Severe cyanosis due to atrial septal defect and an anomalous inferior vena caval valve. 1978

H Laks, and N Ahmad, and J G Mudd

Uncomplicated atrial septal defect is often associated with mild arterial desaturation. A case is reported with severe cyanosis and life-threatening hypoxemia due to an atrial septal defect. Cardiac catheterization revealed normal right heart pressures and an atrial septal defect with shunting of inferior vena caval blood into the left atrium. At surgery an anomalous inferior vena caval valve was found directing inferior vena caval blood into the left atrium. The embryologic basis for this association is discussed. The delayed onset of severe cyanosis may have been contributed to by the thoracic scoliosis.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003490 Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Cyanoses
D005260 Female Females
D005318 Fetal Heart The heart of the fetus of any viviparous animal. It refers to the heart in the postembryonic period and is differentiated from the embryonic heart (HEART/embryology) only on the basis of time. Fetal Hearts,Heart, Fetal,Hearts, Fetal
D006344 Heart Septal Defects, Atrial Developmental abnormalities in any portion of the ATRIAL SEPTUM resulting in abnormal communications between the two upper chambers of the heart. Classification of atrial septal defects is based on location of the communication and types of incomplete fusion of atrial septa with the ENDOCARDIAL CUSHIONS in the fetal heart. They include ostium primum, ostium secundum, sinus venosus, and coronary sinus defects. Atrial Septal Defects,Ostium Primum Atrial Septal Defect,Persistent Ostium Primum,Atrial Septal Defect,Atrial Septal Defect Ostium Primum,Ostium Secundum Atrial Septal Defect,Defect, Atrial Septal,Ostium Primum, Persistent,Primum, Persistent Ostium,Septal Defect, Atrial
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000860 Hypoxia Sub-optimal OXYGEN levels in the ambient air of living organisms. Anoxia,Oxygen Deficiency,Anoxemia,Deficiency, Oxygen,Hypoxemia,Deficiencies, Oxygen,Oxygen Deficiencies
D014682 Vena Cava, Inferior The venous trunk which receives blood from the lower extremities and from the pelvic and abdominal organs. Inferior Vena Cava,Inferior Vena Cavas,Vena Cavas, Inferior
D014684 Venae Cavae The inferior and superior venae cavae. Cavae, Venae

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