Asymmetric apical hypertrophy: ten years experience

Sakamoto, Takafumi; Amano, Keiko; Hada, Yoshiyuki; Tei, Chuwa; Takenaka, Katsu; Hasegawa, Ichiro; Takahashi, Toshiyuki

doi:10.1136/pgmj.62.728.567

Cited by 69 publications

(36 citation statements)

References 9 publications

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“…31 When our analyses excluded the 12 patients with APH, the results were same. Three of the APH patients had NSVT, 4 had a family history of HCM, and 3 had a family history of sudden death apparently caused by HCM.…”

Section: Discussionsupporting

confidence: 64%

Clinical Significance of T-Wave Alternans in Hypertrophic Cardiomyopathy.

Kuroda

Ohnishi

Yoshida

et al. 2002

Circ J

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Section: Discussionsupporting

confidence: 64%

Clinical Significance of T-Wave Alternans in Hypertrophic Cardiomyopathy.

Kuroda

Ohnishi

Yoshida

et al. 2002

Circ J

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“…5,6 It was also associated with a rare occurrence of cardiovascular death and events in Western populations. 7,8 It is not infrequent for AHCM to be associated with arrhythmia and apical infarction with aneurysm.…”

Section: Introductionmentioning

confidence: 99%

Two Hundred Eight Patients With Apical Hypertrophic Cardiomyopathy in China: Clinical Feature, Prognosis, and Comparison of Pure and Mixed Forms

Wang

et al. 2011

Clinical Cardiology

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Background: Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare form of hypertrophic cardiomyopathy (HCM), originally described in Japan and later in the West. Limited information is available on this disease in China. Hypothesis: This study was designed to describe clinical features and prognoses of patients with AHCM in China. Methods: A retrospective study of 208 consecutive patients with AHCM examined at FuWai Hospital was performed. Clinical features, mortality, and cardiovascular morbidity were analyzed. Results: The 208 patients with AHCM represented 16.0% of all HCM patients. Among them, 64.4% were pure form and 35.6% were mixed form. Compared with the pure group, the mixed group had a significantly larger left atrial diameter and thicker apical thickness. One hundred ninety-nine patients had a mean follow-up of 8.0 ± 3.5 years, cardiovascular mortality was 1.0%, and annual cardiovascular mortality was 0.1%. The 2 cardiovascular deaths were both mixed form. The probability of survival was 97.0 ± 2% at 10 years. Of the patients, 17.8% had 1 or more cardiovascular events. The probability of survival without morbid events at 10 years was 77 ± 4%. Three independent predictors of cardiovascular morbidity were identified: age at diagnosis ≥60 years, left atrial diameter ≥36 mm, and New York Heart Association class ≥III at baseline. Conclusions:The prevalence of AHCM is relatively high, and it has a benign prognosis in China. However, 17.8% of patients may develop cardiovascular events. It is important to distinguish the 2 phenotypes of AHCM; the mixed form is less common but more serious than the pure form.

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“…One of the more unusual hypertrophic morphologies is apical HCM: nonobstructive hypertrophy that is localized to the cardiac apex. The ECG in apical HCM can also be unusual and show giant negative T waves [1][2][3][4] that can be labile and even disappear during long-term follow-up. 5 In addition to this unique ventricular morphology, other clinical manifestations can further distinguish apical HCM.…”

mentioning

confidence: 99%

Gene Mutations in Apical Hypertrophic Cardiomyopathy

et al. 2005

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Background-Nonobstructive hypertrophy localized to the cardiac apex is an uncommon morphological variant of hypertrophic cardiomyopathy (HCM) that often is further distinguished by distinct giant negative T waves and a benign clinical course. The genetic relationship between HCM with typical hypertrophic morphology versus isolated apical hypertrophy is incompletely understood. Methods and Results-Genetic cause was investigated in 15 probands with apical hypertrophy by DNA sequence analyses of 9 sarcomere protein genes and 3 other genes (GLA, PRKAG2, and LAMP2) implicated in idiopathic cardiac hypertrophy. Six sarcomere gene mutations were found in 7 samples; no samples contained mutations in GLA, PRKAG2, or LAMP2. Clinical evaluations demonstrated familial apical HCM in 4 probands, and in 3 probands disease-causing mutations were identified. Two families shared a cardiac actin Glu101Lys missense mutation; all members of both families with clinical manifestations of HCM (nϭ16) had apical hypertrophy. An essential light chain missense mutation Met149Val caused apical or midventricular segment HCM in another proband and 5 family members, but 6 other affected relatives had typical HCM morphologies. No other sarcomere gene mutations identified in the remaining probands caused apical HCM in other family members. Conclusions-Sarcomere protein gene mutations that cause apical hypertrophy rather than more common HCM morphologies reflect interactions among genetic etiology, background modifier genes, and/or hemodynamic factors. Only a limited number of sarcomere gene defects (eg, cardiac actin Glu101Lys) consistently produce apical HCM.

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Asymmetric apical hypertrophy: ten years experience

Cited by 69 publications

References 9 publications

Clinical Significance of T-Wave Alternans in Hypertrophic Cardiomyopathy.

Clinical Significance of T-Wave Alternans in Hypertrophic Cardiomyopathy.

Two Hundred Eight Patients With Apical Hypertrophic Cardiomyopathy in China: Clinical Feature, Prognosis, and Comparison of Pure and Mixed Forms

Gene Mutations in Apical Hypertrophic Cardiomyopathy

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