Myocardial interstitial fibrosis assessed by extracellular volume quantification is a determinant of symptoms in aortic valve regurgitation with preserved ejection fraction

Abstract Funding Acknowledgements Type of funding sources: None. Introduction According to current guidelines indication for surgery is straightforward with a class I recommendation in case of severe symptomatic aortic regurgitation (AR) and/or left ventricular ejection fraction (LVEF) decrease ≤50%. However, the management of patients with asymptomatic severe AR with preserved LVEF remains debated, with a cruel lack of prognostic factors to identify patients who may benefit from early intervention. An explanation to the absence of such factors is that the determinants of symptoms, a strong prognostic parameter, have been poorly identified. Beyond LV dilation and systolic dysfunction, which are both recognized prognostic factors in chronic AR, we hypothesized that interstitial myocardial fibrosis, as an early indicator of LV remodeling, may also influence the occurrence of symptoms. Cardiovascular magnetic resonance (CMR)-based myocardial extracellular volume (ECV) quantification by T1 mapping has emerged as a valuable tool to quantify diffuse myocardial fibrosis. Objective To study the relationship between myocardial interstitial fibrosis quantified by T1 mapping and the symptomatic status of patients with chronic aortic valve regurgitation. Methods We retrospectively included 38 consecutive patients with chronic, isolated, mild to severe AR who underwent a CMR at our institution. Exclusion criteria were the presence of any other heart condition that may induce myocardial fibrosis, ≥ mild associated valve disease, AR secondary to endocarditis, genetic, inflammatory or congenital disease except bicuspid aortic valve. T1 mapping of the basal segments was performed before and after contrast administration measuring native and post-contrast T1 relaxation time and ECV. Results Mean age was 56 ± 20 years, 30 patients (79%) were males, and symptoms were reported in 11 patients (29%). Mean LVEF was 57 ± 9% and ≥50% in 30 patients (79%). Aortic valve regurgitation fraction (RF) was 25 ± 13%, ECV 0.27 ± 0.04%, indexed LV end-diastolic volume (LVEDVi) 98 ± 32 ml/m2, end-systolic volume (LVESVi) 46 ± 19 ml/m2, and LV mass 79 ± 21 g/m2. LVESVi (r = 0.41,p = 0.01), LVEF (r=-0.59,p = 0.0001), and ECV (r = 0.42,p = 0.008) were correlated with symptoms, whereas age (r = 0.16,p = 0.33), gender (r=-0.24,p = 0.15), LVEDVi (r = 0.28,p = 0.09), LV mass index (r = 0.08,p = 0.62), and RF (r = 0.31,p = 0.06) were not. In the subgroup of patients with preserved LVEF (≥50%), after adjustment for LVESVi and RF, only ECV remained independently associated with symptoms (p = 0.046). Interestingly, when including the patients with a reduced LVEF < 50% in the multivariable analysis only LVESVi was an independent determinant of symptoms (p = 0.04) and ECV was not (p = 0.07) Conclusion myocardial fibrosis quantified by ECV calculation is a determinant of symptoms in AR with preserved LVEF. Further studies are warranted to determine the prognostic value of ECV that may justify earlier intervention. Abstract Figure. ECV in AR with preserved LVEF

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Aortic valve replacement due to granulomatosis with polyangiitis: a case series

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa307 ◽

2020 ◽

Vol 4 (5) ◽

pp. 1-6

Author(s):

Gilles Uijtterhaegen ◽

Laura De Donder ◽

Eline Ameloot ◽

Kristof Lefebvre ◽

Jo Van Dorpe ◽

...

