Intravascular ultrasound imaging shortly after balloon angioplasty of native aortic coarctation in two neonates.

Two patients who had a balloon angioplasty for native coarctation of the aorta as neonates underwent redilatation for restenosis 1 or 2 months after the initial procedure. We recorded intravascular ultrasound (IVUS) images at redilatation. Before redilatation, there was an abnormal, localized area of ultrasound density equal to the inner layer of the aorta. The enlargement of this tissue was in proportion to the severity of stenosis and diminished after dilatations. No intimal flaps or tears were observed. From the IVUS images it appears that the mechanism to relieve the coarctation of our patients may be compression of this abnormal tissue.

Disopyramide improves hypoxia in patients with tetralogy of Fallot through a negative inotropic action.

The hemodynamic and right ventricular volumetric effects of disopyramide were investigated in patients with tetralogy of Fallot (TF). Intracardiac pressure and oxygen saturation were measured, before and after intravenous administration of disopyramide (2 mg/kg) in 7 patients who had not had previous surgery. Right ventricular volume and the diameter of its outflow tract were analyzed in these 7 and in a further 4 patients with a previous shunt. Aortic oxygen saturation increased from 90.4+/-7.5 (mean+/-SD) to 94.1+/-5.5% (p<0.05) with an increase in pulmonary blood flow and pressure. The systolic pressure gradient between the main pulmonary artery and the right ventricle decreased from 59+/-8 to 42+/-9 mmHg (p<0.01). Aortic pressure fell from 77+/-5 to 67+/-4 mmHg (p<0.05). Systemic vascular resistance increased from 15.3+/-2.2 to 19.4+/-3.3 u x m2 (p<0.05). Pulmonary vascular resistance remained unchanged. The diastolic and systolic diameter indices of the right ventricular outflow tract increased from 17.8+/-3.8 to 20.5+/-3.4 and from 6.5+/-3.0 to 10.4+/-2.2 mm/m2, respectively (p<0.01), whereas the right ventricular ejection fraction decreased. Disopyramide improves systemic oxygen saturation in patients with TF through its negative inotropic action on the right ventricle.

Benign partial epilepsy with affective symptoms: hyperkinetic behavior during interictal periods.

We report a 4-year-old boy with benign partial epilepsy (BPE) with affective symptoms associated with hyperkinetic behavior during interictal periods. He had had hypermobility and restlessness since about age 3. At 4 years, 6 months, he developed episodes consisting of an expression of terror without complete loss of consciousness. Although we first suspected an acute psychic problem, the ictal EEG was abnormal. After carbamazepine (CBZ) therapy, the frequency of the attacks decreased dramatically and the hyperkinetic behavior gradually diminished.

Mitral regurgitation with gross deformity of a mitral leaflet due to Kawasaki disease.

A 4-year-old girl had two episodes of Kawasaki disease which resulted in severe mitral regurgitation. She had not had any coronary arterial lesion during the acute phase of the illness, nor any myocardial ischemia. Severe destruction of the mitral leaflet was documented by two-dimensional echocardiography. It is suggested that this lesion was due to severe carditis and valvulitis which developed during the acute phase of the illness.

Development of cell-mediated cytotoxic immunity to respiratory syncytial virus in human infants following naturally acquired infection.

With virus-infected autologous and allogenic mononuclear cells as specific targets, the development of cell-mediated cytotoxic reactivity to respiratory syncytial virus (RSV) was studied in peripheral blood lymphocytes (PBL) in groups of infants with acute RSV infection and in other control groups of subjects during a community outbreak of RSV infection. No RSV-specific cellular cytotoxicity was observed in cord blood lymphocytes and in other uninfected controls. The PBL of infants with acute RSV infection exhibited significant cellular cytotoxic response. The activity peaked early, usually within 1 week after infection. The response appeared to be age-dependent. Over 65% of infants 6-24 months of age and about 35-38% of infants under 5 months of age exhibited cellular cytotoxicity to RSV. Cellular cytotoxic reactivity was observed against autologous and less frequently against allogenic RSV-infected target cells. These findings suggest the appearance of virus-specific cell-mediated cytotoxic immune response after acute RSV infection. The development of cellular cytotoxic responses may play a role in the mechanisms of protection against or the pathogenesis of RSV infection in man.

Electron microscopic study of the biopsied cardiac muscle in Duchenne muscular dystrophy.

Cardiac muscular biopsies were performed on 4 patients with Duchenne muscular dystrophy (DMD). None of the patients had cardiac symptoms and all of them exhibited normal electro- and echocardiographic findings. Electron microscopic examination of cardiac muscles from 4 patients with DMD disclosed proliferation of the mitochondria, changes in the cristae, abnormalities of the Z-bands, dilatation of the sarcoplasmic reticulum, deposition of glycogen, and changes in the nuclei. The most striking finding in these patients was that the residual bodies were increased and were observed mainly in the perinuclear region. These ultrastructural features suggest that an extreme exhaustion had already been present in myocardial tissue of the patients prior to the onset of cardiac symptoms.