Plus, reduction in scores were observed for scar color, stiffness, thickness, and irregularity, with improvements of 87...
July 2016: International Journal of Pharmaceutical Compounding
https://www.readbyqxmd.com/read/28329109/impact-of-early-fontan-completion-on-postoperative-outcomes-in-patients-with-a-functional-single-ventricle%C3%A2
#3
Masamichi Ono, Melchior Burri, Julie Cleuziou, Jelena Pabst von Ohain, Elisabeth Beran, Martina Strbad, Alfred Hager, Jürgen Hörer, Christian Schreiber, Rüdiger Lange
OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC ≤ 18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3-1.5) years in group A and 2...
February 23, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28321315/prolonged-expiratory-apnoea-with-cyanosis-in-arnold-chiari-ii-malformation
#4
Adnan Zafar, Nahin Hussain
Apnoea associated with Arnold Chiari malformation is a known entity and can be obstructive or central. Differentiating between two types is vital to deciding management pathway and prognosticating disease process.
March 2017: JRSM Open
https://www.readbyqxmd.com/read/28318517/-intracardiac-myxoma-complicated-by-acute-aortic-occlusion-case-report
#5
O Zizi, B Benfor, H Jiber, A Bouarhroum, S Benlamkaddem, N Kanjaa
We report the case for a 44-year-old patient without medical history, admitted in our department for bilateral acute limb ischemia (H6). The patient was hemodynamically stable and the physical examination shows limb sever ischemia (no femoral arterial pulses, coldness, cyanosis and paralysis). Abdominal CT angiography showed a complete occlusion of the infra renal abdominal aorta compatible with embolus. An embolectomy with Fogarty catheters was performed. Echocardiography showed a mass in the left atrium. Pathological examination of the material embolic confirmed the diagnosis of the cardiac myxoma...
March 16, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28296740/diagnosis-and-treatment-of-communicating-bronchopulmonary-foregut-malformation-report-of-two-cases-and-review-of-the-literature
#6
Hongxia Ren, Liqiong Duan, Baohong Zhao, Xiaoxia Wu, Hongyi Zhang, Caixia Liu
RATIONALE: Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory systems. To our best knowledge, most cases of CBPFM reported in the literature were in infancy or adulthood and CBPFM in infantile is even rarer with a high case-fatality rate partly due to misdiagnosis. PATIENT CONCERNS: We presented 2 cases of neonatal CBPFM. A 11-hour male newborn was admitted because of moaning for 7 hours, and a 1-day male newborn was referred to us with profuse foams, choking on breast-milk feeding and facial cyanosis...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28296217/transcatheter-closure-of-unroofed-coronary-sinus-using-covered-stents-in-an-adult-with-drainage-of-the-coronary-sinus-to-the-right-ventricle-after-supra-annular-tricuspid-valve-replacement
#7
Bassel Mohammad Nijres, Damien Kenny, Suhaib Kazmouz, Ziyad M Hijazi
We present a rare case of unroofed coronary sinus in a patient who underwent supra-annular tricuspid valve replacement with consequent drainage of the coronary sinus to the right ventricle. It is unclear whether the coronary sinus was unroofed congenitally or iatrogenically. This rare setup resulted in significant cyanosis. The abnormal drainage was successfully closed via trans-catheter delivery of covered stents with resolution of the cyanosis. © 2017 Wiley Periodicals, Inc.
March 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28285866/obstructed-infradiaphragmatic-total-anomalous-pulmonary-venous-return-in-a-13-day-old-infant-presenting-acutely-to-the-emergency-department-a-case-report
#8
Elizabeth A Siacunco, Garrett S Pacheco, Dale P Woolridge
BACKGROUND: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital heart defect. Obstructed forms are more severe, and typically present earlier in life, usually in the immediate newborn period, with symptoms of severe cyanosis and respiratory failure. CASE REPORT: A 13-day-old boy presented to the emergency department (ED) with respiratory extremis. He appeared cyanotic and limp, and was found to have significant hypoxia with oxygen saturation of 40%...
