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Decreased physical activity is associated with cardiovascular, metabolic and mental health disease. While decreases in physical activity during the COVID-19 pandemic have been described in the general population, there is a paucity of data regarding children with underlying cardiovascular disease. We hypothesized there would be a decrease in physical activity at the onset of the COVID-19 pandemic. Performed a single-center, retrospective cohort study of children aged 80% of dates from February 3, 2020 through June 30, 2020. Patients with significant neurologic/neuromuscular disease were excluded. We identified 144 patients with a median age of 15.4 years. 47% were female. 34% had congenital heart disease, 20% had cardiomyopathy, 19% had an inherited arrhythmia syndrome and 5% had atrioventricular block without congenital heart disease. 47% of patients had an implantable loop recorder, 29% had a permanent pacemaker and 24% had an implantable cardioverter-defibrillator. We observed a significant decrease in device-measured physical activity from baseline (February 3–March 9), with up to a 21% decrease in physical activity during mid-March through early May. Activity levels returned to pre-pandemic levels in June. Physical activity sharply declined in children with cardiac rhythm management devices at the onset of the COVID-19 pandemic. These data highlight the importance of finding strategies to maintain physical activity during the current pandemic and future public health crises.

Introduction: Survivors of congenital diaphragmatic hernia (CDH) are at risk of pulmonary hypertension (PH) due to pulmonary hypoplasia and other pulmonary vascular abnormalities. Hypothesis: Exercise stress echocardiography (ESE) may detect PH, not apparent on echocardiography at rest, in children and young adults with repaired CDH. Methods: Single center retrospective cohort study of all CDH survivors who underwent ESE at Boston Children’s Hospital (January 2006 – June 2020). Severity of PH was assessed at baseline and after exercise. Patients were assigned to one of two categories based on right ventricular pressure: group 1: no or mild PH after exercise; and group 2: moderate or severe PH after exercise (right ventricular pressure ≥ 60 mmHg or ≥ ½ systemic blood pressure). Results: During the study period 84 CDH patients (70% male) underwent 173 ESE with a median age 8.1 (4.8 – 19.1) years at first ESE. Sixty-four patients were classified as group 1, 11 as group 2, and 9 were unable to be classified due to inconclusive ESE. Moderate to severe PH after exercise was found in 8 (10%) patients with no or mild PH at rest. Risk factors for PH after exercise included larger CDH defect size, patch repair, use of extracorporeal membrane oxygenation, and supplemental oxygen at discharge in univariate analysis (Table 1). Exercise and pulmonary function test parameters associated with a group 2 test included higher VE/VCO2 slope, lower peak oxygen saturation, and lower percent predicted FEV1 and FEV1/FVC ratio. Nine of 11 group 2 patients had a change in management based on ESE. PH was found in all 5 group 2 patients with cardiac catheterization after ESE, including 3 patients with no or mild PH on baseline ESE. Conclusions: Among long term CDH survivors, ESE identified 10% with moderate-severe exercise induced PH indicating ongoing pulmonary vascular abnormalities. Further studies are needed to optimally define screening and treatment for patients with repaired CDH.

