scholarly journals Type 1 cluster of differentiation 36 deficiency-related cardiomyopathy accelerates heart failure with co-existing mitral valve prolapse: a case report

2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Midori Miyazaki ◽  
Yasunori Suematsu ◽  
Seiya Kato ◽  
Shin-Ichiro Miura
Keyword(s):  
Heart Failure ◽  
Fatty Acid ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Heart Diseases ◽  
Left Ventricular ◽  
Type I ◽  
Acid Uptake ◽  
Cd36 Deficiency ◽  
Cluster Of Differentiation

Abstract Background Free fatty acid is a major energy source in the healthy heart and cluster of differentiation 36 (CD36) partially regulates the rate of myocardial fatty acid uptake. Here, we report a case of CD36 deficiency-related cardiomyopathy with a unique pathophysiology. Heart failure was accelerated by co-existing mitral valve prolapse (MVP) without a distinct phenotype of hypertrophic or dilated cardiomyopathy. Case summary A middle-aged man was aware of dyspnoea and hospitalized for heart failure with MVP. Cluster of differentiation 36 deficiency was found based on the absence of myocardial 123l-15-(p-iodophenyl)-3-(R,S)-methyl pentadecanoic acid (BMIPP) uptake by myocardial scintigraphy. Type I CD36 deficiency was further diagnosed by the lack of CD36 in both platelets and monocytes by flow cytometry. Left ventricular muscle was obtained intraoperatively, and a histological examination reflected compensative hypertrophy of cardiomyocytes with myofibrillar loss and reactive fibrosis. The microvascular structure around the cardiomyocytes was highlighted by immunohistochemical staining for CD31, while CD36 expression was absent. He had an operation of mitral valve replacement and improved heart failure. Discussion Cluster of differentiation 36 deficiency potentially mediates various pathological conditions in the heart. Incidental CD36 deficiency-related cardiomyopathy may accelerate heart failure in the presence of co-existing heart diseases. BMIPP scintigraphy might be helpful for predicting CD36 deficiency.

scholarly journals Replacement Myocardial Fibrosis in Patients with Mitral Valve Prolapse: Relation to Mitral Regurgitation, Ventricular Remodeling and Arrhythmia

Circulation ◽  
2021 ◽  
Author(s):  
Anne-Laure Constant Dit Beaufils ◽  
Olivier Huttin ◽  
Antoine Jobbe-Duval ◽  
Thomas Senage ◽  
Laura Filippetti ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Ventricular Arrhythmia ◽  
Myocardial Fibrosis ◽  
Mitral Valve Prolapse ◽  
Cardiovascular Events ◽  
Prognostic Significance ◽  
Left Ventricular ◽  
Arterial Embolism

Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) in patients with MVP. Methods: Four hundred patients (53±15 years, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE CMR, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 myocardial wall including 71 basal inferolateral wall, 29 papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45 vs 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (OR 1.01, 95% CI [1.002-1.017], P=0.009) and moderate-severe MR (OR: 2.28, 95% CI [1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ vs 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (HR: 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP, is associated with mitral valve apparatus alteration, more dilated LV, MR grade, ventricular arrhythmia, and is independently associated with cardiovascular events. These findings suggest a MVP-related myocardial disease. Finally, CMR provides additional information to echocardiography in MVP.

scholarly journals Mitral Valve Disease in Thyroid Storm

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A941-A942
Author(s):  
Sandra Rocio Rivera Menjura ◽  
Lia G Moyano Rivas ◽  
Camila Parraguez Gamboa ◽  
Cristobal Balmaceda ◽  
Juan P Peralta ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Invasive Mechanical Ventilation ◽  
Mitral Valve Insufficiency ◽  
Left Ventricular ◽  
Thyroid Storm ◽  
Multiple Infections ◽  
Mitral Insufficiency ◽  
Type I ◽  
Valve Insufficiency

