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Biomedical subjects

P E Daubeney

Publications and source records attributed to P E Daubeney.

14 recordsLinked to original sources

Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children.

Normalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.

Adolescent↗

Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum.

BACKGROUND: Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. METHODS AND RESULTS: From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100,000 live births). The incidence was 4.1 cases per 100,000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland (P=0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100,000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths (P=0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. CONCLUSIONS: PAIVS is rare, occurring in 1 in 22,000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.

Abortion, Induced↗

Cerebral oxygenation during paediatric cardiac surgery: identification of vulnerable periods using near infrared spectroscopy.

OBJECTIVE: Neurologic sequelae remain a well recognised complication of paediatric cardiac surgery. Monitoring of cerebral oxygenation may be a useful technique for identifying vulnerable periods for the development of neurologic injury. We sought to measure regional cerebral oxygenation in children undergoing cardiac surgery using near infrared spectroscopy to ascertain such vulnerable periods. METHODS: Observational study of 18 children (median age 1.3 years) undergoing cardiac surgery (17 with cardiopulmonary bypass, 8 with circulatory arrest). Regional cerebral oxygenation was monitored using the INVOS 3100 cerebral oximeter and related to haemodynamic parameters at each stage of the procedure. RESULTS: Prior to the onset of bypass, 10 patients had a decrease in regional cerebral oxygenation of > or = 15% points, reaching an absolute haemoglobin saturation less than 35% in 5 cases. The most common cause was handling and dissection around the heart prior to and during caval cannulation. With institution of bypass, regional cerebral oxygenation increased by a mean 18% points to a mean maximum of 75%. During circulatory arrest regional cerebral oxygenation decreased with rate of decay influenced by temperature at onset of arrest (0.25%/min at < 20 degrees C; 2%/min at > 20 degrees C). Reperfusion caused an immediate increase in regional cerebral oxygenation followed by a decrease during rewarming. Discontinuation of bypass caused a precipitous decrease in regional cerebral oxygenation in 5 patients, reaching less than 50% in 3 patients. CONCLUSIONS: These observations suggest that the pre- and early post-bypass periods are vulnerable times for provision of adequate cerebral oxygenation. Near infrared spectroscopy is a promising tool for monitoring O2 supply/demand relationships especially during circulatory arrest.

Brain↗

Aortic valve repair of congenital stenosis with bovine pericardium.

BACKGROUND: Conservative surgical options in the treatment of congenital aortic stenosis are limited. To relieve the obstruction necessitates full incision of the raphe of the larger valve leaflet, but this inevitably causes prolapse. METHODS: We performed aortic valve repair in 6 children, aged 14 months to 17 years, with congenital aortic stenosis, 2 having had aortic valvotomy as infants. The repair consisted of suturing the base of a triangular piece of bovine pericardium, with a simple vertical fold, to the free edges of the incised raphe. The pericardial fold was then sutured vertically to the aortic wall. RESULTS: At follow-up of 2 to 60 months, the mean peak systolic Doppler gradients had decreased from 80 +/- 15 mm Hg to 26 +/- 9 mm Hg. The effective valvular orifice area increased from 33% +/- 6% to 64% +/- 3%, allowing blood flow to increase by a factor of 3.76. Two patients have mild and 2 have mild-to-moderate aortic regurgitation. CONCLUSIONS: The described conservative repair renders the valve tricuspid and trisinusoidal, and the deficient interleaflet triangle is recreated, preventing cusp prolapse. Longer follow-up is required to assess the durability of unstented pericardium in the aortic position, but the early results are encouraging.

Adolescent↗

Cerebral oxygenation measured by near-infrared spectroscopy: comparison with jugular bulb oximetry.

BACKGROUND: Near-infrared spectroscopy is a potential tool for measuring adequacy of cerebral oxygenation during cardiac operations. The cerebral microcirculation is predominantly venous (by volume) and therefore regional cerebral oxygenation measured by near-infrared spectroscopy should reflect jugular bulb venous saturations. METHODS: We compared simultaneous regional cerebral oxygenation and jugular bulb venous saturation measurements in 40 children (median age, 4.5 years; range 2 weeks to 14.5 years) in the cardiac catheter laboratory (n = 29) and during cardiac operations (n = 11). RESULTS: For all patients combined the correlation between regional cerebral oxygenation and jugular bulb venous saturation was 0.69 (p < 0.0001) and was similar for the two groups. For individual children undergoing cardiac operations excellent correlations were obtained (r = 0.78 to 0.96; median, 0.91). However, at low values of jugular bulb venous saturation, regional cerebral oxygenation tended to run high, whereas the converse was true for high values of jugular bulb venous saturation. CONCLUSIONS: These findings suggest that near-infrared spectroscopy may be a useful tool for assessing intravascular cerebral oxygenation during pediatric cardiac operations. Prospective studies of neurologic outcome will be required to establish the value of this technique for assessing the adequacy of cerebral protection.

