Transitioning disopyramide to mavacamten in obstructive hypertrophic cardiomyopathy: A case series and clinical guide. Hypertrophic cardiomyopathy (HCM) is a genetic disorder for which first-line treatments for obstructive HCM (oHCM) include beta-blockers, non-dihydropyridine calcium channel blockers, and disopyramide for refractory cases. Mavacamten, a selective cardiac myosin inhibitor , is indicated for symptomatic oHCM to improve functional capacity and symptoms. Use of disopyramide and mavacamten together is not recommended due to concerns of additive negative inotropic effects. Transitioning from disopyramide to mavacamten may be preferred to avoid adverse effects and frequent administration, however, the best approach for making the transition has not been established. We present
Disopyramide An official website of the United States government Here's how you know Log inAccess keysNCBI HomepageMyNCBI HomepageMain ContentMain NavigationBookshelfSearch databaseBooksAll DatabasesAssemblyBiocollectionsBioProjectBioSampleBooksClinVarConserved DomainsdbGaPdbVarGeneGenomeGEO DataSetsGEO ProfilesGTRHomoloGeneIdentical Protein GroupsMedGenMeSHNLM and EffectsSummary of Use during LactationDisopyramide is sometimes found in the plasma of nursing infants at levels of 7.5% to 12.5% of the mother's levels. The N-monodesalkyldisopyramide (NMD) metabolite is more anticholinergic than disopyramide itself and appears in breastmilk in levels higher than disopyramide. However, of the cases reported, there are no reports of infant effects. Disopyramide may be used
Markers of responsiveness to disopyramide in patients with hypertrophic cardiomyopathy. Significant left-ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy (HCM) may result in symptoms and is associated with adverse outcomes. Although disopyramide can reduce resting gradients, nearly 30% of HCM patients do not respond. We sought to study the clinical and echocardiographic variables associated with disopyramide-induced LVOT-gradient reduction. Forty-one disopyramide-treated HCM patients (average daily-dose 305 mg) were subdivided into two groups: (1) nineteen responders, with a reduction of LVOT-gradients of at least 30% from baseline, and (2) twenty-two non-responders, in whom LVOT-gradients did not change or increased following treatment. All patients had
Higher efficacy of direct hemoperfusion using coated activated-charcoal column for disopyramide poisoning: A case report. Cases of severe disopyramide poisoning are rare and few have been reported. We report a case in which activated-charcoal column hemoperfusion was dramatically effective for life-threatening disopyramide poisoning. A teenage girl who had overdosed on disopyramide (total dose , 4950 mg) was brought to our hospital. She was resuscitated from short period cardiopulmonary arrest and subsequently showed severe cardiogenic shock and ventricular arrhythmia. Disopyramide poisoning (self-evident). As hemodynamics remained unstable after providing percutaneous cardiopulmonary support and intra-aortic balloon pumping, we attempted direct hemoperfusion using a coated activated
Severe Hypoglycemia-induced Right Hemiparesis with Reversible Diffusion Restriction in the Left Internal Capsule Due to Combination Therapy Using Disopyramide and Clarithromycin Severe hypoglycemia is known to cause acute focal neurological symptoms. In cases with a medical history of diabetes mellitus (DM), the diagnosis and treatment of hypoglycemia-induced neurological symptoms are simple revealed severe hypoglycemia (27 mg/dl). However, he was not taking insulin or long-acting sulfonylurea drugs but disopyramide and clarithromycin had been administered. In addition, he had kidney dysfunction with an estimated glomerular filtration rate (GFR) of 42.9 ml/min/1.73 m. After the blood glucose level was normalized, the left hemiparesis completely recovered and abnormal findings of magnetic
Computational Analysis of the Mode of Action of Disopyramide and Quinidine on hERG-Linked Short QT Syndrome in Human Ventricles The short QT syndrome (SQTS) is a rare cardiac disorder associated with arrhythmias and sudden death. Gain-of-function mutations to potassium channels mediating the rapid delayed rectifier current, , underlie SQTS variant 1 (SQT1), in which treatment with Na and K channel blocking class Ia anti-arrhythmic agents has demonstrated some efficacy. This study used computational modeling to gain mechanistic insights into the actions of two such drugs, disopyramide and quinidine, in the setting of SQT1. The O'Hara-Rudy (ORd) human ventricle model was modified to incorporate a Markov chain formulation of describing wild type (WT) and SQT1 mutant conditions. Effects
Safety of Outpatient Initiation of Disopyramide for Obstructive Hypertrophic Cardiomyopathy Patients Disopyramide is effective in ameliorating symptoms in patients with hypertrophic cardiomyopathy; however, its potential for proarrhythmic effect has raised concerns about its use in the ambulatory setting. The risk of initiating disopyramide in this manner has never been evaluated. All charts of patients seen in the outpatient hypertrophic cardiomyopathy clinic between 2010 and 2014 were screened for initiation of disopyramide and data were extracted. Disopyramide in our clinic is usually initiated at a dose of 300 mg daily and titrated during follow-up. A total of 2015 patients were seen in the clinic, including 168 who were started on disopyramide. There were no cardiac events within 3 months
Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers Hypertrophic obstructive cardiomyopathy (HOCM) is the most common known cause of sudden death in children beyond infancy and in young athletes. Cases reported indicate that steroid-induced HOCM is usually a benign disorder. The normalization of cardiac patients. We report a critically ill infant with steroid-induced HOCM resistant to beta-blockers who was successfully treated with disopyramide without relevant adverse events. Adult guidelines and pediatric experts suggest pharmacological therapy with beta-blockers or verapamil as the first- and second-line approach. However, these drugs are not always an option, especially in critical patients, hence
