Mai Shimbo

Blood Balloon Induced by an Atrial Myxoma in the Heart

A 75-year-old male with a left atrial (LA) mass was referred to our hospital. The patient had been healthy until 5 months prior, when dyspnea on exertion developed. A chest radiograph showed mild enlargement of the cardiac silhouette. An ECG showed sinus rhythm with P mitrale (Figure 1A). Transthoracic echocardiography (TTE) revealed a 5.4×3.9-cm hypoechoic tumor that had prolapsed through the mitral valve causing left ventricular inflow obstruction (Figure 1B; Movie I in the online-only Data Supplement). The tumor appeared to be soft with smooth surface and had a stalk originating from the interatrial septum. Transesophageal echocardiography (TEE) revealed dynamic smoke-like echoes, indicative of spontaneous echo contrast, inside the tumor (Figure 1C; Movie I in the online-only Data Supplement). M-mode showed multiple echoes and unique tumor behavior with subtle motion, in which the tumor rebounded into LA cavity twice during systole, producing an M-shaped signal (Figure 1D; Movie II in the online-only Data Supplement). These echocardiographic findings implied that the inside of tumor was filled with liquid, rather than clotted blood, giving it a blood-balloon–like appearance. Moreover, color Doppler revealed afferent flow entering the tumor (Figure 2A) and efferent flow draining from the tumor (Figure 2C). Both flows occurred …

Morphological Determinants of Obstructive Hypertrophic Cardiomyopathy Obtained Using Echocardiography

The morphological determinants of left ventricular outflow tract (LVOT) obstruction in hypertrophic cardiomyopathy (HCM) are not completely understood. We aimed to identify the anatomical risks of the obstruction using echocardiography.Fifty patients with untreated HCM were classified into two groups: those with LVOT pressure gradient (LVOTPG) ≥ 30 mmHg (obstructive HCM [HOCM] group) and those with LVOTPG < 30 mmHg (HNCM group). The echocardiographic morphological variables were analyzed to determine whether they were predictive of LVOT obstruction. Systolic anterior motions of the mitral valve were observed in 100% of patients in the HOCM group but only in 58% in the HNCM group. There were no significant differences in wall thickness, end-systolic LV dimension (LVDs), or LVOT diameter between the two groups. However, HOCM subjects had a shorter distance from papillary muscles to the inter-ventricular septum (5.97 ± 2.3 versus 9.20 ± 1.9 mm, respectively, P < 0.0001) and a longer anterior mitral leaflet (AML) length (24.7 ± 5.8 versus 20.1 ± 5.4 mm, respectively, P < 0.01) compared to the HNCM group. The AML length/LVDs ratio was significantly higher in the HOCM group compared to the HNCM group (1.02 ± 0.34 versus 0.78 ± 0.26, P < 0.01), and an LVOT obstruction was predicted with an area under the curve of 0.71 (P < 0.05). Multiple linear regression revealed that only the AML length/LVDs ratio was independently associated with LVOTPG (P < 0.01).The AML length/LVDs ratio has a significant predictive value for LVOT obstruction and a strong relationship with LVOTPGs. The AML length/LVDs ratio determines the anatomical risk of LVOT obstruction in HCM.

A Cardiac Calcified Amorphous Tumor Associated with End-stage Renal Disease: An Emerging Disease Concept

Cardiac calcified amorphous tumor (CAT) is a rare nonneoplastic cardiac mass composed of calcium deposits in a background of amorphous degenerating fibrin material. Recently, cardiac CAT has gained considerable attention. A MEDLINE search of studies published until April 2017 using the term “cardiac calcified amorphous tumor” resulted in 54 articles, 49 of which were reported in the last 10 years. Cardiac CATs were first described by Reynolds et al. in 1997 (1), who reported that they had come to recognize that cardiac CATs can arise in any of the four chambers. However, this situation has begun to change in recent years. Accumulating evidence suggests the presence of specific group of patients with end-stage renal disease (ESRD) in whom cardiac CAT arises from areas of mitral annular calcification (MAC) (2-6). In this issue of the Journal, Yoshimura et al. reported a case of MAC-related CAT in an ESRD patient on hemodialysis, and reviewed the reports of all patients with cardiac CAT (7). Of note, they ascertained that CAT at the mitral annulus is more frequent in patients with ESRD than in patients without ESRD. In contrast, the frequency of CAT in the right ventricle in patients without ESRD is higher than that in patients with ESRD. These clinical findings suggest a particular association between ESRD and MACrelated CAT formation, and supports the hypothesis that MAC-related CAT is etiologically different from CAT arising from other sites. Although the pathogenesis of cardiac CAT remains uncertain, this report by Yoshimura et al. may provide the first clues of the cause. At the very least, it seems likely that the dysfunctional calcium-phosphorus metabolism in ESRD has an impact on the formation of MACrelated CAT (8). Moreover, 21 of the 54 articles on cardiac CAT were reported from Japan. The predominance of cardiac CAT in Japanese patients is of interest as a racial characteristic. Apart from cardiac CAT at other sites, MAC-related CAT represents a diagnostic challenge. The differential diagnosis includes vegetation, papillary fibroelastoma and thrombus. Although Magnetic Resonance Imaging (MRI) is usually helpful in the differential diagnosis of tumors-as it provides additional information on the tissue composition-MRI is of limited value in the diagnosis of MAC-related CAT. One possible reason for this is that MAC-related CAT is highly mobile and small in size. Conversely, the contribution of echocardiography in the characterization tissue is limited but it can evaluate rapid motion with ease. Thus, echocardiography is the diagnostic procedure of choice for assessing MAC-related CAT. The following aspects can be helpful in differentiating MAC-related CAT from vegetation (4): 1) continuity with the MAC, 2) a lack of association with the valve leaflets or regurgitation jet, and 3) revolving movement among the left atrial and ventricular cavities. Some recent reports have demonstrated that cardiac CAT has the potential to cause embolic events (9-11). Excision should thus be considered whenever a highly mobile CAT is found. Over the past 20 years, accumulating reports have significantly extended our knowledge regarding CATs. Nevertheless, our understanding of the pathology of cardiac CATs is still in the preliminary stages. Some questions remain unanswered. First, what is the relationship between MAC-related CAT and the liquefactive necrosis within the area of MAC (also known as annular caseous calcification)? Second, it is unclear whether an adequate intake of calcium-phosphorus can induce the regression of MAC-related CAT. In particular, the mechanisms underlying the formation of CAT represent an attractive target for future research on strategies to prevent the development of CAT.

