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treatment of congenital heart disease

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https://www.readbyqxmd.com/read/29776940/successful-treatment-of-fulminant-neonatal-enteroviral-myocarditis-in-monochorionic-diamniotic-twins-with-cardiopulmonary-support-intravenous-immunoglobulin-and-pocapavir
#1
Shahnawaz M Amdani, Hannah S Kim, Anthony Orvedahl, Audrey Odom John, Ahmed Said, Kathleen Simpson
Neonatal cardiogenic shock most commonly occurs due to critical congenital heart disease, sepsis, metabolic disorder or arrhythmias. In particular, enterovirus infections are common in the neonatal period, and patients can present with fulminant myocarditis. Early recognition is imperative due to its high morbidity and mortality without prompt and aggressive treatment. We present the successful treatment of fulminant neonatal enteroviral myocarditis in a pair of monochorionic diamniotic twins with cardiopulmonary support, intravenous immunoglobulin and pocapavir, an enteroviral capsid inhibitor...
May 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#2
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770292/innovative-interventional-catheterization-techniques-for-congenital-heart-disease
#3
REVIEW
Jeffrey D Zampi, Wendy Whiteside
Since 1929, when the first cardiac catheterization was safely performed in a human by Dr. Werner Forssmann (on himself), there has been a rapid progression of cardiac catheterization techniques and technologies. Today, these advances allow us to treat a wide variety of patients with congenital heart disease using minimally invasive techniques; from fetus to infants to adults, and from simple to complex congenital cardiac lesions. In this article, we will explore some of the exciting advances in cardiac catheterization for the treatment of congenital heart disease, including transcatheter valve implantation, hybrid procedures, biodegradable technologies, and magnetic resonance imaging (MRI)-guided catheterization...
April 2018: Translational pediatrics
https://www.readbyqxmd.com/read/29765932/fluid-overload-and-renal-angina-index-at-admission-are-associated-with-worse-outcomes-in-critically-ill-children
#4
Sidharth K Sethi, Veena Raghunathan, Shilpi Shah, Maninder Dhaliwal, Pranaw Jha, Maneesh Kumar, Sravanthi Paluri, Shyam Bansal, Maroun J Mhanna, Rupesh Raina
Objectives: We investigated the association of fluid overload and oxygenation in critically sick children, and their correlation with various outcomes (duration of ventilation, ICU stay, and mortality). We also assessed whether renal angina index (RAI) at admission can predict mortality or acute kidney injury (AKI) on day 3 after admission. Design and setting: Prospective study, pediatric intensive care in a tertiary hospital. Duration: June 2013-June 2014. Patients: Patients were included if they needed invasive mechanical ventilation for >24 h and had an indwelling arterial catheter...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29764528/symptomatic-myocardial-bridging-a-frequently-occurring-coronary-variation-can-cause-severe-myocardial-ischaemia-in-affected-children-with-underlying-cardiac-conditions
#5
Alexandra Kiess, Marcel Vollroth, Farhad Bakhtiary, Hiroshi Seki, Martin Kostelka, Milan Djukic, Ingo Daehnert, Robert Wagner
Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases...
May 16, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29762234/quality-and-safety-in-health-care-part-xli-the-impact-registry
#6
Jay A Harolds
The IMPACT Registry is a repository of information for heart catheterizations for congenital heart disease regardless of age and also the catheterizations for acquired heart disease in children. The registry collects information on outcomes, provides quality improvement opportunities for participants, provides reports to participants comparing their results with national results, compares the volume of catheter work done at an institution with the frequency of adverse events, and provides information that may be helpful in evaluating the use of medical devices and treatment options...
May 14, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29761681/milestones-in-the-development-of-fetal-cardiac-interventions
#7
REVIEW
Edward G Abinader
Cardiac patients of all ages were managed in the past by internists who specialized in cardiology. During the past 50 years, the medical field has witnessed great strides in the management of congenital heart disease, and thus pediatric cardiology has become a subspecialty in many countries. This review article focuses on the advances in fetal cardiac interventions (FCI) since its inception by our group in 1975. Three major modes of FCI have evolved during the past 42 years: pharmacologic, closed FCI, and open FCI...
May 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29761422/computational-pre-surgical-planning-of-arterial-patch-reconstruction-parametric-limits-and-in-vitro-validation
#8
S Samaneh Lashkarinia, Senol Piskin, Tijen A Bozkaya, Ece Salihoglu, Can Yerebakan, Kerem Pekkan
Surgical treatment of congenital heart disease (CHD) involves complex vascular reconstructions utilizing artificial and native surgical materials. A successful surgical reconstruction achieves an optimal hemodynamic profile through the graft in spite of the complex post-operative vessel growth pattern and the altered pressure loading. This paper proposes a new in silico patient-specific pre-surgical planning framework for patch reconstruction and investigates its computational feasibility. The proposed protocol is applied to the patch repair of main pulmonary artery (MPA) stenosis in the Tetralogy of Fallot CHD template...
