fait clinique

1 65 2 66 3 67 4 Epicardial cavernous : a two-case report. 68 5 69 6 70 7 Atef Ben Youssef, Sarra Zairi, Sonia Ouerghi, Aida Ayadi*, Mouna Bousnina, Ferdaous Berraies, Tarak Kilani. 71 8 72 9 Department of Thoracic and Cardio-Vascular Surgery. A. Mami Hospital.Ariana. Medical School of Tunis. Tunisia. 73 10 * Department of Pathology. A. Mami Hospital. Ariana. Medical School of Tunis. Tunisia. 74 11 75 12 76 13 A. Ben Youssef, S. Zairi, S. Ouerghi, A. Ayadi, M. Bousnina, 77 14 F. Berraies, T. Kilani. A. Ben Youssef, S. Zairi, S. Ouerghi, A. Ayadi, M. Bousnina, 78 15 F. Berraies, T. Kilani. 79 16 L’hémangiome caverneux épicardique: à propos de deux cas. 80 17 Epicardial cavernous hemangioma: a two-case report. 81 18 82 19 LA TUNISIE MEDICALE - 2014 ; Vol 92 (n°04) : 268-271 83 LA TUNISIE MEDICALE - 2014 ; Vol 92 (n°04) : 268-271 20 résumé 84 21 But : Nous rapportons deux cas d'hémangiomes caverneux prenant summary 85 22 naissance au niveau de l'épicarde chez deux femmes âgées aims: We report two-cases of cavernous arising from 86 23 respectivement de 24 et 79 ans. the epicardium in two women aged respectively 24 and 79 years old. 87 24 Observations : La première patiente était symptomatique et avait The first patient was symptomatic and presented with palpitations. 88 25 consulté pour des palpitations. La deuxième patiente nous a été The second patient was referred after a random discovery at 89 26 adressée après une découverte fortuite à l'échographie cardiaque. echocardiography. Chest CT and MRI were performed in the two 90 27 Une exploration par TDM thoracique complétée par une IRM a été cases and showed a mass located in the pericardial cavity. Coronary 91 28 effectuée dans les deux cas, et avait montré une masse située dans la CT was necessary in the first case to ascertain the degree of coronary 92 29 cavité péricardique. Un complément d’exploration par un coro- artery involvement. Both of our patients underwent surgical resection 93 30 scanner était nécessaire dans le premier cas, pour déterminer les under cardiopulmonary bypass with an uneventful postoperative 94 31 rapports avec l'artère coronaire en regard. Les deux patientes ont eu course for the first case. The second one, died postoperatively from 95 32 une résection chirurgicale sous circulation extracorporelle, avec des pneumonia. 96 33 suites simples pour la première. La deuxième est décédée en post- Conclusion: Cardiac cavernous hemangiomas, although rare and 97 34 opératoire suite à une pneumopathie. well tolerated require prompt management and surgery at discovery 98 35 Conclusion : Les hémangiomes caverneux de localisation cardiaque, to avoid further complications which may put at risk the patient’s life 99 36 bien que rares et bien tolérés nécessitent une prise en charge rapide prognosis. 100 37 et une exérèse chirurgicale dès leur découverte. 101 38 102 39 103 40 mots-clés Key-words 104 41 Caverneux, hémangiome, épicarde, chirurgie. Cavernous, hemangioma, epicardium, surgery. 105 42 106 43 107 44 108 45 109 46 110 47 111 48 112 49 113 50 114 51 115 52 116 53 117 54 118 55 119 56 120 57 121 58 122 59 123 60 124 61 125 62 126 63 127 64 128

