Andrew Sands

Incidence and risk factors for ventricular septal defect in “low risk” neonates

AIMS To quantify the incidence of ventricular septal defect in “low-risk” neonates; and to define any associated risk factors. METHODS One hundred and seventy three patients with ventricular septal defects from a scanned population of 3971 clinically normal neonates were compared with scanned controls, considered to be clinically normal. A subset of the group with defects was compared with normal infants delivered over the same period, to identify any seasonal variation. RESULTS Ventricular septal defects were detected in 4.36 % of the “scanned” group (173 out of 3971). Ten had perimembranous defects and the remainder apical or muscular lesions. Eleven neonates had multiple defects. The incidence of ventricular septal defect was independent of most tested risk factors. There were significantly more girls in the group with defects compared with the controls (p = 0.004). The defects group also contained fewer infants born during summer months (p = 0.04). CONCLUSIONS The incidence of ventricular septal defects was much higher than might be expected, given that “high risk infants” were excluded. The observations that gender and season of birth affect the rate suggest that both genetic and environmental factors may be involved in the aetiology.

Determinants of neuropsychological and behavioural outcomes in early childhood survivors of congenital heart disease.

Aims: To evaluate the relative effect of cyanosis, surgical interventions and family processes on neuropsychological and behavioural outcomes in 4-year-old survivors of serious congenital heart disease (CHD). Methods: 90 children with a range of cyanotic and acyanotic conditions, who underwent either corrective or palliative surgery, completed a neuropsychological and behavioural evaluation. Families of participants were also profiled by evaluation of maternal mental health, worry, social support, parenting style and family functioning. Results: Compromised neuropsychological outcomes were associated with a combination of cyanotic conditions and open-heart surgery, but this was not exacerbated by having a complex, palliative, status. Both cyanotic and acyanotic conditions were associated with specific sensorimotor delays, regardless of method of the correction. Only children with complex conditions and palliative interventions seemed at risk of poor behavioural outcomes; indeed, children with cyanosis with complete repair showed favourable behavioural outcomes compared with controls. Multivariate analyses highlighted the sometimes greater relevance of family processes (eg parenting style, maternal mental health and worry), rather than disease or surgical factors, in predicting especially behavioural outcomes. Conclusions: The findings (1) suggest a more complex relationship between cyanosis, surgical methods of correction, neuropsychological and behavioural outcomes than previously charted, (2) highlight that family processes may be aetiologically more important than disease and surgical factors, and (3) indicate specific targets for secondary prevention programmes for this at-risk population.

A controlled trial of early interventions to promote maternal adjustment and development in infants born with severe congenital heart disease

Abstract Background Congenital heart disease can have a negative impact on both infant development and maternal adjustment. This study considered the impact of a new programme of early psychosocial interventions on such outcomes, following the birth of a child with severe congenital heart disease. Methods Seventy infants and their mothers were assigned to an intervention or control group based on order of presentation to the unit. Interventions aimed at bolstering mother-infant transactions, through psychoeducation, parent skills training and narrative therapy techniques were implemented. Results Clinically and statistically significant gains were observed at 6-month follow-up on the mental (but not the psychomotor) scale of the Bayleys-II. Positive gains were also manifested on feeding practices, maternal anxiety, worry and appraisal of their situation. Conclusions A programme of generalizable psychosocial interventions is shown to have a positive impact on the infant with severe congenital heart disease and the mother.

Predictors of psychological functioning in mothers and fathers of infants born with severe congenital heart disease

This study examined mental health and coping styles in both mothers and fathers of infants born with a severe congenital heart defect. Factors associated with mental health outcomes were elucidated. Parents of 70 infants, recently born with a severe congenital heart defect, completed questionnaires which examined psychological functioning and coping strategies. Disease, surgical and psychosocial factors were examined for their significance in predicting psychological functioning. Findings indicated elevated levels of clinically significant psychological distress in mothers, compared to fathers, and differences between parents in coping styles. Regression analyses suggested that the extent of distress in both parents was not primarily predicted by illness or demographic factors. Rather, certain coping styles, knowledge, subjective worry and family functioning emerged as significant predictive variables. Implications for early intervention are discussed.

Home videoconferencing for patients with severe congential heart disease following discharge.

BACKGROUND Centralization of pediatric cardiology services into a small number of tertiary centers and the particular stress that accompanies diagnosis and surgical management of severe congenital heart disease (CHD) renders psychological support for families and clinical monitoring of patients following discharge increasingly important. Telemedicine has an increasing role in clinical and academic medicine. Improvements in quality and reliability of videoconferencing systems have allowed this to become a useful diagnostic tool. OBJECTIVE This study aimed to assess the benefits of home monitoring by videoconferencing compared with contacting by telephone only, in terms of decreasing anxiety levels and clinical monitoring in the postdischarge period. METHODS We performed a prospective, controlled study of children with a recent diagnosis of severe CHD and those recovering from palliative or corrective surgery for severe CHD. We used standardized anxiety scores to assess anxiety after discharge in families followed up by home videoconferencing or telephone calls and assessed the clinical information available through videoconferencing or telephone calls by structured questionnaires. RESULTS Videoconferencing decreased anxiety levels compared with telephone calls (P < .05). Improved clinical information was available in the videoconferencing group, resulting in more appropriate and timely hospital attendance. CONCLUSION Videoconferencing is acceptable to parents and physicians and provides a more effective form of follow-up in terms of clinical observation and parental anxiety levels.

Impact of a regional training program in fetal echocardiography for sonographers on the antenatal detection of major congenital heart disease

The aims of this study were to ascertain the effect of a regional training program in fetal echocardiography for obstetric radiographers on the antenatal detection of major congenital heart disease (CHD) and to document short‐term outcomes for major CHD.

