16例儿童原发性心脏肿瘤的疗效分析

目的总结儿童原发性心脏肿瘤的治疗效果。方法收集2003年6月30日至2017年5月2日由本院确诊并行手术治疗的原发性心脏肿瘤患儿作为研究对象,回顾性分析患儿的年龄分布、临床表现、肿瘤部位、诊断、治疗、病理分型及预后等情况。结果 16例原发性心脏肿瘤患儿中,男童4例,女童12例;年龄范围为1.3个月至12.5岁,平均年龄为2.45岁;临床表现多以胸闷、气促为主;14例为单发肿瘤,2例为多发肿瘤;肿瘤位于左心房4例,左心室2例,右心房4例,右心室4例,室间隔2例;患儿均行心脏彩超检查,其中8例行心脏MRI检查,1例行CTA检查;12例心脏肿瘤完整切除,3例心脏肿瘤部分切除;1例行心脏肿瘤活检术;术后病理学诊断黏液瘤8例,纤维瘤3例,横纹肌瘤2例,婴儿型毛细血管瘤、脂肪瘤、脂肪母细胞瘤各1例。5例失访,11例随访3个月至6年,未出现肿瘤复发或肿瘤继续增长。结论儿童原发性心脏肿瘤症状不典型,多在婴幼儿期诊断,心脏彩超是首选检查方法,部分需行心脏MRI检查,对有明显症状者应及时采取手术治疗,手术以恢复正常血流动力学状态为目的,可根据病变位置及大小选择行完整切除或部分切除,治疗效果良好。     

婴幼儿心脏肿瘤的治疗与预后

目的:探讨婴幼儿心脏肿瘤的诊断、治疗经验以及随访情况。方法:回顾性分析大连医科大学附属大连市儿童医院心脏中心2013年8月至2019年11月收治的9例婴幼儿心脏肿瘤患儿的住院资料及随访结果。其中,男7例,女2例;年龄(6.33±8.06)个月,范围在1 d至27个月;入院体重(6.57±2.89)kg,范围在3.7～1...     

小儿原发性心脏肿瘤超声心动图诊断及临床分析

目的探讨超声心动图对小儿原发性心脏肿瘤的诊断价值.方法对14例原发性心脏肿瘤患儿的临床资料回顾性分析.结果14例中,超声心动图诊断心房肿瘤(块)6例(42.9%),其中粘液瘤3例;心室肿瘤3例(21.4%);心包肿瘤3例(21.4%);巨大肺动脉瘤2例(14.3%).14例中,恶性肿瘤2例(14.3%).超声显示部分病例伴随心脏畸形、下腔静脉扩张等异常.结论超声心动图能确定心脏肿瘤的位置,可初步区分良性与恶性肿瘤,对心房粘液瘤和肺动脉瘤有确诊价值.     

儿童及青少年原发性心脏肿瘤52例临床病理分析

目的 探讨儿童及青少年中罕见的原发性心脏肿瘤的组织学类型和病理特征,为临床诊断治疗提供形态学依据.方法 1976-2006年从中国医学科学院阜外心血管病医院近30年手术切除的562例原发性心脏肿瘤患者中,选择年龄不超过18周岁的病例.分析其临床病理所见,部分病例做了免疫组织化学和透射电镜检查.结果 52例中年龄平均12岁,最小6个月;男女各26例.良性肿瘤48例,占93%,其中黏液瘤最常见(31例),其它依次为纤维瘤(4例)、横纹肌瘤(3例)、海绵状淋巴管瘤(3例)、海绵状血管瘤(2例)、脂肪瘤(2例),还有颗粒细胞瘤、神经纤维瘤、平滑肌瘤各1例.恶性肿瘤4例,占7%,分别为血管肉瘤、纤维肉瘤、黏液纤维肉瘤及低分化肉瘤.3岁以内婴幼儿原发心脏肿瘤以横纹肌瘤(3例)和纤维瘤(3例)较多,还可见恶性的低分化肉瘤(1例).52例临床诊断与病理诊断符合或基本符合的占93%,误诊7%.结论 儿童心脏肿瘤类型多样,年龄较大者也以黏液瘤最多,而婴幼儿期以横纹肌瘤和纤维瘤多见.恶性肿瘤为分化较差的肉瘤,好发于右心,可见于婴幼儿.肿瘤临床诊断时应密切结合患儿年龄、瘤体生长部位及影像形态特点,才能减少误诊.     

