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Biomedical subjects

E Shinebourne

Publications and source records attributed to E Shinebourne.

At least 19 recordsLinked to original sources
PubMed2001

Rupture of pulmonary aneurysms in association with long-standing Waterston shunts.

Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinicopathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.

Anastomosis, Surgical↗
PubMed2001

Are infant behavioural feeding difficulties associated with congenital heart disease?

AIM: To compare the feeding patterns and difficulties of infants with congenital heart disease (CHD) and healthy controls. Information was gathered via parental questionnaires. METHODS: A matched case controlled study of 64 infants with CHD compared with 64 healthy controls. RESULTS: The main findings were: (1) Feeding patterns: mothers with infants with CHD used bottle-feeding as a first method of feeding their babies more often (CHD, 20%, controls, 2%); (2) Specific feeding difficulties: (a) infants with CHD were significantly more breathless when feeding (CHD = 16%, controls, 0%), (b) had more vomiting at mealtimes (CHD = 23%, controls = 11%), but (c) had significantly less spitting (CHD = 19%, controls, 41%); and (3) infants with CHD showed significantly reduced growth. CONCLUSIONS: The feeding difficulties are related to the organic condition and not specific difficulties in mother-infant interaction. Professional support may be required for mothers of infants with CHD to maintain feeding routines and to deal with the difficulties that arise.

Breast Feeding↗
PubMed1997

Coagulation factor abnormalities after the Fontan procedure and its modifications.

OBJECTIVE: Recently we reported the prevalence of thromboembolism in patients who underwent the Fontan procedure and its modifications. Although hemodynamic factors may well contribute to thromboembolism, recent evidence suggests that coagulation factor abnormalities may also play a role. We therefore set out to investigate the coagulation status in a group of patients who had undergone the Fontan procedure. METHODS: The study population consists of 20 children who had undergone the Fontan procedure and its modifications. They were examined for coagulation factor abnormalities. Concentrations of serum albumin, total protein, and liver enzymes were also measured. The median age at the time of the operation was 6.2 years (17 months to 8 years) with a male/female ratio of 2.3:1. The median time from the Fontan repair was 4.9 years (18 to 76 months). RESULTS: Protein C (p < 0.001), protein S (p < 0.02), and factor VII (p < 0.001) were significantly lower than the normal range. The changes in serum albumin and total protein and factors II, IX, and X were not significant. CONCLUSIONS: It is possible that deficiency in protein C, protein S, and factor VII partly account for the prevalence of thromboembolism after Fontan-type repairs. The risk of long-term anticoagulation should be weighed against the best palliative procedure for these patients. We suggest that reduced protein C, protein S, and factor VII levels in this group of patients should be regarded as risk factors and that such patients should be treated with anticoagulants.

Child↗
PubMed1997

Anatomic correction of the syndrome of prolapsing right coronary aortic cusp, dilatation of the sinus of Valsalva, and ventricular septal defect.

BACKGROUND: Although the syndrome of ventricular septal defect and aortic regurgitation was described a long time ago, there is still no agreement about the anatomic and functional components of the syndrome and the optimal methods of management. OBJECTIVE: Our objective was to describe a new simple technique of anatomic correction of all the components of the syndrome, based on redefining the salient anatomic and functional features of the syndrome. METHODS: Anatomic correction of the syndrome is achieved through a transaortic approach with the placement of a series of pledget-supported mattress sutures using autogenous pericardium. The sutures are used to close the ventricular septal defect, plicate the aortic sinus, and correct the outward and downward displacement of the anulus of the aortic valve. The technique is designed to correct all the anatomic functional components including severe aortic regurgitation when present. RESULTS: Between 1972 and 1996, 46 patients with this syndrome underwent surgical treatment. The current technique was used in most of the patients operated on before 1981 and in all patients since that date. There were no early or late deaths during a follow-up period varying from 3 months to 24 years (mean 8.4 years). Aortic regurgitation was abolished in 16 and improved in the remaining patients, The hemodynamic results have been maintained except in five patients operated on early in the series, in whom additional procedures on the cusps were performed. CONCLUSIONS: Anatomic correction of all the components of the syndrome of prolapsing right coronary cusp, dilatation of the sinus of Valsalva, and ventricular septal defect, can be achieved by a very simple technique. This technique can be applied in young children and prevents progression and secondary changes. Early correction in all patients with this syndrome is warranted.

