Predominant, Severe Right Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Kręcki, Radosław; Lipiec, Piotr; Piotrowska-Kownacka, Dorota; Chrzanowski, Łukasz; Królicki, Leszek; Drożdż, Jarosław; Krzemińska‐Pakuła, Maria; Kasprzak, Jarosław D.

doi:10.1161/circulationaha.107.703843

Cited by 12 publications

(7 citation statements)

References 5 publications

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“…[6][7][8][9] Although there are guidelines to aid in the clinical management of HCM patients with LVOT obstruction, there are none addressing RVOT obstruction owing to the infrequent nature of the pathology. 1-3 However, RVOT obstruction remains a rare finding despite appreciation of morphological abnormalities of RV wall thickness and mass in HCM patients.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3] However, RVOT obstruction remains a rare finding despite appreciation of morphological abnormalities of RV wall thickness and mass in HCM patients. [6][7][8][9] Although there are guidelines to aid in the clinical management of HCM patients with LVOT obstruction, there are none addressing RVOT obstruction owing to the infrequent nature of the pathology. Subpulmonic gradients have been observed in conjunction with LV outflow obstruction in the past.…”

Section: Discussionmentioning

confidence: 99%

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Hypertrophic Cardiomyopathy with Right Ventricular Outflow Tract and Left Ventricular Intracavitary Obstruction

et al. 2014

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Obstruction of the right ventricular outflow tract (RVOT) is a rare finding in hypertrophic cardiomyopathy (HCM) patients unlike left ventricular outflow tract (LVOT) obstruction. Although there are guidelines that aid in clinical decision making in patients with LVOT obstruction, there are none addressing RVOT obstruction. As RVOT obstruction may pose serious clinical implications similar to LVOT obstruction, appropriate medical and surgical management is very important. A unique phenotype of HCM with RVOT obstruction in conjunction with left ventricle (LV) intracavitary obstruction is discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Hypertrophic Cardiomyopathy with Right Ventricular Outflow Tract and Left Ventricular Intracavitary Obstruction

et al. 2014

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“…The mechanism of RV outflow tract obstruction is not fully understood. Although systolic anterior motion of the tricuspid valve was hypothesized in the early echocardiographic era [6], bulging of hypertrophied ventricular septum in RV outflow [7], and especially the excessive hypertrophy of RV muscular bands [4,8,9] (especially of crista supraventricularis) seem to be the possible mechanisms of RV outflow tract obstruction. Apart from outflow tract, mid-RV hypertrophy and intense RV apex trabeculation may provoke RV obstruction.…”

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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“…Less frequently, obstruction occurs within the mid-ventricle or the apex [2].On the contrary to LV role in the obstructive mechanisms, the involvement of the right ventricle is very uncommon [3,4].A 26-year-old male with family history of HCM and a previous diagnosis (8 years before) of non-obstructive HCM without pharmacological treatment (the patient had refused therapy) was referred to our department for dyspnea and palpitations.Pulmonary auscultation was normal. Cardiac auscultation revealed rude systolic murmur heard best in the pulmonic area with heart rate 76 bpm.…”

mentioning

confidence: 99%

“…On the contrary to LV role in the obstructive mechanisms, the involvement of the right ventricle is very uncommon [3,4].…”

mentioning

confidence: 99%

Right ventricular outflow tract obstruction in hypertrophic cardiomyopathy

Recupero¹,

Bella²,

Patanè³

et al. 2010

International Journal of Cardiology

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Predominant, Severe Right Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Cited by 12 publications

References 5 publications

Hypertrophic Cardiomyopathy with Right Ventricular Outflow Tract and Left Ventricular Intracavitary Obstruction

Hypertrophic Cardiomyopathy with Right Ventricular Outflow Tract and Left Ventricular Intracavitary Obstruction

Triple type of obstruction in hypertrophic cardiomyopathy

Right ventricular outflow tract obstruction in hypertrophic cardiomyopathy

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