原发性心脏肿瘤的外科治疗

目的探讨小儿原发性心脏肿瘤的外科治疗。方法心脏肿瘤患儿7例均在体外循环下行心脏肿瘤摘除术。结果治愈5例,死亡2例。结论小儿心脏肿瘤一旦确诊,应立即手术治疗。手术切除时应尽可能全层、广泛切除,以免术后复发。房间隔切口直接或补片修复。     

超声心动图诊断儿童心脏肿瘤24例病例系列报告

目的：总结超声心动图诊断儿童及青少年心脏肿瘤的特点及临床治疗经验。方法：纳入2013年1月至2020年1月于首都医科大学附属北京安贞医院儿童心血管病中心住院治疗的心脏肿瘤患儿,对其超声心动图表现及临床资料进行回顾性分析。结果：24例患儿进入本文分析,20例行肿瘤完整切除术,4例行肿瘤部分切除术。术后病理显示良性病变19例（79.2%）,恶性病变1例（4.2%）,生物学行为未明性肿瘤4例（16.7%）;黏液瘤7例,横纹肌瘤6例,炎性肌纤维母细胞瘤4例,纤维瘤3例,纤维肉瘤、畸胎瘤、血管瘤和血栓各1例。心脏肿瘤发生部位以右心室、左心房多见。超声诊断与手术后病理诊断符合率29.2%（7/24）。黏液瘤超声表现特异性强,超声与手术后病理诊断符合率85.7%（6/7）,黏液瘤的超声特征性表现包括：低至中等回声,分叶状,形态不规则,结构松散,活动度大,均有蒂附着;形态会随心脏的收缩和舒张改变,舒张期瘤体不同程度嵌入房室瓣口,引起房室瓣继发狭窄。结论：儿童心脏肿瘤以良性肿瘤多见,病理类型以黏液瘤、横纹肌瘤为主。黏液瘤超声诊断符合率高。     

婴幼儿和儿童心脏肿瘤的临床特点和超声诊断的应用价值

目的评价婴幼儿及儿童心脏肿瘤的临床特点、手术所见、病理学类型、以及治疗情况;评价超声心动图诊断婴幼儿及儿童心脏肿瘤的准确性。方法1983年12月至2003年12月在北京阜外心血管病医院手术和病理证实为心脏肿瘤的患者19例,男8例,女11例;年龄5个月~14岁,平均(7±5)岁。结果19例患者中,良性肿瘤17例(89.5%),其中黏液瘤10例(52.6%),横纹肌瘤4例(21.1%),纤维瘤2例(10.5%),脂肪瘤1例(5.3%);恶性肿瘤2例(10.5%),其中横纹肌肉瘤和转移性上皮肉瘤各1例。19例肿瘤中,11例位于左心(57·9%),7例位于右心(36.8%),1例位于双心室(5.3%)。术前超声10例诊断黏液瘤和1例怀疑横纹肌瘤与术后病理结果一致(11/19,57.9%),其余8例因超声缺乏经验,诊断为心脏占位病变性质待定或黏液瘤可能性大(8/19,42.1%)。结论婴幼儿和儿童心脏原发肿瘤多为黏液瘤,其次为横纹肌瘤,纤维瘤和脂肪瘤。黏液瘤多见于较大儿童,横纹肌瘤多见于婴幼儿,可呈多发性生长。当临床表现严重并难以控制时应选择手术。     

小儿结节性硬化症合并心脏横纹肌瘤临床特征研究

目的探究小儿结节性硬化症(tuberous sclerosis complex,TSC)合并心脏横纹肌瘤(cardiac rhabdomyoma,CRM)的临床特点。方法以2008年5月至2017年11月由浙江大学医学院附属儿童医院确诊合并CRM的TSC患儿。在剑突下、心尖、胸骨旁及胸骨上窝等切面多方位、多角度对心脏进行超声心电图检查,记录心脏肿瘤后重点观察肿瘤的位置、数量、形状、大小、活动及回声情况,进一步行头颅MRI、头颅CT、心电图、脑电图等检查确诊TSC。有手术指征的心脏肿瘤病例行肿瘤切除术,无手术指征的心脏肿瘤病例则通过超声随访观察。结果 34例临床确诊为TSC患儿中,首发临床症状为癫痫23例(68%)。首诊时超声心动图检查发现CRM后确诊为TSC 11例(32%),其中胎儿超声心动图检查发现CRM 1例。34例TSC患儿中心脏肿瘤多发24例(71%),单发10例(29%),以左心室最多。随访21例患儿(随访时间3个月至1年2个月)中7例经手术病理证实为CRM,8例CRM自发缩小或减少,2例CRM完全消退,4例变化不明显。结论癫痫发作为TSC患儿较为常见的首发症状,故此类患儿应常规行心脏超声检查。心脏超声首诊如发现以左心室为主的多发高回声肿瘤应警惕TSC的存在,结合心脏超声检查有助于该病的早期诊断和治疗。     

