Blaysat G, Villain E, Marçon F, Rey C, Lipka J, Lefèvre M, Bourlon F
Service de pédiatrie et génétique médicale, hôpital C.-Nicolie, Rouen.
Arch Mal Coeur Vaiss. 1997 May;90(5):645-8.
Idiopathic dilatation is a rare abnormality corresponding to isolated aneurysmal dilatation of the right atrium, the outcome of which is not well known. Therefore a multicentric retrospective study was set up by the paediatric working group of the French Society of Cardiology recensing 7 boys and 8 girls who were diagnosed with this condition between 1971 and 1993. Ten of the children were asymptomatic and the diagnosis was suggested by the chest X-ray: one neonate had cardiac failure secondary to atrial tachycardia. The diagnosis has been facilitated by echocardiography since 1980. In this series, since 1993, four diagnoses were made antenatally. The outcome was variable : eight children are alive and well with follow-up periods ranging from 2 to 15 years (average 6 years) : four children have had cardiac arrhythmias : benign atrial extrasystoles (1 case), junctional reentrant tachycardia (1 case). The other two had more severe arrhythmias with flutter in a 7 year-old and one neonatal atrial tachycardia. The outcome was favourable with medical treatment. Three children underwent surgical atrial resection : the outcome has been good in these 3 cases with follow-up periods of 4, 13 and 18 years. This series shows that idiopathic dilatation of the right atrium is usually a well tolerated abnormality but unexpected complications may arise which can be severe such as arrhythmias, or which may be potentially threatening such as interatrial thrombosis. Management consists of either follow-up to diagnose complications which require appropriate treatment of systematic surgical correction as some authors suggest.
特发性扩张是一种罕见的异常情况,表现为孤立的右心房动脉瘤样扩张,其预后尚不清楚。因此,法国心脏病学会儿科工作组开展了一项多中心回顾性研究,统计了1971年至1993年间被诊断为此病的7名男孩和8名女孩。其中10名儿童无症状,诊断由胸部X线提示:1名新生儿因房性心动过速继发心力衰竭。自1980年以来,超声心动图有助于诊断。在这个系列中,自1993年以来,有4例在产前确诊。预后各不相同:8名儿童存活且状况良好,随访期为2至15年(平均6年);4名儿童出现心律失常:良性房性早搏(1例)、交界性折返性心动过速(1例)。另外两名儿童有更严重的心律失常,1名7岁儿童出现心房扑动,1名新生儿出现房性心动过速。经药物治疗后预后良好。3名儿童接受了心房切除术:这3例随访期分别为4年、13年和18年,结果良好。该系列研究表明,右心房特发性扩张通常是一种耐受性良好的异常情况,但可能会出现意想不到的并发症,这些并发症可能很严重,如心律失常,或者可能具有潜在威胁,如心房内血栓形成。治疗方法包括随访以诊断并发症,并发症需要适当治疗,或者如一些作者所建议的进行系统性手术矫正。