Patients with primary pulmonary hypertension can be managed based on the severity of their condition and the response to certain drug tests.
Criteria for the diagnosis of primary pulmonary hypertension:
(1) mean pulmonary artery pressure > 25 mm Hg at rest or > 30 mm Hg during exercise
(2) exclusion of left sided cardiac valvular disease, myocardial disease, congenital heart disease, important respiratory disease, important connective tissue disease and thromboembolic diseases
All patients are anticoagulated.
Patient are then divided based on the response to vasoreactivity by challenge test with:
(1) inhaled nitric oxide
(2) intravenous epoprostenol
(3) intravenous adenosine
|
Response to Vasoreactivity Challenge |
Response to Oral Calcium Channel Blocker |
NYHA Class |
Therapy in addition to Anticoagulation |
|
minimal to moderate fall in pulmonary vascular resistance |
NA |
1 or 2 |
possible oral calcium channel blocker therapy |
|
|
NA |
3 or 4 |
epoprostenol; consider transplantation |
|
marked fall in pulmonary vascular resistance |
response |
|
oral calcium channel blocker therapy |
|
|
no response |
1 or 2 |
no additional therapy |
|
|
no response |
3 or 4 |
epoprostenol; consider transplantation |
after Figure 2, page 116
where:
• NA = not applicable
• NYHA = New York Heart Association Class
• oral calcium channel blockers include nifedipine and diltiazem
Specialty: Pulmonology, Cardiology
