Effects of beta-adrenergic antagonists on the QT measurements from exercise stress tests in pediatric patients with long QT syndrome.

It has been proposed that beta-adrenergic antagonist protection against cardiac events in patients with long QT syndrome (LQTS) may be related to a decrease in baseline QTc dispersion. To determine the effects of beta-blocker therapy on QT measurements, we evaluated the exercise tests of 25 pediatric patients with LQTS. Measurements were made of the maximum QTc interval and QTc dispersion during the various segments of the exercise test. There was no statistically significant difference between the pre-beta-blocker and post-beta-blocker maximum QTc interval during the supine (0.473 +/- 0.039 vs 0.470 +/- 0.038 sec), exercise (0.488 +/- 0.044 vs 0.500 +/- 0.026 sec), or recovery (0.490 +/- 0.031 vs 0.493 +/- 0.029 sec) phases of the exercise stress test. There was also no statistically significant difference between the pre-beta-blocker and post-beta-blocker QTc dispersion during the supine (0.047 +/- 0.021 vs 0.058 +/- 0.033 exercise vs 0.063 +/- 0.028 sec), or recovery (0.045 +/- 0.023 vs 0.052 +/- 0.026 sec) phases of the exercise stress test. Therefore, the protection that beta-blockers offer appears not to be related to a reduction of the baseline QTc interval or a decrease of QTc dispersion.

Atrioventricular node reentry tachycardia in pediatric patients.

Atrioventricular node reentry tachycardia (AVNRT) is a significant cause of paroxysmal supraventricular tachycardia (SVT) in the pediatric population. Symptoms can include palpitations, chest pain, fatigue, light-headedness and syncope. AVNRT is a reentry tachycardia that is comprised of dual conduction pathways through the AV node. On electrocardiogram, AVNRT usually manifests as a regular tachycardia with a narrow QRS complex and P waves that are either absent or distort the terminal portion of the QRS complex. Electrophysiology study will reveal dual AV node pathways: a fast pathway with a short AH interval and a long effective refractory period (ERP); and a slow pathway with a longer AH interval and a shorter ERP. During tachycardia, electrophysiologic signals will reveal conduction up the midline. Introduction of premature ventricular contractions and measurement of the HA interval during SVT can help distinguish AVNRT from a SVT utilizing an accessory pathway. Radiofrequency catheter ablation (RFA) has been used increasingly in children as treatment for AVNRT. The initial approach to RFA of AVNRT was modification of AV fast pathway conduction by lesions placed near the anterosuperior aspect of the triangle of Koch, known as the anterior approach method. However, this technique was associated with a significant risk of complete AV block. Now, the posterior approach slow pathway modification is used more commonly, which positions the ablation catheter along the tricuspid annulus immediately anterior to the coronary sinus ostium. This has been associated with a lower risk of complete AV block. Using this technique, RFA should be considered the method of choice for curative therapy of AVNRT in pediatric patients.

Thrombus formation after the Fontan operation.

BACKGROUND: Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed. METHODS AND RESULTS: Between January 1987 and January 1999, 592 patients underwent echocardiography after Fontan operation and 52 (8.8%) had intracardiac thrombus. Median age at Fontan operation was 1.9 years (range 0.8 to 35.1). Freedom from thrombus was 92%, 90%, 84% and 82% at 1, 3, 8, and 10 years after Fontan operation, respectively. There was no difference in freedom from thrombus, based on type of operation (atriopulmonary vs. lateral tunnel) or presence of fenestration. Thrombus was detected in the systemic venous atrium in 26 (48%), in the pulmonary venous atrium in 22 (44%), in both atria in 1 (2%), in the hypoplastic left ventricular cavity in 2 (8%), and in the ligated pulmonary artery stump in 1 (2%). CONCLUSIONS: Thrombus formation occurs with equal frequency in all types of modifications and is seen in the pulmonary, as well as the systemic venous atria. Our study suggests that thrombus formation is inherent to the physiology after Fontan operation and is not related to the type of modification performed.

Airway growth after pediatric lung transplantation.

