Term
| AVOID; significant negative inotropic effects |
|
Definition
|
|
Term
|
Definition
| Amlodipine, Nifedipine, Felodipine, Isradipine, Nicardipine |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Long-acting nifedipine, Diltiazem, Amlodipine |
|
|
Term
| Inhibits the influx of extracellular calcium across myocardial and smooth muscle cell membranes leading to VASODILATION |
|
Definition
|
|
Term
| Primarily affects arteriolar vasculature; no significant effect on sinus node function or cardiac conduction |
|
Definition
| Amlodipine (Norvasc) and Nifedipine (Procardia XL) |
|
|
Term
| Due to gradual onset, reflex tachycardia does not occur as with other dihydropyridines |
|
Definition
|
|
Term
| Dilation of pulmonary and systemic arteries; decreased peripheral resistance, systemic BP, and afterload; increased pulmonary blood flow |
|
Definition
|
|
Term
| Dosing: 2.5-20 mg orally daily |
|
Definition
|
|
Term
| Dosing: 120-180 mg orally daily, initially; titrate to clinical response |
|
Definition
|
|
Term
| Dosing: 180 mg orally daily, initially; titrate to clinical response |
|
Definition
|
|
Term
| Doses up to 720 mg have been used |
|
Definition
|
|
Term
| Dosing: only use long acting formulations in PH; 30-240 mg orally daily |
|
Definition
| Nifedipine (Procardia XL) |
|
|
Term
| Common side effects: H/A, flushing, hypotension, peripheral edema, constipation; Serious: Hypotension, syncope, right ventricular faiure |
|
Definition
|
|
Term
| Block endothelin receptors on vascular endothelium and smooth muscle |
|
Definition
| Endothelin Receptor Antagonists (Can be used alone or in combo with CCBs) |
|
|
Term
| Treatment of PH; to improve exercise capacity and decrease the rate of clinical deterioration (Endothelin Receptor Antagonists) |
|
Definition
| Ambrisentan (letairis); Bosentan (Tracleer) |
|
|
Term
| Initial Dosing: 5 mg orally daily; If tolerated may increased to max 10 mg once daily; selective for endothelin-A receptor |
|
Definition
|
|
Term
| Initial Dosing: 62.5 mg orally twice daily for 4 weeks, increase to maintenance dose of 125 mg twice daily, adults less than 40 kg should be maintained at 62.5 mg twice daily (nonselective endothelin receptor antagonist (A and B) |
|
Definition
|
|
Term
| Adverse Reactions: Edema, Palpitation and Flushing, headache, hemoglobin decrease, hepatotoxicity, transaminases increase, respiratory tract congestion/infection |
|
Definition
| Endothelin Receptor Antagonists |
|
|
Term
| Adverse Reactions: Spermatogenesis inhibition (25%) |
|
Definition
|
|
Term
| Contraindications for use of Endothelin Receptor Antagonists |
|
Definition
|
|
Term
| Only available through a limited distribution program; prescribers and pharmacies must be registered with these programs to prescribe and dispense these medications |
|
Definition
| Bosentan (Tracleer) and Ambrisentan (Letairis) |
|
|
Term
| Patient may be switched from Bosentan to this if liver function test abnormalities arise |
|
Definition
|
|
Term
| Bosentan Drug Interactions |
|
Definition
| Contraceptives; Cyclosporine; Glyburide |
|
|
Term
| may be taken with food; do not crush or chew this medication; report unusual fatigue, N/V, abdominal pain and/or yellowing of the skin/eyes to prescribe immediately; do not get pregnant while taking this medication |
|
Definition
| Bosentan (Tracleer) Counseling points |
|
|
Term
| Phosphodiesterase-5 Inhibitors |
|
Definition
| Sildenafile (Revatio) and Tadalafil (Adcirca) |
|
|
Term
