Purpose: Calculation of mitral annular cross-sectional area (CSA_MV) using the diameters from the apical long-axis and commissural plane (LAX/CC) method could be more accurate than the calculated area obtained by the annular diameters in the conventional apical four- and two-chamber view (4CV/2CV) method. The purpose of the study is to clarify which approach gives better accuracy: to use the 4CV/2CV method from the apical view or to use the LAX/CC method from the apical view or the parasternal view.

Subjects and Methods: Thirty patients without valvular heart diseases were enrolled in this study (22 males, 8 females, 48.7±18.6 years old). CSA_MV was measured by three methods as follows: using the conventional 4CV/2CV method from the apical view, and using the LAX/CC method from the apical view and the parasternal view. Left ventricular inflow volume (Q_LVIT) was calculated using CSA_MV obtained by each method. LV outflow volume (Q_LVOT) was measured by the Doppler method. Correlations and differences between Q_LVOT and Q_LVIT were compared among the three methods.

Results and Discussion: Compared with the 4CV/2CV method, Q_LVIT values by the LAX/CC method from the two views were well correlated with Q_LVOT (4CV/2CV method: r＝0.745, p＜0.01 LAX/CC method from apical view: r＝0.799, p＜0.01 LAX/CC method from parasternal view: r＝0.925, p＜0.01). Further analysis with Bland–Altman plots revealed that the Q_LVIT obtained by the LAX/CC method from the parasternal view exhibited the closest agreement with Q_LVOT.

Conclusions: CSA_MV obtained by the LAX/CC method for both apical and parasternal views is more accurate than that obtained by the conventional 4CV/2CV method. Moreover, the LAX/CC measurement from the parasternal view is better than from the apical view.

Purpose: Diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is often difficult in adult patients, especially those with mild clinical manifestations. The myocardium is susceptible to mitochondrial abnormalities, and therefore, echocardiography might play a role in the early diagnosis of MELAS. However, details of the echocardiographic findings have not yet been fully investigated. Accordingly, the aim of this study was to clarify the characteristic echocardiographic findings in adult patients with MELAS.

Subjects and Methods: Eight patients with MELAS who were undergoing echocardiography were included. Clinical symptoms, laboratory test results, and echocardiographic findings were investigated.

Results and Discussion: Among the 8 patients, neuromuscular symptoms were present in 6, hearing loss in all, diabetes in 6, and short stature in 4 patients Additionally, abnormalities were observed in lactic acid level in 7, lactic acid to pyruvic acid ratio in all, and BNP in 6 patients. Echocardiographic findings showed preserved left ventricular ejection fraction in all patients, apparent left ventricular hypertrophy and abnormal global longitudinal strain in 6, and impaired diastolic function in 5 patients. A fine-particle bright-echo ultrasonic appearance in the hypertrophied myocardium, so-called “granular sparkling echo”, which is seen in glycogen storage disease or amyloidosis, was subjectively observed in 5 patients. Accordingly, in patients with left ventricular hypertrophy with abnormally bright echo accompanied by deafness, diabetes, or short stature, it is important that mitochondrial disease be considered in the differential diagnosis.

Conclusion: Echocardiographic findings of adult patients with MELAS were characterized by apparent left ventricular hypertrophy with fine-particle bright-echo appearance accompanied by left ventricular global longitudinal strain dysfunction and relaxation delay.