Double outlet right ventricle, atrioventricular canal defect with severe pulmonary artery stenosis: a rare cyanotic congenital heart disease. 2014

Shoukat Ali Baig, and Zara Saeed, and Arsala Fatima, and Hasina Chagani, and Sina Aziz

Double outlet right ventricle (DORV) is characterised by arousal of the aorta and the pulmonary vessel from the right ventricle, and is always accompanied by a ventricular septal defect (VSD). Our patient, a twenty days-old female child, presented to the Paediatrics Unit I of Civil Hospital, Karachi, with complaints of generalised cyanosis and reluctance to feed since birth. Apart from the fact that the neonate was cyanosed with increased respiration, the physical examination was unremarkable. Two-dimensional echocardiography revealed two atrial septal defects (ASD), a ventricular septal defect (VSD) in the membranous area, continuous with primum ASD, resulting in an atrioventricular canal defect. The aorta was dilated with the pulmonary artery stenosis. The left ventricle was rudimentary, and both of the great vessels were arising from the dominant right ventricle. Despite the rarity, DORV with complex anatomy should be considered among the probable differential diagnoses for infants presenting with generalised cyanosis since birth.

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
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
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004310 Double Outlet Right Ventricle Incomplete transposition of the great vessels in which both the AORTA and the PULMONARY ARTERY arise from the RIGHT VENTRICLE. The only outlet of the LEFT VENTRICLE is a large ventricular septal defect (VENTRICULAR SEPTAL DEFECTS or VSD). The various subtypes are classified by the location of the septal defect, such as subaortic, subpulmonary, or noncommitted. Taussig-Bing Anomaly,Double Outlet Right Ventricle, Noncommitted VSD,Double Outlet Right Ventricle, Subaortic VSD,Double Outlet Right Ventricle, Subpulmonary VSD,Double-Outlet Right Ventricle,Anomaly, Taussig-Bing,Double-Outlet Right Ventricles,Right Ventricle, Double-Outlet,Taussig Bing Anomaly,Ventricle, Double-Outlet Right
D004694 Endocardial Cushion Defects A spectrum of septal defects involving the ATRIAL SEPTUM; VENTRICULAR SEPTUM; and the atrioventricular valves (TRICUSPID VALVE; BICUSPID VALVE). These defects are due to incomplete growth and fusion of the ENDOCARDIAL CUSHIONS which are important in the formation of two atrioventricular canals, site of future atrioventricular valves. Persistent Common Atrioventricular Canal,Endocardial Cushion Defect,Cushion Defect, Endocardial,Cushion Defects, Endocardial,Defect, Endocardial Cushion,Defects, Endocardial Cushion
D005260 Female Females
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
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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

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