544 fetuses at increased risk of cardiac malformations were examined by echocardiography from gestational week 14 to 40 (median 21), during the period January 1989-March 2002. A heart defect was ...diagnosed in 70 fetuses. Three false positive diagnoses and eight false negative diagnoses occurred (sensitivity 89 % and specificity 99 %). Seven were terminated (three with trisomy, two with concomitant diaphragmatic hernia and two with single ventricles) and eight died in utero. 22 of 52 liveborn infants died during the neonatal period. Fetal arrhythmia was diagnosed in 30 cases of which eight died prenatally. This risk group screening identified only 3.3 % of all infants with heart defects born during the study period. The ambition to diagnose a greater proportion prenatally would require a general screening program. In order to guarantee a high quality of specialized fetal cardiology service, referrals should be centralized to only a few units in the country.
The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We ...compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.
To determine the efficacy of indomethacin to prevent the occurrence of symptomatic patent ductus arteriosus (PDA), a randomized clinical trial was conducted involving 32 preterm infants weighing 750 ...to 1500 g at birth who had hyaline membrane disease. By random assignment, 15 infants were given a single dose of indomethacin, 0.2 mg/kg intravenously, 24 hours after birth. Seventeen infants composed a control group for which indomethacin was reserved as treatment for symptomatic PDA. Birth weight, gestational age, male/female ratio, black/white ratio, and severity of disease were similar for both groups. Only one of the 14 survivors who received prophylactic indomethacin had symptomatic PDA, compared with nine of the 16 survivors in the control group (P = 0.007). There was no difference between the groups in development of bronchopulmonary dysplasia, duration of time endotracheal intubation, was required, duration in oxygen, duration to reach full feedings and regain birth weight, and duration of hospital stay. There was no difference between the two groups in incidence of intraventricular hemorrhage, and none developed necrotizing enterocolitis. These results indicate that the use of prophylactic indomethacin is beneficial in prevention of symptomatic PDA; the lack of differences in pulmonary sequelae or other complications may have been related to a population sample size not large enough to impart sufficient statistical power.
The effects of early left-to-right ductus shunting on left ventricular output (LVO) and cerebral blood flow velocity (CBV) were investigated in 3-day-old preterm infants by means of two-dimensional ...Doppler and M-mode echocardiography. Nineteen infants required mechanical ventilation because of severe lung disease (group A), and 19 had mild or no lung disease (group B). Six infants in each group had predetermined Doppler and M-mode criteria of a hemodynamically significant left-to-right ductus shunt (hsPDA). In group A the LVO was similar in infants with and without hsPDA, but those with hsPDA had lower mean arterial pressure (p = 0.006) and lower mean systolic-diastolic CBV (p = 0.001) than those without hsPDA. In group B the presence of hsPDA was associated with a higher LVO (p = 0.002), whereas neither mean arterial pressure nor mean systolic-diastolic CBV differed significantly in infants with and without hsPDA. In infants without hsPDA, those in group A had higher LVO (p = 0.012), lower mean arterial pressure (p = 0.003), and lower estimated systemic vascular resistance (p = 0.004) than those in group B. These results indicate that severely ill preterm infants receiving mechanical ventilation are less able than spontaneously breathing infants to defend systemic pressures and cerebral perfusion through an increase of LVO when a large ductus shunt develops. Possible reasons include an elevated baseline LVO, caused by systemic vasodilation, and hence a low preload reserve.
Ten children, aged six weeks to 13 years, without intracardiac shunts or lesions that could cause turbulent flow in the ascending aorta or aortic regurgitation, underwent cardiac catheterization, ...including cardiac output measurements by thermodilution. Simultaneously with each of six consecutive thermodilution injections, mean and maximal blood velocities in the ascending aorta were measured by pulsed Doppler echocardiography from the suprasternal notch. Aortic root and aortic orifice diameters were measured with M-mode and cross-sectional echocardiography. One patient had to be excluded from the analysis because of inadequate Doppler recordings. The best agreement with the results of the thermodilution was observed when internal systolic aortic root diameter was combined with mean velocity (r = 0.97, y = 0.90x + 0.28, SEE = 0.31 liters/min). When cardiac output was normalized for body size, there was still a good correlation between the results of these two methods.