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Congenital heart diseases

Ivan Wen Wen Lau, Dongting Liu, Lei Xu, Zhanming Fan, Zhonghua Sun
OBJECTIVE: Current diagnostic assessment tools remain suboptimal in demonstrating complex morphology of congenital heart disease (CHD). This limitation has posed several challenges in preoperative planning, communication in medical practice, and medical education. This study aims to investigate the dimensional accuracy and the clinical value of 3D printed model of CHD in the above three areas. METHODS: Using cardiac computed tomography angiography (CCTA) data, a patient-specific 3D model of a 20-month-old boy with double outlet right ventricle was printed in Tango Plus material...
2018: PloS One
Martin Schweiger, Angela Lorts, Jennifer Conway
PURPOSE OF REVIEW: Increased miniaturization of ventricular assist devices (VADs) and new mechanical support strategies (MCS) has increased the use of MCS in the pediatric and congenital heart disease (CHD) population. This comes with the need for care providers specialized in this field to determine optimal patient and device selection, and to improve outcomes and decrease complication rates for new innovative strategies. A review of the published literature in this field is timely and relevant...
March 20, 2018: Current Opinion in Organ Transplantation
Lidia Capotosto, Francesco Massoni, Simone De Sio, Serafino Ricci, Antonio Vitarelli
Cardiovascular disease (CVD) still remains the main cause of morbidity and mortality and consequently early diagnosis is of paramount importance. Working conditions can be regarded as an additional risk factor for CVD. Since different aspects of the job may affect vascular health differently, it is important to consider occupation from multiple perspectives to better assess occupational impacts on health. Standard echocardiography has several targets in the cardiac population, as the assessment of myocardial performance, valvular and/or congenital heart disease, and hemodynamics...
2018: BioMed Research International
Qian Li, Wenying Zhu, Bei Zhang, Yiping Wu, Yufeng Yuan, Haiyan Zhang, Jie Li, Kai Sun, Hua Wang, Tingting Yu
Background: Many long noncoding RNAs (lncRNAs), including lncRNA MALAT1, are involved in various cardiac diseases. We evaluated the effects of tag single nucleotide polymorphisms (tag-SNPs) on MALAT1 gene in a Chinese population of children with congenital heart disease (CHD). Methods:  In the present study, 713 CHD patients and 730 gender- and age-matched newborns without CHD were genotyped for MALAT1 tag-SNPs rs11227209, rs619586, and rs3200401. Further investigation of SNP's function was performed by luciferase assay...
March 20, 2018: Bioscience Reports
Dehua Wang, Darren Marshall, Gruschen Veldtman, Anita Gupta, Andrew T Trout, Juan Villafane, Kevin Bove
Hepatic dysfunction, including development of hepatocellular carcinoma and other liver lesions has been increasingly reported following Fontan procedure for congenital heart disease. We report a unique case of intrahepatic cholangiocarcinoma 28 years after a Fontan procedure in a 31year old female with heterotaxy syndrome. The subcapsular mass-forming tumor was composed of poorly differentiated tumor cells arranged in small vague glandular or slit-lumen nests, and focally fused or anastomosing large trabecular patterns within the prominent fibrotic stroma...
March 15, 2018: Pathology, Research and Practice
Meghann McKane, Debra A Dodd, Bret A Mettler, Kari A Wujcik, Justin Godown
BACKGROUND: Many pediatric heart transplant recipients live a significant distance from their transplant center. This results in families either traveling long distances or relying on outside physicians to assume aspects of their care. Distance has been implicated to play a role in congenital heart disease outcomes, but its impact on heart transplantation has not been reported. The aim of this study was to assess the impact of distance on pediatric heart transplant outcomes. METHODS: The Scientific Registry of Transplant Recipients database was queried for all pediatric heart transplant recipients from large US children's hospitals (1987-2014)...
January 1, 2018: Progress in Transplantation
Pablo Roa Rojas, Hernán Arango Fernández, Martha Rebolledo Cobos, Jonathan Harris Ricardo
Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia...
April 1, 2018: Archivos Argentinos de Pediatría
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
Jung Min Ko
No abstract text is available yet for this article.
March 2018: Korean Circulation Journal
Bo Geum Choi, Su Kyung Hwang, Jung Eun Kwon, Yeo Hyang Kim
BACKGROUND AND OBJECTIVES: The purpose of the present study was to investigate the advantages and disadvantages of verifying genetic abnormalities using array comparative genomic hybridization (a-CGH) immediately after diagnosis of congenital heart disease (CHD). METHODS: Among neonates under the age of 28 days who underwent echocardiography from January 1, 2014 to April 30, 2016, neonates whose chromosomal and genomic abnormalities were tested using a-CGH in cases of an abnormal finding on echocardiography were enrolled...
March 2018: Korean Circulation Journal
K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
Sharon L Paige, Priyanka Saha, James R Priest
No abstract text is available yet for this article.
March 2018: Circ Genom Precis Med
Justin O Szot, Hartmut Cuny, Gillian M Blue, David T Humphreys, Eddie Ip, Katrina Harrison, Gary F Sholler, Eleni Giannoulatou, Paul Leo, Emma L Duncan, Duncan B Sparrow, Joshua W K Ho, Robert M Graham, Nicholas Pachter, Gavin Chapman, David S Winlaw, Sally L Dunwoodie
BACKGROUND: Congenital heart disease (CHD)-structural abnormalities of the heart that arise during embryonic development-is the most common inborn malformation, affecting ≤1% of the population. However, currently, only a minority of cases can be explained by genetic abnormalities. The goal of this study was to identify disease-causal genetic variants in 30 families affected by CHD. METHODS: Whole-exome sequencing was performed with the DNA of multiple family members...
March 2018: Circ Genom Precis Med
Rui Zhang, Caihong Huo, Xingning Wang, Bo Dang, Yaning Mu, Yuying Wang
BACKGROUND/AIMS: Published studies indicated that the MTHFR gene polymorphisms C677T and A1298C are associated with congenital heart disease (CHD) risk in children, but obtained inconsistent results. Our study aims to reach a more accurate association between these two polymorphisms and CHD risk. METHODS: Eligible studies were obtained by screening the PubMed, Embase, China National Knowledge Infrastructure, Wan Fang and VIP databases based on designed searching strategy...
March 15, 2018: Cellular Physiology and Biochemistry
Mingjie Zhang, Zhiyu Feng, Rui Huang, Chongrui Sun, Zhuoming Xu
BACKGROUND Establishing a shunt-induced pulmonary arterial hypertension (PAH) model in mice would be of great scientific value, but no such models have been reported to date. Here, we established a shunt-associated PAH in mice to investigate the characteristics of pulmonary vascular remodeling, which provides a new platform for the in-depth study of PAH associated with congenital heart disease (CHD). MATERIAL AND METHODS Eighty mice were randomly divided into the heavy shunt group (n=32), the small shunt group (n=32), the sham operation group (n=8), and the control group (n=8)...
March 19, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
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
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