Heart involvement in AL-amyloidosis. Current state of the issue

2019 ◽  
Vol 74 (5) ◽  
pp. 307-316
Author(s):  
Ivan V. Trukhin ◽  
Dmitrij Yu. Schekochikhin ◽  
Nina A. Novikova ◽  
Anna S. Akselrod ◽  
Abram L. Syrkin ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Treatment Options ◽  
Al Amyloidosis ◽  
Primary Amyloidosis ◽  
Current State ◽  
Main Challenge ◽  
Response To Chemotherapy ◽  
Patients With Heart Failure ◽  
Infiltrative Cardiomyopathies

AL cardiac amyloidosis is a relatively rare disorder that belongs to the group of infiltrative cardiomyopathies. Diagnosis of primary amyloidosis is challenging due to many unspecific symptoms and sings, which often leads to late diagnosis when treatment options are limited. Primary amyloidosis particularly needs to be excluded in patients with heart failure with preserved ejection fraction. Therapy in cardiac amyloidosis has to main vectors: 1) chemotherapy to eliminate amyloidogenic plasmatic cells 2) heart failure treatment. The main challenge for cardiologists is to support hemodynamics until response to chemotherapy occurs. In the article the issue of diagnostics, risk stratification and treatment of primary cardiac amyloidosis is addressed.

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.

Frequency of Atrial Fibrillation in Heart Failure Patients

2021 ◽  
Vol 15 (11) ◽  
pp. 3261-3264
Author(s):  
Syed Mohammad Haleem ◽  
Muhammad Hashim Kalwar ◽  
Muhammad Hassan Butt ◽  
Jehangir Hasan ◽  
Javed Khurshed Shaikh ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Demographic Data ◽  
Treatment Options ◽  
Cross Sectional Study ◽  
P Value ◽  
Cross Sectional ◽  
Heart Failure Patients ◽  
Cardiac Symptoms ◽  
Patients With Heart Failure

Background: An increasing number of people around the world suffer from cardiovascular diseases such as atrial fibrillation (AF) and heart failure (HF). Atrial fibrillation (AF) has been linked to poor prognosis in heart failure (HF) patients with mild to moderate LV dysfunction, and it is most prominent in these patients. Despite advancements in care and treatment options like catheter ablation, managing AF is still a therapeutic challenge even with these advances. Aim/Objective: To determine the frequency of atrial fibrillation in heart failure patients. Subjects and Methods: This cross-sectional study was conducted on 246 patients at Adult Cardiology, NICVD, Karachi for Six months after approval from 31-08-2019 till 29-02-2020. Patients' consent was obtained verbally before data were collected prospectively. There were 246 patients with heart failure who had been diagnosed and met the diagnostic criteria. An overview of the event's history was gathered, as well as demographic data. Qualitative variables were presented as frequency and percentages, while quantitative data were given as simple descriptive statistics such as mean and standard deviation. A p-value of 0.05 or lower is considered statistically significant. Results: A total of 246 patients with heart failure were included in this study. The mean age in our study was 56.78±2.81 years. Whereas, mean BNP and ejection fraction in our study was 1144±87.81 pg./ml and 34.65±4.14 %. 136 (55.3%) were male and 110 (44.7%) were female. Out of 246 patients with heart failure, 52 (21.1%) had atrial fibrillation and 194 (78.9%) did not have atrial fibrillation. Conclusion: The results of this research demonstrated that a large percentage of heart failure patients had atrial fibrillation. Thus, it is essential to focus on a comprehensive way of management of heart failure and its comorbidities rather than primarily treating the cardiac symptoms. Keywords: Heart failure and atrial fibrillation.

Atrial fibrillation surgery with a focus on patients with reduced left ventricular function and heart failure

EP Europace ◽  
2020 ◽  
Vol 22 (4) ◽  
pp. 517-521
Author(s):  
Johannes Petersen ◽  
Hermann Reichenspurner ◽  
Simon Pecha
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Surgical Treatment ◽  
Left Ventricular Function ◽  
Treatment Options ◽  
Left Ventricular ◽  
Review Article ◽  
Surgical Ablation ◽  
Ablation Therapy ◽  
Patients With Heart Failure

Abstract This review article aims to give an overview on the different surgical treatment options for atrial fibrillation It includes concomitant- as well as stand-alone surgical ablation therapy and outlines the main issues in patients with heart failure and reduced LVEF.

scholarly journals Echocardiographic markers of cardiac amyloidosis in patients with heart failure and left ventricular hypertrophy

2021 ◽  
Author(s):  
Jorge Melero Polo ◽  
Ana Roteta Unceta-Barrenechea ◽  
Pablo Revilla Martí ◽  
Raquel Pérez-Palacios ◽  
Anyuli Gracia Gutiérrez ◽  
...  
Keyword(s):  
Heart Failure ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Patients With Heart Failure

scholarly journals A Case Report of Cardiac Amyloidosis Highlighting the Importance of Strain Analysis

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Andres Cordova Sanchez ◽  
Ryan Murphy ◽  
Suman Rao ◽  
Fidel Martinez ◽  
Stephanie Bryant ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
High Mortality ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Inverse Relationship ◽  
Cardiac Involvement ◽  
Strain Analysis ◽  
Al Amyloidosis ◽  
Cardiac Symptoms

