Longitudinal changes in aortic distensibility in patients with Marfan syndrome

Background Patients with Marfan syndrome (MFS) may develop aortic dissection due to progressive dilatation in the entire aorta. Increased aortic stiffness, i.e.a. decreased distensibility has been shown to often precede these dismal sequelae. Therefore, we investigated longitudinal changes in aortic distensibility throughout the entire aorta by means of Cardiac Magnetic Resonance (CMR) imaging in patients with MFS. Methods This retrospective study included all MFS patients with four CMR examinations performed between 1996 and 2012. Aortic distensibility was measured and calculated by a single analyst, in the ascending, proximal- and distal descending, and abdominal aorta. Changes in distensibility were studied using linear mixed-effects regression models. Furthermore, we investigated the association between distensibility and age, sex, blood pressure, medication use, FBN1 mutation type, and previous aortic root surgery. Results In total, 35 MFS patients (age at inclusion 28 [IQR 23–32] years, 54% male) were included. Mean aortic distensibility was low in the ascending and proximal descending aorta (resp. 3.25±1.87, 3.91±1.73x10–3 mmHg–1) at the first scan. Distensibility decreased significantly over time at level 2, 3, and 4 (resp. p=0.021, p=0.002, p=0.038) (Figure 1). The rate of distensibility loss per year (x10–3 mmHg–1/year) was respectively 0.04 and 0.06 in the proximal- and distal descending aorta. Men seemed to have a lower but more stable distensibility, whereas women showed a higher distensibility at younger age, but a faster deterioration rate over time (difference in distensibility loss per year between men and women: 0.08, p=0.038). Distensibility did not correlate significantly with medication use, FBN1 mutation type or previous aortic root surgery. Conclusion Patients with MFS have low distensibility at all levels of the aorta at young age, which keeps decreasing over time. Men had lower distensibility at younger age than women. Distensibility was stably low in men, while still deteriorating over time in women. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): AMC FoundationHorstingstuit Foundation

Acute Aortic Syndrome in Marfan Syndrome with Chest Pain and Negative Initial Imaging

A 25-year-old male with Marfan Syndrome and previous aortic root surgery presented with severe chest and upper back pain. Initial CT angiogram showed mild aortic dilatation but no acute dissection. Anti-impulse therapy was initiated. Five days after presentation, follow-up CT angiography revealed a new pseudoaneurysm of the descending thoracic aorta. The patient underwent replacement of the mid-descending thoracic aorta with a collagen-impregnated woven polyester tube graft without complication. Despite negative initial imaging and relatively small aortic size, patients with Marfan Syndrome with acute chest pain should be treated with high suspicion and may benefit from admission and serial imaging.

1655 Thoracic Aortic Aneurysmal Disease: Timing and Outcome of Surgery

Aim A thoracic aortic aneurysm (TAA) is a pathological dilatation of the thoracic aorta. There is significant morbidity associated with a dilated aorta including mortality. Open thoracic surgery is the mainstay of management for proximal lesions, offering definitive treatment. We aim to evaluate the outcomes of patients operated on for TAA and consider high risk associations for mortality. Method Data was retrospectively collected from 234 patients that underwent elective open thoracic surgery at UHS for TAA disease, between 2015 and 2019. Demographics, clinical factors, surgical details, as well as outcome measures were gathered. Results There were 166 males (71%) and 68 females (29%), with a mean age of 66 years. We found that 105 operations involved the aortic root, 171 the ascending aorta, 20 the aortic arch and 12 the descending aorta. 30 day mortality was 5.13%. Female patients that underwent aortic root surgery (P = 0.05), as well as those with prosthetic aortic valves (P = 0.01)and post-operative complications were associated with a significantly higher mortality. Conclusions A number of factors are associated with morbidity and mortality in this high-risk Surgery. These should be considered when discussing risks of intervention with patients.

Clinical analysis of redo aortic root replacement after cardiac surgery: a retrospective study

Objectives To explore the etiology, previous cardiac procedure methods and outcomes of redo aortic root replacement after cardiac surgery. Methods A retrospective analysis of 41 patients who underwent aortic root replacement surgery in our hospital from February 2010 to February 2020 who underwent at least one cardiac surgery in the past, including 27 males and 14 females, with an average age of 49.5 ± 10.2 years old. Indications for reoperation include: aortic sinus dilation and ascending aortic aneurysm in 20 cases (48.8%), recurrent aortic dissection in 7 cases (17.1%), pseudoaneurysm of aortic root in 4 cases (9.8%), prosthetic valve endocarditis in 5 cases (12.2%) and paravalvular leakage in 5 cases (12.2%). According to whether the previous procedure involved aortic root surgery, they were divided into 2 groups, namely aortic root surgery-involved (ARS) group and non-aortic root surgery-involved (NRS) group. After the patients were discharged from hospitals, follow-ups were carried out through outpatient clinic or telephone for 5 years. Kaplan-Meier was used for survival analysis. Results All patients underwent Bentall procedure with a median sternum incision. Six patients (14.6%) died during the postoperative hospitalization and 3 patients (8.6%) died during the follow-up. The 1-year, 3-year, and 5-year survival in ARS group were 92.6, 92.6, and 92.6%, respectively; the 1-year, 3-year, and 5-year survival in NRS group were 100, 85.7, and 85.7%, respectively. There was no statistical difference between the two groups in the cause of redo aortic root replacement, procedure time, postoperative complications, postoperative hospital stay, hospital mortality, and 5-year cumulative survival (p > 0.05). Conclusions Redo aortic root replacement is difficult and high risk. Bentall procedure is still a reliable surgical option for redo aortic root replacement, with good short- and mid-term results. The prognosis of redo aortic root replacement is not necessarily related to the etiology of patient’s surgery and the methods of previous cardiac procedure.

The Skirt and Collar of The Valsalva Graft: One Dress Fits All

In the original pre-formed Valsalva graft, the skirt is the defining feature. It is a self-expanding portion, obtained by 90° rotation of the Dacron fabric corrugation with respect to the rest of the graft. Due to this manufacturing feature, the skirt length is equal to the graft diameter and, once pressurized, it expands by 25-30% (up to 10 mm) from the nominal size. Proximal to this bulged portion, a small collar completes the prosthesis. By virtue of this anatomical design, the Valsalva graft is recommended for most aortic root surgeries including valve-sparing and Bentall procedures. The skirt, by recreating the pseudo-sinuses, represents the key feature of the graft when used for aortic valve-sparing procedures (remodeling and reimplantation). However, in the Bentall procedure, the graft collar is an important feature because it is useful to attach the Valsalva graft to the valve prosthesis sewing ring when making a valved conduit. Moreover, once the graft is pressurized, the whole volume of the skirt better accommodates the struts and the leaflets of biological and mechanical prostheses. Furthermore, the expanded skirt reduces the distance and the tension on the coronary button anastomosis, increasing safety and possibly reducing complication rates. This review demonstrates the versatility of the Valsalva graft in various scenarios of aortic root surgery, with special emphasis on different uses of the skirt and the collar.