Intracardiac extension of metastatic pulmonary leiomyosarcoma

Leiomyosarcomas are uncommon malignancies of soft tissue associated with a poor prognosis due to local and metastatic disease. We describe an example of metastatic pulmonary leiomyosarcoma with intracardiac extension in a patient who had previously undergone resection of a primary retroperitoneal mass. Distinguishing metastatic malignancy within the heart from a primary tumour, such as atrial myxoma, may be difficult, with the use of various imaging modalities assisting in diagnosis and management. In this case, the patient's course was complicated by dramatic progression with widespread emboli resulting in peripheral arterial occlusion and stroke.     

Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature

Intravenous leiomyomatosis is an uncommon clinical entity characterized by the growth of a benign, smooth muscle tumour within the venous system. Intracardiac extension of this tumour is rare, and approximately 35 cases have been reported in the literature. The second case of massive intracardiac extension of intravenous leiomyomatosis is reported in a pregnant patient diagnosed after the unusual presentation of seizure activity. Tumours were successfully removed from the right internal iliac vein, inferior vena cava, and right atrium and ventricle in a single-stage operation using cardiopulmonary bypass and circulatory arrest. Concomitant total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Nine months after diagnosis, the patient was evaluated for recurrent disease. A review of the literature from 1994 to 1998 is presented.     

Intracardiac electrophysiologic study of intravenous diltiazem and combined diltiazem-digoxin in patients

Fifteen patients without sinoatrial (SA) or atrioventricular (AV) node dysfunction underwent electrophysiologic study (EPS) before and after intravenous diltiazem: 0.20 mg/kg bolus followed by 0.0007 mg/kg/min infusion (seven patients) or 0.25 mg/kg bolus followed by 0.0012 mg/kg/min infusion (eight patients). In six patients intravenous digoxin (0.018 mg/kg) was given and 45 minutes later EPS was repeated while the diltiazem infusion continued. Diltiazem prolonged sinus cycle length (+7%, p < 0.01), lengthened AH conduction time (+22% in constant rate atrial paced rhythm, p < 0.001), prolonged AV node functional and effective refractory periods (+6%, p < 0.01 and +16%, p < 0.05, respectively), lengthened AV node Wenckebach cycle length (+13%, p < 0.001), shortened atrial functional refractory period (?3%, p < 0.05), and reduced mean arterial pressure (?8%, p < 0.005 in constant rate atrial paced rhythm). Subsequently, intravenous digoxin further prolonged sinus cycle length (+12%, p < 0.05), AH nonduction time (+17%, p < 0.05), AV node Wenckebach cycle length (+9%, p < 0.05), and AV node functional refractory period (+7%, p < 0.05), shortened atrial effective refractory period (?7%, p < 0.05) and ventricular effective refractory period (?6%, p < 0.05), and increased systolic arterial pressure (+6%, p < 0.05). Diltiazem and digoxin have additive depressant effects on SA and AV node function without significant adverse effects.     

延伸至心脏的静脉内平滑肌瘤7例并文献复习

目的探讨延伸至心脏的静脉内平滑肌瘤的临床特点。方法回顾分析7例心脏受累的静脉内平滑肌瘤患者的临床资料。结果 7例患者均为女性,年龄41～49岁,平均(45±3)岁。其中5例(71%)患者既往因子宫肌瘤行子宫全部或部分切除手术,1例左卵巢畸胎瘤行手术剔除;5例患者分别出现活动后气短、晕厥或肺栓塞症状。超声心动图检查在右心房和(或)右心室内发现肿物,活动度较大,可以堵塞三尖瓣口;肿物均向下腔静脉延续。所有患者均成功接受了静脉和心腔内瘤栓取出术,随诊1例患者盆腔肿瘤复发,余6例患者无复发。结论静脉内平滑肌瘤病的发生率低,侵入心脏的病例更为罕见,明确诊断后应立即进行手术。提高对本病的临床表现和影像学认识有助于早期诊断和治疗。     

心内平滑肌瘤病的临床及心脏超声特点

目的:探讨心内平滑肌瘤病(intracardiac leiomyomatosis,ICL)的临床和心脏超声的特点.方法:回顾性分析经手术和病理证实的9例ICL的临床和心脏超声表现.结果:9例中有7例曾因子宫肌瘤和(或)子宫静脉、宫旁静脉内平滑肌瘤病进行过手术治疗;7例有心脏相关的症状,5例为首发表现,3例为静脉梗阻症状,1例以盆腔包块为首发表现;胸闷、胸痛、呼吸困难、心悸、胸骨左缘3～5肋间收缩期杂音、腹胀、水肿和盆腔包块较为常见.影像学检查中,5例以心脏超声为最初的检查手段,主要表现为下腔静脉向右心腔内匍行延续的团块样或条索样肿物回声,随心动周期在心腔内摆动.结论:ICL具有特定的病史和心脏超声特点,大多数患者有心脏相关的症状;诊断的关键在于对该病的认识和警惕性;心脏超声在疾病的初筛、诊断、病情评估和治疗随访中有重要的作用.     

