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Adult congenital heart

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https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#1
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28439697/heart-transplantation-for-adults-with-congenital-heart-disease-current-status-and-future-prospects
#2
REVIEW
Hikaru Matsuda, Hajime Ichikawa, Takayoshi Ueno, Yoshiki Sawa
Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved...
April 24, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28437783/evacuation-of-a-tertiary-neonatal-centre-lessons-from-the-2016-kumamoto-earthquakes
#3
Osuke Iwata, Akihiko Kawase, Masanori Iwai, Kazuko Wada
BACKGROUND: Newborn infants hospitalised in the neonatal intensive care unit (NICU) are vulnerable to natural disasters. However, publications on evacuation from NICUs are sparse. The 2016 Kumamoto Earthquakes caused serious damage to Kumamoto City Hospital and its level III regional core NICU. Local/neighbour NICU teams and the disaster-communication team of a neonatal academic society cooperated to evacuate 38 newborn infants from the ward. OBJECTIVE: The aim of this paper was to highlight potential key factors to improve emergency NICU evacuation and coordination of hospital transportation following natural disasters...
April 25, 2017: Neonatology
https://www.readbyqxmd.com/read/28436549/atrial-undersensing-secondary-to-quiet-timer-blanking-in-pediatric-and-congenital-heart-disease-patients
#4
Johannes C von Alvensleben, Michael Schaffer, Caitlin Brateng, Kathryn K Collins
BACKGROUND: Atrial undersensing despite normal atrial lead parameters and high amplitude electrograms has been described in adult patients as a consequence of atrial amplifier saturation. Repetitive inputs cause amplifier ringing and undersensing occurs as consequence of quiet timer blanking. High amplitude ventricular electrograms can also result in atrial blanking secondary to cross chamber effect. This has not been described in pediatric patients or epicardial pacing systems and specific risk factors are not known...
April 24, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28432659/current-role-of-blood-and-urine-biomarkers-in-the-clinical-care-of-adults-with-congenital-heart-disease
#5
REVIEW
Saurabh Rajpal, Laith Alshawabkeh, Alexander R Opotowsky
PURPOSE OF REVIEW: There is an increasing number of adult patients with congenital heart disease (CHD). While several biomarkers have been validated and integrated into general cardiology clinical practice, these tests are often applied to adults with CHD in the absence of disease-specific validation. Although these patients are often grouped into a single population, there is heterogeneous pathophysiology, variable disease chronicity, extensive multisystem involvement, and a low event rate relative to acquired heart disease...
June 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28430913/prediction-of-adverse-events-after-catheter-based-procedures-in-adolescents-and-adults-with-congenital-heart-disease-in-the-impact-registry
#6
Ada C Stefanescu Schmidt, Aimee Armstrong, Kevin F Kennedy, David Nykanen, Jamil Aboulhosn, Ami B Bhatt
Aims: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population. Methods and results: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States...
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28427867/outcomes-from-anomalous-origin-of-the-left-coronary-artery-from-the-pulmonary-artery-repair-long-term-complications-in-relation-to-residual-myocardial-abnormalities
#7
Miki Kanoh, Kei Inai, Tokuko Shinohara, Hirofumi Tomimatsu, Toshio Nakanishi
BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery irregularity. This study aimed to clarify the long-term postoperative outcomes in ALCAPA patients, and to compare the infantile and adult types. METHODS: We retrospectively analyzed the clinical data from 33 patients with ALCAPA who underwent surgical repairs after 1980. The patients were grouped based on whether presentation occurred before (infantile type: n=14) or after (adult type: n=19) 1 year of age...
