South Med J. 2003 Aug;96(8):828-31.
Uncommon cause of a common disease.
Iskandar SB, Dittus K, Merrick D.
Veterans Affairs Medical Center, Mountain Home, TN, USA. firstname.lastname@example.org
Myocardial infarction is a common life-threatening condition. Multiple agents can be used to treat acute coronary syndrome (ACS). These therapeutic agents pose potential life-threatening complications when used outside the realm of the acute coronary syndrome. Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac disorder, occurring in 1 in 500 individuals, which may mimic ACS. The hypertrophy most typically involves the septum in patients with HCM. As many as 25% of Japanese patients with HCM have predominately apical involvement. Apical hypertrophic cardiomyopathy (AHC) occurs in only 1 to 2% of the non-Japanese population. Despite its low incidence, physicians caring for patients with chest pain need to consider AHC in their differential diagnosis. We present the case of a patient with chest pain and electrocardiographic changes suggestive of ACS who was later found to have AHC.
Am J Cardiol. 2003 Nov 15;92(10):1183-6.
Comment in: Am J Cardiol. 2004 Oct 1;94(7):981.
Comparison of prevalence of apical hypertrophic cardiomyopathy in Japan and the United States.
Kitaoka H, Doi Y, Casey SA, Hitomi N, Furuno T, Maron BJ.
Department of Medicine and Geriatrics, Kochi Medical School, Kochi, Japan.
The morphologic apical form of hypertrophic cardiomyopathy (HC), in which left ventricular (LV) wall thickening is confined to the most distal region at the apex, has been regarded as a phenotypic expression of nonobstructive HC largely unique to Japanese patients. To investigate this question further, we directly compared unselected and regional hospital-based cohorts of adult patients with HC ( > or =18 years of age) from Japan (Kochi; n=100) and from the United States (US) (Minneapolis; n=361). Japanese and American patients with HC had similar clinical features and did not differ significantly with regard to the severity of symptoms and frequency of outflow obstruction. Although Japanese and American patients also showed similar maximum LV thickness, they differed significantly with respect to the distribution of LV hypertrophy. In particular, the segmental form of HC, with hypertrophy confined to the LV apex, was more frequent in Japanese patients (i.e., apical HC, 15% in Japan vs 3% in US, p<0.0001). Giant negative T waves were also more common in Japanese patients with HC (26% vs 2%, p<0.001), including those with the apical form (64% vs. 30%, p<0.05). Each patient with apical HC had either no or only mild symptoms, and all survived. The morphologic form of nonobstructive HC with hypertrophy limited to the LV apex (apical form of HC) was 5 times more common in an unselected Japanese population. These findings document variability in the phenotypic expression of HC between countries and races, which may be due to differences in environmental factors or genetic background. Patients with the apical form of HC had a benign clinical course.
Nippon Naika Gakkai Zasshi. 2002 Mar 10;91(3):853-5.
History of cardiology in the last 100 years: Japanese contribution to studies on apical hypertrophy
Jpn Circ J. 1998 Feb;62(2):127-31.
Hypertrophic cardiomyopathy with apical left ventricular aneurysm.
Akutsu Y, Shinozuka A, Huang TY, Watanabe T, Yamada T, Yamanaka H, Saitou T, Geshi E, Takenaka H, Takeyama Y, Munechika H, Ban Y, Katagiri T.
Third Department of Internal Medicine, Showa University School of Medicine, Tokyo, Japan.
