BACKGROUND:Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes. METHODS:Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). RESULTS:Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm2, HR ​= ​4.25, 95%CI 1.49-12.17, p ​= ​0.007; as a continuous variable (per mm2 increment), HR ​= ​1.04, 95%CI 1.01-1.07, p ​= ​0.003). Additionally, ROACT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004). CONCLUSION:QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.

Background: The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). Methods: A total of 38 patients with severe PR (mean age 24.2 +/- 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scan-ning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. Results: All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed under -expanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3-8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8-94.7) and 83.5% (95%CI 73.8-93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. Conclusion: TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture.

Although the use of bioabsorbable occluder is expected to reduce the risk of metal occluder-related complications, it has not been approved due to incomplete degradation and new complications. Novel fully bioabsorbable occluders were designed to overcome such limitations. The aim of this study was to investigate the efficacy and safety of a fully biodegradable occluder in patients with ventricular septal defects. 125 patients with perimembranous ventricular septal defect (VSD) larger than 3 mm were screened from April 2019 to January 2020 in seven centers. 108 patients were enrolled and randomized into the bioabsorbable occluder group (n = 54 patients) and nitinol occluder group (n = 54). A non-inferiority design was utilized and all patients underwent transcatheter device occlusion. Outcomes were analyzed with a 24-month follow-up. All patients were successfully implanted and completed the trial. No residual shunt >2 mm was observed during follow-up. Transthoracic echocardiography showed a hyperechoic area corresponding to the bioabsorbable occluder which decreased primarily during the first year after implantation and disappeared within 24 months. Postprocedural arrhythmia was the only occluder-related complication with an incidence of 5.56% and 14.81% for the bioabsorbable and nitinol groups, respectively (P = 0.112). The incidence of sustained conduction block was lower in the bioabsorbable occluder group (0/54 vs. 6/54, P = 0.036) at 24-month follow-up. In conclusion, the novel fully bioabsorbable occluder can be successfully and safely implanted under echocardiography guidance and reduce the incidence of sustained postprocedural arrythmia. The efficacy and safety of this fully biodegradable occluder are non-inferior to that of a traditional nitinol one.

目的:探讨永存第五对主动脉弓(PFAA)的解剖学特征,评价几种常用影像学方法的诊断价值.方法:回顾分析2010年10月至2019年9月中国医学科学院阜外医院诊断的8例PFAA患者,收集其临床、影像及手术资料,总结其解剖及影像学特征、类型和伴发畸形情况,对比X线胸片、超声心动图、CT血管造影(CTA)检查结果.结果:本组8例患者中,7例的PFAA与主动脉弓位于同侧、不形成血管环结构,4例的PFAA伴发狭窄及闭锁,8例PFAA均起自升主动脉远端无名动脉开口旁或对侧,6例止于降主动脉近端,2例止于一侧肺动脉干;8例患者均合并心血管畸形,包括室间隔缺损、主动脉弓离断、法乐四联症、动脉导管未闭、共同动脉干、右心室双出口、肺动脉闭锁,以室间隔缺损和主动脉弓离断最常见;CTA对8例PFAA及伴发的其他心血管畸形患者均做出准确诊断,其准确度优于超声心动图及造影结果.结论:PFAA的解剖特征为:起自升主动脉远端无名动脉开口旁或对侧、止于降主动脉近端或一侧肺动脉干,呈弓状血管结构,部分伴发狭窄及闭锁,常合并其他心血管畸形.CTA为PFAA首选的无创检查方法,可部分替代或补充血管造影结果.