May 14, 2018: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/29757759/mechanical-circulatory-support-of-the-right-ventricle-for-adult-and-pediatric-patients-with-heart-failure
#9
Steven G Chopski, Nohra M Murad, Carson S Fox, Randy M Stevens, Amy L Throckmorton
The clinical implementation of mechanical circulatory assistance for a significantly dysfunctional or failing left ventricle as a bridge-to-transplant or bridge-to-recovery is on the rise. Thousands of patients with left-sided heart failure are readily benefitting from these life-saving technologies, and left ventricular failure often leads to severe right ventricular dysfunction or failure. Right ventricular failure (RVF) has a high rate of mortality caused by the risk of multisystem organ failure and prolonged hospitalization for patients after treatment...
May 10, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29754203/prospective-analysis-of-decision-making-during-joint-cardiology-cardiothoracic-conference-in-treatment-of-107-consecutive-children-with-congenital-heart-disease
#10
Sophie Duignan, Aedin Ryan, Dara O'Keeffe, Damien Kenny, Colin J McMahon
The complexity and potential biases involved in decision making have long been recognised and examined in both the aviation and business industries. More recently, the medical community have started to explore this concept and its particular importance in our field. Paediatric cardiology is a rapidly expanding field and for many of the conditions we treat, there is limited evidence available to support our decision-making. Variability exists within decision-making in paediatric cardiology and this may influence outcomes...
May 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29750285/updates-in-newborn-screening
#11
Farrah Rajabi
Newborn screening in the United States is an important public health measure to provide early detection for specified disorders when early treatment is both possible and beneficial. As technology improves, newborn screening can be offered for many more conditions. In the past 10 years, screening has expanded to include severe combined immunodeficiency, congenital heart disease, lysosomal storage disease, and X-linked adrenoleukodystrophy. This article reviews the current state of newborn screening with updates on recent developments...
May 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29744657/transcatheter-intervention-of-coronary-obstructions-in-infants-children-and-young-adults
#12
Ryan Callahan, James E Lock, Pinak B Shah, Audrey C Marshall
This study assesses the characteristics of coronary obstructions that underwent transcatheter intervention in a pediatric catheterization laboratory, the procedural details, and patient outcomes. Acute cardiac failure due to coronary obstructions in children is rare. The role of catheter based intervention is largely unreported. Single center retrospective review between January 2000 and December 2016. Thirty-three patients (median age 2y/o [0-38], weighing 9.6 kg [2.2-91]) underwent 39 transcatheter interventions on 39 lesions, mainly left main coronary (16/39; 39%) and right coronary (9/39; 23%) arteries...
May 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29740591/postnatal-and-adult-aortic-heart-valves-have-distinctive-transcriptional-profiles-associated-with-valve-tissue-growth-and-maintenance-respectively
#13
Emily Nordquist, Stephanie LaHaye, Casey Nagel, Joy Lincoln
Heart valves are organized connective tissues of high mechanical demand. They open and close over 100,000 times a day to preserve unidirectional blood flow by maintaining structure-function relationships throughout life. In affected individuals, structural failure compromises function and often leads to regurgitant blood flow and progressive heart failure. This is most common in degenerative valve disease due to age-related wear and tear, or congenital malformations. At present, the only effective treatment of valve disease is surgical repair or replacement and this is often impermanent and requires anti-coagulation therapy throughout life...
2018: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29737105/-analysis-of-pathogens-and-clinical-characteristics-in-157-cases-with-infective-endocarditis
#14
Fei-Fei Zhao, Yang Lu, Hui Ye, Ce-Jun Zhong, Liang Huang, Xiao-Ju Lü
OBJECTIVE: To analyze the clinical characteristics of infective endocarditis (IE) in culture-positive patients,so as to provide the evidences for reasonable diagnosis and treatment of IE. METHODS: We performed a retrospective study of 157 culture-positive IE cases,which were diagnosed according to modified Duke criteria for IE from Jan. 2008 to Aug. 2015. RESULTS: The average age of 157 cases of IE was 40.85 years. One hundred and one patients (64...
