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Course: Medical Surgical Nursing

Topic: Pulmonary Hypertension and Pulmonary Embolism

The Nurses International Community

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Module Goals

Learners will be able to

Describe the clinical manifestation of pulmonary hypertension and pulmonary embolism.
Discuss the assessment and diagnostic findings of pulmonary hypertension and pulmonary embolism.
Discuss the complications of pulmonary hypertension and pulmonary embolism.
Describe the medical management of pulmonary hypertension and pulmonary embolism.
Discuss the nursing management of pulmonary hypertension and pulmonary embolism.

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Review of Pulmonary Blood Flow

The right side of the heart receives deoxygenated blood from the body.

Deoxygenated blood moves through the right atrium to the right ventricle.
The right ventricle pumps blood from heart to the lungs via the pulmonary artery.
The deoxygenated blood becomes oxygenated the lungs.

The lungs return the oxygenated blood to the left side of the heart.

Oxygenated blood moves through the left atrium to the left ventricle.
The left ventricle pumps the oxygenated blood to the body.

American Heart Association, 2022

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Pulmonary Hypertension (PH): Definition

An abnormally high amount of pressure in the blood vessels that carry oxygen from the right ventricle of the heart to the lungs.

These vessels are pulmonary arteries.
There is increased musculature and narrowing, which limits blood flow to the lungs.

Results in decreased oxygenation to the lungs and therefore less oxygen to the body.

CDC, 2019

NHS, 2020b

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Pulmonary Hypertension: Pathophysiology

Pathophysiology depends on the underlying process and type of pulmonary hypertension:

Pulmonary arterial hypertension
Pulmonary venous hypertension due to left heart disease
Lung disease and hypoxemia
Blood clots
Unclear mechanism due to multiple factors

CDC, 2019

Rose-Jones & Mclaughlin, 2015

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According to the World Health Organization, pulmonary hypertension can be classified into five groups:

Pulmonary arterial hypertension occurs due to changes in the small branched arteries in the lungs.
Pulmonary venous hypertension linked to left heart disease is when the left side of the heart does not pump adequately, the right side of the heart works harder, and this increases blood pressure in the lungs.
Pulmonary hypertension due to lung disease or hypoxemia is often found in patients with chronic obstructive pulmonary disease; the vessels in the lungs constrict and narrow due to persistently low oxygen levels.
Chronic thromboembolic pulmonary hypertension occurs due to chronic blood clots, which lead to narrowing or blockage of the pulmonary blood vessels; these blood clots are also known as pulmonary emboli.
Pulmonary hypertension due to unclear multifactorial (multiple factors) mechanisms is related to underlying diseases such as glycogen storage disease, thyroid disorder or sarcoidosis.

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Pulmonary Hypertension: Etiology

Congenital heart disease
Connective tissue disease
Coronary artery disease
High blood pressure
Liver disease (cirrhosis)
Pulmonary emboli
Chronic lung diseases

CDC, 2019

NHS, 2020c

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Pulmonary Hypertension: Risk Factors

It affects all ages, however there is increased incidence with age.

Most common in

Women
Non-Hispanic Black people
People age 75 years or older

Associated with other heart and lung conditions.

CDC, 2019

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Pulmonary Hypertension: Clinical Manifestations

Dyspnea, at rest or on exertion
Fatigue
Dizziness or lightheadedness
Chest pain
Palpitations
Edema:

legs
ankles and feet
abdomen

Decreased appetite
Cyanosis

AHA, 2022

NHS, 2020b

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Critical Thinking Question

Indicate whether the following statements about PH are true (T) or false (F):

Dyspnea is only evident in severe cases of PH.
PH might lead to right heart ventricular failure.
PH is characterized by a decreased pulmonary arterial pressure.
PH is more common in older adults.

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Pulmonary Hypertension: Assessment

Obtain patient history and perform clinical assessment

Discuss current symptoms
Evaluate family history
Discuss current medications
Obtain past and present medical conditions

Physical exam

Perform comprehensive cardiac and respiratory assessment
Assess for

hypoxia
edema
cyanosis
vital sign changes

NHS, 2020b

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Pulmonary Hypertension: Diagnostic Findings

Echocardiogram

Utilizes high frequency sound waves.
Estimates pressures in the pulmonary arteries.
Evaluates cardiac function.

