Percutaneous ultrasound-guided versus open cut-down access to femoral vessels for the placement of a REBOA catheter.

Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA) may be useful in treating exsanguinating trauma patients. This study seeks to compare rates of success, complications and time required for vascular access between ultrasound-guidance and surgical cut-down for femoral sheath insertion as a prospective observational case control study. Participating clinicians from either trauma surgery or anesthesiology were allocated to surgical cut-down or percutaneous ultrasound-guided puncture on a 1:1 ratio. Time spans to vessel identification, successful puncture, and balloon inflation were recorded. 80 study participants were recruited and allocated to 40 open cut-down approaches and 40 percutaneous ultrasound-guided approaches. REBOA catheter placement was successful in 18/40 cases (45%) using a percutaneous ultrasound guided technique and 33/40 times (83%) using the open cut-down approach (p < 0.001). Median times [in seconds] compared between percutaneous ultrasound-guided puncture and surgical cut-down were 36 (18-73) versus 117(56-213) for vessel visualization (p < 0.001), 136 (97-175) versus 183 (156-219) for vessel puncture (p < 0.001), and 375 (240-600) versus 288 (244-379) for balloon inflation (p = 0.08) overall. Access to femoral vessels for REBOA catheter placement is safer when performed by cut-down and direct visualization but can be performed faster by an ultrasound-guided technique when vessels can be identified clearly and rapidly.

Nomogram for predicting in-hospital mortality in trauma patients undergoing resuscitative endovascular balloon occlusion of the aorta: a retrospective multicenter study.

Recently, resuscitative endovascular balloon occlusion of the aorta (REBOA) had been introduced as an innovative procedure for severe hemorrhage in the abdomen or pelvis. We aimed to investigate risk factors associated with mortality after REBOA and construct a model for predicting mortality. This multicenter retrospective study collected data from 251 patients admitted at five regional trauma centers across South Korea from 2015 to 2022. The indications for REBOA included patients experiencing hypovolemic shock due to hemorrhage in the abdomen, pelvis, or lower extremities, and those who were non-responders (systolic blood pressure (SBP) < 90 mmHg) to initial fluid treatment. The primary and secondary outcomes were mortality due to exsanguination and overall mortality, respectively. After feature selection using the least absolute shrinkage and selection operator (LASSO) logistic regression model to minimize overfitting, a multivariate logistic regression (MLR) model and nomogram were constructed. In the MLR model using risk factors selected in the LASSO, five risk factors, including initial heart rate (adjusted odds ratio [aOR], 0.99; 95% confidence interval [CI], 0.98-1.00; p = 0.030), initial Glasgow coma scale (aOR, 0.86; 95% CI 0.80-0.93; p < 0.001), RBC transfusion within 4 h (unit, aOR, 1.12; 95% CI 1.07-1.17; p < 0.001), balloon occlusion type (reference: partial occlusion; total occlusion, aOR, 2.53; 95% CI 1.27-5.02; p = 0.008; partial + total occlusion, aOR, 2.04; 95% CI 0.71-5.86; p = 0.187), and post-REBOA systolic blood pressure (SBP) (aOR, 0.98; 95% CI 0.97-0.99; p < 0.001) were significantly associated with mortality due to exsanguination. The prediction model showed an area under curve, sensitivity, and specificity of 0.855, 73.2%, and 83.6%, respectively. Decision curve analysis showed that the predictive model had increased net benefits across a wide range of threshold probabilities. This study developed a novel intuitive nomogram for predicting mortality in patients undergoing REBOA. Our proposed model exhibited excellent performance and revealed that total occlusion was associated with poor outcomes, with post-REBOA SBP potentially being an effective surrogate measure.

Acquired bleeding disorders.

Acquired bleeding disorders can develop in previously healthy people irrespective of age or gender but are particularly common in patients with certain underlying conditions. Here, we review recent advances in the management of acquired haemophilia A (AHA), acquired von Willebrand syndrome (AVWS), and patients with hemostatic abnormalities due to chronic liver disease (CLD). Patients with AHA can now benefit from prophylaxis with emicizumab, a therapeutic antibody that mimics the function of activated coagulation factor VIII. The treatment of AVWS remains challenging in many situations and requires careful consideration of the underlying condition. Haemostatic abnormalities in CLD are often compensated by proportional reduction in pro and anti-haemostatic factors resulting in sustained or even increased thrombin generation. Consequently, bleeding in CLD is rarely caused by haemostatic failure and infusion of plasma or coagulation factor concentrates may not be effective.

