PULMONARY HYPERTENSION

 A type of high blood pressure that affects the arteries in your lungs and the right side of the
heart.
 Syndrome resulting from restricted blood flow through the pulmonary arterial circulation from
vascular proliferation, abnormal remodeling and in situ thrombosis resulting in increased
pulmonary resistance and ultimately right sided heart failure.
 PCWP: >25mmHg at rest; >30mmHg with exercise.

Classification of Pulmonary Hypertension (WHO 1998)

Grp 1: Pulmonary Arterial Hypertension

 Cause: Unknown; Idiophatic pulmonary arterial hypertension
 A specific gene mutation that can cause PH to develop in families, also called heritable HAP
 Drugs: certain prescription diet drug or illegal drugs such as methamphetamines or certain
toxins
 Heart abnormalities at birth: congenital heart disease
 Other condition: connective tissue disorders, HIV infection or chronic liver disease
Grp 2. Pulmonary Hypertension caused by Left-Sided Heart Disease
 Left-sided valvular heart disease, such as mitral valve or aortic valve disease
 Failure of the lower left heart chamber.
 Grp 3. PH cause by Lung disease
 COPD, such as emphysema
 Pulmonary disease: pulmonary fibrosis
 Sleep apnea and other sleep disorders
 Long-term exposure to high altitudes in people who may be at higher risk of pulmonary
hypertension.
Grp 4. PH caused by Blood clots
 Chronic thromboembolic pulmonary hypertension (CTEPH)
 Chronic blood clots in the lungs (emboli)
 Grp5. PH associated with other condition that have unclear reasons why the PH occurs
 Blood disorder
 Disorder that affect several organs in the body
 Metabolic disorders
 Tumors pressing against pulmonary arteries

Pulmonary Hypertension: RISK FACTORS

 Family history.
 Obesity and obstructive sleep apnea.
 Gender.
 Pregnancy.
 Altitude
 Other diseases.
 Drugs and toxins.
Pulmonary Hypertension: PATHOPHYSIOLOGY
1. Abnormal High BP in the pulmonary arteries
2. Inc. pressure damages in the large and small pulmonary arteries
3. Thickness of blood vessel walls
4. Inability to transfer oxygen and carbon dioxide normally
5. Fall of blood oxygen level
6. Constriction of pulmonary arteries
7. Inc. in pressure in pulmonary circulation

Pulmonary Hypertension: COMPLICATIONS:

 Right-sided heart enlargement and heart failure (cor pulmonale)
 Blood clots
 Arrhythmia
 Bleeding

Pulmonary Hypertension: SIGN AND SYMPTOMS

o Shortness of breath
o Fatigue
o Dizziness or fainting spells
o Chest pain
o Swelling (edema)
o Bluish color to your lips and skin (cyanosis)
o Racing pulse or heart palpitations
o Decreased appetite
o Coughing and wheezing
o Distended neck veins
o Enlarged liver

Pulmonary Hypertension: DIAGNOSTIC EXAM

 Chest x ray.
 EKG (electrocardiogram).
 Right heart catheterization.
 Chest CT scan.
 Chest MRI.
 Lung function tests
 Lung ventilation/perfusion (VQ) scan.
 Blood tests.

Pulmonary Hypertension: PHARMACOLOGIC TREATMENT

− Diuretics to decrease fluid accumulation
− Cardiac Glycosides (digitalis) to improve cardiac function
− Calcium Channel Blockers – vasodilation
 − Intravenous prostacyclin - decrease pulmonary hypertension by reducing pulmonary vascular
resistance and pressures and increasing cardiac output
− Anticoagulants - to help prevent the formation of blood clots within the small pulmonary
arteries.
− Endothelin receptor antagonists. These medications reverse the effect of endothelin, a
substance in the walls of blood vessels that causes them to narrow. These drugs may improve
your energy level and symptoms. However, these drugs shouldn't be taken if you're pregnant.
Also, these drugs can damage your liver and you may need monthly liver monitoring.
− Phosphodiesterase-5 inhibitors (Sildenafil and tadalafil.) are sometimes used to treat
pulmonary hypertension. These drugs work by opening the blood vessels in the lungs to allow
blood to flow through more easily. Side effects can include an upset stomach, headache and
vision problems.
− Soluble guanylate cyclase (SGC) stimulator. Soluble guanylate cyclase (SGC) stimulators
(Adempas) interact with nitric oxide and help relax the pulmonary arteries and lower the
pressure within the arteries. These medications should not be taken if you're pregnant. They can
sometimes cause dizziness or nausea.

Pulmonary Hypertension: SURGICAL MANAGEMENT

− Atrial septostomy
− Lung transplant
− Heart–lung transplant

Pulmonary Hypertension: NURSING MANAGEMENT

− Fluid restriction
− Periods of Rest and Physical Activity
− Minimize Sedentary Lifestyle
− Oxygen
− A low-sodium diet that is both low in calories and high in healthy carbohydrates is
recommended
− Anxiety or depression affect approximately one-third of PH patients.