Objective

Patients with hemoglobinopathies and hemolytic anemia constitute unique population predisposed to developing chronic thromboembolic pulmonary hypertension (CTEPH). Although pulmonary endarterectomy (PEA) is the best treatment for CTEPH, this surgery in these patients poses major practical challenges due to the need to cool on cardiopulmonary bypass (CPB). Only isolated reports exist. We present our experience as lessons are translatable to other surgery requiring CPB and hypothermia.

Methods

We performed a retrospective analysis, from our CTEPH database, of all patients who underwent PEA surgery and had abnormal hemoglobin or congenital hemolytic anemia. We reviewed diagnosis, exchange transfusions on cardiopulmonary bypass, preoperative and postoperative pulmonary hemodynamic and functional data and outcomes for this group. Paired data analysis was performed by Student’s T-test; p<0.05 was statistically significant.

Results

There were 19 patients, 11 with sickle cell diseases, 2 thalassemia and 6 with abnormal red cells. Mean age was 52±15 years. CPB time was 310±48mins, ischemic time was 64±18mins, circulatory arrest was 35±12m at 20degreesC. To reduce HbS to <20%, for safe PEA with deep hypothermic circulatory arrest, we used exchange blood transfusion, an average of 8.6±4 units. There was one postoperative death. Remaining 18 patients are alive at 3.4±3 years with improved hemodynamics and functional capacity.

Conclusion

Results of PEA in this complex patient group were satisfactory and similar to those in patients with normal haemoglobin and red cells. The presence of abnormal haemoglobin is not a contra-indication to PEA surgery. The strategy of exchange transfusion and CPB management was successful.