Objective-To determine the effectiveness of clinical examination, chest radiography, and electrocardiography compared with echocardiography in detecting congenital heart disease early in the life of children with Down's syndrome.Design-Prospective two year screening survey.

Examined the behavioral adjustment at school age of 26 children with surgically treated complex congenital heart disease compared to that of 26 children who had been diagnosed as having an innocent murmur. The children with complex heart disease were rated by their parents as more withdrawn, having more social problems, and engaging in fewer activities, and by their teachers as more withdrawn. The families of the children with complex heart disease reported experiencing more stress. Two variables, family strain and exercise tolerance, were strong predictors of teacher-rated school adjustment in the children with complex heart disease, with family strain accounting for 33% of the variance, and exercise tolerance 24%. The impact of the child's chronic condition on the family thus seems to be a critical factor in the school adjustment of these children, more so even than the physical limitations imposed on the child by the chronic condition.

No abstract

We describe the phenotypic characteristics of 25 individuals with oculo-auriculo-vertebral spectrum (OAVS) and its variants, seen in Northern Ireland between 1969-1989, with special reference to cardiovascular defects. We report the type and prevalence of cardiovascular findings and also estimate the minimum prevalence rate of OAVS to be 1 in 45,000.

Objective-To determine whether accurate remote echocardiographic diagnosis of congenital heart disease could be achieved using a low cost telemedicine system. Design-Echocardiographic images obtained by a paediatrician from neonates suspected of having congenital heart disease were transmitted by a telemedicine link across two integrated service digital network (ISDN) lines to a regional paediatric cardiology unit for interpretation by a consultant paediatric cardiologist. The "tele-echo" diagnosis was verified by the paediatric cardiologist on direct consultation and echocardiography. Setting-Neonatal unit of Altnagelvin Hospital, Londonderry (a district general hospital) and the regional paediatric cardiology department, Royal Belfast Hospital for Sick Children. Main outcome measures-Accuracy of the diagnosis made using the telemedicine link; impact on patient management. Results-Between September 1995 and September 1997 echocardiographic images were transmitted on 63 patients. A diagnosis was made in 61 (97%) (transmitted images were unsatisfactory in two). Congenital heart disease was diagnosed in 42 patients. Fourteen patients with major congenital heart disease were accurately diagnosed within 24 hours of admission using the telemedicine link and were transferred to the regional paediatric cardiology unit. A further 28 with less serious congenital heart disease continued to be managed at the district general hospital. Congenital heart disease was excluded in 19. Follow up consultation confirmed accurate diagnosis or exclusion of congenital heart disease in 57 (93%). There were four inaccurate diagnoses (6.3%; three undetected small ventricular septal defects and one pulmonary stenosis). Conclusions-Transmitted images were of suYcient quality to allow confirmation or exclusion of major congenital heart disease. The telemedicine link facilitated early diagnosis and initiation of appropriate management in patients with complex congenital heart disease and avoided the need for transfer in those where significant congenital heart disease was excluded. (Heart 1999;82:217-221)

No abstract

Standards that reflect the complexity of medical practice may best be developed through an "expert systems" analysis of clinical conditions for which desired health care outcomes reflect the contribution of several health professionals within a complex, three-dimensional, contextual model. Examples of the model are provided, but further work is needed to test validity and measurability.

BackgroundThe National Care of the Dying Audit—Hospitals (NCDAH) is used as a method to evaluate care for dying patients in England. An additional component to the 2013/2014 audit was the Local Survey of Bereaved Relatives Views using the ‘Care Of the Dying Evaluation’ (CODE) questionnaire.AimWithin the context of the NCDAH audit, to evaluate quality of care provided to dying patients and their families in acute hospitals from the perspective of bereaved relatives.DesignPostbereavement survey to bereaved relatives.Setting/participantsFor acute hospitals wishing to participate, consecutive ‘expected’ adult deaths occurring between 1 May and 30 June 2013 were identified and the CODE questionnaire was sent to the next-of-kin.ResultsFrom 3414 eligible next-of-kin, 95 (2.8%) were excluded due to being involved in a complaint procedure and 1006 (29.5%) due to insufficient next-of-kin details. From the remaining 2313 potential participants, 858 returned a completed CODE questionnaire (37.1% response rate). Generally, symptoms were perceived to be well controlled with 769 (91%) participants reporting that either no pain was present or only there ‘some of the time’. Unmet information needs, however, was a recognised area for improvement, for example, 230 (29%) reporting having a discussion about hydration would have been beneficial.ConclusionsAdopting a postbereavement survey to NCDAH appears to be feasible, acceptable and a valuable addition. On the whole, the majority of participants reported good or excellent care. A small but significant minority, however, perceived poor quality of patient care with clear and timely communication urgently needed.