Hypertrophic cardiomyopathy is the most common inherited heart disease. Although it was first described over 50 years ago, there has been little in the way of novel disease-specific therapeutic development for these patients. Current treatment practice largely aims at symptomatic control using old drugs made for other diseases and does little to modify the disease course. Septal reduction by surgical myectomy or percutaneous alcohol septal ablation are well-established treatments for pharmacologic-refractory left ventricular outflow tract obstruction in hypertrophic cardiomyopathy patients. In recent years, there has been a relative surge in the development of innovative therapeutics, which aim to target the complex molecular pathophysiology and resulting hemodynamics that underlie hypertrophic cardiomyopathy. Herein, we review the new and emerging therapeutics for hypertrophic cardiomyopathy, which include pharmacologic attenuation of sarcomeric calcium sensitivity, allosteric inhibition of cardiac myosin, myocardial metabolic modulation, and renin-angiotensin-aldosterone system inhibition, as well as structural intervention by percutaneous mitral valve plication and endocardial radiofrequency ablation of septal hypertrophy. In conclusion, while further development of these therapeutic strategies is ongoing, they each mark a significant and promising advancement in treatment for hypertrophic cardiomyopathy patients.
Atrial fibrillation is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), occurring in approximately 25% of patients, which is four to six times more common than in similarly aged patients of the general population. Atrial fibrillation is poorly tolerated by HCM patients, largely due to their dependence on atrial systole for left ventricular filling. HCM patients who develop atrial fibrillation have an increased rate of heart failure related mortality and disabling or fatal thromboembolic events, as well as functional deterioration due to progressive heart failure when left untreated. Atrial fibrillation is both common in HCM and may lead to significant morbidity and mortality. Accurate risk stratification for atrial fibrillation in this population is crucial as contemporary treatments are highly successful. In this paper, we review the current understanding of known risk factors for atrial fibrillation, including different imaging-based parameters that assess left atrial structural and functional remodeling, electrocardiographic changes that reflect left atrial electrical remodeling, and a focus on comorbid obstructive sleep apnea, and in addition we review variables that have been reported to be predictive of atrial fibrillation. Last, we summarize the accumulating evidence for HCM patients having an intrinsic atrial myopathy.
Background Failure of metal-on-metal (MOM) total hip arthroplasty (THA) bearings is often accompanied by an aggressive local reaction associated with destruction of bone, muscle and other soft tissues around the hip. Little is known about whether patient-reported physical and mental function following revision THA in MOM patients are compromised by this soft tissue damage, and whether revision of MOM THA is comparable to revision of hard-on-soft bearings such as metal-on-polyethylene (MOP). Methods We identified 75 first-time MOM THA revisions and compared them to 104 first-time MOP revisions. Using prospective patient-reported measures via the Veterans RAND-12, we compared physical (PCS) and mental function (MCS) at pre-operative baseline and post-operative follow-up between revision MOM THA and revision MOP THA. Results PCS did not vary between the groups pre-operatively and at 1 month, 3 months, and 1 year post-operatively. MCS pre-operatively and 1 and 3 months post-operatively were lower in patients in the MOM cohort compared to patients with MOP revisions (Baseline 43.7 vs. 51.3, P<0.001; 1 month: 44.9 vs. 53.3, P<0.001; 3 months 46.0 vs. 52.3, P=0.016). However, by 1 year, MCS scores were not significantly different between the revision cohorts. Conclusion Post-revision physical function in revised MOM THA patients does not differ significantly from the outcomes of revised MOP THA. Mental function is markedly lower in MOM patients at baseline and early in the post-operative period, but does not differ from MOP patients at 1 year after revision. This information should be useful to surgeons and physicians facing MOM THA revision.
The presentation of a cardiac hamartoma, an exceedingly rare and histologically benign cardiac tumor, can be variable. We describe a case of refractory ventricular tachycardia in a patient with a cardiac mass failing multiple pharmacologic and procedural interventions, ultimately treated by cardiac transplantation and diagnosed with a mesenchymal cardiac hamartoma. ( Level of Difficulty: Intermediate. )
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