{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T16:02:02Z","timestamp":1787673722518,"version":"build-2736575974"},"reference-count":76,"publisher":"Oxford University Press (OUP)","issue":"26","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,7,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Pulmonary arterial hypertension (PAH) is a progressive and lethal pulmonary vascular disease (PVD). Although in recent years outcome has improved by new treatments that delay disease progression, a cure has not yet been achieved. In PAH associated with congenital heart disease (CHD), remodeling of the pulmonary vasculature reaches an irreversible phenotype similar to all forms of end-stage PAH. In PAH-CHD, however, also an early stage is recognised, which can be completely reversible. This reversible phase has never been recognised in other forms of PAH, most likely because these patients are only diagnosed once advanced disease has developed. We propose that the clinical model of PAH-CHD, with an early reversible and advanced irreversible stage, offers unique opportunities to study pathophysiological and molecular mechanisms that orchestrate the transition from reversible medial hypertrophy into irreversible plexiform lesions. Comprehension of these mechanisms is not only pivotal in clinical assessment of disease progression and operability of patients with PAH-CHD; specific targeting of these mechanisms may also lead to pharmacological interventions that transform \u2018irreversible\u2019 plexiform lesions into a reversible PVD: one that is amenable for a cure. In recent years, significant steps have been made in the strive to \u2018reverse the irreversible\u2019. This review provides an overview of current clinical and experimental knowledge on the reversibility of PAH, focussing on flow-associated mechanisms, and the near-future potential to advance this field.<\/jats:p>","DOI":"10.1093\/eurheartj\/ehx034","type":"journal-article","created":{"date-parts":[[2017,1,30]],"date-time":"2017-01-30T13:27:49Z","timestamp":1485782869000},"page":"2034-2041","source":"Crossref","is-referenced-by-count":89,"title":["Pulmonary arterial hypertension in congenital heart disease: translational opportunities to study the reversibility of pulmonary vascular disease"],"prefix":"10.1093","volume":"38","author":[{"given":"Diederik E.","family":"van der Feen","sequence":"first","affiliation":[{"name":"1Centre for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children\u2019s Hospital, University Medical Centre Groningen, University of Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Bartelds","sequence":"additional","affiliation":[{"name":"1Centre for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children\u2019s Hospital, University Medical Centre Groningen, University of Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf A.","family":"de Boer","sequence":"additional","affiliation":[{"name":"2Experimental Cardiology, Department of Cardiology, University Medical Centre Groningen, University of Groningen, Antonius Deusinglaan 1, 9713AV Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf M.F.","family":"Berger","sequence":"additional","affiliation":[{"name":"1Centre for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children\u2019s Hospital, University Medical Centre Groningen, University of Groningen, The 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