Kailyn Anderson¹, James Cnota^2,3, Jeanne James^4,5, Erin M. Miller^2,3, Ashley Parrott³, Valentina Pilipenko^1,2, Kathryn Nicole Weaver^1,2, Amy Shikany³

Congenital Heart Disease, Vol.14, No.2, pp. 264-273, 2019, DOI:10.1111/chd.12721

Abstract Objective: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD.
Design: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected. Chi‐square test, Fisher’s exact test, t test, Wilcoxon rank‐sum test, and ANOVA were used for comparisons among the groups. Logistic regression was used to test for the association between… More >

Joseph T. Poterucha¹, Saraschandra Vallabhajosyula², Alexander C. Egbe², Joseph S. Krien³, Devon O. Aganga⁴, Kimberly Holst⁵, Adele W. Golden⁶, Joseph A. Dearani⁵, Sheri S. Crow⁴

Congenital Heart Disease, Vol.14, No.2, pp. 193-200, 2019, DOI:10.1111/chd.12717

Abstract Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients… More >

Jennifer L. Lapum¹, Suzanne Fredericks¹, Barbara Bailey², Terrence M. Yau^3,4, Jennifer Graham⁵, Ariane J. Marelli^6,7

Congenital Heart Disease, Vol.14, No.2, pp. 185-192, 2019, DOI:10.1111/chd.12716

Abstract Objective: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?
Design/Methods: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order to articulate a… More >

Nayan T. Srivastava^1,2, Roger Hurwitz³, W. Aaron Kay⁴, George J. Eckert⁵, Alisha Kuhlenhoelter⁶, Nicole DeGrave², Eric S. Ebenroth^2,6

Congenital Heart Disease, Vol.14, No.2, pp. 176-184, 2019, DOI:10.1111/chd.12698

Abstract Objective: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d‐transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional ca‐ pacity and quality of life.
Participants: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty‐five of the remaining patients agreed to participate in this current study.
Design: Patients underwent comparable testing to the previous studies when possi‐ ble including exercise stress testing, echocardiography, MRI or… More >

Matthew Lewis¹, William Whang², Angelo Biviano², Kathleen Hickey², Hasan Garan², Marlon Rosenbaum¹

Congenital Heart Disease, Vol.14, No.2, pp. 207-212, 2019, DOI:10.1111/chd.12695

Abstract Background: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined.
Objective: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias.
Methods: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review.
Results: Among 124 patients (mean age: 45 years)… More >

Mariana M. Clavé¹, Nair Y. Maeda², Ana M. Thomaz¹, Sergio P. Bydlowski³, Antonio A. Lopes¹

Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688

Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient’s physical capacity.
Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐ function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.; tada‐ lafil [n… More >

Samantha C. Butler^1,4, Anjali Sadhwani^1,4, Christian Stopp², Jayne Singer^1,3,4, David Wypij^2,5,6, Carolyn Dunbar‐Masterson², Janice Ware^1,3,4, Jane W. Newburger^2,5

Congenital Heart Disease, Vol.14, No.2, pp. 236-245, 2019, DOI:10.1111/chd.12686

Abstract Objective: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery.
Outcome measures: We evaluated the neurodevelopment of a convenience sample of high‐risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation. Factor analysis examined relationships among assessment items and consolidated them into domains of development.
Results: We assessed 237 infants at a median… More >

Shankar Baskar, Andrew N. Redington, Philip R. Khoury, Timothy K. Knilans, David S. Spar, Richard J. Czosek

Congenital Heart Disease, Vol.14, No.2, pp. 201-206, 2019, DOI:10.1111/chd.12684

Abstract Background: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force‐frequency relationship (FFR) using tissue Doppler‐de‐ rived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its vari‐ ability with different ventricular pacing strategies.
Methods: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color‐coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate… More >

Masaki Sato¹, Kei Inai^1,2, Mikiko Shimizu¹, Hisashi Sugiyama¹, Toshio Nakanishi²

Congenital Heart Disease, Vol.14, No.2, pp. 167-175, 2019, DOI:10.1111/chd.12683

Abstract Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and… More >

Varun Aggarwal¹, Christopher J. Petit², Andrew C. Glatz^3，4, Bryan H. Goldstein⁵, Athar M. Qureshi¹

Congenital Heart Disease, Vol.14, No.1, pp. 110-115, 2019, DOI:10.1111/chd.12709

Abstract The use of prostaglandin‐E1 immediately after birth and subsequent surgical crea‐ tion of the modified Blalock‐Taussig shunt (BTS) shunt have remarkably improved the prognosis and survival of children with congenital heart disease and ductal‐depend‐ ent pulmonary blood flow (PBF). Despite the advancement in surgical techniques, bypass strategies, and postoperative management, significant morbidity and mortal‐ ity after BTS still remain. Patent ductus arteriosus stenting has been shown to be as an acceptable alternative to BTS placement in select infants with ductal‐dependent PBF. Newer procedural techniques and equipment, along with operator experience have all contributed to procedural refinement associated with improved outcomes… More >