INTRODUCTION. The prevalence of defects and effective radiation dose from various myocardial perfusion imaging (MPI) strategies in congenital heart disease (CHD) is unknown. METHODS. We studied 75 subjects with complex CHD (ages 5 to 80 years) referred for MPI between 2002 and 2015. A rest and exercise or pharmacologic stress MPI was performed using (99m)Technetium sestamibi, (82)rubidium or (13)N-ammonia, and Sodium iodide SPECT (single-photon emission computed tomography), SPECT/CT or Cadmium zinc telluride (CZT) SPECT or PET (positron emission tomography)/CT scanners. Deidentified images were interpreted semi-quantitatively in three batches: stress only MPI, stress/rest MPI, and stress/rest MPI with taking into account a history of ventricular septal defect repair. Effective radiation dose was estimated for stress/rest MPI and predicted for 1-day stress-first (normal stress scans), and for 2-day stress/rest MPI (abnormal stress scans). RESULTS. The median age was 18.6 years. The most common type of CHD was transposition of the great arteries (63%). Rest/stress MPI was abnormal in 43% of subjects and 25% of the abnormal scans demonstrated reversible defects. Of the subjects with abnormal MPI, 33% had significant underlying anatomic coronary artery obstruction. Estimated mean effective radiation dose ranged from 2.1 ± 0.6 mSv for (13)N-ammonia PET/CT to 12.5 ± 0.9 mSv for SPECT/CT. Predicted effective radiation dose was significantly lower for stress-first MPI and for 2-day stress/rest protocols. CONCLUSIONS. Due to the relatively high prevalence of abnormal stress MPI, tailored protocols with a stress-first MPI as well as the use of 2-day protocols and advanced imaging technologies including CZT SPECT, novel image reconstruction software, and PET MPI could substantially reduce radiation dose in complex CHD.

Introduction: The American Heart Association (AHA) guidelines recommend testing for inducible myocardial ischemia in all patients with a history of coronary artery aneurysm (CAA) after Kawasaki disease (KD). Because the prevalence of clinical coronary complications is exceedingly low in patients whose worst-ever CAA dimension was Methods: Retrospective study including patients Results: A total of 588 stress tests were performed in 208 patients at median age of 12.4 [IQR 8.8, 16.8] years, 8.3 [IQR 5.0, 12.9] years after diagnosis (Table). Tests were performed using either exercise stress (545 [93%] tests) or pharmacologic stress with dobutamine (43 [7%] tests). Echocardiography was the most frequently used modality (n=481, 82%), followed by nuclear imaging (n=133, 23%) and cardiac MRI (n=53, 9%). Inducible ischemia was found on 11 (2%) tests in 6 (3%) patients. All patients with inducible ischemia had a history of giant CAA with persistent CAA at time of testing (moderate CAA in 2 (18%) tests, giant CAA in 9 (82%) tests). After finding inducible ischemia on stress test, coronary artery bypass grafting was performed in 3 cases, cardiac catheterization in 4, stress testing using a different imaging modality in 1, and careful clinical monitoring without intervention in 3. Conclusions: Inducible ischemia was found in 2% of test after KD, and only in patients with a history of giant CAA. Recommendations in the 2017 AHA guidelines for KD for testing for myocardial ischemia in patients with non-giant CAA should be reconsidered in light of these findings.

BACKGROUND Adults with congenital heart disease (ACHD) are vulnerable to contrast-induced acute kidney injury (CI-AKI) after cardiac catheterization. The aim of this study was to identify risk factors for clinically significant CI-AKI and evaluate the predictive value of contrast volume to estimated glomerular filtration rate ratio (V/eGFR) for the risk of CI-AKI following catheterization in the ACHD population. METHODS ACHD patients who underwent catheterization at Boston Children's hospital between 1/2011 and 1/2017 were retrospectively analyzed. CI-AKI was defined as an increase in serum creatinine ≥0.3 mg/dL within 48 hr or ≥1.5 times baseline within 7 days of procedure. Controls without CI-AKI were matched for calendar year of catheterization with cases using a 3:1 ratio. RESULTS Of 453 catheterizations meeting inclusion criteria, 27 catheterizations (5.9%) were complicated by CI-AKI, with dialysis being used to manage renal dysfunction in five of these events. Older age, male gender, admission prior to catheterization, and V/eGFR ratio were found to be related to risk of CI-AKI. Patients with a V/eGFR ≥ 2.6 had a significantly higher risk of CI-AKI (OR = 6.4; 95% CI = 2.0-20.4; P = 0.002). Survival at 3 years post-catheterization, was significantly shorter for CI-AKI cases compared to controls (49% versus 97%; P < 0.001) even in those with return to baseline renal function prior to discharge (60% versus 97%, P < 0.001). CONCLUSION In ACHD patients undergoing cardiac catheterization, a higher V/eGFR ratio is a strong predictor of clinically significant CI-AKI. Development of CI-AKI is a poor prognostic indicator and is associated with decreased survival in this population.

