The Evolution of Longitudinal Strain Across Various Univentricular Morphologies Prior to Superior Cavopulmonary Anastomosis.

BACKGROUND: Infants with single-ventricle physiology have increased morbidity, mortality, and ventricular dysfunction in the pre-superior cavopulmonary anastomosis (pre-SCPA) period. Echocardiography-derived longitudinal strain (LS) is emerging as a reliable marker of single-ventricle performance. We aim to assess evolution of LS during the pre-SCPA period across univentricular morphologies and explore associations of LS with modifiable and nonmodifiable factors. METHODS: Ninety-four term infants (36 females) with univentricular physiology who were discharged home prior to stage 2 palliation were serially analyzed for LS (single apical view) and other echo measures at initial hospital discharge and the last pre-SCPA encounter. Ventricular myocardium was tracked for strain along the septum and respective lateral wall for single right ventricular (RV) and left ventricular (LV) groups, and along both right and left lateral walls in functionally univentricular hearts with biventricular (BiV) morphology. Clinical data were obtained from the medical record. RESULTS: Longitudinal strain improved in the total cohort during the pre-SCPA period (16.48% ± 3.31% to 17.57% ± 3.81%, P = .003). Longitudinal strain improved between encounters in the single LV (P = .04) and BiV groups (P = .02). However, LS failed to improve in the RV group (P = .7) with lower LS at both visits compared with the other groups. The RV group, mostly composed of hypoplastic left heart syndrome patients-87% of cases-had a higher incidence of arrhythmias (57%) and unplanned reinterventions (60%), most of which were arch reinterventions. A subanalysis based on arch reintervention showed that LS improves between encounters in the single LV group needing arch reintervention (P = .05) compared with the single RV group needing arch reintervention (P = .89). Lower LS was independently associated with unplanned reinterventions at both encounters (P = .008 and .02). CONCLUSIONS: Single-ventricle LS evolves differently across ventricular morphologies during the pre-SCPA period and is related to the need for unplanned cardiac reinterventions. Lower LS is noted in the single RV group, who mostly have hypoplastic left heart syndrome.

The association between cardiovascular health and cognition in adults with Down syndrome.

INTRODUCTION: Evidence in the general population suggests that predictors of cardiovascular health such as moderate to vigorous physical activity (MVPA), cardiorespiratory fitness, and systolic blood pressure are associated with cognitive function. Studies supporting these associations in adults with Down syndrome (DS) are limited. The purpose of this study was to examine the associations between systolic blood pressure, cardiorespiratory fitness, and MVPA on cognition in adults with DS. METHODS: This is a cross-sectional analysis using baseline data from a trial in adults with DS. Participants attended a laboratory visit where resting blood pressure, cardiorespiratory fitness (VO2 Peak), and cognitive function (CANTAB® DS Battery) were obtained. The cognitive battery included tests measuring multitasking, episodic memory, and reaction time. Physical activity (accelerometer) was collected over the week following the laboratory visit. Pearson correlations and linear regressions were used to measure the impact of systolic blood pressure, cardiorespiratory fitness, and MVPA on cognitive outcomes. RESULTS: Complete data was available for 72 adults with DS (26.8 ± 9.3 years of age, 57% female). At baseline, VO2 Peak (21.1 ± 4.2 ml/kg/min) and MVPA were low (14.4 ± 14.4 min/day), and systolic blood pressure was 118.3 ± 13.3 mmHg. VO2 Peak was correlated with simple movement time (rho = - 0.28, p = 0.03) but was not significant using a linear regression controlling for age and sex. Systolic blood pressure was significantly associated with episodic memory (first attempt memory score: ß = - 0.11, p = 0.002; total errors: ß = 0.58, p = 0.001) and reaction time (five-choice movement time: ß = 4.11, p = 0.03; simple movement time: ß = 6.14, p = 0.005) using age- and sex-adjusted linear regressions. No associations were observed between MVPA and multitasking, episodic memory, or reaction time. CONCLUSION: Predictors of cardiovascular health, including cardiorespiratory fitness and systolic blood pressure, were associated with some aspects of cognition in adults with DS. While future research should examine the role of improved cardiovascular health on delaying decreases in cognitive function and dementia in adults with DS, we recommend that health care providers convey the importance of exercise and cardiovascular health to their patients with DS. TRIAL REGISTRATION: NCT04048759, registered on August 7, 2019.

Cardiac Structure and Function in Adults with Down Syndrome.