Keyword(s):

Heart Disease ◽

Infective Endocarditis ◽

Aortic Valve ◽

Valve Replacement ◽

Valvular Heart Disease ◽

Granulomatosis With Polyangiitis ◽

Surrounding Tissue ◽

Valve Regurgitation ◽

Aortic Valve Regurgitation ◽

Severe Aortic Regurgitation

Abstract Background Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a systemic inflammatory process predominantly affecting upper and lower respiratory tract and kidneys. Valvular heart disease is a rare manifestation of GPA. Case summary We report two cases of acute valvular heart disease mimicking acute endocarditis caused by GPA. Both patients were middle-aged females with acute aortic valve regurgitation suggestive of possible infective endocarditis. In their recent medical history, atypical otitis and sinusitis were noted. The first patient was admitted with heart failure and the second patient because of persisting fever. Echocardiogram revealed severe aortic regurgitation with an additional structure on two cusps, suggestive of infective endocarditis in both patients. Urgent surgical replacement was performed; however, intraoperative findings did not show infective endocarditis, but severe inflammatory changes of the valve and surrounding tissue. In both patients, the valve was replaced by a prosthetic valve. Microscopic examination of the valve/myocardial biopsy showed diffuse acute and chronic inflammation with necrosis and necrotizing granulomas, compatible with GPA after infectious causes were excluded. Disease remission was obtained in both patients, in one patient with Rituximab and in the other with Glucocorticoids and Cyclophosphamide. Both had an uneventful follow-up. Discussion Granulomatosis with polyangiitis can be a rare cause of acute aortic valve regurgitation mimicking infective endocarditis with the need for surgical valve replacement. Atypical ear, nose, and throat symptoms can be a first sign of GPA. Symptom recognition is important for early diagnosis and appropriate treatment to prevent further progression of the disease.

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Comparison of bicuspid and tricuspid aortic valve repair

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa462 ◽

2020 ◽

Author(s):

Radosław Gocoł ◽

Jarosław Bis ◽

Marcin Malinowski ◽

Joanna Ciosek ◽

Damian Hudziak ◽

...

Keyword(s):

Aortic Valve ◽

Hazard Ratio ◽

Aortic Valve Repair ◽

Pericardial Patch ◽

Valve Regurgitation ◽

Aortic Valve Regurgitation ◽

Valve Repair ◽

Tricuspid Aortic Valve ◽

Valve Annulus ◽

Annulus Diameter

Abstract OBJECTIVES The aim of this study was to compare the outcomes of tricuspid aortic valve (TAV) and bicuspid aortic valve (BAV) repair. METHODS We assessed mortality, freedom from reoperation and the rate of aortic valve regurgitation recurrence. Mortality in both groups was compared with expected survival, and risk factors for reoperation were identified. RESULTS From January 2010 to April 2020, a total of 368 elective aortic valve repair procedures were performed, including 223 (60.6%) in patients with TAV. The perioperative mortality was 0.7% in the BAV group and 3.6% in the TAV group (P = 0.079). Estimated survival at 5 years in the BAV versus TAV group was 97 ± 3% vs 80 ± 6%, respectively (P < 0.001). Freedom from reoperation at 5 years in the TAV versus BAV group was 96 ± 3% vs 93 ± 4%, respectively (P = 0.28). Grade 2 or more aortic valve regurgitation was noted in 9.9% of BAV patients and 11% of TAV patients (P = 0.66). Reoperation was predicted by cusp perforation [hazard ratio 15.86 (4.44–56.61); P < 0.001], the use of pericardial patch [hazard ratio 8.58 (1.96–37.53); P = 0.004] and aortic valve annulus diameter >27.5 mm [hazard ratio 3.07 (0.99–9.58); P = 0.053]. CONCLUSIONS BAV repair is as durable as TAV repair. BAV is not a predictor of a higher rate of reoperations. BAV repair yields survival comparable to expected. Cusp perforation, aortic valve annulus diameter >27.5 mm and the use of pericardial patch adversely impact long-term outcome of aortic valve repair.

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Perioperative management with biologics on severe aortic valve regurgitation caused by Behçet syndrome: the experience from a single center

Therapeutic Advances in Chronic Disease ◽

10.1177/20406223211026753 ◽

2021 ◽

Vol 12 ◽

pp. 204062232110267

Author(s):

Luxi Sun ◽

Jinjing Liu ◽

Xiufeng Jin ◽

Zhimian Wang ◽

Lu Li ◽

...

