Xiaokui Gu

Clinical Manifestations of Fibromuscular Dysplasia Vary by Patient Sex: A Report of the United States Registry for Fibromuscular Dysplasia

To the Editor: Fibromuscular dysplasia (FMD) is an uncommon arteriopathy which can result in stenosis, aneurysm, dissection, and/or occlusion of arteries. It most commonly affects the renal, extracranial carotid, and vertebral arteries but can affect any artery. Although FMD occurs primarily in

The Predictive Ability of the CHADS2 and CHA2DS2-VASc Scores for Bleeding Risk in Atrial Fibrillation: The MAQI2 Experience

INTRODUCTION Guidelines recommend the assessment of stroke and bleeding risk before initiating warfarin anticoagulation in patients with atrial fibrillation. Many of the elements used to predict stroke also overlap with bleeding risk in atrial fibrillation patients and it is tempting to use stroke risk scores to efficiently estimate bleeding risk. Comparison of stroke risk scores to bleeding risk scores to predict bleeding has not been thoroughly assessed. METHODS 2600 patients followed at seven anticoagulation clinics were followed from October 2009-May 2013. Five risk models (CHADS2, CHA2DS2-VASc, HEMORR2HAGES, HAS-BLED and ATRIA) were retrospectively applied to each patient. The primary outcome was the first major bleeding event. Area under the ROC curves were compared with C statistic and net reclassification improvement (NRI) analysis was performed. RESULTS 110 patients experienced a major bleeding event in 2581.6 patient-years (4.5%/year). Mean follow up was 1.0±0.8years. All of the formal bleeding risk scores had a modest predictive value for first major bleeding events (C statistic 0.66-0.69), performing better than CHADS2 and CHA2DS2-VASc scores (C statistic difference 0.10 - 0.16). NRI analysis demonstrated a 52-69% and 47-64% improvement of the formal bleeding risk scores over the CHADS2 score and CHA2DS2-VASc score, respectively. CONCLUSIONS The CHADS2 and CHA2DS2-VASc scores did not perform as well as formal bleeding risk scores for prediction of major bleeding in non-valvular atrial fibrillation patients treated with warfarin. All three bleeding risk scores (HAS-BLED, ATRIA and HEMORR2HAGES) performed moderately well.

Anti-platelet and anti-hypertension medication use in patients with fibromuscular dysplasia: Results from the United States Registry for Fibromuscular Dysplasia.

Fibromuscular dysplasia (FMD), a non-inflammatory arterial disease, may lead to renovascular hypertension (HTN) and cerebrovascular disease. Little is known about medication use in FMD. Clinical features and medication use were reviewed in a national FMD registry (12 US sites). Medication usage was assessed in raw and adjusted analyses. Covariates included demographic characteristics, co-morbid conditions and vascular bed involvement. A total of 874 subjects (93.6% female) were included in the analysis. Mean age was 55.6±13.1 years, 74.5% had HTN, 25.4% had a history of transient ischemic attack or stroke, and 7.5% had a history of coronary artery disease (CAD). Renal and cerebrovascular arteries were affected in 70.4% and 74.7%, respectively. Anti-platelet agents were administered to 72.9% of patients. In multivariate analyses, factors associated with a greater likelihood of anti-platelet agent use were older age (OR=1.02 per year, p=0.005), CAD (OR=3.76, p=0.015), cerebrovascular artery FMD involvement in isolation (OR=2.31, p<0.0001) or a history of previous intervention for FMD (OR=1.52, p=0.036). A greater number of anti-HTN medications was evident in isolated renal versus isolated cerebrovascular FMD patients. Factors associated with a greater number of anti-HTN medications were older age (OR=1.03 per year, p<0.0001), history of HTN (OR=24.04, p<0.0001), history of CAD (OR=2.71, p=0.0008) and a history of a previous therapeutic procedure (OR=1.72, p=0.001). In conclusion, in FMD, medication use varies based on vascular bed involvement. Isolated renal FMD patients receive more anti-HTN agents and there is greater anti-platelet agent use among patients with cerebrovascular FMD. Further studies correlating medication use in FMD with clinically meaningful patient outcomes are necessary.

Smoking and Adverse Outcomes in Fibromuscular Dysplasia: U.S. Registry Report.

The pathophysiology of fibromuscular dysplasia (FMD) is unknown; however, smoking has been implicated as a potential contributing factor [(1,2)][1]. Prior studies have shown a higher prevalence of smoking among those with renal FMD compared with matched hypertensive control subjects [(3)][2]. The

The role of preoperative coronary angiography in the setting of type A acute aortic dissection: Insights from the International Registry of Acute Aortic Dissection

BACKGROUND Performing preoperative coronary angiography (CA) before surgical repair of a type A acute aortic dissection (TA-AAD) remains controversial. Although the information provided by CA may be useful in planning the surgical approach, the potential delay to surgery and complications of CA may confer added risk of death before definitive repair of the aorta. METHODS We analyzed 1,343 patients from January 27, 1996, to May 3, 2010, with TA-AAD from the International Registry of Acute Aortic Dissection who underwent surgical or endovascular repair during the index hospitalization, with (n = 156) or without (n = 1,187) preoperative CA. The main outcomes measured were in-hospital complications and in-hospital and long-term mortality. RESULTS Patients who underwent preoperative CA were more likely to have a history of atherosclerosis and present with electrocardiographic signs of myocardial ischemia/infarction. In the preoperative CA group, significant delays from the onset of symptoms to the time of surgery occurred. In-hospital postoperative complications and mortality rates were largely similar between the 2 groups. On multivariable logistic regression analysis, preoperative CA had no significant effect on in-hospital risk-adjusted mortality when compared to the validated International Registry of Acute Aortic Dissection risk score. Long-term mortality was similar between patients receiving preoperative CA and those who did not; long-term rehospitalization rates were higher, although largely insignificantly, among preoperative CA recipients through 5 years of follow-up. CONCLUSIONS Preoperative CA is infrequently performed on patients with TA-AAD, except, occasionally, on patients at high risk for myocardial ischemia. When performed, preoperative CA was not associated with any significant changes in in-hospital and long-term mortality.