Adolescent↗

Importance of microdeletions of chromosomal region 22q11 as a cause of selected malformations of the ventricular outflow tracts and aortic arch: a three-year prospective study.

OBJECTIVES: To assess the incidence of microdeletions of chromosomal region 22q11 in a population of infants coming to a regional pediatric cardiac center with selected abnormalities of the ventricular outflow tracts and aortic arch and, further, to provide phenotypic/genetic correlations to determine whether patients with 22q11 deletions can be clinically recognized in infancy. BACKGROUND: DiGeorge syndrome and velocardiofacial syndrome are frequently associated with malformations of the ventricular outflow tracts and aortic arch. Both are usually caused by microdeletions of chromosomal region 22q11. The overall importance of such deletions as a cause of these cardiac malformations remains to be established. STUDY DESIGN: All infants with the candidate cardiac phenotypes during a 34-month period were studied. Dysmorphic features, type of cardiac defect, serum calcium concentration, and thymic status were recorded. Cytogenetic studies, including high-resolution karyotyping and fluorescence in situ hybridization using cosmids (cEO or cH748) from the DiGeorge critical region, were performed after clinical assessment. RESULTS: Fifty infants (including 36 with tetralogy of Fallot with or without pulmonary atresia) were seen during the study period. Twenty-six infants (52%) were dysmorphic, including 19 who were considered to have a phenotypic appearance consistent with 22q11 deletion. Genetic analysis confirmed hemizygosity for 22q11 in 8 of these 19 cases. Results of fluorescence in situ hybridization studies were normal in 22 infants without dysmorphic features and in 5 infants with dysmorphic features not suggestive of a 22q11 deletion. CONCLUSIONS: Microdeletions of chromosomal region 22q11 are an important cause of selected malformations of the ventricular outflow tracts and aortic arch and account for about 15% to 20% of cases. These deletions may be clinically recognized in early infancy and can be rapidly confirmed by fluorescence in situ hybridization.

Aorta, Thoracic↗

Cardiac effects of growth hormone in short normal children: results after four years of treatment.

Growth hormone excess is known to have adverse effects on the heart. The long term cardiac effects of growth hormone given to short normal children as part of a prospective randomised controlled trial of growth hormone treatment (Genotropin 30 IU/m2/week v no treatment) were therefore investigated. Echocardiographic findings are presented for 28 children who have been followed up for a minimum of four years. At the outset, the treated (n = 15) and untreated groups (n = 13) did not differ for any anthropometric or echocardiographic parameter. Their mean (SD) age at onset was 7.8 (0.5) years. After four years of treatment mean height SD score increased from -2.4 to -1.2 compared with no change (-2.5) in the untreated group. Left ventricular posterior wall and septal thickness and left ventricular shortening fraction did not differ between the groups, but a tendency towards increased left ventricular mass was seen in the treatment group (93 v 73 g). No such differential was observed after indexing left ventricular mass for body surface area (79 v 71 g/m2) or lean body mass (3.15 v 3.05 g/kg). It is concluded that prolonged growth hormone treatment does not cause important changes to the heart. A tendency towards increased left ventricular mass simply reflects the increase in lean body mass during treatment.

Anthropometry↗

Risk factors at delivery and the need for skilled resuscitation.

A prospective study was devised to investigate how risk factors at delivery contribute to outcome at birth as measured by the type of resuscitation required ('skilled' or 'not skilled') and the Apgar score. In addition the number of unexpected 'crash calls' was recorded. In a 3-month sample, neonatologists attended 494 (38.6%) of 1279 deliveries. Of those attended, 188 (37.2%) required skilled resuscitation including 5 of 20 crash calls. Four risk factors at delivery were associated with skilled resuscitation: meconium, emergency caesarean section, prematurity and multiple births. Following the implementation of a revised resuscitation policy, a subsequent 3-month sample showed that 299 (24.8%) of 1206 deliveries were attended. A total of 175 (58.5%) required skilled resuscitation including 8 of 16 crash calls. The revised resuscitation policy, in our unit, proved to be safe and led to a reduction of attendance by neonatologists at deliveries.

Apgar Score↗

Hypomelanosis of Ito and precocious puberty.

We describe two girls with hypomelanosis of Ito, one of whom was demonstrated to have diploid-triploid mixoploidy in skin fibroblasts. Both had precocious puberty which was probably gonadotrophin-independent. The association of precocious puberty and hypomelanosis of Ito has not been previously reported. We have speculated on the possible mechanism of gonadotrophin-independent precocious puberty.

Child↗

Intrapericardial lymphangioma presenting as neonatal cardiac tamponade.

We report a unique case of cardiac tamponade in a newborn due to an intrapericardial lymphangioma. Echocardiography and magnetic resonance imaging suggested that the mass, which was situated between the right atrium and the aortic root, was unsuitable for primary resection. A pericardial window was performed, and over the next 16 months the tumor spontaneously regressed.

Cardiac Tamponade↗