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https://www.readbyqxmd.com/read/29792339/pulmonary-hypertension-in-congenital-heart-disease
#1
Emma Pascall, Robert Mr Tulloh
Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex...
May 24, 2018: Future Cardiology
https://www.readbyqxmd.com/read/29789917/comparison-of-clinical-profiles-in-patients-with-protein-losing-enteropathy-with-and-without-fontan-circulation
#2
Shin Ono, Hideo Ohuchi, Aya Miyazaki, Osamu Yamada
Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs...
May 22, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29787864/the-neonatal-brain-in-critical-congenital-heart-disease-insights-and-future-directions
#3
REVIEW
Shabnam Peyvandi, Beatrice Latal, Steven P Miller, Patrick S McQuillen
Neurodevelopmental outcomes are impaired in survivors of critical congenital heart disease (CHD) in several developmental domains including motor, cognitive and sensory outcomes. These deficits can extend into the adolescent and early adulthood years. The cause of these neurodevelopmental impairments is multi-factorial and includes patient specific risk factors, cardiac anatomy and physiology as well as brain changes seen on MRI. Advances in imaging techniques have identified delayed brain development in the neonate with critical CHD as well as acquired brain injury...
May 19, 2018: NeuroImage
https://www.readbyqxmd.com/read/29786701/lutembacher-syndrome-with-mitral-valve-calcification-in-a-31-year-old-male
#4
Arsalan Majeed Adam, Ansab Godil, Muhammad Saad Ali Mallick, Fahad Khan, Ather Hasan Rizvi, Inam-Ul-Haq Muhammad Makhdoom
Lutembacher syndrome is characterized by a congenital ostium secundum atrial septal defect and an acquired mitral valve stenosis. We present a similar case in a 31-year old male who came in with orthopnoea, central cyanosis and pedal oedema. Examination revealed cardiac murmurs in tricuspid and apical regions. Chest x-ray showed signs of pulmonary congestion and ventricular enlargement. Electrocardiogaphy (ECG) revealed right axis deviation and right bundle branch block along with atrial fibrillation and Transthoracic Echocardiography (TTE) showed abnormal valves (mitral stenosis with calcification and tricuspid regurgitation) and dilated cardiac chambers...
February 2018: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/29786408/impact-of-additional-tricuspid-valve-annuloplasty-in-tof-patients-undergoing-pulmonary-valve-replacement
#5
Sabrina Lueck, Eike Bormann, Kathrin Rellensmann, Sven Martens, Andreas Rukosujew
BACKGROUND: Many patients with tetralogy of Fallot (TOF) who underwent surgical correction of their congenital cardiac malformation during infancy develop right ventricular dysfunction and exercise intolerance in the long term. The right ventricle (RV) dilates due to the development of severe pulmonary regurgitation (and secondary tricuspid insufficiency). To reduce RV dilation and improve exercise tolerance pulmonary valve replacement (PVR) is the common therapeutic strategy. Whether concomitant tricuspid valve repair (TVR) is beneficial in these pure volume-overload conditions is still unknown...
May 22, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29785061/targeted-neonatal-echocardiography-tne-consult-service-in-a-large-tertiary-perinatal-center-in-canada
#6
Ismina Papadhima, Deepak Louis, Jyotsna Purna, Poorva Deshpande, Yenge Diambomba, Shoo Lee, Prakesh Shah, Dany Weisz, Afif El-Khuffash, Patrick J McNamara, Luc Mertens, Amish Jain
OBJECTIVE: To describe the utilization and study the factors associated with the impact on clinical management of a new TNE consultation service in a perinatal center. METHODS: This retrospective cohort study included all neonates who underwent TNE consultation at the neonatal unit of Mount Sinai Hospital in Toronto, Canada (November 2011 and July 2015). The consults that had "impact" were defined as those that led to a TNE suggested change in the clinical management within 6 h of its recommendation...