Management of Nontraumatic Spontaneous Renal Hemorrhage (Wünderlich Syndrome) through Robotic-Assisted Laparoscopic Nephrectomy: A Case Series.

BACKGROUND Wünderlich syndrome (WS) is a rare diagnosis of nontraumatic spontaneous renal hemorrhage into the subcapsular, perirenal, or pararenal spaces. Prompt and effective intervention is necessary for an accurate pathological diagnosis and preservation of life. In the current literature, open surgery is the primary option when conservative treatment fails, but there can be serious trauma and corresponding consequences. Herein, we present 3 cases of Wünderlich syndrome managed by robot-assisted laparoscopic nephrectomy via a retroperitoneal approach. CASE REPORT Patient 1 was a 44-year-old woman with right flank pain for 6 h. Patient 2 was a 53-year-old woman with a history of diabetes who had pain in her right flank pain and nausea for 1 day. Patient 3 was a 45-year-old man with left flank pain for 1 day. All cases of WS were confirmed by CT. All 3 patients were treated with retroperitoneal robot-assisted nephrectomy after conservative treatment failed. Pathological examination confirmed that patient 1 had angiomyolipoma, and patients 2 and 3 had renal clear cell carcinoma. At the 9-month follow-up, renal function was good and no evidence of recurrence or metastasis has been detected. CONCLUSIONS These cases have highlighted the importance of the clinical history and imaging findings in the diagnosis of Wünderlich syndrome, and show that rapid management can be achieved using robot-assisted laparoscopic nephrectomy. However, it is crucial to have a skilled surgical team and adequate preoperative preparation.

Arterial embolization in the treatment of multiple renal and hepatic hamartomas with spontaneous hemorrhage and 2-year follow-up: a case report.

BACKGROUND: Hamartoma is a common benign tumor that usually occurs in the kidney, liver, lung, and pancreas. Large renal hamartomas may spontaneously rupture and hemorrhage, which is potentially life-threatening. CASE PRESENTATION: This report describes a 46-year-old Han Chinese female patient with multiple renal and hepatic hamartomas with rupture and hemorrhage of giant hamartoma in the left kidney. She underwent arterial embolization three times successively, and her condition was stable during the 2-year follow-up. This report includes a review of the relevant literature CONCLUSIONS: the findings in this report and previous literature suggest that arterial embolization can not only rapidly treat hamartoma hemorrhage in the acute phase but can also effectively control multiple lesions in the long term after repeated multisite arterial embolization.

Pressure-Controlled Intermittent Coronary Sinus Occlusion (PiCSO) in Acute Myocardial Infarction: The PiCSO-AMI-I Trial.

BACKGROUND: Primary percutaneous coronary intervention (pPCI) has improved clinical outcomes in patients with ST-segment-elevation myocardial infarction. However, as many as 50% of patients still have suboptimal myocardial reperfusion and experience extensive myocardial necrosis. The PiCSO-AMI-I trial (Pressure-Controlled Intermittent Coronary Sinus Occlusion-Acute Myocardial Infarction-I) evaluated whether PiCSO therapy can further reduce myocardial infarct size (IS) in patients undergoing pPCI. METHODS: Patients with anterior ST-segment-elevation myocardial infarction and Thrombolysis in Myocardial Infarction flow 0-1 were randomized at 16 European centers to PiCSO-assisted pPCI or conventional pPCI. The PiCSO Impulse Catheter (8Fr balloon-tipped catheter) was inserted via femoral venous access after antegrade flow restoration of the culprit vessel and before proceeding with stenting. The primary end point was the difference in IS (expressed as a percentage of left ventricular mass) at 5 days by cardiac magnetic resonance. Secondary end points were the extent of microvascular obstruction and intramyocardial hemorrhage at 5 days and IS at 6 months. RESULTS: Among 145 randomized patients, 72 received PiCSO-assisted pPCI and 73 conventional pPCI. No differences were observed in IS at 5 days (27.2%±12.4% versus 28.3%±11.45%; P=0.59) and 6 months (19.2%±10.1% versus 18.8%±7.7%; P=0.83), nor were differences between PiCSO-treated and control patients noted in terms of the occurrence of microvascular obstruction (67.2% versus 64.6%; P=0.85) or intramyocardial hemorrhage (55.7% versus 60%; P=0.72). The study was prematurely discontinued by the sponsor with no further clinical follow-up beyond 6 months. However, up to 6 months of PiCSO use appeared safe with no device-related adverse events. CONCLUSIONS: In this prematurely discontinued randomized trial, PiCSO therapy as an adjunct to pPCI did not reduce IS when compared with conventional pPCI in patients with anterior ST-segment-elevation myocardial infarction. PiCSO use was associated with increased procedural time and contrast but no increase in adverse events up to 6 months. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03625869.

