scholarly journals P1684 Cardiac amyloidosis hits the valve first

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Iakovlev ◽  
A I Kalinskaya

Abstract Case report. 60-year-old man without previous history of coronary artery disease was admitted to our hospital. He complained of weakness, low extremities edema. Physical examination revealed also enlargement of the liver, positive hepatojugular reflux, multiple ecchymoses on patient’s face, especially in periorbital area. The arterial blood pressure was 100/60 mm Hg. ECG showed sinus rhythm 83/min, low R waves in V2, V3 with biphasic T waves in V4-V6. The last coronary angiography revealed normal coronary arteries, it was performed 6 months ago because of atypical chest pain and inconclusive stress test. Echocardiography revealed severe tricuspid regurgitation (TR) due to leaflets restriction and malcoaptation. TR gradient was about 10 mm Hg. Interventricular septum 12 mm, left ventricle posterior wall 9 mm, mass index 87 g/m2, relative wall thickness 0.38; no LV regional wall motion abnormalities was noted, left ventricular ejection fraction (LV EF) was about 50%, mitral valve E/A ratio was 1,4, but average E/E" = 17. Left atrium volume 33 ml/m2. Insignificant amount of pericardial effusion also was found. 3D evaluation of tricuspid valve (TV) showed no leaflets defects and chordal ruptures. LV global longitudinal strain (LV GLS) was – 11,1 % with the apex/(mid + base) ratio 1,3 - apical sparing pattern. Cardiac MRI with gadolinium also showed severe TR and diffuse late subendocardial gadolinium enhancement in both ventricles. We suspected cardiac amyloidosis with significant tricuspid valve involvement, torrential TR, and right ventricle volume overload. The rectum biopsy was negative. The cardiac muscle biopsy with congo red straining was positive for amyloidosis. The patient was transferred to hematology clinic where the diagnosis of AL-amyloidosis was confirmed. The treatment with lenalidomide and prednisone was started. Unfortunately, one month later the patient died. The autopsy was not performed due to religious reasons. Discussion AL-amyloidosis is a systemic disease characterized by multiple organ and tissue changes and associated with poor prognosis. Cardiac involvement is a major prognostic factor as it accounts for approximately 75% of death due to heart failure or arrhythmias. Infiltration of myocardium with amyloid leads to diastolic than systolic dysfunction of the heart and to developing of the heart failure. The most common presentation of heart involvement in AL-amyloidosis is fatigue and dyspnea. In this case the main complaint of the patient was peripheral edema, echocardiography showed damaged TV and preserved LV systolic function. In literature, we found only one case report describing cardiac amyloidosis presented as severe TR. Conclusion In patients presenting with significant isolated valvular dysfunction and heart failure the cardiac amyloidosis can be suspected. The comprehensive echocardiography is the most useful tool to detect this problem. Abstract P1684 Figure. 3D picture of tricuspid valve

Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001346
Author(s):  
Aénora Roger-Rollé ◽  
Eve Cariou ◽  
Khailène Rguez ◽  
Pauline Fournier ◽  
Yoan Lavie-Badie ◽  
...  

BackgroundCardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA.MethodsClinical, biological and transthoracic echocardiographic parameters were retrospectively compared among 118 patients with CA. Global work index (GWI) was calculated as the area of left ventricular pressure–strain loop. Global work efficiency (GWE) was defined as percentage ratio of constructive work to sum of constructive and wasted works. Sixty-one (52%) patients performed a cardiopulmonary exercise.ResultsGWI, GWE, global longitudinal strain (GLS), left ventricular ejection fraction (LVEF) and myocardial contraction fraction (MCF) were correlated with N-terminal prohormone brain natriuretic peptide (R=−0.518, R=−0.383, R=−0.553, R=−0.382 and R=−0.336, respectively; p<0.001). GWI and GLS were correlated with peak oxygen consumption (R=0.359 and R=0.313, respectively; p<0.05). Twenty-eight (24%) patients died during a median follow-up of 11 (4–19) months. The best cut-off values to predict all-cause mortality for GWI, GWE, GLS, LVEF and MCF were 937 mm Hg/%, 89%, 10%, 52% and 15%, respectively. The area under the receiver operator characteristic curve of GWE, GLS, GWI, LVEF and MCF were 0.689, 0.631, 0.626, 0.511 and 0.504, respectively.ConclusionIn CA population, MW indices are well correlated with known prognosis markers and are better than LVEF and MCF in predicting mortality. However, MW does not perform better than GLS.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M D M Perez Gil ◽  
V Mora Llabata ◽  
A Saad ◽  
A Sorribes Alonso ◽  
V Faga ◽  
...  