风湿性心脏炎心脏超声改变的研究

目的　了解风湿性心脏炎患儿心脏受累情况 ,探讨超声心动图对本病的价值。方法　总结分析2 2 8例风湿性心脏炎患儿的心电图及超声心动图特点 ,比较两者诊断房室肥大的敏感性 ,观察治疗前后心脏超声改变。结果　心电图异常者 1 66例 (73 .1 % ) ,以房室传导阻滞最多见 (67例 ,40 .4% ) ;治疗前 1 66例行心脏超声检查 ,1 62例 (97.6 % )显示不同程度的房室扩大 ,其中以左房大最多见 (1 0 4例 ,62 .7% ) ,1 66例患儿心脏瓣膜均有改变 ,主要表现为二尖瓣返流、增厚及狭窄和主动脉瓣返流 ,其中以二尖瓣返流最多见 (1 0 8例 ,65 .1 % ) ;超声诊断左房大、左室大的敏感性明显优于心电图 ;部分患儿左室收缩功能左室射血分数、心脏指数降低 ,舒张功能E/A <1 ;治疗后复查心脏超声二尖瓣返流、增厚可消失。结论　超声心动图对儿童风湿性心脏炎的诊断及治疗随访具有重要价值     

小儿原发性心脏肿瘤超声心动图表现及诊断价值

小儿原发性心脏肿瘤较为少见,发病率约0．05％。由于肿瘤位置特殊,可导致血流动力学紊乱、心力衰竭、心律失常等症状,在新生儿期就可引起心功能不全而死亡。1998年5月至2004年2月我院共诊断小儿原发性心脏肿瘤21例,现将其多普勒超声心动图表现报道如下。     

Cardiac Rhabdomyomas Associated with Tuberous Sclerosis Complex in 11 Children: Presentation to Outcome

Cardiac rhabdomyomas (CRs) are the most common heart tumors in children and closely associated with tuberous sclerosis complex (TSC). This study was performed to assess the presentation type, clinical course, treatment modalities, and outcome of the patients with rhabdomyoma, associated with TSC. We reviewed our patients with cardiac rhabdomyomas (CRs), who had received a diagnosis of TSC previously or during the follow-up period between June 1996 and January 2012, retrospectively. Thirty-two patients with TSC were evaluated and among them 11 patients (34%) were associated with CRs. Five patients (45%) had multiple tumors and consequently a total of 29 CRs were analyzed in our study. The median follow-up period was 2 years (range: 1 week–15 years). Clinical presentation was cardiac murmur in three patients, cyanosis in two patients and arrhythmia in one patient. Five patients were asymptomatic at the diagnosis and CRs were detected during routine cardiac evaluation for TSC. Cardiac tumors were diagnosed prenatally in two patients. Spontaneous regression rate was 31% and we experienced a complete regression of a tumor with an echogenic bordered tissue defect and septal thinning in a patient. Three patients had hemodynamically significant tumor obstruction; two of them underwent surgery. The other patient, who had multiple CRs, was treated medically with everolimus because of high-risk potential of surgery. Although surgical resection is the preferred treatment in most of the patients with hemodynamic instability, we need novel alternative medical therapies in some critically ill patients who cannot be operated due to various reasons.     

Diagnosis and treatment of cardiac rhabdomyomas

A diagnosis of primary cardiac tumors was achieved by two-dimensional echocardiography in 3 patients. Two of them were female neonates, born of the 29th and 40th gestational week who had shown intrauterine arrhythmias. Subsequently, we were able to diagnose tuberous sclerosis in both. The third child, a 121/2 year old boy, with already diagnosed tuberous sclerosis, presented an electrocardiographical preexcitation syndrome. Because of the multiplicity of the tumors and the association with tuberous sclerosis we presumed that the tumors were rhabdomyomas. As it is known that rhabdomyomas show no, or only a minimal postnatal growth, we decided neither to perform a heart catheterization nor to treat them surgically unless the children show hemodynamical deterioration, although the tumors were huge in both neonates. The subsequent 14 months showed a clear regression in the size of the tumors. One of the neonates was successfully treated with amiodarone because of ventricular tachycardia. The second neonate didn't require any treatment. We conclude that: Surgical management is only necessary in patients presenting with cardiac insufficiency and/or arrhythmias that are resistant to conventional medical treatment. Primary cardiac tumors should be looked for in patients presenting with arrhythmias, even when occurring already during intrauterine life. Diagnostic measures in order to diagnose tuberous sclerosis in patients with cardiac tumors are necessary and vice versa.     

