Publications by authors named "William D Edwards"

134 Publications

International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.

Radiol Cardiothorac Imaging 2021 Aug 22;3(4):e200496. Epub 2021 Jul 22.

St Paul's Hospital, University of British Columbia, Vancouver, Canada.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes. ©  2021 Jointly between the RSNA, the European Association for Cardio-Thoracic Surgery, The Society of Thoracic Surgeons, and the American Association for Thoracic Surgery. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. All rights reserved. Bicuspid Aortic Valve, Aortopathy, Nomenclature, Classification.
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http://dx.doi.org/10.1148/ryct.2021200496DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8424700PMC
August 2021

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

J Thorac Cardiovasc Surg 2021 Sep 22;162(3):e383-e414. Epub 2021 Jul 22.

St Paul's Hospital, University of British Columbia, Vancouver, Canada.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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http://dx.doi.org/10.1016/j.jtcvs.2021.06.019DOI Listing
September 2021

Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.

J Thorac Cardiovasc Surg 2021 09 22;162(3):781-797. Epub 2021 Jul 22.

St Paul's Hospital, University of British Columbia, Vancouver, Canada; aeCedars Sinai Heart Institute, Los Angeles, Calif; afDivision of Cardiology, Columbia University Irving Medical Center/NY Presbyterian Hospital, New York, NY.

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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http://dx.doi.org/10.1016/j.jtcvs.2021.05.008DOI Listing
September 2021

Summary: International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.

Ann Thorac Surg 2021 09 22;112(3):1005-1022. Epub 2021 Jul 22.

St Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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http://dx.doi.org/10.1016/j.athoracsur.2021.05.001DOI Listing
September 2021

International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.

Ann Thorac Surg 2021 09 22;112(3):e203-e235. Epub 2021 Jul 22.

St Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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http://dx.doi.org/10.1016/j.athoracsur.2020.08.119DOI Listing
September 2021

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

Eur J Cardiothorac Surg 2021 Sep;60(3):448-476

St Paul's Hospital, University of British Columbia, Vancouver, Canada.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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http://dx.doi.org/10.1093/ejcts/ezab038DOI Listing
September 2021

Summary: international consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

Eur J Cardiothorac Surg 2021 Sep;60(3):481-496

St Paul's Hospital, University of British Columbia, Vancouver, Canada.

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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http://dx.doi.org/10.1093/ejcts/ezab039DOI Listing
September 2021

Amyloidosis in surgically resected atrial appendages: a study of 345 consecutive cases with clinical implications.

Mod Pathol 2020 05 13;33(5):764-774. Epub 2019 Nov 13.

Department of Laboratory Medicine & Pathology, Mayo Clinic, Rochester, MN, USA.

Histomorphologic parameters of atrial appendages removed during the Cox-Maze procedure have been shown to correlate with recurrence of atrial fibrillation. While amyloid deposition has been noted within atrial appendages, the incidence and significance remains incompletely understood. More accurate amyloid typing methodologies and targeted pharmacotherapeutics have recently been developed, prompting pathologists to provide more detailed information about the type of amyloid identified in such samples. This study sought to fully characterize the morphologic characteristics of atrial amyloid as well as its incidence and clinical significance. Tissue archives were queried for atrial appendages removed during the cardiac surgeries (2010-2014). Patient demographics, imaging features, and salient clinical findings were recorded. Pattern and extent of amyloid deposition were recorded. Typing of the amyloid protein, when present, was performed on a subset of cases by laser capture microdissection with mass spectrometry-based proteomic analysis. A total of 383 atrial appendages from 345 consecutive patients were included in the study (mean age, 69 years; range, 26-92 years). Amyloid was present in 46% of patients. A linear relationship was observed between age and presence of atrial amyloidosis. Women were more likely to have atrial amyloidosis. Two distinct morphologies of amyloid were observed: filamentous and nonfilamentous, and correlated perfectly with amyloid type (filamentous = AANF-type amyloid; nonfilamentous = ATTR-type amyloid). Filamentous deposits were observed in 91% of those with amyloid. Amyloid was more likely to be found in the left atrial appendage than the right. Patients with atrial amyloid, irrespective of type, were more likely to have experienced stroke or TIA and more likely to have atrial arrhythmia preoperatively. Postoperatively, those with atrial amyloid are more likely to experience recurrence of arrhythmia than those who did not have atrial amyloid. Understanding the morphologic characteristics of AANF-type amyloid will allow for identification by the light microscopy and obviates the need for expensive ancillary typing techniques. The finding of nonfilamentous amyloid, should still prompt confirmation of amyloid type so that targeted therapy may be employed.
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http://dx.doi.org/10.1038/s41379-019-0407-5DOI Listing
May 2020

Neoplastic embolization to systemic and pulmonary arteries.

J Vasc Surg 2018 07 1;68(1):204-212.e7. Epub 2018 Mar 1.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minn; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minn. Electronic address:

Objective: Arterial neoplastic emboli are uncommon, accounting for <1% of thromboemboli in the current literature. Nonetheless, this event may be associated with significant morbidity and mortality. Herein, we report a series of 11 cases of arterial neoplastic emboli from a single tertiary care center along with a comprehensive review of the literature to date. The aim of this study was to document the incidence, clinical presentations, and complications of arterial neoplastic emboli as well as to highlight the importance of routine histologic examination of thrombectomy specimens.

Methods: Pathology archives from a single tertiary care institution were queried to identify cases of surgically resected arterial emboli containing neoplasm (1998-2014). Histopathology was reviewed for confirmation of diagnosis. Patient demographics and oncologic history were abstracted from the medical record. Comprehensive literature review documented 332 patients in 275 reports (1930-2016).

