Biomedical subjects
Publications and source records attributed to T H Goh.
Williams Syndrome (WS) is a condition with multisystemic involvement caused by a genetic deletion in chromosome 7. Colonic diverticulosis has been described in adults with WS; however, it has not previously been reported in adolescents with WS. We report an adolescent boy with WS who developed complicated colonic diverticulitis and briefly review the possible aetiology of diverticular disease.
OBJECTIVE: To compare surgical and device closure of isolated secundum atrial septal defect (ASD) in terms of hospital costs, clinical outcome, and impact on the patient and family. DESIGN: Prospective, observational study. SETTING: Paediatric tertiary referral centre. PATIENTS: Consecutive local children with a secundum ASD, admitted between 1 May 1999 and 1 May 2001. METHODS: Parents completed a standardised questionnaire at recruitment (on admission), at discharge, and one month after the procedure. Clinical and hospital generated cost data were collated at discharge. RESULTS: 62 children were included in the analysis: 19 who underwent surgical repair and 43 who underwent device closure with the Amplatzer septal occluder. Median procedure times and hospital stay were significantly longer for surgical patients (170 (147 to 180) v 92 (70 to 115) minutes and 88 (78 to 112) v 29 (28 to 30) hours, respectively; p < 0.01). There was no difference in the complication rate. No device patients required intensive care or blood products. The median values for postoperative pain score, analgesia use, and convalescence time were greater for surgical patients. The median cost of each procedure was similar, but higher nursing and laboratory costs contributed to a slightly greater total cost for surgical repair (Aus$12 969 ($11 569 to $14 215) v Aus$11 845 ($10 669 to $12 555), p = 0.03). CONCLUSIONS: Device closure of ASD involves a shorter hospital stay, causes less discomfort and familial disturbance, and carries less cost than surgical closure. However, there should be guarded acceptance of this technique until long term data are available.
A patient with scimitar syndrome and dual drainage of a right-sided scimitar vein into the inferior vena cava and the left atrium underwent coil occlusion of the right aortopulmonary collateral artery and device occlusion of the lower scimitar vein drainage, leaving it flowing solely into the left atrium.
BACKGROUND: Congenital heart disease forms the largest group of congenital anomalies and therapy is available for all conditions. The main challenge is the early detection and referral of such cases. OBJECTIVE: The important features of the commonest congenital heart defects are reviewed with an emphasis on the diagnostic features. The pivotal role of the general practitioner in the early detection and referral of these patients is emphasised. DISCUSSION: A high index of suspicion, general understanding of transitional changes, haemodynamic aspects, good listening skills and examination techniques with the appropriate use of investigative tools, especially echocardiography will enable the practitioner to have a higher success with early detection and screening of patients. Rapport with institutions and parents is of great importance.
A 4-month-old girl presented with 2 weeks of symptoms and physical signs of heart failure. Echocardiography demonstrated marked left ventricular dilation, thinning of the myocardium with anterolateral akinesis, mitral regurgitation, a moderate pericardial collection, and an anomalous left coronary artery from the pulmonary artery. At operation there was a tense hemopericardium and a site of imminent rupture through a transmural anterior infarction. The anomalous artery was reimplanted in the ascending aorta, and an extensive infarct resection and ventricular repair performed. Support with a left ventricular assist device was required for 3 days, but the infant subsequently made a satisfactory recovery. Left ventricular rupture is a very rare complication of this lesion, but should be considered if there is evidence of a pericardial collection.
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Device closure of oval fossa atrial septal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The stretched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14+/-5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrieved at the same procedure. One of these patients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not related to the cardiac problems. There was no procedure-related morbidity or mortality and all patients remain well at the present time.
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.
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.
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.
Out of 14,841 women who were serologically examined for syphilis at the antenatal booking clinic, University Hospital, Kuala Lumpur in Malaysia, 1.78% were VDRL positive and 1.05% TPHA positive with significant differences between the Malays, Indians and Chinese. These rates are higher than published series and were attributed to childhood yaws infection among the Malays. As differentiation between yaws and syphilitic infection in the clinic is difficult, all TPHA-reactive women were treated as for syphilis. Congenital syphilis was not diagnosed in those women who had been effectively treated before delivery.
Six cases of primary cardiac tumour have been operated upon in a 7 year period from 1 June 1979 until 1 June 1986. All patients were under 6 months of age at the time of operation and two of the patients were in their first week of life. The principal indication for surgery was obstruction mainly at the right or left ventricular outflow tract level. More recently echo evaluation alone has been adequate to define the problem prior to surgery. Surgical excision has been performed without mortality or significant complications. In most cases resection has been complete, although in one case residual tumour has been left because of attachment of the tumour to vital structures. Follow-up of this case has not resulted in further surgery being required because of regrowth of the tumour. In one case, with co-existent congenital heart disease, the tumour was brought to notice after palliative systemic to pulmonary artery shunt had been performed. From the cardiac view point gratifying results have been obtained both in the short and long term following surgical resection. However, for patients with rhabdomyoma, later development of symptomatic tuberosclerosis should be anticipated in 50% of cases.
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The rate of copper loss from 329 IUDs (Multiload Cu250, TCu-220C and Cu7) which had been terminated for various reasons after 0.5-46 months in-utero were determined. All 3 IUDs showed a wide scatter of release rates with no significant differences between the ML Cu250 and the Cu7. The TCu-220C showed the highest rates and in contrast to the ML Cu250 and Cu7, the decrease with time was not significant. Its rate of copper loss was significantly higher than the ML Cu250 in the 1st year, but comparable after that. The TCu-220C had a similar rate to the Cu7 in the 1st year and significantly higher rates subsequently. The relevance of these findings to observed pregnancy rates and area of copper on the IUDs is discussed.
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Angiographic criteria for the recognition of aortic valve prolapse in isolated ventricular septal defect were based on the degree of aortic cusp deformity and the presence or absence of aortic regurgitation. Ninety eight consecutive patients with isolated perimembranous or infundibular ventricular septal defects who were catheterised and had aortography performed were reviewed. They included five with postoperative ventricular septal defects and three with additional mild right ventricular outflow tract obstruction. Eighteen were found to have aortic valve prolapse. Although eight of the 18 were noted to have aortic regurgitation angiographically, only three had an early diastolic murmur. Only eight of the 18 patients had cross sectional echocardiographic findings suggestive of prolapse. All of these had at least moderate prolapse angiographically. Cross sectional echocardiography was found to be insensitive in diagnosing mild degrees of aortic valve prolapse. A trend towards a decreasing left to right shunt was noted as the degree of aortic valve prolapse increased. Spontaneous decrease in the size of a ventricular septal defect may be due to unrecognised aortic valve prolapse without clinical evidence of aortic regurgitation.
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Two cases of congestive cardiomyopathy were proved to be due to chronic atrial automatic-focus tachycardia. Surgical elimination of the tachycardia resulted in normalization of cardiac function in both cases. We suggest that patients with congestive cardiomyopathy be carefully screened for supraventricular tachycardia. Patients with chronic supraventricular tachycardia should be treated aggressively to prevent myocardial dysfunction.