A 25-year-old patient presented with dyspnea for 6 months. An echocardiogram revealed an 18mm atrial septal defect (ASD) with left-to-right shunting and mild right ventricular dilation. The mean pulmonary artery pressure was elevated at 25mmHg. Device closure of the ASD was performed due to favorable anatomy and mild pulmonary hypertension. Follow up showed a decrease in mean pulmonary artery pressure to 18mmHg, indicating successful treatment of pulmonary arterial hypertension caused by the congenital heart defect. Early detection and advances in closure techniques for congenital heart defects have improved survival outcomes for patients with pulmonary arterial hypertension.

1. Haytham Soliman, MD, FSCAI
Fayoum University

2. Case
A 25 year old patient
Has reports no relevant cardiac history
Complained of dyspnea for 6 month
He had an assessment that lead to an echocardiography
Echo revealed a Secondum ASD 18mm with left to right shunt
Mild RV dilation
Mean PAP 25mmHg

3. Where ?
When?
What?
How?
Four major
questions

4. ASD
TAPVR
Volume overload
Volume and pressure
overload
VSD
PDA
Where is the level of the shunt ?

6. • Minor shunts may be accidentally discovered
• Early discovery means less effect of the shunt on pulmonary artery
• Shunts treated at the 1st year of age can have no residual PHT
• In large defects, treatment after the 2nd years will not change the PAP to
normal levels
• The later and larger the defect the faster we will reach a point of no return
When this defect was discovered ?

8. What changes did happen in the pulmonary
circulation?

10. • Abnormal shear stress, circumferential wall stretch and endothelial
dysfunction
• Decrease vasoactive mediators, such as endothelin-1, prostacyclin
and nitric oxide → vasoconstriction
• Aberrant expression of vascular endothelial and fibroblast growth
factors promotes vascular remodelling
• Smooth muscle hypertrophy and proliferation
• Increased intracellular matrix deposition

12. Diagnostic tools
• Clinical examination
• Laboratory tests
• Echocardiography
• Invasive Right heart cath

14. Laboratory tests
• Total blood cell counts
• serum iron levels, haematocrit
• Infectious diseases
• NT-pro-BNP
• Arterial blood gases
• 6MWT
Functional assessment

15. This is a noninvasive marker of vascular damage and
remodeling
which has been shown to be significantly raised in CHD patients
with irreversible PAH post-surgery

16. Imaging ( Echocardiography)
• The shunt site
• The RV and LV functions
• The PAP mean pressure above 20 mmHg
• The shunt fraction

17. Invasive right heart cath
• Not indicated in bidirectional shunts and Eisenmenger’s
syndrome
• Pulmonary pressure ( mean and peak)
• Systemic vascular resistance
• Pulmonary vascular resistance ( WU)
• Shunt fraction Qp/Qs
• Pulmonary reactivity test

18. How do we treat these patients
• Medical therapy
• Intervention ( surgery or device closure)
• O2 therapy in Eisenmenger’s syndrome for improving symptoms and
QOL

19. Medical therapy
• Diuretics ( in RV failure)
• Vasodilators ( in post repair patients that has vasoactive pulmonary arteries )
• CCbs are contraindicated in Eisenmenger patients
• Anticoagulation with vitamin K antagonists (VKAs) in the absence of atrial
arrhythmia, mechanical valves, or vascular prosthesis is not generally
recommended in PAH-CHD
• Secondary erythrocytosis is beneficial for adequate oxygen transport and
delivery so routine phlebotomy should be avoided

20. PAH directed therapy
- Parenteral prostaglandins ( risk of infection and thrombosis)
- subcutaneous or inhaled forms are generally preferred
- Eisenmenger patients, the endothelin receptor antagonist (ERA)
bosentan has been shown to improve 6MWT and decrease PVR
- less evidence for phosphodiesterase type 5 (PDE-5) inhibitors
sildenafil and tadalafil
- Experience is limited for latest-generation PAH drugs such as
macitentan, selexipag, or riociguat in the setting of CHD

22. Intervention
• Chamber volume overload ( RV or LV)
• Pulmonary vascular resistance ( 3- 5 WU)
• Shunt fraction (Qp/Qs 1.5)

26. Best survival among PAH
• Slow progression
• Less effort
• More RV remodeling to pressure
• Most of shunts are small
• Advance in detection and closure
in early ages

27. Back to our patient
• He had mild RV dilation
• Elevated PAP with a PVR of 3 WU
• He has favorable anatomy for device closure
• So we used a 23mm device to close the ASD
• Follow up showed decrease in mean PAP to 18 mmHg

28. Summary
• Early detection and treatment of PAH-CHD is crucial
• Advances in diagnosis and closure techniques lead to increase
survival
• Proper tailoring according to PVR, shunt fraction and chamber
dilation must be done
• PAH direct therapy has increased survival and improved symptoms
specially in Eisenmenger patients
• But till now there is no change in their mortality

29. Thank you so much