Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease.

OBJECTIVES: To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease. METHODS: Health behaviour outcomes were assessed in 91 children who had surgery or catheter intervention for congenital cardiac disease. The children were classified into four groups according to severity. The main parameters of classification were the presence of residual symptoms, frequency of visits to general practitioner or the Accident and Emergency Department, and ability to participate in physical activity according to a calculated "activity score". RESULTS: Children had very few residual symptoms after "corrective surgery". Those with complex congenital cardiac disease post-Fontan-type repair still had symptoms on average 18.2 days per month. Surprisingly, the complex group had fewer days "sick" from non-cardiac causes and had fewer visits to general practitioner or Accident and Emergency Departments. Regression analysis indicates that three variables had significant relevance to the general practitioner or Accident and Emergency visits: complex congenital cardiac disease, fewer visits; Townsend score - more deprivation - more visits; and maternal worry - higher maternal worry score - more visits. Regression analysis indicates that lower activity score is significantly related to complex cardiac disease and higher maternal worry score. CONCLUSIONS: The majority of this group of 4- and 5-year-old children had few residual symptoms and had good exercise tolerance. Maternal worry is a significant factor in influencing both activity levels and frequency of unscheduled health service demands - general practitioner or Accident and Emergency visits.

Telemedicine applications for the regional paediatric cardiology service in Northern Ireland.

The regional paediatric cardiology centre in Northern Ireland has a telemedicine network linking the neonatal units of three district general hospitals using ISDN and IP-compliant equipment. We have previously reported the use of ISDN transmission at 128 kbit/s for remote echocardiography. In a series of 61 patients, a total of 59 transmitted scans were of sufficient diagnostic quality to confirm or exclude the presence of major congenital heart disease (CHD). There were three diagnostic errors (7%). Subsequently, we have examined the use of ISDN transmission at 384 kbit/s. Echocardiographic studies were carried out on 21 patients and all were followed up. Fourteen patients (67%) had CHD confirmed. There were two diagnostic errors (10%). Our experience of transmitting live echocardiographic images suggests that ISDN at 384 kbit/s provides the optimum balance between the clarity of the transmitted images and costs. We expect that IP videoconferencing will offer similar quality but at a lower running cost, and are currently assessing it in a pilot study. All sites within our network will remain capable of ISDN transmission until the efficacy and reliability of IP transmission has been demonstrated in a controlled trial. The clinical telemedicine service has led to the earlier diagnosis of and instigation of appropriate treatment for CHD.

Transcatheter occlusion of the patent ductus arteriosus: a comparison of two devices.

BACKGROUND: Transcatheter occlusion of the arterial duct is a safe and effective alternative to surgical closure. The Rashkind umbrella occluder and the Cook coil are two established devices, although the former is no longer manufactured. AIMS: To assess any difference in outcomes between the use of the Cook detachable coil and the Rashkind double umbrella in patent ductus arteriosus (PDA) occlusion. METHODS: A retrospective study of 77 patients in whom PDA occlusion was attempted using the Cook detachable PDA coil from March 1996 to March 2000. A comparison was carried out with patients in whom occlusion was attempted using the Rashkind double umbrella between 1989 and 1996. RESULTS: The rate of immediate complete occlusion was 24% compared with 29.9% for the Rashkind device. The figure for complete occlusion after 24 hours with the PDA coil was 63% compared with 61.5% in the Rashkind group (p > 0.1). The overall closure rate in the coil group was 72% versus 74.6% for umbrellas. CONCLUSION: The outcome in terms of complete duct closure using the Cook coil is comparable with figures obtained using the Rashkind umbrella. Both devices have a good safety profile in the short and medium-terms.

The incidence of cardiac lesions in infants born with major gastrointestinal malformations in Northern Ireland.

There is a recognised association between major gastrointestinal (GI) malformations and congenital heart disease (CHD). A retrospective study over 10 years involving 240 infants born with gastrointestinal malformations was conducted in the Royal Belfast Hospital For Sick Children (RBHSC). We felt it was important to look at the incidence of CHD diagnosed in the infants presenting to the tertiary referral centre in Belfast. Comparable figures for the incidence of CHD associated with major GI malformations was found in the literature.

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.

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.

Value of a low-cost telemedicine link in the remote echocardiographic diagnosis of congenital heart defects.

We established a low-cost telemedicine link from a district general hospital to the regional paediatric cardiology department about 120 km away. The link was used to transmit echocardiographic images of newborn infants suspected of having congenital heart disease (CHD) to the referral centre, with simultaneous video and audio contact for consultation. Echocardiograms were transmitted for 61 patients suspected of having CHD, aged from 1 to 42 days. The transmitted images were of adequate quality for the paediatric cardiologist to make a diagnosis in 59 (97%). Congenital heart abnormalities were diagnosed in 38 (64%). Twelve patients (20%) had major CHD diagnosed on the transmitted scan and required transfer to the regional cardiology unit either urgently or electively after initial measures to stabilize the patient. Our findings suggest that, for babies suspected of having CHD, ultrasound images of diagnostic quality can be obtained and transmitted using a low-cost telemedicine system.