Abstract BACKGROUND New echocardiographic phenotypes of heart failure (HF) are focused on myocardial systolic involvement of the left ventricle (LV), either endocardial and/or transmural. PURPOSE. To study the pattern of myocardial involvement in patients (p) with HF with preserved left ventricular ejection fraction (pLVEF) and cardiac amyloidosis (CA). METHODS. Comparative study of 16 p with CA and HF with pLVEF, considering as cut point LVEF &gt; 50%, in NYHA class ≥ II / IV, and a control group of 16 healthy people. Longitudinal Strain (LS) and Circumferential Strain (CS) were calculated using 2D speckle-tracking echocardiography, along with Mitral Annulus Plane Systolic Excursion (MAPSE) and Base-Apex distance (B-A). Also, the following indexes were calculated: Twist (apical rotation + basal rotation, º); Classic Torsion (TorC): (twist/B-A, º/cm); Torsion Index (Tor.I): (twist/MAPSE, º/cm) and Deformation Index (Def.I): (twist/LS, º). We suggest the introduction of these dynamic torsion indexes as Tor.I and Def.I that include twist per unit of longitudinal systolic shortening of the LV instead of using TorC which is the normalisation of twist to the end-diastolic longitudinal diameter of the LV. RESULTS There were no differences of age between the groups (68.2 ± 11.5 vs 63.7 ± 2.8 years, p = 0.14). Global values of LS and CS were lower in p with CA indicating endocardial and transmural deterioration during systole, while TorC and Twist of the LV remained conserved in p with CA. However, there is an increase of dynamic torsion parameters such as Tor.I and Def.I that show an increased Twist per unit of longitudinal shortening of the LV in the CA group (Table). CONCLUSIONS In p with CA and HF with pLVEF, the impairment of LS and CS indicates endocardial and transmural systolic dysfunction. In these conditions, LVEF would be preserved at the expense of a greater dynamic torsion of the LV. Table LS (%) CS (%) Twist (º) TorC (º/cm) Tor.I (º/cm) Def.I (º/%) CA pLVEF (n = 16) -11.7 ± 4.2 17.2 ± 4.8 19.8 ± 8.3 2.5 ± 1.1 27.7 ± 13.5 -1.8 ± 0.9 Control Group (n = 15) -20.6 ± 2.5 22.7 ± 4.9 21.7 ± 6.1 2.7 ± 0.8 16.4 ± 4.7 -1.0 ± 0.3 p &lt; 0.001 &lt; 0.01 0.46 0.46 &lt; 0.01 &lt; 0.01 Dynamic Torsion Indexes and Classic Torion Parameters in pLVEF CA patients vs Control group.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
R Ramos Polo ◽  
S Moral Torres ◽  
C Tiron De Llano ◽  
M Morales Fornos ◽  
J M Frigola Marcet ◽  
...  

Abstract INTRODUCTION Differential diagnosis by echocardiography between cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) is based on the evaluation of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) of the entire myocardial wall. Nevertheless, histopathological studies describe a higher involvement of subendocardial tissue in CA. The aim of our study was to evaluate whether the subanalysis of the GLS by layers (subendocardial and subepicardial) and segments (apical and basal) can provide further information. METHODS Retrospective study including 33 consecutive patients diagnosed with CA (with histological confirmation and imaging tests) or HCM by established criteria. Advanced myocardial deformation analysis software was used for both subendocardial and subepicardial evaluation of the left ventricle wall by transthoracic echocardiography. RESULTS Seventeen patients (52%) had CA and sixteen (48%) had HCM. Differences were observed in LVEF (52.9 ± 10.9% vs 62.4 ±5.0%; p = 0.004), but not in the analysis of the entire wall GLS (-12.3 ± 4.9 vs -13.4 ± 2.8; p = 0.457) nor in the LVEF/GLS ratio (4.7 ± 1.4 vs 4.8 ± 1.1; p = 0.718). In the layered analysis there was no difference in subendocardial GLS (-16.2 ± 5.0 vs -16.4 ± 3.2%; p = 0.916) or subepicardial GLS (-11.7 ± 4.1 vs -11.6 ±2.7%; p = 0.945); however, the increase in GLS from base to apex was greater for CA than for HCM both at subepicardial level (increase: 101% vs 16%; p = 0.006) and subendocardial level (increase: 242% vs 114%; p = 0.006), with inversion of the greatest values for each group (Fig. 1).The ratio (apical GLS/basal GLS) was diagnostic predictor of CA (area under the curve = 86%; p = 0.002): a value &gt;2 presented a sensitivity of 84% and a specificity of 85% for the diagnosis of CA. CONCLUSIONS CA presents an impairment of both subendocardial and subepicardial deformation in transthoracic echocardiography. These patterns provide additional information on differential diagnosis with HCM. Abstract P940 Figure. Subendo vs subepicardial mean values


