LETTER TO THE EDITOR
Long-term outcome in patients with apical hypertrophic cardiomyopathy
Edward G. Abinader, MD, FRCPI*
* Bnai Zion Medical Center, Department of Cardiology, Medical Faculty, Technion, 47 Golomb Street, Haifa 31048, Israel
We read with interest the study by Eriksson et al. (1), which described the long-term outcome in patients with apical hypertrophic cardiomyopathy (A-HCM). The characteristic hallmarks of A-HCM as originally described (2) included the presence of giant negative T waves and a spade-like configuration at end-diastole on left ventriculography. Only 47% of their patients had giant negative T waves at baseline. Other forms of so-called A-HCM such as that described by Maron et al. (3) relate to cases with apical distribution of septal hypertrophy with mid-ventricular constriction rather than the typical concentric apical hypertrophy of the Japanese form. They offer a very different clinical presentation, electrocardiogram, morphology, and prognosis (4,5).
As described initially in Japan, A-HCM is also found in non-Japanese patients (4–6) provided one adheres to the original criteria (2). In a longitudinal study fulfilling the exact "Japanese" criteria of A-HCM, patients were followed for 5 to 20 years (6). Sustained ventricular tachycardia in 18% and atrial fibrillation in another 18% of patients appeared as a very late complication. The T-wave negativity was reduced in 36% and loss of R-wave voltage was found in 27% of cases. Thus, patients may lose some of their electrocardiographic diagnostic characteristics and may develop life-threatening arrhythmias (6,7). One such patient with normal coronary arteries who had an automatic internal pacemaker defibrillator implanted for recurrent ventricular tachycardia developed an apical aneurysm with clot and has since died of severe pulmonary edema. Moreover, knowledge of the possibility of late aneurysmatic formation in A-HCM in the absence of coronary artery disease is mandatory to avoid the misdiagnosis of congenital defects in the form of Cantrell’s syndrome (8,9).
In our study, reduction in T-wave negativity and R-wave voltage was not associated with myocardial infarction (MI) as reported by the investigators (1). In what percentage of their patients were these electrocardiographic changes associated with MI?
Lacking long-term studies and owing to the rarity of true A-HCM outside Japan, the prognosis has generally been considered benign based primarily on Japanese observations. However, considering recent Japanese experience (7) and our own (6) in addition to that of Eriksson et al. (1), in whose patients one-third did develop "unfavorable clinical events and potentially life-threatening complications, such as myocardial infarction, arrhythmia and stroke," all suggest that a complacent approach in this entity is unsafe. Close long-term follow-up is essential to reveal and manage in time potentially fatal complications, particularly in the elderly. Additional studies are needed to investigate further the genetic basis of A-HCM and to define risk-stratification strategies. In our opinion, A-HCM can no longer be viewed as a benign entity neither inside nor outside Japan.
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References
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1. Eriksson MJ, Sonnenberg B, Woo A, et al. Long-term outcome in patients with apical hypertrophic cardiomyopathy. J Am Coll Cardiol. 2002;39:638–645[Abstract/Free Full Text]
2. Yamaguchi M, Ishimura T, Nishiyama S, et al. Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients. Am J Cardiol. 1979;44:401–412[CrossRef][Medline]
3. Maron BJ, Bonow RO, Seshagiri TN, Roberts WC, Epstein SE. Hypertrophic cardiomyopathy with ventricular septal hypertrophy localized to apical region of the left ventricle (apical hypertrophic cardiomyopathy). Am J Cardiol. 1982;49:1838–1848[CrossRef][Medline]
4. Abinader EG. Apical hypertrophic cardiomyopathy. Am J Cardiol. 1983;51:1570–1571[Medline]
5. Chia BL, Tan LK. Apical hypertrophic cardiomyopathy. Am J Cardiol. 1984;53:1413[Medline]
6. Abinader EG, Sharif D, Shefer A, Naschitz J. Novel insights into the natural history of apical hypertrophic cardiomyopathy during long-term follow-up. Isr Med Assoc J. 2002;4:166–169[Medline]
7. Okishige K, Sasano T, Yano K, Azegami K, Suzuki K, Itoh K. Serious arrhythmias in patients with apical hypertrophic cardiomyopathy. Intern Med. 2001;40:396–402[Medline]
8. Gruberg L, Goldstein SA, Pfister AJ, Monsein LH, Evans DM, Leon MB. Cantrell’s syndrome: left ventricular diverticulum in an adult patient. Circulation. 2000;101:109–110[Free Full Text]
9. Abinader EG, Sharif D. Cantrell’s syndrome: left ventricular diverticulum in an adult patient. Circulation. 2001;103:e89[Free Full Text]
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M. Refaat
Prognosis of Apical Hypertrophic Cardiomyopathy
JAMA,
November 7, 2007;
298(17):
2006 - 2006.
[Full Text]
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