X-ray-guided self-expandable metal stent (SEMS) implantation in oesophageal malignancy as an alternative treatment.

<b><br>Indroduction:</b> Significant dysphagia, aspiration pneumonia, and impossible oral nutrition in patients with unresectable or recurrent gastroesophageal malignancy or bronchial cancer invading the oesophagus with a tracheoesophageal fistula lead to cachexia. Dehiscence of the esophago-jejunal or gastroesophageal anastomosis may cause severe oesophageal haemorrhage. We believe that X-ray-guided oesophageal stent implantation (SEMS) is an alternative palliative method for microjejunostomy or full parenteral nutrition.</br> <b><br>Aim:</b> The aim of this paper was to assess the safety and efficacy of a novel X-ray-guided oesophageal stent implantation technique.</br> <b><br>Materials and methods:</b> This retrospective analysis included 54 patients (35 men and 19 women) treated for malignant dysphagia, gastroesophageal/gastrointestinal anastomotic fistula or bronchoesophageal fistula in two Surgical Units between 2010 and 2019, using a modified intravascular approach to oesophageal stent implantation.</br> <b><br>Results:</b> The presented modified intravascular method of oesophageal stent implantation was successfully performed in all described patients requiring oral nutrition restoration immediately following oesophageal stent implantation. Two patients with oesophageal anastomotic dehiscence died on postoperative days 7 and 9 due to circulatory and respiratory failure. One patient was reimplanted due to a recurrent fistula. Two patients with ruptured thoracic aneurysm and thoracic stent graft implantation due to oesophageal haemorrhage, who were implanted with an oesophageal stent, died on postoperative days 4 and 14.</br> <b><br>Conclusions:</b> The modified intravascular X-ray-guided SEMS technique may be a palliative treatment for patients with unresectable oesophageal malignancies.</br>.

Bleeding sites and treatment strategies for cardiac tamponade by catheter ablation requiring thoracotomy: risks of catheter ablation in patients with left atrial diverticulum.

BACKGROUND: There is insufficient information regarding the bleeding sites and surgical strategies of cardiac tamponade during catheter ablation for atrial fibrillation (AF). CASE PRESENTATION: Of the five patients with cardiac tamponade, three required surgical intervention and two required pericardiocentesis. In the first case of three cardiac tamponades requiring surgical intervention, considering that the peripheral route was used, the catecholamines did not reach the heart, and due to unstable vital signs, venoarterial extracorporeal membrane oxygenation (VA-ECMO) was inserted. No bleeding point was identified, but a thrombus had spread around the left atrium (LA) with diverticulum. Hemostasis was achieved with adhesives placed around the LA under on-pump beating. In the second case, pericardiocentesis was performed, but the patient showed heavy bleeding and unstable vital signs. Thus, VA-ECMO was inserted. Heavy bleeding was expected, and safety was enhanced by attaching a reservoir to the VA-ECMO. The bleeding point was found between the left upper pulmonary artery and LA under cardiac arrest to obtain a good surgical view for suturing repair. In the third case, the LA diverticulum was damaged. Pericardiocentesis resulted in stable vitals, but sustained bleeding was present. A bleeding point was found at the LA diverticulum, and suture repair under on-pump beating was performed. CONCLUSIONS: When cardiac tamponade occured in any patient with LA diverticulum, treatment could not be completed with pericardiocentesis alone, and thoracotomy was likely to be necessary. If the bleeding point could be confirmed, suturing technique is a more reliable surgical strategy than adhesive alone that leads to pseudoaneurysm. If the bleeding point is unclear, it is important to confirm the occurrence of LA diverticulum using a preoperative CT, and if confirmed, cover it with adhesive due to a high possibility of diverticulum bleeding. The necessity of CPB should be determined based on whether these operations can be completed while maintaining vital stability.

