Aortic regurgitation secondary to diastolic prolapse of a tubular intimal flap into the left ventricle in a patient with anuloaortic ectasia

A 32-year-old man with distal skeletal manifestations of Marfan's syndrome had experienced shortness of breath and orthopnea for one month. Physical examination showed the presence of severe aortic regurgitation. Both noninvasive and invasive studies revealed that the aortic regurgitation was induced by previously undescribed peculiar and unusual etiology: diastolic prolapse of a circumferentially dissected tubular intimal flap into the left ventricle. The patient underwent surgical repair with striking clinical improvement.     

Surgical Treatment of Aortic Root Aneurysm Related to Marfan Syndrome in Early Childhood

The prognosis of Marfan syndrome in both adult and pediatric patients is primarily related to the cardiovascular complications. In infantile Marfan syndrome, although involvement of the mitral valve is the most frequently encountered cardiovascular lesion, the aortic root can be more worrisome because of its excessive dilatation, leading to aortic insufficiency or dissection. If the role of elective surgery is relatively well defined for adult patients, it is still debated during childhood. We report two patients, aged 22 months and 5 years, each presenting an aortic root aneurysm related to Marfan syndrome, and each treated with the Bentall procedure without specific age-related mortality or morbidity. These two patients experienced normal growth and were free of any complication for a follow-up period of 8 and 2 years, respectively. More than an absolute value of the aortic root dimension, it is the conjunction of the rate of progression of the aortic root dilatation, the degree and the duration of the aortic valve regurgitation, and its resulting left ventricular dysfunction that must be taken into consideration in choosing the surgical option.     

Bentall术后冠状动脉吻合口漏并假性动脉瘤形成的超声诊断价值

目的 探讨二维及彩色多普勒超声在Bentall术后冠状动脉吻合口漏并假性动脉瘤形成诊断中的应用价值。方法 对本院16例Bentall术后临床诊断为冠脉动脉吻合口漏并假性动脉瘤形成患者的二维超声及彩色多普勒超声征像进行总结,并与主动脉造影结果进行对比分析。结果 16例患者人工血管周围二维超声均可探及搏动性无回声区,冠状动脉吻合口处彩色多普勒可显示异常血流信号,其中左侧9例,右侧7例。所有超声结果均与主动脉造影结果一致。结论 超声心动图可以及时、准确、无创诊断Bentall术后冠脉吻合口漏及并发的假性动脉瘤,可作为临床随访检查的首选方法。     

Comparison of aortic root replacement in patients with Marfan syndrome

Objectives: Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in mid-term and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Methods: Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n = 48) or aortic dissection type A (n = 10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4–4.2) years. Results: In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p = 0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p = 0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p = 0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p = 0.086), especially after aneurysm (p = 0.057). After 10 years, freedom from aortic regurgitation ≥II° in the AVS group was 80% for aneurysm and 50% after dissection (p = 0.524). Conclusion: The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients.     

Surgical techniques for improving hemostasis during Bentall procedure

Objective： To evaluate the effect of modified surgical techniques on hemostasis used in aortic root replacement with a composite graft （Bentall procedure）. Methods： Data on 15 patients who underwent Bentall procedure during 2005 to 2007 were analyzed. The first 5 patients （Group 1） received the standard procedure. Then next 10 patients （Group 2） received the modified procedure. Techniques including ＂tandem suture line＂, ＂endo-button buttress＂, ＂sandwich anastomosis＂ and ＂left ventricle filling＂ were added to the standard procedure. Perioperative bleeding and the volume of blood transfusion required were compared to estimate hemostasis in different groups. Results： Between groups 1 and 2, a significant difference was found in postoperative bleeding [（2193±383） ml vs （1012±258） ml, respectively; P〈0.05] and in volume of blood transfusion required [（7242±1416） ml vs （2520±708） ml, respectively; P〈0.05]. Conclusion： The modified surgical techniques used in our study are effective in the improvement of the hemostasis in Bentall procedure.     

Use of coronary computed tomography angiography to detect coronary ostial stenosis after Bentall procedure

Coronary ostial stenosis is a rare complication of the Bentall procedure for aortic root and aortic valve replacement. We report a case of coronary ostial stenosis after a Bentall procedure that was detected by coronary computed tomography angiography and subsequent percutaneous coronary interventions.     

2例马凡氏综合征Bentall术后护理

马凡氏综合征(MFS)是一种常染色体显性遗传与结缔组织异常的先天性病症[1], 累及心血管系统时,病情凶险,死亡率高,矫治手术是目前唯一的治疗手段[2].2003～2007年江阴市人民医院开展2例Bentall手术,术后均进入ICU监护,恢复良好,现将护理体会报告如下.     

升主动脉瘤的外科治疗分析

目的总结升主动脉瘤的外科治疗经验。方法23例主动脉根部病变患者（年龄22～55岁,平均31．8岁）,其中Mafan’s综合征12例,Stanford A型主动脉夹层8例,主动脉瓣狭窄伴升主动脉扩张3例。手术类型：Bentall手术18例,Wheat手术3例,David手术1例,全弓置换加象鼻手术1例。结果全组手术死亡1例,术后随访12例平均41个月,无并发症。结论Bentall手术是治疗升主动脉瘤的主要术式,主动脉瓣叶正常,瓣膜轻度关闭不全,瓣环无明显扩张,David手术可以减少换瓣后服用抗凝药物所致的出血、血栓等并发症。