Optimizing the Care of Pulmonary Arterial Hypertension: The Importance of Functional Class and the Specific Challenges of Eisenmenger Syndrome

Discussedby:GORDONMOE,MD,FRCP(C),FACC Pulmonary arterial hypertension (PAH) is a chronic and pro gressive disease that, if left untreated, eventually leads to right-heart failure and death. One prognostic variable that has a major impact on long-term outcomes in PAH is World Health Organization (WHO) functional class (FC). Patients with PAH in FC I or II at the time of diagnosis fare better than those in FC III or IV, and improvement in FC during therapy is also associated with better prognosis. Eisen menger syndrome (ES) represents the most compromised clinical groups among patients with PAH associated with congenital heart disease (CHD). ES patients typically have a poor prognosis, particularly if they are left untreated. Improved outcomes for ES patients are being sought through intervention with PAH-specific therapies and both evidence and observational data for their utility in this setting is accu mulating. In this issue of Cardiology Scientific Update , evi dence supporting a prognostic role of FC and the benefit of a goal-directed therapy aiming at improving the FC of these patients will be reviewed. As well, the prognosis of a growing population of patients with ES and the increasingly appreciated advantage of treatment options using PAH-spe cific therapy in ES patients will be discussed.