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Biomedical subjects

J L Wilkinson

Publications and source records attributed to J L Wilkinson.

At least 37 records · Page 2Linked to original sources
PubMed1998

The long QT syndrome and seizures in childhood.

Children with the long QT syndrome (LQTS) are prone to life threatening ventricular arrhythmias. These arrhythmias may result in syncope and seizures that are often attributed incorrectly to a seizure disorder or to common fainting. The untreated mortality for symptomatic children with the LQTS is high but is improved significantly with therapy. Paediatricians should be aware of the presentations of the syndrome. Recommendations for screening for the syndrome are given.

Child↗
PubMed1998

Pulmonary valve annulus grows after balloon dilatation of neonatal critical pulmonary valve stenosis.

BACKGROUND: Neonates with critical pulmonary valve stenosis often demonstrate small or hypoplastic right ventricular structures. Relief of the obstruction enhances forward flow across the right ventricle and reduces its pressure load. Growth of the right ventricle and especially of the pulmonary valve annulus was evaluated after balloon dilatation. METHODS: Ten consecutive neonates with critical pulmonary valve stenosis who underwent balloon valvuloplasty were studied by serial echocardiography to assess growth of right ventricular structures at follow-up. RESULTS: The mean diameter of the pulmonary valve annulus increased from 6.1 +/- 1.4 mm to 12.6 +/- 3.5 mm (z scores from -2.9 +/- 1.0 SD to - 1.3 +/- 1.2 SD, p < 0.0001) after a mean follow-up period of 2.7 +/- 2.0 years. The mean diameter of the tricuspid valve annulus increased from 12.9 +/- 3.8 mm to 19.0 +/- 3.1 mm; however, the respective z score did not change significantly (from 0.5 +/- 2.4 SD to -0.5 +/- 1.0 SD). Right ventricular cavity size was hypoplastic in four patients initially and normal in all patients at latest follow-up. CONCLUSIONS: Balloon dilatation of critical pulmonary valve stenosis encourages catch-up growth of the pulmonary valve, and surgery may be avoided even in a hypoplastic pulmonary valve annulus.

Catheterization↗
PubMed1997

Anatomic features and surgical strategies in double-outlet right ventricle.

BACKGROUND: The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. METHODS AND RESULTS: Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.

Adolescent↗
PubMed1997

Gingival overgrowth in pediatric heart and heart-lung transplant recipients.

BACKGROUND: Gingival overgrowth is a recognized side effect of cyclosporine therapy with cosmetic and functional sequelae. This study examines the incidence and severity of gingival overgrowth in pediatric heart and heart-lung transplant recipients. METHODS: Thirty-one pediatric heart and heart-lung transplant recipients underwent a comprehensive dental evaluation. The severity of gingival overgrowth was scored by use of dental plaster casts. Parameters of dental hygiene in each patient included both a plaque index and a gingival inflammation index. The mean cyclosporine level and daily dose (mg/kg/day) at 3 and 12 months after transplantation and at latest follow-up were determined. RESULTS: The mean (+/- SD) patient age at transplantation was 10.5 (+/- 5.5) years, and the mean duration of posttransplantation follow-up was 3.2 (+/- 2.1) years. In all 30/31 (97%) of the cohort had some degree of overgrowth, with children aged less than 10 years at time of transplantation the most severely affected. By univariate analysis gingival overgrowth was inversely related to age at time of transplantation (r = -0.67, p < 0.001). With multiple regression analysis, only age at transplantation was significantly related to gingival overgrowth. CONCLUSIONS: Gingival overgrowth occurs in most pediatric heart and heart-lung transplant recipients treated with cyclosporine and is most severe in the younger patients. Attention to oral hygiene may improve gingival health parameters; however, the daily weight-adjusted dose of cyclosporine is not related to the severity of overgrowth.

Age Factors↗
PubMed1997

Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression.