Cardiac involvement in light-chain (AL) amyloidosis has a high mortality. Once cardiac symptoms are present, it is important to make a diagnosis as there is an inverse relationship between mortality and time of diagnosis. Echocardiography is usually one of the first tests performed. But strain analysis, which can provide important clues, is not routinely performed. This is a case of AL amyloidosis presenting with heart failure in which echocardiographic strain analysis was vital for its diagnosis.

scholarly journals Hyponatremia in Heart Failure: Pathogenesis and Management

2019 ◽  
Vol 15 (4) ◽  
pp. 252-261 ◽  
Author(s):  
Mario Rodriguez ◽  
Marcelo Hernandez ◽  
Wisit Cheungpasitporn ◽  
Kianoush B. Kashani ◽  
Iqra Riaz ◽  
...  
Keyword(s):  
Heart Failure ◽  
Treatment Options ◽  
Volume Overload ◽  
Renal Perfusion ◽  
Loop Diuretics ◽  
Fluid Restriction ◽  
First Case ◽  
Patients With Heart Failure ◽  
Promising Treatment ◽  
Short And Long Term

Hyponatremia is a very common electrolyte abnormality, associated with poor short- and long-term outcomes in patients with heart failure (HF). Two opposite processes can result in hyponatremia in this setting: Volume overload with dilutional hypervolemic hyponatremia from congestion, and hypovolemic hyponatremia from excessive use of natriuretics. These two conditions require different therapeutic approaches. While sodium in the form of normal saline can be lifesaving in the second case, the same treatment would exacerbate hyponatremia in the first case. Hypervolemic hyponatremia in HF patients is multifactorial and occurs mainly due to the persistent release of arginine vasopressin (AVP) in the setting of ineffective renal perfusion secondary to low cardiac output. Fluid restriction and loop diuretics remain mainstay treatments for hypervolemic/ dilutional hyponatremia in patients with HF. In recent years, a few strategies, such as AVP antagonists (Tolvaptan, Conivaptan, and Lixivaptan), and hypertonic saline in addition to loop diuretics, have been proposed as potentially promising treatment options for this condition. This review aimed to summarize the current literature on pathogenesis and management of hyponatremia in patients with HF.

Amyloidosis

2018 ◽  
Author(s):  
Sajjan Mittal
Keyword(s):  
Heart Failure ◽  
Right Heart Failure ◽  
Al Amyloidosis ◽  
Primary Amyloidosis ◽  
Aa Amyloidosis ◽  
Right Heart ◽  
Multisystem Disease ◽  
Reactive Amyloidosis ◽  
Amyloid Light Chain ◽  
Developed World

Amyloidosis is a multisystem disease caused by the extracellular deposition of insoluble abnormal fibrils that injure tissues and organs. The fibrils are formed by the aggregation of misfolded, normally soluble proteins. Systemic amyloid light-chain (AL) amyloidosis (primary amyloidosis) is the commonest type of amyloidosis in the developed world, accounting for 80% of cases. The remainder are due to AA amyloidosis (secondary or reactive amyloidosis), familial amyloidosis, or other rare types of amyloidosis. The most common clinical features at diagnosis are nephrotic syndrome, heart failure (typically with predominant right heart failure), sensorimotor and/or autonomic peripheral neuropathy, and hepatosplenomegaly.

scholarly journals What Type of Patients Did PARAGON-HF Select? Insights from a Real-World Prospective Cohort of Patients with Heart Failure and Preserved Ejection Fraction

2020 ◽  
Vol 9 (11) ◽  
pp. 3669 ◽  
Author(s):  
René Rettl ◽  
Theresa-Marie Dachs ◽  
Franz Duca ◽  
Christina Binder ◽  
Fabian Dusik ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Real World ◽  
Treatment Options ◽  
National Registry ◽  
Preserved Ejection Fraction ◽  
Eligibility Criteria ◽  
Patients With Heart Failure ◽  
Long Term Prognosis

The PARAGON-HF clinical trial suggested that sacubitril/valsartan may become a treatment option for particular subgroups of patients with heart failure and preserved ejection fraction (HFpEF). However, the proportion of real-world HFpEF patients who are theoretically superimposable with the PARAGON-HF population is yet unknown. The present study was performed to define the proportion of real-world PARAGON-HF-like patients and to describe their clinical characteristics and long-term prognosis in comparison with those who would not meet PARAGON-HF criteria. We systematically applied PARAGON-HF inclusion and exclusion criteria to a total of 427 HFpEF patients who have been participating in a prospective national registry between December 2010 and December 2019. In total, only 170 (39.8%) registry patients were theoretically eligible for PARAGON-HF. Patients not meeting inclusion criteria (41.0%) were less impaired with respect to exercise capacity (median 6-min walk distance: 385 m (IQR: 300–450) versus 323 m (IQR: 240–383); p < 0.001) had lower pulmonary pressures (mean pulmonary artery pressure (mPAP): 31.2 mmHg, standard deviation (SD): ±10.2 versus 32.8 mmHg, SD: ±9.7; p < 0.001) and better outcomes (log-rank: p < 0.001) as compared to the PARAGON-like cohort. However, patients theoretically excluded from the trial (19.2%) were those with most advanced heart failure symptoms (median 6-min walk test: 252 m (IQR: 165–387); p < 0.001), highest pulmonary pressures (mPAP: 38.2 mmHg, SD: ±12.4; p < 0.001) and worst outcome (log-rank: p = 0.037). We demonstrate here that < 40% of real-world HFpEF patients meet eligibility criteria for PARAGON-HF. We conclude that despite reasons for optimism after PARAGON-HF, a large proportion of HFpEF patients will remain without meaningful treatment options.

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