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 >

Camilla Sandberg^1,2, Magnus Hedström¹, Karin Wadell², Mikael Dellborg³, Anders Ahnfelt³, Anna-Klara Zetterström⁴, Amanda Öhrn⁴, Bengt Johansson¹

Congenital Heart Disease, Vol.13, No.2, pp. 254-262, 2018, DOI:10.1111/chd.12562

Abstract Objective: The beneficial effects of exercise training in acquired heart failure and coronary artery disease are well known and have been implemented in current treatment guidelines. Knowledge on appropriate exercise training regimes for adults with congenital heart disease is limited, thus further studies are needed. The aim of this study was to examine the effect of home-based interval exercise training on maximal endurance capacity and peak exercise capacity.
Design: Randomized controlled trial.
Methods: Twenty-six adults with complex congenital heart disease were recruited from specialized units for adult congenital heart disease. Patients were randomized to either an intervention group—12… More >

Adam McKillop¹, Brian W. McCrindle^1,2, Gina Dimitropoulos³, Adrienne H. Kovacs³

Congenital Heart Disease, Vol.13, No.2, pp. 232-240, 2018, DOI:10.1111/chd.12553

Abstract Objective: A physically active lifestyle can help maintain positive physical and psychosocial health outcomes among adults with congenital heart disease (CHD). This study explored the physical activity perceptions and behaviors among young adults with CHD.
Design: This was a cross-sectional, mixed-methods study that included objectively measured physical activity assessment (accelerometer), individual semistructured interviews, and psychosocial questionnaires.
Results: Fifteen participants (67% male; 21 ± 3 years old) with moderate (n = 10) or complex (n = 5) CHD were recruited from an outpatient adult CHD clinic. Participants accumulated 26 ± 16 minutes of moderate-to-vigorous physical activity per day, and… More >

Amir-Reza Hosseinpour¹, Marie-Hélène Perez², David Longchamp², Jacques Cotting², Nicole Sekarski³, Michel Hurni¹, René Prêtre¹, Stefano Di Bernardo³

Congenital Heart Disease, Vol.13, No.2, pp. 210-216, 2018, DOI:10.1111/chd.12545

Abstract Objective: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
Design: A retrospective observational study. We defined “late” as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such… More >