Various factors may alter the risk for cardiovascular disease in adults with Down syndrome (Ds), yet few studies have examined differences in cardiac physiology in this population. Previous research suggested lower systolic and diastolic function, but inconsistent methodologies and younger samples warrant research in adults with Ds. Our aim is to compare the cardiac structure and function of adults with Ds to age- and sex-matched adults without Ds. Echocardiography was used to assess systolic function, diastolic function, and cardiac structure in n = 19 adults (Ds n = 9, control n = 10). Regarding cardiac structure, adults with Ds had increased left ventricular posterior wall thickness at end-systole compared to adults without Ds (p = 0.007). Regarding systolic and diastolic function, adults with Ds were found to have lower septal peak systolic annular velocity (S') (p = 0.026), lower lateral and septal mitral annular early diastolic velocity (E') (p = 0.007 and p = 0.025, respectively), lower lateral peak mitral annular late diastolic velocity (A') (p = 0.027), and higher lateral and septal mitral annular early systolic velocity to diastolic velocity ratios (E/e') (p = 0.001 and p = 0.001, respectively). Differences in both cardiac structure and function were found when comparing adults with Ds to matched adults without Ds. Most of the differences were indicative of worse diastolic function.

The promotion of physical activity for the prevention of Alzheimer's disease in adults with Down Syndrome: Rationale and design for a 12 Month randomized trial.

Nearly all individuals with Down Syndrome (DS) display pathology associated with Alzheimer's disease (AD) beginning as early as age 30. Previous research in typically developed adults suggests that increased moderate-to-vigorous physical activity (MVPA) may improve cognitive function and protect against age-related structural and functional changes in the brain; however, the potential impact of increased MVPA on the development of AD in adults with DS has not been evaluated. Despite the potential positive impact of MVPA on cognition and AD risk, participation in MVPA among young adults with DS is low. The limited research evaluating strategies for increasing MVPA in adults with DS has been unsuccessful in increasing MVPA. Results from our preliminary investigation where we remotely delivered real-time MVPA, led by a trained health educator, to groups of adults with DS in their homes via video conferencing on a tablet computer demonstrated high attendance, increased MVPA during group sessions, and improvements in cognitive function. However, the sustainability, impact on total daily MVPA, optimal session frequency, and potential impacts on cognitive function and brain health of remotely delivered group MVPA sessions in adults with DS are unknown. Therefore, we will conduct a trial in 80 non-demented adults with DS to determine the feasibility and potential efficacy of remotely delivered group MVPA sessions to increase daily MVPA, relative to a usual care control. Secondarily we will assess the impact of MVPA on cardiovascular fitness, quality of life, cognitive function and brain parameters related to AD. NCT REGISTRATION: NCT04048759.

Remote Exercise for Adults with Down Syndrome.

PURPOSE: Adults with Down syndrome are less physically active than their typically developed peers. The purpose of this study was to assess the feasibility of delivering moderate-to- vigorous exercise sessions, led by a trained health educator using real-time video conferencing, to groups of young adults with Down syndrome in their homes. METHODS: Participants were randomized to 30-minute group exercise sessions either 1 or 2 times a week delivered on an iPad mini tablet computer using the Zoom video conferencing application, and were asked to attend individual support/education sessions once a week using FaceTime® on the iPad, for 12 weeks. Minutes of MVPA during all group sessions were assessed using a Fitbit Charge HR activity/heart rate monitor. Participants were also asked to complete weekly homework assignments involving MVPA. RESULTS: Twenty-seven participants (n = 14,1 session·wk-1, n =13, 2 sessions·wk-1), mean age 27.9 ± 7.1 yrs., ~ 41% female, enrolled in and completed the 12-wk. intervention. Attendance at group exercise and individual support/education sessions did not differ significantly between those randomized to 1 (exercise sessions =89.9 ± 8.8%, support/education sessions = 81.2 ±18.7%) or 2 sessions·wk-1 (exercise sessions = 88.8 ± 7.7%; p=0.79, support/education sessions= 86.0 ± 20.9%; p=0.87). Participants averaged 27.7 ± 5.7 mins·session-1 of MVPA with no significant difference between the 1 (26.6 ± 3.0 mins·session-1) and 2 session·wk-1 groups (28.8 ± 7.7 mins·session-1, p=0.16). The completion rate for homework assignments did not differ significantly between the 1 (21.4 ± 26.3%) and 2 session·wk-1 groups (37.7 ± 21.7%, p=0.28). CONCLUSION: Exercise delivered by group video conferencing may be a feasible and potentially effective approach for increasing MVPA in adults with Down syndrome.

Evaluation of Right Ventricular Myocardial Mechanics Using Velocity Vector Imaging of Cardiac MRI Cine Images in Transposition of the Great Arteries Following Atrial and Arterial Switch Operations.

