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Supplemental Digital Content is available in the text. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is available in the text. [ABSTRACT FROM AUTHOR]

The article discusses how practice guidelines offer recommendations applicable to patients with or at risk of developing cardiovascular disease. The American College of Cardiology (ACC) and American Heart Association (AHA) sponsor the guidelines without commercial support, and members volunteer their time. The Task Force zealously avoids actual, potential, or perceived conflicts of interest that might arise through relationships with industry or other entities (RWI).

The article reports that managing patients with multiple conditions can be complex, especially when recommendations applicable to coexisting illnesses are discordant or interacting. Management in accordance with guideline recommendations is effective only when followed. Adherence to recommendations can be enhanced by shared decision making between clinicians and patients, with patient engagement in selecting interventions on the basis of individual values, and preferences.

BACKGROUND: Whether carotid artery stenosis predicts stroke after noncardiac surgery remains unknown. We therefore tested the primary hypothesis that degree of carotid artery stenosis is associated with in-hospital stroke or 30-day all-cause mortality after noncardiac surgery. As carotid artery stenosis is also a marker for cardiovascular disease, our secondary hypothesis was that degree of carotid artery stenosis is associated with postoperative myocardial injury. METHODS: We included adults who had noncardiac, noncarotid surgery at Cleveland Clinic from 2007 to 2011 and had carotid duplex ultrasound performed either within 6 months before or 1 month after surgery. Internal carotid artery peak systolic velocity (ICA PSV) was used as a measure of carotid artery stenosis severity. A multivariate (i.e., multiple outcomes per patient) generalized estimating equation model was used to assess the association between highest ICA PSV and the composite of stroke and 30-day mortality after adjusting for predefined potentially confounding variables. RESULTS: Of 2,110 patients included, 112 (5.3%) died within 30 days and 54 (2.6%) suffered postoperative in-hospital stroke. ICA PSV was not associated with this composite outcome (odds ratio of 1.0 [95% confidence interval: 0.99, 1.02] for a 10-unit increase, P = 0.55). ICA PSV was also not associated with postoperative myocardial injury (odds ratio 1.00 [0.99, 1.02], P = 0.49). CONCLUSIONS: This cohort represents a high-risk population, as carotid duplex examinations were likely prompted by neurological symptoms. There was nonetheless no association between carotid artery stenosis and perioperative stroke or 30-day mortality after noncardiac surgery. [ABSTRACT FROM AUTHOR]

The article presents a scientific statement from the American Heart Association about fibromuscular dysplasia (FMD), a nonatherosclerotic, noninflammatory vascular disease that may lead to arterial stenosis, occlusion, aneurysm, or dissection. Topics include an historical perspective on FMD, the prevalence of FMD, and genetic considerations. Histopathological classifications for FMD and clinical manifestations of FMD are also mentioned.

The article describes the case of a 26-year-old woman presented with progressive swelling and pain of the left leg after switching to a different oral contraceptive preparation. Topics discussed include the components of the lower-extremity venous duplex (VDUS) examination to assess for acute deep venous thrombosis (DVT), the acuity of DVT, and the evaluation of patients with suspected pulmonary embolism.

Background: The risk of arterial diseases may be elevated among family members of individuals having multifocal fibromuscular dysplasia (FMD). We sought to investigate the risk of arterial diseases in families of individuals with FMD., Methods: Family histories for 73 probands with FMD were obtained, which included an analysis of 463 total first-degree relatives focusing on FMD and related arterial disorders. A polygenic risk score for FMD (PRS FMD ) was constructed from prior genome-wide association findings of 584 FMD cases and 7139 controls and evaluated for association with an abdominal aortic aneurysm (AAA) in a cohort of 9693 AAA cases and 294 049 controls. A previously published PRS AAA was also assessed among the FMD cases and controls., Results: Of all first degree relatives of probands, 9.3% were diagnosed with FMD, aneurysms, and dissections. Aneurysmal disease occurred in 60.5% of affected relatives and 5.6% of all relatives. Among 227 female first-degree relatives of probands, 4.8% (11) had FMD, representing a relative risk (RR) FMD of 1.5 ([95% CI, 0.75-2.8]; P =0.19) compared with the estimated population prevalence of 3.3%, though not of statistical significance. Of all fathers of FMD probands, 11% had AAAs resulting in a RR AAA of 2.3 ([95% CI, 1.12-4.6]; P =0.014) compared with population estimates. The PRS FMD was found to be associated with an AAA (odds ratio, 1.03 [95% CI, 1.01-1.05]; P =2.6×10 -3 ), and the PRS AAA was found to be associated with FMD (odds ratio, 1.53 [95% CI, 1.2-1.9]; P =9.0×10 -5 ) as well., Conclusions: FMD and AAAs seem to be sex-dimorphic manifestations of a heritable arterial disease with a partially shared complex genetic architecture. Excess risk of having an AAA according to a family history of FMD may justify screening in family members of individuals having FMD.

Objective: While rare variants in the COL5A1 gene have been associated with classical Ehlers-Danlos syndrome and rarely with arterial dissections, recurrent variants in COL5A1 underlying a systemic arteriopathy have not been described. Monogenic forms of multifocal fibromuscular dysplasia (mFMD) have not been previously defined. Approach and Results: We studied 4 independent probands with the COL5A1 pathogenic variant c.1540G>A, p.(Gly514Ser) who presented with arterial aneurysms, dissections, tortuosity, and mFMD affecting multiple arteries. Arterial medial fibroplasia and smooth muscle cell disorganization were confirmed histologically. The COL5A1 c.1540G>A variant is predicted to be pathogenic in silico and absent in gnomAD. The c.1540G>A variant is on a shared 160.1 kb haplotype with 0.4% frequency in Europeans. Furthermore, exome sequencing data from a cohort of 264 individuals with mFMD were examined for COL5A1 variants. In this mFMD cohort, COL5A1 c.1540G>A and 6 additional relatively rare COL5A1 variants predicted to be deleterious in silico were identified and were associated with arterial dissections ( P =0.005)., Conclusions: COL5A1 c.1540G>A is the first recurring variant recognized to be associated with arterial dissections and mFMD. This variant presents with a phenotype reminiscent of vascular Ehlers-Danlos syndrome. A shared haplotype among probands supports the existence of a common founder. Relatively rare COL5A1 genetic variants predicted to be deleterious by in silico analysis were identified in ≈2.7% of mFMD cases, and as they were enriched in patients with arterial dissections, may act as disease modifiers. Molecular testing for COL5A1 should be considered in patients with a phenotype overlapping with vascular Ehlers-Danlos syndrome and mFMD.