Cardiovascular malformation in infant deaths. 10-year clinical and epidemiological study.
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The infant mortality from cardiovascular malformations in a region with a population of 2 million inhabitants during a 10-year period has been studied. The study involved validation of the diagnoses and judgement whether the cardiovascular malformation was the dominating or a contributing cause of death. It is shown that the incidence of fatal cardiovascular malformations is probably overestimated in the official death statistics. Evaluation of the clinical findings and necropsy reports are important aids in obtaining more reliable figures of the incidence. The rate of referral of infants with cardiovascular malformations has increased during the period of this study, so that the number of operable lesions not referred has decreased. The infant mortality rate, found in this study, of 1.33 per 1000 liveborn babies constitutes about 20 per cent of all liveborn infants with cardiovascular malformations. The most common lesions found in those who died belonged to the group constituting the hypoplastic left heart syndrome. The proportion of this type of malformation, about 20 per cent of all those dying, is higher than in other similar studies. This difference can probably be explained by variations in selection and classification. (+info)
Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.
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This review presents the cardiac and non-cardiac malformations in 60 cases with asplenia and polysplenia with special reference to distinguishing factors which may be helpful in the clinical recognition of these syndromes. The asplenia cases were predominantly male and presented with cyanosis. They frequently had transposition of the great arteries (72%) with pulmonary stenosis or atresia (88%) and total anomalous pulmonary venous drainage (72%). Deaths were caused by cardiac failure and anoxia in 57 per cent of cases. Most of the patients died in the first year of life (79%), but longer survival is possible in the asplenia syndrome. The polysplenia cases were predominantly female and survived longer. The characteristic clinical findings were the relatively more benign presenting signs and the leftward or superiorly orientated P wave axis on the electrocardiogram. Conotruncal abnormalities were less common and total anomalous pulmonary venous drainage did not occur. On angiography the inferior vena caval drainage via the azygos system was clearly identified and this was present in all cases at surgery. Our study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable. Three families had two affects sibs but no single genetic factor could be identified. The aetiology of these syndromes remains undetermined. (+info)
A new use for the amplatzer duct occluder device.
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We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel. (+info)
Coronary artery fistula: report of three cases.
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Three children are described with a right coronary artery fistula communicating with a right heart chamber. Each had a continuous murmur like that of a patent ductus arteriosus but situated at a lower level. Aortography established the diagnosis and excluded any accompanying malformation. It is concluded that to prevent complications surgical treatment should be recommended. (+info)
The incidence of congenital heart disease.
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This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times. (+info)
Plasma brain natriuretic peptide as a predictor of haemodynamically significant patent ductus arteriosus in preterm infants.
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Patent ductus arteriosus (PDA) is an important cause of morbidity in extremely preterm infants. As increased plasma brain natriuretic peptide (BNP) is a common feature of adult cardiac disease, we investigated the value of plasma BNP concentration as a predictor of haemodynamically significant PDA in very preterm infants. We studied 18 preterm infants (12 male) of median gestational age 30 weeks (range 24-34), median birth weight 1.46 kg (0.54-2.13) and 11 healthy term controls. Plasma BNP levels were measured by double-antibody radioimmunoassay on days 3, 5 and 7 of life, and an echocardiogram was performed on day 7. Six infants of median gestation 26 weeks (26-30), median birth weight 0.92 kg (0.54-1.04) had PDA proven by echocardiography on day 7. BNP concentrations (pg/ml) on day 3 were significantly higher in these infants than in the remaining twelve [median 2012 (786-2759) versus 42 (7-704), P<0.001]. In four infants PDA was treated successfully (one surgically, three with non-steroidal anti-inflammatory drugs). Two had haemodynamically insignificant closing ducts. In these infants with therapeutic or spontaneous resolution of a PDA, plasma BNP fell to normal values [median after treatment 9 pg/ml (8-27)]. Early measurement of plasma BNP in the first few days of life is a useful method for predicting those preterm infants who may require intervention for PDA. (+info)
Reference values for ductus venosus Doppler flow measurements at 10-14 weeks of gestation.
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OBJECTIVES: To calculate reference ranges for ductus venosus Doppler measurements obtained transabdominally at 10-14 weeks of gestation. DESIGN: Two hundred and one normal fetuses with a crown-rump length (CRL) ranging from 38 to 88 mm were examined in a cross-sectional study. The pulsatility index for veins (PIV), peak velocity during ventricular systole (S-wave), lowest forward velocity during atrial contraction (A-wave) and time-averaged maximum velocity (TAMXV) were recorded from the ductus venosus. Flow velocity waveforms were also classified as normal or abnormal according to the presence (normal) or absence or reversal (abnormal) of frequencies during atrial contraction. RESULTS: Three of 201 fetuses showed an abnormal flow pattern (1.5%; 95% exact confidence interval, 0.3-4.3%). In the 198 fetuses with a normal flow pattern, the mean PIV ranged from 1.07 at a CRL of 38 mm to 1.00 at a CRL of 88 mm (r = -0.093; P = 0.19). A significant increase in mean blood flow velocity with increasing CRL was noted for the S-wave (27.0 cm/s to 33.6 cm/s; r = 0.17; P = 0.02), the A-wave (5.9 cm/s to 7.8 cm/s; r = 0.14; P = 0.04) and the TAMXV (19.4 cm/s to 25.3 cm/s; r = 0.19; P < 0.01). Crown-rump length-specific reference ranges for each parameter were calculated using the method of scaled absolute residuals. CONCLUSIONS: Abnormal ductus venosus flow patterns could be observed in normal fetuses, even if they ocurred with a low prevalence. Reference values for Doppler measurements were established in fetuses with normal patterns of flow. (+info)
Cyclooxygenase-1-selective inhibition prolongs gestation in mice without adverse effects on the ductus arteriosus.
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Preterm delivery is the leading cause of neonatal mortality and contributes significantly to infant morbidity. Classical cyclooxygenase (COX) inhibitors, such as indomethacin, which inhibit both COX-1 and COX-2, are effective for delaying premature labor, but their use is limited by serious complications to the fetus and neonate, including adverse effects on the ductus arteriosus (DA). Using isoform-selective inhibitors, we characterized the roles of the COX isoforms in the initiation of labor and the regulation of fetal and neonatal DA closure in mice. Chronic inhibition of COX-2 during pregnancy (gestation days 15-18) significantly increased neonatal mortality by preventing closure of the DA after birth, whereas acute COX-2 inhibition near the end of term (gestation day 18) constricted the fetal DA. In contrast, the inhibition of COX-1 during pregnancy lacked these prenatal and postnatal adverse effects on the DA and effectively delayed the initiation of full-term labor and LPS-induced preterm labor. These findings suggest that premature fetal DA closure or neonatal patent DA observed following indomethacin tocolysis in women may result from the inhibition of COX-2. Therefore, COX-1-selective inhibitors may provide effective treatment to delay preterm labor with fewer adverse effects on fetal or neonatal health than nonselective or COX-2-selective inhibitors. (+info)