Key result
In connective tissue disorders like Marfan and Loeys-Dietz syndromes, clinical features such as aortic root dimension, rapid growth, and arterial tortuosity predict adverse aortic complications.
Aortic root dimension, family history, rapid aortic growth, and arterial tortuosity are important predictors of aortic complications in pediatric Loeys-Dietz and Marfan syndromes.
In connective tissue disorders, such as Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), aortic root dimension has been the best predictor to date of adverse clinical outcomes and consequently is the usual basis for surgical indications for recommending prophylactic aortic root replacement. Other clinical features, including a family history of aortic dissection, rapid aortic growth, diffuse aortic root and ascending aortic dilation and marked vertebral arterial tortuosity, have also been shown to be associated with increased risk of aortic complications.
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Timothy J. Bradley (2024) conducted an editorial in Marfan syndrome and Loeys-Dietz syndrome. In connective tissue disorders like Marfan and Loeys-Dietz syndromes, clinical features such as aortic root dimension, rapid growth, and arterial tortuosity predict adverse aortic complications.
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