Lahmiti S, Aboussad A
Équipe de recherche pour l'enfance, la santé et le développement, faculté de médecine et de pharmacie de Marrakech, CHU Mohamed VI, Marrakech, Maroc.
Ann Cardiol Angeiol (Paris). 2012 Aug;61(4):299-302. doi: 10.1016/j.ancard.2010.12.001. Epub 2011 Jan 8.
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.
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.
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。