Adolescent↗
PubMed1993

Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients.

OBJECTIVES: The purpose of this study was to determine the incidence of cardiologic abnormalities in Noonan syndrome. BACKGROUND: The incidence of cardiac abnormalities in Noonan syndrome remains unknown, largely because of such difficulties as assembling a substantial cohort, ensuring a correct phenotypic diagnosis and providing accurate definitions of the most frequent abnormalities--pulmonary stenosis and left ventricular hypertrophy. METHODS: A cohort of 145 patients was assembled, and before cardiologic assessment two independent geneticists scrutinized the phenotype. The diagnosis was confirmed in 118 patients, and they were studied by two-dimensional and Doppler echocardiography. RESULTS: A dysplastic pulmonary valve was present in eight patients (7%) and was associated with significant stenosis in six (75%) of the eight. Significant stenosis was present in 22 (20%) of 110 patients without dysplasia. Left ventricular hypertrophy was present in 29 patients (25%) without significant pulmonary stenosis. Localized anterior septal hypertrophy was the most common pattern in 12 (41%) of 29 patients. Diffuse hypertrophy involving the entire septum and the free wall was present in nine patients (31%) and was severe (> 1.7 cm) in five. Other abnormalities included secundum atrial septal defects (10%). CONCLUSIONS: The high incidence of cardiac abnormalities suggests that echocardiographic and Doppler evaluation of patients with the Noonan phenotype is important because it will aid in genetic counseling and in the assessment of the natural history of--and, ultimately, identification of the gene(s) responsible for--Noonan syndrome.

Adolescent↗
PubMed1984

Factors influencing parental participation in a paediatric cardiology outpatient clinic.

Twelve-hundred consultations at a paediatric cardiology outpatient clinic were observed and tape-recorded. A random sample of 102 consultations was selected and an analysis made of the demand for information by parents and the extent to which this was met. Statistically significant differences in numbers of questions asked were found related to stage of treatment, seriousness of condition and outcome of consultation. Most questions were asked either when definitive information was available (i.e. following cardiac catheterisation) or when active intervention was contemplated (i.e. catheterisation or surgery). Contrary to parents' accounts in subsequent home interviews, the number of questions they ask is unrelated to the length of the consultation. Similarly the presence of many medical staff at the consultation, was positively correlated with the number of parents' questions. In general there was a good fit between parental participation and those occasions where most was at stake except at the first outpatient appointment. This was regarded, at least in retrospect, as unsatisfactory by many parents irrespective of its length or content. We suggest that an additional but optional "pre-admission" outpatient appointment where the family visit the wards prior to the child's admission to hospital may help parents cope with their anxieties.

Child↗
PubMed1984

Signal averaged electrocardiography in infants and children with congenital heart disease.

Forty-four patients with congenital cardiac disease underwent surface averaged electrocardiography using a high resolution purpose built module. Twelve patients had complete transposition of the great vessels, 14 had tetralogy of Fallot, 5 had ventricular septal defects, 6 atrial septal defects, 3 atrioventricular septal defects and 4 patients had miscellaneous non-structural abnormalities. All patients with structural disorders underwent corrective or palliative surgery. The aim of the study was to examine the feasibility of the method and its usefulness in detecting His potentials and delayed potentials in the ST segment. A His potential was recorded in 30 patients (68%) with an amplitude ranging from 1.25 to 8.25 microV. Delayed potentials were recorded in only 1 patient with frequent ventricular premature beats. This technique may be useful in assessing intraventricular conduction times in patients with bundle branch block and/or axis deviation especially those who are at risk from intermittent complete AV block after surgical intervention. Detection of delayed potentials may allow better assessment of post-operative risk of ventricular arrhythmias in patients who have undergone ventricular surgery.

Bundle of His↗
PubMed1983

Diminutive pulmonary artery growth following right ventricular outflow tract enlargement.

