This preliminary study demonstrates the incremental value of three-dimensional transesophageal echocardiography (3DTEE) over two-dimensional transesophageal echocardiography in the assessment of intra-cardiac tumors regarding its attachment in two different patients, one with myxoma and other hemangioma, histologically proven. As live/Real time 3DTEE has ability to visualize desired objects in multi-plane inside out, we clearly defined the attached portion of tumor and measured the en face view dimensions of area, which correlate well with surgeonâ€™s finding. In addition with improved tissue characteristics in 3DTEE, heterogeneous myxoma and highly vascular hemangioma were differentiated based on their tissue content, pre-operatively. On the contrary 2DTEE being biplane, measurement of area is cumbersome; also comment on area of attachment has difficulty in complex tumors, and so to differentiate tumor on basis of their tissue characteristics.
Key words: real time three-dimensional transesophageal echocardiography, three-dimensional echocardiography, two-dimensional transesophageal echocardiography, intra-cardiac tumor, tumor attachment, myxoma, hemangioma.
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With universal acceptance of echocardiography as initial choice of examination, along with improved therapeutics, intra-cardiac tumors started falling into arena of one of curable entities (1, 2). With few differentials for intracardiac masses including tumors, thrombi and vegetation, it is customary to differentiate between them as soon as possible to avoid mounting complication risks (1). Traditional 2- dimensional transthoracic echocardiography (2DTTE) can visually distinguish between them and so useful in preliminary, as well as follow-up examination (3). Together with 2DTTE, 2D trans-esophageal echocardiography (2DTEE) provides extensively specific information about intra cardiac masses in almost all cases (2).
It is essential to perform appropriate study before deciding about definitive therapeutic approach. To visualize complex intra-cardiac tumor structures in three dimensional planes, choices left to physicians are between computed tomography (CT), magnetic resonance imaging (MRI) and 3-dimensional (3D) echocardiography. Although with wider plane of focus of study with CT and MRI, they apart from cardiac structures, also concentrates on mediastinal, pulmonary, and thoracic anatomy (4). However they lack in their ability when it comes to live/real time study, hemodynamic assessment, and portability to site of examination while delineating cardiac pathology. Here 3-dimensional echocardiography has an advantage, besides being much cost effective (5).
In this study we reported two different patients of right ventricular (RV) tumor, namely myxoma and hemangioma, evaluated with 2DTEE followed by 3DTEE. In both patients instruments used were Philips iE33 system and a transesophageal X7-3t probe (Andover, MA, USA). For 3DTEE 4.0 MHz 4X matrix probe was used and images cropped online using 6th version of Q-Lab software (14-16).
Although 2DTEE demonstrates tumor size, extent, attachment, mobility and its hemodynamic consequences (17), however 3DTEE apart from this, differentiated tissue content of tumor, which helps in narrowing differential of tumor (10). In case of myxoma we visualized large echolucent foci suggestive of hemorrhage or necrosis, whereas in hemangioma similar echolucent foci appeared in large numbers, highly suggestive of its vascular origin. In addition, on sequential, multi-plane cropping up to the base of tumor reflected brighter area, which is relatively immovable compare to other part of tumor. Anticipating this as an attached portion of tumor, we acquired these images and measured en face offline with help of Philips Sonos 5500 ultrasound system (Andover, MA, USA) as the Philips iE33 does not support this.
47-year-old women presented with symptoms of heart failure (progressive leg swelling, dyspnea on exertion after walk of 200 m (NYHA class- III), generalized malaise) and palpitation of 4 weeks duration. Further clinical examination revealed a grade 3/6 systolic murmur at lower left sternal-edge; other examination was within normal limits. Transthoracic, followed by trans-esophageal echocardiography revealed a pathologic mobile tumor attached under the septal leaflet of tricuspid valve, along interventricular septal portion in right ventricular outflow track (RVOT) with severe tricuspid regurgitation (TR). Dimensions measured in length and breadth is 3.5 and 2.4 cm respectively. Further examination with 3D TEE performed in same setting, obtained pyramidal dataset on systematic sectioning visualized globular, smooth surface mass attached under the septal leaflet of tricuspid valve, along inter-ventricular septum. In Qlab tumor mass measured, 4.17 X 2.45 X 2.27 cm in dimensions. On further sequential cropping we detected brighter area under the base of tumor. Assuming this as attachment, measurement turns out to be 1.78cm and 1.36 cm in length and breadth respectively; whereas area measured 2.15 cm2while circumference 9.79cm. Tumor has great mobility as it protrudes through pulmonary valves during systole albeit in diastole partial bulging into right atrium. Furthermore along the sequential sectioning, we noted the echolucent foci inside tumor, suggestive of hemorrhagic or necrosis, along with brighter speckled foci of calcification.
