Hypertrophic cardiomyopathy: diagnosis and assessment of symptoms

ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Exercise Testing ◽  
Imaging Modality ◽  
Functional Limitation ◽  
Cardiopulmonary Exercise Testing ◽  
Signs And Symptoms ◽  
Left Ventricular ◽  
Myocardial Segment ◽  
Apical Hypertrophy ◽  
Electrocardiogram Monitoring

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.

Hypertrophic cardiomyopathy: diagnosis and assessment of symptoms

ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Exercise Testing ◽  
Imaging Modality ◽  
Functional Limitation ◽  
Cardiopulmonary Exercise Testing ◽  
Signs And Symptoms ◽  
Left Ventricular ◽  
Myocardial Segment ◽  
Apical Hypertrophy ◽  
Electrocardiogram Monitoring

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.

Abstract 16775: Body Mass Index Predicts Exercise Capacity in Patients With Hypertrophic Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Caroline A Ball ◽  
Carolyn M Larsen ◽  
Virginia Hebl ◽  
Jeffrey B Geske ◽  
Kevin C Ong ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Hypertrophic Cardiomyopathy ◽  
Body Mass ◽  
Exercise Testing ◽  
Cardiopulmonary Exercise Testing ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Normal Weight ◽  
Peak Vo2 ◽  
Significant Difference

Introduction: Impaired peak VO2 and obesity are known predictors of morbidity and mortality in Hypertrophic Cardiomyopathy (HCM). The purpose of this study is to determine the degree of exercise impairment due to excess weight in patients with HCM. Methods: Adult HCM patients who underwent cardiopulmonary treadmill testing at our tertiary referral center from 2006 - 2012 and had consented to research participation were identified retrospectively. Percent predicted peak VO2 was calculated by the Astrand formula for men and the Jones formula for women which adjust for age and gender. Baseline echocardiographic features obtained within 1 week of exercise testing and % predicted peak VO2 were compared among four groups of patients stratified by body mass index (BMI). Results: 510 patients were identified, with a mean age at diagnosis of 44.3 ± 16.1 years, 186 (36.5%) female. Mean BMI at the time of cardiopulmonary exercise testing was 29.7 ± 5.3 and 227 (44.6%) patients had a BMI ≥ 30. Overweight and obese patients were older and were more likely to be male than their normal weight peers. However, there was no significant difference in ejection fraction (EF), resting left ventricular outflow tract gradient, right ventricular systolic pressure (RVSP), or septal thickness among the groups. HCM patients show impaired peak VO2 across all BMI groups. While peak VO2 increased progressively across BMI groups consistent with greater O2 demand generated by higher body weight, the adjusted peak VO2 in mL/kg/min fell progressively, indicating progressively greater performance impairment with increasing BMI despite similar degrees of cardiac impairment (p <0.0001) (Table 1). Conclusion: Increased BMI is associated with reduced exercise performance in a graded manner in HCM patients independent of cardiac impairment identified on echocardiography.

Apical hypertrophic cardiomyopathy: what are the risks in our diverse military population?

2018 ◽  
Vol 165 (3) ◽  
pp. 206-209
Author(s):  
Leanne Jane Eveson ◽  
A Williams
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Normal Sinus Rhythm ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Inferior Wall ◽  
Military Population ◽  
Apical Hypertrophic Cardiomyopathy ◽  
Normal Sinus ◽  
Wave Inversion ◽  
Apical Hypertrophy

We present the case of a 50-year-old, fit, asymptomatic gurkha officer. At a routine medical, an ECG showed T-wave inversion in the chest leads V3–6. Transthoracic echo showed left ventricular apical hypertrophy and cavity obliteration consistent with apical hypertrophic cardiomyopathy (ApHCM). Cardiac magnetic resonance imaging showed apical and inferior wall hypertrophy in the left ventricle with no aneurysm or scarring. A 24-hour monitor showed normal sinus rhythm with no evidence of non-sustained ventricular tachycardia. Eighteen-panel genetic testing revealed no specific mutations. Cardiopulmonary exercise testing demonstrated a V̇O2 max, anaerobic threshold and peak V̇O2 consistent with above average cardiopulmonary capacity. There was no family history of either ApHCM or sudden cardiac death (SCD). Risk of SCD by the European Society of Cardiology’s HCM calculator was low. This case generates discussion on the prognosis of ApHCM, factors that worsen prognosis, occupational limitation considerations and appropriate monitoring in this patient group.

