2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

Clinical significance of the hemodynamic gain index in patients undergoing exercise stress testing and coronary computed tomography angiography.

BACKGROUND: Many hemodynamic parameters provide limited information regarding obstructive coronary artery disease (CAD) during exercise stress testing particularly when exercise is suboptimal. Hemodynamic gain index (HGI) is a recent sensitive indicator of ischemia and has been associated with increased mortality. This study evaluated the clinical impact of HGI in patients who underwent concomitant exercise stress testing and coronary computed tomography angiography (CCTA). METHODS: A total of 284 consecutive patients from the executive health program between 2010 and 2018 were identified. Resting and peak heart rate (HR) as well as systolic blood pressure (SBP) measurements were recorded. Framingham risk score (FRS), Duke treadmill score (DTS) and HGI [Formula: see text] were calculated. The latter was divided into quartiles. CCTA was used as a reference test to detect any CAD. Multivariate analysis and artificial neural network were used to determine the independent predictors of obstructive CAD. RESULTS: Mean age was 53 ± 12 years with 83% male. Mean HGI was 1.74 ± 0.67, with cut-off value of severely blunted HGI ≤ 1.25 (Quartile 4). Patients with severely blunted HGI were older, had higher FRS, and worse DTS. Patients with obstructive CAD had lower HGI when compared to those with normal CCTA/non-obstructive CAD (1.36 ± 0.53 vs. 1.77 ± 0.67, P = 0.005), and showed a higher prevalence of severely blunted HGI (44% vs. 22%, P = 0.019). After adjusting for traditional risk factors, HGI remained an independent predictor of obstructive CAD while severely blunted HGI was associated with threefold increased odds of having obstructive CAD (P = 0.05). Using artificial intelligence analysis, severely blunted HGI independently predicted obstructive CAD with an area under the curve of 0.83 and 0.96, and normalized importance of HGI of 100% and 63%, respectively for different models. CONCLUSIONS: Among patients who underwent concomitant exercise stress testing and CCTA, severely blunted HGI independently predicted obstructive CAD after multivariate adjustment for traditional risk factors.

Aortic Stenosis: Risk Stratification and Timing of Surgery.

PURPOSE OF REVIEW: This review summarizes the current management of patients with newly diagnosed aortic stenosis. RECENT FINDINGS: Recent developments include detection of early myocardial dysfunction using serum B-type natriuretic peptide levels and global longitudinal strain, as well as ongoing trials of transcatheter aortic valve replacement in asymptomatic patients and patients with moderate aortic stenosis complicated by symptoms or left ventricular systolic dysfunction. Given the high mortality associated with severe symptomatic aortic stenosis, all symptomatic patients should be referred for aortic valve replacement. Asymptomatic patients with left ventricular systolic dysfunction, abnormal exercise stress test, high degree of stenosis, rapid disease progression, or elevated serum B-type natriuretic peptide level may also have an indication for valve replacement, based on established criteria. The progress in management of severe aortic stenosis has been in the direction of earlier detection and earlier valve replacement.

Multisystem Inflammatory Syndrome in Children (MIS-C): Reduced Exercise Duration and Capacity at Six Month Follow-Up.

Myocarditis is common in Multisystem Inflammatory Syndrome in Children (MIS-C), and the mechanism may differ from idiopathic/viral myocarditis as MIS-C involves a hyper-inflammatory state weeks after COVID-19. We sought to evaluate exercise stress testing (EST) in these patients as EST may help guide return-to-play recommendations. Retrospective cohort study evaluating ESTs (standard Bruce treadmill protocol) from MIS-C patients from 2020 to 2022, compared to myocarditis patients and age, sex, and weight matched controls from 2005 to 2019. ESTs included 22 MIS-C patients (mean age 11.9 years) with 14 cardiopulmonary and 8 cardiovascular tests, 33 myocarditis (15.5 years), and 44 controls (12.0 years). Percent-predicted peak VO2 was abnormal (< 80% predicted) in 11/14 (79%) MIS-C patients, 13/33 (39%) myocarditis, and 17/44 (39%) controls (p = 0.04). Exercise duration was shorter in MIS-C than myocarditis or control cohorts (p = 0.01). Isolated atrial or ventricular ectopy was seen in 8/22 (36%) MIS-C, 9/33 (27%) myocarditis, and 5/44 (11%) controls (p = 0.049). No arrhythmias/complex ectopy or evidence of ischemia were noted, though non-specific ST/T wave abnormalities occurred in 4/22 (18%) MIS-C, 5/33 (15%) myocarditis, and 3/44 (7%) controls. Exercise duration and percent-predicted peak VO2 were significantly reduced in MIS-C at mean 6-month follow-up compared to pre-COVID era idiopathic/viral myocarditis and control cohorts. This may be secondary to deconditioning during the pandemic and/or chronic cardiopulmonary or autonomic effects of COVID/MIS-C. Although there were no exercise-induced arrhythmias in our MIS-C patients, larger cohort studies are warranted. EST in MIS-C follow-up may help evaluate safety and timing of return to play and potentially mitigate further deconditioning.

