Intracardiac Mass as Initial Cardiac Manifestation of Behçet's Disease: Think before You Cut

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder. Cardiac abnormalities including intracardiac thrombi have been described in up to 16% of cases. The clinical presentation of cardiac complications in BD may include fever, dyspnea, chest pain, hemoptysis, and edema. We present 2 cases of patients who underwent surgical excision of intracardiac masses thought to be intracardiac malignancies. Further pathological and clinical evaluation established intracardiac inflammatory masses due to BD as the final diagnosis. As intracardiac masses may be the presenting manifestation of BD, it is crucial for echocardiographers to consider BD in the differential diagnosis. A careful history and physical exam looking for signs and symptoms of BD is critical before considering surgical excision of unexplained intracardiac masses. If the final diagnosis is BD anti‐inflammatory therapy should be considered the basis of treatment.     

In vivo ultrasonic tissue characterization of human intracardiac masses

Interactions between an ultrasonic signal and cardiac tissue have been used to characterize the histologic state of myocardium in vitro. To assess the utility of in vivo ultrasonic tissue characterization, stochastic analysis was applied to the digitized echocardiographic signals from 15 patients with 2-dimensional echocardiograms suggesting intracardiac masses. Ten subjects with echocardiograms suggesting mural thrombi underwent subsequent surgery or necropsy, which confirmed thrombi in 6 and revealed no thrombi (designated artifact) in 4. Five other patients had intracardiac tumors. The amplitudes within the digitized ultrasonic signals were displayed as histograms, which were described by a parameter k that represented the degree to which each histogram departed from a totally random probability density function. In 5 of 6 thrombi, k = 0, but in all 4 artifacts, k greater than 0. The sixth thrombus had k = 0.5 due to the specular effect of the interface between the thrombus' 2 lobes. All 5 tumors had k greater than 0. Ultrasonic tissue characterization using a stochastic analysis of backscatter can be performed in vivo and helps differentiate thrombus from artifact and tumor in the heart.     

Two-dimensional echocardiographic demonstration of left atrial thrombi in patients with prosthetic mitral valves.

Although M-mode echocardiography (MME) is not a reliable method for detecting left atrial thrombi, recent reports suggest that two-dimensional echo (2DE) may be more effective than MME in identifying intracardiac thrombi. In three patients with prosthetic mitral valves who presented with either arterial embolization or prosthetic valvular dysfunction, 2DE demonstrated left atrial masses consistent with thrombi, while MME was either negative (two patients) or suspicious (one patient) for left atrial thrombus. Thrombi were documented by surgical or postmortem examination in all cases. Clear delineation of the atrial cavity and the margins of the masses, visualization on multiple echocardiographic views and comparison of serial examinations were helpful in identifying these masses as thrombi. In addition, the masses visualized had certain patterns of motion which seem unique and may allow characterization of atrial masses as thrombi.     

Real-time, intracardiac, two-dimensional echocardiography: enhanced depth of field with a low-frequency (12.5 mhz) ultrasound catheter

Advances in catheter-based ultrasound imaging technology allow for a unique opportunity to develop two-dimensional intracardiac echocardiography, an imaging method that could have significant clinical applications. In this study, we evaluated the potential of a new, percutaneous, 9-Fr prototype intracardiac echocardiographic catheter with a 12.5-MHz rotating crystal in 13 dogs. In all dogs, we were able to easily advance the intracardiac echocardiographic catheter into the right and left hearts percutaneously and obtain dynamic images of cardiac structures in various imaging planes. With the intracardiac echocardiographic catheter in the right atrium, the whole chamber could be visualized. Minor manipulation allowed visualization of the right atrium, right ventricle, and tricuspid valve in a two-chamber view; further maneuvering yielded four-chamber views. With advancement of the catheter into the right ventricle and pulmonary artery, the right ventricular cavity, right ventricular outflow tract, and pulmonary artery could be imaged. The intracardiac echocardiographic catheter in the aortic root allowed visualization of the pulmonary artery and its bifurcation, superior portions of the atria, interatrial septum, aortic valve, and the proximal left coronary artery. With the intracardiac echocardiographic catheter in the left ventricle, short-axis images of the whole left ventricle were obtained. Manipulating the catheter tip within the left ventricle, we could visualize the left ventricle, left atrium (LA), and the mitral valve in the long axis. We were also able to visualize and identify experimentally-induced ischemic regional left ventricular dyskinesis (four of of five dogs), aortic valvular tear (five out of five dogs), and pericardial effusion with right atrial collapse (two out of two dogs). Intracardiac echocardiography was not associated with any complications. We conclude that percutaneous, low-frequency intracardiac echocardiography with a 12.5-MHz, 9-Fr catheter yields cardiac images in many imaging planes with a good depth of field, allows identification of valvular, myocardial, and pericardial abnormalities, and has excellent clinical potential in the assessment of many cardiovascular disorders.     

