scholarly journals The Importance of Multimodality Imaging in the Diagnosis and Management of Patients with Infiltrative Cardiomyopathies: An Update

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 256 ◽  
Author(s):  
Radu Sascău ◽  
Larisa Anghel ◽  
Alexandra Clement ◽  
Mădălina Bostan ◽  
Rodica Radu ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Multimodality Imaging ◽  
Imaging Techniques ◽  
Left Ventricular ◽  
Widespread Availability ◽  
Infiltrative Cardiomyopathies ◽  
Restrictive Physiology ◽  
Standardized Diagnostic

Infiltrative cardiomyopathies (ICMs) comprise a broad spectrum of inherited and acquired conditions (mainly amyloidosis, sarcoidosis, and hemochromatosis), where the progressive buildup of abnormal substances within the myocardium results in left ventricular hypertrophy and manifests as restrictive physiology. Noninvasive multimodality imaging has gradually eliminated endomyocardial biopsy from the diagnostic workup of infiltrative cardiac deposition diseases. However, even with modern imaging techniques’ widespread availability, these pathologies persist in being largely under- or misdiagnosed. Considering the advent of novel, revolutionary pharmacotherapies for cardiac amyloidosis, the archetypal example of ICM, a standardized diagnostic approach is warranted. Therefore, this review aims to emphasize the importance of contemporary cardiac imaging in identifying specific ICM and improving outcomes via the prompt initiation of a targeted treatment.

scholarly journals Cardiac Amyloidosis with Discordant QRS Voltage between Frontal and Precordial Leads

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Low Voltage ◽  
Disease Process ◽  
Left Ventricular ◽  
Clinical Suspicion ◽  
Immunoglobulin Light Chain ◽  
Different Types

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.

scholarly journals Multimodality imaging in arrhythmogenic cardiomyopathy

2021 ◽  
Vol 31 (1) ◽  
pp. 10-16
Author(s):  
Laura Tapoi ◽  
Alexandra Clement ◽  
Rodica Radu ◽  
Radu Sascau
Keyword(s):  
Cardiac Death ◽  
Multimodality Imaging ◽  
Imaging Techniques ◽  
Left Ventricular ◽  
Arrhythmogenic Cardiomyopathy ◽  
Prognostic Information ◽  
Care Community ◽  
The Past ◽  
Widespread Availability ◽  
Made In

Arrhythmogenic cardiomyopathy, as it has been recently redefi ned, is characterized by progressive myocyte loss with fibrosis and fat infiltration of the myocardium, which finally leads to a broad clinical spectrum ranging from heart failure symptoms to sudden cardiac death. The diagnosis of arrhythmogenic cardiomyopathy is challenging particularly because of its heterogeneity in presentation, which varies from focal right ventricular involvement to biventricular or prominent left ventricular phenotype. In the past decades, the development of new electrocardiographic and imaging diagnostic criteria for arrhythmogenic cardiomyopathy constituted an important area of research and resulted in the elaboration of the Padua criteria. However, even with the widespread availability of modern imaging techniques, there is still a lack of awareness in the health care community and this pathology persist in being under-or misdiagnosed. Given the limited indication of endomyocardial biopsy for the diagnosis of arrhythmogenic cardiomyopathy, one can conclude that the progress that has been made in the last few years in the multimodality imaging field is of utmost importance for the early detection and proper treatment of patients with arrhythmogenic cardiomyopathy, providing valuable prognostic information.

scholarly journals 1962Diagnostic score for the detection of cardiac amyloidosis in patients with left ventricular hypertrophy and impact on prognosis

2017 ◽  
Vol 38 (suppl_1) ◽  
Author(s):  
E. Cariou ◽  
P. Fournier ◽  
G. Victor ◽  
D. Ribes ◽  
P. Pascal ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Left Ventricular

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 Diagnosing Cardiac Amyloidosis: From Heart Failure to Electrical Storm

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Adithya T. Mathews ◽  
Abu-Sayeef Mirza ◽  
Chandrashekar Bohra ◽  
Akshay G. Mathews ◽  
Philip Ritucci-Chinni ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Diastolic Heart Failure ◽  
Diuretic Therapy ◽  
Left Ventricular ◽  
Electrical Storm ◽  
Rapid Progression

