Biomedical subjects
Publications and source records attributed to J L Gibbs.
Multiple peripheral pulmonary artery stenoses were detected in three patients with congenital generalised lipodystrophy. This association, which has not been described before, may be clinically important in patients with lipodystrophy who present with impaired exercise tolerance or heart murmurs.
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.
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.
Enhancement of bone mineral acquisition during growth may be a useful preventive strategy against osteoporosis. The aim of this study was to explore the lean mass, strength, and bone mineral response to a 10-month, high-impact, strength-building exercise program in 71 premenarcheal girls, aged 9-10 years. Lean body mass, total body (TB), lumbar spine (LS), proximal femur (PF), and femoral neck (FN) bone mineral were measured using the Hologic QDR 2000+ bone densitometer. Strength was assessed using a grip dynamometer and the Cybex isokinetic dynamometer (Cybex II). At baseline, no significant difference in body composition, pubertal development, calcium intake, physical activity, strength, or bone mineral existed between groups. At completion, there were again no difference in height, total body mass, pubertal development, calcium intake, or external physical activity. In contrast, the exercise group gained significantly more lean mass, less body fat content, greater shoulder, knee and grip strength, and greater TB, LS, PF, and FN BMD (exercise: TB 3.5%, LS 4.8%, PF 4.5%, and FN 12.0%) compared with the controls (controls: TB 1.2%, LS 1.2%, PF 1.3%, and FN 1.7%). TB bone mineral content (BMC), LS BMC, PF BMC, FN BMC, LS bone mineral apparent density (BMAD), and FN bone area also increased at a significantly greater rate in the exercise group compared with the controls. In multiple regression analysis, change in lean mass was the primary determinant of TB, FN, PF, and LS BMD accrual. Although a large proportion of bone mineral accrual in the premenarcheal skeleton was related to growth, an osteogenic effect was associated with exercise. These results suggest that high-impact, strength building exercise is beneficial for premenarcheal strength, lean mass gains, and bone mineral acquisition.
A child with nephrotic syndrome presenting with right heart failure and severe pulmonary hypertension due to pulmonary artery thrombus was treated with locally administered streptokinase infusion. Streptokinase infusion was continued for 8 days, eventually leading to complete dissolution of thrombus.
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.
The condition of a neonate with severe persistent pulmonary hypertension who became severely hypoxic and acidotic despite intensive conventional treatment improved dramatically after endotracheal administration of tolazoline. This logical mode of administration of vasodilator therapy for this condition has not been reported before. It seemed to be life saving in this case and it warrants further clinical trial.
A patient with complex pulmonary atresia and increasing cyanosis due to stenoses of aortopulmonary collateral vessels improved after implantation of balloon expandable stents into the stenosed vessels. Stent implantation may provide an alternative to surgical palliation in this group of patients.
OBJECTIVE: To assess the feasibility of pulmonary artery banding, atrial septectomy or septostomy, and percutaneous stenting of the arterial duct in babies with the hypoplastic left heart syndrome. PATIENTS: Four infants with hypoplastic left heart syndrome. SETTING: Two supraregional paediatric cardiac centres. METHODS: Ductal patency was maintained initially with prostaglandin E. Banding of the proximal branch pulmonary arteries was performed through a median sternotomy and open atrial septectomy was performed if balloon septostomy was not. Stainless steel stents (Johnson & Johnson) mounted in a balloon catheter were implanted into the arterial duct under radiographic control and expanded to a diameter of approximately 8 mm, prostaglandin treatment was then stopped. RESULTS: All the patients survived the immediate postoperative period and maintenance of wide ductal patency was achieved in three of the four patients by stent implantation. Two weeks after the procedure two babies died of right ventricular failure and respiratory infection: some distal ductal constriction had occurred in one where the stent was not quite sufficiently distally placed. One child was discharged home 15 days after treatment and was well at follow up at age 16 weeks and one was stable but required diuretic therapy five weeks after the procedure. CONCLUSIONS: This new approach is technically feasible. At least in the short term it seems to offer hope of effective palliation for the hypoplastic left heart syndrome and it warrants further study.
Permanent pacing of the left ventricle was achieved by transvenous implantation of a steroid-eluting electrode into the coronary sinus in a patient who had undergone a Fontan operation. This approach to endocardial pacemaker implantation is potentially of considerable value in patients who do not have transvenous access to the right ventricle.
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A case of severe pulmonary stenosis associated with a subpulmonary ventricular septal defect and interruption of the aortic arch is described in a 5-month-old girl. This combination defies explanation by current theories of the development of obstructive abnormalities of the aortic arch secondary to reduced aortic flow during fetal life, and, to the best of our knowledge, is the first reported case with this particular association.
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.
OBJECTIVE: To assess the possibility of maintaining ductal patency in neonates with complex pulmonary atresia by percutaneous implantation of balloon expandable stents. PATIENTS: Two duct-dependent neonates with long segment pulmonary atresia, right sided aortic arch, and left sided arterial duct. RESULTS: Stents with final diameter of 3.5 or 4 mm and initial length of 7 or 15 mm were successfully positioned in the arterial duct. Two stents were required in one child and four in the other in order to stent the entire length of the duct. After the procedures the ducts remained widely patent and arterial oxygen saturations remained above 80%. Complications of the procedures included perforation of a peripheral pulmonary artery and cardiac perforation, both caused by guide wire manipulation. Both babies died suddenly, one at five weeks, and the other at nine days after successful stenting of the duct. Both ducts were patent at necropsy; the exact cause of one death was not clearly defined, but the second seemed to be caused by pneumococcal septicaemia. CONCLUSIONS: Stenting of the arterial duct is technically feasible. It provides adequate palliation for neonates with pulmonary atresia at least in the short term and it seems to result in balanced, central perfusion of both pulmonary arteries. This preliminary report suggests that this previously untried technique may prove to be a promising and attractive alternative to neonatal aortopulmonary shunt operation.
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.
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.
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.