Open Access

EDITORIAL

Andrew N. Redington
Congenital Heart Disease, Vol.12, No.5, pp. 559-560, 2017, DOI:10.1111/chd.12527
Abstract This article has no abstract. More >

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Elise D. Pieterman^1,2, Ricardo P. J. Budde², Danielle Robbers-Visser^1,2, Ron T. van Domburg³, Willem A. Helbing^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 561-569, 2017, DOI:10.1111/chd.12484
Abstract 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, observerdependency 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.
Design: To assess the inter- and intra-observer variability and agreement of knowledge-based versus conventional analysis of magnetic resonance right ventricular volumes, analysis was done by two observers in a mixed… More >

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Amir-Reza Hosseinpour¹, Mathieu van Steenberghe¹, Marc-André Bernath², Stefano Di Bernardo³, Marie-Hélène Pérez⁴, David Longchamp⁴, Mirko Dolci², Yann Boegli², Nicole Sekarski³, Javier Orrit¹, Michel Hurni¹, René Prêtre¹, Jacques Cotting⁴
Congenital Heart Disease, Vol.12, No.5, pp. 570-577, 2017, DOI:10.1111/chd.12485
Abstract 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.
Setting: Regional center for congenital heart disease. University Hospital of Lausanne, Switzerland.
Patients: All patients with Aristotle risk score ≥8 that underwent surgery from 1996 to 2012 were included. Patients operated between 1996 and 2005… More >

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Trahern W. Jones, Michael D. Seckeler
Congenital Heart Disease, Vol.12, No.5, pp. 578-582, 2017, DOI:10.1111/chd.12486
Abstract 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. A cohort of pediatric and combined pediatric/emergency medicine residents were then randomized to intervention and control groups.… More >

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889

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William N. Evans^1,2, Ruben J. Acherman^1,2, Michael L. Ciccolo^1,3, Sergio A. Carrillo^1,3, Gary A. Mayman^1,2, Carlos F. Luna^1,2, Robert C. Rollins^1,2, William J. Castillo^1,2, Alvaro Galindo^1,2, Abraham Rothman^1,2, John A. Alexander^1,2, Tina W. Kwan^1,2, Humberto Restrepo^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 583-587, 2017, DOI:10.1111/chd.12487
Abstract 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. Of the 33 with a vascular ring, 26 (79%) occurred with an isolated right aortic arch, and 7 (21%) had an associated… More >

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812

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Maryanne Caruana¹, Victor Grech²
Congenital Heart Disease, Vol.12, No.5, pp. 588-595, 2017, DOI: 10.1111/chd.12488
Abstract 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 26.13 ± 9.62 (range 1.05–44.55 years). Cardiovascular mortality in repair survivors… More >

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Silvana Molossi^1,2, Hitesh Agrawal^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 596-596, 2017, DOI:10.1111/chd.12490
Abstract 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. More >

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862

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Mark S. Link
Congenital Heart Disease, Vol.12, No.5, pp. 597-599, 2017, DOI:10.1111/chd.12494
Abstract 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. More >

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850

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Julie A. Brothers^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 600-602, 2017, DOI:10.1111/chd.12497
Abstract Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining which unique characteristics place the… More >

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892

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Hitesh Agrawal^1,2, Carlos M. Mery^1,3, Rajesh Krishnamurthy¹, Silvana Molossi^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 603-606, 2017, DOI:10.1111/chd.12518
Abstract Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. As commonly seen in congenital heart disease, a wide spectrum of anatomic variability is seen and hence, it is important for clinicians to document the precise anatomy and course of the anomalous vessel. This article describes coronary artery nomenclature using computerized tomography angiography and virtual angioscopy. These details are important for decision making, useful for surgical planning, and may have prognostic implications. More >

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886

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Silvana Molossi, Hitesh Agrawal
Congenital Heart Disease, Vol.12, No.5, pp. 607-609, 2017, DOI:10.1111/chd.12505
Abstract 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. Additional imaging with echocardiography, computed tomography angiography and/or cardiac magnetic resonance… More >

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802

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Hitesh Agrawal^1,2, Carlos M. Mery^1,3, Patrick E. Day⁴, S. Kristen Sexson Tejtel^1,2, E. Dean McKenzie^1,3,5, Charles D. Fraser Jr^1,3, Athar M. Qureshi^1,2, Silvana Molossi^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 610-614, 2017, DOI:10.1111/chd.12511
Abstract Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long-term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner.
Methods: An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed.
Results: A total of 91 providers… More >

