Surgical treatment of Marfan syndrome and related disorders is all about dealing with uncertainties

Groenink, Maarten; Koolbergen, David R.

doi:10.1136/heartjnl-2017-312081

Cited by 3 publications

(2 citation statements)

References 8 publications

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“…Several surgical procedures have been developed to treat Marfan syndrome; however, the timing and choice of surgical procedure may have become even more complex and uncertain. Patients with Marfan syndrome who undergo different surgical strategies should undergo further observation (10). Aortic valve sparing root surgery (David procedure) has demonstrated equal outcomes in Marfan syndrome patients relative to ARR, although there are theoretical challenges of greater tissue fragility in patients with Marfan syndrome (4).…”

Section: Discussionmentioning

confidence: 99%

Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Zhu¹,

Jian²,

Ma³

et al. 2021

J Thorac Dis

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Section: Discussionmentioning

confidence: 99%

Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Zhu¹,

Jian²,

Ma³

et al. 2021

J Thorac Dis

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“…In an accompanying editorial, Groenink and Koolbergen2 discuss the complexities of timing of surgery in Marfan patients including the type of aortic root surgery, heterogeneity in disease progression rates, reliance on expert opinion alone for current recommendations about timing of intervention and variability in measuring aortic size with various imaging modalities (figure 1). 3 In addition, we still have large knowledge gaps in individual risk assessment, despite recent insights into genotype–phenotype correlations 4.…”

mentioning

confidence: 99%

Communicating with our patients for shared decision making

Otto

2018

Heart

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Mapping microarchitectural degeneration in the dilated ascending aorta with ex vivo diffusion tensor imaging

Wang,

Ching-Johnson,

Yin

et al. 2023

European Heart Journal Open

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Aims Thoracic aortic aneurysms (TAAs) carry a risk of catastrophic dissection. Current strategies to evaluate this risk entail measuring aortic diameter but do not image medial degeneration, the cause of TAAs. We sought to determine if the advanced magnetic resonance imaging (MRI) acquisition strategy, diffusion tensor imaging (DTI), could delineate medial degeneration in the ascending thoracic aorta. Methods and results Porcine ascending aortas were subjected to enzyme microinjection, which yielded local aortic medial degeneration. These lesions were detected by DTI, using a 9.4 T MRI scanner, based on tensor disorientation, disrupted diffusion tracts, and altered DTI metrics. High-resolution spatial analysis revealed that fractional anisotropy positively correlated, and mean and radial diffusivity inversely correlated, with smooth muscle cell (SMC) and elastin content (P < 0.001 for all). Ten operatively harvested human ascending aorta samples (mean subject age 61.6 ± 13.3 years, diameter range 29–64 mm) showed medial pathology that was more diffuse and more complex. Nonetheless, DTI metrics within an aorta spatially correlated with SMC, elastin, and, especially, glycosaminoglycan (GAG) content. Moreover, there were inter-individual differences in slice-averaged DTI metrics. Glycosaminoglycan accumulation and elastin degradation were captured by reduced fractional anisotropy (R2 = 0.47, P = 0.043; R2 = 0.76, P = 0.002), with GAG accumulation also captured by increased mean diffusivity (R2 = 0.46, P = 0.045) and increased radial diffusivity (R2 = 0.60, P = 0.015). Conclusion Ex vivo high-field DTI can detect ascending aorta medial degeneration and can differentiate TAAs in accordance with their histopathology, especially elastin and GAG changes. This non-destructive window into aortic medial microstructure raises prospects for probing the risks of TAAs beyond lumen dimensions.

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Surgical treatment of Marfan syndrome and related disorders is all about dealing with uncertainties

Cited by 3 publications

References 8 publications

Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Communicating with our patients for shared decision making

Mapping microarchitectural degeneration in the dilated ascending aorta with ex vivo diffusion tensor imaging

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