We report a case of hypertrophic cardiomyopathy (HCM) with apical left ventricular aneurysm, which is difficult to review because cases are so rare. A 54-year-old Japanese man was first found to have an electrocardiographic abnormality (T-wave inversion at rest) 19 years ago, and non-obstructive apical HCM without identifiable cause was diagnosed by echocardiography, left ventriculography, and clinical findings. After 19 years, he was admitted because of repeated episodes of palpitation and chest oppression at rest. Widespread left ventricular hypertrophy from the anteroseptal wall to the apex with an apical left ventricular aneurysm was detected by echocardiography, left ventriculography, and cardiac magnetic resonance imaging. Histologic examination of the hypertrophic apical myocardium surrounding the aneurysm showed that the myocardial tissue had been extensively replaced by fibrous tissue containing hypertrophic myocardial fibers, and uptakes of [123I]-metaiodobenzyl guanidine (MIBG) and [123I-] beta-methyliodophenyl pentadecanoic acid (BMIPP) in single-photon emission photography images were reduced despite high myocardial perfusion. On the other hand, histologic examination of the hypertrophic anterior wall revealed myocardial hypertrophy with disorganization; myocardial perfusion and the uptakes of MIBG and BMIPP were preserved. Abnormalities of myocardial fatty acid metabolism and sympathetic neuron activity with preserved perfusion flow and histologic changes such as fibrosis in the apical wall are indicative of apical myocardial injury or ischemia (infarction) without coronary artery stenosis; apical aneurysm may have occurred in severe apical HCM with cavity obliteration up to the midventricular level.
J Mol Cell Cardiol. 1997 Feb;29(2):839-43.
Novel missense mutation in cardiac troponin T gene found in Japanese patient with hypertrophic cardiomyopathy.
Nakajima-Taniguchi C, Matsui H, Fujio Y, Nagata S, Kishimoto T, Yamauchi-Takihara K.
Department of Medicine III, Osaka University Medical School, Japan.
Familial hypertrophic cardiomyopathy (HCM) is a primary cardiomyopathy with an autosomal dominant pattern of inheritance. The disease bearing genes for HCM in HCM families have been identified as beta-myosin heavy chain, alpha-tropomyosin, cardiac troponin T (cTnT) and myosin binding protein-C genes. In the present study, we searched for the mutations in the cTnT gene in Japanese HCM patients. Single-strand conformation polymorphism gel analysis of polymerase chain reaction-amplified product was used to search for the mutations in the exons 8, 9 and 15 of the cTnT gene from 60 familial HCM patients. Clinical studies of the family members were performed and the incidence of sudden or disease-related deaths within the family was also examined. We have identified a novel missense mutation in exon 9 (Ala104Val) of the cTnT gene in a patient with familial HCM. Because the missense mutation was found at the residue conserved through chicken to humans and was not detected in the more than 50 normal controls, it was suggested that this missense mutation is the cause of HCM in this family. Although the affected family members presented moderate hypertrophy of the left ventricular wall, they were symptomatic and there was a high incidence of sudden death in her family members. Among Japanese patients with familial HCM, a novel missense mutation (Ala104Val) in the cTnT gene was identified. Familial HCM is genetically heterogeneous in Japanese HCM patients as observed in Caucasian kindreds. The disease in the kindred was severe and there was a high incidence of sudden or disease-related deaths in the kindred with this mutation.
Jpn Heart J. 1997 Sep;38(5):741-8.
Apical hypertrophic cardiomyopathy of the Japanese type coexistent with a coronary muscle bridge. A case report and review.
Giannitsis E, Haase H, Schmucker G, Sheikhzadeh A.
Department of Cardiology, Medical University of Luebeck, Germany.
We describe the case of a 21-year-old Italian male who presented with giant negative T-waves and left ventricular hypertrophy on the electrocardiogram suggestive of apical hypertrophic cardiomyopathy. Clinically, he suffered from new onset of exertional angina, dyspnea and palpitations during soccer playing or heavy exercise beginning one week before admission. Echocardiography and cardiac catheterization conformed the rare combination of a nonobstructive apical hypertrophic cardiomyopathy of the "Japanese" type coexistent with an extensive muscular bridge involving almost the entire anterior interventricular branch of the left coronary artery. Although the patient complained of exertional angina pectoris, absence of objective evidence of myocardial ischemia by means of treadmill stress test, exercise thallium scan, dobutamine stress echocardiography as well as atrial pacing stress emphasized the benign nature of this complex anomaly.
