CAJ | 학술논문

目的探讨原发性心脏肿瘤的治疗经验.方法回顾性分析1999年5月至2009年5月27例经心脏超声检查确诊为心脏肿瘤患者的临床资料.男性20例,女性7例;年龄24 d～12.6岁,＜1岁者16例,占59.2%.22例为单发心脏肿瘤,5例为多发.其中22例因心律失常、心包积液、呼吸困难、晕厥等症状进行手术切除,5例未施行手术.根据肿瘤的具体位置选择不同的切口径路,14例完整切除肿瘤,8例部分切除;5例行二尖瓣整形,2例行三尖瓣整形.结果术后肿瘤组织学结果:纤维瘤5例,横纹肌瘤8例,黏液瘤4例,毛细血管瘤3例,纤维肉瘤和卵黄囊瘤各1例.16例术后循环稳定;2例出现低心排血量,经治疗后心功能恢复;4例因严重低心排血量并发多器官功能衰竭死亡.18例术后随访1～10年,2例横纹肌瘤残余肿块缩小,1例消失;1例纤维肉瘤、1例血管瘤残余肿块未增大;黏液瘤术后无复发或远处种植转移.5例未手术者随访1～3年,其中2例无明显症状;1例左心室游离壁肿瘤因心律失常死亡,另1例仍存活;1例多发性心脏肿瘤伴低心排血量患者因心力衰竭死亡.结论小儿原发性心脏肿瘤应采用个体化治疗原则,对有明显症状者应行手术治疗,对无明显症状者要密切随访.手术的目的不是完整切除肿瘤,而是要恢复正常的血流动力学状态.
목적 탐토원발성심장종류적치료경험.방법 회고성분석1999년5월지2009년5월27례경심장초성검사학진위심장종류환자적림상자료.남성20례,녀성7례;년령24 d～12.6세,＜1세자16례,점59.2%.22례위단발심장종류,5례위다발.기중22례인심률실상、심포적액、호흡곤난、훈궐등증상진행수술절제,5례미시행수술.근거종류적구체위치선택불동적절구경로,14례완정절제종류,8례부분절제;5례행이첨판정형,2례행삼첨판정형.결과 술후종류조직학결과:섬유류5례,횡문기류8례,점액류4례,모세혈관류3례,섬유육류화란황낭류각1례.16례술후순배은정;2례출현저심배혈량,경치료후심공능회복;4례인엄중저심배혈량병발다기관공능쇠갈사망.18례술후수방1～10년,2례횡문기류잔여종괴축소,1례소실;1례섬유육류、1례혈관류잔여종괴미증대;점액류술후무복발혹원처충식전이.5례미수술자수방1～3년,기중2례무명현증상;1례좌심실유리벽종류인심률실상사망,령1례잉존활;1례다발성심장종류반저심배혈량환자인심력쇠갈사망.결론 소인원발성심장종류응채용개체화치료원칙,대유명현증상자응행수술치료,대무명현증상자요밀절수방.수술적목적 불시완정절제종류,이시요회복정상적혈류동역학상태.
Objective To analyze the experience of treatment strategies for pediatric patients with primary cardiac tumors. Methods The clinical data of 27 patients with primary cardiac tumors which detected by echocardiography from May 1999 to May 2009 was analyzed retrospectively. There were 20 male and 7 female patients, aged from 24 d to 12. 6 years. There were 59. 2% less than 1 year old at the time of diagnosis. A single tumor were present in 22 cases and multiple in 5 cases. Surgery was performed for 22 patients due to the varied significant symptoms such as arrhythmia, pericardial effusion, swoon and congestive heart failure with dyspnoea. Five patients were discharged hospital without surgical treatment. The surgical approachs were adopted according to tumor location. Complete surgical resection was performed in 14 patients and partial resection in 8 patients. Seven patients were underwent valve reconstruction,5 involving the mitral valve and 2 involving the tricuspid valve. Results Histologic examination of the surgically resected tumors showed rhabdomyomas in 8 cases, fibromas in 5 cases, hemangiomas 3 cases,myxomas in 4 cases, fibrosarcoma in 1 case and yolk sac sarcoma in 1 case. Sixteen cases revealed stable haemodynamic status postoperative. Two cases occurred apparent symptoms of low cardiac output and significant arrhythmias, finally recovery after comprehensive treatment of restoration the heart function. There was a total of 4 patients in-hospital death following surgery due to multiorgan system failure. Of the 18 patients who survived after the surgery were followed up from 1 to 10 years, echocardiography showed the residual mass of the tumor with partial resection, rhabdomyoma diminishing in 2 patients and almost vanishing in 1 patient. The residual mass of one fibrosarcoma patient and one hemangioma patient were not increased. Patients with myxomas had no recur or systemic embolisation after the initial surgery. Five nonsurgical patients were followed up from 1 to 3 years, 2 patients without haemodynamic alterations,1 patients with giant tumor of left ventricular free wall was died of arrhythmia, the other one was alive; the patient of multiple cardiac tumor with low cardiac output was died of heart failure. Conclusions Despite the benign histology of most paediatric primary cardiac tumours, there may be significant associated with morbidity and occasional mortality. Therapy strategies should be individualised: surgery is indicated in cases with significant clinical symptoms and close follow-up is necessary for asymptomatic patients. Total resection is not the only therapeutic aim. Most important is the restoration of the normal haemodynamic heart function.