Pulmonary complications of oxygen therapy.

Three cases of the neonatal respiratory distress syndrome are described. The babies were treated by prolonged oxygen given by intermittent positive pressure respiration after endotracheal intubation. Two of the infants died and at necropsy the lungs in both cases were consolidated. Both showed widespread haemorrhagic exudate and interstitial oedema. Early organization of the exudate was apparent in one case. The third infant survived but developed a similar exudative condition and a ;honeycomb' lung. It is suggested that many of the changes found in the lungs of these cases could be attributed to the toxicity of oxygen.

The thalidomide syndrome: risks of exposure and spectrum of malformations.

Echos of the 1959 to 1961 thalidomide disaster are still with us in the form of the continuing appearance, although more rarely, of similarly malformed children. A teratologic timetable could be fashioned retrospectively, supporting the concept that different body parts and systems have periods of vulnerability and of resistance to thalidomide, probably the most potent primate teratogen known. Something between one in every two to one in every ten fetuses exposed at the critical development period were affected. The clinical affects are described from the literature on approximately 2500 children, and from extensive personal experience, and a complex though finite pattern of malformations is described, indistinguishable from sporadic nonthalidomide cases of malformations that continue to occur.

Teratogen update: clinical aspects of thalidomide embryopathy--a continuing preoccupation.

There is much misinformation in the medical community regarding the thalidomide syndrome. Some physicians and scientists are unaware of the fact that organs other than the limbs were frequently affected. Some believe that thalidomide could produce any type of limb reduction defect. Most were aware of the very narrow period of early organogenesis during which the thalidomide-type malformations could be produced. Important features include the fact that limb reduction defects were primarily preaxial, included concomitant girdle hypoplasia when limb reductions were severe, were almost universally bilateral and did not include distal transverse-type defects often called "hemimelia". While it can be said that some spontaneous (non-thalidomide) malformations can mimic the thalidomide syndrome, it can also be said that many limb reduction defects can be determined not to have been produced by thalidomide. The risks of the various defects can be estimated following exposure, with most certainty for limb defects, with less certainty for other defects. Many defects were not associated with exposure to thalidomide such as cleft lip and severe mental retardation.

Linear growth of children with limb deformities following exposure to thalidomide in utero.

The growth of 202 children exposed to thalidomide in utero and having upper (139 children) or lower (63 children) limb deformities has been assess towards the end of pre-pubertal growth. The analyses show that children exposed to thalidomide are shorter than normal children but grow at a normal velocity later. These findings may help in consideration of the mechanism by which thalidomide exerted its teratogenic effect. Analyses of growth may find a wider use in the retrospective assessment of drugs which are potentially harmful in pregnancy.

Subarachnoid cyst with hydrocephalus--a complication of mid-trimester amniocentesis.

We report a child with a subarachnoid cyst with hydrocephalus following a mid-trimester amniocentesis. Although fetal trauma is a rare complication, it is important to stress the need to perform amniocentesis under ultrasound control. Children with neurological disease or convulsions of unknown origin in early infancy who are born to mothers who have had mid-trimester amniocentesis should have a CT scan as part of the investigations to exclude this rare but nevertheless important complication.

Operative survival and 40 year follow up of surgical repair of aortic coarctation.

OBJECTIVE: To study early and late mortality after surgical correction of coarctation of the aorta. DESIGN: Data on 223 patients operated on at the Westminster Hospital, London, between 1946 and 1981, were collected and updated by questionnaire. PARTICIPANTS: All 223 patients recorded as undergoing operation for aortic coarctation up to the end of 1981. Fifteen of 197 survivors were lost to follow up; most of them were patients from overseas. OUTCOME AND RESULTS: The early mortality (within one month of operation) was 12% overall, 2.6% for elective surgery, and 0% for the 77 patients undergoing surgery since 1968. Survivors were followed up for a total of 3288 patient years; in 27 follow up lasted more than 30 years. In a few it reached 40 years. Twenty two patients died during this period, 18 from causes that could be attributed to coarctation or its repair. Mortality was highest more than 20 years after the operation. CONCLUSION: Repair increased life expectancy in patients with aortic coarctation. Late problems caused by persistent hypertension or recoarctation became apparent in long term survivors. The increased risk of late mortality associated with the duration of preoperative hypertension was not statistically significant. There were no deaths from cerebrovascular accidents. (In an earlier necropsy series cerebrovascular accidents accounted for 11.8% of deaths.) The incidence of deaths from aneurysms resembled that in the earlier necropsy series.

