Life-threatening haemoptysis due to tracheal varices secondary to pulmonary vein obstruction

Abstract

A 30-year-old man with a 3 day history of increasing breathlessness and frank haemoptysis was suspected of having pulmonary embolism and anticoagulated with heparin. Haemoptysis worsened so anticoagulation was reversed and he was transferred to our institution.Pulmonary and bronchial angiography revealed a small right pulmonary artery and a prominent right intercostobronchial trunk which was embolised. However, haemoptysis recurred and a contrast enhanced ECG-gated CT scan confirmed a hypoplastic right pulmonary artery and showed thrombosed right pulmonary veins that had no communication to the left atrium, a 'bulky' right hilum and thickened bronchial walls with smooth mucosal indentations into the airway lumen (figures 1 and 2). Bronchoscopy confirmed carinal and right bronchial varices. A ventilation perfusion (V/Q) scan showed normal ventilation but very little perfusion of the right lung.The patient underwent a standard dissectional stapled right pneumonectomy. Intraoperatively, the right main bronchus and distal trachea were encircled by bronchial veins which were the only means by which the lung could drain as the native pulmonary veins had no discernable flow. In addition there were a number of venous collaterals to the chest wall. Postoperatively the patient had no further haemoptysis and repeat bronchoscopy showed resolution of the varices.Histopathology showed thrombosed pulmonary veins with signs of recanalisation and a dilated bronchial venous plexus involving the hilar bronchial submucosa. There was an acquired moderate degree of chronic inflammation involving the pulmonary artery wall causing stenosis. DISCUSSIONTracheal varices from pulmonary venous occlusive disease may cause massive haemoptysis.1 Cases with similar findings to ours have been reported but with a different aetiology; idiopathic hilar fibrosis causing pulmonary vein occlusion. 2In our patient there was no hilar fibrosis and the aetiology of the pulmonary venous thrombosis remains unclear. The prominent collateral circulation within the walls of the airways were tracheal varices caused by pulmonary venous thrombosis. Learning point▸ Massive haemoptysis can occur secondary to non-embolic pathologies and this should be considered in young patients with no risk factors for pulmonary embolism in whom early systemic anticoagulation may be life threatening.

Cardiac catheterization for hemoptysis in a Children’s Hospital Cardiac Catheterization Laboratory: A 15 year experience

“…All these three patients were treated with transcatheter or surgical therapy, resulting in no recurrence. In the literature, severe hemoptysis has been reported in patients with PVO [16,17]. Bronchial and pulmonary venous varices due to the extensive collaterals between the pulmonary and bronchial circulation are postulated to predispose to the occurrence of hemoptysis.…”

Selective pulmonary artery occlusion to treat hemoptysis associated with pulmonary venous obstruction

“…Pulmonary venous obstruction is a rare though serious condition with one collaborative study citing a 49% survival within three years of diagnosis [1] . Several case reports describe life-threatening hemoptysis in patients with pulmonary venous obstruction and bronchial or tracheal varices [2] , [3] , [4] . Surgical reconstruction of pulmonary venous drainage, anticoagulation for an occluding thrombus, and pneumonectomy have been described as options for hemoptysis in this setting [3] , [4] , [5] .…”

“…Several case reports describe life-threatening hemoptysis in patients with pulmonary venous obstruction and bronchial or tracheal varices [2] , [3] , [4] . Surgical reconstruction of pulmonary venous drainage, anticoagulation for an occluding thrombus, and pneumonectomy have been described as options for hemoptysis in this setting [3] , [4] , [5] . Here we report the outcome of catheter-mediated interventions in an individual with refractory hemoptysis associated with severe stenosis of the left upper pulmonary veins and occlusion of the left lower pulmonary veins.…”