Ebstein's malformation. surgical treatment and results

Ebstein's malformation can be defined as an anomaly of the tricuspid valve existing in the setting of a right ventricular dysfunction. The technique introduced by Carpentier in 1980 is based on the concept of mobilization of the restrictive anterosuperior leaflet associated with a longitudinal plication of the inlet component of the right ventricle. From January 1980 to December 1999, 142 patients underwent surgery. The mean age was 25 +/- 15 years (1-65). Cyanosis was present in 48% and associated lesions in 64% of the patients. Patients were classified using a functional approach according to the severity of the lesions. Mild displacement of the septal leaflet, along with small size of the atrialized chamber was seen in 5% (referred to as Type A). Massive displacement of the septal leaflet, but with normal motion of the anterosuperior leaflet and an extensive atrialized chamber, was seen in 35% (Type B). In 51%, the mural (inferior) leaflet was absent, the anterosuperior leaflet was severely restricted by muscular trabeculations and very short tendinous cords, and the anterolateral papillary muscle was incorporated in the right ventricular wall. In these patients (Type C), the atrialized chamber was markedly enlarged and had dyskinetic walls. In such cases, the contractility of the distal (functional) right ventricle was also impaired, and some degree of stenosis of the tricuspid valve was present in one-fifth of them. In the most severe cases (8%), the leaflet tissue of the valve was extremely reduced and the right ventricular walls were thin and contracted poorly. This resulted in the so-called tricuspid sack arrangement (Type D). Valve replacement was needed in only 4 cases, with conservative surgery being achieved in 138 patients by means of mobilization of the anterosuperior leaflet and longitudinal plication of the inlet component of the right ventricle. Additional procedures included the use of a prosthetic ring (94 patients) and partial Glenn anastomosis (30 patients). The hospital mortality was 10%, mainly due to acute postoperative right ventricular failure. Actuarial survival was 75% at 10 years. After operation, 94% of the patients were in functional class I or II of the New York Heart Association, and 88% had no or mild tricuspid valve insufficiency as judged by echocardiography. The rate of reoperation was 9% with a mean delay of 3 years. A second repair was performed in 5 patients. Freedom from reoperation was 87% at 10 years. Sinus rhythm was present in 81%, and 8 pacemaker devices were implanted, 5 for surgically induced atrioventricular block, and 3 because of preoperative conduction disturbances. The use of the partial Glenn anastomosis was introduced recently in cases where the right ventricular contractility was severely impaired, and/or tricuspid valve repair was difficult, and/or permanent atrial fibrillation was present. In those patients with high risk, adding partial Glenn anastomosis reduced the operative mortality from 24% to 6%. Another benefit of the cavo-bipulmonary anastomosis was better functional tolerance of mild residual tricuspid valve incompetence. Those patients with the tricuspid sack arrangement had a high rate of reoperation (2/11) and valve replacement (3/11), but suffered no operative deaths. We conclude that tricuspid valvoplasty associated with longitudinal right ventricular plication is superior to valve replacement. The arrangement producing a tricuspid sack is not suitable for conservative surgery. An associated cavo-pulmonary anastomosis decreases the operative mortality in patients at high risk, and seems to preserve right ventricular function.     

