scholarly journals Primary Carnitine Deficiency: A Rare, Reversible Metabolic Cardiomyopathy

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Stephen Tomlinson ◽  
John Atherton ◽  
Sandhir Prasad
Keyword(s):  
Right Ventricular ◽  
Systolic Function ◽  
Systolic Dysfunction ◽  
Rare Condition ◽  
Left Ventricular ◽  
Carnitine Deficiency ◽  
Dramatic Improvement ◽  
Ventricular Dilation ◽  
Primary Carnitine Deficiency ◽  
Magnetic Resonance Imaging Mri

A 24-year-old female with a diagnosis of primary carnitine deficiency, a rare inherited metabolic disorder predominantly described in the paediatric literature that causes cardiomyopathy, presented for evaluation after three months of nonadherence with prescribed carnitine therapy. Initial echocardiography demonstrated severe left ventricular dilation (104 ml/m2) (normal < 76 ml/m2) with moderate systolic dysfunction (ejection fraction 40%) and severe right ventricular dilation with mild systolic dysfunction. Carnitine replacement was commenced, and a cardiac magnetic resonance imaging (MRI) performed five days later demonstrated dramatic improvement in biventricular function with normalization of left and right ventricular systolic function. To our knowledge, this is only the second case describing the rapid reversal of cardiomyopathy in an adult patient with this rare condition.

scholarly journals Rapid Fire Abstract session: assessment of systolic function: clinical perspectives and future directions782How the echocardiographic parameters of left ventricular function change during the first year after myocardial infarction when the ejection fraction remains normal?783Blunted increase in LV longitudinal deformation during exercise contributes to the transition from an asymptomatic stage to clinically overt HFpEF784A septal flash induced by right ventricular pacing is associated with left ventricular dysfunction and remodeling785Assessment of right ventricular systolic function in patients with significant functional tricuspid regurgitation: longitudinal parameters increase accuracy and predict cardiovascular outcomes786Effect of left ventricular augmentation with alginate biopolymer on right heart function787Left ventricular mechanics: novel tools to evaluate function and dyssynchrony in controls and cardiac resynchronization therapy candidates788How does regional wall thickness influence strain measurements?789TAPSE-estimated right ventricular systolic dysfunction predicts mortality among acute decompensated heart failure with preserved ejection fraction patients: a prospective study in a secondary center

2015 ◽  
Vol 16 (suppl 2) ◽  
pp. S130-S132 ◽  
Author(s):  
T Baron ◽  
W Kosmala ◽  
S I Sarvari ◽  
A Garcia Martin ◽  
S I Dumitrescu ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Right Ventricular ◽  
Acute Decompensated Heart Failure ◽  
Systolic Function ◽  
Systolic Dysfunction ◽  
Decompensated Heart Failure ◽  
Left Ventricular ◽  
Cardiac Resynchronization ◽  
Ventricular Mechanics ◽  
Functional Tricuspid Regurgitation

ACCESSMENT OF RIGHT VENTRICULAR SYSTOLIC FUNCTION USING TAPSE

2011 ◽  
pp. 62-70
Author(s):  
Lien Nhut Nguyen ◽  
Anh Vu Nguyen
Keyword(s):  
Heart Failure ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Systolic Function ◽  
Systolic Heart Failure ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction

Background: The prognostic importance of right ventricular (RV) dysfunction has been suggested in patients with systolic heart failure (due to primary or secondary dilated cardiomyopathy - DCM). Tricuspid annular plane systolic excursion (TAPSE) is a simple, feasible, reality, non-invasive measurement by transthoracic echocardiography for evaluating RV systolic function. Objectives: To evaluate TAPSE in patients with primary or secondary DCM who have left ventricular ejection fraction ≤ 40% and to find the relation between TAPSE and LVEF, LVDd, RVDd, RVDd/LVDd, RA size, severity of TR and PAPs. Materials and Methods: 61 patients (36 males, 59%) mean age 58.6 ± 14.4 years old with clinical signs and symtomps of chronic heart failure which caused by primary or secondary DCM and LVEF ≤ 40% and 30 healthy subject (15 males, 50%) mean age 57.1 ± 16.8 were included in this study. All patients and controls were underwent echocardiographic examination by M-mode, two dimentional, convensional Dopler and TAPSE. Results: TAPSE is significant low in patients compare with the controls (13.93±2.78 mm vs 23.57± 1.60mm, p<0.001). TAPSE is linearly positive correlate with echocardiographic left ventricular ejection fraction (r= 0,43; p<0,001) and linearly negative correlate with RVDd (r= -0.39; p<0.01), RVDd/LVDd (r=-0.33; p<0.01), RA size (r=-0.35; p<0.01), TR (r=-0.26; p<0.05); however, no correlation was found with LVDd and PAPs. Conclusions: 1. Decreased RV systolic function as estimated by TAPSE in patients with systolic heart failure primary and secondary DCM) compare with controls. 2. TAPSE is linearly positive correlate with LVEF (r= 0.43; p<0.001) and linearly negative correlate with RVDd (r= -0.39; p<0.01), RVDd/LVDd (r=-0.33; p<0.01), RA size (r=-0.35; p<0.01), TR (r=-0.26; p<0.05); however, no correlation is found with LVDd and PAPs. 3. TAPSE should be used routinely as a simple, feasible, reality method of estimating RV function in the patients systolic heart failure DCM (primary and secondary).

