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Axenfeld-Rieger syndrome is a rare multi-system disorder associated with cardiac anomalies. All patients with a diagnosis of Axenfeld-Rieger syndrome were identified from our electronic medical record. Chart review was performed to document the presence and types of CHD. Out of 58 patients, 14 (24.1%) had CHD and a wide variety of cardiac lesions were identified.

Competing Interests: The authors declare no conflicts of interest.

Background: Older adults benefit most from engaging in higher-intensity physical activity, which is often determined using step rate thresholds. Fixed step rate thresholds that correspond to moderate (MPA) and vigorous-intensity physical activity (VPA) have been developed for heuristic activity promotion. The activPAL monitor uses step rate thresholds to determine activity intensity. Stepping thresholds may also vary based on body mass index (BMI) or aerobic fitness level in older adults. Despite the various thresholds used in the literature, it is unclear whether they produce similar outcomes., Research Question: How does time spent in physical activity intensities compare between different step rate thresholds in older adults?, Methods: Thirty-eight participants (24♀; 67 ± 4 years; BMI: 26.6 ± 4.4 kg/m 2 ) wore an activPAL monitor 24-hr/day for up to 7-d (total: 205-d). Aerobic fitness (V̇O 2 max: 23 ± 8 ml/kg/min) was determined via indirect calorimetry during a maximal, graded cycling test. Time spent in each intensity category (light-physical-activity [LPA], MPA, VPA) was determined using the fixed (MPA/VPA) 100/130, 110/130, and activPAL step rate thresholds (74/212), as well as BMI-adjusted absolute (108.5 ± 2.5/134.0 ± 4.8) and BMI-adjusted relative (40%/60% V̇O 2 max; 111.4 ± 14.7/132.0 ± 19.0) cut-offs. Times spent in each intensity category were compared between methods., Results: The activPAL and 100/130 thresholds yielded less LPA and more MPA than all other methods. The activPAL had no time spent in VPA at all. The BMI-adjusted absolute and relative thresholds produced statistically equivalent time in LPA and MPA (via equivalence testing), but not VPA. No two methods yielded similar time spent in LPA, MPA, or VPA., Significance: The choice of step rate threshold has a major impact on physical activity intensity outcomes in older adults. Inherently, strategies that adjust for older adults' body size and/or aerobic fitness level provide a more individualized data processing strategy than fixed thresholds that assume the same threshold for all older adults., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Introduction: Acute, laboratory-based bouts of prolonged sitting attenuate lower-limb arterial endothelial-dependent vasodilation. However, the impact of habitual sedentary patterns on popliteal artery endothelial health is unclear. We tested the hypothesis that greater habitual total sedentary time, more time spent in prolonged sedentary bouts, and fewer sedentary breaks would be associated with worse popliteal flow-mediated dilation (FMD) responses., Methods: This cross-sectional study used 98 healthy participants (19-77 years, 53 females) that wore an activPAL monitor on the thigh for 6.4 ± 0.8 days to objectively measure sedentary activity and completed a popliteal ultrasound assessment to determine FMD. Both relative (%baseline diameter) and absolute (mm) FMD were calculated. Using bivariate correlation and multiple regression analyses, we examined if there were relationships between sedentary outcomes and FMD while statistically controlling for any potential confounders., Results: In the multiple regression model, age ( p = 0.006, β = -0.030, 95% CI = -0.051, -0.009) and total time in sedentary bouts > 1 hour ( p = 0.031, β = -0.005, 95% CI = -0.009, -0.001) were independent predictors of relative FMD. Age (β = -0.002, 95% CI = -0.003, -0.001), mean blood flow (β = 0.013, 95% CI = 0.002, 0.024), moderate-intensity physical activity (β = 155.9E -5 , 95% CI = 22.4E -5 , 289.4E -5 ), sedentary breaks (β = 0.036, 95% CI = 0.007, 0.066), and total time spent in sedentary bouts > 1 hour (β = -25.02E -5 , 95% CI = -47.67E -5 , -2.378E -5 ) were predictors of absolute FMD (all, p < 0.047). All independent outcomes remained significant after partially controlling for all other predictor variables (all, p < 0.031)., Conclusions: Habitual prolonged sedentary bouts and sedentary breaks, but not total sedentary time, were predictors of popliteal endothelial-dependent vasodilatory function. The patterns by which sedentary time is accumulated may be more important than the total sedentary time on lower-limb arterial health.

Background: Different approaches have been implemented to calculate stepping cadence (steps/min) that vary in the time demominator used. Given the differences in how stepping intensity are calculated, it is unclear if they are more so associated with total step counts., Research Objective: This study compared three methods of calculating stepping cadence and determined their relationship with total step counts., Methods: 132 participants (74♀; 35 ± 20 years; body mass index: 24.9 ± 4.0 kg•m -2 ) wore an activPAL monitor 24-hr/day for up to 8-d (total: 869-d). The total steps/day, time spent stepping (0.1 s resolution; to calculate bout stepping rate), time spent stepping in 60 s epochs (step accumulation), and awake time (awake cadence) were determined. Each cadence method (in steps/min) were compared via Spearman's rank correlation. The relationships versus total step count were determined, and the strength of these relationships compared between cadence measures (95% confidence interval of correlation differences)., Results: Bout stepping rate (85 ± 14 steps/min) was larger than step accumulation (34 ± 12 steps/min) and awake cadence (10 ± 5 steps/min, both: P < 0.001). Step accumulation was positively strongly related to bout stepping rate (ρ = 0.813; P < 0.001) whereas awake cadence was weakly related to bout stepping rate (ρ = 0.496; P < 0.001). Step accumulation (ρ = 0.634; P < 0.001) and awake cadence (ρ = 0.964; P < 0.001) were more related to step counts than bout stepping rate (ρ = 0.497; P < 0.001; 95% confidence intervals of correlation differences: step accumulation=0.10-0.17, awake cadence: 0.42-0.52)., Significance: Without a precise measure of time spent stepping, stepping cadence is lower using the step accumulation and awake cadence methods. Step accumulation and awake cadence are more related to total step counts than bout stepping rate. Bout stepping rate outcomes reflect continuous stepping rate, does not rely on a preset epoch, and may have less overlap with step counts, which may have implications for determining the unique contributions of step count versus stepping cadence on health outcomes., (Copyright © 2021 Elsevier B.V. All rights reserved.)

In the brachial artery, conflicting evidence exists regarding the relationship between the low-flow-mediated constriction (L-FMC) and subsequent flow-mediated dilation (FMD) responses, which may confound interpretation of the latter. The popliteal artery is a common site for atherosclerotic development, which is preceded by endothelial dysfunction. We aimed to determine whether the magnitude of popliteal L-FMC impacted FMD responses, which is currently unknown. L-FMC and FMD were assessed in the popliteal artery via high-resolution duplex ultrasonography and quantified as the percent change in diameter (from baseline) during ischaemia and in response to hyperaemia, respectively. Using partial correlations and multiple regression analyses, we evaluated the association between popliteal L-FMC on FMD in 110 healthy participants (60 females; 42 ± 22 [19-77] years). All variables univariately associated (p < 0.05) with popliteal relative FMD (relative L-FMC, log-SR AUC , age, systolic blood pressure, diastolic blood pressure, resting shear rate) were inputted into a model that explained 35% of the variance. The reactive hyperaemia stimulus (log-SR AUC : β = 1.10) and relative L-FMC (β = -0.39) were the only independent predictors of FMD (both, p < 0.01). Relative L-FMC was negatively correlated to relative FMD, after controlling for the significant univariate predictor variables listed above (R = -0.30; p = 0.002). An augmented (ie healthier) L-FMC response was linked with a larger FMD response as determined by the independent inverse association observed between these shear-stress-mediated measures of vasoreactivity., (© 2021 Scandinavian Society of Clinical Physiology and Nuclear Medicine. Published by John Wiley & Sons Ltd.)

