右腋下剖胸小儿心内直视术

目的 :探讨右侧剖胸小切口的手术要点及手术指征。　　方法 :本组 10 2例患者经右腋下小切口剖胸及体外循环下行小儿心内直视术 ,其中房间隔缺损 2 4例 ,室间隔缺损 6 8例 ,法乐四联症 5例 ,室间隔缺损合并房间隔缺损 3例 ,右心室双出口、Ebstein畸形各 1例。合并畸形 :左上腔静脉 4例 ,右心室双腔及动脉导管未闭各 1例。　　结果 :全组患者无死亡。　　结论 :切口的正确选择是术野良好显露的前提 ,主动脉插管是手术成功的关键。该切口适用于大部分常见先天性心脏病的矫治。     

经右外侧小切口与正中剖胸切口矫治房间隔与室间隔缺损的对比研究

目的 评价经右外侧小切口矫治房间隔与室间隔缺损手术的优点.方法 回顾性分析经右外侧切口48例患者(右外侧切口组)和正中切口 56例患者(正中剖胸切口组)矫治房间隔与室间隔缺损手术的结果 、主要并发症及随访资料,并进行比较.结果 两组患者的体外循环时间、心肌循环阻断时间、术后机械通气时间、术后住院时间比较,差异无统计学意义(P>0.05);两组均无死亡;术后胸液量右外侧切口组为(103.6±58.3)mL,正中剖胸切口组为(153.1±63.5)mL,两组比较差异统计学意义(P<0.05):随访(60.1±16.3)个月,右外侧切口组无并发鸡胸,正中剖胸切口组有1例并发鸡胸.结论 经右外侧小切口矫治房间隔与室间隔缺损安全、有效,与正中剖胸切口手术相比具有创伤小、出血少、瘢痕隐蔽、不破坏胸廓的骨性连续性等优点.     

右腋下微创小切口在先天性心脏病手术中的应用

目的 总结右腋下微创小切口在先天性心脏病手术中的应用及体会.方法 经右腋下微创小切口剖胸矫治先天性心脏病194例,其中单纯室间隔缺损76例、房间隔缺损92例、房间隔缺损合并室间隔缺损16例、室间隔缺损合并右室流出道狭窄10例.结果 手术顺利,全组无死亡,无并发症发生.本组患者体外循环时间(48.7±29.5)min,术后机械通气时间(4.8±1.4)h,术后引流量(170.5±82.2)ml.结论 右腋下小切口剖胸具有损伤小、恢复快、切口隐蔽美观、不破坏胸廓连续性等优点,符合微创外科的原则.     

小儿先天性心脏病微创小切口治疗体会

目的 探讨胸骨下段小切口与右胸小切口两种微创术式在小儿常见先天性心脏病中的应用.方法 选取解放军105医院心血管外科2011年3月至2013年8月收治的先天性心脏病患儿63例,其中男性27例,女性36例.63例中采用胸骨下段小切口33例、右胸小切口30例;单纯房间隔缺损(ASD)22例,单纯室间隔缺损(VSD)25例,室间隔缺损合并动脉导管未闭(PDA)4例,室间隔缺损合并房间隔缺损10例,室间隔缺损合并肺动脉瓣狭窄(PS)2例.结果 所有患儿均顺利完成手术,无死亡,无低心排血量、肺部感染、切口感染等近期并发症.术后随访6个月至1年,无残余漏、Ⅲ度房室传导阻滞、胸廓畸形等远期并发症.结论 胸骨下段小切口与右胸小切口两种微创术式选择性应用于常见先天性心脏病,安全可靠、创伤小、切口美观,值得推广.     