Regional Left Ventricular Systolic Function in Relation to the Cavity Geometry in Patients With Chronic Right Ventricular Pressure Overload

Circulation ◽  
1995 ◽  
Vol 91 (9) ◽  
pp. 2359-2370 ◽  
Author(s):  
Sheng-Jing Dong ◽  
Adrian P. Crawley ◽  
John H. MacGregor ◽  
Yael Fisher Petrank ◽  
Dale W. Bergman ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Systolic Function ◽  
Pressure Overload ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Ventricular Pressure ◽  
Ventricular Systolic Function

scholarly journals Central and obstructive apneas prevalence in heart failure with reduced, mid-range and preserved ejection fraction

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Borrelli ◽  
P Sciarrone ◽  
F Gentile ◽  
N Ghionzoli ◽  
G Mirizzi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Left Ventricular Systolic Dysfunction ◽  
Systolic Function ◽  
Systolic Dysfunction ◽  
Cardiopulmonary Exercise Testing ◽  
Left Ventricular ◽  
Apnea Hypopnea Index ◽  
Preserved Ejection Fraction ◽  
2D Echocardiography

Abstract Background Central apneas (CA) and obstructive apneas (OA) are highly prevalent in heart failure (HF) both with reduced and preserved systolic function. However, a comprehensive evaluation of apnea prevalence across HF according to ejection fraction (i.e HF with patients with reduced, mid-range and preserved ejection fraction- HFrEf, HFmrEF and HFpEF, respectively) throughout the 24 hours has never been done before. Materials and methods 700 HF patients were prospectively enrolled and then divided according to left ventricular EF (408 HFrEF, 117 HFmrEF, 175 HFpEF). All patients underwent a thorough evaluation including: 2D echocardiography; 24-h Holter-ECG monitoring; cardiopulmonary exercise testing; neuro-hormonal assessment and 24-h cardiorespiratory monitoring. Results In the whole population, prevalence of normal breathing (NB), CA and OA at daytime was 40%, 51%, and 9%, respectively, while at nighttime 15%, 55%, and 30%, respectively. When stratified according to left ventricular EF, CA prevalence decreased from HFrEF to HFmrEF and HFpEF: (daytime CA: 57% vs. 43% vs. 42%, respectively, p=0.001; nighttime CA: 66% vs. 48% vs. 34%, respectively, p&lt;0.0001), while OA prevalence increased (daytime OA: 5% vs. 8% vs. 18%, respectively, p&lt;0.0001; nighttime OA: 20 vs. 29 vs. 53%, respectively, p&lt;0.0001). When assessing moderte-severe apneas, defined with an apnea/hypopnea index &gt;15 events/hour, prevalence of CA was again higher in HFrEF than HFmrEF and HFpEF both at daytime (daytime moderate-severe CA: 28% vs. 19% and 23%, respectively, p&lt;0.05) and at nighttime (nighttime moderate-severe CA: 50% vs. 39% and 28%, respectively, p&lt;0.05). Conversely, moderate-severe OA decreased from HFrEF to HFmrEF to HFpEF both at daytime (daytime moderate-severe OA: 1% vs. 3% and 8%, respectively, p&lt;0.05) and nighttime (noghttime moderate-severe OA: 10% vs. 11% and 30%, respectively, p&lt;0.05). Conclusions Daytime and nighttime apneas, both central and obstructive in nature, are highly prevalent in HF regardless of EF. Across the whole spectrum of HF, CA prevalence increases and OA decreases as left ventricular systolic dysfunction progresses, both during daytime and nighttime. Funding Acknowledgement Type of funding source: None

scholarly journals Prognostic Value of Echocardiographic Right Ventricular Function Parameters in the Presence of Severe Tricuspid Regurgitation

2021 ◽  
Vol 10 (11) ◽  
pp. 2266
Author(s):  
Matthias Schneider ◽  
Varius Dannenberg ◽  
Andreas König ◽  
Welf Geller ◽  
Thomas Binder ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Ventricular Function ◽  
Tricuspid Regurgitation ◽  
Right Ventricular Function ◽  
Prognostic Value ◽  
Large Scale ◽  
Systolic Function ◽  
Left Ventricular ◽  
Severe Tricuspid Regurgitation ◽  
Vena Contracta