Purpose: Acute prolonged bouts of sitting reduce popliteal artery blood flow and flow-mediated dilation (FMD). Individuals with higher aerobic fitness have enhanced popliteal FMD. Conflicting evidence regarding whether more aerobically fit individuals are protected from the negative impacts of sitting on popliteal endothelial function in male-dominated studies have been reported. We further explored the relationship between aerobic fitness and sitting-induced impairments in popliteal blood flow and FMD in a more sex-balanced cohort., Methods: Relative peak oxygen consumption (V̇O 2 peak) was assessed using a cycling-based incremental test in 21 healthy adults (eight males; 23 ± 2 years; 23.9 ± 2.9 kg/m 2 ). Popliteal blood flow and relative FMD (%) were measured via duplex ultrasonography before and after 3 h of uninterrupted sitting. Pearson correlations were performed separately between V̇O 2 peak versus pre-sitting and sitting-induced reductions in popliteal outcomes., Results: Aerobic fitness (41.0 ± 9.7 ml/kg/min) was positively correlated with pre-sitting popliteal blood flow (65 ± 23 mL/min; R = 0.59, P = 0.005) and relative FMD (4.2 ± 1.5%; R = 0.49, P = 0.03). As expected, sitting reduced resting blood flow (19 ± 11 mL/min) and FMD (1.9 ± 0.7%) (both, P < 0.001). V̇O 2 peak was inversely related to sitting-induced declines in blood flow (Δ-46 ± 23 mL/min; R = - 0.71, P < 0.001) and FMD (Δ-2.4 ± 1.5%; R = - 0.51, P = 0.02)., Conclusions: Although higher aerobic fitness was associated with more favorable popliteal endothelial-dependent vasodilator responses, it also corresponded with larger sitting-induced impairments in FMD. This suggests that being more aerobically fit does not protect against sitting-induced vascular endothelial dysfunction. As such, all young adults should minimize habitual prolonged sedentary bouts, regardless of their aerobic fitness level., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Sedentary time has recently been included in the 24-h activity guidelines. However, the impact of habitual sedentary patterns on autonomic cardiovascular regulation are unclear. We tested the hypothesis that more sedentary time and fewer sedentary breaks were associated with lower cardiovagal baroreflex sensitivity. More frequent sedentary breaks, but not total sedentary time, was independently and positively associated with vagally mediated blood pressure control. Breaking up sedentary time could be more important than total sedentary time for cardiovascular health. Novelty: Breaks in sedentary time is an independent predictor of cardiovagal baroreflex sensitivity, with more frequent breaks associated with better vagally mediated blood pressure regulation.

The activPAL linear cadence-metabolic equivalents (METs) equation poorly estimates activity intensity. The magnitude of acceleration in three directional planes may be a superior predictor of activity intensity than stepping cadence, with accelerometry count thresholds developed in children/adolescent populations. We extracted the proprietary accelerometer-derived information to develop a counts-METs model and cross-validates it in laboratory and free-living conditions. Forty adults (25±6 years) wore an activPAL during a 7-stage progressive treadmill protocol (criterion: indirect calorimetry). Tri-axial accelerometry-derived activity counts (vector magnitude) and METs data from a subset of participants (n = 20) were modelled (R 2 =0.76) and the regression equation evaluated in the remaining participants (n = 20). Thirty-two of these participants wore the activPAL during free-living conditions (n = 192-d; criterion: PiezoRxD monitor). The absolute percent error of the counts-METs model in the laboratory cross-validation was 18±13%, with equivalence testing determinining equivalent MET values to indirect calorimetry during the slowest (1.5 mph) and fastest (4.0-4.5 mph) stages. In free-living conditions, the model accurately quantified light- and moderate-intensity physical activity but underestimated vigorous-intensity activity (6.5±11.3 vs. 5.5±20.8 mins/day; p < 0.001). We developed and present a data analysis method using the activPAL tri-axial accelerometry counts to improve estimations of physical activity intensity in controlled laboratory settings and uncontrolled free-living settings., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The activPAL is a valid measure of step counts and posture, but its ability to determine physical activity intensity is unclear. This study tested the criterion validity of the activPAL using its built-in linear cadence-metabolic equivalents (METs) equation (activPAL-linear) versus an individualized height-adjusted curvilinear cadence-METs equation (activPAL-curvilinear) to estimate intensity-related physical activity. Forty adults (25±6 years, 23.3±4.1 kg/m 2 ) wore an activPAL during a 7-stage progressive treadmill walking protocol (criterion: indirect calorimetry). A sub-sample (n=32) wore the device during free-living conditions for 7-days (criterion: PiezoRxD monitor). In the laboratory, the activPAL-linear overestimated METs during slow walking (1.5-3.0 miles•hour -1 ) but underestimated METs during fast walking (3.5-4.5 miles•hour -1 ) (all, p<0.001). In the free-living condition, the activPAL-linear overestimated time in light-intensity activity and underestimated moderate-intensity activity (both, p<0.001), but did not register any vigorous-intensity activity. In contrast, the activPAL-curvilinear estimated values statistically equivalent to indirect calorimetry for treadmill stages 1-6 (1.5-4.0 miles•hour -1 ) and to the PiezoRxD determined light- and moderate-intensity activity during free-living. We present a simple, data processing technique that uses an alternative curvilinear cadence-MET equation that improves the ability of the activPAL to measure intensity-related physical activity in both laboratory and free-living settings.

Lower-limb arteries, such as the popliteal artery, are a common site of atherosclerosis. These arteries are habitually exposed to large fluctuations in blood flow during physical and sedentary activities. Low-flow-mediated constriction (L-FMC) and flow-mediated dilation (FMD) provide indices of endothelial-dependent vasoconstriction and vasodilation, respectively. Age and sex both impact upper-limb FMD. However, it is unclear whether these factors also influence popliteal endothelial-dependent function. Popliteal endothelial function was compared between younger and older males and females (n=14 per group) matched for age- and sex-specific relative aerobic fitness levels (each group's normative percentile: ~45%). Nitroglycerin-mediated dilation (NMD) was also assessed as a measure of endothelial-independent vasodilation. Ageing reduced relative popliteal FMD in both males (older: 4.3±1.8% versus younger 5.7±1.9%) and females (older: 2.9±1.8% versus younger: 6.1±1.6%, both: P<0.046). FMD was also lower in older females versus older males (P=0.04). Popliteal NMD findings followed the same pattern as FMD. Compared to younger adults, relative L-FMC responses were blunted among older males (older: -1.2±1.1% versus younger: -2.2±1.0%) and females (older: -1.0±1.2% versus younger: -2.1±1.3%, both P<0.03) with no sex-differences observed in either age group (all, P>0.60). The adverse age- and sex-related (older adults only) declines in popliteal FMD were mediated, in part, by reduced vascular smooth muscle sensitivity to nitric oxide. Endothelial-dependent vasoconstriction was also attenuated with age, but unaffected by sex. Despite similar normative aerobic fitness percentiles (~45%), older adults exhibited attenuated popliteal endothelial function than their younger counterparts. This was particularly evident in older females who exhibited the lowest endothelial-dependent vasodilatory responses., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Heterogeneous flow-mediated dilation (FMD) and low-flow-mediated constriction (L-FMC) responses have been reported between upper- and lower-limb arteries. Radial artery L-FMC, but not FMD, responses are blunted when endothelial-derived hyperpolarizing factors (EDHFs) or prostaglandin production is inhibited in young adults. However, it is unknown if these mechanisms similarly impact endothelial-dependent responses in the brachial (BA) and popliteal (POP) arteries. We tested whether BA- and POP-L-FMC and FMD would be influenced by independent EDHF and prostaglandin inhibition. Eighteen participants (23 ± 3 yr; 6♀) completed three randomized and double-blinded ultrasound assessments following ingestion of an opaque capsule containing maltodextrin (control), 150 mg of fluconazole (EDHF inhibition), or 500 mg of aspirin (prostaglandin inhibition). POP resting diameter was reduced following fluconazole administration (6.13 ± 0.63 mm vs. 6.19 ± 0.65 mm in control, P = 0.03). Compared with control, fluconazole also blunted the relative L-FMC responses in both the BA (-2.1 ± 0.8% vs. -0.8 ± 1.0%, P = 0.001) and POP (-1.7 ± 1.1% vs. -0.8 ± 0.9%, P = 0.009). In contrast, aspirin did not impact either the BA (-1.9 ± 0.7%) or POP-L-FMC (-1.3 ± 0.6%) responses (both, P > 0.35). The FMD response was unchanged following fluconazole or aspirin administration in either artery (both, P > 0.36). Our findings demonstrate that EDHF mediates L-FMC responses in both the brachial and popliteal arteries. Complementary to the nitric oxide-mediated FMD response, L-FMC appears to provide information regarding the EDHF pathway. Future research should uncover if these mechanisms impact older adults and/or patient populations characterized by vascular endothelial dysfunction associated with low aerobic fitness and habitual physical activity levels. NEW & NOTEWORTHY We compared changes in upper- and lower-limb artery endothelial-dependent vasodilatory and vasoconstrictor responses between control, prostaglandin inhibition, and endothelial-derived hyperpolarizing factor inhibition conditions. Neither prostaglandins nor endothelial-derived hyperpolarizing factor influenced flow-mediated dilation responses in either the brachial or popliteal artery. In contrast, endothelial-derived hyperpolarizing factor, but not prostaglandins, reduced resting brachial artery blood flow and shear rate and resting popliteal artery diameter, as well as low-flow-mediated constriction responses in both the popliteal and brachial arteries.

