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 ± 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 ≥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.
Retinal vascular abnormalities in aortic coarctation were commented on by Granstrom at a meeting of the Swedish Ophthalmological Society in 1948. Granstrom summarized his observations:
As in other cases of hypertension, these patients are sent as a matter of routine for examination to the eye department. As soon as I had seen a few such cases, it became evident that the retinal picture in coarctation of the aorta is often characteristic, the principle feature being pronounced tortuosity of the arteries. I therefore expressed the opinion that it should be possible to distinguish between coarctation of the aorta and other cases of juvenile hypertension by means of ophthalmoscopy.
Granstrom correctly concluded that the retinal abnormality in coarctation was benign and that “retinal changes usually present in hypertensive disorders are entirely lacking.” In 1951, he published observations on 40 patients with coarctation of the aorta, 60% of whom had tortuous retinal arterioles and, occasionally, “swinging pulsations of the entire arterial branches.” However, this unique retinal vascular pattern has received very little attention during the past 7 decades. The report by Granstrom included unoperated patients. Our report extends his observations by using current, high-technology ophthalmologic techniques to investigate vascular patterns of the retina in patients who had undergone surgical repair of aortic isthmic coarctation.
Methods
The University of California, Los Angeles, institutional review board approved the study. Ten patients (4 men and 6 women) were recruited from the Outpatient Clinic of the Ahmanson/UCLA Adult Congenital Heart Disease Center (Los Angeles, California). The criterion for enrollment was postoperative coarctation of the aortic isthmus, simple or complex. Patient age at enrollment was 35 ± 4.2 years (range 25 to 66).
The cardiac evaluation included upper and lower extremity blood pressure, anteroposterior and lateral chest radiography, 12-lead scalar electrocardiography, and ≥1 transthoracic echocardiograms with Doppler interrogation and color flow imaging. The paracoarctation aorta was also evaluated by magnetic resonance imaging or computed tomographic angiography.
The ophthalmic investigations were performed ≥3 years after the last coarctation repair (median 7 years, interquartile range 11, range 4 to 15). These investigations included visual acuity, intraocular tonometry, slit lamp examination, and dilated funduscopic examination that used either a standard fundus camera (Carl Zeiss Meditec, Jena, Germany) or wide-field digital photography (Optos Panoramic 200, Optos, Dunfermline, Scotland, United Kingdom).
Results
Postoperative coarctation gradients were absent in 4 patients, mild in 4, and mild to moderate in 1 patient. The systemic blood pressure averaged 130/70 mm Hg (range 100 to 140/56 to 82) and was controlled with an average of 2 medications.
Visual acuity ranged from 20/16 to 20/64. One patient with a visual acuity of 20/64 was amblyopic. The intraocular pressures and slit lamp examination findings were within normal limits. The retinal vascular patterns were bilaterally similar in 8 patients. In 2 patients, the left eye was significantly more affected than the right.
The results were compared with the normal retina, as defined by criteria of the Retina Division at the University of California, Los Angeles, Jules Stein Eye Institute. The results are summarized in Table 1 . Retinal arteriolar tortuosity ( Figure 1 ) was detected in 70% of the patients, an incidence that approximates the 60% found by Granstrom. The arteriolar tortuosity was graded as severe in 1 subject, moderate in 3, and mild in 3. In 1 subject, the tortuosity was moderate in the right eye and mild in the left eye.
| Pt. No. | Gender | Age at Surgical Repair or Intervention (yrs) | Age at Retinal Study (yrs) | Residual Gradient | Retinal Arterial Abnormality | Retinal Venous Abnormality |
|---|---|---|---|---|---|---|
| 1 | Male | 57 | 60 | 0 | Mild | 0 |
| 2 | Male | 22 | 37 | Mild | Moderate | Moderate |
| 3 | Female | 4 | 25 | Mild | Mild | 0 |
| 4 | Male | 1, 20, and 21 | 26 | 0 | Severe | Mild |
| 5 | Female | 13, 32 | 39 | Mild/moderate | Moderate | 0 |
| 6 | Female | 4 | 32 | Mild | 0 | 0 |
| 7 | Female | 4, 23 | 38 | 0 | Moderate | Moderate |
| 8 | Female | 17 | 51 | Mild | 0 | Mild |
| 9 | Male | 21, 49 | 62 | 0 | Mild | 0 |
| 10 | Female | 19 | 66 | Mild | 0 | 0 |
