European Heart Journal Advance Access originally published online on February 28, 2008
European Heart Journal 2008 29(7):948; doi:10.1093/eurheartj/ehn019
Normal systolic function in hypertrophic cardiomyopathy: reality or myth?
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Stilp. Kiriakidi 1
54 636 Thessaloniki
Greece
Tel: +30 2310994830
Fax: +30 2310994673
Email: efthymos{at}med.auth.gr
Laboratory of Histology
Embryology and Anthropology
Aristotle University of Thessaloniki
Thessaloniki
Greece
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Thessaloniki
Greece
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Thessaloniki
Greece
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Greece
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Thessaloniki
Greece
Cardiology Department
AHEPA General Hospital
Aristotle University of Thessaloniki
Thessaloniki
Greece
We read with great interest the article by Ganame et al.1 published in the European Heart Journal. By using strain rate imaging technique, the authors found that the myocardial systolic deformation was significantly and inhomogeneously reduced in children with hypertrophic cardiomyopathy (HCM). The main clinical message of the aforementioned study is that in HCM although left ventricular ejection fraction is normal or supranormal, a subclinical myofibrillar systolic dysfunction exists. A crucial matter is whether this systolic dysfunction precedes the development of hypertrophy. In an experimental study published a decade ago by Geisterfer-Lowrance et al.,2 it was clearly demonstrated that in a mouse model of familial HCM, cardiac dysfunction, by means of reduced myofibrillar shortening, was found to precede histopathological changes, i.e. myocyte disarray, hypertrophy, and fibrosis, and notably when ECG was still normal. Therefore, the classic aspect that myocardial systolic function in HCM is normal or hyperdynamic is rather a ‘myth’. On the other hand, alterations in relaxation pattern detected by pulsed-wave Doppler in healthy first-degree relatives of patients with HCM,3 and reduced myocardial contraction and relaxation velocities detected by tissue Doppler imaging in patients genotype positive for familial HCM, but with no hypertrophy and with a normal ECG,4 have been described.
Which one of the above two myocardial functions precedes cannot easily be determined. In our opinion, ‘pure’ systolic or diastolic dysfunction does not exist since the two phenomena of cardiac cycle are closely interdependent and additionally, myocardial relaxation process involves both systole (systolic relaxation) and diastole (diastolic relaxation). In the current era, we think that the traditionally referred disease phenotype, i.e. hypertrophy, should be reconsidered, taking into account the myocardial systolic or diastolic subclinical abnormalities consistent or not with an abnormal ECG, in the absence of hypertrophy.
Another interesting point of the study is that the reduction in systolic myocardial function was related to maximal wall thickness and decreased exercise capacity. It would be very interesting if the authors provided data on how reduced systolic deformation affected patients' blood pressure response on exercise. The authors also found that the patients who developed ventricular tachycardia had more prominent reduced systolic deformation parameters and higher values of septal E/E' ratio compared with patients with no ventricular tachycardia. Since this cohort was quite small, multivariate analysis in order to predict the prognostic significance of the above parameters was not performed. In a study by our group, a septal E/E' > 15 was found to be an independent predictor of adverse outcome in 96 adult patients with HCM.5
In conclusion, in the view of recently developed techniques, such as tissue Doppler and cardiac MRI, the secrets of HCM have become obvious and familiar to the medical community.
References
- Ganame J, Mertens L, Eidem BW, Claus P, D'hooge J, Havemann LM, McMahon CJ, Elayda MA, Vaughn WK, Towbin JA, Ayres NA, Pignatelli RH. Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations. Eur Heart J (2007) 28:2886–2894.
[Abstract/Free Full Text] - Geisterfer-Lowrance AA, Christe M, Conner DA, Ingwall JS, Schoen FJ, Seidman CE, Seidman JG. A mouse model of familial hypertrophic cardiomyopathy. Science (1996) 272:731–734.[Abstract]
- Efthimiadis GK, Parharidis GE, Karvounis HI, Papadopoulos CE, Gemitzis KD, Styliadis IH, Karoulas TN, Louridas GE. Left ventricular Doppler characteristics in first-degree relatives of patients with hypertrophic cardiomyopathy. Angiology (2005) 56:319–322.
[Abstract/Free Full Text] - Nagueh SF, Bachinski LL, Meyer D, Hill R, Zoghbi WA, Tam JW, Quiñones MA, Roberts R, Marian AJ. Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy. Circulation (2001) 104:128–130.
[Abstract/Free Full Text] - Efthimiadis GK, Giannakoulas G, Parcharidou DG, Karvounis HI, Mochlas ST, Styliadis IH, Papadopoulos CE, Kounatiadis P, Pliakos CI, Parcharidis GE, Louridas GE. Clinical significance of tissue Doppler imaging in patients with hypertrophic cardiomyopathy. Circ J (2007) 71:897–903.[CrossRef][Medline]
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