Jing Wu¹, Li Kuang¹, Lijuan Fu²

Congenital Heart Disease, Vol.13, No.2, pp. 194-202, 2018, DOI:10.1111/chd.12586

Abstract Objective: The aim of this study was to evaluate the effects of inspiratory muscle training (IMT) in chronic heart failure (CHF) patients.
Design: We searched MEDLINE, EMBASE, Cochrane Library, CINHAL, and CBMdisc to collect controlled trials on the application of inspiratory muscle training in CHF patients from the establishment of these databases to November 2016. Two reviewers independently screened literature according to the inclusion and exclusion criteria, extracted data, and assessed the quality of literature. Meta-analysis was conducted by software RevMan5.3.
Results: Eight studies involving 302 patients were identified. Meta-analysis indicated that IMT significantly improved PI_max, V_E/VCO₂ slope and 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 >

Fariborz Soheili^1,2, Zahra Jalili³, Mahtab Rahbar⁴, Zahed Khatooni¹, Amir Mashayekhi⁵, Hossein Jafari⁶

Congenital Heart Disease, Vol.13, No.2, pp. 295-304, 2018, DOI:10.1111/chd.12571

Abstract Background: The mutations in GATA4 gene induce inherited atrial and ventricular septation defects, which is the most frequent forms of congenital heart defects (CHDs) constituting about half of all cases.
Method: We have performed High resolution melting (HRM) mutation scanning of GATA4 coding exons of nonsyndrome 100 patients as a case group including 39 atrial septal defects (ASD), 57 ventricular septal defects (VSD) and four patients with both above defects and 50 healthy individuals as a control group. Our samples are categorized according to their HRM graph. The genome sequencing has been done for 15 control samples and… 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 >

Xinyan Li¹, Xiaoxia Qiu¹, Huan Huang¹, Yili Zhao², Xueqin Li¹, Meng Li¹, Xiaoxian Tian¹

Congenital Heart Disease, Vol.13, No.2, pp. 282-287, 2018, DOI:10.1111/chd.12568

Abstract Objective: To evaluate the efficacy of using fetal heart size measurements derived from axial echocardiography to predict homozygous α-thalassemia-1.
Design: Prospective diagnostic study.
Setting: The carrier rate of α-thalassemia-1 (–/αα) in China’s Guangxi Zhuang Autonomous Region is approximately 15%. If both parents are carriers, the risk of homozygous a-thalassemia-1 in one pregnancy is 25%.
Patients: Singleton mid-pregnancies at risk of homozygous α-thalassemia-1 were enrolled.
Outcome Measures: Fetal heart measurements, including heart diameter (HD), heart length (HL), heart circumference (HC), and heart area (HA), were measured. The z-scores for these heart parameters were then calculated separately based on previously constructed z-score… More >

Yu Zhu^1*, Yong Chen^1*, Yu Feng², Di Yu¹, Xuming Mo¹

Congenital Heart Disease, Vol.13, No.2, pp. 271-281, 2018, DOI:10.1111/chd.12567

Abstract We conducted this meta-analysis to address the open question of a possible association between maternal body mass index (BMI) and congenital heart defects (CHDs) in infants. We conducted a comprehensive computerized search of PubMed, Web of Science, Medline, and Embase databased (January 1980 through August 2017). We assessed the association between maternal BMI and the risk for congenital heart defects in their offspring. Study-specific relative risk estimates were polled according to random-effect or fixed-effect models. From 2567 citations, a total of 13 case-control studies and 4 cohort studies were selected for a meta-analysis, including more… 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 >