Pulmonary hypertension is a condition in which pressures in the pulmonary artery are persistently elevated. It is therefore not a diagnosis, but instead a physiological condition that may accompany a number of diagnoses.
We have found it useful to think of the causes of pulmonary hypertension as belonging to one of the following three categories:
- Increased vascular resistance
- Increased left atrial pressure
- Increased pulmonary flow
Patients with pulmonary hypertension often complain of shortness of breath and weakness. Additionally, they may experience dizziness, palpitations and/or syncope.
We believe that patients with pulmonary hypertension should receive a basic evaluation that includes the following:
- All patients should have an echocardiogram done to estimate pulmonary artery pressures and to rule out valvular heart disease, left atrial myxoma, atrial/ventricular septal defect, and patent ductus arteriosis.
- A careful history should be done with an emphasis on discovering possible connective tissue disease, a history of venous thromboembolism, sleep apnea, or a family history of primary pulmonary hypertension.
- Pulmonary function tests should be done that include lung volumes and a room air arterial blood gas.
- In the appropriate patient, a V/Q scan should be done to rule out chronic pulmonary emboli.
The treatment of pulmonary hypertension should be directed toward the causative disorder if it can be discovered. In cases in which a secondary cause of pulmonary hypertension cannot be discovered, the treatment is much more challenging. It is our policy to treat these patients with oxygen, diuretics when necessary and calcium channel blockers. We are routinely referring patients to the university centers in which Prostacyclin therapy is delivered with the use of the long-term intravenous pump. We have been very encouraged by the literature and our own experience with the long-term infusion of Prostacyclin.