Ibarrola Martin, Pérez-Riera Andrés Ricardo, González Mario D
Centro Cardiovascular BV, Bella Vista, Buenos Aires, Argentina.
Design of Studies and Scientific Writing Laboratory in the ABC School of Medicine, Santo André, São Paulo, Brazil.
Oxf Med Case Reports. 2019 Mar 29;2019(3):omz021. doi: 10.1093/omcr/omz021. eCollection 2019 Mar.
Isolated left ventricular noncompaction (LVNC) was described for the first time in 1984. It is a rare congenital disease, characterized by prominent trabecular meshwork pattern and deep intertrabecular recesses, communicated with the left ventricular chamber. Clinical presentation varies from asymptomatic patients, to those developing supraventricular and ventricular arrhythmias, thromboembolism, heart failure and sudden cardiac death. We present an unusual case, where the patient with Neurofibromatosis type 1 presented with a wide complex orthodromic atrioventricular reentrant tachycardia (AVRT) and a diagnosis of left posterior paraseptal accessory pathway in association with LVNC.
孤立性左心室心肌致密化不全（LVNC）于1984年首次被描述。它是一种罕见的先天性疾病，其特征是小梁网状结构突出和小梁间深陷凹，与左心室腔相通。临床表现从无症状患者到出现室上性和室性心律失常、血栓栓塞、心力衰竭和心源性猝死的患者不等。我们报告一例不寻常的病例，一名1型神经纤维瘤病患者出现宽QRS波正向房室折返性心动过速（AVRT），诊断为左后间隔旁道合并LVNC。
