scholarly journals Evaluation of Perugini score and myocardial mass and voltage abnormalities in transthyretine cardiac amyloidosis

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Costa ◽  
L Bichon ◽  
A Maouche ◽  
P Durdon ◽  
R Pouy ◽  
...  
Keyword(s):  
Bone Scintigraphy ◽  
Cardiac Amyloidosis ◽  
Structural Parameters ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Cardiac Mass ◽  
Mass Ratio ◽  
Myocardial Mass ◽  
Complete Evaluation ◽  
Cardiac Uptake

Abstract Background Transthyretine cardiac amyloidosis (TTR-CA) is an emerging treatable disease in cardiology. Severity of TTR infiltration can be assessed by bone scintigraphy with Perugini score, depending on HPD-Tc99M myocardial intensity uptake. Less is known on how Perugini score interplays with routine cardiac structural parameters, especially cardiac mass and voltage. Aim To evaluate correlation between Perugini score and myocardial mass and voltage abnormalities in patients with TTR-CA. Methods Patients diagnosed with ATTR-CA and addressed to our center for treatment underwent complete evaluation including electrocardiogram (ECG) and transthoracic echocardiography (TTE). Routine electrical and echographic parameters were obtained such as: Sokolow indice (SV1+RV5) in millivolt (mV), PR delay (ms), QRS width (in ms), left ventricular mass (LVM, in grams) and diastolic interventricular septum (dIVS) thickness (in mm). Patients were classified in three categories according to Perugini score: I (cardiac uptake < chest bones), II (cardiac uptake = chest bones) and III (cardiac uptake > bones). Main end-point were LVM and Voltage/Mass ratio (VMR) variations, depending on Perugini score. Results Among the 17 patients included, 14 were male (82.4%) and mean age was 82±8 years old. On ECG, 6 patients (35.3%) had permanent atrial fibrillation (AF). Mean voltage according to Sokolow index, mean PR delay and mean QRS width were respectively 1.5±0.5 mV, 212±54 ms and 113±19 ms. On TTE, mean LVM and mean dIVS width were respectively 333±98 g (188±55 g/m2) and 19±4 mm. Mean voltage/mass ratio (VMR) was 0.48±0.26 uV/g (see table). On bone scintigraphy, 3 patients were scored with Perugini I (17.6%), 4 with Perugini II (23.5%) and 10 with Perugini III (58.8%). According to Pearson test, there was a strong correlation between Perrugini score and LVM (r=+0.56, p=0.03) (graph A), and a strong negative correlation between Perrugini score and VMR (r=−0.70, p=0.003) (graph B). There were a trend for correlation between Perugini score and Voltage according to Sokolow (r=−0.46, p=0.07) and between Perugini score and dIVS width (r=0.49, p=0.07). Conclusion In TTR-CA patients, Perugini score appears to strongly correlate with left ventricle mass and voltage. Larger scale studies are needed to confirm these results. FUNDunding Acknowledgement Type of funding sources: None. ECG and TEE trends according to Perugini Cardiac mass and voltage with Perugini

scholarly journals P1829CARDIOVASCULAR DISORDERS IN CHILDREN WITH CHRONIC KIDNEY DISEASE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Aigul Nugmanova ◽  
Nuriya Meriken ◽  
Saule Dikanbayeva ◽  
Zeinep Zhumagulova ◽  
Zhuldyz Baidauletova ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Clinical Laboratory ◽  
Interventricular Septum ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Myocardial Mass ◽  
Cardiac Events ◽  
Initial Stage ◽  
Echocardiographic Examination ◽  
Left Ventricular Myocardial Mass

