22q11.2 deletion detected by endoscopic observation of pharyngeal pulsations in a child with submucous cleft palate and persistent velopharyngeal insufficiency.

22q11.2 microdeletion syndrome (22q11.2DS) is the most common syndrome associated with cleft palate and velopharyngeal insufficiency (VPI).

A quadricuspid pulmonic valve diagnosed in a live newborn by two-dimensional echocardiography.

Quadricuspid pulmonic valve is a rare congenital anomaly which appears to occur in the absence of other cardiac or systemic anomalies. It predominates in males and tends to be clinically quiescent. The first case of quadricuspid pulmonic valve in a live newborn infant diagnosed by two-dimensional echocardiography is presented here with a review of the literature.

Spontaneous closure of atrial septal defects in premature vs. full-term neonates.

Our hypotheses were that the following factors influenced closure of atrial septal defects (ASDs) detected in neonates: estimated gestational age (EGA), the presence of a persistent ductus arteriosus (PDA), severity of pulmonary disease, gender, and the initial size of the ASD. Our population consisted of 82 newborns (38 premature and 44 term) who were found before the age of 1 month to have an ASD. Closure of ASDs was analyzed using both Kaplan-Meier survival analysis and the Cox proportional hazards model, each with the five covariates.

Double-outlet right ventricle: an antenatal diagnostic dilemma.

Objective: The purpose of this study was to describe the antenatal ultrasonographic findings of fetuses with double-outlet right ventricle (DORV).

Design: The records were reviewed of all fetuses scanned in our ultrasound unit which were suspected of having DORV during a 6-year period ending in April 1996. A medical record search for all infants with a postnatal diagnosis of DORV was also undertaken to identify cases that were not detected antenatally.

Doppler velocity and flow through the aortic isthmus in normal term neonates.

Our hypothesis was that the relationship between the internal aortic diameter and the Doppler flow velocity across the aortic isthmus could be modeled by applying the principle of conservation of mass flow. The aortic diameter decreased at the isthmus by a mean of 18% (t = 11.02, p < 0.

Recurrence risk of supraventricular tachycardia in pediatric patients.

We analyzed risk of recurrence of supraventricular tachycardia (SVT) in 70 pediatric patients using both Kaplan-Meier survival analysis and logistic regression of likelihood of recurrence, each with covariates: (1) age at onset of SVT; (2) presence of Wolff-Parkinson-White syndrome (WPW), and (3) gender. Among 38 patients who had onset of SVT <1 year, only 11 had a recurrence, while among 32 older patients, 30 had a recurrence of SVT (p < 0.00001, Fisher's exact test).

Fetal cardiac asymmetry: a marker for congenital heart disease.

Objective: To determine the sensitivity of prenatally detected fetal cardiac asymmetry as a sonographic marker for congenital heart disease.

Methods: The normal ratios of pulmonary artery to aorta diameters and of right ventricle to left ventricle diameters were derived from normal fetuses scanned at 17 weeks or more in a 65-month period. Cross-sectional diameters of cardiac ventricles and great arteries were measured at the level of the valves at the time of the scan.

Sonographic screening to detect fetal cardiac anomalies: a 5-year experience with 111 abnormal cases.

Objective: To determine whether there is a difference between the types of cardiac lesions detected as abnormal prenatally and those that are not detected.

Methods: Consecutive fetuses at 14 weeks' gestation or more were scanned in our unit from February 1990 through July 1995 and later were delivered at our hospital. Outcome information was obtained from neonatal echocardiograms and autopsies.

Tetralogy of Fallot: prenatal diagnosis and postnatal survival.

Objectives: To correlate prenatal echocardiographic findings with infant outcome in a large screening population affected by tetralogy of Fallot.

Methods: Inclusion criteria required confirmed postnatal cardiac diagnosis, at least one fetal ultrasound examination with satisfactory heart visualization, and infant delivery at our institution. Aortic and pulmonary artery diameters were measured from real-time ultrasound or videotaped scans and compared against published nomograms.

Prenatal screening for cardiac anomalies: the value of routine addition of the aortic root to the four-chamber view.

Objective: To determine whether the addition of the aortic-root view would detect more congenital cardiac anomalies than a standard four-chamber view alone.

Methods: The study included fetuses of 14 weeks' gestation or more who were scanned in our unit during a 28-month period. Outcome information was obtained from postnatal echocardiograms and autopsies.

Review of 180 episodes of chest pain in 134 children.

