Fulminant primary cardiac lymphoma with sudden cardiac death: A case report and brief review.

Primary cardiac lymphoma (PCL) is very rare, with the variable clinical manifestations potentially leading to a delayed diagnosis. PCL is usually detected incidentally through image studies, whereas the diagnosis can be confirmed via analysis of pericardial effusion, endomyocardial biopsy tissue, or surgical specimens. Although no standard therapy has been established for PCL, without treatment, the prognosis is grave, with the estimated overall survival being approximately 1 year. We report a difficult diagnosis and complicated case of fulminant PCL, which is the first comprehensively reported case of PCL with secondary hemophagocytosis. A man presented with progressive dyspnea for 3 weeks, and then sudden cardiac death with ventricular fibrillation occurred. After resuscitation, echocardiography revealed a thickened left ventricular wall and severe mitral regurgitation, and computed tomography showed a right atrial mass with diffuse myocardial lesions. PCL was confirmed through a pathological analysis of specimens collected during mitral valvuloplasty, which also implied extensive myocardial involvement. Bone marrow biopsy demonstrated no evidence of lymphoma involvement, but secondary hemophagocytosis was noted. Despite aggressive chemotherapy, the patient died of sepsis with multiorgan failure 26 days after the operation.

Right Anterolateral Minithoracotomy versus Median Sternotomy Approach for Resection of Left Atrial Myxoma.

BACKGROUND: Minimally invasive surgery has become the standard approach for several cardiac diseases. In this retrospective study, we compared right anterolateral minithoracotomy (RALT) with standard median sternotomy (SMS) for resection of left atrial myxoma (LAM). MATERIALS AND METHODS: From January 2009 to June 2015, the clinical data of patients who underwent RALT (n = 30) and SMS (n = 36) for resection of LAM in our hospital were collected. The preoperative clinical data and operative results were compared between the two groups. RESULTS: There were no significant differences in aortic cross-clamp and cardiopulmonary bypass time between the two groups. The total incision length was significantly shorter in RALT group compared with SMS group (p < 0.001). For RALT and SMS groups, respectively, the intensive care unit length of stay was 29.2 ± 6.5 versus 43.5 ± 6.9 hours (p < 0.001), and the postoperative hospital length of stay was 5 days (interquartile range [IQR]: 4-6) versus 8 days (IQR: 7-10) (p < 0.001). The total cost in RALT group was 27,000 RMB (IQR: 25,000-29,000) versus 33,000 RMB (IQR: 31,000-35,000) in SMS group (p < 0.001). There were no significant differences in mortality and postoperative complications between the two groups. CONCLUSION: RALT approach for LAM resection can be performed safely with favorable cosmetic outcome, accepted clinical results, and lower cost. It should be considered as a promising alternative to SMS and merit additional study.

Intrapericardial paraganglioma: The role of integrated advanced multi-modality cardiac imaging for the assessment and management of rare primary cardiac tumors.

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Cardiac Sarcoma Arising From Malignant Transformation of a Preexisting Atrial Myxoma.

A 49-year-old man presented with shortness of breath on exertion, orthopnea, and chest pain. On transthoracic echocardiography, a myxomatous mass was seen in the left atrium. A diagnosis of atrial myxoma was made and the patient underwent urgent surgical intervention. At operation, the mass, which extended into the anterior leaflet of the mitral valve, was excised. Histopathologic examination showed a primary cardiac sarcoma arising within a preexisting atrial myxoma. The patient received no further treatment and died 7 months postoperatively. We report the first case of a malignant transformation of an atrial myxoma to a cardiac sarcoma.

Computerized tomography angiography in preoperative assessment of intravenous leiomyomatosis extending to inferior vena cava and heart.

