European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
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Eur J Cardiothorac Surg · Sep 2014
Outcome of aortic surgery in patients with Loeys-Dietz syndrome primarily treated as having Marfan syndrome.
Loeys-Dietz syndrome (LDS) is characterized by acute aortic dissection (AAD) at aortic diameters below thresholds for intervention in patients with Marfan syndrome (MFS). The aim was to evaluate the outcome of LDS patients primarily treated as having MFS. ⋯ Although early surgical intervention in LDS is warranted to avoid AAD, the current data suggest that once the diseased segment is repaired, there seems to be no additional burden in terms of mortality or reoperation rate compared with that in MFS patients, with or without confirmed FBN1 mutation.
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Eur J Cardiothorac Surg · Sep 2014
Comparative StudyComparison of the Ross/Ross-Konno aortic root in children before and after the age of 18 months.
Evaluation of long-term results after the Ross/Ross-Konno operation in patients <18 months of age, focusing on pulmonary autograft performance. ⋯ Ross/Ross-Konno can be invaluable in the younger age group but not without risk in the setting of ventricular dysfunction. Long-term performance of the neoaortic valve is significantly better than in older children, which is related to maintenance of normal root dimensions compared with progressive dilatation in patients undergoing Ross/Ross-Konno at older ages.