Importance of exercise stress testing in evaluation of unexplained cardiac arrest survivor.

BACKGROUND: In sudden cardiac arrest survivors without an immediately identifiable cause, additional extensive yet individualised testing is required. METHODS: We describe 3 survivors of sudden cardiac arrest in whom exercise stress testing was not performed during the initial hospital admission. RESULTS: All 3 patients were incorrectly diagnosed with long QT syndrome based on temporary sudden cardiac arrest-related heart rate-corrected QT interval prolongation, and exercise stress testing was not performed during the initial work-up. When they were subjected to exercise stress testing during follow-up, a delayed diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT) was made. As a result, these patients were initially managed inappropriately, and their family members were initially not screened for CPVT. CONCLUSION: In sudden cardiac arrest survivors without an immediately identifiable cause, omission of exercise stress testing or erroneous interpretation of the results can lead to a delayed or missed diagnosis of CPVT, which may have considerable implications for survivors and their family.

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Collaboration, Agency for Healthcare Research and Quality reports, and other relevant databases. Structure Many recommendations from the earlier hypertrophic cardiomyopathy guidelines have been updated with new evidence or a better understanding of earlier evidence. This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy. For both guideline-directed medical therapy and other recommended drug treatment regimens, the reader is advised to follow dosing, contraindications and drug-drug interactions based on product insert materials.

Resting electrocardiographic differences in ventricular repolarization between children and young adults with congenital heart disease and those with a structurally normal heart are diminished by exercise.

OBJECTIVE: Exercise-induced repolarization changes have not been systematically evaluated in children and young adults with congenital heart disease (CHD). We carried out this study to assess the QTc responses during exercise in children and young adults (≤ 21 years) with CHD with comparison to those with structurally normal hearts. METHODS: Baseline QRS duration, calculated baseline QTc, QTc at 4 min of recovery and delta QTc was measured in 360 exercise stress tests which were performed in 360 subjects (137 stress tests in patients with CHD [CHD group] and 223 stress tests in patients with structurally normal hearts). The effects of presence of CHD and potential confounders on primary outcome measure, change in QTc (delta QTc), and secondary outcome measures (QTc at baseline and QTc at 4 min of recovery) were determined using multiple linear regression analyses. RESULTS: The baseline QTc and the QTc at 4 min of recovery in the CHD group was longer than patients with structurally normal hearts (respective p values = .00 and .001). No significant difference was noted in delta QTc between the CHD and structurally normal heart groups. CONCLUSIONS: While patients with CHD had a longer QRS duration and QTc interval at baseline than those with structurally normal hearts, these differences did not persist or augment with exercise.

A high exercise workload of ≥ 10 METS predicts a low risk of significant ischemia and cardiac events in older adults.

BACKGROUND: Patients who achieve ≥ 10 METS during exercise SPECT myocardial perfusion imaging (MPI) have very low rates of significant ischemia and major adverse cardiac events (MACE). It is unknown how many older adults can achieve ≥ 10 METS, and if low risk extends to this subgroup. METHODS AND RESULTS: We examined the workload achieved, prevalence and predictors of ischemia, and MACE (cardiac death, non-fatal MI, late revascularization) in a cohort of 382 patients ≥ 65 years of age who underwent exercise 99mTc SPECT MPI. The cohort was 64.4% male and 36.9% had known coronary artery disease (CAD). All achieved ≥ 85% of maximum age-predicted heart rate. A workload of ≥ 10 METS was achieved in 25.4%; 50.3% attained 7-9 METS, and 24.4% reached < 7 METS. There was a stepwise decrease in prevalence of any ischemia and significant ischemia (≥ 10% of the left ventricle (LV)) as workload increased (P = 0.037). Patients achieving ≥ 10 METS had a 3.1% prevalence of ≥ 10% LV ischemia (1.2% in those without ST depression). Cardiac death and MACE rates in the ≥ 10 METS subgroup were 0.6%/year and 2.6%/year over a median 7.0 years of follow-up. CONCLUSIONS: A substantial proportion of older adults who undergo exercise SPECT MPI can achieve ≥ 10 METS. This subgroup has low rates of significant LV ischemia and MACE. The favorable diagnostic and prognostic implications of achieving a high workload in an older adult population suggest it is feasible, with certain exceptions, to include this subgroup in workload-based strategies of provisional imaging.