Cardiac amyloidosis is a condition when amyloid fibers are deposited in the extracellular space of the heart causing tachyarrhythmias, heart failure, or sudden cardiac death. We present a 71-year-old woman presenting with dyspnea on admission. Echocardiogram revealed diastolic heart failure and left ventricular hypertrophy with strain pattern concerning for an infiltrative process. She was discharged with diuretic therapy and scheduled for a cardiac magnetic resonance imaging. One week after discharge, she was readmitted with progressive shortness of breath and syncope. She was found to be in shock and had multiple episodes of cardiac arrest with both ventricular tachycardia and pulseless electrical activity. She developed electrical storm and eventually passed within 24 hours. Autopsy revealed gross cardiomegaly and left ventricular hypertrophy with Congo red staining revealing amyloid fibrils with apple-green birefringence. This case demonstrates the rapid progression of cardiac amyloidosis from acute-onset diastolic heart failure to uncontrollable ventricular tachycardia, and eventually death. We review the literature regarding multiple diagnostic modalities that facilitate the confirmation of cardiac amyloidosis.

scholarly journals Cardiac amyloidosis screening using a relative apical sparing pattern in patients with left ventricular hypertrophy

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yasuhisa Nakao ◽  
Makoto Saito ◽  
Katsuji Inoue ◽  
Rieko Higaki ◽  
Yuki Yokomoto ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Low Voltage ◽  
Characteristic Curve ◽  
Multiple Logistic Regression Analysis ◽  
Left Ventricular ◽  
Discrimination Ability ◽  
Incremental Benefit ◽  
Deformation Parameters

Abstract Background Cardiac amyloidosis (CA) mimics left ventricular hypertrophy (LVH). It is treatable, but its prognosis is poor. A simple screening tool for CA would be valuable. CA is more precisely diagnosed with echocardiographic deformation parameters (e.g., relative apical sparing pattern [RASP]) than with conventional parameters. We aimed to 1) investigate incremental benefits of echocardiographic deformation parameters over established parameters for CA screening; 2) determine the resultant risk score for CA screening; and 3) externally validate the score in LVH patients. Methods We retrospectively studied 295 consecutive non-ischemic LVH patients who underwent detailed diagnostic tests. CA was diagnosed with biopsy or 99mTc-PYP scintigraphy. The base model comprised age (≥65 years [men], ≥70 years [women]), low voltage on the electrocardiogram, and posterior wall thickness ≥ 14 mm in reference to the literature. The incremental benefit of each binarized echocardiographic parameter over the base model was assessed using receiver operating characteristic curve analysis and comparisons of the area under the curve (AUC). Results Fifty-four (18%) patients had CA. RASP showed the most incremental benefit for CA screening over the base model. After conducting multiple logistic regression analysis for CA screening using four variables (RASP and base model components), a score was determined (range, 0–4 points). The score demonstrated adequate discrimination ability for CA (AUC = 0.86). This result was confirmed in another validation cohort (178 patients, AUC = 0.88). Conclusions We developed a score incorporating RASP for CA screening. This score is potentially useful in the risk stratification and management of LVH patients.

scholarly journals Comparison between echocardiography and cardiac magnetic resonance for differential diagnosis of left ventricular hypertrophy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Corsi ◽  
G Todiere ◽  
A Barison ◽  
C Grigoratos ◽  
G.D Aquaro
Keyword(s):  
Differential Diagnosis ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Athlete’S Heart ◽  
Kaplan Meier ◽  
Athlete's Heart

Abstract Background Left ventricular hypertrophy (LVH) may be due to different causes, ranging from, benign secondary forms (athlete's heart) to severe prognosis cardiomyopathies (i.e. cardiac amyloidosis). Early and accurate differential diagnosis is important to proper patient management. LVH may be detected by echocardiography signs of hypertrophy or other abnormalities often associated to hypertrophic phenotypes. Cardiac magnetic resonance (CMR) is often used to confirm the initial diagnostic suspicion. On the best of our knowledge, there are no study specifically designed to evaluate the final impact of CMR in changing or confirming the initial diagnostic echocardiographic suspicion. Aim To evaluate the clinical prognostic correlates of CMR in patients with echocardiographic or ECG suspicion of LVH (or cardiomyopathies with hypertrophic phenotype). Methods and results We enrolled 275 pts with echocardiographic evidence of LVH. Using current guidelines, the initial echocardiographic diagnostic suspicion was: hypertrophic cardiomyopathy (HCM) in 46.9% of pts; cardiac amyloidosis in 14.5%; hypertensive LVH in 17%; aortic stenosis in 1.5%; athlete's heart in 0.3%; undetermined LVH in 17%. CMR changed the diagnosis in 42% cases: the diagnosis of HCM increased from 44% to 72% of pts; hypertensive and undetermined LVH decreased significantly (respectively to 4% and 5%). Finally, the change in diagnostic suspicion was associated to reclassification of risk of patients: Kaplan-Meier curves demonstrated that HCM and cardiac amyloidosis had worst prognosis than undetermined or hypertensive LVH. Conclusions CMR changed the echocardiographic suspicion in almost half of patients with LVH. This study highlights the indication of CMR in patient with ECG or echocardiographic suspicion of LVH. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

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