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 >

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 >

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 >

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 >

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 >

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 >

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 >

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 >

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 >

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 >