Integrated Imaging in Hypertrophic Cardiomyopathy

doi:10.1016/j.amjcard.2016.09.033

The American Journal of Cardiology

Volume 119, Issue 2, 15 January 2017, Pages 328-339

https://doi.org/10.1016/j.amjcard.2016.09.033 Get rights and content

Hypertrophic cardiomyopathy (HC) has a very heterogeneous clinical spectrum and lends itself to multimodality imaging for evaluation and management. This review addresses clinical applications of cardiac imaging in patients with HC. Integrating various techniques of echocardiography and cardiac magnetic resonance (CMR) is discussed in the clinical context such as diagnosis, evaluation, management, risk stratification, and family screening of patients with HC. The utility of periprocedural imaging techniques is highlighted for guiding surgical and transcatheter septal reduction procedures. More limited roles of invasive or computed tomography coronary angiography are discussed for patients with HC with chest pain and risk factors for coronary artery disease. Nuclear techniques although available for decades play a more limited role in contemporary routine management but may assist in risk assessment. Newer CMR and echo imaging techniques are discussed in their emerging roles for further characterization of patients with HC and family members with prospects of preclinical diagnosis. The strengths of the different imaging modalities are presented as well as a flow diagram summarizing integrated imaging in this disease. In conclusion, integrated imaging using the various imaging techniques predominantly echocardiography and CMR based on the clinical picture plays an essential role in the management of patients with HC.

Section snippets

Diagnosis of HC

Patients with HC are often asymptomatic and diagnosed because of a heart murmur, an abnormal electrocardiogram, or incidentally by an echocardiogram performed for unrelated purposes. The diagnosis is also often made during evaluation of cardiac symptoms or screening because of a family history of HC or sudden death. Once HC is suspected, the diagnosis must be confirmed by imaging.

Echocardiography is the principal test for establishing the HC diagnosis, using the criteria of increased LV wall

Evaluation of symptoms

Major pathophysiological mechanisms of symptoms in HC are LV diastolic dysfunction, LVOT obstruction, mitral regurgitation, myocardial ischemia, and arrhythmias.1, 2 Imaging plays an important role in elucidating the cause of symptoms.

Dyspnea results from the complex interplay between diastolic dysfunction, LVOT obstruction, and mitral regurgitation, any or all of which can produce significant pulmonary hypertension. Diastolic dysfunction is assessed echocardiographically by mitral inflow

Management

When patients with HC are determined to have symptoms due to LVOT obstruction, the recommended initial management is medical therapy. The role of imaging in stable patients with HC on medical therapy is mainly relegated to serial evaluations of LV wall thickness, LVOT obstruction, mitral regurgitation, and cavity dimensions and function.1, 2 Assessing LVOT gradients with stress echocardiography is useful to evaluate ongoing symptoms on medical therapy.

Echocardiography is also useful to identify

Disclosures

The authors have no conflicts of interest to disclose.