Risk stratification in hf with mid-range LVEF: the role of cardiopulmonary exercise testing

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B.M.L Rocha ◽  
G.J Lopes Da Cunha ◽  
P.M.D Lopes ◽  
P.N Freitas ◽  
F Gama ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Exercise Testing ◽  
Primary Endpoint ◽  
Prognostic Significance ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Cardiopulmonary Exercise

Abstract Background Cardiopulmonary exercise testing (CPET) is recommended in the evaluation of selected patients with Heart Failure (HF). Notwithstanding, its prognostic significance has mainly been ascertained in those with left ventricular ejection fraction (LVEF) &lt;40% (i.e., HFrEF). The main goal of our study was to assess the role of CPET in risk stratification of HF with mid-range (40–49%) LVEF (i.e., HFmrEF) compared to HFrEF. Methods We conducted a single-center retrospective study of consecutive patients with HF and LVEF &lt;50% who underwent CPET from 2003–2018. The primary composite endpoint of death, heart transplant or HF hospitalization was assessed. Results Overall, 404 HF patients (mean age 57±11 years, 78.2% male, 55.4% ischemic HF) were included, of whom 321 (79.5%) had HFrEF and 83 (20.5%) HFmrEF. Compared to the former, those with HFmrEF had a significantly higher mean peak oxygen uptake (pVO2) (20.2±6.1 vs 16.1±5.0 mL/kg/min; p&lt;0.001), lower median minute ventilation/carbon dioxide production (VE/VCO2) [35.0 (IQR: 29.1–41.2) vs 39.0 (IQR: 32.0–47.0); p=0.002) and fewer patients with exercise oscillatory ventilation (EOV) (22.0 vs 46.3%; p&lt;0.001). Over a median follow-up of 28.7 (IQR: 13.0–92.3) months, 117 (28.9%) patients died, 53 (13.1%) underwent heart transplantation, and 134 (33.2%) had at least one HF hospitalization. In both HFmrEF and HFrEF, pVO2 &lt;12 mL/kg/min, VE/VCO2 &gt;35 and EOV identified patients at higher risk for events (all p&lt;0.05). In Cox regression multivariate analysis, pVO2 was predictive of the primary endpoint in both HFmrEF and HFrEF (HR per +1 mL/kg/min: 0.81; CI: 0.72–0.92; p=0.001; and HR per +1 mL/kg/min: 0.92; CI: 0.87–0.97; p=0.004), as was EOV (HR: 4.79; CI: 1.41–16.39; p=0.012; and HR: 2.15; CI: 1.51–3.07; p&lt;0.001). VE/VCO2, on the other hand, was predictive of events in HFrEF but not in HFmrEF (HR per unit: 1.03; CI: 1.02–1.05; p&lt;0.001; and HR per unit: 0.99; CI: 0.95–1.03; p=0.512, respectively). ROC curve analysis demonstrated that a pVO2 &gt;16.7 and &gt;15.8 mL/kg/min more accurately identified patients at lower risk for the primary endpoint (NPV: 91.2 and 60.5% for HFmrEF and HFrEF, respectively; both p&lt;0.001). Conclusions CPET is a useful tool in HFmrEF. Both pVO2 and EOV independently predicted the primary endpoint in HFmrEF and HFrEF, contrasting with VE/VCO2, which remained predictive only in latter group. Our findings strengthen the prognostic role of CPET in HF with either reduced or mid-range LVEF. Funding Acknowledgement Type of funding source: None

scholarly journals Role of confirmed and potential predictors of an unfavorable outcome in heart failure in everyday clinical practice

2021 ◽  
Author(s):  
Anna Chuda ◽  
Maciej Banach ◽  
Marek Maciejewski ◽  
Agata Bielecka-Dabrowa
Keyword(s):  
Heart Failure ◽  
Exercise Testing ◽  
Clinical Decision Making ◽  
Cardiopulmonary Exercise Testing ◽  
High Sensitivity ◽  
Left Ventricular ◽  
Unfavorable Outcome ◽  
Left Ventricular Ejection ◽  
Monitoring Method ◽  
Ventricular Ejection Fraction