Prescribing trends of atrial fibrillation patients who switched from warfarin to a direct oral anticoagulant

Direct oral anticoagulant (DOAC) agents offer several lifestyle and therapeutic advantages for patients relative to warfarin in the treatment of atrial fibrillation (AF). These alternative agents are increasingly used in the treatment of AF, however the adoption practices, patient profiles, and reasons for switching to a DOAC from warfarin have not been well studied. Through the Michigan Anticoagulation Quality Improvement Initiative, abstracted data from 3873 AF patients, enrolled between 2010 and 2015, were collected on demographics and comorbid conditions, stroke and bleeding risk scores, and reasons for anticoagulant switching. Over the study period, patients who switched from warfarin to a DOAC had similar baseline characteristics, risk scores, and insurance status but differed in baseline CrCl. The most common reasons for switching were patient related ease of use concerns (37.5%) as opposed to clinical reasons (16.5% of patients). Only 13% of patients that switched to a DOAC switched back to warfarin by the end of the study period.

Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia: A Report From the US Registry for Fibromuscular Dysplasia

Importance The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. Objective To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. Design, Setting, and Participants This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. Main Outcomes and Measures Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. Results Of 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years). Of the 669 patients included in the analysis, 86 (12.9%; 95% CI, 10.3%-15.9%) had at least 1 intracranial aneurysm. Of these 86 patients, 25 (53.8%) had more than 1 intracranial aneurysm. Intracranial aneurysms 5 mm or larger occurred in 32 of 74 patients (43.2%), and 24 of 128 intracranial aneurysms (18.8%) were in the posterior communicating or posterior arteries. The presence of intracranial aneurysm did not vary with location of extracranial FMD involvement. A history of smoking was significantly associated with intracranial aneurysm: 42 of 78 patients with intracranial aneurysm (53.8%) had a smoking history vs 163 of 564 patients without intracranial aneurysm (28.9%; P < .001). Conclusions and Relevance The prevalence of intracranial aneurysm in women diagnosed with FMD is significantly higher than reported in the general population. Although the clinical benefit of screening for intracranial aneurysm in patients with FMD has yet to be proven, these data lend support to the recommendation that all patients with FMD undergo intracranial imaging if not already performed.

The United States Registry for Fibromuscular Dysplasia

Background— Fibromuscular dysplasia (FMD), a noninflammatory disease of medium-size arteries, may lead to stenosis, occlusion, dissection, and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis, and outcomes since its first description in 1938. Methods and Results— Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 US sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years; range, 5–83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%), and sudden death (19.8%) were common, but FMD in first- or second-degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, and vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had experienced arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection. Conclusions— In this registry, FMD occurred primarily in middle-aged women, although it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. Although a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.

CLINICAL FEATURES AND PRESENTING SYMPTOMS AND SIGNS OF FIBROMUSCULAR DYSPLASIA: A REPORT OF THE FIBROMUSCULAR DYSPLASIA PATIENT REGISTRY

Results: Most pts were female (91.4%) with a mean age at diagnosis of 51.5 + 14.3 years. The most common presenting symptoms and signs are shown below; only 4.3% were asymptomatic. The median interval from first symptom to diagnosis was within the first year (IQR 0-4 years). Family history (FH) of stroke (51.7%) or aneurysm (22.7%) was common, but FH of diagnosed FMD was uncommon (7.9%). Ultrasound was the most common diagnostic modality, followed by angiography, CTA, and MRA. The most common vascular beds involved were: renal (61.3%), extracranial carotid (55.3%), vertebral (16.6%), mesenteric (11.3%), lower extremity (8.0%), intracranial (7.3%). Medial fibroplasia was the most common type, followed by intimal disease.

Structure and function of anticoagulation clinics in the United States: an AC forum membership survey

Many anticoagulation clinics have adapted their services to provide care for patients taking direct oral anticoagulants (DOAC) in addition to traditional warfarin management. Anticoagulation clinic scope of service and operations in this transitional environment have not been well described in the literature. A survey was conducted of United States-based Anticoagulation Forum members to inquire about anticoagulation clinic structure, function, and services provided. Survey responses are reported using summary or non-parametric statistics, when appropriate. Unique clinic survey responses were received from 159 anticoagulation clinics. Clinic structure and staffing are highly variable, with approximately half of clinics (52%) providing DOAC-focused care in addition to traditional warfarin-focused care. Of those clinics managing DOAC patients, this accounts for only 10% of their clinic volume. These clinics commonly have a DOAC follow up protocol (75%). Clinics assign a median of 190.5 (interquartile range 50–300) patients per staff full-time-equivalent, with more patients assigned in phone-based care clinics than in face-to-face based care clinics. Most clinics (68.5%) report receiving reimbursement, which occur either through a combination of patient and insurance provider billing (78.2%), insurance reimbursement only (19.5%) or patient reimbursement only (2.3%). There is wide heterogeneity in anticoagulation clinic structure, function, and services provided. Half of all survey-responding anticoagulation clinics provide care for DOAC-treated patients. Understanding how changes in healthcare policy and reimbursement have impacted these clinics remains to be explored.