Echocardiographic and Doppler Assessment of Hypertrophic Cardiomyopathy

doi:10.1016/S0894-7317(88)80062-2

Journal of the American Society of Echocardiography

Volume 1, Issue 1, January–February 1988, Pages 31-47

https://doi.org/10.1016/S0894-7317(88)80062-2 Get rights and content

Abstract

Echocardiographic and Doppler studies are invaluable in the detection and management of patients with hypertrophic cardiomyopathy, since virtually all aspects of the disorder can be evaluated. Echocardiography is the primary method of diagnosis, and hemodynamic studies are required usually only when surgery is contemplated. All patients should if possible undergo complete M-mode, two-dimensional, Doppler, and color flow studies to define the extent of hypertrophy, the hemodynamic subgroup, quantitation of left ventricular outflow obstruction, mitral regurgitation when present, and an assessment of diastolic function. Patients undergoing medical or surgical therapy for this disorder should have repeat studies performed to assess the effects of therapy on the degree of obstruction and diastolic dysfunction.

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Cited by (79)

Surgical Ventricular Septal Myectomy for Patients with Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction
2015, American Journal of Cardiology
Citation Excerpt :
Preoperative and postoperative transthoracic echocardiography (TTE) was performed using standard equipment in routine clinical practice. Myocardial wall thickness and cardiac dimensions were obtained using previously described methods.10–13 Left ventricular ejection fraction was determined with Simpson's biplane method of disks.14,15
Approximately 20% to 30% of patients with Noonan syndrome (NS) have asymmetric left ventricular hypertrophy (LVH) and LV outflow tract obstruction (LVOTO). The role of surgical myectomy in such patients is unknown. We sought to compare clinical features and outcomes of patients with NS and LVOTO with age- and gender-matched patients with nonsyndromic, obstructive hypertrophic cardiomyopathy (HC) after myectomy. Two cohorts were selected and retrospectively analyzed using Mayo Clinic databases from 1996 to 2014. Subjects included patients with NS with LVH and LVOTO and nonsyndromic controls with obstructive HC. Twenty-three patients with NS and LVH were identified, of whom 12 (8 males) underwent myectomy (10 septal and 2 combined septal/apical) for severe LVOTO (10 pediatric and 2 adults; 13 ± 10 year old [range 1 to 39]). Similar echocardiographic improvements were noted in both groups. There were no perioperative deaths. Residual gradients were slightly higher in patients with NS. No improvement was noted in left atrial volume after myectomy in patients with NS. At early follow-up, the majority showed improvement in the New York Heart Association class (88% in NS vs 82% in HC, median of 6 and 2 months, respectively). At late follow-up (median of 7 years), the survival rate was 92% in NS and 100% in HC. In patients with NS with LVH and symptomatic LVOTO, myectomy reduces both gradient and the New York Heart Association class, similar to patients with nonsyndromic obstructive HC. Residual gradients were slightly higher, and left atrial dilation persisted in patients with NS. In conclusion, myectomy should be considered in patients older than 1 year with NS and symptomatic LVOTO.
Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy: Comparison of conservative versus invasive treatment
2011, Journal of the American College of Cardiology
Citation Excerpt :
The diagnosis of HCM was established by the presence of asymmetric septal hypertrophy (septum ≥13 mm), in the absence of another condition that could account for the degree of hypertrophy observed (5). Echocardiographic data were obtained, as described previously (11), and LVOT gradients were determined by continuous wave Doppler assessment (11,12). Only patients with resting LVOT obstruction, defined as a resting gradient of ≥30 mm Hg, were included.
The aim of this study was to compare the survival of patients with hypertrophic cardiomyopathy (HCM) and resting left ventricular outflow tract (LVOT) obstruction managed with an invasive versus a conservative strategy.
In patients with resting obstructive HCM, clinical benefit can be achieved after invasive septal reduction therapy. However, it remains controversial whether invasive treatment improves long-term survival.
We studied a consecutive cohort of 649 patients with resting obstructive HCM. Total and HCM-related mortality were compared in 246 patients who were conservatively managed with 403 patients who were invasively managed by surgical myectomy, septal ethanol ablation, or dual-chamber pacing.
Multivariable analyses (with invasive therapy treated as a time-dependent covariate) showed that an invasive intervention was a significant determinant of overall mortality (hazard ratio: 0.6, 95% confidence interval: 0.4 to 0.97, p = 0.04). Overall survival rates were greater in the invasive (99.2% 1-year, 95.7% 5-year, and 87.8% 10-year survival) than in the conservative (97.3% 1-year, 91.1% 5-year, and 75.8% 10-year survival, p = 0.008) cohort. However, invasive therapy was not found to be a significant independent predictor of HCM-related mortality (hazard ratio: 0.7, 95% confidence interval: 0.4 to 1.3, p = 0.3). The HCM-related survival was 99.5% (1 year), 96.3% (5 years), and 90.2% (10 years) in the invasive cohort, and 97.8% (1 year), 94.6% (5 years), and 86.9% (10 years) in the conservative cohort (p = 0.3).
