心脏乳头状弹力纤维瘤的研究进展

心脏乳头状弹力纤维瘤（PFEs）是一种罕见的良性心脏肿瘤,通常位于左心瓣膜上。PFEs对心脏血流动力学和瓣膜功能影响微弱,所以大多数患者无临床症状,但也可能会出现危及生命的并发症。PFEs在经胸超声心动图检查、心脏手术和尸检中偶有发现。PFEs的治疗方法目前还存在争议。该病的发病率及危险因素尚无确切报道。随着超声心动检查技术的发展和普及,该病的检出率不断提高。     

A pedunculated left ventricular hemangioma initially misdiagnosed as thrombus in a woman with atypical chest pain

The incidence of cardiac masses increased as echocardiography is becoming increasingly popular. Benign tumors of the heart constitute about 72% of all primary cardiac neoplasms and hemangioma accounts for 5–10% of benign cardiac tumors. Cardiac hemangiomas are generally asymptomatic and diagnosed incidentally during echocardiography or magnetic resonance imaging (MRI). We reported a 52-year-old woman presented with atypical chest pain and exertional dyspnea. The echocardiographic examination revealed a hyperechoic round mass in the left ventricle. With an initial diagnosis of left ventricular thrombus, the patient underwent cardiac MRI. The mass was found compatible with cardiac hemangioma. It was removed surgically and histopathologic evaluation identified a cardiac hemangioma. As reports of cardiac hemangioma are extremely rare and cardiac masses are mostly thought to be thrombi or myxomas (being the most common primary cardiac tumor), such hemangioma cases warrant attention as possibility of hemangioma should also be kept in mind.     

Cardiac tumors: clinical and echocardiographic diagnosis of 65 cases

65 cases of cardiac tumors were diagnosed and studied by echocardiography. Among them 49 were primary tumors including 43 cases of myxoma, one case each of hamartoma, lipomatous infiltration, fibroma, hemangioma, rhabdomyosarcoma, pericardial mesothelioma, and 16 cases of secondary cardiac tumors. It was found that the nature of primary cardiac tumors could be speculated by two-dimensional echocardiography based on their pathological features. Most patients with large left atrial myxoma had obstructive symptoms of mitral valves, abnormal ECG and enlarged left atrium, whereas patients with small atrial myxoma, embolic phenomenon was liable to occur. Echocardiography of secondary cardiac tumors showed that the tumors usually invaded most frequently both the myocardium and pericardium as single or multiple nodular echoes in the myocardium under pericardium or within the pericardial cavity, with profuse pericardial effusion. Occasionally, the secondary tumor appeared as a large mobile intracavitary mass or an extracardiac one compressing the heart or large vessels. It was noticed that cardiac symptoms might be the clinical clue in certain patients with extracardiac primary malignancy.     

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.     

Cardiac papillary fibroelastoma. Experience of an institution

Primary intracardiac tumors are rare, with prevalence between 0.0017% and 0.19% from non-selected autopsy studies. Approximately 75% are benign and almost half of them are myxomas. The remaining tumors are divided among rhabdomyomas, lipomas and fibroelastomas. Myxomas are the most common intracardiac tumors in adult age and rhabdomyomas the most common among pediatric population. Papillary fibroelastoma (PFE) is a relative rare benign heart tumor, corresponding to approximately 8% of intracardiac tumors. They most commonly manifested in cardiac valves. In the past, they either consisted of necropsy findings or were found in surgical procedures at random. In vivo diagnosis was sporadic. With the improvement of echocardiography techniques, PFE has been more frequently diagnosed. They are usually described as a movable, pedunculate, well-delimited mass and with predilection for valve endocardium. Therapeutic proposal, when they are pedunculate, is surgical resection, preventing cerebral, pulmonary, coronary or peripheral embolic phenomena. Five cases diagnosed in our institution, in the period from August 1995 to June 2004, will be presented.     

Metastatic involvement of the heart and pericardium--MR case reports

Secondary metastatic tumors of the heart and pericardium are in comparison with primary cardiac tumors more common, but their overall prognosis is very poor. Metastases can occur in every part of the heart and pericardium. The most common source are tumors--lung cancer, breast cancer, melanoma and lymphoma. In this case report we described two patients with metastatic involvement of the heart. We are pointing out the possibilities of diagnostic imaging of cardiac tumors using echocardiography and magnetic resonance.     