March 9, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28274236/isolation-and-genetic-characterization-of-a-novel-2-2-1-2a-h5n1-virus-from-a-vaccinated-meat-turkeys-flock-in-egypt
#9
Ahmed H Salaheldin, Jutta Veits, Hatem S Abd El-Hamid, Timm C Harder, Davud Devrishov, Thomas C Mettenleiter, Hafez M Hafez, Elsayed M Abdelwhab
BACKGROUND: Vaccination of poultry to control highly pathogenic avian influenza virus (HPAIV) H5N1 is used in several countries. HPAIV H5N1 of clade 2.2.1 which is endemic in Egypt has diversified into two genetic clades. Clade 2.2.1.1 represents antigenic drift variants in vaccinated commercial poultry while clade 2.2.1.2 variants are detected in humans and backyard poultry. Little is known about H5N1 infection in vaccinated turkeys under field conditions. CASE PRESENTATION: Here, we describe an HPAI H5N1 outbreak in a vaccinated meat-turkey flock in Egypt...
March 9, 2017: Virology Journal
https://www.readbyqxmd.com/read/28265211/modified-cone-reconstruction-of-the-tricuspid-valve-for-ebstein-anomaly-as-performed-in-siberia
#10
Evgeny V Krivoshchekov, Jaeger P Ackerman, Olga S Yanulevich, Alexander A Sokolov, Nadezhda V Ershova, Joseph A Dearani, Frank Cetta
The cone reconstruction technique, first described by da Silva and modified by Dearani and by others, has become the repair method of choice in patients with Ebstein anomaly of the tricuspid valve. This report details the outcome of the modified cone reconstruction technique in 6 children who underwent surgical correction of Ebstein anomaly at the Tomsk Institute of Cardiology in Siberia. From 2012 through 2015, 4 boys and 2 girls (age range, 11 mo-12 yr) underwent surgery to correct Ebstein anomaly. All had presented with cyanosis, exertional dyspnea, fatigue, or new-onset atrial arrhythmia, and none had undergone previous cardiac surgery...
February 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28260780/pulmonary-arterio-venous-malformation-pavm-a-rare-case-report
#11
A H Mollah, M S Islam, N Ghafoor, S S Morshed, T K Kar, A L Kabir, N Tabassum
A 6-year-old boy from Comilla, was admitted in Dhaka Medical College Hospital with exertional dyspnea, central cyanosis, clubbing and was finally diagnosed as pulmonary Arterio-Venous Malformation (PAVM) by bubble contrast echocardiography, and pulmonary CT angiography. As PAVM is rare in children, it is often not thought of in differential diagnoses and the diagnosis remains in disguise. In this report, we described the clinical presentation of 6-year-old child with PAVM and also how to investigate the case to get the diagnosis...
January 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28237971/body-mass-index-in-adult-congenital-heart-disease
#12
Margarita Brida, Konstantinos Dimopoulos, Alexander Kempny, Emmanouil Liodakis, Rafael Alonso-Gonzalez, Lorna Swan, Anselm Uebing, Helmut Baumgartner, Michael A Gatzoulis, Gerhard-Paul Diller
OBJECTIVE: Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. METHODS: We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18...
February 25, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28237697/heart-transplantation-in-adults-with-congenital-heart-disease
#13
REVIEW
Lucile Houyel, Ngoc-Tram To-Dumortier, Yannick Lepers, Jérôme Petit, Régine Roussin, Mohamed Ly, Emmanuel Lebret, Elie Fadel, Jürgen Hörer, Sébastien Hascoët
With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients...
February 22, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28222032/unexplained-cyanosis-caused-by-hepatopulmonary-syndrome-in-a-girl-with-apeced-syndrome
#14
Fatih Celmeli, Abdullah Kocabas, Ishak A Isik, Mesut Parlak, Kai Kisand, Serdar Ceylaner, Doga Turkkahraman
Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy (APECED) is a rare but devastating primary immunodeficiency disease caused by loss-of-function mutations in autoimmune regulator (AIRE) gene on chromosome 21q22.3. The clinical spectrum of the disease is characterized by a wide heterogeneity because of autoimmune reactions toward different endocrine and non-endocrine organs. Here, we report a 17-year-old Turkish girl diagnosed with APECED at 9 years in whom a novel homozygote mutation in AIRE gene p...