Ten years' experience in aortic valve replacement with homografts in 389 cases

BACKGROUND AND AIM OF THE STUDY: Aortic valve replacement using homografts is an accepted alternative to the use of other replacement devices, and has been established at the authors' institution for more than 10 years. METHODS: Since 1992, a total of 389 homografts was implanted, and 332 patients (mean age 54 years, 72% males) were followed up. The initial patients (n = 75) had subcoronary implantation, all subsequent patients had root replacement. Both aortic grafts (AG) and pulmonary grafts (PG) were used. Follow up was conducted with regard to the factors 'graft origin', 'implantation technique' and 'gender', and included clinical examination, ECG and transthoracic echocardiography on an annual basis. RESULTS: Overall 30-day mortality was 5.4% (AG patients 3.9%, PG patients 13.5%; p = 0.09). Among late deaths (n = 22), six were valve-related (all prosthetic infection). Four minor thrombembolic events were recorded due to amaurosis fugax and transient ischemic attacks (TIA). Freedom from reoperation was 86.5%. Indication for graft replacement was greater after subcoronary implantation than after root implantation (p = 0.04). Reoperation was necessary in 24 patients due to restenosis (n = 4), regurgitation grade >II (n = 5), paravalvular leak (n = 2) and prosthetic infection (n = 13). At the latest echocardiographic follow up, mean peak pressure gradient was 15.60 +/- 11.76 mmHg, homograft regurgitation grade was 0.82 +/- 0.66, left ventricular end-diastolic diameter (EDD) was 49.1 +/- 7.54 mm, and mean aortic root diameter was 30.54 +/- 5.48 mm. When comparing parameters at a mean of five years postoperatively, the pressure gradient increased from 10.26 to 15.02 mmHg, regurgitation grade increased from 0.53 to 0.81, and EDD decreased from 52.3 to 50.4 mm. Other variables showed no significant differences. CONCLUSION: The present results confirmed good midterm-results for aortic valve replacement with homografts. These prostheses are vulnerable to infection, and root replacement was superior to the subcoronary implantation technique.     

Effect of LDL apheresis on oxygen tension in skeletal muscle in patients with cardiac allograft vasculopathy and severe lipid disorder

In this study, the extent to which intramuscular pO2 is influenced by a single HELP-apheresis (Heparin-induced Extracorporeal LDL Precipitation) was investigated in 10 patients with cardiac allograft vasculopathy (CAV) and severe lipid disorder. For this purpose, a sterile flexible pO2 microcatheter was inserted into the anterior tibial muscle and pO2 monitoring was begun 10 minutes before starting apheresis treatment. The intramuscular pO2 values were recorded continuously until the end of apheresis treatment and a subsequent 30-minute further observation phase. The patients with CAV and severe lipid disorder presented with 11.6+/-3.8 mmHg significantly and pathologically reduced intramuscular pO2 (p<0.001). LDL apheresis resulted in a significant increase in pO2 in the anterior tibial muscle. Thirty minutes after the end of HELP-apheresis, intramuscular partial oxygen pressure had increased by 162% and showed values at this point, 30.3+/-9.8 mmHg, similar to those found in healthy subjects.     

Prenatal management of congenital diaphragmatic hernia

Recently, two randomized controlled, prospective trials, the Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trials, reported the outcomes on fetal endoluminal tracheal occlusion (FETO) for isolated left congenital diaphragmatic hernia (CDH). FETO significantly improved outcomes for severe hypoplasia. The effect in moderate cases, where the balloon was inserted later in pregnancy, did not reach significance. In a pooled analysis investigating the effect of the heterogeneity of the treatment effect by the time point of occlusion and severity, the difference may be explained by a difference in the duration of occlusion. Nevertheless, FETO carries a significant risk of preterm birth. The primary objective of this review is to provide an overview of the rationale for fetal intervention in CDH and the results of the randomized trials. The secondary objective is to discuss the technical aspects of FETO. Finally, recent developments of potential alternative fetal approaches will be highlighted.     