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Goebel ◽  
S Schwuchow-Thonke ◽  
O Hahad ◽  
M Brandt ◽  
U Von Henning ◽  
...  

Abstract Background Cardiac amyloidosis (CA) is increasingly recognized as an underlying cause of heart failure with preserved ejection fraction (HFpEF), associated with high morbidity and mortality. However, most studies, solely investigated the prevalence of CA in special subgroups including HFpEF and severe aortic valve disease. Purpose With the present study we sought to investigate prevalence of different phenotypes of CA in an all comer-population of patients with non-ischaemic heart failure (HF) and to analyze the impact of CA on all-cause mortality. Methods The My Biopsy HF-Study (German clinical trials register number: 22178) is a retrospective monocentric study investigating the underlying etiology of HF in an all-comer population of patients with HF of unknown etiology. Patients presenting with symptoms of HF at the University Medical Centre between 14/10/2012 and 01/03/2021, who underwent endomyocardial biopsy (EMB) were enrolled in the present study. Ischaemic HF and valvular HF were ruled out prior to EMB. Specimens were sent for further examination to a specialized laboratory approved by the Food and Drug Administration Results Between October 2012 and March 2021, 767 patients (71.6% men) with HF of unknown etiology were included. Mean age at the time of presentation was 55.4 years (±14.4). Altogether, 72.5% of the patients presented with HF with reduced ejection fraction (HFrEF), 7.1% were diagnosed with HF with mid-range ejection fraction (HFmrEF) and 20.4% with HFpEF. Based on histological examination and genotyping, CA was diagnosed in 44 (5.7%) patients (immunglobulin light chain [AL] CA: 15 patients; variant transthyretin [ATTRv] CA: 6 patients; wild type transthyretin [ATTRwt] CA: 21 patients; de novo CA: 2 patients). Patients with CA were older compared with patients without CA (69.4±11.4 vs. 54.1±14.5; p&lt;0.0001), had a higher prevalence of arterial hypertension (68.2% vs. 50.9%; p=0.045) and showed a better left ventricular ejection fraction based on echocardiographic examination (47.5% vs. 32.6%; p&lt;0.0001). With respect to biomarker expression, levels of both brain natriuretic peptide and high-sensitive troponin I were significantly higher in patients without CA (BNP: 914.1 vs 612; p=0.01; troponin I: 812.8 vs. 171.7; p=0.006). In univariate logistic regression analysis CA was associated with a significant all-cause mortality (hazard ratio [HR] per unit increase [ui], 5.17, 95% CI, 2.93–9.08; p&lt;0.0001), even after adjustment for classical cardiovascular risk factors (HRperui 3.12, 95% CI, 1.11–8.76; p=0.03) and comorbidities like chronic obstructive pulmonary disease, chronic kidney disease and stroke (HRperui 2.93, 95% CI, 1.2–7.15; p=0.018). Conclusions Among patients presenting with HF of unknown etiology, including patients with HFpEF, HFmrEF and HFrEF, cardiac amyloidosis is the underlying cause of HF in 5.7% of patients and is independently associated with all-cause mortality. FUNDunding Acknowledgement Type of funding sources: None.


EP Europace ◽  
2020 ◽  
Vol 22 (8) ◽  
pp. 1216-1223
Author(s):  
Eun-Jeong Kim ◽  
Benjamin B Holmes ◽  
Shi Huang ◽  
Ricardo Lugo ◽  
Asad Al Aboud ◽  
...  

