-
JACC. Case Reports Dec 2023An adult with unrepaired tetralogy of Fallot presented with frequent tet spells. Her course was complicated by severe cyanotic spells and tachycardia-bradycardia...
An adult with unrepaired tetralogy of Fallot presented with frequent tet spells. Her course was complicated by severe cyanotic spells and tachycardia-bradycardia syndrome that limited beta blocker use to stabilize her spells. She markedly improved after disopyramide initiation and underwent successful tetralogy of Fallot repair with excellent functional outcome.
PubMed: 38204534
DOI: 10.1016/j.jaccas.2023.102093 -
The Medical Letter on Drugs and... Jan 2024
Topics: Humans; Atrial Fibrillation
PubMed: 38180321
DOI: 10.58347/tml.2024.1693a -
Circulation Nov 2023Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with clinical and subclinical episodes occurring in nearly one-half... (Review)
Review
Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with clinical and subclinical episodes occurring in nearly one-half of patients. AF in HCM historically has been characterized as a decisive disease complication associated with substantial risk for thromboembolic stroke and increased morbidity and mortality. However, there have been many advances in treatment strategy resulting in improved outcomes for this patient group. For example, stroke risk in HCM has been greatly reduced by using systemic oral anticoagulation initiated after the first clinical (symptomatic) AF episode, usually with preference given to direct anticoagulants over warfarin. In contrast, stroke risk scoring systems (such as CHADS-VASc score) are not informative in HCM given the substantial potential for stroke events in patients with low scores, and therefore should not be used for anticoagulation decisions in this disease. A novel risk score specifically designed for HCM (HCM-AF score) can reliably identify most patients with HCM at risk for future AF. Although a strategy focused on controlling ventricular rate is effective in asymptomatic (or minimally symptomatic) patients with AF, restoring and maintaining sinus rhythm is required for most patients with marked AF symptom burden and impaired quality of life. Several antiarrhythmic drugs such as sotalol, disopyramide, and amiodarone, can be effective in suppressing AF episodes; albeit safe, long-term efficacy is supported by only limited data. Catheter AF ablation has emerged as an important treatment option for some patients, although freedom from AF after a single ablation is relatively low (35% at 3 years), multiple ablations and the concomitant use of antiarrhythmic drugs can control AF with more than two-thirds of patients maintaining sinus rhythm at 5 years. Surgical AF ablation with biatrial Cox-Maze IV performed as an adjunctive procedure during myectomy can reduce symptomatic AF episodes (70% of patients free from AF at 5 years). For the vast majority of patients who have HCM with AF, the implementation of contemporary therapies has allowed for improved quality of life and low HCM-related mortality.
Topics: Humans; Atrial Fibrillation; Anti-Arrhythmia Agents; Quality of Life; Risk Factors; Anticoagulants; Stroke; Cardiomyopathy, Hypertrophic; Catheter Ablation; Treatment Outcome
PubMed: 38011245
DOI: 10.1161/CIRCULATIONAHA.123.065037 -
Future Cardiology Oct 2023Hypertrophic cardiomyopathy (HCM) is a phenotypically heterogeneous disease with a genetic basis and variable penetrance. The hallmarks of HCM include dynamic left... (Review)
Review
Hypertrophic cardiomyopathy (HCM) is a phenotypically heterogeneous disease with a genetic basis and variable penetrance. The hallmarks of HCM include dynamic left ventricular outflow tract obstruction, typically caused by asymmetric septal hypertrophy. However, abnormal papillary muscle placement, abnormal mitral valve and subvalvular apparatus and apical hypertrophic forms have also been described. Typical medical treatment has been stagnant for decades, although there have been significant advances in surgical treatment of patients with obstructive HCM. Herein, we describe a new class of drugs targeting the specific pathophysiology of HCM.
Topics: Humans; Cardiomyopathy, Hypertrophic; Cardiomyopathy, Hypertrophic, Familial; Heart Valve Diseases; Ventricular Outflow Obstruction, Left
PubMed: 37933625
DOI: 10.2217/fca-2023-0056 -
European Heart Journal. Case Reports Oct 2023Left ventricular outflow tract obstruction (LVOTO) sometimes presents with aortic stenosis (AS). Echocardiography is used to assess the diagnosis and severity of LVOTO...
