Bhutia Euden, Kumar Dinesh, Shankar Binoy, Das Shakti Pad, Kishore Sunil
Department of Neonatology and Pediatric Medicine, PGIMER, Dr. RML Hospital, New Delhi, India.
Iran J Med Sci. 2017 May;42(3):310-313.
Bicuspid aortic valve is traditionally considered an innocuous congenital anomaly. Due to a better and widespread availability of non-invasive imaging techniques, it has come to the fore that 30% of these cases develop complications, viz., valve abnormality (aortic regurgitation and stenosis), and aneurysm of aortic root and ascending aorta. Sinus of Valsalva aneurysm is an uncommon complication of bicuspid aortic valve and more so those arising from the left coronary sinus are the rarest. These complications generally occur in the third or fourth decade of life. We present a case of the left sinus of Valsalva aneurysm in conjunction with bicuspid aortic valve and ascending aorta aneurysm at a very young age in a girl in her early adolescence. This case is to remind the paediatricians about the not so "innocuous image", but the serious implications of the bicuspid aortic valve and to regularly follow these cases for early diagnosis of potential complications so as to prevent catastrophic outcomes.
传统上，二叶式主动脉瓣被认为是一种无害的先天性异常。由于非侵入性成像技术的更好普及和广泛应用，人们逐渐认识到这些病例中有30%会出现并发症，即瓣膜异常（主动脉瓣反流和狭窄）以及主动脉根部和升主动脉瘤。主动脉窦瘤是二叶式主动脉瓣的一种罕见并发症，而起源于左冠状动脉窦的主动脉窦瘤则最为罕见。这些并发症通常发生在生命的第三个或第四个十年。我们报告了一例在青春期早期的年轻女孩中，非常罕见地出现了左冠状动脉窦瘤合并二叶式主动脉瓣和升主动脉瘤的病例。该病例旨在提醒儿科医生，二叶式主动脉瓣并非“无害表象”，而是具有严重的影响，需要定期对这些病例进行随访，以便早期诊断潜在并发症，从而预防灾难性后果。