右胸前外侧与胸骨正中切口矫治先天性心脏病的临床对比研究

目的 :研究比较右胸前外侧切口和胸骨正中切口在临床上矫治先天性心脏病的优缺点。方法 :将术前超声心动图检查及手术确诊为单纯房间隔缺损或单纯室间隔缺损的患者共 15 1例 ,分成右胸前外侧切口和胸骨正中切口手术 2组 ,从术后呼吸机辅助呼吸时间、术后 2 4h引流量、体外循环时间、术后住院天数、并发症的发生率等方面进行统计分析比较。结果 :术后呼吸机辅助时间及术后住院时间 ,2组病人无明显差别 ;术后 2 4h引流量 ,右胸前外侧切口组较少 ;体外循环时间胸骨正中切口组较短 ;胸骨正中切口组切口感染发生率为 3 42 %、胸骨畸形发生率为 4 2 7% ,右胸前外侧切口组无切口感染和胸骨畸形。结论 :右胸前外侧切口可对经右房或房间沟入路完成的简单的心内直视手术 ,具有美容效果好、术后渗血少、切口感染发生率低、无胸骨畸形等突出优点。缩短住院时间不明显。是否能减少手术创伤 ,有待进一步研究     

389例先天性心脏病右外侧小切口剖胸心内直视手术

目的:总结经右胸外侧小切口体外循环下行先天性心脏病心内直视手术经验。方法:1996年11月至2011年12月,我科室应用右胸小切口完成各类先天性心脏病(先心病)的心内直视手术389例。年龄1.5～57岁,平均13.5岁。体质量7.6～68 kg,平均24.5 kg。手术切口后缘自右腋中线第3肋间处,向前下斜行达锁骨中线第6肋间处做8～12 cm弧形切口,第4肋间入胸。手术方式:房间隔缺损修补157例,室间隔缺损修补150例,部分房室隔缺损矫治8例;右心室流出道疏通术24例;法洛三联症矫治32例,法洛四联症矫治5例以及其他畸形矫治13例。随机选择同期常规手术(胸正中切口)患者100例作为对照。结果:研究组切口长度、手术时间及术后引流量都显著低于对照组;研究组术后呼吸机辅助时间明显低于对照组;术后监护时间各组间差异无统计学意义。研究组手术死亡4例(1.03%),2例死于术后脑栓塞,另外2例死于低心排出量综合征(低心排)。结论:右胸小切口手术入路,可安全有效地行常见先心病的矫治。该技术创伤小、恢复快、美观,并提高了患者的生活质量,值得进一步推广应用。     

胸骨下段小切口心内直视手术40例

目的介绍胸骨下段小切口心内直视手术体会。方法选择10岁以下房间隔缺损3例、室间隔缺损20例,纵劈胸骨上至第二肋间;10岁以上房间隔缺损5例、室间隔缺损3例、风湿性二尖瓣狭窄9例,纵劈胸骨上至第二肋间并向右侧横断。常规体外循环,胸正中小切口第三胸肋关节水平至剑突根部,完成心内直视手术。结果无手术死亡,平均主动脉阻断时间、体外循环时间、术后24h胸液量与对照组无明显差异。切口长度约为常规胸骨正中切口的60%。结论胸骨下段小切口可以顺利完成部分心内直视手术,美观且保留了胸廓的连续性,无胸骨前凸畸形。     

右外侧小切口剖胸矫治合并心底部畸形的室间隔缺损体会

目的:总结经右外侧小切口剖胸矫治合并心底部畸形的室间隔缺损(VSD)手术方法及临床疗效。方法:总结经右侧剖胸入路矫治合并简单心底部畸形(动脉导管未闭和/或永存左上腔静脉)的VSD 16例(R组),同期经右侧剖胸入路矫治不合并心底部畸形的VSD 144例(C组),R组在矫治心内畸形的同时,游离结扎动脉导管或游离阻断左上腔静脉。通过对比体外循环时间、主动脉阻断时间、术后机械通气时间、术后引流量、监护室时间以及术后住院时间,观察处理简单心底部畸形对手术的影响和治疗效果。结果:与C组相比较R组体外循环时间[(65.31±17.10)vs.(55.02±15.97)min P<0.05]明显延长,但主动脉阻断时间[(33.44±8.02)vs.(31.40±10.12)min]、术后机械通气时间4.25 hvs.13.08 h、术后引流量88.44 mL vs.89.83 mL、监护室时间2.38 d vs.1.95 d、术后住院时间[(7.88±1.82)vs.(7.88±2.30)d],差异无统计学意义。2组均无死亡及严重并发症,C组有8例患儿术后血痰,1例肺部感染;R组2例血痰。结论:经右外侧小切口剖胸入路矫治合并简单心底部畸形的VSD安全可行。     

