scholarly journals A Case of Early Hereditary Transthyretin Amyloid Cardiomyopathy Recognition With Genetic Screening: A Case Report

2022 ◽  
Vol 13 ◽  
pp. 215013192110626
Author(s):  
Michel Juarez ◽  
Gaspar Del Rio-Pertuz ◽  
Kanak Parmar ◽  
Melanie C. Bois ◽  
Scott Shurmur ◽  
...  
Keyword(s):  
Heart Failure ◽  
African American ◽  
Genetic Screening ◽  
Signs And Symptoms ◽  
Restrictive Cardiomyopathy ◽  
Cardiac Amyloid ◽  
Carpal Tunnel Surgery ◽  
History Of ◽  
Amyloid Cardiomyopathy ◽  
Men Elderly

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the most common types of cardiac amyloidosis. Amyloid cardiomyopathy more commonly affects men, elderly, and 3% to 4% of the African-American population. ATTR-CM suspicion and diagnosis is challenging; however, awareness of the disease is increasing, and best practices to identify it are being proposed. The approach to suspected cases of ATTR-CM relies on the presence of heart failure, red flag signs and symptoms, and age >65 or >70 for men and women respectively. Little is known about cases when it presents in early ages. Case: We report a 62-year-old African American male with past medical history of hyperlipidemia, prostate cancer, hypertension, bilateral carpal tunnel surgery that had debuted with a cardiac arrhythmia at age 55 and was diagnosed with heart failure several years later. Restrictive cardiomyopathy was suspected, and genetic screening was sent for ATTRm which confirmed a pathogenic trasnthyretin gene mutation. Endomyocardial biopsy was performed which confirmed cardiac amyloid deposition. Discussion: ATTR-CM is a rare disease with an increasing prevalence. Cases with out of proportion signs and symptoms of heart failure with preserved ejection fractions should raise the suspicion of ATTR-CM despite age.

Lipid inclusions in cardiac myocytes – a rare case of cardiolipotoxicity

2020 ◽  
Author(s):  
Rahul Gupta ◽  
Purva Ranchal ◽  
Sugandhi Mahajan ◽  
Rugved Pattarkine ◽  
Saikrishna Patibandla ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Myocytes ◽  
Amorphous Material ◽  
Restrictive Cardiomyopathy ◽  
Fatty Acid Uptake ◽  
Heart Tissue ◽  
Acid Uptake ◽  
Lipid Inclusions ◽  
History Of ◽  
Accumulation Of Lipids

The heart oxidizes fatty acids for its energy production. The physiological balance between fatty acid uptake and its oxidation prevents lipid accumulation in cardiac myocytes. However, accumulation of lipids due to various processes such as obesity, diabetes, heart failure, myocardial ischemia or infarction can result in damage to the heart tissue, also known as cardiolipotoxicity. We present a unique case of a 69-year-old gentleman with a history of heart failure and ventricular tachycardia. Endomyocardial biopsy to assess for restrictive cardiomyopathy/amyloid showed no evidence of amyloid, significant inflammation or fibrosis, but did show intracellular accumulation of significant amorphous material in most cardiac myocytes. We review the literature regarding the pathogenesis of cardiolipotoxicity, which has no definite cause or treatment yet identified.

scholarly journals Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis

2017 ◽  
Vol 10 ◽  
pp. 117954761772364 ◽  
Author(s):  
Amit Alam ◽  
Shankar Thampi ◽  
Shahryar G Saba ◽  
Rita Jermyn
Keyword(s):  
Heart Failure ◽  
Surgical Intervention ◽  
Diastolic Heart Failure ◽  
Treatment Options ◽  
Hypereosinophilic Syndrome ◽  
Restrictive Cardiomyopathy ◽  
Endomyocardial Fibrosis ◽  
History Of ◽  
The Right ◽  
Loeffler Endocarditis

Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis. The severe progression of the disease required surgical intervention with endocardial stripping to treat the right-sided diastolic heart failure.

scholarly journals Ruptured Coronary Sinus of Valsalva in the Setting of a Supracristal Ventricular Septal Defect

2020 ◽  
Vol 4 (2) ◽  
pp. 154-157
Author(s):  
Abilio Arrascaeta-Llanes ◽  
Akanksha Kashyap ◽  
Diana Meyler ◽  
Ravi Gupta ◽  
Zubin Tharayil ◽  
...  
Keyword(s):  
Heart Failure ◽  
Emergency Department ◽  
Ventricular Septal Defect ◽  
Coronary Sinus ◽  
Septal Defect ◽  
Surgical Repair ◽  
Signs And Symptoms ◽  
Sinus Of Valsalva ◽  
Sinus Of Valsalva Aneurysm ◽  
History Of

A sinus of Valsalva aneurysm (SOVA) is usually a silent entity until one of its complications arises, such as heart failure. SOVA itself is uncommon, but it is more frequently associated with a supracristal ventricular septal defect (SVSD). We present a 67-year-old man with a history of an asymptomatic SVSD who presented to the emergency department with signs and symptoms of heart failure. He was subsequently found to have a ruptured SOVA and underwent urgent surgical repair.

