Faculty Bibliography

Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Echocardiographic findings were consistent with worsening left ventricular outflow tract obstruction at rest. Cardiac magnetic resonance imaging revealed patchy areas of midwall late gadolinium enhancement. Right ventricular endomyocardial biopsy did not reveal amyloid deposition, and cardiac technetium-99m pyrophosphate scintigraphy did not suggest transthyretin amyloidosis. The patient underwent septal myectomy with resection of an accessory papillary muscle. Pathological examination of the myectomy specimen was consistent with HCM. In addition, there was a thick layer of diffuse endocardial and vascular amyloid deposition that was identified as AA type by laser-microdissection with liquid chromatography-coupled tandem-mass spectrometry. This case report highlights the presence of 2 distinct disease processes occurring simultaneously and the importance of tissue diagnosis of AA amyloidosis, a condition that is not commonly associated with HCM.

The relationships of osteocalcin (OC) and C-telopeptide of type I collagen (CTX) with long-term incidence of hip fracture were examined in 1680 post-menopausal women from a population-based study. CTX, but not OC, levels were associated with incident hip fracture in these participants, a relationship characterized by an inverted U-shape.

Background Hypertrophic cardiomyopathy is defined as unexplained left ventricular ( LV ) hypertrophy (wall thickness ≥15 mm) and is prevalent in 0.2% of adults (1:500) in population-based studies using echocardiography. Cardiac magnetic resonance imaging ( MRI ) allows for more accurate wall thickness measurement across the entire ventricle than echocardiography. The prevalence of unexplained LV hypertrophy by cardiac MRI is unknown. MESA (Multi-Ethnic Study of Atherosclerosis) recruited individuals without overt cardiovascular disease 45 to 84 years of age. Methods and Results We studied 4972 individuals who underwent measurement of regional LV wall thickness by cardiac MRI as part of the MESA baseline exam. American Heart Association criteria were used to define LV segments. We excluded participants with hypertension, LV dilation (≥95% predicted end-diastolic volume) or dysfunction (ejection fraction ≤50%), moderate-to-severe left-sided valve lesions by cardiac MRI , severe aortic valve calcification by cardiac computed tomography (aortic valve Agatston calcium score >1200 in women or >2000 in men), obesity (body mass index >35 kg/m²), diabetes mellitus, and current smoking. Sixty-seven participants (aged 64±10 years, 9% female) had unexplained LV hypertrophy (wall thickness ≥15 mm in at least 2 adjacent LV segments), representing 1.4% (1 in 74) participants, 2.6% of men and 0.2% of women. Prevalence was similar across categories of race/ethnicity. Hypertrophy was focal in 17 (25.4%), intermediate in 44 (65.7%), and diffuse in 5 (7.5%) participants. Conclusions The prevalence of unexplained LV hypertrophy in a population-based cohort using cardiac MRI was 1.4%. This may have implications for the diagnosis of patients with hypertrophic cardiomyopathy and will require further study.

Background: Hypertrophic cardiomyopathy is defined as unexplained left ventricular (LV) hypertrophy (wall thickness >= 15 mm) and is prevalent in 0.2% of adults (1:500) in population-based studies utilizing echocardiography. Cardiac magnetic resonance imaging (MRI) allows for more accurate wall thickness measurement across the entire ventricle than echocardiography. However, the prevalence of unexplained LV hypertrophy has not been examined in a cohort utilizing cardiac MRI. The Multi-Ethnic Study of Atherosclerosis (MESA) recruited individuals without overt cardiovascular disease 45-84 years of age. Method(s): We studied 4,972 individuals who underwent measurement of regional LV wall thickness by cardiac MRI as part of the MESA baseline exam. American Heart Association criteria were used to define LV segments. We excluded participants with hypertension, LV dilation (>= 95% predicted end-diastolic volume) or dysfunction (ejection fraction <= 50%), moderate-severe left-sided valve lesions by cardiac MRI or severe aortic valve calcification by cardiac computed tomography (aortic valve Agatston calcium score > 1,200 in women or > 2,000 in men), diabetes mellitus and current smoking. Result(s): 67 participants (age 64 +/- 10 years, 9% female) had unexplained LV hypertrophy (wall thickness >= 15 mm in at least 2 adjacent LV segments), representing 1.4% (1 in 74) participants, 2.6% of men and 0.2% of women. Prevalence was similar across categories of race/ethnicity. Hypertrophy was focal (2 segments) in 17 (25.4%), intermediate (3 to 7 segments) in 44 (65.7%) and diffuse (>= 8 segments) in 5 (7.5%) participants. The most commonly hypertrophied segments in each level were the basal anterior septal (46%), midventricular anterior lateral (40%) and apical lateral (33%) segments. Conclusion(s): The prevalence of unexplained LV hypertrophy in a population-based cohort utilizing cardiac MRI was 1.4%. This may have implications for diagnosis and management of patients with hypertrophic cardiomyopathy and will require further study.2019 American College of Cardiology Foundation. All rights reserved