BACKGROUND:Nitinol-containing devices are widely used in clinical practice. However, there are concerns about nickel release after nitinol-containing device implantation. This study aimed to compare the efficacy and safety of a parylene-coated occluder vs. a traditional nitinol-containing device for atrial septal defect (ASD). METHODS:One-hundred-and-eight patients with ASD were prospectively enrolled and randomly assigned to either the trial group to receive a parylene-coated occluder (n = 54) or the control group to receive a traditional occluder (n = 54). The plugging success rate at 6 months after device implantation and the pre- and post-implantation serum nickel levels were compared between the two groups. A non-inferiority design was used to prove that the therapeutic effect of the parylene-coated device was non-inferior to that of the traditional device. The Cochran-Mantel-Haenszel chi-squared test with adjustment for central effects was used for the comparison between groups. RESULTS:At 6 months after implantation, successful ASD closure was achieved in 52 of 53 patients (98.11%) in both the trial and control groups (95% confidence interval (CI): [-4.90, 5.16]) based on per-protocol set analysis. The absolute value of the lower limit of the 95% CI was 4.90%, which was less than the specified non-inferiority margin of 8%. No deaths or severe complications occurred during 6 months of follow-up. The serum nickel levels were significantly increased at 2 weeks and reached the maximum value at 1 month after implantation in the control group (P < 0.05 vs. baseline). In the trial group, there was no significant difference in the serum nickel level before vs. after device implantation (P > 0.05). CONCLUSIONS:The efficacy of a parylene-coated ASD occluder is non-inferior to that of a traditional uncoated ASD occluder. The parylene-coated occluder prevents nickel release after device implantation and may be an alternative for ASD, especially in patients with a nickel allergy.

1临床资料 室间隔缺损(ventricular septal defect,VSD)是最常见的先天性心脏病,其中膜周部VSD是最常见的类型,约占全部VSD的70％左右[1].膜周部VSD的治疗方法包括经典的外科修补手术和介入封堵术.经皮介入封堵术治疗VSD最早由Lock等在1988年报道[2],但是在这项技术开展的早期,由于并发症较多,尤其是传导阻滞发生率较高,其推广应用受到较大限制.2002年以来,随着Amplatzer VSD封堵器的问世,尤其是国产封堵器的应用,VSD介入封堵治疗病例数迅速增加,成功率明显提高,但是严重迟发并发症也时有报道[3-6].为进一步探讨VSD封堵治疗的并发症发生原因及防治措施,现将中国医学科学院阜外医院膜周部VSD经皮介入封堵治疗的情况总结如下.

卵圆孔未闭(PFO)发生率高,约1/4的成年人均存在PFO.随着4项经导管封堵PFO和药物治疗相对比的随机对照研究结果的相继发表,各国相继更新了 PFO相关指南或共识.我国关于PFO治疗的推荐仍停留在2017年的"卵圆孔未闭预防性封堵术中国专家共识"上,鉴于PFO人群基数庞大,为规范PFO封堵术的临床应用,在借鉴国内外最新研究的基础上,结合我国具体情况,国内本领域专家形成了卵圆孔未闭相关卒中预防中国专家指南,以供参考.

目前中国大陆每年先天性心脏病(先心病)介入治疗3万多例,其中继发孔型房间隔缺损(atrial septal defect,ASD)封堵术约占44.30％,室间隔缺损(ventricular septal defect,VSD)封堵术约占15.13％,二者成功率分别为98.78％及96.16％[1].如何进一步提高先心病介入治疗成功率,降低严重并发症的发生率,是每位介入医师面临的挑战.除严格掌握先心病介入治疗适应证、规范化操作外,选择直径合适的封堵器至关重要.若封堵器直径选择过小,术后则易发生残余分流、封堵器移位及封堵器脱落.若封堵器直径选择过大,术后则有发生内皮化不全、血栓形成、心脏磨蚀及严重心律失常的潜在风险[2].为保证术前获得准确的ASD和特殊类型卵圆孔未闭(patent foramen ovale,PFO)的直径,介入医师深入探索ASD及PFO直径的测量方法.本期宋会军等[3]采用三维CT横断序列辅助技术测量ASD大小,其结果优于二维经胸超声心动图及二维CT血管造影(computed tomography angiography,CTA),能准确测量ASD上下径长度并消除其薄弱边缘.另外,CTA还可以除外ASD合并畸形或疾患,如部分性肺静脉畸形引流、静脉窦型或冠状静脉窦型ASD及冠状动脉疾病等.但CTA检查需要注射对比剂以及X线辐射是其不足之处.介入医师需结合患者临床情况酌情应用该检查技术,以保证患者最大获益.徐吉 等[4]对ASD/PFO合并房间隔瘤患者采用超声心动图指导下导丝参比封堵器选择策略,提高了封堵器选择准确率,22例ASD及7例PFO患者全部封堵成功,无二次封堵器更换,且避免多个封堵器置入.