When controlling for baseline diameter, males have greater brachial flow-mediated dilation (BA-FMD) responses than females. It is unclear whether sex differences in baseline diameter also influences popliteal FMD (POP-FMD), which may be impacted by cardiorespiratory fitness and physical activity levels. We hypothesized that males would exhibit greater BA-FMD and POP-FMD when allometrically scaled to baseline diameter. FMD (ultrasonography), cardiorespiratory fitness (indirect calorimetry), and objectively measured physical activity were assessed in males ( n = 13; age, 23 ± 3 years; peak oxygen consumption, 48.0 ± 7.1 mL·kg -1 ·min -1 ) and females ( n = 13; age, 24 ± 2 years; peak oxygen consumption, 36.8 ± 6.0 mL·kg -1 ·min -1 ). Both groups had similarly high levels of moderate-to-vigorous intensity physical activity (503 ± 174 vs. 430 ± 142 min·week -1 , p  = 0.25). However, males were more aerobically fit ( p < 0.001) and females accumulated more light-intensity physical activity (182 ± 67 vs. 127 ± 53 min·week -1 , p  = 0.03). Relative and allometrically scaled BA-FMD were similar (both, p  ≥ 0.09) between sexes. In contrast, relative (6.2% ± 1.0% vs. 4.6% ± 1.4%, p  = 0.001) and scaled (6.8% ± 1.7% vs. 4.7% ± 1.7%, p  = 0.03) POP-FMD were greater in females. Relative POP-FMD was related to light-intensity physical activity in the pooled sample ( r = 0.43; p  = 0.04). However, the enhanced relative POP-FMD in females remained after adjusting for higher light-intensity physical activity levels ( p  = 0.01). Young females have enhanced popliteal, but not brachial, endothelial health than males with similar moderate-to-vigorous intensity physical activity levels and higher cardiorespiratory fitness. Novelty In physically active adults, females had greater POP-FMD but not BA-FMD than males. The enhanced POP-FMD in females was not related to greater vascular smooth muscle sensitivity to nitric oxide or their smaller baseline diameters. POP-FMD was associated with light physical activity levels in the pooled sample.

Uninterrupted sitting can impair popliteal flow-mediated dilation (FMD) responses in young, premenopausal females when endogenous or exogenous estrogen levels are low. However, it is unknown whether sitting-induced FMD responses are altered when estrogen levels are elevated in females who naturally menstruate (NAT) or those using combined, monophasic oral contraceptive pills (OCP). This study tested the hypothesis that the decline in popliteal FMD following an acute bout of prolonged sitting would be attenuated during the later versus earlier phases of a natural menstrual or OCP cycle. Popliteal FMD was measured before and after 3 h of sitting in NAT females ( n = 9; 23 ± 3 yr) and OCP females ( n = 9; 23 ± 3 yr) during both of their respective phases. At pre-sit, relative FMD was greater in the later phase versus earlier phase in NAT (4.6 ± 1.6% to 5.8 ± 1.5%; P = 0.002) but not between pill phases among OCP (4.4 ± 1.2% to 4.8 ± 1.6%; P = 0.32). Both groups exhibited similar prolonged sitting-induced impairments in popliteal FMD (pre- to post-sitting time: P < 0.001; group ΔFMD: P = 0.66; phase ΔFMD: P = 0.42; interaction ΔFMD: P = 0.72) regardless of menstrual cycle phase (earlier: -2.5 ± 1.5%; later: -2.4 ± 1.0%) or pill phase (inactive pill: -2.4 ± 1.4%; active pill: -2.1 ± 1.1%). Our findings demonstrate that lower-limb arterial endothelial-dependent vasodilatory function was enhanced in the later versus earlier menstrual phase among NAT but unaffected by combined, monophasic pill phases in OCP. Furthermore, healthy, young females exhibited pronounced negative lower-limb vascular effects in response to prolonged sitting regardless of whether they were in the earlier or later phases of a natural menstrual or contraceptive pill cycle. NEW & NOTEWORTHY We compared changes in popliteal artery endothelial function to a 3-h bout of sitting in females across their natural menstrual or oral contraceptive pill cycles. Pre-sitting endothelial-dependent vasodilation was greater in females who naturally menstruate during the later versus earlier phase but unchanged among contraceptive pill phases. Neither menstrual nor oral contraceptive pill phases attenuated the robust decline in conduit artery health following an acute period of uninterrupted sitting in young females.

