Background Published nomograms of pediatric echocardiographic measurements are limited by insufficient sample size to assess the effects of age, sex, race, and ethnicity. Variable methodologies have resulted in a wide range of Z-scores for a single measurement. This multicenter study sought to determine Z-scores for common measurements adjusted for body surface area (BSA) and stratified by age, sex, race, and ethnicity. Methods and Results Data collected from healthy non-obese children ≤18 years old at 19 centers with a normal echocardiogram included age, sex, race, ethnicity, height, weight, echocardiographic images, and measurements performed at the Core Laboratory. Z-score models involved indexed parameters (X/BSAα) that were normally distributed without residual dependence on BSA. The models were tested for the effects of age, sex, race, and ethnicity. Raw measurements from models with and without these effects were compared, and <5% difference was considered clinically insignificant because interobserver variability for echocardiographic measurements are reported as ≥5% difference. Of the 3566 subjects, 90% had measurable images. Appropriate BSA transformations (BSAα) were selected for each measurement. Multivariable regression revealed statistically significant effects by age, sex, race, and ethnicity for all outcomes, but all effects were clinically insignificant based on comparisons of models with and without the effects, resulting in Z-scores independent of age, sex, race, and ethnicity for each measurement. Conclusions Echocardiographic Z-scores based on BSA were derived from a large, diverse, and healthy North American population. Age, sex, race, and ethnicity have small effects on the Z-scores that are statistically significant but not clinically important.

Pregnancy after the Fontan operation appears to have been well tolerated in 13 to 14 gravidas. There does appear to be an increased risk of miscarriage. The tendency to routinely discourage pregnancy may need to be reconsidered.

Bulboventricular foramen obstruction may complicate the management of patients with single left ventricle. Bulboventricular foramen size was measured in 28 neonates and infants greater than 5 months old and followed up for 2 to 5 years in those patients whose only systemic outflow was through the foramen. The bulboventricular foramen was measured in two planes by two-dimensional echocardiography, its area calculated and indexed to body surface area. One patient died before surgical treatment. The mean initial bulboventricular foramen area index was 0.94 cm2/m2 in 12 patients (Group A) in whom the foramen was bypassed as the first procedure in early infancy. The remaining 15 patients underwent other palliative operations but the bulboventricular foramen continued to serve as the systemic outflow tract. There was one surgical death. Six (Group B) of the 14 survivors developed bulboventricular foramen obstruction during follow-up (mean initial bulboventricular foramen area index 1.75 cm2/m2). The remaining eight patients (Group C) did not develop obstruction during follow-up and had an initial bulboventricular foramen larger than that in the other two groups (mean initial bulboventricular foramen area index 3.95 cm2/m2). All patients with an initial bulboventricular foramen area index less than 2 cm2/m2 who did not undergo early bulboventricular foramen bypass developed late obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Although Fontan-type repair of single ventricle early in life is associated with reversal of the abnormal contractile mechanics associated with age and volume load, this capacity for recovery diminishes with age at surgery.

Video-assisted endoscopic techniques have reduced operative trauma in adult thoracic and general surgery, but applications in children with congenital heart disease have been limited. We report the development of video-assisted thoracic surgery procedures for neonates and infants with cardiovascular disease. Endoscopic instruments and techniques for pediatric cardiovascular procedures were designed and tested in the animal laboratory. Forty-eight operations were subsequently performed in 46 pediatric patients ranging in age from 2 hours to 14 years (median 9 months), weighing from 575 grams to 54 kg (median 8.5 kg). Clinical applications included seven different surgical procedures: patent ductus arteriosus interruption in infants (n = 26) and premature neonates (n = 5), vascular ring division (n = 8), pericardial drainage and resection (n = 3), arterial and venous collateral interruption (n = 2), thoracic duct ligation (n = 2), epicardial pacemaker lead insertion (n = 1), and diagnostic thoracoscopy (n = 1). There was no operative mortality. Technical success, defined as a video-assisted procedure completed without incising chest wall muscle or spreading the ribs, was achieved in 39 of 48 procedures (82%), with thoracotomy required to complete nine procedures. Most patients (22/25, 88%) undergoing elective ductus ligation were extubated in the operating room and discharged from the hospital within 48 hours of the operation. Eight of the last 10 patients having ductus ligation were discharged on the first postoperative day. Residual ductal flow was assessed by (1) transesophageal echocardiography in the operating room (incidence: 0/25, 0%, 70% CL 0% to 7.3%); (2) discharge auscultation (incidence: 1/30, 3%, 70% CL 0.5% to 10.8%); and (3) follow-up Doppler echocardiography (incidence: 3/25, 12%, 70% CL 5.4% to 22.6%). Video-assisted thoracoscopic techniques can be safely applied to pediatric patients with patent ductus arteriosus and vascular rings and may become an effective addition to the staged management of more complex forms of congenital heart disease.

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