Int J Cardiol. 1996 Sep;56(1):41-51.
Apical hypertrophic cardiomyopathy: a study of 14 patients and their first degree relatives.
Barbosa MM, Coutinho AH, Motta MS, Fortes PR, Roza OZ, God EM.
Hospital Vera Cruz, Belo Horizonte, Brazil.
Although apical hypertrophic cardiomyopathy is generally accepted as a form of hypertrophic cardiomyopathy, its underlying genetic factors, clinical course and complications may be different. The characteristics of 14 Brazilian patients with a diagnosis of apical hypertrophic cardiomyopathy are described. Symptoms were frequent and abnormal filling of the left ventricle by Doppler criteria was recorded in all patients. Rest electrocardiograms showed giant negative T waves in 5 patients, all had low exercise capacity on an exercise stress test while significant arrhythmias were detected by 24-h ambulatory monitoring in just one patient. Forty-eight first degree relatives were studied and 3 had some form of hypertrophy of the left ventricle as seen by echocardiography. Apical hypertrophic cardiomyopathy in Brazilians seems to be expressed somewhat differently from that reported in Japanese patients, since "giant' T waves are less frequent and women more involved.
J Am Coll Cardiol. 1995 Dec;26(7):1672-8.
Disappearance of giant negative T waves in patients with the Japanese form of apical hypertrophy.
Koga Y, Katoh A, Matsuyama K, Ikeda H, Hiyamuta K, Toshima H, Imaizumi T.
Third Department of Medicine, Kurume University School of Medicine, Japan.
OBJECTIVES. The present study investigated the long-term changes in the electrocardiographic (ECG) hallmarks of the Japanese form of apical hypertrophy. BACKGROUND. Giant negative T waves and tall R waves in the left precordial leads are the ECG hallmarks of the Japanese form of apical hypertrophy. However, the long-term course is largely unknown. METHODS. Twenty-nine patients with apical hypertrophy (26 men, 3 women, mean age +/- SD 50.4 +/- 8.2 years) who showed left precordial giant negative T waves (< or = -10 mm) and tall R waves (> or = 26 mm) and spade configuration in the left ventriculogram were followed up for 10.9 +/- 3.7 years. RESULTS. The intermediate follow-up ECGs (5 to 9 years) showed disappearance of giant negative T waves in 31% and of tall R waves in lead V5 in 6%. At the long-term follow-up study (> or = 10 years), loss of giant negative T waves increased to 71%, with average T wave negativity in lead V4 or V5 decreasing from -16.5 +/- 5.1 to -6.9 +/- 4.2 mm. These T wave changes were associated with decreases in R wave amplitude in lead V5 from 40.7 +/- 9.6 to 26.1 +/- 13.8 mm, with loss of tall R waves in lead V5 in 38% of patients and development of abnormal Q waves in two patients. CONCLUSIONS. During the long-term follow-up of the Japanese form of apical hypertrophy, giant negative T waves disappeared in association with decreases in R wave amplitude in lead V5, indicating that these ECG hallmarks are clinical features that evolve progressively during the natural course of the disease.
J Am Soc Echocardiogr. 1993 May-Jun;6(3 Pt 1):327-31.
A reversible form of apical left ventricular hypertrophy associated with pheochromocytoma.
Schuiki ER, Jenni R, Amann FW, Ziegler WH.
Department of Internal Medicine, University Hospital, Zurich, Switzerland.
A patient with pheochromocytoma was found to have typical features of apical left ventricular hypertrophy similar to apical hypertrophic cardiomyopathy of the Japanese type. The electrocardiogram showed giant negative T waves (1.0 mV), and echocardiography as well as angiographic examination revealed left ventricular hypertrophy confined to the apex. Surgical removal of the tumor resulted in striking regression of apical hypertrophy and essential normalization of the electrocardiogram within 1 year of operation. These findings emphasize the possible role of catecholamines in the cause of apical hypertrophic cardiomyopathy and illustrate the potential reversibility of this condition in association with pheochromocytoma. It is suggested that patients with signs of apical left ventricular hypertrophy should undergo thorough screening for a pheochromocytoma.
