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Pulmonary Hypertension Clinic

The Pulmonary Hypertension Clinic at The Heart Center is equipped to evaluate, characterize and treat patients with pulmonary hypertension. We have a team of physicians, nurses and medical technicians experienced in the management of this complex condition.

Pulmonary hypertension (PH) refers to any condition that causes elevated pressure within the lung blood vessels, and subsequently strain, on the right side of the heart. The right side of the heart collects unoxygenated (venous) blood coming back to the heart and pumps it to the lungs, where it is re-oxygenated and delivered to the left side of the heart. The left side then pumps the oxygenated (arterial) blood to the rest of the body.

Click here to see a general diagram of the pulmonary systemic circuit.
Click here to see a detailed diagram of the pulmonary systemic circuit.

The World Health Organization (WHO) classifies PH into 5 major categories.

  1.  Group I: Pulmonary Arterial Hypertension
  2.  Group II: PH secondary to left heart diseases
  3.  Group III: PH secondary to lung diseases
  4.  Group IV: PH secondary to chronic lung clots (pulmonary embolism)
  5.  Group V: PH secondary to various reasons such as chronic leukemia, sickle cell anemia and others 

The vast majority of PH is caused by abnormal conditions of the left side of the heart or the lungs. This type of PH can often be improved by conventional methods of treating heart failure and lung diseases like COPD.

By contrast, Pulmonary Arterial Hypertension (PAH) is a rare, progressive condition instigated by a number of diseases that cause characteristic abnormalities of the blood vessels in the lungs. PAH can occur secondary to connective tissue diseases (scleroderma, lupus, etc.), congenital heart diseases or idiopathic (unknown cause). When PAH is present, the left side of the heart is functioning normally. PAH may be treated with a number of specific medications meant to reverse the abnormality of the blood vessels in the lungs and subsequently restore normal pressure to the right side of the heart.

Pulmonary hypertension is often suspected in patients with shortness of breath, lower extremity swelling, chest pain, or in the most extreme of cases, syncope (passing out).

PH can be evaluated noninvasively by an echocardiogram (ultrasound of the heart). However, it must be further characterized and definitively diagnosed by an invasive cardiac catheterization procedure. This involves placement of a catheter enabling measurement of pressures inside the heart and lungs -- a low-risk procedure known as right heart catheterization.

Patients with PAH often present to the PH specialist late due to the delay between the onset of symptoms and diagnosis. Patients with PAH require specialized care, medications and follow up. Early diagnosis is paramount to delay illness progression, improve lifestyle and reduce mortality.


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