Coarctation of the aorta life and health 20-44 years after surgical repair.

OBJECTIVE: To examine the health and lifestyle of a group of patients who had repair of coarctation of the aorta 20-44 years ago (these were the first such operations in the United Kingdom) and to see how the results would influence current management strategies. DESIGN: Attempts were made to contact all patients by questionnaire. They were then requested to attend for a clinical examination. SETTING: Patients had their initial surgery at the Westminster Hospital (by Charles Drew) and the follow up examination at the same hospital. PATIENTS: 149 operations were performed. 70 of the 106 patients presumed to be alive were traced and 62 replied. 42 attended for examination. Only patients with the diagnosis of simple coarctation were included. Some patients had had coincidental ligation of a patent ductus arteriosus but none had any other cardiac abnormality requiring surgical or medical treatment. Those who died during the follow up period were described in paper by Bobby et al (Br Heart J 1991;65:271-6). MAIN OUTCOME MEASURES: Current symptoms and life situations, evidence of cardiac disease, further cardiac surgery, current and retrospective blood pressures, and Doppler echocardiographic examination. RESULTS: 29 (69%) had cardiovascular disorder. Doppler echocardiography did not show previously unrecognised major recoarctation. 19 (46%) had hypertension at follow up and there was evidence of enlargement of the aortic root or arch in seven (16%) patients, who tended to have had surgery at a later age. No evidence of cerebrovascular accident was found. CONCLUSIONS: In this group of patients with surgically repaired simple coarctation, late morbidity (particularly aortic aneurysm, aortic valve disease, and ischaemic heart disease) was common. The incidence of intracranial haemorrhage seemed to have been reduced by surgical repair. The integrity of the surgery remained good. Many patients did not have any regular cardiovascular review. Long-term anxiety related to early surgical experiences was evident. Even after apparently successful surgical repair of aortic coarctation. It would be prudent for all patients to have long-term review.

Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance.

BACKGROUND: We have shown previously that transient right ventricular restriction after tetralogy of Fallot repair prolongs postoperative course. This is a prospective study of right ventricular diastolic performance in late follow-up patients. METHODS AND RESULTS: We studied biventricular function, using Doppler echocardiographic examination. Pulmonary arterial, tricuspid, and mitral valves and superior vena cava Doppler spectrals were obtained in 41 patients (mean age, 28.8 years), 15 to 35 years (mean, 23.6) after complete repair of tetralogy of Fallot. Patients were considered to have evidence of right ventricular restriction if antegrade diastolic flow was detected in the main pulmonary artery, coinciding with atrial systole (A wave), throughout the respiratory cycle. Exercise function was measured by graded treadmill testing with respiratory mass spectrometry. Three patients were excluded because of pulmonary outflow obstruction (Doppler gradient > 40 mm Hg) or residual intracardiac shunts. Of the 38 patients, 37 were in sinus rhythm. Twenty (52.6%) had definite evidence of restriction with an A wave in the pulmonary artery, augmented during inspiration. In all 20 cases, there was superior vena caval flow reversal with atrial systole. Both inspiratory and expiratory transtricuspid E-wave deceleration time was significantly shorter in the restrictive group (P < .003 and P < .03, respectively). All patients had Doppler evidence of pulmonary regurgitation, but its duration was shorter in the restrictive group (P < .01) during inspiration. Cardiothoracic ratio was significantly lower in the restrictive group (P < .01), suggesting less severe pulmonary regurgitation. Both restrictive and nonrestrictive groups had reduced exercise MVO2 compared with healthy age- and sex-matched control subjects, but those with restrictive physiology had significantly better maximum oxygen uptake than the nonrestrictive group (P < .001). CONCLUSIONS: Isolated right ventricular restriction late after tetralogy of Fallot repair is common. Although it reflects abnormal hemodynamics, the A wave contributes to forward pulmonary arterial flow and shortens the duration of pulmonary regurgitation. Consequently, there is less cardiomegaly and improved exercise performance in those patients.

Proton MR spectroscopy in herpes simplex encephalitis: assessment of neuronal loss.

We present here the case of an 11-year-old boy with herpes simplex encephalitis diagnosed on the basis of clinical features, serology, and response to acyclovir, who relapsed after 3 weeks of therapy. In vivo proton magnetic resonance spectroscopy (1H MRS) of the brain, at 8 and 16 weeks after the onset of symptoms, showed abnormalities, most prominently a reduction in the N-acetylaspartate/choline ratio. The role of 1H MRS in assessing disease activity is discussed.