Conway Samuel, Herrey Anna S, Rakhit Roby D
Department of Cardiology, Royal Free Hospital, Pond Street, Hampstead, London, NW3 2QG, UK.
Barts Heart Centre, St. Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.
Eur Heart J Case Rep. 2021 Jan 12;5(2):ytaa552. doi: 10.1093/ehjcr/ytaa552. eCollection 2021 Feb.
Coronary arterial fistulae are rare yet have been associated with hypertrophic cardiomyopathy (HCM). We present a patient who was found to have a left circumflex (LCx) to left ventricular (LV) fistula in combination with apical HCM.
A 72-year-old female presented with syncope after exercise. She sustained facial injuries including fracture of her nasal bones. There were no previous episodes, no cardiac history, and she denied chest pain or anginal symptoms. Electrocardiogram showed sinus rhythm with T-wave inversion throughout the chest leads. Echocardiography suggested apical HCM with hypertrophy of the LV apex but good systolic function. This was confirmed on cardiac magnetic resonance imaging with a characteristically spade-shaped LV cavity. Coronary angiography demonstrated a distal LCx to LV fistula from the apical hypertrophy but no coronary artery disease. She was started on beta-blockers and has had no further episodes, remaining well.
Coronary fistulae are present in 0.002% of the population but clinical outcomes are poorly understood. The majority are asymptomatic but anginal chest pains can occur through the 'coronary steal' phenomenon. Apical HCM is a subtype of HCM characterized by spade-shaped LV cavity obliteration. It is unclear whether the association between fistulae and HCM occur because of the increased vascularization and fibrosis associated with HCM or whether congenital malformation leads to hypertrophy. Both can produce a constellation of cardiac symptoms. Our patient has the previously unreported combination of apical HCM and an LCx fistula; two rarer subtypes of rare conditions appearing together.
冠状动脉瘘虽罕见，但与肥厚型心肌病（HCM）有关。我们报告一名患者，发现其存在左旋支（LCx）至左心室（LV）的瘘管，并伴有心尖部HCM。
一名72岁女性在运动后出现晕厥。她面部受伤，包括鼻骨骨折。既往无发作史，无心脏病史，否认胸痛或心绞痛症状。心电图显示窦性心律，胸前导联T波倒置。超声心动图提示心尖部HCM，左心室心尖肥厚，但收缩功能良好。心脏磁共振成像证实了这一点，左心室腔呈特征性的铲形。冠状动脉造影显示，由于心尖肥厚导致远端LCx至LV瘘管形成，但无冠状动脉疾病。她开始服用β受体阻滞剂，此后未再发作，情况良好。
冠状动脉瘘在人群中的发生率为0.002%，但其临床结局了解甚少。大多数患者无症状，但通过“冠状动脉窃血”现象可出现心绞痛。心尖部HCM是HCM的一种亚型，其特征为左心室腔呈铲形闭塞。目前尚不清楚瘘管与HCM之间的关联是由于HCM相关的血管化和纤维化增加所致，还是先天性畸形导致肥厚。两者均可产生一系列心脏症状。我们的患者患有此前未报道的心尖部HCM和LCx瘘管的组合；两种罕见疾病的更罕见亚型同时出现。