Abstract Aims Cardiac amyloidosis (CA) is associated with increased mortality due to arrhythmias, heart failure, and electromechanical dissociation. However, the role of an implantable cardioverter-defibrillator (ICD) remains unclear. We conducted case-control study to assess survival in CA patients with and without a primary prevention ICD and compared outcomes to an age, sex, and device implant year-matched non-CA group with primary prevention ICD. Methods and results There were 91 subjects with CA [mean age= 71.2 ± 10.2, female 22.0%, 49 AL with Mayo Stage 2.9 ± 1.0, 41 transthyretin amyloidosis (ATTR), 1 other] followed by Vanderbilt Amyloidosis centre. Patients with ICD (n = 23) were compared with those without (n = 68) and a non-amyloid group with ICD (n = 46). All subjects with ICD had implantation for primary prevention. Mean left ventricular ejection fraction was 36.2% ± 14.4% in CA with ICD, 41.0% ± 10.6% in CA without ICD, and 33.5% ± 14.4% in non-CA patients. Over 3.5 ± 3.1 years, 6 (26.1%) CA, and 12 (26.1%) non-CA subjects received ICD therapies (P = 0.71). Patients with CA had a significantly higher mortality (43.9% vs. 17.4%, P = 0.002) compared with the non-CA group. Mean time from device implantation to death was 21.8 months in AL and 22.8 months in ATTR patients. There was no significant difference in mortality between CA patients who did and did not receive an ICD (39.0% vs. 46.0%, P = 0.59). Conclusions Despite comparable event rates patients with CA had a significantly higher mortality and ICDs were not associated with longer survival. With the emergence of effective therapy for AL amyloidosis, further study of ICD is needed in this group.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Negareh Mousavi ◽  
Timothy Tan ◽  
Mohammad Ali ◽  
Elkan F. Halpern ◽  
Lin Wang ◽  
...  

Objectives: The aim of this study was to assess whether baseline echocardiographic measures of left ventricular (LV) size and function predict the development of symptomatic heart failure or cardiac death (major adverse cardiac events, MACE) in patients treated with anthracyclines who have a pre-chemotherapy left ventricular ejection fraction (LVEF) in the low normal range (between 50-59%). Background: Anthracycline-induced symptomatic heart failure and impaired LVEF are late and often irreversible manifestations of anthracycline-induced cardiotoxicity. The value of echocardiographic parameters of myocardial size and function before chemotherapy to identify patients at high-risk for development of symptomatic heart failure in patients with low normal LVEF was studied. Methods: Patients with a LVEF between 50 and 59% before anthracyclines were selected. In these patients, LV volumes, LVEF and peak longitudinal strain (GLS) were measured. Individuals were followed for MACE and all-cause mortality over a median of 659 days (range; 3-3704 days). Results: Of 2234 patients undergoing echocardiography for pre-anthracycline assessment, 158 (7%) had a resting ejection fraction of 50-59%. Their average LV end-diastolic volume (LVEDV) was 101±22ml, LVEF was 54 ±3% and global longitudinal strain (GLS) was -17.7±2.6%. Twelve patients experienced a MACE (congestive heart failure) at a median of 173 days (range; 15-530). Age, diabetes, previous coronary artery disease, LVEDV, LVESV and GLS were all-predictive of MACE (P= 0.015, 0.0043 and 0.0065 for LVEDV, LVESV, and GLS respectively). LVEDV and GLS remained predictive of MACE when adjusted for age. Age and GLS were also predictive of overall mortality (p<0.0001 and 0.0105 respectively). Conclusions: In patients treated with anthracyclines with an LVEF of 50-59%, both baseline EDV and GLS predict the occurrence of MACE. These parameters may help target patients who could bene[[Unable to Display Character: &#64257;]]t from closer cardiac surveillance and earlier initiation of cardioprotective medical therapy.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Rodolfo Caminiti ◽  
Antonio Parlavecchio ◽  
Giampaolo Vetta ◽  
Giuseppe Pelaggi ◽  
Francesca Lofrumento ◽  
...  