Clin Cardiol. 1992 Nov;15(11):833-7.
Comparison of clinical, morphological, and prognostic features in hypertrophic cardiomyopathy between Japanese and western patients.
Chikamori T, Doi YL, Akizawa M, Yonezawa Y, Ozawa T, McKenna WJ.
Department of Cardiological Sciences, St. George's Hospital Medical School, London, U.K.
Apical hypertrophic cardiomyopathy appears to be more common in Japan than in the West. Explanations for this difference include variable methods and criteria for the diagnosis. To assess morphological, clinical, and prognostic differences, 45 consecutive Japanese and 45 age- and gender-matched Western patients with hypertrophic cardiomyopathy were evaluated in two referral institutions by the same individuals. The diagnosis of hypertrophic cardiomyopathy was based on the echocardiographic demonstration of unexplained left ventricular hypertrophy. Patients were aged 8 to 64 years (mean 50); there were 66 males and 24 females. The pattern of left ventricular hypertrophy was similar in Japanese and Western patients: asymmetric septal 64 vs. 76%, concentric 22 vs. 13%, and apical 13 vs. 11% (p = NS). The incidence of an echocardiographic or Doppler calculated left ventricular gradient of > 30 mmHg was similar (11 vs. 18%; p = NS). The maximal left ventricular wall thickness was greater in Western patients (23 +/- 7 vs. 20 +/- 4 mm; p = 0.03), but was not different when adjusted for body surface area. Clinical features including incidence of family history and ventricular tachycardia during 24-h ambulatory electrocardiography were similar. During follow-up (4.9 +/- 4.0 years for Western vs. 4.4 +/- 2.0 years for Japanese), disease-related mortality was worse in Western patients (p < 0.05; 10 versus 2 patients). This evaluation, using the same diagnostic methods and criteria, reveals a worse prognosis in Western patients despite a similar clinical and morphological spectrum of hypertrophic cardiomyopathy.
South Med J. 1990 Dec;83(12):1465-8.
Apical hypertrophic cardiomyopathy in a non-Oriental man.
Williams J, Kreshon M, Khan AA.
Department of Cardiology, James H. Quillen College of Medicine, Johnson City, Tenn. 37614-0002.
Japanese investigators first described apical HCM in 2.9% of patients who had diagnostic left ventricular catheterization for suspected ischemic heart disease or cardiomyopathy. This entity was initially thought to be limited to individuals of Asian origin and has been uncommonly described in patients of Western origin. Patients of Western origin differ in several ways from those in the original description of Yamaguchi et al, but they both share the same classic criterion of hypertrophy of the left ventricular apex. The major differences probably relate to the anatomic variation in the distribution of the left ventricular hypertrophy as described by Maron et al. It is not known whether racial, genetic, or environmental factors account for the variation of disease expression in Asian and Western patients. Our case illustrates that this diagnosis should be considered in patients who have chest pain (anginal or atypical) and markedly abnormal findings on electrocardiograms in the absence of hypertension or significant coronary artery disease.
J Korean Med Sci. 1989 Dec;4(4):163-9.
Clinical and morphological features of hypertrophic cardiomyopathy in Korean patients.
Park YB, Lee WS, Kim DK, Choi YS, Seo JD, Lee YW.
Department of Internal Medicine, College of Medicine, Seoul National University, Korea.