Abstract Introduction: The cardiovascular effects that thyroid gland causes are widely studied. In fact, there is a known correlation between Graves’ Disease and mitral valve damage. We present the case of a patient admitted with thyroid storm and heart failure associated with severe structural damage of the mitral valve papillary muscle. Case Report: 24 year old woman with hyperthyroidism diagnosed 12 years ago, treated irregularly with thiamazole and propranolol, leaving treatment a year ago, presents dyspnea, class III functional capacity, diarrhea and logic dysphagia of a month of evolution. Heart rate over 170 bpm, respiratory rate 48 rpm and blood pressure 143/84 mmHg. Physical exam positive for exophthalmos, grade III goiter, crackles in both lung bases, pretibial myxedema and fulfilling criteria for a thyroid storm (65 points in Burch-Wartofsky Point Scale). First Lab Results: TSH&lt;0.005µU/mL, free T4&gt;7.7ng/dl and TRAB 37.8UI/L. Chest ray: Global cardiomegaly and pulmonary edema. EKG: Narrow complex supraventricular tachycardia. Thyroid ultrasound: Intrathoracic goiter. Transesophageal echocardiogram: Severe mitral insufficiency (Carpentier Type I and IIIB), right cavities and left ventricular enlargement, preserved right ventricular function and severe pulmonary hypertension (PSAP 71-76 mmHg). First treated with thiamazole, hydrocortisone IV, cholestyramine and sedation, falling time after into ventilatory failure and developing delirium, requiring invasive mechanical ventilation. Tested positive for COVID- 19. Starts preparation with Lugol and undergoes Total Thyroidectomy. After surgery develops severe hypocalcemia secondary to transitory hypoparathyroidism. During hospitalization presents multiple infections including pneumonia (Pseudomonas Aeruginosa), lung aspergillosis, bacteriuria (Enteroccocus Faecium) and candiduria (Candida Albicans and Glabrata), each one treated with multiple antibiotics and vasoactive drugs. Once stable, mitral valve replacement is realized, after which, the patient progresses favorably being discharged with programmed ambulatory controls. Conclusion: We report a case of a patient who was presented with positive thyroid storm criteria associated with heart failure and severe mitral valve insufficiency. The case gets complicated as multiple infections take place, including COVID-19. Fortunately, because of the early and aggressive multidisciplinary management, the patient evolved favorably, overcoming the life-threatening conditions she went through. Key Words: Thyroid storm, mitral valve insufficiency, heart failure. Bibliography: Klein I, Danzi S. Thyroid disease and the heart. Circulation. 2007 Oct 9;116(15):1725-35. doi: 10.1161/CIRCULATIONAHA.106.678326. Erratum in: Circulation. 2008 Jan 22;117(3):e18. PMID: 17923583.

scholarly journals P302 Forgotten and neglected: LV hypertrophy and LV remodeling as important predictors in patients with mitral valve prolapse

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Trifunovic ◽  
O Petrovic ◽  
M Tomic-Dragovic ◽  
I Paunovic ◽  
V Tutus ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Left Ventricular ◽  
Secondary Outcome ◽  
Multivariable Model ◽  
Chi Square ◽  
Increased Risk ◽  
Lv Mass

Abstract Current ESC guidelines recommends left ventricular (LV) end-systolic diameter (ESD), LV ejection fraction (LV EF), systolic pulmonary arterial pressure (SPAP) as key parameters in a multifactorial treatment algorithm for chronic severe primary MR. However, LV hypertrophy (LVH) and LV remodelling during the process of adaptation to chronic MR can influence further clinical course. Aim of this study was to test whether LVH and distinctive LV geometry are coupled with increased risk for heart failure (HF) development and occurrence of major adverse cardiac event (MACE) among patients with MVP and can they improve power of statistical models for HF and MACE prediction based on parameters supported by the current guidelines. Methods 376 pts diagnosed with mitral valve prolapse (MVP) between 1. January 2014. and 31. December 2017 and with complete medical chart and follow-up data from central echo laboratory in the tertiary health center were enrolled in the study. Four types of LV geometry were identified: Type 1 (normal LV mass with normal geometry), Type 2 (normal LV mass with concentric remodeling), Type 3 (eccentric hypertrophy) and Type 4 (concentric hypertrophy). The primary outcome was HF and secondary outcome was MACE (HF development, myocardial infarction, myocardial revascularisation (both PCI and/or ACBG) and cardiac death). Results The distribution of patients was as follow: 51.2% (Group 1) vs 3.3% (Group 2) vs 41.4 % (Group 3) vs 4.1% (Group 4). In multivariable model the highest OR for HF development after adjustment for age, ESD and LVH, had concentric LVH (OR= 5.361, p= 0.004, 95% CI 1.696-16.648), then EF &lt; 60% (OR= 3.025, p = 0.004, 95% CI 1.427-6.411) and the lowest OR had SPAP &gt; 40 mmHg (OR = 2.274, p = 0.039, 95% 1.43-4.958). Adding LVH significantly increased model’s power to predict HF above traditional parameters (Chi-square from 19.386 to 23.640, p &lt; 0.001; Nagelkerke R square from 0.090 to 0.110), whereas addition of LV geometry increased it even more (Chi-square from 23.640 to 28.729, p &lt; 0.001; Negelkerke R square from 0.110 to 0.132). Independent MACE predictors in multivariable model were: EF &lt; 60% (OR 3.645, p &lt; 0.001, 95% CI 1.808- 7.50), new onset atrial fibrillation during the follow-up (OR =3.327, p = 0.012, 95% CI 0.305-8.484), concentric LVH (OR= 4.241, p = 0.015, 95% CI 1.327-13.550) and normal LV geometry without LVH (OR= 0.514, p = 0.002, 95% CI 0.288-0.918), even after adjustment for MV surgery. Adding LVH significantly improved model’s power (Chi-square from 29.026 to 35.112, p &lt; 0.001; Nagelkerke R square 0.121 to 0.146) to predict MACE and addition of type of LV geometry provided additional strength (Chi-square from 35.112 to 39.707, p &lt; 0.001; Nagelkerke R square from 0.146 to 0.164). Conclusion LVH and especially concentric LVH are independent predictors of heart failure development and MACE in mitral valve prolapse and significantly improves predictive powers of the models based on traditional parameters.