Results: Eleven patients (six men) with a median age of 63 years (interquartile range, 42-71 years) were identified through institutional archives. Embolism was the primary form of diagnosis in seven (64%) cases. Cardiac involvement (primary or metastasis) was present in more than half of the cohort. Comprehensive literature review revealed that pulmonary primaries were the most common anatomic origin of arterial neoplastic emboli, followed by gastrointestinal neoplasia. Cardiac involvement was present in 18% of patients, and sentinel identification of neoplasia occurred in 30% of cases. Postmortem evaluation was the primary means of diagnosis in 27%.

Conclusions: This study highlights the importance of routine histopathologic evaluation of embolectomy specimens in patients with and without documented neoplasia.
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http://dx.doi.org/10.1016/j.jvs.2017.09.045DOI Listing
July 2018

Global Pulmonary Vascular Remodeling in Pulmonary Hypertension Associated With Heart Failure and Preserved or Reduced Ejection Fraction.

Circulation 2018 04 15;137(17):1796-1810. Epub 2017 Dec 15.

Department of Cardiovascular Medicine (A.U.F., J.J.M., E.A.K., B.A.B., R.P.F., M.M.F.)

Background: We hypothesized that pulmonary venous hypertension in heart failure (HF) leads to predominate remodeling of pulmonary veins and that the severity of venous remodeling is associated with the severity of pulmonary hypertension (PH) in HF.

Methods: Patients with HF (n=108; 53 preserved and 55 reduced ejection fraction) with PH (HF-PH; pulmonary artery systolic pressure [PASP] ≥40 mm Hg) were compared to normal controls (n=12) and patients with primary pulmonary veno-occlusive disease (PVOD; n=17). In lung specimens from autopsy (control, HF-PH, and 7 PVOD) or surgery (10 PVOD), quantitative histomorphometry was performed in all analyzable arteries (n=4949), veins (n=7630), and small indeterminate vessels (IV; n=2168) to define percent medial thickness (arteries) and percent intimal thickness (%IT) (arteries, veins, and IV) relative to external diameter.

Results: The average arterial percent medial thickness (control, 6.9; HF-PH, 11.0; PVOD, 15.0), arterial %IT (control, 4.9; HF-PH, 14.9; PVOD, 31.1), venous %IT (control, 14.0; HF-PH, 24.9; PVOD, 43.9), and IV %IT (control, 10.6; HF-PH, 25.8; PVOD, 50.0) in HF-PH were higher than controls (<0.0001 for all) but lower than PVOD (≤0.005 for all). PASP (mm Hg) was lower in HF-PH (median, 59 [interquartile range, 50-70]) than in PVOD (median, 91 [interquartile range, 82-103]). PASP correlated with arterial percent medial thickness (=0.41) and arterial %IT (=0.35) but more strongly with venous %IT (=0.49) and IV %IT (=0.55) (<0.0001 for all). Associations between PASP and venous or IV %IT remained significant after adjusting for arterial percent medial thickness and %IT and did not vary by HF type. In patients with right heart catheterization (30 HF-PH, 14 PVOD), similar associations between the transpulmonary gradient and pulmonary vascular remodeling existed, with numerically stronger associations for venous and IV %IT. Although the PASP was slightly higher in patients with HF-PH with right ventricular dysfunction, pulmonary vascular remodeling was not more severe. Pulmonary vascular remodeling severity was associated with reductions in the diffusing capacity of the lungs.

Conclusions: In HF, PH is associated with global pulmonary vascular remodeling, but the severity of PH correlates most strongly with venous and small IV intimal thickening, similar to the pattern observed in PVOD. These findings expand our understanding of the pathobiology of PH in HF.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.031608DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5915920PMC
April 2018

Myocardial Cobalt Levels Are Elevated in the Setting of Total Hip Arthroplasty.

J Bone Joint Surg Am 2017 Nov;99(22):e118

1College of Medicine (T.C.W. and S.P.W.) and Departments of Orthopedic Surgery (C.C.W. and R.T.T.), Laboratory Medicine & Pathology (M.C.B., M.S.A., A.F., P.L.D., D.L.M., W.D.E., and J.J.M.), Health Sciences Research (S.M.J.), and Cardiovascular Diseases (N.S.A. and J.J.M.), Mayo Clinic, Rochester, Minnesota.

Background: Arthroplasty implants commonly contain elemental metal that may undergo wear-related release. Recently, cases of hip implant-associated myocardial injury have been reported. However, we are not aware of any previous study that has systematically measured myocardial metal levels or examined the relationship with total hip arthroplasty (THA).

Methods: Archives of our institution were queried for autopsies of individuals who had undergone THA between 1990 and 2013. Myocardial tissue samples were analyzed for cobalt (Co) and chromium (Cr) levels with inductively coupled plasma mass spectroscopy. Seventy-five Co/Cr-on-polyethylene THA cases were included (mean age at time of death = 77.4 years; 49% women) as were 73 non-arthroplasty controls matched for age, sex, and history of hypertension and diabetes mellitus.

Results: Significantly higher median myocardial concentrations of Co were observed in individuals with THA compared with controls (0.12 versus 0.06 μg/g, p < 0.0001). The median Co concentration was 69% higher in patients who had undergone THA revision (0.169 μg/g) than in those who underwent primary THA (0.100 μg/g; p = 0.004). In general, higher Co levels were observed in those with multiple replaced joints, although this finding only trended toward significance. Cardiomegaly, interstitial fibrosis, and decreased ejection fraction were observed more frequently in the postmortem samples of patients with implants than in those of controls (p = 0.0002, 0.044, and 0.0039, respectively).

Conclusions: We believe this to be the first study to quantify metal levels in cardiac tissue in patients with and without joint replacement. The elevated Co levels, in concert with cardiomegaly and increased interstitial fibrosis found during autopsy, in the arthroplasty cohort are novel, important findings. Although Co levels were significantly elevated above those in controls, the majority were below those seen in clinical case reports of death from Co cardiotoxicity associated with metal-on-metal prostheses.

Level Of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.
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http://dx.doi.org/10.2106/JBJS.17.00159DOI Listing
November 2017

Comparative study of bicuspid vs. tricuspid aortic valve stenosis.