Thirty three cases of hypertrophic cardiomyopathy (HCMP) were reviewed to estimate the relative frequencies of the subtypes of HCMP and to clarify whether there is any racial difference in clinical and morphological features of HCMP. The diagnosis was made by echocardiography, cardiac catheterization and left ventriculography. Twenty four patients underwent coronary angiogram. Numbers of cases by the types of HCMP were 20 (61%) with asymmetrical septal hypertrophy (ASH), 11 (33%) with apical hypertrophy (APH) and 2 (6%) with midventricular hypertrophy (MVH). Mean ages of the patients with APH, ASH and MVH were 54, 46 and 31 years respectively, and the differences were statistically significant (p less than 0.05). The giant negative T wave on electrocardiogram was seen in 4 patients (20%) of ASH and 5 patients (45%) of APH. On echocardiogram mean ratio of interventricular septal to left ventricular posterior wall thickness was 1.9 in ASH, 1.2 in APH and 1.6 in MVH, and the differences were statistically significant (p less than 0.05). All patients with APH showed "spade of ace" deformity in left ventriculography. Coronary angiograms were normal in all patients who had the procedure. Our study showed high frequency of APH of which characteristics were similar to those of the Japanese type APH.
J Cardiol Suppl. 1987;16:25-34.
A comparative study of apical hypertrophy with giant negative T waves and non-obstructive hypertrophic cardiomyopathy: evaluation by computed tomography, echocardiography and left ventriculography]
Kakinoki S, Sakuma I, Anzai T, Kudo T, Kanamori K, Sakamoto S, Yasuda H.
Department of Cardiovascular Medicine, School of Medicine, Hokkaido University, Sapporo.
To assess the relationships between patterns of left ventricular hypertrophy and giant negative T waves (greater than 15 mm, GNT), 24 patients with GNT were categorized in two groups: Group A consisted of 12 patients with GNP but without left ventricular hypertrophy (greater than 13 mm at the chorda level of the interventricular septum and/or left ventricular posterior wall by UCG, LVH); and group-B consisted of 12 patients with GNT and LVH. Twelve patients with LVH but no GNT compromised as group-C. Left ventriculography (LVG) was performed, and left ventricular mass (LVM) and apical hypertrophy were assessed by CT. The configurations of the end-diastolic left ventricle by LVG (RAO 30 degrees) were as follows, by group: A: spade in eight patients and papillary muscle hypertrophy (PMH) shape in four. B: spade in five, PMH in four, banana in one and oval in two. C: banana in nine and oval in three. The LVM (g/BSAm2) were as follows; A: 121.5 +/- 14.9, B: 130.5 +/- 14.8, C: 190.6 +/- 42.1 (C greater than A, B, p less than 0.001). The apex/base ratios calculated by CT were as follows; A: 1.43 +/- 0.31, B: 1.21 +/- 0.19, C: 1.09 +/- 0.20 (C greater than A, p less than 0.05). The left ventricular mass in patients with GNT was less than that of those without GNT. Giant negative T waves were most often associated with apical hypertrophy.
Ann Intern Med. 1987 May;106(5):663-70.
Apical hypertrophic cardiomyopathy: clinical and two-dimensional echocardiographic assessment.
Louie EK, Maron BJ.
Of 965 patients with hypertrophic cardiomyopathy evaluated by echocardiography at the National Institutes of Health during a 7-year period, 23 (2%) had a nonobstructive morphologic form, in which wall thickening occurred predominantly in the apical (distal) portion of the left ventricle. The patients ranged in age from 15 to 69 years (mean, 37) and were predominantly male (14 patients) and white (only 1 was of oriental descent). Fifteen patients had significant functional limitation, which was usually caused by exertional dyspnea and fatigue. Several electrocardiographic patterns were identified in the study group, but only 4 of these patients showed "giant" negative T waves. Only 3 patients had a morphologic expression of apical hypertrophy that closely resembled that described in Japanese patients--that is, hypertrophy that was particularly localized and confined to the true left ventricular apex (2 of these patients had giant negative T waves). Hence, hypertrophy located predominantly in the distal left ventricle was uncommon in our primarily North American patient population with hypertrophic cardiomyopathy. Most of our patients showed morphologic and clinical features that were dissimilar to those found previously in Japanese patients with apical hypertrophy.
J Cardiogr Suppl. 1985;(6):53-64.
