Biomedical subjects
Publications and source records attributed to A Benatar.
A fetus with intrapericardial teratoma had a large pericardial effusion diagnosed by echocardiography. Cardiac tamponade, a common cause of fetal death, was averted by intrauterine pericardiocentesis. Planned obstetric and neonatal management permitted surgical treatment before cardiorespiratory distress developed. The tumor was completely excised and the infant is asymptomatic.
The long-term complications of patch plasty repair for coarctation of the aorta were assessed retrospectively in 119 patients who were operated upon from 4 days to 13 years of age. There were 7 late deaths and 17 patients were lost to follow-up. Thus 95 patients were followed up for a minimum period of 3 years (mean 6.3 years). In addition, graded exercise tests were performed on 15 patients and 11 normal controls, measuring systolic blood pressure response and arm-leg blood pressure gradients. In this series, 16 patients (17%) were hypertensive, while 25 (26%) had a resting systolic arm-leg gradient greater than 20 mm Hg. Re-coarctation occurred more frequently when surgery had been undertaken under 1 month of age. Review of chest X-rays revealed calcification in the patch in 4 patients, one of whom subsequently went on to aneurysm formation. In a second child aneurysm formation was detected at repeat operation. Exercise tests showed a significantly higher systolic arm pressure in patients (mean: 165.3 mm Hg) when compared to controls (mean: 139.2 mm Hg) (P = 0.017) and a significant increase in arm-leg systolic gradient viz. 36 and 5.9 mm Hg, respectively (P = 0.0016). A good correlation was found between the systolic arm pressure and the systolic arm-leg gradient after exercise (r = 0.822; P = 0.0001). We conclude that the most important long-term complication following an aortic patch plasty, is re-stenosis. The development of an aneurysm was observed only twice. We believe that this apparently rare occurrence, as assessed on chest radiographs; is related to the young age (53% under 1 year) at which the repairs were carried out. Another possible reason may be that the follow-up period is not yet long enough.
An unusual case of posterior transposition of the great arteries, with previously undescribed aortic obstruction is described. The patient, who underwent an arterial switch operation, was found at surgery to have a mass of myxomatous tissue arising from the tricuspid valve. This finding was responsible for the hemodynamically significant aortic stenosis.
Interruption of the aortic arch (IAA) is a rare but lethal congenital cardiovascular anomaly. Over a 3-month period we encountered 5 neonates with IAA, all of whom presented in the 1st week of life with congestive cardiac failure and diminished or absent pulses. All 5 underwent surgery, and 3 died from causes unrelated to the surgical repair. The diagnosis of this condition is difficult to make, although the absence of pulses may provide an important clinical clue (particularly if the left brachial and femoral pulses are involved). Using prostaglandins to re-establish ductal patency may be life-saving in these patients, since this restores blood flow to the lower trunk and kidneys. Administration of oral prostaglandin E2 to infants in whom this anomaly is suspected before they are referred to a tertiary care centre is therefore vital.
Immunological functions were investigated in 10 children with acute rheumatic fever and 11 children with acute nephritis to try and elucidate the cause of heart damage in acute rheumatic fever. Children with acute rheumatic fever and carditis showed an increase in serum IgG, IgA and antistreptococcal antibodies during the acute stage. Lymphocyte transformation responses to phytohaemagglutinin and streptococcal antigens were reduced but this was due to a serum suppressor effect. After recovering from acute rheumatic fever a lymphocytosis and an increased lymphocyte blastogenic response to streptococcal antigen were found. T-cells, T-helper cells and T-suppressor cells showed some changes in acute rheumatic fever but these were not statistically significant in our study. None of the changes in immunological responses that were seen in acute rheumatic fever were found in acute nephritis. These results support the hypothesis that an abnormal immune response to streptococcal products is involved in the development of carditis and the other phenomena observed in acute rheumatic fever.
Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.
The management of and results of treatment in 57 children with coarctation of the aorta are presented. In this series, 75% of the patients presented in the 1st year of life and 51% presented as neonates. Among the 50 patients who underwent surgery the total surgical mortality rate was 14%. The higher mortality in young infants is closely related to associated cardiac anomalies. We suggest that neonates with coarctation presenting with heart failure should be operated on early, as this significantly reduces the mortality.
A patient with tuberculosis and polyarthritis is described. The investigations and rapid resolution of the arthritis in response to therapy suggested the diagnosis of Poncet 's disease. The distinction between Poncet 's disease and tuberculous arthritis is prognostically important.
Syncopal episodes in 5 children have been shown to be caused by the prolonged QT syndrome. The clinical features, pathogenesis and treatment of this syndrome are discussed, and the poor prognosis without treatment is stressed. The prolonged QT syndrome should be considered and an ECG performed in all cases of recurrent unexplained syncope in the paediatric age group.
Four children are described, (three black and one white, two boys and two girls) with type A postaxial polydactyly. All four of them, in addition, had either a partial or complete atrioventricular septal defect (AVSD). None of these children had associated major malformations. Minor anomalies were observed (e.g., two patients with hypersegmentation of the sternal segments, one patient with undescended testes, one patient with hypoplastic lumbar vertebra, and one patient with a degree of craniofacial abnormality). Chromosome analysis was carried out for three of the four patients, and was normal in all of them. It is suggested that there is a specific association between type A postaxial polydactyly and the AVSD found in each of these patients. This picture does not conform to, but bears some resemblance to, the Ellis-van Creveld syndrome.