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Biomedical subjects

J L Wilkinson

Publications and source records attributed to J L Wilkinson.

At least 55 records · Page 3Linked to original sources

Transposition of the great arteries with intact ventricular septum. Arterial switch repair in patients 21 days of age or older.

Since 1985 we have performed arterial switch repair for infants with simple transposition of the great arteries (with intact or virtually intact ventricular septum) who were younger than 2 months of age. As of December 1990, a total of 118 arterial switch operations had been performed for transposition of the great arteries with intact ventricular septum. Although correction is usually performed in the first 14 days of life, 18 infants had operations at or after 21 days (range 21 to 118 days; mean 38 days). Fourteen of 18 patients had echocardiographic or catheter evidence (or both) of low left ventricular pressure at the time of operation. None of these patients had preliminary pulmonary artery banding. Mortality was 5.6% (1 of 18 patients, confidence limit 0.8% to 17%) for the older group versus 0% (0 of 100 patients; confidence limit, 0% to 1.8%) for patients less than 21 days of age. The single death occurred in a 22-day old, 1.8 kg premature baby, probably as a result of a problem with a coronary anastomosis. One patient required a left ventricular assist device for 2 days after the operation. These data suggest that with appropriate perioperative management and support, the arterial switch procedure can be safely carried out as a primary procedure for patients up to the age of 1 month and probably up to the age of 2 months.

Age Factors↗

Complete thoracic ectopia cordis with double-outlet right ventricle: neonatal repair.

A case of total thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect is presented. Prenatal diagnosis allowed single-stage correction immediately after birth. This approach proved to be technically feasible. Death occurred on the twelfth postoperative day owing to sepsis unrelated to the repair.

Abnormalities, Multiple↗

Cardiac conduction abnormalities and rhythm changes after neonatal anatomical correction of transposition of the great arteries.

Seventy three infants who underwent neonatal anatomical correction for transposition of the great arteries with or without a ventricular septal defect were reviewed for evidence of conduction and rhythm abnormalities on preoperative and postoperative 12 lead electrocardiograms and during 24 hour Holter monitoring. There was a partial right bundle branch block pattern in 47% (29/62) of all patients and in 60% (24/40) of those with simple transposition. Complete right bundle branch block was noted in 21% including 5% with simple transposition. Holter monitoring showed sinus rhythm in all patients except three: one had episodes of supraventricular tachycardia, another an intermittent second degree heart block, and a third a complete heart block. Atrial extrasystoles were noted in 47% (29/62) of patients but were frequent in only three patients. Occasional unifocal ventricular extrasystoles were encountered in 37% (23/62) of patients and were frequent in a further 3% (2/62). Only one patient (2%) developed multifocal ventricular extrasystoles. The frequency of important cardiac arrhythmias after neonatal anatomical correction of transposition of the great arteries was 5%, significantly less than that reported after atrial inflow diversion for the same malformation.

Arrhythmias, Cardiac↗

Very small pulmonary arteries: central end-to-side shunt.

Between 1980 and 1989, 28 patients with pulmonary atresia, ventricular septal defect, and very small pulmonary arteries with major aortopulmonary collateral arteries underwent direct central end-to-side shunts as staging procedures. Age range was 2 months to 32 years, with 19 patients less than 1 year of age. Pulmonary artery diameters ranged from 1 to 4 mm, with 24 less than 3 mm. Two patients (7%; 70% confidence limits, 2% to 16%) died after the shunt, and there were two further deaths after subsequent staging or correction. Acute shunt complications included congestive cardiac failure (mild to moderate, n = 8; severe, n = 3) and endocarditis (n = 1). Proximal right pulmonary artery stenoses have occurred in 75% of patients and left pulmonary artery stenoses, in 50%. Satisfactory pulmonary artery growth was achieved, however, in 16 of 24 hospital survivors investigated postoperatively. Twelve patients have proceeded through unifocalization to biventricular repair (in 2 the ventricular septal defect patch was subsequently fenestrated) with one death (8.3%; 70% confidence limits, 1% to 25%). Eight patients are still in staging, and 4 have been excluded from the program because of inadequate unifocalization. The direct central end-to-side shunt has proven satisfactory in attaining pulmonary artery growth in patients with very small central pulmonary arteries.