Differential diagnosis of cardiac masses using contrast echocardiographic perfusion imaging

OBJECTIVES: We investigated the usefulness of echocardiographic contrast perfusion imaging in differentiating cardiac masses. BACKGROUND: Two-dimensional echocardiography is the primary diagnostic modality for cardiac masses. However, differentiation between the different types of cardiac masses may be difficult at times. We hypothesized that echocardiographic contrast perfusion imaging would differentiate the neo-vascularization of malignancies from the avascularity of thrombi and the sparse vascularity of stromal tumors. METHODS: Sixteen patients with cardiac masses underwent power-modulation imaging after echocardiographic intravenous contrast administration. Pixel intensities in the mass and an adjacent section of myocardium were analyzed visually and by dedicated software. All masses had a pathologic diagnosis or resolved after anticoagulation. In a subset of patients, video-intensity curves of contrast replenishment in the mass and myocardium over time were generated. The post-impulse steady-state pixel intensity (A) and initial rate of contrast replenishment after impulse (beta) were compared with an index of blood vessel area on pathology. RESULTS: In seven of 16 patients, contrast enhancement resulted in greater pixel intensity in the mass than in the adjacent myocardium. All of these masses were classified pathologically as malignant (n = 6) or benign and vascular (n = 1). Nine masses demonstrated decreased pixel intensity, compared with the myocardium, and were diagnosed pathologically as myxomas (n = 2) or thrombi (n = 5), or they resolved with anticoagulation (n = 2). For the subset of patients, beta correlated with the vessel area index (r = 0.60). CONCLUSIONS: Echocardiographic contrast perfusion imaging aids in the differentiation of cardiac masses. Compared with the adjacent myocardium, malignant and vascular tumors hyper-enhanced, whereas stromal tumors and thrombi hypo-enhanced.     

Intracardiac thrombi in primary antiphospholipid syndrome: two case reports

We describe two patients who developed stroke and ischemia of the left lower limb, related to intracardiac thrombi, as the first manifestation of primary antiphospholipid syndrome (PAPS). Transesophageal echocardiography (TEE) revealed intracardiac thrombi as abnormal hyperechogenic, nonmobile masses, firmly attached to the left atrial appendage and the anterior part of the mitral annulus, respectively. Our patients received high-intensity oral anticoagulant therapy (INR 3.0–4.0), which resulted in the rapid disappearance of clinical symptoms, without subsequent recurrence of thromboembolic manifestations, and the disappearance of intracardiac thrombi on TEE. Our findings underscore that PAPS should be suspected in patients presenting with intracardiac thrombi, even if they have no previous history of thromboembolic disorders. Moreover, because intracardiac thrombi may precede other manifestations of PAPS, this finding in young patients without underlying heart disease should invoke a search for both antiphospholipid and anticardiolipin antibodies.     

Intracardiac Thrombi in Acute Myeloid Leukemia: An Echocardiographic and Autopsy Correlation

Two-dimensional echocardiography is the primary diagnostic imaging modality for the evaluation of cardiac masses. We describe an adult male suffering from acute myeloid leukemia who was detected to have right atrial and right ventricular mass on echocardiography. Based on the clinical data metastasis, coincidental primary cardiac tumor, vegetation, and thrombi were considered as possible differential diagnosis. Chemotherapy for acute myeloid leukemia failed and patient succumbed to septicemia. Later, clinical autopsy confirmed the diagnosis of intracardiac thrombi. Occurrence of intracardiac thrombi in acute myeloid leukemia is extremely rare. This report also emphasises the importance of histopathological or clinical autopsy examination of the mass in certain clinical scenario with diagnostic dilema. (Echocardiography 2010;27:E4-E8)     