Eur Heart J Cardiovasc Imaging 2018 01;19(1):3-8

Department of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

Aims: To examine differences and similarities in bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) stenosis occurring during the same age and whether any differences impact outcomes following aortic valve replacement (AVR).

Methods And Results: An age-matched cohort of 198 BAV stenosis and 198 TAV stenosis patients was identified from 888 consecutive patients undergoing AVR for severe AS. Mean age 68 ± 6 years; 68% male. Patients with BAV were less likely to have multiple comorbidities, as measured by the Charlson Comorbidity Index (CCI) >2 (3 vs. 10%, P = 0.007). Indexed aortic valve area (0.44 ± 0.09 vs. 0.42 ± 0.08 cm2/m2, P = 0.17) and left ventricular ejection fraction (LVEF) were similar (62 ± 11 vs. 61 ± 12%, P = 0.12), but E/e' ≥15 (46 vs. 66%, P = 0.0002) was less common in BAV stenosis. A tissue aortic valve prosthesis was more commonly utilized in both groups (81 vs. 78%, P = 0.54). Overall indexed effective orifice area was larger in BAV compared with TAV (1.08 ± 0.33 vs. 0.96 ± 0.25 cm2/m2, P = 0.0008). Five-year survival following AVR was lower in TAV compared with BAV stenosis (61 vs. 79%, P = 0.02). Independent predictors of survival following AVR were LVEF < 50% [hazard ratio (HR): 4.8, P = 0.0005], CCI > 2 (HR: 3.1, P = 0.015), effective orifice area index ≤0.85 cm2/m2 (HR: 2.5, P = 0.004), and bioprosthesis (HR: 3.7, P = 0.02).

Conclusion: In an age-matched cohort, TAV compared with BAV stenosis is associated with greater prevalence of cardiovascular risk factors and cardiac impairment and worse survival after AVR.
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http://dx.doi.org/10.1093/ehjci/jex211DOI Listing
January 2018

Clinical presentation and echocardiographic diagnosis of postinfarction papillary muscle rupture: A review of 22 cases.

Echocardiography 2017 Jul 30;34(7):973-977. Epub 2017 May 30.

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

Background: Acute myocardial infarctions (AMI) continue to be common in the United States. Mechanical complications of AMI can lead to cardiogenic shock (CS) and death. The aim of this study was to review the cases of papillary muscle ruptures in the setting of myocardial infarctions at a tertiary care center, with a focus on the clinical presentation and echocardiographic diagnosis.

Methods: This was a retrospective study from January 1, 2000 through December 31, 2014. In all, 22 patients with AMI and papillary muscle rupture (AMI-PMR) who had surgical intervention were identified.

Results: The average age was 70 (±11) with 16 (73%) males. Six patients presented with ST-elevation myocardial infarctions (STEMI) and all underwent emergent revascularization with primary percutaneous coronary intervention (PCI) prior to the diagnosis of AMI-PMR. The other 16 patients presented with a non-STEMI. In total, 17 (77%) of the 22 patients were diagnosed with an AMI-PMR within 7 days from their onset of symptoms. In all, 12 patients (55%) had anterolateral papillary muscle ruptures (ALPMR), and the other 10 had posteromedial papillary muscle ruptures (PMPMR). Ruptures were complete in 10 patients (45%). Patients presented with pulmonary edema early (<7 days) more commonly than late (>14 days). Transthoracic echocardiography was able to demonstrate severe mitral regurgitation in 86% and a definitive or suggestive diagnosis in 93%. All 22 patients survived to operative management, and the overall in-hospital mortality rate was 9%.

Conclusion: In conclusion, ischemic papillary muscle ruptures continue to occur, but with prompt diagnosis by echocardiography and rapid surgical management, the mortality rate continues to decline.
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http://dx.doi.org/10.1111/echo.13585DOI Listing
July 2017

Quadricuspid Aortic Valve: Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes.

Circulation 2016 Jan 3;133(3):312-9. Epub 2015 Dec 3.

From Divisions of Cardiovascular Diseases (M.Y.C.T., N.M.A., V.T.N., P.A.P.) and Anatomic Pathology (W.D.E.), Mayo Clinic, Rochester, MN; and Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ (M.M.A., T.Z.N.).

Background: Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes.

Methods And Results: Fifty patients (mean ± SD age, 43.5 ± 21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean ± SD follow-up of 4.8 ± 5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years.

Conclusions: Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.115.017743DOI Listing
January 2016

Trends in Coronary Atherosclerosis: A Tale of Two Population Subgroups.

Am J Med 2016 Mar 6;129(3):307-14. Epub 2015 Nov 6.

Department of Health Sciences Research, Mayo Clinic, Rochester, Minn. Electronic address:

Background: We previously investigated trends in subclinical coronary artery disease and associated risk factors among autopsied non-elderly adults who died from nonnatural causes. Although grade of atherosclerosis declined from 1981 through 2009, the trend was nonlinear, ending in 1995, concurrent with increasing obesity/diabetes in this population. The previous study used linear regression and examined trends for all 4 major epicardial coronary arteries combined. The present investigation of coronary artery disease trends for the period 1995 through 2012 was prompted by a desire for more detailed examination of more recent coronary artery disease trends in light of reports that the epidemics of obesity and diabetes have slowed and are perhaps ending.

Methods: This population-based series of cross-sectional investigations identified all Olmsted County, Minnesota residents aged 16-64 years who died 1995 through 2012 (N = 2931). For decedents with nonnatural manner of death, pathology reports were reviewed for grade of atherosclerosis assigned each major epicardial coronary artery. Using logistic regression, we estimated calendar-year trends in grade (unadjusted and age- and sex-adjusted) for each artery, initially as an ordinal measure (range, 0-4); then, based on evidence of nonproportional odds, as a dichotomous variable (any atherosclerosis, yes/no) and as an ordinal measure for persons with atherosclerosis (range, 1-4).

