Stiller B, Hetzer R, Meyer R, Dittrich S, Pees C, Alexi-Meskishvili V, Lange P E
Department of Pediatric Cardiology, Deutsches Herzzentrum, Augustenburger Platz 1D-13353, Berlin, Germany.
Eur J Cardiothorac Surg. 2001 Nov;20(5):1002-6. doi: 10.1016/s1010-7940(01)00951-4.
Primary cardiac tumours are rare. The literature predominantly contains series on myxomas in adults and only a few long-term series that involve the very different primary cardiac tumours in early childhood. As foetal ultrasonography has continued to improve, cardiac tumours are increasingly detected early before significant symptoms develop. It is a challenge for paediatric cardiologists and surgeons to ascertain which patients need surgery and which will benefit from conservative follow-up.
A retrospective review of a 10-year period revealed 51 tumours in 26 children (median age: 1 month). Analysis was by presentation, location, associated findings, interventions, histological findings, and clinical course.
The most common tumours were rhabdomyomas (29), fibromas (nine), teratomas (two), and haemangiomas (two). The tumour location was the right ventricle in 24 and the left ventricle in 22 patients. The symptoms varied between abnormal heart murmur (20), arrhythmia and conduction abnormalities (ten), obstruction of the outflow tract >30 mmHg (nine), severe cyanosis (three) and congestive heart failure (two). Fourteen children with haemodynamic compromises underwent surgery. There was one post-operative death and one heart transplantation after bridging with an assist device. There was no tumour recurrence even when resection was incomplete. Nine of 13 children with rhabdomyomas had spontaneous tumour regression without intervention.
Most of the cardiac tumours in children are benign. Spontaneous regression is possible not only in rhabdomyoma. Surgical intervention is only required for children who develop relevant clinical symptoms. Total resection of the tumour is not the only therapeutic aim; more important is the restoration of the best possible heart function.
原发性心脏肿瘤较为罕见。文献中主要是关于成人黏液瘤的系列报道，仅有少数长期系列报道涉及幼儿期截然不同的原发性心脏肿瘤。随着胎儿超声检查不断改进，心脏肿瘤在出现明显症状之前越来越多地被早期检测出来。确定哪些患者需要手术以及哪些患者将从保守随访中获益，对儿科心脏病学家和外科医生来说是一项挑战。
对10年期间的病例进行回顾性分析，发现26名儿童（中位年龄：1个月）患有51个肿瘤。分析内容包括临床表现、肿瘤位置、相关发现、干预措施、组织学结果和临床病程。
最常见的肿瘤是横纹肌瘤（29个）、纤维瘤（9个）、畸胎瘤（2个）和血管瘤（2个）。肿瘤位于右心室的有24例，位于左心室的有22例。症状包括心脏杂音异常（20例）、心律失常和传导异常（10例）、流出道梗阻>30 mmHg（9例）、严重发绀（3例）和充血性心力衰竭（2例）。14名有血流动力学障碍的儿童接受了手术。术后有1例死亡，1例在使用辅助装置过渡后进行了心脏移植。即使切除不完全，也没有肿瘤复发。13例横纹肌瘤患儿中有9例未经干预肿瘤自发消退。
儿童心脏肿瘤大多为良性。不仅横纹肌瘤可能自发消退。只有出现相关临床症状的儿童才需要手术干预。肿瘤的完全切除并非唯一的治疗目标；更重要的是尽可能恢复最佳心脏功能。
