A case of Takotsubo cardiomyopathy with apical hypertrophic cardiomyopathy-like morphological changes during recovery / Journal of Rural Medicine

Objective: Few cases of Takotsubo cardiomyopathy with apical hypertrophic cardiomyopathy (APH)-like morphological changes during the recovery process have been reported.Patient: A 56-year-old woman diagnosed with Takotsubo cardiomyopathy showed a morphology similar to that of APH during recovery. We examined this patient using 2D speckle-tracking echocardiography based on the method used for hypertrophic cardiomyopathy, which suggested that the circumferential strain (CS) of the middle wall indicated myocardial function of the left ventricle, and the CS of the inner wall was associated with left ventricular chamber function.Results: We measured the CS of the endocardial, middle, and epicardial layers and found that the apical inner layer CS (CSinner), middle layer CS, and outer layer CS were all decreased at the onset. CSinner showed a strong tendency to recover on echocardiography performed when APH-like morphology was observed.Conclusion: The morphology of the apex in our case likely contributed to the maintenance of chamber function.

Diferencias electrocardiográficas en miocardiopatía hipertrófica apical e infarto apical sin elevación del segmento ST / Electrocardiographic differences between apical hypertrophic cardiomyopathy and apical non-ST segment myocardial infarction

La miocardiopatía hipertrófica apical (MCHA) es una variante fenotípica dentro de las miocardiopatías hipertróficas que presenta alteraciones de la repolarización ventricular. Estos cambios electrocardiográficos pueden simular en muchos casos un infarto anterior, lo que dispara una serie de estudios y tratamientos que pueden ser innecesarios. El objetivo del trabajo fue describir y comparar las diferencias electrocardiográficas en una serie de pacientes con MCHA e infarto sin elevación del ST apicales (IAMSESTa) que presenten cambios tipo T en el electrocardiograma (ECG). Se realizó un estudio observacional y retrospectivo, incluyendo pacientes con diagnóstico de MCHA (n = 19) e IAMSESTa (n = 19) con ondas T negativas en ECG de ingreso en derivaciones V1-V6. Se excluyeron aquellos con MCHA y enfermedad coronaria asociada. Se analizaron las características clínicas y electrocardiográficas entre ambos grupos. Los pacientes con MCHA presentaron mayor voltaje de ondas T (7 mV vs. 5 mV; p = 0.001) y sumatoria de voltaje de las mismas (29 mV vs. 17 mV; p = 0.003), mayor voltaje de ondas R (25 mV vs. 10 mV; p = 0.0001), con una sumatoria de máximo voltaje de R y T (R+T) significativamente mayor (33 vs. 14; p = 0.00001). Presentaron además mayor asimetría de las ondas T negativas, objetivado mediante una relación TiTp/TpTf > 1. Con un valor de corte de 26.5 mV para la variable R+T, se obtuvo un 68% de sensibilidad y 100% de especificidad para diagnosticar MCHA. El presente trabajo demuestra la existencia de diferencias en el patrón del ECG en MCHA e IAMSESTa.

Apocal hypertrophic cardiomyopathy (AHCM) is a phenotypic variant within hypertrophic cardiomyopathies, in which ventricular repolarization alterations are present. These electrocardiographic disturbances can mimic an anterior infarction which triggers a series of studies and treatments that may be unnecessary. The aim of this study was to describe and compare electrocardiographic differences in a series of patients with AHCM and apical non-ST segment elevation myocardial infarction in patients (NSTEMI) with T-wave changes. We conducted an observational and retrospective study, including patients with diagnosed AHCM (N = 19) and apical NSTEMI (N = 19) with negative T waves in V1 and V6 lead of the EKG. Those with AHCM presented higher T-wave voltage (7 mV vs. 5 mV, p = 0.001) and peak voltage (29 mV vs. 17 mV, p = 0.003), higher R-waves (25 mV vs. 10 mV, p = 0.0001), and a maximum voltage of R and T sum (R + T) significantly higher (33 vs. 14, p = 0.00001). They also showed a greater T-wave asymmetry, with a TiTp / TpTf ratio > 1. At a cut-off value of 26.5 mV for the R + T variable, 68% sensitivity and 100% specificity were obtained to diagnose AHCM. This study shows the existence of major differences in electrocardiographic presentation of AHCM and apical NSTEMI.

