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Objective: Improved survival has yielded to growing importance of functional outcome measures in patients with congenital heart disease (CHD). This study applied the International Physical Activity Questionnaire (IPAQ) to assess self-reported physical activity (PA) in patients with CHD and their association with exercise capacity and health-related quality of life (HrQoL).Patients and Methods: Prior to cardiopulmonary exercise testing (CPET), 786 consecutive patients (335 female, 31.1±11.6 years) with CHD filled in the short form of the IPAQ and the HrQoL questionnaire 36-Item Short Form.Results: In total, 393 (50.0%) patients reported health-enhancing physical activity (HEPA), 237 (30.2%) minimal activity and 156 (19.8%) inactivity. In comparison with the HEPA group, the inactive group had significant lower peak oxygen uptake (74.2%±20.7% vs 86.8%±22.3%; p<0.001) as well as lower physical (91.0%±16.9% vs 97.4%±13.6%; p<0.001) and mental (97.1%±22.2% vs 104.1%±15.6%; p<0.001) HrQoL. Independent of severity class, surgery, age, beta-blocker, pacemaker and oxygen saturation, the HEPA group in comparison with the inactive group showed significantly less probability for impairments in mental (OR: 0.21, 95% CI: 0.10 to 0.45; p<0.001) and physical (OR: 0.46, 95% CI: 0.23 to 0.92; p=0.027) HrQoL and exercise capacity (OR: 0.36, 95% CI: 0.22 to 0.59; p<0.001).Conclusions: Categorisation of patientswith CHD with the IPAQ quickly provides clinical information as HEPA patients have a less probability for impaired HrQoL and diminished exercise capacity. Nevertheless, the IPAQ cannot substitute an accelerometer-based assessment for PA, nor a CPET for exercise capacity. [ABSTRACT FROM AUTHOR]

Objective Multiple studies have demonstrated the predictive value of arterial stiffness parameters like augmentation index (AIx) for cardiovascular events, the onset of hypertension, and the progression of heart failure. There is evidence that arterial stiffness is increased in some diagnostic subgroups of patients with congenital heart defects (CHD). This study aims to investigate AIx in a large cross-sectional cohort of patients with CHD. Patients and methods We prospectively examined 1125 consecutive patients with various congenital heart defects (27.3 ± 12.1 years, 464 female) referred for routine cardiopulmonary exercise testing (CPET) in our institution, and 322 healthy volunteers (29.4 ± 18.4 years, 165 female). AIx was estimated in supine position using the oscillometric Vicorder device (SMT medical, Würzburg, Germany). Afterward patients performed a CPET. Results In multivariable regression, presence of a CHD emerged as independent risk factor for higher AIx (p < .001). AIx was also higher in older (p < .001), smaller (p < .001) and heavier (p < .001) patients and in females (p = .008). Patients with aortic stenosis (p < .001), Tetralogy of Fallot (p < .001), transposition of the great arteries after atrial switch (p < .001) or Rastelli procedure (p = .013) and after Fontan procedure (p = .002) had higher AIx. Higher peak oxygen uptake (p < .001) and an ACE-inhibitor (p = .088) were associated with a lower AIx. Conclusions AIx is increased in patients with CHD. Several diagnostic subgroups are at risk. A better understanding of pathophysiologic mechanisms, genetic predisposition, the role of surgical aortic scars or implanted conduits/patches and medication is needed to define the value of AIx for further cardiovascular risk assessment in this cohort. [ABSTRACT FROM AUTHOR]

Abstract: Objective: This study aimed to compare situational and trait anxiety levels in patients with congenital heart disease (CHD) with that in healthy controls and to investigate their correlation to the perceived health status. Patients and methods: From November 2007 to December 2009 in total 879 patients (405 female, 15–71years) with various CHD and 40 healthy controls completed the State-Trait Anxiety Inventory (STAI) to assess state and trait anxiety, the depression scaling instrument CES-D, and the health-related quality of life questionnaire SF-36. Results: In patients with CHD no increased anxiety as a trait could be found (Mann–Whitney Utest, p=.186). Only situational anxiety in the setting of an outpatient department in a tertiary center was increased compared to healthy controls (Mann–Whitney U test, p=.004). Anxiety was closely related to depression and to all of the nine SF-36 dimensions (r=−.149 to r=−.745, p<.001). Conclusions: In general, patients with CHD show a rather normal anxiety pattern compared to healthy controls. These symptoms of anxiety were strongly correlated with the perceived health status. Only situational anxiety levels at the hospital are increased and still remain an important challenge for doctors and psychologists. [Copyright &y& Elsevier]