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Work up of her palpitation reveled hyperthyroidism. She made euthyroid before surgery. During surgery, in order to excise tumor in totality, tricuspid valve resected and repaired. Tumor was attached with broad base, measuring 1.5 X 1.5 cm. Post-op 2 DTTE showed no evidence of tumor, with minimal TR. The pathological specimen revealed dispersed network of spindle cells and myxoid matrix along with evidence of congestion and hemorrhages consistent with a right ventricular myxoma. Her further hospital stay was uneventful except from few hiccoughs of allergic reaction to anti-thyroid medications, which managed conservatively, and she discharged in stable condition with her thyroid medication.
58-year-old woman diagnosed outside of cardiac tumor on CT scan, has intermittent nonspecific chest pain (chest discomfort) for 7-8 years duration. She was operated for total abdominal hysterectomy due to intra-uterine myoma, 16 years ago, also has right hemidiaphragm paralysis (cause unevaluated). There were no evidence of embolic events in past. Subsequent 2-dimensional trans-thoracic echocardiography and transesophageal echocardiography reveled 3.0cm X 1.6cm, intra- cardiac globular mass, attached along the free wall of right ventricle. No evidence of valve abnormality noted. Further 3DTEE examination performed to delineate tumor thoroughly before surgery. It showed hyper lucent tumor attached along the free wall of right ventricle as broad base with up and down movement rather to and fro during ventricular contraction, dimensions measured in Qlab are 3.08 X 2.39 X 1.55 cm. Acquired pyramidal shaped dataset on appropriate cross- sectioning visualized multiple echolucent foci of hemorrhages with little tissue sparing between them. In order to delineate the attached margin of tumor, tumor cropped from its free margin toward immobile attached portion. Visualized brighter portion of tumor as possible attachment site, measurements in first case was 2.79 cm length, and 2.26 cm breadth; whereas area measured 3.74 cm2 and calculated circumference 7.5 cm. Dimensions measured on Chest CT was 2.7X2.0 cm. Also some of incidental findings noted were small nodule of 0.6X0.5X0.4 cm in right median lobe of lung and 67.0 mm calcified mass in liver. She further underwent surgery after obtained medical and surgical fitness. Surgery was uneventful. Post surgical specimen measured 2.5X3.0 cm. Attachment was along right ventricular free wall with broad base. On histological examination tumor identified as hemangioma comprising of multiple foci of vascular tissues along with few plasma cells
Previously, studies described the incremental value of 3DTEE over 2DTEE with respect to tumor morphology, localizing attachment (6, 7), relation with surrounding anatomy, hemodynamic complications (8, 9). Despite the fact that 2DTEE can localize the possible attachment site of tumors, and so in present study; however no studies till date described its ability to measure the attachment portion of tumor, mainly due to its limited vision. 3DTEE however, besides focusing on the brighter attached portion of both tumors, it showed here its competency to measure this area en face, which later correlate well with surgical measurements. In case of hemangioma as tumor has broad base attachment as per surgical notes, its measurement correlates with 3DTEE findings. 2DTEE underestimated tumor dimensions in both patients (11, 18). Tissue characteristics are better delineated with the help of 3DTEE (6, 10). Myxoma histologically constitutes mucinous tissue mixed with cystic foci of hemorrhages. Presence of calcifications, rarely bony tissues and hematopoietic cells makes its nature heterogeneous (1, 12); whereas hemangiomas are common vascular tumors filled with multiple blood and blood vessels (12, 13). Cardiac presentation of hemangioma is very rare, in ratio of 1:15 compare to uncommon Myxoma (13). On 3DTEE Myxoma appears as protruding irregular mass into cardiac cavity, with similar contrast with surrounding structures, attached to one of cardiac walls or rarely to the valves (1). Sequential cropping of myxoma visualized foci of echolucent spaces inside tumor consistent with hemorrhage or necrosis; calcification appears as speckled, brighter structures, indicative of their long time presence (9, 10). While in second patient of hemangioma similar echolucencies noted as in myxoma, however those were multiple, extensive occupying almost whole content of tumor at the cost of tissue structure in between. These findings were very suggestive of the nature of tumors pre-operatively. Being relatively immobile, attached portion gives firm consistency to other part of tumor. This portion of tumor is rich in ground substance of collagen (19) which appears brighter on echocardiography examination (6, 10). Similarly, findings noted in fibroma; tumor with rich content of fibrous tissue collagen appears very brighter on 3D echocardiography platform (10, 12).
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Although many literatures has published on intra-cardiac tumor till date, and role of CT, MRI and 2DTTE previously explained in studies, however to our knowledge non has shown its ability to measure the attached portion of tumor. With wide expanding arm of minimal invasive cardiac surgeries in practice, in future 3DTEE will be crucial in demonstrating these findings in our belief. However further quantitative analytic study may ascertain the fact of our preliminary demonstration.
Eventually, 3DTEE has incremental value over 2DTEE, in respect to defining attached portion of tumor, their measurements as well as tissue characteristics. Retrieved full volume pyramidal shape dataset of desired area of interest with 3D echocardiography has much meaningful information hidden inside it, to reveal this absolutely one needs skillful and knowledgeable cardiologist for its meticulous dissection.