Sergey B. Zaets

Recombinant Factor XIII Mitigates Hemorrhagic Shock-Induced Organ Dysfunction

BACKGROUND Plasma factor XIII (FXIII) is responsible for stabilization of fibrin clot at the final stage of blood coagulation. Since FXIII has also been shown to modulate inflammation, endothelial permeability, as well as diminish multiple organ dysfunction (MOD) after gut ischemia-reperfusion injury, we hypothesized that FXIII would reduce MOD caused by trauma-hemorrhagic shock (THS). MATERIALS AND METHODS Rats were subjected to a 90 min THS or trauma sham shock (TSS) and treated with either recombinant human FXIII A(2) subunit (rFXIII) or placebo immediately after resuscitation with shed blood or at the end of the TSS period. Lung permeability, lung and gut myeloperoxidase (MPO) activity, gut histology, neutrophil respiratory burst, microvascular blood flow in the liver and muscles, and cytokine levels were measured 3 h after the THS or TSS. FXIII levels were measured before THS or TSS and after the 3-h post-shock period. RESULTS THS-induced lung permeability as well as lung and gut MPO activity was significantly lower in rFXIII-treated than in placebo-treated animals. Similarly, rFXIII-treated rats had lower neutrophil respiratory burst activity and less ileal mucosal injury. rFXIII-treated rats also had a higher liver microvascular blood flow compared with the placebo group. Cytokine response was more favorable in rFXIII-treated animals. Trauma-hemorrhagic shock did not cause a drop in FXIII activity during the study period. CONCLUSIONS Administration of rFXIII diminishes THS-induced MOD in rats, presumably by preservation of the gut barrier function, limitation of polymorphonuclear leukocyte (PMN) activation, and modulation of the cytokine response.

Comparative assessment of Fontan operation in modifications of atriopulmonary and total cavopulmonary anastomoses

OBJECTIVES The optimal technique of the Fontan operation remains disputable. This investigation was aimed at the comparison of atriopulmonary and total cavopulmonary anastomoses. METHODS The results of 81 operations of total cavopulmonary and 69 operations of atriopulmonary anastomosis, performed from 1983 to 1995 were analysed. A control study was carried out 1-10 (3.7 + 0.2) years after the operation in 80 patients (36 after total cavopulmonary and 44 after atriopulmonary anastomoses). 70 patients were studied several times. Cardiac catheterization was done in 70 patients. In 78 patients central hemodynamical indices were studied during exercise. RESULTS Preoperative hemodynamical indices were not reliably different in the two groups. Early mortality after total cavopulmonary anastomosis was 15%, after atriopulmonary anastomosis 23%. The highest mortality was seen when the criteria of Choussat et al. (Choussat et al. Pediatric Cardiology. Edinburgh: 1977:559-566) were surpassed. In the early postoperative period after total cavopulmonary anastomosis circulatory insufficiency was less marked, transsudation from pleural cavities was reliably lower (15.3 + 1.2 versus 25.5 + 1.8 ml/kg/day, P < 0.01). Arrhythmias were more common after total cavopulmonary anastomosis (18.5 versus 12%). Late mortality after total cavopulmonary anastomosis was 6%, after atriopulmonary anastomosis 12%. 82% of patients after atriopulmonary anastomosis and 81% after total cavopulmonary anastomosis were in NYHA classes 1 and 2.7% of patients after total cavopulmonary anastomosis and 11% after atriopulmonary anastomosis were reoperated. Physical tolerance rose stepwise in both groups and by the third post-operative year reached 75% of normal level. At this time we saw the most optimal hemodynamical indices during exercise. However, the best hemodynamics during exercise were seen after atriopulmonary anastomosis. CONCLUSIONS Total cavopulmonary anastomosis is accompanied by lower mortality rate and a more favourable course of early postoperative period. However, the best long-term functional results are obtained after atriopulmonary anastomosis.

Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery

OBJECTIVES Experience with the palliative treatment of congenital heart defects (CHDs) associated with unilateral absence of the pulmonary artery (UAPA) is limited. There is a description of 32 interventions in the available literature. The aim of this retrospective study was to present our experience with palliative interventions in patients with cyanotic CHDs associated with UAPA and to suggest a rational surgical strategy. METHODS Twenty-nine patients were subjected to palliative interventions. All of them had the following cyanotic CHDs with agenesis of the left pulmonary artery: tetralogy of Fallot (n = 26) or double outlet right ventricle (n = 3). Twenty patients were subjected to a single and 9 to multiple staged palliative operations. Patients were grouped according to the type of initial palliation to assess the clinical and haemodynamic results of each surgical intervention: Group 1: systemic-to-pulmonary shunts (n = 13); Group 2: transluminal balloon pulmonary valvuloplasty (n = 5); Group 3: palliative reconstruction of the right ventricular outflow tract (n = 11). The median age of patients at the initial palliative intervention was 2.6 years. Twenty-three of 27 discharged patients were followed up for a median period of 3 years. RESULTS Hospital mortality in our series reached 7% (2 of 29 patients). Both lethal outcomes occurred after palliative reconstruction of the right ventricular outflow tract was performed as a sole intervention. The assessment of angiographic parameters has shown that palliative reconstruction of the right ventricular outflow tract provided more significant and uniform enlargement of the pulmonary artery than systemic-to-pulmonary shunts or transluminal balloon pulmonary valvuloplasty. Fifty-nine percent of patients (17 of 29 patients) were subjected to complete repair of CHDs during the follow-up. CONCLUSIONS Palliative surgical treatment of CHDs associated with UAPA can be performed with a relatively low risk. Systemic-to-pulmonary artery shunt and transluminal balloon pulmonary valvuloplasty are methods of choice in patients with non-severe hypoplasia of the single pulmonary artery. The intravascular intervention is indicated more in patients with a prevailing valvular component of the pulmonary stenosis. Palliative reconstruction of the right ventricular outflow tract is a more favourable procedure for patients with a severe hypoplasia of the single pulmonary artery.

Results of Fontan operation in patients with atrioventricular valve regurgitation.

OBJECTIVES The influence of concomitant atrioventricular valve regurgitation on the results of Fontan operation remains disputable. The goal of this study was to compare early and late results of Fontan operation performed in patients with mild or moderate-to-severe atrioventricular valve regurgitation. METHODS For retrospective analysis, patients with atrioventricular valve insufficiency assessed by echocardiography were divided into three groups based on the severity of regurgitation and its repair or non-repair during Fontan operation: Group 1: unrepaired regurgitation 1-2+ (n = 33); Group 2: unrepaired regurgitation 3-4+ (n = 11); Group 3: repaired regurgitation 3-4+ (n = 35). Actuarial survival was estimated by the Kaplan-Meier method, followed by the log-rank test to compare survival curves between groups. RESULTS Hospital mortality rates in Groups 1 through 3 reached 3, 27 and 14%, respectively (P = 0.015, Group 1 vs Group 2). The frequency of non-lethal complications did not significantly differ between groups. However, patients from Group 2 required significantly more intensive inotropic support, longer mechanical ventilation and had larger pleural effusion. Predicted survival after Fontan operation was the worst in Group 2 (P = 0.016, Group 2 vs Group 1). The frequency of non-lethal late complications was also the highest in Group 2 (50 vs 17 or 11%); however, the difference did not reach statistical significance (P = 0.13 and 0.069, respectively). The severity of atrioventricular valve regurgitation during the follow-up did not significantly change when compared with discharge after the repair in Group 2 or Group 3 (P = 0.19 and 0.52, respectively), and significantly increased in Group 1 (P = 0.003). However, this increase did not have clinical significance during the reported period of follow-up. CONCLUSIONS If unrepaired, concomitant moderate-to-severe atrioventricular valve regurgitation significantly worsens the results of the Fontan procedure. Longer observation is needed to define the strategy in patients with mild atrioventricular valve regurgitation that remained unrepaired during Fontan operation.

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.

Poor Left Ventricular Performance in a Child With Coronary Sinus Stenosis: Successful Surgical Repair.

Atresia or stenosis of the coronary sinus is a rare congenital defect that can cause marked clinical manifestations, depending on the presence of concomitant anomalies securing adequate coronary venous outflow. We describe a 4.5-year-old boy admitted to the clinic with signs of congestive heart failure (left ventricular ejection fraction, 29%). His condition had deteriorated during the previous 4 months. The examination revealed stenosis of the coronary sinus ostium, coronary sinus-to-left atrium fistula, mitral regurgitation of the second grade, and moderate pulmonary hypertension. The surgical intervention consisted of enlargement and plastic reconstruction of the coronary sinus as well as closure of the coronary sinus-to-left atrium fistula. The hospital period was uneventful. The left ventricular ejection fraction at discharge increased up to 50%.

