Long-term outcome after nonsurgical management of Paget-Schroetter syndrome

Paget-Schroetter syndrome (PSS) is an uncommon disease with potentially debilitating long-term effects. The optimal therapy for PSS is unclear, and the role of surgical decompression of the thoracic outlet is still being questioned. In this study, we present long-term results of patients treated with catheter-directed thrombolysis (CDT) and anticoagulation without surgical management.

This is a retrospective case series of all patients who previously underwent treatment of PSS in our institution between the years 2007 and 2019. Patients were evaluated for clinical signs of post-thrombotic syndrome (PTS) using a modified Villalta scoring scale, including measurements of the circumference of the treated and untreated arms. Duplex ultrasound examination of the treated vein was performed, and quality of life was evaluated using the shortened Disabilities of the Arm, Shoulder, and Hand questionnaire.

Eighteen consecutive patients previously treated for PSS with CDT and anticoagulation compose the cohort of this study. None underwent surgical thoracic outlet decompression. All were contacted and invited for clinical and ultrasound evaluation. Follow-up was available for all patients. Mean age at diagnosis was 29 years (range, 16-46 years), and 15 (79%) were male. Mean time from the index event to the follow-up clinic visit was 109 months (range, 37-176 months). Patients were treated with anticoagulation for a mean period of 26 months (range, 6-120 months). Seventeen patients (94%) had a Villalta score of 0 to 3, consistent with nonexistence of PTS. Fourteen patients (78%) were completely asymptomatic. Seven patients (39%) had no difference in arm circumference. A difference in arm circumference between the treated arm and the healthy arm of 1 cm and 2 cm was seen in nine (50%) and two (11%) patients, respectively. Based on the shortened Disabilities of the Arm, Shoulder, and Hand score, none of the patients suffered from impaired quality of life. Duplex ultrasound scanning of the affected veins was performed on 16 of the 18 patients (89%). The vein appeared patent in all examined patients. In three patients, the wall of the examined vein was thickened and irregular.

This study suggests that PSS patients can be treated with anticoagulation and CDT alone, without the need for surgical thoracic outlet decompression. This is based on long-term follow-up of these patients objectively evaluated by means of valid scoring systems. These findings suggest that symptoms or signs of PTS rarely develop, the patients do not suffer from impaired quality of life, and patency of the diseased vein is commonly maintained.