Objective Health-related quality of life questionnaires are frequently used to involve patients' impressions and feelings in the outcome evaluation. In patients with congenital heart disease (CHD) methodological heterogeneities, assessment in different age and diagnostic groups led to controversial findings. This study aims to give a comprehensive answer to the health-related quality of life in patients with CHD. Patients and methods From July 2001 to June 2013, 2360 patients (1058 female, 28.6±11.2-years, range 14-75-years) with various kinds of CHD underwent a quality of life assessment with the SF-36 questionnaire and underwent a cardiopulmonary exercise test as part of their routine follow-up. Results Physical component summary score (PCS) develops from 96.1 %predicted in patients younger than 20-years, to 96.2 %predicted in patients aged 20 to 30-years, 92.3 %predicted in patients aged 30 to 40-years, and 92.6 %predicted in patients 40-years or older (r=-.114; p<.001). The decline was more prominent in the mental component summary score (MCS) declining from 104.1 %predicted in patients younger than 20years, to 103.4 %predicted, 99.9 %predicted, and 97.5 %predicted (r=-.132; p<.001). Exercise capacity was impaired with 80.1±23.0 %predicted and also declined slowly with age (r=-.084; p<.001). Conclusions Health-related quality of life in patients with CHD is progressively reduced in older age-groups. The decline is small, but more prominent in the mental components. This should be considered in the interpretation of studies, and strategies have to be developed to reduce this effect in future. [ABSTRACT FROM AUTHOR]

Background: The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD–APAH). Methods: Patients with invasively confirmed CHD–APAH received in-hospital exercise training for 3weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21±14months. Results: Twenty consecutive CHD–APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60±23mmHg) were included. Patients significantly improved the mean distance walked in 6min compared to baseline by 63±47m after 3weeks (p<0.001) and by 67±59m after 15weeks (p=0.001). Quality of life-score (p=0.05), peak oxygen consumption (p=0.002) and maximal workload (p=0.003) improved significantly by exercise training after 15weeks. The 1- and 2‐year survival rates were 100%, the transplantation-free survival rate was 100% after 1year and 93% after 2years. Conclusion: Exercise training as add-on to medical therapy may be effective in patients with CHD–APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results. [ABSTRACT FROM AUTHOR]

The aim of the study was to assess the quantity and nature of emergencies affecting adults with congenital cardiac disease (CCD) and evaluate infrastructural requirements for adequate management. There is an increasing number of adults with CCD requiring specialized complex care. This multicenter study evaluated all emergency admissions to 1 of 5 centers for adults with CCD within 1 year. Within 1 year, there were 1,033 admissions of adults with CCD, and 201 (160 patients; age 16 to 71 years) were emergencies. Underlying cardiac anomalies were univentricular heart (22%), complete transposition (14%), tetralogy of Fallot (21%), and others (43%). Seventy percent of patients had undergone previous cardiac surgery. The main reason for acute admission was cardiovascular (arrhythmia, heart failure, syncope, aortic dissection, and endocarditis). Diagnostic procedures most often assigned were echocardiography (n = 223), chest x-ray (n = 95), Holter electrocardiography (n = 85), cardiac catheterization/electrophysiologic study (n = 39), and others (n = 143). Forty-six patients underwent surgery (cardiovascular n = 41, general n = 5) or electrophysiologic treatment (n = 41). One hundred twenty-six of 201 emergencies (63%) required cooperation with another specialized department: surgery (n = 46), internal medicine (n = 42), neurology (n = 12), ophthalmology (n = 6), otorhinolaryngology (n = 5), gynecology (n = 5), psychiatry (n = 4), radiology (n = 3), dermatology (n = 2), and orthopedics (n = 2). In conclusion, physicians and consultants attending adult patients with CCD need a high degree of specialized experience concerning the cardiac anomaly to manage emergencies properly. Furthermore, a wide range of noncardiac diagnostic and therapeutic procedures must be available. Data support the demand for a multidisciplinary approach in specialized centers for adequate care of adults with CCD. [Copyright &y& Elsevier]