Frank Casey

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.

Application of a low cost telemedicine link to the diagnosis of neonatal congenital heart defects by remote consultation

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 sufficient 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.

Quality of life in surgically palliated complex congenital heart disease.

The outcome of surgical palliation was evaluated in 26 children with complex cyanotic congenital heart disease. Outcome was examined in terms of ongoing symptoms, exercise tolerance, and the ability to participate in normal childhood activities. An activity score was calculated and each child performed graded treadmill exercise testing. Breathlessness (24 (92%) children), respiratory infections (nine (35%) children), and leg cramps (eight 31%) children) were the most common physical disorders. Although formal exercise testing showed a clear reduction in exercise tolerance compared with age and sex matched controls, palliation had allowed 23 (89%) to function with moderate exercise limitation, three (11%) having severely limited activity. Parents underestimated the childs exercise tolerance in 80% of cases. Sixteen (62%) patients attended school full time, eight (31%) attended part time, and two (8%) received only home tuition. Palliative surgery can give children with a single functional ventricle a level of activity which allows them to take part in most childhood activities. Subjective estimates of exercise tolerance are inaccurate in this group of children, and formal exercise testing can contribute useful information to decision making about further surgical intervention.

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.

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.

Recommendations for the use of palivizumab as prophylaxis against respiratory syncytial virus in infants with congenital cardiac disease

New data are emerging on the use of palivizumab as prophylaxis against infection with the respiratory syncytial virus in infants with congenital cardiac disease. Following a 4-year multicentre randomised trial, it was shown that prophylactic injections with palivizumab were effective and safe for such children. Prophylaxis consists of 5, monthly, intramuscular injections of palivizumab, at a dose of 15 mg/kg, given during the season for infection with the respiratory syncytial virus. Timing is at the discretion of the physician, depending on the onset of the season locally. It is suggested that, in the United Kingdom, this should be commenced in mid-September. To help clinicians to identify appropriate candidates for palivizumab, a working group of the British Paediatric Cardiac Association has developed recommendations. Infants, namely those under 1 year old, with congenital cardiac disease likely to benefit from prophylaxis include those with haemodynamically significant lesions, particularly increased pulmonary blood flow with or without cyanosis; pulmonary venous congestion, pulmonary hypertension or long-term pulmonary complications, residual haemodynamic abnormalities following medical or surgical intervention (patients who have undergone cardiopulmonary bypass should receive an injection as soon as they are medically stable), cardiomyopathy requiring treatment, and congenital cardiac disease likely to need hospital admission for medical or surgical intervention during the season of infection with the virus. Prophylaxis with palivizumab may also be indicated, at the discretion of the physician, in some children with complex cardiac disease over the age of 1 year. Children less likely to benefit from prophylaxis are those with haemodynamically insignificant disease, or those with lesions adequately corrected by medical or surgical intervention.

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.

Diagnosis of neonatal congenital heart defects by remote consultation using a low-cost telemedicine link.

To determine whether telemedicine could assist in the earlier diagnosis of neonates with congenital heart disease (CHD) in an area hospital remote from a paediatric cardiologist, we established a low-cost telemedicine link between the neonatal unit of a district general hospital and the regional paediatric cardiology unit. Realtime ultrasound images of babies suspected of having CHD were obtained by a paediatrician and transmitted for realtime interpretation by a paediatric cardiologist. In a four-month pilot study, 10 neonates were studied in this way. In eight of the ten cases, the diagnosis made over the telemedicine link was confirmed subsequently in a direct examination at the regional unit. In one case the patient died before the direct examination was possible. In one case two small muscular ventricular septal defects were missed on the remote examination. Our early experience suggests that, with realtime guidance by a paediatric cardiologist, transmitted images of sufficient quality to allow confirmation or exclusion of major cardiac defects can be obtained. This form of remote consultation should improve morbidity and mortality rates by reducing the waiting time for specialist diagnosis and treatment.

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.