• Primary Pulmonary Hypertension
• Pulmonary Hypertension
• Pulmonary Arterial Hypertension
• PPH Symptoms
• PPH Diagnosis
• PPH Causes
• PPH Treatment
  • Ambrisentan
  • Tracleer
  • Flolan
  • Remodulin
  • Prostacyclin
  • Lung Transplants
  • Thelin
• Diet Drugs and PPH
• Legal Remedies for PPH

Primary Pulmonary Hypertension Diagnosis PPH Diagnosis

Diagnosis of primary pulmonary hypertension (PPH) typically requires several tests. It is often not detected in a routine clinical examination because many of the PPH symptoms are similar to other disease conditions of the heart and lung, such as shortness of breath, dizziness, weakness, swelling of the ankles and extremities, etc. Treatment with diet drugs including Fen Phen, Redux (dexfenfluramine) and Pondimin (fenfluramine) have been shown to cause primary pulmonary hypertension in some patients.

Elevated Pulmonary Pressure

Pulmonary hypertension is characterized by elevated pressure in the artery connecting the heart and lung (pulmonary artery). The average normal pressure in this artery is 18 mm Hg, while for a patient with PPH, the average pressure is 25 mm Hg. In order to measure the pulmonary artery pressure directly, a heart catheterization is needed. During catheterization to measure pulmonary artery pressure, a thin, flexible tube, called a Swan-Ganz catheter, is inserted into a vein in the upper chest, neck or upper leg area. The catheter is specially designed to measure heart and artery pressure, it is highly flexible and has a balloon tip. The catheter is treaded through the major arteries of the body, through the right side of the heart and into the pulmonary artery. Once in the pulmonary artery, the balloon tip is inflated and a direct measurement of the pressure can be determined.

In some cases, there are additional components to the catheterization procedure, such as exercise or introduction of vasodilator drugs. These additional components can aid the physician in determining the extent of the disease and how well the patient will respond to certain types of treatment. During exercise, the physician can determine when the patient reaches his or her anaerobic threshold, or the level at which the body cannot increase its intake of oxygen. Pulmonary hypertension patients reach this threshold much more quickly than normal patients because of the inability of the pulmonary artery to carry enough blood to the lungs. It has also been found that some patients have normal pulmonary artery pressure during rest, but during exercise the presence of the disease becomes apparent. Such tests help the doctor determine the level of dysfunction of the pulmonary artery and how advanced the PPH is.

During catheterization, the patient is sometimes treated with a vasodilator, or a drug that dilates the blood vessels and reduces blood pressure. If the pulmonary artery pressure falls significantly when a patient is dosed with a vasodilator, such as a calcium channel blocker, then it is possible the patient may respond well to such treatment.

Primary Versus Secondary Pulmonary Hypertension

When a patient has elevated pulmonary pressure, it does not necessarily indicate a diagnosis of primary pulmonary hypertension. The elevated pressure can also be directly related to heart or lung disease. When pulmonary hypertension is caused by other factors, the disease is called secondary pulmonary hypertension. In order to specifically diagnose PPH, the doctor must rule out other causes of the elevated pulmonary pressure. Once these have been eliminated as causes, and no other direct cause can be determined, then the diagnosis is primary pulmonary hypertension. Other tests that are typically used to rule out other causes of elevated pulmonary hypertension include standard pulmonary tests, X-rays, Doppler Flow studies and ventilation-perfusion testing.

Diet Drugs and PPH

Use of diet drugs, such as Fen Phen, fenfluramine (Pondimin) and dexfenfluramine (Redux) have been known to cause primary pulmonary hypertension. These patients typically present with a defective heart valve. These heart valve defects can be detected with echocardiography, which is a non-invasive ultrasound examination of the heart. Patients with this known heart defect and primary pulmonary hypertension should be screened for previous treatment with these diet drugs. Some studies have suggested that diet drug patients have normal pulmonary artery pressure during rest, but abnormal levels during exercise. An exercise test during catheterization (described above) can be used to diagnose PPH for these patients.

Last Revision: April 9, 2008

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