Results: Of 474 nonnatural deaths, 453 (96%) were autopsied; 426 (90%) had coronary stenosis graded. In the ordinal-logistic model for trends in coronary artery disease grade (range, 0-4), the proportional odds assumption did not hold. In subsequent analysis as a dichotomous outcome (grades 0 vs 1-4), each artery exhibited a significant temporal decline in the proportion with any atherosclerosis. Conversely, for subjects with coronary artery disease grade 1-4, age- and sex-adjusted ordinal regression revealed no change over time in 2 arteries and statistically significant temporal increases in severity in 2 arteries.

Conclusions: Findings suggest that efforts to prevent coronary artery disease onset have been relatively successful. However, statistically significant increases in the grade of atherosclerosis in 2 arteries among persons with coronary artery disease may be indicative of a major public health challenge.
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http://dx.doi.org/10.1016/j.amjmed.2015.10.032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4755914PMC
March 2016

Sex-related differences in calcific aortic stenosis: correlating clinical and echocardiographic characteristics and computed tomography aortic valve calcium score to excised aortic valve weight.

Eur Heart J 2016 Feb 27;37(8):693-9. Epub 2015 Oct 27.

Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

Aims: Calcific aortic valve stenosis (AS) is purportedly associated with less calcium burden in women than in men. We sought to examine sex-related differences and correlates of surgically excised aortic valve weight (AVW) in pure AS.

Methods And Results: Clinical and echocardiographic characteristics of 888 consecutive patients who underwent aortic valve replacement for severe AS were correlated to AVW, and in 126 patients, AVW was also correlated to computed tomography aortic valve calcium (AVC) score. Women and men had similar indexed valve area (0.42 ± 0.09 vs. 0.42 ± 0.07 cm (2)/m(2), P = 0.95) and mean systolic gradient (53 ± 15 vs. 52 ± 13 mmHg, P = 0.11), but women had higher New York Heart Association class (2.63 ± 0.70 vs. 2.50 ± 0.70, P = 0.01) and less prevalent coronary artery disease (38 vs. 52%, P < 0.0001). Aortic valve weight was lower in women (1.94 ± 0.88 vs. 3.08 ± 1.32 g, P < 0.0001) even when indexed to body surface area (1.09 ± 0.48 vs. 1.48 ± 0.62 g/m(2), P < 0.0001) or left ventricular outflow tract (LVOT) area (0.54 ± 0.23 vs. 0.71 ± 0.29 g/cm(2), P < 0.0001). Using multivariate analysis, male sex (P < 0.0001), bicuspid valve (P < 0.0001), and larger LVOT area (P < 0.0001) were the major determinants of increased AVW, along with current cigarette smoking (P = 0.007). Diabetes (P = 0.004) and hypertension (P = 0.03) were independently associated with lower AVW. Aortic valve calcium correlated well with AVW (r = 0.81, P < 0.0001) and was lower in women than in men (2520 ± 1199 vs. 3606 ± 1632 arbitrary units, P < 0.0001).

Conclusions: Despite the same degree of AS severity, women have less AVC and lower AVW compared with men, irrespective of valve morphology. Aortic valve calcium is correlated to excised AVW. Hypertension, diabetes, and current cigarette smoking were independently associated with AVW.
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http://dx.doi.org/10.1093/eurheartj/ehv560DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370337PMC
February 2016

Echocardiographic Features of Cardiac Angiosarcomas: The Mayo Clinic Experience (1976-2013).

Echocardiography 2016 Feb 13;33(2):186-92. Epub 2015 Oct 13.

Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.

Objective: Cardiac angiosarcoma is the most common primary malignant cardiac tumor. The dismal prognosis and nonspecific symptomatology underscore the need for an accurate and cost-effective approach to the identification and characterization of this rare tumor.

Methods: Mayo Clinic tissue registry archives were queried for all histologically confirmed cases of cardiac angiosarcoma (1976-2013) with available imaging data. Echocardiograms were retrospectively reviewed.

Results: Thirty-three cases of cardiac angiosarcoma were identified; of these, 17 had echocardiograms available (mean age, 46 years; six men). Transthoracic echocardiography (TTE) as the initial diagnostic test had 75% sensitivity for visualizing primary cardiac angiosarcoma (9/12 patients). Tumor extension into the pericardium was common and pericardial effusion was present in 15 patients (88%); however, pericardial fluid cytology was negative for malignancy in all tested patients (n = 15). Left ventricular ejection fraction (LVEF) was preserved in 16 patients (94%) (average LVEF, 62%). Right ventricular function was mildly reduced in two patients (12%) at initial presentation. Tricuspid valve obstruction was present in three patients (18%; mean diastolic gradient, 6.3 mmHg [range, 3-11 mmHg]).

Conclusion: The sensitivity of TTE as the first diagnostic imaging modality compared favorably with computed tomography. Pericardial effusion was common, but pericardial fluid cytology was negative in all patients who underwent pericardiocentesis. The absence of a stalk was a universal finding that may help distinguish angiosarcoma from benign, primarily pedunculated tumors such as myxoma and papillary fibroelastoma.
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http://dx.doi.org/10.1111/echo.13060DOI Listing
February 2016

Correlation of histomorphological pattern of cardiac amyloid deposition with amyloid type: a histological and proteomic analysis of 108 cases.

Histopathology 2016 Apr 28;68(5):648-56. Epub 2015 Sep 28.

Department of Laboratory Medicine & Pathology, Mayo Clinic, Rochester, MN, USA.

Aims: Prognostication and treatment selection for cardiac amyloidosis requires accurate amyloid typing. The aim of this study was to investigate the utility of histomorphology for predicting type.

