pulmonary hypertension

Pulmonary Hypertension

Definition

a type of high blood pressure that affects the arteries in your lungs and the right side of your heart.

Usually pressure in the lung arteries are normally lower than the pressures in the systemic circulation

PH occurs when pressure in the pulmonary circuit becomes abnormally elevated

Classifications of Pulmonary Hypertension

according to WHO 1998 Group 1: Pulmonary arterial hypertension

Cause unknown, known as idiopathic pulmonary arterial hypertension

A specific gene mutation that can cause pulmonary hypertension to develop in families, also called heritable pulmonary arterial hypertension

Certain drugs — such as certain prescription diet drugs or illegal drugs such as methamphetamines — or certain toxins

Heart abnormalities present at birth (congenital heart disease)

Other conditions, such as connective tissue disorders (scleroderma, lupus, others), HIV infection or chronic liver disease (cirrhosis)


according to WHO 1998 Group 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 (left

ventricle)


according to WHO 1998 Group 3: Pulmonary hypertension caused by lung

disease Chronic obstructive pulmonary disease, such as

emphysema Lung disease such as pulmonary fibrosis, a

condition that causes scarring in the tissue between the lungs' air sacs (interstitium)

Sleep apnea and other sleep disorders Long-term exposure to high altitudes in people

who may be at higher risk of pulmonary hypertension


according to WHO 1998 Group 4: Pulmonary hypertension caused by chronic

blood clots Chronic blood clots in the lungs (pulmonary

emboli)


according to WHO 1998 Group 5: Pulmonary hypertension associated with

other conditions that have unclear reasons why the pulmonary hypertension occurs

Blood disorders Disorders that affect several organs in the body,

such as sarcoidosis Metabolic disorders, such as glycogen storage

disease Tumors pressing against pulmonary arteries

Risk Factors Family history. If two or more members of your family have PH or if a

family member in your lineage is known to have a PH-causing gene mutation, the risk of getting PH is more likely.

Obesity and obstructive sleep apnea. In isolation, obesity is not a risk factor. However, if obesity is combined with obstructive sleep apnea (meaning that oxygen levels fall while a person is sleeping), mild PH may occur.

Gender. Idiopathic PAH and heritable PAH (also known as familial PAH) are at least two-and-a-half times more common in women than in men. Females of childbearing age are also more susceptible.

Pregnancy. Pregnancy is a possible risk factor suggested by registries and expert opinion.

Altitude. Living at a high altitude for years can make you more predisposed to PH. When travelling to high altitudes, your PH symptoms can be aggravated by the altitude.

Risk Factors

Other diseases. Other diseases, including congenital heart disease, lung disease, liver disease and connective tissue disorders like scleroderma and lupus, can lead to the development of pulmonary hypertension.

Drugs and toxins. Certain drugs, such as methamphetamines and the diet drug “fen phen,” are known to cause pulmonary hypertension.

Pathophysiology of Pulmonary Hypertension

Abnormally high BP in pulmonary arteries

Increased pressure damages large and small pulmonary arteries

Thickness of Blood vessel walls

Inability to transfer oxygen and carbon dioxide normally

Levels of oxygen in blood fall

Constriction of pulmonary arteries

increase in pressure in pulmonary circulation

Complications of Pulmonary Hypertension

Right-sided heart enlargement and heart failure (cor pulmonale). In cor pulmonale, your heart's right ventricle becomes enlarged and has to pump harder than usual to move blood through narrowed or blocked pulmonary arteries.

At first, the heart tries to compensate by thickening its walls and expanding the chamber of the right ventricle to increase the amount of blood it can hold. But this thickening and enlarging works only temporarily, and eventually the right ventricle fails from the extra strain.


Blood clots. Clots help stop bleeding after you've been injured. But sometimes clots form where they're not needed. A number of small clots or just a few large ones dislodge from these veins and travel to the lungs, leading to a form of pulmonary hypertension that can generally be reversible with time and treatment.

Having pulmonary hypertension makes it more likely you'll develop clots in the small arteries in your lungs, which is dangerous if you already have narrowed or blocked blood vessels.


Arrhythmia. Irregular heartbeats (arrhythmias) from the upper or lower chambers of the heart are complications of pulmonary hypertension. These can lead to palpitations, dizziness or fainting and can be fatal.

Bleeding. Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood (hemoptysis). This is another potentially fatal complication.

