Objective Physical Activity Assessment in Clinical Congenital Heart Disease Research: A Systematic Review on Study Quality, Methodology, and Outcomes.

BACKGROUND: The shift toward a preventative approach in medical aftercare of congenital heart disease (CHD) patients has led to encouragement of regular physical activity (PA) in this patient population. Objective measures are crucial in accurately displaying PA levels and have increasingly found their way into clinical research. This review aims to give an overview about quality, methodology, and outcomes of current scientific work on accelerometers objectively assessing PA in patients with CHD. METHODS: Systematically researched literature in all relevant databases (PubMed, Cochrane, and Scopus) over the past decade (2009-2019) with history of CHD and accelerometer-based PA assessment was evaluated by 2 independent reviewers according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood Institute. RESULTS: Eight articles with 664 pediatric patients with CHD aged 3-18 years (range 10-162 patients), 5 studies with 574 adults with CHD aged 18-63 years (range 28-330 patients), and 3 studies with 177 pediatric patients and adults with CHD aged 8-52 years were included. Two studies were rated "good"; 9, "fair"; and 5, "poor." Methodologies and devices differed substantially across all studies. CONCLUSIONS: Overall study quality was fair at best, and due to difficult methodological comparability of the studies, no clear answer on how active patients with CHD really are can currently be given. Larger studies carefully considering collection and processing criteria, and correct reporting standards exploring PA in patients with CHD from different angles are needed.

A rare HCN4 variant with combined sinus bradycardia, left atrial dilatation, and hypertrabeculation/left ventricular noncompaction phenotype.

INTRODUCTION AND OBJECTIVES: HCN4 variants have been reported to cause combined sick sinus syndrome (SSS) and left ventricular noncompaction (LVNC) cardiomyopathy. This relationship has been proven in few cases and no previous patients have associated left atrial dilatation (LAD). Our objective was to study a familial disorder characterized by SSS, LAD, and hypertrabeculation/LVNC and to identify the underlying genetic and electrophysiological characteristics. METHODS: A family with SSS and LVNC underwent a clinical, genetic, and electrophysiological assessment. They were studied via electrocardiography, Holter recording, echocardiography, and exercise stress tests; cardiac magnetic resonance imaging was additionally performed in affected individuals. Genetic testing was undertaken with targeted next-generation sequencing, as well as a functional study of the candidate variant in Chinese hamster ovary cells. RESULTS: Twelve members of the family had sinus bradycardia, associated with complete criteria of LVNC in 4 members and hypertrabeculation in 6 others, as well as LAD in 9 members. A HCN4 c.1123C>T;(p.R375C) variant was present in heterozygosis in all affected patients and absent in unaffected individuals. Electrophysiological analyses showed that the amplitude and densities of the HCN4 currents (IHCN4) generated by mutant p.R375C HCN4 channels were significantly lower than those generated by wild-type channels. CONCLUSIONS: The combined phenotype of SSS, LAD, and LVNC is associated with the heritable HCN4 c.1123C>T;(p.R375C) variant. HCN4 variants should be included in the genetic diagnosis of LVNC cardiomyopathy and of patients with familial forms of SSS, as well as of individuals with sinus bradycardia and LAD.

Current state of home-based exercise interventions in patients with congenital heart disease: a systematic review.

Home-based exercise training is a promising alternative to conventional supervised training for patients with congenital heart disease (CHD). Even though the beneficial effect of exercise interventions is well established in patients with CHD, knowledge concerning variety and utility of existing programmes is still lacking. Therefore, the aim of this review is to give an overview about existing home-based exercise interventions in patients with CHD. A systematic search was performed in PubMed, Cochrane, Scopus and PEDro (2008-2018) for relevant clinical trials that provided any kind of home-based exercise with patients with CHD. All articles were identified and assessed by two independent reviewers. Seven articles with 346 paediatric CHD (18 months to 16 years) and five articles with 200 adults with CHD (21-41 years) were included. Most studies performed a supervised home-based exercise intervention with children and adolescents exercising at least three times per week with duration of 45 min for 12 weeks. Reported outcome measurements were health-related quality of life and physical activity, but mostly exercise capacity measured as peak oxygen uptake that improved in four studies (1.2%, 7%, 7.7%, 15%; p<0.05), walking distance in two (3.5%, 19.5%, p<0.05,) or walking time (2 min, p=0.003) in one. The dropout rates were high (15%), and compliance to the training programme was not reported in the majority of the studies (58%). Home-based exercise interventions are safe, feasible and a useful alternative to supervised cardiac rehabilitation for all age groups of patients with CHD. Nevertheless, training compliance represents a major challenge.

Valoración preconcepcional de mujeres en edad fértil con cardiopatías congénitas / Preconception assessment of women of childbearing age with congenital heart disease

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Role of rhinovirus C respiratory infections in sick and healthy children in Spain.

BACKGROUND: Recently a new genogroup of human rhinovirus (HRV) has been described and named HRV-C. The relative importance of HRV-C in viral respiratory tract illnesses is unknown. OBJECTIVE: We looked for HRV-C in pediatric patients with respiratory tract infections to determine the incidence of HRV-C and its role in sick and healthy children. We describe the clinical differences associated with HRV-C infections and other HRV genogroups. PATIENTS AND METHODS: From January 2004 to December 2008, a prospective study was conducted in children younger than 14 years who were admitted with respiratory infection to the Pediatrics Department of the Severo Ochoa Hospital in Madrid, Spain. Specimens of nasopharyngeal aspirate were taken for virologic study with polymerase chain reaction, and clinical data were recorded. HRV specimens were genotyped. We studied the frequency of HRV-C infections, the clinical course of these patients and the differences with other HRV genogroups (HRV-A and HRV-B). Presence of HRV-C was also studied in a group of healthy children. RESULTS: HRV was detected in 424 of 1555 episodes of illness (27.2%) and in 26 of 211 healthy children (12.3%) (P < 0.001). We amplified at random 248 of them (227 hospitalized children and 21 healthy children): 132 (53.2%) had HRV-A, 28 (11.2%) had HRV-B, and 88 (35.4%) HRV-C. HRV-C infections were associated with asthma, recurrent wheezing, and bronchiolitis but were not significantly different from the HRV-A genogroup. Nevertheless, significant clinical differences were observed between the HRV-B genogroup and the other groups: more frequent infiltrate on chest radiograph (P = 0.017), fever (P = 0.052), diagnosis of pneumonia (P = 0.01), and antibiotic treatment (P = 0.004). CONCLUSIONS: HRV-C infections were frequent in hospitalized children with respiratory diseases and were associated with asthma, recurrent wheezing, and bronchiolitis. No clinical differences were found with the HRV-A group: HRV-B group had clinical differences with both the other groups.