PAH is a silently progressive disease[1]

PAH, a subgroup of pulmonary hypertension,[2] is a rare, severe and progressive disease characterised by chronic elevation in pulmonary vascular resistance and arterial pressure.*[3]If left untreated, PAH eventually results in right ventricular failure and death.[3][4]

Vascular remodelling in PAH leads to narrowing of the pulmonary arteries and increased pulmonary vascular resistance[3]

Increased pulmonary vascular resistance leads to high right ventricular afterload, hypertrophy, dilatation and, eventually, right heart failure[5]

*The current haemodynamic definition of PAH is mPAP ≥25 mmHg, PAWP ≤15 mmHg and PVR >3 Wood units.[2]

Early identification and intervention is key to changing the course of PAH[6]

PAH is a common complication of CHD[2][7]

CHD-PAH is a common PAH subtype, accounting for 10–20% of cases,[8] and represents a heterogeneous patient population.[9] Patients with CHD-PAH can be classified into one of four main subgroups according to the 2015 ESC/ERS guidelines:[9]

Adapted from Savale et al. 2019[10]

1. Eisenmenger's syndrome[2]

  • Includes large defects that develop due to systemic-topulmonary shunts
  • Progression to severe elevation of PVR
  • Reversed or bidirectional shunting Cyanosis, erythrocytosis and multiple organ involvement
  • PAH is present by definition[10]
Adapted from Savale et al. 2019[10]

2. PAH associated with prevalent systemic-to-pulmonary shunts[2]

  • Includes moderate-to-large defects
  • Mild-to-moderate increase in PVR
  • Systemic-to-pulmonary shunt is still present
  • No cyanosis detectable at rest
Adapted from Savale et al. 2019[10]

3. PAH with small/coincidental defects[2]

  • Small defects (usually ASD <2 cm and VSD <1 cm)
  • Marked elevation of PVR
  • Clinical presentation similar to idiopathic PAH
  • Closing the defects is contraindicated PAH is present by definition[10]
Adapted from Savale et al. 2019[10]

4. PAH after defect correction[2]

  • Defect is repaired
  • PAH persists immediately after correction or recurs/ develops months or years after defect closure in the absence of significant post-operative haemodynamic lesions

Patients with CHD are at risk of PAH, even when congenital heart defects are corrected[11]

Patients with corrected defects are still at risk of developing PAH[^10]

[10]: D’Alto M, Mahadevan VS. Eur Respir Rev 2012; 21(126):328–337.


Since PAH can develop over time despite surgery, patients with CHD need regular, long-term screening for PAH after defect correction[10][12]

PAH associated with corrected CHD is increasing in prevalence[^14]

Advances in the treatment of CHD over the past few decades have led to an increase in adult CHD survivors, who may then go on to develop PAH following defect correction.[^14] The overall prevalence of PAH in patients with corrected simple defects, including ASD and VSD, ranges from 3% to 12%.*[13][7]

[14]: Van Dissel A et al. J Clin Med 2017; 6(4):40.


The number of patients who develop PAH after congenital defect correction is increasing[14]

*Data from adult patients with CHD in CONCOR, a Dutch registry (N=2,389),[13] and the Euro Heart survey database (N=1,877).[7]

Patients with corrected CHD-PAH have poor outcomes[15][7]

In patients with corrected CHD, development of PAH is associated with significant worsening in functional limitations and poor long-term survival.*[15][7]

CHD and PAH graph

More than 1 in 3 patients with corrected CHD-PAH die within 10 years of PAH diagnosis[15]

*Data from patients with CHD-PAH in an Italian database study (N=192)16 and from patients with CHD in the Euro Heart survey database (N=1,877).[7]


Marina Meurice

Product Specialist Pulmonary
Hypertension BELUX
+32 491 15 34 24

Sévérine Gara

Product Specialist Pulmonary
Hypertension BE
+32 475 82 71 39

Pascaline Vancraeyenest

Medical Advisor
+32 492 58 48 48


ACHD, adult congenital heart disease; ASD, atrial septal defect; CHD, congenital heart disease; CI, confidence interval; CT, computerised tomography; ECG, electrocardiography; ERS, European Respiratory Society; ESC, European Society of Cardiology; mPAP, mean pulmonary arterial pressure; MRI, magnetic resonance imaging; PA, pulmonary artery; PAH, pulmonary arterial hypertension; PAWP, pulmonary artery wedge pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; TRV, tricuspid regurgitation velocity; VSD, ventricular septal defect; WSPH, World Symposium on Pulmonary Hypertension


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