Open AccessUnilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome
Author(s) -
Jane David,
Smilu Mohanlal,
Punam Sankhe,
Radha Ghildiyal
Publication year - 2016
Publication title -
lung india
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 25
eISSN - 0974-598X
pISSN - 0970-2113
DOI - 10.4103/0970-2113.188982
Subject(s) - medicine , right pulmonary artery , cardiology , pulmonary artery , ductus arteriosus , pulmonary hypertension , ventricle , lung , left pulmonary artery , scimitar syndrome , right to left shunt , radiology , patent foramen ovale , migraine
We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).