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Biomedical subjects

D F Dickinson

Publications and source records attributed to D F Dickinson.

At least 19 recordsLinked to original sources

Natural history of cardiac involvement in geleophysic dysplasia.

Geleophysic dysplasia is an autosomal recessive short-limbed, dysmorphic syndrome. The condition is frequently associated with cardiac valvular disease, which may result in secondary hypertrophy and cardiac failure. We describe two distantly related Pakistani children with classical features of geleophysic dysplasia. Pulmonary stenosis was recognized in both within a few months of birth. The younger child shows no signs of cardiac decompensation at the age of 7 years whilst her older cousin has developed a significant pulmonary gradient requiring surgical intervention in the second decade of life. The natural history of his disorder highlights that the cardiac involvement seen in apparently stable forms of geleophysic dysplasia is frequently progressive.

Child↗

Haemolysis following implantation of duct occlusion coils.

OBJECTIVES: To describe the incidence and management of haemolysis after transcatheter coil occlusion of the arterial duct. DESIGN: Prospective clinical and echocardiographic follow up of patients who have undergone implantation of the Cook detachable duct occlusion coil. SETTING: Tertiary paediatric cardiac centre. PATIENTS: Five cases of haemolysis (two girls aged 6 and 11 months; three boys aged 6, 17, and 14 months) from a series of 137 duct coil implantations. MAIN OUTCOME MEASURES: The occurrence of clinically significant haemolysis after implantation of duct occlusion coils and resolution of haematuria after completion of duct occlusion. RESULTS: Haemolysis was detected in five of 137 procedures following implantation of Cook detachable duct coils. Four patients became symptomatic 12 hours after the procedure but in one haemolysis was detected three months later. Resolution of ongoing haemolysis was achieved within 48 hours of detection with further coil implantations, but haematuria persisted for up to 10 days. In one patient the extensive destruction of erythrocytes resulted in acute renal failure requiring peritoneal dialysis. CONCLUSIONS: Haemolysis is an important complication after duct coil implantation. It occurred in 3.6% of 137 procedures in this series and is most likely to occur in young patients with relatively large ducts. Further coil implantation to occlude the duct completely is not only successful but technically relatively straightforward and should be undertaken early if major complications such as severe anaemia and renal failure are to be avoided.

Acute Kidney Injury↗

Right ventricular outflow stent implantation: an alternative to palliative surgical relief of infundibular pulmonary stenosis.

OBJECTIVE: Preliminary assessment of the use of stents for palliative relief of right ventricular infundibular stenosis as an alternative to palliative surgical ventricular outflow enlargement. DESIGN: Descriptive clinical study. PATIENTS: Four patients with right ventricular outflow obstruction, aged between 2 and 15 years. One had had previous palliative surgery for pulmonary atresia, one had hypoplastic pulmonary arteries after palliative surgery for tetralogy of Fallot, one had multiple congenital abnormalities, and one had hypertrophic cardiomyopathy. SETTING: Tertiary paediatric cardiac centre. METHODS: After initial echocardiographic diagnosis the extent of right ventricular outflow obstruction was assessed by angiography. Balloon expandable stainless steel stents (Johnson & Johnson) were deployed in the right ventricular infundibulum. MAIN OUTCOME MEASURES: Improvement in right ventricular outflow assessed by ventriculography and change in right ventricular/ left ventricular pressure ratio, change in systemic oxygen saturation, freedom from arrhythmias, and sustained improvement in echocardiographic indices of obstruction. RESULTS: Mean right to left ventricular pressure ratio fell from 0.95 to 0.35 in the three patients with intact ventricular septum. Oxygen saturation increased from 76% to 91% in the patient with tetralogy. No arrhythmias were detected. Improvement was maintained at mean follow up of 9.7 months in three cases, but one patient required stent enlargement 17 months later because of neoendothelial proliferation within the stent. CONCLUSION: Stent implantation provides an effective alternative to palliative surgical enlargement of the right ventricular infundibulum. Neoendothelial proliferation causes reduction in lumen in some cases, but this may respond to redilatation.

Adolescent↗

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.

Catheterization↗

Transcatheter occlusion of the arterial duct with Cook detachable coils: early experience.

OBJECTIVE: To assess the effectiveness of modified, controlled release Gianturco coils for transcatheter occlusion of the arterial duct. DESIGN: Prospective study, approved by local medical ethics committee. SETTING: Tertiary paediatric cardiac centre. PATIENTS: 43 patients with left to right shunting through the arterial duct, two of whom had a residual leak after surgical ligation and three had residual shunting after previous Rashkind umbrella implantation. INTERVENTION: Transcatheter delivery of one or more coils to the arterial duct. MAIN OUTCOME MEASURES: Complete occlusion of the arterial duct, based on intention to treat and judged by Doppler echocardiography. Absence of flow disturbance in the branch pulmonary arteries and the descending aorta following the procedure. Assessment of cost of the disposable items used. RESULTS: At a median follow up period of three months complete duct occlusion was achieved in 37 (86%) of the 43 patients. No flow disturbance in the branch pulmonary arteries or the descending aorta was detected in any patient. The median cost of disposable items used during the procedure was 342 pounds. CONCLUSIONS: The Cook detachable coil is an effective and financially attractive alternative to the Rashkind umbrella for closure of the arterial duct.

Adolescent↗

Anaemia in children following cardiac transplantation: treatment with low dose human recombinant erythropoietin.

Anaemia is common in children following cardiac transplantation. In a series of 5 children with anaemia beyond the immediate post-operative period one had a hypochromic, microcytic anaemia which corrected with oral iron. The other four had normochromic, normocytic anaemias unresponsive to iron or folate supplementation and associated with inappropriately low levels of erythropoietin. Subcutaneous administration of low dose human recombinant erythropoietin to these four patients resulted in correction of their anaemia. Our findings suggest that erythropoietin deficiency is an important cause of anaemia in transplant recipients and should be sought in cases of anaemia refractory to conventional haematinic therapy. In cases of proven erythropoietin deficiency, treatment with erythropoietin is effective, acceptable to patients and preferable to repeated blood transfusion.

Adolescent↗

Transoesophageal echocardiography in neonates, infants and children: applicability and diagnostic value in everyday practice of a cardiothoracic unit.

OBJECTIVE: To determine the applicability of and information obtained by transoesophageal echocardiography in neonates, infants, and children in every day practice of a cardiothoracic unit. DESIGN: Four month prospective study. SETTING: Supraregional centre for paediatric cardiothoracic services. PATIENTS AND METHODS: 58 patients aged between four days and 16 years with a wide range of cardiovascular disease underwent transoesophageal echocardiography under sedation or general anaesthetic. One of two paediatric probes (6 or 7 mm diameter), or an adult probe (13 mm in diameter) was used. Whenever possible the investigation was immediately preceded by precordial echocardiography. MAIN OUTCOME MEASURES: Success, failure, technical difficulties, and complications of probe introduction and, when possible, comparison of the information obtained with that obtained from precordial echocardiography. RESULTS: Introduction of the probe was successful in 57 of the 58 patients. The only complication encountered was transient bradycardia during manipulation of the probe in a 2.2 kg baby. The adult (13 mm) probe was successfully used in children as small as 7.0 kg. Below this weight a smaller paediatric probe was required. In 56% of cases transoesophageal ultrasound provided information not obtained from the precordial approach. The technique was of particular value perioperatively and in the immediate postoperative period in neonates and infants and in the presence of valve prostheses and the investigation of mediastinal tumours in older children. CONCLUSIONS: Transoesophageal echocardiography is a valuable additional investigative tool for children of all ages. It is of particular value when acquisition of precordial image is impaired around the time of and after operation and in children with prosthetic valves or mediastinal tumour. Image quality was superior with the adult probe and we recommend the use of this probe unless the patient's weight is below 7 kg, when a paediatric probe allows this technique to be used usefully and safely in babies as small as 2.2 kg.

Adolescent↗

Continuous wave Doppler echocardiography after surgical repair of coarctation of the aorta.

