Plasma lipoprotein(a) levels in patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary artery hypertension is a common sequelae of a variety of cardiac and lung diseases. Pathogenesis of primary and secondary pulmonary artery hypertension is still debatable. METHODS AND RESULTS: We studied the serum lipoprotein(a) levels in patients with primary (n=27) and secondary (n=19) pulmonary artery hypertension (Eisenmenger syndrome). The results were compared with age and sex matched controls (n=46). We also studied the frequency of high levels of lipoprotein(a) (> 30 mg/dl) in pulmonary artery hypertension. Mean lipoprotein(a) levels were significantly higher in the pulmonary artery hypertension group compared to age- and sex-matched controls (31.60+/-15.49 mg/dl v. 14.66+/-14.7; p=0.0001). All patients were classified into two groups on the basis of their lipoprotein(a) levels (<30 mg/dl and >30 mg/dl). There was a higher frequency of lipoprotein(a) >30 mg/dl in patients of pulmonary artery hypertension v. controls (52% v. 24%; p= <0.001). Younger age, higher functional class, more severe congestive heart failure, shorter duration of symptoms. and more cases of hemoptysis were observed in the group with lipoprotein(a) >30 mg/dl. CONCLUSIONS: High lipoprotein(a) may be a marker and be associated with a more adverse prognosis in severe pulmonary artery hypertension. Larger prospective studies are needed to establish lipoprotein(a) as a risk factor for the development of pulmonary artery hypertension.

Plasma anticoagulant system in patients with pulmonary hypertension

Adults with pulmonary hypertension and polycythemia (N=22) have low levels of plasma antithrombin III (84 ñ 18 vs 98 ñ 13½ for controls, N=35, P<0.005) and protein C (66ñ21 vs 125 ñ 30%, N 8, P<0.0002 but normal levels of total protein S. Data are reported as means ñ SD and percent normal values obtained for pooled plasma from normal healthy adults. Children with the same disorder (N = 6) also had low protein C levels (66 ñ 16 vs 85 ñ 5½, P < 0.025). Total protein S was normal for children, but free protein S was decreased (66 ñ 13 vs 91 ñ 23,, P < 0.02). Since the levels observed in these patients are above those reported for congenital deficiencies, the reduction in plasma levels of anticoagulant proteins may be the result of cronic intravascular coagulation. Furthermore, normal levels of plasminogen and fibrin degradation products suggested a localized disorder or an acquired decrease in fibronolytic activity