Comparison of Apical and Asymmetric Septal Phenotype in Patients With Hypertrophic Cardiomyopathy: Clinical Features and Prognosis / 中国循环杂志

Objectives: To compare the clinical features and long-term outcomes of patients with apical hypertrophic cardiomyopathy (ApHCM) and patients with asymmetric septal hypertrophic cardiomyopathy (ASHCM). Methods: Data from 600 patients (300 with ApHCM and 300 with ASHCM) identified in a consecutive single-center cohort between 1996 and 2014 were retrospectively analyzed. The two groups were 1:1 matched by age of diagnosis, gender and the presence of outflow tract obstruction. Clinical features, cardiovascular mortalities, incidence of sudden cardiac death and cardiovascular morbidity (including unexplained syncope, atrial fibrillation, nonsustained ventricular tachycardia, progressive heart failure, embolic stroke or transient ischemic attack and myocardial infarction) were compared between the two groups. Results: Forty-two patients (14.0%) had a maximum LV wall thickness of ≥30 mm in the ASHCM group compared to only 11 patients (3.7%) in the ApHCM group (P<0.01). 156 patients in ApHCM group (52.0%)and 168 patients in ASHCM group(56.0%)underwent cardiovascular NMR examination, the incidence of late gadolinium enhancement was significantly lower in ApHCM group than in ASHCM group(26.9% vs 76.2%,P<0.01). The mean follow-up durations for ApHCM and ASHCM were (7.5 ± 4.0) years and (6.6 ± 5.4) years, respectively. The incidence of cardiovascular death (1.0% vs 5.7%), sudden cardiac death (0.33% vs 3.3%) and major adverse cardiovascular event (18.3% vs 40.3%) were significantly lower in the ApHCM group than in the ASHCM group (all P<0.01). Unexplained syncope, nonsustained ventricular tachycardia, and progressive heart failure were less common in ApHCM group than in ASHCM group (all P<0.05). Multivariate COX regression analysis showed that late gadolinium enhancement positivity (HR=4.62, 95% CI: 2.28- 68.0, P=0.02) and unexplained syncope (HR=8.56, 95% CI: 2.1-16.6, P<0.01) were independent predictors of cardiovascular mortality. Unexplained syncope was independent predictor for sudden cardiac death (HR=4.40, 95% CI: 1.5-15.2, P=0.02). Conclusions: After eliminating the interference of age at diagnosis, gender and outflow tract obstruction, patients with ApHCM represent a more benign prognosis with a lower incidence of cardiovascular mortality and morbidity than patients with ASHCM.

Evaluation the Value of Left Ventricular Rotation and Torsion in Pre-apical Hypertrophic Cardiomyopathy Diagnosis Using Two-dimensional Speckle Tracking Imaging / 中国医科大学学报

Objective To discuss the diagnostic value of two-dimensional speckle tracking imaging in the evaluation of left ventricular rotation and torsion in pre-apical hypertrophic cardiomyopathy(PAHCM)patients. Methods A total of 26 patients with PAHCM,26 patients with hyperten-sive left ventricular hypertrophy(HLVH)and 26 healthy volunteers were recruited for the study. Two-dimensional echocardiographs were performed carefully. Two-dimensional images were obtained from the left ventricular apical four-chamber section,two-chamber section and two short-axis sec-tions,including mitral valve and apical levels. The peak subendocardial rotation(endo-rot),epicardial rotation(epi-rot),transmural torsion(mural-tor)and bulk rotation(bulk-rot)of each short-axis section were measured and analyzed using QLAB 9.1 software. Results In the mitral level, there was no statistically significant difference in endo-rot,epi-rot,mural-tor and bulk-rot between the PAHCM group and normal control group (P>0.05). There was statistically significant difference in endo-rot,epi-rot,mural-tor and bulk-rot between the PAHCM group and HLVH group (all P0.05). There was statisti-cally significant difference in endo-rot,mural-tor and bulk-rot between each two groups(P<0.05). There was statistically significant difference in G-tor between each two groups(P<0.05). The receiver operating characteristic curve analysis showed that the accuracy of differential diagnosis of G-tor PAHCM was high(the area under the receiver operating characteristic curve is 0.89),the sensitivity was 73.08%,and the specificity was 92.31%. Conclusion Two-dimensional speckle tracking imaging could accurately and quantitatively measure the left ventricular rotation and tor-sion in PAHCM patients. The G-tor could accurately screen and identify between PAHCM and HLVH.