BACKGROUND: The true incidence and prognosis of myocarditis in children with acute dilated cardiomyopathy (DCM) at presentation remains uncertain. This study examines the incidence of lymphocytic myocarditis in a consecutive cohort of children with acute DCM at presentation and outcome after dual therapy immunosuppression with cyclosporine and steroids. METHODS: Twenty-nine consecutive children with acute DCM underwent early endomyocardial biopsy. Children with "definite" myocarditis comprised group I (n = 9) and were treated with cyclosporine and prednisolone. Group II (n = 2) had "borderline" myocarditis, and group III (n = 18) nonspecific histologic findings. Outcome was assessed by echocardiographic measurement of left ventricular end-diastolic dimension and fractional shortening, with follow-up endomyocardial biopsy in group I subjects. RESULTS: Myocardial inflammation with or without myocardial necrosis (groups I and II) was present in 38% of all cases. There were no initial clinical, electrocardiographic, or echocardiographic features to distinguish patients in group I from patients in group III. At presentation, the mean +/- SEM left ventricular end-diastolic dimension and fractional score-Z scores of group I patients were 4.6 +/- 1.7 and -5.1 +/- 0.8, respectively, compared with 0.8 +/- 0.3 and -0.9 +/- 0.4, respectively, at withdrawal of immunosuppression (p < 0.001 for both). Both of these parameters did not differ significantly from normal controls at least follow up. Two group I patients had a biopsy-proven relapse after withdrawal of therapy that responded to reinstitution of immunosuppression. At latest follow-up, all nine group I patients had regained normal left ventricular function compared with four of 18 group III patients (p < 0.001). CONCLUSION: Lymphocytic myocarditis is frequent in children with dilated cardiomyopathy and cannot be predicted from noninvasive investigations. The use of cyclosporine and steroids is associated with a favorable outcome, and a controlled trial of dual therapy immunosuppression in children is therefore warranted.

Anti-Inflammatory Agents↗
PubMed1996

Treatment of critical pulmonary valve stenosis by balloon dilatation in the neonate.

Critical pulmonary valve stenosis represents an emergency, and immediate treatment is mandatory. The purpose of this study was to evaluate the immediate and medium-term results of pulmonary valve dilatation. We report 18 neonates in whom pulmonary valvuloplasty was attempted. The procedure could be accomplished in 14 patients. The angiographically determined diameters of the pulmonary and tricuspid valve at the time of procedure were 5.6 +/- 1.5 mm and 14.0 +/- 5.4 mm. The mean Doppler gradient decreased from 71 +/- 27 mm Hg to 27 +/- 14 mm Hg. Perforation of the right ventricular outflow tract was the major complication in three patients with one fatal event. Infusion of prostaglandin E1 could be discontinued 1 to 5 days after the procedure. On follow-up three children required a second balloon dilatation with good results. Seven patients monitored for more than 9 months with a mean follow-up time of 34.4 +/- 16 months had a residual gradient of 11.6 +/- 6.7 mm Hg. In spite of a hypoplastic pulmonary valve annulus in seven of the patients, results were good and surgery could be avoided.

Catheterization↗
PubMed1996

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis.

Early intervention is necessary in neonates with critical aortic stenosis. The advent of alternate therapy, particularly balloon aortic valvuloplasty, requires a reappraisal of the traditional surgical approach, including the efficacy of initial transvalvar gradient reduction and freedom from recurrence of obstruction in the longer term. This report describes a series of 33 consecutive infants who underwent surgical aortic valvotomy in the first month of life. The hospital mortality was 18 percent with a 5-year probability of survival of 66 percent (90 percent CI, 50 percent to 79 percent). Fourteen reinterventions, nine reoperations and five balloon dilatations, were required at a median age of 0.8 years (range 9 days to 6 years). Three patients died after reintervention (one early and two late). The median follow-up time was 5.8 years (range 0.2 to 14 years). At last follow-up Doppler investigation the average Doppler mean and peak gradients were 34 mm Hg and 18 mm Hg, respectively. Open valvotomy in neonatal aortic valve stenosis allows the use of an appropriate surgical approach with low initial mortality and satisfactory 5-year freedom from reintervention.

Aortic Valve Stenosis↗
PubMed1996

Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome.

OBJECTIVE: Aortic arch obstruction is a commonly associated problem in the Taussig-Bing anomaly. Between 1983 and 1995, 28 consecutive patients with Taussig-Bing anomaly underwent arterial switch operation with baffling of the left ventricle to neoaorta. Group A: 11/28 had associated aortic arch obstruction. Group B: 17/28 had isolated Taussig-Bing anomaly. We assessed whether the coexistence of subpulmonary ventricular septal defect and aortic arch obstruction pose an incremental risk factor. PATIENTS AND RESULTS: Group A: Mean age and weight were 1.4+/-1.3 months and 3.5+/-0.4 kg. The aortic arch obstruction included: hypoplasia (5/11), interruption (4/11) and discrete coarctation (2/11). Seven patients had a one-stage correction, and four had initial arch repair followed by arterial switch operation. There were no hospital deaths (CL 0-28%). Over a follow-up of 638 patient-months (mean 64+/-39), there have been no late deaths, and all patients are in New York Heart Association class 1. There have been three cases of recurrent aortic arch obstruction (two requiring reoperation, and one requiring balloon dilation). One patient has been reoperated upon for right ventricular outflow tract obstruction. The actuarial survival and freedom from reoperation rates at 6 years were 100% (CL = 66-100%) and 72.9% (CL=38-92%) respectively. Group B: Mean age and weight were 5.9+/-8.4 months and 5+/-2.1 kg. All patients had a one-stage operation. There were two early deaths (11.8%, CL = 1-36%) and one late death over a follow-up of 678 patient-months (mean 52+/-31). All survivors are in New York Heart Association class 1 and there have been no reoperations. The actuarial survival and freedom from reoperation rates at 6 years were 81% (CL = 56-93%) and 100% (CL = 76-100%) respectively. CONCLUSIONS: 1. Aortic arch obstruction has not adversely affected early or late survival (P>.05) or late functional class. 2. Patients with Taussig-Bing anomaly and aortic arch obstruction may have a higher reoperation rate than those with normal arch anatomy. 3. Taussing-Bing anomaly, with or without aortic arch obstruction, can be repaired with arterial switch operation during the neonatal period with good outcome.

Aged↗
PubMed1996

Left ventricular abnormalities in children, adolescents and young adults with renal disease.

The cardiac abnormalities that complicate chronic renal failure and renal replacement therapy are not well characterized in young people. These abnormalities are becoming more important because successful renal transplantation has resulted in children with end-stage renal failure living longer. Echocardiographic abnormalities of cardiac function and structure were studied in children and young adults (< 27 years old) with chronic renal failure (CRF, N = 32), end-stage renal failure treated with chronic peritoneal dialysis (CPD, N = 10) or renal transplantation (N = 30) or controls (N = 60). Left ventricular mass indexed for height (LVM/Ht and LVM/Ht2.7) and body surface area (LVM/SA), fractional shortening, measurement of left ventricular diastolic function (peak E and A wave velocities and the EA ratio) and structural (such as valvular) abnormalities were determined by echocardiography. The median (and range) of LVM/Ht in the groups were control 51.8 (23.1 to 119.8), CRF 60.2 (22.2 to 135.8), CPD 80.2 (14.5 to 100.9) and transplant group 97.8 (51.2 to 182.1) g/m. The increases in LVM/Ht, LVM/Ht2.7 and LVM/SA in the transplant group were significant (P < 0.01). The CRF group had significantly increased LVM/Ht2.7 and LVM/SA (P < 0.01). Systolic function was not significantly different between the groups. A significant correlation between creatinine and LVM indexed for height was found in the CRF group. Systolic or diastolic blood pressure could not be correlated with LVM indices in the transplant group. Changes in diastolic function were found (increased peak A wave velocity and decreased E/A ratios in the CRF and CPD groups, and increased peak E wave velocity in the transplant group). The study demonstrated that left ventricular hypertrophy is a frequent and often severe finding in children with chronic renal failure and those treated with renal replacement therapy. Factors other than hypertension and anaemia are important, and evidence was found for a link between serum creatinine and increased left ventricular mass prior to end-stage renal failure.