Pulmonary arterial hypertension in a cat with restrictive cardiomyopathy – a case report

2018 ◽  
Vol XXIII (132) ◽  
pp. 66-74
Author(s):  
Elizabeth Regina Carvalho
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Thromboembolism ◽  
Clinical Signs ◽  
Restrictive Cardiomyopathy ◽  
Domestic Cat ◽  
Heartworm Disease ◽  
History Of ◽  
Pulmonary Arterial ◽  
Patent Ductus

Feline pulmonary arterial (PAH) is an unusual and minimally reported condition that can lead to right ventricular insufficiency and death. In cats, PAH was reported in cases in which it was secondary to heartworm disease, pulmonary thromboembolism and patent ductus arteriousus. This is a case report of an elderly domestic cat with medical history of dyspnea, fatigue and pleural effusion, which was diagnosed with restrictive cardiomyopathy and PAH upon echocardiography. The progression of restrictive cardiomyopathy can be silent due to the owners’ inability to recognize clinical signs of heart failure. In spite of the few reports in cats, it is reasonable that PAH be part of differential diagnosis on patients presenting signs of cardiopulmonary decompensation.

scholarly journals Cardiac amyloid presenting as cardiogenic shock: case series

2021 ◽  
Vol 5 (7) ◽  
Author(s):  
Monique Oye ◽  
Pooja Dhruva ◽  
Fadi Kandah ◽  
Melissa Oye ◽  
Emil Missov
Keyword(s):  
Heart Failure ◽  
African American ◽  
Ejection Fraction ◽  
Cardiogenic Shock ◽  
Cardiac Amyloidosis ◽  
Clinical Status ◽  
Case Series ◽  
Reduced Ejection Fraction ◽  
Cardiac Amyloid ◽  
First Case

Abstract Background Amyloidosis is a systemic infiltrative disease that can affect nearly every organ in the human body. It is characterized by the deposition of misfolded protein within various tissues and organs. Once there is cardiac involvement this portends a worse prognosis. Case summary We describe a case series of two patients with cardiac amyloidosis presenting as a cardiogenic shock. There were several missed opportunities in diagnosing cardiac amyloid prior to their fatal presentations. In the first case, a 65-year-old African-American male patient presented with worsening shortness of breath and signs of heart failure. Echocardiography revealed preserved ejection fraction. He was diagnosed with light chain subtype of cardiac amyloidosis, and rapidly deteriorated during his admission. Patient in the second case is a 75-year-old African-American female who presented with worsening heart failure and hypotension. Echocardiography revealed reduced ejection fraction. She was diagnosed with transthyretin cardiac amyloid. Her clinical status worsened during admission and she went into cardiogenic shock requiring multiple vasopressors. Discussion This case series discusses two incidences of cardiac amyloidosis presenting as cardiogenic shock in African-American patients. This article postulates that cardiac amyloidosis may be misdiagnosed for more common causes of heart failure especially among this demographic group. Once patients with cardiac amyloid present with cardiogenic shock their clinical course is typically rapidly fatal despite aggressive measures. Earlier detection is imperative to prevent poor outcomes.

scholarly journals Clinical Characteristics and Outcomes of Community- and Hospital-Acquired Acute Kidney Injury with COVID-19 in a US Inner City Hospital System

2020 ◽  
Vol 10 (4) ◽  
pp. 223-231 ◽  
Author(s):  
Jerald Pelayo ◽  
Kevin Bryan Lo ◽  
Ruchika Bhargav ◽  
Fahad Gul ◽  
Eric Peterson ◽  
...  
Keyword(s):  
Heart Failure ◽  
Acute Kidney Injury ◽  
African American ◽  
Clinical Outcomes ◽  
Clinical Characteristics ◽  
Kidney Injury ◽  
Hospital Death ◽  
City Hospital ◽  
History Of ◽  
Hospital Acquired

