Abstract
Eisenmenger syndrome (ES) is defined as the process in which a long-standing left-to-right
cardiac shunt caused by a congenital heart defect is eventually reversed into a cyanotic
right-to-left shunt. It is crucial to recognize this grave pathology at the earliest
because once it develops, treatment by medical or surgical means becomes even more
challenging. In past decades, various therapeutic options have been developed that
address the specific pathophysiological aspects of the disease and have shown to improve
functional capacity and quality of life. There are three major therapeutic pathways
in pulmonary hypertension treatment - endothelin receptor antagonists, phosphodiesterase
type-5 inhibitors, and prostacyclin derivatives. These therapies not only improve
hemodynamic parameters and exercise capacity but they also improve prognosis with
various form of Pulmonary hypertension including ES. We report a case of a 35-year-old
female of ostium secundum atrial septal defect with ES, started on upfront combination
therapy of ambrisentan and tadalafil who demonstrated marked improvement after 3 months
of medical therapy.
Keywords
ostium secundum atrial septal defect - pulmonary hypertension - endothelin–receptor
antagonists - phosphodiesterase type-5 inhibitors - prostacyclin derivatives - echocardiography
- Eisenmenger syndrome