Right heart catheterization

A catheter is inserted into the neck, arm or groin and threaded to the heart.
The catheter measures the pressures in the right side of the heart and pulmonary arteries.

NHS, 2020d

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Pulmonary Hypertension: Other Diagnostic Findings

Electrocardiogram (ECG)
Chest X-ray
Pulmonary function tests
Exercise tests
Ventilation-perfusion scan (VQ scan)
Blood tests

NHS, 2020d

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Critical Thinking Question

The nurse cares for a client with PH. Which of the following assessment findings would the nurse expect in PH? (Select all that apply)

Cyanosis
Edema
Hypoxia
Neutropenia
Night sweats
Changes in vital signs

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Complications of Pulmonary Hypertension

Vascular:

Pulmonary dilation
Pulmonary artery dissection
Hemorrhage to the alveoli
Hemoptysis
In situ thrombosis
Complications from balloon angioplasty

AHA, 2022

Mak et al., 2017

Oldroyd et al., 2023

Pulmonary Hypertension, n.d.

Cardiac:

Right heart failure
Pericardial effusion and cardiac tamponade
Cardiac cirrhosis
Arrhythmias

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Dilation of the pulmonary artery occurs due to increased stress on the walls of the artery caused by increased pressure. The dilated pulmonary artery can compress the left main stem coronary artery, which could result in chest pain, acute MI, or sudden death.

Pulmonary artery dissection is rare and is the presence of a tear in the pulmonary artery. It occurs due to chronic pulmonary hypertension or as an adverse effect of cardiac catheterization.

Hemorrhage to the alveoli can be associated with anticoagulation therapy or pulmonary vasodilators. It can occur secondary to the rupture of an aneurysm in the pulmonary artery.

In situ thrombosis is a blood clot that originates in the pulmonary arteries. Unlike a pulmonary embolism, which is a blood clot that has traveled from another location to the lungs, the in situ thrombosis forms in the vessels of the lungs. This is most common in cases of severe pulmonary hypertension regardless of underlying etiology.

A balloon pulmonary angioplasty may be indicated for patients unable to undergo surgery. Complications from balloon angioplasty include reperfusion edema and perforation of the pulmonary artery (which is often fatal).

Increased pressure in the pulmonary vessels increases the workload of the right ventricle, weakening the ventricle over time. Eventually, the heart can no longer efficiently pump enough blood to the lungs. This result is right heart failure. (AHA, 2022).

The exact mechanism of action for the development of pericardial effusion and cardiac tamponade is unknown; however, it is hypothesized that it is related to increased pressure to the right side of the heart.

Cardiac cirrhosis refers to the damage to the liver that is caused by congestion from right-sided cardiac dysfunction. When the right ventricle cannot pump blood adequately to the lungs, the deoxygenated blood can build up in the right atrium, IVC, and hepatic. The result is congestion in the liver, which leads to liver fibrosis/cirrhosis.

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Complications of Pulmonary Hypertension

Central line complications
Pulmonary:

Cavitation
Infection

Other:

Pregnancy complications
Anemia

Mak et al., 2017

Oldroyd et al., 2023

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Critical Thinking Question

Give example of PH complications.

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Pulmonary Hypertension: Medical Management

Pulmonary arterial hypertension

Anticoagulants
Diuretics
Oxygen
Digoxin

Specialized medications

Endothelin receptor antagonists
Phosphodiesterase 5 inhibitors
Prostaglandins
Soluble guanylate cyclase stimulators
Calcium channel blockers

NHS, 2020e

NHLBI, 2022b

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Treatment for pulmonary hypertension depends on cause and severity. The following can be indicated for pulmonary arterial hypertension:

Anticoagulants may be prescribed when blood clots are present.
Diuretics can help remove excess fluid in cases of heart failure
Oxygen helps to reduce hypoxia
Digoxin helps improve heart contractility and decrease the heart rate

There are multiple different classes of medications that can be prescribed to help reduce the pressure in the lungs and dilate the pulmonary vasculature. Examples of endothelin receptor antagonists include bosentan and ambrisentan. Phosphodiesterase 5 inhibitors include sildenafil and tadalafil. Prostaglandins include epoprostenol and treprostinil. Riociguat is a type of soluble guanylate cyclase stimulator. Calcium channel blockers include nifedipine, amlodipine, and nicardipine.

