The left ventricular regional systolic functions in patients with hypertrophic cardiomyopathy (HCM) were assessed by using quantitative tissue velocity imaging (QTVI). Left ventricular (LV) regional myocardial velocity along long- and short-axis in 31 HCM patients and 20 healthy subjects were analyzed by QTVI, and the regional myocardial systolic peak velocities (MVS) were measured. Mean MVS at each level including mitral annular, basal, middle and apical segments were calculated. The ratio of MVS along long-axis to that along short-axis (Ri) at basal and middle segments of the LV posterior wall and ventricular septum were calculated. The results showed that mean MVS was slower at each level including mitral annular, basal, middle and apical segments in the HCM patients than that in the healthy subjects (P < 0.01). There were no significant differences in mean MVS between obstructive and non-obstructive groups in HCM patients. MVS of all regional myocardial segments along long-axis in the HCM patients were significantly slower than that in the healthy subjects (P < 0.05), but there was no significant difference in MVS of all regional myocardial segments along long-axis between hypertrophied and non-hypertrophied group in the HCM patients. Ri was significantly lower in the HCM patients than that in the healthy subjects. The LV regional myocardial contractility along long-axis was impaired not only in the hypertrophied wall but also in the non-hypertrophied one in patients with HCM, suggesting that QTVI can assess accurately LV regional systolic function in patient with HCM and provides a novel means for an early diagnosis before and independent of hypertrophy.
Cardiomyopathy, Hypertrophic/*physiopathology ; Cardiomyopathy, Hypertrophic/ultrasonography ; *Echocardiography, Doppler/methods ; Ventricular Function, Left/*physiology

Cardiomyopathy, Hypertrophic ; diagnostic imaging ; Humans ; Liver Diseases ; Ultrasonography

About half of all cases of hypertrophic cardiomyopathy(HCMP) have a positive family history. All first-degree relatives of patients with HCMP should be screened with echocardiography. The prenatal diagnosis of abnormal septal hypertrophy in fetuses of mothers with HCMP has not yet been documented. We report a prenatal diagnosis in a case of familial HCMP by ultrasonography which was confirmed by autopsy. Fetal echocardiography provides a valuable aid in diagnosis of familial HCMP.
Autopsy ; Cardiomyopathy, Hypertrophic, Familial* ; Diagnosis ; Echocardiography ; Fetus ; Humans ; Hypertrophy ; Mothers ; Prenatal Diagnosis* ; Ultrasonography ; Ultrasonography, Prenatal*

To assess the left ventricular regional relaxation abnormalities in patients with hypertrophic cardiomyopathy (HCM) by quantitative tissue velocity imaging (QTVI), Doppler echocardiography and QTVI were performed in HCM (n=10) and healthy subjects (n=11) at apical long-axis, two-chamber and four-chamber views. Regional early diastolic velocity (rVe) and regional atrial contraction (rVa) were measured at each segment of ventricular middle, basal and annular levels. Mean rVe and mean rVa at three levels as well as mean rVe/rVa ratio were calculated. Our results showed that transmitral inflow peak velocities during early diastole (E) and atrial contraction (A) were also measured and E/A ratio was calculated. The rVe of all left ventricular segments in HCM were lower than those in healthy subjects (P<0.05), but compared with healthy subjects majority of rVa in HCM were not different except inferior wall and anterior wall. E between HCM and healthy subjects was different (P=0.036), while mean rVe between them was significantly different (P<0.0001). Mean rVa and mean rVe/rVa of three levels were lower in HCM than in healthy subjects (P<0.05), but there were no differences in A and E/A between them (P=0.22, P=0.101). Left ventricular regional myocardial relaxation is reduced in HCM. Transmitral inflow E and A are influenced by preload, relaxation of myocardium and atrial contraction, etc., while rVe and rVa reflect myocardial relaxation function independently. QTVI is more sensitive and more accurate than conventional Doppler imaging for characterizingregional diastolic properties in HCM.
Cardiomyopathy, Hypertrophic/*physiopathology ; Cardiomyopathy, Hypertrophic/*ultrasonography ; Diastole ; Echocardiography, Doppler, Color/methods ; Hypertrophy, Left Ventricular/*physiopathology ; Hypertrophy, Left Ventricular/ultrasonography ; Ventricular Function, Left

Intracoronary stent implantation can improve coronary hemodynamics and myocardial ischemia in patients with symptomatic bridging. However, percutaneous coronary intervention for this lesion is limited due to the high prevalence of restenosis and risk of complications. We present a case of a totally occluded long-segment myocardial bridge in a patient with hypertrophic cardiomyopathy who was successfully implanted with a bare metal stent under intravascular ultrasound guidance without complications. The patient has been free of ischemic symptoms with stent patency for 10 years.
Cardiomyopathy, Hypertrophic* ; Follow-Up Studies* ; Hemodynamics ; Humans ; Myocardial Bridging ; Myocardial Ischemia ; Percutaneous Coronary Intervention* ; Prevalence ; Stents ; Ultrasonography

OBJECTIVETo assess the left ventricular rotation and twist in patients with hypertrophic cardiomyopathy (HCM) by 2-dimensional ultrasound speckle-tracking imaging (STI).

METHODSTwo-dimensional images of left ventricule (LV) at basal and apical short-axis views were acquired in 20 patients with HCM and 20 healthy subjects to evaluate LV rotation. LV twist were defined as rate of apical LV rotation to the basal. Peak rotation (Prot) and the time to Prot in basal and apical short axis views were measured separately. Peak twist (Ptw), twist at aortic valve closure (AVCtw), twist at mitral valve opening (MVOtw), untwisting rate (Untw R), and half time of untwisting (HTU) were calculated.