The role of hypertension in apical hypertrophy
Fujiwara S, Umemoto M, Miyamoto Y, Ota A, Arita M, Yokote Y, Nakamura Y, Ueno Y, Nishio I, Masuyama Y.
Recent advances in echocardiography have revealed that hypertension causes several types of cardiac hypertrophy. We classified hypertensive patients by type of cardiac hypertrophy, and evaluated left ventricular function and the severity of hypertension. The subjects consisted of 257 hypertensive patients, 13 patients with cardiac hypertrophy, and 95 normotensive controls. The hypertensives were classified in four groups: no hypertrophy, concentric hypertrophy, asymmetrical septal hypertrophy (ASH), and asymmetrical apical hypertrophy (AAH). The normotensive patients with cardiac hypertrophy included nine with ASH and four with AAH. Cardiac functions in these patients were determined by echocardiography, RI-angiocardiography and cardiac catheterization. The results were as follows: Among 257 hypertensive patients, the incidence of concentric hypertrophy, ASH, and AAH was 53%, 10%, and 4%, respectively. In patients with AAH and hypertension, the hypertensive blood pressure levels and hypertensive organ involvements were mild. The blood pressures of most of these patients fell to the normal range after admission. The cardiac index and left ventricular systolic function (FS, mVCF, and ejection fraction) were significantly higher in AAH with hypertension than in the other hypertensive groups or in the normotensive controls. The hypertensive patients showed lower E-F slopes and higher A/E ratios than the normotensive controls, as well as the normotensive patients with ASH or AAH. Cardiac function did not differ appreciably between normotensive AAH and hypertensive AAH. Left ventricular dimension and left ventricular end-diastolic volume index were larger in AAH with hypertension. The total peripheral resistance of the hypertensive AAH was significantly lower than that of the hypertensives with concentric hypertrophy (p less than 0.01), though it was higher than that of the normotensive AAH (p less than 0.01). It was, therefore, concluded that mild hypertension observed in patients with AAH may be the result of regulatory mechanisms in the hyperkinetic states.
J Cardiogr Suppl. 1985;(6):23-33.
Distribution of left ventricular hypertrophy and electrocardiographic findings in patient with so-called apical hypertrophic cardiomyopathy
Fujii J, Saihara S, Sawada H, Aizawa T, Kato K.
The distribution of left ventricular hypertrophy determined by two-dimensional echocardiography was compared with the ECG findings of 15 patients with so-called apical hypertrophic cardiomyopathy. In most patients, hypertrophy was localized from the anterior septum at the mid-level of the left ventricle to the apex. Giant negative T waves and ST depressions were observed in II, III, aVF and V4-6 in all cases. Negative U waves in the same leads and prolongation of the QTc were also observed in most cases. The cause of these ECG abnormalities was unknown, but ST-T changes and negative U waves may be related to marked, localized hypertrophy of the apex. The depth of the negative T waves and the ST segments changed significantly by hour, from day to day, or from year to year. However, these changes were not associated with significant changes in blood pressure, cardiothoracic ratio, physical condition or therapy. The cause of these changes was unknown, but alterations in activity of the sympathetic nervous system may be one possible cause. In patients with monthly or yearly changes in ST-T, the progression of hypertrophy may also be the cause. ST-T variations revealed in this study might be one of the causes for the different incidences of giant negative T waves in the reports with apical hypertrophic cardiomyopathy. In some patients, an exercise test was positive, but, the coronary angiogram and left ventriculogram were normal for all patients.
Am Heart J. 1984 Aug;108(2):351-9.
Prognosis in hypertrophic cardiomyopathy.
Koga Y, Itaya K, Toshima H.
