ABSTRACT
Left ventricular-right atrial shunt is a relatively uncommon defect, which permits as communication between the left ventricle and right atrium. This paper is fifth case report in our country. This patient was noted to have congenital heart disease in two years old. The pathology of this lesion was classified into four types by Perry. The type C is compatible with this case. The first successful surgical correction was made by Kirby in 1957. The pathology of this lesion was classified into four types by Perry. The type C is compatible with this case. The first successful surgical correction was made by Kirby in 1957. In 12 years old boy, we have experienced a case of left ventricular-right atrial shunt. In selective left ventricular angiocardiography, we found opacification to right atrium and main pulmonary artery simultaneously, and also opacified right ventricle later. Operation was performed during cardiopulmonary bypass. We opened right atrium and found out a defect just below the annulus of the tricuspid valve. The defect measuring 6mm in diameter communicate the left ventricle with right atrium through a segment of the septal leaflet fused to the margins of the septal defect. In the tricuspid valve, a cleft, 4mm in length and a isolated perforation(2mm in diameter) were found in the septal leaflet. Associated cardiac anomaly was a non-functioning patent foramen ovale. The recovery was uneventful and discharged healthily on fourteenth day after operation.
Subject(s)
Child , Humans , Male , Angiocardiography , Cardiopulmonary Bypass , Foramen Ovale, Patent , Heart Atria , Heart Defects, Congenital , Heart Ventricles , Pathology , Pulmonary Artery , Tricuspid ValveABSTRACT
Hereditary anhidrotic ectodermal dysplasia is a rare hereditary condition in which certain structures derived from the ectoderm are undeveloped or underdeveloped, although, on a rarity, mesodermal or endodermal derivatives may be associated. Recently, we enperienced 3-cases of anhidrotic ectodermal dysplasia in 3 months male infant and in brothers aged 4(1/2) months and 22 months. They had abscence of sweating, hypotrichosis and defective dentition, which was characteristic features of this disorder. They seemed to be inherited a sex linked recessive fashion in their family ground. We established the diagnosis with this clinical features, their familial history, and skin biopsy. The first case of these was associated with congenital heart disease, a mesodermal derivative. But, we couldn't explain the associations of this anhidrotic ectodermal dysplasia and the occurance of C.H.D.