Successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance associated with dextrocardia and pulmonary stenosis. 1978

G K Danielson, and I F Tabry, and D G Ritter, and J D Maloney

The association of complete atrioventricular canal with other complex congenital cardiac anomalies has represented a significant challenge for the cardiac surgion. The combination of double-outlet right ventricle with complete atrioventricular canal has been particularly difficult to correct, with no surgical successes having been reported until recently. This is a report of the first successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance (ventricular incersion) associated with common atrium, bilateral superior venae cavae, dextrocardia, and pulmonary stenosis. The specialized conduction tissue was identified by intracardiac electrophysiological mapping, and normal sinus rhythm was preserved. Postoperative cardiac catheterization showed excellent hemodynamics. One year postoperatively, the patient was attending school, playing swimming without difficulty, and taking no cardiac medications. This good result lends encouragement for considering total repair for similar patients with the combination of double-outlet right ventricle, complete atrioventricular canal, and other associated congenital cardiac anomalies.

UI MeSH Term Description Entries
D011666 Pulmonary Valve Stenosis The pathologic narrowing of the orifice of the PULMONARY VALVE. This lesion restricts blood outflow from the RIGHT VENTRICLE to the PULMONARY ARTERY. When the trileaflet valve is fused into an imperforate membrane, the blockage is complete. Pulmonary Stenosis,Pulmonary Stenoses,Pulmonary Valve Stenoses,Pulmonic Stenosis,Stenoses, Pulmonary,Stenoses, Pulmonary Valve,Stenosis, Pulmonary,Stenosis, Pulmonary Valve,Valvular Pulmonic Stenosis,Pulmonary Stenose,Pulmonic Stenoses,Pulmonic Stenoses, Valvular,Pulmonic Stenosis, Valvular,Stenose, Pulmonary,Stenoses, Pulmonic,Stenosis, Pulmonic,Valvular Pulmonic Stenoses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003914 Dextrocardia A congenital defect in which the heart is located on the right side of the THORAX instead of on the left side (levocardia, the normal position). When dextrocardia is accompanied with inverted HEART ATRIA, a right-sided STOMACH, and a left-sided LIVER, the combination is called dextrocardia with SITUS INVERSUS. Dextrocardia may adversely affect other thoracic organs. Dextrocardias
D004562 Electrocardiography Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY. 12-Lead ECG,12-Lead EKG,12-Lead Electrocardiography,Cardiography,ECG,EKG,Electrocardiogram,Electrocardiograph,12 Lead ECG,12 Lead EKG,12 Lead Electrocardiography,12-Lead ECGs,12-Lead EKGs,12-Lead Electrocardiographies,Cardiographies,ECG, 12-Lead,EKG, 12-Lead,Electrocardiograms,Electrocardiographies, 12-Lead,Electrocardiographs,Electrocardiography, 12-Lead
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
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
D006330 Heart Defects, Congenital Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life. Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006343 Heart Septal Defects Abnormalities in any part of the HEART SEPTUM resulting in abnormal communication between the left and the right chambers of the heart. The abnormal blood flow inside the heart may be caused by defects in the ATRIAL SEPTUM, the VENTRICULAR SEPTUM, or both. Cardiac Septal Defect,Heart Septal Defect,Cardiac Septal Defects,Defect, Cardiac Septal,Defect, Heart Septal,Defects, Cardiac Septal,Defects, Heart Septal,Septal Defect, Cardiac,Septal Defect, Heart,Septal Defects, Cardiac,Septal Defects, Heart
D006352 Heart Ventricles The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation. Cardiac Ventricle,Cardiac Ventricles,Heart Ventricle,Left Ventricle,Right Ventricle,Left Ventricles,Right Ventricles,Ventricle, Cardiac,Ventricle, Heart,Ventricle, Left,Ventricle, Right,Ventricles, Cardiac,Ventricles, Heart,Ventricles, Left,Ventricles, Right

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