Cases reported "Heart Defects, Congenital"

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1/8. In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.

    Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.
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ranking = 1
keywords = animal
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2/8. PAGOD syndrome: eighth case and comparison to animal models of congenital vitamin a deficiency.

    We observed a 46, XY infant with atrophy of the optic nerve, complex congenital heart disease including a double outlet right ventricle, hypoplasia of the right pulmonary artery and lung, eventration of the diaphragm, and ambiguous genitalia. The baby died of cardiac arrhythmias at 204 days. The pattern of malformations was compatible with pulmonary tract and pulmonary artery, agonadism, omphalocele, diaphragmatic defect, and dextrocardia (PAGOD) syndrome. The condition may resemble the malformation complex associated with developmental deficiency of vitamin A or retinoic acid, as described in animal models.
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ranking = 5
keywords = animal
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3/8. A histologic study of nonmorphogenetic forms of hereditary hearing impairment.

    It appears that many forms of syndromic and nonsyndromic hereditary hearing impairment are secondary to either neuroepithelial or cochleosaccular dysfunction. Making this distinction can be difficult in human temporal bone specimens; however, this added knowledge may ultimately provide prognostic and therapeutic information in hearing habilitation. Fundamental studies using animal models of different types of hereditary deafness may also prove useful in this respect.
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ranking = 1
keywords = animal
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4/8. Cardiovascular anomalies associated with prenatal exposure to theophylline.

    theophylline has been shown to be a potent cardiovascular teratogen in animals, but there has been no evidence linking theophylline with congenital anomalies in infants. The cardiovascular anomalies in our three patients were aortic anomalies, double-outlet right ventricle, transposition of the great arteries, total anomalous pulmonary venous connection, and hypoplasia of the left ventricle. Some of these rare defects were also induced frequently in animal experiments with theophylline, which suggests that this drug can be a cardiovascular teratogen in a susceptible human fetus.
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ranking = 2
keywords = animal
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5/8. The acardiac anomaly.

    karyotype analysis of a premature human acardiac twin disclosed normal chromosomes. A review of previous cytogenetic, placental and animal studies suggests that chromosomal errors are not the cause of the acardiac anomaly. Rather, they point to the placental vascular anastomoses as the principal pathogenetic event.
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ranking = 1
keywords = animal
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6/8. Biologic reconstruction of the right ventricular outflow tract. Preliminary experimental analysis and clinical application in a neonate with type I truncus arteriosus.

    A unique concept of right ventricular outflow tract reconstruction is presented. Applications of this concept, employing a glutaraldehyde-preserved heterograft mitral valve leaflet mounted directly to the right ventricle, was studied in six immature goat models. Interval evaluation of these models demonstrated no significant outflow tract obstruction or aneurysm. Only one animal had significant pulmonary outflow regurgitation angiographically at the time of sacrifice. All heterograft valve leaflets showed a degree of calcification and/or retraction directly related to the time interval between implant and sacrifice. Encouraging early results were obtained with this method of pulmonary outflow tract reconstruction in a 2,400 gram neonate with type I truncus arteriosus.
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ranking = 1
keywords = animal
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7/8. Diprosopia revisited in light of the recognized role of neural crest cells in facial development.

    OBJECTIVE--The aim of this study is to compare the theory of embryogenesis of the face with human diprosopia. This peculiar form of conjoined twinning is of great interest because 1) only the facial structures are duplicated and 2) almost all cases have a rather monomorphic pattern. The hypothesis is that an initial duplication of the notochord leads to two neural plates and subsequently duplicated neural crests. In those conditions, derivatives of the neural crests will be partially or totally duplicated; therefore, in diprosopia, the duplicated facial structures would be considered to be neural crest derivatives. If these structures are identical to those that are experimentally demonstrated to be neural crest derivatives in animals, these findings are an argument to apply this theory of facial embryogenesis in man. methods--Serial horizontal sections of the face of two diprosopic fetuses (11 and 21 weeks gestation) were studied macro- and microscopically to determine the external and internal structures that are duplicated. Complete postmortem examination was performed in search for additional malformations. RESULTS--The face of both fetuses showed a very similar morphologic pattern with duplication of ocular, nasal, and buccal structures. The nasal fossae and the anterior part of the tongue were also duplicated, albeit the posterior part and the pharyngolaryngeal structures were unique. Additional facial clefts were present in both fetuses. Extrafacial anomalies were represented by a craniorachischisis, two fused vertebral columns and, in the older fetus, by a complex cardiac malformation morphologically identical to malformations induced by removal or grafting of additional cardiac neural crest cells in animals. CONCLUSION--These pathological findings could identify the facial structures that are neural crest derivatives in man. They are similar to those experimentally demonstrated to be neural crest derivatives in animals. In this respect, diprosopia could be considered as the end of a spectrum, whereas the other end is agnathia-holoprosencephaly complex. This assumption has to be discussed, but we want to draw attention to the fact that diprosopia must not be considered as a curious form of conjoined twinning, but as a major means of bringing us a better knowledge of the facial embryogenesis in man.
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ranking = 3
keywords = animal
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8/8. fluconazole-induced congenital anomalies in three infants.

    fluconazole has been associated with various teratisms in animals, including craniofacial ossification defects, thin, wavy ribs, and renal pelvis defects. We describe three infants born to women who were receiving fluconazole through or beyond the first trimester of pregnancy. All of the infants had congenital anomalies; no other drug was implicated. Only one of the three infants survived. Their anomalies, similar to those observed in animal studies, were largely craniofacial, skeletal (i.e., thin, wavy ribs and ossification defects), and cardiac. One of these infants was previously reported as having Antley-Bixler syndrome; however, given the chronology described herein and the similarity of this infant to the others, we conclude that her deformities also represent the potent teratogenic effect of fluconazole.
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ranking = 2
keywords = animal
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