April 17, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28420661/person-centred-transition-programme-to-empower-adolescents-with-congenital-heart-disease-in-the-transition-to-adulthood-a-study-protocol-for-a-hybrid-randomised-controlled-trial-stepstones-project
#8
Mariela Acuña Mora, Carina Sparud-Lundin, Ewa-Lena Bratt, Philip Moons
INTRODUCTION: When a young person grows up, they evolve from an independent child to an empowered adult. If an individual has a chronic condition, this additional burden may hamper adequate development and independence. Transition programmes for young persons with chronic disorders aim to provide the necessary skills for self-management and participation in care. However, strong evidence on the effects of these interventions is lacking. Therefore, as part of the STEPSTONES project (Swedish Transition Effects Project Supporting Teenagers with chrONic mEdical conditionS), we propose a trial to assess the effectiveness of a structured, person-centred transition programme to empower adolescents with congenital heart disease in the transition to adulthood...
April 17, 2017: BMJ Open
https://www.readbyqxmd.com/read/28416028/attitudes-and-perceptions-of-pregnant-women-with-chd-results-of-a-single-site-survey
#9
Aarthi Sabanayagam, David Briston, Ali N Zaidi
Introduction CHD occurs in about 1% of the United States population, and is now the most common cardiac condition affecting women during pregnancy. METHODS: An anonymous, single-site, cross-sectional, 50-question survey was performed at a national Adult Congenital Heart Disease conference to assess the level of knowledge, attitudes, and perceptions regarding cardiac care during pregnancy in women with CHD. RESULTS: A total of 77 women completed the survey. Among them, 50% (n=39) had moderate and 38% (n=29) had severely complex disease; 30% (n=23) of women were told that pregnancy was contraindicated given their underlying cardiac condition...
April 18, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28414679/misses-opportunity-tof-diagnoses-in-4th-decade-of-life
#10
O Baniahmad, T Jarreau, A Johnson
CASE: A 40 year old woman with a history of HIV, congestive heart failure secondary to an unknown congenital heart defect, and hypertension presented to our emergency department with worsening edema. On room air, oxygen saturation was 55 percent . On 5L of oxygen via nasal cannula, oxygen saturation was 88 percent . Physical examination was notable for central cyanosis, facial and lid edema, a II/VI holosystolic murmur across right chest radiating to entire right back hemithorax, decreased breath sounds at bases with pulmonary crackles, clubbing of fingers and edema of bilateral lower extremities...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#11
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28403448/pregnancy-in-adult-congenital-heart-disease-special-delivery
#12
William R Davidson
No abstract text is available yet for this article.
April 12, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28402450/sudden-cardiac-death-in-adults-with-congenital-heart-disease-does-qrs-complex-fragmentation-discriminate-in-structurally-abnormal-hearts
#13
Jim T Vehmeijer, Zeliha Koyak, Jouke P Bokma, Werner Budts, Louise Harris, Barbara J M Mulder, Joris R de Groot
Aims: Sudden cardiac death (SCD) causes a large portion of all mortality in adult congenital heart disease (ACHD) patients. However, identification of high-risk patients remains challenging. Fragmented QRS-complexes (fQRS) are a marker for SCD in patients with acquired heart disease but data in ACHD patients are lacking. We therefore aim to evaluate the prognostic value of fQRS for SCD in ACHD patients. Methods and results: From a multicentre cohort of 25 790 ACHD patients, we included tachyarrhythmic SCD cases (n = 147), and controls (n = 266) matched by age, gender, congenital defect and (surgical) intervention...
April 10, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28393785/modifying-risks-to-improve-outcome-in-cardiac-surgery-an-anesthesiologist-s-perspective
#14
REVIEW
Murali Chakravarthy
Challenging times are here for cardiac surgical and anesthesia team. The interventional cardiologist seem to have closed the flow of 'good cases' coming up for any of the surgery,; successful percutaneous interventions seem to be offering reasonable results in these patients, who therefore do not knock on the doors of the surgeons any more . It is a common experience among the cardiac anesthesiologists and surgeons that the type of the cases that come by now are high risk. That may be presence of comorbidities, ongoing medical therapies, unstable angina, uncontrolled heart failure and rhythm disturbances; and in patients with ischemic heart disease, the target coronaries are far from ideal...
