Rohit S. Loomba^1,2, Andrew N. Pelech^1,2

Congenital Heart Disease, Vol.13, No.1, pp. 92-97, 2018, DOI:10.1111/chd.12510

Abstract Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.
Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior… More >

James E. Lock^1,2,3

Congenital Heart Disease, Vol.12, No.6, pp. 697-698, 2017, DOI:10.1111/chd.12548

Abstract This article has no abstract. More >

Andrew D. Spearman

Congenital Heart Disease, Vol.12, No.6, pp. 828-833, 2017, DOI:10.1111/chd.12543

Abstract A genetic basis of congenital heart disease (CHD) has been known for decades. In addition to the sequence of the genome, the contribution of epigenetics to pediatric cardiology is increasingly recognized. Multiple epigenetic mechanisms, including DNA methylation, histone modification, and RNA-based regulation, are known mediators of cardiovascular disease, including both development and progression of CHD and its sequelae. Basic understanding of the concepts of epigenetics will be essential to all pediatric cardiologists in order to understand mechanisms of pathophysiology, pharmacotherapeutic concepts, and to understand the role of epigenetics in precision medicine. More >

Gruschen R. Veldtman¹, Alexander R. Opotowsky², Samuel G. Wittekind¹, Jack Rychik³, Daniel J. Penny⁴, Mark Fogel³, Bradley S. Marino⁵, Marc Gewillig⁶

Congenital Heart Disease, Vol.12, No.6, pp. 699-710, 2017, DOI:10.1111/chd.12526

Abstract Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The More >

Juan Villafane¹, Thomas C. Edwards², Karim A. Diab³, Gary M. Satou⁴, Elizabeth Saarel⁵, Wyman W. Lai⁶, Gerald A. Serwer⁷, Peter P. Karpawich⁸, Russell Cross⁹, Russell Schiff¹⁰, Devyani Chowdhury¹¹, Thomas J. Hougen¹²

Congenital Heart Disease, Vol.12, No.6, pp. 762-767, 2017, DOI:10.1111/chd.12523

Abstract Objective: The objective of this study was to develop quality metrics (QMs) relating to the ambulatory care of children after complete repair of tetralogy of Fallot (TOF).
Design: A workgroup team (WT) of pediatric cardiologists with expertise in all aspects of ambulatory cardiac management was formed at the request of the American College of Cardiology (ACC) and the Adult Congenital and Pediatric Cardiology Council (ACPC), to review published guidelines and consensus data relating to the ambulatory care of repaired TOF patients under the age of 18 years. A set of quality metrics (QMs) was proposed by the… More >

Raysa Morales-Demori

Congenital Heart Disease, Vol.12, No.6, pp. 820-827, 2017, DOI:10.1111/chd.12521

Abstract Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%–60%), atrial (16%-21%), or ventricular septal defects (14%–27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. More >

Joseph M. Krepp¹, Mary J. Roman², Richard B. Devereux², Adrienne Bruce¹, Siddharth K. Prakash³, Shaine A. Morris⁴, Dianna M. Milewicz³, Kathryn W. Holmes⁵, William Ravekes⁵, Ralph V. Shohet⁶, Reed E. Pyeritz⁷, Cheryl L. Maslen⁸, Barbara L. Kroner⁹, Kim A. Eagle¹⁰, Liliana Preiss⁹, GenTAC Investigators^*, Federico M. Asch¹

Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520

Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17… More >

Jonathan N. Johnson^1,2, Cindy S. Barrett³, Wayne H. Franklin⁴, Eric M. Graham⁵, Nancy J. Halnon⁶, Brandy A. Hattendorf⁷, Catherine D. Krawczeski⁸, James J. McGovern⁹, Matthew J. O’Connor¹⁰, Amy H. Schultz¹¹, Jeffrey M. Vinocur¹², Devyani Chowdhury¹³, Jeffrey B. Anderson¹⁴

Congenital Heart Disease, Vol.12, No.6, pp. 756-761, 2017, DOI:10.1111/chd.12519

Abstract Introduction: In 2012, the American College of Cardiology’s (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee’s metric design process.
Methods: The infection prevention metrics team consisted of 12 members from 11 institutions in North America. The group agreed to work… More >

Michael D. Seckeler¹, Katri Typpo², Jendar Deschenes², Ruth Higgins³, Ricardo Samson¹, Peter Lichtenthal⁴

Congenital Heart Disease, Vol.12, No.6, pp. 815-819, 2017, DOI:10.1111/chd.12517

Abstract Objective: To determine the accuracy of a continuous cardiac output monitor (FloTrac sensor) for measuring cardiac index in children with congenital heart disease undergoing cardiac catheterization. Cardiac index is a critical hemodynamic parameter measured during catheterizations in children with congenital heart disease. This has been challenging to measure accurately and many clinicians rely on predictive equations for calculating cardiac index.
Design: Prospective, nonrandomized trial.
Setting: Tertiary care congenital heart center.
Patients: Consecutive participants ≤18 years old undergoing clinically indicated cardiac catheterizations from September 2014 through August 2015.
Interventions: Oxygen consumption was measured using the Vmax Encore 229 monitor attached to… More >

Navaneetha Sasikumar, Antony Hermuzi, Chun-Po Steve Fan, Kyong-Jin Lee, Rajiv Chaturvedi, Edward Hickey, Osami Honjo, Glen S. Van Arsdell, Christopher A. Caldarone, Arnav Agarwal, Lee Benson

Congenital Heart Disease, Vol.12, No.6, pp. 808-814, 2017, DOI:10.1111/chd.12516

Abstract Objectives: Mortality associated with the modified Blalock–Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity.
Design: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox’s proportional hazard regression.
Results: Median age was 8 (0–126) days, weight 3.1(1.7–5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent… More >