Chieko Sakai

Diagnostic performance of ultrasonic tissue characterization for subendocardial ischaemia in patients with hypertrophic cardiomyopathy

AIMS Hypertrophic cardiomyopathy (HCM) patients often develop left--ventricular subendocardial ischaemia, a cause of chest symptoms, despite normal epicardial coronary arteries. The aim of this study was to examine whether ultrasonic tissue characterization or late gadolinium enhancement on cardiac magnetic resonance imaging can detect subendocardial ischaemia in patients with HCM. METHODS AND RESULTS Subendocardial ischaemia was quantified on exercise Tc-99m tetrofosmin myocardial scintigraphy in 29 non-obstructive HCM patients with asymmetric septal hypertrophy. Ultrasonic tissue characterization using cyclic variation of integrated backscatter (CV-IB) and late gadolinium enhancement on cardiac magnetic resonance imaging were analysed separately in the right halves and the left halves of the ventricular septum in relation to subendocardial ischaemia. Subendocardial ischaemia was identified in 17 (59%) patients. The ratio of CV-IB in the right-to-left halves of the ventricular septum was significantly higher in patients with subendocardial ischaemia (1.19 ± 0.10) than those without (0.84 ± 0.10, P = 0.04). The optimal cutoff for the detection of subendocardial ischaemia was the ratio of CV-IB >1.0, with a sensitivity of 80%, specificity of 71%, and accuracy of 76%. On the other hand, late gadolinium enhancement was not associated with subendocardial ischaemia in our cohort. CONCLUSION Ultrasonic tissue characterization using CV-IB separately in the right and left halves of the ventricular septum, but not late gadolinium enhancement on magnetic resonance imaging, provided useful information in detecting subendocardial ischaemia in patients with HCM. Ultrasonic tissue characterization may be useful in selecting patients who will benefit from medications to relieve chest symptoms.

Holter Monitoring and Long-Term Prognosis in Hypertrophic Cardiomyopathy

Objectives: Limited data are available regarding Holter monitoring for cardiovascular events except for ventricular tachycardia as a risk marker for sudden death in hypertrophic cardiomyopathy (HCM). We aimed to examine Holter findings in relation to the long-term prognosis in patients with HCM. Methods: Ambulatory Holter monitoring was performed in 106 HCM patients with sinus rhythm. All were prospectively followed for the composite endpoint of sudden death, cardiovascular death, and hospitalization for heart failure or stroke associated with atrial fibrillation. Results: Cardiovascular events occurred in 19 patients during a mean follow-up of 10.1 years. Neither arrhythmia information nor autonomic information as assessed by heart rate variability and turbulence significantly differed between HCM patients with and without cardiovascular events. Average heart rates were lower in HCM patients with cardiovascular events (64.7 ± 11.2 beats/min) than in those without (73.7 ± 10.2 beats/min, p = 0.001). Multivariate Cox proportional hazards regression analysis after adjustment for baseline characteristics showed that lower average heart rate remained an independent predictor of cardiovascular events (HR: 0.47 per 10 increase; 95% CI: 0.25–0.87; p = 0.016). Conclusion: Average heart rate on Holter monitoring predicted long-term prognosis in our cohort. Further multicenter studies are needed to confirm our results.

Silent pulmonary artery dissection in a centenarian

Pulmonary artery dissection is an extremely rare and fatal disease that is difficult to diagnose. We report a case of a 97-year-old woman with heart failure, who was diagnosed as having silent pulmonary artery dissection by chance during transthoracic echocardiography. Surgical treatment for pulmonary artery dissection was not performed, but the patient has been doing well in a sanatorium for more than 2 years, being a centenarian.