Medical Management (β Blocker ± Disopyramide) of Left Ventricular Outflow Gradient Secondary to Systolic Anterior Motion of the Mitral Valve After Repair. Systolic anterior motion of the mitral valve (SAM) occurs intraoperatively after mitral valve repair (MVRr) in up to 14% of cases and typically resolves in the operating room with conservative measures. Less commonly SAM may also occur in the early or late postoperative period. The clinical course and optimal management of such cases is poorly defined, but reoperation is common. We describe our experience using disopyramide to successfully treat postoperative SAM refractory to beta blockade. Seven patients were retrospectively identified with mitral valve prolapse who underwent MVRr from 2003 to 2015 and were found during follow-up to have
Disopyramide for Hypertrophic Cardiomyopathy: A Pragmatic Reappraisal of an Old Drug. Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disorder characterized by unexplained left ventricular hypertrophy in the absence of other cardiac or systemic etiologies. Approximately two-thirds of patients with HCM develop left ventricular outflow tract (LVOT) obstruction with or without provocation , whereas nearly half develop heart failure with preserved ejection fraction. Medical management of heart failure with preserved ejection fraction is based on the presence of symptoms and LVOT obstruction and frequently includes β-blockers or verapamil. Disopyramide is a class Ia antiarrhythmic that historically was used for the treatment of arrhythmias; however, its contemporary use is often reserved
class of 2 to 3. It is recommended only if: • it is an add-on to individually optimised standard care that includes beta-blockers, non-dihydropyridine calcium-channel blockers or disopyramide, unless these are contraindicated, and • the company provides it according to the commercial arrangement. 1.2 This recommendation is not intended to affect treatment with mavacamten that was started in the NHS care is either beta-blockers or non-dihydropyridine calcium-channel blockers and if symptoms persist then disopyramide may be added. Some people with uncontrolled symptoms may choose to have surgery. Mavacamten is the first treatment that specifically treats the condition rather than the symptoms. For this evaluation, the company asked for mavacamten to be considered only as an add-on treatment
titrated according to guidelines (not specified at this point) at the investigator’s discretion prior to study inclusion. Disopyramide treatment was disallowed. The concomitant therapy was to have been well tolerated for at least 2 weeks before screening. It was to be kept stable during the study, unless safety or tolerability concerns arose. Since not all patients in the EXPLORER-HCM study received
into account non-vasodilating beta-blockers, verapamil, and diltiazem a. Presented is the ACT specified by the G-BA. b. In the guideline [1], non-vasodilating beta blockers or calcium channel blockers (verapamil or diltiazem) are recommended for the treatment of symptomatic HOCM if beta-blockers are insufficient or not tolerated. c. The drug disopyramide is neither approved nor marketed in Germany. d. Given therapy of physician’s choice with nonvasodilating beta-blockers or calcium channel blockers. As per study protocol, all patients who received concomitant medication for HOCM were to be optimally adjusted according to guidelines at the investigator’s discretion prior to their enrolment. Disopyramide treatment was disallowed. The concomitant therapy was to have been well tolerated for at least 2 weeks
of symptoms is strongly suggestive of obstruction. If the goal is to achieve the highest success of showing someone has obstructive physiology then the patient should abstain from medications (disopyramide, β-blockers, and calcium channel blockers), for 48 hours before the study. Otherwise, there can be a role for patients to continue taking medications to assess the efficacy of gradient reduction therapy
for more than one year, it is considered longstanding persistent. Permanent AF – When a decision has been made not to pursue rhythm control strategies, the patient is said to have permanent AF Class I antiarrhythmic agents – quinidine, procainamide, disopyramide, mexiletine, flecanide, propafenone Class III antiarrhythmic agents – amiodarone, dronedarone, dofetilide, ibutilide, sotalol, vernakalant
— concurrent use with diltiazem or verapamil may lead to increased plasma concentrations of digoxin. Increased risk of bradycardia. Manufacturer advises monitor and adjust dose. * Disopyramide — concurrent use with verapamil may lead to increased risk of cardiodepression and asystole. * Domperidone — diltiazem and verapamil are predicted to increase exposure to domperidone. Manufacturer advises avoid
, sedating antihistamines, antimuscarinic drugs for urinary incontinence, and disopyramide). * Diabetic autonomic neuropathy and neurogenic bladder. * Urethral stricture and phimosis (constriction of the foreskin). * Cancer of the prostate or bladder. [NICE, 2015; BMJ Best Practice, 2021] What are the causes of storage lower urinary tract symptoms? * The exact cause of overactive bladder is not well
blockers and/or disopyramide is ineffective or poorly tolerated); they advise that it can be co-administered with beta-blockers or non-dihydropyridine calcium channel blockers but cannot recommend its use with disopyramide.3 The submitting company did not consider disopyramide to be a relevant comparator because it is associated with poor tolerability and supply problems, and therefore is not widely used in clinical practice. However, whilst SMC clinical experts acknowledged these issues, they did consider disopyramide as a potential treatment option in the second-line setting. 3 If pharmacological treatment is ineffective, non-pharmacological options for oHCM involve invasive cardiac surgery to relieve the LVOTO by reducing septal hypertrophy. The two approaches (ventricular septal myectomy and alcohol