BACKGROUND: Intravenous leiomyomatosis (IVL) extending to inferior vena cava and heart is one of the most challenging conditions for surgical treatment. We explored the use of computerized tomography angiography (CTA) in preoperative assessment for this disease. METHODS: A cohort of 31 patients with IVL extending to inferior vena cava and heart were reviewed from the year 2002 to 2014, focusing on the preoperative CTA imaging characteristics and the surgical procedures in clinical treatment. RESULTS: All patients were diagnosed correctly combining the clinical medical history and CTA imaging. Thirteen patients had tumors confined within the inferior vena cava, and 18 patients had tumors intruding into the right heart. Furthermore, 15 tumors were located in the right atrium alone, and 3 tumors involved both the right atrium and the right ventricle. All patients had simple or multiple soft tissue masses from the pelvis, with 22 tumors extending into inferior vena cava through the iliac veins and 9 tumors through the ovarian veins. Three patients had tumors invading into lung and underwent tumor thrombus resection in the pulmonary artery. Patients received either one-stage surgery or two-stage surgery dependent on patient general condition and tumor status. All operations were successfully performed by multidisciplinary cooperation, including gynecology, cardiac surgery, and vascular surgery, without severe surgical-related complications or deaths. CONCLUSIONS: CTA imaging can present location, size, and full-scale extension pathway of IVL lesions, and can be used as first-line imaging technique in preoperative assessment, having great significance in making surgical plan and obtaining successful outcome.

Incremental value of live/real time three-dimensional transesophageal echocardiography over the two-dimensional technique in the assessment of primary cardiac malignant fibrous histiocytoma.

We describe a case of primary cardiac malignant fibrous histiocytoma where live/real time three-dimensional transesophageal echocardiography added incremental value to the two-dimensional modalities. Specifically, the three-dimensional technique allowed us to delineate the true extent and infiltration of the tumor, to identify characteristics of the tumor mass suggestive of its malignant nature, and to quantitatively assess the total tumor burden.

Incremental value of live/real time three-dimensional transesophageal echocardiography over the two-dimensional modality in the assessment of cardiac lymphoma.

We describe a case of cardiac lymphoma where live/real time three-dimensional transesophageal echocardiography provided additional information compared to two-dimensional transesophageal echocardiography regarding the extent of tumor infiltration. In addition, it gave a quantitative assessment of the tumor burden by providing its volume.

Integrated 18F-FDG PET/MR imaging in the assessment of cardiac masses: a pilot study.

UNLABELLED: The objective of the present study was to evaluate whether integrated (18)F-FDG PET/MR imaging could improve the diagnostic workup in patients with cardiac masses. METHODS: Twenty patients were prospectively assessed using integrated cardiac (18)F-FDG PET/MR imaging: 16 patients with cardiac masses of unknown identity and 4 patients with cardiac sarcoma after surgical therapy. All scans were obtained on an integrated 3-T PET/MR device. The MR protocol consisted of half Fourier acquisition single-shot turbo spin-echo sequence, cine, and T2-weighted images as well as T1-weighted images before and after injection of gadobutrol. PET data were acquired simultaneously with the MR scan after injection of 199 ± 58 MBq of (18)F-FDG. Patients were prepared with a high-fat, low-carbohydrate diet in a period of 24 h before the examination, and 50 IU/kg of unfractionated heparin were administered intravenously 15 min before (18)F-FDG injection. RESULTS: Cardiac masses were diagnosed as follows: metastases, 3; direct tumor infiltration via pulmonary vein, 1; local relapse of primary sarcoma after surgery, 2; Burkitt lymphoma, 1; scar/patch tissue after surgery of primary sarcoma, 2; myxoma, 4; fibroelastoma, 1; caseous calcification of mitral annulus, 3; and thrombus, 3. The maximum standardized uptake value (SUVmax) in malignant lesions was significantly higher than in nonmalignant cases (13.2 ± 6.2 vs. 2.3 ± 1.2, P = 0.0004). When a threshold of 5.2 or greater was used, SUVmax was found to yield 100% sensitivity and 92% specificity for the differentiation between malignant and nonmalignant cases. T2-weighted hyperintensity and contrast enhancement both yielded 100% sensitivity but a weak specificity of 54% and 46%, respectively. Morphologic tumor features as assessed by cine MR imaging yielded 86% sensitivity and 92% specificity. Consent interpretation using all available MR features yielded 100% sensitivity and 92% specificity. A Boolean 'AND' combination of an SUVmax of 5.2 or greater with consent MR image interpretation improved sensitivity and specificity to 100%. CONCLUSION: In selected patients, (18)F-FDG PET/MR imaging can improve the noninvasive diagnosis and follow-up of cardiac masses.

Assessment of pericardial diseases and cardiac masses with cardiovascular magnetic resonance.