Background: Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD). Methods: We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O2 saturation ≥92%, and had peak O2 consumption ([Formula: see text]) < 80% predicted. We assessed exercise capacity, physical activity, quality of life, self-reported health status, and other variables at baseline and after 12 weeks. The prespecified primary end point was change in [Formula: see text]. Results: We analyzed data on 28 participants (aged 41.1 ± 12.1 years, 50% male), 13 randomized to CR and 15 to SOC. [Formula: see text] averaged 16.8 ± 3.8 mL/kg/min, peak work rate = 95 ± 28 W, and median Minnesota Living with Heart Failure Questionnaire (MLHFQ) score = 27 (interquartile range: 11-44). Cardiac rehabilitation participants were older (48 ± 9 years vs 36 ± 12 years; P = .01), but there were no significant between-group differences in other variables. There were no adverse events related to CR. [Formula: see text] increased in the CR group compared with SOC (+2.2 mL/kg/min, 95% confidence interval: 0.7-3.7; P = .002, age-adjusted +2.7 mL/kg/min; P = .004); there was a nonsignificant improvement in work rate (+8.1 W; P = .13). Among the 25 participants with baseline MLHFQ > 5, there was a clinically important >5-point improvement in 72.7% and 28.6% of CR and SOC participants, respectively ( P = .047). Cardiac rehabilitation was also associated with improved self-assessment of overall health ( P < .04). Conclusions: Cardiac rehabilitation is safe and is associated with improvement in aerobic capacity and self-reported health status compared with SOC in adults with CHD.

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD). Methods: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire). Results: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0–2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4–2.0] (n = 6) per year, with 1.1% [95%CI 0.5–2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5–8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80–93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05). Conclusions: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease., SCOPUS: ar.j, info:eu-repo/semantics/published

Exercise stress echocardiography (ESE) is a versatile diagnostic tool that has been integrated into the standard clinical assessment of adult patients with coronary artery disease, hypertrophic cardiomyopathy (HCM), and pulmonary hypertension. Experience with ESE in children with these disorders is more limited. Given the potential value of this technology is significant, its role in the clinical diagnosis and management of pediatric patients continues to evolve. Since 2006, Boston Children’s Hospital has been performing stress echocardiography in children for both ischemia and assessment of hemodynamics of exercise.

This chapter summarizes the basic approach for evaluation and characterization of ventricular septal defects (VSD) in adults. A brief description of nomenclature and associated cardiac evaluation is included. Finally, a schematic for a systemic approach to VSD evaluation is provided.

This chapter provides a brief review of complex congenital heart disease. Included is a general approach to complex anatomy as well as examples of coarctation, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, and Ebstein anomaly.

Learning objectives Malcolm is a 35-year-old patient followed at a safety net hospital. He describes growing up with limited resources both in his family and at his school. As the child of a single mother, his afternoons were often spent hanging out in his neighbourhood. He recalls that there were two options ‘basketball or gangs’. Because of his complex congenital heart disease, he was not allowed to play sports and his exercise capacity was never evaluated. Now, 20 years later, he feels that was a turning point in his life which ultimately resulted in a 5-year incarceration for weapons charges. ‘I loved basketball and not playing made me angry and feel different. I made my own choices, but playing would have made a huge difference’. Malcolm has his life back on track now, but providers have a responsibility to ensure that kids like him have providers who consider their whole lives and the lifelong impact of their recommendations. Restriction is not only a physical limitation. It affects patient’s self-perception, acceptance and psyche. Thus, providers must consider the risks of not playing sports versus that of playing, and recommend level of activity based on objectively obtained data. The benefits of exercise are well known to most patients and providers. Due to exercise restrictions placed of patients with congenital heart disease (CHD) as well as concerns for exercise-related morbidity and mortality, many providers and patients are uncertain on ways to …

