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Number 10-12: Spiral hypertrophic cardiomyopathy. An unusual distribution of hypertrophy in HCM
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Number 10-12: Spiral hypertrophic cardiomyopathy. An unusual distribution of hypertrophy in HCM

Case from:  Albert Teis, Peter Drivas, Chiara Bucciarelli-Ducci
CMR Unit, Royal Brompton Hospital, London, UK 

Clinical history: A 44 year old African male with a background of severe hypertension for the last 2 years presented with atypical chest pain at the emergency department. The ECG was highly suggestive of hypertrophic cardiomyopathy (HCM), with deep T wave inversion in all antero-lateral leads (Figure 1). The echocardiogram suggested septal asymmetric HCM. He was referred to our institution for a CMR scan to confirm the diagnosis of HCM and to assess the presence and extent of myocardial fibrosis and microvascular dysfunction

 

 

Figure 1-Click to zoom

 


      

Movie 1                                                               Movie 2

 

Figure 2-Click to zoom

 


 

Movie 3

Figure 4-Click to zoom

 

Cine CMR images: short axis cine images showed asymmetrical left ventricular hypertrophy, starting from the basal antero-septum (22mm wall thickness, versus 9mm in the lateral wall). The region of maximal wall hypertrophy progressively rotated counter-clockwise and at the apical level the lateral wall measured 22mm versus 9mm in the septum (Movie 1 and 2; Figure 2, arrows). There was no systolic anterior movement of the mitral valve or LV outflow track obstruction at rest.

Perfusion CMR images: Perfusion images were obtained after 3 minutes of 140µg/min/Kg of adenosine infusion and at rest. There was an almost circumferential subendocardial inducible perfusion abnormality in the apical and mid-cavity segments (Movie 3 and Figure 3, arrows).

Late gadolinium enhancement (LGE): There was patchy myocardial enhancement in the anteroseptal and anterior walls at the mid-cavity level and in the antero-lateral wall in the apical segments, in keeping with rotated counter-clockwise progression of the maximum wall hypertrophy (Figure 4, asterisk).

Perspective: We report a very peculiar spiral distribution of maximal wall hypertrophy in a patient with HCM. A similar anatomical pattern was previously reported but it is unclear whether this should be considered a new entity.1 In our case, both the mid-wall myocardial fibrosis and microvascular dysfunction (increasingly important hallmarks of risk stratification in patients with HCM)2,3 followed the counter-clockwise rotation pattern of the hypertrophy.

Reference:


1. Masci PG, De Bondt J, Bogaert J. Helical form of hypertrophic cardiomyopathy: a new entity? Eur Heart J. 2008;29:706

2. Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ. Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol. 2003;41:1561-7

3. Maron MS, Olivotto I, Maron BJ, Prasad SK, Cecchi F, Udelson JE, Camici PG. The case for myocardial ischemia in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009;54:866-75

 

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COTW handling editor: Rory O' Hanlon

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