| Increased cGMP concentration results in pulmonary vasculature relaxation; vasodilation in the pulmonary bed and the systemic circulation (to a lesser degree) may occur |
|
Definition
| Phosphodiesterase-5 Inhibitors |
|
|
Term
| Treatment of PH (WHO Group I) to improve exercise ability and delay clinical worsening |
|
Definition
|
|
Term
| Dosing: 20 mg orally 3 times/day, taken 4-6 hours apart |
|
Definition
|
|
Term
| Dosing?: 40 mg orally once daily; may need dosing adjustments with CYP 3A4 inhibitors |
|
Definition
|
|
Term
| Adverse Reactions include HA, Dyspepsia, Flushing, Pyrexia, Diarrhea, UTIs, Increased LFTs, Myalgia, abnormal vision, Epistaxis, Nasal congestion |
|
Definition
|
|
Term
| PDE-5 Inhibitors Contraindications |
|
Definition
| Hypersensitivity to sildenafil or tadalafil; concurrent use of organic nitrates in any form (nitroglycerin, isosorbide dinitrate) |
|
|
Term
|
Definition
| Epoprostenol, Treprostinil, Iloprost |
|
|
Term
| Strong vasodilator of all vascular beds; decreases thrombogenesis by inhibiting platelet aggregation |
|
Definition
|
|
Term
| Intial Dosing: 1-2 ng/kg/minute, increase dose in increments of 1-2 ng/kg/minute every 15 minutes or longer until dose-limiting side effects are noted or tolerance limit is observed |
|
Definition
|
|
Term
| Initial Dosing: 18 mcg (or 3 inhalations) every 4 hours 4 times/day; if 3 inhalations not tolerated, reduce to 1-2 inhalations, then increased to 3 inhalations as tolerated |
|
Definition
| Treprostinil (Remodulin): Inhalation |
|
|
Term
| Initial Dosing: new to prostacyclin therapy: 1.25 ng/kg/minute continuous, if dose cannot be tolerated due to systemic effects, reduce to 0.625 ng/kg/minute |
|
Definition
| Treprostinil (Remodulin): SubQ or IV |
|
|
Term
| Initial Dosing: 2.5 mcg/dose; administer 6-9 times daily |
|
Definition
| Illoprost (Ventavis): Inhalation |
|
|
Term
| Adverse Reactions/Events include anxiety, diarrhea, flu-like syndrome, flushing, HA, jaw pain, N/V, nervousness, pump malfunction, local site infection, catheter obstruction, sepsis |
|
Definition
|
|
Term
| Epoprostenol (Flolan) Contraindications |
|
Definition
| Hypersensitivity, chronic use with pts w/ heart failure due to severe left ventricular systolic dysfunction, pts who develop pulmonary edema during dose initiation |
|
|
Term
| Epoprostenol (Flolan) Drug Interactions |
|
Definition
| Anticoagulants and Antiplatelets, Antihypertensives |
|
|
Term
| Class II Preferred Agents |
|
Definition
| Ambristentan, Bosentan, Sildenafil |
|
|
Term
| Class II Alternative Agents |
|
Definition
|
|
Term
| Class III Preferred Agents |
|
Definition
| Ambrisentan, Bosentan, IV Epoprostenol, IV or SubQ Treprostinil, Inhaled Iloprost, Sildenafil |
|
|
Term
| Class III Alternative Agents |
|
Definition
|
|
Term
| Class IV Preferred Agents |
|
Definition
|
|
Term
| Class IV Alternative Agents |
|
Definition
|
|
Term
|
Definition
|
|
Term
| Creates a right or left shunt in the atria of the heart; decompresses the right heart chamber and increases LV preload and CO; improves systemic O2 transport despite arterial O2 desaturation |
|
Definition
|
|
Term
| Option for a select group of pts when medication therapy is no longer effective; this is the only "curative" treatment for PH |
|
Definition
| Lung Transplant or Heart-Lung Transplant |
|
|
Term
| Guidelines for Referral of patients for Transplantation |
|
Definition
| WHO Functional Class III or IV; MAP > 10 mmHg; MPAP > 50 mm Hg; Cardiac index < 2.5 L/min per m2; failure to improve functionally despite medical therapy; rapidly progressive disease |
|
|