While the Fontan procedure has improved life expectancy, patients with single ventricle physiology have impaired exercise capacity due to limited increase in pulmonary blood flow during activity. Enhancing the “thoracic pump” using inspiratory muscle training (IMT) may ameliorate this impairment. Adult nonsmokers with Fontan physiology were recruited through Boston Children's Hospital's outpatient clinic. Participants underwent cardiopulmonary exercise testing and pulmonary function testing, followed by 12 weeks of IMT and then repeat testing. The primary endpoint was change in % predicted peak oxygen consumption (VO2). Secondary endpoints were changes in other exercise metrics. Eleven patients (6 male) were enrolled. Median ages at time of enrollment and Fontan completion were 28.8 years (25.7, 45.5) and 7.8 years (3.9, 16.5), respectively. Average baseline maximal inspiratory pressure (MIP) was normal; only 2 patients had MIP

Key Points Patients with transposition of the great arteries (TGA) and systemic right ventricles have premature congestive heart failure; there is also a growing concern that athletes who perform extraordinary endurance exercise may injure the right ventricle. Therefore we felt it essential to determine whether exercise training might injure a systemic right ventricle which is loaded with every heartbeat. Previous studies have shown that short term exercise training is feasible in TGA patients, but its effect on ventricular function is unclear. We demonstrate that systemic right ventricular function is preserved (and may be improved) in TGA patients with exercise training programmes that are typical of recreational and sports participation, with no evidence of injury on biomarker assessment. Stroke volume reserve during exercise correlates with exercise training response in our TGA patients, identifying this as a marker of a systemic right ventricle (SRV) that may most tolerate (and possibly even be improved by) exercise training. Abstract We aimed to assess the haemodynamic effects of exercise training in transposition of the great arteries (TGA) patients with systemic right ventricles (SRVs). TGA patients have limited exercise tolerance and early mortality due to systemic (right) ventricular failure. Whether exercise training enhances or injures the SRV is unclear. Fourteen asymptomatic patients (34 ± 10 years) with TGA and SRV were enrolled in a 12 week exercise training programme (moderate and high-intensity workouts). Controls were matched on age, gender, BMI and physical activity. Exercise testing pre- and post- training included: (a) submaximal and peak; (b) prolonged (60 min) submaximal endurance and (c) high-intensity intervals. Oxygen uptake (VO2; Douglas bag technique), cardiac output (Qc, foreign-gas rebreathing), ventricular function (echocardiography and cardiac MRI) and serum biomarkers were assessed. TGA patients had lower peak VO2, Qc, and stroke volume (SV), a blunted Qc/VO2 slope, and diminished SV response to exercise (SV increase from rest: TGA = 15.2%, controls = 68.9%, P

Background— Exercise oscillatory ventilation (EOV) refers to regular oscillations in minute ventilation (V E ) during exercise. Its presence correlates with heart failure severity and worse prognosis in adults with acquired heart failure. We evaluated the prevalence and predictive value of EOV in patients with single ventricle Fontan physiology. Methods and Results— We performed a cross-sectional analysis and prospective survival analysis of patients who had undergone a Fontan procedure and subsequent cardiopulmonary exercise test. Data were reviewed for baseline characteristics and incident mortality, heart transplant, or nonelective cardiovascular hospitalization. EOV was defined as regular oscillations for >60% of exercise duration with amplitude >15% of average V E . Survival analysis was performed using Cox regression. Among 253 subjects, EOV was present in 37.5%. Patients with EOV were younger (18.8±9.0 versus 21.7±10.1 years; P =0.02). EOV was associated with higher New York Heart Association functional class ( P =0.02) and V E /V CO 2 slope (36.8±6.9 versus 33.7±5.7; P =0.0002), but not with peak V O 2 (59.7±14.3 versus 61.0±16.0% predicted; P =0.52) or noninvasive measures of cardiac function. The presence of EOV was associated with slightly lower mean cardiac index but other invasive hemodynamic variables were similar. During a median follow-up of 5.5 years, 22 patients underwent transplant or died (n=19 primary deaths, 3 transplants with 2 subsequent deaths). EOV was associated with increased risk of death or transplant (hazard ratio, 3.9; 95% confidence interval, 1.5–10.0; P =0.002) and also predicted the combined outcome of death, transplant, or nonelective cardiovascular hospitalization after adjusting for New York Heart Association functional class, peak V O 2 , and other covariates (multivariable hazard ratio, 2.0; 95% confidence interval, 1.2–3.6; P =0.01). Conclusions— EOV is common in the Fontan population and strongly predicts lower transplant-free survival.

