Background: Strong arguments have been made both for and against anticoagulation for primary prevention in Eisenmenger syndrome, based on the known risk of hemoptysis and pulmonary artery thrombosis, though the issue has never been clinically studied.
Methods: After initial evaluation with laboratory, echocardiographic and hemodynamic data, anticoagulation in Eisenmenger patients was based on physician preference alone. We retrospectively identified patients who were started on anticoagulants (AC group) within 12 months of initial presentation and followed for at least 2 years, and compared them to non-anticoagulated patients (Non-AC).
Results: Out of 144 patients screened, We identified 48 anticoagulated and 44 non-anticoagulated patients with Eisenmenger physiology (mean PAP=73±19 mmHg, oxygen saturation 82±9%, PaO2 48±8 mmHg, hemoglobin 18.6±4 g/dL). There were more ASD patients in the AC group, but otherwise no baseline differences in clinical, functional, or hemodynamic data. After mean follow-up of 7±5.4 years (range 1–31), 11 patients died in the AC and 10 in the Non-AC group. There was no survival difference (log rank test = 1.78; p=NS). For the entire cohort, mortality was significantly associated with NYHA class 3-4 (HR=4.2), evidence of right heart failure (HR=13.6), and a mean corpuscular volume<80 µ3 (HR=3.8). Use of anticoagulation did not impact survival. Bleeding complications occurred in 7 (16%) of AC patients, including two fatalities.
Conclusion: Anticoagulation had no impact on long-term survival and was not benign. The data do not support the routine use of warfarin in these patients.