The Spanish Registry of Pulmonary Arterial Hypertension (REHAP)
Jose Cifrian, MD
Hospital University Marques Valdecilla
Recently, large national observational registries in different countries have provided information on current PAH epidemiology, increasing awareness about the disease. Furthermore, these national registries have made possible the reassessment of patient survival under present conditions, leading to the formulation of new predictive survival equations. Epidemiological data from different countries, sometimes with different healthcare organizations, drug availability and financial outreach, may provide a more comprehensive view of current management of the disease worldwide.
Different efforts have been done in United States (U.S Reveal), Scotland (Scottish-SMR), France (French Registry), China (New Chinese Registry), United Kingdom, but also in Spain to try to analyze prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), and to assess the applicability of survival prediction equations.
The REHAP registry is a voluntary, nonpaid registry that was launched in January 2007. All patients evaluated in 31 hospitals in Spain since January 1, 1998 were included in the registry. Centers reporting patients to the registry covered 15 of the 17 administrative regions of Spain. Patients diagnosed in the period 1998-2006 were entered retrospectively and prospectively thereafter. Data was collected by means of electronic data capture, starting at the initial baseline assessment reported in the medical records. Patients with newly or previously diagnosed PH were eligible for enrolment if they met the definition of PAH and CTEPH and pre-specified hemodynamic criteria by right heart catheterization.
3072 patients have been included in the Spanish registry until February 2017. The REHAP registry is collaborating with important studies in genetic aspects of PAH and phenotypic expression in Spanish population.
The registry is working now in the impact of age on survival of patients with idiopathic and connective tissue disease, pulmonary arterial hypertension, and also in the probable underuse of lung transplantation in severe pulmonary hypertension.
Results of the REHAP registry support the validity of multicenter, international clinical trials and indicate that despite differences in health systems and populations, the evolution of the disease is quite homogeneous when access to advanced specific therapies is guaranteed.
Registries have been extremely helpful in improving our understanding of PAH. It is clear that more registry data will be needed to answer emerging questions.
Since the pioneer NIH Registry of PAH, recent information gathered from national (REHAP) and international registries have shown changes in PAH phenotypes and outcomes in the management of these patients. ■
Disclosure Statement: The author has no conflicts of interest to disclose.