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Pulmonary Hypertension: Medical Management

Surgeries and procedures

Pulmonary endarterectomy
Balloon angioplasty
Atrial septostomy
Heart valve repair
Transplant

Additional treatments

Iron supplements
Blood transfusion or hydroxyurea
Blood pressure medications

NHS, 2020e

NHLBI, 2022b

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Pulmonary endarterectomy is a surgical procedure that removes blood clots from the pulmonary arteries.

During a balloon pulmonary angioplasty, a balloon is inserted through the pulmonary arteries and inflated to reduce the blockage and improve blood flow. This procedure may be indicated when the patient is not suitable for surgery.

A heart valve repair may be indicated if a dysfunctional valve is the underlying cause.

During an atrial septostomy, a catheter creates a hole between the two atria to reduce the workload and pressure on the right side of the heart.

A lung transplant or heart-lung transplant, while rare, can be performed in severe cases.

Iron supplements may be indicated in cases of anemia. Blood transfusions or hydroxyurea may be indicated if the underlying cause is related to sickle cell disease. Blood pressure medications are indicated if the left-sided heart dysfunction is the underlying cause.

Hydroxyurea is a chemo drug used to treat chronic myeloid leukemia and us used in sickle cell disease to help prevent the formation of sickle-shaped red blood cells.

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Pulmonary Hypertension: Nursing Management

Patient monitoring
Oxygen therapy
Assist with diagnosis: blood work, imaging
Patient education

Medications
Fall prevention
Healthy lifestyle changes
Adherence
Immunizations

AHA, 2022

Kingman & Vaqar 2022

NHLBI, 2022a

Contact info: info@nursesinternational.org

The nurse should monitor patient vital signs, assess for signs of pulmonary hypertension, and evaluate for clinical deterioration. Oxygen therapy may be indicated if the patient exhibits hypoxia. The nurse may need to assist with obtaining blood work and assisting with imaging for diagnostic purposes.

The nurse’s role is pivotal in providing education to patients to improve outcomes. It is important to discuss any adverse reactions the patient may experience from medications. For example, many intravenous therapies used to treat pulmonary hypertension can result in hypotension, flushing, or headache.

Patients are often at increased risk for falls related to signs and symptoms of pulmonary hypertension (lightheadedness, syncope, fatigue, dyspnea on exertion). Nurses can provide education on healthy lifestyle changes such as eating a heart-healthy diet and participating in an exercise program such as through pulmonary rehabilitation. Other lifestyle changes include encouraging the patient to quit smoking, maintain a healthy weight, avoid hot tubs and saunas (that lower blood pressure), participate in regular physical activity, be cautious with air travel and high altitude locations, find a support group for people with pulmonary hypertension, and reduce anxiety and stress as much as possible. Nurses should assess and address barriers to adherence with medication regimens, oxygen therapy, and healthy lifestyle choices. The nurse can administer immunizations to reduce further complications.

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Critical Thinking Question

A client is being discharged from the hospital after a 7-day stay for complications of PH. Which of the following lifestyle modifications is a priority in this case?

Cereals are a great option for breakfast and snack
Eat a low-sodium diet
Eliminate iron-rich foods
Increase fiber and cheese intake

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Pulmonary Embolism (PE): Definition

Blood clot in the lungs.
Causes blockage of the arteries that supply the lungs.
Blockage can be complete or partial.
Blood clot also originates as a thrombus by forming in another part of the body.

When it breaks off and spreads, it becomes an embolism.
May originate in the veins of the legs, heart, pelvis, abdomen.
Blood clots that originate in the legs or pelvis cause the most clinically significant pulmonary embolisms.

Healthdirect, 2021

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Pulmonary Embolism: Pathophysiology

Blood clot breaks off and enters the pulmonary artery.
Large blood clots block the main pulmonary artery and smaller ones may block a smaller pulmonary artery.
Leads to V/Q mismatch.
Mediators trigger vasoconstriction in parts of the lung not impacted.
Increased pulmonary artery pressure negatively impacts the heart.
Can lead to myocardial ischemia with severe decompensation and sudden death.

