Pulmonary hypertension is a condition in which there are high pressures within pulmonary circulation. 

Pathophysiology of pulmonary hypertension

In normal cardiopulmonary circulation, blood flows easily through the vessels of the lungs where it participates in gas exchange – offloading carbon dioxide and picking up oxygen. However, pathologic changes to pulmonary vasculature lead to vasoconstriction, endothelial dysfunction, smooth muscle hypertrophy, thrombosis, and/or vascular remodeling. The result is that blood flow through the pulmonary vessels is decreased or even occluded, which causes pressures to increase leading to pulmonary hypertension (PH) and increased pulmonary vascular resistance. This causes the right ventricle to have to work harder to overcome this elevated vascular pressure and results in right ventricular hypertrophy and eventually, heart failure.

When PH occurs in newborns it is called persistent pulmonary hypertension of the newborn (PPHN). These babies can have a range of long-term complications including significant neurodevelopmental impairments, hearing impairments, cerebral palsy, and decreased ability to engage in physical activity due to diminished lung function.

Pulmonary Hypertension Subgroups

Group 1 PH: Pulmonary arterial hypertension (PAH) caused by a variety of factors including connective tissue disease, sickle cell anemia, congenital heart disease, HIV, liver disease, certain drugs and toxins (including methamphetamine), and schistosomiasis (a parasitic infection). In some cases there is no known cause, in which case it is “idiopathic PAH” and in other cases it can be inherited. 

Group 2 PH: This type of pulmonary hypertension is caused by left sided heart failure which can be associated with a variety of conditions such as mitral valve disease, congenital cardiomyopathy and long-term hypertension. 

Group 3 PH: In some cases, pulmonary hypertension is a result of lung disease such as COPD, sleep apnea, interstitial lung diseases (including sarcoidosis and pulmonary fibrosis), and chronic hypoxia.These conditions cause pulmonary vasoconstriction, which leads to higher pressures and pulmonary hypertension.

Group 4 PH: This form of pulmonary hypertension is caused by chronic blood clots (pulmonary emboli), the scars from blood clots, and other clotting disorders.

Group 5 PH: This subtype includes pulmonary hypertension that is related to other health conditions such as polycythemia vera, vasculitis, kidney disease, metabolic disorders including toxic multinodular goiter, glycogen storage disease, and tumors that compress the pulmonary arteries.

Prognosis and survival rates for pulmonary hypertension

Survival rates vary based on the subtype of pulmonary hypertension. Untreated, the prognosis for all types is poor, which is why it is important patients be diagnosed and start treatment early in the disease. For example, pulmonary arterial hypertension (PAH) registries report survival rates of between 68% and 93% at 1 year and 39% and 77% at 3 years. Even with treatment, the leading cause of death for patients with PH is right ventricular failure.

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Who is most at risk for pulmonary hypertension?

In general, pulmonary hypertension affects individuals with underlying lung or heart conditions. 

  • Valve disease – Almost 100% of individuals with severe mitral valve disease and about 65% of those with aortic valve disease develop PH.
  • Scleroderma – Approximately 30% of individuals with this connective tissue disease develop PH. 
  • Sickle cell disease – PH affects about 20 to 40% of individuals with SCD
  • Some other factors that increase an individual’s risk are asbestos exposure, living at high altitude, smoking, and recreational drug use.

Pulmonary hypertension with the LATTE Method

Now that you have a baseline understanding of pulmonary hypertension, let’s dive into caring for these patients using the Straight A Nursing LATTE Method

L: How does the patient LOOK?

The signs of symptoms of pulmonary hypertension develop gradually and many patients may not notice them for months or even years. As the condition progresses, signs and symptoms include:

  • Dyspnea on exertion (as the disease progresses, patients experience dyspnea at rest)
  • Syncope or dizziness
  • Fatigue
  • Chest pain or pressure, heart palpitations, tachycardia, and/or dysrhythmias such as atrial fibrillation or atrial flutter
  • Decreased appetite
  • Cyanosis due to hypoxia
  • Signs and symptoms of right-sided heart failure: peripheral edema, ascites, hepatomegaly, and JVD

The World Health Organization has defined four functional classes to help define the severity of disease in patients with PH. 

  • Class 1 – The patient has no limitations on their activity and does not notice any symptoms
  • Class 2 – The patient has slight limitations on activity. Physical activity causes some symptoms, but there are no symptoms while the patient is resting.
  • Class 3 – The patient notices symptoms even with light physical activity or ordinary daily activities, but there are no symptoms while resting.
  • Class 4 – The patient has noticeable symptoms even when resting.