Background: Presence of severe tricuspid regurgitation (TR) has a significant impact on assessment of right ventricular function (RVF) in transthoracic echocardiography (TTE). High trans-valvular pendulous volume leads to backward-unloading of the right ventricle. Consequently, established cut-offs for normal systolic performance may overestimate true systolic RVF. Methods: A retrospective analysis was performed entailing all patients who underwent TTE at our institution between 1 January 2013 and 31 December 2016. Only patients with normal left ventricular systolic function and with no other valvular lesion were included. All recorded loops were re-read by one experienced examiner. Patients without severe TR (defined as vena contracta width ≥7 mm) were excluded. All-cause 2-year mortality was chosen as the end-point. The prognostic value of several RVF parameters was tested. Results: The final cohort consisted of 220 patients, 88/220 (40%) were male. Median age was 69 years (IQR 52–79), all-cause two-year mortality was 29%, median TAPSE was 19 mm (15–22) and median FAC was 42% (30–52). In multivariate analysis, TAPSE with the cutoff 17 mm and FAC with the cutoff 35% revealed non-significant hazard ratios (HR) of 0.75 (95%CI 0.396–1.421, p = 0.38) and 0.845 (95%CI 0.383–1.867, p = 0.68), respectively. TAPSE with the cutoff 19 mm and visual eyeballing significantly predicted survival with HRs of 0.512 (95%CI 0.296–0.886, p = 0.017) and 1.631 (95%CI 1.101–2.416, p = 0.015), respectively. Conclusions: This large-scale all-comer study confirms that RVF is one of the main drivers of mortality in patients with severe isolated TR. However, the current cut-offs for established echocardiographic parameters did not predict survival. Further studies should investigate the prognostic value of higher thresholds for RVF parameters in these patients.

Abstract P024: Pharmacological Treatment of Hypertension and Lv Dysfunction Predictors in ESRD Patients With AV Fistula

Hypertension ◽  
2015 ◽  
Vol 66 (suppl_1) ◽  
Author(s):  
Sayed Tariq ◽  
James Anderson ◽  
Rohit Dhingra ◽  
Mikhail Torosoff
Keyword(s):  
Systolic Function ◽  
Beta Blockers ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Channel Blockers ◽  
Ca Channel ◽  
Av Fistula ◽  
Fistula Surgery ◽  
Lv Dysfunction ◽  
Ca Channel Blockers

Background: Effects of anti-hypertensive medications on left ventricular dimensions and systolic function in patients with arterio-venous (AV) fistulas have not been well investigated. Material and Methods: Medical charts and echocardiograms of 346 patients with AV fistula were reviewed. Of 346, 149 patients had TTE prior to the AV fistula surgery, 197 had TTE after the AV fistula surgery, and 76 patients had TTE before and after the AV fistula surgery. Data on medication use was available in 314 patients. ANOVA, chi-square, and logistic regression tests were employed. Results: In patients scheduled for AV fistula placement, 20% (31/149) patients had systolic dysfunction and 15% (22/142) had increased LV end-diastolic dimensions (LVEDD). Moderate systolic LV dysfunction was observed in 6% (9/149) and additional 8% (12/149) had severe LV dysfunction. Increased LVEDD with some LV dysfunction was noted in 27% (38/142).Following the AV fistula placement, 18% (36/197) of patients had systolic dysfunction and 12% (22/187) had increased LV end-diastolic dimensions (LVEDD). Moderate or severe systolic LV dysfunction was observed in 6% (5/197). LV systolic dysfunction or dilatation was noted in 23% (43/187). Of 314 patients, 63% were on beta-blockers (BB), 25% were on ACE inhibitor or an ARB , 43% on calcium-channel blocker , and 15% on alpha-antagonist . BB, ACEi-ARB, or AA were not associated with increased LVEDD or systolic dysfunction before or after the AV fistula placement. Prior to AV fistula, CCB treatment was not related to LV dilatation (36% in each group, p=0.981) Post AV fistula, CCB treatment was associated with increased LV dimensions (71% vs. 46%, p=0.029) but not LV systolic dysfunction (49% in LV dysfunction vs. 38% in the rest, p=0.446) . This association persisted after adjustment for co-morbidities and demographic parameters. Conclusions: LV systolic dysfunction and/or dilatation are common in patients undergoing AV fistula surgery. Despite decreased use of Ca-channel blockers in patients with LV dysfunction prior to AV fistula, Ca-channel blockers are associated with increased LV dimensions post AV fistula, and probably should be avoided in this vulnerable patient population.

