The average time from symptom onset to diagnosis has been reported to be approximately 2 years. Despite recent attempts at increasing the awareness of pulmonary arterial hypertension (PAH), especially associated PAH (APAH), this delay in diagnosis has not changed appreciably in recent years.
Early symptoms are nonspecific. Often, neither the patient nor the physician recognizes the presence of the disease, which leads to delays in diagnosis. Complicating matters, idiopathic PAH (IPAH) requires an extensive workup in an attempt to elucidate an identifiable cause of the elevated pulmonary artery pressure.
The most common symptoms and their frequency, reported in a national prospective study, are as follows:
Dyspnea (60% of patients)
Recurrent syncope (13%)
Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper quadrant pain. Cough, hemoptysis, and hoarseness are less common symptoms.
Women are more likely to be symptomatic than men.