Multiple Intracardiac Masses Research Articles

P281 CARDIAC RICHTER‘S SYNDROME: A RARE CASE OF CHRONIC LYMPHOCYTIC LEUKAEMIA TRANSFORMATION

Abstract A 71–year–old woman with chronic lymphocytic leukaemia (CLL) presented to our Cardiology clinic complaining of recent–onset chest pain and dysphonia. Physical examination and ECG were normal. A blood test revealed high levels of high–sensitivity troponin T (373 ng/L) and of C–reactive protein (7 mg/dL); blood counts and differential were normal. Transthoracic echocardiography showed a hyperechogenic appearance of the interatrial septum, the interventricular septum and the right ventricular free wall, as well as a mild circumferential pericardial effusion. Cardiac magnetic resonance imaging revealed multiple intracardiac masses involving both ventricles and the interatrial septum, with evidence of oedema and fibrosis. A chest computed tomography showed the presence of multiple cervical, pulmonary, and mediastinal masses, one of which was biopsied through video–thoracoscopy. At the histological examination, the mass mainly consisted of large pleomorphic peripheral CD10–, CD20+/–, CD79a+, PAX5+, BCL2+, BCL6–, c–MYC+ B cells, with high proliferative activity (Ki–67/MIB1+ 80%). A diagnosis of CLL transformation into diffuse large B–cell lymphoma (DLBCL), also known as Richter’s syndrome (RS), was established (Figure). Despite indication for specific haematological chemotherapy, the patient rapidly deteriorated and died few days after the diagnosis. RS is a rare evolution of CLL and of small lymphocytic lymphoma, affecting 2–8% of such patients (transformation <1%/ year), yet is burdened with very poor prognosis (median overall survival 8–16 months). Extra–nodal RS is uncommon, and cardiac involvement has been reported in only 3 cases in the literature (in none with the panel of techniques here used). Despite its rarity, CLL patients with rapidly worsening cardiac symptoms should be suspected of RS transformation involving the heart. The main differential diagnosis of cardiac RS is primary cardiac lymphoma. The characterization of cardiac masses relies on multimodality imaging, but histologic analysis is required to reach a definite diagnosis, which is essential to guide subsequent therapeutic choices.

Multiple intra‐cardiac masses: A life‐threatening complication of Behçet’s disease

The incidence of multiple intra-cardiac masses is rare, and the differential diagnosis may be challenging sometimes. The most common etiologies of multiple intra-cardiac masses are thrombus, vegetation, and tumors. Intra-cardiac thrombus is more common in patients with central catheters, with a wall-motion abnormality and global hypokinesis. Certain autoimmune disorders may favor the development of intra-cardiac thrombus, and very rarely, multiple thrombi may be seen. Here, a patient with multiple intra-cardiac masses in the right ventricle and diagnosed with Behçet's disease is presented.

Incidental finding of multiple intracardiac masses in severe trauma patient: The challenges in the diagnosis and management of these masses and its implications on patients’ outcome

Intracardiac masses can be presented in multiple forms. Differentiating the type of the mass is often challenging but crucial to direct the management. This report describes a 31-year-old female who presented with multiple intracardiac masses after one hour of tranexamic acid administration. Multiple imaging modalities used in our patient to reach the final diagnosis of intracardiac thrombosis and to follow up the response to treatment. Tranexamic acid has been used commonly in trauma patients to reduce the risk of death with a good safety profile. Large prospective trials have failed to show a significant association of vascular occlusive events with TXA administration. This case demonstrates the need for close monitoring in post‐trauma TXA recipients to avoid thrombotic complications. Heparin was used successfully to treat these thrombi, and the patient was discharged on enoxaparin to finish six months course of treatment.

Manual Respiratory Support Increases Cardiac Parasympathetic Activity in Healthy Adults

Intracardiac masses can be presented in multiple forms. Differentiating the type of the mass is often challenging but crucial to direct the management. This report describes a 31-year-old female who presented with multiple intracardiac masses after one hour of tranexamic acid administration. Multiple imaging modalities used in our patient to reach the final diagnosis of intracardiac thrombosis and to follow up the response to treatment. Tranexamic acid has been used commonly in trauma patients to reduce the risk of death with a good safety profile. Large prospective trials have failed to show a significant association of vascular occlusive events with TXA administration. This case demonstrates the need for close monitoring in post‐trauma TXA recipients to avoid thrombotic complications. Heparin was used successfully to treat these thrombi, and the patient was discharged on enoxaparin to finish six months course of treatment.