A right atrial mass and a pseudomass

Right atrial (RA) masses are rare entities often detected incidentally during imaging studies. Leading etiologies of right atrial masses are tumor, thrombi, and vegetations. We present two cases of right atrial masses, a cardiac lipoma and an artifact. Clinical and echocardiographic characteristics of benign cardiac tumors are reviewed. We then highlight the importance of considering artifact in the differential diagnosis of atrial masses. Finally, we discuss echocardiographic characteristics of right atrial masses that may provide clues for diagnosis. Right atrial masses, often detected incidentally during imaging studies, are uncommon and can be due to many etiologies including tumors, thrombus, vegetations, normal variants, and artifacts. We describe 2 patients with RA masses detected on routine transthoracic echocardiogram.     

Diagnostic performance of two-dimensional versus three-dimensional transesophageal echocardiographic images of selected pathologies evaluated by receiver operating characteristic analysis

The sensitivity and specificity of 2-D and 3-D echocardiographic images for the detection of selected morphological abnormalities were compared using receiver operating characteristic (ROC) analysis. Five experienced clinical echocardiographers blinded to the patients' diagnoses evaluated the 20 original static 2-D image sets and 20 corresponding 3-D reconstructions using a five point categorical scale that ranged from definitely abnormal to definitely normal. The ROC curve for the 3-D images was significantly (P < 0.05) closer to the ideal discrimination function than was the ROC curve for the 2-D transesophageal images (i.e., the sensitivity of the 3-D images was higher than that of the 2-D sequential images at the same specificity). In conclusion: 3-D transesophageal images provided better visual clues for the identification of morphological abnormalities than did serial 2-D echocardiographic images despite the same input information in both image formats. The use of ROC analysis assisted in the comparison of these two imaging techniques.     

Invtracardiac thrombi: Frequency,location, etiology,and complications: A morphologvic review—Part V

Intracardiac thrombus may develop as a consequence of multiple underlying cardiac disorders. Other systemic disorders may predispose formation of thrombus within the heart, or the heart may be the site of emboli in transitthrombus originating elsewhere and traveling through the heart to the pulmonary or arterial circulation. Part V of this five-port series on intracardiac thrombus will focus on “migrating thrombi,” miscellaneous systemic conditions associated with intracardiac thrombi, and echocardiographic detection of intracardiac thrombus.     

Left ventricular thrombi: In vivo detection by indium-111 platelet imaging and two dimensional echocardiography

Indium-111 platelet Imaging, which can Identify sites of active intravascular platelet deposition, and two dimensional echocardlography, which can identify intracardiac masses, can both be used to detect left ventricular thrombi noninvasively. We compared these techniques in 44 men at risk for thrombi from remote transmural myocardial infarction (31 patients) or cardiomyopathy (13 patients). All 44 patients underwent platelet imaging; 35 underwent echocardlography.On platelet imaging nine patients had thrombi and one had a possible thrombus. Of these 10 studies, none were positive at 2 hours, 5 were positive at 24 hours and all were positive 48 or 72 hours after platelet labeling. Nine of these patients underwent echocardlography, and all had an intraventricular mass. The findings on platelet scanning were negative in six patients who had positive (four patients) or equivocally positive (two patients) findings on echocardiography. All patients with thrombi detected by either noninvasive method had transmural anterior myocardial infarction with ventricular aneurysm. Of the seven patients who underwent cardiac surgery or autopsy, three had thrombi. Platelet imaging failed to Identify one thrombus in a patient in whom imaging was performed only at 24 hours after labeling. There were no false positive platelet images in this group. Five of these seven patients (two with thrombi, three without) underwent echocardiography; in all cases the echocardiographic findings agreed with the pathologic findings.Both platelet Imaging and echocardiography detect ventricular thrombi. Platelet imaging may detect only the most hematologically active thrombi. Both techniques may help define patients at risk of embolization and may be useful for in vivo assessment of antithrombotic drugs.     

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis.From January 2003 through July 2012, 7 women (age range, 24–59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure''s wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients.When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.     