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864

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Athar M. Qureshi^1,2,3, Hitesh Agrawal^2,3
Congenital Heart Disease, Vol.12, No.5, pp. 615-618, 2017, DOI:10.1111/chd.12500
Abstract 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. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar… More >

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Ian S. Rogers^1,2, Jennifer A. Tremmel¹, Ingela Schnittger¹
Congenital Heart Disease, Vol.12, No.5, pp. 619-623, 2017, DOI:10.1111/chd.12499
Abstract A myocardial bridge is a segment of a coronary artery that travels into the myocardium instead of the normal epicardial course. Although it is general perception that myocardial bridges are normal variants, patients with myocardial bridges can present with symptoms, such as exertional chest pain, that cannot be explained by a secondary etiology. Such patients may benefit from individualized medical/ surgical therapy. This article describes the prevalence, clinical presentation, classification, evaluation, and management of children and adults with symptomatic myocardial bridges. More >

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Peter Ermis
Congenital Heart Disease, Vol.12, No.5, pp. 624-626, 2017, DOI:10.1111/chd.12495
Abstract 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. Stress echocardiography is well suited for routine screening and monitoring in these patients. Also, due to the ability of stress echocardiography to provide real-time cardiac… More >

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924

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Cory Noel^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 627-629, 2017, DOI:10.1111/chd.12501
Abstract Myocardial ischemia is an insult that is primarily thought of in an adult population. However, there are several congenital and acquired cardiac lesions that may lead to myocardial ischemia in a pediatric population. One of the prominent congenital lesions is anomalous aortic origin of a coronary artery (AAOCA). Anomalous aortic origin of a coronary artery is one of the leading causes sudden cardiac death in pediatric and young adult patients, and thus the assessment of myocardial perfusion is of the utmost importance. Over the past decade, pharmacologic stress MRI has proven to be a highly sensitive and accurate diagnostic examination… More >

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835

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Carlos M. Mery^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 630-632, 2017, DOI:10.1111/chd.12493
Abstract 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. The main objective of this article is to use what is known and unknown about this disease and to provide a… More >

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Jane W. Newburger
Congenital Heart Disease, Vol.12, No.5, pp. 633-635, 2017, DOI:10.1111/chd.12498
Abstract 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. Echocardiography should be performed at the time of diagnosis, then 1–2 weeks and 4–6 weeks later, with more frequent studies in individuals with coronary… More >

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Carolyn A. Altman^1,2
Congenital Heart Disease, Vol.12, No.5, pp. 636-640, 2017, DOI:10.1111/chd.12496
Abstract Echocardiography is an excellent noninvasive imaging modality for evaluation and follow-up of cardiac lesions, especially coronary artery changes occurring as a result of Kawasaki disease. The information obtained has prognostic implications and can be complemented with other modes of imaging for risk stratification and optimization of both medical and interventional therapy. The aim of this article is to describe the time line of echocardiographic follow-up of patients affected with Kawasaki disease. The classification of coronary artery changes and transthoracic echocardiographic views recommended for detailed evaluation of the coronary arteries are delineated in detail in this report. More >

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Jane W. Newburger
Congenital Heart Disease, Vol.12, No.5, pp. 641-643, 2017, DOI:10.1111/chd.12502
Abstract 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. Adjunctive therapy with a longer course of corticosteroids, together with IVIG and aspirin, may be considered for primary treatment in patients at high risk for… More >

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850

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Constantine Mavroudis
Congenital Heart Disease, Vol.12, No.5, pp. 644-646, 2017, DOI:10.1111/chd.12491
Abstract 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. More >

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EDITORIAL

Brett Snodgrass, Aruna Chilakala
Congenital Heart Disease, Vol.12, No.5, pp. 647-648, 2017, DOI:10.1111/chd.12522
Abstract This article has no abstract. More >

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Anoymous
Congenital Heart Disease, Vol.12, No.5, pp. 649-691, 2017, DOI:10.1111/chd.12538
Abstract This article has no abstract. More >

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EDITORIAL

Christopher Snyder
Congenital Heart Disease, Vol.12, No.5, pp. 692-692, 2017, DOI:10.1111/chd.12542
Abstract This article has no abstract. More >

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