Discussion In this report, we presented a case with severe PAH associated with secundum type ASD who was successfully treated with operation

and transient use of oral bosentan. ASD is most common congenital heart disease in adults. PAH can occur as a result of chronic exposure of the pulmonary vessels to increased blood flow through the shunt.1) Histologic changes in the intima and media of the pulmonary vessels can be resulted in the luminal narrowing and subsequent development of PAH.5) According to previous studies, the prevalence of PAH is Inhibitors,research,lifescience,medical 6-17% of patients with ASD.6),7) The presence of PAH is associated with poor prognosis in the patients with ASD.1),2) Increased pulmonary arterial pressure can be inhibitors lowered with septal closure. Balint et al.8) reported successful outcomes after

transcatheter closure in selected patients with secundum ASD and PAH. Inhibitors,research,lifescience,medical Initial pulmonary vasodilator therapy may be beneficial in patients with irreversible anatomic changes of pulmonary vessels in the previously published data.3),4),9),10) Schwerzmann et al.4) described a 38-year-old woman with ASD and severe PAH, Inhibitors,research,lifescience,medical who showed significant symptomatic and hemodynamic improvement after 1 year of treatment with intravenous prostacyclin. The ASD was closed percutaneously after the pulmonary vasodilator therapy. Kim et al.3) Inhibitors,research,lifescience,medical reported a 41-year-old woman with Eisenmenger syndrome who was initially managed with oral sildenafil for 2 years and ASD was successfully closed. In our case, we successfully managed severe PAH with the surgical repair of ASD and subsequent use of oral bosentan therapy. Although decision of operative closure in this patient was difficult, we decided to operate the ASD on the basis of clinical situation and the result of cardiac catheterization. Several reports already showed that the Inhibitors,research,lifescience,medical hemodynamic determination of operability

in patients with ASD and severe PAH was problematic.5),9),11) However, there are reported cases with transient use of vasodilator therapy was associated Rutecarpine with good result in the management of PAH associated with ASD. There is a report that younger age was associated with good prognosis in the surgical correction of ASD.2) In conclusion, we experienced a case of dramatic improvement of severe PAH and right ventricular dysfunction after ASD closure followed by an oral bosentan treatment. Our case suggests that the operability in patients with ASD and severe PAH should be decided with discretion on a case by case. The corrective repair of ASD and subsequent oral bosentan treatment can be an option in the treatment of selected patients with severe PAH and right ventricular dysfunction.