2. Understand the physiology of pulmonary
hypertension.
Understand the signs and symptoms of pulmonary
hypertension.
Types pulmonary hypertension.
Understand the treatment of a pulmonary
hypertension and a crisis.
8. Any condition that causes chronic low oxygen levels in
the blood
Autoimmune diseases that damage the lungs, such as
scleroderma and rheumatoid arthritis
Certain birth defects of the heart
Certain diet medications
Congestive heart failure
History of a blood clot in the lung
HIV infection
Lung or heart valve disease
Obstructive sleep apnea
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Shortness of breath or light-headedness during
activity is often the first symptom.
Over time, symptoms occur with lighter activity or
even while at rest
10. Ankle and leg swelling
Bluish color of the lips or skin (cyanosis)
Chest pain or pressure, usually in the front of the
chest
Dizziness or fainting spells
Fatigue
Weakness
13. In congenital heart disease with a left to right
shunt, high pulmonary blood flow increases shear
stress and causes obstructive pulmonary
hypertension.
Existing pulmonary hypertension is exacerbated
post cardiopulmonary bypass due to reperfusion
injury causing further endothelial dysfunction.
14. Abnormal heart sounds (especially a splitting of
the second heart sound)
Enlargement of the veins in the neck
Feeling of a pulse over the breastbone
Heart murmur
Leg swelling
Liver and spleen swelling
Normal breathing sounds
15. Cardiac catheterization
Chest x-ray
CT scan of the chest
Echocardiogram
ECG
Nuclear lung scan
Pulmonary arteriogram
Pulmonary function tests
Sleep study
17. Right heart strain (RVH and RA enlargement)
Right axis deviation
ST depression and T wave inversion V1-V3
18. There is no known cure.
The goal of treatment is to control symptoms. It is
important to treat medical disorders that cause
pulmonary hypertension, such as obstructive
sleep apnea, lung conditions, and heart valve
disorders
21. 1. Bag the child in 100% oxygen with high PEEP
and hyperventilate them.
2. Sedate and paralyse the child immediately.
Fentanyl is the drug of choice with these children.
3. Nitric Oxide at 20ppm
A pulmonary hypertensive crisis is an
EMERGENCY. If it is not treated immediately, the
child will DIE!!!
22. Avoid pregnancy
Avoid strenuous activities and heavy lifting
Avoid traveling to high altitudes
Keep up to date with yearly flu and pneumococcal
pneumonia vaccines
Stop smoking
23. The long-term outlook has been poor, but new
therapies may produce better results. Some
people with this condition may develop
progressive heart failure that may lead to death
#21: Bosentan is used to treat pulmonary arterial hypertension (PAH, high blood pressure in the vessels that carry blood to the lungs). Bosentan may improve the ability to exercise and slow the worsening of symptoms in patients with PAH. Bosentan is in a class of medications called endothelin receptor antagonists. It works by stopping the action of endothelin, a natural substance that causes blood vessels to narrow and prevents normal blood flow in people who have PAH.
For female patients:
Do not take bosentan if you are pregnant or plan to become pregnant. Bosentan may harm the fetus. If you are sexually active and able to become pregnant, you should not begin taking bosentan until a pregnancy test has shown that you are not pregnant. You must use a reliable method of birth control and be tested for pregnancy every month during your treatment. Hormonal contraceptives (birth control pills, patches, rings, shots, implants, and intrauterine devices) may not work well when used with bosentan and should not be used as your only method of birth control. Talk to your doctor about birth control methods that will work for you. Call your doctor immediately if you miss a period or think that you may be pregnant while you are taking bosentan