A Scimitar Syndrome Variant: Anomalous Systemic Arterial Supply to the Right Lung With Normal Pulmonary Venous Return Luis Gorospe, MD, Amparo Esteban-Peris, MD, Alberto Caba~ nero-S anchez, MD, and Gemma María Mu~ noz-Molina, MD, PhD Departments of Radiology and Thoracic Surgery, Ram on y Cajal University Hospital, Madrid, Spain
Fig 1.
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Fig 4. Fig 3.
n asymptomatic 23-year-old woman underwent computed tomography after a chest roentgenogram that was performed because of a positive tuberculin test result and showed a small right lung volume and dextroposition of the heart (Fig 1). Physical examination was unremarkable. Her medical history was significant for unilateral renal agenesis and an L5 hemivertebra. Thoracic computed tomography showed a combination of lung and vascular congenital anomalies. First, the right lung was hypoplastic, the middle lobe was absent, and there were no fissures in the right hemithorax. Second, the right pulmonary artery appeared small (Fig 2) and vascularized the upper two thirds of the right lung. There was a large systemic Address correspondence to Dr Gorospe, Department of Radiology, Ram on y Cajal University Hospital, Ctra. de Colmenar Viejo Km 9.100, 28034 Madrid, Spain; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
arterial supply to the basal segments of the right lung that originated from the abdominal aorta, without associated pulmonary sequestration (Fig 3, arrows). Pulmonary venous return was directed into the left atrium through a single common pulmonary vein on both sides (Fig 4, asterisks). Because the patient was asymptomatic, we opted for clinical follow-up. Anomalous systemic arterialization of the lung without pulmonary sequestration is the rarest form of anomalous systemic arterial supply to the lung and, unlike our patient, more commonly involves the left lung. The anomalous systemic artery usually arises from the thoracic aorta, although in our patient it originated from the abdominal aorta. Our case represents a rare variant of the scimitar syndrome with “normal” pulmonary venous return to the left atrium associated with developmental anomalies of the bronchial tree and lung parenchyma.
Ann Thorac Surg 2015;99:339 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.081
FEATURE ARTICLES
A
Fig 1.
Fig 2.
Fig 4. Fig 3.
n asymptomatic 23-year-old woman underwent computed tomography after a chest roentgenogram that was performed because of a positive tuberculin test result and showed a small right lung volume and dextroposition of the heart (Fig 1). Physical examination was unremarkable. Her medical history was significant for unilateral renal agenesis and an L5 hemivertebra. Thoracic computed tomography showed a combination of lung and vascular congenital anomalies. First, the right lung was hypoplastic, the middle lobe was absent, and there were no fissures in the right hemithorax. Second, the right pulmonary artery appeared small (Fig 2) and vascularized the upper two thirds of the right lung. There was a large systemic Address correspondence to Dr Gorospe, Department of Radiology, Ram on y Cajal University Hospital, Ctra. de Colmenar Viejo Km 9.100, 28034 Madrid, Spain; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
arterial supply to the basal segments of the right lung that originated from the abdominal aorta, without associated pulmonary sequestration (Fig 3, arrows). Pulmonary venous return was directed into the left atrium through a single common pulmonary vein on both sides (Fig 4, asterisks). Because the patient was asymptomatic, we opted for clinical follow-up. Anomalous systemic arterialization of the lung without pulmonary sequestration is the rarest form of anomalous systemic arterial supply to the lung and, unlike our patient, more commonly involves the left lung. The anomalous systemic artery usually arises from the thoracic aorta, although in our patient it originated from the abdominal aorta. Our case represents a rare variant of the scimitar syndrome with “normal” pulmonary venous return to the left atrium associated with developmental anomalies of the bronchial tree and lung parenchyma.
Ann Thorac Surg 2015;99:339 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.08.081
FEATURE ARTICLES
A