Adolescent↗
PubMed1996

Alveolar epithelial lining fluid cellularity, protein and endothelin-1 in children with congenital heart disease.

This study applied bronchoalveolar lavage (BAL) to children with congenital heart disease (CHD) prior to elective cardiac catheterization (n = 48), to determine the influence of pulmonary blood flow and viral infection on the alveolar epithelial lining fluid (ELF) concentration of leucocytes, protein and endothelin-1 (ET-1). Lower respiratory tract (LRT) viral infection was defined as either a positive immunofluorescence for virus, or a virus cultured from the bronchoalveolar lavage fluid (BALF). Haemodynamic status was determined at cardiac catheterization. Normative data for BALF, but not ELF parameters, were obtained from 26 asymptomatic, noninfected normal children undergoing elective surgery. In the absence of LRT infection, the BALF macrophage, lymphocyte and neutrophil differential in CHD was not significantly different from the normal controls. In CHD, both increased pulmonary-to-systemic flow ratio (Q'p/Q's) and increased pulmonary artery-to-left ventricular pressure ratio PAP/LVP were associated with a decrease in ELF protein (rs = -0.59; p < 0.0001; and rs = -0.50; p < 0.0001 respectively). A respiratory virus was isolated from the BALF in 8 (17%) of CHD children. Virus isolation was associated with an increased ELF total protein (p < 0.05 vs no infection), a decreased alveolar macrophage differential count (p < 0.01), and an increased neutrophil differential count (p < 0.05). ET-1 was detected in the BALF of 83% of the noninfected CHD children compared to only 23% of the controls (p < 0.001). ELF ET-1 concentrations did not correlate with haemodynamic status in CHD, but were up to 100 times higher than paired plasma levels. We conclude that, in congenital heart disease, both lower respiratory tract viral infection and increased pulmonary blood flow and/or pulmonary vascular pressure influence the alveolar milieu. High alveolar epithelial lining fluid concentrations of endothelin-1 occur in congenital heart disease, but the stimulus for pulmonary endothelin-1 production is unclear.

Bronchoalveolar Lavage Fluid↗
PubMed1995

Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance.

BACKGROUND: Limited information is available concerning long-term results, especially systemic right ventricular (RV) or tricuspid valvular function, after intracardiac repair of anomalies associated with discordant atrioventricular (AV) and ventriculoarterial (VA) connections ("congenitally corrected transposition of the great arteries"). METHODS AND RESULTS: We retrospectively reviewed the intermediate-term follow-up of 28 patients, totaling 158 patient-years (median, 60 months), after intracardiac repair involving closure of a ventricular septal defect (VSD) with or without additional surgery. Seven patients had VSD closure alone, 5 had VSD repair with pulmonary stenosis relief, and 16 had VSD closure with conduit insertion between left ventricle and main pulmonary artery. Hospital mortality was 4% (1 of 28 patients; 70% confidence limits, 0.07% to 12%) and the 1-, 5-, and 10-year actuarial survival probabilities were 89%, 83%, and 83%, respectively. Twenty-one of 24 long-term survivors were in New York Heart Association functional class I and 3 were in class II. Sixteen of 24 patients showed increasing tricuspid regurgitation (TR) of more than moderate degree, which occurred within 3 years after surgery in 7 patients. Twelve of 22 patients showed deterioration of RV pump function, mainly (9 of 12 patients) within 3 years postoperatively. The pulmonary to systemic flow ratio at the preoperative cardiac catheter study was significantly (P < .05) higher in patients who developed RV dysfunction (2.3 +/- 1.0, mean +/- SD) than in those with well-maintained RV function (1.4 +/- 0.6). CONCLUSION: Intermediate-term results of intracardiac repair for AV and VA discordance were satisfactory in terms of survival and clinical functional status; however, there is concern about systemic RV dysfunction with development of TR relatively early after the operation. Alternative surgical approaches such as anatomic correction or Fontan repair for cases unsuitable for biventricular repair may improve the long-term results, including ventricular and valvular function.

