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) [
1
,
2
,
3
,
4
]. The reported prevalence of ES among adult CHD patients is ≈1–6% [
2
,
5
,
6
].Keywords
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Article info
Publication history
Published online: September 13, 2016
Accepted:
September 6,
2016
Received:
September 5,
2016
Identification
Copyright
© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