One hundred thirty-six patients with hypertrophic cardiomyopathy were followed up for 1 to 17 years. Twenty-one patients had died, 14 of them suddenly, two from heart failure, two from cerebral embolism, and three from noncardiac causes. Life table analysis revealed that sudden death was significantly associated with young age less than 20 years (relative risk [rr] = 8.63, when compared with those greater than 40 years) and with positive Master's single two-step test (rr = 3.55). Heart failure was more frequent in patients with positive Master's single test (rr = 4.27) and with left ventricular end-diastolic pressure greater than 20 mm Hg (rr = 2.58). Atrial fibrillation, observed in 15 patients, was a poor prognostic sign, resulting in five cardiac deaths and seven heart failures. In contrast, prognosis was favorable in patients with apical hypertrophy with giant negative T wave. Thus Japanese patients with hypertrophic cardiomyopathy showed a prognosis consistent with Western patients, except for excellent outcome of apical hypertrophy.
J Cardiogr. 1984 Aug;14(2):281-8. Related Articles, Links
Correlation between left ventricular wall thickness and the depth of negative T waves in apical hypertrophic cardiomyopathy
Nishiyama S, Shiratori K, Nishimura S, Araki R, Takeda K, Nagasaki F, Nakanishi S, Yamaguchi H, Kuwayama M.
Electrocardiographic features of apical hypertrophic cardiomyopathy are high QRS voltage and giant negative T waves greater than 10 mm in left precordial leads. We analyzed thirty patients with apical hypertrophic cardiomyopathy to clarify the correlation between left ventricular (LV) wall thickness and the depth of negative T waves. LV anterior wall thickness was measured by the left ventriculogram (LVG) in the RAO projection. LV posterior wall and septal thickness were measured by echocardiograms recorded from the left sternal border. THA and THM were defined as apical thickness and mid ventricular wall thickness, respectively. The results were as follows: LVG disclosed that the depth of negative T waves in apical hypertrophic cardiomyopathy was significantly related to the absolute thickness of apical segment itself rather than the difference in the distribution of thickness from the basal to apical segment of the left ventricle. This results support the clinical observation that giant negative T waves in some cases are associated with diffuse hypertrophy of the LV without a spade like configuration in LVG. Results of the echocardiographic analysis, however, revealed that the depth of negative T waves had no relation to the distribution of left ventricular hypertrophy. This is partly because of unsatisfied and difficult recordings and measurements of the true LV apical segment by echocardiography. The presence of both high QRS voltage and giant negative T waves is indispensable for the diagnosis of apical hypertrophy.
J Cardiogr. 1984 Aug;14(2):289-300.
Apical hypertrophy as a part of the morphologic spectrum of hypertrophic cardiomyopathy
Mori H, Ogawa S, Nakazawa H, Noma S, Fujii I, Nagata M, Akiyama H, Yamazaki H, Handa S, Nakamura Y.
Clinical and morphological features in 10 cases of "apical hypertrophic cardiomyopathy" (apical type) were analyzed and compared with those in classic hypertrophic cardiomyopathy with asymmetric septal hypertrophy (ASH). The hypertrophic pattern of the interventricular septum (IVS) was evaluated on the two-dimensional echocardiographic (2-D) left ventricular (LV) long-axis view. Thirty-one of 70 patients with ASH were characterized by predominant hypertrophy of the basal IVS (Type I). Seventeen patients had predominant hypertrophy of the apical IVS (Type III). Diffuse IVS hypertrophy was noted in 22 patients (Type II). Electrocardiographic similarity was documented between cases with Type III and apical type; that is, a low incidence of abnormal Q waves, a high incidence of giant negative T waves, and frequent absence of Q waves. In Type I and II, abnormal Q waves were frequently observed, while giant negative T waves were rare. On the 2-D LV short-axis view at the papillary muscle level, three groups with ASH revealed similar distributions of myocardial hypertrophy characterized by extension of hypertrophy to the LV anterior free all and papillary muscles. Most cases with apical type had a similar distribution of hypertrophy, but its degree was significantly less severe than that of ASH. Comparison of diastolic LV configurations on left ventriculograms and/or the 2-D apical two-chamber views indicated the morphologic continuity among the three types with ASH and the apical type. Clinical features in cases of the apical type were obviously less severe than those of three groups with ASH, as indicated by a significantly larger proportion of asymptomatic patients (90%), absence of sudden death and rare documentation of malignant ventricular arrhythmias on 24-hours ambulatory electrocardiography. In conclusion, apical type can be categorized as a part of hypertrophic cardiomyopathy with a wide morphologic spectrum rather than the separate disease entity, and it is characterized by giant negative T waves with minimum clinical manifestations.
