Three-dimensional Transesophageal Echocardiography in Tumors of ...

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transesophageal echocardiography was found to be a valuable way to define the morphologic and spatial characteristics of cardiac and paracardial tumors in.
Three-dimensional Transesophageal Echocardiography in Tumors of the Heart Nilda Espinola-Zavaleta, MD, PhD, Gunther Herna´ndez Morales, MD, Jesu´s Vargas-Barro´n, MD, Candace Keirns, MD, and Alberto Aranda Fraustro, MD, Tlalpan, Mexico

Three-dimensional echocardiography was used to study 5 women, 4 with intracardiac tumors and 1 with a paracardial neoplasia. Three-dimensional transesophageal echocardiography was found to be a valuable way to define the morphologic and spatial

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characteristics of cardiac and paracardial tumors in vivo, establish their relationships with adjacent structures, and assess the hemodynamic effects they can produce. (J Am Soc Echocardiogr 2002;15:972-9.)

rimary tumors of the heart are rare. Their incidence in autopsies varies between 0.0017% and 0.28%. Approximately 75% are benign,1,2 and surgical resection is often curative; even the indication of extensive resection to prevent recurrences is still doubtful.3 Treatment of primary malignant tumors of the heart is not as clear because of the biologic aggressiveness of these tumors and the limited experience in their management.4 Secondary cardiac tumors are much more common in adults and can arise from any malignant tumor except those of the central nervous system.2,5 Up until a few years ago identification of metastases to heart was only reached by autopsy6 despite advances in both diagnostic techniques and treatment schemes for different cancers. Tumors of the heart are the least studied in oncology.7 Today diagnosis is achieved in vivo with less invasive diagnostic methods such as transthoracic or transesophageal echocardiography, computed tomography (CT), and magnetic resonance imaging that have displaced cardiac catheterization, once the “gold standard” of evaluation. Angiography, including selective coronary angiogram, is now indicated only when the tumor exerts an effect on or compresses these arteries or coexists with coronary artery disease.3,8-11 It is clear that echocardiography has become the principal diagnostic technique of cardiac tumors and masses. Since the introduction of echocardiography in 1954, it has undergone important modifications, both in the nature of the images and instrumenta-

tion.12-14 It is now possible to create 3-dimensional (3D) reconstructions of normal and pathologic structures of the heart that improve anatomic details, particularly when a transesophageal probe is used. The internal structure of the heart is represented in a realistic way, which means that in the future this technique may become a routine way to perform many serial sections, opening up a new field of exploration. Moreover, it has proved to be a powerful tool in the 3D visualization of the heart beating.15,16 In this article we will describe our experience with 5 patients on whom multiplane transesophageal echocardiography was performed with 3D reconstruction. Echocardiographic images were analyzed and compared with surgical findings.

Reprint requests: Jesu´s Vargas-Barro´n, MD, Department of Echocardiography, Insituto Nacional de Cardiologı´a Ignacio Cha´vez, Juan Badiano No. 1, Colonia Seccio´n XVI, Tlalpan, 14080 Me´xico, DF, Me´xico. Copyright 2002 by the American Society of Echocardiography. 0894-7317/2002/$35.00 ! 0 27/1/121535 doi:10.1067/mje.2002.121535

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MATERIALS AND METHODS Five women with an average age of 39.4 years (range 19 to 70) were included. Three had primary cardiac tumors, 1 had a primary paracardial tumor, and 1 had a metastatic mass. All underwent 2-dimensional transthoracic and 3D transesophageal studies. Four patients received operation, and histopathologic examination was performed. One of them also underwent CT-guided biopsy of a mass in her lung. Diagnosis was exclusively on the basis of clinical suspicion and echocardiographic findings in the other 2 patients.

Two-dimensional and color, pulsed, and continuous wave Doppler echocardiographic studies were performed with a 2.5 MHz phased-array probe (HP Sonos 5500, HewlettPackard, Andover, Mass). Images were recorded on VHS videotape for later interpretation.