[Discussion on the surgical timing of rupture and hemorrhage of renal angiomyolipoma].

OBJECTIVE: To investigate the effect of different surgical timing on the surgical treatment of renal angiomyolipoma (RAML) with rupture and hemorrhage. METHODS: The demographic data and perioperative data of 31 patients with rupture and hemorrhage of RAML admitted to our medical center from June 2013 to February 2023 were collected. The surgery within 7 days after hemorrhage was defined as a short-term surgery group, the surgery between 7 days and 6 months after hemorrhage was defined as a medium-term surgery group, and the surgery beyond 6 months after hemorrhage was defined as a long-term surgery group. The perioperative related indicators among the three groups were compared. RESULTS: This study collected 31 patients who underwent surgical treatment for RAML rupture and hemorrhage, of whom 13 were males and 18 were females, with an average age of (46.2±11.3) years. The short-term surgery group included 7 patients, the medium-term surgery group included 12 patients and the long-term surgery group included 12 patients. In terms of tumor diameter, the patients in the long-term surgery group were significantly lower than those in the recent surgery group [(6.6±2.4) cm vs. (10.0±3.0) cm, P=0.039]. In terms of operation time, the long-term surgery group was significantly shorter than the mid-term surgery group [(157.5±56.8) min vs. (254.8±80.1) min, P=0.006], and there was no significant difference between other groups. In terms of estimated blood loss during surgery, the long-term surgery group was significantly lower than the mid-term surgery group [35 (10, 100) mL vs. 650 (300, 1 200) mL, P < 0.001], and there was no significant difference between other groups. In terms of intraoperative blood transfusion, the long-term surgery group was significantly lower than the mid-term surgery group [0 (0, 0) mL vs. 200 (0, 700) mL, P=0.014], and there was no significant difference between other groups. In terms of postoperative hospitalization days, the long-term surgery group was significantly lower than the mid-term surgery group [5 (4, 7) d vs. 7 (6, 10) d, P=0.011], and there was no significant difference between other groups. CONCLUSION: We believe that for patients with RAML rupture and hemorrhage, reoperation for more than 6 months is a relatively safe time range, with minimal intraoperative bleeding. Therefore, it is more recommended to undergo surgical treatment after the hematoma is systematized through conservative treatment.

Efficacy and Safety of Long-Term Dual Antiplatelet Therapy: A Systematic Review and Meta-Analysis.

BACKGROUND: Dual antiplatelet therapy (DAPT) with aspirin and a P2Y12 inhibitor is a standard therapy in patients with ischemic vascular diseases (IVD) including coronary artery, cerebrovascular and peripheral arterial diseases, although the optimal duration of this treatment is still debated. Previous meta-analyses reported conflicting results about the effects of long-term and short-term as well as non-DAPT use in various clinical settings. Herein, we conducted a comprehensive meta-analysis to assess the efficacy and safety of different durations of DAPT. METHODS: We reviewed relevant articles and references from database, which were published prior to April 2023. Data from prospective studies were processed using RevMan5.0 software, provided by Cochrane Collaboration and transformed using relevant formulas. The inclusion criteria involved randomization to long-term versus short-term or no DAPT; the endpoints included at least one of total or cardiovascular (CV) mortalities, IVD recurrence, and bleeding. RESULTS: A total of 34 randomized studies involving 141 455 patients were finally included. In comparison with no or short-term DAPT, long-term DAPT reduced MI and stroke, but did not reduce the total and CV mortalities. Meanwhile, bleeding events were increased, even though intracranial and fatal bleedings were not affected. Besides, the reduction of MI and stroke recurrence showed no statistical significance between long-term and short-term DAPT groups. CONCLUSION: Long-term DAPT may not reduce the mortality of IVD besides increasing bleeding events, although reduced the incidences of MI and stroke early recurrence to a certain extent and did not increase the risk of fatal intracranial bleeding.

[Analysis of Influencing Factors of Complications for CT-guided 
Percutaneous Lung Biopsy].

