Severe Pulmonary Vein Stenosis Presenting With Hemoptysis

Abstract

INTRODUCTION: Pulmonary vein stenosis (PVS) is an uncommon cause of hemoptysis. We report a case of severe pulmonary vein stenosis in a patient who had undergone catheter ablation for atrial fibrillation who later presented with hemoptysis. CASE PRESENTATION: A 51-year-old man was referred for evaluation of sudden onset daily hemoptysis, dyspnea and progressive anemia. He had no fever, chills, night sweats or weight loss. Medical history included positive tuberculin test and atrial fibrillation for which he had undergone three catheter ablation procedures, latest 8 months ago. At presentation he was in no acute distress, afebrile, and vital signs were stable. His physical examination was unremarkable. He was admitted to the hospital and subsequently underwent bronchoscopy which showed irregular mucosa in the anterolateral wall of the left upper bronchus and ongoing active oozing. Endobronchial biopsies of the irregular mucosa showed reactive follicles and germinal center but no malignancy and cultures (bacterial, mycobacterial and fungal) were negative. Contrast-enhanced computed tomography (CT) of the chest showed non visualization and near complete occlusion of the superior pulmonary veins. Quantitative perfusion scan showed markedly decreased perfusion in the left upper lobe. Cardiac catherization was performed via transeptal approach. Angiogram revealed severe stenosis of the left superior pulmonary vein. Balloon dilatation followed by stent placement was performed. His hemoptysis resolved and he was discharged home. DISCUSSION: Pulmonary vein stenosis (PVS) is a relatively rare condition and can be congenital or acquired. Congenital PVS has been proposed to be secondary to abnormal incorporation of the common pulmonary vein into the left atrium in the later stages of cardiac development. Affected patients most often become symptomatic in the first few months to years of life and frequently have one or more additional cardiac anomalies (30-80%). The most commonly associated congenital heart defects are septal defects. The most common cause of acquired PVS in adult patients is radiofrequency ablation procedures for treatment of atrial fibrillation. PVS secondary to neoplasm growth, sarcoidosis, or fibrosing mediastinitis has been reported. The frequency of PVS post ablation procedure varies and had been reported to be as high as 42.4% in small early case series but recently due to improved technique and improved experience reported to be as low as 1.3 %. Patients can be asymptomatic, but more extensive and severe stenosis may cause dyspnea, hemoptysis, cough or chest pain. Due to the variability of symptoms diagnosis can be missed. Chest radiographs may show oligemia but this is not diagnostic. Quantitative perfusion scan is useful in assessing the extent of flow in the distribution of PVS. CT angiography and magnetic resonance imaging can provide accurate location and extent of stenosis. Suboptimal angiography can often overestimate the severity. Untreated congenital PVS carries a poor prognosis. Repair of PVS has low success rates and patients usually require multiple repairs. Even with newer techniques the freedom from reoperation or death at 5 years is still only 50 %. Pneumonectomy may be required for patients with massive hemoptysis. In patients with severe pulmonary hypertension, lung transplantation has been successful. Acquired PVS has been treated almost exclusively by transcatheter techniques. Balloon angioplasty of the involved vessels usually leads to a reasonably good initial result but restenosis is reported in more than 50% of patients within 1 year. Stent implantation has been associated with better success rates. CONCLUSIONS: Clinicians should be aware of the possibility of PVS as a cause of hemoptysis in a patient who has had ablation procedure for atrial fibrillation.