Introduction: Emerging data have described poor clinical outcomes from infection with the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV 2) among African American patients and those from underserved socioeconomic groups. We sought to describe the clinical characteristics and outcomes of acute kidney injury (AKI) in this special population. Methods: This is a retrospective study conducted in an underserved area with a predominance of African American patients with coronavirus disease 2019 (COVID-19). Descriptive statistics were used to characterize the sample population. The onset of AKI and relation to clinical outcomes were determined. Multivariate logistic regression was used to determine factors associated with AKI. Results: Nearly half (49.3%) of the patients with COVID-19 had AKI. Patients with AKI had a significantly lower baseline estimated glomerular filtration rate (eGFR) and higher FiO2 requirement and D-dimer levels on admission. More subnephrotic proteinuria and microhematuria was seen in these patients, and the majority had a pre-renal urine electrolyte profile. Patients with hospital-acquired AKI (HA-AKI) as opposed to those with community-acquired AKI (CA-AKI) had higher rates of in-hospital death (52 vs. 23%, p = 0.005), need for vasopressors (42 vs. 25%, p = 0.024), and need for intubation (55 vs. 25%, p = 0.006). A history of heart failure was significantly associated with AKI after adjusting for baseline eGFR (OR 3.382, 95% CI 1.121–13.231, p = 0.032). Conclusion: We report a high burden of AKI among underserved COVID-19 patients with multiple comorbidities. Those who had HA-AKI had worse clinical outcomes compared to those who with CA-AKI. A history of heart failure is an independent predictor of AKI in patients with COVID-19.

Abstract 15242: Increased Mortality Among African American Patients With Heart Failure Caused by Hereditary Transthyretin Amyloid Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Meena Zareh ◽  
Alina Levine ◽  
John L Berk ◽  
Omar K Siddiqi ◽  
Deepa M Gopal ◽  
...  
Keyword(s):  
Heart Failure ◽  
African American ◽  
Retinol Binding Protein ◽  
P Value ◽  
Retinol Binding Protein 4 ◽  
Risk Of Death ◽  
Kaplan Meier ◽  
Increased Risk ◽  
All Cause Mortality ◽  
Amyloid Cardiomyopathy

Introduction: Hereditary transthyretin amyloid cardiomyopathy (pV142I hATTR-CM) is an under-recognized cause of heart failure (HF) in elderly patients of African origin. While we have previously demonstrated that the endogenous TTR ligand retinol binding protein 4 (RBP4) identifies patients with hATTR-CM, its capacity to predict outcomes is unexplored. Further, comparative data are lacking describing clinical outcomes in hATTR-CM vs. matched patients with HF. Hypothesis: Reduced survival is associated with pV142I hATTR-CM when compared to an age, race, sex matched patients with HF. Methods: Retrospective, single center, cohort study of self-reported African American patients ≥ 60 years of age with normal TTR genotype, HF, and LV wall thickness ≥ 12mm (n=84) were compared to a cohort with biopsy-proven pV142I hATTR-CM (n=30). Patients did not receive ATTR-specific therapies, as study occurred prior to FDA approvals. Baseline laboratory values were measured. All-cause mortality was estimated and compared between groups based on a median followup of 45.4 months. Analyses were conducted with Fisher Exact, Kruskal Wallis, and Kaplan-Meier with log-rank testing. Results: hATTR-CM patients were slightly older but less likely to have concomitant HTN or DM (Table 1). Baseline RBP4 concentration was lower while troponin level higher in patients with hATTR-CM (Table 1). hATTR-CM patients had a higher hazard of all-cause mortality after adjusting for age (HR=3.5, 95% CI: 1.8 – 6.7, p<0.001). Baseline RBP4 was not a significant predictor of mortality (p-value=0.195). Conclusions: Patients pV142I hATTR-CM demonstrate significantly reduced survival (a 3.5-fold increased risk of death, 43% 4-year survival) compared to control subjects with HF in the absence of amyloidosis. Baseline RBP4 concentration did not associate with survival. These data suggest that hATTR-CM recognition is essential to permit implementation of available therapies that can improve survival.

scholarly journals Epidemiology of cardiomyopathies and incident heart failure in a population-based cohort study

Heart ◽  
2021 ◽  
pp. heartjnl-2021-320181
Author(s):  
Jack RW Brownrigg ◽  
Vincenzo Leo ◽  
Joel Rose ◽  
Eric Low ◽  
Sarah Richards ◽  
...  
Keyword(s):  
Heart Failure ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Restrictive Cardiomyopathy ◽  
Population Based ◽  
Ventricular Cardiomyopathy ◽  
Health Records ◽  
The Past ◽  
Population Prevalence ◽  
History Of ◽  
Incident Heart Failure