BACKGROUND: Lung transplantation (LT) has been successfully offered to pediatric patients. Very little is known about the growth of the transplanted lung, especially in the infant population. Computerized tomography (CT) scanning is a simple method for studying pediatric patients who have undergone LT. We evaluated the use of CT scans to assess airway growth after pediatric LT, compare airway diameter indexed to somatic growth between LT patients and normals, and compare the growth of pre-anastomotic and post-anastomotic airways indexed to somatic growth in pediatric LT patients. METHODS: We reviewed CT scans on all pediatric patients who underwent primary LT before their fifteenth birthday between January 1995 and September 1998. Uniform measurements of diameter were made in pre-anastomotic (trachea, and proximal right and left bronchi) and post-anastomotic (distal right and left bronchi) sites. These measurements were then correlated with height and compared to previously published normal values. RESULTS: Of the 16 patients who underwent LT during the study period, 11 had at least 2 sequential CT scans (LT age 3 months to 14 years, median 2 years). Thirty-one CT scans were reviewed. Inter-observer variability was within 1 standard deviation (2 mm) in 93% of the measurements and inter-observer reliability was 0.91 by analysis of variance. Tracheal transverse diameter plotted against body height (slope 0.0072, correlation coefficient 0.88) was virtually identical to previously published norms. A similar relationship between airway diameter and height was observed in pre-anastomotic and post-anastomotic segments. CONCLUSION: CT scanning is a reliable method for assessing airway growth in pediatric LT recipients. Tracheal growth in pediatric LT recipients is similar to that of normal children. Post-anastomotic large airways grow similarly to native, pre-anastomotic airways.

Outcome of infants listed for lung or heart/lung transplantation.

BACKGROUND: Little is known about outcome, characteristics, or organ availability for infants listed for lung or heart/lung transplantation. METHODS: Within a 45-month period at one institution, all pediatric patients who were listed for primary lung or heart/lung transplantation and who reached the end point of either transplant or death prior to transplant were identified. Outcomes for those patients listed as younger than and older than 1 year of age were compared. RESULTS: Among 48 pediatric patients, 19 were infants less than one year of age. The median age among infants at listing was 3.7 months (range 0.5 to 8.9 months). Death before transplant occurred in 10 of 19 infants (53%) compared with 14 of 29 (48%) children. When comparing those infants who died prior to transplant with those who received organs, there were no significant differences with respect to size, blood type, age at listing, presence of pulmonary hypertension, or type of transplant for which the patient was listed. There was a trend toward poorer pre-transplant survival for infants when compared with children. Waiting times were significantly shorter for infants vs children (p = 0.02). The incidence of acute cellular rejection and serious infection was similar in the 2 groups. Infants had significantly longer hospitalization post-transplant and a trend toward poorer hospital survival, although survival at 1 year was comparable between the 2 groups. CONCLUSION: The outcome for infants listed for lung or heart/lung transplantation is similar to that of children; thus, very young age should not be considered a contraindication to lung or heart/lung transplantation. Earlier diagnosis and listing may decrease pre-transplant mortality.

Precision measurements of water droplet evaporation rates.

The rate at which a motionless droplet evaporates can be measured continuously and precisely by determining the period of the intensity fluctuation of laser light reflected off the center of the droplet. The back scattered light is the coherent sum of light reflected from the external and the internal surfaces of the droplet; under typical conditions the back scattered light intensity oscillates at about 2 Hz or 3 Hz. This method is applied to pure water droplets in the diameter range from 0.6 mm to 1.8 mm, supported by bead thermistors and beaded glass fibers, and the results are compared with the quasi-stationary theory of Maxwell. Our measurements show that, after steady state is reached, the rate of change of diameter is inversely proportional to the diameter, as predicted in the quasi-stationary theory. Our experiments give a somewhat slower evaporation rate than the theory predicts; this discrepancy can be eliminated if one assumes that the surface temperature of the droplet is somewhat lower than measured with the bead thermistor. Using the optical method, mechanical resonances of the droplet can be sensitively detected; acoustically induced shape resonances are briefly investigated.