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Biomedical subjects

R Radley-Smith

Publications and source records attributed to R Radley-Smith.

At least 37 records · Page 2Linked to original sources

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↗

[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↗

An evaluation of right and left ventricular function after anatomical correction and intra-atrial repair operations for complete transposition of the great arteries.

Anatomical correction of complete transposition of the great arteries has the potential advantage over intra-atrial repair in that the left ventricle becomes the systemic pump. To investigate the importance of this, we evaluated right and left ventricular function in 21 patients after anatomical correction and in 21 patients after Mustard or Senning operations. First-pass and equilibrium-gated radionuclide angiography were used to measure right and left ventricular ejection fractions between 17 and 78 (mean, 47) months after anatomical correction and between 3 and 187 (mean, 67) months after intra-atrial repair. The mean age of the patient groups at the time of study was 52 and 84 months, respectively. The right ventricular ejection fraction ranged from 35% to 78% (mean, 58%) in patients after anatomical correction and from 27% to 68% (mean, 51%) after intra-atrial repair (p = 0.066). The left ventricular ejection fraction ranged from 39% to 74% (mean, 58%) after anatomical correction and from 35% to 74% (mean, 58%) after intra-atrial repair (p = 0.86). The mean right and left ventricular ejection fractions of both groups were significantly lower than those of normal children. Individuals with systemic ventricular dysfunction were identified after both types of operations; however, symptomatic dysfunction occurred only after intra-atrial repair (p = 0.24).

Cardiac Surgical Procedures↗

Cardiac transplantation in the seventh decade of life.

Twenty-five patients older than 60 years of age underwent cardiac transplantation using an immunosuppression protocol with cyclosporin and azathioprine, but without routine use of oral steroids. There were 24 men and 1 woman (age range 60 to 69 years, mean 63). The etiology of heart disease was coronary artery disease in 21 and idiopathic dilated cardiomyopathy in 4. Six patients had previous coronary artery bypass operations, 1 had undergone repair of an abdominal aneurysm and 1 had pulmonary embolism. Sixteen patients were in New York Heart Association class IV and 9 in class III. Donor mean age was 30 (14 to 46) years. Hospital stay after transplantation was 10 to 90 days (median 11). Four died within 30 days and none from 5 to 59 months (mean 22). The 1-year actuarial survival was 84%. The incidence of rejection was 2.16 episodes per patient. Only 1 patient (4%) had serious infection. Six patients received antihypertensive treatment, 3 had reversible impairment of renal function, 2 had gout and 1 had drop foot. No patient had convulsions, transient ischemic attack or cerebrovascular accident. None had significant psychological problems. The 21 patients currently alive are in New York Heart Association class I. Quality of life, assessed by the Nottingham Health Profile, showed marked improvement. It is concluded that the initial results of cardiac transplantation in the seventh decade of life are encouraging.

Actuarial Analysis↗

Primary cardiac tumours--is there a place for cardiac transplantation?

Between 1979 and 1985, seven patients (five children and two adults) were treated for primary cardiac tumours other than benign atrial myxomas. There were five malignant neoplasms (two non-classifiable sarcomas, one haemangiosarcoma, one histiocytoma and one neurofibrosarcoma) and two benign tumours (fibromas). Echocardiography, cardiac catheterisation, computed tomography and magnetic resonance imaging provided diagnostic confirmation. The two patients with fibroma are alive and well 4 and 5 years after radical resection of the tumours from the interventricular septum. The patient with a neurofibrosarcoma underwent orthotopic cardiac transplantation and is well 5.5 years postoperatively with no evidence of residual disease or recurrence. One patient died awaiting a donor heart for transplantation. Another patient who was a candidate for heart and lung transplantation was found to have an unresectable tumour at the time of operation. One patient with sarcoma who underwent a successful emergency partial resection for relief of cardiac tamponade died 18 months later from widespread metastases. The seventh patient was inoperable due to multiple secondaries. It is concluded that radical resection of large, benign, cardiac tumours can give good results and that early cardiac transplantation probably offers the only hope for patients with malignant tumours of the heart.

Adolescent↗

One-stage correction of interrupted aortic arch combined with heart-lung transplantation.

A technique for correction of interrupted aortic arch during heart-lung transplantation is described. This operation was performed in a 14-year-old patient with situs solitus, absent right atrioventricular connection, large atrial septal defect, ventricular septal defect, transposition of the great arteries, type B interrupted aortic arch, and severe pulmonary hypertension. The postoperative course was uneventful. Clinical, radiologic, and nuclear magnetic resonance investigation have shown evidence of good correction of the aortic arch anomaly. We concluded that donor tissue available during heart-lung transplantation is useful in correcting extracardiac anomalies.

Adolescent↗

A quantitative evaluation of aortic regurgitation after anatomic correction of transposition of the great arteries.

Twenty patients who had undergone anatomic correction of transposition of the great arteries were assessed by Doppler echocardiography or cardiac catheterization, or both, to identify the presence of aortic regurgitation. The severity of aortic regurgitation was evaluated by radionuclide angiographic measurement of the stroke volume index a mean of 47.1 months postoperatively. The stroke volume index was defined as the ratio of the stroke counts between the left and right ventricles. A value greater than 1.8 was considered to indicate significant left ventricular volume overload. Eight patients (40%) were shown to have various degrees of aortic regurgitation by Doppler echocardiography or cardiac catheterization, or both. The mean (+/- SD) stroke volume index was 1.03 +/- 0.15 in these patients and 1.01 +/- 0.21 in the 12 patients without aortic regurgitation (p = NS). The stroke volume index was not above the normal range in any patient, indicating that the degree of aortic regurgitation present was trivial. This medium-term study indicates that trivial or mild aortic regurgitation is a frequent finding after anatomic correction of transposition of the great arteries. However, it rarely results in an audible cardiac murmur or significant left ventricular volume overload. Long-term evaluation is required to determine its importance.