Abstract Background and Aims Pathology of the cardiovascular system is the leading cause of death in patients with CKD, while determining the causes of the formation of cardiac events is often difficult. Method We conducted an analysis of echocardiography data performed by 50 children with CKD. On the echoCG, the final systolic and diastolic sizes of the left ventricle (FSS and FDS), the final systolic and diastolic volumes of the left ventricle (FSV and FDV) were determined; measured the thickness of the interventricular septum (IVS) and the posterior wall of the left ventricle (PWLV). Left ventricular myocardial mass (LVMM) was determined by the formula proposed by R. Devereux and N. Reichek: LVMM = 1.04x (/ IVS+PWLV+FDS/3-FDS3) - 13.6where, IVS - thickness of IVS in diastole,PWLV-thickness of PWLV in diastole, FDS-final diastolic size of the left ventricle. Results We conducted an analysis of echocardiography data performed by 50 children with CKD. On the echoCG, the final systolic and diastolic sizes of the left ventricle (FSS and FDS), the final systolic and diastolic volumes of the left ventricle (FSV and FDV) were determined; measured the thickness of the interventricular septum (IVS) and the posterior wall of the left ventricle (PWLV). Left ventricular myocardial mass (LVMM) was determined by the formula proposed by R. Devereux and N. Reichek: LVMM = 1.04x (/ IVS+PWLV+FDS/3-FDS3) - 13.6where, IVS - thickness of IVS in diastole,PWLV-thickness of PWLV in diastole, FDS-final diastolic size of the left ventricle. Conclusion The mechanisms of damage to the heart and blood vessels in patients with CKD begin to function already in the initial stage of renal failure and increase as it progresses. The need to know the data of clinical, laboratory and instrumental examination methods at the terminal stage of CKD is dictated, first of all, by the possibility of exposure to them. An important stimulus for conducting an echocardiographic examination is the early detection and correction of cardiovascular disorders, in connection with the prospect of increasing the survival of patients after kidney transplantation.

scholarly journals Incidental cardiac uptake in bone scintigraphy: increased importance and association with cardiac amyloidosis

2021 ◽  
pp. 20200161
Author(s):  
Francis T Delaney ◽  
Philip Dempsey ◽  
Ivan Welaratne ◽  
Bryan Buckley ◽  
Donagh O'Sullivan ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Differential Diagnosis ◽  
Coronary Artery ◽  
Soft Tissue ◽  
Bone Scintigraphy ◽  
Cardiac Amyloidosis ◽  
Cardiac Uptake ◽  
Undiagnosed Disease ◽  
Radiotracer Uptake ◽  
Artery Disease

Extraosseous radiotracer uptake during bone scintigraphy must be carefully assessed and it offers the potential to detect previously undiagnosed disease processes. A range of neoplastic, metabolic, traumatic, ischaemic and inflammatory disorders can cause soft tissue accumulation of bone avid radiopharmaceuticals. Accordingly, cardiac uptake in bone scintigraphy has a broad differential diagnosis and is commonly attributed to ischaemia/infarction related to coronary artery disease. However, there has been renewed focus on incidental cardiac uptake in recent years in light of significant developments in the diagnosis and management of cardiac amyloidosis.

scholarly journals Evaluation of myocardial strain assessed by CMR tissue-tracking to predict adverse cardiovascular events in patients with cardiac amyloidosis

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
R Fernandez Galera ◽  
I Dentamaro ◽  
Y Belahnech ◽  
X Cia ◽  
L Gutierrez ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Myocardial Mass ◽  
Transthyretin Amyloidosis ◽  
Ventricular Ejection Fraction ◽  
Tissue Tracking

Abstract Background Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease. Purpose The aim of our study was to assess the prognostic value of cardiac magnetic resonance parameters, including Tissue deformation, in cardiac amyloidosis patients. Methods We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. Contrast-enhanced CMR was performed on a 1.5 T clinical scanner. Routine analysis was performed with a semi-automatic software for volumetric analysis. When late gadolinium enhancement was present, percentage of area of enhancement (LGE) was calculated.All strain parameters were measured off-line using dedicated software. Left ventricular circumferential strain (CS) measurements were obtained using mid-ventricular level short-axis cine views. Longitudinal strain (LS) derived from cine SSFP of 2-, 3-, and 4-chamber long axis views.Finally we evaluated the CMR parameters related with the combined event of mortality and heart failure in the follow-up. Results A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 59p (60,8%) died or present heart failure during the follow-up. Patients who died or present heart failure in the follow-uo had lower left ventricular ejection fraction (48,25±12,2% vs 56,13±11,03%, p=0,003), higher myocardial mass (156,05±54,8g vs 120,84±56,1g, p=0,007), higher LGE (8,6±6,8% vs 4,4±2%), worst GLS (−14,76±6,1% vs −18,67±6,2%) and worst GCS (−23,3±9,7% vs −30,04±9,1%). Both, GLS and GCS were independently associated with the combined event of dead or heart failure when evaluated within a multivariate analysis in a Cox regression model, but GCS was the stronger predictor of events in the follow-up over other CMR parameters like LGE an myocardial mass (p<0,001). Tertile distribution for GCS identified subgroups with different adverse events, particularly for the lowest-risk tertile: GCS <−34%, who had a combinated event in 13,6% of cases, significantly lower than patients in the mid-risk tertile (41,5%) and highest-risk tertile (53,8%) (Log-rank p=0,02) Conclusion Mortality and appearance of heart failure in cardiac amyloid patients is high. The assessment of myocardial strain parameters by CMR tissue-tracking in this population is useful to predict adverse outcomes in the follow-up. Particularly, GCS, stratify patients in subgroups with different risk of events, with added value to classical CMR parameters. FUNDunding Acknowledgement Type of funding sources: None. Death & Heart Failure by GCS

