Clinical Profile and Consequences of Atrial Fibrillation in Hypertrophic Cardiomyopathy.

BACKGROUND: Atrial fibrillation (AF), the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), is capable of producing symptoms that impact quality of life and is associated with risk for embolic stroke. However, the influence of AF on clinical course and outcome in HCM remains incompletely resolved. METHODS: Records of 1558 consecutive patients followed at the Tufts Medical Center Hypertrophic Cardiomyopathy Institute for 4.8±3.4 years (from 2004 to 2014) were accessed. RESULTS: Of the 1558 patients with HCM, 304 (20%) had episodes of AF, of which 226 (74%) were confined to symptomatic paroxysmal AF (average, 5±5; range, 1 to >20), whereas 78 (26%) developed permanent AF, preceded by 7±6 paroxysmal AF episodes. At last evaluation, 277 patients (91%) are alive at 62±13 years of age, including 89% in New York Heart Association class I or II. No difference was found in outcome measures for patients with AF and age- and sex-matched patients with HCM without AF. Four percent of patients with AF died of HCM-related causes (n=11), with annual mortality 0.7%; mortality directly attributable to AF (thromboembolism without prophylactic anticoagulation) was 0.1% per year (n=2 patients). Patients were treated with antiarrhythmic drugs (most commonly amiodarone [n=103] or sotalol [n=78]) and AF catheter ablation (n=49) or the Maze procedure at surgical myectomy (n=72). Freedom from AF recurrence at 1 year was 44% for ablation patients and 75% with the Maze procedure (P<0.001). Embolic events were less common with anticoagulation prophylaxis (4/233, 2%) than without (9/66, 14%) (P<0.001). CONCLUSIONS: Transient symptomatic episodes of AF, often responsible for impaired quality of life, are unpredictable in frequency and timing, but amenable to effective contemporary treatments, and infrequently progress to permanent AF. AF is not a major contributor to heart failure morbidity or a cause of arrhythmic sudden death; when treated, it is associated with low disease-related mortality, no different than for patients without AF. AF is an uncommon primary cause of death in HCM virtually limited to embolic stroke, supporting a low threshold for initiating anticoagulation therapy.

Is medial elbow pain correlated with cubital tunnel syndrome? An electrodiagnostic study.

INTRODUCTION: Medial elbow pain is often considered to be a symptom associated with ulnar neuropathy at the elbow (UNE). We examined the relationship between medial elbow pain and a positive electrodiagnostic (EDx) test result for UNE. METHODS: We performed a retrospective review of 884 patients referred for EDx evaluation of UNE. Regression models were used to determine the odds ratios between clinical findings and a positive EDx result for UNE. RESULTS: Patients reported medial elbow pain in 44.3% of cases. Clinical factors that correlated with a positive EDx study result for UNE included male gender, small and ring finger numbness, ulnar intrinsic weakness, and age. Medial elbow pain was negatively correlated with a positive EDx result. CONCLUSIONS: This study demonstrates a negative correlation between medial elbow pain and a positive EDx result for UNE. Medial elbow pain should not be considered a clear diagnostic symptom of UNE.

Impact of Advanced Therapies for Improving Survival to Heart Transplant in Patients with Hypertrophic Cardiomyopathy.

Heart transplant has become an increasingly important option for patients with end-stage nonobstructive hypertrophic cardiomyopathy (HC). However, clinical details related specifically to the overall HC transplant experience remain sparse. We assessed outcomes of HC heart transplants, from 2002 to 2016, at Tufts Medical Center. Fifty-two nonobstructive severely symptomatic patients underwent evaluation at 47 ± 13 years; 11 (21%) declined or failed to qualify, most commonly because of co-morbidities (n = 7). Of the remaining 41 patients ultimately listed, 6 (15%) died of heart failure awaiting transplant (11%/year), 26 underwent transplant, and 9 remained active on the list. Survival rates on the waiting list depended on ≥1 treatment intervention: inotropic medications (n = 20), ventricular assist devices (n = 7), or implantable defibrillators terminating ventricular tachyarrhythmias (n = 7). Of the 26 transplanted patients, 24 survived for 4.8 ± 3.4 years (up to 12), including 23 who are currently alive. The survival rate 5 years post transplant is 92%. Compared with heart transplants for other cardiomyopathies, patients with HC had similar mortality while wait-listed and post transplant (p = 0.77 and 0.13, respectively). In conclusion, a large proportion of patients with HC considered for transplant ultimately received hearts and experienced excellent short- and long-term survival rates. The survival rate on the waiting list was directly attributable to major interventions: implantable cardioverter-defibrillators, inotropic drugs, and ventricular assist devices, and the perception that patients with HC have low wait-list mortality risk does not appear justified. Neither normal ejection fraction nor peak oxygen consumption > 14 ml/kg/min should exclude drug refractory severely symptomatic patients with HC from heart transplant consideration.