Pulmonary hypertension is considered present when the mean PA pressure is
greater than 25 mm Hg at rest or 30 mm Hg during exercise.
Etiology and Classification
- Secondary pulmonary hypertension is present when there is a identifiable cause
with hemodynamic sequelae leading to pulmonary hypertension. Disease of the left heart,
pulmonary embolism, chronic hypoxemia and left to right shunts are common etiologies.
- Primary pulmonary hypertension is obviously idiopathic. However, it is often
associated with cirrhosis , AIDS and can be familial.
- Primary pulmonary hypertension often occurs in young females. Dyspnea on
exertion, fatigue, syncope, dizziness, edema and angina occur during the course of
- Loud pulmonary component of the second heart sound, pulmonary ejection click, RV
lift, S4 gallop, syst murmur of tricuspid regurgitation and a diastolic murmur of
pulmonary regurgitation can be recognized. Findings are subtle and the diagnosis is often
- Complex testing is often required to establish the presence of pulmonary
hypertension and to rule out secondary causes.
- Prognosis is poor and five year survival is between 22-33%.
- See cor pulmonale for therapeutic measures for secondary pulmonary hypertension.
- Anticoagulation, Calcium channel blocks, Nitric oxide and Prostacyclin are
current therapeutic options.
- Anticoagulation has to given indefinitely.
- 20-35 % of patients respond to vasodilators acutely and benefit from their use.
- Infusion of prostacycline, continuously and chronically, improves hemodynamics,
symptoms and survival. This requires implantation of a permanent central venous catheter,
use of an external portable infusion pump and comprehensively trained support staff.
- Lung transplant is an option for patients refractory to medical management.