Usefulness of catheter pressure measurement using the Navvus RXi system to determine left ventricular outflow tract obstruction and aortic stenosis severity: a case report.
BACKGROUND
Left ventricular outflow tract obstruction (LVOTO) sometimes presents with aortic stenosis (AS). Echocardiography is used to assess the diagnosis and severity of LVOTO or AS. However, LVOTO is one of the conditions that makes AS assessment difficult, and catheter pressure measurement is frequently useful in such cases.
CASE SUMMARY
An 84-year-old female patient presented with New York Heart Association functional Class III dyspnoea. Transthoracic echocardiography revealed LVOTO caused by upper septal hypertrophy, mitral valve systolic anterior motion with moderate mitral regurgitation, and a highly calcified aortic valve, which suggested the possibility of severe AS. The continuous Doppler echocardiography revealed a late-systolic peaking dagger-shaped profile with a peak jet velocity of 5.6 m/s. Cardiac catheterization was performed to determine the contribution of AS or LVOTO to her symptoms. Catheter pressures were measured at the ascending aorta (using a coronary catheter) and the LV (using the Navvus RXi system). The initial mean pressure gradient between the apex of the LV, just below the aortic valve and aorta, was measured at 65 and 25 mmHg, respectively. The mean pressure gradient between the apex and the aorta decreased from 65 to 40 mmHg after a 50 mg disopyramide administration. Oral medication therapy effectively stabilized her symptom after catheterization.
DISCUSSION
To the best of our knowledge, this is the first reported case of assessing the severity of LVOTO and AS using the Navvus RXi system. Catheter pressure measurement using the Navvus RXi system is a useful method of determining the severity of LVOTO and AS.
PubMed: 37811156
DOI: 10.1093/ehjcr/ytad471 -
Annals of Medicine and Surgery (2012) Oct 2023Aluminum phosphide (ALP) is a commonly used suicidal agent in an agrarian country like Nepal. The unmasking of the Brugada pattern in the electrocardiogram (ECG)...
INTRODUCTION AND IMPORTANCE
Aluminum phosphide (ALP) is a commonly used suicidal agent in an agrarian country like Nepal. The unmasking of the Brugada pattern in the electrocardiogram (ECG) associated with ALP poisoning is a rare phenomenon, and studies pertaining to it are scarce in the medical literature.
CASE PRESENTATION
An 18-year-old female presented to the emergency department with multiple episodes of vomiting, headache, blurring of vision, and abdominal pain after 4 h of consumption of ALP with suicidal intent. A 12-lead ECG revealed a coved ST-segment elevation and T-wave inversion in leads V1-V3 with right bundle branch block suggestive of a type 1 Brugada pattern. Her past medical and family history was not significant. The patient made an uneventful recovery with the required supportive treatments.
CLINICAL DISCUSSION
Cardiac arrhythmias are the major cause of death in ALP poisoning. Unmasking of the Brugada ECG pattern is a rare but potentially fatal complication implicated in various pharmacological toxicities, including tricyclic antidepressants, cocaine, procainamide, disopyramide, flecainide, and rarely with ALP.
CONCLUSIONS
ALP poisoning can unmask the Brugada ECG pattern, which can lead to ventricular fibrillation and/or sudden cardiac death.
PubMed: 37811028
DOI: 10.1097/MS9.0000000000001129 -
Giornale Italiano Di Cardiologia (2006) Oct 2023Hypertrophic cardiomyopathy is the most common genetic cardiomyopathy. Main complications include the development of arrhythmias and heart failure, and the latter may be... (Review)
Review
Hypertrophic cardiomyopathy is the most common genetic cardiomyopathy. Main complications include the development of arrhythmias and heart failure, and the latter may be triggered by left ventricular outflow tract obstruction. The treatment of left ventricular outflow tract obstruction includes pharmacological therapies (beta-blockers, calcium channel blockers, disopyramide) and septal reduction therapies (alcohol septal ablation, surgical myectomy). Myosin inhibitors represent a new therapeutic opportunity and in recent clinical trials proved effective in symptom relief, improvement of functional capacity and quality of life in patients with obstructive hypertrophic cardiomyopathy. In this narrative review we will summarize the available and under development therapeutic approaches for hypertrophic cardiomyopathy.