Aortic Valve Insufficiency↗

Pulmonary atresia and aortic valve stenosis.

A case is described of pulmonary atresia with ventricular septal defect and severe aortic valve stenosis. The aortic valve gradient measured at the time of insertion of an aortic to pulmonary arterial shunt was only 5 mm Hg and aortic valvotomy was deferred. Post-operative low output cardiac failure resulted in the death of the patient. The severity of the aortic stenosis was confirmed by post-mortem examination. We suggest consideration of aortic valvotomy in similar cases as the intraoperative pressure gradient may be unreliable.

Abnormalities, Multiple↗

Arrhythmias before and after anatomic correction of transposition of the great arteries.

Cardiac rhythm has been evaluated using 24 hour ambulatory electrocardiographic (ECG) recordings in 92 survivors of anatomic correction of transposition of the great arteries. A low incidence of arrhythmias was found in preoperative recordings made in 41 patients. Thirty-four (83%) patients showed sinus rhythm exclusively; one (2.4%) patient had supraventricular premature beats and one (2.4%) paroxysmal atrial fibrillation. Six patients (14.6%) had ventricular arrhythmias, five (12.2%) with modified Lown grade 1 ventricular premature beats and one (2.4%) with a modified Lown grade 5 arrhythmia. The incidence of arrhythmias was unchanged after a first stage operation consisting of banding of the pulmonary artery with or without insertion of a systemic to pulmonary shunt. During a mean 3.1 year follow-up, there have been no cases of arrhythmic death or symptomatic arrhythmias requiring treatment. Postoperative recordings in 40 patients have shown a low incidence of important arrhythmias with 62% having recordings free from arrhythmias. There was an increase in the incidence of infrequent supraventricular premature beats from 4.9% preoperatively to 23% postoperatively (p less than 0.05), with no significant change in the incidence of ventricular arrhythmias. One patient has acquired complete heart block but all others had sinus rhythm postoperatively. Preoperative arrhythmias are uncommon in patients with transposition of the great arteries and are similar to those described in normal children. The incidence of important arrhythmias after anatomic correction is low during the medium-term period of follow-up. Further longitudinal studies will be required to determine whether this promising result is maintained.

Age Factors↗

Lung biopsy findings in transposition of the great arteries with ventricular septal defect: potentially reversible pulmonary vascular disease is not always synonymous with operability.

Pulmonary vascular structure was analyzed in lung biopsy specimens taken from 28 children, aged 2 months to 15 years, with transposition of the great arteries and ventricular septal defect. Cellular intimal proliferation occurred in infants as young as 2 months, but it increased markedly between ages 7 to 9 and 10 to 12 months, and the increased obstruction was associated with a lower mean percent arterial medial thickness in patients older than 10 months than was found in younger patients. Early generalized arterial dilation appeared without the intimal fibrosis and dilation lesions characteristic of classical grade III and IV pulmonary vascular disease. Intimal abnormalities increased with age and pulmonary artery pressure, but mean percent arterial medial thickness was inversely related to mean pulmonary artery pressure (r = -0.5; p less than 0.0001). Nine patients survived intracardiac repair and six did not. Five of the patients who died were of similar age (12 months or less), had similar preoperative hemodynamic and pulmonary vascular abnormalities compared with the survivors and died after a clinical course compatible with pulmonary vascular disease. The findings emphasize that potential structural reversibility is not synonymous with "operability." Further studies are indicated on the function of the excessively muscularized pulmonary vascular bed of such infants.

Adolescent↗

Acquired supravalvar membranous stenosis of the left atrioventricular valve.

Acquired supravalvar membranous stenosis of the left atrioventricular valve developed in a nine year old boy with atrioventricular discordance. The supravalvar membrane had not been present when a partial annuloplasty was performed on the left atrioventricular valve eight years before. The stenosis was relieved by operative excision of the membrane.

Child↗

Familial persistent ductus arteriosus.

The occurrence of isolated persistent ductus arteriosus in three generations of one family with surgical correction is described. The high incidence of the malformation in this family suggests autosomal dominant inheritance rather than a polygenic mode of inheritance.

Adult↗

Computer assisted echocardiographic assessment of left ventricular function before and after anatomical correction of transposition of the great arteries.

Left ventricular function before and after anatomical correction of transposition of the great arteries was assessed by computer assisted analysis of 78 echocardiographs from 27 patients obtained one year before to five years after operation. Sixteen patients had simple transposition, and 11 had complex transposition with additional large ventricular septal defect. Immediately after correction mean shortening fraction fell from 46(9)% to 33(8)%. There was a corresponding drop in normalised peak shortening rate from 5.4(3.7) to 3.3(1.1) s-1 and normal septal motion was usually absent. Systolic shortening fraction increased with time after correction and left ventricular end diastolic diameter increased appropriately for age. The preoperative rate of free wall thickening was significantly higher in simple (5.6(2.8) s-1) and complex transposition (4.5(1.8) s-1) than in controls (2.9(0.8) s-1). After operation these values remained high in both the short and long term. Thus, computer assisted analysis of left ventricular dimensions and their rates of change before and after anatomical correction showed only slight postoperative changes which tended to become normal with time. Septal motion was commonly absent after operation. This was associated with an increase in the rate of posterior wall thickening that suggested normal ventricular function associated with an altered contraction pattern. Computer assisted echocardiographic analysis may be helpful in the long term assessment of ventricular function after operation for various heart abnormalities.

Child↗