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Hypertrophic Cardiomyopathy: Current Treatment and Future Options
2023, Current Problems in Cardiology
Hypertrophic cardiomyopathy (HCM) is a disease involving the cardiac sarcomere. It is associated with various disease-causing gene mutations and phenotypic expressions, managed with different therapies with variable prognoses. The heterogeneity of the disease is evident in the fact that it burdens patients of all ages. HCM is the most prevalent cause of sudden death in athletes. However, several technological advancements and therapeutic options have reduced mortality in patients with HCM to 0.5% per year. In addition, rapid advances in our knowledge of the molecular defects accountable for HCM have strengthened our awareness of the disorder and recommended new approaches to the assessment of prognosis. Despite all these evolutions, a small subgroup of patients with HCM will experience sudden cardiac death, and risk stratification remains a critical challenge. This review provides a practical guide to the updated recommendations for patients with HCM, including clinical updates for diagnosis, family screening, clinical imaging, risk stratification, and management.
Echocardiographic Applications of M-Mode Ultrasonography in Anesthesiology and Critical Care
2019, Journal of Cardiothoracic and Vascular Anesthesia
Citation Excerpt :
In general, SAM occurs in hypovolemic and hypercontractile states. The onset and duration of mitral leaflet-septal approach, which is used to grade SAM, are best depicted by M-mode imaging on TTE or TEE.163,164 With TTE, the parasternal long-axis view is used; with TEE, the midesophageal long-axis view is used.
Proficiency in echocardiography and lung ultrasound has become essential for anesthesiologists and critical care physicians. Nonetheless, comprehensive echocardiography measurements often are time-consuming and technically challenging, and conventional 2-dimensional images do not permit evaluation of specific conditions (eg, systolic anterior motion of the mitral valve, pneumothorax), which have important clinical implications in the perioperative setting. M-mode (motion-based) ultrasonographic imaging, however, provides the most reliable temporal resolution in ultrasonography. Hence, M-mode can provide clinically relevant information in echocardiography and lung ultrasound–driven approaches for diagnosis, monitoring, and interventional procedures performed by anesthesiologists and intensivists. Although M-mode is feasible, this imaging modality progressively has been abandoned in echocardiography and is often underutilized in lung ultrasound. This article aims to comprehensively illustrate contemporary applications of M-mode ultrasonography in the anesthesia and critical care medicine practice. Information presented for each clinical application will include image acquisition and interpretation, evidence-based clinical implications in the critically ill and surgical patient, and limitations. The present article focuses on echocardiography and reviews left ventricular function (mitral annular plane systolic excursion, E-point septal separation, fractional shortening, and transmitral propagation velocity); right ventricular function (tricuspid annular plane systolic excursion, subcostal echocardiographic assessment of tricuspid annulus kick, outflow tract fractional shortening, ventricular septal motion, wall thickness, and outflow tract obstruction); volume status and responsiveness (inferior vena cava and superior vena cava diameter and respiratory variability [collapsibility and distensibility indexes]); cardiac tamponade; systolic anterior motion of the mitral valve; and aortic dissection.
Quantification of mitral regurgitation in patients with hypertrophic cardiomyopathy using aortic and pulmonary flow data: Impacts of left ventricular outflow tract obstruction and different left ventricular segmentation methods
2017, Journal of Cardiovascular Magnetic Resonance
Cardiovascular magnetic resonance (CMR) imaging in patients with hypertrophic cardiomyopathy (HCM) enables the assessment of not only left ventricular (LV) hypertrophy and scarring but also the severity of mitral regurgitation. CMR assessment of mitral regurgitation is primarily based on the difference between LV stroke volume (LVSV) and aortic forward flow (Ao) measured using the phase-contrast (PC) technique. However, LV outflow tract (LVOT) obstruction causing turbulent, non-laminar flow in the ascending aorta may impact the accuracy of aortic flow quantification, leading to false conclusions regarding mitral regurgitation severity. Thus, we decided to quantify mitral regurgitation in patients with HCM using Ao or, alternatively, main pulmonary artery forward flow (MPA) for mitral regurgitation volume (MRvol) calculations.
