Video viewing as an alternative to sedation for young subjects who have cardiac ultrasound examinations.

Sedation is often required for extended or quantitative echocardiographic examination of young patients. To test the hypothesis that patient viewing of entertaining videotapes during echocardiographic examinations would reduce the need for sedation and that Doppler pressure gradients so obtained would correspond satisfactorily with subsequent catheterization pressure gradients, 38 patients (age, 5 to 64 months; mean age, 18.6 months) with a variety of cardiac defects were studied. Because of excessive activity, resistance, fear, or crying, all of these patients would have normally been sedated. Instead, an age-appropriate videotape was used for patient viewing. In 35 of 38 patients, complete examinations were obtained with video viewing with no sedation. Twelve study patients later underwent catheterization while receiving sedation. The Doppler pressure gradients obtained while patients were viewing videotapes corresponded well with catheterization (r = 0.94). However, those pressure gradients were higher than those obtained with catheterization (mean, 8.3 mm Hg), but there was only one significant discrepancy of 22 mm Hg. In this series, complete examinations were obtained without sedation in 92% of subjects who would have normally required sedation (p less than 0.001), with minimal reduction in the accuracy of prediction of subsequent sedated catheterization pressure gradients. This suggests that near baseline conditions existed while patients were viewing television. Video viewing during echocardiographic examinations appears to be advantageous.

Symptomatic mitral valve prolapse in children and adolescents: catecholamines, anxiety, and biofeedback.

It has been proposed that symptomatic mitral valve prolapse may be associated with a hyperadrenergic state and/or increased anxiety. To test this hypothesis, Spielberger State-Trait Anxiety (STAI) scores and 24-hour urinary catecholamine collections were gathered from 11 children and adolescents without mitral valve prolapse, 6 with asymptomatic mitral valve prolapse, and 14 who had chest pain (some with additional symptoms of shortness of breath, palpitations, and fatigue). STAI scores and catecholamine excretion values were not significantly different between groups. Ten symptomatic patients were randomly assigned to either eight sessions of skin temperature biofeedback with daily home practice of relaxation-mental imagery techniques or an attention-placebo condition. Change in 24-hour urinary catecholamine excretion values and STAI scores from baseline to end of treatment did not differ significantly between treatment and placebo conditions. Although not evident at the end of treatment, a significant decrease in chest pain was found in the biofeedback group at 6-month follow-up evaluation. In summary, results of this study did not show evidence of increased sympathetic tone or levels of anxiety in symptomatic pediatric patients with mitral valve prolapse. A behavioral treatment program using biofeedback and relaxation-mental imagery techniques was associated with decreased chest pain at 6-month follow-up.

Surgical repair of anatomically corrected malposition of the great arteries.

Anatomically corrected malposition of the great arteries is a rare malformation in which the aorta and pulmonary artery arise from their appropriate ventricles but in an abnormal spatial relationship. This report describes 2 patients with anatomically corrected malposition who underwent closure of a ventricular septal defect and placement of a right ventricle-pulmonary artery conduit. A review of the literature indicates that surgical results have been good (92% survival) in those patients with situs solitus and atrioventricular concordance [S,D,L]. However, when there is atrioventricular discordance, that is, [S,L,D] or [I,D,L], hypoplastic right heart structures, or both conditions, the outcome after palliative procedures has been poor (29% survival). The results of surgical treatment should improve as this entity is recognized earlier and prompt surgical treatment is undertaken.

Relationships of cardiac and somatic growth in infancy and childhood.

Recent advances at clinical and experimental levels bespeak the need for a more complete understanding of cardiac growth and its relationship to somatic growth. The size of the heart is integral to several physiologic processes, including aerobic and work capacity, athletic performance, and reproduction. Because the morphologic foundations for cardiac size are laid in large part before adulthood, cardiac growth and the factors controlling it are important in achieving optimal cardiovascular fitness. We provide a baseline for normal cardiac growth during the late fetal to adolescent periods. Sexual differences are not evident in this prepubertal population. Heart weight is related to three measures of body and chest size. These relationships follow a hierarchy, whereby heart weight is most closely related to body weight, followed by body length, then chest circumference. The association of heart weight with chest circumference is a secondary one, reflecting more primary relationships between heart weight and body weight and length. Deviations from this normal hierarchy occur in certain environments and disease states.

The spectrum of the DiGeorge syndrome.

Clinical and autopsy data on 25 patients with DiGeorge syndrome and its variants are presented. Congenital heart disease was the most common presenting complaint; 15 patients came to medical attention in the first 48 hours of life because of cyanosis, cardiac murmurs, or tachycardia and tachypnea. Two unusual anomalies, interrupted aortic arch or truncus arteriosus, were seen in 17 patients. Clinically documented hypocalcemia associated with seizures was seen in ten patients, with a median age at onset of eight days. Fifteen of our 25 patients died at less than one month of age. Most of the patients surviving the first month of life developed purulent rhinitis, maculopapular rashes, failure to thrive, and developmental delay. Sixteen patients had major congenital anomalies not localized to the anterior neck and thorax; these anomalies included arhinencephaly, cleft lip, palate, or uvula, diaphragmatic abnormalities, hydronephrosis, malrotation of the gut and imperforate anus. The 24 autopsied cases constitute 0.7% of the 3,469 sequential postmortem studies done in the period 1950--1975 at The Children's Orthopedic Hospital and Medical Center.

Subdural hematoma following open-heart operations.

Six patients who died following open-heart operations were found at postmortem examination to have acute subdural hematomas. On the basis of the clinical and postmortem findings, two factors in the pathogenesis of the hemorrhage are postulated. In the presence of intraoperative heparin administration, significant hematoma formation may result from damage to the bridging dural veins from minor, inadvertent head trauma or alterations in cerebral volume from fluid shifts. Manipulation of the head in patients who have been given heparin should be undertaken with extreme care, particularly in infants. In any patient with neurological dysfunction who has also had an open-heart operation, the possibility of an expanding subdural hematoma must be considered.