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https://www.readbyqxmd.com/read/28096041/advance-care-planning-in-adults-with-congenital-heart-disease-a-patient-priority
#1
Lisa X Deng, Lacey P Gleason, Abigail M Khan, David Drajpuch, Stephanie Fuller, Leah A Goldberg, Christopher E Mascio, Sara L Partington, Lynda Tobin, Yuli Y Kim, Adrienne H Kovacs
BACKGROUND: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. METHODS: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles...
January 4, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28094679/a-retrospective-analysis-of-a-pediatric-tele-echocardiography-service-to-treat-triage-and-reduce-trans-pacific-transport
#2
Christopher A Rouse, Brandon T Woods, C Becket Mahnke
Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28092290/clinical-recommendations-of-cardiac-magnetic-resonance-part-ii-inflammatory-and-congenital-heart-disease-cardiomyopathies-and-cardiac-tumors-a-position-paper-of-the-working-group-applicazioni-della-risonanza-magnetica-of-the-italian-society-of-cardiology
#3
Gianluca Pontone, Gianluca Di Bella, Castelletti Silvia, Viviana Maestrini, Pierluigi Festa, Lamia Ait-Ali, Pier Giorgio Masci, Lorenzo Monti, Gabriella di Giovine, Manuel De Lazzari, Alberto Cipriani, Andrea I Guaricci, Santo Dellegrottaglie, Alessia Pepe, Martina Perazzolo Marra, Giovanni Donato Aquaro
The current document was developed by the working group on the 'application of cardiac magnetic resonance' of the Italian Society of Cardiology to provide a perspective on the current state of technical advances and clinical cardiac magnetic resonance applications and to inform cardiologists how to implement their clinical and diagnostic pathway with the introduction of this technique in the clinical practice. Appropriateness criteria were defined using a score system: score 1-3 = inappropriate (test is not generally acceptable and is not a reasonable approach for the indication), score 4-6 = uncertain (test may be generally acceptable and may be a reasonable approach for the indication but more research and/or patient information is needed to classify the indication definitively) and score 7-9 = appropriate (test is generally acceptable and is a reasonable approach for the indication)...
January 13, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28091794/the-impact-of-liver-disorders-on-perioperative-management-of-reoperative-cardiac-surgery-a-retrospective-study-in-adult-congenital-heart-disease-patients
#4
Koko Adachi, Hiroaki Toyama, Yu Kaiho, Osamu Adachi, Hiroto Hudeta, Masanori Yamauchi
PURPOSE: We evaluated the preoperative prevalence of risk factors for liver disorders and the relationship between the liver disorders and perioperative outcomes in adult congenital heart disease (ACHD) patients. METHODS: This retrospective study included 32 ACHD patients who underwent reoperative cardiac surgery. RESULTS: Preoperatively, 38% of the study patients had risk factors, including congestive liver (CL) due to right heart failure (31%), chronic hepatitis C (HC) (22%), and both CL and HC (16%)...
January 16, 2017: Journal of Anesthesia
https://www.readbyqxmd.com/read/28077509/the-basic-approach-a-single-centre-experience-with-a-cost-reducing-model-for-paediatric-cardiac-extracorporeal-membrane-oxygenation
#5
Massimo A Padalino, Chiara Tessari, Alvise Guariento, Anna C Frigo, Vladimiro L Vida, Andrea Marcolongo, Fabio Zanella, Michael J Harvey, Ravi R Thiagarajan, Giovanni Stellin
OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) is a lifesaving but expensive therapy in terms of financial, technical and human resources. We report our experience with a 'basic' ECMO support model, consisting of ECMO initiated and managed without the constant presence of a bedside specialist, to assess safety, clinical outcomes and financial impact on our health system. METHODS: We did a retrospective single-centre study of paediatric cardiac ECMO between January 2001 and March 2014...
January 10, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28077189/cardiac-haemangioma-associated-with-a-duct-dependent-congenital-heart-disease-in-a-newborn-infant
#6
Stefan A Djordjevic, Sofija Glumac, Jasna Kalanj
Cardiac haemangiomas are exceedingly rare; however, they can cause significant haemodynamic impairment and disturbances in heart rhythm. Rarely, cardiac tumours may also coexist with congenital heart lesions. We present an extremely unusual case of a cardiac haemangioma in the setting of complex transposition of the great arteries that caused functional tricuspid atresia. To our knowledge, this is the first such case described in the literature.