BACKGROUND:Mitral annular calcification (MAC) is associated with cardiovascular events and mitral valve dysfunction. However, the underlying pathophysiology remains incompletely understood. In this prospective longitudinal study, we used a multimodality approach including positron emission tomography, computed tomography, and echocardiography to investigate the pathophysiology of MAC and assess factors associated with disease activity and progression. METHODS:F-Fluorodeoxyglucose (inflammation activity) positron emission tomography, computed tomography calcium scoring, and echocardiography. Sixty patients underwent repeat computed tomography and echocardiography after 2 years. RESULTS:F-Fluorodeoxyglucose uptake, as well as female sex and renal function. Similarly, MAC progression was closely associated with local factors, in particular, baseline MAC. Traditional cardiovascular risk factors and calcification activity in bone or remote atherosclerotic areas were not associated with disease activity nor progression. CONCLUSIONS:MAC is characterized by increased local calcification activity and inflammation. Baseline MAC burden was associated with disease activity and the rate of subsequent progression. This suggests a self-perpetuating cycle of calcification and inflammation that may be the target of future therapeutic interventions.

Continued improvement in medical and device therapy for heart failure (HF) has led to better survival with this disease. Longer survival and increasing numbers of unhealthy lifestyle factors and behaviors leading to occurrence of HF at younger ages are both contributors to an increase in the overall prevalence of HF. Clinicians treating this complex disease tend to focus on pharmacological and device therapies, but often fail to capitalize on the significant opportunities to prevent or treat HF through lifestyle modification. Herein, the authors review the evidence behind weight management, exercise, nutrition, dietary composition, supplements, and mindfulness and their potential to influence the epidemiology, pathophysiology, etiology, and management of stage A HF.

OBJECTIVE:To investigate the relationship of osteocalcin (OC), a marker of bone formation, and C-telopeptide of type I collagen (CTX), a marker of bone resorption, with incident diabetes in older women. RESEARCH DESIGN AND METHODS/METHODS:The analysis included 1,455 female participants from the population-based Cardiovascular Health Study (mean [SD] age 74.6 [5.0] years). The cross-sectional association of serum total OC and CTX levels with insulin resistance (HOMA-IR) was examined using multiple linear regression. The longitudinal association of both markers with incident diabetes, defined by follow-up glucose measurements, medications, and ICD-9 codes, was examined using multivariable Cox proportional hazards models. RESULTS:= 0.031]), associations that were comparable in magnitude and approached or achieved statistical significance. CONCLUSIONS:In late postmenopausal women, lower OC and CTX levels were associated with similarly increased risks of insulin resistance at baseline and incident diabetes over long-term follow-up. Further research to delineate the mechanisms linking abnormal bone homeostasis and energy metabolism could uncover new approaches for the prevention of these age-related disorders.

Oral retinoids are commonly prescribed for many dermatological conditions and may induce hyperlipidemia. We document the case of a 35-year-old man taking acitretin for congenital lamellar ichthyosis associated with a homozygous deleterious mutation in NIPAL4 who developed retinoid-induced hyperlipidemia that responded dramatically to a whole-food plant-based (WFPB) diet. On presentation, his diet consisted of chicken, fish, low fat meats and dairy, grains, and some fruits and vegetables. He then adopted a WFPB diet without making changes to his medications. His serum lipid levels dropped and his exercise capacity improved. Five months later, after discontinuing the plant-based diet and returning to his baseline diet, his hyperlipidemia returned and persisted despite adjustments to his medications. After a year and a half, the patient again adopted a plant-based diet and his lipid levels fell sharply again. A WFPB diet helped improve and control serum lipid levels in a patient with retinoid-induced hyperlipidemia. Future interventions should focus on ways to help patients successfully adopt and maintain a WFPB diet, as increased adherence to a healthy lifestyle is associated with greater health benefits.