RESULTSCompared with the control group, the value of Prot-MV, Prot-AP, Ptw, time to Ptw, AVCtw, MVOtw, and HTU significantly increased (all P < 0.05) and the Untw R significantly decreased (P < 0.05) in the HCM group. In the HCM group, time to Prot in apical view was significantly higher than that in basal view.

CONCLUSIONSTI can noninvasively evaluate the characteristics of LV twist and rotation in patients with HCM.

Cardiomyopathy, Hypertrophic ; complications ; diagnostic imaging ; Humans ; Hypertrophy, Left Ventricular ; diagnostic imaging ; etiology ; Torsion Abnormality ; diagnostic imaging ; etiology ; Ultrasonography

BACKGROUND: We sought to test whether patients with apical hypertrophic cardiomyopathy (APH) have different clinical features compared to those with typical asymmetric septal hypertrophy (ASH). METHODS: Among 32, 534 patients who underwent routine echocardiography at Asan Medical Center from January 2000 to December 2001, 305 patients (0.9%), who were finally diagnosed with hypertrophic cardiomyopathy (HCMP), were evaluated. The type of HCMP was classified according to the echocardiographic findings. RESULTS: ASH was the most frequent type (n=160, 53%, group I), and APH was the second most frequent (n=91, 30%, group II). Mean age (60.8 +/- 10 vs. 48.2 +/- 14 years, p< 0.001) and prevalence of hypertension (32% vs. 19%, p=0.022) were significantly higher in group II than in group I. Family history of HCMP (4.4% vs. 0% p=0.043) and sudden cardiac death (8.8% vs. 1.1% p=0.014) was more prevalent in group I. During the follow-up period of 32.0 +/- 37.2 months, cardiac events occurred at a significantly higher rate in group I (25.5% vs. 8.8%, p=0.003). CONCLUSION: APH comprises a significant proportion of HCMP in Korea and patients with APH show different clinical features compared to those with ASH.
Cardiomyopathy, Hypertrophic/epidemiology/*ultrasonography ; Comparative Study ; Echocardiography ; Female ; Follow-Up Studies ; *Heart Septum/ultrasonography ; *Heart Ventricles/ultrasonography ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Prevalence ; Retrospective Studies

OBJECTIVETo compare the safety and efficacy of myocardial contrast enhancement (MCE)-guided and angio-pressure (AP)-guided transcoronary ablation of septal hypertrophy (TASH) for patients with hypertrophic obstructive cardiomyopathy (HOCM).

METHODSTASH was performed under MCE-guide (n = 47, group I) or AP-guide (n = 25, group II) for drug-refractory patients with HOCM. Myocardial perfusion imaging (MPI) data as well as other clinical data were compared.

RESULTSTASH both under MCE-guide or AP-guide resulted in similar and significant reduction of left ventricular outflow tract gradient (PG) and associated with significant symptom improvement (all P < 0.001). Dosage of ethanol use, peak-level of CK-MB and ablated myocardial area and incidence of arrhythmia were also similar between the two groups.Similar left ventricular/atrial dimension changes post TASH were observed in the 2 groups during follow-up. However, the first selected septal vessels were changed under MCE in 6 patients.

CONCLUSIONSOur data demonstrated that the MCE-guided TASH was not superior to AP-guided TASH in safety and efficacy. However, MCE-guided TASH can avoid the misplace of ethanol to avoid innocent myocardial ablation.

Adult ; Cardiac Catheterization ; methods ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; therapy ; Catheter Ablation ; methods ; Female ; Humans ; Male ; Middle Aged ; Myocardial Perfusion Imaging ; Ultrasonography

OBJECTIVETo investigate the clinicopathologic manifestations of non-compaction of ventricular myocardium (NVM).

METHODSClinical data, electrocardiograms, echocardiography images and pathologic changes were studied in five cases of non-compaction of ventricular myocardium.

RESULTSThe patient's ages ranged from 29 to 57 years old, all were males. Abnormal electrocardiograms were obtained in all of the 5 cases. Among them, 3 were diagnosed using echocardiography. Histopathologic examination showed that there were abnormally coarse muscle trabeculation and deep recesses, interlacing in arrangement, over the inner wall of the heart chambers. The compacted myocardium became thinning down gradually from the base to the apex of the heart. The non-compacted myocardium bundles locating close to the endocardium were coarse and orderless in arrangement, nuclei were irregular and abnormal, nevertheless, the arrangement and appearance of the muscle bundles near by the pericardium part were essentially normal and the cell nuclei were evenly distributed.

CONCLUSIONThere are no specific clinical manifestations obtained in patients with non-compaction of ventricular myocardium, however, the pathologic changes are characteristic and a clinical diagnosis can be made by using echocardiography.

Adult ; Cardiomyopathy, Dilated ; diagnostic imaging ; pathology ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Electrocardiography ; Heart Ventricles ; pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Myocardium ; pathology ; Ultrasonography

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.
Aged ; Cardiomyopathy, Hypertrophic/*complications/diagnosis ; Coronary Angiography ; Coronary Vessel Anomalies/*complications/diagnosis/*ultrasonography ; Echocardiography ; Electrocardiography ; Female ; Heart Defects, Congenital/*complications/diagnosis/*ultrasonography ; Heart Ventricles ; Human ; Hypertrophy, Left Ventricular/complications/diagnosis