OBJECTIVE: The aim of the study was to determine right and left ventricle deformation parameters in patients with transposition of the great arteries who had undergone atrial or arterial switch procedures. SETTING: Patients with transposition are born with a systemic right ventricle. Historically, the atrial switch operation, in which the right ventricle remains the systemic ventricle, was performed. These patients have increased rates of morbidity and mortality. We used cardiac MRI with Velocity Vector Imaging analysis to characterize and compare ventricular myocardial deformation in patients who had an atrial switch or arterial switch operation. DESIGN: Patients with a history of these procedures, who had a clinically ordered cardiac MRI were included in the study. Consecutive 20 patients (75% male, 28.7 ± 1.8 years) who underwent atrial switch operation and 20 patients (60% male, 17.7 ± 1.9 years) who underwent arterial switch operation were included in the study. Four chamber and short-axis cine images were used to determine longitudinal and circumferential strain and strain rate using Vector Velocity Imaging software. RESULTS: Compared with the arterial switch group, the atrial switch group had decreased right ventricular ejection fraction and increased end-diastolic and end-systolic volumes, and no difference in left ventricular ejection fraction and volumes. The atrial switch group had decreased longitudinal and circumferential strain and strain rate. When compared with normal controls multiple strain parameters in the atrial switch group were reduced. CONCLUSIONS: Myocardial deformation analysis of transposition patients reveals a reduction of right ventricular function and decreased longitudinal and circumferential strain parameters in patients with an atrial switch operation compared with those with arterial switch operation. A better understanding of the mechanisms of right ventricle failure in transposition of great arteries may lead to improved therapies and adaptation.

Right ventricular echocardiographic indices predict poor outcomes in infants with persistent pulmonary hypertension of the newborn.

AIMS: Infants with persistent pulmonary hypertension of the newborn (PPHN) have elevated pulmonary vascular resistance that can lead to right ventricular (RV) failure and death. Clinicians must decide which infants will fail conventional therapy and require transfer to extra corporeal membrane oxygenation (ECMO) centres, but accurate echocardiographic predictors have not been identified. We assessed echocardiographic measurements of RV pressure and function in predicting progression to death or ECMO in infants with PPHN. METHODS AND RESULTS: Echocardiograms for infants ≥35-week gestation with a clinical diagnosis of PPHN were retrospectively reviewed. Traditional and strain echocardiographic measures were compared for those with or without the primary outcome of ECMO/cardiovascular death. Receiver operator curves identified cut points for measures that were significantly different. Of the 86 subjects analysed, 25 (29%) of the patients had the primary outcome of ECMO/death. The ECMO/death group had diminished tricuspid annular plane systolic excursion (TAPSE; P = 0.002) and RV global longitudinal peak strain (GLPS; P = 0.03), a predominant right-to-left shunt across the patent ductus arteriosus (PDA; P = 0.05), and an elevated oxygenation index (OI; P < 0.001). Sensitivity/specificity for TAPSE <4 mm was 56 and 85%, and for GLPS greater than or equal to -9% was 52 and 77%. CONCLUSION: TAPSE, GLPS, and right-to-left PDA shunting were associated with progression to death/ECMO. RV free wall strain was not associated with the outcome, suggesting that diminished global strain better reflects clinical outcomes in this group. These thresholds may assist in the decision-making to transfer high-risk infants to ECMO centres.

Comprehensive assessment of right ventricular function in patients with pulmonary hypertension with global longitudinal peak systolic strain derived from multiple right ventricular views.

BACKGROUND: Right ventricular (RV) function is a strong predictor of mortality in pulmonary hypertension (PH), but two-dimensional (2D) echocardiography-derived assessments of RV function that could aid in risk assessment and management of patients with PH are of limited utility. RV longitudinal peak systolic strain (RVLS) derived from 2D speckle-tracking echocardiography is a relatively novel method for quantifying RV function but typically is derived from a single apical four-chamber view of the right ventricle and may have inherent limitations. The objective of this study was to determine the utility of regional and global RVLS calculated from multiple views of the right ventricle to comprehensively assess RV function in a cohort of patients with PH. METHODS: Regional and global RVLS were obtained from multiple views of the right ventricle (centered on the right ventricle-focused apical position) in 40 patients with PH, defined as a mean pulmonary artery pressure ≥ 25 mm Hg, most of whom also had pulmonary capillary wedge pressures ≤ 15 mm Hg and were thus defined as having pulmonary arterial hypertension. This was compared with other 2D echocardiography-derived parameters of RV function and functional parameters. RESULTS: Global RVLS calculated from multiple views had a superior correlation with 6-min walk distance compared with other parameters of RV function, including tricuspid annular plane systolic excursion, RV myocardial performance index, and fractional area change. Although global RVLS calculated from multiple views displayed a similar correlation with 6-min walk distance as global RVLS calculated from a single four-chamber view, analysis of regional strains provided by multiple views identified distinct patterns of RV dysfunction, consisting of global, free wall, or septal dysfunction, that were associated with specific clinical characteristics. CONCLUSIONS: Global RVLS derived from multiple right ventricle-focused views yields a comprehensive quantitative assessment of regional and global RV function that correlates moderately with functional parameters and may be useful in the assessment of PH. Distinct patterns of regional RV dysfunction are associated with different clinical characteristics.