Biomedical subjects
Publications and source records attributed to J L Wilkinson.
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.
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.
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.
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.
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.
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.
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)
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The angiographic and haemodynamic findings in 2 cases of Pompe's disease (glycogenosis type II) indicated an abnormal trabecular pattern, not previously reported, on the left ventricular angiogram of both patients. This feature may be helpful in distinguishing Pompe's disease from other forms of myocardial abnormality.
Two pathological specimens are described in which a bizarre distortion of atrioventricular and ventriculoarterial relations existed. In both cases anterior and leftward displacement of the tricuspid valve coexisted with rightward displacement of the infundibular regions and semilunar valves. Abnormal expansion of the AV canal, possibly secondary to malrotation of the cardiac tube, is considered as a possible cause of the anomaly. One of the hearts showed severe subpulmonary stenosis produced by a valve-like structure which was well differentiated, with a complete annulus and chordae tendineae that inserted in a parachute-like fashion on the infundibular septum. The morphology of this structure was clearly different from that fo the fibrous tags described in other anomalies. Its origin is uncertain. These anomalies have important implications in relation to the nomenclature and classification of congenital heart malformations, and illustrate the value of recent modifications of the segmental approach.
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Two cases are described of a most unusual variant of two-chambered right ventricle. In both the ventricular septal defect was between the distal chamber of the right ventricle and the left ventricle. However the extensive dividing 'septum' between proximal and distal parts of the right ventricle converted the latter, haemodynamically, into part of the left ventricle. In the first case the distal chamber supported the aorta in the left anterior position, the pulmonary artery arising from the proximal part of the right ventricle. In the second the pulmonary artery arose from the distal chamber and the aorta from the proximal chamber. Though in both the ventriculoarterial connection was double outlet right ventricle, functionally there was arterial concordance in case 1 and discordance in case 2. A further disconcerting feature was the resemblance of the distal right ventricular chamber to the rudimentary chamber of a univentricular heart of left ventricular type.
Three hearts are described in which a fibrous membrane was interposed between the right atrium and a formed but hypoplastic right ventricle, which possessed recognisable inlet, trabecular, and infundibular components. In these hearts the distribution of the conducting tissue was as expected for concordant atrioventricular connections, and contrasts with that seen in the classical type of 'tricuspid atresia'. The distinctive morphological and histological features of these specimens lend further support to our view that the majority of cases of atresia of the right atrioventricular orifice should be regarded as coming within the designation of 'the univentricular heart'.
Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.
Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved possible to subdivide the hearts, depending on the morphology of the valve leaflets. Five leaflets were distinguished by the commissural pattern and their insertion to major papillary muscles. They were a posterior bridging leaflet, right and left lateral leaflets, and right and left anterior leaflets. Subdivision was made on the basis of the disposition of the anterior leaflets. In six hearts the left anterior leaflet was committed to the left ventricle and the right anterior leaflet to the right ventricle, the commissure between them being on the crest of the ventricular septum. In 39 hearts there was minimal bridging of the left anterior leaflet so that it extended between the anterior papillary muscle of the left ventricle and the medial papillary complex of the right ventricle. In eight hearts the right margin of the left anterior leaflet was attached to an apical papillary muscle, while in 17 hearts it was attached to the anterolateral papillary muscle of the right ventricle. As the bridging of the left anterior leaflet increased, so the size of the right anterior leaflet decreased, but in all hearts both leaflets were identified. These findings were compared with previous classifications of complete atrioventricular defects.
Seventeen cases are described in which both atria connect directly to a chamber with right ventricular characteristics. The atria connected through separate atrioventricular valves in six hearts and a common valve in 11. All hearts had a posterior rudimentary chamber. The septum which separated it from the main chamber was directed to the crux of the heart. Ten hearts were from patients with atrial situs solitus and seven from patients with atrial situs ambigous. Arterial connections were concordant in three cases, had a double outlet from the main ventricular chamber in nine and single outlet of the heart in five. The patent artery always arose from the main chamber, with pulmonary atresia in three and aortic atresia in two. This and other studies indicate that double inlet atrioventricular connection does not predict the morphology of the main chamber. Although usually associated with a main chamber of left ventricular type, it may also be associated with a main chamber having right ventricular characteristics. Both types should be considered as univentricular hearts; the posterior chamber in hearts of right ventricular type are analogous to the anterior chamber in univentricular hearts of left ventricular type and are a rudimentary chamber rather than a hypoplastic ventricle. In the right ventricular form of univentricular heart, the trabecular zone of the rudimentary chamber is of left ventricular type.
The cardiac specialized conduction tissue was studied by serial sectioning in 13 cases of situs ambiguus. In four cases of right isomerism, we found paired sinus nodes in relation to a crista terminalis, and in each case a sling of conduction tissue between two atrioventricular nodes was present regardless of the ventricular morphology or cardiac position. In the cases with left isomerism, the sinus node was hypoplastic and abnormally located. We saw two types of atrioventricular conduction systems. In the three cases in which the morphologically right ventricle lay to the right of the morphologically left ventricle (presumed d-loop), a single atrioventricular bundle arose from a normally located atrioventricular node. In the five cases in which the morphologically right ventricle lay to the left of the morphologically left ventricle (presumed l-loop) and in the one case with a univentricular heart, paired atrioventricular nodes were present, linked or potentially linked by a sling of conduction tissue.
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