PubMed Health⌕ Search

Biomedical subjects

R Radley-Smith

Publications and source records attributed to R Radley-Smith.

At least 19 recordsLinked to original sources
PubMed2001

Returning to school after heart or heart-lung transplantation: how well do children adjust?

BACKGROUND: Up to 40% of children and adolescents with chronic illness experience school-related problems, including learning difficulties and problems in social adjustment and peer relationships. Despite the life-threatening nature of heart and heart-lung transplantation and the severity of illness, which results in the necessity for surgery, there is little information on the school performance of children after transplantation. METHODS: Eighty-one children and adolescents were assessed with regard to their academic attainments and behavior at school at regular intervals after heart (n=47) or heart-lung (n=34) transplantation and comparisons made with a group of healthy children. RESULTS: Cognitive ability and performance on academic attainments were within the normal range and did not change significantly as a function of time since transplant. However, performance was at a significantly lower level than that of the healthy children. Although the prevalence of behavior problems was only 8% at 6 months posttransplant, at 3 years, it had increased to 29% and, at 5 years, it was still 27%. Children with an initial diagnosis of congenital heart disease had more academic and behavioral difficulties than those with either cardiomyopathy or cystic fibrosis. CONCLUSIONS: A significant number of children who had undergone successful transplantation experienced difficulties at school. Contrary to expectations, educational problems were more prevalent in the medium term, rather than short term, after transplant. Initial diagnosis was a salient factor in posttransplant psychological functioning at school. Early intervention and close liaison with schools is indicated to reduce psychological morbidity and enhance adaptation within the school environment.

Absenteeism↗
PubMed1999

Early and long-term results of a valve-sparing operation for Marfan syndrome.

BACKGROUND: We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. METHODS AND RESULTS: Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63. 6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. CONCLUSIONS: Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

Adolescent↗
PubMed1999

Mobilization of the left and right fibrous trigones for relief of severe left ventricular outflow obstruction.

BACKGROUND: There is still no agreement about the optimal method of surgical relief of fixed subaortic stenosis, particularly the severe forms. OBJECTIVES: The purpose of this study was to describe a new technique for the relief of subaortic stenosis based on analysis of the functional anatomy of the left ventricular outflow tract and pathophysiologic features of subaortic stenosis. METHODS AND PATIENTS: We propose that one of the basic abnormalities in subaortic stenosis is interference with the hinge mechanism provided by the 2 fibrous trigones with progressive deposition of fibrous tissue in these angles. The technique described in this paper consists of excision of all components of the fibrous "ring," with mobilization of the left and right fibrous trigones. This results in the restoration of the normal dynamic behavior of the left ventricular outflow tract with maximal widening of the outflow tract as the result of backward displacement of the subaortic curtain and anterior leaflet of the mitral valve. This technique has been used in 57 consecutive patients who ranged in age between 5 months and 56 years (mean, 15.5 +/- 10.6 years). Gradients across the left ventricular outflow tract were between 45 and 200 mm Hg (mean, 86.7 mm Hg). Additional lesions were present in 10 patients, and 7 patients had had 8 previous operations on the left ventricular outflow tract. At operation, in addition to resection of subaortic stenosis, 3 patients had aortic valvotomy, 2 patients had homograft replacement of the aortic valve, 7 patients had patch closure of a ventricular septal defect, and 1 patient had open mitral valvotomy. RESULTS: There were 2 early deaths and 1 late sudden death during the follow-up period that ranged from 1 month to 25 years (mean, 15. 2 years). One patient experienced the development of endocarditis on the aortic valve 7 years after operation, which was successfully treated by homograft replacement. Postoperative gradients across the left ventricular outflow tract varied from no gradient to 30 mm Hg (mean, 8 mm Hg). There were no instances of recurrence of a gradient across the left ventricular outflow tract. CONCLUSION: It is concluded that mobilization of the left and right fibrous trigones results in durable relief of subaortic stenosis.

Adolescent↗
PubMed1999

The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

Child, Preschool↗
PubMed1998

Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root.

