A 20-day-old female child born at term to a primigravida by normal vaginal hospital delivery was admitted in neonatal intensive care unit with respiratory distress. Immediate postnatal period was uneventful. The baby had severe tachypnea, tachycardia and chest retractions. These was no cyanosis and the heart sounds were audible on the left side of the chest with grade II systolic murmur in second left parasternal area. There was no evidence of any external congenital anomaly. Investigations revealed normal hemogram, X-ray chest showed density in the right paraspinal region along with medial inferior lung suggestive of sequestration of right lower lobe (Fig. 1), no cardiomegaly or dextroposed heart. Colour Doppler echocardiography revealed ostium secundum ASD with anomalous pulmonary venous drainage. CT scan of chest and abdomen with angiography showed complex cardiac anomaly with aorta draining into the right lung. The consolidated right lower lobe represented an area of sequestration. The patient underwent coil repair of collateral to relieve pulmonary hypertension with clinical improvement of symptoms.

Fig. 1. Sequestration of right lower lobe.

The Scimitar syndrome is a rare condition of cardiopulmonary anomalies(1) accounting for 0.5-1% of congenital heart disease. The incidence of the associated congenital cardiovascular abnormalities is 36% in pediatric age group and is highest (75%) among the neonates and include ASD, VSD, coarctation of aortic arch and abnormal relationship of the pulmonary arteries and bronchi(2). The age of presentation is variable. In a series of 32 patients over a period of 20 years, the median age at diagnosis was 7 months(3). Severe respiratory insufficiency is always present in symptomatic cases in early age group as Scimitar syndrome results in pulmonary hypertension, heart failure and right lung infection. In older children and adults, the diagnosis of Scimitar syndrome is often made incidentally who undergo chest radiography for diverse reasons. Recurrent respiratory infections and heart murmur may be the mode of presentation in them. Children who are diagnosed with Scimitar syndrome after infancy have fewer associated defects and less pulmonary hypertension.

Treatment for symptomatic Scimatar syndrome consists of surgical repair. Repair of the anomalous venous return and ligation of colloaterals is generally recommended, although right pneumonectomy also provides similar results(4).

Our patient responded for 6 weeks but then deteriorated and therefore was referred to cardio-thoracic center in Mumbai for further surgical management.

S.S. Kashyape,
A.M. Tilak,
Shree Medical Research Center,
c/o. Kashyape Children Hospital,
Opp. Hotel Mazda, Trimbak Naka,
Nasik 422 002, Maharashtra,
India.

1. Canter CE, Martin TC, Spray TL, Weldon CS, Strauss AW. Scimitar syndrome in childhood. Am J Cardiol 1986; 58: 652.

2. Gilonyo DK, Tandon R, Lucas RV Jr, Edwards JE. Scimitar syndrome in neonates: report of four cases and review of the literature. Pediatr Cardiol 1986; 6: 193-197.

3. Najm HK, Williams WG, Coles JG, Rebeyka IM. Freedom RM. Scimitar syndrome: twenty year’s experience and results of repair. J Thorac Cardiovasc Surg 1996; 112: 1161-1168.

4. Huddleston CB, Exil V, Canter CE, Mendeloff En. Scimitar syndrome presenting in infancy. Ann Thorac Surg 1999; 67: 154-159.