title: Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is common in patients with congenital heart disease (CHD). Although Eisenmenger syndrome may be on decline, other types of PAH-CHD are increasing and little is known on long-term outcome of this population. We report the PAH-CHD population of Spain via a national registry with focus on long-term survival.
  
  
  RESULTS
  A total of 240 consecutive patients (age 37.7 ± 14.1 years, 67.9% females) with PAH-CHD included in the REHAP registry were analysed. Patients were classified into 3 groups: 1) Eisenmenger syndrome, 2) postoperative-PAH and 3) PAH associated with small defects. Over a median follow-up time of 4.5[1.6-7.1]years, 50 patients (20.8%) died or underwent lung/heart-lung transplantation. Patients with Eisenmenger syndrome had better survival than postoperative-PAH (HR 0.1 95% CI: 0.2-0.9, p=0.048) but no advantage compared to small defects (HR 4.4, 95% CI 0.6-31.4, p=0.15). In the overall PAH-CHD population, patients in NYHA functional class III-IV had a 3-fold increased risk of death (HR 3.0, 95% CI: 1.5-5.9, p=0.001). Amongst patients with Eisenmenger syndrome, a pre-tricuspid shunt had a 2.6-fold increase risk of death (HR 2.6, 95% CI: 1.2-5.6, p=0.03). There was no significant difference in survival between patients with postoperative-PAH and patients with iPAH (HR 0.99 95% CI: 0.6-1.7, p=0.97).
  
  
  CONCLUSIONS
  PAH-CHD is associated with mid to long-term mortality. Outcome relates closely to functional class, type of PAH-CHD and within the Eisenmenger cohort, with location of the shunt. Adults with postoperative-PAH have the worse prognosis in the PAH-CHD cohort, reinforcing the need for lifelong close follow-up of such patients.
authors.affiliation: Cardiology Department, Hospital Universitario Infanta Cristina, Badajoz, Spain; Adult Congenital Heart Disease Centre, Royal Brompton Hospital NIHR Cardiovascular BRU, London, UK; National Centre for Pulmonary Hypertension, Royal Brompton Hospital NIHR Cardiovascular BRU, London, UK. Electronic address: rafa.alonso.g@gmail.com.
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso-Gonzalez
authors.affiliation: Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Carmen Jiménez
authors.initials: CJ
authors.lastname: Lopez-Guarch
authors.affiliation: Integrated Adult Congenital Heart Disease Unit Vall d'Hebron - Sant Pau, Cardiology Department Hospital de la Sta. Creu i Sant Pau, Barcelona, Spain.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Subirana-Domenech
authors.affiliation: Adult Congenital Heart Disease Unit, Cardiology Department, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: José María Oliver
authors.initials: JMO
authors.lastname: Ruíz
authors.affiliation: Pneumonology Department, Hospital A Coruña, Coruña, Spain.
authors.forename: Isabel Otero
authors.initials: IO
authors.lastname: González
authors.affiliation: Cardiology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain.
authors.forename: Javier Segovia
authors.initials: JS
authors.lastname: Cubero
authors.affiliation: Paediatric and Adult Congenital Heart Disease Unit, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: Maria Jesus
authors.initials: MJ
authors.lastname: Del Cerro
authors.affiliation: Cardiology Department, Hospital Universitario Virgen de la Salud, Toledo, Spain.
authors.forename: Maria Lázaro
authors.initials: ML
authors.lastname: Salvador
authors.affiliation: Integrated Adult Congenital Heart Disease Unit Vall d'Hebron - Sant Pau, Cardiology Department Hospital Vall d'Hebron, Barcelona, Spain.
authors.forename: Laura Dos
authors.initials: LD
authors.lastname: Subira
authors.affiliation: Heart Area, Hospital Universitario Virgen Macarena, Hospital Universitario Virgen del Rocío, Sevilla, Spain.
authors.forename: Pastora
authors.initials: P
authors.lastname: Gallego
authors.affiliation: Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. Electronic address: pilar.escribano.subias@gmail.com.
authors.forename: Pilar
authors.initials: P
authors.lastname: Escribano-Subias
mesh_terms: D000328:Adult; D004541:Eisenmenger Complex / Q000175:diagnosis* / Q000453:epidemiology*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012042:Registries; D013030:Spain / Q000453:epidemiology; D055815:Young Adult
keywords: Adult; Congenital heart disease; Eisenmenger syndrome; Pulmonary hypertension; Survival