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 >

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 >

Constanze Pfitzer^1,2,3, Paul C. Helm⁴, Hannah Ferentzi^1,5, Lisa-Maria Rosenthal¹, Ulrike M. M. Bauer^4,6, Felix Berger^1,3,7, Katharina R. L. Schmitt^1,3

Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515

Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
Design: Cross-sectional registry study.
Setting: We analyzed data from patients with CHD born between 1996 and 2015.
Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic… More >

Brian A. Boe¹, Mark D. Norris², Jeffrey D. Zampi², Albert P. Rocchini², Gregory J. Ensing²

Congenital Heart Disease, Vol.12, No.6, pp. 733-739, 2017, DOI:10.1111/chd.12514

Abstract Objective: We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis.
Design: Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo)… More >

Dmitry Tumin^1,2, Helen Chou¹, Don Hayes Jr^1,3,4, Joseph D. Tobias^1,2,5, Mark Galantowicz^6,7, Patrick I. McConnell^6,7

Congenital Heart Disease, Vol.12, No.6, pp. 794-799, 2017, DOI:10.1111/chd.12513

Abstract Objective: Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease.
Design: Retrospective review of a prospective registry.
Setting: United Network for Organ Sharing registry of transplant recipients in the United States.
Patients: Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015.
Interventions: None.
Outcome measures: Employment status reported… More >

Rahul H. Rathod^1,2, Brittney Jurgen^1,2, Rose A. Hamershock³, Kevin G. Friedman^1,2, Audrey C. Marshall^1,2, Mihail Samnaliev⁴, Dionne A. Graham³, Kathy Jenkins^1,2, James E. Lock^1,2, Andrew J. Powell^1,2

Congenital Heart Disease, Vol.12, No.6, pp. 768-776, 2017, DOI:10.1111/chd.12508

Abstract Background: Standardized Clinical Assessment and Management Plans (SCAMPs) are a quality improvement initiative designed to reduce unnecessary utilization, decrease practice variation, and improve patient outcomes. We created a novel methodology, the SCAMP managed episode of care (SMEOC), which encompasses multiple encounters to assess the impact of the arterial switch operation (ASO) SCAMP on total costs.
Methods: All ASO SCAMP patients (dates March 2009 to July 2015) were compared to a control group of ASO patients (January 2001 to February 2009). Patients were divided into “younger” (<2 years) and “older” (2–18 years) subgroups. Utilization included all cardiology visits,… More >

Hitesh Agrawal^1,2, Oriana K. Wright³, Kathleen E. Carberry^1,4, S. Kristen Sexson Tejtel^1,2, Carlos M. Mery^1,5, Silvana Molossi^1,2

Congenital Heart Disease, Vol.12, No.6, pp. 721-725, 2017, DOI:10.1111/chd.12473

Abstract Background: Long-term outcome data on patients with anomalous aortic origin of coronary arteries (AAOCA) is sparse and they are often managed in a nonuniform manner. There is subjective perception of anxiety and unmet needs in these patients and families.
Methods: An online survey of 13 questions was sent to 74 families of patients with AAOCA between May and October 2015. Descriptive statistics were performed.
Results: A total of 31 (47%) families responded. Of these, 27 expressed the need to interact with other patients/families with AAOCA. The majority were interested in either face-to-face meetings (77%) or online support groups… 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… 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 >