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Figure 1 A, Three-dimensional (3D) reconstruction of myxoma from 4-chamber apical section. In systole, myxoma is in left atrium, and in middle portion area of multiple cavities can be seen corresponding to necrosis (arrow). In diastole, myxoma prolapses into left ventricle. Arrows indicate rounded area of different acoustic density (necrotic area), and pedicle of myxoma implanted in interatrial septum on left side. B, 3D image of myxoma at 70 degrees. Arrow shows pedicle. C, 3D study of myxoma at 40 degrees. Pedicle is observed with implantation in interatrial septum (arrow) and round area of necrosis (arrow). LA, Left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle. 3D Echocardiography Tranesophageal echocardiography was performed on all patients using the HP Sonos 5500 system (Hewlett-Packard), which has the capability for acquisition of serial cross-sectional images by a multiplane probe. With patients under conscious sedation, cross-sectional images with increments of 2 degrees were acquired with cardiac and respiratory cycle gating. Ninety sequential cross sections were obtained during each complete heart cycle, resulting in a conical volumetric data set encompassing tumor shape and morphology. To estimate the degree of neoplastic invasion at the atrial level, electronic parallel slicing (paraplane echocardiography) of both atria was performed in 1 case (leiomyosarcoma). The stored serial cross-sectional data in the optical disk were transferred to computer using software (Echoscan, version 3.1, Tomtec Imaging System) for analysis of the 3D echocardiographic data set as previously described.15,17 The analysis program of the 3D echocardiographic system performed data processing off-line. Cardiac cross

sections were formatted in their correct sequence according to their ECG phase in cubic data sets (256 " 256 pixels/8 bits). The postprocessing of the data set was performed offline with the reconstruction software. Image data were converted from polar to Cartesian coordinate format and interpolated to “fill the gaps” between sequential crosssections and reduce spatial artifacts created by patients, probe movement, or respiration. Image Display The tumors could be visualized in computer-generated transgastric, transesophageal apical 4- and 5-chamber views and upper transesophageal images from the 3D set. Volume-rendered Display Once an appropriate cut plane had been chosen, a threshold value was selected to differentiate cardiac structures from the blood pool and background. The gray scale

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Figure 2 Pathologic specimen of myxoma. Upper image is longitudinal section of myxoma showing interior areas of necrosis. Lower image shows external aspect of whole tumor.

Figure 3 Pathologic specimen of cardiac leiomyosarcoma. Multiple rounded masses of different sizes can be seen. information of the structures was rendered and a gradient shading algorithm enhanced the views. Image Analysis Two experienced observers analyzed all the echocardiograms.

