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Retinal vascular patterns in adults with cyanotic congenital heart disease
Irena Tsui1, Kamran Shamsa, Joseph K Perloff
1Retina Division, Department of Ophthalmology, Jules Stein Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, CA, USA. itsui@jsei.ucla.edu
Insights
Adults with cyanotic congenital heart disease often show increased retinal vascular tortuosity. This finding is linked to hypoxemia and high red blood cell counts, but good vision is maintained.
Area of Science:
- Ophthalmology
- Cardiology
- Vascular Biology
Background:
- Cyanotic congenital heart disease (CCHD) significantly impacts adult systemic physiology.
- Understanding the ocular manifestations of CCHD is crucial for comprehensive patient care.
Observation:
- Retinal examinations were performed on four adult patients (27-47 years) diagnosed with CCHD.
- Diagnostic tests included echocardiography, ECG, chest X-ray, and blood work, alongside detailed ocular assessments like fundus photography and fluorescein angiography.
Findings:
- All examined eyes (8/8) displayed increased retinal vascular tortuosity.
- Despite vascular changes, patients reported no ocular symptoms and maintained good visual acuity.
- Physiological parameters revealed low arterial oxygen saturation (mean 77%) and high hematocrit (mean 64.5%).
Implications:
- Increased retinal vascular tortuosity is a prevalent finding in adults with CCHD.
- This vascular change is likely an adaptive response to chronic hypoxemia and erythrocytosis.
- Normalization of retinal vasculature post-surgical correction of cyanosis supports this hypothesis.
Introduction:
The purpose of this report is to describe retinal findings in adults with cyanotic congenital heart disease.
Methods:
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.
Results:
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.
Conclusions:
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.
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