Clinical characteristics and survival of patients with pulmonary hypertension: a 40-month mean follow-up.

BACKGROUND AND AIMS: The aim of our study was to describe the characteristics and clinical outcomes of pulmonary hypertension (PH) patients who underwent their follow-up at a single centre. METHODS: We included patients who fulfilled the criteria for the diagnosis of PH by right heart catheterisation. A detailed clinical history and complete physical examination were recorded for each patient. Demographic data, laboratory testing, haemodynamic values and treatment records were also collected. A 6-min walk test was used to evaluate exercise capacity. Survival analysis was performed using the Kaplan-Meier method, and a log-rank test was used to compare survival curves among groups. A multivariate Cox's proportional hazards model was used in order to identify independent predictors of mortality in the overall PH population. RESULTS: A total of 93 patients (64 women and 29 men) were studied. The patients were distributed across the groups as follows: 50.5% in group 1, 20.4% in group 2, 9.7% in group 3, 14% in group 4 and 5.4% in group 5. The most common type of PH in our population was portopulmonary hypertension followed by PH associated with left heart disease, and idiopathic and chronic thromboembolic disease. Nineteen deaths, constituting 20.5% of the sample population, were reported. The Kaplan-Meier estimates of the 1-, 2- and 3-year survival rates were 91%, 89% and 85%, respectively. There were no statistically significant differences in survival among the groups (P = 0.18). A multivariate analysis revealed that the cardiac index (CI) was an independent predictor of death. CONCLUSIONS: The survival rates of PH patients are higher than previously reported. In our study, the largest number of deaths occurred in groups 1 and 2, and CI was found to be an independent predictor of mortality.

Home mechanical ventilation for restrictive thoracic diseases: effects on patient quality-of-life and hospitalizations.

Forty-five patients with restrictive respiratory diseases, including thoracic wall diseases (TWD, n = 27) and neuromuscular diseases (NMD, n = 18), underwent 18 months of home mechanical ventilation (HMV) treatment. Treatment consisted of a two-level pressure system for 7h at night, with oxygen available if needed. Questionnaire-based assessments of health-related quality-of-life (HRQL) were evaluated before treatment and at 3, 6, 9, 12 and 18 months of follow-up. Hospitalization rates pre- and post-treatment were recorded, and the numbers need to treat (NNT) to avoid hospitalization and absolute risk reduction (ARR) rates were calculated. Several categories of HRQL, including physical function and vitality, improved significantly with treatment in both groups of patients; these improvements persisted over the entire 18 months. In contrast, other categories such as social function and mental health improved initially and declined subsequently. Hospitalizations decreased significantly with treatment. NNT calculations indicated that treatment would be needed for two TWD patients (ARR 63%) and one NMD patient (ARR 78%) to prevent one hospitalization per year per disease group. We conclude that improved quality-of-life and decreased hospitalizations make home non-invasive mechanical ventilation an useful treatment for patients with restrictive respiratory disorders.