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Chronic thromboembolic pulmonary hypertension. Clinical characterization, hemodynamics, and survival

Julio Sandoval, Arturo Gómez, Andrés Palomar, Guillermo Cueto, María Luisa Martínez-Guerra, Efrén Santos





2003, Number 2

2003; 62 (2)






ABSTRACT

Background: Although average survival in chronic, major vessel, thromboembolic pulmonary hypertension (CTPH) has been estimated in the vicinity of 2.5 to 3 years from diagnosis, the natural history of patients has not been clearly elucidated. The aim of the present study is to characterize mortality in a Mexican population of patients with CTPH and to investigate factors associated with their survival. Methods: Retrospective clinical review of a dynamic cohort of fifteen patients with CTPH, mean (SD) age of 48 (16) years, enrolled between 1979 and 1989 and followed through May 1994 in a tertiary referral center. Diagnosis of CTPH was established in the basis of: 1) demonstration of pulmonary arterial hypertension and cor pulmonale, 2) no evidence of primary heart and/or lung disease, 3) the presence of lobar or at least segmental defect (s) in the lung scan, 4) confirmation of perfusion defects by pulmonary angiogram. Measurements included hemodynamic and functional variables in addition to information on demographic data and medical history. All these patients received long-term oral anticoagulation for treatment. As an index for determining survival we used the initial diagnostic catheterization. Results: Mean (SD) baseline hemodynamic values were: mean pulmonary artery pressure 6.93 (2.13) kPa; cardiac index 3.09 ± 0.81 L•min-1•m2, and calculated pulmonary vascular resistance of 921 dynes•sec•cm5. The right ventricular end-diastolic pressure was 1.73 (0.79) kPa. The estimated median survival of the group was 2.16 years (95% CIs; 0.08 to 5.91 years) which is statistically different from the 3.12 years (95% CIs; 0.5 to 13.25 years) median survival of adult patients with primary pulmonary hypertension also followed at our institution (Chi2 log rank = 10.03, p < 0.0015). Except for female gender (hazard ratio: 0.08), and a decreased arterial oxygen content (hazard ratio: 13.9), none of the other demographic, clinical, functional, and hemodynamic variables was significantly associated to mortality (Cox’s proportional hazards model). Conclusions: Despite medical treatment, patients with CTPH have a poor survival expectancy which appears to be worse than that of adult patients with primary pulmonary hypertension.

KEYWORDS

Circulación pulmonar, hipertensión arterial pulmonar, tromboembolia pulmonar crónica.

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2003, Number 2

2003; 62 (2)