Quantitative Anatomy of Taussig-Bing Anomaly

Background: Taussig-Bing is one of the variants of the double outlet right ventricle. The characteristic feature of the Taussig-Bing anomaly (TBA) that makes it different from partial transposition of the great arteries is sub pulmonary ventricular septal defect. Morphometric studies of hearts with TBA are limited and in the majority of cases do not contain quantitative assessment of intracardiac structures. The aim of this study was to measure intracardiac structures (e.g. cardiac mass, diameter of valves, thickness of the myocardium), to assess their deviation from the individual norm, as well to determine how these parameters are influenced by anatomical variant of the lesion, anthropometric variability or pulmonary hypertension. Methods: Thirty pediatric hearts with TBA underwent morphometric assessment. The median age at the moment of death was 0.4 years. All patients had pulmonary hypertension of grade I-II (n=24) or grade III-IV (n=6) per Heath- Edwards. Morphometric assessment included the measurement of the following parameters: cardiac mass, diameter of the fibrous annulus of all cardiac valves (mitral, tricuspid, aortic, and pulmonary), myocardial thickness of the both right and left ventricle. Results of valvular morphometry were compared with individual normative data presented by Schulz DM and Giordano DA, and Z-score index was calculated. Results: Cardiac mass, right ventricular myocardial thickness and the diameter of pulmonary valve significantly exceeded normal values in TBA hearts. Cardiac mass median Z-score in TBA hearts with pulmonary hypertension of III-IV grade was significantly higher if compared to pulmonary hypertension of I-II grade. Diameters of tricuspid and pulmonary valve were significantly bigger in TBA hearts without aortic obstruction. Conclusions: There is an increase in cardiac mass, right ventricular myocardial thickness as well as pulmonary valve diameter in TBA hearts if compared to age adjusted normal values. The degree of pulmonary hypertension may contribute to the increase in cardiac mass.

Repair of congenital heart defects associated with single pulmonary artery.

Background Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. Methods Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n = 25) and ventricular septal defect (n = 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. Results Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm2·m−2). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 ± 0.11. Conclusions Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects.

Potts shunt in children with pulmonary arterial hypertension: institutional experience

OBJECTIVES Since 2004, different techniques for the Potts shunt have been used in patients with incurable pulmonary arterial hypertension. However, the experience with these interventions in a single institution is limited. We present our series of Potts shunts in children with pulmonary arterial hypertension. METHODS Eight patients with suprasystemic pulmonary arterial hypertension (pulmonary artery-to-aorta mean pressure ratio of 1.39 ± 0.30) had a Potts shunt at the median age of 13.5 months. Six patients were followed up for 2-32 (median 17) months after the operation. RESULTS In all cases, the Potts shunt caused a decrease in arterial blood oxygen saturation at the lower extremities and was the genesis of the arterial blood oxygen saturation gradient between the upper and lower extremities (10.5 ± 1.8% at discharge). Postoperative echocardiography revealed flattening of the ventricular septum as well as a decrease in the right ventricle-to-left ventricle end-diastolic diameter ratio from 1.36 ± 0.14 preoperatively to 0.99 ± 0.22 at discharge (P = 0.010). The 2 patients with the highest preoperative pulmonary artery-to-aorta mean pressure ratio (1.79-1.86) died during the early postoperative period from repeated pulmonary hypertension crises and uncontrolled hypoxaemia. There were no deaths during the follow-up period. The paediatric functional class of the patients improved from IIIb-IV to I-II, syncopal episodes no longer persisted and the weight deficit decreased from 21.8 ± 7.5% preoperatively to 11.5 ± 5.9% at the latest follow-up examination (P = 0.004). The arterial blood oxygen saturation gradient between the upper and lower extremities and the right ventricle-to-left ventricle end-diastolic diameter ratio were the same as they were while the patients were in the hospital. CONCLUSIONS Our limited experience demonstrates that the Potts shunt lowers suprasystemic pulmonary hypertension to the systemic level, limits manifestations of right-sided heart failure and improves the overall functional status of the patients.

Repair of isolated partial anomalous pulmonary venous connection of the right upper pulmonary veins to the left vertical vein

The most common form of partial anomalous pulmonary venous connection is drainage of the right pulmonary veins to the superior vena cava. On the contrary, partial anomalous venous connection of right pulmonary veins to the left vertical vein is described in few reports. The goal of this publication is to present the case of diagnosis and surgical repair of this extremely rare pathology in a 19-year old female. Methods of preoperative examination included but were not limited to spiral computed tomography and angiocardiography. Intraoperative examination confirmed that the collector of right upper pulmonary veins was connected to the vertical vein that continued to the innominate vein and then to the superior vena cava draining into the right atrium. During surgical intervention, the vertical vein was transected in the area of connection to the innominate vein with the suturing of inlet area. The collector of the right upper pulmonary veins was then anastomosed with the left atrial appendage. Postoperative course was uneventful. In conclusion, isolated partial anomalous pulmonary venous connection of the right upper pulmonary veins to the left vertical vein is a very rare congenital defect. Existing modern techniques contribute to the precise and timely diagnosis of this anomaly.