scholarly journals Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis: a case report

Author(s):  
Jihad Hamudi ◽  
Basheer Karkabi ◽  
Devy Zisman ◽  
Avinoam Shiran

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare multisystem disease characterized by asthma, rhinosinusitis, and eosinophilia. Cardiac involvement, present in half the patients, may be life threatening. Case summary A young woman with long-standing asthma and nasal polyposis was admitted with new-onset dyspnoea, sinus tachycardia, and eosinophilia. She had severe biventricular thrombosis and severe tricuspid regurgitation (TR) on echocardiography, with preserved ejection fraction of both ventricles. Cardiac magnetic resonance (CMR) imaging showed diffuse subendocardial late gadolinium enhancement (LGE). She had a positive test for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) confirming the diagnosis of ANCA positive EGPA. She was treated with anticoagulation, high-dose corticosteroids, cyclophosphamide, and rituximab with gradual resolution of her symptoms. Follow-up echocardiography showed significant improvement in ventricular thrombi and TR but could not reliably exclude residual ventricular thrombus. Repeat CMR at 11 months confirmed complete resolution of both ventricular thrombi and near complete resolution of LGE. Discussion Cardiac involvement in EGPA, a rare cause of heart failure, can manifest as severe biventricular thrombosis and severe TR, resulting in heart failure with preserved ejection fraction. Combined immunosuppression and anticoagulation can lead to complete remission within a year. CMR is instrumental for both diagnosis and follow-up of EGPA, allowing for safe discontinuation of oral anticoagulation.

2020 ◽  
Vol 26 (8) ◽  
pp. 673-684
Author(s):  
CAMILLA HAGE ◽  
ULRIKA LÖFSTRÖM ◽  
ERWAN DONAL ◽  
EMMANUEL OGER ◽  
AGNIESZKA KAPŁON-CIEŚLICKA ◽  
...  

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Huang ◽  
C Liu

Abstract Background Lower systolic blood pressure (SBP) at admission or discharge was associated with poor outcomes in patients with heart failure and preserved ejection fraction (HFpEF). However, the optimal long-term SBP for HFpEF was less clear. Purpose To examine the association of long-term SBP and all-cause mortality among patients with HFpEF. Methods We analyzed participants from the Treatment of Preserved Cardiac Function Heart Failure with an Aldosterone Antagonist (TOPCAT) study. Participants had at least two SBP measurements of different times during the follow-up were included. Long-term SBP was defined as the average of all SBP measurements during the follow-up. We stratified participants into four groups according to long-term SBP: <120mmHg, ≥120mmHg and <130mmHg, ≥130mmHg and <140mmHg, ≥140mmHg. Multivariable adjusted Cox proportional hazards models were used to estimate hazard ratios (HRs) and 95% confidence intervals (CI) for all-cause mortality associated with SBP level. To assess for nonlinearity, we fitted restricted cubic spline models of long-term SBP. Sensitivity analyses were conducted by confining participants with history of hypertension or those with left ventricular ejection fraction≥50%. Results The 3338 participants had a mean (SD) age of 68.5 (9.6) years; 51.4% were women, and 89.3% were White. The median long-term SBP was 127.3 mmHg (IQR 121–134.2, range 77–180.7). Patients in the SBP of <120mmHg group were older age, less often female, less often current smoker, had higher estimated glomerular filtration rate, less often had history of hypertension, and more often had chronic obstructive pulmonary disease and atrial fibrillation. After multivariable adjustment, long-term SBP of 120–130mmHg and 130–140mmHg was associated with a lower risk of mortality during a mean follow-up of 3.3 years (HR 0.65, 95% CI: 0.49–0.85, P=0.001; HR 0.66, 95% CI 0.50–0.88, P=0.004, respectively); long-term SBP of <120mmHg had similar risk of mortality (HR 1.03, 95% CI: 0.78–1.36, P=0.836), compared with long-term SBP of ≥140mmHg. Findings from restricted cubic spline analysis demonstrate that there was J-shaped association between long-term SBP and all-cause mortality (P=0.02). These association was essentially unchanged in sensitivity analysis. Conclusions Among patients with HFpEF, long-term SBP showed a J-shaped pattern with all-cause mortality and a range of 120–140 mmHg was significantly associated with better outcomes. Future randomized controlled trials need to evaluate optimal long-term SBP goal in patients with HFpEF. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): China Postdoctoral Science Foundation Grant (2019M660229 and 2019TQ0380)