January 12, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28077182/functional-tricuspid-stenosis-a-rare-presentation-of-suspected-rhabdomyoma-as-congenital-cyanotic-heart-disease
#7
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/28074790/incidence-and-progression-of-cardiac-surgery-associated-acute-kidney-injury-and-its-relationship-with-bypass-and-cross-clamp-time
#8
Habib Md Reazaul Karim, Mohd Yunus, Manuj Kumar Saikia, Jyoti Prasad Kalita, Mrinal Mandal
INTRODUCTION: Cardiac surgery-associated kidney injury (CSA-AKI) is common but relatively less is known about its progression. The present study is aimed at evaluating the incidence and course of CSA-AKI and its relationship with the different durations of cardiopulmonary bypass (CPB) and cross clamp times. MATERIALS AND METHODS: Occurrences of CSA-AKI are evaluated as per the Akin Kidney Injury Network (AKIN) criteria over the course of 5 postoperative day (POD) in 100 patients...
January 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28072699/prevention-preferable-to-treatment-3-case-reports-of-patients-experiencing-right-sided-heart-failure-after-ebstein-anomaly-correction
#9
Ming Luo, Jing Lin, Zhen Qin, Lei Du
RATIONALE: Ebstein anomaly is a common congenital heart disease that may induce severe tricuspid regurgitation and dilation of the "atrialized" portion of the right ventricle. Patients who undergo surgery to correct Ebstein anomaly are at high risk of postoperative right-sided heart failure, yet little is known about what pre-, peri-, or postoperative procedures may help reduce this risk. PATIENT CONCERNS: Here, we describe 3 cases of adults with Ebstein anomaly who underwent corrective surgery and in whom right-sided heart failure occurred with severe tricuspid regurgitation detected by transesophageal echocardiography...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28068786/topical-and-low-dose-intravenous-tranexamic-acid-in-cyanotic-cardiac-surgery
#10
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/28063686/extubation-failure-after-neonatal-cardiac-surgery-a-multicenter-analysis
#11
Christopher W Mastropietro, Katherine Cashen, Lisa M Grimaldi, Keshava Murty Narayana Gowda, Kurt D Piggott, Michael Wilhelm, Eleanor Gradidge, Elizabeth A S Moser, Brian D Benneyworth, John M Costello
OBJECTIVES: To describe the epidemiology of extubation failure and identify risk factors for its occurrence in a multicenter population of neonates undergoing surgery for congenital heart disease. STUDY DESIGN: We conducted a prospective observational study of neonates ≤30 days of age who underwent cardiac surgery at 7 centers within the US in 2015. Extubation failure was defined as reintubation within 72 hours of the first planned extubation. Risk factors were identified with the use of multivariable logistic regression analysis and reported as OR with 95% CIs...
January 4, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28062939/early-transverse-tubule-development-begins-in-utero-in-the-sheep-heart
#12
Michelle L Munro, Christian Soeller
The ventricular cardiomyocytes of adult mammals contain invaginations of the plasma membrane known as transverse (t)-tubules. These regular structures are essential for the synchronisation of excitation-contraction (EC) coupling throughout the cell, which is a vital process for cardiac function. T-tubules form a close association with the sarcoplasmic reticulum (SR) to form junctions, where several key proteins involved in EC coupling are localised, including the SR calcium release channels-the ryanodine receptors (RyR)...
January 6, 2017: Journal of Muscle Research and Cell Motility
https://www.readbyqxmd.com/read/28062416/blood-flow-patterns-underlie-developmental-heart-defects
#13
Madeline Midgett, Kent L Thornburg, Sandra Rugonyi
Although cardiac malformations at birth are typically associated with genetic anomalies, blood flow dynamics also play a crucial role in heart formation. However, the relationship between blood flow patterns in the early embryo and later cardiovascular malformation has not been determined. We used the chicken embryo model to quantify the extent to which anomalous blood flow patterns predict cardiac defects that resemble those in humans, and found that restricting either the inflow to the heart or the outflow led to reproducible abnormalities with a dose-response type relationship between blood flow stimuli and the expression of cardiac phenotypes...
January 6, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28054902/novel-goal-directed-hemodynamic-optimization-therapy-based-on-major-vasopressor-during-corrective-cardiac-surgery-in-patients-with-severe-pulmonary-arterial-hypertension-a-pilot-study
#14
Shuwen Li, Qing Ma, Yanwei Yang, Jiakai Lu, Zhiquan Zhang, Mu Jin, Weiping Cheng
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a common and fatal complication of congenital heart disease (CHD). PAH-CHD increases the risk for postoperative complications. Recent evidence suggests that perioperative goal-directed hemodynamic optimization therapy (GDHOT) significantly improves outcomes in surgery patients. Standard GDHOT is based on major solution volume, vasodilators and inotropic therapy, while novel GDHOT is based on major vasopressor and inotropic therapy...