Methods And Results: Autopsy cases with cardiac amyloidosis (1998-2010) were typed by the use of mass spectrometry-based proteomics. Deposition patterns were correlated with amyloid type. Among 108 decedents (mean age 75 years; 69% men), 107 had a single type, including transthyretin (ATTR) (60 cases), amyloid light chain (AL) (32 λ; 12 κ), amyloid A (AA) (two), and apolipoprotein AIV (AApoAIV) (one). Interstitial deposition was more extensive in AL amyloidosis cases than in ATTR cases [odds ratio (OR) 6.8, P = 0.0004]. Histomorphological patterns of interstitial deposition were mixed in 61% of AL amyloidosis cases and in 61% of ATTR cases, but diffuse pericellular deposits favoured AL amyloidosis (OR 10.7, P = 0.0001), nodular deposits favoured ATTR (OR 3.1, P = 0.0229), and discrete pericellular deposits tended to partially favour ATTR (OR 1.7, P = 0.1970). Arterial and venous deposits each favoured AL amyloidosis (OR ranging from 9.3 to 192.0, P-value ranging from 0.0022 to <0.0001), and were severe in AL amyloidosis. Endocardial deposits favoured AL amyloidosis (OR 46.3, P < 0.0001) and were also more severe in AL amyloidosis.

Conclusions: The extent and distribution of cardiac amyloidosis strongly correlate with amyloid type, suggesting fundamental differences in the pathobiology of deposition. The tendency for mixed patterns to occur limits the practicality and accuracy of using histopathology for amyloid typing.
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http://dx.doi.org/10.1111/his.12793DOI Listing
April 2016

Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas.

J Am Coll Cardiol 2015 Jun;65(22):2420-9

Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Papillary fibroelastomas (PFE) are benign neoplasms with little available outcome data.

Objectives: This study sought to describe the frequency and clinical course of patients with surgically removed PFE and echocardiographically suspected, but unoperated, PFE.

Methods: Mayo Clinic pathology and echocardiography databases (January 1, 1995, to December 31, 2010) were queried, resulting in 511 patients: group 1 (n = 185), including patients with surgically removed, histopathologically confirmed PFE; group 1a (n = 94; 51%) with PFE removed at primary surgery; and group 1b (n = 91; 49%) with PFE removal at time of another cardiac surgery. Group 2 (n = 326) patients had echocardiographic evidence of PFE but no cardiac surgery to remove PFE.

Results: Group 1 had mean age of 63 ± 14 years (116 women [63%]). During the study period, we identified 112 cardiac myxomas in the pathology database and 142 in the echocardiographic database. Mean age in group 2 was 67 ± 14 years (162 women [50%]). PFE occurred most commonly on cardiac valves (n = 400 [78%]). In group 1, transient ischemic attack or stroke was the presenting symptom in 58 patients (32%). With surgical removal of valvular PFE, the valve was preserved in 92 (98%). Recurrence was documented in 3 patients (1.6%). Follow-up stroke risk in groups 1, 1a, and 1b at 1 year was 2%, 0%, and 4%; at 5 years, 8%, 5%, and 11%, respectively. Cerebrovascular accident risk in group 2 at 1 and 5 years was 6% and 13%.

Conclusions: In patients with echocardiographically suspected PFE who do not undergo surgical removal, rates of cerebrovascular accident and mortality are increased.
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http://dx.doi.org/10.1016/j.jacc.2015.03.569DOI Listing
June 2015

Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas.

J Am Coll Cardiol 2015 Jun;65(22):2420-9

Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Papillary fibroelastomas (PFE) are benign neoplasms with little available outcome data.

Objectives: This study sought to describe the frequency and clinical course of patients with surgically removed PFE and echocardiographically suspected, but unoperated, PFE.

Methods: Mayo Clinic pathology and echocardiography databases (January 1, 1995, to December 31, 2010) were queried, resulting in 511 patients: group 1 (n = 185), including patients with surgically removed, histopathologically confirmed PFE; group 1a (n = 94; 51%) with PFE removed at primary surgery; and group 1b (n = 91; 49%) with PFE removal at time of another cardiac surgery. Group 2 (n = 326) patients had echocardiographic evidence of PFE but no cardiac surgery to remove PFE.

Results: Group 1 had mean age of 63 ± 14 years (116 women [63%]). During the study period, we identified 112 cardiac myxomas in the pathology database and 142 in the echocardiographic database. Mean age in group 2 was 67 ± 14 years (162 women [50%]). PFE occurred most commonly on cardiac valves (n = 400 [78%]). In group 1, transient ischemic attack or stroke was the presenting symptom in 58 patients (32%). With surgical removal of valvular PFE, the valve was preserved in 92 (98%). Recurrence was documented in 3 patients (1.6%). Follow-up stroke risk in groups 1, 1a, and 1b at 1 year was 2%, 0%, and 4%; at 5 years, 8%, 5%, and 11%, respectively. Cerebrovascular accident risk in group 2 at 1 and 5 years was 6% and 13%.

Conclusions: In patients with echocardiographically suspected PFE who do not undergo surgical removal, rates of cerebrovascular accident and mortality are increased.
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http://dx.doi.org/10.1016/j.jacc.2015.03.569DOI Listing
June 2015

Novel association of elastofibroma with aortic stenosis: report of a case report interfering with a thoracotomy procedure and a reassessment of typical patient demographics and tumor location.

J Thorac Oncol 2015 Apr;10(4):e18-20

Departments of *Surgery-Thoracic Surgery, †Opthalmology, ‡Pathology, §Upstate Heart and Vascular Center, and ‖Radiology, State University of New York, Upstate Medical University, Syracuse, New York; and ¶Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, New York.

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http://dx.doi.org/10.1097/JTO.0000000000000452DOI Listing
April 2015

Coronary microvascular rarefaction and myocardial fibrosis in heart failure with preserved ejection fraction.

Circulation 2015 Feb 31;131(6):550-9. Epub 2014 Dec 31.

From the Division of Cardiovascular Diseases (S.F.M., S.H., M.M.R.), Mayo Graduate School (S.F.M.), Mayo Medical School (S.A.M.), and Division of Anatomic Pathology (W.D.E., J.J.M.), Mayo Clinic, Rochester, MN.

