The right heart in cardiac amyloidosis: a comparison between subtypes and with other genetic and non-genetic causes of left ventricular hypertrophy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Palmiero ◽  
M Rubino ◽  
E Monda ◽  
M Caiazza ◽  
M.G Trinchillo ◽  
...  

Abstract Background Right chambers involvement is common in cardiac amyloidosis (CA) but has been ever compared to control groups. Purpose Aim of this study is to compare right heart involvement between CA subgroups (AL vs. ATTR amyloidosis) and between CA and other forms of genetic and non-genetic left ventricular hypertrophy. Methods We enrolled 25 patients with CA (10 pts with AL and 15 pts with wild type ATTR amyloidosis) and 75 patients with LVH (25 HCM pts; 25 HypCMP pts; 25 AS pts). Beside routine echocardiographic measurements, we analysed right chambers dimensions and classical and novel parameters for right ventricular (RV) function [TAPSE (Tricuspid Anulus Plane Systolic Excursion), St (S' wave at RV TDI), global and free-wall strain]. Results ATTR group showed higher right dimensions compared to AL, without differences in RV systolic parameters (see table). CA patients, compared to LVH group, showed no differences in right dimensions. RV systolic parameters were significantly reduced while diastolic Doppler parameters were higher (E/E' 21.7±9.0 vs. 11.2±5.0; p<0.0001). At ROC curve analysis TAPSE showed the best ability in discriminating CA among other forms of LVH (AUC 0.936; 95% CI: 0.879–0.993; p<0.0001), with a sensibility of 94.7% and specificity of 87.3% for a cut-off value of 19.5 mm. At Kaplan-Meier estimation CA patients showed a significantly higher cardiovascular mortality compared to LVH group (9/25 deaths vs. none). At multivariate analysis TAPSE was the only independent prognostic factor (β 1.324; 95% IC: 1.086–1.614; p<0.006). Discussion CA group showed a significantly impaired RV systolic function with higher pulmonary pressures compared to LVH group. TAPSE proved to be the only able to discriminate CA among genetic and non-genetic forms of LVH and also to have prognostic significance. Funding Acknowledgement Type of funding source: None

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  

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.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Nelson Samesima ◽  
Carlos A Pastore ◽  
Luciana D de Matos ◽  
Fernanda F Fumagalli ◽  
Mariane V Ferreira ◽  
...  

Introduction. The widely known electrocardiographic criteria for diagnosing left ventricular hypertrophy (LVH) use QRS complex voltages to define whether there is left ventricle enlargement or not. Mild myocardial hypertrophy is detected in many professional athletes and this is a consequence of their daily intensity of training. Thus it is not unusual that athlete’s ECGs show large QRS voltages with normal hearts. Objective. To evaluate the applicability of the usual electrocardiographic criteria for LVH - Sokolow-Lyon, Romhilt-Estes, Cornell and Gubner - in a population of professional athletes. Methods. The four LVH criteria for diagnosing LVH were applied to analyse ECGs of 107 professional athletes (71% soccer players, 29% marathonists, all male, age 25± 10 years, training for 9± 8 years) by the same observer unaware of echocardiographic results. ECG was considered to be indicative of LVH if: Sokolow-Lyon ≥35mm (V 1or 2 S wave+V 5or 6 R wave); Romhilt-Estes score ≥5 points (frontal plane: R or S waves ≥ 20mm, horizontal plane: R or S waves ≥ 30mm, Morris indices, V 5or 6 strain pattern, left axis deviation ≥ − 30°, intrinsecoid deflection ≥ 0.04s, QRS duration ≥ 0.10s) ; Cornell ≥ 28mm (aV L R wave + V 3 S wave); Gubner ≥ 22mm (D I R wave + D III S wave). Hypertrophy was considered whenever: LV diastolic diameter ≥ 60mm and/or septum ≥ 13mm and/or LV posterior wall ≥ 13mm. Kruskal-Wallis was used to statistically analyse quantitative variables, corrected chi-square test for categorical variables. Significance level: p ≤ 0.05. Results. Romhilt-Estes showed the best results (75% sensitivity, 84% specificity, 16 false-positives, 1 false-negative), and was the only criteria with statistical significance (p = 0.047). Sokolow-Lyon showed 100% sensitivity, 15% specificity, p = 0.545, 88% false-positives, 0% false-negative. Cornell and Gubner showed 25% and 0% sensitivity, 95% and 99% specificity, p=0.205 and p = 0.449, respectively. Conclusion. In this male population of professional athletes, Romhilt-Estes score proved to be the best criterion for identifying left ventricular hypertrophy, while Sokolow-Lyon criterion did not discriminate normal from abnormal hearts. Cornell and Gubner criteria should not be used in this population because of their low sensitivity.