A: How do you ASSESS the patient with pulmonary hypertension?

Priority assessments for a patient with pulmonary hypertension involve assessing both the respiratory and cardiovascular system.

  • Respiratory assessment – Auscultate lungs, assess respiratory rate and work of breathing, assess for dyspnea at rest and with exertion. Assess SpO2 and look for cyanosis which will look different depending on the patient’s skin color. In individuals with lighter skin tones, cyanosis appears as blu-ish or purple-ish hue. In those with more yellow-toned skin, cyanosis appears more green or gray-ish. And in those with darker skin tones, cyanosis presents as a gray-ish white color around the lips or tongue and a bluish-tinge at the conjunctiva, nail beds and palms.
  • Cardiovascular assessment – Assess heart rate and rhythm. Measure blood pressure and capillary refill. Make note of skin signs which could indicate poor perfusion such as coolness and pallor or mottling. Assess for jugular vein distention and peripheral edema, both of which are signs of right-sided heart failure. Grade edema from mild (+1) to deep pitting edema (+4). Make note of how far up the legs the edema is present. Palpate pulses to determine if the heart rhythm is regular but also to determine the quality of the pulse (0 is no pulse and 3 is a bounding pulse). Some patients may have an audible murmur if a valve disorder is present, and S3 may be audible in patients whose condition has progressed to heart failure. Assess for chest pain and palpitations as well.
  • I/O – Keep careful track of I/Os and weigh the patient daily.
  • Fatigue – Ask the patient about symptoms of fatigue and how much impact it has on their ADLs.

T: What TESTS will be conducted for a patient with pulmonary hypertension?

The diagnosis of PH can be difficult, and is often arrived at when other possible causes for the patient’s symptoms have been ruled out. In addition to the MD conducting a full physical assessment, diagnostic tests include: 

  • Echocardiogram – This test measures pulmonary artery pressure and right atrial pressure. It also evaluates the degree of both right and left ventricular dysfunction. It can also help determine if the pulmonary hypertension is arterial or venous in nature. A patient with PH has a mean pulmonary artery pressure > 25 mmHg at rest, or higher than 30 mmHg with activity. For reference, a normal value is about 12 to 16 mmHg. 
  • 12-lead ECG – An ECG could show right ventricular hypertrophy and right-axis deviation as well as any dysrhythmias such as atrial fibrillation.
  • Right-sided cardiac catheterization – This is the “gold standard” test for pulmonary hypertension. It is used to provide detailed measurements and evaluation of pulmonary artery pressure, pulmonary artery occlusion pressure (POAP), mixed venous oxygen saturation, and conduct the vasoreactivity test. This test involves the administration of a vasoactive substance and the response of the pulmonary vasculature is assessed. If the pulmonary vessels dilate, this indicates that medications that cause pulmonary arterial vasodilation (CCBs) may be helpful for the patient and these patients tend to have a better prognosis. 
  • Imaging studies – Imaging studies such as chest CT or cardiac MRI provide detailed images of the structure of the heart and surrounded structures.
  • Chest X-ray – A chest X-ray shows the size and shape of the heart, which can be helpful in monitoring for worsening heart failure.
  • The 6-minute walk distance (6MWD) test – In this test, the patient walks as far as they can on a treadmill for six minutes. When done regularly, this test provides a way to monitor for disease progression and response to therapy.
  • ABG – An ABG may be utilized to determine the degree of hypoxemia
  • Blood tests – Blood tests could provide information about potential causes for pulmonary hypertension such as thyroid disorders, kidney disease, polycythemia vera, or a clotting disorder (among many others!). BNP is an important biomarker that is elevated in heart failure, and can be utilized to monitor the progression of this complication. Genetic tests may also be conducted if the PH is thought to be inherited.
  • Ventilation/perfusion (V/Q) scan – If blood clots are suspected to be the cause of PH, this test can show if clots are present. In a V/Q scan, a radioactive substance is injected through an IV catheter so that blood flow can be visualized. Airflow through the lungs can also be visualized by having the patient inhale a radioactive tracer as well.
  • Pulmonary function tests (PFTs) – PFTs may be utilized to determine if the patient has underlying pulmonary disease which could be the cause of pulmonary hypertension.
  • Sleep study – Since OSA can cause pulmonary hypertension, this test may be conducted to help determine the underlying cause.
  • Exercise stress test – This test evaluates how the heart functions during stress, which can help determine the cause of the patient’s symptoms.

