Hypoplastic right lung associated with right pulmonary vein stenosis and systemic collateral.
We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidetector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities.
Khositseth
Anant
A
Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital Mahidol University, Bangkok, Thailand. alaks@diamond.mahidol.ac.th
Siripornpitak
Suvipaporn
S
Laohakunakorn
Preecha
P
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D006328:Cardiac Catheterization; D003097:Collateral Circulation; D003251:Constriction, Pathologic; D003914:Dextrocardia / Q000150:complications / Q000175:diagnosis; D018618:Echocardiography, Doppler, Color; D005260:Female; D006801:Humans; D007223:Infant; D008168:Lung / Q000002:abnormalities* / Q000098:blood supply*; D010690:Phlebography; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000503:physiopathology; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed