Scimitar syndrome in an infant with right lung hypoplasia, ventricular septal defect, and severe pulmonary hypertension

A 5-month-old boy was presented for surgical repair of scimitar syndrome associated with right lung hyperplasia, severe pulmonary hypertension, ventricular septal defect (VSD), and atrial septal defect. The calculated shunt fraction (Qp/Qs) was 3.1:1.0, pulmonary vascular resistance was 4.6, and the...

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Bibliographic Details
Published in:General thoracic and cardiovascular surgery 2010-10, Vol.58 (10), p.524-527
Main Authors: Shinohara, Gen, Morita, Kiyozo, Uno, Yoshimasa, Yamashiro, Masahito, Hashimoto, Kazuhiro
Format: Article
Language:English
Subjects:
Abnormalities, Multiple
Azygos Vein - transplantation
Blood Pressure
Cardiac Catheterization
Cardiac Surgery
Cardiology
Case Report
Heart Septal Defects, Atrial - complications
Heart Septal Defects, Atrial - surgery
Heart Septal Defects, Ventricular - complications
Heart Septal Defects, Ventricular - physiopathology
Heart Septal Defects, Ventricular - surgery
Humans
Hypertension, Pulmonary - etiology
Hypertension, Pulmonary - physiopathology
Hypertension, Pulmonary - surgery
Infant
Lung - abnormalities
Lung - blood supply
Male
Medicine
Medicine & Public Health
Perfusion Imaging
Pulmonary Circulation
Pulmonary Veno-Occlusive Disease - etiology
Scimitar Syndrome - complications
Scimitar Syndrome - physiopathology
Scimitar Syndrome - surgery
Surgical Oncology
Thoracic Surgery
Treatment Outcome
Vascular Grafting - adverse effects
Vascular Resistance
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Summary:A 5-month-old boy was presented for surgical repair of scimitar syndrome associated with right lung hyperplasia, severe pulmonary hypertension, ventricular septal defect (VSD), and atrial septal defect. The calculated shunt fraction (Qp/Qs) was 3.1:1.0, pulmonary vascular resistance was 4.6, and the perfusion lung scan showed a marked decrease (11%) in right pulmonary blood flow. Surgical repair was performed through the right fourth intercostal space with the patient supine. The anomalous vein was divided and interposed with a short azygos vein graft, followed by closure of the VSD. Finally, the interposed azygos vein was anastomosed to the left atrium. Although pulmonary artery pressure was normalized at the 2-year follow-up, cardiac catheterization 6 months after the operation demonstrated right pulmonary vein obstruction.
ISSN:1863-6705
1863-6713
DOI:10.1007/s11748-009-0567-1