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Review
. 2017 Dec 12;6(12):118.
doi: 10.3390/jcm6120118.

Hypertrophic Cardiomyopathy-Past, Present and Future

Alphonsus C Liew  1 , Vassilios S Vassiliou  2   3   4 , Robert Cooper  5 , Claire E Raphael  6   7
Affiliations
Review

Hypertrophic Cardiomyopathy-Past, Present and Future

Alphonsus C Liew et al. J Clin Med. .

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with a prevalence of 1 in 500 in the general population. Since the first pathological case series at post mortem in 1957, we have come a long way in its understanding, diagnosis and management. Here, we will describe the history of our understanding of HCM including the initial disease findings, diagnostic methods and treatment options. We will review the current guidelines for the diagnosis and management of HCM, current gaps in the evidence base and discuss the new and promising developments in this field.

Keywords: HCM; LVOT; hypertrophic cardiomyopathy; left ventricular outflow tract obstruction.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Brockenbrough sign after a premature ventricular contraction (PVC) in HCM (Hypertrophic cardiomyopathy). Pulse pressure, as recorded in the femoral artery, decreases in the post-extrasystolic beat (circled) while intracavity pressure in the LV (Left ventricle) increases due to greater obstruction in the LVOT (Left ventricular outflow tract). (Reproduced from [12] with permission).
Figure 2
Figure 2
(A) Schematic demonstrating how left ventricular hypertrophy (LVH) with unfavourable mitral valve anatomy may result in obstruction of the left ventricular outflow tract. Echocardiography allows high quality imaging of the left ventricular outflow tract due to systolic anterior motion of the anterior mitral valve leaflet; (B) Abnormal motion of the mitral valve is well demonstrated on M-mode echocardiography. This may result in high velocities through the LVOT seen on continuous wave Doppler imaging as seen in (C). LA—Left atrium. MV—Mitral valve. LV—Left ventricle. LVOT—Left ventricular outflow tract, LVH—Left ventricular hypertrophy, Ao—Aort.
Figure 2
Figure 2
(A) Schematic demonstrating how left ventricular hypertrophy (LVH) with unfavourable mitral valve anatomy may result in obstruction of the left ventricular outflow tract. Echocardiography allows high quality imaging of the left ventricular outflow tract due to systolic anterior motion of the anterior mitral valve leaflet; (B) Abnormal motion of the mitral valve is well demonstrated on M-mode echocardiography. This may result in high velocities through the LVOT seen on continuous wave Doppler imaging as seen in (C). LA—Left atrium. MV—Mitral valve. LV—Left ventricle. LVOT—Left ventricular outflow tract, LVH—Left ventricular hypertrophy, Ao—Aort.
Figure 3
Figure 3
Hypertrophic cardiomyopathy imaged using cardiovascular magnetic resonance (CMR). (A) Short axis through the heart and asymmetrical left ventricular hypertrophy (starred); (B) Four chamber view of the heart; (C) Late gadolinium imaging showing patchy fibrosis of the hypertrophied area; (D) Same heart in the long axis. LV—Left ventricle, RV—Right ventricle, LA—Left atrium, RA—Right atrium.
See this image and copyright information in PMC

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