scholarly journals P797 Assessment of right ventricular systolic function with 3D echocardiography following cardiac resynchronization therapy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Jenei ◽  
E Papp ◽  
M Clemens ◽  
Z Csanadi
Keyword(s):  
Cardiac Resynchronization Therapy ◽  
Right Ventricular ◽  
Systolic Function ◽  
Left Ventricular ◽  
3D Echocardiography ◽  
Cardiac Resynchronization ◽  
Resynchronization Therapy ◽  
S Wave ◽  
Ventricular Systolic Function ◽  
Significant Difference

Abstract Background In approximately 30-40% of cases, the left ventricular systolic function does not improve following cardiac resynchronization therapy (CRT; non-responders). Currently, the role of right ventricular (RV) systolic function is not yet completely clear in the background. Our aim was to assess the RV systolic function with 3D echocardiography in CRT patients. Methods We selected 19 patients who received CRT in our department between May and June 2017, and whose 1-year follow-up data were available. We characterized several 2D parameters of RV systolic function, such as RV free wall strain (RV GLSFW), annular s’ wave velocity (TDI s), tricuspid annulus plane systolic excursion (TAPSE), RV fractional area change (RV FAC). A number of 3D parameters were also assessed, such as RV ejection fraction (EF), end-diastolic (EDV) and end-systolic (ESV) volumes, using a dedicated RV analysis software. Moreover, we measured the LV EF and considered the patients "responder", when the LV EF improved with at least 10% after CRT implantation. Results From 19 patients, 12 was identified as responders (R) and 7 as non-responders (NR). No significant difference was seen in the mean age of patients in the two groups (NR: 68 ± 6 year; R: 67 ± 9 year, p = 0.76), however, the proportion of male individuals was higher in the NR group (8/12 vs. 1/7). The RV EF was higher in the R group (41 ± 8% vs.29 ± 10%; p = 0.012), while the EDV or ESV did not differ between the two groups. The RV GLSFW (–21.2 ± 7% vs.–13.9 ± 7%, p = 0.045) and the TAPSE (16.8 ± 5 mm vs.11.4 ± 3 mm, P = 0.03) values were significantly different between the two groups. Based on logistic regression analysis, the RV EF was an independent predictor of non-respondence. Conclusions The lower RV EF indicates non-respondence to CRT, however, it is not associated with RV dilation, i.e.adverse remodelling. These results suggest mechanical abnormality of RV function in the background of impaired EF.

scholarly journals Correlation between hemoglobin level and left ventricular systolic functions and dimensions in children with chronic severe anemia

2011 ◽  
Vol 51 (2) ◽  
pp. 79
Author(s):  
Erlina Masniari Napitupulu ◽  
Fera Wahyuni ◽  
Tina Christina L. Tobing ◽  
Muhammad Ali ◽  
Bidasari Lubis
Keyword(s):  
Systolic Function ◽  
Pearson Correlation ◽  
Systolic Dysfunction ◽  
Univariate Analysis ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Lv Function ◽  
Severe Anemia ◽  
Cross Sectional ◽  
Lv Systolic Function

Background Chronic severe anemia is a connnon disease. Cardiac output may increase when the hemoglobin (Hb) level decreases to < 7 g/dL for 3 months or more. Alteration of left ventricular (LV) function occurs frequently in children 'With chronic severe anemia, in the {onn of concentric LV hypertrophy, LV dilatation with or v.ithout LV hypertrophy, or systolic dysfunction. Objective To examine the correlation between Hb level and alteration of LV systolic function in children with chronic severe anemia. Methods We conducted a cross-sectional study in Adam Malik Hospital from October to December 2009. Subjects were chronic severely anemic children. Left ventricular systolic function (ejection fraction/EF, fractional shortening/FS) and dimensions (left ventricular end diastolic diameter/LVEDD and left ventricular end systolic diameter/LVESD) were measured using Hitachi EUB 5500 echocardiography unit. Univariate analysis  and Pearson correlation were performed.Results Thirty children were enrolled in the study. The mean of age was 113.5 months (SD 53.24). Hb values ranged from 2.1 to 6.9 g/dL with mean value of 4.6 g/dL (SD 1.44). Mean duration of anemia was 3.9 months (SD 0.70). Chronic severe anemia was not associated \\lith decreased LV systolic function [EF 62.2% (SD 9.16), r =0.296, P=0.112; FS 33.8% (SD 7.26), r =0.115, P=0.545], nor LV dimension changes [LVEDD 40.2 mm (SD 6.85), r = -0.192, P=0.308; LVESD 26.2 mm (SD 4.98), r=-0.266, P=0.156]. Conclusion There was no correlation between Hb level in chronically anemic children and changes in LV systolic function or dimension.

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