A case of pulmonary stenosis due to extrinsic compression of the pulmonary artery and right ventricular infundibulum is reported. It is believed that this is the first such case recorded in the English literature where the cause of the compression was a bronchiogenic cyst.Pulmonary stenosis due to compression of the right ventricular outflow tract by an extrinsic mass is an uncommon but well-documented event. The recorded causes include lymphoma, teratoma, thymoma, bronchial carcinoma, pericardial sarcoma, and aneurysm of the ascending aorta (Gough, Gold, and Gibson, 1967;Seymour, Emmanuel, and Pattinson, 1968;Littler, Meade, and Hamilton, 1970).The case reported here appears to be the first in the English literature in which the cause of the compression was a bronchiogenic cyst.
CASE REPORTThe patient was a 3-year-old girl who was referred from Barbados to the University Hospital of the West Indies, Jamaica on 29 October 1970 for investigation of suspected pulmonary valve stenosis. She was free of symptoms but a chest radiograph taken at the time of a respiratory tract infection had revealed a large intrathoracic opacity. The antenatal and obstetric history was normal.Physical examination showed that she was small for her age but alert and lively. A left-sided praecordial bulge was present and the apex beat was in the left fifth intercostal space on the nipple line. A systolic thrill was palpable along the left sternal border and there was an ejection systolic murmur which was loudest in the third and fourth intercostal spaces just to the left of the sternum. Signs of cardiac failure were absent. There was a dull percussion note and absence of breath sounds over the upper half of the left side of the chest anteriorly. No other abnormal physical signs were found.INVESTIGATIONS Chest radiographs (Fig. 1) (Fig. 2). Angiocardiograms showed that the infundibulum, the main pulmonary artery, and left pulmonary artery were all pushed inferiorly and to the left by a large, avascular mass in the position of the left upper lobe (Fig. 3).OPERATION A large cystic mass was removed by median sternotomy on 9 November 1970. The mass was situated in the superior anterior mediastinum, extending inferiorly in front of the right ventricle and laterally to fill the upper third of the left hemithorax. It occupied the bay between the ascending aorta and the main pulmonary artery, compressing the latter and the adjacent infundibulum of the right ventricle. The right ventricle was hypertrophied proximal to the site of compression.The mass was found to be extrapleural and extrapericardial and showed no evidence that it infiltrated adjacent tissues. It was not connected to the trachea or bronchi. After aspiration of 50 ml of thick white mucus, it was removed completely. A withdrawal pressure tracing across the right ventricular outflow tract after removal of the mass showed a residual, but greatly reduced, systolic pressure gradient of 20 mmHg. PATHOLOGY The excised specimen consisted of a cystic mass (8 x 5 x 3 cm; weight 70 g) with a sm...