Primary pulmonary hypertension

A new disease profile is added to our database – Primary pulmonary hypertension (PPH). It is an uncommon disease with an estimated incidence of 2 cases per million. There is a strong female predominance, with most patients presenting in the fourth and fifth decades, although the age range is from infancy to >60 years. The natural history of PPH is uncertain because initially the disease can be asymptomatic. Because the predominant symptom is dyspnea, which can have an insidious onset, the disease is typically diagnosed late in its course. Prior to current therapies, a mean survival of 2 to 3 years from the time of diagnosis was reported. Functional class remains a strong predictor of survival, with patients who are in New York Heart Association (NYHA) functional class IV having a mean survival of <6 months. The cause of death is usually RV failure, which is manifest by progressive hypoxemia, tachycardia, hypotension, and edema. Familial PPH accounts for 12 to 20% of cases of PPH and is characterized by autosomal dominant inheritance, variable age of onset and incomplete penetrance. To receive a full description of this rare disease absolutely free of charge, please click here and send us a request. The profile was written by our consultant in internal diseases – Dr. Nikolay Botushanov, MD.