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 >

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 >

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 >

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 >

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 >

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 >

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 >