Topics: Humans; Quality of Life; Cardiomyopathy, Hypertrophic; Heart Failure; Ventricular Outflow Obstruction, Left
PubMed: 37767831
DOI: 10.1714/4100.40979 -
Pharmacotherapy Dec 2023Hypertrophic cardiomyopathy (HCM) is a genetic disorder for which first-line treatments for obstructive HCM (oHCM) include beta-blockers, non-dihydropyridine calcium...
BACKGROUND
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.
CASES
We present a series of seven patients with oHCM who transitioned from disopyramide to mavacamten and underwent echocardiograms mandated by a Risk Evaluation and Mitigations Strategies program. Two methods were employed. The first approach, involving washout of disopyramide before starting mavacamten, resulted in worsening of heart failure symptoms in the first two cases. The second approach, involving tapering disopyramide when starting mavacamten, was successfully implemented in the last five cases, with no adverse effects or worsening of systolic dysfunction.
CONCLUSION
Our method of tapering disopyramide when starting mavacamten using a stepwise approach is feasible and safe. Our report fulfills an unmet need by serving as a guide for other clinicians who seek to transition their patients from disopyramide to mavacamten.
Topics: Humans; Disopyramide; Cardiomyopathy, Hypertrophic; Benzylamines; Adrenergic beta-Antagonists
PubMed: 37688422
DOI: 10.1002/phar.2874 -
Progress in Cardiovascular Diseases 2023Hypertrophic cardiomyopathy (HCM) is a genetic condition with multiple different genetic and clinical phenotypes. As awareness for HCM increases, it is important to also... (Review)
Review
Hypertrophic cardiomyopathy (HCM) is a genetic condition with multiple different genetic and clinical phenotypes. As awareness for HCM increases, it is important to also be familiar with potential treatment options for the disease. Treatment of HCM can be divided into two different categories, medical and interventional. Typically for obstructive forms of the disease, in which increased septal hypertrophy, abnormally placed papillary muscles, abnormalities in mitral valve or subvalvular apparatus, lead to dynamic left ventricular outflow tract (LVOT) obstruction, treatment is targeted at decreasing obstructive gradients and therefore symptoms. Medications like beta blockers, calcium channel blockers, disopyramide can often accomplish this. However, in patients with severe obstruction or symptoms refractory to medical therapy, either surgical correction of the LVOT obstruction or percutaneous via alcohol septal ablation, are treatment options. In this review, we will focus on the invasive treatment of hypertrophic obstructive cardiomyopathy.
PubMed: 37652213
DOI: 10.1016/j.pcad.2023.08.003 -
Cardiology Research Aug 2023Hypertrophic cardiomyopathy is one of the most common genetic inherited diseases of myocardium, which is caused by mutation in genes encoding proteins for the cardiac... (Review)
Review
Hypertrophic cardiomyopathy is one of the most common genetic inherited diseases of myocardium, which is caused by mutation in genes encoding proteins for the cardiac sarcomere. It is the most frequent cause of sudden death in young people and trained athletes. All diagnostic methods, including heart catheterization, transthoracic and transesophageal echocardiography, magnetic resonance imaging, genetic counseling and tissue biopsy are required for risk and therapy stratification and should be individualized depending on phenotype and genotype. Current therapy has not been tested adequately. Beta-blockers and verapamil can cause hypotension which can make hypertrophic cardiomyopathy worse. Disopyramide has been inadequately studied, and mavacamten was only studied in small trials. More definitive trials are currently ongoing. Novel invasive and noninvasive diagnostics, medical therapies, interventional and surgical approaches tend to influence the natural history of the disease, favoring a better future for this patient population.
PubMed: 37559708
DOI: 10.14740/cr1514