A male with multiple cardiac masses

SummaryThirty-seven-year-old male presented with cough, dyspnea, significant weight loss (20 kg) and subacute fever for the past 2 months. Physical examination revealed inspiratory and expiratory wheezing bilaterally. A normal S1, S2 and a 3/6 systolic ejection murmur at the left upper parasternal border with respiratory variation were found during cardiac auscultation. Kidney and bone marrow biopsy reported a high-grade B-cell lymphoma. Echocardiography and cardiac CT findings consisted of multiple intracardiac masses affecting the right ventricular (RV) outflow track, RV apex, medial portion of the right atrium and posterior left atrium, as well as mild impairment of the RV systolic function. The masses in the RV outflow track caused partial obstruction (pulmonary valve peak velocity 2.3 m/s) with a RV systolic pressure of 43 mmHg. The infiltrative mass in the interatrial septum extended into both the right and left atrial cavities. The right superior pulmonary vein was occluded. This patient was treated with aggressive chemotherapy and had a good clinical response that resulted in mass size reduction after the first course of chemotherapy. Multimodality imaging techniques such as echocardiography, cardiac CT and PET scan can provide complementary information to better evaluate, stage and manage these patients.

Concomitant lipomatous hypertrophy and left atrial mass: Distinguishing benign from malignant

Masses in and near the interatrial septum may be either benign or malignant. The most common mass near the interatrial septum is lipomatous atrial septal hypertrophy (LASH). LASH can be present in patients with intracardiac malignancies, myxomas, lipomas, or other cardiac masses. It is important to recognize the transesophageal echocardiography (TEE) characteristics of these pathologies to arrive at an accurate diagnosis with an appropriate plan for intraoperative resection. At the authors' institution, patients have been referred for surgery due to a finding of significant LASH masquerading as a left atrial myxoma. In challenging cases, TEE offers a thorough evaluation of the interatrial septum to delineate between multiple intracardiac masses.

Antenatally diagnosed fetal cardiac tumor associated with tuberous sclerosis

Term male baby was born by normal vaginal delivery. The mother was followed-up in fetomaternal unit. Ultrasound report showed a huge mass arising externally from left ventricle free wall with multiple intracardiac masses with moderate pericardial effusion. A possibility of rhabdomyoma was considered. Baby was born with poor respiratory effort needed resuscitation and mechanical ventilation. Immediate postnatal echocardiography confirmed the presence of the extra and intra cardiac masses. Baby remained hemodynamically stable, developed one attack of supraventricular tachycardia and heart failure for which medical treatment given. Investigations confirmed the presence of tuberous sclerosis complex in this baby including magnetic resonance imaging and computed tomography (CT) scan that showed multiple subcortical and periventricular tubers'. CT abdomen showed angiomyolipoma of the left kidney, which disappeared completely after that. Baby remained free from neurological symptoms, discharged home on oral propranolol with multidisciplinary outpatient follow-up. His cardiac masses regressed significantly over his 2 years of life

Resection of Multiple Recurrent Cardiac Myxomas in an Adult Man With Carney's Complex

Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier.Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene.Fig 2View Large Image Figure ViewerDownload (PPT)The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.Fig 3View Large Image Figure ViewerDownload (PPT) Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier. Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene. The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.

Mitral and tricuspid valve papillary fibroelastomas: A rare cause of multiple intracardiac masses