The analysis included 143 prospectively recruited subjects with HCM and 15 controls. MRvol was calculated as the difference between LVSV computed with either the inclusion (LVSV_incl) or exclusion (LVSV_excl) of papillary muscles and trabeculations from the blood pool and either Ao (MRvol_Aoi or MRvol_Aoe) or MPA (MRvol_MPAi or MRvol_MPAe). The presence or absence of LVOT obstruction was determined based on Doppler echocardiography findings.
MRvol_Aoi was higher than MRvol_MPAi in HCM patients with LVOT obstruction [47.0 ml, interquartile range (IQR) = 31.5–60.0 vs. 35.5 ml, IQR = 26.0–51.0; p < 0.0001] but not in non-obstructive HCM patients (23.0 ml, IQR = 16.0–32.0 vs. 24.0 ml, IQR = 15.3–32.0; p = 0.26) or controls (18.0 ml, IQR = 14.3–21.8 vs. 20.0 ml, IQR = 14.3–22.0; p = 0.89). In contrast to controls and HCM patients without LVOT obstruction, in HCM patients with LVOT obstruction, aortic flow-based MRvol (MRvol_Aoi) was higher than pulmonary-based findings (MRvol_MPAi) (bias = 9.5 ml; limits of agreement: −11.7–30.7 with a difference of 47 ml in the extreme case). The differences between aortic-based and pulmonary-based MRvol values calculated using LVSV_excl mirrored those derived using LVSV_incl. However, MRvol values calculated using LVSV_excl were lower in all the groups analyzed (HCM with LVOT obstruction, HCM without LVOT obstruction, and controls) and with all methods of MRvol quantification used (p ≤ 0.0001 for all comparisons).
In HCM patients, LVOT obstruction significantly affects the estimation of aortic flow, leading to its underestimation and, consequently, to higher MRvol values than those obtained with MPA-based MRvol calculations.
Comparison of Pulmonary Venous and Left Atrial Remodeling in Patients With Atrial Fibrillation With Hypertrophic Cardiomyopathy Versus With Hypertensive Heart Disease
2017, American Journal of Cardiology
Citation Excerpt :
The study protocol was approved by the local institutional review board, and all patients provided their written informed consent. The diagnosis of HC was determined by marked LV thickness, a pattern of LV hypertrophy, LV outflow tract obstruction with systolic anterior motion, and a family history of HC.1 The diagnosis of HHD was defined as having hypertension requiring multiple antihypertensive drugs and an LV wall thickness of ≥12 mm.
Left ventricular diastolic dysfunction in hypertrophic cardiomyopathy (HC) increases susceptibility to atrial fibrillation. Although phenotypical characteristics of the hypertrophied left ventricle are clear, left atrial (LA) and pulmonary venous (PV) remodeling has rarely been investigated. This study aimed to identify differences in LA and PV remodeling between HC and hypertensive heart disease (HHD) using 3-dimensional computed tomography. Included were 33 consecutive patients with HC, 25 with HHD, and 29 without any co-morbidities who were referred for catheter ablation of atrial fibrillation. Pre-ablation plasma atrial and brain natriuretic peptide levels, post-ablation troponin T level, and LA pressure were measured, and LA and PV diameters were determined 3 dimensionally. LA transverse diameter in the control group was smaller than that in the HHD or HC group (55 ± 6 vs 63 ± 9 vs 65 ± 12 mm, p = 0.0003). PV diameter in all 4 PVs was greatest in the HC group and second greatest in the HHD group (21.0 ± 3.1 vs 23.8 ± 2.8 vs 26.8 ± 4.1 mm, p <0.0001 for left superior PV). Differences in PV size between the HHD and HC groups were enhanced by indexing to the body surface area (12.4 ± 1.9 vs 13.1 ± 1.4 vs 16.1 ± 3.3 mm/m², p <0.0001). The PV/LA diameter ratio was greater in the HC than in the other groups (0.38 ± 0.06 vs 0.38 ± 0.05 vs 0.42 ± 0.07, p = 0.01). Atrial natriuretic peptide, brain natriuretic peptide, troponin T levels, and LA pressure were highest in the HC group (all p <0.05). In conclusion, the stiff LA caused from atrial hypertrophy may account for higher levels of biomarkers, higher LA pressure, and PV-dominant remodeling in HC.
Assessment of atrial function by myocardial deformation techniques in hypertrophic cardiomyopathy
2021, Echocardiography
Risk stratification in hypertrophic cardiomyopathy
2020, Herz

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ReviewIntegrated Imaging in Hypertrophic Cardiomyopathy

Section snippets

Diagnosis of HC

Evaluation of symptoms

Management

Disclosures

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Thorac Cardiovasc Surg

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Coll Cardiol

J Am Coll Cardiol

J Thorac Cardiovasc Surg

Lancet

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Thorac Cardiovasc Surg

J Am Coll Cardiol

J Am Coll Cardiol

Lancet

J Am Soc Echocardiogr

Hum Pathol

J Am Coll Cardiol

J Am Coll Cardiol

Am Heart J

Am J Cardiol

J Am Coll Cardiol

2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines

Circulation

2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy. The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)

Eur Heart J

Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy

Circulation

Review
Integrated Imaging in Hypertrophic Cardiomyopathy