scholarly journals Pursuing the non-invasive assessment of cardiac contractility: the added value of pressure-area-strain loop analysis in volume overload-induced heart failure

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Tokodi ◽  
BK Lakatos ◽  
M Ruppert ◽  
A Olah ◽  
AA Sayour ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Cardiac Contractility ◽  
Volume Overload ◽  
Left Ventricular ◽  
Stroke Work ◽  
Loading Conditions ◽  
Innovation And Technology ◽  
Pressure Area ◽  
Area Strain

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): This work was supported by the New National Excellence Programme (ÚNKP-19-3-I) of the Ministry for Innovation and Technology in Hungary, and the Artificial Intelligence Research Field Excellence Programme of the National Research, Development and Innovation Office of the Ministry of Innovation and Technology in Hungary. Background Global longitudinal strain (GLS) by speckle-tracking echocardiography (STE) is a sensitive parameter of left ventricular (LV) systolic function. Nevertheless, GLS is dependent on loading conditions. Through the analysis of pressure-strain loops, myocardial work was recently introduced and tested in different clinical scenarios. Myocardial work incorporates afterload, but still, it neglects changes in preload and LV geometry. Purpose Accordingly, our aim was to test our hypothesis that adding instantaneous LV size to myocardial work calculation can further mitigate the load-dependency of GLS, and therefore, a better correlation with intrinsic myocardial contractility can be achieved. Methods Volume overload-induced heart failure was established by an aortocaval fistula (ACF) in male Wistar rats (n = 12). Age-matched sham-operated animals served as controls (n = 12). STE was performed to assess GLS, which was immediately followed by invasive pressure-volume (P-V) analysis to assess LV pressure and to compute a gold-standard index of cardiac contractility (preload recruitable stroke work [PRSW]). Global myocardial work index (GMWI) was calculated from GLS and the invasively measured LV pressure. To compute GMWI indexed to LV area (GMWIA), the instantaneous power (calculated by multiplying the strain rate and the instantaneous LV pressure) was divided by the instantaneous LV area, and then it was integrated from mitral valve closure until mitral valve opening. Results LV ejection fraction did not differ significantly (ACF vs. controls: 59 ± 4 vs. 65 ± 9%, p = NS), whereas GLS (Figure 1A - representative animals) was slightly decreased in the ACF group (-13.2 ± 2.3 vs. -15.4 ± 1.9%, p &lt; 0.05). In contrast, PRSW, GMWI (Figure 1B - representative animals) and GMWIA (Figure 1C - representative animals) were considerably reduced in ACF compared to controls (57 ± 13 vs. 111 ± 38mmHg, 1383 ± 382 vs. 1928 ± 281mmHg%, 11.6 ± 3.7 vs. 47.9 ± 22.8mmHg%/mm2, all p &lt; 0.01). GLS showed moderate correlation with PRSW (r=-0.550, p &lt; 0.01), whereas GMWI correlated more significantly, but still moderately with the invasively measured LV contractility (r = 0.681, p &lt; 0.001). Correlation between the pressure-area-strain loop-derived GMWIA and P-V analysis-derived PRSW (Figure 1D) was found to be very strong (r = 0.924, p &lt; 0.001). Conclusions In the case of LV volume overload-induced heart failure, our pressure-area-strain loop-derived metric reflected LV contractility better than GLS and even GMWI. Therefore, the incorporation of instantaneous LV size into myocardial work calculation represents a promising clinical tool to assess and monitor intrinsic myocardial function independently of loading conditions. Abstract Figure 1