How to achieve patient-centered testing: role of the protocol nurse.

INTRODUCTION: Patient-centered cardiac testing is predicated on choosing the right test for the right patient. We studied the effects of changing from script-driven scheduling to nurse-driven protocoling of stress tests. METHODS AND RESULTS: A protocol nurse reviewed records before scheduling and communicated with patients and ordering providers if needed. We found that instituting nurse protocolling of all non-imaging (ETT) and nuclear (MPI) stress tests (N = 3071) resulted in protocol changes in 37% of our patients, and reduced the proportion of tests that could not be performed as scheduled by 56% and cancelations by 71% (P < 0.001 for each). These changes were sustained over two successive 6-month periods following a baseline observation period of 6 months. For MPI, the most frequent nurse interventions were re-protocoling as stress-first MPI (12% of tests), changing test location for clinical reasons (13%), changing stress modality (7%), and care coordination (5%). CONCLUSIONS: Changing from script-driven scheduling to protocol nursing contributed measurably to patient-centered testing.

Utilization of stress testing for low-risk patients with chest discomfort in the emergency department.

BACKGROUND: The management of patients presenting to an emergency department with chest discomfort at low-risk for acute coronary syndrome represents a common clinical challenge. Such patients are often triaged to chest pain units for monitoring and cardiac stress testing for further risk stratification. METHODS: We conducted a retrospective study of 292 low-risk patients who presented to an emergency department with chest discomfort. We performed physician-adjudicated chart reviews of all patients with positive stress tests to assess downstream testing, subsequent coronary revascularization, and outcomes. RESULTS: Of the 292 patients, 33 (11.3%) had stress tests positive for ischemia, and 12 (4.1%) underwent diagnostic cardiac catheterization. Of the 292 patients, 4 (1.4%) underwent coronary revascularization that may have resulted in a mortality benefit. CONCLUSION: These data suggest a very low yield of detecting clinically significant coronary disease with stress testing low-risk patients with chest discomfort in emergency department chest pain units.

Comparative Cardiorespiratory Fitness in Children: Racial Disparity May Begin Early in Childhood.

African American (AA) adults are reported to have lower levels of cardiorespiratory fitness (CRF) as compared to Caucasian adults. CRF is linked to cardiovascular morbidity and mortality. We hypothesized that the disparities start early in childhood. This was a retrospective analysis of the cardiopulmonary exercise test (CPET). We included normal healthy children, ≤ 18 years of age, who had normal electrocardiograms and normal cardiac function. We excluded patients with congenital heart disease, obesity and suboptimal exercise test. The entire cohort was divided into two groups based on race (Caucasian vs. AA) and then further subcategorized by gender. The cohort of 248 patients had a mean ± SD age of 14.4 ± 2.1 years. 158 (60.8%) were males and 158 (60.8%) were Caucasians. Oxygen consumption was higher among Caucasian children when compared to the AA children (48.7 ± 7.9 vs. 45.4 ± 7 mL/kg/min, p = 0.01). This racial disparity continued to persist when comparisons were performed separately for girls and boys. Upon comparing the four groups, the AA females were found to have the lowest values of VO2max, exercise time and METS (p = 0.001). Thus, in conclusion, the AA children have significantly lower level of CRF, as measured by VO2max and exercise time. The racial disparity is independent of gender. African American females show the lowest level of aerobic capacity. The findings of our study suggest that the racial disparity in the CRF levels seen in the adult population may begin early in childhood.

Comparative efficiency of exercise stress testing with and without stress-only myocardial perfusion imaging in patients with low-risk chest pain.