OBJECTIVES: There is still no agreement about the best method of dealing with malfunction of the aortic valve caused by aneurysm or dissection of the aortic root. The experience, rationale, and development of a valve-preserving technique introduced and used since 1979 is described. METHODS: During this period 158 patients (78% of all patients undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Their ages ranged from 2 to 72 years (mean 46.6 years). Of the patients 107 were male and 51 were female. A total of 68 patients had skeletal manifestations of Marfan's syndrome. The original disease was chronic aneurysm of the ascending aorta or root in 92 (58.2%), chronic dissection in 17 (10.8%), and acute dissection in 49 (31%) patients. One hundred eleven additional procedures were performed in 84 patients. In all there were five early deaths (4.6% +/- 2%) in the 109 patients with chronic aneurysm and one death in the 103 patients operated on electively (0.97% +/- 0.9%). Actuarial survival for patients operated on for chronic aneurysm was 93.3%, 88.0%, 79.0%, and 57.9% at 1, 5, 10, and 15 years and 96.8%, 91.2%, 82.0%, and 60.0% for those operated on electively. Actuarial survival for patients operated on for acute dissection was 72.8%, 63.4%, and 53.3% at 1, 5, and 10 years. The probability of needing reoperation was 3.0% +/- 2%, 11% +/- 0.5%, and 11% +/- 0.5% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in two patients operated on early in the series who had cusp extension. No anticoagulants were used. Echocardiography showed reduction in left ventricular end-systolic and end-diastolic dimensions, which was maintained. At the end of follow-up trivial or no aortic regurgitation was demonstrated in 63.6%, mild to moderate in 33.3%, and severe in 3%. CONCLUSIONS: Valve-sparing operations are possible in a large proportion of patients with aneurysms of the ascending aorta and the medium and long-term results are encouraging.

Adolescent↗
PubMed1997

Heterotopic cardiac transplantation in infants and children.

BACKGROUND: Children with advanced heart failure, particularly those with elevated pulmonary vascular resistance, pose a difficult management problem because the normal donor right ventricle cannot cope with the high pulmonary resistance and because of the relative shortage of donor organs of an appropriate size for this age group. METHODS: In an attempt to address these issues and evaluate the role of heterotopic transplantation in this context, we operated on 12 children, six boys and six girls, in the period between January 1, 1991, and March 31, 1996. Their ages ranged from 11 months to 15.2 years (mean 81.6 +/- 62.8 months) and their mean weight was 23.3 kg (range 7.6 to 56.8 kg). Eight patients (66.6%) had significant elevation of pulmonary artery pressure (pulmonary artery systolic pressure = 66 +/- 9.4 mm Hg, mean transpulmonary gradient = 22.3 +/- 3.4 mm Hg). In all patients the donor pulmonary artery was anastomosed to the recipient right atrium without the use of any prosthetic material. Ischemic times varied between 135 and 255 minutes (mean 182.1 +/- 30.7 minutes). The immunosuppression regimen included cyclosporine and azathioprine. Steroids were not routinely used. RESULTS: One patient died in the hospital of acute rejection on postoperative day 16. Three patients had lobe collapse within 1 week and all were treated successfully. Two late deaths (18.2%) occurred as a result of cardiac rejection 3 months and 2 years after the operations. Nine survivors (75%) are alive, active, and growing normally at a mean follow-up of 2.2 years (range 11 months to 4.75 years). Repeated cardiac catheterization performed in seven patients with preoperative pulmonary hypertension showed a slow progressive drop in mean pulmonary artery pressure. No significant change was observed in the function of the recipient hearts. CONCLUSION: We conclude that heterotopic heart transplantation is feasible for a selected group of children with good medium-term results, notably regression of pulmonary artery pressure, normal growth, and lack of long-term chest complications.

Adolescent↗
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↗
PubMed1995

Reoperative aortic valve operation after homograft root replacement: surgical options and results.