AbstractHeart failure (HF) is the only cardiovascular disease with an ever increasing incidence. HF, through reduced functional capacity, frequent exacerbations of disease, and repeated hospitalizations, results in poorer quality of life, decreased work productivity, and significantly increased costs of the public health system. The main challenge in the treatment of HF is the availability of reliable prognostic models that would allow patients and doctors to develop realistic expectations about the prognosis and to choose the appropriate therapy and monitoring method. At this moment, there is a lack of universal parameters or scales on the basis of which we could easily capture the moment of deterioration of HF patients’ condition. Hence, it is crucial to identify such factors which at the same time will be widely available, cheap, and easy to use. We can find many studies showing different predictors of unfavorable outcome in HF patients: thorough assessment with echocardiography imaging, exercise testing (e.g., 6-min walk test, cardiopulmonary exercise testing), and biomarkers (e.g., N-terminal pro-brain type natriuretic peptide, high-sensitivity troponin T, galectin-3, high-sensitivity C-reactive protein). Some of them are very promising, but more research is needed to create a specific panel on the basis of which we will be able to assess HF patients. At this moment despite identification of many markers of adverse outcomes, clinical decision-making in HF is still predominantly based on a few basic parameters, such as the presence of HF symptoms (NYHA class), left ventricular ejection fraction, and QRS complex duration and morphology.

The role of cardiopulmonary exercise testing in a contemporary heart failure population

2021 ◽  
Vol 28 (Supplement_1) ◽  
Author(s):  
P Garcia Bras ◽  
A Valentim Goncalves ◽  
J Reis ◽  
T Pereira Da Silva ◽  
R Ilhao Moreira ◽  
...  
Keyword(s):  
Heart Failure ◽  
Risk Stratification ◽  
Exercise Testing ◽  
Predictive Power ◽  
Cardiopulmonary Exercise Testing ◽  
Composite Endpoint ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Discriminative Power ◽  
Cardiopulmonary Exercise

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiopulmonary exercise testing (CPET) is used for risk stratification in patients with chronic heart failure (CHF). However, there is a lack of information regarding CPET prognostic power in patients under new HF therapies such as sacubitril/valsartan, Mitraclip, IV iron or SGLT2 inhibitors. The aim of this study was to evaluate the prognostic value of CPET parameters in a contemporary subset of patients with optimal medical and device therapy for CHF. Methods Retrospective evaluation of patients with CHF submitted to CPET in a tertiary center. Patients were followed up for 24 months for the composite endpoint of cardiac death, urgent heart transplantation or left ventricular assist device. CPET parameters, including peak oxygen consumption (pVO2) and VE/VCO2 slope, were analysed and their predictive power was measured. HF events were stratified according to cut-off values defined by the International Society for Heart and Lung Transplantation (ISHLT) guidelines: pVO2 of ≤12 mL/Kg/min and VE/VCO2 slope of &gt;35. Results CPET was performed in 204 patients, from 2014 to 2018. Mean age was 59 ± 13 years, 83% male, with a mean left ventricular ejection fraction of 33 ± 8%, and a mean Heart Failure Survival Score of 8.6 ± 1.3. The discriminative power of CPET parameters is displayed in the Table. In patients with pVO2 ≤12 mL/Kg/min, the composite endpoint occurred in 18% of patients. A pVO2 value of ≤12 mL/Kg/min had a positive predictive power of 18% while pVO2 &gt;12 had a negative predictive power of 93%. Regarding VE/VCO2 slope &gt;35, the composite endpoint occurred in 13% of patients. A VE/VCO2 slope value of &gt;35 had a positive predictive power of 13% while VE/VCO2 slope &lt;35 had a negative predictive power or 94%. Conclusion Using ISHLT guideline cut-off values for advanced HF therapies patient selection, there was a reduced number of HF events (&lt;20%) at 24 months in patients under optimal CHF therapy. While pVO2 and VE/VCO2 slope are still valuable parameters in risk stratification, redefining cut-off values may be necessary in a modern HF population. Discriminative power of CPET parameters Parameters HR; 95% CI AUC p-value Peak VO2 0.824 (0.728-0.934) 0.781 0.001 Percent of predicted pVO2 0.942 (0.907-0.978) 0.774 0.002 VE/VCO2 slope 1.068 (1.031-1.106) 0.756 0.008 Cardiorespiratory optimal point 1.118 (1.053-1.188) 0.746 0.004 PETCO2 maximum exercise 0.854 (0.768-0.950) 0.775 0.003 Ventilatory Power 0.358 (0.176-0.728) 0.796 0.002 HR Hazard ratio, AUC: Area under the curve, PETCO2: end-tidal CO2 pressure

scholarly journals Impairments in Blood Pressure Regulation and Cardiac Baroreceptor Sensitivity Among Patients With Heart Failure Supported With Continuous-Flow Left Ventricular Assist Devices

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Christine Sailer ◽  
Hannah Edelmann ◽  
Cullen Buchanan ◽  
Pedro Giro ◽  
Matthew Babcock ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Rate ◽  
Exercise Testing ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Advanced Heart Failure ◽  
Ventricular Assist Devices ◽  
Left Ventricular Assist Devices ◽  
Baroreceptor Sensitivity ◽  
Cardiopulmonary Exercise