Am J Cardiol. 1982 Jun;49(8):1838-48.
Hypertrophic cardiomyopathy with ventricular septal hypertrophy localized to the apical region of the left ventricle (apical hypertrophic cardiomyopathy).
Maron BJ, Bonow RO, Seshagiri TN, Roberts WC, Epstein SE.
Clinical and morphologic features are described in a unique subgroup of seven patients with hypertrophic cardiomyopathy. Five patients either died suddenly or are alive but severely symptomatic. In each patient ventricular septal hypertrophy was demonstrated on two dimensional echocardiography or at necropsy to be virtually confined to its apical one-half. However, conventional M mode echocardiography was unreliable in identifying this site of hypertrophy because it was often inaccessible to the path of the M mode beam. Apical distribution of septal hypertrophy does not constitute a separate disease entity, but rather appears to be part of the morphologic spectrum of hypertrophic cardiomyopathy, as judged from two findings: (1) genetic transmission of hypertrophic cardiomyopathy in relatives of each study patient; and (2) marked disorganization of cardiac muscle cells in the left ventricular wall of the two patients studied at necropsy. Apical distribution of septal hypertrophy in these patients was associated with relatively mild T wave inversion in the electrocardiogram and characteristic angiographic appearance of the left ventricle with mid ventricular constriction and a small, often poorly contractile apical segment. These electrocardiographic and angiographic features differ from those previously described in Japanese patients with "apical hypertrophic cardiomyopathy" in whom "giant" T wave inversion and a "spade-like" appearance of the left ventricle were characteristic.
Am J Cardiol. 1979 Sep;44(3):401-12.
Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients.
Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F, Nishijo T, Umeda T, Machii K.
Jpn Heart J. 1976 Sep;17(5):611-29.
Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle. Echocardiographic and ultrasono-cardiotomographic study.
Sakamoto T, Tei C, Murayama M, Ichiyasu H, Hada Y.
Left ventricular scanning by echocardiography and ultrasono-cardiotomography was performed to search the possible muscular abnormality in 9 cases with giant T wave inversion without documented cause. The deeply inverted T wave was more than 1.2 mV (average was 1.63 mV) in the left precordial leads. All the cases had electrocardiographic left ventricular hypertrophy of obscure origin and ischemic episode was absent. Conventional echo beam direction to measure the short axis of the left ventricle disclosed almost normal thickness and movement of both interventricular septum (IVS) and the posterior wasll (PW), so that the report of these cases is frequently within normal limits. However, ultrasono-cardiotomography (sector B scan) disclosed the fairly localized hypertrophy near the left ventricular apex, and conventional echocardiography also revealed the same area of either IVS or PW or both below the insertion of the papillary muscles, when the scanning towards the apex was performed (asymmetrical apical hypertrophy: AAH). Control study of 9 cases with IHSS showed asymmetrical septal hypertrophy (ASH) with almost equally hypertrophied IVS from base to apex. All cases had inverted T waves, but these were of lesser degree. Three cases had relatively deep T wave compatible with those of AAH, and these cases also had the apical hypertrophy of considerable degree (unusual type of IHSS, i.e., intermediate type between AAH and ASH). The close relationship between the depth of the inverted T waves and the Apex/Mid wall thickness ratios suggests that the altered recovery process of the hypertrophied apical musculature is responsible for the giant T wave inversion of heretofore unsolved origin. Until the connective link of AAH to the other forms of hypertrophic cardiomyopathy is disclosed, the cases with such a T wave and the apical hypertrophy may be designated as asymmetrical apical hypertrophy (AAH).
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