April 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28393063/neuropsychological-and-psychiatric-outcomes-in-dextro-transposition-of-the-great-arteries-across-the-lifespan-a-state-of-the-art-review
#15
REVIEW
Leila Kasmi, Damien Bonnet, Michèle Montreuil, David Kalfa, Nikoletta Geronikola, David C Bellinger, Johanna Calderon
Advances in prenatal diagnosis, perioperative management, and postoperative care have dramatically increased the population of survivors of neonatal and infant heart surgery. The high survival rate of these patients into adulthood has exposed the alarming prevalence of long-term neuropsychological and psychiatric morbidities. Dextro-transposition of the great arteries (d-TGA) is one of the most extensively studied cyanotic congenital heart defect (CHD) with regard to neurodevelopmental outcomes. Landmark studies have described a common neurodevelopmental and behavioral phenotype associated with d-TGA...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28382463/micrornas-association-in-the-cardiac-hypertrophy-secondary-to-complex-congenital-heart-disease-in-children
#16
Ma C Sánchez-Gómez, K A García-Mejía, M Pérez-Díaz Conti, G Díaz-Rosas, I Palma-Lara, R Sánchez-Urbina, M Klünder-Klünder, J A Botello-Flores, N A Balderrábano-Saucedo, A Contreras-Ramos
Complex congenital heart disease (CHD) affects cardiac blood flow, generating a pressure overload in the compromised ventricles and provoking hypertrophy that over time will induce myocardial dysfunction and cause a potential risk of imminent death. Therefore, the early diagnosis of complex CHD is paramount during the first year of life, with surgical treatment of patients favoring survival. In the present study, we analyzed cardiac tissue and plasma of children with cardiac hypertrophy (CH) secondary to CHD for the expression of 11 miRNAs specific to CH in adults...
April 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28377475/diagnosis-imaging-and-clinical-management-of-aortic-coarctation
#17
REVIEW
Elles J Dijkema, Tim Leiner, Heynric B Grotenhuis
Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV)...
April 4, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28375677/cardiopulmonary-exercise-testing-in-adult-congenital-heart-disease
#18
Valentina Mantegazza, Anna Apostolo, Alfred Hager
Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries and new issues are emerging regarding them: the evaluation of their capacity to cope with physical activity, and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, pulmonary hypertension) and help prescribe an individualized rehabilitation program when needed...
April 4, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28374180/coronary-artery-anomalies-when-you-need-to-worry
#19
REVIEW
Ajar Kochar, Todd Kiefer
PURPOSE OF REVIEW: There is a broad spectrum of coronary artery anomalies that cardiologists may encounter either incidentally or during evaluation for cardiac symptoms. These anomalies include anomalous coronary arteries arising from the opposite sinus of Valsalva (ACAOS), coronary fistulae, and coronary artery aneurysms. This manuscript outlines the unique features, diagnostic characteristics, and treatment considerations for these lesions. RECENT FINDINGS: Intravenous ultrasound (IVUS), computed tomographic angiography (CTA), and magnetic resonance imaging (MRI) are becoming more sophisticated and will be increasingly used to facilitate the optimal treatment approach for coronary anomalies...
May 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28373784/left-ventricular-noncompaction-a-rare-form-of-cardiomyopathy-revelation-modes-and-predictors-of-mortality-in-adults-through-23-cases
#20
Iliyasse Asfalou, Sanae Boulaamayl, Maha Raissouni, Najat Mouine, Mohamed Sabry, Jamal Kheyi, Nawal Doghmi, Aatif Benyass
OBJECTIVES: To describe modes of clinical presentation and echocardiographic, angiographic, and rhythmic features, and prognostic characteristics of left ventricular noncompaction cardiomyopathy (LVNC) in North African adults, through one of the first series in Morocco. BACKGROUND: LVNC is a rare congenital disorder, described for the first time by Engberding in 1984. The suspected diagnosis in thromboembolic, hemodynamic, or rhythm events requires both echocardiography and cardiovascular magnetic resonance (CMR)...
April 2017: Journal of the Saudi Heart Association
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