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Pulmonary hypertension in adults with congenital heart disease: First data from Latvian PAH registry

Published:September 13, 2016DOI:https://doi.org/10.1016/j.ejim.2016.09.002
      Pulmonary arterial hypertension (PAH) is a common complication in patients with congenital heart disease (CHD), particularly in those with prominent left-to-right (systemic-to-pulmonary) shunt. However, PAH can also develop after partially successful repair (due to remaining residual lesions) or with full repair of cardiac defect if it is not done in a timely manner. Persistent exposure of the pulmonary vasculature to increased blood flow and pressure may result in pulmonary obstructive arteriopathy which histopathologically is similar to those observed in idiopathic or other associated forms of PAH. Pulmonary vascular remodeling and dysfunction leads to increased pulmonary vascular resistance (PVR) which, approaching or exceeding systemic resistance, will result in the reversal of the shunt manifesting as the most advanced form of PAH, known as Eisenmenger syndrome (ES) [
      • Galie N.
      • Manes A.
      • Palazzini M.
      • Negro L.
      • Marinelli A.
      • Gambetti S.
      • et al.
      Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome.
      ,
      • Duffels M.G.J.
      • Engelfriet P.M.
      • Berger R.M.F.
      • RLE van L.
      • Hoendermis E.
      • Vriend J.W.J.
      • et al.
      Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry.
      ,
      • Simonneau G.
      • Robbins I.M.
      • Beghetti M.
      • Channick R.N.
      • Delcroix M.
      • Denton C.P.
      • et al.
      Updated clinical classification of pulmonary hypertension.
      ,
      • D'Alto M.
      • Mahadevan V.S.
      Pulmonary arterial hypertension associated with congenital heart disease.
      ]. The reported prevalence of ES among adult CHD patients is ≈1–6% [
      • Duffels M.G.J.
      • Engelfriet P.M.
      • Berger R.M.F.
      • RLE van L.
      • Hoendermis E.
      • Vriend J.W.J.
      • et al.
      Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry.
      ,
      • Bouzas B.
      • Gatzoulis M.A.
      Pulmonary arterial hypertension in adults with congenital heart disease.
      ,
      • Engelfriet P.M.
      • Duffels M.G.J.
      • Möller T.
      • Boersma E.
      • Tijssen J.G.P.
      • Thaulow E.
      • et al.
      Pulmonary arterial hypertension in adults born with a heart septal defect: the euro heart survey on adult congenital heart disease.
      ].

      Keywords

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