Congenital hypertrichosis, cardiomegaly and mild osteochondrodysplasia.

We report on a boy with congenital hypertrichosis, cardiomegaly and a mild osteochondrodysplasia, a rare syndrome of which there is only one previous report [Cantú et al., Hum Genet 60:36-41, 1982]. In all, five patients now are known to have this syndrome (2 females, 3 males). As the syndrome has been described in males and females and also in two sibs, inheritance is probably autosomal recessive.

Behavioral adjustment of children with surgically palliated complex congenital heart disease.

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.

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.

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 child's 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.

Kawasaki disease--the Northern Ireland experience.

In the period 1985-1991 inclusive, forty five cases of Kawasaki Disease were identified in Northern Ireland. The number of cases increased each year until 1988 when the incidence was 19 per million population under 16 years, the highest reported for any region in the United Kingdom (data from British Paediatric Surveillance Unit). A high incidence of cardiac involvement was found, with 18(40%) having proximal coronary artery dilatation or aneurysm. and 8(17.7%) with pericardial effusion detected by 2D Echocardiography during or shortly after the acute illness. One year after disease onset, persistent coronary artery changes were detectable by 2D Echocardiography in 53% of those initially affected. There have been no deaths and no patient has so far developed myocardial infarction. One patient has required coronary artery bypass grafting. A protocol for the acute management and long term follow-up of Kawasaki Disease is suggested.

Cardiovascular abnormalities in the oculo-auriculo-vertebral spectrum (Goldenhar syndrome).

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.

Congenital heart disease in Down's syndrome: two year prospective early screening study.

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. SETTING: Regional paediatric cardiology service, Northern Ireland. PATIENTS: 81 newborn infants with Down's syndrome born in Northern Ireland between November 1987 and November 1989. INTERVENTIONS: Clinical examination, chest radiography, and electrocardiography soon after birth followed by cross sectional Doppler echocardiography. MAIN OUTCOME MEASURES: Diagnostic ability of clinical examination, radiography, and electrocardiography compared with echocardiographic findings. RESULTS: 34 babies had congenital heart disease detected by echocardiography (13 had atrioventricular septal defects, seven secundum atrial septal defects, six a solitary patent ductus arteriosus, five isolated ventricular septal defects, and three combinations of heart defects). Individual examination methods were insensitive (the sensitivity of clinical examination was 0.53, of radiography 0.44, and of electrocardiography 0.41) but highly specific (the specificity of clinical examination was 0.94, of radiography 0.98, and of electrocardiography 1.0), although sensitivity improved when the three techniques were combined (the sensitivity was 0.71, the specificity 0.91). CONCLUSION: Echocardiography performed early in life can detect congenital heart disease that might otherwise be missed. Early detection may help prevent complications such as pulmonary vascular disease that may adversely affect the outcome of cardiac surgery.

Ventricular tachycardia associated with Coxsackie B4 virus infection.

We describe a case of ventricular tachycardia in association with acute Coxsackie B4 virus infection occurring in an otherwise normal infant. The dysrhythmia responded to flecainide acetate.

Cardiovascular studies in the mucopolysaccharidoses.

Cardiovascular studies were performed on 22 patients with mucopolysaccharidosis ascertained from an epidemiological study on the mucopolysaccharidoses in Northern Ireland. None of the patients had attended a cardiologist before the study. The main echocardiographical findings were thickening of the interventricular septum and left ventricular posterior wall in the absence of ECG evidence of ventricular hypertrophy. Moreover, reduced QRS voltages were present in the majority of the patients (77%) and some had reduced shortening fraction (33%). These findings suggest an infiltrative cardiomyopathy owing to mucopolysaccharide deposition as a cause of the cardiac thickening rather than true ventricular hypertrophy. Thickening of the mitral valve was present in one case and thickening of the aortic valve in two cases. Involvement of the other heart valves was minimal and aortic valve disease was not found in any of the cases of Morquio's disease type A. In conclusion, the clinical, ECG, and chest x ray findings and echocardiographical evidence for valvular involvement were significantly less than in other studies. Hence, the incidence of clinically significant cardiovascular disease in patients with mucopolysaccharidosis has probably been overestimated.

Origin of the left coronary artery from the right pulmonary artery: a report of successful surgery in a 3-month-old child.

Successful reimplantation into the aorta of an anomalous left coronary artery arising from the right pulmonary artery is described in a 3-month-old child. Preoperative and postoperative angiograms are included, and serial echocardiography demonstrates the marked improvement in left ventricular function.

Chylopericardium: a rare complication of a Waterston shunt.

A case of chylopericardium occurring after a Waterston shunt was successfully managed by pericardial drainage of chyle and substitution of dietary fats with medium chain triglycerides.