We have assessed the clinical results and angiographic pulmonary artery growth following construction of the right ventricular outflow tract in 10 children with tetralogy of Fallot or pulmonary atresia. All cases were associated with diminutive pulmonary arteries and considered unsuitable for total corrective surgery. The mean age of the children was 34 months, mean weight 10 kg and mean body surface area 0.48 m2. The right ventricular outflow tract was constructed by insertion of a patch of dura mater (5 patients), pericardium (2 patients), homograft valved conduit (2 patients) and infundibulectomy (1 patient). The ventricular septal defect was not closed. Study of the patients between 13 and 37 months postoperatively revealed an increase in mean arterial oxygen saturation of 22% (P less than 0.01) and a fall in mean haemoglobin concentration of 1.6 g% (P less than 0.05). Overall change in pulmonary artery diameter compared to that of the trachea was insignificant although in individual cases a 50% increase in diameter was shown. Palliation of symptoms was equivalent to conventional shunting procedures. Closure of the ventricular septal defect was performed successfully in 2 patients at a later date.

Child↗
PubMed1977

Transatrial repair of ventricular septal defects with reference to their anatomic classification.

A consecutive series of 50 children undergoing elective operations for ventricular septal defect (VSD) is presented. Atriotomy was performed routinely. Repair of the defect through the tricuspid valve was attempted in all cases and achieved in 72 percent. There was a 24 percent incidence of right bundle branch block. All patients improved symptomatically after the operation. The mortality rate was 4 percent. The right atrial route is considered to be preferable as a primary approach and has no disadvantages.

Child↗
PubMed1977

Development of a highly sensitive radioimmunoassay for digoxin and its application in pediatric practice.

The sensitivity of two established routine digoxin radioimmunoassay methods has been increased to enable the provision of a rapid and relatively atraumatic inpatient and outpatient service for neonates and small children, using capillary blood samples obtained by heel-prick. The methods employ 125I- or 3H-labelled digoxin, a rabbit antiserum raised against a digoxin: bovine serum albumin conjugate and only 10 or 25 microliter of plasma as the sample. The results obtained using these highly sensitive assays correlate closely with those found using conventional assays, requiring larger sample volumes. An apparent difference in sensitivity to digoxin has been demonstrated between infants and children more than 1 yr old. Thus infants appear to tolerate plateau phase plasma levels (mean value for non toxic infants 2.6 +/- 1.8 ng/ml) that in older children or adults would be associated with digoxin toxicity.

Binding Sites, Antibody↗
PubMed1976

Surgical correction in complete levotransposition of the great arteries with an unusual subaortic ventricular septal defect.

Six children with an uncommon variant of complete (that is, physiologically uncorrected) transposition of the great arteries are described. In this malformation, levoposition of the aorta is coincident with situs solitus and concordant atrioventricular relations. All patients underwent successful surgical correction. Four had a ventricular septal defect; in three, the defect was subaortic and because of its unusual anatomic features, a right ventriculotomy was required for repair. Interatrial transposition of venous return was carried out in all cases by insertion of a baffle, as in complete dextrotransposition of the great arteries. The surgical verification of the arterial positions in these cases illustrated the fallibility of the so-called loop rule. The significance of the cases in relation to terminology, classification and morphogenesis of this variant is discussed.

Angiocardiography↗
PubMed1970

Haemodynamic studies in hypertensive patients treated by oral proranolol.

Haemodynamic studies have been performed in 9 patients whose blood pressures had been satisfactorily controlled with oral propranolol for 7 to 29 months. A postural drop of blood pressure or a fall of blood pressure on exercise did not occur. Pulse rate and cardiac output were below normal at rest and on exercise; peripheral resistance was high. The results are similar to those obtained in a comparable group of hypertensives after acute administration of propranolol. These findings are discussed in relation to the mode of action of propranolol in hypertension.

Adult↗
PubMed1969

Significance of electrocardiographic changes in hypertension.

Studies in 17 hypertensive patients showed that the electrocardiographic features of T-wave inversion and S-T segment depression distinguish those patients with higher systolic blood pressures. They do not indicate impaired cardiac function.

Adult↗