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Introduction: Safety of vigorous exercise for individuals with appropriately-treated HCM remains debated. Physical activity practices in this population have not been described. The objective of this report is to describe physical activity pattern in HCM patients. Long term follow up which is ongoing will determine safety of vigorous exercise. Methods: The NIH-funded LIVE-HCM study prospectively enrolled individuals age 8-60 years with clinically overt HCM or patients with a positive genetic test for sarcomeric HCM. Participants (or parents, for children) answered surveys describing activity patterns. Vigorous exercise was defined as > 6 METS for > 60 hours per year. Clinical and demographic data were derived from medical record review. Results: Among 1798 participants, 745 (41%) reported exercising vigorously, including 296 competitive athletes, with 59 at high-school or college varsity level, 770 (43%) were exercising at moderate, and 283 (16%) at low levels. Of those working, 6% described jobs more active than walking, including 16 in protective services, and 11 in sports/coaching. Clinical/demographic data including imaging characteristics and treatments, are shown in Table, and were mostly similar amongst the exercise-groups. Conclusions: Many patients with HCM are engaged in vigorous exercise including competitive athletics. Prospective follow-up of this cohort is ongoing. Comparison of arrhythmic outcomes outcomes in vigorous exercisers vs moderate/low level exercisers will help determine the safety of exercise in HCM patients.

To determine whether abnormal blood pressure response (ABPR), with or without left ventricular outflow tract obstruction (LVOTO), is associated with adverse heart failure and arrhythmia outcomes in hypertrophic cardiomyopathy (HCM).A retrospective, single-center analysis was performed for adult HCM patients who underwent exercise stress testing.Of 589 patients included in the study, 192 (33%) demonstrated ABPR. A similar proportion of patients with ABPR had LVOTO compared to those without ABPR (56% vs 63%, p = 0.11). Patients with ABPR demonstrated lower percent predicted VO2 and METs achieved than those with LVOTO (16.9 ± 6.8 vs 21.6 ± 7.9, p = 0.002 and 5.3 ± 2.4 vs 7.4 ± 3.1, p 0.001). In a subgroup of 17 patients with LVOTO and ABPR who subsequently underwent successful myectomy, 5 (30%) demonstrated persistent ABPR. 23 patients (3.8%) experienced sudden cardiac death or ventricular arrhythmias, which were not associated with ABPR, regardless of age group. In multivariable analysis, syncope (p = 0.04), left ventricular hypertrophy (p = 0.02) and left atrial diameter (p = 0.006) were significantly associated with the composite outcome of sudden death or severe ventricular arrhythmia, whereas ABPR was not (p = 0.38). In contrast, ABPR was associated with subsequent heart failure hospitalization (p = 0.002), regardless of presence or absence of LVOTO (p = 0.04, p = 0.02).ABPR is associated with reduced functional capacity in HCM regardless of the presence of LVOTO but is not associated with adverse arrhythmia outcomes. Patients with ABPR have a higher incidence of subsequent heart failure hospitalization.

PurposeHypertrophic cardiomyopathy (HCM) is considered a hereditary autosomal dominant condition, but genetic testing is positive in only half of patients. In patients with negative genetic tests, the inheritance pattern and utility of family screening are unclear.MethodsSubjects with HCM were prospectively enrolled in a registry. A survey at a median follow-up of 4 years determined the yield of family screening.ResultsThe outcome of cardiac screening on 267 family members was reported by 120 survey respondents. Subjects with positive genetic test or family history (n=74, 62%) reported an HCM diagnosis in 34 of 203 first-degree relatives who were screened (17%). Affected family members were diagnosed at a mean age of 30-39 years, and 22 of 34 experienced HCM-related adverse events (65%). Gene test-negative subjects with no prior family history of HCM (n=46, 38%) reported an HCM diagnosis in only 2 of 64 first-degree relatives who were screened (3%, p0.001). These two individuals were diagnosed at age40 years without HCM-related adverse events.ConclusionHypertrophic cardiomyopathy is a heterogeneous disorder, only half of which tracks with a Mendelian inheritance pattern. Negative genetic testing and family history indicates a more complex genetic basis corresponding to low risk for family members.