Patients treated invasively have an overall survival advantage compared with conservatively treated patients, with the latter group more likely to die from noncardiac causes. The HCM-related mortality is similar, regardless of a conservative versus invasive strategy.
Delayed Left Ventricular Untwisting in Hypertrophic Cardiomyopathy
2009, Journal of the American Society of Echocardiography
Citation Excerpt :
Furthermore, untwisting rate from peak systolic twist to MVO (−31 ± 16°/s vs −46 ± 24°/s, P < .05) was lower in patients with HCM with reverse septal curvature (Table 2). Echocardiography has been used since its early days to gain insight into the complex pathophysiology of HCM, because it provides a practical and comprehensive assessment of cardiac structure and function.16,17 HCM is usually associated with alterations in LV diastolic function, whereas global systolic function is preserved until the later stages of the disease.
Almost all patients with hypertrophic cardiomyopathy (HCM) have some degree of left ventricular (LV) diastolic dysfunction. Nevertheless, the pathophysiology remains incompletely characterized. Conceptually, an ideal therapeutic agent should target the underlying mechanisms that cause LV diastolic dysfunction. Assessment of diastolic LV untwisting could potentially be helpful to gain insight into the mechanism of diastolic dysfunction. The purpose of this study was to investigate LV untwisting in patients with HCM and control subjects.
LV untwisting parameters were assessed using speckle-tracking echocardiography in 75 consecutive patients with HCM and compared with those from 75 healthy control subjects.
Untwisting at 5%, 10%, and 15% of diastole was lower in patients with HCM (all P values < .001) compared with control subjects. Peak diastolic untwisting velocity (−92 ± 32°/s vs −104 ± 39°/s, P < .05) and untwisting rate from peak systolic twist to mitral valve opening (MVO) (−37 ± 20°/s vs −46 ± 22°/s, P < .01) were lower, while the for diastolic duration normalized time-to-peak diastolic untwisting velocity (17 ± 9% vs 13 ± 9%, P < .05) was higher in patients with HCM. Untwisting rate from peak systolic twist to MVO was negatively correlated with the E/A ratio (R² = 0.15, P < .01). Peak diastolic untwisting velocity and untwisting rate from peak systolic twist to MVO were increased in mild but decreased in moderate and severe diastolic dysfunction compared with control subjects.
LV untwisting is delayed in HCM, which probably significantly contributes to diastolic dysfunction.
Echocardiography in the Evaluation and Management of Patients with Hypertrophic Cardiomyopathy
2008, Practice of Clinical Echocardiography, Thrid Edition
Assessment of Mitral Annulus Size and Function by Real-time 3-Dimensional Echocardiography in Cardiomyopathy: Comparison with Magnetic Resonance Imaging
2007, Journal of the American Society of Echocardiography
We sought to assess mitral annular (MA) size and function in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) using real-time 3-dimensional (3D) echocardiography (RT3DE).
The study included 30 patients with HCM, 20 patients with DCM, and 30 control subjects. RT3DE measurements included end-systolic and end-diastolic MA area (MAA) (MAA_3D), MA diameter_3D, MA fractional area change (MAFAC), and MA fractional shortening. In subgroup of 50 patients, magnetic resonance imaging (MRI) was used for MAA_MRI and MA diameter_MRI measurement.
End-diastolic MAA_3D was larger in HCM than in control group (P < .0001). Higher MAFAC and MA fractional shortening were present in HCM than in control group (P = .001 and P = .006, respectively). End-systolic and end-diastolic MAA_3D in DCM were higher than in HCM and control groups (P < .0001). Lower MAFAC and MA fractional shortening were present in DCM than in HCM and control groups (P < .0001). MAFAC correlated well with left ventricular function in control subjects (r = 0.94, P < .0001), whereas correlation was less in DCM (r = 0.53, P = .02) and HCM (r = 0.42, P < .01). RT3DE and MRI measurements were comparable.
RT3DE assessment of MA size and function in control subjects and patients with cardiomyopathy is accurate and well correlated with MRI.
Hypertrophic Cardiomyopathy
2021, Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult: Third Edition

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Echocardiographic and Doppler Assessment of Hypertrophic Cardiomyopathy

Abstract

Am J Cardiol

J Am Coll Cardiol

Prog Cardiovasc Dis

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Mayo Clin Proc

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

Am Heart J

Am J Cardiol

Am J Cardiol

Asymmetrical hypertrophy of the heart in young adults

Br Heart J

Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy

Circulation

Ultrasound in the diagnosis and evaluation of therapy in idiopathic hypertrophic subaortic stenosis

Circulation

Role of echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis

Circulation

Idiopathic hypertrophic subaortic stenosis viewed by wide angle phased-array echocardiography

Circulation

The role of echocardiography in the assessment of hypertrophic cardiomyopathy

Postgrad Med J

Giant negative T-wave inversion as a manifestation of asymmetric apical hypertrophy (AAH) of the left ventricle. Echocardiographic and ultrasonocardiotomographic study

Jpn Heart J