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Congenital heart disease

Maanasi S Mistry, Sara M Trucco, Timothy Maul, Mahesh S Sharma, Li Wang, Shawn West
BACKGROUND: Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides respiratory and hemodynamic support to pediatric patients in severe cardiac failure. We aim to identify risk factors associated with poorer outcomes in this population. METHODS: A retrospective chart review was conducted of pediatric patients requiring VA-ECMO support for cardiac indications at our institution from 2004 to 2015. Data were collected on demographics, indication, markers of cardiac output, ventricular assist device (VAD) insertion, heart transplantation, or left atrial (LA) decompression...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
Paul C Helm, Sebastian Kempert, Marc-André Körten, Wiebke Lesch, Katharina Specht, Ulrike M M Bauer
BACKGROUND: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients' and parents' perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions. MATERIALS AND METHODS: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD)...
January 25, 2018: Congenital Heart Disease
Tatiana Marcela Pérez, Sandra Milena García, Martha Lucía Velasco, Angela Paola Sánchez
Interrupted aortic arch is an extremely rare congenital malformation representing about 1% of congenital heart disease. Early symptoms usually occur early in the neonatal period and clinical deterioration is often rapid and long-term prognosis is limited. Nonetheless, this condition has been identified later in adult life in rare cases. We report a case in an adult male with absence of hypertension history and no further cardiac compromise, with a severe posterior chest pain alongside dyspnea and sweating. Computed tomography angiography revealed interrupted aortic arch type A, bivalve aorta, hemopericardium, aortic dissection Stanford A, and important collateral circulation...
February 2018: Radiology Case Reports
Abdulrahman Masrani, Sebastian McWilliams, Sanjeev Bhalla, Pamela K Woodard
PURPOSE: To report the anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. MATERIALS AND METHODS: Retrospective review of the medical records between February 2001 and February 2016 was performed. To identify patients, radiological reports were queried for "Scimitar" or "partial anomalous pulmonary venous return." Patients with radiological findings of Scimitar syndrome were included. Patients without cross-sectional imaging were excluded...
February 3, 2018: Journal of Cardiovascular Computed Tomography
James K Kirklin, Rongbing Xie, Jennifer Cowger, Theo M M H de By, Takeshi Nakatani, Stephan Schueler, Rhiannon Taylor, Jenny Lannon, Paul Mohacsi, Jan Gummert, Daniel Goldstein, Kadir Caliskan, Margaret M Hannan
The second annual IMACS registry report includes over 14, 000 patients from 35 countries. Survival, adverse events, and an updated risk model is presented. Continuous flow pumps continue to dominate the world's experience. One and Two-year survival remains at 80% and 70%. Congenital heart disease and biventricular support are the most dominant risk factors. The database is poised for major novel analyses.
January 31, 2018: Journal of Heart and Lung Transplantation
Nathaly M Sweeney, Shareef A Nahas, Shimul Chowdhury, Miguel Del Campo, Marilyn C Jones, David P Dimmock, Stephen F Kingsmore, Rcigm Investigators
Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2...
March 16, 2018: Cold Spring Harbor Molecular Case Studies
María Guadalupe Jiménez-Carbajal, Didier López Pérez, Claudia Paola Fernández Luna
A review is presented of data published in medical literature related to the screening used for the early detection of complex congenital heart disease in apparently healthy newborns in several cities of the world, including those reported in Mexico. The screening was performed due to the knowledge of the pathophysiology of indirect hypoxia data, observation of differential cyanosis and the consequent difference in the values of pre- and post-ductal pulse oximetry derived from the ductal and/or atrial septal defect dependence of several severe congenital heart diseases...
March 13, 2018: Archivos de Cardiología de México
Qiyu Lu, Yi Sun, Yuyin Duan, Bin Li, Jianming Xia, Songhua Yu, Guimin Zhang
BACKGROUND: Valvular heart disease is a leading cause of cardiovascular mortality, especially in China. More than a half of valvular heart diseases are caused by acute rheumatic fever. microRNA is involved in many physiological and pathological processes. However, the miRNA profile of the rheumatic valvular heart disease is unknown. This research is to discuss microRNAs and their target gene pathways involved in rheumatic heart valve disease. METHODS: Serum miRNA from one healthy individual and four rheumatic heart disease patients were sequenced...
March 16, 2018: BMC Cardiovascular Disorders
Ashley Gionfriddo, Mika L Nonoyama, Peter C Laussen, Peter N Cox, Megan Clarke, Alejandro A Floh
OBJECTIVES: To promote standardization, the Centers for Disease Control and Prevention introduced a new ventilator-associated pneumonia classification, which was modified for pediatrics (pediatric ventilator-associated pneumonia according to proposed criteria [PVAP]). We evaluated the frequency of PVAP in a cohort of children diagnosed with ventilator-associated pneumonia according to traditional criteria and compared their strength of association with clinically relevant outcomes. DESIGN: Retrospective cohort study...
March 15, 2018: Pediatric Critical Care Medicine
Benjamin H Goot, Jon Kaufman, Zhaoxing Pan, David W A Bourne, Francis Hickey, Mark Twite, Jeffrey Galinkin, Uwe Christians, Jeannie Zuk, Eduardo M da Cruz
OBJECTIVES: To assess if morphine pharmacokinetics are different in children with Down syndrome when compared with children without Down syndrome. DESIGN: Prospective single-center study including subjects with Down syndrome undergoing cardiac surgery (neonate to 18 yr old) matched by age and cardiac lesion with non-Down syndrome controls. Subjects were placed on a postoperative morphine infusion that was adjusted as clinically necessary, and blood was sampled to measure morphine and its metabolites concentrations...