The surgical options available and the associated operative risks for repeat aortic valve replacement after free-standing homograft root replacement with reimplantation of the coronary arteries are as yet undefined. We therefore reviewed our experience with repeat aortic valve replacement between January 1976 and July 1994 and identified 22 such procedures performed on 21 patients after homograft or autograft root replacement. Reoperation was indicated for structural deterioration in 16 and for bacterial endocarditis in 6 patients. Associated procedures were undertaken in 5 patients, and 4 procedures were performed emergently. Significant calcification of the homograft wall was noted in over half of all patients. There were no coronary ostial complications. In 13 patients new valves were inserted within the previous root: 9 mechanical valves and 4 subcoronary homografts. In 9 patients the root was re-replaced. The mean cross-clamp time was 85 (+/- 20) minutes and mean cardiopulmonary bypass time was 123 (+/- 32) minutes. There were no early deaths and 5 late deaths. Two patients required reoperation for bleeding and 2 experienced low cardiac output syndromes postoperatively. Univariate analysis failed to identify any variables predictive of outcome. We conclude that repeat aortic valve replacement after homograft root replacement, even in the presence of significant calcification, can be undertaken with an acceptable operative risk and should not be delayed until irreversible ventricular dysfunction has occurred. The same options available at initial valve replacement may be employed at reoperation, although extensive calcification mandates repeat root replacement.

Adolescent↗
PubMed1995

Fourteen-year experience with homovital homografts for aortic valve replacement.

Two hundred seventy-five unprocessed, viable homograft ("homovital") aortic valves were used for aortic valve replacement in patients aged 1.5 to 79 years (mean 45.8 +/- 19 years) with maximum follow-up of a 14-year period (mean 4.8 years). Ninety-two percent (252 patients) had New York Heart Association class III or IV functional status before operation and 25 underwent emergency operation. Valves were harvested under sterile conditions and kept in nutrient medium 199. Freehand (subcoronary) technique was used in 147 patients and freestanding root replacement was used in 128. Cumulative survival rates for the whole group were 92% +/- 2% at 5 years and 85% +/- 3% at 10 years, as compared with 96% +/- 2% and 94% +/- 4%, respectively, for the 98 patients who underwent isolated root replacement. Multivariate analysis determined that root replacement with associated procedures and operation for prosthetic endocarditis were risk factors for death, whereas previous xenograft valve, operation for endocarditis, and operation for aortic regurgitation were risk factors for reoperation. Actuarial rates for freedom from degenerative valve failure diagnosed at operation, by postmortem examination, or by routine echocardiography were 94% +/- 2% at 5 years and 89% +/- 3% at 10 years. Recipient age younger than 30 years and previous xenograft valve were risk factors for late degeneration. We conclude that homovital valves demonstrate good durability, particularly in patients older than 30 years, who had a 10-year freedom from degeneration rate of 97%.

Adolescent↗
PubMed1995

Visceral leishmaniasis after cardiac surgery.

An English child developed visceral leishmaniasis (kala-azar) after cardiac surgery. Neither he nor his mother had ever been out of the UK, and his disease was probably transmitted by blood transfusion. Kala-azar should be considered in patients with unexplained fever and hepatosplenomegaly, even if there is no history of foreign travel.

Blood Transfusion↗
PubMed1994

Cognitive function and behavioural status in paediatric heart and heart-lung transplant recipients: the Harefield experience.

OBJECTIVE: To assess the psychological impact of cardiac and cardiopulmonary transplantation on children. DESIGN: Retrospective cross sectional study. SETTING: One British centre performing paediatric heart and heart-lung transplant operations, four cardiac units in London, three London schools, two London health centres, and the dental department of a London children's hospital. SUBJECTS: 65 children who had been given heart or heart-lung transplants and two reference groups of 52 children who had had other types of cardiac surgery and 45 healthy children. MAIN OUTCOME MEASURES: Development, cognition, and behaviour at home and at school as assessed by measures with proved validity and reliability. RESULTS: Developmental and cognitive measures indicated that children given transplants had significantly lower scores on several parameters, particularly in terms of development in children under 4 1/2 years of age. Performance on all tests, however, was within the normal range. There were no significant differences in behavioural ratings between the transplant and reference groups, though problem behaviour at home was more prevalent in the transplant group. CONCLUSIONS: Though cognitive development may be within the normal range, there are adverse psychological effects associated with cardiac and cardiopulmonary transplantation. These data indicate the need for a controlled prospective study in which children and their families are seen before and at regular intervals after transplantation. Interventions should be developed that are tailored to the particular needs of this very specialised group of paediatric patients and their families.