经右外侧小切口矫治室间隔缺损合并复杂畸形技术的应用

目的:分析经右外侧小切口剖胸矫治合并需行心底部操作的室间隔缺损(VSD)手术方法及临床疗效。方法:比较经右外侧小切口剖胸矫治需行心底部复杂操作(干下型VSD或右心室流出道狭窄或主动脉瓣下狭窄)的VSD 84例(A组),和经右外侧小切口剖胸矫治不合并心底部畸形的VSD450例(B组),A组在矫治心内畸形的同时需切开主动脉、肺动脉或者右心室流出道。通过对比体外循环时间、主动脉阻断时间、术后机械通气时间、术后引流量、监护室时间以及术后住院时间,观察在心底部进行复杂操作对手术的影响和治疗效果。结果:尽管A组比B组需要更长的体外循环时间[(60.68±16.12)vs.(47.07±16.55)分,P<0.05]和主动脉阻断时间[(37.68±12.84)vs.(25.16±12.60)分,P<0.05],但在术后评价指标,机械通气时间9.18vs.8.51小时、术后引流量(96.98 vs.92.30m L)、监护室停留时间(1.44 vs.1.40)天、术后住院时间[(7.00±1.76)vs.(7.06±2.23)天,P<0.05]。两组均无死亡及严重并发症。A组有3例血痰、肺部感染1例,室上性心动过速1例。B组有9例患儿术后血痰,2例肺部感染,临时起搏器4例。结论:经右外侧小切口剖胸矫治VSD的同时行心底部复杂操作安全可行。     

右外侧小切口剖胸矫治小儿先天性心脏病1972例

目的:总结右外侧小切口剖胸矫治先天性心脏病(先心病)的经验,探讨右外侧切口在先心病的应用与推广。方法 :2002年1月至2011年10月,本手术组经右外侧剖胸小切口完成1 972例小儿先天性心脏畸形矫治。其中男性1 143例,女性829例。年龄平均38.7个月(3～489个月),体质量平均11.6 kg(4.8～69 kg)。主要病种:室间隔缺损、房间隔缺损及法洛四联症等。结果 :术后并发症81例(4.1%):低心排出量综合征(低心排)26例(死亡3例)、严重肺部感染22例(死亡2例)、二次开胸止血8例(死亡1例)、多脏器功能衰竭4例(死亡1例)、一过性脑功能障碍5例、术后残余分流6例(经原切口再次手术1例)、右膈神经麻痹5例(1例膈肌折叠)、术后房室传导阻滞4例(1例置永久起搏器)、乳糜胸2例。随访3～108个月,3例法洛四联症存在残余梗阻26～50 mmHg(1 mmHg=0.133 kPa)观察中,1例二尖瓣成型术后大量反流行二尖瓣置换。结论:先心病可以在右外侧剖胸小切口下完成,该入路安全可靠、创伤小、暴露好并恢复快。     

Ochronosis: a report of a case and a review of literature

A patient with alkaptonuria and ochronotic pigment deposited in articular cartilage and sclerae clinically manifested a serious osteoarthritis of the peripheral and axial joints and synchondrosis, typically involved in long lasting cases of this hereditary defect of homogentisic acid oxidase. This is the first patient with this disorder reported, where a non-cemented total knee prosthesis (PCAR) was applied on both knees. This was possible due to the good quality of the bone stock, which did not seem to be impaired by ochronosis. Our patient had no cardiac symptoms or murmurs, but had a slight calcification in the annulus of aorta observed with echocardiography, a useful new method for screening this disease manifestation. A third new aspect reported is the immunopathology of the synovial tissue. Small pieces of torn-off cartilage were seen embedded in the synovial stroma. This was associated with a slight hyperplasia of the C3bi-receptor positive and proline hydroxylase positive type A and B synovial lining cells. Perivenular infiltrates contained CD2 positive T lymphocytes, mostly belonging to the CD4 subset, and some C3bi-receptor positive monocytes. Activated CD25 positive and immunoglobulin light chain positive T and B lymphocytes were absent or few. Because modern medicine has much to offer to those suffering from this ancient inborn error of metabolism in the form of new specific diagnostic methods and new surgical modes of treatment, such as endoprosthesis surgery and cardiac valve replacement, we also present a literature overview of this interesting condition.     