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129 Cardiac cavernous hemangioma is an uncommon benign close to the left anterior descending coronary artery, without 193 130 194 primary tumor of the heart. It may originate in any part but less opening of heart cavities. One diagonal branch running into the 131 195 132 commonly from the pericardium or epicardium. We report tumor has been sacrificed. The cardiac defect was closed in two 196 133 herein two cases of epicardial cavernous hemangioma managed layers with Teflon felt pledgets. Histological examination 197 134 during the past 5 years in the cardio-vascular surgery showed large vascular channels within the tumor, lined by 198 135 department of A. Mami Hospital. flattened endothelial cells lining a fibrous stroma suggestive of 199 136 cavernous hemangioma. The lining cells expressed endothelial 200 137 Case n°1 markers CD34 and CD31 at immunohistochemistry studies. 201 138 A 24-year-old woman, presented with a two-month history of The postoperative clinical course was uneventful and the 202 139 203 chest pain and palpitations. Physical examination was patient was discharged 10 days after surgery. Transthoracic 140 204 141 unremarkable. Electrocardiogram showed a normal sinus echocardiography eight months later showed a remnant of 205 142 rhythm with normal axis and T-wave inversion in leads V4 hemangioma with a-69% left ventricular ejection fraction. 206 143 through V6. Chest X-ray showed a bulging of the left middle Coronary CT 10 months later showed the small remnant of the 207 144 heart segment without pulmonary vascular redistribution or tumor with normal cardiac cavities. The patient is currently 208 145 cardiomegaly. Tumor markers (CEA, AFP, HCG) were within asymptomatic 4 years after surgery. 209 146 normal limit. Transthoracic echocardiography revealed an 210 147 anterior intrapericardial polycystic mass of 50cm2. It was in Figure 2 : The tumor surrounding completely the segments 2 and 3 of 211 148 212 front of the pulmonary infundibulum and extended from the the left anterior descending coronary artery. 149 213 150 apex to the base of the heart, without compression of the cardiac 214 151 cavities. Magnetic resonance imaging (MRI) revealed a 9×7cm 215 152 pericardial tissue mass which was isointense on T1-weighted 216 153 imaging and hyperintense on T2-weighted imaging. The mass 217 154 showed a delayed, inhomogeneous enhancement after 218 155 gadolinium administration because of interspersed septa within 219 156 the mass. Coronary CT showed close contact with the 220 157 221 myocardium and heterogeneous density with a tissue 158 222 159 component. There was delayed enhancement in the post 223 160 contrast imaging, with hypo-intense, unenhanced areas within 224 161 the mass (Fig.1). 225 162 Figure1 : The mass showing delayed enhancement areas in the post 226 163 contrast imaging. 227 164 228 165 229 166 230 Case n°2 167 231 168 A 79-year-old woman with hypertension, a history of external 232 169 electrical cardio-version for atrial fibrillation 4 years ago as 233 170 well as a prior hysterectomy for a uterine cancer 12 years ago, 234 171 was referred to our department for a random discovery of an 235 172 intra-pericardial tumor with a large contact with the right 236 173 atrium. The patient had a good general condition. Physical 237 174 examination revealed an 80 beats/min cardiac arrhythmia with 238 175 239 normal heart sounds. Chest X-ray showed a cardiomegaly with 176 240 177 a bulging left middle arc. Electrocardiogram showed an atrial 241 178 arrhythmia. Trans-thoracic echocardiography showed a 3x4cm 242 179 round sessile fixed mass developing in the right atrium next to 243 180 the superior vena cava contracting a large contact to the inter- 244 181 atrial septum with a bi-atrial dilatation. Trans-esophageal 245 182 The tumor surrounded completely the segments 2 and 3 of the echography showed a soft tissue mass of 65x45mm, with 246 183 left anterior descending coronary artery (LAD) and was compression on the right atrium developing in the space 247 184 248 associated to pericardial effusion (Fig.2). Patient was explored bounded in front by the aorta and in below by the right atrium 185 249 186 via sternotomy and after pericardotomy, the tumor was found and the superior vena cava. A recent clot was visualized in the 250 187 on the left face of the heart, extended to the left and behind the left atrium of 11x7mm. CT scan showed that the mass was 251 188 pulmonary artery. Tumor infiltrated into the myocardium at the adherent to the cardiac cavity and to the pericardium, presenting 252 189 anterior wall of the right ventricle and lateral wall of the left a homogeneous density without extension to the cardiac 253 190 ventricle. After cardiopulmonary bypass and under cardioplegic cavities or vascular compression (Fig.3). MRI showed a well- 254 191 arrest, the tumor was incompletely resected, leaving a remnant defined mass which was infiltrating the right atrial wall without 255 192 256