Keyword(s):

Aortic Valve ◽

Perioperative Management ◽

Perioperative Period ◽

Valve Regurgitation ◽

Aortic Valve Regurgitation ◽

Behçet Syndrome ◽

Reactive Protein ◽

Behcet Syndrome ◽

Sparing Effect ◽

Median Interval

Background: To investigate the efficacy and safety of biologics in the perioperative management of severe aortic valve regurgitation (AR) caused by Behçet syndrome (BS). Methods: We retrospectively analyzed 20 patients with severe AR caused by BS who were all treated with biologics during the perioperative period of cardiac surgeries in our center between February 2016 and October 2020. Results: A total of 20 patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1 ± 8.8 years and a median course of 8 [interquartile range (IQR) 5.25–10.00] years. Before biologic administration, 92.9% of the patients who underwent aortic valve replacement had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics were administered with background glucocorticoids (GCs) and immunosuppressants during the perioperative period for 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR 2.75–4.25) months in 13 cases and within 3 months postoperatively in 9 cases. After a median follow up of 21 (IQR 15–32) months, 2 out of 13 cases (15.4%) preoperatively, and 1 out of 9 cases (11.1%) postoperatively treated with biologics developed PVL, and the rest were event free. The Behçet’s Disease Current Activity Form score improved significantly (7 versus 0, median, p < 0.0001). Decrease of erythrocyte sedimentation rate [25.0 (IQR 11.00–36.25) mm/h versus 6.5 (IQR 4.0–8.8) mm/h, p < 0.001], and C-reactive protein [20.77 (IQR 7.19–29.58) mg/l versus 1.53 (IQR 0.94–2.92) mg/l, p = 0.001] were achieved rapidly and effectively. The GC dosage tapered from 40 (IQR 30–60) mg/d to 10 (IQR 5–11.25) mg/d, p < 0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed. Conclusion: Our study suggests that biologics were effective and well tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had favorable GC- and immunosuppressant-sparing effect.

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Proteoglycan remodeling is accelerated in women with angina pectoris and diffuse myocardial fibrosis: the iPOWER Study

European Heart Journal ◽

10.1093/ehjci/ehaa946.1280 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

N.D Mygind ◽

S Holm Nielsen ◽

M Mide Michelsen ◽

A Pena ◽

D Bechsgaard Frestad ◽

...

Keyword(s):

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Angina Pectoris ◽

Myocardial Fibrosis ◽

T1 Mapping ◽

Obstructive Coronary Artery Disease ◽

Extracellular Volume ◽

Diffuse Myocardial Fibrosis ◽

Public Institution ◽

Funding Source

Abstract Background Women with angina and no obstructive coronary artery disease (CAD) have an unfavourable prognosis, possibly due to coronary microvascular disease and diffuse myocardial fibrosis (DMF). In DMF myocardial extracellular matrix (ECM) proteins are actively remodeled by matrix metalloproteinase (MMP). Purpose We investigated MMP-mediated degradation of the protegoglycans biglycan and versican in women with angina pectoris and possible DMF assessed by cardiac magnetic resonance T1 mapping. Methods Seventy-one women with angina pectoris and no obstructive CAD were included. Asymptomatic age-matched women served as controls (n=32). Versican and biglycan were measured in serum by specific competitive enzyme-linked immunosorbent assays. T1 mapping was performed by cardiac magnetic resonance with gadolinium measuring T1 and extracellular volume (ECV). Results Both biglycan and versican levels were higher in symptomatic women compared with controls; 31.4 ng/mL vs. 16.4 ng/mL (p<0.001) and 2.1 ng/mL vs. 1.8 ng/mL (p<0.001), respectively (Figure 1) and were moderately correlated to global ECV (r2=0.38, p<0.001 and r2=0.26, p=0.015 respectively). Conclusion Turnover of biglycan and versican was increased in symptomatic compared to asymptomatic women and associated to ECV, supporting a link between angina with no obstructive CAD and fibrotic cardiac remodeling. The examined biomarkers may prove to be suitable for monitoring active ECM remodeling. Figure 1. Levels of BGM and VCANM Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): This work was supported by The Danish Heart Foundation, the Danish Research Fund (Den Danske Forskningsfond) and by University of Copenhagen.

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