Background: Characterization of myocardial structural changes in heart failure with preserved ejection fraction (HFpEF) has been hindered by the limited availability of human cardiac tissue. Cardiac hypertrophy, coronary artery disease (CAD), coronary microvascular rarefaction, and myocardial fibrosis may contribute to HFpEF pathophysiology.

Methods And Results: We identified HFpEF patients (n=124) and age-appropriate control subjects (noncardiac death, no heart failure diagnosis; n=104) who underwent autopsy. Heart weight and CAD severity were obtained from the autopsy reports. With the use of whole-field digital microscopy and automated analysis algorithms in full-thickness left ventricular sections, microvascular density (MVD), myocardial fibrosis, and their relationship were quantified. Subjects with HFpEF had heavier hearts (median, 538 g; 169% of age-, sex-, and body size-expected heart weight versus 335 g; 112% in controls), more severe CAD (65% with ≥1 vessel with >50% diameter stenosis in HFpEF versus 13% in controls), more left ventricular fibrosis (median % area fibrosis, 9.6 versus 7.1) and lower MVD (median 961 versus 1316 vessels/mm(2)) than control (P<0.0001 for all). Myocardial fibrosis increased with decreasing MVD in controls (r=-0.28, P=0.004) and HFpEF (r=-0.26, P=0.004). Adjusting for MVD attenuated the group differences in fibrosis. Heart weight, fibrosis, and MVD were similar in HFpEF patients with CAD versus without CAD.

Conclusions: In this study, patients with HFpEF had more cardiac hypertrophy, epicardial CAD, coronary microvascular rarefaction, and myocardial fibrosis than controls. Each of these findings may contribute to the left ventricular diastolic dysfunction and cardiac reserve function impairment characteristic of HFpEF.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.114.009625DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4324362PMC
February 2015

Impact of incidental amyloidosis on the prognosis of patients with hypertrophic cardiomyopathy undergoing septal myectomy for left ventricular outflow tract obstruction.

Am J Cardiol 2014 Nov 12;114(9):1396-9. Epub 2014 Aug 12.

Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota.

To determine the impact of amyloid on the prognosis of patients with hypertrophic cardiomyopathy (HC), we reviewed outcomes of patients who underwent septal myectomy for HC from March 7, 1996, to October 9, 2012, with amyloid deposits identified in operative specimens. Amyloid subtypes were differentiated by mass spectrometry-based proteomics. The survival rate was compared with that of an age-matched population (2:1) without amyloid who underwent septal myectomy for HC. Sixteen patients (mean age ± SD 71 ± 8 years; 12 men) met study criteria. All 16 had intraventricular peak systolic gradients reduced intraoperatively from 105 ± 53 mm Hg to 3 ± 7 mm Hg (p <0.001). Amyloid deposits in specimens ranged from minimal to mild. Nine patients had senile (transthyretin-type) amyloidosis, 4 had immunoglobulin-associated amyloidosis, 2 had apolipoprotein A4 amyloidosis type, and 1 had serum amyloid A type. There were no deaths before 30 days. Twelve patients had New York Heart Association class III or IV function preoperatively, and at last follow-up (median 3 years), class I or II. Only 1 patient received postoperative amyloidosis treatment. The postoperative survival rate at 2 and 4 years was 100% (n = 11 at risk) and 91% (n = 6 at risk), respectively, similar to that of the age-matched population with HC without amyloid who underwent myectomy (p = 0.13). Patients undergoing septal myectomy for HC who have histologic evidence of mild amyloidosis have early outcomes and midterm survival similar to those of patients with HC without amyloidosis who undergo myectomy. In conclusion, although longer follow-up is necessary, small amounts of amyloid, regardless of subtype, do not confer a poor prognosis on patients with HC who undergo septal myectomy.
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http://dx.doi.org/10.1016/j.amjcard.2014.07.058DOI Listing
November 2014

Electrogram guidance: a method to increase the precision and diagnostic yield of endomyocardial biopsy for suspected cardiac sarcoidosis and myocarditis.

JACC Heart Fail 2014 Oct 3;2(5):466-73. Epub 2014 Sep 3.

Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Department of Pediatrics and Adolescent Medicine Mayo Clinic, Rochester, Minnesota. Electronic address:

Objectives: The aim of this study was to describe the method used to perform electrogram-guided EMB and correlate electrogram characteristics with pathological and clinical outcomes.

Background: Endomyocardial biopsy (EMB) is valuable in determining the underlying etiology of a cardiomyopathy. The sensitivity, however, for focal disorders, such as lymphocytic myocarditis and cardiac sarcoidosis (CS), is low. The sensitivity of routine fluoroscopically guided EMB is low. Abnormal intracardiac electrograms are seen at sites of myocardial disease. However, the exact value of electrogram-guided EMB is unknown.

Methods: We report 11 patients who underwent electrogram-guided EMB for evaluation of myocarditis and CS.

Results: Of 40 total biopsy specimens taken from 11 patients, 19 had electrogram voltage <5 mV, all of which resulted in histopathologic abnormality (100% specificity and positive predictive value). A voltage amplitude cutoff value of 5 mV had substantially higher sensitivity (70% vs. 26%) and negative predictive value (62%) than 1.5 mV. Abnormal electrogram appearance at biopsy site had good sensitivity (67%) and specificity (92%) in predicting abnormal myocardium. Normal signals with voltage >5 mV signified normal myocardium with no significant diagnostic yield. Biopsy results guided therapy in all patients, including 5 with active myocarditis or CS, all of whom subsequently received immunosuppressive therapy. There were no procedural complications.

Conclusions: In patients with suspected myocarditis or CS, electrogram-guided EMB targeting sites with abnormal or low-amplitude electrograms may increase the diagnostic yield for detecting abnormal pathological findings.
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http://dx.doi.org/10.1016/j.jchf.2014.03.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4459501PMC
October 2014

The role of Iex-1 in the pathogenesis of venous neointimal hyperplasia associated with hemodialysis arteriovenous fistula.