Background: Ice hockey players often lack hip mobility which may be attributed to excessive time spent in a flexed position during competition and the intensive use of hip external rotators and extensors. Previous investigations demonstrate that foam rolling increases range-of-motion in healthy populations. It is unclear whether such benefits occur in athletes with chronic hip tightness. Further, athletes often perform pre-competition warm-up up to 60-min prior to a game, therefore we investigated the retention of the effects of foam rolling. The purpose of this study was to analyze the effects of foam rolling the anterior and posterior aspects of the thigh on passive hip flexion in ice hockey players, up to 60-min post-rolling., Methods: Sixteen amateur ice hockey players (8 ♀, 22 ± 2 years, 25.1 ± 3.5 kg m -2 ) warmed up for 5-min then performed 2, 1-min bouts of foam rolling using a high density multi-rigid roller on the quadriceps and hamstrings, with 1-min break between sets. Passive hip flexion was measured before and immediately after rolling, 10-min, 30 and 60-min post-rolling using the Coach's Eye application during a passive straight leg raise., Results: Compared to baseline, passive hip flexion was greater immediately after foam rolling (p < 0.01), 10 min (p = 0.05), 30 (p < 0.01) and 60-min post-rolling (p < 0.01)., Conclusions: Regardless of measurement time point, significant increases in passive hip flexion occurred up to 60-min compared to baseline. Incorporation of foam rolling into pre-competition warm-ups may be beneficial when increases in hip mobility are warranted, up to 1-h before competition., Competing Interests: Declaration of competing interest Neither author declares any conflicts of interest., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Existing analytical approaches used to measure free-living stepping cadence (steps per minute; spm) characterize stepping two-ways: 1) 60 s epochs without considering time spent stepping during the epoch (step accumulation; SA), or 2) a bout-based analysis that considers both number of steps and time spent stepping during stepping bouts (total event cadence; TEC). SA and TEC may incorrectly characterize cadence in epochs that do not consist of continuous stepping or if there are marked changes in cadence within a stepping bout, respectively., Research Question: How does a bout-based analytical method that examines each stepping bout individually and considers within-bout changes in stepping cadence during epochs ≥120 s (Bout Cadence; BC) compare to SA and TEC?, Methods: ActivPAL™-derived data from 122 participants were analyzed (790 total days). SA and TEC were calculated according to previous literature. BC calculated cadence bout-by-bout and stride-by-stride for bouts lasting <120 s and ≥120 s, respectively. Time spent stepping between 0-140 spm (divided into 20 spm bins) or >140 spm were determined for each method. Time spent in slow (0-80 spm), medium (80-120 spm) and fast (>120 spm) cadences for each method were compared via Bland-Altman analyses., Results: Almost half (43 %) of the total number of 60 s epochs included ≥2 stepping bouts, and 37 % of total stepping time was accumulated in continuous stepping bouts ≥120 s. Compared to TEC, BC identified more daily time spent in the 20-40 spm and >120 spm cadence bins, but less time spent in the 60-120 spm range. Both SA (fixed bias: -11.0 ± 12.4 min/day) and TEC (fixed bias: -10.0 ± 13.6 min/day) underestimated faster stepping cadences compared to BC., Significance: Existing analytical approaches largely underestimate faster stepping cadences, resulting in inaccurate measurements of vigorous-intensity stepping activity. The BC better characterizes higher intensity stepping activity, which could have important implications for assessing participants' true habitual stepping activity levels., Competing Interests: Declaration of Competing Interest All authors declare no conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose: It is unclear if high-intensity interval training (HIIT) elicits superior improvements in brachial artery (BA) flow-mediated dilation (FMD) responses (i.e., endothelial-dependent vasodilation) than moderate-intensity continuous training (MICT) or resistance training (RT) in otherwise healthy older adults. Whether HIIT enhances lower-limb FMD responses and/or augments low flow-mediated constriction (L-FMC) (endothelial-dependent vasoconstriction) responses more than MICT or RT is also unknown. We tested the hypothesis that HIIT would improve BA and popliteal artery (POP) FMD and L-FMC responses more than MICT or RT in healthy older adults., Methods: Thirty-eight older adults (age, 67 ± 6 yr) performed 6 wk of either HIIT (2 × 20 min bouts alternating between 15-s intervals at 100% of peak power output [PPO] and passive recovery [0% PPO]; n = 12), MICT (34 min at 60% PPO; n = 12), or whole-body RT (8 exercises, 2 × 10 repetitions; n = 14). The L-FMC and FMD were measured before and after training using high-resolution ultrasound and quantified as the percent change in baseline diameter during distal cuff occlusion and after cuff release, respectively., Results: Resting BA blood flow and vascular conductance (both, P < 0.003) were greater after HIIT only. The HIIT and MICT similarly increased BA-FMD (pre-post: both, P < 0.001), but only HIIT improved BA L-FMC (P < 0.001). Both HIIT and MICT similarly enhanced POP FMD and L-FMC responses (both, P < 0.045). Resistance training did not impact FMD or L-FMC responses in either artery (all, P > 0.20)., Conclusions: HIIT and MICT, but not RT, similarly improved lower-limb vasodilator and vasoconstrictor endothelial function in older adults. Although HIIT and MICT groups enhanced BA vasodilator function, only HIIT improved resting conductance and endothelial sensitivity to low-flow in the BA. In the short-term, HIIT may be most effective at improving peripheral vascular endothelial function in older adults.

Purpose: Cardiovagal baroreflex sensitivity (cvBRS) reflects the efficiency of modulating heart rate in response to changes in systolic blood pressure. International guidelines recommend that older adults achieve at least 150 min of moderate-vigorous physical activity per week. We tested the hypothesis that older adults who achieve these guidelines will exhibit greater cardiovagal baroreflex sensitivity versus those who do not., Methods: A cross-sectional comparison of older adults who did (active, 66 ± 5 years, 251 ± 79 min/week; n = 19) and who did not (inactive, 68 ± 7 years, 89 ± 32 min/week; n = 17) meet the activity guidelines. Beat-by-beat R-R intervals (electrocardiography) and systolic blood pressure (finger photoplethysmography) were recorded. Spontaneous cardiovagal baroreflex sensitivity was assessed using the sequence technique from 10 min of resting supine data. Cardiovagal baroreflex function was also measured during early phase II and phase IV of the Valsalva maneuver. Peak oxygen uptake was determined during maximal cycle ergometry. Moderate-vigorous intensity physical activity and time spent sedentary were assessed over 5 days using the PiezoRx and activPAL, respectively., Results: Groups had similar peak oxygen uptake (active 25 ± 9 vs. inactive 22 ± 6 ml/kg/min; p = 0.218) and sedentary time (active 529 ± 60 vs. inactive 568 ± 88 min/day; p = 0.130). However, the active group had greater (all, p < 0.019) cvBRS at rest (9.1 ± 2.7 vs. 5.0 ± 1.9 ms/mmHg), during phase II (8.2 ± 3.8 vs. 5.4 ± 2.1 ms/mmHg), and during phase IV (9.9 ± 3.8 vs. 5.6 ± 1.6 ms/mmHg). In the pooled sample, moderate-vigorous physical activity was positively correlated (all, p < 0.015) with spontaneous (R = 0.427), phase II (R = 0.447), and phase IV cvBRS (R = 0.629)., Conclusions: Independent of aerobic fitness and sedentary time, meeting activity guidelines was associated with superior cardiovagal baroreflex sensitivity at rest and during the Valsalva maneuver in older adults.

An acute bout of prolonged sitting (PS) impairs the popliteal artery flow-mediated dilation (FMD) response. Despite equivocal reductions in mean shear rate, young women demonstrate an attenuated decline in popliteal FMD versus young men. However, it is uncertain whether popliteal endothelial-dependent vasoconstrictor responses [low-flow-mediated constriction (L-FMC)] are similarly affected by PS and/or whether sex differences exist. We tested the hypothesis that women would have attenuated reductions in both popliteal FMD and L-FMC responses following an acute bout of PS. Popliteal FMD and L-FMC responses were assessed via duplex ultrasonography before and after a 3-h bout of PS. These responses were then compared between 10 men (24 ± 2 yr) and 10 women (23 ± 2 yr) with similar ( P > 0.13) levels of objectively measured habitual physical activity (via PiezoRx) and sedentary time (via activPAL). At baseline, men and women exhibited similar ( P > 0.46) popliteal FMD (4.8 ± 1.2 vs. 4.5 ± 0.6%) and L-FMC (-1.7 ± 1.0 vs. -1.9 ± 0.9%) responses. Both sexes experienced identical (group: P > 0.76; time: P < 0.001) PS-induced impairments in popliteal FMD (-2.8 ± 1.4 vs. -2.6 ± 0.9%) and L-FMC (1.3 ± 0.7% vs. 1.4 ± 0.7%). In young adults, sex did not influence the negative PS-induced FMD, L-FMC, or microvascular responses in the lower limb. As such, our findings suggest that young men and women are similarly susceptible to the acute negative vascular effects of PS. Future studies should extend these findings to older, less physically active adults and/or patients with vascular disease. NEW & NOTEWORTHY We compared changes in popliteal artery endothelial function to a single 3-h bout of sitting between young men and women. Both groups exhibited similar endothelial-dependent vasodilation (i.e., flow-mediated dilation) and endothelial-dependent vasoconstrictor responses (i.e., low-flow-mediated constriction) at baseline and equivocal impairments in these measures of endothelial function following prolonged sitting. These findings demonstrate that acute impairments in conduit artery endothelial health associated with uninterrupted sitting are not influenced by sex in young, healthy adults.

