Fibromuscular Dysplasia and Abdominal Aortic Aneurysms Are Dimorphic Sex-Specific Diseases With Shared Complex Genetic Architecture.

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.

Histologic and morphologic character of pediatric abdominal aortic developmental coarctation and hypoplasia.

Objectives: Abdominal aortic coarctation and hypoplasia are uncommon diseases, recognized most often in pediatric-aged individuals. Comprehensive studies regarding the pathologic spectrum of these aortopathies are nonexistent. This investigation was undertaken to better define the histologic and morphologic character of abdominal aortic narrowings affecting children and assess its potential relevance to contemporary clinical practice.

Molecular genetic evaluation of pediatric renovascular hypertension due to renal artery stenosis and abdominal aortic coarctation in neurofibromatosis type 1.

The etiology of renal artery stenosis (RAS) and abdominal aortic coarctation (AAC) causing the midaortic syndrome (MAS), often resulting in renovascular hypertension (RVH), remains ill-defined. Neurofibromatosis type 1 (NF-1) is frequently observed in children with RVH. Consecutive pediatric patients (N = 102) presenting with RVH secondary to RAS with and without concurrent AAC were prospectively enrolled in a clinical data base, and blood, saliva and operative tissue, when available, were collected.

Industry Compensation to Physician Vascular Specialist Authors of Highly-referenced Aortic Aneurysm Studies.

Background: Industry payments to physicians may influence their attitudes toward medical devices and products. Disclosure of industry compensation by authors of scientific manuscripts usually occurs at the authors' discretion and is seldom audited as part of the peer review process. The purpose of this analysis was to characterize industry compensation among highly cited research articles related to aortic aneurysm.

A Novel Recurrent Genetic Variant Is Associated With a Dysplasia-Associated Arterial Disease Exhibiting Dissections and Fibromuscular Dysplasia.

Objective: While rare variants in the gene have been associated with classical Ehlers-Danlos syndrome and rarely with arterial dissections, recurrent variants in 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 pathogenic variant c.

Chromosome 1q21.2 and additional loci influence risk of spontaneous coronary artery dissection and myocardial infarction.

Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic cause of myocardial infarction (MI), typically in young women. We undertook a genome-wide association study of SCAD (N = 270/N = 5,263) and identified and replicated an association of rs12740679 at chromosome 1q21.2 (P = 2.

Histologic and morphologic character of pediatric renal artery occlusive disease.

Objective: The pathologic nature of pediatric renal artery occlusive lesions causing renovascular hypertension has been the subject of numerous anecdotal reports. This study was undertaken to define the character of childhood renal artery stenoses. A better understanding of this disease is particularly germane, given its unknown etiology and the limited success of certain contemporary treatment options.

Surgical management of pediatric renin-mediated hypertension secondary to renal artery occlusive disease and abdominal aortic coarctation.

Background: Renovascular hypertension (RVH) associated with renal artery and abdominal aortic narrowings is the third most common cause of pediatric hypertension. Untreated children may experience major cardiopulmonary complications, stroke, renal failure, and death. The impetus of this study was to describe the increasingly complex surgical practice for such patients with an emphasis on anatomic phenotype and contemporary outcomes after surgical management as a means of identifying those factors responsible for persistent or recurrent hypertension necessitating reoperation.

Industry compensation and self-reported financial conflicts of interest among authors of highly cited peripheral artery disease studies.

Objective: Industry compensation to authors may influence the interpretation of study results. Scientific journals often require author disclosure of a relevant financial conflict of interest (FCOI) but seldom quantify compensation and leave reporting up to the author's discretion. Professional and public concerns related to potential bias introduced into medical research by FCOI have arisen, especially when physician compensation from manufacturers is not disclosed.

The Association of Intracranial Aneurysms in Women with Renal Artery Aneurysms.

Background: Renal artery aneurysms (RAAs) may reflect a systemic dysplastic arteriopathy, independent of a recognized connective tissue disease. It is hypothesized that RAAs are associated with an increased risk of intracranial aneurysms (IcAs). The objective of this study was to better define the association of IcAs in women with RAAs.

Computational analysis of renal artery flow characteristics by modeling aortoplasty and aortic bypass interventions for abdominal aortic coarctation.

Objective: Suprarenal abdominal aortic coarctation (SAAC) alters flow and pressure patterns to the kidneys and is often associated with severe angiotensin-mediated hypertension refractory to drug therapy. SAAC is most often treated by a thoracoabdominal bypass (TAB) or patch aortoplasty (PA). It is currently unclear what effect these interventions have on renal flow and pressure waveforms.

Arterial reconstructions for pediatric splanchnic artery occlusive disease.

Objective: Pediatric splanchnic arterial occlusive disease is uncommon and a rare cause of clinically relevant intestinal ischemia. This study was undertaken to better define the clinical manifestations and appropriate treatment of celiac artery (CA) and superior mesenteric artery (SMA) occlusive disease in children.

Methods: Clinical courses of 30 consecutive children undergoing operations for splanchnic arterial occlusive disease at the University of Michigan from 1992 to 2017 were retrospectively analyzed.

Arterial reconstructions for chronic lower extremity ischemia in preadolescent and adolescent children.

Objective: Chronic lower extremity ischemia in pediatric patients is uncommon. The intent of this study was to better define the arterial reconstructive options and their long-term durability in preadolescent and adolescent children having clinically relevant arterial occlusions affecting the lower extremity.

Methods: The medical records of 33 consecutive pediatric patients who underwent lower extremity revascularization for chronic ischemia at the University of Michigan from 1974 to 2016 were reviewed.

Pancreaticoduodenal and Gastroduodenal Artery Aneurysms Associated with Celiac Artery Occlusive Disease.

Background: The purpose of this study is to better define the clinical relevance of aneurysms affecting collateral vessels in patients with celiac artery (CA) occlusive disease.

Methods: True pancreaticoduodenal artery (PDA) and gastroduodenal artery (GDA) aneurysms associated with CA stenoses or occlusions reported from 1970 to 2010 in the English literature and similar cases treated at the University of Michigan were reviewed. Clinical presentations and differing treatment modalities were documented and analyzed.

Surgical treatment of abdominal aortic aneurysms in infancy and early childhood.

Objective: Pediatric abdominal aortic aneurysms (AAAs) are rare. The intent of this report was to review the presentation and surgical management of AAAs in infancy and early childhood.

Methods: The clinical courses of young children undergoing AAA surgery were subjected to a retrospective review and analysis.

Infantile Renovascular Hypertension with Failure to Thrive.

Severe hypertension in infancy is a rare cause of failure to thrive. The successful surgical management of this disease in an infant having refractory renovascular hypertension and growth failure is reported.

Pediatric nonaortic arterial aneurysms.

Objective: Pediatric arterial aneurysms are extremely uncommon. Indications for intervention remain poorly defined and treatments vary. The impetus for this study was to better define the contemporary surgical management of pediatric nonaortic arterial aneurysms.

Remedial operations for failed endovascular therapy of 32 renal artery stenoses in 24 children.

Background: Percutaneous transluminal angioplasty (PTA) for the treatment of pediatric renovascular hypertension (RVH) in contemporary practice is accompanied with ill-defined complications. This study examines the mode of pediatric renal PTA failures and the results of their surgical management.

Methods: Twenty-four children underwent remedial operations at the University of Michigan from 1996 to 2014 for failures of renal PTA.