May 22, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29781990/fetal-mouse-cardiovascular-imaging-using-a-high-frequency-ultrasound-30-45mhz-system
#7
Marlin Touma
Congenital heart defects (CHDs) are the most common cause of childhood morbidity and early mortality. Prenatal detection of the underlying molecular mechanisms of CHDs is crucial for inventing new preventive and therapeutic strategies. Mutant mouse models are powerful tools to discover new mechanisms and environmental stress modifiers that drive cardiac development and their potential alteration in CHDs. However, efforts to establish the causality of these putative contributors have been limited to histological and molecular studies in non-survival animal experiments, in which monitoring the key physiological and hemodynamic parameters is often absent...
May 5, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29780722/coarctation-repair-redo-challenges-in-the-adults-what-to-do
#8
REVIEW
Erik Beckmann, Arminder S Jassar
Aortic coarctation is one of the most common congenital cardiac pathologies. Repair of native aortic coarctation is nowadays a common and safe procedure. However, late complications, including re-coarctation and aneurysm formation, are not uncommon. The incidence of these complications is dependent on the type of the initial operation. Both endovascular and conventional open repair play important roles in the treatment of late complications after previous coarctation repair. This article will review the incidence of late complications after coarctation repair and will discuss the treatment options for redo coarctation repair in adult patients...
2018: Journal of Visualized Surgery
https://www.readbyqxmd.com/read/29779802/feasibility-of-initiating-early-enteral-nutrition-after-congenital-heart-surgery-in-neonates-and-infants
#9
Rajat Kalra, Rohit Vohra, Malti Negi, Reena Joshi, Neeraj Aggarwal, Mridul Aggarwal, Raja Joshi
OBJECTIVE: To assess the feasibility of initiating enteral nutrition support with first 24 h of congenital heart repairs in neonates and Infants and its impact on outcomes following surgery. DESIGN: It is a prospective randomized control single blind study. SETTING: It is a single centre prospective study carried out in a tertiary care centre at Pediatric cardiac intensive care unit. PATIENTS: All patients with the cyanotic congenital heart disease with increase pulmonary blood flow, weighing less than 5 kg and undergoing congenital heart repair during the study period were included in the study...
June 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29776940/successful-treatment-of-fulminant-neonatal-enteroviral-myocarditis-in-monochorionic-diamniotic-twins-with-cardiopulmonary-support-intravenous-immunoglobulin-and-pocapavir
#10
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/29776810/congenital-heart-disease-in-adults-assessmentof-functional-capacity-using-cardiopulmonary-exercise-testing
#11
Sílvia Aguiar Rosa, Ana Agapito, Rui M Soares, Lídia Sousa, José Alberto Oliveira, Ana Abreu, Ana Sofia Silva, Sandra Alves, Helena Aidos, Fátima F Pinto, Rui Cruz Ferreira
AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction...
May 15, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29776787/the-value-of-peripheral-perfusion-index-measurements-for-early-detection-of-critical-cardiac-defects
#12
Ozgun Uygur, Ozge Altun Koroglu, Erturk Levent, Merve Tosyali, Mete Akisu, Mehmet Yalaz, Nilgun Kultursay
BACKGROUND: Approximately 25% of congenital heart diseases (CHD) are estimated to be critical and require an intervention. In this study, we aimed to investigate the additional value of peripheral perfusion index (PPI) measurements to pulse oximetry screening for critical CHD (CCHD). METHODS: Infants born at Ege University Hospital between May 2013 and September 2015 were prospectively included in the study. In addition to physical examination, pre- and postductal oxygen saturations and PPI values were measured with a new generation pulse oximeter before discharge from the hospital...
