scholarly journals Combining ECG and echocardiography to identify transthyretin cardiac amyloidosis in heart failure

2021 ◽  
Author(s):  
V Löfbacka ◽  
OB Suhr ◽  
B Pilebro ◽  
J Wixner ◽  
T Sundström ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis

scholarly journals Cardiac Amyloidosis Therapy: A Systematic Review

2021 ◽  
Vol 11 (1) ◽  
pp. 10-17
Author(s):  
Franco Iodice ◽  
Marco Di Mauro ◽  
Marco Giuseppe Migliaccio ◽  
Angela Iannuzzi ◽  
Roberta Pacileo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Systematic Review ◽  
Cardiac Amyloidosis ◽  
Restrictive Cardiomyopathy ◽  
Novel Therapies ◽  
Preserved Ejection Fraction ◽  
Cardiac Damage ◽  
Transthyretin Amyloidosis ◽  
To Come ◽  
Almost All

Heart involvement in Cardiac Amyloidosis (CA) results in a worsening of the prognosis in almost all patients with both light-chain (AL) and transthyretin amyloidosis (ATTR). The mainstream CA is a restrictive cardiomyopathy with hypertrophic phenotype at cardiac imaging that clinically leads to heart failure with preserved ejection fraction (HFpEF). An early diagnosis is essential to reduce cardiac damage and to improve the prognosis. Many therapies are available, but most of them have late benefits to cardiac function; for this reason, novel therapies are going to come soon.

Total 12-Lead QRS Voltage in Patients Having Orthotopic Heart Transplantation for Heart Failure Caused by Adriamycin-Induced Cardiomyopathy

Cardiology ◽  
2018 ◽  
Vol 141 (3) ◽  
pp. 172-175 ◽  
Author(s):  
William C. Roberts ◽  
Sarah Haque ◽  
Shelley A. Hall
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Cardiac Amyloidosis ◽  
Cardiac Sarcoidosis ◽  
Medical Center ◽  
Severe Heart Failure ◽  
Orthotopic Heart Transplantation ◽  
S Wave ◽  
Baylor University ◽  
Before And After

Objective: Although several studies have described the effects of adriamycin on the heart, electrocardiographic total 12-lead QRS voltage (distance in millimeters from the peak of the R wave to the nadir of either the Q or S wave, whichever was deeper, with 10 mm [1 mV] being standard) both before and after orthotopic heart transplantation (OHT) has not been reported. This study describes the total 12-lead QRS voltage in 8 patients studied at Baylor University Medical Center at Dallas, from 1994 to June 2018, who underwent OHT for severe heart failure caused by anthracycline-induced cardiomyopathy. Method: Prior to OHT, the total 12-lead non-paced QRS voltages ranged from 86 to 189 mm (mean 125 ± 56) and for paced QRS voltages from 82 to 113 mm (mean 97 ± 15). The total 12-lead QRS voltages post-OHT ranged from 100 to 190 mm (mean 130 ± 30). Total 12-lead QRS voltages were lower in patients with a pacemaker than without. Results/Conclusion: These low voltages are like those found in patients with carcinoid syndrome, severe cardiac adiposity, cardiac amyloidosis, and cardiac sarcoidosis.

scholarly journals Multimodality imaging can shift the clinical approach and prognosis of a patient: from heart failure and angina to cardiac amyloidosis

2021 ◽  
Vol 31 (1) ◽  
pp. 103-110
Author(s):  
Alexandra Maria Chitroceanu ◽  
Alina Ioana Nicula ◽  
Roxana Cristina Rimbas ◽  
Mihaela Andreescu ◽  
Cristina Popp ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Multimodality Imaging ◽  
Clinical Status ◽  
Decompensated Heart Failure ◽  
Response To Therapy ◽  
Al Amyloidosis ◽  
Clinical Approach ◽  
Non Invasive ◽  
Life Threatening

AL (light chain) amyloidosis is a life threatening disease. Untreated patients with involvement of the heart, a condition known as cardiac amyloidosis (CA), tend to have the most rapid disease progression and worst prognosis. Therefore, it is essential to early recognize the signs of symptoms of CA, and to identify the affected individuals with readily available non-invasive tests, as timely therapy can prolong life. Different imaging tests are used to diagnose and stratify the risk of the disease noninvasively, and to follow-up of the disease course and response to therapy. In this light, we present a case of a woman with cardiovascular risk factors, initially admitted for typical angina and decompensated heart failure (HF), who was later diagnosed with AL amyloidosis with cardiac involvement, by using multimodality imaging assessment in a step-by-step fashion. This changed completely the prognosis of the patient. Timely chemotherapy and stem cell transplantation led to an improvement in clinical status, biomarkers, and in a regression of amyloid myocardial infi ltration showed by imaging.