Imaging plays an important diagnostic and prognostic role in the assessment of pericardial diseases and cardiac tumors and in differentiating these conditions from other cardiac and noncardiac diseases. A number of imaging modalities are available for this task; each has advantages and limitation. Cardiovascular magnetic resonance (CMR) is a highly versatile imaging modality that provides detailed anatomical information, tissue characterization, cardiac function assessment, and evaluation of the impact of these conditions on hemodynamics. In this review we focus on the current state-of-the-art application of cardiovascular magnetic resonance in assessing pericardial diseases and cardiac tumors.

Assessment of cardiac masses: magnetic resonance imaging versus transthoracic echocardiography.

OBJECTIVE: The purpose of this study is to compare the role of magnetic resonance imaging (MRI) with transthoracic echocardiography (TTE) for characterization of cardiac masses. METHODS: Twenty-three patients were examined with a 1.5T MRI Scanner and TTE for the evaluation of suspected intracardiac masses. MRI examinations were performed with a protocol of steady cine imaging, HASTE and 3D IR-FLASH sequence after administration of gadolinium chelate. All patients were examined with MRI within a period of 1-2 weeks after TTE. RESULTS: According to the results of MRI, 15 patients underwent cardiac surgery. All of the operated patients were proven to have cardiac tumors with no false positive diagnosis on MRI. In 3 of 8 unoperated patients who were diagnosed to have cardiac tumors on TTE, cardiac thrombi were identified on MRI. Two patients with Eustachian valve in the right atrium on MRI were misinterpreted as having cardiac masses on TTE. In one patient with known renal cancer, metastases were diagnosed within the myocardium on MRI. TTE demonstrated a mass in the left atrium in one patient with a history of myxoma operation, whereas MRI was able to show the paracardiac extension of the mass into pulmonary veins and lung metastases, which are indicators of malignant transformation. In one patient, a mediastinal mass compressing the left atrium on MRI was misinterpreted as an intracavitary lesion with TTE. CONCLUSION: Contrast enhanced MRI is a noninvasive method superior to TTE in diagnosis, differentiation, and detection of extension of cardiac masses.

Utility of cardiac CT and MRI for the diagnosis and preoperative assessment of cardiac paraganglioma.

BACKGROUND: Cardiac paragangliomas are rare cardiac tumors that are usually benign. Surgical excision can be curative. METHODS: We report a case of 39-year-old male who, during the work up of acute coronary syndrome with coronary angiography, cardiac computed tomography (CT) and magnetic resonance imaging (MRI), was found to have cardiac paraganglioma. RESULTS: The tumor was intrapericardial, arising at the level of proximal left anterior descending artery. The tumor was completely resected and the postoperative course was uneventful. At 3-months follow-up the patient was asymptomatic with normal ventricular function. CONCLUSION: Cardiac CT and MRI are valuable in characterizing and preoperative planning of primary cardiac paragangliomas.

Assessment of the vascularity of a left atrial mass using myocardial perfusion contrast echocardiography.

Emerging applications of myocardial contrast echocardiography (MCE) include the evaluation of myocardial perfusion, the improvement of the definition of intracavitary structures, and evaluation of the relative perfusion of a cardiac mass. We present a case of a patient that was found incidentally to have a cardiac mass on transthoracic echocardiography. MCE was used to evaluate the vascularity of the mass. This case is compared with another patient with a left atrial thrombus, which represents an "avascular" cardiac mass by MCE.

Imaging of cardiac masses and myocardial disease: self-assessment module.

The educational objectives for this self-assessment module on imaging of cardiac masses and myocardial disease are for the participant to exercise, self-assess, and improve his or her knowledge of imaging and the clinical features of cardiac masses and myocardial disease, with emphasis on CT and MRI.

Asymptomatic cardiac papillary fibroelastoma: diagnostic assessment and therapy.

Papillary fibroelastoma is a rare benign cardiac tumor with elevated risk for embolization. This report describes the case of a 65-year-old man, admitted for the occasional finding of a round, pedunculate mass adherent to the chordae of the anterior mitral valve leaflet, mimicking an endocarditic mass. Appropriate diagnostic evaluations lead to the suspect of a papillary fibroelastoma. Because of the elevated risk of thromboembolism, surgery was emergently performed with complete removal of the mass and preservation of the integrity of the mitral valve. Histologic evaluation confirmed the diagnosis. Papillary fibroelastoma should be always considered in the differential diagnosis of intracardiac masses.