Management of hypertrophic cardiomyopathy
BMJ 2006; 332 doi: https://doi.org/10.1136/bmj.332.7552.1251 (Published 25 May 2006) Cite this as: BMJ 2006;332:1251All rapid responses
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Because of space limitations imposed by the editor, it was difficult
to squeeze into our brief review a complex and heterogeneous disease, as
hypertrophic cardiomyopathy. Therefore, we are glad to have the
opportunity to answer the question of Dr. Anand et al. regarding the
clinical course and prognosis of the apical form of hypertrophic
cardiomyopathy, an issue not addressed in the review. In centres with
experience in the management of hypertrophic cardiomyopathy, it was
generally perceived that patients with hypertrophy confined to the cardiac
apex probably had a more favorable prognosis and a lower rate of sudden
death than patients with a more standard distribution of the left
ventricular hypertrophy. Recently, Eriksson et al. (JACC 2002) have
investigated the clinical course of a particularly large cohort of
patients with the apical form of hypertrophic cardiomyopathy, which
included a total of 105 patients. The results of this study have
confirmed that the apical form of hypertrophic cardiomyopathy is indeed
associated with a low risk of sudden death and cardiovascular mortality.
Dr. Wald asserts that coronary artery disease, rather than
hypertrophic cardiomyopathy, is the most common cause of sudden and
unexpected cardiac death in the young. This criticism is based on the
results of a recent paper published by Dr. Wald on this issue, as well as
on a number of other studies reported in his letter. Our differing
conclusions were based on the recent ACC/ESC guidelines on hypertrophic
cardiomyopathy (JACC 2003) and on the results of a large and systematic
evaluation of the causes of sudden death in competitive athletes (Maron et
al. JAMA 1996). We believe that this discrepancy has two major
explanations. First, the important age difference between the patient
populations discussed. In our review, we were referring to patients in
their “youth” (adolescents or young adults) and to athletes. Instead,
most of the studies quoted by Dr. Wald include adult patients, some with
ages ranging up to 71 years. Second, two-dimensional echocardiography,
the technique which first permitted an immediate identification of the
morphologic features typical of hypertrophic cardiomyopathy, became
available only in the early 1980s’. However, the papers from the
Liberthson review quoted by Dr. Wald in support of his thesis were based
on epidemiological studies performed from the 1950s’ to the early 1980s’
(5), an age in which the diagnosis of hypertrophic cardiomyopathy remained
a major clinical challenge.
Dr. Uriach comments that the combination of beta-blockers and
verapamil may cause bradycardia and could be too risky for patients with
hypertrophic cardiomyopathy. Dr. Uriach also points out that such
treatment strategy is not recommended by the recent ACC/ESC Guidelines on
hypertrophic cardiomyopathy (JACC 2003). In our review, as in others
(Elliott P, McKenna Lancet 2004), the use of either beta blockers or
verapamil, or the combination of both drugs, is suggested exclusively to
control the ventricular rate in patients with HCM and chronic atrial
fibrillation. Conversely, the use of the two drugs combined is not
appropriate for the treatment of heart failure. The statement quoted by
dr. Uriach from the ACC/ESC Guidelines, ”there is no evidence that
combined medical therapy with beta-blockers and verapamil is more
advantageous than the use of either drug alone.”, refers to the control of
symptoms of heart failure.
Competing interests:
None declared
Competing interests: No competing interests
Sir, we read with interest the comprehensive review on Hypertrophic
cardiomyopathy (HCM) by Spirito and Autore (1). However authors do not
sufficiently emphasise the apical variant of HCM, characterized by apical
hypertrophy. This variant is often associated with giant negative T waves
on the electrocardiogram and a “spade shaped” left ventricular cavity on
angiography: it usually has a benign course(2).
References
1.Spirito P, Autore C. Hypertrophic cardiomyopathy. BMJ 2006;352:1251-
1255.
2.
Wynne J,Braunwald E. Cardiomyopathy and myocarditis.
Kasper DL, Braunwald E, Fauci AS, et al Eds. In; Harrison’s principles of
internal medicine. 16 th Ed. Vol II. McGraw-Hill:New York. 2005:1408-1414.