Survival and Clinical Behavior of Hypertrophic Cardiomyopathy in a Latin American Cohort in Contrast to Cohorts from the Developed World

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with diverse phenotipyc, genetic expession and clinical presentations. The evolution of patients with HCM in Latin America has not been properly described being the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of this study was to determine the survival rate of HCM patients having different phenotypes in a Mexican cohort of patients. METHODS: Clinical and echocardiographic data obtained from 77 Mexican patients with recently diagnosed HCM were analyzed. The follow-up was of 12.5 years. RESULTS: 96.1% of patients were in functional class I/II according to the New York Heart Association, 2.6% in class III and 1.3% in class IV. Only 3.9% of them went to surgery for myectomy. During the follow-up, 17 patients (22%) died: 4/9 (44%) had apical HCM, 5/20 (25%) had obstructive septal asymmetric HCM, 6/35 (17%) had nonobstructive septal asymmetric HCM and 2/3 (15%) had concentric HCM. The survival rate was worse for patients with apical HCM, followed by those with obstructive and nonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significant difference in survival rates which declined in 65% in a 9 years-period. Log rank test showed significant differences (p < 0.002). CONCLUSION: The survival rate of patients with HCM was worse in those with apical variety. The majority of patients received medical treatment. The indication for myectomy was below that observed in other international centers.

Characteristics of Arrhythmia With its Prognosis in Patients of Apical Hypertrophic Cardiomyopathy / 中国循环杂志

Objective: To summarize the characteristics of arrhythmia with the relevant factors affecting its prognosis in patients of apical hypertrophic cardiomyopathy (AHCM). Methods: A total 283 AHCM patients with echocardiography or cardiac magnetic resonance (CMR) conifrmed diagnosis in our hospital from 2005-01 to 2012-08 were summarized. The patients were divided into 2 groups: With arrhythmia group, n=103 and Without arrhythmia group,n=180. The endpoint event was followed-up by clinical and telephone visits in both groups and the relevant risk factors affecting AHCM prognosis were investigated by Cox regression analysis. Results: There were 269 patients ifnished the follow-up investigation, 98 in With arrhythmia group, 171 in Without arrhythmia group, and the death rate was 4.08% vs 1.17%, the occurrence of endpoint event was 18.37% vs 5.58%respectively. Cox regression analysis indicated that age (HR=23.051, 95% CI 1.08-1.068,P<0.005), left atrial diameter (HR=4.113, 95%CI 1.002-1.119,P=0.043) and NT-proBNP (HR= 18.653, 95% CI 3.433-26.650,P<0.005) were the independent risk factors affecting prognosis in AHCM patients. Conclusion: Arrhythmia is one of the common presentations of AHCM, it does not have much impact on survival, while it may cause ventricular ifbrillation and increase the occurrence of cardiovascular events.

Apical Hypertrophic Cardiomyopathy with Apical Aneurysm and Thrombus Diagnosed by Contrast Echocardiography / 영남의대학술지

Apical hypertrophic cardiomyopathy is rare disease and a variant of hypertrophic cardiomyopathy with prevalence of 1 in 500 in the general population. Apical hypertrophic cardiomyopathy with apical aneurysm and intramural thrombus is extremely rare. We report a case of apical hypertrophic cardiomyopathy progressing to left ventricular apical aneurysm with intramural thrombus diagnosed by contrast echocardiography.

A Case of Apical Hypertrophic Cardiomyopathy Combined Acute Myocardial Infarction with Multiple Coronary Thrombosis

We report a case of apical hypertrophic cardiomyopathy combined with acute myocardial infarction and multiple coronary thrombosis. The patient presented with signs and symptoms of acute myocardial infarction, showing ST elevation in the lateral leads and ST depression in anterior chest leads on initial electrocardiogram. The coronary angiogram on admission, multiple thrombosis of distal left anterior descending artery, left circumflex, diagonal and obtuse marginal arteries were found. The present case shows that apical hypertrophic cardiomyopathy can be combined with acute myocardial infarction and multiple coronary thrombosis.

Multiple Coronary Artery-Left Ventricular Microfistulae in a Patient with Apical Hypertrophic Cardiomyopathy: A Demonstration by Transthoracic Color Doppler Echocardiography

Among the congenital coronary artery fistulae, multiple coronary artery microfistulae arising from the left and right coronary artery and emptying into the left ventricle are very rare and little is known of their anatomic and clinical features, especially in apical hypertrophic cardiomyopathy. A 67-year- old woman was referred for the evaluation of chest pain at exertion, and shortness of breath. Electrocardiographic and echocardiographic findings were typical of apical hypertrophic cardiomyopathy. Coronary arteriography showed normal epicardial coronary arteries, but multiple coronary artery-left ventricular microfistulae arising from the left and right coronary arteries. Transthoracic color Doppler echocardiography, using a high frequency transducer with a low Nyquist limit, demonstrated multiple coronary artery-left ventricular microfistulae just beneath the apical impulse window.