Achievement↗
PubMed1994

Influence of anatomic correction for transposition of the great arteries on myocardial perfusion: radionuclide imaging with technetium-99m 2-methoxy isobutyl isonitrile.

OBJECTIVES: We sought to determine the incidence of late perfusion defects attributable to coronary artery mobilization in patients undergoing anatomic correction for complete transposition of the great arteries. BACKGROUND: Anatomic correction (arterial switch procedure) is currently the surgical treatment of choice for complete transposition. From its conception, there has been concern about the impact on myocardial perfusion of the coronary artery mobilization and reimplantation involved in the correction. Previous studies have demonstrated myocardial perfusion defects in patients after correction, although a causal relation between coronary mobilization, and perfusion abnormality has not been established. METHODS: In a case-comparison study designed to test this hypothesis, 29 children underwent imaging with technetium-99m 2-methoxy isobutyl isonitrile (technetium-99m mibi). Ten had undergone anatomic correction (arterial switch group; interval from operation 6.9 +/- 1.42 years [range 4.9 to 9.1]); 9 had required noncoronary open heart surgery for other cardiac lesions (post-bypass group; interval from operation 5.6 +/- 3.6 years [range 1.0 to 13.25]); and 10 had had no surgical procedure (control group). The latter group comprised children with atrial or ventricular septal defects who required a radionuclide study for shunt calculation. Planar studies were performed in all 29 children, and additional tomographic acquisition was achieved in 25. To assess reversibility of perfusion defects both an exercise and a rest planar study were performed in the arterial switch group. RESULTS: Perfusion abnormalities were observed in seven of the nine children in the postbypass group and in all 10 children in the arterial switch group. The frequency of perfusion defects in these two groups was similar, with at least 25% of the tomographic segments reported being abnormal. The control group had significantly fewer defects than the other two groups (p = 0.02), with only 8% of the tomographic segments judged to be abnormal. In all except one patient in the arterial switch group, the segments reported as abnormal on the planar exercise study were either abnormal or equivocal on the rest study, indicating a fixed abnormality. CONCLUSIONS: Although the precise etiology of these perfusion abnormalities cannot be defined from this study, these data suggest that their origin is related more to the insult of open heart surgery itself than to the coronary manipulation involved in the arterial switch procedure. The functional importance requires further study.

Cardiac Surgical Procedures↗
PubMed1993

Long-term growth following neonatal anatomic repair of transposition of the great arteries.

Despite generally normal prenatal growth, surviving infants with transposition of the great arteries (TGA) frequently develop severe and progressive growth impairment which is not always fully reversed by elective atrial repair within the first year of life. This study was undertaken to determine the effect of neonatal anatomic repair of TGA on long-term growth. Twenty-three children with uncomplicated TGA were followed for a mean of 60 (12-90) months after anatomic repair at a mean age of 11 (1-40) days. Standardized measurements of weight, height, and head circumference for both patients and normal siblings were expressed as percentiles as well as in Z scores (in standard deviations from the mean for age and sex) based on internationally recognized standards. At latest follow-up, 22 (96%) of the patients were above the 3rd percentile for weight and 21 (91%) for both height and head circumference, with 13 (57%), 11 (48%), and 13 (57%) above the 50th percentile for each respective parameter. The mean Z scores (+/- SD) for weight, height, and head circumference for the patient group were -0.1 +/- 1.2, -0.2 +/- 1.3, and -0.1 +/- 1.1, respectively, and did not differ significantly from those of the reference population (p > 0.05 for each comparison). Paired comparisons of mean Z scores for each growth parameter with those of 35 normal siblings demonstrated no significant difference for weight or height and a small but significant difference for head circumference. Age at surgical repair (within the first 6 weeks of life), duration of follow-up and the development of moderate supravalvar pulmonary stenosis were not statistically related to long-term growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Birth Weight↗
PubMed1992

Intermediate-term results of heart-lung transplantation for cystic fibrosis.

