Recurrent intracardiac leiomyomatosis

An intravenous leiomyoma, a histologically benign smooth muscle tumour, arises from either a uterine myoma or the walls of a uterine vessel, with extension into veins. The present report describes echocardiographic features of an intravenous leiomyoma that spread into the right-sided cardiac chambers in a middle-aged woman who had undergone a hysterectomy two years earlier. Echocardiographic features included an elongated mobile mass extending from the inferior vena cava and multiple masses in the right atrium and right ventricle. Intracardiac leiomyomatosis should be considered in women who present with a cardiac mass in the right-sided chambers.     

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.     

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)     

Intravenous leiomyomatosis: A rare cause of intracardiac mass

Intravenous leiomyomatosis is an unusual clinical condition characterized by histologically benign smooth muscle lesions extending from the uterus into pelvic and systemic veins and, more rarely, into the right cardiac chambers. We report the case of a 45-year-old woman who presented with a three-week history of dyspnea on exertion, shortness of breath and fatigue. Echocardiography showed a large mobile mass in the right atrium prolapsing into the right ventricle and extending to the inferior vena cava. A computed tomography scan revealed a large mass extending from the right atrium to the inferior vena cava and through the systemic veins as far as the popliteal veins. A presumptive diagnosis of large thrombus was made; the correct diagnosis of intravenous leiomyomatosis with intracardiac involvement was obtained only after surgical resection and histologic examination.     

Intravenous leiomyoma with cardiac involvement.

We report a case of intravenous leiomyomatosis (IVL) in a 44-year-old woman, presenting with cardiothoracic clinical manifestations. Such a rare neoplastic condition most often arises from the uterine veins. At times it may reach the right cavities of the heart and it must be considered in the differential diagnosis with a cardiac myxoma or a thrombus.     

Partial Anomalous Pulmonary Venous Return with Intact Atrial Septum

Partial anomalous pulmonary venous connection in association with an intact atrial septum is uncommonly encountered in adults. Symptoms may result if the single anomalously draining vein results in elevation of right-sided heart pressures, enlargement of these chambers, and pulmonary hypertension. The condition is clinically indistinguishable from an atrial septal defect of the ostium secundum type and often requires cardiac catheterization with oximetry, selective dye dilution curves, and angiography of the pulmonary veins for diagnosis. Correction of the abnormality surgically is associated with mini-mum morbidity and mortality, and often results in the complete resolution of symptoms and signs of right-sided hemodynamic overload.     

A Case of Uterine Leiomyomatosis Extending into the Right Heart with an Unusual Echocardiographic Appearance

Intravenous extension of uterine leiomyomatosis is a rare condition characterized by tumor invasion of the venous system and propagation toward the large abdominal veins. A few cases with extension to the right atrium have been reported, and the echocardiographic appearance of this entity is well described. The case we herein report is of interest because of its unusual echocardiographic findings.     

Human Coronary Venous Anatomy: Implications for Interventions

The coronary venous system is a highly variable network of veins that drain the deoxygenated blood from the myocardium. The system is made up of the greater cardiac system, which carries the majority of the deoxygenated blood to the right atrium, and the smaller cardiac system, which drains the blood directly into the heart chambers. The coronary veins are currently being used for several biomedical applications, including but not limited to cardiac resynchronization therapy, ablation therapy, defibrillation, perfusion therapy, and annuloplasty. Knowledge of the details of the coronary venous anatomy is essential for optimal development and delivery of treatments using this vasculature. This article is part of a JCTR special issue on Cardiac Anatomy.     

Validity of the hepatojugular reflux as a clinical test for congestive heart failure

This study describes observations designed to test the validity of the hepatojugular reflux as an indicator of actual or incipient heart failure. The central venous pressure (CVP) could be predicted from the height of the jugular venous pulsations in 44 of 48 comparisons. In the remaining comparisons, discrepancies ranged from 5 to 7 mm Hg. In patients with normal resting cardiac function, abdominal compression did not cause an increase in CVP of >2 mm Hg (2.7 mm H₂O). In 16 of 19 patients with impaired function, CVP increased by ≥3 mm Hg. The increase in CVP was estimated from neck veins to within 2 mm Hg in all but 3 instances. CVP stabilized by 10 seconds and did not change over the subsequent 60 seconds. Abdominal compression caused no consistent change in cardiac output. Changes in venous pressure could not be attributed to changes in esophageal pressure or to compression of the heart by elevation of the diaphragm. Observations were consistent with the hypothesis that an increase in right-sided cardiac filling pressures resulting from abdominal compression carried out as described here, reflects both the volume of blood in the abdominal veins and the ability of the ventricles to respond to increased venous return, and constitutes a useful clinical test for detecting congestive cardiac failure. An increase of 3 cm in the height of neck vein distention is a reasonable upper limit of normal.     

