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Congenital Heart Disease

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https://www.readbyqxmd.com/read/28643441/coronary-artery-anomalies-a-multidisciplinary-approach-to-shape-the-landscape-of-a-challenging-problem
#1
Silvana Molossi, Hitesh Agrawal
Coronary artery anomalies, particularly anomalous aortic origin of a coronary artery with an interarterial course, are associated with sudden cardiac arrest or death in the young. There is paucity of data on risk stratification and longitudinal follow up is lacking in these patients. Collaboration and sharing of data among specialized centers might shed much needed light in this complex problem.
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#2
Monique M Gardner, Meryl S Cohen
BACKGROUND: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. METHODS: We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28643385/the-effect-of-balloon-valvuloplasty-for-bioprosthetic-valve-stenosis-at-pulmonary-positions
#3
Eun Young Choi, Jinyoung Song, Heirim Lee, Chang Ha Lee, Jun Huh, I-Seok Kang, Ji Hyuk Yang, Tae Gook Jun
BACKGROUND: Balloon dilatation of a bioprosthetic valve in the pulmonary position could be performed to delay valve replacement. We proposed to identify the long-term effectiveness of such a procedure. METHODS: We reviewed the medical records of 49 patients who underwent balloon valvuloplasty between January 2000 and December 2015. The primary goal was to determine the time interval until the following surgical or catheter intervention. RESULTS: The mean age at bioprosthetic valve insertion was 5...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28639359/interobserver-variability-in-the-classification-of-congenital-coronary-abnormalities-a-substudy-of-the-anomalous-connections-of-the-coronary-arteries-registry
#4
Athanasios Koutsoukis, Xavier Halna du Fretay, Patrick Dupouy, Phalla Ou, Jean-Pierre Laissy, Jean-Michel Juliard, Fabien Hyafil, Pierre Aubry
OBJECTIVE: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort. PATIENTS AND DESIGN: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort...
June 21, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28621042/clinical-evaluation-of-anomalous-aortic-origin-of-a-coronary-artery-aaoca
#5
Silvana Molossi, Hitesh Agrawal
The clinical evaluation of patients with an anomalous aortic origin of a coronary artery (AAOCA), a congenital abnormality of the origin or course of a coronary artery that arises from the aorta, is challenging given its first presentation being sudden cardiac arrest in about half of the patients. Symptoms of chest pain, shortness of breath and syncope during exertion should be of concern in evaluating young athletes and nonathletes. The lack of abnormal signs on the physical exam and electrocardiogram further adds to the difficulty in establishing the diagnosis...
June 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28618202/effects-of-persistent-fontan-fenestration-patency-on-cardiopulmonary-exercise-testing-variables
#6
M Elisabeth Heal, Lanier B Jackson, Andrew M Atz, Ryan J Butts
Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration -4.9 ± 3.8 v. nonfenestration -3 ± 3...
June 15, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28618149/sudden-cardiac-death-in-the-young-epidemiology-and-overview
#7
Mark S Link
Sudden cardiac death (SCD), particularly in the young athlete, is a rare though devastating event for families, institutions, and communities at large. It can also affect the nonathlete and occur at rest, although most commonly associated with exercise activities and/or sports participation. Common causes of SCD include cardiomyopathies, particularly hypertrophic cardiomyopathy in the United States, congenital coronary artery anomalies, channelopathies, among others. This report will explore an overview of the prevalence and causes of SCD in the young...
June 15, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28608519/catheter-based-anatomic-and-functional-assessment-of-coronary-arteries-in-anomalous-aortic-origin-of-a-coronary-artery-myocardial-bridges-and-kawasaki-disease
#8
Athar M Qureshi, Hitesh Agrawal
Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients...
June 13, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28608434/use-of-3d-models-of-vascular-rings-and-slings-to-improve-resident-education
#9
Trahern W Jones, Michael D Seckeler
OBJECTIVE: Three-dimensional (3D) printing is a manufacturing method by which an object is created in an additive process, and can be used with medical imaging data to generate accurate physical reproductions of organs and tissues for a variety of applications. We hypothesized that using 3D printed models of congenital cardiovascular lesions to supplement an educational lecture would improve learners' scores on a board-style examination. DESIGN AND INTERVENTION: Patients with normal and abnormal aortic arches were selected and anonymized to generate 3D printed models...
June 13, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28598588/nutritional-outcomes-in-infants-with-food-allergy-after-cardiac-surgery
#10
Wen-Yi Luo, Zhuo-Ming Xu, Li Hong, Qian-Yue Wu, Yue-Yue Zhang
BACKGROUND: Food allergy is a rapidly growing public health concern because of its increasing prevalence, as well as life-threatening potential. There is limited knowledge on the nutritional status for the pediatric congenital heart disease (CHD) patients with food allergy. OBJECTIVE: This study investigated both clinical and nutritional outcomes according to the CHD infants with food allergy. METHODS: Forty CHD infants with food allergy and 39 controls were recruited in Shanghai, China...
June 9, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28598551/coronary-artery-bypass-grafting-in-infants-children-and-young-adults-for-acquired-and-congenital-lesions
#11
Constantine Mavroudis
Coronary artery disease ranges from congenital in origin such as anomalous aortic origin of a coronary artery (AAOCA) to acquired diseases such as Kawasaki disease, and previously repaired conditions such as transposition of the great arteries. It is not uncommon, in the long run, for proximal coronary stenosis to develop following arterial switch, AAOCA repair, Ross procedure and Kawasaki disease leading to coronary artery bypass grafing (CABG). The objective of this report is to discuss the indications, challenges, and outcomes of CABG in infants, children, and young adults with acquired and congenital lesions...