A 55-year-old woman with no cardiac history presented with increasing shortness of breath on exertion, worsening cough and orthopnea for three weeks. A transthoracic echocardiogram identified two intracardiac masses. Transesophageal echocardiography demonstrated a 1.8 cm × 1.5 cm mass attached to the base of the posterior tricuspid leaflet (Figure 1A) and a 1.0 cm × 0.5 cm mass attached to the atrial side of the left mitral leaflet (Figure 1B). Both masses displayed indistinct surface features and neither was attached to the atrial wall or interatrial septum. No other intracardiac pathology was identified. The differential diagnosis included primary cardiac tumour, metastatic malignancy, thrombus or vegetation. After an extensive workup, the patient ultimately went on to have surgical excision of these masses. Surgical pathology confirmed the diagnosis: mitral and tricuspid valve cardiac papillary fibroelastomas (CPFs). Figure 1 CPFs are the second most common primary cardiac tumour in adults. They are benign avascular tumours made of papillary elastin fibrils in a hyaline stroma covered with a single layer of endothelium. These tumours occur most frequently on the aortic and mitral valves, and infrequently on the tricuspid and pulmonic valves. On echocardiography, CPFs are typically small, well-demarcated, highly mobile masses attached to the endocardium by a pedicle. They can have a highly refractive appearance, often with areas of echolucency and a ‘speckled’ appearance around the perimeter. Multiple CPFs are an exceedingly rare finding. While typically asymptomatic, CPFs can present with stroke, sudden death, myocardial infarction, heart failure, syncope, pulmonary embolism or peripheral infarction. To date, no recurrences of CPFs after surgical excision have been reported, and the long-term prognosis of these patients appears to be excellent (1–4).

Issue Highlights

HomeCirculationVol. 113, No. 10Issue Highlights Free AccessIn BriefPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessIn BriefPDF/EPUBIssue Highlights Originally published14 Mar 2006https://doi.org/10.1161/circ.113.10.1271Circulation. 2006;113:1271HEMODYNAMIC EFFECTS OF LONG-TERM CARDIAC RESYNCHRONIZATION THERAPY: ANALYSIS BY PRESSURE-VOLUME LOOPS, by Steendijk et al.Although it has been shown that chronic resynchronization therapy for heart failure exerts beneficial effects on clinical outcomes and cardiac remodeling, little is known about the long-term effects on myocardial function. Pressure-volume loop analyses were performed before and after 6 months of biventricular pacing in 22 patients with NYHA class III–IV heart failure. At 6 months there were improvements in left ventricular ejection fraction, dP/dtmax, and stroke work at both resting and increased heart rates. Of note, there were improvements in ventricular-arterial coupling and mechanical efficiency, as well. These data indicate that the initial improvements in myocardial function with resynchronization therapy are sustained during chronic therapy. See p 1295.IMPROVEMENT IN STROKE MORTALITY IN CANADA AND THE UNITED STATES, 1990 TO 2002, by Yang et al.The US Public Health Service recommended in September 1992 that all women of childbearing age consume 400 micrograms (μg) of folic acid daily to reduce their risk of having a pregnancy affected with spina bifida or other neural tube defects. Owing to the relationship between folic acid fortification and blood homocysteine levels, some experts have speculated that fortification may also have a favorable effect on cardiovascular health and stroke. The United States and Canada implemented policies of mandatory fortification in the late 1990s, and the populations of both countries experienced a reduction in blood homocysteine levels, a factor that appears to be related to the risk of stroke. Investigators from both countries collaborated to compare trends in stroke mortality in the United States and Canada, where these policies were mandatory, with England and Wales, where the policies were not. See p 1335.ADVANCED GLYCATION END PRODUCTS ACTIVATE A CHYMASE-DEPENDENT ANGIOTENSIN II–GENERATING PATHWAY IN DIABETIC COMPLICATIONS, by Koka et al.Angiotensin II is intimately involved in the development of cardiovascular complications of diabetes. Indeed, angiotensin-converting enzyme (ACE) inhibitors reduce, but do not prevent, the occurrence of clinical events in these patients. Interestingly, there are alternative pathways such as the enzyme chymase expressed in vascular tissue that are able to generate angiotensin II even in the presence of ACE inhibitors. In this study, Koka et al report that chymase is upregulated in the vasculature of diabetic patients. The upregulation of this alternative pathway is associated with tissue disposition of advanced glycation end products (AGEs), another consequence of hyperglycemia. AGEs induce chymase expression via the receptor for AGEs (RAGE)–Erk1/2 MAP kinase pathway. Under these conditions, chymase appears to account for the majority of angiotensin II produced in the vasculature, and this can be prevented by RAGE antibodies or inhibitions of Erk1/2 MAP kinase. This study therefore provides new insights into the cellular mechanisms of angiotensin II–induced vascular complications in diabetes and also a novel therapeutic target for the treatment of these high-risk patients. See p 1353.Visit http://circ.ahajournals.org:Clinician UpdateA Clinician’s Guide to Tissue Doppler Imaging. See p e396.Images in Cardiovascular MedicineOvarian Malignancy Presenting as Multiple Intracardiac Masses. See p e399.Bubble in the Heart: A Rare Cause of Mitral Regurgitation. See p e401. Download figureDownload PowerPointSpontaneous Coronary Dissection: Computed Tomography Appearance and Insights From Intravascular Ultrasound Examination. See p e403.CorrespondenceSee p e406. Previous Back to top Next FiguresReferencesRelatedDetails March 14, 2006Vol 113, Issue 10 Advertisement Article InformationMetrics https://doi.org/10.1161/circ.113.10.1271 Originally publishedMarch 14, 2006 PDF download Advertisement

