Abstract
Background: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are serious diseases with similar pathophysiologic aspects. The prognosis of patients with these conditions is highly uncertain, particularly incident cases. Methods: A Cox proportional hazards model was applied to a group of 85 patients (81% women, mean age 52 (18 - 82) years) with PAH (80%) and non-surgical CTEPH (20%) to evaluate risk factors for mortality. The following variables were included in the model: age, etiology, baseline 6-minute walk test (6 mWT), cardiac index, and improvement in the 6 mWT following initiation of first medical treatment. Results: In the multivariate analysis, the response to treatment, assessed by an improvement on the 6 mWT, was the most relevant prognostic factor in these patients (RR, 4.832 (95% CI, 1.888 - 12.364); p = 0.001). The remaining variables studied in this model had less influence on the prognosis: age > 50 years (RR, 0.744 (95% CI, 0.26 - 2.133); p = 0.582); etiology of connective tissue disease-associated PAH (RR, 3.145 (95% CI, 0.995-9.946); p = 0.051) or CTEPH (RR, 0.654 (95% CI, 0.179 - 2.387); p = 0.521) with respect to idiopathic PAH; baseline 6 mWT (RR, 1.173 (95% CI, 0.599 - 4.895); p = 0.315); or cardiac index (RR, 2.295 (95% CI, 0.793 - 6.642); p = 0.125). Conclusions: There is a high degree of uncer-tainty regarding the prognosis of PAH and CTEPH at the start of appropriate treatment. Our results support the idea that the initial treatment response is of paramount importance as prognostic factor in these patients.
Highlights
The incidence of pulmonary arterial hypertension (PAH) is estimated to be 1.1 cases per million [1], and its natural evolution leads to death at an average of 3 years from the time of the diagnosis [2]
The remaining variables studied in this model had less influence on the prognosis: age > 50 years (RR, 0.744; p = 0.582); etiology of connective tissue disease-associated Pulmonary arterial hypertension (PAH) (RR, 3.145; p = 0.051) or chronic thromboembolic pulmonary hypertension (CTEPH) (RR, 0.654; p = 0.521) with respect to idiopathic PAH; baseline 6-minute walk test (6 mWT) (RR, 1.173; p = 0.315); or cardiac index (RR, 2.295; p = 0.125)
This study attempts to clarify the factors predictive of the prognosis in PAH and in a subgroup of CTEPH patients who had an indication for medical treatment similar to those with PAH
Summary
The incidence of pulmonary arterial hypertension (PAH) is estimated to be 1.1 cases per million [1], and its natural evolution leads to death at an average of 3 years from the time of the diagnosis [2]. In non operable CTEPH patients, natural course of the disease is similar to that of PAH Because of these low incidences, it is difficult to assemble large groups of patients to better study their prognoses. The remaining variables studied in this model had less influence on the prognosis: age > 50 years (RR, 0.744 (95% CI, 0.26 - 2.133); p = 0.582); etiology of connective tissue disease-associated PAH (RR, 3.145 (95% CI, 0.995 - 9.946); p = 0.051) or CTEPH (RR, 0.654 (95% CI, 0.179 - 2.387); p = 0.521) with respect to idiopathic PAH; baseline 6 mWT (RR, 1.173 (95% CI, 0.599 4.895); p = 0.315); or cardiac index (RR, 2.295 (95% CI, 0.793 - 6.642); p = 0.125). Our results support the idea that the initial treatment response is of paramount importance as prognostic factor in these patients
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