A Case of Cerebral Thromboembolism Occurred after Restoration to Sinus Rhythm of Paroxysmal Atrial Flutter in Apical Hypertropic Cardiomyopathy with Spontaneous Echo Contrast(SEC)

Atrial flutter occurs most often in patients with organic heart disease. It appears that chronic atrial flutter is associated with a remarkably high risk of clinically apparent thromboembolism and effective anticoagulation appears to reduce this risk, but acute or recent onset, postoperative atrial flutter may have a lower risk of thromboembolism than those with chronic atrial flutter. In chronic atrial flutter or fibrillation with organic heart disease, anticoagulation is generally justified but there is some debate about anticoagulation in paroxysmal atrial flutter. The spontaneous echo contrast is generally accepted one of the major risk factor of thromboembolism and usually occurred in mitral stenosis, dilated cardiomyopathy, and enlarged left atrium, but rarely observed in apical hypertrophic cardiomyopathy. We experienced a patient with apical hypertrophic cardiomyopathy, who visited to emergency medical center due to dizziness and suffered from cerebral thromboembolism after restoration of sinus rhythm. In transesophageal echocardiography, there was moderate to severe spontaneous echo contrast in left atrium. This patient showed that transesophageal echocardiography evaluation of left atrium might be mandatory in patients with paroxysmal atrial flutter and organic heart disease.

Apical Hypertrophic Cardiomyopathy: Clinical and Echocardiographic Follow Up

BACKGROUND: An unusual form of hypertrophic cardiomyopathy localized to the left ventricular apex has racial differences in phenotypic expression between many Japanese reports and most reports from outside Japan. In Japanese patients follow up study of apical hyertrophy has shown benign clinical course without demonstrable genetic transmission, but other ethnic patients with this variant was clinically different from Japanese patients. The purpose of this study was to evaluate the clinical course and the progression of hypertrophy of apical hyper- trophic cardiomyopathy by echocardiography and to define the relationship between the severity of apical hypertrophic cardiomyopathy and the clinical course. METHODS: Between June 1990 and August 1996, 35 out of 53 patients with apical hypertrophic cardiomyopathy diagnosed by echocardiography were studied. In 26 out of 35 patients, two- dimensional echocardiography and EKG were obtained at initial visit and follow up. We analyzed the sum of S wave in lead Vl and R wave in lead VS(mm) and T wave negativity in lead V4 on EKG. Measured echocardiographic parameters were apical thickness and apical cross-sectional area of left ventricle at end-diastole in apical tour chamber view, anteroposterior left atrial dirnension at end systole in parasternal short axis view and pulsed-wave Doppler pattern of transmitral inflow. RESULTS: 1) Mean age at presentation was 57.9+8.3 years(range 37 to 72). Mean follow up duration of echocardiography and EKG were 29.5+/-13.5 months and 27.7+ -1.4 months, respectively. 2) Eleven(31%) out of 35 patients were asymptomatic at initial presentation. In the remaining 24 patients, major symptoms included atypical chest pain(n=7), angina (n=11), dyspnea(n=12), palpitation(n=4) and fatigue(n=l), During follow-up, symptoms aggravated in 5/35(14%), no change in 22/35(63%) and allenated in 8/35(23%). The clinical event during follow up was transient ischemic attack in 1 patient, syncope in 1 patient and death in 1 patient with cerebral infarction and upper gastrointestinal bleeding. 3) Electrocardiography revealed normal sinus rhphm in 24 patients and atrial fibrillation in 2 patients at initial presentation. Paroxysmal atrial fibrillation was observed in 1 patient during follow up period at 24 hours Holter monitoring. Negative T wave amplitude was increased from 11.5+/-5.5 to 13.1+/-6.5mm(p<0.05), however the sum of SV, and RV, did not change significantly. 4) The apical thickness and apical cross-sectional area changed over time, frorn 19.9+/- 3.2 to 21.8+/-4.lmm(p<0.005) with interobservers difference of 2.3+/-1.2mm and from 11.4+/-2.4 to 12.5+/-3.1cm(p<0.05) with interobservers difference of 1.9+/-1.5cm, respectively. Left atrial dimension increased from 43.5+/-6.6 to 46.2+/-6.1mm(p<0.005). Transmitral inflow revealed norrnal E/A ratio and deceleration time of 150~ 40msec in 11 patients with changed to relaxation abnormalities in three and pseudonormalization in ovo and relaxation abnormalities in 14 patients with changed to pseudonormalization in three at follow-up. CONCLUSIONS: Patients with apical hypertrophic cardiomyopathy have relatively favorable prognosis during follow up period without any significant clinical event and symptomatic deterioration. T wave negativity on EKG and left atrial dimension on echocardiographic examination were increased during follow up, but these parameters were not associated with clinical presentation.