Acute aortic regurgitation in a bicuspid aortic valve due to the rupture of an anomalous cord

A 64-year-old male with acute-onset dyspnea and diastolic murmur was referred to our hospital. Eight months earlier, he had developed atrial fibrillation. At that time, echocardiography showed a reduced ejection fraction of 41% and a bicuspid aortic valve (BAV) with mild aortic stenosis (Fig. 1a, b, Video 1). On admission, echocardiography showed prolapse of the conjoined cusp and severe aortic regurgitation (AR) accompanied by an eccentric jet (Fig. 1c, Video 2). Careful observation revealed a 10-mm-long, highly mobile, thread-like structure attached to the aortic valve on the ventricular surface, which mimicked valvular vegetation (Fig. 1d, Video 2). However, laboratory testing showed no inflammatory reaction. Blood cultures were negative for pathogens. Enhanced chest computed tomography showed mild dilation but not dissection of the ascending aorta. The patient’s hemodynamic deterioration prompted urgent surgical intervention. The aortic valve was resected and replaced with a 22-mm ATS Medical prosthesis. Grossly, the excised aortic valve was bicuspid. The conjoined cusp had a small raphe with incomplete commissural fusion, implying a forme fruste BAV. Moreover, it contained an anomalous cord attached by one-and to the raphe near the free margin (Fig. 2). Any signs of infective endocarditis were not found. We diagnosed acute-onset AR caused by the rupture of an anomalous cord in BAV, in which the conjoined cusp had completely lost its cooptation and suspension. If acute-onset severe AR develops in BAV patients, in addition to infective endocarditis and aortic dissection, the rupture of an anomalous cord should be considered.

Tricuspid valve chordae tendineae as protective filter to prevent massive pulmonary embolism

Although several cases of thrombus-in-transit associated with pro-thrombotic conditions have been reported (1-3), a transit thrombus located in the right ventricular (RV) cavity is extremely rare (4,5). Here, we report a case of nephrotic syndrome (NS) with a RV thrombus-in-transit that threatened to cause a fatal pulmonary embolism (PE).

Rapidly growing intraventricular mass in a patient with hereditary haemorrhagic telangiectasia

A 55-year-old man with hereditary haemorrhagic telangiectasia manifesting with giant pulmonary arteriovenous malformations (PAVMs; Panel A ) was admitted for a brain abscess and subsequent antibiotic-resistant fever. An echocardiogram revealed a mobile 9 × 7 mm mass attached to the posterior papillary muscle (PPM), with no valvular involvement, masquerading as an intracardiac tumour or …

Isolated Atrial Septal Defect Complicated by Tricuspid Valve Infective Endocarditis

Infective endocarditis (IE) associated with atrial septal defect (ASD) is extremely rare. However, tricuspid regurgitation (TR) secondary to right ventricular overload is a potential cause of IE, and once it occurs, the development of a paradoxical embolism may lead to fatal complications. We herein report the case of a 50-year-old woman who was admitted due to a persistent fever resistant to antibiotics. Echocardiography showed secundum ASD, moderate TR and a mobile vegetation measuring 15×10 mm attached to the tricuspid valve. Given the risk of developing a paradoxical embolism, urgent surgery was successfully performed.

Obstructed bi-leaflet prosthetic mitral valve imaging with real-time three-dimensional transesophageal echocardiography

Real‐time three‐dimensional transesophageal echocardiography (RT3D‐TEE) can provide unique visualization and better understanding of the relationship among cardiac structures. Here, we report the case of an 85‐year‐old woman with an obstructed mitral prosthetic valve diagnosed promptly by RT3D‐TEE, which clearly showed a leaflet stuck in the closed position. The opening and closing angles of the valve leaflets measured by RT3D‐TEE were compatible with those measured by fluoroscopy. Moreover, RT3D‐TEE revealed, in the ring of the prosthetic valve, thrombi that were not visible on fluoroscopy. RT3D‐TEE might be a valuable diagnostic technique for prosthetic mitral valve thrombosis.