We previously observed that brachial artery (BA) low-flow-mediated constriction (L-FMC) is inversely related to aerobic fitness (i.e., V̇o 2peak ) in older adults (OA). However, it is unclear if an L-FMC response is elicited in the popliteal artery (POP) or if a similar inverse relationship with aerobic fitness exists. Considering that the POP experiences larger shear stress fluctuations during sedentary behaviors and traditional lower limb modes of aerobic exercise, we tested the hypotheses that 1 ) heterogeneous L-FMC responses exist between the BA versus POP of OA, and 2 ) that aerobic fitness will be inversely related to POP L-FMC. L-FMC was assessed in 47 healthy OA (30 women, 67 ± 5 yr) using duplex ultrasonography and quantified as the percent decrease in diameter (from baseline) during the last 30 s of a 5-min distal cuff occlusion period. When allometrically scaled to baseline diameter, the BA exhibited a greater L-FMC response than the POP (-1.3 ± 1.6 vs. -0.4 ± 1.6%; P = 0.03). Furthermore, L-FMC responses in the BA and POP were not correlated ( r  = 0.22; P = 0.14). V̇o 2peak was strongly correlated to POP L-FMC ( r  = -0.73; P < 0.001). The heterogeneous BA versus POP L-FMC data indicate that upper limb L-FMC responses do not represent a systemic measure of endothelial-dependent vasoconstrictor capacity in OA. The strong association between V̇o 2peak and POP L-FMC suggests that localized shear stress patterns, perhaps induced by lower limb dominant modes of aerobic exercise, may result in greater vasoconstrictor responsiveness in healthy OA. NEW & NOTEWORTHY We compared low-flow-mediated constriction responses between the brachial and popliteal arteries of healthy older adults. Vasoconstrictor responses were not correlated between arteries. A strong relationship between aerobic fitness and low-flow-mediated vasoconstriction was observed in the popliteal artery. These findings suggest that brachial vasoconstrictor responsiveness is not reflective of the popliteal artery, which is exposed to larger shear stress fluctuations during bouts of sedentary behavior and traditional lower limb modes of exercise.

Objective: Traditional vital sign thresholds reflect an increased risk of mortality, which may occur hours, days, or weeks following illness/injury, limiting immediate clinical significance to guide rescue therapy to avoid arrest. Our objective is to explore vital sign patterns prior to arrest due to shock., Design: This retrospective observational analysis utilized physiological data from adult helicopter patients suffering provider-witnessed arrest. Pre-arrest values for systolic blood pressure (SBP), mean arterial pressure (MAP), heart rate (HR), shock index, and end-tidal carbon dioxide (EtCO2) were modeled against time using polynomial linear regression. The "terminal inflection point" beyond which arrest was imminent was identified where slope equals 1.0 (shock index) or -1.0 (SBP, MAP, HR, EtCO2) and was then compared to initial values., Setting: Air ambulance services., Patients: 70 helicopter patients over age fourteen suffering cardiac arrest., Results: SBP and MAP demonstrated a gentle decline followed by acceleration beyond the inflection point (SBP 80.7 mmHg, MAP 61.9 mmHg). HR demonstrated an increase followed by a terminal drop, but inflection point values fell within normal range. Shock index increased gradually from a mean of 0.9 to the inflection point of 1.1. Initial EtCO2 values declined gradually from normal (34.4 mmHg) to the inflection point (24.7 mmHg), then dropped precipitously into arrest. All inflection points occurred 2-5 min prior to arrest., Conclusions: Vital sign patterns were defined for SBP, MAP, HR, shock index, and EtCO2 with clear inflection points identified 2-5 min prior to arrest. These patterns may help guide therapy to reverse deterioration and prevent arrest., (Published by Elsevier B.V.)

Purpose: Aerobic fitness is directly related to favorable vasodilatory (i.e., flow-mediated dilation; FMD) and vasoconstrictor functions (i.e., low-flow-mediated constriction; L-FMC) in young adults. Furthermore, aerobically fit older adults (OA) have larger FMD responses than their less fit peers. However, the relationship between aerobic fitness and vasoconstrictor responsiveness is unknown in OA. We hypothesized that OA who are more aerobically fit will exhibit a greater L-FMC response than their less fit counterparts., Methods: Forty-seven healthy OA (67 ± 5 years) were divided into less (LF; n = 27) and more aerobically fit (MF; n = 20) groups based on peak oxygen consumption (VO 2 peak). VO 2 peak was determined from an incremental maximal cycle ergometer test via indirect calorimetry. FMD and L-FMC were assessed in the brachial artery via high-resolution duplex ultrasonography., Results: VO 2 peak (18.3 ± 3.2 versus 29.1 ± 5.8 ml/kg/min; P < 0.001) and L-FMC were both greatest in the MF versus LF groups (-1.2 ± 0.9 vs. - 0.5 ± 0.6%; P = 0.01). Furthermore, the MF group had an enhanced FMD response (5.6 ± 1.5 versus 3.9 ± 1.2%; P < 0.001). In the pooled sample, there was a negative correlation (r = - 0.52; P < 0.001) between VO 2 peak (22.9 ± 7.0 ml/kg/min) and L-FMC (-0.8 ± 0.8%)., Conclusions: In an older population, greater aerobic fitness was associated with a more favorable vasoconstrictor response to low-flow conditions. Interventional or longitudinal aerobic exercise training studies are warranted in this population to determine the impact of training-induced increases in VO 2 peak on L-FMC.

Background: Antitachycardia pacing (ATP) can reduce implantable cardioverter-defibrillator shocks, but its use in children and patients with congenital heart disease (CHD) is not well described., Objective: To review the efficacy of ATP in children and patients with CHD., Methods: We reviewed implantable cardioverter-defibrillator therapies in children and patients with CHD (aged 2-52 years) at our institution. Appropriate therapies were defined as those delivered for true ventricular tachycardia (VT) or ventricular fibrillation; other therapies were defined as inappropriate., Results: During a median follow-up of 4 years (range 0.5-15 years), 17 of 79 patients (23%) received appropriate therapy and 14 received ATP for 100 episodes of VT. ATP was highly successful (88%) in terminating VT, and only 10 of 100 episodes required a shock. Shocks were effective in terminating VT/ventricular fibrillation in 21 of 24 episodes (87%). The outcomes of appropriate therapy were similar for ATP and shocks (success 88% vs 87%, failure 9% vs 8%, acceleration 3% vs 4% for ATP and shocks, respectively). Thirty-one patients (39%) received inappropriate therapy. Inappropriate ATP (without subsequent shocks) was delivered to 11 patients for the following: sinus tachycardia (19 episodes in 7 patients) with slowing of the rate after ATP, T-wave oversensing (2 episodes in 2 patients) with loss of oversensing after ATP, and reentrant supraventricular tachycardia (14 episodes in 2 patients) terminated with ventricular ATP., Conclusions: ATP is highly efficacious for VT in children and patients with CHD. In addition to reducing appropriate shocks, inappropriate shocks due to sinus or supraventricular tachycardia can be significantly reduced with ATP., (Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Transvenous pacemaker leads in children are often placed with redundant lead length to allow for anticipated patient growth. This excess lead may rarely prolapse into the pulmonary artery and potentially interfere with valve function. We sought to determine the response to lead repositioning on pulmonary valve insufficiency., Methods: Retrospective reviews of demographics, lead type, implant duration, and radiography and echocardiography., Results: A total of 11 pediatric patients were identified with lead prolapse through the pulmonary valve, of which nine patients underwent procedures to retract and reposition the lead (age at implant 9 +/- 4 years, age at revision 13 +/- 4 years). The implant duration prior to revision was 4 +/- 3 years. Two leads required radiofrequency extraction sheaths for removal, two pulled back using a snare, while five leads were simply retracted and repositioned. Tricuspid regurgitation was none/trivial (three), mild (four), or moderate (two) and only two improved with repositioning or replacement. Pulmonary regurgitation preoperatively was mild (three), mild-moderate (two), or moderate (four) compared with trivial (three), mild (four), and moderate (two) after revision. Patients with longer-term implanted leads had less improvement in pulmonary insufficiency. Two patients had mild pulmonary stenosis from lead-related obstruction., Conclusions: Prolapse of transvenous pacing leads into the pulmonary artery can occur when excess slack is left for growth. Leads can often be repositioned, but may require extraction and replacement, particularly if chronically implanted and adherent to valve apparatus. Lead revision does not always resolve pulmonary insufficiency, potentially leaving permanent valve damage.

