Resting Echocardiographic Features of Latent Left Ventricular Outflow Obstruction in Hypertrophic Cardiomyopathy

doi:10.1016/S0002-9149(96)00386-6

The American Journal of Cardiology

Volume 78, Issue 6, 15 September 1996, Pages 662-667

https://doi.org/10.1016/S0002-9149(96)00386-6 Get rights and content

Abstract

We determined resting echocardiographic features predictive of latent left ventricular (LV) outflow obstruction in 50 consecutive patients with nonobstructive hypertrophic cardiomyopathy (26 provocable, 24 nonprovocable with amyl nitrite inhalation) to have a better understanding of the pathophysiology of this condition and to identify such patients without pharmacologic provocation. Measurements included wall thickness, type of hypertrophy, LV outflow tract diameter, degree of mitral systolic anterior motion, outflow pressure gradient, and ventricular volume. The direction of the ejection streamline was measured to assess the magnitude of the drag force acting on the mitral valve. Thirteen of 16 patients (81%) with proximal septal bulge were provocable, whereas only 3 of 8 patients (38%) with asymmetric septal hypertrophy and 10 of 26 (38%) with concentric hypertrophy were provocable (p <0.05). LV outflow tract was significantly narrower and the angle between the ejection flow and the mitral valve was larger in provocable patients. The sensitivity for predicting provocable patients by a combination of a narrow outflow tract (≤2 cm) and a large angle ( ≥35°) was 65%, with a specificity of 80% and a positive predictive value of 79%. When these criteria were combined with the presence of septal bulge, the sensitivity was 35%, but the specificity and the positive predictive value were both 100%. Patients with nonobstructive hypertrophic cardiomyopathy with proximal septal bulge, a narrow LV outflow tract, and an oblique angle between the ejection flow and the mitral valve appeared to be predisposed for latent outflow obstruction. These features are consistent with the presence of the large Venturi and drag forces. Thus, the left ventricle, which is capable of increasing both the Venturi and the drag forces on the basis of the morphologic change, contributes to the development of outflow obstruction with amyl nitrite inhalation.

(Am J Cardiol 1996;78:622–667)

Section snippets

METHODS

Provocation with amyl nitrite: In our echocardiography laboratory, patients routinely undergo provocative testing with amyl nitrite inhalation if they have anatomic features of HC[8]with clinical suspicion of intermittent LV outflow tract obstruction but do not demonstrate significant obstruction at rest.[3]Amyl nitrite (Vaporole, Burroughs Wellcome, Research Triangle Park, North Carolina) is administered after crushing a 0.3-ml glass capsule. The patient inhales amyl nitrite from the capsule

RESULTS

Type of hypertrophy: There were 8 patients with asymmetric septal hypertrophy, 26 with concentric hypertrophy, and 16 with proximal septal bulge. In 3 of 8 patients (38%) with asymmetric septal hypertrophy and in 10 of 26 patients (38%) with concentric hypertrophy, LV outflow obstruction was provoked by amyl nitrite inhalation. In contrast, patients with a proximal septal bulge had a significantly higher incidence of latent obstruction; 13 of 16 patients (81%) were provocable (p <0.05 by

DISCUSSION

Effect of the site of hypertrophy: Correlation between the site of hypertrophy and the hemodynamic subgroups has been previously reported. In a study with 100 patients with HC, Wigle et al[1]demonstrated that latent obstruction was found in 72% of patients with localized subaortic hypertrophy. They also showed that when the hypertrophy involved the full-length septum, only 8% of the patients had provocable obstruction. In agreement with their findings, we showed that the incidence of latent

