Hemostasis assessment in Fontan patients using the new thrombodynamics test.

BACKGROUND: Thromboembolic events are a well-known risk for Fontan patients and often lead to morbidity and mortality in cyanotic patients and patients with a single ventricle. Coagulopathy and thrombophilia, in addition to disturbed Fontan blood flow and endothelial injury, are major contributors to thromboembolic complications. However, there is currently no consensus regarding the optimal medication to prevent or treat these events. Identification of coagulation disorders is therefore crucial for selecting appropriate management strategies and evaluating long-term outcomes in these patients. CASE PRESENTATION: We present the case of a 35-year-old male who underwent the Fontan procedure with a total cavopulmonary modification during childhood due to tricuspid atresia. He was admitted with complaints of headaches and visual disturbances, but no clear cardiovascular cause was identified. Standard coagulation test parameters were normal, but the thrombodynamics test indicated severe hypercoagulation and spontaneous clot formation. Anticoagulation therapy was initiated, and a subsequent thrombodynamics assay showed normalization of the coagulation parameters. The patient remained asymptomatic during the six-month follow-up period. CONCLUSIONS: The thrombodynamics test is a valuable tool for the diagnosis of coagulation disorders, as it can assess coagulation parameters and clot growth in vitro. This method can also aid in the optimization of antithrombotic therapy. The presented clinical case highlights the potential use of the thrombodynamics test in Fontan patients to diagnose coagulation disorders and improve long-term outcomes.

Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis†.

OBJECTIVES: This study aimed to investigate the indications for and the results of axillary arterio-venous fistula in patients with a functionally single ventricle at mid-term follow-up after bidirectional cavopulmonary connection. METHODS: From 2012 to 2017, 20 patients aged 21.1 ± 8.7 years (median 20.5) underwent axillary arterio-venous fistula after bidirectional cavopulmonary connection in our institution. The interval between bidirectional cavopulmonary connection and axillary arterio-venous fistula was 13.9 ± 7.2 years. The clinical and haemodynamic parameters of the patients were assessed before and after surgery. RESULTS: There was no in-hospital mortality. Paraesthesia of the arm persisted for 1-7 days. The mean arterial oxygen saturation increased from 72.4 ± 7.1% pre-surgery to 81.6 ± 5.5% (P < 0.001) after surgery, and was 83.8 ± 6.5% at discharge (P = 0.002 Wilcoxon matched pairs test). The haemoglobin, haematocrit, McGoon index and ejection fraction values did not change significantly. The speed and minute volume of the blood flow in the superior vena cava increased from from 21.1 ± 8.3 cm/s to 28.8 ± 14.9 cm/s (P < 0.001) and from 10.6 ± 3.7 ml/s to 12.5 ± 2.9 ml/s (P < 0.001), respectively. At the mid-term follow-up (1-5 years), 2 patients died from concomitant pathology. Stable improvement in the state and tolerance of physical activity was noted: before surgery, 17 patients were classified as New York Heart Association functional capacity (FC) III and 3 patients as FC IV; after surgery, 9 patients were FC II and 3 patients were FC III. One patient underwent a Fontan operation within 3 years. CONCLUSIONS: Axillary arterio-venous fistula can be used as the stage of surgical treatment and a bridge to Fontan and/or heart transplantation for this category of 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.

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.

Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults.

The purpose of this study is to analyze the immediate results of bidirectional cavopulmonary anastomosis (BCPA) and Fontan operations performed in adults, and to reveal the risk factors. During the years 1983-2010, 681 consecutive patients underwent BCPA or a Fontan operation. Fifty-three of 681 patients were more than 18 years of age. Twenty-nine adults underwent BCPA and 24 underwent a Fontan operation. Immediate results of surgical treatment were followed during the hospital period. The average number of exceeded 'operability' criteria by Choussat et al. [Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchhill Livingstone, 1977:559-566] was significantly higher in patients from the BCPA group compared to the Fontan group (1.3±0.8 vs. 0.9±0.7, P=0.034). Hospital mortality after BCPA in adults was 6.9% (2/29) and did not differ from children (7.1%, 19/268), P=0.634. Hospital mortality after Fontan operation in adults was 8.3% (2/24) and did not differ from children (11.9%, 43/360), P=0.419. The frequency of non-lethal hospital complications was higher in patients after a Fontan operation. Patients from the Fontan group more frequently developed arrhythmias and prolonged pleural effusions. Preoperative regurgitation at atrioventricular valves was an independent risk factor for hospital mortality and morbidity after a Fontan operation. BCPA and Fontan operations performed in adults are accompanied by good immediate results and considerably improves patients' condition.