scholarly journals Concordance between quantitative and semi-quantitative analysis of Tc-99m-pyrophosphate scintigraphy for the diagnosis of transthyretin cardiac amyloidosis in patients with HFpEF

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Murat ◽  
H.E Yalvac ◽  
G.O Mert ◽  
I.A Sivrikoz ◽  
Y Cavusoglu
Keyword(s):  
Quantitative Analysis ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Visual Assessment ◽  
Interatrial Septum ◽  
Left Ventricular ◽  
Myocardial Uptake ◽  
Cardiac Uptake ◽  
Funding Sources ◽  
American Society

Abstract Background Transthyretin (TTR) cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF). Cardiac scintigraphy with 99mTechnetium-pyrophosphate (99mTc-PYP) is referred as a simple, non-invasive and reliable method in the diagnosis of TTR-CA. American Society of Nuclear Cardiology Practice Points recommends two interpretative approaches: the quantitative heart-to-contralateral lung ratio (H/CL) at 1 hour or the semi-quantitative visual assessment at 3 hours after radiotracer injection. Purpose In this study, we evaluated the concordance between semi-quantitative and quantitative approaches in the diagnosis of TTR CA in patients with HFpEF. Methods This single-center, prospective study included 78 patients who had a diagnosis of HFpEF according to 2016 ESC HF guidelines. 99mTc-PYP cardiac scintigraphy was performed in 43 patients who have ≥2 red flags for TTR-CA including left ventricular hypertrophy (LVH) (wall thickness ≥12 mm), biventricular hypertrophy, sparkling pattern, reduction in longitudinal strain with apical sparing, thickening of the interatrial septum (>6mm), low-voltage, pseudo infarct pattern or atrioventricular block on ECG. In the absence of monoclonal protein in the serum and urine, Grade 2 to 3 myocardial uptake in semi-quantitative analysis at 3 hours or a H/CL ratio of ≥1.5 in quantitative analysis at 1 hour post injection of 99mTc-PYP is considered positive for TTR-CA. Grade 2–3 uptake with a H/CL ratio ≥1.5 or Grade 0–1 uptake with a H/CL ratio <1.5 were considered as concordant results. Grade 2–3 uptake with a H/CL ratio <1.5 or Grade 0–1 uptake with a H/CL ratio ≥1.5 were considered as discordant results. Results Mean age of study population was 68.26±9.97 years. 17 (39.5%) of 43 patients who underwent 99mTc-PYP cardiac scintigraphy showed a Grade 2 or 3 cardiac uptake and in these patients with Grade 2–3 uptake, 11 patients (65%) had a H/CL ratio ≥1.5 (concordant results) and 6 patients (35%) had a H/CL ratio <1.5 (discordant results). 26 (60.5%) of 43 patients showed Grade 0–1 cardiac uptake. All patients (100%) with Grade 0–1 uptake had a H/CL ratio <1.5 and therefore, showed concordant results. Overall, 37 (86%) patients had concordant and 6 (14%) patients had discordant results (Table 1). Conclusion The results of this study showed that although there was a high agreement between semi-quantitative and quantitative analysis of 99mTc-PYP cardiac scintigraphy, 14% of patients have discordant results and need further workup to confirm TTR-CA in patients with HFpEF. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Pfizer independent grant.