Atrioventricular nodal reentry tachycardia (AVNRT) is an uncommon mechanism of supraventricular tachycardia in neonates in whom orthodromic reciprocating tachycardia (ORT) predominates. We report three patients with structurally normal hearts who presented with neonatal ORT, documented by transesophageal electrophysiology studies at 2 to 3 weeks of age. At follow-up study at 8-12 months of age, no infant had inducible ORT, suggesting spontaneous regression of congenital accessory pathways; however, each had inducible tachycardia consistent with the typical form of AVNRT. The clinical significance of this finding is unknown and warrants further study.

Background: The parenteral administration of oestradiol acutely protects against exercise-induced myocardial ischaemia in women, but whether this effect is sex-specific is not known., Aims: The effects of acutely administered conjugated equine oestrogen on exercise-induced myocardial ischaemia in men with established coronary heart disease were investigated in a randomized, placebo-controlled, double-blind cross-over trial., Methods: Twenty men, aged 62 +/- 11.6 years, with reproducible exercise-induced myocardial ischaemia were treated with either intravenous conjugated equine oestrogen (25 mg) or saline prior to undergoing an exercise stress test. Primary end-points were total exercise time and time to 1 mm ST-segment depression., Results: All participants completed the protocol. Total exercise time exceeded the baseline value in 17 of the 20 men following saline, and in 17 of the 20 men following oestrogen pretreatment. Time to 1 mm ST-segment depression exceeded the baseline value in 14 of the 19 men following saline, and following oestrogen administration, exceeded baseline in 13 of the 19 men. There was no significant difference between the two treatments in either time to 1 mm ST-segment depression or total exercise time. A period effect was apparent for total exercise time (P = 0.05) but not for time to ST-segment depression., Conclusion: Acute parenteral oestrogen therapy did not increase total exercise time or time to the onset of electrocardiographic changes of ischaemia in men with chronic stable coronary artery disease. These findings contrast the favourable effects of oestrogen in women in comparable studies and indicate a sex specificity for the acute cardiovascular effects of oestrogen.

The percutaneous use of stents for the treatment of superior vena cava (SVC) syndrome is well described in the adult population. We report the successful use of intravascular stents to treat an infant with severe SVC syndrome.

Pacemakers have been implanted in pediatric patients since the late 1960s. Indications for cardiac pacing in infants and children have evolved to include controlling symptoms as well as providing a life-sustaining cardiac rhythm. Pacemakers have become smaller and able to perform more complex functions. Current pacemakers offer a variety of operation modes, which are chosen on the basis of the pacing indication or the potential hemodynamic benefit. Today's pacemakers have the ability to maintain AV synchrony, perform rate-responsive pacing, and sometimes prevent or treat tachyarrhythmias. Recent advances in pacing lead technology, such as steroid elution, have narrowed the gap between epicardial and endocardial leads in terms of chronic pacing thresholds and sensing characteristics. The choice of a permanent pacing system is determined by the patient's size, underlying arrhythmia, and the anatomic details of any structural heart disease. Selection of temporary pacing modalities depends on the urgency of initiating pacing and the anticipated duration of temporary pacing. The decision to implant a permanent pacemaker in a pediatric patient commits the patient to long-term follow-up of pacemaker function. Such follow-up is important to ensure adequate safety margins for pacing and sensing, anticipate the need for pacemaker replacement, screen for pacemaker malfunctions, and optimize programmable settings. Pediatric cardiac pacing has evolved into its own subspecialty over the past decade. As more infants with complex congenital heart disease are being successfully treated with surgical palliation and repair, the population of pediatric patients with permanent pacemakers is likely to increase.

Objective: Substantial improvements in lipoprotein-lipid profiles have previously been shown with both simvastatin and combined estrogen-progestin therapy in postmenopausal hypercholesterolemic women. Since little is known about the impact of the concomitant use of these therapies, the effects of concurrent hormone therapy and simvastatin in hypercholesterolemic postmenopausal women have been evaluated., Methods: Twenty-three postmenopausal women with fasting serum total cholesterol levels greater than 250 mg/dl received, in a randomized cross-over design, simvastatin (10 mg daily) for 8 weeks or postmenopausal hormone therapy (up to 1.25 mg of conjugated equine estrogens plus 5 mg of medroxyprogesterone acetate daily) for 8 weeks, with an 8-week wash-out interval between the two treatment periods. In a third, non-randomized treatment period after a second wash-out interval, each woman received a combination of simvastatin and postmenopausal hormone therapy in the same dosage regimens as above. Fasting blood was sampled monthly from baseline to measure total cholesterol, high- and low-density lipoprotein (HDL and LDL) cholesterol, triglycerides and lipoprotein(a)., Results: For total cholesterol, the mean decreases with hormone therapy, simvastatin and combination therapy were 12% (95% confidence interval 6-17%), 26% (20-31%) and 28% (24-31%), respectively, and for LDL cholesterol 21% (14-27%), 37% (30-44%) and 46% (41-51%), respectively. Simvastatin was more effective than hormone therapy (p < 0.001), while the effect of the combined therapy was even greater (total cholesterol, p = 0.012; LDL cholesterol, p < 0.001). The level of HDL cholesterol increased similarly with each treatment: 4% (-3-11%), 6% (2-10%) and 7% (2-13%), respectively. Triglyceride levels increased with hormone therapy and decreased with simvastatin (p < 0.001), while there was little change with the combination (effect of combined therapy vs. simvastatin, p = 0.002; vs. hormone therapy, p < 0.001). Both hormone therapy and combined therapy reduced lipoprotein(a) similarly (-23% and -14%, respectively, p = 0.078). Simvastatin had no effect on lipoprotein(a) levels., Conclusion: For postmenopausal women with hypercholesterolemia, use of a statin in combination with continuous combined oral estrogen and progestin therapy can result in a more cardioprotective lipoprotein-lipid profile than that achieved with either therapy used alone.

An infant with hypoplastic left heart syndrome, excessive pulmonary blood flow, and tachypnea was placed on subatmospheric oxygen (supplemental nitrogen) to increase pulmonary vascular resistance and decrease pulmonary blood flow. His cardiorespiratory status stabilized without mechanical ventilation, but 2 weeks later he developed spontaneous subcutaneous emphysema. The emphysema worsened over approximately 1 month. During this time his left-to-right shunt gradually decreased, and he was weaned to room air. Even without the use of supplemental oxygen the emphysema resolved without complication, and the patient underwent successful orthotopic heart transplantation at 65 days of age.

Chaotic atrial tachycardia was observed in 7 infants without underlying structural heart disease. Clinical presentation and approach to management are discussed, with particular attention to the use of propafenone for this uncommon pediatric arrhythmia.