Conflict of interest-none
Source of funding-nil
Kuldip Parkash Anand MD,
Professor and Head,
Department of Medicine,
Command Hospital (Eastern Command),
Kolkata 700 027,
India
*Ajit Singh Kashyap MD *Corresponding author,
Department of Endocrinology,
Command Hospital (Central Command),
Lucknow Cantt 226 002,
India
Surekha Kashyap MD,
Department of Hospital Administration,
Command Hospital (Central Command),
Lucknow Cantt 226 002,
India
Competing interests:
None declared
Competing interests: No competing interests
Dear Sir,
In their excellent review of management of Hypertrophic
Cardiomyopathy, Spirito & Autore [1] have not discussed Apical form of
hypertrophic cardiomyopathy. Approximately 25% of cases of hypertrophic
cardiomyopathy may belong to apical variant in Japan[2]. 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 [3].
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 [3]. Japanese workers have described this
subgroup of hypertrophic cardiomyopathy to be relatively benign[4].
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.
References :
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
Cardiomyopathy).
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 :
p1967-1987.
Competing interests:
None declared
Competing interests: No competing interests
Sudden death is rare in young people with hypertrophic cardiomyopathy
Spirito and Autore, in their review on the management of hypertrophic cardiomyopathy (HCM),1 claim that HCM is
the most common cause of sudden death in young asymptomatic people. This is incorrect. The table summarises the results of 12
studies that provided data on the number of deaths due to HCM in young people,2-5 (nine of which were summarised
by Liberthson3). Less than 10% of all sudden deaths in young people were due to HCM. The commonest cause, despite
the young age, was coronary artery disease.3,4
Most deaths from HCM, contrary to perception, occur in older people.2 In the general young adult population the
annual death rate from previously undiagnosed HCM is one per million or less (table). Among asymptomatic people known to have
the disorder the death rate (case-fatality) is 0.2% per year or less. These results are important with respect to advising
patients of their prognosis.
Spirito and Autore recommend screening for the disorder in families of affected cases but provide no quantitative evidence
to show that this would be worthwhile. It would undoubtedly lead to a large number of diagnosed cases, but few would die
unexpectedly from the disorder and there is no treatment that could reasonably be offered to all.
David S Wald, Senior Lecturer
d.s.wald{at}qmul.ac.uk
Malcolm Law, Professor
Wolfson Institute of Preventive Medicine, Barts and The London, Queen Mary's School of Medicine , London EC1M 6BQ
References
- Spirito P, Autore C. Management of hypertrophic cardiomyopathy. BMJ 2006;332:1251-5
- Wald DS, Law M, Morris JK. Mortality from hypertrophic cardiomyopathy in England and Wales: clinical and screening
implications. Int J Cardiol 2004;97:479-84
- Liberthson RR. Suden death from cardiac causes in children and young adults. N Engl J Med 1996;334:1039-44
- Corrado D, Basso C, Maurizio S, et al. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med
1998;339:364-9
- Takagi E, Yamakado T, Nakano T. Prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy. J
Am Coll Cardiol 1999;33:206-11
Sudden deaths from hypertrophic cardiomyopathy (HCM) in young people
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Competing interests:
None declared
Competing interests: No competing interests
EDITOR - Doctors Spirito and Autore recommend beta- blockers or
verapamil, or the two drugs combined to control heart rate in patients
with hypertrophic cardiomyopathy and chronic atrial fibrillation (1). To
recommend the combination of beta-blockers and verapamil without more
specifications is, in our opinion, too risky. As it is well known, both
beta-blockers and verapamil have negative inotropic effects, which can be
additive. Given together they can cause marked bradycardia and may depress
ventricular contraction (2) as well as increase the risk of AV block (3).
Verapamil can also raise the serum levels of beta-blokers that are
extensively metabolised in the liver (e.g. metoprolol, propranolol),
possibly by inhibiting their metabolism (2). Moreover, as was mentioned in
the ACC/ESC Expert Consensus Document on Hypertrophyc Cardiomyopathy,
published in 2003 (4), there is no evidence that combined medical therapy
with beta-blockers and verapamil is more advategeous that the use of
either drug alone. In our knowledge, the situation has not changed.
