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 - 10 November 2023

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;… 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 - 15 July 2020

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… 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 - 23 June 2020

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… More >

Alessandro Sgrò¹, Thomas M. Drake², Pedro Lopez‐Ayala³, Kevin Phan⁴

Congenital Heart Disease, Vol.14, No.6, pp. 1102-1112, 2019, DOI:10.1111/chd.12855

Abstract Background: Left cardiac sympathetic denervation (LCSD) has been proposed as useful therapy for long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT), in addition to anti‐arrhythmic agents and implant‐ able cardioverter defibrillators. This study aimed to assess the current evidence for LCSD and compare the open vs the video‐assisted thoracoscopic surgery (VATS) approaches.
Methods: MEDLINE, Embase and Cochrane library databases were searched up to December 2018 for studies reporting the long‐term outcomes of LCSD in LQTS, CPVT patients. The incidence of cardiac events (CEs) before and after surgery, the change in QTc interval, and surgical complications… More >

Jim T. Vehmeijer¹, Zeliha Koyak¹, A. Suzanne Vink¹, Werner Budts^2,3, Louise Harris⁴, Candice K. Silversides⁴, Erwin N. Oechslin⁴, Aeilko H. Zwinderman⁵, Barbara J.M. Mulder^1,6, Joris R. de Groot¹

Congenital Heart Disease, Vol.14, No.6, pp. 952-957, 2019, DOI:10.1111/chd.12847

Abstract Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured… 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 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 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 More >