This study shows a significantly lower incidence of TE after Fontan procedure if either aspirin or warfarin is used. This meta-analysis suggests no significant difference in incidence of early or late TE in patients receiving aspirin compared with warfarin.

Fontan patients managed with warfarin are at risk not only for thrombotic events, but also for bleeding episodes as a consequence of anticoagulation treatment. The aim of this study was to determine whether time spent in patient specified therapeutic range (TTR), when managed in a cardiology-based pharmacist managed anticoagulation clinic (PMAC), is a useful target metric for monitoring, as well as improving outcomes. A single center retrospective review was conducted evaluating TTR of all Fontan patients (n = 45) on warfarin managed in our outpatient cardiology pharmacist managed anticoagulation clinic (PMAC) during a 19 month time frame. The primary outcome was time spent within, above, and below therapeutic range. Secondary outcomes were thrombotic event (TE) incidence pre- and post PMAC enrollment and bleeding event incidence during PMAC management. Of the Fontan patients included, 55.6% were male and the median age at latest anticoagulation clinic follow-up was 19 years (IQR 13, 29). A composite 52.9 patient years of warfarin therapy was evaluated during the study time frame. The mean TTR for patients was 84.1 ± 5.2%. The most frequent reasons for non-therapeutic INRs were diet changes (42.8%), medication non-compliance (13.7%), and drug interactions (8.8%). Only one TE occurred during the study time frame. The incidence of TE in this population was decreased after PMAC enrollment (1 per 52.9 patient year versus 1 event per 17.4 patient year; p < 0.0002). Two major bleeds that required emergency department visit occurred during this time, none were cerebral or gastrointestinal. In Fontan patients anticoagulated with warfarin, a greater than 80% TTR can be achieved in a PMAC. Such high time in therapeutic range was associated with excellent outcomes, despite the obvious complexity of this population.

Despite the common occurrence of thrombosis in Fontan circulations, the mid-term thrombotic risk beyond the first two postoperative years is poorly defined especially in total cavo pulmonary Fontan. This study examines the thrombotic incidence and risk beyond the first 2 years after contemporary Fontan surgery. Using a retrospective cohort study design, 89 Fontan patients, 50 male, were included and evaluated with a median of 8.3 years (IQR 6.8-11.4) follow-up. Hospital records were reviewed for known risk factors of thrombosis, thrombotic events, antiplatelet and anticoagulation management, and basic characteristics. Forty seven patients (52%) had a dominant left ventricle, and 28 (32%) had hypoplastic left heart syndrome. Eight patients had thrombotic events post Fontan surgery at a median age of 9 years (IQR 5.6-13), 5.7 (IQR 2.0-9.7) years following surgery, not including events that occurred immediately peri-operatively. Four thrombotic events were intracardiac whereas the remainder were extra-cardiac. There was no significant univariate correlation between thrombosis and the presence of ventricular morphology, pulmonary arterial reconstruction, or type of cavopulmonary anastomosis (lateral tunnel vs. extracardiac conduit). Thrombosis continues to be an important intermediate-term risk even for patients with contemporary Fontan circulations. These results strongly suggest that thrombophilic risk is not dictated purely by vascular pathway and hemodynamic variables. Further investigation into the pathophysiology, individualized risk, and effectiveness of anticoagulation strategies are required in this high risk population.

Triple therapy with cilostazol has been shown to reduce MACEs by providing increased inhibition of platelet aggregation and reducing the rates of in-stent thrombosis compared to DAT without increasing the risk of bleeding complications. Further studies are needed to identify proper patient selection based on risk factors for the addition of cilostazol. Additionally, studies comparing cilostazol with newer antiplatelet therapies, such as prasugrel and ticagrelor, are needed.

Selexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial hypertension in WHO Group 1 adult patients. Compared to parenteral prostacyclin formulations, selexipag offers a significant improvement in patient’s and caregiver’s quality of life because of its oral formulation, frequency of administration, and mechanism of action. Although experience in the pediatric population is limited to case reports in older adolescent patients and selexipag is not approved for use in the pediatric pulmonary hypertension population, many pediatric centers are expanding the use of this therapy to this population. We report our institution's experience in the use of selexipag to treat pulmonary hypertension in children under 10 years of age, between 10 and 30 kg. Seven patients were initiated on selexipag therapy including de novo initiation and transition from intravenous treprostinil to oral selexipag. All patients were on stable background therapy with phosphodiesterase-5 inhibitor and endothelin receptor antagonist therapies at baseline. All patients reached their planned goal selexipag dose during admission without the need for changes to the titration schedule and without hemodynamic deterioration. In our experience, oral selexipag is safe and well-tolerated in young pediatric patients with pulmonary hypertension. Based on our favorable experience, we developed an institution-specific selexipag process algorithm for continued successful use in the pediatric population.

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blood components units within two 6-hour periods, before and after CFCs administration (Table 1). Results: Eighty-three patients (median 58 years [IQR 45, 65] years, 59 % male, SOFA 7 [4,10]) were included. Thirty-six (43.4 %) underwent cardiac surgery, 31 (37.3 %) liver transplantation and 16 (19.3 %) other surgeries. Fifty-seven (68.7%) received Riastap® (2 g. [2, 4]), nine (10.8%) Prothromplex® (1,200 U. [600, 1800] and 17 (20.5%) both CFCs. Crude mortality was 50.6% (42 patients) with 62 % out of all deaths being considered as due to bleeding. Table 1. Laboratory values and blood transfusion within 6 hour before [1] and 6 hour after [2] of CFCs administration INR [1]=2.0 (1.2 -2.8); [2]=1.6 (1.3 -2.2) Fibrinogen (g/L) [1]=1.1 (0.8 -2.3);[2]=1.8 (1.2 -2.4) Hemoglobin (g/L) [1]= 83 (65, 99);[2]= 89 (75, 100) Platelets (x 109) [1]=85 (44, 164);[2]=81 (58, 113) RBC (units) [1]= 4 (3, 7);[2]=2 ( 1, 4); overall= 9 (6, 15) FFP (units) [1]= 2 (0, 3); [2]= 0 (0, 2); overall= 3 (1, 5) Platelets (pool) [1]= 1 (0, 2);[2]= 0 (0, 1); overall= 2 (1, 3) Bleeding (mL) [1]= 1200 (700, 2000);[2]= 500 (240, 1250); overall=1900 (500, 2700) All differences (6h. before vs. after, were significative); RBC: red blood cells; FFP: fresh frozen plasma Conclusions: In bleeding, over transfused patients with coagulopathy, CFCs administrations were associated with a decrease of transfusion requirements, although mortality rates were unacceptably high. Further investigation to ascertain whether an earlier, specific and sufficient (ROTEM-guided) administration of CFCs might improve patient outcome is warranted