[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).


RESULTS
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.
Colorio
Cecilia
C
Instituto de Cardiología y Cirugia Cardiovascular, Fundación Favaloro, Buenos Aires. ccolorio@intramed.net.ar
Rossi
Andrea
A
Tabares
Maria
M
Martinuzzo
Marta
M
Pombo
Gonzalo
G
Favaloro
Roberto
R
Forastiero
Ricardo
R
D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000032:analysis; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013923:Thromboembolism / Q000150:complications / Q000503:physiopathology* / Q000628:therapy
D017152:Antibodies, Antiphospholipid