Report of a case of a giant phytobezoar in a patient with a duodenal bulb ulcer.

A case of a large phytobezoar (750 g weight and with the length of 29 cm) due to Khormalou (Persimmon) is being reported in a young patient with chief complaint of abdominal pains and concomitant duodenal ulcer. Review of the literature in this subject shows that bezoars of this size and weight are relatively rare in healthy individuals.     

Charge of a quasiparticle in a superconductor

Nonlinear charge transport in superconductor–insulator–superconductor (SIS) Josephson junctions has a unique signature in the shuttled charge quantum between the two superconductors. In the zero-bias limit Cooper pairs, each with twice the electron charge, carry the Josephson current. An applied bias V_SD leads to multiple Andreev reflections (MAR), which in the limit of weak tunneling probability should lead to integer multiples of the electron charge ne traversing the junction, with n integer larger than 2Δ/eV_SD and Δ the superconducting order parameter. Exceptionally, just above the gap eV_SD ≥ 2Δ, with Andreev reflections suppressed, one would expect the current to be carried by partitioned quasiparticles, each with energy-dependent charge, being a superposition of an electron and a hole. Using shot-noise measurements in an SIS junction induced in an InAs nanowire (with noise proportional to the partitioned charge), we first observed quantization of the partitioned charge q = e*/e = n, with n = 1–4, thus reaffirming the validity of our charge interpretation. Concentrating next on the bias region eV_SD ～ 2Δ, we found a reproducible and clear dip in the extracted charge to q? ～ 0.6, which, after excluding other possibilities, we attribute to the partitioned quasiparticle charge. Such dip is supported by numerical simulations of our SIS structure.Excitations in superconductors (Bogoliubov quasiparticles) can be described according to the Bardeen–Cooper–Schrieffer (BCS) theory (1) as an energy-dependent superposition of an electron with amplitude u(ε), and a hole with amplitude v(ε), where the energy ε is measured relative to the Fermi energy (2). Evidently, the expectation value of the charge operator (applied to the quasiparticle wave function), which we address as the quasiparticle charge e* = q(ε)e, is smaller than the charge of an electron, q(ε) = |u(ε)|² ? |ν(ε)|² (3). Solving the Bogoliubov–de Gennes equations, one finds that |u(ε)|2=1/2[1+(ε2−Δ2/ε)] and |v(ε)|2=1/2[1−(ε2−Δ2/ε)], with the expected charge evolving with energy according to q(ε)=ε2−Δ2/ε––vanishing altogether at the superconductor gap edges (3). Note, however, that the quasiparticle wave function is not an eigenfunction of the charge operator (3, 4). Properties of quasiparticles, such as the excitation spectra (5), lifetime (6–10), trapping (11), and capturing by Andreev bound states (12, 13), had already been studied extensively; however, studies of their charge are lagging. In the following we present sensitive shot-noise measurements in a Josephson junction, resulting in a clear observation of the quasiparticle charge being smaller than e, q(eV_SD∼2Δ) < 1, and evolving with energy, as expected from the BCS theory.To observe the BCS quasiparticles in transport we study a superconductor–insulator–superconductor (SIS) Josephson junction in the nonlinear regime. The overlap between the wave functions of the quasiparticles in the source and in the drain is expected to result in a tunneling current of their effective charge. This is in contrast with systems which are incoherent (14, 15) or with an isolated superconducting island, where charge conservation leads to traversal of multiples of e – Coulomb charge (16). As current transport in the nonlinear regime results from “multiple Andreev reflections” (MAR), it is prudent to make our measurements credible by first measuring the charge in this familiar regime.In short, the MAR process, described schematically in Fig. 1, carries a signature of the shuttled charge between the two superconductors (SCs), being a consequence of n traversals through the junction (as electron-like and hole-like quasiparticles), with n an integer larger than 2Δ/eV_SD. A low transmission probability t (via tunneling through a barrier) in the bias range 2Δ/n < eV_SD < 2Δ/(n ? 1) assures dominance of the lowest order MAR process (higher orders are suppressed as tⁿ), with the charge evolving in nearly integer multiples of the electron charge. Although there is already a substantial body of theoretical (3, 17–23) and experimental (24–29) studies of the MAR process, charge determination without adjustable parameters is still missing. An important work by Cron et al. (27) indeed showed a staircase-like behavior of the charge using “metallic break junctions;” however, limited sensitivity and the presence of numerous conductance channels some of which with relatively high transmission probabilities did not allow exact charge quantization. Our shot-noise measurements, performed on a quasi-1D Josephson junction (single-mode nanowire) allowed clear observation of charge quantization without adjustable parameters. To count a few advantages: (i) the transmission of the SIS junction could be accurately controlled using a back-gate; (ii) this, along with our high sensitivity in noise measurements, enabled us to pinch the junction strongly (thus suppressing higher MAR orders); and (iii) with the Fermi level located near the 1D channel van Hove singularity, a rather monoenergetic distribution could be injected (SI Appendix, section S7).Open in a separate windowFig. 1.MAR. Illustrations of the leading processes contributing to the current as function of bias. In general, for 2Δ/(n ? 1) > eV_SD > 2Δ/n the leading charge contribution to the current is ne. An electron-like quasiparticle is denoted by a full circle, whereas a hole-like quasiparticle is denoted by an empty circle. (A) When the bias is larger than the energy gap, eV_SD > 2Δ, the leading process is a single-path tunneling of single quasiparticles from the full states (Left) to the empty states (Right). This current is proportional to the transmission coefficient t. Higher-order MAR process (dashed box), being responsible for tunneling of Cooper pairs, is suppressed as t². (B) For 2Δ > eV_SD > Δ, the main charge contributing to the current is 2e with probability t². (C) For Δ > eV_SD > 2Δ/3, the main charge contributing to the current is 3e with probability t³.     