Signs and Symptoms Shortness of breath during routine activity, such as climbing a flight of stairs Fatigue Dizziness or fainting spells (syncope) Chest pain Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites) Bluish color to your lips and skin (cyanosis) Racing pulse or heart palpitations Decreased appetite coughing (sometimes with blood) and wheezing distended neck veins enlarged liver

Functional Classification of PH according to WHO

Functional Classification• Class I- Patients with pulmonary hypertension but without resulting limitation of

physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope.

B. Class II- patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope.

C. Class III- patients with pulmonary hypertension resulting in marked. Limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea, fatigue, and chest pain or near syncope.

D. Class IV -patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. these patients manifest signs of right heart failure. Dyspnoea and /or fatigue may be present even at rest. Discomfort is increased by any physical activity

This is done by either a 6-minute walk test or a cardiopulmonary exercise test

Diagnostic Examinations

Assessment/Interview history of present illnesses past medical history Family history Any past or present medications that

the patient may have taken physical examination will also take

place.

Diagnostic Examinations Echocardiography. or echo, uses sound waves to

create a moving picture of your heart. This test can estimate the pressure in your pulmonary arteries. Echo also can show the size and thickness of your right ventricle and how well it's working.

Diagnostic Examinations Chest x ray. takes pictures of the structures in

your chest, such as your heart, lungs, and blood vessels. This test can show whether your pulmonary arteries and right ventricle are enlarged.

The pulmonary arteries and right ventricle may get larger if the right ventricle has to work hard to pump blood through the pulmonary arteries.

A chest x ray also may show signs of an underlying lung disease that's causing or contributing to PH.

Diagnostic Examinations EKG (electrocardiogram). An EKG is a simple,

painless test that records the heart's electrical activity. This test also shows whether your heart's rhythm is steady or irregular. An EKG may show whether your right ventricle is enlarged or strained.


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

During this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your groin (upper thigh) or neck. The tube is threaded into the right side of your heart and into the pulmonary arteries. Through the tube, your doctor can do tests and treatments on your heart.

Diagnostic Examinations, others Chest CT scan. A chest computed tomography (to-MOG-ra-fee) scan, or chest CT scan, creates pictures of

the structures inside your chest, such as your heart, lungs, and blood vessels. These pictures can show signs of PH or a condition that may be causing PH.

Chest MRI. Chest magnetic resonance imaging, or chest MRI, shows how your right ventricle is working. The test also shows blood flow in your lungs. Chest MRI also can help detect signs of PH or an underlying condition causing PH.

Lung function tests. Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests can help detect a lung disease that may be causing PH.

Polysomnogram (PSG). This test records brain activity, eye movements, heart rate, and blood pressure while you sleep. A PSG also measures the level of oxygen in your blood. A low oxygen level during sleep is common in PH, and it can make the condition worse.

A PSG usually is done while you stay overnight at a sleep center. For more information about this test, go to the Diseases and Conditions Index Sleep Studies article.

Lung ventilation/perfusion (VQ) scan. A lung VQ scan measures air and blood flow in your lungs. This test can help detect blood clots in your lung's blood vessels.

Blood tests. Blood tests are used to rule out other diseases, such as HIV, liver disease, and autoimmune diseases (such as rheumatoid arthritis).

Treatments for Pulmonary Hypertension

Pharmacological Treatments 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.


Pharmacological Treatments 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.


Pharmacological Treatments 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.


Surgical Interventions Atrial septostomy (sep-TOS-toe-me). For this procedure, a

thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the upper chambers of your heart). This wall is called the septum. A tiny balloon on the tip of the tube is inflated. This creates an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow.

Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.


Surgical Interventions Atrial septostomy (sep-TOS-toe-me). For this procedure, a thin,

flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the upper chambers of your heart). This wall is called the septum. A tiny balloon on the tip of the tube is inflated. This creates an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow.

Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.

Heart–lung transplant. A heart–lung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.

Heart–lung transplant


Nursing Interventions Fluid restriction

Periods of Rest and Physical Activity/ Minimize Sedentary Lifestyle

Oxygen, especially if you live at a high altitude or have sleep apnea. Some people who have pulmonary hypertension eventually require continuous oxygen therapy.

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. An interdisciplinary team approach is best as appropriate medications can be dispersed for distressing symptoms, and psychological, social, and spiritual support can be provided.

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