A Case of Regressed Apical Hypertrophic Cardiomyopathy

An unusual form of hypertrophic cardiomyopathy localized to the left ventricular apex is called apical hypertrophic cardiomyopathy. In the classic form is characterized by giant T wave inversion in electrocardiogram and an angiographic silhouette showing enddiastolic deformation of the left ventricular cavity resembling a "spade". Apical hypertrophic cardiomyopathy appears functionally similar to other forms of nonobstructive hypertrophic cardiomyopathy. The diagnosis of apical hypertrophic cardiomypathy is made chiefly from the 12 lead electrocardiogram, two-dimensional echocardiography, left ventriculogram and magnetic resonance imaging. The prognosis appears relatively favorable course. We report a case of regressed apical hypertrophic cardiomyopathy which was diagnosed by two-dimensional echocardiography and 12 lead electrocardiogram, with related literatures.

2 Cases of Apical Hypertrophic Cardiomyopathy with Left Ventricular Endomyocardial Calcification

Calcification of myocardium is most common in the site of an old infarction or in an aneurysmal wall. In addition, Myocardial calcification may occur in association with endomyocardial fibrosis and hyperparathyroidism, as a result of focal toxic or inflammatory myocardial necrosis, as well as in patients undergoing hemodialysis. Calcium deposits due to parasites and due to neoplastic disease may also be seen, But, left ventricular endomyocardial calcification associated with apical hypertrophic cardiomyopathy is very rare. This report describes 2 cases of apical hypertrophic cardiomyopathy with left ventricular endomyocardial calcification, diagnosed by the echocardiographic, angiographic and histologic findings.

Classification of the Site of Ventricular Septal Defect with 2-Dimensional Doppler Echocardiography

Seventeen cases of apical hypertrophic cardiomyopthy were reviewed to clarify whether there is any racial difference in phenotypic expression of apical hypertrophic cardiomopathy and to study whether there is any clinical or morphological difference between apical hypertrophic cardiomyopathy of Japanese type (J-APH) and apical hypertrophic cardiomyophthy of non-Japanese type (NJ-APH). The diagnosis was made by cardiac catheterization, left ventriculography and echocardiography. Seventeen patients were divided into 9 (53%) patients with J-APH and 8 (47%) patients with NJ-APH. Mean ages of patients with J-APH and NJ-APH were 55 years and 57 years, respectively. The most common clinical symptoms were chest pain(100%), dyspnea(59%) and palpitation(35%). Mean values of S1+R5 and T wave depth were 58+/-16mm and 16+/-6mm in J-APH and 42+/-11mm and 7+/-3mm in NJ-APH(p<0.05, respectively). Seven patients experienced transient or permanent atrial fibrillation without significant symptoms. Seven patients showed progression or regression of T wave depth greater than 5mm during the period of follow-ups. On echocardiography IVS/LVPW thickness(mm) at the base were 11+/-2/10+/-2 in J-APH and 17+/-7/11+/-2 in NJ-APH, and IVS/LVPW thickness(mm) at the apex were 25+/-7/24+/-5 in J-APH and 26+/-3/26+/-2 in NJ-APH. Three patients with NJ-APH showed asymmetrical septal hypertrophy, cardiac catheterization disclosed elevated left ventricular end diastolic pressure in 11 out of 19 patients. Dye entrapment at the apex during systole was noted in 5 out of 18 patients. In conclusion, our study showed high prevalence rate expression of J-APH in contrast to the Western countries, there may be racial difference in phenotype of apical hypertrophic cardiomyopathy. Considering progression or regression of T-wave depth, NJ-APH may be a forme fruste or regressed form of J-APH.