Background: Continuous-flow (CF) left ventricular assist devices (LVADs) improve outcomes for patients with advanced heart failure (HF). However, the lack of a physiological pulse predisposes to side-effects including uncontrolled blood pressure (BP), and there are little data regarding the impact of CF-LVADs on BP regulation. Methods: Twelve patients (10 males, 60±11 years) with advanced heart failure completed hemodynamic assessment 2.7±4.1 months before, and 4.3±1.3 months following CF-LVAD implantation. Heart rate and systolic BP via arterial catheterization were monitored during Valsalva maneuver, spontaneous breathing, and a 0.05 Hz repetitive squat-stand maneuver to characterize cardiac baroreceptor sensitivity. Plasma norepinephrine levels were assessed during head-up tilt at supine, 30 o and 60 o . Heart rate and BP were monitored during cardiopulmonary exercise testing. Results: Cardiac baroreceptor sensitivity, determined by Valsalva as well as Fourier transformation and transfer function gain of Heart rate and systolic BP during spontaneous breathing and squat-stand maneuver, was impaired before and following LVAD implantation. Norepinephrine levels were markedly elevated pre-LVAD and improved—but remained elevated post-LVAD (supine norepinephrine pre-LVAD versus post-LVAD: 654±437 versus 323±164 pg/mL). BP increased during cardiopulmonary exercise testing post-LVAD, but the magnitude of change was modest and comparable to the changes observed during the pre-LVAD cardiopulmonary exercise testing. Conclusions: Among patients with advanced heart failure with reduced ejection fraction, CF-LVAD implantation is associated with modest improvements in autonomic tone, but persistent reductions in cardiac baroreceptor sensitivity. Exercise-induced increases in BP are blunted. These findings shed new light on mechanisms for adverse events such as stroke, and persistent reductions in functional capacity, among patients supported by CF-LVADs. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03078972.

Abstract 3281: The Prognostic Characteristics of Cardiopulmonary Exercise Testing in Caucasian and African-American Patients with Heart Failure

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ross Arena ◽  
Jonathan Myers ◽  
Mary Ann Peberdy ◽  
Daniel Bensimhon ◽  
Paul Chase ◽  
...  
Keyword(s):  
African American ◽  
Exercise Testing ◽  
Event Rate ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Cardiac Events ◽  
Assist Device ◽  
Heart Transplants ◽  
Ventricular Assist ◽  
Left Ventricular Assist

Introduction: Peak oxygen consumption (VO 2 ) and the minute ventilation (VE)/carbon dioxide production (VCO 2 ) slope are prognostically important in the heart failure (HF) population. Hypothesis: We assessed the hypothesis that the prognostic characteristics of peak VO 2 and the VE/VCO 2 slope would be comparable between Caucasian and African-American subjects with HF. Methods: Four hundred and ninety one HF patients (339 Caucasian/152 African-American) underwent cardiopulmonary exercise testing and were tracked for major cardiac events for three years. Results: The following comparisons are reported as Caucasian vs. African-American subgroups, respectively. Age (56.7 ±14.4 vs. 47.1 ±13.4 years, p<0.001) and ejection fraction (30.6 ±12.9 vs. 25.2 ±11.7%, p<0.001) were significantly lower in the African-American subgroup. Peak VO 2 (15.7 ± 5.6 vs. 14.8 ± 5.7 mlO 2 ·kg −1 ·min −1 , p<0.11) and the VE/VCO 2 slope (35.4 ±9.8 vs. 36.8 ±9.7, p=0.15) were not significantly different. There were 44 (annual event rate: 8.3%) major cardiac events (25 deaths/14 heart transplants/5 left ventricular assist device implantations) in the Caucasian subgroup and 25 (annual event rate: 10.1%) major cardiac events (18 deaths/5 heart transplants/2 left ventricular assist device implantations) in the African-American subgroup. Receiver operating characteristic (ROC) curve analysis and hazard ratios for exercise test variables are listed in Table 1 . Peak VO 2 and the VE/VCO 2 slope were prognostically significant in both subgroups. Conclusions: Despite differences in baseline characteristics between Caucasian and African-American subjects, the optimal prognostic threshold values of established exercise testing variables were similar. The VE/VCO 2 slope was the superior prognostic marker in both subgroups. While, peak VO 2 was prognostically significant in Caucasian and African-American subjects, its value was diminished in the latter subgroup.

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