22, 2016: Heart Surgery Forum
https://www.readbyqxmd.com/read/28052532/persistent-left-superior-vena-cava-accompanying-congenital-heart-disease-in-children-experience-of-a-tertiary-care-center
#15
Mehmet Emre Ari, Vehbi Doğan, Senem Özgür, Özben Ceylan, İlker Ertuğrul, Şeyma Kayalı, Tamer Yoldaş, Utku Arman Örün, Özkan Kaya, Selmin Karademir
BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed...
January 3, 2017: Echocardiography
https://www.readbyqxmd.com/read/28051234/neonatal-lupus-erythematosus-with-congenital-heart-block-in-twins
#16
Lamia Gargouri, Faiza Safi, Bayen Maalej, Souad Mallek, Fatma Turki, Imen Majdoub, Malek Akrout, Dorra Abid, Samir Kamoun, Abdelmajid Mahfoudh
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Cases report - We report neonatal lupus erythematosus with complete congenital heart block in twins...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28049534/case-report-maternal-mosaicism-resulting-in-inheritance-of-a-novel-gata6-mutation-causing-pancreatic-agenesis-and-neonatal-diabetes-mellitus
#17
Daphne Yau, Elisa De Franco, Sarah E Flanagan, Sian Ellard, Miriam Blumenkrantz, John J Mitchell
BACKGROUND: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism. CASE PRESENTATION: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents...
January 3, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28049487/endothelial-microparticles-are-increased-in-congenital-heart-diseases-and-contribute-to-endothelial-dysfunction
#18
Ze-Bang Lin, Hong-Bo Ci, Yan Li, Tian-Pu Cheng, Dong-Hong Liu, Yan-Sheng Wang, Jun Xu, Hao-Xiang Yuan, Hua-Ming Li, Jing Chen, Li Zhou, Zhi-Ping Wang, Xi Zhang, Zhi-Jun Ou, Jing-Song Ou
BACKGROUND: We previously demonstrated that endothelial microparticles (EMPs) are increased in mitral valve diseases and impair valvular endothelial cell function. Perioperative systemic inflammation is an important risk factor and complication of cardiac surgery. In this study, we investigate whether EMPs increase in congenital heart diseases to promote inflammation and endothelial dysfunction. METHODS: The level of plasma EMPs in 20 patients with atrial septal defect (ASD), 23 patients with ventricular septal defect (VSD), and 30 healthy subjects were analyzed by flow cytometry...
January 4, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28043739/canadian-cardiovascular-society-canadian-pediatric-cardiology-association-position-statement-on-pulse-oximetry-screening-in-newborns-to-enhance-detection-of-critical-congenital-heart-disease
#19
Kenny K Wong, Anne Fournier, Deborah S Fruitman, Lisa Graves, Derek G Human, Michael Narvey, Jennifer L Russell
Congenital heart disease is the most common congenital malformation and approximately 3 in 1000 newborns have critical congenital heart disease (CCHD). Timely diagnosis affects morbidity, mortality, and disability, and newborn pulse oximetry screening has been studied to enhance detection of CCHD. In this position statement we present an evaluation of the literature for pulse oximetry screening. Current detection strategies including prenatal ultrasound examination and newborn physical examination are limited by low diagnostic sensitivity...
October 26, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28043466/increasing-complexity-of-heart-transplantation-in-patients-with-congenital-heart-disease
#20
William Y Shi, Pankaj Saxena, Matthew S Yong, Silvana F Marasco, David C McGiffin, Anne Shipp, Robert G Weintraub, Yves d'Udekem, Christian P Brizard, Igor E Konstantinov
Owing to improved surgical results, there is a growing population of patients with repaired congenital heart disease (CHD) requiring heart transplantation. The objective of the study was to review our experience in these patients. A retrospective review of the outcomes of heart transplantation in patients with CHD (n = 77) between 1988 and 2014 was performed. Outcomes of early (1988-1999) and late (2000-2014) eras were compared. In results, the mean age was 18 ± 14 years (range: 16 days-58 years). Seventy (91%) patients underwent a mean of 2...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
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