T: What TREATMENTS will be provided for pulmonary hypertension?

Treatment for pulmonary hypertension will depend on which group of PH the patient has and current direct treatments only exist for pulmonary arterial hypertension (Group 1) and chronic thromboembolic pulmonary hypertension (Group 4). Other therapies aim to address the underlying causative factors and decrease symptoms.

Since there is no cure for pulmonary hypertension, the goal of therapy is to minimize symptoms and prevent or delay progression while preserving as much pulmonary and cardiac function as possible. Pharmacologic treatment includes:

  • Calcium channel blockers – The evidence is suggesting that CCBs may be useful in patients with Group 1 PH who had a positive vasoreactivity test during their heart catheterization. Commonly used medications are long-acting nifedipine and diltiazem. Note that administering CCBs to a nonreactive individual could cause significant hypotension and death.
  • Phosphodiesterase-5 inhibitors (sildenafil/Revatio) – PDE-5 inhibitors are utilized to promote pulmonary vasodilation and decrease proliferation of vascular muscle cells in individuals with Group 1 PH. Common adverse effects include GI upset, headache, and muscle/joint pain.
  • Endothelin receptor antagonists (ambrisentan/Letairis) – Ambrisentan antagonizes endothelin which results in vasodilation. It is often used in combination with sildenafil for patients with Group 1 PH. Another medication you may see utilized in this class is bosentan/Tracleer. 
  • Riociguat/Adempas – This medication is primarily used for those with Group 4 (thromboembolic PH) but also has shown benefit for those with Group 1 PH. It is used to improve vascular resistance, exercise tolerance, and symptoms while also increasing time to clinical worsening.
  • Prostacyclin pathway agonists – Selexipag/Uptravi has vasodilatory properties and is used for individuals with Group 1 PH. It has been shown to delay disease progression and reduce the risk for hospitalization due to PH.
  • Parenteral prostanoids – Patients with class IV or high risk PH may be treated with a parenteral prostanoid such as IV epoprostenol or subcutaneous treprostinil. These medications are utilized for their vasodilatory properties. Note that epoprostenol is administered via an implanted central venous catheter using a portable infusion pump. 
  • Diuretics – Diuretics such as furosemide/Lasix are used to remove excess fluid and decrease edema.
  • Anticoagulants – For patients with thromboembolic PH, anticoagulants can help decrease blood clots.

Other potential treatments include:

  • Oxygen as needed to maintain adequate SpO2 and/or SaO2. 
  • Treating the underlying cause, when possible (ex: mitral valve repair)
  • Treat right-sided heart failure and any associated complications such as dysrhythmias.
  • Balloon pulmonary angioplasty (BPA) is a procedure that uses a balloon to widen the pulmonary artery and is a possible treatment option for those with CTEPH.
  • Pulmonary thromboendarterectomy is a surgical procedure that removes blood clots from the lungs and could be potentially curative for those with CTEPH. Most patients experience significant relief of symptoms and improvement in hemodynamics.
  • Lung transplant for patients who are not responsive to treatment.

E: How do you EDUCATE the patient/family?

  • Important lifestyle modifications for a patient with PH are to get regular moderate exercise such as walking or cycling, follow a heart-healthy diet (avoid processed foods, foods high in fat and sodium), maintaining a consistent sleep schedule, and quitting smoking
  • Pregnancy with pulmonary hypertension is risky so patients should be encouraged to discuss birth control options and family planning with their pulmonologist.
  • Patients taking a diuretic should be encouraged to enjoy foods high in potassium and magnesium such as bananas, oranges, peanuts and broccoli. 
  • Patients with PH may need to take special precautions when flying, such as using supplemental oxygen to minimize pulmonary vasoconstriction, and may need to avoid traveling to high altitude locations.
  • Patients with heart failure should be taught how to monitor their fluid intake and recognize signs of fluid overload.
  • Patients should know when to seek medical attention. This includes sustained tachycardia, dyspnea not resolved with rest, or sudden and severe chest pain. 
  • Patients should be advised to take measures to avoid infection which means receiving recommended vaccinations.
  • Patients with an implanted central venous catheter need to know how to monitor for complications such as infection, dislodged catheters, bleeding, IV pump malfunctions or occlusions. If any problems occur, they need to seek medical treatment.

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