Tarbox & Swaroop, 2013

Contact info: info@nursesinternational.org

Blood clots that form may also be termed deep venous thrombosis. When the blood clot breaks off, it moves with the flow of blood back to the heart. If the blood clot is large, it can block the main pulmonary artery. If the blood clot is smaller, it may travel through the main pulmonary artery and block one of the smaller pulmonary arteries.

V/Q mismatch occurs when there is ventilation of the lungs, but the blood flow in the vessels of the lungs is not adequate, resulting in a “mismatch” in the exchange of gas molecules between the capillaries and alveoli.

When the embolus enters the lung, certain chemicals, known as mediators, can be released in response to the activated platelets. Unfortunately, these mediators can trigger vasoconstriction in the areas not impacted by the embolus, which can worsen pulmonary function.

When there is a blood clot in one of the pulmonary arteries and vasoconstriction results, there is a build-up of pressure in the pulmonary artery as the blood cannot flow through it as easily. The increased pressure in the pulmonary artery increases the workload of the right ventricle. The right ventricle must pump harder to perfuse the lungs and maintain cardiac output. Eventually, the right ventricle becomes overworked and right heart failure results. As right heart failure develops, the same amount of blood no longer reaches the left ventricle from pulmonary circulation, causing decreased filling of the left ventricle. When this happens, myocardial ischemia can occur with inadequate perfusion of the coronary arteries. Syncope, hypotension, pulseless electrical activity and sudden death may follow.

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Pulmonary Embolism: Etiology and Risk Factors

Virchow’s triad:

Hypercoagulability
Venous stasis
Injury to the vessel wall

Risk factors can be classified as

Inherited or acquired
Strong, medium, or weak association

Tarbox & Swaroop, 2013

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Virchow’s triad provides a model for three mechanisms that lead to the formation of blood clots: hypercoagulability(a state in which the blood has a tendency to coagulate or form clots), venous stasis (blood in the veins does not move back to the heart effectively), and vessel wall injury.

Examples of inherited risk factors: hyperhomocysteinemia (weak association), mutation in the factor V Leiden, deficiency in coagulation inhibitors (strong association)

Examples of acquired risk factors: bed rest, obesity, knee replacement, hormone replacement therapy, fracture, or major surgery

Examples of strong risk factors: myocardial infarction, stroke, surgery, trauma, immobilization, prior blood clots, advanced age, central venous catheter, thrombophilia, cancer

Examples of medium risk factors: hormone replacement therapy, smoking, pregnancy, obesity

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Pulmonary Embolism: Clinical Manifestations

Level of severity: asymptomatic to sudden death
Variability in signs and symptoms:

Chest or upper back pain
Shortness of breath
Tachycardia
Hypoxemia
Syncope
Shock
Leg pain or swelling
Cough

NHS, 2020a

Tarbox & Swaroop, 2013

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Critical Thinking Question

The nurse conducts the physical examination of a client with PE. Which of the following clinical manifestations are consistent with the diagnosis? (Select all that apply).

Chest pain
Cough
Dyspnea
Hypoxemia
Leg pain or swelling
Tachycardia

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Pulmonary Embolism: Assessment

Evaluate risk factors and probability.
Assess clinical stability.
Scoring systems assist providers with determining probability of pulmonary embolism.

Patient stability and signs and symptoms are used for scoring.
Results help direct further diagnostic testing.

Tarbox & Swaroop, 2013

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Pulmonary Embolism: Diagnosis

Blood tests: D-dimer
Extremity venous ultrasound
Computed tomography angiography
V/Q scan
ECG
Echocardiogram

Tarbox & Swaroop, 2013

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A D-dimer test may be indicated in a stable patient if there is less clinical suspicion and the patient has not undergone a recent surgery or trauma. Because the D-dimer test reflects the breakdown of blood clots, it is often not useful in certain settings, such as with recent surgery or trauma.

An extremity ultrasound can be ordered to diagnose a blood clot in an extremity. However, there are many limitations to this modality, such as a lack of qualified personnel to perform the test, patient factors (obesity, edema, dressings) that impact accuracy and feasibility, and the inability to detect blood clots in the pelvic veins.

