Which connective tissue diseases most commonly cause pulmonary hypertension?

Scleroderma (especially CREST syndrome)

Mixed connective tissue disease Systemic lupus erythematosus Rheumatoid arthritis Dermatomyositis

Which occurs more frequently, primary or secondary pulmonary hypertension?

Primary (unexplained) pulmonary hypertension is a rare disorder. Secondary pulmonary hypertension is seen considerably more often in practice.

What population group is most frequently affected by primary pulmonary hypertension?

Although primary pulmonary hypertension occurs in both sexes and virtually all age groups, it has a tendency to affect young females. The female-to-male predominance is 1.7:1, and the mean age is 36 years.

Is surgical therapy now an option for patients with pulmonary hypertension secondary to chronic recurrent thromboembolism?

Appropriate prevention of recurrent thromboembolism continues to be extremely important. In addition to this prevention, it is now possible to remove organized thrombus surgically from the proximal pulmonary arteries of patients with pulmonary hypertension secondary to chronic recurrent thromboembolism. Operative mortality was 8.7% in a recent study from the most experienced center.

What is the average survival for the patient with primary pulmonary hypertension?

According to the National Institutes of Health Registry on Primary Pulmonary Hypertension, the median survival is approximately 2.8 years from the date of diagnosis. It should be noted, however, that there is large interindividual variability. Although some patients progress quickly to death over a period of months, others live for years with little change in symptoms or hemodynamics. Response to therapy with calcium channel antagonists has recently been reported to be associated with an improved prognosis.


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