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CHD and PAH

Specialties
Pulmonary arterial hypertension (PAH)

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]

EISENMENGER’S SYNDROME
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]
PAH ASSOCIATED WITH PREVALENT SYSTEMIC-TO-PULMONARY SHUNTS
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
PAH WITH SMALL/COINCIDENTAL DEFECTS
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]
PAH AFTER DEFECT CORRECTION
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.

CHD and PAH

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.

CHD and PAH

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]

Contact

Helena Sercu

Product Specialist Pulmonary
Hypertension BE
+ 32 472 501 443
hsercu@its.jnj.com

Nele Van Mieghem

Product Specialist Pulmonary
Hypertension BE
+32 479 79 12 74
nvanmieg@its.jnj.com

Pascaline Vancraeyenest

Medical Advisor
+32 492 58 48 48
pvancrae@its.jnj.com

Abbreviations

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

References

[1]
Lau EMT et al. Nat Rev Cardiol 2015; 12(3):143–155.
[1]
[2]
Gali. N et al. Eur Heart J 2016; 37(1):67–119.
[2]
[3]
Gali. N et al. Eur Heart J 2010; 31(17):2080–2086.
[4]
Vachiery JL et al. Eur Respir Rev 2012; 21(123):40–47.
[5]
Vonk Noordegraaf A et al. J Am Coll Cardiol 2017; 69(2):236–243
[6]
National Pulmonary Hypertension Centres of the UK and Ireland. Heart 2008; 94(Suppl 1):i1–i41
[7]
Engelfriet PM et al. Heart 2007; 93(6):682–687.
[8]
Hoeper MM, Gibbs JSR. Eur Respir Rev 2014; 23(134):450–457
[9]
Gali. N et al. Eur Heart J 2016; 37(1):67–119
[10]
Savale L, Manes A. Eur Heart J Suppl 2019; 21(Suppl K):K37–K45.
[11]
D’Alto M, Mahadevan VS. Eur Respir Rev 2012; 21(126):328–337.
[12]
Dimopoulos K et al. J Am Coll Cardiol 2018; 72(22):2778–2788.
[13]
Duffels MGJ et al. Int J Cardiol 2007; 120(2):198–204.
[14]
Van Dissel A et al. J Clin Med 2017; 6(4):40.
[15]
Manes A et al. Eur Heart J 2014; 35(11):716–724.
[16]
Frost A et al. Eur Respir J 2019; 53(1):1801904.
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Baumgartner H et al. Eur Heart J 2020; doi:10.1093/eurheartj/ehaa554.
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Bhatt AB et al. Circulation 2015; 131(21):1884–1931.
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Khou V et al. Respirology 2020; doi:10.1111/resp.13768.
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Mackie AS et al. Circulation 2009; 120(4):302–309.
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Dimopoulos K et al. Eur Heart J 2014; 35(11):691–700
[22]
Vis JC et al. Int J Cardiol 2011; 149(2):246–247.
[23]
Constantine A et al. American College of Cardiology. 2019. Available at: https://www.acc.org/latest-in-cardiology/articles/2019/07/02/15/22/echocardiographic-screening-for-ph-in-chd (last accessed January 2021)
[24]
Pulido T, et al. N Engl J Med 2013; 369(9):809-818
[25]
Sitbon O, et al. N Engl J Med 2015; 373(26):2522-2533
[26]
Gaine S, et al. Eur Respir J 2017; 50(2):1-9
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OPSUMIT US Prescribing Information. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/204410s017lbl.pdf (accessed February 2020)
[28]
Humbert M et al. Eur Respir Rev 2012; 21(126):306–312.
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