Objectives The frozen elephant trunk (FET) technique has become an important tool in the treatment of acute type A aortic dissection. The aim of this study was to evaluate the effect of long FET on spinal cord injury (SCI) and distal aortic remodeling after acute type A aortic dissection based on clinical and radiological outcomes. Methods From January 2018 to November 2019, 158 patients (mean age 51.8 years [range: 32–78 years], 88.6% male) with acute type A aortic dissection were treated by FET with 100 mm (n = 113) or 150 mm (n = 45) open hybrid stent graft prosthesis. Patients were divided into two groups according to the length of FET. The clinical and radiological outcomes of the patients were reviewed retrospectively. Results Postoperative outcomes did not differ significantly: in‐hospital mortality (9.7% vs. 6.7%, p = .758) and SCI (5.3% vs. 2.2%, p = .674). Aortic remodeling, which was evaluated by aortic diameter, true lumen diameter, false lumen (FL) diameter and the rate of FL complete thrombosis, was more positive in long FET group in the descending thoracic aorta during the follow‐up period. At the abdominal level, there was no statistically significant difference between the two groups. Conclusions The long version of FET does not increase the risk of SCI in patients with acute type A aortic dissection. The application of long FET can achieve better results in terms of remodeling of the thoracic aorta in the short‐ and medium‐term follow‐up.

Background. Marfan syndrome (MFS) is a connective tissue disease involving multiple systems, with thoracic aortic aneurysm (TAA) as the most common life-threatening manifestation. Method. A pedigree with TAA was investigated, and peripheral venous blood was extracted from six family members. After whole exome sequencing (WES) and chromosomal microarray analysis (CMA) in these individuals, bioinformatics and inheritance analyses were performed. Result. WES revealed a novel, small, 0.76 Mb microdeletion in 15q21.1, which cosegregated with the disease phenotype in the family and led to the haploinsufficiency of the fibrillin 1 (FBN1) gene, which is associated with MFS. This small copy number variant (CNV) was confirmed by CMA. Conclusion. Our study expands the phenotypic spectrum of the pathogenic CNV associated with MFS, thereby facilitating clinical genetic diagnosis and future genetic counseling for this family.

Aorto-oesophageal fistula is a rare but fatal disease. Open surgical repair or endotube repair of aorta and oesophagus save patients from circulatory deterioration, major haematemesis and severe infection. Here we present a rare case of an aorto-oesophageal fistula in the right hemi-arch caused by a fish bone treated with an alternative surgical strategy. Total aortic arch repair using the frozen elephant trunk procedure combined with oesophageal repair were performed. No aortic fistula, oesophageal fistula or artificial graft infection occurred in the follow-up.