Remote Diagnosis of Congenital Heart Disease: The Impact of Telemedicine

Objectives To determine the accuracy of remote diagnosis of congenital heart disease (CHD) by real-time transmission of echocardiographic images via integrated services digital network (ISDN) lines, to assess the impact on patient management and examine cost implications. Design Prospective comparison of echocardiograms on infants with suspected significant CHD performed as follows: (1) hands-on evaluation and echocardiogram by a paediatrician at a district general hospital (DGH) followed by (2) transmission of the echocardiogram via ISDN 6 with guidance from a paediatric cardiologist and finally (3) hands-on evaluation and echocardiogram by a paediatric cardiologist. The economic analysis compares the cost of patient care associated with the telemedicine service with a hypothetical control group. Setting Neonatal units of three DGH and a UK regional paediatric cardiology unit. Results Echocardiograms were transmitted on 124 infants. In five cases scans were inadequate for diagnosis. Of the remaining 119 tele-echocardiograms, a follow-up echocardiogram was performed on 109/119 (92%). Major CHD was diagnosed in 39/109 infants (36%) and minor CHD in 45 (41%). The tele-echo diagnosis was accurate in 96% of cases (κ=0.89). Unnecessary transfer to the regional unit was avoided in 93/124 patients (75%). Despite relatively high implementation costs, telemedicine care was substantially cheaper than standard care. Each DGH potentially saved money by utilising the telemedicine service (mean saving: £728/patient). Conclusions CHD is accurately diagnosed by realtime transmission of echocardiograms performed by paediatricians under live guidance and interpretation by a paediatric cardiologist. Remote diagnosis and exclusion of CHD affects patient management and may be cost saving.

Major congenital heart disease: antenatal detection, patient characteristics and outcomes

Objective. To determine the antenatal detection rate in Northern Ireland (NI) for major congenital heart disease (CHD) between 1st January 2002 and 31st December 2006. To compare characteristics and prognosis of antenatal versus postnatal groups. Methods. Data were obtained from fetal, neonatal and post-mortem records. Variables recorded: antenatal diagnosis, karyotype, referral indication, type of defect and neonatal outcome. Results. Twenty-seven per cent (73/272) of patients with major CHD were diagnosed antenatally. Forty-one per cent (30/73) of these died before 28 days. Eighty-two per cent (60/73) of the antenatal diagnosis group were referred with suspected cardiac abnormality on obstetric screening. There were more lethal trisomies amongst the antenatal diagnosis group (15%, 11/73) than the postnatal diagnosis group (2%, 4/191) p < 0.001. Significantly more of the antenatal group had functionally univentricular hearts than the postnatal/post-mortem group (47%vs. 7%). Eighty-nine per cent (65/73) of defects diagnosed antenatally were ‘four chamber view’ defects. 11% (8/73) were ‘outflow tract view’ defects. Conclusions. Antenatal detection in NI is around the European average. Those with an antenatal diagnosis have more complex CHD, higher risk of karyotype abnormality and poorer neonatal outcomes. Screening is responsible for the majority of antenatal diagnoses of major CHD. A regional training programme for obstetric sonographers has now been delivered.

Exercise training improves activity in adolescents with congenital heart disease

Objectives To ascertain if motivational techniques and a structured exercise programme can increase activity in adolescents afflicted with congenital heart disease (CHD). Design Prospective randomised controlled trial. Setting One hundred and forty-three patients aged 12–20 years attending the tertiary centre for paediatric cardiology in Northern Ireland. Main outcome measures Increase in exercise capacity as assessed by duration of exercise stress test, and number of minutes spent in moderate to vigorous physical activity (MVPA) per day. Results Eighty-six patients were men (60%), mean age was 15.60±2.27 years. Seventy-three percent were considered to have major CHD. Seventy-two participants were randomised to the intervention group. Following intervention, duration of exercise test increased by 1 min 5 s for the intervention group (p value 0.02) along with increase in predicted VO2Max (p value 0.02). There was a significant increase in minutes of MVPA per day for the intervention group from baseline to reassessment (p value <0.001) while MVPA remained much the same for the control group. Fourteen patients met the current recommendation for more than 60 min MVPA per day at baseline. This doubled to 29 participants at reassessment. There were no adverse effects or mortalities reported. Conclusions Exercise training is safe, feasible and beneficial in adolescents with CHD. Psychological techniques can be employed to maximise the impact of interventions. Trial Registration Number ISRCTN27986270.

Echocardiographic screening for congenital heart disease: a randomized study

Abstract Objectives: To assess the effectiveness and potential cost of an echocardiographic screening program for congenital heart disease (CHD). Patients and methods: Between 01/11/94 and 28/02/98 there were 9697 deliveries in The Royal Maternity Hospital Belfast. Mothers were randomized before delivery. 4875 infants were allocated to the scan group, while 4822 were assigned to clinical assessment alone. High-risk infants were considered separately. Cases of CHD detected before hospital discharge were documented. The annual cost of screening was estimated and the time to accurate diagnosis in each group was assessed. Results: During the study 124 scan allocated infants and 50 controls were identified as having significant CHD before hospital discharge. With a minimum of 3 years follow-up there were 27 additional late diagnoses in controls and 1 in scanned infants. During a single year of the study the mean time to complete diagnosis was 2 days for scanned cases and 110 in controls. The projected cost of screening for all infants was £ 22/infant for the first year. Conclusions: Adding echocardiography to clinical examination greatly enhances early detection of CHD. Although screening is expensive, once established it may reduce the cost of unnecessary outpatient referrals.