AimsThe population prevalence of cardiomyopathies and the natural history of symptomatic heart failure (HF) and arrhythmia across cardiomyopathy phenotypes is poorly understood. Study aims were to estimate the population-diagnosed prevalence of cardiomyopathies and describe the temporal relationship between a diagnosis of cardiomyopathy with HF and arrhythmia.MethodsPeople with cardiomyopathy (n=4116) were identified from linked electronic health records (~9 million individuals; 2000–2018) and categorised into hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM) and cardiac amyloidosis (CA). Cardiomyopathy point prevalence, rates of symptomatic HF and arrhythmia and timing relative to a diagnosis of cardiomyopathy were determined.ResultsIn 2018, DCM was the most common cardiomyopathy. DCM and HCM were twice as common among men, with the reverse trend for ARVC. Between 2010 and 2018, prevalence increased for ARVC by 180% and HCM by 9%. At diagnosis, more patients with CA (66%), DCM (56%) and RCM (62%) had pre-existing HF compared with ARVC (29%) and HCM (27%). Among those free of HF at diagnosis of cardiomyopathy, annualised HF incidence was greatest in CA and DCM. Diagnoses of all cardiomyopathies clustered around the time of HF onset.ConclusionsThe recorded prevalence of all cardiomyopathies increased over the past decade. Recognition of CA is generally preceded by HF, whereas individuals with ARVC or HCM more often developed HF after their cardiomyopathy diagnosis suggesting a more indolent course or better asymptomatic recognition. The clustering of HF and cardiomyopathy diagnoses suggests opportunities for presymptomatic or earlier diagnosis.

scholarly journals Prevalence of undiagnosed pre-diabetes or diabetes in patients with HFpEF presenting in the emergency department

2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
M Stratinaki ◽  
K Milaki ◽  
S Stavrakis ◽  
M Pitarokoilis ◽  
E Rogdakis ◽  
...  
Keyword(s):  
Heart Failure ◽  
Emergency Department ◽  
Ejection Fraction ◽  
Signs And Symptoms ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Funding Sources ◽  
History Of

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Heart failure with preserved ejection fraction (HFpEF) has become a common cause of visiting the emergency department (ED). Type 2 diabetes mellitus (T2DM) is one of the important predisposing factors. Purpose To study the prevalence of undiagnosed pre-diabetes or T2DM in the patients visiting the ED due to HFpEF. Methods 93 consecutive patients without known history of T2DM visited the ED with signs and symptoms of heart failure. Left ventricular ejection fraction (LVEF) was calculated using bedside ECHO. LVEF &gt; 50% was used to classify HFpEF patients. HbA1c was measured to all patients and the cutoff for T2DM was HbA1c &gt; 6.5% and for pre-diabetes 5.7-6.4%. Body mass index (BMI), brain natriuretic peptide (BNP) , creatinine  and  hemoglobin (Hb) were also recorded. Results are interpreted as mean ± SD. Results Mean age was 56 ± 11.8 years. 64/93 (68.8%) were female and 29/93(26.97%) male. Mean BMI was 36.52 ± 7.34 kg/m2 and mean Hb 13.51 ± 3.45 g/dl. Mean BNP was 584.82 ± 212.97 pg/ml. Mean creatinine was 1.11 ± 0.36mg/dl. 19/93 (17.67%) patients had HbA1c &gt; 5.7-6.4% and were classified as pre-diabetics and 23/93(21.93%) had HbA1c &gt; 6.5% and were classified as diabetics. We did not identify any correlation between the rest of the parameters. Coclusion 44.64% of the patients that visited ED due to signs or symptoms of HFpEF were identified as having either pre-diabetes or T2DM. Taking into consideration the importance of the presence of T2DM in the prognosis of the patients with HFpEF, it would be reasonable to screen all patients with HFpEF for T2DM and suggest specialized treatment.

Not as It Is Written

2020 ◽  
Vol 42 (2) ◽  
pp. 78-100
Author(s):  
Benjamin Houston
Keyword(s):  
African American ◽  
Civil Rights ◽  
Oral History ◽  
Civil Rights Movement ◽  
International Exhibition ◽  
Black Experience ◽  
American Perspective ◽  
History Of ◽  
Black Life ◽  
Made In

This article discusses an international exhibition that detailed the recent history of African Americans in Pittsburgh. Methodologically, the exhibition paired oral history excerpts with selected historic photographs to evoke a sense of Black life during the twentieth century. Thematically, showcasing the Black experience in Pittsburgh provided a chance to provoke among a wider public more nuanced understandings of the civil rights movement, an era particularly prone to problematic and superficial misreadings, but also to interject an African American perspective into the scholarship on deindustrializing cities, a literature which treats racism mostly in white-centric terms. This essay focuses on the choices made in reconciling these thematic and methodological dimensions when designing this exhibition.

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