Atrial myxoma: case report and a review of the literature

Atrial myxomas are the most common benign primary tumor of the heart and occur in as many as 3 in 1000 patients. These tumors are a major cause of patient morbidity and mortality. Although the majority of atrial myxomas occur in the left atrium, 3 separate familial myxoma syndromes can result in multiple myxomas in atypical locations. Approximately 50% of patients with myxomas may experience symptoms due to central or peripheral embolism or intracardiac obstruction, but 10% of patients may be completely asymptomatic. Screening for myxomas should involve a thorough history and physical examination and a transthoracic and/or transesophageal echocardiogram. Transthoracic echocardiography is approximately 95% sensitive for the detection of cardiac myxomas, and transesophageal echocardiography approaches 100% sensitivity. Though the majority of atrial myxomas are sporadic, it is imperative that first-degree relatives of patients with documented myxomas undergo screening for occult myxomas. Surgical removal of the myxoma is the treatment of choice and usually curative; however, myxoma recurrence does occur and is most frequently associated with a familial syndrome.     

Transesophageal versus transthoracic echocardiography in the evaluation of right atrial tumors

The purpose of this study was to define the contribution of transesophageal echocardiography to the diagnosis of right atrial tumors in a large series of patients with this rare finding. Transesophageal echocardiography (TEE) has been found to be valuable in evaluating patients with intracardiac masses and has been shown ton be superior to transthoracic echocardiography (TTE) in evaluating left heart masses. Between 1989 and 1993, 23 patients with either known tumors elsewhere or right atrial masses that were detected on TTE were evaluated with TEE. TEE studies were performed in the noninvasive cardiology laboratory. All patients were studied with 5 MHz biplane or omniplane transducers. The right atrium was evaluated for the presence, characteristics, extent, and attachment of any masses and for extension of these masses into the great vessels or other cardiac chambers. No complications occurred. Six patients had primary right atrium tumors. In 10 patients the tumors reached the right atrium via the inferior vena cava. Seven patients had malignant secondary tumors. TEE demonstrated three tumors not detected by TTE. Furthermore TEE provided 16 additional findings not seen on TTE. In conclusion, TEE is superior to TTE in the evaluation of right atrial tumors. TEE should be considered in patients with right atrial tumors even when these tumors have been demonstrated with TTE.     

Use of echocardiography in detecting cardiac sources of embolus

Up to 20% of all ischemic strokes are felt to be the result of emboli from the heart. High resolution transthoracic (TTE) and transesophageal (TEE) echocardiography have been the principal diagnostic tools for detecting associated cardiac abnormalities and for guiding medical and surgical approaches to these patients. In addition to identifying the precise location and morphological characteristics of intracardiac masses, echocardiography has improved our ability to predict embolic potential of these masses. Specific cardiac lesions that are predisposed to stroke and are readily identifiable by echocardiography include: cardiac thrombi, valvular vegetations, cardiac tumors, aortic atheroma, atrial septal aneurysm, and regional left ventricular wall abnormalities. Careful interrogation of patients with cerebrovascular accidents has identified a potential cardiac source of embolus in approximately 30%. This is largely due to the advent of TEE, which has provided much better assessment of posterior cardiac chambers including left atrium and left atrial appendage. Use of TEE in identifying a cardiac source of embolus is indicated in patients with stroke who are young, have no apparent cerebrovascular disease, or have recurrent embolic events. Echocardiography is an essential diagnostic tool in evaluating patients with a suspected cardiac source of embolus. TTE and TEE provide invaluable information regarding the majority of cardiac sources of embolus.     

Transseptal Biopsy of a Left Atrial Mass with 3-Dimensional Transesophageal Echocardiographic Guidance

A 31-year-old man presented with a large cardiac mass that originated from the basal posterior left atrial wall and occupied most of the dilated atrium. Minimally invasive studies yielded inconclusive results, but the patient was considered at high risk for an open cardiac biopsy due to the size of the mass. To establish a tissue diagnosis for definitive treatment, we performed a transseptal cardiac biopsy guided by 3-dimensional transesophageal echocardiography. We thereby determined that the mass was a primary left atrial sarcoma. To the best of our knowledge, this is the first report concerning the use of 3-dimensional transesophageal echocardiography for biopsy of a left atrial mass.Key words: Biopsy/methods, echocardiography, 3-dimensional, echocardiography, transesophageal, heart atria/ultrasonography, heart neoplasms/diagnosis, sarcoma/diagnosis, tumors, cardiacCardiac tumors are infrequently encountered in clinical practice. Treatment options are guided by location, stage, and tissue diagnosis. If imaging does not provide a conclusive diagnosis, a biopsy is performed. In obtaining specimens for biopsy, the operator encounters more difficulty with left-sided than with right-sided masses. We describe a left atrial tumor in which the biopsy specimen was obtained by means of a transseptal puncture with 3-dimensional (3D) transesophageal echocardiographic (TEE) guidance.     