目的 分析中国地方医院12年先天性心脏病(先心病)室间隔缺损(VSD)介入治疗的状况.方法 搜集并整理国家卫生健康委员会先心病介入网络直报系统的数据,分析自2009年1月～2020年12月经皮VSD介入治疗的状况.结果 12年中国150家地方医院共完成经皮VSD封堵术58,156例,技术成功率95.990％,严重并发症发生率0.349％,病死率0.021％.结论 室间隔缺损封堵术是一种有效的介入方法,近期疗效可靠,远期疗效仍需进一步观察.

目的 探讨COOK普通弹簧圈联合可解脱带纤维毛弹簧圈栓塞系统栓塞体肺侧支在复杂先天性心脏病(先心病)外科治疗中的应用价值及经验.方法 回顾性分析2016年1月至2018年8月中国医学科学院阜外医院收治的15例复杂先心病合并体肺侧支患者的临床资料,评价COOK普通弹簧圈联合可解脱带纤维毛弹簧圈栓塞系统在体肺侧支栓塞术中的可行性、安全性和有效性.结果 15例患者中共发现33支体肺侧支血管,均栓塞成功,无血管损伤、弹簧圈移位及脱落等术中并发症发生.术中共用COOK普通弹簧圈34枚,直径3～8 mm,长度3～8 cm;共用Interlock可解脱弹簧圈28枚,其中15枚为钻石型,13枚为普通2D型,弹簧圈直径为2～6 mm,长度为2.3～8.0 cm.15例患者中10例属于杂交手术,即在杂交手术室行体肺侧支栓塞术后即刻行外科手术;5例因先心病外科术后发生灌注肺或左心功能不全需持续呼吸机辅助通气,不能拔除气管插管而行心血管造影及体肺侧支栓塞术,术后均顺利拔除气管插管.结论 COOK普通弹簧圈联合可解脱带纤维毛弹簧圈栓塞系统栓塞体肺侧支在复杂先心病治疗中疗效确切,可推广应用.

2020年8月29日,《2020 ESC成人先天性心脏病(ACHD )管理指南》[1]在欧洲心脏病学会(ESC)年会重磅发布.距前一版指南(2010 年[2] )已有10年之久.10年间,随着治疗方式的改进与经验的积累,大量的临床证据涌现,新指南的更新内容涉及疾病诊断、复杂程度分级、治疗团队组建、风险评估、心律失常管理、肺动脉高压治疗、抗凝方案、终身管理计划、高危妊娠分类以及导管介入治疗领域的关键临床问题.

目的 评价经导管封堵先天性心脏病(先心病)外科矫治术后及室间隔缺损封堵术后室水平残余分流的安全性、有效性以及中长期随访结果.方法 回顾性分析2011年1月至2019年12月我院收治的35例先心病术后室水平残余分流患者的临床资料,结合介入封堵治疗情况和随访结果进行描述性分析.结果 35例患者中23例合并室间隔膜部瘤,其中6例右室面有两处或多发破口.34例封堵成功,其中4例采用2枚封堵器进行封堵.所封堵的右室面破口直径3～10.2 mm,平均(4.89±1.58)mm.所选择的封堵器直径5～20 mm,平均(9.16±3.59)mm,封堵器直径较缺损直径大1.8～10.4 mm,平均大(4.29±2.39)mm.封堵成功的患者中,31例随访1～51个月,与封堵术前相比,患者术后末次随访的左心室舒张末期内径、左心房内径及心胸比均有降低(P＜0.05).所有随访患者术后均未出现房室传导阻滞、感染性心内膜炎、封堵器脱落、主动脉瓣中大量反流及死亡等严重并发症.结论 经导管介入封堵术治疗先心病术后室水平残余分流是一种安全有效的方法,中长期随访效果良好,应成为此类患者首选的治疗方法.

目的 探讨影像学诊断先天性左冠状动脉主干闭锁(LMCAA)的可行性.方法 回顾性分析6例LMCAA患者,男3例、女3例,1例成人、5例婴幼儿.6例均接受超声心动图、多排螺旋CT(MDCT)及心血管造影检查.结果 1例超声提示左冠状动脉内径偏细,起源显示欠清晰,不除外左冠状动脉异常起源于肺动脉;1例超声提示左冠状动脉近段发育细,似壁内走行、开口狭窄;另4例超声仅提示二尖瓣脱垂并大量反流,冠状动脉未探及异常.5例经MDCT明确诊断为左冠状动脉主干闭锁,1例不除外左冠状动脉主干开口重度狭窄或闭锁.6例经心血管造影检查均明确诊断为LMCAA.结论 LMCAA属罕见先天性心脏病,超声可提示该病;CT对多数患者可明确诊断;心血管造影检查是诊断LMCAA的金标准.