269 Ben Youssef A. - Epicardial cavernous hemangioma

257 intraluminal extension. It was adherent to the right atrial heart cells (Fig.5). The diagnosis of intra-pericardial cavernous 321 258 322 walls and infiltrating the base of the aorta and both sides of the hemangioma was confirmed. The post-operative course was 259 323 260 upper and lower cave system. An invasive cardiac tumor was uneventful at the beginning. At the 7th day, the patient 324 261 highly suspected and a surgical approach was decided. A right presented an ischemic with peripheral signs of clotting. 325 262 thoracoscopy with pericardotomy was performed. The laboratory tests revealed an immuno-induced 326 263 thrombopenia. A respiratory distress secondary to nosocomial 327 264 Figure 3 : The mass adherent to the cardiac cavity and to the pneumonia was also diagnosed and the patient required 328 265 pericardium, presenting a homogeneous density. mechanical ventilation and intra-venous antibiotics. The patient 329 266 developed a septic shock two weeks later and died. 330 267 331 268 332 Figure 5 : TA vascular proliferation vessels with vessels of various 269 333 widths, lined by regular endothelial cells with enlarged lumen. 270 334 271 335 272 336 273 337 274 338 275 339 276 340 277 341 278 342 279 343 280 344 281 345 282 346 283 The tumor was sessile and tightly adherent to the right atrium. 347 284 A conversion to sternotomy was decided. The tumor was 348 285 349 developing at the postero-lateral face of the right atrium, behind 286 350 287 the superior vena cava, extending to the aortico-caval space in 351 288 contact with the roof of the left atrium and the pulmonary artery 352 Discussion 289 from which it was easily dissected (Fig.4). 353 290 Primary cardiac tumors are very rare and accounted for 0.056% 354 291 Figure 4 : The tumor developing at the postero-lateral face of the right 355 atrium. among 12 485 consecutive autopsies [1]. Up to 75% of primary 292 cardiac tumors are benign and cardiac hemangioma accounts 356 293 357 294 for 1 to 2% of all benign tumors. We report two rare cases of 358 295 cardiac hemangioma, which is a non-malignant 359 296 that consists of closely packed capillary structures or widely 360 297 dilated vascular channels lined by flattened endothelial cells 361 298 and with focal connective tissue in the walls. It can be classified 362 299 as capillary, arteriovenous or cavernous like in both of our cases 363 300 [2]. It may originate from any part of the heart [3], but a 364 301 previous review of 56 cases of cardiac hemangiomas, revealed 365 302 366 303 a predilection for ventricles at 70% (36% right, 34% left 367 304 ventricle) and 23% in the right atrium [4]. The pericardium is a 368 305 rarely reported location [5] with hemangiomas arising from 369 306 visceral pericardium, and most of the cavernous type [6]. Both 370 307 of our patients illustrate this fact. 371 308 Hemangiomas may be encountered at any age and a slightly 372 309 Given the tight adherence to the right atrium, a female predominance has been reported [7]. Cardiac 373 310 cardiopulmonary bypass was decided. Complete dissection of hemangiomas are frequently well tolerated and asymptomatic 374 311 the tumor from the right atrium was achieved without atriotomy 375 312 in most of the patients with incidental discovery at imaging. For 376 and a total tumorectomy was possible. Frozen section was in 313 our cases, the presenting symptoms were chest pain and 377 314 favor of an hemangioma. Histological examination showed a palpitations in the first case and an imaging finding in the 378 315 6x5x1.5 cm tender encapsulated mass with micro-cystic areas second case. 379 316 and important hemorrhagic reshuffle. There was a benign well Echocardiography is an efficient tool in determining the 380 317 limited vascular proliferation with vessels of various widths. characteristics of any cardiac tumor with an accuracy rate of 381 318 These were lined by regular endothelial cells with enlarged 81% [9]. Hemangiomas appear at echocardiography as 382 319 lumen and areas of intercommunications filled with red blood hyperechoic . Further examinations are recommended in 383 320 384

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case of symptoms of coronary artery disease or a doubtful natural course of a hemangioma is not well known: spontaneous echography. CT and MRI are highly contributive in determining regression has been reported as well as various complications the extension of the tumor to the neighboring vessels and and even sudden death [13, 14]. For these reasons, hemangioma myocardium [9]. (such as any cardiac tumor) when resectable, has to be removed. Chest CT shows a heterogeneous mass at unenhanced Complete excision is the mainstay for treatment: it eases the sequences and intensely enhanced after contrast material symptoms and prevents complications. The long-term administration [10]. On MRI, hemangioma appears prognosis for operated patients is favorable, without relapses heterogeneous isotense or hypointense on T1-weighted, and for completely removed hemangiomas. However, a regular usually hyperintense on T2-weighted images [11]. Cardiac screening with different imaging modalities is highly catheterization studies reveal an intra-cavitary filling defect recommended in all cases, especially for incompletely resected which may help the diagnosis of a cardiac tumor in 40% of tumors. cases. Coronary arteriography helps to establish the diagnosis with the classic finding of a vascular blush, which reflects the vascular nature of the tumor [10, 12]. Coronary CT is indicated conclusion in some patients, such our first case, in which an accurate Cavernous epicardial hemangiomas are benign tumors but determining of the tumor extent to the coronary vessels was require prompt surgery when discovered, even for mandatory. The different images modalities, even if accurate, asymptomatic patients, to confirm the diagnosis and prevent can’t certify the diagnosis which remains histological. The farther complications.

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