Abstract Aims Left ventricular function recovery (LV-REC) or left ventricular adverse remodelling (LV-REM) after acute myocardial infarction (AMI) play an important role for identifying patients at risk of heart failure. In this study we aim to evaluate the usefulness of non-invasive myocardial work (MW), a new index of global and regional myocardial performance, to predict LV-REC or LV-REM after AMI. Methods and results Fifty patients with AMI (mean age, 63.8 ± 13.4 years), treated by primary percutaneous coronary intervention (PCI), were prospectively enrolled. They underwent a baseline transthoracic Doppler echocardiography (TTE) within 48 h after PCI and a second TTE after a median of 31 days during the follow-up. MW was derived from the strain-pressure loops, integrating in its calculation the non-invasive arterial pressure, according to standard speckle tracking echocardiography recommendations. LV-REC was defined as an absolute improvement of left ventricular ejection fraction (LVEF) ≥ 5% from LVEF at baseline, whereas LV-REM was defined as an increase of ≥ 20% of the LV end diastolic volume (LVEDV) at 1 month follow-up. We overall found a significant improvement from baseline to one-month follow-up for values of LVEF (49.8 ± 9.5% vs. 52.8 ± 9.3%, P = 0.001), global longitudinal strain (GLS) (−13.4 ± 3.9% vs. −18.7 ± 5.4%, P = 0.016), global work index (GWI) (1368.6 ± 435.2 vs. 1788 ± 493 mmHg/%, P = 0.0001), global work efficiency (GWE) (89.96 ± 9.3% vs. 91.3 ± 6.4%, P = 0.001), global constructive work (GCW) (1619.16 ± 497.9 mmHg/% vs. 2008.6 ± 535.3 mmHg/%, P = 0.0001), global wasted work (GWW) (188.8 ± 19.8 mmHg/% vs. 149.2 ± 16.5 mmHg/%). However, LV-REC at 1 month of follow-up was observed only in 36% of the population enrolled, whereas LV-REM was described in 18% of cases. Using ROC curve analysis, we identified a cut off value of 202 mmHg/% for baseline GWW (sensitivity 75%, specificity 62%, AUC 0.6667, CI 95%: 0.51618–0.81715, P = 0.0001) to identify patients with LV-REM at 1 month. With regards to conventional echo parameters, patients with LV-REC showed lower baseline wall motion score index (WMSI) than those without LV-REC (1.73 vs. 1.38, P = 0.007). Conclusions Among standard and advanced TTE parameters, only baseline GWW is able to predict early LV-REM at 1 month after primary PCI. Therefore, it could be used during baseline evaluation of AMI patients for a more accurate stratification of those at higher risk of heart failure. However, further larger scale studies are needed to validate these findings.


2020 ◽  
Vol 41 (25) ◽  
pp. 2366-2375 ◽  
Author(s):  
Wunan Zhou ◽  
Jenifer M Brown ◽  
Navkaranbir S Bajaj ◽  
Alvin Chandra ◽  
Sanjay Divakaran ◽  
...  

Abstract Aims Hypertension is a well-established heart failure (HF) risk factor, especially in the context of adverse left ventricular (LV) remodelling. We aimed to use myocardial flow reserve (MFR) and global longitudinal strain (GLS), markers of subclinical microvascular and myocardial dysfunction, to refine hypertensive HF risk assessment. Methods and results Consecutive patients undergoing symptom-prompted stress cardiac positron emission tomography (PET)-computed tomography and transthoracic echocardiogram within 90 days without reduced left ventricular ejection fraction (&lt;40%) or flow-limiting coronary artery disease (summed stress score ≥ 3) were included. Global MFR was quantified by PET, and echocardiograms were retrospectively analysed for cardiac structure and function. Patients were followed over a median 8.75 (Q1–3 4.56–10.04) years for HF hospitalization and a composite of death, HF hospitalization, MI, or stroke. Of 194 patients, 155 had adaptive LV remodelling while 39 had maladaptive remodelling, which was associated with lower MFR and impaired GLS. Across the remodelling spectrum, diastolic parameters, GLS, and N-terminal pro-B-type natriuretic peptide were independently associated with MFR. Maladaptive LV remodelling was associated with increased adjusted incidence of HF hospitalization and death. Importantly, the combination of abnormal MFR and GLS was associated with a higher rate of HF hospitalization compared to normal MFR and GLS [adjusted hazard ratio (HR) 3.21, 95% confidence interval (CI) 1.09–9.45, P = 0.034), including in the adaptive remodelling subset (adjusted HR 3.93, 95% CI 1.14–13.56, P = 0.030). Conclusion We have demonstrated important associations between coronary microvascular dysfunction and myocardial mechanics that refine disease characterization and HF risk assessment of patients with hypertension based on subclinical target organ injury.


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