Echocardiographic study of an intravenous leiomyoma: case report and review of the literature

Intravenous leiomyoma is a rare tumor of smooth muscle with invasion to veined channels that affects women at reproductive age. The case of a 45-year-old woman is described, with history of oophorectomy and hysterectomy, in addition to abdominal surgery due to mesenteric tumor. Several months later, the patient developed syncope and dyspnea secondary to pulmonary embolism. Echocardiographic study reported a mass in right side of heart proceding from inferior vena cava. The patient underwent tumor surgical resection from left iliac vein and histologic study concluded intravascular leiomyomatosis.     

Intracardiac echography

The role of echocardiography during non-coronary interventions is of increasing importance. They include percutaneous closure of atrial septal defects (ASD) or patent foramen ovale (PFO), percutaneous balloon mitral valvuloplasty (PMV), septal alcoholization, or interventional procedures managing arrhythmia. In all cases, echographic monitoring enables to guide the procedures, optimize and assess their results and avoid complications. This role could even increase with the development of other interventional techniques such as left auricle appendage exclusion or the percutaneous treatment of valvular diseases. This article reviews this new approach and its value in interventional cardiology.     

Intracardiac myxoma

Six intracardiac myxomas and 2 thrombi were subjected to scanning electron microscopy (SEM). Surface morphology of myxomas was distinct from thrombi. The surface of myxoma was either smooth or thrown into projections which had a continuous endothelial lining. This was lacking in thrombi. Cut surface of myxoma revealed the presence of round to polyhedral cells having blunt villi and/or pit-like depressions on their surface in a stroma of fibres, granular material and blood vessels. Thrombi were composed of blood elements enmeshed within fibres. No cells of the type encountered in myxomas were present.     

Intracardiac echocardiography

This article describes currently available intracardiac ultrasound (ICE) technology contrasting it with intravascular ultrasound (IVUS) highlighting their differences. Clinical applications in the electrophysiologic and cardiac catheterization laboratory are discussed and current limitations addressed. Intracardiac echocardiography (ICE) is possible because lower frequency transducers (in contrast to higher frequency IVUS devices) have been miniaturized and mounted onto catheters capable of percutaneous insertion into the heart. These lower frequency transducers are capable of enhanced tissue penetration, permitting high-resolution 2D "whole heart" imaging. Also, with the introduction of the newest phased array transducer, Doppler hemodynamic data in addition to high resolution imaging can also be obtained. ICE facilitates electrophysiologic procedures by guiding transseptal catheterization, enabling endocardial anatomy visualization and targeting of arrhythmogenic substrate, ensuring optimal ablation electrode/tissue contact and promptly diagnosing procedural complications. Promising non-electrophysiologic applications include guidance of percutaneous closure of septal defects, percutaneous mitral balloon valvuloplasty and complex cardiac biopsy. Current limitations include monoplanar imaging, narrow field of view, and relatively large size of the catheter. Intra-cardiac imaging is now a clinical tool and has the potential to play an important role in diagnostic and therapeutic interventional procedures. Further refinement and miniaturization of these transducers, through continued technological progress, will make way for primary operator controlled, integrated ultrasound-guided interventional devices.     

Unexpectedly long intravenous and intracardiac extension of a small-sized pulmonary pleomorphic carcinoma

Pleomorphic carcinoma of the lung is one of the uncommon histological types of lung cancers, which shows an aggressive behavior. Intravenous extension (not metastasis or direct invasion) of the tumor into the heart is a rare complication of lung cancers. We present a case of a 64-year-old man, who was admitted to hospital due to severe dyspnea. Chest CT scan revealed a 2-cm nodule in the upper lobe of the right lung. Echocardiography demonstrated a giant mass in the left atrium. Because of a considerable distance between the lung nodule and heart, the relation of these two lesions was unclear. He died four days after the admission. At autopsy, the lung nodule was pleomorphic carcinoma composed of spindle and giant cells, which invaded the pulmonary vein and extended intravenously to the left atrium. The intravenous component of the tumor measured approximately ten cm in length. At the tip of the extension, an 8 cm × 5 cm × 3 cm mass was formed in the left atrium, which obstructed the mitral valve. This case highlights a possibility that even a small-sized, peripherally located pleomorphic carcinoma of the lung could extend for an unexpectedly long distance to the heart, causing cardiac complications.     

Unknown Intracardiac Electrogram

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Unknown Intracardiac Electrogram

Watch a video presentation of this article. For additional educational resources, please visit Medtronic Academy  .     

Unknown Intracardiac Electrogram

Watch a video presentation of this article. For additional educational resources, please visit Medtronic Academy  .     

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)     

Unknown Intracardiac Electrogram

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