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European Journal of Echocardiography 2008 9(2):344-345; doi:10.1093/ejechocard/jen018
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Significant obstruction of the right and left ventricular outflow tract in a patient with biventricular hypertrophic cardiomyopathy

Thomas Butz1,*, Dieter Horstkotte1, Christoph Langer1, Hermann Esdorn2, Georg Kleikamp3, Reiner Körfer3 and Lothar Faber1

1 Department of Cardiology, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Georgstrasse 11, D-32545 Bad Oeynhausen, Germany
2 Institute of Radiology, Nuclear Medicine and Molecular Imaging, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany
3 Department of Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany

Received 18 October 2007; accepted after revision 7 November 2007.

* Corresponding author. Tel: +49 5731 971258; fax: +49 5731 972194. E-mail address: akohlstaedt{at}hdz-nrw.de


   Abstract
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Echocardiography demonstrated pronounced asymmetric left ventricular (LV) hypertrophy and thickened right ventricular muscular components in a 54-year-old woman with a history of dyspnoea (NYHA III), and recurrent syncopes. Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre.

Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) provided striking images of a right ventricular outflow tract obstruction and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis. Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to right ventricular (RV) outflow was due to massive hypertrophy of the crista supraventricularis with an ‘hour-glass’ deformity. A surgical intervention with LV septal myotomy-myectomy and RV ventriculotomy was performed successfully.

Hypertrophic obstructive cardiomyopathy with significant RV and LV outflow tract obstruction is a very rare finding.

Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because mechanisms of obstruction are different in each ventricle.

Keywords: Biventricular hypertrophic cardiomyopathy; Outflow tract obstruction; Right ventricle; Left ventricle

Hypertrophic obstructive cardiomyopathy (HOCM) with significant left and right ventricular (RV and LV) outflow tract obstruction is a very rare finding.

A 54-year-old woman with a history of HOCM, significant LV outflow obstruction despite a β-blocker-medication, dyspnoea (New York Heart Association grade III), and recurrent syncopes was referred to our hospital for septal ablation (PTSMA, TASH).1,2

Echocardiography demonstrated pronounced asymmetric LV hypertrophy and thickened RV muscular components (crista supraventricularis; Figure 1, see Supplementary material online, Movies S1 and S2).3 Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre.


Figure 1
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  		Figure 1 Echocardiographic short-axis view of the right ventricular outflow with a hypertrophied ventricular septum and crista supraventricularis (arrow); LV, left ventricle; RV, right ventricle; RVOT, right ventricular outflow tract.

 
Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) performed at 1.5 T provided striking images of the RV outflow tract obstruction (Figure 2, see Supplementary material online, Movie S3) and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis (Figure 3). Magnetic resonance imaging proved superior to two-dimensional echocardiography for visualization of the RV outflow obstruction.


Figure 2
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  		Figure 2 Cardiac cine T1 weighted MRI (CMR, 1.5 T; balanced TFE resp. SSFP; short-axis view) providing striking images of the right ventricular outflow tract obstruction. LV, left ventricle; RV, right ventricle; PV, pulmonary valve.

 


Figure 3
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  		Figure 3 Cardiac gadolinium-enhanced short-axis view MRI (CMR, 1.5 T; late enhancement) showing a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis (arrow). LV, left ventricle; RV, right ventricle; RVOT, right ventricular outflow tract.

 
Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to RV outflow was due to massive hypertrophy of the crista supraventricularis with an ‘hour-glass’ deformity (Figure 4, see arrow; see Supplementary material online, Movie S4).


Figure 4
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  		Figure 4 Impediment to right ventricular outflow was due to massive hypertrophy of the crista supraventricularis with an ‘hour-glass’ deformity (arrow) shown by right ventricular angiography (LAO 90°, caudal 0°). PV, pulmonary valve.

 
A surgical intervention with LV septal myotomy–myectomy and RV ventriculotomy was performed successfully and the post-operative course showed a very good functional result.

There has been little agreement on the incidence of RV outflow obstruction in patients with HOCM, varying from 15 to 92% as documented by catherization in previous studies.3,4

Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because the mechanism of obstruction is different in each ventricle.5 In contrast to LV outflow obstruction, which is characteristically dynamic and due to mitral valve systolic anterior motion, RV obstruction is the result of a static and fixed impediment to outflow by excessive hypertrophy and increased mass of RV muscular components.


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Supplementary material associated with this article can be found in the online version.

Conflict of interest: none declared.


   References
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 Abstract
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 References
 

  1. Faber L, Seggewiss H, Gleichmann U. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: results with respect to intraprocedural myocardial contrast echocardiography. Circulation (1998) 98:2415–21.[Abstract/Free Full Text]
  2. Faber L, Seggewiss H, Fassbender D, Bogunovic N, Strick S, Schmidt HK, et al. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: acute results in 66 patients with reference to myocardial contrast echocardiography. Z Kardiol (1998) 87:191–201.[CrossRef][Web of Science][Medline]
  3. Shimizu M, Kawai H, Yokota Y, Yokoyama M. Echocardiographic assessment of right ventricular obstruction in hypertrophic cardiomyopathy. Circ J (2003) 67:855–60.[CrossRef][Web of Science][Medline]
  4. Doshi SN, Kim MC, Sharma SK, Fuster V. Right and left ventricular outflow tract obstruction in hypertrophic cardiomyopathy. Circulation (2002) 106:3–4.[CrossRef]
  5. Maron BJ, McIntosh CL, Klues HG, Cannon RO, Roberts WC. Morphologic basis for obstruction to right ventricular outflow in hypertrophic cardiomyopathy. Am J Cardiol (1993) 71:1089–94.[CrossRef][Web of Science][Medline]

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