Subject(s)
Catheter Ablation , Heart Neoplasms/complications , Rhabdomyoma/complications , Tachycardia, Ventricular/surgery , Female , Follow-Up Studies , Humans , Infant , Tachycardia, Ventricular/etiology , Torsades de Pointes/etiology , Torsades de Pointes/surgery , Tuberous Sclerosis/complications , Ventricular Premature Complexes/etiology , Ventricular Premature Complexes/surgeryABSTRACT
Head-up tilt test has been useful in evaluating children with neurocardiac syncope. In this study patients with positive baseline and isoproterenol tests had repeat tilt testing done after normal saline infusion. If the symptoms persisted, the test was then repeated with phenylephrine infusion. Of the 101 patients studied, 58 (57%) had a positive tilt sign. Normal saline infusion was given to 53 patients. Three patients were excluded because of structural heart disease. Fifty patients (aged 14 +/- 4 years) comprised the study group. Forty-two (84%) of 50 patients had a negative repeat tilt sign after normal saline infusion, and these patients were treated with 0.5 to 1 gm of salt three times a day and/or fludrocortisone (20 patients). Of the 8 (16%) patients who did not respond to normal saline infusion, 5 had negative results when given phenylephrine and were treated with pseudoephedrine. Follow-up data on 42 patients (range 4 to 40 months, median 18 months) showed that all 35 patients who responded to normal saline were either asymptomatic or had improved. Two patients were successfully treated with pseudoephedrine; however, two patients in this group required pacemaker therapy. We conclude that (1) normal saline infusion mitigates the hemodynamic effects of neurocardiac syncope, (2) high-salt diet treatment in these patients was economical and effective, and (3) failure to respond to normal saline test may indicate a less favorable prognosis.
Subject(s)
Saline Solution, Hypertonic/administration & dosage , Syncope/drug therapy , Tilt-Table Test , Adolescent , Adrenergic Agents/administration & dosage , Adrenergic Agents/pharmacology , Age Factors , Child , Ephedrine/administration & dosage , Ephedrine/pharmacology , Female , Fludrocortisone/administration & dosage , Fludrocortisone/pharmacology , Heart Diseases/complications , Heart Diseases/physiopathology , Hemodynamics/drug effects , Humans , Infusions, Parenteral , Male , Prognosis , Saline Solution, Hypertonic/pharmacology , Syncope/diagnosis , Syncope/etiologyABSTRACT
Pediatric cardiologists are often consulted to evaluate continuous murmurs in children. This case report describes a child with a congenital vertebral-jugular fistula who presented with torticollis and a continuous murmur. The differential diagnosis of continuous murmurs in this setting is discussed.
Subject(s)
Arteriovenous Malformations/diagnostic imaging , Jugular Veins/abnormalities , Vertebral Artery/abnormalities , Arteriovenous Malformations/complications , Cineangiography , Diagnosis, Differential , Humans , Infant, Newborn , Jugular Veins/diagnostic imaging , Male , Torticollis/etiology , Vertebral Artery/diagnostic imagingABSTRACT
In one family a duplicated 21q was shown to be a true isochromosome, which segregates from mosaic mother to non-mosaic child with full Down syndrome phenotype. Densitometric analysis of Southern blots, using probe pPW228C for the distal long arm of chromosome 21, indicated that the 21q duplication contains two copies of the allele detected by the probe. Maternal mosaic karyotype of 45,XX,-21/46,XX/46, XX,-21,+21i(21q) also suggested transverse mitotic centromere division as the origin of the 21q isochromosomes. Morphologic analysis of chromosome heteromorphisms strengthened this interpretation because the free 21 missing in the cell line with 45 chromosomes was also missing in cells with the isochromosome. In a second family the cytogenetic data also suggested transmission of an i(21q) from mosaic mother to non-mosaic Down syndrome child but molecular evidence did not prove identity of alleles in the duplicated chromosome 21.