Vascular Smooth Muscle Tumors: 13 Cases and a Review of the Literature

Vascular smooth muscle tumors, benign or malignant, are rare and encompass a variety of neoplastic lesions characterized histologically by their similarity to adult smooth muscle tissue. Leiomyomatosis, a benign neoplasm, primarily arising from within the uterine and extrauterine venous systems, and leiomyosarcoma, a malignant neoplasm arising predominantly in larger veins, while relatively rare, are nevertheless a cause of significant morbidity and mortality. We present a review of case of leiomyomatosis and leiomyosarcomas seen over a 35-month period, and an 18-year review of the literature. All tissues were fixed in 10% formalin, photographed, examined, and gross features described. Tissue samples were processed for light microscopy and stained with hematoxylin–eosin. The clinical records were reviewed. Eight cases of intravascular leiomyomatoses and five cases of leiomyosarcomas are presented in this series. Gross findings of resected intravascular leiomyomatosis were remarkably similar, although tumor size varied greatly between patients. Histologically, the tumor masses were similar. Gross and histological analysis of leiomyosarcoma masses revealed tumors of varying grades of malignancy. Vascular smooth muscle tumors that extend through or directly involve the vasculature are rare and difficult to diagnose and manage. Surgical resection remains the most effective treatment, although chemotherapy in isolated cases has been shown to have a positive effect. Appropriate interpretation and diagnosis are crucial for effective management.     

Unusual thrombotic cardiac complications of <Emphasis Type="Italic">Pemphigus vulgaris</Emphasis>: a new link?

We are reporting a case of Pemphigus vulgaris with extensive thrombosis of deep veins, pulmonary veins, and cardiac chambers complicated by myocardial infarction. The extensive skin lesions prohibited the administration of thrombolytics and coronary intervention. The patient was treated conservatively with heparin, and oral anticoagulation in addition to steroids and cyclosporine with significant resolution of thrombosis.     

Intravascular leiomyomatosis: report of a case and review of the literature

So far, only 13 cases with extension of an intravascular leiomyoma into the heart have been reported. We present the second known case of leiomyomatosis with extension of the tumor through the tricuspid valve into the right ventricle, which was first removed successfully. Ten years after an abdominal hysterectomy for a myomatous uterus, a 53-year old woman presented with a intracavitary mass in the right-sided heart chambers which could be followed retrogradely into the inferior vena cava and right pelvic veins, representing an intravasal extension from the pelvic leiomyoma. The clinical picture, the two-stage operative management, and the postoperative course are reviewed and the literature on the subject is presented.     

Hepatic portal venous gas and "the aquarium sign" due to intussusception in kawasaki disease

The presence of hepatic portal venous gas (HPVG) may be secondary to bowel necrosis, mechanical distension, or intraabdominal sepsis. We describe an unusual and hitherto unreported presence of HPVG manifesting as gas embolization and the unique "aquarium sign" in a patient of Kawasaki's disease. Continuous passage of bubble-like echoes flowing from the hepatic portal venous system into the inferior vena cava and right-sided chambers of heart was noted on echocardiography. The patient was treated with intravenous immune-globulins and made an uneventful recovery.     

Simplifying the estimation of jugular venous pressure

The classic technique of estimating jugular venous pressure, with variable inclination of the upper body and the sternal angle as the reference point, is complicated and little used in general practice. The aim of this prospective, comparative study was to assess whether estimating neck vein distension with the patient in the sitting position could be used to detecting elevated venous pressure. Patients (n = 96) who underwent right-sided cardiac catheterization or endomyocardial biopsy were evaluated. The visible height of the right internal jugular venous column above the clavicle was estimated, and the mean pressure in the right atrium or superior vena cava at cardiac catheterization was measured. Invasive venous pressure was elevated (>8 mm Hg) in 23 patients. A deep venous column visibly distended above the right clavicle in the sitting position had sensitivity of 65% and specificity of 85% to identify truly elevated venous pressure. Abdominal compression increased sensitivity to 77% but decreased specificity to 68%. In conclusion, studying the deep neck veins of a sitting patient simplifies the estimation of jugular venous pressure and has moderate to high diagnostic performance in detecting elevated central venous pressure.     

Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum—does it matter?

Cardiac variant of total anomalous pulmonary venous return is a rare entity, whereby all the pulmonary veins drain directly to the right atrium or coronary sinus. The effective left heart blood flow channels through a small stretched patent foramen ovale and can often be confused with a variant of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly where pulmonary veins drain to a persistent chamber above the left atrium with a membrane separating these two. There persists either a small aperture directly from the true to the accessory left atrium or none at all. Where there is no such aperture, it is often physiologically akin to the cardiac variant of total anomalous pulmonary venous return described above. Such morphological differentiation is often challenging in a clinical situation, but the effective treatment remains the same. It involves removal of the common wall between the two chambers and baffling the pulmonary veins effectively to the left atrium. We describe such a case where the pulmonary venous return is to the right atrium, managed recently in our centre, and discuss the morphological differences between these two.     