BACKGROUND: Computed tomography guided percutaneous lung biopsy (CT-PLB) is a widely used method for the diagnosis of lung lesions. However, it is invasive, and the most common complications are pneumothorax and pulmonary hemorrhage, which can be life-threatening in severe cases. Therefore, the aim of this study is to analyze the independent risk factors affecting the occurrence of different complications of CT-PLB, so as to reduce the incidence of complications. METHODS: The 605 patients with complete clinical data who underwent CT-PLB in our hospital from May 2018 to December 2019 were retrospectively analyzed. According to the location of the lesions, they were divided into subpleural group and non-subpleural group. The patients were divided into pneumothorax group, pulmonary hemorrhage group, pneumothorax with pulmonary hemorrhage group and non-pneumothorax/pulmonary hemorrhage group according to the complications. The risk factors affecting the incidence of different complications and the independent risk factors of each complication were analyzed. RESULTS: The incidence of pneumothorax was 34.1%, the incidence of pulmonary hemorrhage was 28.1%, and the incidence of pneumothorax complicated with pulmonary hemorrhage was 10.8% (63 cases). The independent risk factor affecting the incidence of subpleural pneumothorax was lesion size (P=0.002). The independent risk factors affecting the occurrence of pneumothorax in the non-subpleural group were plain scan CT value (P=0.035), length of needle through lung tissue (P=0.003), and thickness of needle through chest wall (P=0.020). Independent risk factors affecting the occurrence of pulmonary hemorrhage in the non-subpleural group were length of needle through lung tissue (P<0.001), △CT value of needle travel area (P=0.001), lesion size (P=0.034) and body position (P=0.014). The independent risk factors affecting the co-occurrence of pneumothorax and pulmonary hemorrhage were the length of needle through lung tissue (P<0.001) and the △CT value of needle travel area (P<0.001). CONCLUSIONS: CT-PLB is a safe and effective diagnostic method, which of high diagnostic value for lung lesions. Selecting the appropriate puncture program can reduce complications such as pneumothorax and pulmonary hemorrhage, and improve diagnosis and treatment efficiency.

Resuscitation and Care in the Trauma Bay.

Start balanced resuscitation early (pre-hospital if possible), either in the form of whole blood or 1:1:1 ratio. Minimize resuscitation with crystalloid to minimize patient morbidity and mortality. Trauma-induced coagulopathy can be largely avoided with the use of balanced resuscitation, permissive hypotension, and minimized time to hemostasis. Using protocolized "triggers" for massive and ultramassive transfusion will assist in minimizing delays in transfusion of products, achieving balanced ratios, and avoiding trauma induced coagulopathy. Once "audible" bleeding has been addressed, further blood product resuscitation and adjunct replacement should be guided by viscoelastic testing. Early transfusion of whole blood can reduce patient morbidity, mortality, decreases donor exposure, and reduces nursing logistics during transfusions. Adjuncts to resuscitation should be guided by laboratory testing and carefully developed, institution-specific guidelines. These include empiric calcium replacement, tranexamic acid (or other anti-fibrinolytics), and fibrinogen supplementation.

Management of Pelvic Trauma.

Pelvic fractures are common after blunt trauma with patients' presentation ranging from stable with insignificant fractures to life-threatening exsanguination from unstable fractures. Often, hemorrhagic shock from a pelvic fracture may go unrecognized and high clinical suspicion for a pelvic source lies with the clinician. A multidisciplinary coordinated effort is required for management of these complex patients. In the exsanguinating patient, hemorrhage control remains the top priority and may be achieved with external stabilization, resuscitative endovascular balloon occlusion of the aorta, preperitoneal pelvic packing, angiographic intervention, or a combination of therapies. These modalities have been shown to reduce mortality in this challenging population.

Adrenal Hemorrhage: A Comprehensive Analysis of a Heterogeneous Entity-Etiology, Presentation, Management, and Outcomes.