OBJECTIVES: To compare major adverse cardiac event (MACE), downstream resource utilization, and direct cost of care for low-risk chest pain patients observed in the clinical decision unit (CDU) with exercise treadmill testing (ETT) and with stress-only myocardial perfusion imaging (sMPI). BACKGROUND: CDUs are poised to increase efficiency and resource utilization. However, the optimal testing strategy that would assure favorable outcomes while decreasing cost is not defined. METHODS: 1016 subjects from 2 locations were propensity score-matched (PSM) by age, gender, pre-test likelihood, Duke treadmill score, and test results. Outcomes were length of stay >24 hours, MACE (acute coronary syndrome, revascularization, cardiac death), downstream resource use (admission for chest pain, repeat testing, angiography), and mean direct cost per patient. RESULTS: PSM yielded 680 patients (340 matches). 98% of all tests were normal. 96.6% of patients were discharged from the CDU within 24 hours but twice as many exceeded 24 hours in the sMPI group. There were no cardiac deaths. MACE rate was 1.47% at 72 hours and 1% at 1 year. Downstream resource use was 4.82% at 72 hours, and 7.69% at 1 year. The sMPI group was event-free longer than the ETT group reflecting less repeat testing. The mean direct cost was 30% higher for sMPI ($3168.70) vs. ETT ($2226.96). CONCLUSION: Low-risk chest pain patients in the observation unit had low MACE rate, not different for ETT vs. sMPI. The majority of ETT and sMPI tests were normal. The sMPI reduced additional testing, but resulted in greater expense and longer stay.

The hidden risk: Incorporating inflammation and HIV serostatus into coronary artery disease screening.

CAD is a well-established comorbidity associated with HIV infection. This association is in large part due to ongoing inflammation propagated by viremia and dysregulation of the immune system. Despite this knowledge, evidence to guide clinical management and screening for CAD among HIV-infected patients is lacking. The following editorial discusses recent evidence that HIV-infected patients with abnormal cardiovascular stress testing are more likely to undergo subsequent percutaneous coronary intervention. Importantly, the cardiovascular consequences of HIV infection and potential clinical implications are discussed.

Maximum QTc on Holter electrocardiography in children.

BACKGROUND: Corrected QT interval (QTc) on electrocardiography (ECG) at rest and after exercise in a short daytime recording period may be insufficient for the diagnosis and management of long QT syndrome (LQTS), especially for LQTS type 2 and 3. Therefore, examination of QTc on Holter ECG is important. We designed a method of analyzing QTc on Holter ECG that can be performed in daily clinical practice by combining automatic and manual measurements. METHODS: We reviewed the charts of healthy children (n = 210) and LQTS patients (n = 35) aged <16 years and analyzed QTc at rest and after exercise, and the maximum QTc on Holter ECG. RESULTS: The QTc (Fridericia's correction) of most controls and LQTS patients reached the maximum at night or early in the morning. QTc differed according to sex and age. In the control group, QTc on ECG of all three types tended to lengthen with age. QTc after exercise was slightly longer than QTc at rest, and the maximum QTc on Holter ECG was much longer than both, reaching >450 ms in boys and 500 ms in girls. In most LQTS type 1 patients, QTc after exercise and that on Holter ECG tended to be long. In most LQTS type 2 and 3 patients, QTc at rest and that after exercise was long, and that on Holter ECG tended to be much longer. CONCLUSIONS: The present method of analyzing QTc on Holter ECG, in which automatic and manual measurements are combined, is practical and may be useful for diagnosis and risk stratification of LQTS.

Initiation and outcomes with Class Ic antiarrhythmic drug therapy.

BACKGROUND: Expert opinion recommends performing exercise testing with initiation of Class Ic antiarrhythmic medication. OBJECTIVE: To evaluate the rate and reason for discontinuation of Ic agent within the first year of follow up, with particular attention to rate of proarrhythmia and the value of routine treadmill testing. METHODS: This is a single center retrospective cohort study including consecutive patients with atrial arrhythmias who were initiated on a Class Ic agent from 2011 to 2016. Data was collated from chart review and pharmacy database. RESULTS: The study population included 300 patients (55% male, mean age 61; mean ejection fraction, 56%) started on flecainide (n = 153; 51%) and propafenone (n = 147; 49%). Drug initiation was completed while hospitalized on telemetry and the staff electrophysiologists directed dosing. There was one proarrhythmic event during initiation (0.3%). The primary reason for not being discharged on Ic agent was due to detection of proarrhythmia (n = 15) or ischemia (n = 1) with treadmill testing (5.3%). Exercise testing was the single significant variable to affect the decision to discontinue Ic drug, p < 0.0001 (95% CI: 1.89-6.08%). During follow up, the primary reason for discontinuation of Ic agent was lack of efficacy, 32%. CONCLUSIONS: With proper screening, initiation of Class Ic agent is associated with very low rate of proarrhythmia. Treadmill testing is of incremental value and should be completed in all patients after loading Class Ic antiarrhythmic.

Gender disparities in the functional significance of anemia among apparently healthy adults.