June 9, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28598033/decision-making-in-anomalous-aortic-origin-of-a-coronary-artery
#12
Carlos M Mery
Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death (SCD) in young athletes in United States. The exact pathophysiological mechanisms of SCD are unknown. There is lack of long-term outcome data on repaired and unrepaired AAOCA and our current risk stratification scheme for these patients is suboptimal. These patients are evaluated in a nonuniform manner across institutions in United States, and even by different providers residing in the same institution...
June 9, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580763/knowledge-based-reconstruction-for-measurement-of-right-ventricular-volumes-on-cardiovascular-magnetic-resonance-images-in-a-mixed-population
#13
Elise D Pieterman, Ricardo P J Budde, Daniëlle Robbers-Visser, Ron T van Domburg, Willem A Helbing
OBJECTIVE: Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observer-dependency of manual analysis of right ventricular volumes limit its use. Knowledge-based reconstruction is a new semiautomatic analysis tool that uses a database including knowledge of right ventricular shape in various congenital heart diseases. We evaluated whether knowledge-based reconstruction is a good alternative for conventional analysis...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580760/stress-echocardiography-an-overview-for-use-in-pediatric-and-congenital-cardiology
#14
Peter Ermis
Currently, the role of stress echocardiography primarily resides in diagnosing acquired coronary artery disease (CAD) in adults. Besides an increasing concern for traditional CAD in young patients due to obesity and other chronic pediatric diseases, there is also a growing population of adolescents and young adults with "at risk" coronary arteries due to: reimplanted coronaries in congenital heart disease, anomalous origin of the native coronary arteries, coronary abnormalities in Kawasaki's disease, and posttransplant coronary vasculopathy...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580730/right-aortic-arch-with-situs-solitus-frequently-heralds-a-vascular-ring
#15
William N Evans, Ruben J Acherman, Michael L Ciccolo, Sergio A Carrillo, Gary A Mayman, Carlos F Luna, Robert C Rollins, William J Castillo, Alvaro Galindo, Abraham Rothman, John A Alexander, Tina W Kwan, Humberto Restrepo
OBJECTIVE: We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS: From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch and situs solitus. From the 50 patients, 6 did not meet inclusion criteria for further analysis. RESULTS: Of the 44 remaining, 33 (75%) had a vascular ring...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580713/mitral-valve-prolapse-and-marfan-syndrome
#16
REVIEW
Amitabh Thacoor
Marfan syndrome is a multisystemic genetic condition affecting connective tissue. It carries a reduced life expectancy, largely dependent on cardiovascular complications. More common cardiac manifestations such as aortic dissection and aortic valve incompetence have been widely documented in the literature. Mitral valve prolapse (MVP), however, has remained poorly documented. This article aims at exploring the existing literature on the pathophysiology and diagnosis of MVP in patients with Marfan syndrome, defining its current management and outlining the future developments surrounding it...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580712/kawasaki-disease-state-of-the-art
#17
Jane W Newburger
Kawasaki disease is an acute febrile arteritis of childhood that can result in coronary artery aneurysms if untreated in the first 10 and ideally 7 days of illness. Kawasaki disease begins as a necrotizing arteritis with neutrophilic infiltrate, followed by subacute/chronic changes and luminal myofibroblastic proliferation that can cause coronary artery stenosis. Manifestations include the presence of ≥5 days of fever, together with clinical criteria of extremity changes, rash, conjunctivitis, oral changes, and unilateral cervical lymphadenopathy...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580660/long-term-outcomes-after-aortic-coarctation-repair-in-maltese-patients-a-population-based-study
#18
Maryanne Caruana, Victor Grech
OBJECTIVES: To investigate survival and freedom from reintervention after aortic coarctation repair in Maltese patients and to compare cardiovascular mortality in coarctation repair survivors with that in the general population. DESIGN: All 72 aortic coarctation patients with any type of repair, born by end-1997 and logged in the local database were included. Trends in timing and type of repair were determined by comparing patients born before and after 1985. Kaplan-Meier analyses of survival and reintervention-free survival were performed on the 59 repair survivors with complete follow-up data (mean follow-up 26...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580658/improvement-in-perioperative-care-in-pediatric-cardiac-surgery-by-shifting-the-primary-focus-of-treatment-from-cardiac-output-to-perfusion-pressure-are-beta-stimulants-still-needed
#19
Amir-Reza Hosseinpour, Mathieu van Steenberghe, Marc-André Bernath, Stefano Di Bernardo, Marie-Hélène Pérez, David Longchamp, Mirko Dolci, Yann Boegli, Nicole Sekarski, Javier Orrit, Michel Hurni, René Prêtre, Jacques Cotting
OBJECTIVE: An important aspect of perioperative care in pediatric cardiac surgery is maintenance of optimal hemodynamic status using vasoactive/inotropic agents. Conventionally, this has focused on maintenance of cardiac output rather than perfusion pressure. However, this approach has been abandoned in our center in favor of one focusing primarily on perfusion pressure, which is presented here and compared to the conventional approach. DESIGN: A retrospective study...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580631/kawasaki-disease-medical-therapies
#20
Jane W Newburger
Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10-12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti-inflammatory and antipyretic effects until the patient has been afebrile for ∼2 days, then lowered to an antiplatelet dose...
June 5, 2017: Congenital Heart Disease
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