Tsui Irena, Shamsa Kamran, Perloff Joseph K, Lee Eric, Wirthlin Robert S, Schwartz Steven D
Retina Division, Department of Ophthalmology, Jules Stein Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.
Semin Ophthalmol. 2009 Nov-Dec;24(6):262-5. doi: 10.3109/08820530903400739.
The purpose of this report is to describe retinal findings in adults with cyanotic congenital heart disease.
Four patients, 27 to 47 years of age, with cyanotic congenital heart disease were enrolled from the Outpatient Clinic of the Ahmanson/UCLA Adult Congenital Heart Disease Center. Each patient had a medical history and physical examination by a cardiologist, chest X-ray, electrocardiogram, echocardiogram, complete blood count, and arterial oxygen saturation. In addition, each patient had an ocular examination by a retina specialist, color fundus photography, and fluorescein angiography.
The chest X-ray, electrocardiogram, and echocardiogram were diagnostic of cyanotic congenital heart disease (Eisenmenger Syndrome). Mean systemic arterial oxygen saturation was 77% (range 71-81%) and mean hematocrit was 64.5% (range 53.7-69.5%). Eight out of eight eyes had evidence of increased retinal vascular tortuosity, but no patient had ocular symptoms, and all eyes had good visual acuity.
Increased retinal vascular tortuosity appears to be prevalent in adults with cyanotic congenital heart disease and is likely to be in response to hypoxemia and erythrocytosis. This conclusion is consistent with normalization of the retinal vascularity patterns after surgical relief of cyanosis resulting in resolution of hypoxemia and erythrocytosis.
本报告旨在描述患有青紫型先天性心脏病的成年人的视网膜检查结果。
从艾哈迈森/加州大学洛杉矶分校成人先天性心脏病中心门诊招募了4名年龄在27至47岁之间的青紫型先天性心脏病患者。每位患者均有心脏病专家进行的病史和体格检查、胸部X光、心电图、超声心动图、全血细胞计数以及动脉血氧饱和度检查。此外，每位患者还接受了视网膜专科医生的眼部检查、彩色眼底照相和荧光素血管造影。
胸部X光、心电图和超声心动图诊断为青紫型先天性心脏病（艾森曼格综合征）。平均体循环动脉血氧饱和度为77%（范围71 - 81%），平均血细胞比容为64.5%（范围53.7 - 69.5%）。8只眼中有8只出现视网膜血管迂曲增加的迹象，但没有患者有眼部症状，且所有眼睛视力良好。
视网膜血管迂曲增加在患有青紫型先天性心脏病的成年人中似乎很普遍，并且可能是对低氧血症和红细胞增多症的反应。这一结论与青紫症手术缓解后视网膜血管形态正常化相一致，青紫症缓解导致低氧血症和红细胞增多症得到解决。
