Congenitally corrected transposition with pulmonary atresia and intact ventricular septum. 2000

C R Patel, and M L Spector, and K G Zahka
Department of Pediatrics, Rainbow Babies and Childrens Hospital, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA. cxp24@po.cwru.edu

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D004452 Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Echocardiography, Contrast,Echocardiography, Cross-Sectional,Echocardiography, M-Mode,Echocardiography, Transthoracic,Echocardiography, Two-Dimensional,Transthoracic Echocardiography,2-D Echocardiography,2D Echocardiography,Contrast Echocardiography,Cross-Sectional Echocardiography,Echocardiography, 2-D,Echocardiography, 2D,M-Mode Echocardiography,Two-Dimensional Echocardiography,2 D Echocardiography,Cross Sectional Echocardiography,Echocardiography, 2 D,Echocardiography, Cross Sectional,Echocardiography, M Mode,Echocardiography, Two Dimensional,M Mode Echocardiography,Two Dimensional Echocardiography
D006328 Cardiac Catheterization Procedures in which placement of CARDIAC CATHETERS is performed for therapeutic or diagnostic procedures. Catheterization, Cardiac,Catheterization, Heart,Heart Catheterization,Cardiac Catheterizations,Catheterizations, Cardiac,Catheterizations, Heart,Heart Catheterizations
D006346 Heart Septum This structure includes the thin muscular atrial septum between the two HEART ATRIA, and the thick muscular ventricular septum between the two HEART VENTRICLES. Cardiac Septum,Heart Septa,Septa, Heart,Septum, Cardiac,Septum, Heart
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D014188 Transposition of Great Vessels A congenital cardiovascular malformation in which the AORTA arises entirely from the RIGHT VENTRICLE, and the PULMONARY ARTERY arises from the LEFT VENTRICLE. Consequently, the pulmonary and the systemic circulations are parallel and not sequential, so that the venous return from the peripheral circulation is re-circulated by the right ventricle via aorta to the systemic circulation without being oxygenated in the lungs. This is a potentially lethal form of heart disease in newborns and infants. Dextro-TGA,Dextrotransposition of Great Vessels,Levo-Looped Transposition of the Great Arteries,Levo-TGA,Levotransposition of Great Vessels,Dextro-Looped Transposition of the Great Arteries,Transposition of Great Arteries,Dextro Looped Transposition of the Great Arteries,Dextro TGA,Dextro-TGAs,Great Arteries Transposition,Great Arteries Transpositions,Great Vessels Dextrotransposition,Great Vessels Dextrotranspositions,Great Vessels Levotransposition,Great Vessels Levotranspositions,Great Vessels Transposition,Great Vessels Transpositions,Levo Looped Transposition of the Great Arteries,Levo TGA,Levo-TGAs
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D018633 Pulmonary Atresia A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. Clinical features include rapid breathing, CYANOSIS, right ventricle atrophy, and abnormal heart sounds (HEART MURMURS). Atresia, Pulmonary,Pulmonary Valve Atresia,Atresia, Pulmonary Valve,Atresias, Pulmonary,Atresias, Pulmonary Valve,Pulmonary Atresias,Pulmonary Valve Atresias,Valve Atresia, Pulmonary,Valve Atresias, Pulmonary

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