Unilateral Pulmonary Vein Stenosis with Life-threatening Hemoptysis: A case report / 대한흉부외과학회지

ABSTRACT

A case is described in a girl who presented with recurrent life-threatening hemoptysis at the age of 18 months, and had been diagnosed as atrial septal defect with severe cardiomegaly which was presumed to result in pulmonary vein stenosis at the age of 6 months. Closure of atrial septal defect was associated with decreased heart size and improved pulmonary venous flow. However, recurrent life-threatening hemoptysis occurred during follow-up. Computed tomography scan demonstrated left pulmonary vein stenosis and extrinsic compression of the left bronchus by multiple soft tissue density-masses. Exploratory thoracotomy revealed single stenotic left pulmonary vein, and flat left main bronchus compressed by multiple hypertrophied lymph nodes. Unexpected endotrachial tube bleeding during left hilar dissection mandated to proceed to left pneumonectomy. The patient's postoperative course was uneventful. Follow-up chest roentgenography revealed acceptable left hydrothorax without mediastinal shifting. Nevertheless, a long-term follow-up is necessary.