Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry

International Journal of Cardiology
Rafael Alonso-GonzalezREHAP investigators

Abstract

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. 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 s...Continue Reading

References

Jun 28, 2006·Circulation·Nazzareno GalièUNKNOWN Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators
Feb 28, 2007·Circulation·Gerhard-Paul Diller, Michael A Gatzoulis
Jul 31, 2007·International Journal of Cardiology·Michael A GatzoulisUNKNOWN BREATHE-5 Investigators
Oct 20, 2010·European Respiratory Review : an Official Journal of the European Respiratory Society·M A GatzoulisM Beghetti
Mar 1, 2012·The European Respiratory Journal·Pilar Escribano-SubiasUNKNOWN REHAP investigators
Mar 7, 2012·International Journal of Cardiology·Gerhard-Paul DillerMichael A Gatzoulis
Aug 18, 2012·Circulation·Pamela MoceriWei Li
Oct 31, 2013·European Heart Journal·Konstantinos DimopoulosMichael A Gatzoulis
Dec 18, 2013·International Journal of Cardiology·Craig S BrobergMichael A Gatzoulis
Dec 21, 2013·Journal of the American College of Cardiology·Gerald SimonneauRogiero Souza

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Citations

Feb 18, 2016·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Sheila RamjugRobin Condliffe
Feb 4, 2016·Revista Española De Cardiología·P Escribano Subias
Dec 1, 2015·International Journal of Cardiology·P Escribano-SubíasUNKNOWN REHAP Investigators
May 27, 2015·International Journal of Cardiology·Uğur AksuIbrahim Halil Tanboga
Dec 15, 2015·Circulation. Cardiovascular Imaging·Eric V Krieger, Anne Marie Valente
Nov 11, 2018·Paediatric Anaesthesia·Gregory J Latham, Delphine Yung
May 24, 2019·Expert Review of Cardiovascular Therapy·Carla FavocciaKonstantinos Dimopoulos
Jun 27, 2018·BMC Pulmonary Medicine·Ana Beatriz ValverdeRogerio Souza
Oct 4, 2020·Genes·Jair Antonio Tenorio CastañoPilar Escribano-Subías
Sep 8, 2018·General Thoracic and Cardiovascular Surgery·Daisuke YoshinagaToshio Heike
Dec 10, 2020·Respiratory Medicine Case Reports·Ali A AlakhfashAbdulrahman A Almesned
Jan 14, 2020·International Journal of Cardiology·Astrid Elisabeth LammersUNKNOWN German Competence Network for Congenital Heart Defects Investigators
Apr 16, 2021·Expert Review of Cardiovascular Therapy·Ana Barradas-PiresKonstantinos Dimopoulos
May 12, 2021·Revista Española De Cardiología·Alejandro Cruz-UtrillaPilar Escribano Subias

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