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[孤立性右心室心肌致密化不全:两例报告]

[Isolated non-compaction of the right ventricular myocardium: two cases report].

作者信息

Lahmiti S, Aboussad A

机构信息

Équipe de recherche pour l'enfance, la santé et le développement, faculté de médecine et de pharmacie de Marrakech, CHU Mohamed VI, Marrakech, Maroc.

出版信息

Ann Cardiol Angeiol (Paris). 2012 Aug;61(4):299-302. doi: 10.1016/j.ancard.2010.12.001. Epub 2011 Jan 8.

Abstract

BACKGROUND

The noncompaction of myocardium is a rare myocardiopathy. The isolated right ventricular involvement is exceptional. We report two cases of isolated noncompaction of the right ventricular myocardium. CASE 1: I., 2 years old, investigated for a congenital cyanosis with severe functional repercussion. Her echocardiography revealed a complex congenital heart disease associated with isolate noncompaction of the right ventricle. CASE 2: A., 5 years old, was referred for history of syncope for the 2 last years. The echocardiography showed a severe pulmonary hypertension and an isolate noncompaction of the right ventricle.

CONCLUSION

Due to the rarity of the isolate non-compaction of the right ventricle, it diagnosis remains difficult. The treatment and the outcomes of this disease are still unclear. An early diagnosis may help to reduce its complication.

摘要

背景

心肌致密化不全是一种罕见的心肌病。孤立的右心室受累情况极为罕见。我们报告两例孤立的右心室心肌致密化不全病例。病例1:I.,2岁,因先天性青紫伴严重功能影响接受检查。她的超声心动图显示患有复杂先天性心脏病,伴有孤立的右心室致密化不全。病例2:A.,5岁,因过去两年的晕厥病史前来就诊。超声心动图显示严重肺动脉高压和孤立的右心室致密化不全。结论:由于孤立的右心室致密化不全病例罕见,其诊断仍然困难。这种疾病的治疗方法和预后仍不明确。早期诊断可能有助于减少其并发症。

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