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Systolic anterior motion of mitral valve (MV) leaflets is a main pathophysiologic feature of left ventricular outflow tract (LVOT) obstruction in hypertrophic obstructive cardiomyopathy. Thus, restricted leaflet motion that occurs with MV stenosis might be expected to minimize outflow tract obstruction related to systolic anterior motion.
From January 1993 through February 2015, we performed MV replacement and septal myectomy in 12 patients with mitral stenosis and hypertrophic obstructive cardiomyopathy at Mayo Clinic Hospital in Rochester, Minn. Preoperative data, echocardiographic images, operative records, and postoperative outcomes were reviewed.
Mean (standard deviation) age was 70 (7.6) years. Preoperative mean (standard deviation) maximal LVOT pressure gradient was 75.0 (35.0) mm Hg; MV gradient was 13.7 (2.8) mm Hg. From echocardiographic images, 4 mechanisms of outflow tract obstruction were identified: systolic anterior motion without severe limitation in MV leaflet excursion, severe limitation in MV leaflet mobility with systolic anterior motion at the tip of the MV anterior leaflet, septal encroachment toward the LVOT, and MV displacement toward the LVOT by calcification. Mitral valve replacement and extended septal myectomy relieved outflow gradients in all patients, with no death or serious morbidity.
Patients with mitral stenosis and hypertrophic obstructive cardiomyopathy have multiple LVOT obstruction mechanisms, and MV replacement may not be adequate treatment. We favor septal myectomy and MV replacement in this complex subset of hypertrophic obstructive cardiomyopathy.
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Careful evaluation, including provocation tests, is needed to specify an indication for septal reduction therapy in patients with drug-refractory hypertrophic obstructive cardiomyopathy. This study aimed to evaluate the outcome of alcohol septal ablation (ASA) using an intravenous nitroglycerin test (IV-NTG). Of consecutive 156 patients, after excluding cases of severe valvular disease and repeat septal reduction therapy, we investigated the clinical characteristics of patients with labile obstruction (n = 32) and the outcomes after ASA using the IV-NTG test; comparisons were made with those exhibiting basal obstruction (a resting gradient of ≥30 mm Hg). The patients with labile obstruction had less left ventricular mass (141 ± 47 vs 182 ± 59 g, p = 0.003) and less brain natriuretic peptide values (414 ± 576 vs 744 ± 625 pg/ml, p <0.001) than those with basal obstruction. Immediately after ASA, the gradients improved from 15 ± 7 to 5 ± 5 mm Hg and the IV-NTG-provoked gradients improved from 74 ± 25 to 13 ± 9 mm Hg, respectively. At 1-year follow-up, the New York Heart Association functional class had improved from 2.7 ± 0.5 to 1.3 ± 0.5. There was no sudden cardiac death during the follow-up period (5.1 ± 3.0 years), and 8-year survival free from cardiovascular death was 94%. In conclusion, patients with labile obstruction had less-severe left ventricular hypertrophy but exhibited symptoms comparable to those with basal obstruction. The IV-NTG test is a useful method for rapidly confirming acute reduction of the latent gradient after the ASA procedure, and the outcome of ASA for labile obstruction was favorable.
Differences in global and regional left ventricular myocardial mechanics in various morphologic subtypes of patients with obstructive hypertrophic cardiomyopathy referred for ventricular septal myotomy/myectomy
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Patients with obstructive hypertrophic cardiomyopathy (HC) have various left ventricular (LV) shapes: reverse septal curvature (RSC, commonly familial), sigmoid septum (SS, common in hypertensives), and concentric hypertrophy (CH). Longitudinal (systolic and early diastolic) strain rate (SR) is sensitive in detecting regional myocardial dysfunction. We sought to determine differences in longitudinal SR of patients with obstructive HC, based on LV shapes. We studied 199 consecutive patients with HC (50% men) referred for surgical myectomy. Clinical and echocardiographic parameters were recorded. LV shapes were classified on echocardiography, using basal septal 1/3 to posterior wall ratio: RSC = ratio >1.3 (extending to mid and distal septum), SS = ratio >1.3 (extending only to basal 1/3), and concentric = ratio ≤1.3. Longitudinal systolic and early diastolic SRs were measured from apical 4- and 2-chamber views (VVI 2.0; Siemens, Erlangen). Distribution of RSC, SS, and CH was 50%, 28%, and 22%, respectively. Patients with RSC were significantly younger (47 ± 12 vs 64 ± 10 and 57 ± 11, respectively) with lower hypertension (40% vs71% and 67%, respectively) than patients with SS or CH (both p <0.001). Patients with RSC had lower global systolic (−0.99 ± 0.3 vs −1.05 ± 0.3 and −1.17 ± 0.3) and early diastolic SR (0.95 ± 0.4 vs 0.98 ± 0.3 and 1.16 ± 0.4) versus patients with SS and CH (in 1/s, both p <0.01), despite being much younger and less hypertensive. RSC was associated with abnormal global LV systolic (beta 0.16) and early diastolic (beta −0.17) SR (both p <0.01). In conclusion, patients with HC with RCS have significantly abnormal LV mechanics, despite being younger and less hypertensive. A combination of LV mechanics and shapes could help differentiate between genetically mediated and other causes of obstructive HC.
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Hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy, disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia, which contribute to disease progression. Longitudinal systolic and early diastolic strain rate (SR) measurements by speckle tracking echocardiography are sensitive markers of regional myocardial function. We sought to determine the association between septal SR and histopathologic findings in symptomatic HCM patients who underwent surgical myectomy.
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^*: This study was supported in part by a grant from the Uehara Memorial Foundation, Tokyo, Japan.

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Resting Echocardiographic Features of Latent Left Ventricular Outflow Obstruction in Hypertrophic Cardiomyopathy*

Abstract

Section snippets

METHODS

RESULTS

DISCUSSION

Prog Cardiovasc Dis

Prog Cardiovasc Dis

Am J Cardiol

Am Heart J

Chest

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

Am Heart J

J Am Coll Cardiol

J Am Coll Cardiol