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Case 1. This patient was 70 years old and had a 10-year history of arterial hypertension. Her illness began 6 months before with shortness of breath with moderate exertion that advanced to shortness of breath at rest and paroxysmal nocturnal dyspnea. On occasion she perceived palpitations but had no syncope. The physical examination revealed hypertension, tachycardia, and tachypnea. The point of maximum impulse was displaced to the fifth intercostal space on the anterior axillary line; and there was a constant fourth sound and loud second sound. Blood cytology, coagulation tests, urinalysis, and renal function tests produced normal findings. The ECG demonstrated biatrial enlargement. Cardiac catheterization was performed, and a pedunculated vascular image was observed in left atrium that prolapsed into the left ventricle. The coronary arteries showed no obstructive lesions. Transthoracic echocardiography was suboptimal. Transesophageal echocardiography showed an irregular, pedunculated left atrial mass of 58 " 19 mm and heterogeneous density that prolapsed into the left ventricle during diastole. Implantation was in the interatrial septum. Threedimensional echocardiography demonstrated a mass that occupied a large part of the left atrium. The pedicle implanted in the interatrial septum, and the mass prolapsed into the left ventricle during diastole. Numerous cavities could be seen (Figure 1, A, B, and C). At operation, a pedunculated myxomatous tumor with implantation in the interatrial septum was found. Histopathologic examination confirmed the myxomatous origin of the 50 " 20-mm mass. Old hemorrhagic areas corresponded to the necrotic zones discovered with 3D echocardiogram (Figure 2). Case 2. This 31-year-old patient had never had menstrual irregularity. Three weeks before she began to experience progressive shortness of breath, dry cough, dysphagia, fatigue, weakness, and weight loss of 3 kg. On physical examination hypotension, tachycardia, and tachypnea were found with mild jugular ingurgitation, bilateral crepitating rales, no murmurs, a constant third heart sound, and a loud second sound. Enlarged abdominal organs were not palpated. Laboratory findings were within normal limits. The ECG showed repolarization abnormalities of the inferior wall and elevation of the J point from V1 to V3. The chest radiograph revealed grade II cardiomegaly and venocapillary pulmonary hypertension. There was no evidence of pulmonary metastases. Abdominal ultrasound produced normal findings. A transthoracic echocardiogram showed normal-sized cardiac chambers. A solid mass filling the entire left atrium was observed that caused obstruction of left ventricular filling and severe tricupsid regurgitation with a systolic pulmonary arterial pressure of 86 mm Hg. Transesophageal echocardiography demonstrated a large solid mass in left atrium adhering to the interatrial septum and roof of the left atrium that protruded into the superior pulmonary veins and atrial appendage with obstruction of left atrial emptying. The early/late filling ratio of mitral flow was inverted.

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Figure 4 Transesophageal 2D color Doppler image demonstrates shunt at interatrial septum (arrow). LA, Left atrium; RA, right atrium.

Figure 5 Transesophageal 3D image at 50 degrees shows multiple tumors (arrows) in right ventricle, left ventricle (LV), left ventricular outlet, and left atrium (LA). AO, aorta. The surgical findings included a large infiltrative tumor in left atrium that involved superior pulmonary veins. The neoplasia was completely removed (Figure 3). Histopathologic study showed it to be a leiomyosarcoma. Immunochemical analysis was positive for vimentin, desmin, smooth muscle-specific actin, and HHF35 and negative for S 100 protein, factor VIII, and myoglobin. A control transesophageal study performed 4 months later revealed multiple neoplastic masses in left atrium, left atrial appendage, interatrial septum, and right ventricular outlet. A solid mass with heterogeneous reflectance and some irregular, mobile projections was seen at the junction of the superior vena cava. Color Doppler dem-

Figure 6 Three-dimensional tomographic section at level of both atria. Tumor masses can be observed in left atrial chamber (head arrows) with infiltration of interatrial septum. Arrow points to loss of continuity of interatrial septum corresponding to a perforation. LA, Left atrium; RA, right atrium. onstrated an interatrial shunt secondary to septal perforation from infiltration (Figure 4). Three-dimensional echocardiography confirmed multiple tumors and aided in clarifying the morphological characteristics of the left ventricle, left ventricular outlet (Figure 5), and interatrial septum (Figure 6). The perforation of the interatrial septum secondary to infiltration was again observed (Figure 6). The patient died 2 months after the last echocardiographic study. Case 3. Patient No. 3 was a 19-year-old woman with a history of a heart murmur detected when she was 2 years

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Figure 7 Three-dimensional reconstruction at 120 degrees. Round mass is evident in left ventricular outlet with homogeneous density of a probably fibroma (arrow). AO, Aorta; LA, left atrium.

