ObjectiveReporting a case of hybrid procedure of extensive thoracoabdominal aortic aneurysm (TAAA) with type B dissection due to Marfan syndrome (MFS) using a prosthetic graft as the distal landing zone for stent-graft.MethodsRetrospectively summarize in-hospital profiles of a patient for who was diagnosed as MFS complicated with TAAA and type B dissection and admmited to Vascular Surgery Department of West China Hospital in May 2018. A GORE-TEX 18 mm×9 mm Y-shaped graft was sewn side-to-end to the bifurcation of left common iliac artery as the inflow site, and a self-made penta-limb graft was sewn side-to-end to the bifurcation of the 18 mm graft. The visceral and bilateral iliac arteries were reconstructed subsequently. Then, the release of the stent-graft was designed from distal to proximal. The distal part of the stent-graft was anchored into the main body of the 18 mm Y-shaped graft.ResultsThe patient underwent the operation successfully with a duaration of 6 h, blood loss of about 800 mL. No serious postoperative complications occurred. Computed tomography angiography at 2-year follow-up showed that the bypass grafts were patent without endoleak, stent migration, stent infolding or infections of the vessel graft and endograft.ConclusionThis modified management of the landing zone could be a proper choice for this kind of rare case as extensive aneurysm or dissection involved in patients with MFS.
Pregnancy complicated by aortic root aneurysm in patients with Marfan syndrome is one of the main causes of termination of pregnancy or even death in pregnant women. A very small number of pregnant women require cardiac surgery to preserve pregnancy under extracorporeal circulation, and all surgeries use aortic root replacement. We reported a 30-year-old patient with severe aortic regurgitation combined with giant aortic root aneurysm and Marfan syndrome in mid-pregnancy. Valve-sparing root replacement using reimplantation technology was performed via a multidisciplinary cooperation model. This not only achieved the patient’s desire to continue pregnancy but also avoided the anticoagulation and bleeding complications brought by mechanical valve replacement, reduced pregnancy risks and improved long-term quality of life. Postoperative echocardiography showed trace aortic regurgitation, aortic valve coaptation height of 0.6 cm, effective height of 1.1 cm, maximum aortic flow velocity of 1.4 m/s, mean transvalvular pressure gradient of 4.4 mm Hg, and satisfactory clinical results.
Objective To evaluate the incidence and characteristics of spontaneous pneumothorax in patients with Marfan syndrome (MFS), and to analyze its related factors. Methods The medical record of patients with MFS who were hospitalized in the First Affiliated Hospital of University of Science and Technology of China from March 1, 2017 to December 31, 2022 were retrospectively collected, to analyze the incidence of pneumothorax and related risk factors. Results A total of 53 patients (including 29 males, 55%) were included, with a median age of 32 years (ranging from 13 to 65 years). All patients denied family history of pneumothorax. Four cases (8%) had one episode of spontaneous pneumothorax, with pulmonary bullae involved the ipsilateral apex. Among them, 3 cases (75%) had single pulmonary bulla and thoracic deformity. Of the 49 patients without previous pneumothorax history, 9 (18%) had pulmonary bullae, of which 6 (67%) were located at the apex of the lung, and the remaining 3 (33%) were in the middle and lower lobes of the lung; In addition, 6 cases (12%) were coexisted with thoracic deformity. The incidence of pneumothorax in patients with pulmonary bullae in MFS was significantly higher than those without pulmonary bullae (31% vs. 0%, P=0.002), and the incidence of pneumothorax in patients with thoracic deformities was significantly higher than those with normal thorax (33% vs. 2%, P=0.013). Of the 4 patients with pneumothorax, 2 underwent bullectomy, 1 underwent thoracic drainage, and the other underwent thoracic puncture and aspiration procedures, respectively. No recurrence of pneumothorax was observed during the follow-up period. Sixteen cases of MFS with spontaneous pneumothorax (11 males, 69%) were reported in Chinese literature, with a median age of 17 years (ranging from 13 to 28 years). Among them, 3 cases (19%) had a family history of pneumothorax, and 14 cases (88%) had thoracic deformities. Bullae was found in all 5 patients with pneumothorax, 4 of them with chest CT images involved the ipsilateral upper lobe. Forty-four percents of 16 patients had pneumothorax recurrence. Conclusions The incidence of MFS complicated with spontaneous pneumothorax is significantly higher. However, most of them have no family history of pneumothorax. The incidence of pneumothorax with MFS is associated with pulmonary bullae (especially the apex of lung) and thoracic deformity. Timely surgical intervention should be carried out when pneumothorax happened.