RESUMO

Down syndrome is commonly associated with significant congenital heart disease with the potential for early development of pulmonary hypertension. As such, children with Down syndrome may be at increased risk for both perioperative and long-term mortality. The purpose of this study, using data collected from a population-based outcomes study, is to analyze the potential role that Down syndrome plays in the outcome of surgically "corrected" congenital heart disease. Data were collected from a registry of all Oregon residents who, in the period 1958 to the present, had a reparative operation for one of 14 congenital cardiac malformations when younger than 18 years (N = 3965 patients). Down syndrome was present in 289 (7%) of the total registry patients. In evaluating the cardiac mortality associated with Down syndrome for each of the repaired cardiac malformations, only complete atrioventricular septal defect was associated with significantly higher perioperative (13% vs 5%) as well as higher overall late cardiac mortality through 20 years after the operation (20% vs 5%; p = 0.04). The survival outcomes for each of the other cardiac malformations were similar for children with and without Down syndrome.

Assuntos

RESUMO

Infants with congenital heart disease and chronic lung disease are at risk for development of systemic-to-pulmonary collateral arteries (SPCA). This study characterizes associated clinical findings in 20 premature infants without CHD who were diagnosed as having SPCA with echocardiography. SPCA can occur in premature infants without chronic lung disease and may represent a transient phenomenon.

Assuntos

RESUMO

Advances in echocardiography have enhanced our diagnostic imaging capabilities for congenital heart defects. In addition to improved resolution of two-dimensional images, cardiac hemodynamic assessment is possible with the use of Doppler, color Doppler, and stress echocardiography. Transesophageal echocardiography has allowed intraoperative assessment of cardiac repairs, and fetal echocardiography has allowed development of the field of fetal cardiology. The developing areas of intravascular ultrasonography and three-dimensional echocardiography show promise for the future. Echocardiography continues to revolutionize our ability to diagnose congenital heart defects accurately.

Assuntos

RESUMO

A series of investigations has been performed to assess the timing of physiologic closure of the ductus arteriosus in premature infants with and without respiratory distress syndrome. The data from these studies emphasize the concept of physiologic ductal patency and give normative data for expected closure rates through the fourth day of life. On the basis of these data, patency on or beyond the fourth day of life is abnormal irrespective of gestational age, and prematurity, in the absence of respiratory distress syndrome, is not a risk factor for persistent patent ductus arteriosus. We also found that persistent patent ductus arteriosus in larger premature infants (> or = 30 weeks of gestation) with respiratory distress syndrome is relatively uncommon. Last, ductal patency was evaluated in a group of low birth weight infants with severe respiratory distress syndrome in a randomized, double-blind trial of exogenous surfactant administration. We concluded that the beneficial effects of exogenous surfactant are not associated with either a greater clinical need for indomethacin or any increased risk of delayed closure of the ductus arteriosus.

Assuntos

Assuntos

RESUMO

The purpose of this investigation was to assess the duration of ductal shunting after birth in healthy preterm infants (30 to 37 weeks gestational age) without evidence of respiratory distress. Thirty-six infants were evaluated in the first 12 hours of life by means of two-dimensional echocardiography and color flow Doppler techniques, and then once daily until no ductal flow was detected (defined as functional closure). Preterm infants were subdivided into two groups by gestational age: group 1 = 30 to 33 weeks (n = 12); group 2 = 34 to 37 weeks (n = 24). Sixteen full-term infants (38 to 41 weeks) were similarly evaluated as control subjects (Group 3). One infant from each group had a closed ductus at the time of the first study (performed at a mean of 7.7 +/- 3.2 hours). Subsequent studies for the entire group were performed at a mean of 31.3 +/- 5.4 hours (day 2), 55.0 +/- 4.5 hours (day 3), and 80.3 +/- 6.1 hours (day 4). For the three groups, the rates of ductal closure ranged from 50.0% to 58.3% on day 2 and 81.3% to 87.5% on day 3. For the entire group, all but one infant had demonstrated closure of the ductus arteriosus by day 4. Within the range of gestational ages studied, we conclude that prematurity, in the absence of respiratory distress syndrome, does not prolong the initial duration of physiologic ductal shunting.

Assuntos

RESUMO

The purpose of this investigation was to use Doppler echocardiography to evaluate respiratory problems significant enough to warrant supplemental oxygenation in newborn infants. Of 17 infants (mean gestational age 37.5 weeks, mean birth weight 3070 g) 14 (82%) had detectable tricuspid regurgitation. By modified Bernoulli equation, all infants had right ventricular pressure greater than 60% of systemic pressure, and nine of 14 had estimated right ventricular pressure at or near systemic pressures. In eight infants for whom sequential evaluations could be obtained, right ventricular pressure as a percentage of systemic pressure gradually decreased, and corresponded to decreasing needs for supplemental oxygenation. Tricuspid regurgitation could no longer be detected between 3 and 16 days after the first study, and was associated with decreased right ventricular pressures (less than 50% systemic) and weaning from supplemental oxygenation. We conclude that in the near-term infant with early respiratory difficulties, tricuspid regurgitation is common and is associated with increased right ventricular pressure.

Assuntos

RESUMO

We evaluated two groups of diabetic women in pregnancy who differed primarily in the time of initiation of careful diabetes management. Group A (early) were entered in the first trimester (n = 35); group B (late) were entered in the late second or early third trimester (n = 28). Normal women delivering at the same period were used as controls (n = 23). All infants were evaluated by a thorough clinical and echocardiographic examination between 24 and 72 hours of life. Both groups of infants of diabetic mothers had mild increase in mean thickness of ventricular and septal walls compared with those of normal newborn infants, and both had a significant percentage with septal hypertrophy (43% vs 39%). None of the infants in the early group had respiratory symptoms requiring oxygen therapy, compared with 19% in the late group. The early group had significantly fewer infants with elevated right ventricular systolic time interval ratios than did the late group (20% vs 50%); none of the normal infants had elevated ratios. We conclude that careful management of diabetes in pregnancy reduces the severity of hypertrophic cardiomyopathy, although no advantage of early vs late management was obvious. Early management does significantly reduce the number of infants of diabetic mothers who develop respiratory symptoms requiring oxygen therapy.

Assuntos

RESUMO

The clinical effects of fluid therapy designed to maintain different degrees of negative water balance during the first five days of life were determined prospectively in 88 very low-birth-weight infants. Infants with birth weights of 750 to 1,500 gm were matched for birth weight in 250 gm increments. RDS or no RDS, asphyxiated or not, and inborn or outborn. Each infant was randomized to either Group 1--fluids managed to allow 1 to 2% loss of BW per day to a maximum loss of 8 to 10%, or Group 2--fluids managed to allow 3 to 5% loss of BW per day to a maximum loss of 13 to 15%. The mean five-day cumulative fluid input in Group 2 was 220 ml/kg less than in Group 1, yet Group 2 lost only 41 gm/kg more than did Group 1 (8.8% of BW lost in Group 1 vs 12.9% of BW lost in Group 2, P less than 0.001). There were no statistically significant differences between the groups in incidence of clinically significant patent ductus arteriosus, intracranial hemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis, dehydration, acute renal failure, or metabolic disturbances. There was no difference in duration of respiratory support required, in time to regain BW, or in time to discharge. There was no difference in the neonatal mortality rate. Fluid input in VLBW infants can be flexible to allow the gradual loss of 5 to 15% of birth weight during the first week of life without adversely affecting outcome.

Assuntos