Figure 8 Three-dimensional transgastric image that provides macroscopic view of cystic mass. Various cavities of differing sizes (arrow) and acoustic density can be observed within it. old but not followed-up. A year and a half before admission she began to experience oppressive precordial pain of an intensity rating 5 of 10 without radiation, lasting 30 minutes. This occurred on 3 occasions. She did not have fever or weight loss. Her vital signs were at normal levels with an apical grade II/VI regurgitant murmur with radiation to axilla. The point of maximum impulse and pulses were at normal levels. Hepatosplenomegaly were not palpated. Laboratory findings included polyglobulia (hemoglobin 18.8 mg% and Ht 46%). The ECG showed abnormal repolarization of the left ventricular inferior wall. The chest radiograph showed no abnormalities. A transthoracic echocardiogram showed a thickening originating in the subaortic membranous septum without increased left ventricular outflow velocity. Transesophageal echocardiography demonstrated a small, round mobile image in the left ventricular outlet. A subaortic ring was dismissed and

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it was concluded that the image in the left ventricular outflow could correspond to an old vegetation or a fibroma. Aortic regurgitation was not found. Three-dimensional echocardiography made it possible to determine precise size, morphologic characteristics, and location of the small mass and strongly suggested that it was a fibroma. Vegetation was dismissed by absence of clinically relevant data and because of the echocardiographic characteristics of the mass (Figure 7). Case 4. Patient No. 4 was 23 years old and had an unremarkable history. Her symptoms began 6 months before with epigastric distress, exertional dyspnea, and paresthesias of the left arm. When she was examined her vital signs were within normal limits. The point of maximum impulse was palpated in the fifth intercostal space on the midclavicular line. There was a grade I/VI pulmonary ejection murmur and fixed splitting of the second heart sound. The neurological examination produced normal findings, as did the ECG. Cardiomegaly was not seen on the chest radiograph. Laboratory findings were within normal limits. A cystic extracardiac mass measuring 11 " 9 cm was observed on a transthoracic echocardiogram. This finding was corroborated by transesophageal echocardiography. The 3D echocardiogram showed a mass with multiple cystic areas in its interior of different sizes and densities (Figure 8) located on the anterior mediastinum, beside the heart. The patient had undergone and operation and the histopathologic examination of cystic mass was compatible with mature teratoma (Figure 9). Case 5. Patient 5, 54 years old, had a history of asthma during her adolescence. Four years before she had undergone a hysterectomy because of a uterine leiomyosarcoma. Her presenting illness began 1 month before with shortness of breath on moderation exertion that progressed to mild exertion, orthopnea, and paroxysmal nocturnal dyspnea. She also developed a productive cough. The chest radiograph showed radio-opaque images in both lungs. Computed tomographic scan of the chest was then performed showing solid images in both lungs, 1 of which was in contact with the left edge of the heart. She was sent to our institution with a diagnosis of probable secondary cardiac tumor. On physical examination her vital signs were within normal limits. The point of maximum impulse was palpated in the fifth left intercostal space on the midclavicular line. The first heart sound was loud and the second heart sound was within normal limits. A grade II/VI regurgitant murmur was auscultated over the apex with a short rumble. Pulmonary sounds were within normal limits. Liver and spleen were of normal size. Laboratory determinations produced normal findings. The ECG showed in sinus rhythm and biatrial enlargement and right ventricular dilatation. The chest radiograph demonstrated grade I cardiomegaly and 3 radio-opaque images, 1 in the middle third of the right lung, 1 at the level of the aortic knob, and the other in the base of the left lung. All were quite large. Effusion between lobes of the right lung was also reported. The transthoracic echocardiogram showed normal

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Figure 9 Benign cystic teratoma in transversal section with abundant hair, sebaceous material, adipous tissue, cavities, and calcifications. image was visualized at the level of the porta hepatis that surrounded and compressed the inferior vena cava and the portal vein. A CT-guided pulmonary biopsy was performed. The histopathologic findings were compatible with leiomyosarcoma. Because the patient showed evidence of segmental pulmonary hypertension secondary to infiltration of the left superior pulmonary vein, she underwent resection of the cardiac mass. She is now in functional class II.

