Elevated pulmonary artery pressures can be found in a very diverse population of patients. Elevated pulmonary artery pressure can be the patient’s primary problem or secondary to other medical problems. The treatment and prognosis are intimately related to the underlying cause. It is prevalent enough to be seen in most general medicine practices. Five important facts to know about pulmonary hypertension are listed below.



1. Although transthoracic echocardiography (TTE) is the initial screening test of choice, the results vary by more than 10 mm Hg from the gold standard, right heart catheterization, approximately 50% of the time. If pulmonary hypertension (PH) is suggested by TTE, the patient should be referred for right heart catheterization, which both establishes the diagnosis and severity and can test for vasoreactivity, which has implications for treatment.


2. Although vigorous exercise may be detrimental and risk leading to syncope, moderate exercise should be encouraged. In fact, cardiopulmonary rehabilitation has been shown to lead to an improved exercise capacity and quality of life for PH patients.


3. Chronic thromboembolic pulmonary hypertension (CTEPH) is the only potentially curable form of PH; therefore, it is especially important to recognize and treat it. The majority of these patients lack the history of an acute venous thromboembolic event. Many centers continue to use the ventilation/perfusion as the diagnostic test of choice, whereas others have moved to computed tomography as the technology has improved.


4. Combination therapy for the initial management of some patients may be better then monotherapy. The recent AMBITION trial (AMBrIsentan and Tadalafil in patients with pulmonary arterial hypertensION) compared those treated with the combination of ambrisentan and tadalafil with those treated with either one alone as initial management for patients with group 1 PH (idiopathic pulmonary arterial hypertension, hereditary pulmonary arterial hypertension, or pulmonary arterial hypertension that was associated with connective tissue disease, drugs or toxins, HIV [stable disease status], or repaired congenital heart defects).1 The results demonstrated a 50% reduction in first clinical failure (defined as the first occurrence of a composite end point of death, hospitalization for worsening pulmonary arterial hypertension, disease progression, or an unsatisfactory long-term clinical response).


5. Full systemic anticoagulation is indicated for all patients with CTEPH but is not clearly beneficial for all others with PH and, according to some studies, may be harmful.


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  1. Galiè N, Barberà JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015;373:834–44.