April 12, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29774307/bethlem-myopathy-in-a-portuguese-patient-case-report
#13
Ana Inês Martins, Cristin Maarque, Jorge Pinto-Basto, Luis Negrão
Mutations of the encoding genes of collagen VI (COL6A1, COL6A2 and COL6A3 ), are responsible for two classical phenotypes (with a wide range of severity), the Ullrich congenital muscular dystrophy (UCMD) and the Bethlem myopathy (BM). We present a male patient of 49 years old, with symptoms of muscle weakness beginning in childhood and of very slowly progression. At the age of 42, the neurological examination revealed proximal lower limb muscle weakness and contractures of fingers flexors muscles, positive Gowers manoeuvre and a waddling gait...
September 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29772208/influence-of-blood-flow-on-cardiac-development
#14
REVIEW
Katherine Courchaine, Graham Rykiel, Sandra Rugonyi
The role of hemodynamics in cardiovascular development is not well understood. Indeed, it would be remarkable if it were, given the dauntingly complex array of intricately synchronized genetic, molecular, mechanical, and environmental factors at play. However, with congenital heart defects affecting around 1 in 100 human births, and numerous studies pointing to hemodynamics as a factor in cardiovascular morphogenesis, this is not an area in which we can afford to remain in the dark. This review seeks to present the case for the importance of research into the biomechanics of the developing cardiovascular system...
May 14, 2018: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/29770300/tissue-engineered-vascular-grafts-for-pediatric-cardiac-surgery
#15
REVIEW
Toshihiro Shoji, Toshiharu Shinoka
New technologies and science have contributed to improved surgical outcomes in patients with congenital cardiovascular diseases. However, current materials display shortcomings, such as risk of infection and lack of growth capacity when applied to the pediatric patient population. Tissue engineering has the potential to address these limitations as the ideal tissue engineered vascular graft (TEVG) would be durable, biocompatible, nonthrombogenic, and ultimately remodel into native tissue. The traditional TEVG paradigm consists of a scaffold, cell source, and the integration of the scaffold and cells via seeding...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#16
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/29770295/recent-innovations-in-perfusion-and-cardiopulmonary-bypass-for-neonatal-and-infant-cardiac-surgery
#17
REVIEW
David Sturmer, Claude Beaty, Sean Clingan, Eric Jenkins, Whitney Peters, Ming-Sing Si
The development and refinement of cardiopulmonary bypass (CPB) has made the repair of complex congenital heart defects possible in neonates and infants. In the past, the primary goal for these procedures was patient survival. Now that substantial survival rates have been achieved for even the most complex of repairs in these patients, focus has been given to the reduction of morbidity. Although a necessity for these complex neonatal and infant heart defect repairs, CPB can also be an important source of perioperative complications...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770294/three-dimensional-modelling-and-three-dimensional-printing-in-pediatric-and-congenital-cardiac-surgery
#18
REVIEW
Laszlo Kiraly
Three-dimensional (3D) modelling and printing methods greatly support advances in individualized medicine and surgery. In pediatric and congenital cardiac surgery, personalized imaging and 3D modelling presents with a range of advantages, e.g., better understanding of complex anatomy, interactivity and hands-on approach, possibility for preoperative surgical planning and virtual surgery, ability to assess expected results, and improved communication within the multidisciplinary team and with patients. 3D virtual and printed models often add important new anatomical findings and prompt alternative operative scenarios...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770292/innovative-interventional-catheterization-techniques-for-congenital-heart-disease
#19
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/29770291/a-review-of-the-nunn-modified-single-patch-technique-for-atrioventricular-septal-defect-repair
#20
REVIEW
Tracy R Geoffrion, Kanchana Singappuli, John S K Murala
Atrioventricular septal defect (AVSD) is a common congenital cardiac surgical problem. Over the years, younger and smaller infants are having operations for this condition before irreversible cardio pulmonary changes occur. Traditionally a single or two patch techniques have been used to repair this defect. However, in the past two decades an innovative method of modified single patch technique popularized by Dr. Graham Nunn has gained worldwide popularity. This review discusses the origin, surgical principles, technique and outcomes of this method, popularly known as Nunn or Australian technique...
April 2018: Translational Pediatrics
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