Primary Heart Tumors in the Pediatric Age Group: A Review of Salient Pathologic Features Relevant for Clinicians

Because primary tumors of the heart in infants and children are extremely rare, most knowledge is based on collections of case reports rather than large cohort studies. The types of heart tumors encountered in the pediatric age group differ from those seen in adults. In the latter, cardiac myxomas are by far the most common tumor; in infants and children the most common primary tumor of the heart is the rhabdomyoma. Spontaneous regression of these tumors has been well established so that surgical intervention is no longer indicated unless there are clinical manifestations from the heart. There is a high incidence of associated tuberous sclerosis. Any intracavitary mass in infants is suggestive for a cardiac rhabdomyoma unless otherwise proven. The second most common tumor in this age group is cardiac fibroma. These tumors probably represent hamartomatous lesions and this has led to a strategy in which radical surgical excision is not indicated if the procedure endangers postoperative heart function. Cardiac myxomas are of interest in this age group because of their familial occurrence as part of the myxoma syndrome. Other types of primary heart tumors occur, including malignant variants, but all are exceedingly rare. It is because of the sporadic nature of these tumors that treatment and insights into prognosis are based largely on case documentations and analogies from similar tumors originating elsewhere.     

Cardiac rhabdomyoma as an unusual mediastinal mass in a newborn

A newborn case with cardiac rhabdomyoma is presented to discuss the differential diagnosis of mediastinal tumors around the heart in children. The diagnosis of rhabdomyoma can be suggested by modern visualization methods such as computerized tomography, magnetic resonance imaging and echocardiography. However, surgery is clearly indicated in cases with obstruction of flow tracts, valvular dysfunction and dysrhythmia, and in cases with indefinite diagnosis, or in the absence of association with tuberous sclerosis. Cardiac rhabdomyoma should be included in the differential diagnosis of masses located adjacent to the heart in infants.     

Cardiac rhabdomyoma and tuberous sclerosis: Prenatal diagnosis and follow-up

This article reports 4 cases with cardiac rhabdomyomas diagnosed during intrauterine life. Echocardiographic follow-up at 9–30 months showed regression of the tumors in 3 cases (75%). Two cases (50%) developed tuberous sclerosis during that period. Fetal echocardiography promotes early diagnosis of tuberous sclerosis through prenatal detection of cardiac rhabdomyoma, and facilitates genetic counselling of families at risk     

Effective everolimus treatment of inoperable, life-threatening subependymal giant cell astrocytoma and intractable epilepsy in a patient with tuberous sclerosis complex

We present successful everolimus treatment of a huge subependymal giant cell astrocytoma in a 10-year old boy with tuberous sclerosis complex. The patient underwent several partial tumor resections complicated by intraoperative cardiac arrest. The tumor has been regrowing and produced severe clinical symptoms. Everolimus treatment resulted in marked tumor regression, significant improvement in patient’s ambulation and cessation of seizures. Moreover, the therapy was well tolerated. These findings indicate that everolimus treatment should be considered as a therapeutic option alternative to surgery in patients with tuberous sclerosis complex.     

Tumors of the heart in newborn infants and infants. Apropos of 5 cases

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was a hemangioma, originating from outside the left ventricular wall, and was resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively in to the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.     

Heart transplantation in an infant with rhabdomyoma

Rhabdomyoma is the most common primary cardiac tumor in infants and children and is often associated with tuberous sclerosis. Surgical resection may be indicated and, if so, is usually curative. We describe a rhabdomyoma in an infant who presented with severe myocardial ischemia necessitating orthotopic heart transplantation.     

Cardiac rhabdomyoma presenting as fetal hydrops

Summary Fetal hydrops and possible congenital heart disease with atrioventricular block was diagnosed one day before birth in a male infant of 35 weeks' gestation. Echocardiography and angiocardiography soon after birth revealed a cardiac tumor. The child died three days after birth. Necropsy showed tuberous sclerosis involving the heart (type-I rhabdomyoma), kidneys, retina, and central nervous system.     

Primary Mitral Valve Sarcoma in Infancy

Primary cardiac sarcoma is a rare tumor, found especially in children, particularly in the left side of the heart. This report describes a rare case of primary mitral valve sarcoma in a 7-month-old male infant with hemiparesis and heart murmur who underwent mitral valve replacement because of excessive invasion of the mitral valve by the tumor. The patient remains well, free of recurrence and cerebral metastasis, and without adjuvant therapy 18 months after the operation.