The role of primary prophylactic factor replacement therapy in children with severe factor X deficiency

Severe factor X (FX) deficiency is one of the severest inherited coagulation disorders. Clinical manifestations include umbilical cord, mucosal, joint and central nervous system bleeding. Four Irish children with severe FX deficiency presented with umbilical cord bleeding. One developed an intraperitoneal haemorrhage and another an intracranial bleed. Prophylaxis, using intermediate purity Factor IX concentrate, was commenced within the first month of life, necessitating the insertion of central venous access devices in two of the children. All children have normal joint function, suggesting that prophylaxis commenced early in life reduces the incidence of arthropathy and improves quality of life.     

IMPACT OF HEPARIN LOCKING FREQUENCY ON PREVENTING TEMPORARY DIALYSIS CATHETER DYSFUNCTION IN HAEMODIALYSIS PATIENTS

A prospective analysis was conducted comparing dysfunction attributable to catheter thrombosis in subjects who received a heparin catheter lock three times a week (n = 15) to those who received a heparin lock six times a week (n = 15) immediately after the insertion of a temporary haemodialysis catheter. Thrombus related catheter removal occurred in two patients in control but no patients in the experiment group. Heparin locking six times a week was found to be effective in prolonging the mean of the first day where difficulty was experienced in aspiration. It also prevented any possible difficulty in catheter flushing. Increased locking frequency prevents any thrombus accumulation within the temporary catheter, while it has limited but significant preventive effect on thrombus accumulated around the catheter.     

经桡动脉与经桡股复合途径行冠状动脉慢性闭塞病变介入治疗的有效性和安全性分析

目的：比较经双侧桡动脉与经桡股复合途径行冠状动脉慢性闭塞病变介入治疗的有效性和安全性。方法：研究纳入336例采用双侧桡动脉途径或桡股动脉复合途径实施冠状动脉慢性闭塞病变介入治疗的病例。对比两组患者基线数据、手术策略、术中参数（手术时间、曝光时间、射线量、造影剂用量和肝素用量等）、手术成功率和术后并发症情况。结果：76.8%的病例采用双侧桡动脉途径完成介入治疗。与桡股动脉复合途径组相比,双侧桡动脉途径组手术时间短,造影和肝素用量少（P < 0.05）。双侧桡动脉途径组手术成功率不劣于桡股动脉复合途径组（P > 0.05）。经双侧桡动脉途径行冠状动脉慢性闭塞病变介入治疗,出现冠脉穿孔、血管入路并发症及急性肾损伤的比例明显降低（P<0.05）。结论：双侧桡动脉途径已成为冠状动脉慢性闭塞病变介入治疗的重要入路,得益于其较高的手术成功率和突出的安全性优势,值得临床进一步推广应用。     

The PARTNER 3 Bicuspid Registry for Transcatheter Aortic Valve Replacement in Low-Surgical-Risk Patients

ObjectivesThe study compared 1-year outcomes between transcatheter aortic valve replacement (TAVR) patients with bicuspid aortic valve (BAV) morphology and clinically similar patients having tricuspid aortic valve (TAV) morphology.BackgroundThere are limited prospective data on TAVR using the SAPIEN 3 device in low-surgical-risk patients with severe, symptomatic aortic stenosis and bicuspid anatomy.MethodsLow-risk, severe aortic stenosis patients with BAV were candidates for the PARTNER 3 (Placement of Aortic Transcatheter Valves 3) (P3) bicuspid registry or the P3 bicuspid continued access protocol. Patients treated in these registries were pooled and propensity score matched to TAV patients from the P3 randomized TAVR trial. Outcomes were compared between groups. The primary endpoint was the 1-year composite rate of death, stroke, and cardiovascular rehospitalization.ResultsOf 320 total submitted BAV patients, 169 (53%) were treated, and most were Sievers type 1. The remaining 151 patients were excluded caused by anatomic or clinical criteria. Propensity score matching with the P3 TAVR cohort (496 patients) yielded 148 pairs. There were no differences in baseline clinical characteristics; however, BAV patients had larger annuli and they experienced longer procedure duration. There was no difference in the primary endpoint between BAV and TAV (10.9% vs 10.2%; P = 0.80) or in the rates of the individual components (death: 0.7% vs 1.4%; P = 0.58; stroke: 2.1% vs 2.0%; P = 0.99; cardiovascular rehospitalization: 9.6% vs 9.5%; P = 0.96).ConclusionsAmong highly select bicuspid aortic stenosis low-surgical-risk patients without extensive raphe or subannular calcification, TAVR with the SAPIEN 3 valve demonstrated similar outcomes to a matched cohort of patients with tricuspid aortic stenosis.     