Objectives: To illustrate the use of transoesophageal echocardiography in the detection of the morphological features of central pulmonary artery thromboemboli and their clinical implications., Design: Review of five cases of central pulmonary artery thromboemboli detected by transoesophageal echocardiography., Setting: University teaching hospital., Patients: Five patients (three men and two women) admitted under general medical units., Results: Central pulmonary artery thromboemboli were detected by the use of transoesophageal echocardiography in all the patients presented. Presentations were acute, subacute, or chronic. The morphological features of the thromboemboli on transoesophageal echocardiography were used to correlate with the time course of the illness, and to guide treatment. Two patients received thrombolytic treatment, one patient was treated with anticoagulation alone, and two patients had inferior vena caval filters implanted., Conclusions: Transoesophageal echocardiography is an alternative diagnostic tool in the detection of central pulmonary artery thromboemboli. Morphological features of central pulmonary thromboemboli on echocardiography can provide useful information that may help to guide treatment.

Objectives: This study evaluated the pacing and sensing characteristics of a new porous-tipped steroid-eluting epicardial lead in a group of pediatric patients., Background: Pacing in children may be complicated by small patient size, patient growth and the prevalence of structural congenital heart disease in children requiring pacing. Epicardial pacing has been associated with a high incidence of problems with sensing and capture, prompting the use of transvenous endocardial pacing when possible. In some children, epicardial pacing may still be desirable because of small patient size, potential for caval obstruction, previous cardiac surgery limiting transvenous access to the heart, or the need to repair congenital heart disease at the time of pacemaker insertion., Methods: Twelve patients aged 3 weeks to 18 years underwent placement of 23 epicardial pacing leads (8 atrial, 15 ventricular). Pulse width thresholds, sensing thresholds and lead impedance were measured weekly for 6 weeks, then at 3, 6, 12 and 18 months after pacemaker implantation. The median duration of follow-up was 12 months., Results: Ventricular pulse width thresholds did not change over time, whereas atrial pulse width thresholds improved significantly. At 6 months, the mean pulse width threshold at 2.5 V for the atrial and ventricular leads was 0.10 +/- 0.03 and 0.19 +/- 0.09 ms, respectively. The thresholds were slightly lower at 12 and 18 months. At the most recent follow-up, all atrial leads sensed appropriately at 2.5 mV and all ventricular leads at 5 mV., Conclusions: These encouraging early results suggest that steroid-eluting epicardial pacing leads may be an attractive option for children needing epicardial pacing. Their excellent pacing and sensing characteristics may allow reliable dual-chamber pacing in infants who are too small for transvenous pacing.

In 1978 Choussat and Fontan established ten criteria, which should be fulfilled to achieve a successful outcome in Fontan operation. Recent data suggest that while some of these ten criteria need not be necessarrily be fulfilled, new criteria should be added. These include 1. good diastolic function, 2. normal or only slightly increased ventricular mass, and 3. absence of systemic outflow obstruction. In addition the morphology of the single ventricle may be important as long-term results in patients with single ventricle of right ventricular morphology may be worse than results in patients with single ventricle of left ventricular morphology. Ventricular size and pump function can be assessed by cardiac catherization, echocardiography or magnetic resonance imaging. Estimation of ejection fraction under stress by nuclear angiography may be indicated. Diastolic function can be examined using Doppler echocardiography or nuclear angiography. Myocardial mass may be assessed by echocardiography or magnetic resonance imaging. Normal reference values for different parameters of systolic and diastolic function are listed in the enclosed tables. Patients scheduled for a Fontan operation should have an ejection fraction less than 50%. Patients with borderline ejection fraction should be examined by echocardiography to determine the end-systolic wall stress, a parameter of ventricular contraction, which is independent of pre- and afterload. As afterload may decrease after a Fontan operation some patients with reduced ejection fraction but normal end-systolic wall stress may still be suitable candidates for Fontan operation.(ABSTRACT TRUNCATED AT 250 WORDS)

Physical performance and haemodynamic parameters at rest and with exercise were compared in a prospective, cross-over fashion in 12 anaemic haemodialysis patients (Hb 6.4 +/- 0.5, mean +/- SEM) at two levels of haemoglobin (Hb 9 and 12 g/dl) before and after long-term treatment with recombinant human erythropoietin (rHuEpo). Patients were divided into two groups and measurements made prior to treatment, upon reaching, and after 4 months at the first target Hb (9 g/dl group A, 12 g/dl group B), and after 4 months at the alternative target Hb. Tests included an exercise radionuclide ventriculogram, Doppler echocardiogram, and respiratory function exercise test. Compared to pretreatment, there was a significant reduction in resting pulse rate (P < 0.001), and in pulse rate (P < 0.001) and arterial lactate (P < 0.01) concentrations at specified levels of exercise. Work capacity improved 60% (P < 0.001), and left ventricular mass fell by 26% (P < 0.001). Although cardiac output (CO) during and after exercise was reduced (P < 0.05), resting CO, cardiac index, stroke volume and ejection fraction (rest and exercise) were not significantly altered. There appeared little benefit in having the higher target Hb: no significant difference could be found between target levels for almost any measure. In addition, despite marked improvement from pretreatment levels, performance parameters were still below those of non-uraemic age-matched controls. These results demonstrate the beneficial but incomplete effect of rHuEpo on resting and exercise-related factors, and suggest that most improvement is achieved with modest increments in haemoglobin.

With the current trend to performing surgical valvotomy for infantile aortic stenosis without cardiac catheterization, there is a need to develop echocardiographic criteria for adequacy of left ventricular size. The echocardiograms and catheterization data of all 25 infants less than 3 months of age undergoing aortic valvotomy for isolated aortic valve stenosis from September 1980 through July 1990 were reviewed. Significant differences (p less than 0.05) between the survivors and nonsurvivors were noted for age at operation (30 +/- 28 vs. 3 +/- 1.5 days), mitral valve diameter (10.1 +/- 1.7 vs. 7.7 +/- 1.5 mm), left ventricular end-diastolic dimension (18.4 +/- 6.4 vs. 11.4 +/- 3 mm), left atrial dimensions (15.3 +/- 3.8 vs. 10 +/- 2.4 mm), left ventricular cross-sectional area on the parasternal long-axis echocardiogram (4 +/- 1.9 vs. 2 +/- 1.9 cm2) and angiographically determined left ventricular end-diastolic volume (43 +/- 23 vs. 11 +/- 5 ml/m2). There was no difference with respect to patient weight, body surface area, aortic root dimension or left ventricular ejection fraction. Left ventricular cross-sectional area less than 2 cm2 as measured on the parasternal long-axis echocardiogram was found in 5 of 7 nonsurvivors and 0 of 12 survivors, making this a risk factor for perioperative death (p less than 0.05). Left ventricular end-diastolic dimension less than 13 mm was found in 5 of 6 nonsurvivors and 2 of 17 survivors, making this another risk factor for early mortality (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Pulsatile three-dimensional retinal arteriolar tortuosity has been previously reported in about 50% of patients with coarctation of the aorta. In a contemporary series of 20 patients with coarctation of the aorta, none exhibited this characteristic retinal vascular abnormality. Our findings suggest that the retinal vascular abnormalities in patients with coarctation of the aorta represent secondary hemodynamic changes. The prevalence of these abnormalities may be decreasing because of earlier surgical repair of coarctation.

We undertook a study of premature infants with cyanotic congenital heart disease to determine whether these infants develop retinopathy of prematurity despite a persistent hypoxemic state. Using the computerized registry of the neonatal intensive care unit of Vanderbilt University Medical Center, Nashville, Tenn, we identified six premature infants (less than 37 weeks' gestational age, with birth weights of 1100 to 2050 g) with cyanotic congenital heart disease who survived the neonatal period and underwent ophthalmologic evaluation. Review of their charts revealed that three of six infants developed retinopathy of prematurity (two had grade 1 and one had grade 3 disease), but none required treatment. Our data support the findings of other investigators that elevated arterial oxygen tension is not the sole factor leading to the development of retinopathy of prematurity. Premature infants with cyanotic congenital heart disease can develop retinopathy of prematurity despite persistent hypoxemia. Cyanotic premature infants should be screened for retinopathy of prematurity with the same thoroughness as other premature infants.