March 15, 2018: Pediatric Critical Care Medicine
C Le Roy, G Larios, D Springmüller, C Clavería
INTRODUCTION: Children with congenital heart disease (CHD) present a high percentage of undern utrition and the interpretation of their nutritional assessment is difficult. OBJECTIVE: To describe the nutritional status of infants with CHD using two anthropometric classifications and compare them. PATIENTS AND METHOD: Non-concurrent cohort study. We studied children under 12 months under going cardiac surgery. We excluded preterm infants, small for gestational age, carriers of genetic syndrome or other disease with nutritional compromise...
December 2017: Revista Chilena de Pediatría
Wail Alkashkari, Amani Alsubei, Ziyad M Hijazi
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital heart disease (CHD). Percutaneous valve interventions are now a cornerstone of not just the congenital cardiologist treating patients with congenital heart disease, but also-and numerically more importantly-for adult interventional cardiologists treating patients with acquired heart valve disease. Transcatheter pulmonary valve replacement (tPVR) is one of the most exciting recent developments in the treatment of CHD and has evolved to become an attractive alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction...
March 15, 2018: Current Cardiology Reports
Shu Meng, Qilin Gu, Xiaojie Yang, Jie Lv, Iris Owusu, Gianfranco Matrone, Kaifu Chen, John P Cooke, Longhou Fang
Background -Angiogenesis is integral for embryogenesis, and targeting angiogenesis improves the outcome of many pathological conditions in patients. TBX20 is a crucial transcription factor for embryonic development, and its deficiency is associated with congenital heart disease. However, the role of TBX20 in angiogenesis has not been described. Methods -Loss- and gain-of-function approaches were used to explore the role of TBX20 in angiogenesis both in vitro and in vivo Angiogenesis gene array was used to identify key downstream targets of TBX20...
March 15, 2018: Circulation
S Arques
Cardiovascular diseases are the leading cause of death worldwide. Conceptually, endothelial dysfunction, inflammatory status and oxidative stress are at the forefront in the onset and development of most cardiovascular diseases, particularly coronary artery disease and heart failure. Serum albumin, the most abundant plasma protein, has many physiological properties, including anti-inflammatory, antioxidant and antiplatelet aggregation activity. It also plays an essential role in the fluid exchange across the capillary membrane...
March 12, 2018: Annales de Cardiologie et D'angéiologie
Faisal Junejo, Mohammad Irfan Akhtar, Mohammad Hamid, Syed Shabbir Ahmed, Fauzia Minai, Muneer Amanullah
OBJECTIVE: To compare the intravenous boluses and intravenous continuous infusion of tranexamic acid (TXA) to reduce postoperative bleeding in cyanotic congenital heart disease surgeries. STUDY DESIGN: Single-blinded randomised clinical trial. PLACE AND DURATION OF STUDY: Anaesthesia Department, The Aga Khan University Hospital, Karachi, from July 2016 to April 2017. METHODOLOGY: Sixty patients of cyanotic congenital heart disease, undergoing either palliative or corrective surgery involving cardiopulmonary bypass (CPB), were recruited...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Michael L O'Byrne, Michael G McBride, Stephen Paridon, Elizabeth Goldmuntz
BACKGROUND: Obesity is associated with increased lifelong morbidity and reduced life span and is increasingly prevalent in the congenital heart disease population. Habitual exercise is an important aspect of a healthy lifestyle and primary prevention of obesity in the general population. The association between habitual activity and body mass index (BMI) has not been studied in children with congenital heart disease. METHODS: A cross-sectional analysis of two previously collected cohorts was performed, including participants 8 to 18 years old with tetralogy of Fallot, transposition of the great arteries, and single ventricle heart disease after a Fontan operation...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Alexander R Opotowsky, Jonathan Rhodes, Michael J Landzberg, Ami B Bhatt, Keri M Shafer, Doreen DeFaria Yeh, Scott E Crouter, Ana Ubeda Tikkanen
BACKGROUND: Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD). METHODS: We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O2 saturation ≥92%, and had peak O2 consumption ([Formula: see text]) < 80% predicted...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Cathy Liu, Jade Lodge, Christopher Flatley, Alexander Gooi, Cameron Ward, Karen Eagleson, Sailesh Kumar
OBJECTIVE: To determine obstetric, intrapartum and perinatal outcomes for pregnancies with isolated fetal congenital heart defects (CHD). METHODS: This was a retrospective cohort study of women that delivered an infant with an isolated major CHD between January 2010 and April 2017 at a major Australian perinatal centre. The study cohort was compared with a cohort of women with infants without CHD. Cardiac abnormalities were broadly subdivided into the following five categories using the International Classification of Diseases Tenth Revision (ICD-10) as a guide - transposition of the great arteries (TGA), septal defects, right heart lesions (RHL), left heart lesions (LHL) and "other"...
March 16, 2018: Journal of Maternal-fetal & Neonatal Medicine
Rafael Arboleda Salazar, Jane Heggie, Piotr Wolski, Eric Horlick, Mark Osten, Massimiliano Meineri
BACKGROUND: Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center...
March 14, 2018: Anesthesia and Analgesia
Rachel C Lombardo, Aleksey Porollo, James F Cnota, Robert J Hopkin
PurposeCongenital central hypoventilation syndrome (CCHS, OMIM 209880) is a rare autosomal dominant disorder caused by mutation in PHOX2B that manifests as a consequence of abnormal neural crest cell migration during embryogenesis. Unlike other neurocristopathies, however, its impact on the cardiovascular system has not been previously assessed. This study was an effort to characterize the association between congenital heart disease (CHD) and mutations in PHOX2B in patients with CCHS.MethodsA retrospective review of patients with CCHS in conjunction with functional analysis of PHOX2B mutations associated with CHD was performed...
March 15, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
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