BACKGROUND: Data on the functional impact of anemia on cardiorespiratory fitness (CRF) and survival in healthy individuals are limited. Our aim was to evaluate the association between anemia thresholds, low CRF, and survival in otherwise healthy adults. METHODS: Study population included 16 334 apparently healthy subjects attending annual periodic health screening examinations (71 200 annual visits), including exercise stress testing (EST). Anemia was defined by the World Health Organization (WHO) or Beutler and Waalens' (BW) criteria. Low CRF was defined as the lowest fitness quintile according to the Bruce protocol. RESULTS: The mean age was 46±10 years, and 70% were men. Mean Hb levels were 13±1 and 15±1 among women and men, respectively, with higher proportion of anemia among women. The majority of anemic subjects had mild anemia. When analyzing repeated annual visits, anemia was associated with a significant 39% and 64% increased risk of low CRF according the WHO and BW criteria only in women (n=18 672). Baseline anemia at first visit was associated with 2.6- and 1.9-fold increased risk of all-cause mortality using the WHO and BW criteria, exclusively in men (n=11 511). CONCLUSIONS: Overall, the functional and prognostic impact of anemia is gender dependent, based on the WHO and BW arbitrary criteria, suggesting differing mechanism and responses.

The short-term impact of the catheter ablation on noninvasive autonomic nervous system parameters in patients with paroxysmal atrial fibrillation.

BACKGROUND: The autonomic nervous system (ANS) is a potentially potent modulator of the initiation and perpetuation of atrial fibrillation (AF), whereas the presence of AF can activate and alter the ANS. The catheter ablation of AF (AFCA) may cause the cardiac ANS dysfunction, whereas restoration of sinus rhythm or sympathovagal imbalance by AFCA can reverse this process. Our principal goal was to investigate the short-term effect of AFCA on ANS functions evaluated by noninvasive chronotropic (CI), resting heart rate (RHR), and heart rate recovery (HRR) indices. METHOD: A total of 45 patients were enrolled with symptomatic, drug refractory paroxysmal AF undergoing first cryoballoon (CB) pulmonary vein antrum isolation (PVAI) with one 28-mm CB using single 3-minute freeze techniques without bonus applications. All patients underwent symptom-limited exercise treadmill testing to evaluate noninvasive parameters of ANS before PVAI. For those patients who remained in sinus rhythm, an additional exercise test was repeated after 1 and 3 months after discharge. RESULTS: The autonomic CI and RHR/HRR indices were impaired after PVAI and persisted post-PVAI 3 months. However, these parameters were not different in patients with and without recurrence. CONCLUSION: This study demonstrated that the successful AFCA might concurrently impair the ANS parameters. The autonomic imbalance between the sympathetic and parasympathetic activity after AFCA could either become antiarrhythmic and/or proarrhythmic based on which of the two components was going to prevail after successful AFCA. The impaired ANS balance after PVAI might also be another hypothetical mechanism for AF recurrence particularly in the absence of PV reconnection.

Evaluation of Serial High Sensitivity Troponin T Levels in Individuals Without Overt Coronary Heart Disease Following Exercise Stress Testing.

BACKGROUND: Detectable levels of high sensitivity (cardiac) troponin T (HsTnT), occur in the majority of patients with stable coronary heart disease (CHD), and often in 'healthy' individuals. Extreme physical activity may lead to marked elevations in creatine kinase MB and TnT levels. However, whether HsTnT elevations occur commonly after exercise stress testing (EST), and if so, whether this has clinical significance, needs clarification. METHODS: To determine whether HsTnT levels become elevated after EST (Bruce protocol) to ≥95% of predicted maximum heart rate in presumed healthy subjects without overt CHD, we assayed HsTnT levels for ∼5h post-EST in 105 subjects (median age 37 years). RESULTS: Pre-EST HsTnT levels <5 ng/L were present in 31/32 (97%) of females and 52/74 (70%) of males. Post-EST, 13 (12%) subjects developed HsTnT levels >14 ng/L, with troponin elevation occurring at least three hours post-EST. Additionally, a detectable ≥ 50% increase in HsTnT levels (4.9→9ng/L) occurred in 28 (27%) of subjects who during EST achieved ≥ 95% of their predicted target heart rate. The median age of the subjects with HsTnT elevations to > 14ng/L post-EST was higher than those without such elevation (42 and 36 years respectively; p=0.038). At a median follow-up of 13 months no adverse events were recorded. CONCLUSION: The current study demonstrates that detectable elevations occur in HsTnT post-EST in 'healthy' subjects without overt CHD. Future studies should evaluate the clinical significance of detectable elevations in post-EST HsTnT with long-term follow-up for adverse cardiac events.