[Chronic thromboembolic pulmonary hypertension with and without antiphospholipid syndrome]

Chronic thromboembolic pulmonary hypertension (CTE-PH) is defined as the chronic obstruction by organized thrombi in pulmonary artery and their branches causing pulmonary hypertension. The objective is to evaluate features and outcome of CTE-PH in patients with and without coexisting antiphospholipid syndrome (APS). All patients studied at our Institution with CTE-PH between June 1993 and June 2005 were analyzed retrospectively. Sixteen out of 38 patients were APS positive (group A), and 22/38 patients (group B) disclosed normal results (n = 10) or other thrombophilic abnormalities (n =12).

both groups were similar in age (mean 41 vs. 50 years), NYHA class at diagnosis (81 vs. 100% in III-IV) and the presence of previous or coexistent thrombosis in other territories (31 vs. 27%). Sixty three percent of patients in group A and 55% of patients in group B underwent thromboendarterectomy. The patients were followed during an average of 45 months (0.5-144). At the end of follow up all the APS patients and all the chirurgical patients were in I-II functional class (p=0.053). The median survival from diagnosis was 59 months for group A and 27 months for group B (p=0.199). The mean survival of patients who underwent thromboendarterectomy was 56 months for group A vs. 33 months for group B (p=0.08). We conclude that patients with CTE-PH and APS disclosed a trend to a better survival than patients with CTE-PH without APS. Those patients with CTE-PH and APS who underwent thromboendarterectomy tended to achieve a better functional class and survival than those who received medical treatment.