PULMONARY HYPERTENSION
Pulmonary hypertension is also called pulmonary arterial hypertension
WHO PH Group / Causes / TreatmentGroup 1
Pulmonary Arterial Hypertension / Idiopathic, Genetic, Drug/Toxin Exposure, Connective Tissue Disease, HIV, Portal Hypertension, Congenital Heart Disease / Medications (pills, inhalers, and continuous infusions developed specifically to dilate and reduce inappropriate growth of cells in pulmonary arteries
Group 2
Left Heart Disease / Coronary Artery Disease, HTN, Valvular Disease, Age / Therapies focused on treating underlying heart disease
Group 3
Lung Disease / COPD, Interstitial Lung Disease, Other Lung Disease causing low blood oxygen levels / Therapies focused on treating underlying lung disease
Group 4
Chronic Thromboembolic Pulmonary Hypertension / Old, organized blood clots in the lungs forming a physical barrier to blood flow within the pulmonary arteries / Surgical removal of clot, oral medication if surgery not possible or PH remains after surgery
Group 5
Unclear Mechanisms / Sarcoidosis, Sickle Cell Anemia, Chronic Hemolytic Anemia, Spleen Removal, Metabolic Disease / Therapies focused on treating underlying disease
Exercise
Because of the varying severity of PH from patient to patient (and other health-related factors), recommendations for exercise are different for each individual, and may vary over time.
Walking on an incline or exercising arms and legs simultaneously can produce greater increase in blood pressure, and should be done conservatively, or avoided.
Light functional resistance exercises may be considered, but should be done below shoulder height and with frequent breaks.
Exercise/activity should be stopped at the first sign of lightheadedness, fatigue, chest pressure, palpitations, or excessive shortness of breath.
Avoid exercise/activity in outdoor temperature extremes. Cold, heat, humidity, high elevation worsen exercise tolerance and warrants additional caution.
Recovery time of five to ten minutes without exhaustion or worsening of symptoms can give a measure of how much exercise/activity is appropriate.
Nutrition
Controlling Salt and Sodium Consumption
Fluid building up in the tissues is a common problem for PH patients. Verify patients are following a low sodium diet, and instruct in reading a food label and all ways sodium can be hidden in food
Managing Nausea and Vomiting
Nausea, early satiety, and lack of appetite can be symptoms of worsening right-sided heart failure. Prolonged nausea and vomiting more than 24 hours, or symptoms of increasing intensity, require contacting a physician.
Ways patients can avoid nausea:
Don’t eat large meals, drink large amounts of liquids with meals, drink large amounts of carbonated beverages.
Avoid greasy or fatty foods.
Sit up while eating and don’t lie down immediately after eating.
Keep teeth and tongue brushed or use mouthwash.
If nauseated:
Snack on crackers, pretzels, or dry toast.
Sip on cold non-acidic liquids.
Put an ice pack behind the neck.
Open windows for fresh air or circulate air with a fan.
Medications
Conventional Medication Therapy
Calcium channel Blockers – only appropriate for small minority of patients
Digoxin
Diuretics
Oxygen
Warfarin
Pulmonary Hypertension Medications
Endothelian receptor Antagonists (ERAs) – help prevent blood vessels from narrowing
Ambrisentan (Letairis)
Bosentan (Tracleer)
Macitentan (Opsumit)
Phosphodiesterase Inhibitors (PDE 5) – allow the lungs to produce more of its own natural vasodilators
Sildenafil (Revatio) + pediatric formula
Tadalifil (Adcirca)
Prostacyclin Analogue – slows blood vessels in the lungs to relax
Oral Treprostinil (Orenitram)
Selective IP Receptor Agonist – targets and activates a prostacyclin receptor helping blood vessels in the lungs to relax
Selexipag (Uptravi)
Soluble Guanylate Cyclase Stimulators – increases the interaction of sGC with nitric oxide to help blood vessels in the lungs relax
Riocoguat (Adempas)
Inhaled Medications
Iloprost (Ventavis)
Inhaled Treprostinil (Tyvaso)
Intravenous Medications
Intravenous Treprostinil (Remodulin)
Epoprostenol (Flolan)
Room Temperature Stable Epoprostenol (Veletri)
Subcutaneous Medications
Subcutaneous Treprostinil (Remodulin)
Treatment Side Effects
Endothelian receptor antagonists: headache, nausea, nasal congestion, hypotension, liever test abnormalities, decreased sperm count, edema, decreased appetite, metallic taste in mouth
PDE-5 inhibitors: headache, hypotension, visual disturbances, increased indigestion
Prostacyclins: headache nausea, diarrhea, flushing, hypotension, jaw pain, low platelets, rash
IV delivery: site pain, redness, infections
Inhaled route: sore throat, cough, chest discomfort
Help patients talk to a pharmacist about any interactions or side effects that may occur. Patient should contact PH team member before starting any new medication prescribed by another health care professional.
It is not a good idea to make up for a missed dose of PH medication.
Over-the-Counter Medications
All decongestants and medications that contain stimulants should be avoided as the can cause vasoconstriction and worsen PH and increase blood pressure and heart rate.
Medications containing antihistamines may be used to treat cold/allergy symptoms provided they do not contain decongestants.
There is limited information on the effects of herbal medications on PH. Products labeled as natural should not be assumed to be safe. Some can affect heart and lung function and interact with prescribed medication. Herbal therapies should be viewed as medications and not as “natural or supplements.”
Consult and PH team member before beginning an OTC or herbal medication.
Health Management
Help Patients Take Charge of Health Plan
Get educated on all medications taken including side effects and drug interactions.
Keep information on medications and dosages, allergies, medical history, physicians, and insurance information on person at all times.
Comply with medication regimen. Set an alarm to prompt you to take medications. Arrange a private place, if away from home, to take medication.
Plan ahead for travel.
Use O2 as prescribed, even when reluctant to use in public.
Purchase a Medic Alert bracelet or necklace.
Join support group.
Listen to body signals.
How Patients Can Work with Medical Team
Talk to PH team about medications options and expectations for day-to-day management.
Share medical information with all health care teams.
Ask questions if you don’t understand something.
Establish “ringleader” physician who possesses the most knowledge of the condition and treatment. This is usually the PH physician.
Let healthcare team know if struggling to follow mediation regimen.
Sources:
Pulmonary Hypertension Association
phassociation.org
National Heart Lung and Blood Institute
nhlbi.nih.gov