ABSTRACT

Treatment Algorithm and Assessing Response to Therapy The most recent treatment guideline from the Dana Point meeting is shown in Figure 10.7 (9). Incorporating risk-based approach by combining known factors that determine prognosis in PAH in selecting therapy has been recommended and is widely utilized by clinicians (Fig. 10.8) (16). The ACCF/AHA

Figure 10.8 PAH treatment algorithm based on risk assessment. Background therapies include warfarin anticoagulation, which is recommended in all patients with IPAH without contraindication. Diuretics are used for management of right heart failure. Oxygen is recommended to maintain oxygen saturation greater than 90%.aAcute vasodilator testing should be performed in all IPAH patients who may be potential candidates for long-term therapy with CCBs. Patients with PAH due to conditions other than IPAH have a very low rate of longterm responsiveness to oral CCBs, and the value of acute vasodilator testing in such patients needs to be individualized. IPAH patients in whom CCB therapy would not be considered, such as those with right heart failure or hemodynamic instability, should not undergo acute vasodilator testing. aCCBs are indicated only for patients who have a positive acute vasodilator response, and such patients need to be followed closely for both safety and efficacy. bFor patients who did not have a positive acute vasodilator testing and are considered lower risk on the basis of clinical assessment (Table 10.4), oral therapy with endothelin receptor antagonist (ERA) or phosphodiesterase-5 inhibitor (PDE-5 I) would be the first line of therapy recommended. If an oral regimen is not appropriate, the other treatments would need to be considered on the basis of patient’s profile and the side effects and risk of each therapy. cFor patients who are considered high risk on the basis of clinical assessment (Table 10.4), continuous treatment with intravenous (IV) prostacyclin (epoprostenol or treprostinil) would be the first line of therapy recommended. Combination therapy should be considered when patients are not responding adequately to initial monotherapy. Timing for lung transplantation and/or atrial septostomy is challenging and is reserved for patients who progress despite optimal medical treatment. Abbreviations: IPAH, idiopathic pulmonary arterial hypertension; PAH, pulmonary arterial hypertension; CCBs, calcium channel blockers. Source: From Ref. 68.