Objective Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality. Design All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test. Results Of the 42 cases, 27 had a Fontan circulation and 15 with TOF. All Fontan patients had a low peak VO2 but there was no significant difference between cases and controls (52.5 ± 14.7 v. 57.4% ± 13.5% predicted, P = .11). Spirometry values were significantly lower in Fontan cases than controls (eg, FVC 67.4 ± 19.1 v 77.6% ± 14.9% predicted, P = .007). Spirometry values were also lower in TOF cases than controls (% predicted FVC 62.8 ± 16.7 v 75 ± 14, P = .006). In contrast to the Fontan analysis, both %peak predicted VO2 and VE/VCO2 slope were worse in TOF cases than controls (50.1 ± 13.5 v. 68.5% ± 15.0% predicted VO2 , P = .0004; 33.9 ± 12.9 v 26.6 ± 4.4, P = .002). Multivariable analysis also identified different predictors of mortality among the anatomic subgroups. Spirometric data (FVC) correlated most strongly with mortality in Fontan patients while the VE/VCO2 slope was most associated with outcome in TOF patients. Conclusions Variables most predictive of mortality in Fontan and TOF patients diverge but spirometry was abnormal and associated with mortality in both groups. When compared with age-matched controls, reduced FEV1 and FVC correlated most strongly with mortality in Fontan patients while VE/VCO2 slope correlated with mortality for TOF patients. These findings further support the importance of lung health in patients with complex congenital heart disease.

Objectives In patients with systemic right ventricles (RVs) in a biventricular circulation, exercise capacity and RV function often deteriorate over time and echocardiographic assessment of systemic RV function is difficult. The purpose of this study was to examine the relationship between exercise capacity and RV function and to determine which noninvasive imaging parameters correlate most closely with exercise capacity. Design Patients with a systemic RV (D-loop transposition of the great arteries [TGA] after atrial switch procedure or physiologically “corrected” TGA) who underwent cardiopulmonary exercise testing (CPX) and noninvasive imaging (cardiac magnetic resonance [CMR] and echocardiography [echo]) within 1 year of CPX were identified. Regression analysis was used to evaluate the relationship between exercise variables and noninvasive indices of ventricular function. Results We identified 92 patients with 149 encounters (mean age 31.0 years, 61% men, 70% D-loop TGA) meeting inclusion criteria. Statistically significant correlations between % predicted peak oxygen uptake (%pVO2) and RV ejection fraction (EF) (r = 0.29, P = .0007), indexed RV end-systolic volume (r = −0.25, P = .002), and Tei index (r = −0.22, P = .03) were found. In patients without additional hemodynamically significant lesions, the correlations between %pVO2 and RV EF (r = 0.37, P = .0007) and the Tei index (r = −0.28, P = .03) strengthened and a correlation emerged between %pVO2 and dP/dtic (r = 0.31, P = .007). On multivariable analysis, Tei index was the only statistically significant correlate of %pVO2 (P = .04). Conclusions In patients with systemic RVs in a biventricular circulation, CMR-derived RVEF and echo-derived Tei index correlate with %pVO2. On multivariable analysis, the Tei index was the strongest predictor of peak %pVO2 response.