Calcified mass in the right atrium extending into the inferior vena cava with pulmonary artery embolization. Typical or atypical myxoma?

Cardiac masses are divided into neoplastic and non-neoplastic. They usually represent a diagnostic challenge given their relative rarity, their infrequent symptoms, and the overall difficulty with dynamic imaging of the heart. While echocardiography is useful in the initial evaluation of a suspected mass, cardiac magnetic resonance (CMR) imaging is the best imaging modality to characterize cardiac tumors due to its superior tissue characterization and its higher contrast resolution. For neoplastic, primary cardiac tumors are rare (0.05%). Atrial myxoma is the most common cardiac (50%) mass. About 75%-80% of myxoma are seen in the left atrium. Atypical myxoma is a term describing myxoma arising in other nonleft atrial locations. 20%-25% myxomas arise from the right atrium and 5% or less from the ventricles. We present a case of a 59-year-old female patient presenting with severe dyspnea. Her chest noncontrast CT showed a calcified mass lesion in the right atrium extending into the inferior vena cava. She underwent cardiac MRI for better tissue characterization. The cardiac MRI revealed a very irregular, highly spiculated, heavily calcified, heterogeneous, and nonenhancing lesion within the right atrium extending into the inferior vena cava. Via dynamic imaging, no evidence of mobile components was present. Via T1, T2 along with pre- and postcontrast imaging, the mass was confirmed to be calcified without a fibrotic component or evidence of thrombus. The above findings raised the possibility of atypical myxoma.     

Carcinoid heart disease and the utility of 3D trans-thoracic and trans-esophageal echocardiography: Two clinical cases

Carcinoid tumors are rare neuroendocrine malignancies. We present two cases of metastatic carcinoid tumors, complicated by carcinoid syndrome and by cardiac valve involvement. Carcinoid syndrome is characterized by secretory diarrhea, episodic flushing, and bronchospasm. Cardiac involvement occurs in up to 50% of patients with metastatic carcinoid tumors which commonly causes abnormalities of the right sided valves. Echocardiography is the best available and non invasive technique for diagnosis. Characteristic features of carcinoid heart disease are thickened, shortened, retracted, and fixed or partially fixed valve leaflets. Three-dimensional (3D) echocardiography provided an en face view of pulmonary and tricuspid valve, not obtainable by two-dimensional echocardiography, and improved delineation of the relationship between these structures and cardiac chambers.     

Extracardiac manifestations of atrial myxomas

Primary cardiac tumors are extremely rare and constitute only about 5% of all cardiac tumors. Cardiac myxomas are noncancerous primary tumors of the heart and constitute about of 50% of all primary heart tumors. Left-sided atrial myxomas are more common than right-sided atrial myxomas. Atrial myxomas can lead to a triad of complications. The most common symptoms are associated with obstruction due to the size and location of the tumor. The next most common symptoms are associated with pulmonary and systemic embolization. Patients may also present with constitutional symptoms. Diagnosis is made via means of transesophageal echocardiography and magnetic resonance imaging. Early diagnosis and surgical resection remain the treatment of choice to prevent complications. Patients usually have a good prognosis after resection.     

Cystic tumor in the left atrium: an unusual aspect of a myxoma

Most cardiac tumors are benign, whereas up to 50 % of the diagnosed cases are histologically myxomas. The common clinical signs are rhythm disturbances, myocardial ischemia, pulmonary edema, syncope and cardiac arrest. They do normally lead to the diagnostic hypothesis of an intracardiac mass. The primary modality for imaging is echocardiography which usually confirms the suspected diagnosis. But in rare cases there are masses which cannot be exactly identified by this technique. Here we present a patient with an atypical echocardiography of an unusual intracardiac tumor.     

Cardiac hamartoma. Case report and literature review

Primary cardiac tumors are infrequent and usually benign. They can manifest as dyspnea, chest pain, palpitations, sudden death, peripheral embolism, cyanosis, or general symptoms. They are sometimes an incidental finding in an asymptomatic patient. We describe a 33-year-old man who was seen because of dyspnea and palpitations. Transthoracic echocardiography revealed, on the lateral wall of the left ventricle, an intramyocardial mass that was successfully resected surgically. The pathologic diagnosis was hamartoma of mature cardiac myocytes. We discuss the usefulness of imaging techniques for identifying cardiac masses.     

L'imagerie en coupe dans le bilan des tumeurs intra-cardiaques

The etiology of cardiac masses is often oncological or thrombotic, rarely inflammatory. Among heart tumors, the vast majority are metastatic. We describe the most frequent benign primary cardiac tumors and the most frequent malignant primary cardiac tumors and give information about the advantages of using a multi-modality approach for the accurate diagnosis of a cardiac mass using Computed Tomography Scanner and Magnetic Resonance Investigation.     