Importance Formulating exercise recommendations for patients with hypertrophic cardiomyopathy is challenging because of concern about triggering ventricular arrhythmias and because a clinical benefit has not been previously established in this population. Objective To determine whether moderate-intensity exercise training improves exercise capacity in adults with hypertrophic cardiomyopathy. Design, Setting, and Participants A randomized clinical trial involving 136 patients with hypertrophic cardiomyopathy was conducted between April 2010 and October 2015 at 2 academic medical centers in the United States (University of Michigan Health System and Stanford University Medical Center). Date of last follow-up was November 2016. Interventions Participants were randomly assigned to 16 weeks of moderate-intensity exercise training (n = 67) or usual activity (n = 69). Main Outcomes and Measures The primary outcome measure was change in peak oxygen consumption from baseline to 16 weeks. Results Among the 136 randomized participants (mean age, 50.4 [SD, 13.3] years; 42% women), 113 (83%) completed the study. At 16 weeks, the change in mean peak oxygen consumption was +1.35 (95% CI, 0.50 to 2.21) mL/kg/min among participants in the exercise training group and +0.08 (95% CI, −0.62 to 0.79) mL/kg/min among participants in the usual-activity group (between-group difference, 1.27 [95% CI, 0.17 to 2.37]; P = .02). There were no occurrences of sustained ventricular arrhythmia, sudden cardiac arrest, appropriate defibrillator shock, or death in either group. Conclusions and Relevance In this preliminary study involving patients with hypertrophic cardiomyopathy, moderate-intensity exercise compared with usual activity resulted in a statistically significant but small increase in exercise capacity at 16 weeks. Further research is needed to understand the clinical importance of this finding in patients with hypertrophic cardiomyopathy, as well as the long-term safety of exercise at moderate and higher levels of intensity. Trial Registration clinicaltrials.gov Identifier:NCT01127061

Sleep apnea affects more than half of patients with acute ischemic stroke and is associated with poor stroke outcome. This pilot study assessed the feasibility of a randomized, sham-controlled continuous positive airway pressure (CPAP) trial in subjects with acute ischemic stroke. Subjects identified with sleep apnea based on an apnea-hypopnea index ≥5 on overnight polysomnography or portable respiratory monitoring within 7 days of onset of stroke symptoms were randomized to receive active or sham CPAP for a 3-month period. Objective usage was ascertained by compliance data cards. Subjects, treating physicians, and outcome assessors were masked to intervention allocation. Among 87 subjects who provided consent, 74 were able to complete sleep apnea screening, 54 (73%) of whom had sleep apnea. Thirty-two subjects agreed to randomization. Of the 15 subjects who commenced active titration, 11 (73%) took the device home, and 8 (53%) completed the 3-month follow-up. Of the 17 subjects who commenced sham titration, 11 (65%) took the sham device home and completed the 3-month follow-up. The median cumulative usage hours over the 90 days were similar in the active group (53 hours; interquartile range, 22-173 hours) and the sham group (74 hours; interquartile range, 17-94 hours), and blinding to subject condition was successfully maintained. This first-ever randomized, sham-controlled trial of CPAP in patients with recent stroke and sleep apnea demonstrates that sham treatment can be an effective placebo.

Denervation of oropharyngeal muscles in obstructive sleep apnea (OSA) has been suggested by needle electromyography (EMG) and muscle biopsy, but little is known about oropharyngeal nerve conduction abnormalities in OSA. We sought to compare hypoglossal nerve conduction studies in patients with and without OSA. Unilateral hypoglossal nerve conduction studies were performed on 20 subjects with OSA and 20 age-matched controls using standard techniques. Median age was 48 years in OSA subjects and 47 years in controls. Hypoglossal compound muscle action potential (CMAP) amplitudes were significantly reduced (P = 0.01, Wilcoxon signed-rank test), but prolongation of latencies in OSA subjects did not reach significance in comparison to those of controls. Among a subgroup of subjects without polyneuropathy (15 pairs), reduced amplitudes in OSA subjects retained borderline significance (P = 0.05). Hypoglossal nerve conduction abnormalities may distinguish patients with OSA from controls. These abnormalities could potentially contribute to, or arise from, OSA.

