Total anomalous pulmonary venous drainage to superior vena cava associated with preductal coarctation of aorta. Successful correction in a 12-day-old infant.
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Aorta↗
Biomedical subjects
M R de Leval
Publications and source records attributed to M R de Leval.
Clinical prolonged extracorporeal circulation for respiratory insufficiency: hematological effects.
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Adolescent↗
Heart transplantation in an infant with rhabdomyoma.
Rhabdomyoma is the most common primary cardiac tumor in infants and children and is often associated with tuberous sclerosis. Surgical resection may be indicated and, if so, is usually curative. We describe a rhabdomyoma in an infant who presented with severe myocardial ischemia necessitating orthotopic heart transplantation.
Diagnosis, Differential↗
Heart transplantation for Barth syndrome.
Barth syndrome is an X-linked recessive disorder comprising dilated cardiomyopathy, muscular hypotonia, and cyclical neutropenia. Affected children usually die during infancy as a consequence of septicemia, cardiac failure, or both. We report a patient with Barth syndrome who underwent successful heart transplantation.
Cardiomyopathies↗
The Fontan circulation: What have we learned? What to expect?
Our knowledge of the Fontan operation tends to indicate that it remains a palliative procedure for patients with a functionally single ventricle. There is a continuing attrition and the life expectancy of these patients is likely to be different from the life expectancy of a population of individuals having a biventricular circulation. This article is an essay on the rational approach for the future management of these patients. It is suggested that a better understanding of the continuing Fontan attrition and a more subtle way to predict outcomes of patients who do receive the Fontan could help in establishing better selection criteria and designing ways to prevent, delay, or treat the side-effects of this late attrition.
Collateral Circulation↗
Identification of systems failures in successful paediatric cardiac surgery.
Patient safety will benefit from an approach to human error that examines systemic causes, rather than blames individuals. This study describes a direct observation methodology, based on a threat and error model, prospectively to identify types and sources of systems failures in paediatric cardiac surgery. Of substantive interest were the range, frequency and types of failures that could be identified and whether minor failures could accumulate to form more serious events, as has been the case in other industries. Check lists, notes and video recordings were employed to observe 24 successful operations. A total of 366 failures were recorded. Coordination and communication problems, equipment problems, a relaxed safety culture, patient-related problems and perfusion-related problems were most frequent, with a smaller number of skill, knowledge and decision-making failures. Longer and more risky operations were likely to generate a greater number of minor failures than shorter and lower risk operations, and in seven higher-risk cases frequently occurring minor failures accumulated to threaten the safety of the patient. Non-technical errors were more prevalent than technical errors and task threats were the most prevalent systemic source of error. Adverse events in surgery are likely to be associated with a number of recurring and prospectively identifiable errors. These may be co-incident and cumulative human errors predisposed by threats embedded in the system, rather than due to individual incompetence or negligence. Prospectively identifying and reducing these recurrent failures would lead to improved surgical standards and enhanced patient safety.
Adolescent↗
Pseudoaneurysm of the aorta after heart-lung transplantation: diagnosis by color flow Doppler mapping.
Five months after heart-lung transplantation for treatment of end-stage cystic fibrosis, a 14-year-old girl had a swelling over the manubrium that was identified as a pseudoaneurysm at the aortic anastomotic site by means of cross-sectional echocardiography with color flow Doppler mapping. The diagnosis was confirmed at operation, despite which she died. Biopsy material taken during the operation revealed chronic sternal osteomyelitis caused by Pseudomonas aeruginosa. Disruption of the aortic anastomosis by infection may be a major complication of heart-lung transplantation for treatment of cystic fibrosis.
Adolescent↗
Surgery for congenital abnormalities of the mitral valve at the Hospital for Sick Children, London from 1969-1983.
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.
Actuarial Analysis↗