A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations.Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated.866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3-and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar.Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.
Background Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic/familial pulmonary arterial hypertension (IPAH/FPAH). Aims To compare the characteristics, survival, prognostic factors and management of PoPH and IPAH/FPAH patients and to assess the impact of treatment on survival of PoPH patients. Methods Analysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first‐line PAH‐targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients). Results Compared to IPAH/FPAH patients (n = 678), patients with PoPH (n = 237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age‐ and sex‐adjusted 5‐year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (P < 0.001). Treated PoPH had better survival than non‐treated. PAH‐ and liver‐related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first‐line PAH‐targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first‐line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation. Conclusions PoPH patients are undertreated and show poorer survival than IPAH/FPAH patients. First‐line treatment with PAH‐targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.
Introduction Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. Children born in recent years with CHD have benefited from advances in pediatric cardiac surgery that have modified their evolution compared to adults with the same pathologies. Purpose Our objective was to define and compare the characteristics of the adult and pediatric population with PAH associated with CHD. Methods REHAP and REHIPED are Spanish, voluntary, multicenter registries that include patients with PAH associated with CHD. REHAP started in 2007 and includes patients over 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2020. Patients who were followed in the participating centres and diagnosed after January 1998 were retrospectively included in the registry. REHIPED started in 2009 and collects patients between 2 months and 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2020. Patients who were followed and diagnosed after January 1998 were retrospectively included. The baseline characteristics, hemodynamic profile, treatment, and 10-year survival of the REHAP patients were compared to those included in the REHIPED. In addition, the change in the distribution of etiologies of REHAP over time was analyzed. Results 664 patients were included from REHAP (mean age 32±20 years) and 153 from REHIPED (mean age 5±5 years). Among adults, there was a significantly higher proportion of women (67% versus 56%, p 0.004), patients with Eisenmenger syndrome and simple heart disease. The frequency of the different clinical groups has varied over the years, becoming progressively similar to the distribution observed in REHIPED. Adults presented greater hemodynamic severity, with higher mean pulmonary arterial pressure and greater pulmonary vascular resistance index. There were no differences in initial therapy or in the percentage of patients who received prostanoids at follow-up. Survival at 10 years was better in the group of children (99% at 1 year, 95% at 5 and 80% at 10) than in the adult group (94% at 1 year, 83% at 5 and 71% at 10) with a HR of 1.93 [95% CI 1.2–3] for REHAP compared to REHIPED. Conclusions Patients with PAH associated with CHD have different clinical and prognostic characteristics in childhood and adulthood. Knowing the characteristic of the pediatric age can help predict how the future patients with PAH associated with CHD will be and how we can plan their care. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Ferrer, MSD, Janssen, GSK Table 1 Figure 1
Introduction Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. Our aim was to investigate how the location of the shunt influences the outcome of these patients. Methods The REHAP registry is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with congenital heart disease and PAH, starting in 2007. Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. We performed a cox regression model for assessing the impact of the type of shunt on prognosis, after looking for confusing and effect modifying variables. Results 664 patients were analyzed, their characteristics are detailed in the table. Mean follow up time was 10 years. We performed a survival analysis adjusted for the confounding variables: age (Hazard ratio (HR) 1.02 p<0.001), functional class (HR 2.45 p 0.001) and clinical group. The clinical group was also an effect-modifying variable, which means there is no unique effect (HR) of the type of shunt, instead it depends on the clinical group. We did a multiple regression analysis, adjusting for the confounding variables, to calculate the HR for each clinical group: in patients with Eisenmenger syndrome, the HR is 0.27 (p<0.001) for simple posttricuspid shunt and HR 0.38 (p<0.001) for complex posttricuspid shunt (reference group: pretricuspid shunt). In the others clinical groups, the type of shunt did not reach statistical significance. Conclusion The type of shunt significantly affects prognosis in Eisenmenger patients, with a much worse outcome for those with pretricuspid shunts. Functional class and clinical group are also closely related to outcome. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): MSD, GSK
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