Background: Obstructive sleep apnea is a very common condition after stroke, and it predicts poor outcomes. Unfortunately, stroke patients often do not tolerate continuous positive airway pressure. We hypothesized that a 1-piece head frame headgear would be easier and quicker to use than a traditional strap headgear. Methods: A convenience sample of ischemic stroke patients was taught to use 2 different headgear systems, a head frame and straps. Subjects were timed while putting on and taking off the 2 headgears, and they were queried about their ease of use. Results: All the 30 enrolled subjects found the head frame to be easier to apply and to remove than the straps. The patients took longer to put on (p < 0.01) and to remove (p < 0.01) the straps than the head frame. Conclusions: Headgear selection should be considered when fitting a stroke patient with a continuous positive airway pressure mask.

Background and Purpose— Sleep apnea is very common after stroke and is associated with poor outcome. Supine sleep is known to exacerbate apneas in the general sleep apnea population. We therefore investigated the pattern of sleep positions in the acute stroke period. Methods— Inpatients with acute ischemic stroke underwent full polysomnography that included continuous monitoring of sleep positions. Sleep apnea severity was measured using the apnea-hypopnea index (AHI). Stroke severity was measured by the NIH Stroke Scale (NIHSS) at the time of study enrollment by certified study personnel. Percent total sleep time spent in the supine position was calculated and compared by stroke severity based on a median split of NIHSS using a Wilcoxon rank-sum test. Results— Of the 30 patients, the median age was 67. The median AHI was 23 (IQR: 6, 47). Twenty-two patients (73%) had sleep apnea with an AHI ≥5. The vast majority of sleep time among the stroke cases was spent supine, with a median percent sleep time spent supine of 100 (IQR: 62, 100). The majority (63%) of subjects spent no time asleep in any of the nonsupine positions (prone, left, right). Median percent sleep time supine was 100 (IQR: 100, 100) in those with a higher NIHSS and 63 (IQR: 51, 100) in those with a lower NIHSS ( P Conclusions— Given the high prevalence of supine sleep identified, research into positional therapy for stroke patients with sleep apnea seems warranted.

This cross-sectional study of acute ischemic stroke patients examined relationships between hypoglossal nerve conduction, sleep-disordered breathing (SDB), and its severity.Patients within 7 days of stroke underwent nocturnal respiratory monitoring with the ApneaLink device and hypoglossal nerve conduction studies.Eighteen of 52 subjects (35% [95% confidence interval: 22%, 49%]) had an abnormal hypoglossal amplitude and 23 (44% [95% confidence interval: 30%, 59%]) had an abnormal hypoglossal latency. No differences were identified in hypoglossal nerve latency or amplitude between those with (n = 26) and without (n = 26) significant SDB, defined by an apnea-hypopnea index ≥ 15. However, hypoglossal nerve conduction latency was associated (linear regression p0.05) with SDB severity as reflected by the apnea-hypopnea index.Acute ischemic stroke patients have a high prevalence of hypoglossal nerve dysfunction. Further studies are needed to explore whether hypoglossal nerve dysfunction may be a cause or consequence of SDB in stroke patients and whether this association can provide further insight into the pathophysiology of SDB in this population.

Obstructive sleep apnea (OSA) is common after stroke and associated with poor stroke outcomes. Whether OSA after acute stroke is caused by anatomic, physiologic, or both etiologies has not been studied. We therefore used brain magnetic resonance imaging (MRI) scans to assess oropharyngeal anatomy in stroke patients with and without OSA.Patients within 7 days of ischemic stroke underwent nocturnal polysomnography. Sagittal T1-weighted MRI performed for clinical purposes was used to measure retropalatal distance, soft palatal length, soft palatal thickness, retroglossal space, and tongue length. Nasopharyngeal area and high retropharyngeal area were measured from axial T2-weighted images, and lateral pharyngeal wall thickness from coronal T1-weighted images.Among 27 subjects, 18 (67%) had OSA (apnea/hypopnea index (AHI)5). Demographics, vascular risk factors, and stroke severity were similar in the two groups. Median retropalatal distance was shorter in subjects with OSA (Wilcoxon rank-sum test, p=0.03). Shorter retropalatal distance was associated with higher AHI (linear regression, p=0.04). None of the other morphological characteristics differed.Anatomic difference between awake acute stroke patients with and without OSA shows that the sleep disorder cannot be attributed solely to sleep, sleeping position, or changes in neuromuscular control that are specific to the sleep state.