1. Indications and Usage
\nObstructive Hypertrophic Cardiomyopathy (oHCM):
\nIndicated for the treatment of adult patients with symptomatic NYHA class II-III obstructive HCM to improve exercise capacity and symptoms.
\n2. Dosage and Administration
\nPatient Selection and Monitoring:
\nBaseline Assessment: Assess LVEF by echocardiogram prior to initiation. Do not initiate in patients with LVEF < 55%.
\nMonitoring: Perform echocardiograms at Weeks 4, 8, and 12 after initiation or dose modification, then every 12 weeks thereafter.
\nLVEF Hold: Interrupt treatment if LVEF < 50%. Resume at a lower dose once LVEF recovers to \u2265 50%.
\nRecommended Dosage:
\nInitial Dose: 5 mg orally once daily. (Note: 2.5 mg is recommended for patients on weak CYP2C19 inhibitors or moderate CYP3A4 inhibitors).
\nTitration: Dose adjustments are based on echocardiographic assessment of LVEF and LVOT gradient. Maximum dose is 15 mg once daily.
\n3. Mechanism of Action
\nMyosin Inhibition:
\nMavacamten is a selective, allosteric, reversible inhibitor of cardiac myosin.
\nPhysiological Effect:
\nIt reduces the number of myosin heads available to interact with actin, thereby decreasing hypercontractility. This reduces the left ventricular outflow tract (LVOT) gradient and improves ventricular filling pressures without compromising heart rate or systolic function excessively.
\n4. Safety and Warnings
\nNew or Worsening Heart Failure:
\nMavacamten can reduce myocardial contractility, which may lead to heart failure. Monitor for signs and symptoms of heart failure.
\nDecreased LVEF:
\nMavacamten can decrease LVEF. Monitor LVEF by echocardiogram regularly.
\nConcomitant Medications:
\nAvoid concomitant use with moderate to strong CYP2C19 or CYP3A4 inhibitors and inducers.
\n5. Adverse Reactions
\nMost Common:
\nFatigue, dyspnea, diarrhea, and heart failure.
\nSerious Adverse Events:
\nDecreased LVEF (< 50%) and heart failure requiring hospitalization.
\n6. Drug Interactions
\nCYP2C19 Inhibitors (e.g., fluconazole):
\nIncrease mavacamten exposure. Reduce mavacamten dose by one level (e.g., 15 mg \u2192 10 mg).
\nCYP3A4 Inhibitors (e.g., clarithromycin):
\nIncrease mavacamten exposure. Reduce mavacamten dose by one level.
\nMetabolic Enzymes:
\nMavacamten is metabolized primarily by CYP2C19, and secondarily by CYP3A4 and CYP2C9.
\n7. Pharmaceutical Information
\nChemical Composition:
\nActive Ingredient: Mavacamten.
\nStorage:
\nStore at 20\u00b0C to 25\u00b0C (68\u00b0F to 77\u00b0F); excursions permitted to 15-30\u00b0C (59-86\u00b0F).<\/p>\n","protected":false},"excerpt":{"rendered":"
Brand Name: \u6cfd\u7ef4\u53ef \u00ae(Zavesca\u00ae)
\nGeneric Name: Miglustat
\nStrength: 0.1g (100mg) per capsule, 84 capsules per box
\nManufacturer: Almac Pharma Services Limited
\nMarketing Authorization Holder: Janssen-Cilag International N.V.\uff08\u56fd\u5185\u6301\u8bc1\uff1a\u897f\u5b89\u6768\u68ee\u5236\u836f\u6709\u9650\u516c\u53f8\uff09
\nApproval Date in China: November 23, 2016
\nRegistration Number: \u56fd\u836f\u51c6\u5b57 HJ20160635
\nStorage: Sealed, store below 30\u2103, avoid high temperature and humidity, keep out of reach of children<\/p>\n
Price&Cost:Contact Us now for the best price of Miglustat(Zavesca\uff09<\/p>\n","protected":false},"featured_media":3331,"comment_status":"open","ping_status":"closed","template":"","meta":[],"product_brand":[],"product_cat":[26],"product_tag":[],"class_list":["post-3330","product","type-product","status-publish","has-post-thumbnail","product_cat-capsules","first","instock","shipping-taxable","product-type-simple"],"yoast_head":"\n