Signs and Symptoms of Primary Pulmonary Hypertension (PPH)

Because symptoms of Primary Pulmonary Hypertension (PPH) may develop very gradually, patients may delay seeing a physician for years. Common symptoms of Primary Pulmonary Hypertension (PPH) are shortness of breath fatigue, non-productive cough, angina pectoris fainting or syncope, peripheral edema (swelling around the ankles and feet), and rarely hemptysis (coughing up blood).

Primary Pulmonary Hypertension (PPH) venous typically presents with shortness of breath while lying flat or sleeping (orthopnea) or paroxysmal nocturnal dyspnea), while Primary Pulmonary Hypertension (PPH) arterial typically does not.

A detailed family history is established to determine whether the Primary Pulmonary Hypertension (PPH) might be familial. A history of exposure to drugs such as cocaine, methamphetamines, alcohol leading to cirrhosis, and tobacco leading to emphysema are considered significant. A physical examination is performed to look for typical signs of Primary Pulmonary Hypertension (PPH), including a loud P2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal edema, ascites, hepatojugular reflux, clubbing etc. Evidence of tricuspid insufficiency is also sought and, if present, is consistent with the presence of Primary Pulmonary Hypertension (PPH).