Aust. Paediatr. J. zyxwvutsrqp (1 985) zyxwvutsr 21, 279-280 zyxwvutsr Pulmonary sequestration in a newborn mimicking cardiac disease: A trap for diagnosis GARY F. SHOLLER, CHRISTOPHER M. WHIGHT and GRAHAM R. NUNN Adolph Basser Institute of Cardiology, The Childrens Hospital, Camperdown, Sydney Abstract A case of pulmonary sequestration in a neonate presenting with hyperdynamic circulation and a murmur is reported. Clinical, radiographic and echocardiographic data suggested the diagnosis and this was confirmed by angiography. The surgical findings were those of sequestration of the right lower lobe, in conjunction with an accessory right lung complete with bronchial and vascular supply arising from the region of the lower oesophagus. Pulmonary sequestration may mimic primary cardiovascular disease in the neonate. Key words: cardiac failure; congenital heart disease; infancy; pulmonary sequestration. Pulmonary sequestration (PS) is a condition where there is a segment (or segments) of lung whose tissue is dislocated from the bronchial system of normal lung segments, and which receives its arterial supply from the systemic circulation.'** The diagnosis is made mainly in young adults with respiratory symptom^,^ but presentation may occur at any age and includes detection of murmurs and cardiac failure in the neonate.435 This report describes a neonate who presented following detection of a cardiac murmur. CASE REPORT A boy, KL, was born at 36 weeks gestation following a normal pregnancy and delivery. His birthweight was 2.35 kg. At the age of 4 days he was noted to have a systolic murmur and hyper- dynamic pulses, and the diagnosis of persistent ductus arteriosus was made. At 5 weeks of age he presented to the Children's Hospital, Camperdown, with a history of cyanotic episodes (often associated with feeding) and rapid shallow respirations. He had brisk pulses and a grade 216 systolic ejection murmur, maximal at the upper left sternal edge and in the back. The second heart sound was normal. Chest X-ray (Fig. 1) showed the heart size and pulmonary vascularity to be normal. A right lower lobe opacity was present. Barium swallow and meal revealed moderate gastro-oesophageal reflux. Two-dimensional, doppler echocardiography demonstrated an enlarged left atrium and overactive left ventricle with otherwise normal intracardiac anatomy and no evidence of a persistent ductus arteriosus. A provisional diagnosis of pulmonary sequestration was made and this was confirmed by cardiac catheterization and angiography. A large supradiaphragmatic systemic vessel arose from the descending aorta to supply the lower portion of right lung, while pulmonary venous drainage Reprints: Dr G. F. Sholler. Adolph Basser lnstttute of Cardiology, The Childrens Hospital. Camperdown 2050, Sydney, Australia. G F. Sholler. MB, BS, FRACP, Fellow in PaediatricCardiology. C. M. Whight, MB. BS. FRACP. Cardiologist. G. R. Nunn, MB. BS. FRACS. Visiting Cardiac Surgeon. Received 4 March 1985 was normal (Fig. 2). There was no pulmonary hypertension or left to right shunt. At operation the whole of the right lower lobe, with the exception of a small area anteriorly, was non-aerated. The oblique and transverse fissures were normally developed and a 6 mm diameter systemic artery entered this lower lobe through the pulmonary ligament. This vessel arose from the descending aorta at the level of the twelfth thoracic vertebra. Pulmonary venous drainage from the lobe was zyx to the lower right pulmonary vein, which drained to the left atrium. The bronchial anatomy at the level of the oblique fissure appeared normal although intralobar bronchial anatomy was not deter- mined at the time of surgery. In addition, an accessory lung measuring 2.5 cmx2.0 cm lay in the right paravertebral gutter at the level of the eleventh thoracic vertebra; this had a well developed oblique fissure and an investing pleura with vascular structures entering a normally placed hilum. This was the only Fig. 1 Chest radiograph with right lower lobe opacity