SummaryWe report analysis of surgical results in 110 consecutive infants and children who underwent atrial repair of simple complete transposition using the Senning operation between February 1978 and May 1990. Mean age at operation was 5.4 months ± 6.1 (range 1 week to 4 years); 75 were less than 6 months old. There were 72 males and 38 females. Operative mortality rate was 5.5%, with one late death. Average follow-up is 48.1 months with 44 followed greater than 3 years, and 27 greater than 5 years. Postoperative cardiac catheterization was performed in 48 patients. Right ventricular ejection fraction averaged 0.52 ± 0.08 and was normal in 28 patients. Response of right ventricular ejection fraction to afrerload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 12 patients. Mild obstruction of the superior caval vein was noted in 4 patients. Baffle leak requiring reoperation occurred in one patient. Fifty-seven of 90 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiologic studies were performed in 34 patients, and Holter monitoring in 25. Significant arrhythmia occurred in 26 patients: 6 patients required pacemakers for slow junctional rhythm or complete heart block; 20 additional patients have a junctional rhythm. Six patients have delayed sinus nodal recovery time. At last follow-up, 88 children (98%) are in New York Heart Association functional Class I, and 2 (2%) are in Class II. The Senning operation for compete transposition can be accomplished with a low operative and late mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function may limit their long-term functional status.

Nuclear magnetic resonance (NMR) imaging has shown potential in the detection and characterization of acute myocardial infarction in humans. This study was performed to evaluate the capability of NMR imaging in the measurement of infarct size in patients with recent myocardial infarction. Electrocardiographic (ECG)-gated spin-echo NMR imaging was performed in 26 patients a mean of 9 +/- 3 days (range 5 to 20) after infarction. The imaging technique used provided single-slice, spin-echo (time to echo [TE] = 60 ms) images of the left ventricle in its true short axis, allowing direct correlation of NMR infarct location and size with the region of severe hypokinesia on left ventriculography. In all 20 patients with complete NMR studies, infarct location was correctly identified by using specific, objective criteria. The correlation between the mean infarct volume (29 +/- 11 ml) and the quantitated left ventricular hypokinetic segment (7.5 +/- 4.0 cm) was good (r = 0.84, p = 0.0002), suggesting that NMR imaging of the heart may have a role in the noninvasive assessment of myocardial infarct size in patients.

Indium-111 monoclonal antimyosin Fab scintigraphy was used to detect myocardial necrosis in 52 of 54 patients (96.3%) with acute myocardial infarction. Infarcts were visualized when coronary arteries were persistently occluded (n = 10), became patent after thrombolysis (n = 33), or became patent after spontaneous reperfusion (n = 7). Posteroinferolateral visualizations were obtained in two patients with clinical and enzymatic evidence of infarction but normal electrocardiograms. Of the two patients in whom no infarcts were visualized, one had an anterior myocardial infarct. This patient underwent successful thrombolytic therapy, with attendant minimization of creatine kinase release. The other patient had a small, nonreperfused inferior myocardial infarct. Five patients with a history of remote infarction and acute necrosis showed antimyosin uptake only in regions concordant with the acute episodes of infarction, and radiolabeled antimyosin Fab localized in neither old infarcts nor normal, noninfarcted myocardium. Antimyosin Fab scintigraphy, thus, appears to be a highly specific means of delineating necrotic myocardium, at least in this limited and selected group of patients.

The pharmacokinetics, thrombolytic profile and effects on hemostasis of graded intravenous doses of recombinant human tissue-type plasminogen activator (rt-PA) were studied in 45 patients with acute myocardial infarction. Infusion of rt-PA at a rate of 4 to 8.3 micrograms/kg/min resulted in plateau levels of the drug in plasma of 0.52 to 1.4 micrograms/ml. A linear relation between infusion rate and plasma rt-PA concentration was observed, although plasma drug levels varied substantially among subjects who received infusions at the same rate. The ratio between plateau levels of rt-PA in plasma and infusion rate was inversely related to initial distribution volume (7.3 +/- 2.9 liters, n = 21). The decline in plasma concentration of rt-PA, x(t), as a function of time after cessation of the infusion, was described adequately by the biexponential equation: x(t) = 0.71exp(-0.13t) + 0.29exp(-0.015t). The initial and terminal half-lives of rt-PA in the blood were 5.3 +/- 1.7 and 46.2 +/- 14 minutes, respectively. The efficacy of rt-PA for coronary thrombolysis was dose-dependent. With 4 micrograms/kg/min of rt-PA for 90 minutes, no reperfusion was achieved, whereas infusion rates of 5 micrograms/kg/min or more for 90 minutes accomplished reperfusion in more than 80% of the patients. However, the frequency of occurrence of residual intraluminal thrombus was significantly lower with an infusion rate of 7 micrograms/kg/min for 90 minutes.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical characteristics and angiographic ventricular volume data were obtained in 25 infants aged 1 to 66 days who presented with coarctation of the aorta, ventricular septal defect and congestive heart failure to determine if left ventricular volume loading was present and if there were hemodynamic or volumetric variables that were predictive of operative mortality in this group. Pulmonary to systemic flow ratio averaged 2.8 +/- 0.8 and right ventricular/left ventricular peak pressure ratio was 0.96 +/- 0.12. Left ventricular end-diastolic volume averaged 116 +/- 49% of normal and was less than the investigators' lower limit of normal in 5 (20%) of 25 patients. In contrast, right ventricular end-diastolic volume, measured in eight patients, averaged 173 +/- 47% of normal and was greater than the investigators' upper limit of normal in seven (88%) of eight. Left ventricular ejection fraction averaged 0.47 +/- 0.17 and was below normal (less than 0.55) in 14 (58%) of 24 patients. Preoperative volume and ejection fraction data did not differ in infants with coarctation plus ventricular septal defect and a similar group of 19 infants with isolated coarctation. Abnormal left ventricular operative volume distensibility was inferred by normal or decreased left ventricular end-diastolic volume and increased left ventricular end-diastolic pressure (greater than 12 mm Hg) in 12 (55%) of 24 patients. Early plus late mortality was related to left ventricular size: 3 of 5 patients with a small left ventricular end-diastolic volume died, compared with only 4 of 20 with a normal or increased volume (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Twenty-four patients with unstable angina pectoris, defined as chest pain at rest with transient ST segment deviation of at least 1 mm, were randomly assigned to blinded treatment with either placebo or intravenous recombinant human tissue-type plasminogen activator (rt-PA). Before randomization, all patients were treated with oral beta-blockers, calcium antagonists, nitrates, and continuous intravenous heparin for a monitoring period of 12 to 28 hr. After this monitoring period the 24 patients were randomly assigned to receive either placebo or 1.75 mg/kg intravenous rt-PA given over 12 hr at a rate of 0.75 mg/kg over 1 hr, 0.5 mg/kg over 4 hr, and 0.5 mg/kg over 7 hr. One patient, assigned to receive placebo, developed acute myocardial infarction after randomization but before receiving the study drug. Ischemic events were recorded during a hospital follow-up period of at least 4 days unless a further intervention was indicated or the coronary angiogram was normal. The follow-up period was 7 +/- 5 days (mean +/- SD) after the placebo infusion and 8 +/- 4 days after the infusion of rt-PA. Unstable angina pectoris persisted after the completion of the infusion in six of 11 patients receiving placebo and only one of 12 patients receiving rt-PA (p less than .03). Coronary angiography, performed 38 +/- 19 hr after the infusion, demonstrated subocclusive thrombus in eight of 11 patients receiving placebo but in none of 11 patients treated with rt-PA (p less than .002). One patient on rt-PA refused coronary angiography.(ABSTRACT TRUNCATED AT 250 WORDS)