Background Peak oxygen consumption (pVO 2 ) measured by cardiopulmonary exercise test (CPET) predicts mortality in adults with a Fontan circulation. The purpose of this study was to assess the additive prognostic value of change in pVO 2 over time. Methods We analyzed a cohort of adults (≥18years-old) with a Fontan circulation who underwent at least 2 maximal CPETs separated by 6–30months at Boston Children's Hospital between 2000 and 2015. Survival analysis was performed to determine whether changes in CPET variables, including pVO 2 between consecutive tests were associated with subsequent clinical events. The primary outcome was transplant-free survival. Results The study included 130 patients with 287 CPET test pairs. Average age was 26.6±9.5years. Baseline pVO 2 averaged 22.0±5.7mL/kg/min or 60.9±13.7% predicted. In the cohort overall, there was no change in mean pVO 2 between sequential CPETs. Eleven patients died and 2 underwent transplant. On average, pVO 2 declined for patients who subsequently died or underwent transplant but remained stable among those who did not (−9.8±14.6% vs. 0.0±13.0%, P 2 between CPETs were at greater risk of death or transplantation (per 10% decrease in pVO 2 HR=2.0, 95%CI 1.2–3.1, P =.004). Change in pVO 2 remained a significant predictor of death or transplant after adjusting for pVO 2 at first CPET (per 10% decline in pVO 2 , HR=2.5, 95%CI 1.5–4.2, P Conclusions A decline in pVO 2 between consecutive CPETs predicts increased risk for death or transplant in adults with a Fontan circulation independent of baseline pVO 2 . These results support the additive clinical value of serial CPET in this population.

Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair.In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years). Our analysis categorized participants in these three groups: with structurally normal hearts as controls (n=21), with BiV circulation (n=20) and with UniV circulation (n=14). We acquired ESE images of the systemic ventricle including pulsed-wave flow and spectral tissue Doppler imaging (TDI) of lateral free wall before and immediately after standard, symptom-limited exercise tests on an electronically braked cycle ergometer.During ESE we obtained inflow E-wave and TDI systolic (S') and early diastolic (E') velocities in 93% to 100% of participants at rest and in 90% to 100% of participants post exercise. Feasibility to obtain Doppler imaging parameter was the same across study groups. The myocardial response to exercise was increase in heart rate (HR), S' and inflow E-wave velocity in all participants. Patients with BiV circulation had preserved ventricular function at rest. While patients with UniV circulation had low S', E', and E-wave velocities at rest in comparison to controls and to BiV group (all P.001), both patients with BiV and UniV circulation showed significant increases in HR, S' velocity and inflow E-wave velocity post exercise, with magnitudes of these increases higher in controls than in the BiV and UniV group. The S' and E' velocities were strongly associated with lower percent predicted peak oxygen consumption VOESE with Doppler imaging is a practical noninvasive diagnostic method and sufficiently robust for the assessment of morphologic LV/systemic ventricles under exercise in patients after biventricular and univentricular CHD repair. Although patients with BiV and UniV circulation had both preserved myocardial response to exercise, the magnitude of this response was the lowest in patients with UniV circulation.

Sedentary aging leads to left ventricular (LV) and vascular stiffening due in part to advanced glycation end-products (AGEs) cross-linking of extracellular matrix proteins. Vigorous lifelong exercise ameliorates age-related cardiovascular (CV) stiffening and enhances exercise LV function, although this effect is limited when exercise is initiated later in life. We hypothesized that exercise training might be more effective at improving the impact of age-related CV stiffening during exercise when combined with an AGE cross-link breaker (Alagebrium). Sixty-two seniors (≥60 yr) were randomized into four groups: sedentary + placebo, sedentary + Alagebrium, exercise + placebo, and exercise + Alagebrium for 1 yr. Moderate-intensity aerobic exercise was performed 3-4 sessions/wk; controls underwent similar frequency of yoga/balance training. Twenty-four similarly-aged, lifelong exercisers (4–5 sessions/wk) served as a comparator for the effect of lifelong exercise on exercising LV function. Oxygen uptake (Douglas bags), stroke index (SI; acetylene rebreathing), and effective arterial elastance (Ea) were collected at rest and submaximal and maximal exercise. Maximum O2 uptake (23 ± 5 to 25 ± 6 ml·kg−1·min−1) increased, while SI (35 ± 11 to 39 ± 12 ml/m2) and Ea (4.0 ± 1.1 to 3.7 ± 1.2 mmHg·ml−1·m−2) were improved across all conditions with exercise, but remained unchanged in controls (exercise × time, P ≤ 0.018). SI or Ea were not affected by Alagebrium (medication × time, P ≥ 0.468) or its combination with exercise (interaction P ≥ 0.252). After 1 yr of exercise plus Alagebrium, exercise SI and Ea remained substantially below that of lifelong exercisers (15–24 and 9–22%, respectively, P ≤ 0.415). In conclusion, Alagebrium plus exercise had no synergistic effect on exercise LV function and failed to achieve levels associated with lifelong exercise, despite a similar exercise frequency.

