M.D. The goal of the diagnostic workup for patients with pulmonary hypertension is primarily to differentiate between primary and secondary forms of this disorder. Additionally, if a secondary form is diagnosed, to determine precisely what is the cause so that specific treatment can be applied. The diagnosis of primary pulmonary hypertension is a diagnosis of exclusion. Therefore, the diagnostic workup is basically a search for a secondary cause of this disorder.
History There are essentially four causes of secondary pulmonary hypertension.
The patient's history should focus on obvious associated symptoms that might help define one of these secondary causes, including history of heart murmur or valvular disease, ischemic heart disease or congestive heart failure, chronic obstructive pulmonary disease, high altitude exposure, collagen vascular disease, pulmonary hypoventilation disorders, history of intracardiac shunt or murmur, history of pulmonary embolus or phlebitis and exposure to certain drugs, including weight loss medications. In patients with significant pulmonary hypertension, physical examination will often show features such as right ventricular lift, accentuated P2, murmurs of pulmonary and tricuspid regurgitation as well as features of right heart failure with elevated jugular venous pressure, hepatic enlargement, and peripheral edema. In addition, other physical findings suggestive of a secondary cause, such as those of connective tissue disease, chronic obstructive pulmonary disease, cirrhosis, etc., should be sought. These common tests may show clues to an etiology such as prior myocardial infarction or parenchymal pulmonary disease but in many patients will not define the etiology of the pulmonary hypertension. The role of transesophageal echocardiography is particularly important in the latter.