DISCUSSION

Figure 10 Three-dimensional reconstruction at 100 degrees of multilobulated mass that prolapses into left ventricle (LV) during diastole (arrows). Asterisks indicate mitral valve ring.

chamber diameter and left ventricular wall thickness. Ventricular function was within normal limits. A large mass adhering to the lateral wall of the left atrium was observed. With 3D transesophageal echocardiogram a large multiloculated tumor of variable reflectance measuring 8 " 4 cm was found in the left atrium. During diastole it prolapsed into the left ventricular inlet (Figure 10). Its implantation was identified in left superior pulmonary vein (Figure 11). Hepatic and biliary ultrasound revealed the liver to have homogeneous reflectance. A 3.7 " 3.0-cm hyperechoic

Primary tumors of the heart are rare, although they are seen with some frequency in concentration hospitals. The echocardiogram has become the diagnostic technique of choice because of its availability and noninvasiveness. Advances in echocardiography have enhanced the quality of the images, and today it is possible to create 3D reconstructions of normal and abnormal cardiac structures, including tumors and other masses. In some cases it is possible to visualize neoplasias that are outside but adjacent to the heart. Three-dimensional reconstructions can be derived from both transthoracic and transesophageal studies. In the case of the former approach problems often arise when deficient acoustic windows produce poor quality images. The latter technique is somewhat invasive and requires cooperation on the part of the patient. The rotational acquisition technique with a multiplane transesophageal probe makes it possible to obtain images of excellent quality if the probe is stabilized and the

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Figure 11 Three-dimensional image shows implantation of tumor (arrow). Head arrows point to tumor in left atrium (LA). AO, Aorta.

study is gated to respiration and heart rate.15 All of our reconstructions were performed using this technique. In the first case, myxoma was suspected because of the localization of the mass, its morphologic characteristics, and the patient’s symptoms. Histopathologic examination corroborated the diagnosis. The 3D reconstruction was a dynamic representation of the surgical specimen. These tumors are usually visible in a transverse plane of the 4-chamber image or in a section of the left ventricular outlet at 0 degree and 120 to 130 degrees. With 3D reconstruction, anatomy and pathology of the heart can be examined without having to perform a mental reconstruction of the problem.18 The second case involved a young woman whose echocardiogram performed 4 months after surgical resection of a leiomyosarcoma revealed multiple neoplastic implants in both left and right chambers. Implants in the right heart could have been disseminated by hematogenous route through the interatrial perforation. The chest radiograph showed no images suggesting pulmonary metastases and abdominal ultrasound produced normal findings. Immunohistochemical studies of the mass provided evidence of its origin. Patients with primary leiomyosarcoma have a poor prognosis; most of them die within 1 year after diagnosis,18 as occurred with our patient. These tumors require meticulous scrutiny with all possible transverse and longitudinal sections. The 3D reconstruction was created from images at the level of the aortic root with visualization of the left atrial appendage in an intermediate section (30 degrees) and transverse section (0 de-

grees) and the interatrial septum and both atria from paraplanar tomographic sections. In the third case, the 3D reconstruction helped to establish the precise echocardiographic characteristics of the tumor and its relationship with contiguous cardiac structures and showed a probable fibroma in the left ventricular outlet. Because there was no clinical evidence of left ventricular outflow obstruction conservative management with observation and echocardiography was continued. The fourth case involved an extracardiac tumor with multiple cavities of different sizes, shapes, and spatial relationships well-characterized by 3D echocardiography as corresponding to a probable teratoma, which was confirmed by histologic study. Paracardial masses are usually visualized in transgastric sections at 0 and 90 degrees. The final patient was a woman with a history of hysterectomy because of an uterine leiomyosarcoma. Metastatic masses were found in the lung and heart that originated the signs and symptoms of infiltration of the left superior pulmonary vein. The cardiac tumor was removed, also the obstruction of the left superior pulmonary vein. In 50% of autopsy cases of these tumors there are metastases in various organs from hematogenous dissemination.19 In this case the tumor corresponded to a secondary cardiac leiomyosarcoma with implantation in the left superior pulmonary vein from a primary uterine neoplasia. Three-dimensional echocardiography revealed the macroscopic appearance, implantation, shape, and acoustic density of the cardiac tumor using transesophageal images at 0 and 100 degrees. Metastatic liver disease was also identified.

