Annelieke C M J van Riel1, Mark J Schuuring2, Irene D van Hessen2, Aielko H Zwinderman3, Luc Cozijnsen4, Constant L A Reichert5, Jan C A Hoorntje6, Lodewijk J Wagenaar7, Marco C Post8, Arie P J van Dijk9, Elke S Hoendermis10, Barbara J M Mulder1, Berto J Bouma11. 1. Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands. 2. Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands. 3. Department of Clinical Epidemiology and Biostatistics, Academic Medical Centre, Amsterdam. 4. Department of Cardiology, Gelre Hospital, Apeldoorn, The Netherlands. 5. Department of Cardiology, Medical Centre Alkmaar, Alkmaar, The Netherlands. 6. Department of Cardiology, Isala Klinieken, Zwolle, The Netherlands. 7. Department of Cardiology, Medisch Spectrum Twente Hospital, Enschede, The Netherlands. 8. Department of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands. 9. Department of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. 10. Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands. 11. Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands. Electronic address: b.j.bouma@amc.nl.
Abstract
BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population. METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed. RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million. CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.
BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population. METHODS: A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed. RESULTS: Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million. CONCLUSIONS: This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.
Authors: Christian Opitz; Ekkehard Grünig; Stephan Rosenkranz; Ann-Sophie Kaemmerer; Matthias Gorenflo; Dörte Huscher; David Pittrow; Peter Ewert; Christine Pausch; Marion Delcroix; Hossein A Ghofrani; Marius M Hoeper; Rainer Kozlik-Feldmann; Andris Skride; Gerd Stähler; Carmine Dario Vizza; Elena Jureviciene; Dovile Jancauskaite; Lina Gumbiene; Ralf Ewert; Ingo Dähnert; Matthias Held; Michael Halank; Dirk Skowasch; Hans Klose; Heinrike Wilkens; Katrin Milger; Christian Jux; Martin Koestenberger; Laura Scelsi; Eva Brunnemer; Michael Hofbeck; Silvia Ulrich; Anton Vonk Noordegraaf; Tobias J Lange; Leonhard Bruch; Stavros Konstantinides; Martin Claussen; Judith Löffler-Ragg; Hubert Wirtz; Christian Apitz; Rhoia Neidenbach; Sebastian Freilinger; Attila Nemes Journal: Cardiovasc Diagn Ther Date: 2021-12
Authors: I M Blok; A C M J van Riel; M J Schuuring; M G Duffels; J C Vis; A P J van Dijk; E S Hoendermis; B J M Mulder; B J Bouma Journal: Neth Heart J Date: 2015-05 Impact factor: 2.380