Wong A R, Amelia A, Mohd Zain M R, Sayuti K A
Universiti Sultan Zainal Abidin, Faculty of Medicine, Dept of Pediatrics, Malaysia.
Hospital Raja Perempuan Zainab II, Dept of Pediatrics, Malaysia.
Med J Malaysia. 2022 Jan;77(1):101-103.
Left aorto-ventricular tunnel is a rare congenital heart disorder. A then 19-year-old young lady was referred to our clinic for chronic rheumatic heart disease with aortic regurgitation. However, because of an almost continuous murmur, and an unusual transthoracic echo, she underwent various investigations, finally revealing a rare type 2 left aorto-ventricular tunnel. She had been variously diagnosed as chronic rheumatic heart disease with aortic regurgitation, bicuspid aortic valve with aortic regurgitation, and then possibly coronary arterial (cameral) fistula (based on 2D transthoracic echo) and finally left aorto-ventricular tunnel. This is the first case described in South East Asia who is asymptomatic, apart from a murmur, and her long journey to this final diagnosis illustrates the need to "chase shadows" on echoes, to come to a proper conclusion for the patient.
左主动脉-心室通道是一种罕见的先天性心脏病。一名当时19岁的年轻女性因慢性风湿性心脏病伴主动脉瓣反流被转诊至我们诊所。然而，由于几乎持续存在的杂音以及经胸超声心动图表现异常，她接受了各种检查，最终发现是一种罕见的2型左主动脉-心室通道。她曾被诊断为慢性风湿性心脏病伴主动脉瓣反流、二叶式主动脉瓣伴主动脉瓣反流，之后又可能被诊断为冠状动脉（心腔）瘘（基于二维经胸超声心动图），最终确诊为左主动脉-心室通道。这是东南亚地区报道的首例除杂音外无症状的病例，她漫长的确诊过程表明，在超声心动图检查中需要“追踪疑点”，以便为患者得出正确结论。