Multiple recurrences of nonfamilial cardiac myxomas: a report of two cases

Cardiac myxomas are the most common primary cardiac tumors. Surgical resection usually provides definitive treatment; however, postoperative tumor recurrence has been reported, especially when myxomas occur as part of a familial pattern. Only a few cases of 2nd recurrence of nonfamilial cardiac myxoma have been reported. We report 2 cases of nonfamilial cardiac myxoma, with multiple recurrences after surgical resection. The possibility of repeated recurrence of cardiac myxomas demonstrates the importance of regular echocardiography after surgical resection in order to detect such recurrence. Future studies, including genetic analysis of patients with recurrent cardiac myxomas, are warranted to investigate the nature of these tumors.     

Cardiac infections in the immunocompromised host

Bacterial infections of the heart in compromised patients are uncommon but may be increasing in relative frequency. They are associated with near-equal frequencies in patients who have pre-existing or simultaneous infections at other sites, patients with neoplasms (usually solid tumors), those who have undergone cardiac surgery, and intravenous drug or ethanol abusers. Staphylococcus aureus is the bacteria most often identified, in contrast to a preponderance of Streptococcus isolated in the pre- and early antibiotic era. Gram-negative bacilli are the causative organisms in a significant number of cases. The endocardium remains the most common site of infection, and left-sided valves are most commonly involved, especially the mitral valve, often in the absence of pre-existing valvular damage. The majority of cases are associated with involvement at other sites, and premortem blood cultures are positive in approximately two thirds of patients who undergo autopsy. Fungal infections involving the heart do not occur as often as bacterial infections. The frequency, however, is increasing. Fungal infections usually occur in patients who have received treatment with antineoplastic agents, antibiotics, or corticosteroids, alone or in combination, or who have had abdominal or cardiac surgery. Candida is the organism found most frequently and usually causes myocarditis or endocarditis, whereas Aspergillus involves the myocardium but may be invasive to the endocardium or pericardium. Other fungi that occasionally infect the heart include Phycomycetes and Cryptococcus. Dissemination is usually present and involves the lungs, kidneys, brain, or gastrointestinal tract. The diagnosis may be difficult prior to death, because blood cultures are positive in only one third of patients who come to autopsy. Precipitin tests and antibody titers for specific organisms may be helpful in providing early diagnosis. Survival rates can be improved by early diagnosis and the institution of aggressive antifungal treatment.     

Redundant Mitral Valve Simulating an Intracardiac Mass on Transesophageal Echocardiography

Cardiac ultrasound is a useful diagnostic modality for the evaluation of cardiac masses and tumors. However, normal cardiac structures and normal variants can mimic masses with both transthoracic echocardiography and transesophageal echocardiography. We report two patients in whom a redundant mitral leaflet simulated cardiac tumor attached to the mitral valve by transesophageal echocardiography. (ECHOCARDIOGRAPHY, Volume 11, May 1994)     

Transthoracic echocardiographic features of cardiac pheochromocytoma: a single-institution experience

Background: Cardiac pheochromocytoma is extremely rare. Previous papers usually are reports of a single case. Transthoracic echocardiography (TTE) offers a useful option, but the features of cardiac pheochromocytoma on TTE have not been favorably reported. In this study, the findings of cardiac pheochromocytoma on TTE in nine cases were presented. Methods: TTE images (especially two‐dimensional ultrasound) of nine patients with cardiac pheochromocytomas were analyzed retrospectively and compared with the findings from surgery. Results: Among the nine patients with cardiac pheochromocytomas identified in Peking Union Medical College Hospital (PUMCH) clinical and echocardiographic database, TTE identified one cardiac tumor in seven cases (77.8%), two cardiac tumors in one case (11.1%), and a false‐negative result in another (11.1%). Cardiac pheochromocytomas were usually located on the base of the heart, near the origin of great arteries. The tumors were usually round or ovoid, ranging from 1.4 cm to 7.7 cm in diameter, with homogeneous and moderate echoes and low activity. They could press or invade surrounding cardiac structures and influence hemodynamics. In this study the majority of cardiac pheochromocytoma seemed marginated and appeared to be encapsulated on TTE. Apical four‐chamber view and parasternal short‐axis view of the aortic valve were most effective in identifying cardiac pheochromocytomas. The findings on TTE were similar to those from surgical procedures. Conclusion: Cardiac pheochromocytomas presented characteristic TTE appearances in aspect of location, size, texture, and shape of tumors. Understanding of these characteristics on TTE can help correctly recognize this extremely rare disease. (Echocardiography 2012;29:153‐157)