不同缺血时间再灌注损伤对大鼠骨骼肌的影响

目的探讨不同缺血时间再灌注损伤对大鼠骨骼肌的影响。方法选取35只雄性Wistar大鼠,采用单侧夹闭股动脉和压力绷带施压的方法构建下肢骨骼肌缺血再灌注损伤(IRI)模型。根据不同缺血时间分为2 h缺血24 h再灌注(I2R24组)、2.5 h缺血24 h再灌注(I2.5R24组)、3 h缺血24 h再灌注(I3R24组)、4 h缺血24 h再灌注(I4R24组)、假手术组,每组7只。在再灌注终点,收集腓肠肌组织和血浆进行分析。采用湿重/干重比值(W/D)评估组织水肿情况;3-(4,5-二甲基噻唑-2)-2,5二苯基四氮唑溴盐(MTT)检测组织活力;HE染色观察组织病理学变化;免疫荧光染色检测补体C1q和C3b/c沉积、凝血组织因子(TF)表达和纤维蛋白原(FN)沉积、缓激肽受体1(BR1)和BR2表达、内皮血管细胞黏附分子-1(VCAM-1)和E选择素表达、炎症纤维介素蛋白-2(FGL-2)和髓过氧化物酶(MPO)表达;ELISA法检测血浆干扰素-γ(IFN-γ)、白细胞介素-7(IL-7)、IL-18、巨噬细胞炎症蛋白-1α(MIP-1α)、单核细胞趋化蛋白-1(MCP-1)水平。结果延长缺血时间再灌注,组织水肿逐渐加重,I2R24组、I2.5R24组、I3R24组、I4R24组W/D分别为5.3±0.2、6.1±0.3、6.9±0.2、7.6±0.3,高于假手术组的4.5±0.1(P均<0.01)。组织活力逐渐降低,I2R24组、I2.5R24组、I3R24组、I4R24组分别为(62.4±3.5)%、(45.3±3.3)%、(35.4±3.4)%、(27.1±5.9)%,低于假手术组的(93.8±7.2)%(P均<0.01)。病理组织损伤逐渐加重,最重为I4R24组,有严重肌细胞损伤、间质水肿和大量炎性细胞浸润,余依次为I3R24组、I2.5R24组、I2R24组,假手术组肌细胞结构完整、排列整齐。免疫荧光染色提示C1q、C3b/c、FN、BR1、VCAM-1、E选择素、FGL-2水平逐渐升高,由低到高依次为假手术组、I2R24组、I2.5R24组、I3R24组、I4R24组。MPO阳性细胞数/高倍镜(×200)细胞总数的大体比例逐渐升高,从高到低依次为I4R24组、I3R24组、I2.5R24组、I2R24组、假手术组。而TF和BR2表达在各组间无明显改变。血浆IFN-γ、IL-7、IL-18、MIP-1α、MCP-1浓度随缺血时间延长均逐渐升高(P均<0.01),从低到高依次为假手术组、I2R24组、I2.5R24组、I3R24组、I4R24组(P均<0.01)。结论延长缺血时间再灌注增加补体、凝血、激肽、内皮细胞激活及炎症因子释放,从而加重大鼠骨骼肌组织损伤。     

Ultrastructural changes of coronary artery endothelium induced by cardioplegic solutions

Coronary artery endothelial and myocardial ultrastructure was studied in guinea-pig heart-lung preparations (HLP) subjected to ischemic cardiac arrest induced by three hypothermic solutions. Two of the solutions used had high potassium chloride concentration ("Alabama" and "St. Thomas") while the third, instead, was a bicarbonate buffer (Kreb's solution). Five experimental groups were studied. In group 1 (control) the HLP were not subjected to cardiac arrest. Groups 2, 3, and 4 were subjected to a period of cardiac arrest of 30, 60, and 120 minutes respectively. In group 5, HLP were reperfused with blood for 30 minutes after 60 minutes of cardiac arrest. A thin ring of the left anterior descending coronary artery and myocardial fragments were obtained at the end of each experiment and were analyzed by means of transmission electron microscopy (TEM). Functional parameters were recorded in group 5. HLP perfused with Alabama solution showed a well-preserved endothelium and myocardium. HLP perfused with Krebs solution showed slight changes of the endothelial glycocalix only in group 4. Further, HLP perfused with Krebs solution showed extensive myocardial lesions (groups 3 and 4). These ischemic changes were not completely reversed after reperfusion (group 5). HLP perfused with St. Thomas solution showed only endothelial changes. These lesions were mainly characterized by: disappearance of the glycocalix and pynocytotic vesicles, endothelial cell bulging (group 2), and loss of the endothelial continuity (groups 3, 4, and 5). Hemodynamic parameters were significantly changed only in the Krebs-perfused HLP which showed a deterioration of the cardiac function related to the ischemic damage.(ABSTRACT TRUNCATED AT 250 WORDS)     