We think that currently, when drug safety is an important concern,
authors of medical papers should be very cautious when making this kind of
recommendation.
1.- Spirito P, Autore C. Management of hypertrophyc cardiomyopathy.
Br Med J 2006; 332: 1251-5.
2.- Baxter K (ed.). Stockley's Drug Interactions, 7th ed., London,
Pharmaceutical Press.2006.
3.- López-Sendón J, Swedberg K, McMurray J, Tamargo J, Maggioni A P,
Dargie H, et al. Expert consensus document on beta-adrenergic receptor
blockers. Eur Heart J 2004; 25: 1341-62.
4.- Maron B J, McKenna W J, Danielson G K, Kappemberger L J, Kuhn H
J, Seidman C E, et al. American College of Cardiology/European Society of
Cardiology Clinical Expert Consensus Document on Hypertrophic
Cardiomyopathy. J Am Coll Cardiol 2003; 42: 1687-713.
Competing interests:
None declared
Competing interests: No competing interests
Risk Stratification in hypertrophic cardiomyopathy with cardiovascular magnetic resonance.
Editor,
I read with interest the excellent recent review on the management of
hypertrophic cardiomyopathy (HCM) by Spirito and Autore(1). One area not
touched upon by their article was the emergence of cardiovascular magnetic
resonance (CMR) as a potential tool for sudden death risk stratification
in these patients.
CMR is a rapidly expanding area of cardiology with increasing indications.
It is currently the gold standard for the assessment of cardiac volumes
and dimensions (2) and has long been an established modality for the
assessment of congenital heart disease, pericardial and aortic pathology.
The development of contrast enhanced CMR, along with improved imaging
techniques, allows characterisation of myocardial tissue with a degree of
transmural resolution that is not possible with other imaging modalities.
The contrast agent, Gd-DTPA, accumulates in the extra-cellular space and
causes tissue enhancement on CMR images. It is now known that Gd-DTPA
accumulates in areas of necrosis and fibrosis, due to interstitial
expansion and slowed washout kinetics (3) . This phenomenon is seen in the
“late phase” (i.e. 5-10 minutes) after an intra-venous bolus and is known
as delayed hyperenhancement (DHE). This has been an exploding area of
cardiac imaging over recent years, mostly with respect to ischaemic heart
disease. The DHE technique has also been used to assess myocardial
fibrosis in HCM patients. Moon et al (4) investigated the significance of
the presence of DHE in 53 HCM patients. They found a positive correlation
between the percentage of the left ventricle displaying DHE and the
presence of “classical” factors used to stratify for risk of sudden
cardiac death (SCD) as described in the recent BMJ review. They also noted
the pattern of DHE was significant with a diffuse (as opposed to
confluent) pattern of distribution being associated with the presence of
more SCD risk factors.
Hence it was demonstrated for the first time that the presence of the
classical risk factors for SCD directly correlated to a demonstrable
abnormality of the myocardial interstitium and the degree to which it is
present.
A non invasive imaging technique which can assist in risk stratification
in HCM patients would be a powerful tool but further work is required to
ascertain how CMR could be most effectively integrated into clinical
practice.
Jonathan R Dalzell,
SHO in Cardiology,
Aberdeen Royal Infirmary,
Aberdeen, AB25 2ZN, UK.
References
(1)Spirito P, Autore C. Management of hypertrophic cardiomyopathy.
BMJ 2006;332:1251-55.
(2)Bellenger NG, Burgess MI, Ray SG et al. Comparison of left
ventricular ejection fraction and volumes in heart failure by
echocardiography, radionuclide ventriculography and cardiovascular
magnetic resonance. Are they interchangeable? Eur Heart J 2000;21:1387-96.
(3)Judd RM, Lugo-Olivieri CH, Masazumi A et al. Physiological basis
of myocardial contrast enhancement in fast magnetic resonance images of 2-
day old reperfused canine infarcts. Circulation 1995;92:1902-10
(4)Moon JC, McKenna WJ, McCrohon JA et al. Toward clinical risk
assessment in hypertrophic cardiomyopathy with cardiovascular magnetic
resonance. J Am Coll Cardiol 2003;41:1561-67.
Competing interests:
None declared
Competing interests: No competing interests