PLoS One 2014 18;9(7):e102542. Epub 2014 Jul 18.

Vascular and Interventional Radiology Translational Laboratory, Department of Radiology, University of Cincinnati, Cincinnati, Ohio, United States of America; Department of Biochemistry and Molecular Biology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, United States of America.

Arteriovenous fistulas (AVFs) used for hemodialysis fail because of venous neointimal hyperplasia (VNH). There are 1,500,000 patients that have end stage renal disease worldwide and the majority requires hemodialysis. In the present study, the role of the intermediate early response gene X-1 (IEX-1), also known as IER-3 in the pathogenesis of VNH was evaluated. In human samples removed from failed AVF, there was a significant increase in IEX-1 expression localized to the adventitia. In Iex-1-/- mice and wild type (WT) controls, chronic kidney disease was induced and an AVF placed 28 days later by connecting the carotid artery to jugular vein. The outflow vein was removed three days following the creation of the AVF and gene expression analysis demonstrated a significant decrease in vascular endothelial growth factor-A (Vegf-A) and monocyte chemoattractant protein-1 (Mcp-1) gene expression in Iex-1-/- mice when compared to WT mice (P<0.05). At 28 days after AVF placement, histomorphometric and immune-histochemical analyses of the outflow vein demonstrated a significant decrease in neointimal hyperplasia with an increase in average lumen vessel area associated with a decrease in fibroblast, myofibroblast, and Ly6C staining. There was a decrease in proliferation (Ki-67) and an increase in the TUNEL staining in Iex-1 KO mice compared to WT. In addition, there was a decrease in Vegf-A, Mcp-1, and matrix metalloproteiniase-9 (Mmp-9) staining. Iex-1 expression was reduced in vivo and in vitro using nanoparticles coated with calcitriol, an inhibitor of Iex-1 that demonstrated that Iex-1 reduction results in decrease in Vegf-A. In aggregate, these results indicate that the absence of IEX-1 gene results in reduced VNH accompanied with a decrease in proliferation, reduced fibroblast, myofibroblast, and Ly6C staining accompanied with increased apoptosis mediated through a reduction in Vegf-A/Mcp-1 axis and Mmp-9. Adventitial delivery of nanoparticles coated with calcitriol reduced Iex-1 and VNH.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0102542PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4103828PMC
November 2015

Left ventricular amyloid deposition in patients with heart failure and preserved ejection fraction.

JACC Heart Fail 2014 Apr;2(2):113-22

Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address:

Objectives: This study sought to determine the frequency of left ventricular amyloid in heart failure with preserved ejection fraction (HFpEF).

Background: Left ventricular amyloid deposition can cause diastolic dysfunction and HFpEF.

Methods: Autopsy of left ventricular specimens from patients with antemortem diagnosis of HFpEF without clinically apparent amyloid (n = 109) and from control subjects (n = 131) were screened with sulfated Alcian blue and subsequent Congo red staining with microdissection for mass spectrometry-based proteomics to determine amyloid type. Fibrosis was assessed with quantitative whole-field digital microscopy.

Results: The presence of wild-type transthyretin (wtTTR) amyloid was associated with age at death and male sex, but the age- and sex-adjusted prevalence of wtTTR amyloid was higher in HFpEF patients than in control subjects (odds ratio: 3.8, 95% confidence interval: 1.5 to 11.3; p = 0.03). Among HFpEF patients, moderate or severe interstitial wtTTR deposition, consistent with senile systemic amyloidosis as the primary etiology of HFpEF, was present in 5 (5%) patients (80% men), with mild interstitial and/or variable severity of intramural coronary vascular deposition in 13 (12%) patients. While, wtTTR deposition was often mild, adjusting for age and presence of HFpEF, wtTTR amyloid was associated with more fibrosis (p = 0.005) and lower age, sex, and body size-adjusted heart weight (p = 0.04).

Conclusions: Given the age- and sex-independent association of HFpEF and wtTTR deposition and an emerging understanding of the pathophysiology of the amyloidoses, the current findings support further investigation of the role of wtTTR in the pathophysiology of HFpEF.
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http://dx.doi.org/10.1016/j.jchf.2013.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3984539PMC
April 2014

PRKAR1A in the development of cardiac myxoma: a study of 110 cases including isolated and syndromic tumors.

Am J Surg Pathol 2014 Aug;38(8):1079-87

*Division of Anatomic Pathology, Mayo Clinic, Rochester, MN †Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA ‡Department of Pathology, Dalhousie University, Halifax, NS, Canada.

Cardiac myxoma usually occurs as a solitary mass, but occasionally develops as part of a familial syndrome, the Carney complex (CNC). Two thirds of CNC-associated cardiac myxomas exhibit mutations in PRKAR1A. PRKAR1A mutations occur in both familial and sporadic forms of CNC but have not been described in isolated (nonsyndromic) cardiac myxomas. A total of 127 consecutive cardiac myxomas surgically resected at Mayo Clinic (1993 to 2011) from 110 individuals were studied. Clinical, radiologic, and pathologic findings were reviewed. Of these, 103 patients had isolated cardiac myxomas, and 7 patients had the tumor as a component of CNC. Age and sex distributions were different for CNC (mean 26 y, range 14 to 44 y, 71% female) and non-CNC (mean 62 y, range 18 to 92 y, 63% female) patients. PRKAR1A immunohistochemical analysis (IHC) was performed, and myxoma cell reactivity was graded semiquantitatively. Bidirectional Sanger sequencing was performed in 3 CNC patients and 29 non-CNC patients, to test for the presence of mutations in all coding regions and intron/exon boundaries of the PRKAR1A gene. IHC staining showed that all 7 CNC cases lacked PRKAR1A antigenicity and that 33 (32%) isolated cardiac myxomas were similarly nonreactive. Of tumors subjected to sequencing analysis, 2 (67%) CNC myxomas and 9 (31%) non-CNC myxomas had pathogenic PRKAR1A mutations. No germline mutations were found in 4 non-CNC cases tested. PRKAR1A appears to play a role in the development of both syndromic and nonsyndromic cardiac myxomas. Routine IHC evaluation of cardiac myxomas for PRKAR1A expression may be useful in excluding a diagnosis of CNC.
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http://dx.doi.org/10.1097/PAS.0000000000000202DOI Listing
August 2014

Pneumatic compression for embolic protection during upper extremity endovascular intervention.