Adolescent↗
PubMed1995

Tooth morphology and archaeology: a problem solving exercise.

A knowledge of tooth morphology is often a valuable aid in forensic science but another less well-known application is the analysis of skeletal remains from archaeological sites, particularly when these involve children. This article describes one such case.

Adult↗
PubMed1995

Cardiac anatomy in patients undergoing the Fontan procedure.

BACKGROUND: Controversies in nomenclature, especially of hearts with "single ventricle," have clouded discussion and understanding of the anatomy. Many patients with such malformations are submitted to the Fontan procedure as definitive surgical palliation. The spectrum of anatomy among these patients is wide and deserves analysis in an effort to provide a simple framework for description and to eliminate confusion. METHODS: We reviewed 138 successive patients undergoing the Fontan operation at one institution to demonstrate the variability in pathology. RESULTS: Only 89 patients (65%) had a univentricular type of atrioventricular connection. All but 5 patients had two ventricular chambers. Among the 49 patients with biventricular atrioventricular connections, 43 had a hypoplastic ventricle that precluded a biventricular repair. CONCLUSIONS: Full understanding of the malformations that may preclude a "biventricular" repair and hence necessitate a Fontan procedure requires knowledge of the different forms of univentricular atrioventricular connection that, although usually associated with two ventricular chambers, are seldom amenable to a "two-pump repair." Understanding of those types of "biventricular heart" that preclude a two-pump repair (eg, severe hypoplasia of the left ventricle or the right ventricle) or are associated with high risks (eg, straddling atrioventricular valve) is also important.

Fontan Procedure↗
PubMed1995

Pulmonary artery size after bidirectional cavopulmonary connection.

Pulmonary arterial size was measured during cineangiography in 23 patients, 1.9 months before, and 14 months after bidirectional cavopulmonary connection (BCPC). Measurements were standardized for body surface area using the method of Nakata and co-workers (pulmonary artery index). There was a significant reduction in pulmonary artery index after BCPC. These data suggest that pulmonary arterial growth is impaired after the creation of a BCPC. This may be related to an absolute reduction in pulmonary arterial flow, and/or the loss of systolic expansion of the pulmonary artery.

Cardiac Catheterization↗
PubMed1993

Long-term follow-up after two coronary repair of anomalous left coronary artery from the pulmonary artery.

A retrospective analysis of ten patients with anomalous left coronary artery arising from the pulmonary artery operated between 1979 and 1990 was undertaken. All presented with evidence of left ventricular dysfunction and "ischemic" mitral regurgitation. Surgical repair consisted of an aortopulmonary tunnel (Takeuchi) procedure in eight and direct left coronary artery reimplantation in two. Two patients required postoperative support with a left ventricular assist device. There were no operative or late deaths (CL 0% to 17%) for a follow-up of over 670 patient months. All patients are in New York Heart Association Class I or II, though two patients are still receiving anticongestive medications. One patient has required further surgery for pulmonary artery stenosis, and another has had a mitral valve replacement because of severe mitral regurgitation. One additional patient has moderate-to-severe residual mitral regurgitation and two have a trivial left coronary to main pulmonary artery fistula. All have a patent, nonstenotic left coronary artery and much improved left ventricular function and perfusion as assessed by echocardiography, thallium scan, gated blood pool scan, and angiography. There have been no documented arrhythmias, clinically or on Holter monitoring. The ECGs have shown resolution or improvement of the initial changes of ischemia/infarction in all patients. Chest X-rays have shown normalization of cardiothoracic ratio in eight of ten patients. Excellent early and late results can be achieved following timely surgical repair. Marked improvement in left ventricular function has been observed in patients with poor preoperative left ventricular function, even in the presence of extensive ischemia/infarction.

Child, Preschool↗