Rick D. Vavolizza^1,2, Pe’er Dar^3,4, Barrie Suskin^3,4, Robert M. Moore^4,5, Kenan W.D. Stern^1,4

Congenital Heart Disease, Vol.13, No.3, pp. 407-412, 2018, DOI:10.1111/chd.12584

Abstract Objective: Suboptimal cardiac imaging on obstetric ultrasound is a frequent referral indication for fetal echocardiography, even in the absence of typical risk factors for fetal cardiac disease. The clinical profile of patients and findings of examinations performed for such an indication are not well defined. Given the increased cost, time and resource utilization of fetal echocardiography, we sought to determine the clinical findings of such referrals.
Study Design: We performed a single-center review of such referrals from January 2010 to June 2016. Patients with commonly accepted indications for fetal echocardiography were excluded. Demographic variables and echocardiogram findings… More >

Christina E. Holbein¹, Nicholas D. Fogleman^1,2, Kevin Hommel¹, Silke Apers³, Jessica Rassart³, Philip Moons^3,4, Koen Luyckx³, Maayke A. Sluman⁵, Junko Enomoto⁶, Bengt Johansson⁷, Hsiao-Ling Yang⁸, Mikael Dellborg^4,9, Raghavan Subramanyan¹⁰, Jamie L. Jackson¹¹, Werner Budts^3,12, Adrienne H. Kovacs¹³, Stacey Morrison¹, Martha Tomlin¹, Kathy Gosney¹, Alexandra Soufi¹⁴, Katrine Eriksen¹⁵, Corina Thomet^3,16, Malin Berghammer^4,17, Luis Alday¹⁸, Edward Callus¹⁹, Susan M Fernandes²⁰, Maryanne Caruana²¹, Samuel Menahem²², Stephen C. Cook²³, Gwen R. Rempel²⁴, Kamila White²⁵, Paul Khairy²⁶, Shelby Kutty²⁷, Gruschen Veldtman¹

Congenital Heart Disease, Vol.13, No.3, pp. 392-400, 2018, DOI:10.1111/chd.12583

Abstract Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL.
Design: Cross-sectional observational study.
Setting: Twenty-four cardiology centers from 15 countries across five continents.
Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years.
Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions… More >

Chien-Jung Lin¹, Eric Novak¹, Michael W. Rich¹, Joseph J. Billadello^1,2

Congenital Heart Disease, Vol.13, No.3, pp. 384-391, 2018, DOI:10.1111/chd.12582

Abstract Background: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort.
Methods: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other. Diagnosis and lesion severity of ACHD were extracted from ICD-10 diagnostic codes… More >

Paul C. Helm¹, Sebastian Kempert², Marc-André Körten¹, Wiebke Lesch³, Katharina Specht³, Ulrike M. M. Bauer^1,3

Congenital Heart Disease, Vol.13, No.3, pp. 377-383, 2018, DOI:10.1111/chd.12581

Abstract Background: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients’ and parents’ perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions.
Materials and Methods: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD). The total sample (N = 818) was divided into four groups: “Children” (176 patients), “Adolescents” (142 patients), “Adults” (269 patients), and “Parents” (231 parents).… More >

Lindsay A. Edwards^1,2, Christine Bui^2,3, Antonio G. Cabrera^1,2, Jill Ann Jarrell^2,4,5

Congenital Heart Disease, Vol.13, No.3, pp. 362-368, 2018, DOI:10.1111/chd.12579

Abstract Objective: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology.
Design: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions.
Patients and Setting: Patients ≥18 years of age seen in the HF and Transplant Clinic for followup visit were included in analysis.
Interventions: Interventions focused on five main areas: identifying and training providers to have ACP discussions, standardizing the ACP discussion, standardizing ACP and advance directive (AD) documentation… More >

Andrew D. Spearman¹, Rohit S. Loomba², Michael Danduran¹, Joshua Kovach¹

Congenital Heart Disease, Vol.13, No.3, pp. 369-376, 2018, DOI:10.1111/chd.12577

Abstract Objective: Multiple studies demonstrate the association of intrauterine growth restriction (IUGR) with impaired aerobic fitness in adolescents and adults. To our knowledge, there are no studies including individuals with the history of both IUGR and congenital heart disease (CHD). Thus, we sought to evaluate the impact of IUGR on exercise capacity in adolescents with CHD.
Study Design: We conducted a retrospective chart review of patients <18 years of age who underwent cardiopulmonary exercise testing (CPET) between August 1, 2003 and July 1, 2016. Individuals with birth weight <10th percentile for gestational age were defined as IUGR. Patients… More >

Djoeke Wolff¹, Henricus B. M. van de Wiel², Mirthe E. de Muinck Keizer¹, Joost P. van Melle³, Petronella G. Pieper³, Rolf M. F. Berger¹, Tjark Ebels⁴, Willebrord C. M. Weijmar Schultz⁵

Congenital Heart Disease, Vol.13, No.2, pp. 319-326, 2018, DOI:10.1111/chd.12576

Abstract Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.
Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.
Results: Fontan patients scored significantly lower on general health than their healthy peers (t (19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable… More >

Jeffery B. Anderson¹, Devyani Chowdhury², Jean A. Connor³, Curt J. Daniels⁴, Craig E. Fleishman⁵, Michael Gaies⁶, Jeffrey Jacobs^7,8, John Kugler⁹, Nicolas Madsen¹, Robert H. Beekman⁶, Stacey Lihn¹⁰, Kay Stewart-Huey¹¹, Robert Vincent¹¹, Robert Campbell¹¹

Congenital Heart Disease, Vol.13, No.2, pp. 167-180, 2018, DOI:10.1111/chd.12575

Abstract Pediatric cardiovascular services are responding to the dynamic changes in the medical environment, including the business of medicine. The opportunity to advance our pediatric cardiology field through collaboration is now realized, permitting us to define meaningful quality metrics and establish national benchmarks through multicenter efforts. In March 2016, the American College of Cardiology hosted the first Adult Congenital/Pediatric Cardiology Section Congenital Heart Community Day. This was an open participation meeting for clinicians, administrators, patients/parents to propose metrics that optimize patient care and outcomes for a state-of-the-art congenital heart center of the 21st century. Care center… More >

Laura S. Kverneland^1,2, Peter Kramer², Stanislav Ovroutski²

Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570

Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published… More >

Peter Bak¹, Cristel S. Hjortshøj², Peter Gæde³, Lars Idorn⁴, Lars Søndergaard², Annette S. Jensen²

Congenital Heart Disease, Vol.13, No.2, pp. 263-270, 2018, DOI:10.1111/chd.12565

Abstract Objective: Cyanotic congenital heart disease is a systemic disease, with effects on multiple organ systems. A high prevalence of subclinical hypothyroidism (SCH) has been reported in a small cohort of cyanotic congenital heart disease patients. Subclinical hypothyroidism has been associated with various adverse cardiovascular effects, as well as an increased risk of progression to overt hypothyroidism. The aim of this study was to examine the prevalence of SCH in cyanotic congenital heart disease patients, consider possible etiologies, and evaluate thyroid function over time.
Methods: First, 90 clinically stable cyanotic congenital heart disease patients were examined with blood… More >