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28 years
How is pulmonary hypertension diagnosed? What is the treatment?
Dec 23, 2014

Dr. Zakia Dimassi Pediatrics

Pulmonary hypertension may be the
result of a number of underlying many medical conditions. 
Pulmonary hypertension is known to have an insidious onset,
i.e., it can develop slowly and a patient may have it for years without being
aware. This is because the condition has no early signs or symptoms.

When symptoms do occur, they're often
like those of other heart and lung conditions, such as asthma. This is why pulmonary hypertension is difficult to diagnose.

By taking a complete medical history and performing a
thorough physical exam, other diseases are ruled out, and the correct diagnosis
is reached. During the physical exam, your health care provider will:

·    - listen for abnormal heart sounds.

·    - examine the jugular vein in the neck for engorgement.

·   - examine the abdomen, legs, and ankles for fluid retention
(manifests as swelling, or edema).

·   - examine nail beds for bluish discoloration.

·   - look for signs of other underlying diseases that might be
causing pulmonary hypertension.

The tests that are usually ordered include blood tests
(including tests that assess your liver, kidney, and thyroid gland functions),
in addition to more specialized tests:

A-   Tests and procedures to confirm the diagnosis

1.  1. Echocardiography. This is required in order to estimate
the blood pressure in the pulmonary arteries (the arteries that bring blood
back to the heart from the lung). Echo also can show the size and thickness of
the right ventricle and how well it's functioning.

     2. Chest X-ray. To see whether the pulmonary arteries and
right ventricle are enlarged; this occurs in the event of having a right
ventricle that is pumping very hard to push blood through the pulmonary
arteries because it’s pushing against elevated pressure in the lungs. A chest x
ray also may show signs of an underlying lung disease that's causing or
contributing to pulmonary hypertension.

b   3. EKG (electrocardiogram). Tracing of the heart's
electrical activity and detects the regularity of the heart's rhythm and can
provide indications if the right ventricle is enlarged or strained.

     4. Right heart catheterization. This procedure directly
measures the pressure in the pulmonary arteries. It also shows how well the
heart is pumping blood to the rest of the body. It can detect any leaks
between the left and right side of the heart.

B- Tests to coin the underlying cause of pulmonary hypertension

1.    Chest CT scan or MRI.

2.    Lung function tests. Measure how much air the patient
can breathe in and out, how fast they can breathe air out, and how well the
lungs deliver oxygen to the blood.

3.    Polysomnogram (PSG). This test records brain activity,
eye movements, heart rate, and blood pressure during sleep. A PSG also measures
the level of oxygen in blood. A low oxygen level during sleep is common in
pulmonary hypertension.

4.    Lung ventilation/perfusion (VQ) scan. Measures the
amount of air and blood flow in the lungs. This test can help detect blood
clots in the lungs vasculature which manifest as a mismatch between the amounts
of blood and air.

Severity if pulmonary hypertension:
this is assessed through exercise testing, which consists of either a 6-minute
walk test or a cardiopulmonary exercise test. A 6-minute walk test measures the
distance that the patient can quickly walk in 6 minutes. A cardiopulmonary
exercise test measures how well the lungs and heart work during exercise on a
treadmill or bicycle. The activity level is rated, and the level is linked to
the severity of pulmonary hypertension. The rating system ranges from class 1
to class 4.

Class 1 has no limits: the patient can do regular physical
activities, such as walking or climbing stairs. These activities don't cause
pulmonary hypertension symptoms, such as tiredness, shortness of breath, or
chest pain.

Class 2 has slight or mild limits: the patient comfortable while
resting, but regular physical activity causes pulmonary hypertension symptoms.

Class 3 has marked or noticeable limits; the patient is
comfortable while resting. However, walking even one or two blocks or climbing
one flight of stairs can precipitate symptoms.

Class 4 has severe limits: almost any physical activity leads to
discomfort and pulmonary hypertension symptoms may also occur while at rest.

Treatment of pulmonary hypertension varies depending on the different underlying causes but
generally includes:

1.   Medications: including oxygen, anticoagulants or
"blood thinners"
, diuretics or "water
pills", Sildenafil

2.   Surgical therapies