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ORIGINAL ARTICLE

Scimitar Syndrome: Experience With 6 Patients

Ranjith Baskar Karthekeyan, MD, Richard Saldanha, MCh, M Ranjith Sahadevan, MD, Suresh KG Rao, MD, Mahesh Vakamudi, MD, Balakrishnan K Rajagopal, MCh

Sri Ramachandra Medical College and Research Institute Porur, Chennai, India

Ranjith Baskar Karthekeyan, MD, Tel: +91 44 24986880, Fax: + 91 44 24769033, Email: ranjithb73{at}gmail.com, Department of Cardiac Anesthesiology, Sri Ramachandra Medical College and Research Institute, No. 1 Ramachandra Nagar, Porur, Chennai – 116, India.

ABSTRACT

Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous drainage to the inferior vena cava, causing a left-to-right shunt. Six patients with scimitar syndrome were diagnosed in our hospital between 2002 and 2008. There were 4 girls and 2 boys; 4 < 5 kg in weight, 2 < 8 kg in weight. Scimitar syndrome was suspected in 5 cases because of dextroversion, and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Computed tomography confirmed the diagnosis in all patients. Two patients had horseshoe lung, 2 had a unilobar right lung, 1 had a hypoplastic right lung, and 1 had a hypoplastic right lower lobe. Three patients had severe pulmonary arterial hypertension, 2 had moderate pulmonary arterial hypertension, and one had normal pulmonary arterial pressure. All patients had lower respiratory tract infections, volume loss of the right lung, a normal or hyperinflated left lung, dextroversion of the heart, and scimitar arteries from the descending aorta. Pneumonectomy was performed in 3 patients, lobectomy in 1, ligation of anomalous vessels in 1, and 1 died before surgery.

Key Words: Heart Defects • Congenital • Hypertension • Pulmonary • Scimitar Syndrome

Asian Cardiovasc Thorac Ann 2009; 17:266-271
© 2009 by SAGE Publications
DOI: 10.1177/0218492309104750




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