Contribution of complementary examinations in the diagnosis and treatment of emboligenic cardiopathies. Retrospective study apropos of 46 cases

The aim of this prospective study was to assess the value of complementary investigations in the diagnosis and follow-up of embolic heart disease. Forty-six patients having presented a systemic embolism cardiac origin underwent a standard work up which included clinical examination, ECG, chest X-ray and 2D echocardiography. Other investigations were carried out in some patients: CT cardiac scan (11 cases), gamma scintigraphy with Indium III labelled platelets (6 cases) and angiocardiography (12 cases). The diagnosis of an embolic cardiac lesion was made after the standard investigations in 82% of cases. The remaining 18% of cases hall had echocardiographic abnormalities and enable the diagnosis of clinically imapparent conditions: mitral valve prolapse, aneurysm of the interatrial septum, valvular calcification and cardiomyopathy. A potentially embolic mass was visualised in 18 patients; 6 valvular vegetations, 12 left atrial or ventricular thrombi. The other specialised radionuclide, angiographic and CT investigations only confirmed the echocardiographic diagnosis of intravavitary thrombosis. These results were confirmed surgically in 19% of cases. This study shows that complementary investigations especially echocardiography, allow diagnosis of latent embolic cardiac lesions, some of which may benefit from surgical treatment. In addition, potentially embolic intracardiac masses may be visualised, so confirming the origin of systemic emboli. When surgery is not indicated, echocardiography is a good method of following up the results of medical treatment in some of these masses.     

Echocardiographic diagnosis of left ventricular thrombi

Left ventricular thrombi have not been commonly recognized by M-mode or by cross-sectional echocardiographic techniques despite their frequency at postmortem examination in patients dying of cardiovascular disease. We discuss two patients, with left ventricular thrombi recognized echocardiographically and confirmed by pathologic and/or angiographic evaluation, whose M-mode and cross-sectional echocardiographic abnormalities add to the variable spectrum of appearance of left ventricular thrombi. The sensitivity and specificity of echocardiographic techniques in the diagnosis of intracardiac thrombi are discussed.     

Echocardiographic characteristics of intravenous leiomyomatosis with intracardiac extension: a single-institution experience

Background: Intravenous leiomyomatosis (IVL) is a rare smooth‐muscle proliferation arising from a uterine myoma and occasionally extending into cardiac chambers. Methods and Results: A series of 10 consecutive patients with histologically and surgically proven intracardiac IVL between 2000 and 2010 in our hospital were reviewed. The echocardiographic features of 10 cases with IVL and extensive spread into the right‐sided cardiac chambers were described for the first time. All patients were female and the mean age was 42 ± 7 years old. The first symptoms of six patients (60%) were exertional dyspnea and palpitation of cardiac origin. Echocardiography showed that all the tumors originated from the inferior vena cava (IVC) and located in cardiac right chambers (70% in right atrium alone, 30% in right ventricle and atrium). Eight masses (80%) were oval, whereas the others (20%) were serpentine, all with well‐demarcated borders and most (70%) with heteroechogenic texture. Five tumors (50%) intermittently prolapsed into right ventricle through the tricuspid valve. Two patients with nodules adhering to the top of the tumors had pulmonary tumorous thromboembolism. Conclusion: Echocardiography is a simple and important technique to diagnose IVL with intracardiac extension. This disease should be considered in a female patient presenting with an extensive mass from IVC with well‐demarcated border in the right‐sided cardiac chambers. (Echocardiography 2011;28:934‐940)     

Transesophageal echocardiography in the study of intracardiac thrombosis, tumors and vegetations]

Of 200 consecutive patients, studied with transesophageal echocardiography the findings were analyzed with respect to the presence of thrombi, tumors or vegetations. Thrombus formation was detected in 18 patients; their location and associated pathology are described. Tumors were present in 9 cases and in 10 patients endocarditis in native or prosthetic valves. The information obtained by transesophageal echocardiography was compared with transthoracic echocardiographic data. Transoesophageal echocardiography was demonstrated to be more sensitive, especially in the detection of intracavitary masses in the posterior structures of the heart.     