Abstract 14664: Reduced Activin Like Kinase 1 Activity Promotes Cardiac Fibrosis in Heart Failure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Kevin Morine ◽  
Vikram Paruchuri ◽  
Xiaoying Qiao ◽  
Emily Mackey ◽  
Mark Aronovitz ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Remodeling ◽  
Cardiac Fibrosis ◽  
Type I Collagen ◽  
Transforming Growth Factor ◽  
Left Ventricular ◽  
Lv Function ◽  
Type I ◽  
Mrna Levels ◽  
Protein Levels

Introduction: Activin receptor like kinase 1 (ALK1) mediates signaling via transforming growth factor beta-1 (TGFb1), a pro-fibrogenic cytokine. No studies have defined a role for ALK1 in heart failure. We tested the hypothesis that reduced ALK1 expression promotes maladaptive cardiac remodeling in heart failure. Methods and Results: ALK1 mRNA expression was quantified by RT-PCR in left ventricular (LV) tissue from patients with end-stage heart failure and compared to control LV tissue obtained from the National Disease Research Interchange (n=8/group). Compared to controls, LV ALK1 mRNA levels were reduced by 85% in patients with heart failure. Next, using an siRNA approach, we tested whether reduced ALK1 levels promote TGFb1-mediated collagen production in human cardiac fibroblasts. Treatment with an ALK1 siRNA reduced ALK1 mRNA levels by 75%. Compared to control, TGFb1-mediated Type I collagen and pSmad-3 protein levels were 2.5-fold and 1.7-fold higher, respectively, after ALK1 depletion. To explore a role for ALK1 in heart failure, ALK1 haploinsufficient (ALK1) and wild-type mice (WT; n=8/group) were studied 2 weeks after thoracic aortic constriction (TAC). Compared to WT, baseline LV ALK1 mRNA levels were 50% lower in ALK1 mice. Both LV and lung weights were higher in ALK1 mice after TAC. Cardiomyocyte area and LV mRNA levels of BNP, RCAN, and b-MHC were increased similarly, while SERCa levels were reduced in both ALK1 and WT mice after TAC. Compared to WT, LV fibrosis (Figure) and Type 1 Collagen mRNA and protein levels were higher among ALK1 mice. Compared to WT, LV fractional shortening (48±12 vs 26±10%, p=0.01) and survival (Figure) were lower in ALK1 mice after TAC. Conclusions: Reduced LV expression of ALK1 is associated with advanced heart failure in humans and promotes early mortality, impaired LV function, and cardiac fibrosis in a murine model of heart failure. Further studies examining the role of ALK1 and ALK1 inhibitors on cardiac remodeling are required.

Abstract P132: Increased Cardiovascular Parasympathetic Tone in African Americans With CD36 Partial Deficiency

Hypertension ◽  
2017 ◽  
Vol 70 (suppl_1) ◽  
Author(s):  
Shahram Ejtemaei Mehr
Keyword(s):  
Heart Rate ◽  
Fatty Acid ◽  
African Americans ◽  
Heart Function ◽  
Taste Perception ◽  
Acid Uptake ◽  
High Fat ◽  
Cd36 Deficiency ◽  
Parasympathetic Tone ◽  
Fat Meal

Cardiovascular disease is the leading cause of death among African Americans (AA). Reduced parasympathetic tone as measured by high frequency heart rate variability (HF RRI ) predicts cardiovascular mortality. HF RRI is reduced after a high fat meal through caveolar sequestration of muscarinic M2 receptors. The fatty acid translocase CD36 is a protein abundant in the myocardium and important for heart function and lipid metabolism. CD36 plasma membrane localization and function in fatty acid uptake is modulated by its interaction with caveolin. One in four AAs are G-allele carriers for CD36 SNP rs3211938 resulting in ~50% decreased CD36 expression. CD36 deficiency also reduces fat taste perception, which might lead to higher fat intake to reach taste saturation. We tested the hypothesis that obese AAs with partial CD36 deficiency have altered parasympathetic tone during fasting and after a high-fat meal. We recruited 13 G-allele carriers and 39 non-carriers. Subjects were matched by age (P=0.820), BMI (P=0.751), and blood pressure (P=0.701). There was a trend towards reduction in heart rate in carriers (P=0.07). Baseline HF RRI was elevated in G carriers (557.1 [251 to 942] vs. 224 [95 to 655] ms 2 , P=0.046). Eleven subjects received a high-fat meal (700 Cal/m 2 BSA, 80% fat). HF RRI was measured at baseline and 30, 60, 120, 240 minutes after meal. Non-carriers (n=4) showed a time-dependent decline in the percent change in HF RRI (-23, -32, -70, -84, respectively). In G-allele carriers (N=6), the decline in HF RRI (21, -11, -61, -70 min) was attenuated. Conclusion: AAs with partial CD36 deficiency have enhanced fasting parasympathetic tone and a blunted response to a high fat meal.

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