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 >
Open Access
ARTICLE
Elise D. Pieterman1,2, Ricardo P. J. Budde2, Danielle Robbers-Visser1,2, Ron T. van Domburg3, Willem A. Helbing1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
William N. Evans1,2, Ruben J. Acherman1,2, Michael L. Ciccolo1,3, Sergio A. Carrillo1,3, Gary A. Mayman1,2, Carlos F. Luna1,2, Robert C. Rollins1,2, William J. Castillo1,2, Alvaro Galindo1,2, Abraham Rothman1,2, John A. Alexander1,2, Tina W. Kwan1,2, Humberto Restrepo1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Silvana Molossi1,2, Hitesh Agrawal1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Julie A. Brothers1,2
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 >
Open Access
ARTICLE
Hitesh Agrawal1,2, Carlos M. Mery1,3, Rajesh Krishnamurthy1, Silvana Molossi1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Hitesh Agrawal1,2, Carlos M. Mery1,3, Patrick E. Day4, S. Kristen Sexson Tejtel1,2, E. Dean McKenzie1,3,5, Charles D. Fraser Jr1,3, Athar M. Qureshi1,2, Silvana Molossi1,2
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 >
Open Access
ARTICLE
Athar M. Qureshi1,2,3, Hitesh Agrawal2,3
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 >
Open Access
ARTICLE
Ian S. Rogers1,2, 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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Cory Noel1,2
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 >
Open Access
ARTICLE
Carlos M. Mery1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
Carolyn A. Altman1,2
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 >
Open Access
ARTICLE
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 >
Open Access
ARTICLE
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 >
Open Access
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 >
Open Access
ARTICLE
Anoymous
Congenital Heart Disease, Vol.12, No.5, pp. 649-691, 2017, DOI:10.1111/chd.12538
Abstract This article has no abstract. More >
Open Access
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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