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Axel Orozco-Hernandez, Fernando Schoonewolff, Jeanette Mercado, R.V. Paul Chan, Virgilio Morales-Canton, Gerardo Garcia-Aguirre, Maria Ana Martinez-Castellanos; New Insights in Retinal Vascular Morphology in Neonates with Congenital Heart Disease. Invest. Ophthalmol. Vis. Sci. 2012;53(14):5868.
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The aim of the present study is to report the ocular findings in neonates with congenital heart disease, with novel descriptions in retinal vascular changes demonstrated by wide field fundus images and fluorescein angiography (FA).
Retrospective case series including four eyes of infants with complicated congenital heart disease in the Neonatal Intensive Care Unit (NICU) who underwent complete systemic and ophthalmologic evaluation, including 120 degrees wide-field color fundus imaging and FA (RetCam II, Clarity Medical Systems, Inc.)
The identified anatomic cardiac anomalies were persistence of arterial conduct with pulmonary hypertension and interventricular communication with left ventricle hypertrophy, both with hypertrophic cardiomyopathy. After a complicated course in the NICU, both infants died of cardiogenic shock. One patient was a 32 week preterm neonate with retinopathy of prematurity (ROP) and thrombocytopenia, the other was full term. Ophthalmologic evaluation and FA showed in all eyes venous tortuosity in the posterior pole as well as in the extreme periphery. At first, retinal vascular tortuosity of the preterm infant was thought to be plus disease of an Aggressive Posterior ROP (APROP), because of its association with thrombocytopenia, nevertheless FA showed zone 2, stage 1 ROP without plus disease or neovascularization and no progression was seen within the follow-up.
We demonstrate an early characteristic retinal vascular tortuosity pattern in neonates with congenital heart disease and hypertrophic cardiomyopathy. This vascular tortuosity suggested plus disease in the preterm infant. However, as FA and follow-up demonstrates no neovascularization or progression and also these findings were present in a non-ROP patient, we concluded that the clinical picture was related to congenital heart disease and should not be confused with the plus disease or APROP seen in premature babies.
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