Echocardiographic Assessment of Right Ventricular Obstruction in Hypertrophic Cardiomyopathy

Shimizu, Masatoshi; Kawai, Hiroya; Yokota, Yoshiyuki; Yokoyama, Mitsuhiro

doi:10.1253/circj.67.855

Cited by 26 publications

(17 citation statements)

References 21 publications

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“…A high flow velocity signal was detected via color flow mapping, confirming the obstruction location. Maximal apical wall thickness was evaluated using apical 2-chamber and apical 4-chamber views at end-diastole, and the mean values of these parameters were measured [14]. …”

Section: Methodsmentioning

confidence: 99%

The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

et al. 2017

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IntroductionSevere right ventricular hypertrophy (SRVH) is a rare phenotype in hypertrophic cardiomyopathy (HCM) for which limited information is available. This study was undertaken to investigate the clinical, prognostic and genetic characteristics of HCM patients with SRVH.MethodsHCM with SRVH was defined as HCM with a maximum right ventricular wall thickness ≥10 mm. Whole-genome sequencing (WGS) was performed in HCM patients with SRVH. Multivariate Cox proportional hazards regression models were used to identify risk factors for cardiac death and events in HCM with SRVH. Patients with apical hypertrophic cardiomyopathy (ApHCM) were selected as a comparison group. The clinical features and outcomes of 34 HCM patients with SRVH and 273 ApHCM patients were compared.ResultsCompared with the ApHCM group, the HCM with SRVH group included younger patients and a higher proportion of female patients and also displayed higher cardiovascular morbidity and mortality. The multivariate Cox proportional hazards regression models identified 2 independent predictors of cardiovascular death in HCM patients with SRVH, a New York Heart Association class ≥III (hazard ratio [HR] = 8.7, 95% confidence interval (CI): 1.43-52.87, p = 0.019) and an age at the time of HCM diagnosis ≤18 (HR = 5.5, 95% CI: 1.24-28.36, p = 0.026). Among the 11 HCM patients with SRVH who underwent WGS, 10 (90.9%) were identified as carriers of at least one specific sarcomere gene mutation. MYH7 and TTN mutations were the most common sarcomere mutations noted in this study. Two or more HCM-related gene mutations were observed in 9 (82%) patients, and mutations in either other cardiomyopathy-related genes or ion-channel disease-related genes were found in 8 (73%) patients.ConclusionsHCM patients with SRVH were characterized by poor clinical outcomes and the presentation of multiple gene mutations.

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Section: Methodsmentioning

confidence: 99%

The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

et al. 2017

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“…Small RV outflow tract gradients (less than 30 mmHg) are very often present in patients with HCM [5], but severe RV outflow tract obstruction combined with mid-LV and LV outflow tract obstruction is a very rare condition. In one study [3], RV obstruction at any site (mid-RV, RV outflow tract, or RV apical trabecular obstruction) was present in approximately 15% of patients with HCM. However, only 5.5% of patients had significant gradients (30 mmHg) and none of them had combined double LV obstruction (LV outflow and mid-LV) with significant RV obstruction.…”

Section: Discussionmentioning

confidence: 97%

“…It should also be suspected when a turbulent flow is found at mid-LV in a four-chamber view with an absent or minor systolic anterior motion of the mitral valve. RV outflow tract obstruction may infrequently occur, usually in combination with LV outflow tract obstruction [2][3][4]. Small RV outflow tract gradients (less than 30 mmHg) are very often present in patients with HCM [5], but severe RV outflow tract obstruction combined with mid-LV and LV outflow tract obstruction is a very rare condition.…”