Vasc Endovascular Surg 2014 Jan 30;48(1):70-3. Epub 2013 Oct 30.

1Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

Embolic protection devices are occasionally employed during endovascular interventions to prevent complications caused by embolic debris. However, these devices have imperfect efficacy, confer risk of endovascular trauma, and are expensive. We report a patient with giant cell arteritis and symptomatic axillary artery stenosis, with a perceived elevated risk of distal embolization during endovascular intervention. We describe a straightforward embolic protection technique of brachial pressure cuff inflation during endovascular intervention and aspiration of displaced thrombotic material from the static column of blood. This novel, effective, and cost-free technique could also be employed in other vascular beds during endovascular intervention.
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http://dx.doi.org/10.1177/1538574413510622DOI Listing
January 2014

Type A aortic dissection in patients with bicuspid aortic valves: clinical and pathological comparison with tricuspid aortic valves.

Heart 2013 Nov 4;99(22):1668-74. Epub 2013 Sep 4.

Division of Cardiovascular Diseases, Mayo Clinic, , Rochester, Minnesota, USA.

Objective: Bicuspid aortic valve (BAV) is associated with a higher risk of type A aortic dissection (AD) compared with tricuspid aortic valve (TAV). We sought to study differences between patients with BAV and TAV with AD.

Design And Setting: Observational descriptive analysis of clinical, imaging and pathological characteristics of all patients with confirmed BAV and AD from 1980-2010, compared with a consecutive TAV group with AD.

Results: Of 47 patients with BAV (mean age 58 ± 14, 77% male), 31 (66%) had acute AD, 16 (34%) had chronic AD, 40 (85%) had typical BAV, 32 (68%) had hypertension and 11 (23%) had previous aortic coarctation repair. Of 53 patients with TAV (mean age 66 ± 13 (p=0.007), 76% male), 34 (66%) had acute AD (p=1.0) and 46 (87%) had hypertension (p=0.03). More patients with BAV had known aortic dilatation prior to AD (49% versus 17%, p=0.001). Presentation symptoms were identical between groups (p=NS). Maximal ascending aortic diameter at AD was higher in patients with BAV (66 ± 15 mm vs 56 ± 11 mm, p=0.0004). Previous aortic valve replacement (AVR) was more common in BAV (23% vs 6%, p=0.02). Of 11 patients with BAV with previous isolated AVR, 7 had ≥ moderate ascending aorta dilatation at the time of surgery. Patients with BAV had increased aortic jet velocity (28% vs 10%) and more severe aortic stenosis (19% vs 0%) at presentation (p=0.04 and 0.002, respectively). In acute AD, aortic medial degeneration affected 75% of BAV specimens and 41% TAV specimens (p=0.01) while aortic atherosclerosis was more frequent in TAV (56% vs 26%, p=0.02).

Conclusions: Compared with patients with TAV, patients with BAV with type A AD are younger, have less hypertension, more valve stenosis and previous AVR, higher maximal aortic dimension, worse aortic medial degeneration, high prevalence of aortic coarctation, and 1 out of 2 have known aortic dilatation prior to AD. Implementation of current guidelines could have theoretically prevented AD in several patients with BAV.
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http://dx.doi.org/10.1136/heartjnl-2013-304606DOI Listing
November 2013

Surgical pathology of hypothenar hammer syndrome with new pathogenetic insights: A 25-year institutional experience with clinical and pathologic review of 67 cases.

Am J Surg Pathol 2013 Nov;37(11):1700-8

Divisions of *Anatomic Pathology ‡Cardiovascular Diseases §Biomedical Statistics and Informatics †Department of Plastic and Reconstructive Surgery, Mayo Clinic, Rochester, MN.

Hypothenar hammer syndrome (HHS) is characterized by digital ischemia after repetitive traumatic injury to the ulnar artery. Some hypothesize that susceptible patients have an intrinsic vasculopathy such as fibromuscular dysplasia (FMD). To investigate this hypothesis, we reviewed our institutional experience with this syndrome over 25 years. Clinical records were reviewed from all patients who underwent surgical treatment for HHS (1987 to 2011), and histologic features of excised ulnar arteries were characterized. A total of 67 patients (mean age 45 y; range, 21 to 75 y; 65 men) were treated for unilateral or bilateral disease. Common symptoms included digital pain (96% of cases), cold intolerance (79%), cyanosis (70%), numbness (54%), tingling (51%), and ulceration (40%). Angiography showed ulnar artery occlusion (89%), irregularity (56%), tortuosity (46%), and digital emboli (89%). Common pathologic features (from 75 specimens) included: luminal thrombosis (87%); intimal thickening (60%) and fibrosis (57%); internal elastic membrane disruption (95%); medial fibrosis (96%), hypertrophy (43%), neovascularization (49%), dilatation (29%), and disruption (25%); and adventitial neovascularization (53%). Specific features of dysplasia were present in 10 cases (15%), including smooth muscle disorganization and additional smooth muscle bundles outside the external elastic membrane, but typical FMD was not identified. Histologic features in HHS most often represent secondary changes consistent with repetitive trauma. Dysplastic features can be found in occasional cases, but FMD does not appear to contribute to HHS in most patients. Angiography should be interpreted with caution in superficial locations, as a string-of-beads appearance may simply reflect a posttraumatic corkscrew deformity of the ulnar artery.
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http://dx.doi.org/10.1097/PAS.0b013e318297fa37DOI Listing
November 2013
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