Adult↗

Familial fixed subaortic stenosis.

In this report, we give details of two families in which fixed subaortic stenosis was found in more than one member. It is rare for this entity to show familial incidence.

Adolescent↗

Discordant atrioventricular connexion with concordant ventriculo-arterial connexion (so-called "isolated ventricular inversion") with usual atrial arrangement (situs solitus).

The combination of a discordant atrioventricular connexion with a concordant ventriculo-arterial connexion (so-called "isolated ventricular inversion") is an extremely rare entity described, to the best of our knowledge, on only 15 occasions in patients with usual atrial arrangement. Initial diagnosis at cross-sectional echocardiography has only been reported three times, but the combination has been diagnosed in life on seven occasions, with successful Mustard or Senning operations having been performed in six of these patients. We report a neonate referred with an initial diagnosis of complete transposition, who was correctly diagnosed at cross-sectional echocardiography, and had closure of an associated ventricular septal defect with a Senning procedure in the neonatal period.

Crisscross Heart↗

Intraocular pressure and eye enlargement in chicks.

In chicks, the application of optical devices that blur vision results in eye enlargement and ametropia. The research presented here investigated changes in intraocular pressure (IOP), specifically intravitreal pressure, that occur with eye enlargement induced by this means. Plastic goggles (domes) were glued over one eye in 25 domestic chicks. Plastic rings were glued around one eye in 25 control chicks to control for the mechanical effects of the treatment but which left their vision undisturbed. Following two weeks of treatment, domes and rings were removed and IOP was measured. Mean IOP of the domed eyes was 1.14 mm Hg lower than that of control eyes, a difference that was not statistically significant. The eyes of an additional group of 20 chicks that experienced similar treatments were refracted; these refractions revealed that the dome-treated eyes had a mean refractive state that was 7.98 D myopic relative to the ring controls, which was statistically significant. The results indicate that IOP does not change significantly in chicks with experimental eye enlargement induced by retinal-image degradation.

Animals↗

Normal variations in the relationship of the tricuspid valve to the membranous septum in the human heart.

The anatomy of the antero-septal commissure of the tricuspid valve and its relationship to the membranous part of the septum are described in 72 normal hearts from subjects ranging in age from 25 weeks gestation to 13 months. Three major patterns were found. According to whether the contiguous leaflets were fused at the commissure, joined at the circumference of the orifice, or left a gap at the commissural site, the varieties are described as undivided, annular, or gap commissure, respectively. Commissures of annular type showed further morphological varieties, as did the patterns of division of the membranous septum into its atrioventricular and interventricular components. Dominance of the atrioventricular component and/or absence of the interventricular membranous septum were encountered in 38 cases out of 72 (52%). This finding demonstrates that dominance of the atrioventricular membranous septum during fetal life and infancy is not universal. The variability noted in the morphology and position of the antero-septal commissure was not associated with other positional variations of the tricuspid valve.

Fetus↗

Morphology of pulmonary atresia with intact ventricular septum in patients dying after operation.

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.

Aorta, Thoracic↗

The congenital long QT syndromes in childhood.

