Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) that significantly affects its prognosis. Down syndrome is a common comorbidity among patients with PAH associated with CHD. 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. Our objective was to know and compare the characteristics of the adult and pediatric population with DS and PAH associated with CHD. 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 2021. Patients who were followed in the participating centers 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 2021. Patients who were followed and diagnosed after January 1998 were retrospectively included. 131 adults and 47 children with DS and PAH associated with CHD were analyzed. Their baseline characteristics, hemodynamics, treatment, and 10-year survival were compared. Adult patients with DS presented more frequently complete atrioventricular canal defect as underlying heart disease and were mostly in Eisenmenger's situation (84%). In pediatric patients the largest group was post-repair PAH (50%). Functional class was similar in both groups, although adults presented significantly greater hemodynamic severity. Regarding medical treatment, no differences were observed between both groups: there was a very low use of prostanoids both at baseline and at follow-up, and a majority use of oral monotherapy. There were no significant differences in survival. Corrective surgery in patients with DS has become more frequent over the years: adults are mostly in Eisenmenger's situation, while post-repair PAH is the most numerous group in children. Although adults show greater hemodynamic severity, survival is similar in both groups. Medical treatment, which showed no differences between pediatric and adult age, is very conservative, with most patients on oral monotherapy and rare use of prostanoids. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Janssen MSD
Background Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. There are four large clinical groups of CHD-related PAH: Eisenmenger syndrome, PAH associated with non-restrictive shunt, PAH associated with restrictive shunt and postoperative PAH. Our purpose was to study the clinical and prognostic differences among them. Methods The REHAP is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with CHD and PAH, starting in 2007. 664 patients were analyzed: Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. Results 664 patients were analyzed: characteristics are detailed in the table. Patients with Eisenmenger were more frequently treated with oral monotherapy as a first line therapy and received less frequently prostanoids during the follow-up. Patients with Eisenmenger had significantly better prognosis, with the best long-term survival of the 4 groups. In a cox regression model, postoperative PAH has a 1.7 hazard ratio (HR) (reference group: Eisenmenger) after adjustment for age (HR 1.02 p 0,001), functional class (HR NYHA III-IV 2.3 p<0,001), sex (p 0.8) and pulmonary vascular resistance (p 0.7). Conclusion The clinical classification of PAH associated with CHD defines both the baseline characteristics and the prognosis of patients. Outcome relates closely to functional class and type of PAH–CHD. Eisenmenger group, which has the most severe hemodynamics, is the one with the best prognosis despite a less aggressive treatment. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Ferrer, Janssen
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 rare disease that affects the small pulmonary arteries, producing gradual obliteration of arterial lumen leading to the progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. Idiopathic PH refers to patients without an identifiable underlying cause, although some of them may have a genetic cause. Our aim was to define the baseline characteristics, initial therapy and risk factors for survival of patients with idiopathic PAH. Methods The REHIPED registry is a Spanish, multicenter, observational and prospective registry on pulmonary hypertension in the pediatric population (<18 years of age) that began in 2007. 47 patients with idiopathic PAH were analyzed. Results The mean age at diagnosis was 4.5 years, and there was a similar proportion of both sexes. 64% of patients were in functional class I-II. The mean pulmonary arterial pressure was 47,3±16,3 mmHg, with mean pulmonary vascular resistance index of 12±8,3 WU·m2. Initial therapy was oral monotherapy in 67%, prostanoids monotherapy in 11% and combination therapy with prostanoids in 9%. At the end of follow up, 55% of patients had received prostanoids. The mean follow-up time was 8 years. The survival rates (free of death or transplantation) was 91% (95% confidence interval (CI): 79–97%), 82% (95% CI: 67–91%) and 70% (95% CI: 52–82%) at 1, 5 and 10 years. Lung transplantation was performed in 7 patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV (Hazard Ratio (HR) 4.6, p=0.022, 95% CI: 1.3–15.3) and diagnosis under the age of 2 (HR 4, p=0.031, 95% CI: 1.1–14.4). Conclusions Idiopathic Pulmonary hypertension in pediatric age affects both sexes equally and begins at a young age. Prostanoids were used in 55% of patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV and diagnosis under the age of 2. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Janssen, Ferrer
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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