Eleven patients died during the first year of follow-up. Seven patients had primary pulmonary hypertension, and the remaining patients had either congenital heart disease (n = 3) or collagen vascular disease (n = 1). Survival was not influenced by patient age, sex, or mean pulmonary arterial pressure (Tables 1 and 2). However, the proportion of patients with primary pulmonary hypertension who died in the interval was significantly higher than that for patients with secondary pulmonary hypertension (p < 0.005, Table 1). In addition, the nonsurvivors had higher vWF:Ag values at the beginning of the follow-up period than the survivors, and this was highly significant (p < 0.0001, Table 2). In the subgroup of patients with secondary pulmonary hypertension, vWF:Ag was significantly higher in the nonsurvivors than in the survivors (247.5 ± 78.7% and 124.8 ± 58.3% activity, respectively; p < 0.05). In patients with primary pulmonary hypertension, a clear trend toward higher v\VF:Ag levels for the nonsurvivors also was observed (261.8 ± 94.6% activity for nonsurvivors vs 177.2 ± 49.7% activity for survivors). Thus, vWF:Ag values were similar for the nonsurviving patients from both subgroups (p = NS). Buy dexone online read more For the whole group of 40 patients, a vWF:Ag of > 240% (p = 0.003) was 54% sensitive for and 93% specific for identifying patients who were unlikely to survive 1 year, with an overall predictive value of 75%. The influence of plasma vWF:Ag levels on the likelihood of fatal outcome during the first year of follow-up in patients with primary and secondary pulmonary hypertension is shown in Figure 1. Multivariate analysis showed that the etiology of pulmonary hypertension (ie, primary vs secondary form) and plasma vWF:Ag levels influenced patient outcomes independently. The equation that represents the probability of death as a function of initial vWF:Ag values was:

Eleven patients died during the first year of follow-up. Seven patients had primary pulmonary hypertension, and the remaining patients had either congenital heart disease (n = 3) or collagen vascular disease (n = 1). Survival was not influenced by patient age, sex, or mean pulmonary arterial pressure (Tables 1 and 2). However, the proportion of patients with primary pulmonary hypertension who died in the interval was significantly higher than that for patients with secondary pulmonary hypertension (p < 0.005, Table 1). In addition, the nonsurvivors had higher vWF:Ag values at the beginning of the follow-up period than the survivors, and this was highly significant (p < 0.0001, Table 2). In the subgroup of patients with secondary pulmonary hypertension, vWF:Ag was significantly higher in the nonsurvivors than in the survivors (247.5 ± 78.7% and 124.8 ± 58.3% activity, respectively; p < 0.05). In patients with primary pulmonary hypertension, a clear trend toward higher v\VF:Ag levels for the nonsurvivors also was observed (261.8 ± 94.6% activity for nonsurvivors vs 177.2 ± 49.7% activity for survivors). Thus, vWF:Ag values were similar for the nonsurviving patients from both subgroups (p = NS). For the whole group of 40 patients, a vWF:Ag of > 240% (p = 0.003) was 54% sensitive for and 93% specific for identifying patients who were unlikely to survive 1 year, with an overall predictive value of 75%. The influence of plasma vWF:Ag levels on the likelihood of fatal outcome during the first year of follow-up in patients with primary and secondary pulmonary hypertension is shown in Figure 1. Multivariate analysis showed that the etiology of pulmonary hypertension (ie, primary vs secondary form) and plasma vWF:Ag levels influenced patient outcomes independently. The equation that represents the probability of death as a function of initial vWF:Ag values was:  where P and S are 0 and 1, respectively, for primary and secondary pulmonary hypertension.

where P and S are 0 and 1, respectively, for primary and secondary pulmonary hypertension.

Table 1—Correlation of Sex, Age, and Etiology of Pulmonary Hypertension With 1-Year Survival

Variable Category- Deceased Alive p> x2 p, Fisher
Sex Male 4 7
(%) (36.36) (63.64) 0.439 0.455
Female 7 22
(%) (24.14) (75.68)
Age (yr) <14 3 8
(%) (27.27) (72.73) 0.984 1.000
>14 8 21
(%) (27.59) (72.41)
Etiology of pulmonary hypertension PPH* 7 4
(%) (63.64) (36.36) 0.002 0.004
SPH 4 25
(%) (13.79) (86.21)
Total 11 29

Table 2—Correlation of Age, Mean Pulmonary Arterial Pressure, and vWF:Ag with 1-Year Survival in Pulmonary Hypertension

Variable Status at 1 yr n Mean ± SD Range P(t test)
Age Deceased 11 26.4 ± 13.7 3-43 0.2686
Alive 29 21.6 ± 11.5 1.2-45
Mean PAP* (mm Hg) Deceased 11 70.3 ± 25.2 30-112 0.4720
Alive 29 64.8 ± 19.9 27-105
vWFAg (% activity) Deceased 11 256.6 ± 85.3 141-379 <0.0001
Alive 29 132.0 ± 59.3 56-244

 

Figure 1. The likelihood of fatal outcome in pulmonary hypertension as a function of plasma antigenic activity of vWF. Parameters for curve fitting were estimated by logistic regression analysis of data obtained from 11 primary (P) and 29 secondary (S) pulmonary hypertensive patients. The slope corresponding to the influence of vWF:Ag on the probability of death was 0.02 ± 0.01 (estimate, ± SEE; p = 0.0087). The odds ratio associated with vWF:Ag values was 1.022 with a 95% Cl of 1.01 to 1.04.

Figure 1. The likelihood of fatal outcome in pulmonary hypertension as a function of plasma antigenic activity of vWF. Parameters for curve fitting were estimated by logistic regression analysis of data obtained from 11 primary (P) and 29 secondary (S) pulmonary hypertensive patients. The slope corresponding to the influence of vWF:Ag on the probability of death was 0.02 ± 0.01 (estimate, ± SEE; p = 0.0087). The odds ratio associated with vWF:Ag values was 1.022 with a 95% Cl of 1.01 to 1.04.