Natural history of the distal aorta following elective root replacement in patients with Marfan syndrome.

OBJECTIVE: It is unclear if the addition of a prophylactic arch operation is beneficial at the time of root replacement in patients with Marfan syndrome (MFS). This project aims to understand the fate of the distal aorta following elective root replacement in patients with MFS. METHODS: Between 2000 and 2019, 124 adult patients with MFS were identified as having undergone elective aortic root replacement with a clamped distal aortic anastomosis during their lifetime. Serial axial imaging was analyzed. The primary outcome was a composite of subsequent type B aortic dissection (TBAD), aneurysmal degeneration (>4 cm), and aortic reintervention. Secondary outcomes included subsequent TBAD and mortality. RESULTS: Mean age at root replacement was 33.3 years. Median follow-up was 11.3 years. Thirty-one patients (25%) experienced the primary outcome, with no survival difference (P = .9). The crossclamp and aortic cannulation sites were stable (growth rate, 0.33 mm/year), and 2 patients (1.6%) required reintervention there. Twenty-three patients (19.8%) experienced subsequent TBAD. Patients with TBAD had a higher rate of distal degeneration (P < .001), but no significant change in survival (P = .2). Preoperative hypertension (odds ratio, 3.96; P < .05) and younger age at root replacement (odds ratio, 1.05; P < .05) increased the risk of TBAD, based on regression analysis. CONCLUSIONS: The distal aorta (including the clamp and cannulation site) overall appears stable in patients with MFS following elective root replacement without prophylactic arch operation. Development of TBAD seems to be the primary driver of distal degeneration. Factors associated with TBAD development included hypertension, underscoring the importance of strict blood pressure control in these patients.

Use of a sutureless aortic valve in reoperative aortic valve replacement.

Objectives: Management of degenerated bioprosthetic aortic valves remains a challenge. Valve-in-valve transcatheter aortic valve replacement (AVR) has limited utility in the presence of small annuli/prosthetic valves. Sutureless valves may offer an advantage over traditional redo AVR by maximizing effective orifice area due to their unique design as well as ease of implant. Methods: Twenty-two patients undergoing redo AVR received a sutureless valve in our institution over the past 5 years. All patients were determined to be poor candidates for valve-in-valve transcatheter AVR due to a combination of small annulus size, low coronary heights, and/or underlying valve characteristics (ie, mechanical valves). Results: Median time from implant to redo AVR was 8 years. One patient died within 30 days. In the 13 patients who had a 21 mm or smaller valve explanted, 5 small, 7 medium, and 1 large Perceval valves were implanted (all with larger internal diameters than the explanted valve). The average postoperative gradient of the cohort valves was 14.8 mm Hg compared with 38.8 mm Hg preoperatively. Conclusions: In addition to their ease of use and rapid deployment, sutureless bioprosthetic aortic valves offer significant physiological advantages in patients with degenerated prosthetic aortic valves and small anatomical annuli. It can also simplify the surgical approach to redo AVR following a Bentall procedure. If long-term durability is confirmed, sutureless valves should be considered in a broader population of patients for both redo and primary aortic valve replacement surgery.

Giant Cell Tumor of Bone Presenting as Left Posteromedial Chest Wall Tumor.

Giant cell tumor is a relatively uncommon bone tumor rarely originating from the chest wall. Given its proximity to vital structures in the thoracic cavity, treatment options may be challenging. We report the case of a patient with a giant cell tumor of the posterolateral chest wall with invasion of the thoracic spine treated with neoadjuvant denosumab, followed by surgical resection.