1997 ◽  
Vol 272 (5) ◽  
pp. H2416-H2424 ◽  
Author(s):  
M. Qi ◽  
T. R. Shannon ◽  
D. E. Euler ◽  
D. M. Bers ◽  
A. M. Samarel

To determine whether reduced sarcoplasmic reticulum (SR) Ca(2+)-adenosinetriphosphatase (ATPase) (SERCA2) activity contributes to delayed myocardial relaxation during chronic left ventricular hypertrophy (LVH) progression, LVH was produced in rats by abdominal aortic coarctation. Systolic and diastolic functions were assessed in vivo 8 and 16 wk after surgery, and compositional alterations in LV myocardium [SERCA2 concentration, myosin heavy chain (MHC) isoenzymes, and tissue collagen] were correlated with the development of prolonged isovolumic relaxation and impaired cardiac performance over time. Myocardial relaxation was prolonged in 8-wk banded rats, despite normal isovolumic systolic function and LV end-diastolic pressure (LVEDP). No significant alterations in SERCA2 protein, beta-MHC, or fibrillar collagen levels were observed at this early time point. In contrast, LV SERCA2, beta-MHC, and fibrillar collagen concentrations were all significantly altered in 16-wk banded rats. These late compositional changes were associated with reduced cardiac performance, as manifested by a significant elevation in LVEDP (14 +/- 2 mmHg). The 34% decrease in SERCA2 protein was associated with reduced SR Ca2+ uptake and an even greater reduction (76%) in SERCA2 mRNA. SERCA2 mRNA levels were also significantly reduced to 43 +/- 10% of sham-operated rats 8 wk after banding, despite unchanged SERCA2 protein levels and normal SR Ca2+ uptake. These results argue against a significant contribution of SERCA2 downregulation to the subtle alterations in myocardial relaxation observed in compensated LVH. However, the early reduction in SERCA2 mRNA levels may serve as a molecular marker for impaired cardiac performance during the transition from compensated LVH to heart failure.


2004 ◽  
Vol 286 (6) ◽  
pp. R1085-R1092 ◽  
Author(s):  
Marja Luodonpää ◽  
Hanna Leskinen ◽  
Mika Ilves ◽  
Olli Vuolteenaho ◽  
Heikki Ruskoaho

We examined whether adrenomedullin, a vasoactive peptide expressed in the heart, modulates the increase in blood pressure, changes in systolic and diastolic function, and left ventricular hypertrophy produced by long-term administration of ANG II or norepinephrine in rats. Subcutaneous administration of adrenomedullin (1.5 μg·kg−1·h−1) for 1 wk inhibited the ANG II-induced (33.3 μg·kg−1·h−1 sc) increase in mean arterial pressure by 67% ( P < 0.001) but had no effect of norepinephrine-induced (300 μg·kg−1·h−1 sc) hypertension. Adrenomedullin enhanced the ANG II-induced improvement in systolic function, resulting in a further 9% increase ( P < 0.01) in the left ventricular ejection fraction and 19% increase ( P < 0.05) in the left ventricular fractional shortening measured by echocardiography, meanwhile norepinephrine-induced changes in systolic function were remained unaffected. Adrenomedullin had no effect on ANG II- or norepinephrine-induced left ventricular hypertrophy or expression of hypertrophy-associated genes, including contractile protein and natriuretic peptide genes. The present study shows that adrenomedullin selectively suppressed the increase in blood pressure and augmented the improvement of systolic function induced by ANG II. Because adrenomedullin had no effects on ANG II- and norepinephrine-induced left ventricular hypertrophy, circulating adrenomedullin appears to act mainly as a regulator of vascular tone and cardiac function.


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