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Association of atrioventricular valve atresia with single ventricle, truncus arteriosus communis and transposition. A basic reorientation in the approach to the definition of congenital heart defects.

A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided.

Child

Surgery for congenital heart disease in Ibadan. I: Non-cyanotic heart defects.

Congenital heart disease represents about 5% of all cardiovascular diseases in Nigeria today. Its incidence has increased in the last decade because of improved neonatal care, increased awareness and clinical acumen of physicians, better diagnostic tools and the introduction of newer techniques in cardiac catheterization. It is now possible to make the diagnosis with certainty and to define the extent of the pathology thus making it possible to select the appropriate surgical therapy for these children. This article reviews the commonest types of noncyanotic heart disease, the indications for surgery and the types of surgical procedures available in Nigeria today. The simple extracardiac defects, such as patent ductus arteriosus and coarctation of the aorta can be corrected with little or no mortality, but the more complex intracardiac defects can only be palliated pending the introduction of open heart surgery in Nigeria.

Aortic Coarctation

[Pathologic diagnosis of certain congenital heart defects].

Morphometrical examinations of 265 normally formed hearts collected at autopsies of infants under 2 years of age were used as a control of the heart weight and size of the pulmonary trunk and aorta. The time of anatomical closing of the arterial duct and oval window were established. Comparisons of the results of morphometry of normal and 173 malformed hearts with the tabulated data were used to develop objective criteria for pathological diagnosis of congenital heart diseases: open arterial duct and centrally located defect of the interatrial septum in infants dying within the first two months of life. The criteria for the detection of marginal forms of stenosis of the pulmonary trunk ostium and isolated coarctation of the aortal isthmus were established.

Age Factors

[Oxygen transport in the blood of children with congenital heart defects].

In 55 children with congenital heart defects (aged 6 months and older), hematocrit, hemoglobin concentration, acid-base status, oxygen pressure, oxygen saturation, and 2,3-DPG content of erythrocytes were determined in blood taken from the vena cava superior. The data were correlated with each other as well as with parameters obtained during cardiac catheterization. Whereas an increased hematocrit was seen only at a difference in arteriovenous oxygen saturation of more than 30%, or a central venous saturation below 65%, a close correlation between 2,3-DPG concentration in erythrocytes and these parameters was obtained already in the normal range (r = 0.79, and r = 0.78, respectively). A relatively close correlation was also found between 2,3-DPG concentration and half-maximal oxygen saturation pressure (T50 value; r equals 0.73), and the T50 values and the central venous oxygen saturation (r equals 0.61), respectively. Because of the shift to the right of the O2-binding curve the functional oxygen transport capacity was increased by an average 15% in children with acyanotic heart defects (mean T50 value of 31.6 mm Hg), and by 40% in children with cyanotic heart defects (mean T50 value of 34.6 mm Hg), as compared to healthy children (mean T50 value of 28.1 mm Hg). A marked influence on the position of the O2-binding curve and hence on oxygen release of the blood in the periphery is therefore exerted by the 2,3-DPG concentration, whose effect was about twice that of the Bohr effect in the pH range measured.

Biological Transport

Surgical treatment of complex congenital heart defects: recent developments with valved external conduits.

The use of valved external conduits has majorly expanded the place of corrective operations for children with a variety of congenital cardiac defects. Initially they were employed to correct patients with congenital pulmonary atresia and ventricular septal defect and subsequently placed between the venous ventricle and pulmonary artery for a number of other complex malformations. More recently they have been placed between the right atrium and pulmonary artery and between the systemic (left) ventricle and aorta. Surgical techniques, early and late results of these procedures are discussed.

Aorta, Thoracic