目的 探讨Revolution CT对先天性左冠状动脉主干闭锁的诊断价值.方法 回顾性分析2012年2月至2019年3月我院收治的6例先天性左冠状动脉主干闭锁患者的资料,男女各3例;年龄10个月至48岁,体重7.7～55 kg.采用GE公司Revolution CT扫描仪进行前瞻单心跳收缩期(45％～50％)扫描成像.5例婴幼儿于基础麻醉及自由呼吸状态下行冠状动脉扫描,1例成人患者在清醒状态及屏气条件下进行检查.所有CT图像均由放射影像科2名具有5年以上心血管影像阅片经验的医师在AW4.6工作站进行阅读和评估.诊断结果与心血管造影结果作对比.结果 Revolution CT成像显示所有患者右冠状动脉均开口于主动脉右冠状动脉窦,5例患者诊断为先天性左冠状动脉主干闭锁,1例患儿可疑左冠脉主干开口闭锁或狭窄.所有患者左心房室均有扩大,2例患者二尖瓣下腱索走行区可见钙化.3例患者行手术治疗并证实为左冠脉主干闭锁,其中2例婴幼儿患者左主干开口及近段闭锁,1例成人患者左主干开口闭锁.结论 Revolution CT检查可在低辐射剂量条件下显示冠状动脉及心脏的病理解剖学信息,对绝大多数的先天性左冠脉主干闭锁患者可做出明确诊断.

目的:评价第二代Amplatzer动脉导管未闭封堵器(ADO II)在非动脉导管未闭结构性心脏病中临床应用的疗效.方法:2011年5月至2015年1月应用ADO II治疗非动脉导管未闭结构性心脏病的患者28例,年龄中位数5岁(5个月～42岁),体重中位数19.3 kg(4.2～91.0 kg).封堵后观察封堵器的位置、形态及封堵效果.结果:28例患者应用ADO II封堵手术均成功.膜部室间隔缺损患者14例,肺动脉闭锁合并室间隔缺损、行体肺动脉侧支血管封堵患者10例,其他适合应用ADO II封堵的患者4例.28例患者未发生与股动脉穿刺有关的并发症、溶血和血栓栓塞并发症,无死亡病例.结论:ADO II形态小、可控性好,容易输送,对血管损伤小,适用于小型膜部室间隔缺损、粗大迂曲体肺侧支血管及一些少见的结构性心脏病,封堵安全、有效.

早在1877年,德国病理学家Cohnheim[1]就提出反常栓塞的概念,随之不断被尸检证实.1985年Nellessen等[2]首次用超声证实了卵圆孔未闭(patent foramen ovale,PFO)处骑跨血栓.1994年Brogno等[3]不仅在PFO处发现骑跨血栓并于左、右心房内检测到血凝块,为反常栓塞提出了直接证据.因此,PFO有"脑的后门"之称,甚至有人提出"洞在心,病在脑"的理论.遗憾的是,临床上找到血栓的概率极低,大多数反常栓塞的诊断仍为推测性.因而PFO究竟是作为"无辜的旁观者"还是"参与者"成为争论的焦点.

目的 分析总结经皮肺动脉瓣球囊扩张术(PBPV)单中心临床应用30年治疗肺动脉瓣狭窄的临床价值及疗效.方法 1986年4月至2016年4月阜外医院共完成1229例PBPV,对其中临床资料完整的655例患者进行了6个月及以上的有效随访,随访时间为0.5～11.5年,平均(2.6±1.3)年,主要随访包括电话随访及门诊随访(以复查超声心动图为主).结果 1229例患者术后即刻导管测量的跨瓣压差(PG)由术前(74.40±29.64)mmHg下降至(25.05±8.62)mmHg,下降明显(P<0.001),最后1次随访时超声心动图测量的平均PG维持在(21.91±17.22)mmHg.PBPV成功率达到98.5％(1211/1229),发生术中及围术期死亡3例(死亡率0.2％),其他严重并发症(包括三尖瓣腱索断裂、肺动脉瓣重度反流)发生率为0.4％(5/1229).超声心动图随访中3.1％(20/655)肺动脉瓣反流程度较术后24 h有所增加,再狭窄发生率为1.1％(7/655).结论 30年的临床应用经验表明PBPV作为一种主要治疗单纯性肺动脉瓣狭窄的介入技术,其总体近、中、远期疗效满意,并发症发生率低,可作为一项瓣膜病微创治疗时代的主流技术继续应用于临床.

