Retinal Vascular Patterns After Operative Repair of Aortic Isthmic Coarctation

Shamsa, Kamran; Perloff, Joseph K.; Lee, Eric; Wirthlin, R.; Tsui, Irena; Schwartz, Steven D.

doi:10.1016/j.amjcard.2009.09.046

Cited by 12 publications

(6 citation statements)

References 11 publications

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“…In some other human patients, changes in thickness of vessels are correlated with occurrence of abnormally shaped vessels. In patients with aortic isthmic coarctation, retinal arteriolar, but not venular diameter, was reduced [42], [43]. In the Elk3 deficient arteries, proliferation was unchanged, as we did not detect any change in diameter of abnormally shaped adult arteries.…”

Section: Discussioncontrasting

confidence: 43%

“…Blood pressure or hypertension could be expected to affect arterial shape. However, human patients with aortic isthmic coarctation after operative repair had retinal arteriolar tortuousity, but no hypertension, and blood pressure was normal [42], [43], suggesting that vessel tortuousity is not necessarily correlated with hypertension. In agreement, we did not detect any abnormalities in blood pressure of Elk3(−/−) knockout animals.…”

Section: Discussionmentioning

confidence: 96%

“…In the Elk3 deficient arteries, proliferation was unchanged, as we did not detect any change in diameter of abnormally shaped adult arteries. Venular tortuousity has been observed in some human patients diagnosed with aortic isthmic coarctation [42], [43] and in a screen for abnormalities of ocular fundi in monkeys [44]. Interestingly, only arteries are affected in the Elk3(−/−) eyes, as in the rat model of OIR [38], in humans with ATS (arterial tortuousity syndrome) [45], and in the mouse TNFα injection model [40].…”

Section: Discussionmentioning

confidence: 99%

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Elk3 Deficiency Causes Transient Impairment in Post-Natal Retinal Vascular Development and Formation of Tortuous Arteries in Adult Murine Retinae

et al. 2014

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Serum Response Factor (SRF) fulfills essential roles in post-natal retinal angiogenesis and adult neovascularization. These functions have been attributed to the recruitment by SRF of the cofactors Myocardin-Related Transcription Factors MRTF-A and -B, but not the Ternary Complex Factors (TCFs) Elk1 and Elk4. The role of the third TCF, Elk3, remained unknown. We generated a new Elk3 knockout mouse line and showed that Elk3 had specific, non-redundant functions in the retinal vasculature. In Elk3(−/−) mice, post-natal retinal angiogenesis was transiently delayed until P8, after which it proceeded normally. Interestingly, tortuous arteries developed in Elk3(−/−) mice from the age of four weeks, and persisted into late adulthood. Tortuous vessels have been observed in human pathologies, e.g. in ROP and FEVR. These human disorders were linked to altered activities of vascular endothelial growth factor (VEGF) in the affected eyes. However, in Elk3(−/−) mice, we did not observe any changes in VEGF or several other potential confounding factors, including mural cell coverage and blood pressure. Instead, concurrent with the post-natal transient delay of radial outgrowth and the formation of adult tortuous arteries, Elk3-dependent effects on the expression of Angiopoietin/Tie-signalling components were observed. Moreover, in vitro microvessel sprouting and microtube formation from P10 and adult aortic ring explants were reduced. Collectively, these results indicate that Elk3 has distinct roles in maintaining retinal artery integrity. The Elk3 knockout mouse is presented as a new animal model to study retinal artery tortuousity in mice and human patients.

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Section: Discussioncontrasting

confidence: 43%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

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Elk3 Deficiency Causes Transient Impairment in Post-Natal Retinal Vascular Development and Formation of Tortuous Arteries in Adult Murine Retinae

et al. 2014

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“…However, since 1951, retinal venular tortuosity has been recognised as another hallmark of coarctation retinopathy. 20,21 In addition to tortuosity and vascular narrowing, the earlier studies 22 also reported sclerosis and a swinging pulsation of the retinal arteries, which appeared as side-to-side shift of the arterial branches in time to the heartbeat. In contemporary coarctation patients, the incidence of such abnormalities is probably lower, owing to earlier surgical intervention in coarctation patients.…”

Section: Coarctation Of the Aortamentioning

confidence: 95%

The eye in CHD

et al. 2018

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In recent years, there has been a rise in the number patients with CHD surviving into adulthood. Many have complications related to their CHD or its treatments, outside the heart, including ocular abnormalities. The objective of this review is to highlight the ocular abnormalities that occur in adults with CHD, either from their condition or related to the common drugs prescribed to manage it. In particular, we reviewed the effects of cyanosis, coarctation of the aorta, endocarditis, and the side effects of Sildenafil and Amiodarone. A change in the retinal vasculature is a common observation with cyanosis or coarctation of the aorta. Occlusion of the retinal vessels may also be observed in cyanotic patients, as well as those with infectious endocarditis. Sildenafil has established ocular side effects; here they are explored in the context of therapy for pulmonary hypertension. Similarly, Amiodarone has established ocular risks, which are summarised. The high prevalence of ocular consequences in adult CHD patients reinforces the need for knowledge of the risks involved and for frequent ophthalmological screening where appropriate.

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“…This is an important issue as some interesting relationships between retinal vascular patterns and certain heart diseases are beginning to emerge. 23,24 A case has been reported in which branch retinal artery occlusion was caused by calcific embolization secondary to calcific aortic valvulopathy, 25 and anticoagulation has been hypothesized to be linked to intraocular hemorrhage in ARMD. 26 A general limitation of our study results from the ceiling effect, a limited ability to discriminate among those who are in relatively good health, which also limits the ability of the EQ-5D to make distinctions for those with relatively less visual impairment.…”

Section: Mobilitymentioning

confidence: 97%