This study intends to establish the etiology of chest pain in the pediatric population, to determine the causal relationship between a cardiac problem and chest pain in acutely sick children, and to provide a systematic approach to the management of these patients. The charts of 134 patients, up to 19 years of age, seen in the emergency department (ED) of Children's Hospital of Wisconsin (CHW) with the primary diagnosis of "chest pain" during a five-year period were reviewed. In 15% of these patients, a relationship was established between chest pain and actual cardiac disease.

Cardiac tamponade complicating a case of fever of unknown origin.

A 16-year-old male was transferred to Children's Hospital of Wisconsin (CHW) after 17 days of fever of unknown origin. An echocardiogram revealed a small pericardial effusion only. Serial echocardiography demonstrated an increased size of this effusion with collapse of the right atrium during diastole.

Cor pulmonale as a complication of methylmalonic acidemia and homocystinuria (Cbl-C type).

We report an infant with a bronchiolitis-like illness and rapid deterioration who developed a cor pulmonale-like picture with a dilated right ventricle. Urinary organic acid assays established a probable diagnosis of Cbl-C-type methylmalonic aciduria, later confirmed by complementation studies. Despite medical intervention and cyanocobalamin treatment the patient died on his tenth hospital day.

Increasing extracellular calcium concentration does not prevent hypotensive effects of verapamil in neonatal swine.

Effects of CaCl2 on cardiac function and regional circulatory responses to verapamil (V) infusion were studied in pentobarbital-anesthetized 2-week-old swine. V 100 micrograms/kg (n = 15) or 300 micrograms/kg (n = 15), given as a 2-min intravenous infusion, was repeated after 30 min. Only V was given to 15 of these.

Treatment of severe imipramine poisoning complicated by a negative history of drug ingestion.

The wide use of imipramine (Tofranil) for the treatment of nocturnal enuresis continues in spite of the unique dangers associated with this drug. Young children are particularly susceptible to the potentially lethal central nervous system and cardiovascular toxicities, yet the toxic potential of imipramine remains unrecognized by both parents and too many physicians. Management of severe imipramine intoxication can be difficult.

Young long distance runners. Physiological and psychological characteristics.

This study presents the physiological and psychological characteristics and the running histories of 16 subjects who began long distance running at age 4-12 years. Running duration was 3-15 years (mean 8.4 +/- 3.

Age-related digoxin-verapamil interactions in the adult rat.

We have previously described a method for inducing atrial ectopic tachycardia (AET) in the anesthetized adult rat using digoxin, 30 mg/kg s.c. In this study, we used this model to study digoxin-verapamil interactions in young and older male rats and have found age-related differences in their responses to digoxin alone and to digoxin-verapamil interactions as well.

Dilated cardiomyopathy complicating a case of epidermolysis bullosa dystrophica.

A child with epidermolysis bullosa dystrophica, recessive type (EBDR) developed significant anemia at 9 years of age and was treated with long-term transfusion therapy. At age 17 he had symptoms of congestive heart failure secondary to dilated cardiomyopathy. Treatment with digoxin and vasodilators for the past year has failed to improve his cardiomyopathy significantly.

Acute rheumatic fever in Wisconsin.

Medical records of patients discharged from Children's Hospital of Wisconsin from January 1980 to May 1988 who fulfilled the revised Jones criteria for acute rheumatic fever were reviewed. A total of 13 patients were hospitalized with a first attack of acute rheumatic fever during this period. Migratory polyarthritis was present in 92% of the patients and carditis in 62%.

Bilateral origin of the left anterior descending coronary artery in a young man.

Abnormalities of coronary artery origin from the aorta have been implicated in sudden death in sports among young athletes. We describe an 18-year-old patient who presented with nonspecific chest pain, developed ventricular couplets during recovery from a stress test, and whose coronary angiograms revealed bilateral left anterior descending coronary arteries, an abnormality that has not been previously reported.

Cardiac glycoside toxicity in small laboratory animals.

Cardiac glycosides are frequently administered to laboratory animals for research purposes. The effects achieved depend not only upon the particular glycoside and dose administered, but also upon an entire array of variables from the species of animal to the temperature of the animal housing facility. We review a number of these factors and their influence upon the effects achieved by the administration of cardiac glycosides to laboratory animals.

A model of atrial ectopic tachycardia in the rat.

A model of atrial ectopic tachycardia (AET) in the adult rat is described. Pentobarbital-anesthetized adult male rats given digoxin 30 mg/kg s.c.