Familial hypercholesterolemia (FH) is a genetic disorder of lipoprotein metabolism resulting in elevated serum low-density lipoprotein (LDL) cholesterol levels leading to increased risk for premature cardiovascular diseases (CVDs). The diagnosis of this condition is based on clinical features, family history, and elevated LDL-cholesterol levels aided more recently by genetic testing. As the atherosclerotic burden is dependent on the degree and duration of exposure to raised LDL-cholesterol levels, early diagnosis and initiation of treatment is paramount. Statins are presently the mainstay in the management of these patients, although newer drugs, LDL apheresis, and other investigational therapies may play a role in certain subsets of FH, which are challenging to treat. Together these novel treatments have notably improved the prognosis of FH, especially that of the heterozygous patients. Despite these achievements, a majority of children fail to attain targeted lipid goals owing to persistent shortcomings in diagnosis, monitoring, and treatment. This review aims to highlight the screening, diagnosis, goals of therapy, and management options in patients with FH.

Purpose: To describe a precannulated fenestrated endograft system utilizing externalized guidewires to facilitate aortic arch endovascular repair and to report its use in 2 patients with challenging anatomy. Technique: For distal arch repair, a fenestration for the left subclavian artery (LSA) is made onsite in a standard thoracic endograft tailored to the patient anatomy; it is precannulated with a nitinol guidewire (NGw), which is passed from the femoral artery and externalized from the left brachial artery prior to endograft delivery system introduction over a parallel stiff guidewire. Steps are then taken to remove guidewire intertwining, prevent NGw wrapping around the delivery system, and orient the LSA fenestration superiorly when the delivery system moves into the arch. Gentle traction on the ends of the NGw during endograft deployment facilitates proper fenestration alignment. A covered stent is deployed in the LSA fenestration. The technique is illustrated in a patient with congenital coarctation of the aorta and descending aortic aneurysm. For total arch repair, endograft fenestrations are made for all 3 arch branches; the left common carotid artery (LCCA) and LSA fenestrations are each cannulated with NGws, which travel together from the femoral artery, pass through a LSA snare loop, and are exteriorized from the LCCA. After endograft deployment, the innominate artery fenestration is separately cannulated using right brachial access. Placement of a parallel externalized hydrophilic guidewire passing through the LCCA fenestration (but not the LSA snare loop) and removal of the LCCA fenestration NGw allows exteriorization of the LSA fenestration NGw from the left brachial artery by pulling the LSA snare. Covered stents are deployed in all 3 fenestrations. The technique is presented in a patient with type B aortic dissection. Conclusion: Use of the precannulated fenestrated endograft system described is feasible and has the potential to make aortic arch endovascular repair simpler, more reliable, and safer.

Background: Because of the widespread acceptance of percutaneous coronary intervention with drug-eluting stents as an effective treatment strategy for in-stent restenosis, it is common to encounter multimetal layer stent restenosis in the recent years. This study aimed to evaluate the clinical outcomes of such patients treated with intravascular brachytherapy (IVBT) in comparison with other percutaneous options. Methods and Results: We enrolled patients who underwent percutaneous coronary intervention during the period between 2011 and 2015 for recurrent drug-eluting stents in-stent restenosis with at least 2 layers of stents at the lesion site. This analysis compared patients who underwent treatment with IVBT and those who did not (non-IVBT group). The primary end point measured was major adverse cardiac events defined as a composite of target lesion revascularization, myocardial infarction, and all-cause mortality at 12 months. Adjusted associations were measured using propensity score matching. A total of 328 percutaneous coronary intervention patients met the eligibility criteria, of which 197 patients received IVBT, and 131 patients underwent routine percutaneous intervention. The primary end point was significantly lower in patients undergoing IVBT (13.2% and 28.2%; P =0.01). A propensity score matching for risk factors of in-stent restenosis identified 182 patients. The advantages of IVBT with regard to 1-year major adverse cardiac events were confirmed in this matched cohort (13.2% and 30.8%; adjusted hazard ratio [95% CI]: 0.37 [0.18–0.73]; P <0.01). Conclusions: In this analysis, IVBT led to significantly lower major adverse cardiac events in patients with multilayered drug-eluting stents restenosis when compared with other percutaneous options at 1-year follow-up.

Very severe OSA is associated with significant diastolic dysfunction as well as early systolic abnormalities as evidenced by abnormal global longitudinal strain. Sleep apnea severity as measured by AHI was the only significant predictor of abnormal longitudinal strain in these patients.