Eisenmenger Syndrome:

Is It Terminal State or Not?

Ratih Rachmanyati Pasah
Izzati Nadhifa R.
 Introduction
Eisenmenger Syndrome

A congenital heart defect that initially

causes chronic large left to right shunt that
induces severe pulmonary vascular and PAH

8% of patients with CHD and 11% of those with left-

to-right intracardiac shunting develop the ES
Introduction
Pathophysiology
Management

Supportive therapy

Targeted therapy

Combination therapy

Corrective intervention
 Supportive Therapy
O2 • Controversial
supplementa
tion
• No impact on haematology, exercise
capacity, natural history & survival

• Right heart failure

Digoxin • Greater utility in the treatment of
arrhythmias

Diuretics • Symptomatic relief of congestion

• Risk of worsening hyperviscosity
 Supportive Therapy
• Controversial
Anticoa • PA thrombosis & stroke vs. risk of
gulants haemorrhage & hemoptysis
• Only in Afib, intracardial mechanical
prostheses & conduits w/ advanced HF

• Reduce pulmonary vascular resistance with

minimal systemic effects
NO • Does not play any role in the long-term therapy
• Important role in acute post-operative therapy
• For assessment of pulmonary vascular reactivity
 Supportive Therapy
• May decrease systemic arterial pressure and
CCBs increase right- to-left shunting
• Not recommended
in patients with ES

• Routine phlebotomy should be avoided.

• Lead to anemia, exercise impairment,
Phlebo
reduced QoL, chronic iron deficiency, &
tomy increased risk of thromboembolic events.
• Supplemental iron treatment in patients
with low ferritin plasma levels.
Supportive Therapy
Patient Education
• At particular risk during surgery, anesthesia,
dehydration, chest infection, pregnancy, high
altitude, and intravenous lines
• Avoid strenuous exercise and competitive
sports
Targeted Therapy
Endothelin Receptor Antagonist (ERA)
ET-1 plays a major role in structural & functional abnormalities in
pulmonary vasculature and progression of PAH-CHD & ES

Lowers the pressure & pulmonary vascular resistance, decreases

fibrotic & inflammatory changes of the vessel

BREATHE-5 study : bosentan significantly reduced PVR & improved

exercise capacity vs. placebo of ES patients in WHO FC 3

The most efficacious targeted PAH therapy

Targeted Therapy
Phosphodieterase-5 Inhibitor (PDE-5i)
Sildenafil selectively inhibit PDE-5

Sildenafil enhances vasodilation of nitric oxide by increasing

cGMP concentration and has antiproliferative properties towards
smooth muscles of the vessels

Sildenafil increases contractility of the hypertrophic right ventricle

 Targeted Therapy
Prostacyclin and Prostacyclin Analogs

Data limited to case reports and small studies

Approved agents for PAH

• Epoprostenol
Epoprostenol
• Iloprost
•• Can only be administered by continuous intravenous infusion
Treprostinil
• Has the strongest effect of all known agents.
• Positive impact in CHD patients improving effort tolerance and
saturation
Combination Therapy
Combination therapy may be considered for
symptomatic patients who failed to improve with
first-line, monodrug treatment

Combination therapy with sildenafil and bosentan in

21 ES patients : significant improvement in 6MWD,
PVR and pulmonary blood flow
Corrective Intervention

ES has been synonymous with in-operability

Definitive treatment : lung-transplantation with

shunt closure, or heart-lung-transplantation 
associated with high perioperative mortality

Available data on closure of intra- or extracardiac

communications in the presence of severe PAH, with
or without the use of advanced therapies, are scarce
and still limited to case reports
Treatment Algorithm
Conclusion
Eisenmenger syndrome is a severe and devastating condition
that is associated with considerable morbidity and mortality

The evaluation of targeted therapies, including ERA, PDE-5i,

prostacyclin, and prostacyclin analogs, in patients with ES
showed improvements in exercise capacity, functional class,
and hemodynamics without compromising oxygen saturation

These treatments have challenged the notion that ES is a

stable disease, not amenable to treatment

However, until now, the only definitive treatment of ES is lung-

transplantation with shunt closure, or heart-lung-
transplantation
Thank you…