Section: Discussionmentioning

confidence: 99%

Triple type of obstruction in hypertrophic cardiomyopathy

Efthimiadis

Parcharidou

Vassilikos

et al. 2008

Journal of Cardiovascular Medicine

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A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred to our department for sudden death risk assessment. The diagnosis was made by a private cardiologist to whom the patient was presented due to a history of cardiac murmur. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. The patient's blood pressure (BP) was 130/70 mmHg and a grade 3-4/6 systolic ejection murmur was present between the left parasternal border and the apex. The ECG showed a left ventricular (LV) hypertrophy with strain pattern. A two-dimensional echocardiogram revealed an asymmetrical type LV hypertrophy; anterior ventricular septum equal to 35 mm; posterior ventricular septum equal to 20 mm; anterolateral free wall equal to 12 mm; and posterior free wall equal to 6 mm ( Fig. 1). The patient had three types of obstruction; LV outflow obstruction due to systolic anterior motion of both mitral leaflets with a Doppler-estimated 34 mmHg gradient at rest ( Fig. 2a and b); a midventricular obstruction with a Doppler-estimated 55 mmHg gradient at rest but without apical aneurysm or dyskinesia ( Fig. 2a and c); and right ventricular (RV) outflow tract obstruction with a Doppler-estimated 40 mmHg gradient at rest (Fig. 3). The LV end-diastolic dimension was 45 mm and the left atrium was 45 mm. The RV dimension was 30 mm and the RV free-wall thickness was 8 mm. No mitral or tricuspid regurgitation was detected. The patient had a normal BP response on exercise test with a VO 2max of 20 ml/kg per minute and no episodes of nonsustained ventricular tachycardia in 24-h ECG recording. Cardiac magnetic resonance imaging (MRI) showed a gross late enhancement at the septum. On the basis of the extreme degree of LV hypertrophy, an implantable cardioverter defibrillator (ICD) was implanted prophylactically for primary prevention of sudden death. After an uneventful 6-month period, a routine ICD-device interrogation revealed an episode of sustained monomorphic ventricular tachycardia suppressed by ICD antitachycardia pacing. DiscussionThis is a very rare case of a triple type of obstructive HCM (mid-LV, LV outflow tract, and RV outflow tract) with severe LV hypertrophy, normal RV free-wall thickness and ventricular tachycardia suppressed by ICD antitachycardia pacing. HCM is considered predominantly a disease of LV outflow tract obstruction in which 70% of patients present LV outflow obstruction at rest and with exercise [1].Midcavity obstruction is due to the systolic apposition of hypertrophied papillary muscle and LV wall at the level of the mid-LV, producing two distinct LV chambers. It may be easily overlooked during echocardiography but a modified left two-chamber apical view can detect the narrow neck between basal and apical segments. It should also be suspected when a turbulent flow is found at mid-LV in a four-chamber view with an absent or minor systolic anterior motion of the mitral valve. Case ...

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“…1 Although it is difficult to differentiate between a DCRV with no other anomaly and an intraventricular RV obstruction in hypertrophic cardiomyopathy (HCM), we think that the absence of marked thickening of the ventricular septum in this case is supportive of the diagnosis of DCRV. 8 The only established therapy for DCRV is the surgical removal of the anomalous muscle band, and no medical therapy has been established to reduce the intraventricular pressure gradient. 1 In the present case, we demonstrated for the first time the synergetic effect of RV apical pacing and cibenzoline in reducing the RV pressure gradient in DCRV.…”

Section: Images In Cardiovascular Medicine Harimura Y Et Almentioning

confidence: 99%

Utility of 320-Slice Multi-Detector Computed Tomography for the Diagnosis and Evaluation of Cardiac Structures in a Patient With a Double-Chambered Right Ventricle

Harimura

Tada

Ishizu

et al. 2011

Circ J

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Figure 1. (A) 320-slice multidetector computed tomography (MDCT): coronal section through the right ventricular outflow tract (RVOT) indicating hypertrophy of the right ventricle (RV) wall, an anomalous muscle band dividing the RV, and a post-stenotic dilated RV outflow trunk. (B) 320-slice MDCT: sagittal section through the RVOT. Hypertrophy of the infundibulum and RV free wall and anomalous muscle band resulted in the division of the RV into 2 chambers. Note that severe stenosis was found within the RV in the end-systole images (arrows). All examinations were performed using an Aquilion ONE CT scanner (Toshiba, Otawara, Japan). Contrast medium (Iopamiron, Nihon Shering, Osaka, Japan; 65 ml) was given i.v. at the rate of 4.0 ml/s. A bolus tracking program was used to time the start of the scanning after the contrast material injection. The trigger threshold level was set at an increase of 100 HU over the baseline CT number of the ascending aorta. The parameters were as follows: 120 kV; 350 mA; prospective ECG gating; 0-100% phase window; 16-cm craniocaudal coverage; detector configuration, 320×0.5 mm; gantry rotation time, 350 ms). IMAGES IN CARDIOVASCULAR MEDICINE

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Echocardiographic Assessment of Right Ventricular Obstruction in Hypertrophic Cardiomyopathy

Cited by 26 publications

References 21 publications

The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

Triple type of obstruction in hypertrophic cardiomyopathy

Utility of 320-Slice Multi-Detector Computed Tomography for the Diagnosis and Evaluation of Cardiac Structures in a Patient With a Double-Chambered Right Ventricle

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