Between September, 1984, and March, 1991, 79 patients underwent heart-lung transplantation for end-stage cystic fibrosis at the Harefield Hospital. Short-term outcome has already been reported, and we now present intermediate-term results. The overall actuarial patient survival was 69% at 1 year, 52% at 2 years, and 49% at 3 years. 17 patients had diabetes mellitus with a survival of 62% to 1 year and 51% to 2 years. 23 patients had one or more other possible high-risk factors, and survival of these patients was 64% at 1 year and 57% at 2 years, compared with 71% and 49%, respectively, in the low-risk group (n = 56). Pseudomonas aeruginosa infection was the most common respiratory infection encountered postoperatively. 92% of patients had at least one episode of acute rejection during the first 3 postoperative months. Lung function was greatly improved after transplantation, the mean forced expiratory volume in 1 s and forced vital capacity increasing from 22% and 35% predicted, respectively, preoperatively to 68% and 70% predicted, respectively, by the sixth postoperative month. This improvement was maintained at 1, 2, and 3 years after transplantation. Lymphoproliferative disorders (4 patients) were successfully treated. Obliterative bronchiolitis developed in 17 patients and the cumulative probability of getting this complication at 1, 2, and 3 years postoperatively was 17%, 23%, and 48%, respectively. Overall, 7 patients were retransplanted. There was no coronary artery disease in the 37 patients who underwent coronary angiography at 1 year, 14 at 2 years, and 9 at 3 years after surgery. 58 patients donated their hearts for subsequent "domino" heart transplantation. Our 5 1/2-year experience with heart-lung transplantation is encouraging but the shortage of donor organs and the complication of obliterative bronchiolitis are the two main obstacles to be overcome.

Adolescent↗
PubMed1992

Effect of cardiac or heart-lung transplantation on the quality of life of the paediatric patient.

Despite the increase in the use of heart and heart-lung transplantation as methods of treatment for children with end stage heart or lung disease, there is little documented research about the psychological implications of such procedures or about the effects of transplantation on quality of life. Twenty-eight children were studied before and 3 months after heart or heart-lung transplantation and compared with 28 normal children. Developmental and cognitive function were within the normal range, although performance was at a significantly lower level on a number of parameters compared with the normal group. There were no significant changes in any of the developmental or cognitive parameters after transplantation. Pre-operatively the prevalence of problem behaviour at home was significantly higher in the transplant group compared with the normal group, but there was a significant reduction in the prevalence of problem behaviour following transplantation. Early postoperative findings indicate an improvement in quality of life after heart or heart-lung transplantation but longer term follow-up is now necessary.

Adolescent↗
PubMed1992

[Treatment of dilated cardiomyopathy in childhood].

Authors reviewed available information concerning etiology and pathophysiology of dilated cardiomyopathy. Diagnostic criteria and current poor results of conservative treatment are discussed. Between August 1984 and August 1991 67 children with various types of cardiomyopathy underwent heart and heart and lung transplantation at Harefield Hospital, U.K. Dilated cardiomyopathy was the indication for transplantation in 47 patients (mean age at operation was 6.5 years, range 0.3-4.8 years). Eighty-one percent actuarial survival at 4 years after operation represents reasonable progress in the treatment of this severe acquired disease. Currently, early transplantation is recommended in children with dilated cardiomyopathy and with low shortening fraction of left ventricle (below 0.13), without any improvement after 3 months of conservative treatment, will familial trait of dilated cardiomyopathy and/or with severe myocardial fibrosis.

Cardiomyopathy, Dilated↗