Computed tomography angiography is a test that can be used to evaluate the blood vessels in the lungs. This may be preferred over the V/Q scan as it is a faster way to diagnose a pulmonary embolism and may also provide information about other disease processes that are present. If the patient has an allergy to contrast dye, a V/Q scan can identify the pulmonary embolism. The V/Q scan assesses the ventilation and perfusion within the lung and can detect whether there is a mismatch between the two.

An ECG may be helpful to identify electrical changes to the heart related to a PE. An echocardiogram provides a direct sonographic image of the heart and can detect right ventricular dysfunction and a specific sign known as McConnell’s sign that has a high predictive value for a PE.

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Pulmonary Embolism: Complications

Complications often depend on the size of the PE
A large PE could lead to

cardiac dysfunction
pulmonary dysfunction
sudden death

Recurrence affects 1 in 3 people

Healthdirect, 2021

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Pulmonary Embolism: Medical Management

Anticoagulation

Systemic anticoagulants
Intravenous, subcutaneous, or oral routes

Supportive care

Monitoring
Oxygen administration

Fibrinolytic treatment
Surgery
Inferior vena cava filter

Healthdirect, 2021

Tarbox & Swaroop, 2013

Contact info: info@nursesinternational.org

In inpatient settings, anticoagulants may be given through the intravenous route, such as with a heparin drip or via the subcutaneous route, such as with low molecular weight heparin (LMWH). Unfractionated heparin, or LMWH, is the primary treatment. Newer oral options for anticoagulation include rivaroxaban.

Prompt initiation of anticoagulation is necessary to prevent decompensation. In patients who present to the emergency department, anticoagulation therapy should be started promptly once a diagnosis has been confirmed.

Supportive care should include close patient monitoring, such as in an intensive care setting with the administration of oxygen for the treatment of hypoxemia.

Fibrinolytic therapy may be indicated in patients with hemodynamic instability. This treatment may be given pharmacologically (in the form of an intravenous medication) and mechanically (using a catheter to directly insert the medication to break up the embolus). The blood clot may also be removed surgically in severe cases.

Inferior vena cava filters may be indicated in patients who have experienced bleeding, have contraindications to anticoagulation therapy, or have persistent PEs. These filters help to reduce the recurrence of PEs but do not reduce mortality.

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Pulmonary Embolism: Nursing Management

Hemodynamic monitoring
Oxygen administration
Facilitate diagnostic testing
Anticoagulation administration

Monitoring for bleeding

Patient education

Anticoagulant
Monitoring
Risk reduction

Healthdirect, 2021

Tarbox & Swaroop, 2013

Contact info: info@nursesinternational.org

One of the primary roles of the nurse includes close patient monitoring to evaluate for signs of clinical decompensation. For example, the patient may be placed on continuous telemetry with a pulse oximeter. The nurse may administer oxygen if the patient experiences hypoxia. Due to the risk of sudden death and the need for immediate anticoagulation in the case of a PE, the nurse often plays a pivotal role in the timeliness of diagnostic testing. The nurse communicates with the radiology team and prepares the patient for testing. The nurse administers anticoagulation therapy, which requires monitoring of therapeutic levels and signs of bleeding and thrombocytopenia. The nurses can educate the patient about the anticoagulation therapy, discuss steps for monitoring, and determine ways to reduce the risk of further blood clots.

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Pulmonary Embolism:

Nursing Management (Continued)

Prevention

Compression stockings
Promote physical activity
Discharge education

When to seek emergency help:

Respiratory distress, fast heart beat, or fainting

Healthdirect, 2021

NHS, 2020a

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Critical Thinking Question

Which nursing intervention would be of highest priority for a hospitalized client who has been diagnosed with PE?

Administer oxygen
Advise to avoid physical activity
Encourage compression stockings
Increase dietary intake of protein

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Red Flags

Respiratory distress
Chest or upper back pain
Fainting or dizziness
Palpitations
Leg swelling or pain
Fatigue
Cyanosis

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Cultural Considerations

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Health beliefs: In some cultures talking about a possible poor health outcome will cause that outcome to occur.
Health customs: In some cultures family members play a large role in health care decision-making.
Ethnic customs: Differing gender roles may determine who makes decisions about accepting & following treatment recommendations.