Providing medical care for adults with congenital heart disease (CHD) is a complex endeavor requiring an understanding of all aspects of the health system. Adult patients with CHD often have multiple medical issues related to the underlying CHD, the sequelae of surgical or catheter interventions, and the addition of typical conditions of adulthood superimposed on the CHD. In this article, the authors first frame the assessment of quality in the health care system as a whole and then focus on CHD and the unique approach required in the care of the adult with CHD.

Background— Lifelong exercise training maintains a youthful compliance of the left ventricle (LV), whereas a year of exercise training started later in life fails to reverse LV stiffening, possibly because of accumulation of irreversible advanced glycation end products. Alagebrium breaks advanced glycation end product crosslinks and improves LV stiffness in aged animals. However, it is unclear whether a strategy of exercise combined with alagebrium would improve LV stiffness in sedentary older humans. Methods and Results— Sixty-two healthy subjects were randomized into 4 groups: sedentary+placebo; sedentary+alagebrium (200 mg/d); exercise+placebo; and exercise+alagebrium. Subjects underwent right heart catheterization to define LV pressure–volume curves; secondary functional outcomes included cardiopulmonary exercise testing and arterial compliance. A total of 57 of 62 subjects (67±6 years; 37 f/20 m) completed 1 year of intervention followed by repeat measurements. Pulmonary capillary wedge pressure and LV end-diastolic volume were measured at baseline, during decreased and increased cardiac filling. LV stiffness was assessed by the slope of LV pressure–volume curve. After intervention, LV mass and end-diastolic volume increased and exercise capacity improved (by ≈8%) only in the exercise groups. Neither LV mass nor exercise capacity was affected by alagebrium. Exercise training had little impact on LV stiffness (training×time effect, P =0.46), whereas alagebrium showed a modest improvement in LV stiffness compared with placebo (medication×time effect, P =0.04). Conclusions— Alagebrium had no effect on hemodynamics, LV geometry, or exercise capacity in healthy, previously sedentary seniors. However, it did show a modestly favorable effect on age-associated LV stiffening. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT01014572.

Background— Hemodynamic assessment after volume challenge has been proposed as a way to identify heart failure with preserved ejection fraction. However, the normal hemodynamic response to a volume challenge and how age and sex affect this relationship remain unknown. Methods and Results— Sixty healthy subjects underwent right heart catheterization to measure age- and sex-related normative responses of pulmonary capillary wedge pressure and mean pulmonary arterial pressure to volume loading with rapid saline infusion (100–200 mL/min). Hemodynamic responses to saline infusion in heart failure with preserved ejection fraction (n=11) were then compared with those of healthy young (−1 ·m 2 ) than the other 3 groups ( P ≤0.019). Saline infusion resulted in a greater increase in mean pulmonary arterial pressure relative to cardiac output in women compared with men regardless of age. Subjects with heart failure with preserved ejection fraction exhibited a steeper increase in pulmonary capillary wedge pressure relative to infused volume (25±12 mm Hg·L −1 ·m 2 ) than healthy young and older subjects ( P ≤0.005). Conclusions— Filling pressures rise significantly with volume loading, even in healthy volunteers. Older women and patients with heart failure with preserved ejection fraction exhibit the largest increases in pulmonary capillary wedge pressure and mean pulmonary arterial pressure.

