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Absent pulmonary valve or pulmonary atresia with intact ventricular septum: Which is it? 2020

Erin Nealon, and Darren Berman, and Daniel Rowland, and Brian Boe, and Eric Lloyd, and Clifford L Cua
Heart Center, Nationwide Children's Hospital, Columbus, OH, USA.

A fetal echocardiogram noted valvar pulmonary atresia vs critical pulmonary valve stenosis, confluent pulmonary arteries, patent ductus arteriosus, and an apical muscular ventricular septal defect vs coronary sinusoid. Postnatal echocardiogram documented an absent pulmonary valve, coronary artery fistula to the right ventricle, confluent pulmonary arteries, and a patent ductus arteriosus. Catheterization confirmed the postnatal echocardiographic findings except there was no antegrade flow from the right ventricle to the pulmonary arteries. Close evaluation of all imaging modalities revealed the final diagnosis of absent pulmonary valve with a double-chambered right ventricle with no egress from the right ventricle to pulmonary artery.

UI MeSH Term Description Entries
D011664 Pulmonary Valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Pulmonary Valves,Valve, Pulmonary,Valves, Pulmonary
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
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D054088 Ventricular Septum The muscular structure separating the right and the left lower chambers (HEART VENTRICLES) of the heart. The ventricular septum consists of a very small membranous portion just beneath the AORTIC VALVE, and a large thick muscular portion consisting of three sections including the inlet septum, the trabecular septum, and the outlet septum. Septum, Ventricular
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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