International Hand Function Study Following Distal Radial Access: The RATATOUILLE Study

BackgroundDistal radial access (DRA) has been proposed to improve procedure ergonomics and favor radial artery patency. Although promising data, nothing is known on evolving hand function after DRA.ObjectivesThis study sought to comprehensively evaluate hand function in patients undergoing DRA.MethodsReal-world patients undergoing DRA undertook a thorough multimodality assessment of hand function implementing multidomain questionnaires (Disabilities of the Arm, Shoulder and Hand and Levine-Katz), and motor (pinch grip test) and sensory (Semmes-Weinstein monofilaments test) examinations of both hands. All assessments were performed at preprocedural baseline and planned at 1-, 6-, and 12-month follow-up (FU). Adverse clinical and procedural events were documented too.ResultsData of 313 patients (220 men, age 66 ± 10 years) from 9 international centers were analyzed. The Disabilities of the Arm, Shoulder and Hand and the Levine-Katz scores slightly improved from baseline to FU (P = 0.008 and P = 0.029, respectively). Pinch strength mildly improved from baseline to FU (P < 0.001 for both the left and right hands). Similarly, touch pressure threshold appeared to faintly improve in both the left and right hands (P < 0.012 for all the sites). For both motor and sensory function tests, comparable findings were found for the DRA hand and the contralateral one, with no significant differences between them. Repeated assessment of all tests over all FU time points similarly showed lack of worsening hand function. Access-related adverse events included 19 harmless bleedings and 3 forearm radial artery and 3 distal radial artery occlusions. None affected hand function at FU.ConclusionsIn a systematic multidimensional assessment, DRA was not associated with hand function impairment. Moreover, DRA emerges as a safe alternative vascular access.     

Coronary Access Following Redo TAVR: Impact of THV Design,Implant Technique,and Cell Misalignment

BackgroundThe implications and potential challenges of coronary access after redo transcatheter aortic valve replacement (TAVR) are unknown.ObjectivesThe authors sought to evaluate the impact of different transcatheter heart valve (THV) designs, neoskirt height, implant technique, and cell misalignment on coronary access after redo TAVR.MethodsDifferent THV designs (Sapien 3 [Edwards Lifesciences LLC], Evolut Pro [Medtronic], ACURATE neo [Boston Scientific Corporation], and Portico [Abbott Structural Heart]) and sizes were implanted inside Sapien XT (Edwards Lifesciences LLC) and Evolut R (Medtronic) THVs, which were modeled as the “failed” THVs, at different implant depths. Valve combinations underwent micro–computed tomography to determine the neoskirt height and dimensions of the lowest accessible cell for potential coronary access. This was compared with dimensions of 6-F/7-F/8-F coronary guiding catheters.ResultsRedo TAVR combinations resulted in a wide range of neoskirt heights (15.4-31.6 mm) and a variable diameter of the lowest accessible cell (1.9-21.8 mm). An ACURATE neo implanted in a Sapien XT resulted in the largest accessible cells, whereas a Portico implanted in a Sapien XT resulted in the lowest neoskirt heights. The smallest accessible cell was observed in the Evolut Pro–in–Evolut R configuration with higher neoskirt heights. Redo TAVR in a tall frame valve with supra-annular leaflets caused a taller neoskirt height. In Evolut-in-Evolut combinations, misalignment of the cells of the 2 THVs reduced the cell area by 30% to 50% compared with an aligned configuration.ConclusionsThis study demonstrates that different redo TAVR combinations are not equivalent in terms of future coronary access. Redo TAVR using a tall frame valve in a failed tall frame valve and misaligned cells may lead to potentially challenging coronary access.