Metastatic chordoma to the left ventricle: A case report

Chordoma is a rare neoplasm of bone that develops from the remnants of the primitive notochord. We present a 78-year-old woman with metastatic chordoma to the left ventricle. She had been diagnosed with sacral chordoma 5 years earlier, and chordoma gradually spread to numerous muscles, the ankle joint of the left leg, bilateral lungs, and the brain, despite repeated surgical excisions and carbon heavy-ion radiotherapy. Positron emission tomography/computed tomography showed an abnormal accumulation in the heart, with features similar to the other metastatic lesions. Transthoracic echocardiography could not provide a clear view of the heart, but multidetector computed tomography revealed that a giant abnormal mass was attached to the apical inferior segment of the left ventricle. The patient stated that she did not want to undergo surgical resection for the cardiac mass. The chordoma has been slowly increasing in size, but she has remained asymptomatic without cardiovascular events for more than a year since the diagnosis of cardiac metastasis. <Learning objective: Multidetector computed tomography provided information not only about the structure morphology of the left ventricular mass, but also about the pathological features using the attenuation values of computed tomography, enabling us to make a diagnosis of a metastatic lesion to the left ventricle from sacral chordoma.>.

Imaging of Right-to-Left Shunt in an Adult Patient with Unroofed Coronary Sinus with Persistent Left Superior Vena Cava: Diagnostic Clue and Pitfall

A 54-year-old woman with a history of multiple cardiac surgeries suffered from hypoxemia caused by a right-to-left intra-cardiac shunt due to coronary sinus (CS) anomaly with persistent left superior vena cava (PLSVC). Both the contrast echocardiography and enhanced computed tomography (CT) provided conclusive diagnosis of this rare congenital anomaly, which was overlooked for a long time. However, an important diagnostic clue was left-arm injection of the contrast media. In the present case, previously performed enhanced CT with its routine manner, i.e., contrast through the right arm, missed this anomaly. It is crucial to note that the unusual type of unroofed CS with PLSVC, presenting with an entirely right-to-left intra-cardiac shunt, cannot be delineated on an enhanced routine chest CT if the contrast media is injected through the right arm.

Mitral Valve Systolic Anterior Motion-Associated Sounds in Hypertrophic Cardiomyopathy

patient had not taken any regular medication. His mother had hypertrophic cardiomyopathy (HCM). An electrocardiogram showed left ventricular (LV) hypertrophy with a QRS width of 115 ms. Echocardiography indicated an LV ejection fraction of 65%, asymmetric septal hypertrophy with a maximum wall thickness of 21 mm, systolic anterior motion (SAM) of the mitral valve, LV outflow tract (LVOT) obstruction with a resting pressure gradient of 92 mmHg, and mild mitral regurgitation (Movie S2). The peak pressure gradient across the LVOT was 96 mmHg during the Valsalva maneuver, and increased to 185 mmHg in a sitting position. A diagnosis of obstructive A 51-year-old man presented with exertional dyspnea, especially during post-prandial periods. On physical examination the fourth sound and mid-late systolic murmurs were heard. The systolic murmur did not radiate to the neck, but it did increase with the Valsalva maneuver and in the standing position. Interestingly, an extra sound was audible between the first sound (S1) and a paradoxically split, second sound (S2) over a wide area, that is, from the second to fourth left sternal borders as well as the apex, with the intensity being loudest at the third left sternal border (Movie S1). No third sound was heard. The remainder of the examination was normal. The

Sick sinus syndrome as a late complication after ventricular septal defect surgery

Ventricular septal defect is a common congenital heart disease for which patch closure technique using cardiopulmonary bypass has been applied for a few decades, resulting in a low incidence of perioperative complications. We report a 26-year-old woman with bradycardia of 25-30 beats per minute, which was considered to be a late-onset complication of surgical closure of an isolated ventricular septal defect performed 21 years earlier. She has been stable for more than 9 months without an implanted pacemaker, although her heart rate was always around 40 beats per minute in scheduled follow-up visits. <Learning objective: We experienced a 26-year-old woman with bradycardia of 25-30 beats per minute, which was considered to be a late-onset complication of surgical closure of an isolated ventricular septal defect performed 21 years earlier. Our case highlights the importance of close follow-up for patients who underwent successful surgical closure of a ventricular septal defect and have shown nonsignificant bradycardia, even though they have no symptoms.>.