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 Association between Neonatal Intakes and Hyperglycemia, and Left Heart and Aortic Dimensions at 6.5 Years of Age in Children Born Extremely Preterm

2021 ◽  
Vol 10 (12) ◽  
pp. 2554
Author(s):  
Jawwad Hamayun ◽  
Lilly-Ann Mohlkert ◽  
Elisabeth Stoltz Sjöström ◽  
Magnus Domellöf ◽  
Mikael Norman ◽  
...  
Keyword(s):  
Interventricular Septum ◽  
Posterior Wall ◽  
Root Diameter ◽  
Left Ventricular ◽  
Left Heart ◽  
Extremely Preterm ◽  
Extremely Preterm Infants ◽  
Nutritional Data ◽  
Aortic Dimensions

Survivors of extremely preterm birth (gestational age < 27 weeks) have been reported to exhibit an altered cardiovascular phenotype in childhood. The mechanisms are unknown. We investigated associations between postnatal nutritional intakes and hyperglycemia, and left heart and aortic dimensions in children born extremely preterm. Postnatal nutritional data and echocardiographic dimensions at 6.5 years of age were extracted from a sub-cohort of the Extremely Preterm Infants in Sweden Study (EXPRESS; children born extremely preterm between 2004–2007, n = 171, mean (SD) birth weight = 784 (165) grams). Associations between macronutrient intakes or number of days with hyperglycemia (blood glucose > 8 mmol/L) in the neonatal period (exposure) and left heart and aortic dimensions at follow-up (outcome) were investigated. Neonatal protein intake was not associated with the outcomes, whereas higher lipid intake was significantly associated with larger aortic root diameter (B = 0.040, p = 0.009). Higher neonatal carbohydrate intake was associated with smaller aorta annulus diameter (B = −0.016, p = 0.008). Longer exposure to neonatal hyperglycemia was associated with increased thickness of the left ventricular posterior wall (B = 0.004, p = 0.008) and interventricular septum (B = 0.004, p = 0.010). The findings in this study indicate that postnatal nutrition and hyperglycemia may play a role in some but not all long-lasting developmental adaptations of the cardiovascular system in children born extremely preterm.

Epicardial fat thickness in infants of diabetic mothers

2021 ◽  
pp. 1-6
Author(s):  
M.M. Vela-Huerta ◽  
N. Amador-Licona ◽  
R. Domínguez-Damiá ◽  
A. Heredia-Ruiz ◽  
H.V. Orozco-Villagomez ◽  
...  
Keyword(s):  
Interventricular Septum ◽  
Left Ventricular ◽  
Epicardial Fat ◽  
Left Ventricular Ejection ◽  
Epicardial Fat Thickness ◽  
Infants Of Diabetic Mothers ◽  
Significant Difference ◽  
Echocardiographic Parameters ◽  
Fat Thickness ◽  
Diabetic Mothers

BACKGROUND: Epicardial fat thickness (EFT) in adults and children has been related to the metabolic syndrome and other cardiovascular risk factors; however, scarce studies have evaluated it in infants of diabetic mothers (IDM) in whom, alterations in the thickness of the interventricular septum have been reported. This study compares the EFT in IDM versus infants of non-diabetic mothers (INDM) and its association with others echocardiographic parameters. METHODS: We performed a cross sectional study in 93 infants (64 IDM and 29 INDM). To evaluate EFT dimensions, an echocardiogram was performed within the first 24h of extrauterine life in both groups. In diabetic mothers, HbA1c was also determined. RESULTS: There was no significant difference in birth weight between the groups although gestational age was lower in IDM. The EFT (3.6 vs. 2.5 mm, p <  0.0001), the interventricular septum thickness (IVST) (6.2 vs. 5.2 mm, p <  0.0001) and the IVST / left ventricle posterior wall (1.3 vs. 1.1, p = 0.001) were higher in the IDM; while the left ventricular expulsion fraction [LVFE] (71.1 vs. 77.8; p <  0.0001) was lower than in the INDM, respectively. We found a positive correlation between EFT with IVST (r = 0.577; p = 0.0001), LVPW (r = 0.262; p = 0.011), IVST/LVPW index (r = 0.353; p = 0.001), and mitral integral early velocity (r = 0.313; p = 0.002), while a negative correlation with LVFE was observed (r = –0.376; p = 0.0001). CONCLUSIONS: The EFT is higher in IDM than in INDM. It was positively related with echocardiographic parameters of left ventricular thickness and negatively with left ventricular ejection function.

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