Is Apical variant of Hypertrophic Cardiomyopathy benign ?
In their excellent review of management of Hypertrophic
Cardiomyopathy, Spirito & Autore  have not discussed Apical form of
hypertrophic cardiomyopathy. Approximately 25% of cases of hypertrophic
cardiomyopathy may belong to apical variant in Japan. It is quite
frequently seen in other tropical countries like India. We in Indian Armed
Forces see it quite frequently as routine ECG is part of annual medical
examination. A ‘horrible’ looking ECG with giant T-wave inversions
(defined as more than 10 mm in depth) is the usual cause of referral to
physician and confirmation of diagnosis by echocardiography. Apical
hypertrophic cardiomyopathy is rare in Western Countries. Strangely mild T
-wave inversions have been reported from West as compared to giant T-wave
inversions in the East .
Does the management of Apical hypertrophic cardiomyopathy differ from
other cases of hypertrophic cardiomyopathy? Sudden death has been reported
to occur in Apical hypertrophic cardiomyopathy patients and in their first
degree relatives in west . Japanese workers have described this
subgroup of hypertrophic cardiomyopathy to be relatively benign.
However, their long term prognosis and risk of sudden cardiac death has
not been clearly defined. Perhaps the management strategies in these cases
will be dictated by patient’s symptoms. Majority of these patients do not
need infective endocarditis prophylaxis as they usually have no features
of obstruction. Should their first degree relatives need to be
aggressively screened specially in countries with scarce health resources
is not very clear.
1. Spirito P & Autore C. Management of Hypertrophic
Cardiomyopathy. BMJ 2006; 332 : 1251-1255.
2. Wynne J & Braunwald E. The Cardiomyopathies. In Zipes DP,
Libby P, Bonow RO& Braunwald E (Eds) Braunwald’s Heart Disease, 7th
ed, Elsevier Saunders 2005: p1659-1696.
3. Maron BJ, Bonow RO, Seshagiri TNR, Roberts WC & Epstein SE.
Hypertrophic Cardiomyopathy With Ventricular Septal Hypertrophy Localized
to the Apical Region of the Left Ventricle (Apical Hypertrophic
Am J Cardiol 1982; 49: 1838-1848.
4. Maron BJ. Hypertrophic Cardiomyopathy in Fuster V, Alexander RW
, O’Rourke RA et al (Eds) Hurst’s The Heart, 10th ed Mcgraw-Hill 2001 :