Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

Congenital Heart Disease, Vol.18, No.5, pp. 507-523, 2023, DOI:10.32604/chd.2023.028829

Abstract Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA; 14 (1.0%) had… More >

Tânia Branco Mano^1,*, João Ferreira Reis¹, Ana Figueiredo Agapito¹, André Monteiro¹, Mário Oliveira¹, Luísa Moura Branco¹, José Fragata², Fátima Pinto³, Rui Cruz Ferreira¹, Lídia de Sousa¹

Congenital Heart Disease, Vol.15, No.3, pp. 153-162, 2020, DOI:10.32604/CHD.2020.011500

Abstract Introduction: Due to the low prevalence and wide variation of severity of Ebstein’s Anomaly (EA), long-term follow-up data are scarce. The aim was to evaluate the long-term outcome of an adult population with EA. Methods: Retrospective analysis of EA adults followed in the past 42 years in a tertiary congenital heart disease outpatient clinic. Predictors of complications and mortality were assessed. Results: We studied 53 patients: 53% females, mean age 46 ± 19 years, 36% cyanotic, 55% diagnosed at adult age. Tricuspid regurgitation was moderate or severe in 33% and 46%, respectively, and during follow-up (mean 12 ± 10… More >

Coralie Katharina Dicks¹, Gerhard-Paul Diller¹, Kristina Wasmer², Paul C. Helm^3,4, Ulrike M. M. Bauer^3,4, Helmut Baumgartner¹, Stefan Orwat^1,#, Alicia Jeanette Fischer^1,#,*

Congenital Heart Disease, Vol.15, No.2, pp. 117-125, 2020, DOI:10.32604/CHD.2020.011520

Abstract Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to… More >

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 >

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 >

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 >

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 >