Hemoptysis in a patient with a previous surgical correction of a tetralogy of Fallot

Aorto-bronchial fistula is a rare but associated with a height rare of mortality. Although most reported cases are secondary to infectious aneurysms, cases arising after surgery to correct congenital cardiovascular abnormalities have recently been described. We report the case of a 41-year-old patient with recurrent hemoptysis and a history of Fallot's tetralogy corrected in childhood. Given such a case of hemoptysis in a patient with a history of cardiovascular surgery, the correct diagnostic approach includes fiberoptic bronchoscopy, helicoidal CAT and/or NMR, and aortography. Diagnostic confirmation should be followed soon by corrective surgery.     

Dissection of a viral autoprotease elucidates a function of a cellular chaperone in proteolysis

Replication of positive-strand RNA viruses involves translation of polyproteins which are proteolytically processed into functional peptides. These maturation steps often involve virus-encoded autoproteases specialized in generating their own N or C termini. Nonstructural protein 2 (NS2) of the pestivirus bovine viral diarrhea virus represents such an enzyme. Bovine viral diarrhea virus NS2 creates in cis its own C terminus and thereby releases an essential viral replication factor. As a unique feature, this enzyme requires for proteolytic activity stoichiometric amounts of a cellular chaperone termed Jiv (J-domain protein interacting with viral protein) or its fragment Jiv90. To obtain insight into the structural organization of the NS2 autoprotease, the basis for its restriction to cis cleavage, as well as its activation by Jiv, we dissected NS2 into functional domains. Interestingly, an N-terminal NS2 fragment covering the active center of the protease, cleaved in trans an artificial substrate composed of a C-terminal NS2 fragment and two downstream amino acids. In the authentic NS2, the 4 C-terminal amino acids interfered with binding and cleavage of substrates offered in trans. These findings strongly suggest an intramolecular product inhibition for the NS2 autoprotease. Remarkably, the chaperone fragment Jiv90 independently interacted with protease and substrate domain and turned out to be essential for the formation of a protease/substrate complex that is required for cleavage. Thus, the function of the cell-derived protease cofactor Jiv in proteolysis is regulation of protease/substrate interaction, which ultimately results in positioning of active site and substrate peptide into a cleavage-competent conformation.