Twenty-three children and young persons with a congenital long QT syndrome were identified; the median age at the time of referral was 10 years (range 4 days to 19 years) and 14 patients (61%) had a family history of the syndrome. Among the 19 patients with symptoms, the initial symptom was syncope in 13 (69%), aborted sudden death in 5 (26%) and near drowning in 1 (5%). There were three deaths during a combined follow-up period of 67 patient-years (average annual mortality rate 4.5%). Patients who did not respond to therapy with a beta-adrenergic blocker and those who died were significantly younger than the remaining patients at the time of diagnosis (p less than or equal to 0.05 for both). Analysis of 44 treadmill exercise tests performed by 16 patients revealed significant prolongation of the median corrected QT (QTc) interval in response to exercise, with maximal prolongation present after 2 min of recovery (median QTc interval 0.52 s versus a baseline value of 0.47 s, p less than 0.001). Characteristic changes in T wave configuration were noted in 8 of 15 patients on at least one occasion during ambulatory Holter electrocardiographic monitoring, including T wave alternation in two patients, both of whom died shortly afterward. It is suggested that the congenital long QT syndrome is associated with a significant mortality rate in childhood despite the use of conventional therapy in symptomatic patients. Ambulatory Holter monitoring and treadmill exercise testing may be helpful, both in confirming the diagnosis of a long QT syndrome and in monitoring the adequacy of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenergic beta-Antagonists↗

Anomalous origin of the left coronary artery from the pulmonary trunk. Anatomic findings in relation to pathophysiology and surgical repair.

Anomalous origin of the left coronary artery from the pulmonary trunk, though a discrete anatomic malformation, manifests a spectrum of clinical and pathologic consequences. The objectives of this study were to characterize the primary anatomic findings in a group of specimens with anomalous left coronary artery and the extent of secondary morphologic and pathologic changes. Although the cases studied probably represent the least favorable end of the spectrum, the observed pathogenesis and evolution of secondary changes suggest that reconstruction of a two-coronary arterial system supplied through two coronary arteries would be advantageous to most patients. A high origin of the right coronary artery or location of the left coronary artery adjacent to a pulmonary cusp or branch may complicate the tunnel-type repair. In these cases, transfer of the left coronary artery to the aorta may be preferable.

Abnormalities, Multiple↗

The medial papillary muscle complex and its related septomarginal trabeculation. A normal anatomical study on human hearts.

The morphology of the medial papillary muscle complex of the right ventricle was studied in 81 normal hearts from subjects ranging in age from 20 weeks of gestation to 13 months. The position of the main medial papillary muscle (of Lancisi) was differentiated in terms of a root and a belly. The anterior aspect of the root was found in a constant position at the basal bifurcation of the septomarginal trabeculation, whereas its belly showed considerable positional variation. Three groups of minor papillary muscles were identified and localised in specific areas in the base of the right ventricle. Taken overall, they formed part of the medial papillary complex. The complex itself was found to be constantly related to the antero-septal commissure of the tricuspid valve. A separate group of septal papillary muscles could be differentiated from the medial papillary complex. The anchorage of the septal leaflet of the tricuspid valve to the ventricular septum was also studied. The antero-superior and postero-inferior portions of this leaflet were found to be supported by the medial and posterior papillary complexes, respectively. Its midportion was connected to the septal group of papillary muscles but much variability was evident. The portion of the septomarginal trabeculation supporting the medial papillary complex, namely the postero-basal division, was studied further. This showed considerable variability and did not form a continuous anatomical spectrum.

Fetus↗

Protein-losing enteropathy caused by baffle obstruction after Mustard's operation.

Three patients developed protein-losing enteropathy caused by intra-atrial obstruction of the systemic venous return after Mustard's operation. The enteropathy resolved in one case after reoperation and in the others after balloon dilatation of the stenosed caval pathways. Protein-losing enteropathy may occur as a complication of Mustard's operation. Balloon dilatation of the obstructed baffle is an effective alternative to reoperation.

Humans↗

Balloon dilatation of the pulmonary valve in the first year of life in patients with tetralogy of Fallot: a preliminary study.

Fifteen infants with tetralogy of Fallot, who would otherwise have required a palliative operation, underwent balloon dilatation of the right ventricular outflow tract. The mean period of palliation was 8.5 months (range 0-26 months). The procedure was performed without serious complications on 88% of occasions. This preliminary study suggests that balloon dilatation may be useful in the management of tetralogy of Fallot.

Catheterization↗