January 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29731834/diagnosis-and-treatment-of-postoperative-intestinal-perforation-in-infants-and-young-children-with-congenital-heart-disease-a-report-of-three-cases
#15
Yong-Hong Zhang, Zhen-Liang Chen, Lei Shi, Zhong-Jian Chen, Xiang-Yang Dong, Bo Zhai
The aim of the present study was to analyze risk factors of intestinal perforation following surgery for the treatment of congenital heart disease in infants and young children, and to summarize experiences of diagnosis and treatment. A total of 3,270 children, who underwent congenital heart disease surgery under extracorporeal circulation from January 2010 to July 2015, were retrospectively analyzed. Among these children, three (0.09%) developed postoperative intestinal perforation. Primary diseases were Tetralogy of Fallot (two cases) and ventricular septal defect combined with atrial septal defect (one case)...
May 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29721915/clinical-biochemical-and-molecular-overview-of-transaldolase-deficiency-and-evaluation-of-the-endocrine-function-update-of-34-patients
#16
M Williams, V Valayannopoulos, R Altassan, W K Chung, A C Heijboer, W T Keng, R Lapatto, P McClean, M F Mulder, A Tylki-Szymańska, M J E Walenkamp, M Alfadhel, H Alakeel, G S Salomons, W Eyaid, M M C Wamelink
BACKGROUND: Transaldolase deficiency (TALDO-D) is a rare autosomal recessive inborn error of the pentose phosphate pathway. Since its first description in 2001, several case reports have been published, but there has been no comprehensive overview of phenotype, genotype, and phenotype-genotype correlation. METHODS: We performed a retrospective questionnaire and literature study of clinical, biochemical, and molecular data of 34 patients from 25 families with proven TALDO-D...
May 2, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29720396/primary-repair-versus-surgical-and-transcatheter-palliation-in-infants-with-tetralogy-of-fallot
#17
Dan M Dorobantu, Alireza S Mahani, Mansour T A Sharabiani, Ragini Pandey, Gianni D Angelini, Andrew J Parry, Robert M R Tulloh, Robin P Martin, Serban C Stoica
OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered...
May 2, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29719528/co-occurrence-of-pheochromocytoma-paraganglioma-and-cyanotic-congenital-heart-disease-a-case-report-and-literature-review
#18
Bingbin Zhao, Yi Zhou, Yi Zhao, Yumo Zhao, Xingcheng Wu, Yalan Bi, Yufeng Luo, Zhigang Ji, Shi Rong
Pheochromocytoma and paraganglioma (PHEO-PGL) and cyanotic congenital heart disease (CCHD) are both rare diseases. We reported a 30-year-old patient with a right adrenal gland nodule and a retroperitoneal mass and history of functional single atrium and ventricle. 123 I-metaiodobenzylguanidine scintigraphy showed intense uptake in both lesions. Laboratory investigation demonstrated elevated urinary norepinephrine. Preoperative α-blockade was initiated. A successful open resection of right adrenal and retroperitoneal masses was performed...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29717916/risk-factors-associated-with-the-development-of-acquired-airway-disease-after-congenital-heart-surgery-a-retrospective-cohort-study
#19
Andrew J Matisoff, Pranathi Ari, David Zurakowski, Alexandra G Espinel, Nina Deutsch, Brian K Reilly
OBJECTIVE: In this single-center, retrospective review, we sought to determine the risk factors associated with the development of severe acquired airway disease (AAD; vocal cord paralysis [VCP] or subglottic stenosis [SGS]) in pediatric patients who had undergone surgery for congenital heart disease (CHD) with cardiopulmonary bypass. All patients who required surgical treatment for CHD using cardiopulmonary bypass at our institution between 2010 and 2015 were reviewed. We defined severe AAD as either clinically significant VCP, SGS, or both, requiring consultation with the otolaryngology (ENT) service for evaluation...
April 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29716711/cardiac-magnetic-resonance-imaging-myocardial-scar-and-coronary-flow-pattern-in-anomalous-origin-of-left-coronary-artery-from-the-pulmonary-artery
#20
Parag Bhalgat, Abhijeet Naik, Prasanna Salvi, Nilesh Bhadane, Kshiti Shah, Bhawan Paunipagar, Suresh Joshi
BACKGROUND: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect characterized by myocardial ischemia and ultimately scaring. The scar burden will determine eventual recovery of left ventricular function after corrective surgery. MATERIAL METHOD: All patients with proven diagnosis of ALCAPA and who underwent treatment at present centre were included. Detail echocardiography and cardiac magnetic resonance imaging (CMR) (delayed Gadolinium enhancement) was performed before and after surgery...
March 2018: Indian Heart Journal
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