Background— Aging and sedentary lifestyles lead to cardiac atrophy, ventricular stiffening, and impaired diastolic function. Both conditions are marked by increased adiposity, which can lead to ectopic fat deposition in nonadipocyte tissues including the myocardium. The effect of excess intramyocardial fat on cardiac function in nonobese individuals is unknown. Methods and Results— Cardiac lipid content was measured by magnetic resonance spectroscopy in 153 healthy nonobese subjects with varying fitness levels quantified by peak oxygen uptake during treadmill exercise. Cardiac function (echo) and left ventricular (LV) filling pressures (right heart catheterization) were measured under varying preloads. LV stiffness was calculated from a curve fit of the diastolic portion of the pressure–volume curve. The strongest clinical predictors of lipid content were body mass index (β=+0.03; 95% confidence interval, 0.001–0.06) and peak oxygen uptake (β=−0.02; 95% confidence interval, −0.03 to −0.009; R 2 =0.14; P 2 ; P Conclusions— Body mass index and fitness levels are the strongest predictors of myocardial lipid content in nonobese humans. Cardiac lipid content is associated with decreased ventricular distensibility, and it may provide a causal mechanism linking changes in LV function related to age and fitness.

Objectives The aim of this study was to determine the relative contribution of the muscle and ventilatory pumps to stroke volume in patients without a subpulmonic ventricle. Background In patients with Fontan circulation, it is unclear how venous return is augmented to increase stroke volume and cardiac output during exercise. Methods Cardiac output (acetylene rebreathing), heart rate (electrocardiography), oxygen uptake (Douglas bag technique), and ventilation were measured in 9 patients age 15.8 ± 6 years at 6.1 ± 1.8 years after Fontan operation and 8 matched controls. Data were obtained at rest, after 3 min of steady-state exercise (Ex) on a cycle ergometer at 50% of individual working capacity, during unloaded cycling at 0 W (muscle pump alone), during unloaded cycling with isocapnic hyperpnea (muscle and ventilatory pump), during Ex plus an inspiratory load of 12.8 ± 1.5 cm water, and during Ex plus an expiratory load of 12.8 ± 1.6 cm water. Results In Fontan patients, the largest increases in stroke volume and stroke volume index were during zero-resistance cycling. An additional increase with submaximal exercise occurred in controls only. During Ex plus expiratory load, stroke volume indexes were reduced to baseline, non-exercise levels in Fontan patients, without significant changes in controls. Conclusions With Fontan circulation increases in cardiac output and stroke volume during Ex were due to the muscle pump, with a small additional contribution by the ventilatory pump. An increase in intrathoracic pressure played a deleterious role in Fontan circulation by decreasing systemic venous return and stroke volume.

We appreciate the interest and thoughtful comments regarding our recently published paper and are pleased to take this opportunity to discuss our techniques and data further.

A 28-year-old woman experienced hypotension and unresponsiveness. She had undergone bronchoscopy with needle biopsy 2 weeks before admission to evaluate hilar lymphadenopathy given suspicion of sarcoidosis. She had an elevated white blood cell count of 28,000/μL and a serum creatinine level of 4.0 mg/dL. Echocardiography showed a large pericardial effusion. Pericardiocentesis resulted in removal of 400 mL of yellow-green purulent material that grew Streptococcus milleri, Prevotella, Veillonella, and Peptostreptococcus species. Pericardiectomy and mediastinal washout were performed. Subsequently, her condition rapidly improved. She returned home after 26 days of admission. At 12-month follow-up, the patient had made a full recovery.

Exercise test variables including ventilatory efficiency (VE/VCO2 slope) and peak oxygen uptake (pVO2) correlate with mortality in adults with congenital heart disease. O2 pulse (VO2 divided by heart rate) is used as a surrogate for stroke volume. However, correlation of these exercise values with