OBJECTIVE: To find how closely pressure gradients across the aortic arch derived from Doppler echocardiography reflect gradients measured by catheter after surgical repair of coarctation of the aorta. DESIGN: Pressure drop across the aortic arch was measured simultaneously by continuous wave Doppler and double lumen catheter in 20 patients with repaired coarctation of the aorta. RESULTS: The peak pressure drop estimated by Doppler was almost invariably higher than the peak to peak gradient measured by catheter, as might be expected. Wide variation was seen between the Doppler measured pressure drop and instantaneous peak gradient measured by catheter, ranging from +22 to -17 mm Hg. The reasons for these differences are unclear but are probably related to a combination of complex flow dynamics in the aortic arch, difficulty in closely aligning the Doppler beam with flow, and inability to measure flow velocity immediately proximal to the site of the surgical repair with continuous wave Doppler. CONCLUSIONS: Continuous wave Doppler echocardiography may significantly overestimate or underestimate the pressure drop after repair of coarctation and it should be interpreted with caution in individual patients. Catheterisation with angiography remains the reference standard for assessment of surgical repair of the aortic arch.

Adolescent↗

Chorea after cardiopulmonary bypass: exacerbation by captopril.

Chorea is a rare complication of major cardiac surgery, which has been related to profound hypothermia and circulatory arrest. We describe a case of choreic syndrome in a child which followed normothermic cardiac bypass and only became apparent clinically when the child was treated with captopril.

Captopril↗

Long-term outcome for children with acute renal failure following cardiac surgery.

Acute renal failure requiring dialysis occurred in 34 children (2.9%) following cardiac surgery over a five year period. 17 children (50%) recovered renal function with 11 (32%) long-term survivors. The long-term outcome for the survivors, in terms of renal function, was studied from 1 to 5 years after their episodes of acute renal failure. Three children had significant abnormalities of renal function despite normal urinalysis. Detailed assessment of renal function is advocated for children who survive acute renal failure following cardiac surgery.

Acute Kidney Injury↗

Day case ligation of patent ductus arteriosus in preterm infants: a 10 year review.

Since 1978, 136 preterm babies received ligation of a patent ductus arteriosus as day cases. A total of 122 babies whose notes were available for review, with a median gestational age of 27 weeks (range 23-35) and median birth weight of 960 g (range 470-2750), were transported distances of up to 80 miles. The median ages at ligation with and without previous medical management with indomethacin were 23 and 15 days, respectively. One hundred and ten (90%) babies were dependent on ventilatory support, but extubation was achieved at a median time of 10 days after ligation, regardless of postnatal age at the time of ligation. There were no deaths associated with the operation, and no complications resulting from transportation. The hospital mortality was 15 (12.3%), and the most important (and significant) adverse factor was a preoperative fractional inspiratory oxygen content greater than 0.3. The results of this study show that day case ligation of the patent ductus arteriosus is safe, and if it is carried out early will reduce the time before extubation and discharge from the intensive care unit.

Birth Weight↗

Functional aortic atresia in congenitally corrected transposition.

An infant with congenitally corrected transposition and severe regurgitation through the left atrioventricular valve presented with clinical and Doppler echocardiographic features of aortic atresia. This eventually proved to be functional. This clinical situation had not, to the best of our knowledge been previously reported before.

Aortic Valve Stenosis↗

Limitations of Doppler ultrasound in the diagnosis of neonatal coarctation of the aorta.

Blood flow velocities were recorded using Doppler ultrasound in 40 babies aged between 1 day and 4 weeks who had coarctation of the aorta or interruption of the aortic arch. High velocity jets of 3 m/sec or more in the descending aorta were detected in only 3 patients, two of whom had interruption of the aortic arch. There was very poor agreement between upper and lower limb blood pressure difference and Doppler derived peak pressure drop. Doppler ultrasound may provide useful information on coexisting intracardiac abnormalities, but in the presence of ductal patency is of little value in the diagnosis of coarctation itself.

Aortic Coarctation↗

Balloon dilatation of stenosed Blalock-Taussig shunts.

Three patients with complex cyanotic congenital heart disease, who had previously undergone Blalock-Taussig shunt operations, developed severe stenoses at the pulmonary anastomosis of the shunts. All were successfully treated with balloon dilatation, which can thus be considered an effective alternative to reoperation.

Anastomosis, Surgical↗