Clinical and echocardiographic diagnosis,follow up and management of right-sided cardiac thrombi

BackgroundRight-sided cardiac masses are infrequent and have varied clinical presentation. The present study describes the clinical features, echocardiographic findings and management of 19 patients presenting with right-sided cardiac thrombi in a tertiary care center in north India.MethodsThis is a retrospective, single center observational study of consecutive patients over the period January 2003–2008 admitted in our emergency intensive care unit (EICU). We identified 38 patients with right-sided cardiac masses admitted to EICU diagnosed by transthoracic echocardiography of which 19 patients had right-sided thrombus. The echocardiographic findings were reviewed by two cardiologists in all patients. Treatment was not standardized and choice of therapy was based on judgment of attending physician.ResultsThe mean age of patients with cardiac thrombus was 36.6 ± 11.8 years. Right atrial (n = 17) and right ventricle (n = 2) thrombi were associated with deep vein thrombosis (DVT) in 7 (36.8%) and pulmonary embolism in 3 (15%) patients. 13 (68.4%) patients appeared to have in situ mural thrombus. 12 patients were managed with oral anticoagulants, 3 patients underwent surgery and 4 patients were thrombolysed. All the survivors had a mean follow-up of 40 ± 6 months (range – 18–50 months).ConclusionsPrompt echocardiographic examination in an appropriate clinical setting facilitates faster diagnosis and management of patients with right-sided cardiac thrombi. High incidence of in situ mural thrombus and varied comorbidities predisposing to right-sided cardiac thrombi besides DVT and pulmonary embolism need to be recognized. Oral anticoagulation and thrombolysis appear to be the mainstay of treatment with surgery limited for selected patients.     

Diagnosis of noninfective cardiac mass lesions by two-dimensional echocardiography. Comparison of the transthoracic and transesophageal approaches

This study was conducted in 46 patients with cardiac thrombi, 15 patients with atrial myxomas, and 32 patients with other cardiac or paracardiac tumors. Diagnoses were subsequently proven by surgery, autopsy, computed tomography, magnetic resonance imaging, or angiography in all patients. All patients underwent precordial and transesophageal two-dimensional echocardiography to assess the various mass detection rates. Atrial myxomas and predominantly left-sided cardiac tumors were identified by both echocardiographic techniques with comparable detection rates. Left ventricular apical thrombi were detected more frequently by precordial echocardiography. In contrast, transesophageal echocardiography was superior in visualizing left atrial appendage thrombi, small and flat thrombi in the left atrial cavity, thrombi and tumors in the superior vena cava, and masses attached to the right heart and the descending thoracic aorta. These data indicate that transesophageal echocardiography leads to a clinically relevant improvement of the diagnostic potential in patients in whom cardiac masses are suspected or have to be excluded in order to ensure the safety of clinical procedures.     

Intracardiac masses detected by echocardiography: case presentations and review of the literature

Intracardiac masses are often diagnosed by transthoracic echocardiography (TTE). Transesophageal echocardiography (TEE) improves overall visualization of masses, especially those located in the posterior cardiac structures. Masses in the heart are most commonly due to thrombi or valvular vegetations; however, a variety of tumors may also present as cardiac masses on echocardiography. Tumors of the heart most commonly occur in the setting of metastatic disease, usually from malignancies of the breast, lung, or from malignant melanoma. Primary cardiac tumors occur much less frequently and are usually benign. Atrial myxomas constitute nearly one-half of reported primary cardiac tumors. The following discussion details the findings of five cases that illustrate the spectrum of intracardiac tumors detected by echocardiography and reviews the relevant literature.     

Successful three-dimensional reconstruction using transesophageal echocardiography in a patient with a left atrial myxoma

Two-dimensional echocardiography can provide intracardiac images. However, the cross-sectional images require mental reconstruction to understand a three-dimensional intracardiac structure. It is sometimes hard for inexperienced echocardiographers to engage in reconstruction. Thus, three-dimensional echocardiography is potentially beneficial because these images can provide extra information without mental reconstruction. Herein we demonstrate three-dimensional reconstruction using transesophageal echocardiography in a patient with a left atrial myxoma. It contributed to clarifying the surgical considerations, including whether the tumor was adhering to the left atrium or the mitral valve.