Which of the following medications should be initiated for this patient?
A previously healthy 43-year-old woman presents to your office complaining of progressive dyspnea of 6 months duration. After an extensive evaluation, you diagnose idiopathic pulmonary arterial hypertension (IPAH). The patient's transthoracic echocardiogram revealed right heart failure with elevated pulmonary artery pressures. Other causes of pulmonary arterial hypertension were excluded. During right heart catheterization, the patient was noted to have a marked response to inhaled nitric oxide.
A. Oral diltiazem B. Oral bosentan C. Inhaled iloprost D. Intravenous epoprostenol
Oral diltiazem-vasodilators may be helpful for only a select portion of patients with pulmonary hypertension who have evidence of significant vasoreactivity. in general, only 10% of those with ipah and very few with other forms of pah demonstrate such vasoreactivity. for patients who have an acute vasodilator response to inhaled nitric oxide, a trial of a calcium channel blocker, such as nifedipine or diltiazem, should be initiated. the patient should be monitored closely during this time because some patients will deteriorate despite this therapy. if the patient responds, the therapy can be continued and titrated to maximum benefit. patients who do not show a vasodilator response during right heart catheterization should be started on one of the other available agents, such as the oral endothelin receptor antagonists (e.g., bosentan), oral phosphodiesterase type-5 inhibitor (sildenafil), subcutaneous or intravenous treprostinil, inhaled iloprost, or intravenous epoprostenol.