Abstract 16124: Implantable Cardioverter Defibrillators in Patients With Hereditary Transthyretin Cardiac Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Sreeram Yalamanchili ◽  
Matthew T Brown ◽  
Evan A Blank ◽  
Melissa A Lyle ◽  
Kunal N Bhatt
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Cardiac Amyloidosis ◽  
Clinical Decision Making ◽  
Systolic Heart Failure ◽  
Heart Association ◽  
High Rate ◽  
Implantable Cardioverter Defibrillators ◽  
Antitachycardia Pacing ◽  
Icd Implantation

Introduction: Implantable cardioverter defibrillator (ICD) implantation in patients with cardiac amyloidosis is controversial, with no clear guidelines for clinical decision-making.. The role of ICD implantation in hereditary Transthyretin Amyloid Cardiomyopathy (hATTR-CM) is unclear. We sought to establish the burden of ventricular arrhythmias and the outcomes of ICD implantation in a single center cohort of hATTR-CM patients. Methods: A total of 69 patients with a confirmed diagnosis of hATTR-CM from genetic testing, and technetium pyrophosphate (PYP) scanning, or endomyocardial biopsy underwent retrospective chart review for demographic, clinical, and arrhythmia data. Results: Seventy-four percent of the cohort was male, with a mean age at diagnosis of 68 (SD=18 years). Sixty-five patients (94.2%) patients were African-American; all of whom carried the Valine 122 Isoleucine mutation. Most had systolic heart failure (New York Heart Association Staging II [18, 26%] and III [40, 58.8%]); 37 (54%) patients had an LVEF ≤ 35%. Thirty-six (52.2%) patients had documented episodes of non-sustained ventricular tachycardia (NSVT), three (4.3%) with ventricular tachycardia (VT), and one (1.5%) with ventricular fibrillation (VF). A total of 15 (21.7%) patients had ICDs placed for prevention of sudden cardiac death in the setting of low LVEF (EF <35%). All recorded VT/VF episodes occurred in three patients with ICDs. Of these patients, one experienced two episodes of VT each successfully abated by antitachycardia pacing (ATP), another experienced a single episode of VT abated by ATP, while the final experienced VF with successful 36J shock as well as two episodes of VT each successfully treated with 36J shocks. No inappropriate ICD shocks were delivered, however, there were two instances of ATP for inappropriately detected atrial arrhythmias. Conclusions: In a cohort of patients with hATTR-CM, we observed a high incidence of NSVT, yet only 41% of patients with severe systolic heart failure had ICDs implanted. A high rate of successful defibrillation and no inappropriate ICD shocks were noted, suggesting that ICDs should be strongly considered, and may be underutilized, in patients with systolic heart failure and/or arrhythmias in the setting of hATTR-CM.

Cardiac amyloidosis

ESC CardioMed ◽  
2018 ◽  
pp. 1545-1549
Author(s):  
Martha Grogan
Keyword(s):  
Heart Failure ◽  
Median Survival ◽  
Cardiac Amyloidosis ◽  
Beta Blockers ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Wild Type ◽  
Transthyretin Amyloidosis ◽  
Converting Enzyme Inhibitors ◽  
Right Ventricular Wall ◽  
Immunoglobulin Light Chain Amyloidosis

Cardiac amyloidosis is an important cause of heart failure and cardiac arrhythmias, yet cardiologists often miss the diagnosis. Immunoglobulin light-chain amyloidosis (AL) is relatively rare, but likely underdiagnosed. The median survival of untreated patients with cardiac AL is 6 months after the onset of heart failure, highlighting the importance of early diagnosis. Wild-type transthyretin amyloidosis (ATTR) is increasingly recognized, especially in males over the age of 60 years. Although the clinical course of wild-type ATTR is more indolent, the median survival is approximately 3.5 years from diagnosis. Typical echocardiographic findings of increased left and right ventricular wall thickness, diastolic dysfunction, and pericardial effusion may suggest cardiac amyloidosis, along with abnormal delayed gadolinium enhancement and difficulty nulling the myocardium on cardiac magnetic resonance imaging. For AL, a tissue diagnosis is required. In contrast, ATTR may be diagnosed non-invasively with grade 2/3 uptake by nuclear scintigraphy in the absence of a monoclonal protein. Treatment of cardiac amyloidosis is entirely dependent on the type of amyloid and is directed at the underlying precursor protein or disrupting existing deposits. Cardiac care is supportive and challenging. Standard heart failure medications such as beta blockers and angiotensin-converting enzyme inhibitors are not routinely indicated and often cause haemodynamic deterioration. Outcomes of source-directed therapy for AL are improving and several clinical trials of treatment for ATTR are ongoing.

Prevalence of cardiac amyloidosis among elderly patients with systolic heart failure or conduction disorders

Amyloid ◽  
2019 ◽  
Vol 26 (3) ◽  
pp. 156-163 ◽  
Author(s):  
Ángela López-Sainz ◽  
F. Javier de Haro-del Moral ◽  
Fernando Dominguez ◽  
Alejandra Restrepo-Cordoba ◽  
Almudena Amor-Salamanca ◽  
...  
Keyword(s):  
Heart Failure ◽  
Elderly Patients ◽  
Cardiac Amyloidosis ◽  
Systolic Heart Failure ◽  
Conduction Disorders

A simple case of heart failure? Isolated cardiac amyloidosis

2019 ◽  
Vol 80 (12) ◽  
pp. 735-735
Author(s):  
A Shahid ◽  
F Javed ◽  
B Renton
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis

scholarly journals Cardiac Care of Patients with Cardiac Amyloidosis

2020 ◽  
Vol 143 (4) ◽  
pp. 343-351
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Ran Kornowski
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Management Strategies ◽  
Treatment Strategies ◽  
Multidisciplinary Care ◽  
Cardiac Care ◽  
Immunoglobulin Light Chain ◽  
Transthyretin Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Specific Management

Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the importance of multidisciplinary care.

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