Retinal vascular patterns after operative repair of aortic isthmic coarctation.

The retinal arteriolar abnormality that accompanies aortic isthmic coarctation is as distinctive as it is unique but has thus far been virtually ignored. Accordingly, 10 patients (4 men and 6 women) were recruited for a study from the Ahmanson/UCLA Adult Congenital Heart Disease Center Outpatient Clinic. Patient age at enrollment was 35 + or - 4 years (range 25 to 66). The inclusion criterion was postoperative coarctation of the aortic isthmus, simple or complex. Coarctation gradients were absent, mild, or moderate, as determined by standard echocardiographic criteria. Ophthalmic investigations were done > or = 3 years after the last coarctation repair and included visual acuity, intraocular tonometry, slit lamp examination, and dilated funduscopic examination that used either a standard fundus camera or wide-field digital photography. The intraocular pressure and slit lamp examination findings were within normal limits. The retinal vascular patterns were bilaterally similar in all but 2 patients, in whom the left eye was significantly more affected than the right. Mild to severe retinal arteriolar tortuosity was present in 70% of our subjects, 4 of whom also had tortuosity of the retinal venules. Hypertensive retinopathy was absent. In conclusion, the unique retinal arteriolar tortuosity that accompanies coarctation of the aorta persists long after ideal surgical repair and normalization of systemic blood pressure. These retinal patterns were present in 70% of our patients, differing only by degree. A hitherto unrecognized tortuosity of venules was identified but to a lesser extent.