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With these findings we can conclude that 3D echocardiography is the ideal method for morphologic and spatial characterization of cardiac and paracardial tumors in vivo. It provides the most complete information on the relationships of adjacent structures and the hemodynamic effects that tumors can produce.

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REFERENCES 1. McAllister HA Jr. Primary tumors of the heart and pericardium. Ann Pathol 1979;14:325-55. 2. McAllister HA Jr, Hall RJ, Cooley DA. Tumors of the heart and pericardium. Curr Probl Cardiol 1999;24:57-116. 3. Heath D. Pathology of cardiac tumors. Am J Cardiol 1968; 21:315-27. 4. Gabelman Ch, Al-Sadir J, Lamberti J. Surgical treatment of recurrent malignant rumor of the left atrium. J Thorac Cardiovasc Surg 1979;77:914-21. 5. Dein JR, Frist WH, Stinson EB, Miller DC, Baldwin JC, Oyer PE, et al. Primary cardiac neoplasms. J Thorac Cardiovasc Surg 1987;93:502-11. 6. Cardiovascular pathology. 2nd ed. New York (NY): Churchill Livingstone; 1991. p. 1297-333. 7. Chan HS, Sonley MJ, Moes F, Daneman A, Smith CR, Martin DJ. Primary and secondary tumors of childhood involving the heart pericardium and great vessels. Cancer 1983;56:825-36. 8. Lam KY, Dickens P, Chan AC. Tumors of the heart. Arch Pathol Lab Med 1993;117:1027-31. 9. Freedom RM, Lee KJ, MacDonald G, Taylor G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol 2000;21:299-316. 10. Boxer RA, LaCorte MA, Singh S, Shapiro J, Schiller M,

14.

15.

16. 17.

18.

19.

Goldman M, et al. Diagnosis of cardiac tumors in infants by magnetic resonance imaging. Am J Cardiol 1985;56:831-2. Mugge A, Daniel WG, Haverich A, Lichtlin PR. Diagnosis of noninfective cardiac mass lesions by two-dimensional echocardiography. Circulation 1991;83:70-8. Edler I, Hertz CH. Use of ultrasonic reflectoscope with a continuous recording of movements of heart wall. Kungl Fysiorg Sallsk Lund Forhandl 1954;24:40. Fyke FE, Seqard JB, Edwards WD, Miller FA Jr, Reeder GS, Shattenberg TT, et al. Primary cardiac tumors: experience with 30 consecutive patients since the introduction of twodimensional echocardiography. J Am Coll Cardiol 1985;5: 1465-73. Reeder GS, Khanderia BK, Seward JB, Tajik AJ. Transesophageal echocardiography and cardiac masses. Mayo Clin Proc 1991;66:1101-9. Veiga M de F, Lopes MG, Pinto FJ. Dynamic three-dimensional reconstruction of the heart by transesophageal echocardiography. Arq Brasil Cardiol 1999;72:559-68. Roelandt JR. Three-dimensional echocardiography: the future today. Acta Cardiol 1998;53:323-36. Borges AC, Witt C, Bartel T, Muller S, Konertz W, Baumann G. Preoperative two- and three-dimensional transesophageal echocardiographic assessment of heart tumors. Ann Thorac Surg 1996;61:1163-7. Handke M, Scho¨ nlein A, Scha¨fer DM, Beyersdorf F, Geibell A. Myxoma of the mitral valve: diagnosis by 2 dimensional and 3 dimensional echocardiography. J Am Soc Echocardiogr 1999;12:773-6. Fukuoka M, Hagiwara M, Shimoshige S, Hirada A, Adachi T, Komura H, et al. Primary leiomyosarcoma of the heart subsequent to double carcinomas of the thyroid and lung. Heart Vessels 2000;15:100-2.