OBJECTIVE: To investigate the etiology, presentation, management, and outcomes of patients with adrenal hemorrhage (AH). PATIENTS AND METHODS: Longitudinal study of consecutive adult patients with radiologically confirmed AH (January 1, 2017, through December 31, 2021). RESULTS: Of the 363 patients with AH (median age, 62 years [interquartile range (IQR, 52-70 years]; 128 women [35%]), 338 (93%) had unilateral AH and 25 (7%) had bilateral AH. It was discovered incidentally in 152 patients (42%) and during the evaluation of trauma in 103 (28%), abdominal/back pain in 90 (25%), critical illness in 13 (4%), and symptoms of adrenal insufficiency in 5 (1%). Etiologies included postoperative complications in 150 patients (41%), trauma in 107 (30%), coagulopathy in 22 (6%), anticoagulant/antiplatelet therapy in 39 (11%), adrenal neoplasm in 22 (6%), and sepsis in 11, (3%). Overall, 165 patients (46%) were hospitalized, and no deaths occurred due to AH. Median (IQR) baseline AH size was 34 mm (24-40 mm) on the right and 29 mm (22-37 mm) on the left. Among 246 patients with follow-up imaging, AH resolution was complete in 155 (63%) and incomplete in 74 (30%) at a median of 15 months (IQR, 6-31 months). Patients with bilateral AH were more likely to have underlying coagulopathy (44% vs 3%) and to develop primary adrenal insufficiency (72% vs 0%) than those with unilateral AH (P<.001). CONCLUSION: Often, AH presents as an incidental unilateral lesion with normal adrenal function, commonly attributed to postoperative complications or trauma. In contrast, bilateral AH is rare and typically linked to underlying coagulopathy, with primary adrenal insufficiency developing in most patients.

Segmental artery angioembolization as an efficient treatment modality for delayed hematuria with normal angiography: two case reports.

BACKGROUND: Percutaneous nephrolithotomy is the most commonly used modality for the removal of kidney stones larger than 2 cm in size. Like other stone removal methods, percutaneous nephrolithotomy also has some complications, including bleeding and delayed hematuria. These complications are improved with conservative management and bed rest most of the time. However, it may require more invasive treatments. Angioembolization following an abnormal renal angiography is an efficient treatment modality for delayed hematuria. Furthermore, nephrectomy is suggested in uncontrolled cases of delayed hematuria when renal angiography is normal. CASE PRESENTATION: We described two cases of uncontrolled delayed hematuria after percutaneous nephrolithotomy and angioembolization were carried out rather than potential nephrectomies. The first case was a 61-year-old Iranian man with left kidney stones, for whom percutaneous nephrolithotomy was planned. The patient was referred to the hospital after discharge with massive hematuria and had normal angiographic findings. An angioembolization was suggested for the patient and was carried out. His hematuria was dramatically improved within 30 minutes, and his hemoglobin level started to increase 2 days later. The second case was a 53-year-old Iranian man with kidney stones who was a candidate for right kidney percutaneous nephrolithotomy. The patient was referred to the hospital 4 days after discharge with a decreased hemoglobin level and massive hematuria. The patient had normal angiographic findings and was planned for angioembolization to control his hemorrhage, which dramatically decreased after the angioembolization within 60 minutes. CONCLUSION: Embolization of the segmental arteries of the targeted calyx can eliminate hematuria of the patient and prevent further nephrectomy.

Benefits and risks of non-factor therapies: Redefining haemophilia treatment goals in the era of new technologies.

INTRODUCTION: Over the last decades progress in haemophilia treatment has been remarkable and prophylaxis with clotting factor concentrates in haemophilia A and B has been established as the standard of care in individuals with haemophilia and a severe bleeding phenotype. Besides clotting factor products with prolonged half-life non-factor therapies were developed which enable prophylaxis via subcutaneous administration. Factor VIIIa mimetics like emicizumab facilitate the coagulation pathway and are used in routine clinical practice for indivdiduals with haemophilia A. Rebalancing therapeutic agents like fitusiran, concizumab, marstacimab and serpin PC block the anticoagulant pathway and clinical trials using these products in individuals with haemophilia A and B are ongoing. AIM AND METHODS: A narrative review to asess the benefits and risks of non-factor therapies taking in to account re-defined haemophilia treatment goals. RESULTS: Prophylaxis for prevention of bleeds using non-factor products by subcutaneous administration is effective and results in reductions of bleeding episodes in individuals with haemophilia A or B with and without inhibitors. The treatment with emicizumab showed tolerable safety both in clinical trials and long-term real-world observations with few thrombotic events. In some clinical trials with rebalancing therapies (fitusiran and concizumab) thrombotic events occurred. Monitoring of the haemostatic function of novel therapies especially with concomitant haemostatic treatment is not yet established. CONCLUSION: With the advent of novel therapeutic agents including factor concentrates with ultra-long half-life and improved FVIIIa mimetics aimed at raising the bar of protection into the non-hemophilic range redefinition of haemophilia treatment goals is eagerly needed.