Superiority of mitral valve replacement with preservation of subvalvular structure to conventional replacement in severe rheumatic mitral valve disease: a modified technique and results of one-year follow up

BACKGROUND AND AIM OF THE STUDY: Mitral valve replacement with preservation of the subvalvular apparatus (MRVP) has been proven superior to conventional mitral valve replacement (MVR). We devised a simple modified MVRP method in this prospective, randomized study to investigate the clinical effects and one-year follow up echocardiographic results of MVRP compared with MVR in patients with severe rheumatic mitral insufficiency (MI). METHODS: Sixty-eight patients with severe rheumatic MI with or without stenosis were randomized to MVRP (n = 35) and MVR (n = 33) groups. In MVRP patients, the preserved tissue was pulled back posteriorly to the posterior wall of the left ventricle, then plicated and reaffixed to one-fourth of the annular circumference in the posterior annulus, in order to prevent left ventricular outflow tract (LVOT) obstruction. Clinical data including cumulative ventricular arrhythmias and use of inotropes were collected. Echocardiography examination was performed before surgery, and at five days, three months and one year thereafter. RESULTS: There were no preoperative differences patient data. The cross-clamp time was 2.2 min longer in MVRP patients. The one-month mortality rate after surgery was lower in MVRP patients (2.9% versus 15.2%, p = 0.074). Mechanical ventilation and ICU times were shorter in the MVRP group (17.6 versus 24.8 and 52.5 versus 70.6 h, p = 0.001 and 0.1, respectively). There were fewer ventricular arrhythmias and less need for inotropic support in this group. One year follow up echocardiography data showed better preserved left ventricular ejection fraction (LVEF) and better recovery of heart size after MRVP. There was no indication that preserved valvular tissue interfered with mechanical valve function, or caused LVOT obstruction. CONCLUSION: This modified MVRP technique is simple, effective and without risk of LVOT obstruction. In severe rheumatic MI patients the outcome of MVRP is superior to that of conventional MVR in term's of mortality, postoperative care needs, left ventricular function and heart dimensions.     

Jamming of prosthetic heart valves by suture trapping: experimental findings

The vulnerability of the Medtronic-Hall, Bj?rk-Shiley Monostrut, Duromedics, and St. Jude Medical valves to occluder immobilization by sutures was determined under static and pulsatile flow conditions. Variables were cardiac output, cross-sectional diameter of suture, type of suture (braided versus monofilament) and position of the offending suture along the circumference of the valve ring. Under static conditions, pressures, ranging from 40 to 340 mmHg and 10 to 170 mmHg, were required to decompress obstructed Medtronic-Hall and Bj?rk-Shiley Monostrut valves, respectively. As a result of different design characteristics and different occluder/orifice clearances the Medtronic-Hall valve showed its maximum opening pressure in case of interference with sutures at the axis of symmetry in both minor and major orifices, whereas for the Bj?rk-Shiley Monostrut valve this was reached in the minor orifice. Under pulsatile flow conditions, in case of interference with Prolene 2-0 suture, the Duromedics valve showed irregularly delayed opening and an opening pressure difference of 50 mmHg at a cardiac output of 8 L/min, whereas leaflet motion and pressure difference in the St. Jude Medical valve were undisturbed under similar conditions. The necessary pressure difference for opening the Medtronic Hall valve reached 44mmHg at a cardiac output of 8 L/min. High and low risk of extrinsic leaflet obstruction in the Duromedics and St. Jude Medical valves, respectively, is related to the design of the hinge mechanisms and the wedge angle of their leaflets (2 degrees versus 25 degrees). Precautionary principles in implantation of prosthetic heart valves are stressed to prevent the potentially lethal complication of occluder immobilization.(ABSTRACT TRUNCATED AT 250 WORDS)