March 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28196557/anomalous-drainage-of-isolated-superior-caval-vein-to-the-left-atrium-the-oldest-reported-unrepaired-case
#15
Maria Boutsikou, Dudley J Pennell, Eva Nyktari
Isolated right superior caval vein drainage into the left atrium is a rare congenital cardiac anomaly usually presenting with hypoxaemia and cyanosis. Surgical repair is the definitive treatment for this condition. In this report, we present the case of a 72-year-old patient with uncorrected anomalous drainage of a right superior caval vein into the left atrium, first diagnosed in the 1960s. To the best of our knowledge, this is the oldest reported unrepaired case.
February 15, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28178121/a-case-report-of-congenital-umbilical-arteriovenous-malformation-complicated-with-liver-failure-after-surgical-excision
#16
Ji-Won Han, Hyun-Young Kim, Sung-Eun Jung
RATIONALE: Few case reports of umbilical arteriovenous malformation (AVM) have been reported. Herein, we report a neonatal case of umbilical AVM who underwent liver failure after surgical excision. PATIENT CONCERNS: The patient was a girl delivered at a gestational age of 39+5 weeks showing cyanosis and heart murmur. DIAGNOSES: Cardiac echography, abdominal ultrasonography (USG), and computed tomography revealed suspecting the umbilical AVM...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28177675/what-factors-contribute-to-the-elevation-of-serum-free-fatty-acids-ffa-in-newborns-in-the-cardiac-surgical-setting
#17
Carin Wittnich, Michael P Belanger
Elevations in serum free fatty acids (FFA) levels during heart surgery have been reported in humans and experimental animals, causing increased arrhythmias, decreased heart function, and contributing to mortality. Factors such as heparin administration, age, cardiopulmonary bypass (CPB) and cyanosis/hypoxia have been implicated but not proven. This study was designed to clarify the contribution of these factors using an experimental pig model as follows: a) adult (n=10) versus 3-day old piglets (n=18) had FFA levels assessed before and after heparin administration; b) 3 day old piglets, the additional effect of CPB (n=8) or just severe hypoxia (PaO2 = 20-25 mmHg; n=6) exposure on FFA levels...
January 9, 2017: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28176173/massive-left-atrial-calcification-a-case-report-and-review-of-the-literature
#18
Saeid Hosseini, Yousef Rezaei, Niloufar Samiei, Anita Sadeghpour, Mohammad Mehdi Peighambari, Carlos A Mestres, Frank W Sellke
Left atrial (LA) calcification is mostly observed in patients with rheumatic mitral valve (MV) diseases who underwent surgical treatment. We report a case of LA calcification in a 69-year-old woman presented with dyspnea and cyanosis. Admission electrocardiogram showed atrial fibrillation and biventricular hypertrophy. Her chest roentgenogram revealed cardiomegaly and a curvilinear whitish shadow in the area of LA. Transthoracic echocardiography revealed severe mitral stenosis and massive LA thrombosis. The patient underwent an emergent surgery, during which the whitish line on chest roentgenogram proved to be a calcified LA inner wall...
February 7, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28167694/uncommon-cause-of-adult-onset-cyanosis-single-left-ventricle
#19
Harsh Agrawal, Jessica D Schaefer, Mohamed Teleb, Laura M Cashin
No abstract text is available yet for this article.
February 6, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28126028/acute-airway-obstruction-due-to-postoperative-retropharyngeal-hematoma-after-anterior-cervical-fusion-a-retrospective-analysis
#20
Kyung-Jin Song, Byung-Wan Choi, Dong-Hyun Lee, Dong-Ju Lim, Seung-Yeol Oh, Sung-Soo Kim
BACKGROUND: Acute airway obstruction (AAO) after anterior cervical fusion (ACF) can be caused by postoperative retropharyngeal hematoma, which requires urgent recognition and treatment. However, the causes, evaluation, and appropriate treatment of this complication are not clearly defined. The purpose of this retrospective review of a prospective database was to investigate etiologic factors related to the development of AAO due to postoperative hematoma after ACF and formulate appropriate prevention and treatment guidelines...
January 26, 2017: Journal of Orthopaedic Surgery and Research
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