AHRQ, 2020

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Cultural Considerations (Continued)

Religion, culture, beliefs, and ethnic customs can influence how families understand and use health concepts:

Religious beliefs: Faith and spiritual beliefs may affect health seeking behavior and willingness to accept treatment.
Dietary customs: Dietary advice may be difficult to follow if it does not fit the foods or cooking methods of the family.
Interpersonal customs: Eye contact or physical touch may be ok in some cultures but inappropriate or offensive in others.

AHRQ, 2020

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References

Agency for Healthcare Research and Quality (AHRQ). (2020). Health literacy universal precautions toolkit (2nd ed.). U.S. Department of Health and Human Services, National Institutes of Health. https://www.ahrq.gov/health-literacy/improve/precautions/tool10.html

American Heart Association. (2022). Pulmonary hypertension-high blood pressure in the heart-to-lung system. https://www.heart.org/en/health-topics/high-blood-pressure/the-facts-about-high-blood-pressure/pulmonary-hypertension-high-blood-pressure-in-the-heart-to-lung-system

Centers for Disease Control and Prevention [CDC]. (2019). Pulmonary hypertension. https://www.cdc.gov/heartdisease/pulmonary_hypertension.htm#:~:text=What%20is%20pulmonary%20hypertension%3F,wall%20of%20the%20blood%20vessels

Healthdirect. (2021). Pulmonary embolism. https://www.healthdirect.gov.au/pulmonary-embolism

Contact info: info@nursesinternational.org

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References

Kingman, M., & Vaqar, S. (2022). Increasing awareness about pulmonary arterial hypertension treatment for nurses. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK580476/

Mak, S. M., Strickland, N., Gopalan, D. (2017). Complication of pulmonary hypertension: A pictorial review. The British Journal of Radiology. 90(1070), 20160745.
https://doi.org/10.1259/bjr.20160745

National Health Service [NHS]. (2020a). Pulmonary embolism. https://www.nhs.uk/conditions/pulmonary-embolism/

Contact info: info@nursesinternational.org

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References

National Health Service [NHS]. (2020b). Pulmonary hypertension. https://www.nhs.uk/conditions/pulmonary-hypertension/

National Health Service [NHS]. (2020c). Pulmonary hypertension: Causes. https://www.nhs.uk/conditions/pulmonary-hypertension/causes/

National Health Service [NHS]. (2020d). Pulmonary hypertension: Diagnosis. https://www.nhs.uk/conditions/pulmonary-hypertension/diagnosis/

National Health Service [NHS]. (2020e). Pulmonary hypertension: Treatment. https://www.nhs.uk/conditions/pulmonary-hypertension/treatment/

Contact info: info@nursesinternational.org

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References

National Heart, Lung and Blood Institute [NHLBI]. (2022a). Pulmonary hypertension: Living with. https://www.nhlbi.nih.gov/health/pulmonary-hypertension/living-with

National Heart, Lung and Blood Institute [NHLBI]. (2022b). Pulmonary hypertension: Treatment. https://www.nhlbi.nih.gov/health/pulmonary-hypertension/treatment

Oldroyd, S. H., Manek, G., Sankari, A., & Bhardwaj., A. (2023). Pulmonary hypertension. National Institutes of Health. National Library of Medicine. https://www.ncbi.nlm.nih.gov/books/NBK482463/

Pulmonary Hypertension. (n.d.) Pulmonary hypertension complications. Bio News. https://pulmonaryhypertensionnews.com/pulmonary-hypertension-complications/

Contact info: info@nursesinternational.org

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References

Rose-Jones, L. J., & Mclaughlin, V. V. (2015). Pulmonary hypertension: Types and treatments. Current Cardiology Reviews, 11(1), 73–79. https://doi.org/10.2174/1573403x09666131117164122

Tarbox, A. K., & Swaroop, M. (2013). Pulmonary embolism. International Journal of Critical Illness and Injury Science, 3(1), 69–72. https://doi.org/10.4103/2229-5151.109427

Contact info: info@nursesinternational.org

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