Disorders of hemodynamics and pulmonary gas exchange in the acute period of myocardial infarct

In 40 patients with massive myocardial infarction, the central hemodynamics was examined by probing the right-sided chambers of the heart and the pulmonary artery. The cardiac output was measured by thermodilution. The gas exchange (respiratory minute volume, capnography, gas analysis of arterial and mixed venous blood) was explored. Arterial hypoxemia was found to be associated with increased intrapulmonary blood shunting in patients with acute myocardial infarction complicated by congestive heart failure. Deterioration of arterial hypoxemia was promoted by a combination of increased overall pulmonary shunting and decreased residual venous oxygenation. Abnormalities in the ventilation-perfusion relations are of great importance.     

Retrograde coronary venous contrast echocardiography: assessment of shunting and delineation of regional myocardium in the normal and ischemic canine heart

Coronary venous injections of sonicated Renografin-76 were performed in seven closed chest dogs during two-dimensional echocardiography to study the ability of this new technique to opacify regional myocardium before and after occlusion of the left anterior descending coronary artery. The balloon of a 4F double lumen catheter was inflated in the great cardiac vein for each contrast injection to prevent backflow through the coronary sinus into the right atrium. Retrograde injections before coronary artery occlusion generally resulted in patchy myocardial contrast uptake. Injections after coronary occlusion always resulted in confluent and transmural myocardial opacification which occupied 42.8 +/- 8.6% (range 26 to 54) (mean +/- standard deviation) of the myocardial circumference. Retrograde opacification always extended into adjacent myocardium beyond the ischemic zone, which was assessed in echocardiograms with antegrade contrast injections into the left main coronary artery and which measured 30 +/- 6.3% of the ventricular circumference. Shunting from the coronary venous system to cardiac chambers was evaluated in a parasternal four chamber view and was graded on a scale of 0 to 4+. Contrast appearance was equally intense in the right atrium and right ventricle (3.5 +/- 0.6+, range 2+ to 4+), less intense in the left ventricular cavity (1.5 +/- 0.6+, range 1+ to 3+) and absent in the left atrium. Postmortem anatomic validation with retrograde great cardiac vein injections of indocyanine green corroborated and in vivo contrast appearance in chambers. Retrograde coronary venous contrast echocardiography appears capable of providing in vivo information about the extent and location of myocardial zones that can be reached by retrograde infusions of therapeutic agents and about the ability of these agents to reach ischemic myocardium. In addition, this new method allows for in vivo evaluation of shunts between coronary veins and cardiac chambers, which may influence the efficacy of retrograde interventions.     

Pathophysiologic mechanisms of cardiac tamponade and pulsus alternans shown by echocardiography

A pericardial effusion is easily recognized by echocardiography, but the diagnosis of cardiac tamponade by echocardiography is controversial. Recently, several reports have indicated that right ventricular (RV) or right atrial (RA) diastolic collapse represent highly specific and sensitive signs of a hemodynamically significant pericardial effusion. This report evaluates the pathophysiologic significance of these findings in 3 patients. One patient had classic clinical and hemodynamic features of tamponade without typical echocardiographic features; 1 had typical echocardiographic features of tamponade without the characteristic clinical or hemodynamic features; and 1 had all the findings characteristic of tamponade, including mechanical and electrical alternans. The first patient had increased right-sided cardiac pressures and RV hypertrophy, which prevented RV or RA collapse. The second patient had low right-sided intracardiac pressures, which allowed RV and RA diastolic compression to occur during early and mid-diastole. In the third patient, severe holodiastolic impairment of right-sided filling, and presumed decreased pulmonary venous and pericardial compliance, in the setting of tamponade produced a beat-to-beat alternation of RV and left ventricular filling with associated electrical and mechanical alternans. RV or RA collapse during diastole occurs when intrapericardial pressure equals or exceeds intracardiac pressure. Increases in wall stiffness of chamber pressures may prevent diastolic collapse in the setting of tamponade. Conversely, extremely low intracardiac pressures may allow diastolic collapse to occur in the absence of overt cardiac tamponade. The extent and timing of the RA or RV collapse, rather than its mere occurrence, are important in the diagnosis of cardiac tamponade by echocardiography.     

Drenagem venosa pulmonar anômala em gestante identificada pela ressonância magnética cardíaca

Anomalous pulmonary venous return (APVR) is a rare cardiac anomaly defined as one or more pulmonary veins draining into a structure other than the left atrium, with venous return directly or indirectly to the right atrium. The most common form is partial APVR, in which one to three pulmonary veins drain into systemic veins or into the right atrium. We report the case of a woman diagnosed with partial APVR by magnetic resonance imaging during pregnancy.     

Intravenous leiomyomatosis with cardiac extension

A woman with intravenous leiomyomatosis experienced syncope 7 years after a total abdominal hysterectomy and oophorectomy for a uterine fibroid. The workup revealed a tumor that extended from the iliac veins to the right ventricle. It was totally removed under cardiopulmonary bypass. One year later, the patient was found to be asymptomatic and without evidence of tumor recurrence.