Ovarian Malignancy Presenting as Multiple Intracardiac Masses

A 38-year-old woman with no significant previous illness presented with progressive breathlessness and abdominal distension of 6 months’ duration. Examination noted tachycardia, a blood pressure level of 100/60 mm Hg, an elevated jugular venous pressure to the angle of the mandible, a middiastolic heart sound, dullness at both lung bases, and hepatomegaly. Chest x-ray confirmed cardiomegaly and bilateral pleural effusions. Echocardiography demonstrated 6 intracardiac masses, 3 in the left atrium, 2 in the right atrium, and 1 …

Localized Nodular Hypertrophy Mimicking Rhabdomyoma in the Fetal Heart: Prenatal Sonographic and Pathology Findings

Multiple intracardiac masses (ICM) are considered to be diagnostic of rhabdomyoma often associated with tuberous sclerosis. We describe a fetus with multiple ICM detected by fetal sonography at 18.7 wk gestation. The appearance and number were consistent with leading diagnosis of rhabdomyoma. Due to complications of pregnancy and extreme prematurity, the fetus did not survive. Autopsy showed the cardiac masses to be localized nodular hypertrophy (LNH) of the myocardium. No features of rhabdomyoma or tuberous sclerosis were present. In a review of the literature, similar lesions were reported in a child and two adults, perhaps as localized variants of hypertrophic cardiomyopathy. Our case does not, however, show the histopathologic features of hypertrophic cardiomyopathy. Isolated developmental abnormalities, such as in this case, can have a mass effect mimicking cardiac tumors. At the time of autopsy, the largest mass in the anterior wall of the right ventricle extended to and obstructed the right ventricular outflow tract.

Primary cardiac Burkitt lymphoma in a child

Primary cardiac lymphoma (PCL) is a rare and usually fatal malignancy, seldom reported in children. This report describes the case of a 10-year-old boy who presented with multiple intracardiac masses which, when biopsied, proved to be small non-cleaved cell (Burkitt's) lymphoma. The first two cycles of chemotherapy according to the LMB 96 protocol were given under close cardiological supervision, with good response. The treatment was then continued with full-dose chemotherapy, without any cardiological complication. The patient who was treated by chemotherapy alone remains in complete remission 36 months after the end of treatment and can presently be considered as cured, without late cardiac effect.

Cardiac tuberculosis with multiple intracardiac masses: A case report

In this report, clinical, echocardiographic, and pathologic findings of a patient with multiple masses caused by tuberculosis both in the left and right side of the heart are presented. After antituberculosis treatment some of the masses disappeared and some became smaller. Although an intracardiac mass caused by tuberculosis is very rare, it should be considered in the list of masses detected by echocardiography. (J Am Soc Echocardiogr 2002;15:756-8.)

Multiple intracavitary cardiac masses; an uncommon presentation of African Burkitt's lymphoma

A 29-yr-old patient is described, who presented with rapid general deterioration and right heart failure. Two-dimensional echocardiography revealed massive intracardiac tumor involving the right atrium, the right ventricle and the left atrium. At histology the diagnosis of Burkitt's lymphoma was made. This report presents what we believe to be the first case of a patient with primary cardiac manifestations and multiple intracardiac masses in this disease.

Multiple intracardiac masses complicating peritoneo-venous (LeVeen) shunt

Multiple intracardiac masses complicating peritoneo-venous (LeVeen) shunt