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 >

Fred H. Rodriguez III^1,2,3,4, Georges Ephrem^1,2, Jennifer F. Gerardin^1,2, Cheryl Raskind-Hood⁵, Carol Hogue⁵, Wendy Book^1,2

Congenital Heart Disease, Vol.13, No.1, pp. 59-64, 2018, DOI:10.1111/chd.12563

Abstract Objective: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for “rule-out” congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age.
Design: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination… More >

Yumi Shiina¹, Tomoaki Murakami², Noriko Matsumoto³, Daisuke Okamura⁴, Yuta Takahashi⁴, Yosuke Nishihata¹, Nobuyuki Komiyama¹, Koichiro Niwa¹

Congenital Heart Disease, Vol.13, No.1, pp. 79-84, 2018, DOI:10.1111/chd.12555

Abstract Objectives: To assess body composition and relationships among body composition, appetiterelated hormones, adipocytokines, and heart failure (HF) in adult patients with congenital heart disease (CHD).
Patients: This prospective study enrolled 46 consecutive adult patients with CHD and 12 agematched healthy controls. The patients and control subjects were divided into four groups: 13 patients with Fontan circulation (group A), 16 patients with cyanosis (group B), 17 patients who previously underwent biventricular repair (group C), and 12 age-matched healthy controls.
Design: Body composition was measured using InBody730, and levels of appetite-related hormones (ghrelin and leptin) and adipocytokines (leptin, interleukin-6, and… More >

Daniel E. Ehrmann¹, Matthew Mulvahill², Shaunda Harendt^1,3, Jessica Church¹, Amy Stimmler¹, Piyagarnt Vichayavilas^1,4, Sanja Batz^1,5, Jennifer Rodgers^1,5, Michael DiMaria¹, James Jaggers¹, Cindy Barrett¹, Jon Kaufman¹

Congenital Heart Disease, Vol.13, No.1, pp. 31-37, 2018, DOI:10.1111/chd.12550

Abstract Background: Feeding practices after neonatal and congenital heart surgery are complicated and variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic assessment of feeding skills after cardiac surgery may earlier identify those likely to have protracted feeding difficulties, which may promote standardization of care.
Methods: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center. A systematic feeding readiness assessment (FRA) was utilized to score infant feeding skills. FRA scores were assigned immediately prior to surgery and 1, 2, and… More >

Sumeet S. Vaikunth^1,2, Roberta G. Williams³, Merujan Y. Uzunyan⁴, Han Tun⁵, Cheryl Barton³, Philip M. Chang⁵

Congenital Heart Disease, Vol.13, No.1, pp. 85-91, 2018, DOI:10.1111/chd.12549

Abstract Objective: Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease.
Design, Setting, and Patients: We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January… More >