Natural and modified history of single-ventricle physiology in adult patients.

OBJECTIVE: To define the evolution of the single-ventricle (SV) heart in adult patients in terms of morbidity, mortality and quality of life. METHODS: Sixty-two patients with SV physiology and aged older than 16 years were retrospectively reviewed. Three patients (5%) were in natural history, one had received a Blalock-Taussig shunt, one a Waterstone anastomosis, one a pulmonary artery banding, three a bidirectional cavopulmonary anastomosis, eight a classic Fontan procedure and 46 a total cavopulmonary connection (TCPC). The morphology of the SV was left in 48 patients (77%), right in nine (14%) and indeterminable in five (8%). Thirty-three patients underwent magnetic resonance imaging (MRI) to assess ventricular mass (VM), ventricular systolic function, pulmonary artery branch diameter and potential thrombosis of the conduit. Cardiopulmonary exercise testing (CPTE) was carried out to evaluate exercise tolerance. The quality of life was monitored with two different specific tests, the Short Form-36 (SF-36) and the congenital heart disease-TNO/AZL adult quality of life (CHD-TAAQOL). The mean follow-up time was 8.0 ± 9.1 years. RESULTS: Two of the three patients in natural history underwent primary TCPC. Re-interventions were necessary in seven patients (11%). Three patients (5%) died during follow-up. Five patients (8%) underwent cardiac transplantation. Protein losing enteropathy appeared in six (10%), while the arrhythmic disorder was detected in 13 patients. On the MRI, the mean end-diastolic ventricular volume was 106 ± 448 ml/m(2), the mean ejection fraction (EF) was 52.3 ± 10% and VM was 56 ± 22.1 g/m(2). On CPTE, the peak of oxygen uptake (peak VO(2)) was moderately impaired in 92% of patients, while 4% presented a severely impaired and 4% a normal peak of VO(2). No correlations were found among the peak of VO(2) and the quality-of-life evaluation. CONCLUSIONS: Adult patients with SV are at high risk of reoperations and need of transplant and complications. Nevertheless, in the presence of a moderately reduced peak of VO(2) and a moderate reduction in the EF detected at the MRI, the results of the evaluation of daily quality of life are incredibly high.

Patient education in Parkinson's disease: Formative evaluation of a standardized programme in seven European countries.

OBJECTIVE: To evaluate a newly developed education programme for Parkinson's disease (PD) patients. METHODS: The programme consisted of eight sessions and aimed at improving knowledge and skills related to self-monitoring, health promotion, stress management, depression, anxiety, social competence, and social support, all with special reference to PD. The programme was formatively evaluated in seven European countries (Spain, Finland, Italy, The Netherlands, United Kingdom, Estonia, Germany) with 151 patients diagnosed with idiopathic PD. The evaluation included patients' ratings of the comprehensibility and feasibility of the programme as well as mood ratings before and after each session. Patients also completed questionnaires at the beginning and end of the programme to explore possible changes in disease-related psychosocial problems, quality of life, and depression. RESULTS: The programme was feasible to run, and patients were able to understand its elements. Patients reported mood elevations following individual sessions and reduced disease-related psychosocial problems after completing the programme. There were no substantial differences in results between cultures. CONCLUSION: Patient education appears to have potential as a useful and feasible intervention, complementing medical treatment in PD. PRACTICE IMPLICATIONS: The present programme will soon be available in seven European languages and can be tested in different health care systems.