MNACVPR



PULMONARY HYPERTENSIONPulmonary hypertension is also called pulmonary arterial hypertensionWHO PH GroupCausesTreatmentGroup 1Pulmonary Arterial HypertensionIdiopathic, Genetic, Drug/Toxin Exposure, Connective Tissue Disease, HIV, Portal Hypertension, Congenital Heart DiseaseMedications (pills, inhalers, and continuous infusions developed specifically to dilate and reduce inappropriate growth of cells in pulmonary arteriesGroup 2Left Heart DiseaseCoronary Artery Disease, HTN, Valvular Disease, AgeTherapies focused on treating underlying heart diseaseGroup 3Lung DiseaseCOPD, Interstitial Lung Disease, Other Lung Disease causing low blood oxygen levelsTherapies focused on treating underlying lung diseaseGroup 4Chronic Thromboembolic Pulmonary HypertensionOld, organized blood clots in the lungs forming a physical barrier to blood flow within the pulmonary arteriesSurgical removal of clot, oral medication if surgery not possible or PH remains after surgeryGroup 5Unclear MechanismsSarcoidosis, Sickle Cell Anemia, Chronic Hemolytic Anemia, Spleen Removal, Metabolic DiseaseTherapies focused on treating underlying diseaseExerciseBecause 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.NutritionControlling Salt and Sodium ConsumptionFluid 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 foodManaging Nausea and VomitingNausea, 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.MedicationsConventional Medication TherapyCalcium channel Blockers – only appropriate for small minority of patientsDigoxinDiureticsOxygenWarfarinPulmonary Hypertension MedicationsEndothelian receptor Antagonists (ERAs) – help prevent blood vessels from narrowingAmbrisentan (Letairis)Bosentan (Tracleer)Macitentan (Opsumit)Phosphodiesterase Inhibitors (PDE 5) – allow the lungs to produce more of its own natural vasodilatorsSildenafil (Revatio) + pediatric formulaTadalifil (Adcirca)Prostacyclin Analogue – slows blood vessels in the lungs to relaxOral Treprostinil (Orenitram)Selective IP Receptor Agonist – targets and activates a prostacyclin receptor helping blood vessels in the lungs to relaxSelexipag (Uptravi)Soluble Guanylate Cyclase Stimulators – increases the interaction of sGC with nitric oxide to help blood vessels in the lungs relaxRiocoguat (Adempas)Inhaled MedicationsIloprost (Ventavis)Inhaled Treprostinil (Tyvaso)Intravenous MedicationsIntravenous Treprostinil (Remodulin)Epoprostenol (Flolan)Room Temperature Stable Epoprostenol (Veletri)Subcutaneous MedicationsSubcutaneous Treprostinil (Remodulin)Treatment Side EffectsEndothelian receptor antagonists: headache, nausea, nasal congestion, hypotension, liever test abnormalities, decreased sperm count, edema, decreased appetite, metallic taste in mouthPDE-5 inhibitors: headache, hypotension, visual disturbances, increased indigestionProstacyclins: headache nausea, diarrhea, flushing, hypotension, jaw pain, low platelets, rashIV delivery: site pain, redness, infectionsInhaled route: sore throat, cough, chest discomfortHelp 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 MedicationsAll 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 ManagementHelp Patients Take Charge of Health PlanGet 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 ply 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 TeamTalk 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 National Heart Lung and Blood Institutenhlbi. ................
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