1 临床资料 患者 女,34岁.因"活动后气促3个月"来阜外医院就诊.既往病史:患者1年前在外院因"二尖瓣重度狭窄"行二尖瓣生物瓣置换术(术中置入圣犹达27号三叶生物瓣膜),术后6个月发生一次严重上呼吸道感染,治疗1个月后好转,且于术后6个月停用华法林.入院查体:脉搏65次/min,血压 105/63 mmHg(1 mmHg=0.133 kPa),双肺呼吸音清,心律齐,心尖部可闻及3/6级隆隆样舒张期杂音.入院心电图示:窦性心律,P波异常(既往心电图提示阵发性心房颤动).胸部X线示:双肺淤血,左心房右心室大,心胸比率0.55.经胸超声心动图示:二尖瓣换瓣术后生物瓣再狭窄(重度),二尖瓣瓣口面积(MVA)0.76 cm2,左心房前后径(LAD)45 mm,左心室舒张末期内径(LVEED) 47 mm.心脏多排螺旋CT增强扫描示:生物瓣未见明显钙化,左心房未见明确血栓形成.实验室检查未见明显异常.由于患者有生育要求,优先考虑经皮二尖瓣球囊成形术(PBMV),经与外科共同会诊及术前讨论后,决定给患者尝试行PBMV.手术经过:局麻下穿刺右股动、静脉行左右心导管检查,在透视及超声心动图引导下穿刺房间隔,采用直径26 mm进口Inoue球囊以17 mm、18 mm、19 mm、20 mm、21 mm、22 mm扩张生物瓣6次(图1～2),每次扩张后复查超声心动图,以22 mm球囊直径扩张后出现少量二尖瓣反流,遂停止扩张.MVA增加至1.32 cm2,肺动脉压由53/27(37) mmHg下降至45/13(27)mmHg,左心房平均压由22 mmHg下降至17 mmHg,左心室压由117/10 (45)mm Hg上升至119/12(46) mmHg,跨瓣压差由17 mmHg下降至10 mmHg.术后患者活动后气促症状明显减轻,6个月复查超声心动图示:MVA 1.3 cm2,左心房前后径40 mm,较术前明显缩小.胸部X线示:肺淤血较术前明显减轻,心胸比率0.52.

目的 探讨Interlock可解脱弹簧圈系统应用于冠状动脉-肺动脉瘘栓塞术的安全性及有效性.方法 回顾性分析2016年12月23日至2017年9月7日于中国医学科学院阜外医院选用Interlock可解脱弹簧圈系统行冠状动脉-肺动脉瘘栓塞术的6例冠状动脉-肺动脉瘘患者临床资料,其中包括男性2例,女性4例.所有患者均常规采用右侧桡动脉入径行冠状动脉造影以明确诊断,沿导管置入并释放Interlock可解脱弹簧圈于瘘管或瘤腔内,栓塞后行选择性冠状动脉造影评价疗效,观察术中及术后有无并发症发生.结果 6例选用Interlock可解脱弹簧圈行冠状动脉-肺动脉瘘栓塞术的患者均手术成功,无血管损伤及心肌梗死等术中并发症发生.有4例合并动脉瘤形成,其中3例应用弹簧圈填塞在动脉瘤体,1例栓塞在动脉瘤近心端瘘管.术中共用Interlock可解脱弹簧圈18枚,其中3枚为钻石型,15枚为普通2D型;弹簧圈直径为2～12 mm,长度为2.3～30 cm;瘘口直径为2～3 mm.即刻行选择性冠状动脉造影,其中3例为完全栓塞,3例有少量残余分流.术后所有患者胸闷、胸痛症状均得以缓解;超声心动图提示无异常血流,X线胸片提示弹簧圈位置、形态良好,心电图检查无新发心律失常或心肌缺血等改变.随访12个月,无心肌梗死、心源性休克、心室颤动、心室扑动、心脏破裂或猝死等主要不良心血管事件发生.结论 Interlock可解脱弹簧圈系统可控性高、安全性好,尤其适用于伴有动脉瘤形成、血管迂曲需要准确定位的冠状动脉-肺动脉瘘患者介入治疗.