Atypical development and behaviour in some non-verbal children.
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Biomedical subjects
Publications and source records attributed to J Stark.
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Unexpected arterial oxygen desaturation occurred immediately after cardiopulmonary bypass in two children. Epicardial echocardiography demonstrated unroofing of the coronary sinus after a Fontan procedure in the first child and rapidly excluded this abnormality in the second, who had undergone closure of a sinus venosus defect and in whom there was also a left superior vena cava draining to the coronary sinus. Epicardial echocardiography determined management in both patients: Oversewing of the ostium of the coronary sinus was undertaken in the first case during an additional period of cardiopulmonary bypass. Oxygen saturation values improved with aggressive endotracheal lavage and aspiration of secretions in the second child. Epicardial echocardiography can identify unroofing of the coronary sinus (which may be difficult to diagnose by transesophageal echocardiography) and can identify or exclude this condition when unexpected arterial oxygen desaturation is present after cardiac surgery.
Extracardiac valved conduits are routinely employed in the correction of complex congenital heart lesions. Compression of a conduit and/or of the heart may present serious operative and postoperative complications. We believe its occurrence can be minimized and hemodynamic results improved by adhering to simple surgical techniques. It is important to select a conduit which will produce minimal resistance to flow. Partial or subtotal thymectomy may increase the mediastinal space to accommodate the conduit. The conduit should be cut into the appropriate shape and should be placed in such a way that it does not cross the midline. Finally, the posterior pericardium may be opened to allow the heart to move posteriorly and to rotate to the left, thus moving the conduit away from the sternum.
Extracardiac valved conduits were introduced in 1966. Currently, both aortic and pulmonary homografts, preserved in antibiotic/nutrient solution or cryopreserved, are used. Conduits are implanted between the right ventricle and pulmonary artery, left ventricle and pulmonary artery, right atrium and right ventricle, and left atrium to left ventricle. Several factors can influence longevity of valved conduits: young age at implantation, small size of homograft, and immunological response. In the recent study from our department, we evaluated 405 homografts implanted between 1971 and 1993 in patients who survived 90 days after surgery. Freedom from conduit replacement at 5 and 15 years was 84% and 31% (95% confidence limits: 80-88% and 19-43%), respectively. In multivariate analysis, there were only two predictors of conduit longevity: (1) conduits used at reoperation lasted less well than those used at original operation; and (2) conduits used earlier in the series lasted longer. Conduits may have to be replaced because of obstruction, conduit valve regurgitation, aneurysm/pseudoaneurysm, and endocarditis. Obstructed conduits are either replaced or outflow tract patch is used after removal of the conduit with or without pulmonary valve implantation. Current mortality of conduit insertion is low (5-6%). The risk of conduit replacement has also decreased in recent years to 2-3.5%
The authors report the results of a March 1992 survey of 650 baccalaureate nursing education programs concerning the education that they provide in the area of death, dying, and bereavement. The survey inquired about death education content in the curriculum, theoretic models, and whether organ/tissue donation is addressed. Results indicate that many schools are using outdated models. The authors suggest ways to address these issues more adequately.
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One-hundred and eleven patients with tricuspid atresia were seen during 1972-1982 and thirteen of these were not operated upon. The remaining 98 patients have undergone at least one operation. Those with low pulmonary blood flow had 1-4 shunt procedures; in 15 patients with high pulmonary blood flow the pulmonary artery was banded. The mortality rate after the first shunt was 10% (8/82), 9% after the second shunt (3/32) and 12% after the third and fourth shunts. Three out of 15 (20%) patients died after pulmonary artery banding. Twenty three patients underwent a Fontan operation and five died (22%); eighteen survivors are clinically well (after 1-7 years). One patient required a pacemaker implantation and revision of his Fontan procedure. The survivors of the palliative operations and the unoperated patients were more restricted, the older ones having developed pulmonary vascular obstructive disease, chronic heart failure or arrhythmias.
The clinical course and outcome of 111 patients with tricuspid atresia, seen during 1972-1982, were reviewed. All clinical and investigation data, progress and management were analysed. The mean age at presentation was 21.9 +/- 8.8 days. Patients with reduced pulmonary blood flow presented earlier. Patients with normal or increased pulmonary blood flow usually presented in heart failure. "True" natural history was observed in 13 unoperated patients. Six of these died at the age of 7 days to 8 months (mean 32.2 days). Seven patients survived without surgery for 11 months to 24 years, the older ones developing pulmonary vascular obstructive disease. The overall mortality in the whole series was 32.4% (36/111), the mean age at death was 3.1 years (range 3 days-48 years). The mortality rate within the first year of life was 22.5%. The mean age of survivors at follow-up was 10.7 +/- 0.9 years (range 11 months-27 years).
The surgical management of congenital mitral valve (MV) anomalies remains controversial and complex. Valve repair has been said to be preferable to valve replacement. To assess the validity of this suggestion we have reviewed our experience of these procedures at The Hospital for Sick Children, Great Ormond Street, London from 1969-1983. In that time 48 patients have undergone surgery; in 23 repair was practicable (Group A) and in 25 replacement of the MV was required (Group B). Group A patients were slightly older than group B patients (5.9 +/- 1.0 years (SEM) vs 3.6 +/- 0.5 years). Björk Shiley valves were the predominant prosthesis used. Overall mortality was 33%, with 17% in Group A and 48% in Group B. Hospital mortality was 4.5% in Group A and 28% in Group B. Actuarial survival for Group A was 90% at 1 year, and 75% at 2 and 5 years; for Group B it was 52% at 1 and 2 years and 42% at 5 years. Mortality was significantly higher in those aged less than 5 years. Four patients in Group A have required re-operation, in 3 valve replacement was required; 2 of these died in hospital. One patient in Group B has required reoperation for valve-related thromboembolism. Three patients are awaiting replacement of calcified bioprostheses. These data support the concept that repair of MV should be performed where possible. The severity of the anomalies was greater in Group B and a rethink as to whether valve replacement is the best option for severe mitral stenosis in those aged less than 5 years seems indicated, in view of the very high mortality.
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Eleven of 32 patients (34%) with advanced ovarian cancer previously treated with conventional therapies had partial or complete responses to 5-day courses of cis-diamminedichloroplatinum(II) (CDDP) (20 mg/m2/day) with nine showing objective response prior to initiation of the second course. Median duration of response was 6 months (range, 2-18 +). Response to CDDP was not correlated with age, pathologic subtype, tumor grade, or bulk disease but was correlated with response to prior treatment. Eight of 15 patients previously responsive to either single alkylating agents or cyclophosphamide plus doxorubicin (Adriamycin) responded to CDDP. In contrast, response to CDDP was observed in only three of 17 patients showing no response to this prior chemotherapy. The highest response rate to CDDP was seen in that subset of patients sensitive to cyclophosphamide and doxorubicin (seven of ten patients).
A successful operative treatment of a three day old girl with transposition of the great arteries is described. Post-operative course was complicated by furosemide resistant anuria which was treated by peritoneal dialysis. At the age of eight months, she is a pink, active baby, developing normally. This case report demonstrates the importance of aggressive treatment of severe congenital heart lesions in the first few days of life. The importance of post-operative care is emphasized.