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
X.T Cui ◽  
E Thunstrom ◽  
U Dahlstrom ◽  
J.M Zhou ◽  
J.B Ge ◽  
...  

Abstract Background It remains unclear whether the readmission of heart failure (HF) patients has decreased over time and how it differs among HF with preserved ejection fraction (EF) (HFpEF) versus reduced EF (HFrEF) and mid-range EF (HFmrEF). Methods We evaluated HF patients index hospitalized from January 2004 to December 2011 in the Swedish Heart Failure Registry with 1-year follow-up. Outcome measures were the first occurring all-cause, cardiovascular (CV) and HF readmissions. Results Totally 20,877 HF patients (11,064 HFrEF, 4,215 HFmrEF, 5,562 HFpEF) were included in the study. All-cause readmission was highest in patients with HFpEF, whereas CV and HF readmissions were highest in HFrEF. From 2004 to 2011, HF readmission rates within 6 months (from 22.3% to 17.3%, P=0.003) and 1 year (from 27.7% to 23.4%, P=0.019) in HFpEF declined, and the risk for 1-year HF readmission in HFpEF was reduced by 7% after adjusting for age and sex (P=0.022). Likewise, risk factors for HF readmission in HFpEF changed. However, no significant changes in cause-specific readmissions were observed in HFrEF. Time to the first readmission did not change significantly from 2004 to 2011, regardless of EF subgroup (all P-values>0.05). Conclusions Although the burden of all-cause readmission remained highest in HFpEF versus HFrEF and HFmrEF, a declining temporal trend in 6-month and 1-year HF readmission rates was found in patients with HFpEF, suggesting that non-HF-related readmission represents a big challenge for clinical practice. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): The SwedeHF was funded by the Swedish National Board of Health and Welfare, the Swedish Association of Local Authorities and Regions.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pankaj Garg ◽  
Hosamadin Assadi ◽  
Rachel Jones ◽  
Wei Bin Chan ◽  
Peter Metherall ◽  
...  

AbstractCardiac magnetic resonance (CMR) is emerging as an important tool in the assessment of heart failure with preserved ejection fraction (HFpEF). This study sought to investigate the prognostic value of multiparametric CMR, including left and right heart volumetric assessment, native T1-mapping and LGE in HFpEF. In this retrospective study, we identified patients with HFpEF who have undergone CMR. CMR protocol included: cines, native T1-mapping and late gadolinium enhancement (LGE). The mean follow-up period was 3.2 ± 2.4 years. We identified 86 patients with HFpEF who had CMR. Of the 86 patients (85% hypertensive; 61% males; 14% cardiac amyloidosis), 27 (31%) patients died during the follow up period. From all the CMR metrics, LV mass (area under curve [AUC] 0.66, SE 0.07, 95% CI 0.54–0.76, p = 0.02), LGE fibrosis (AUC 0.59, SE 0.15, 95% CI 0.41–0.75, p = 0.03) and native T1-values (AUC 0.76, SE 0.09, 95% CI 0.58–0.88, p < 0.01) were the strongest predictors of all-cause mortality. The optimum thresholds for these were: LV mass > 133.24 g (hazard ratio [HR] 1.58, 95% CI 1.1–2.2, p < 0.01); LGE-fibrosis > 34.86% (HR 1.77, 95% CI 1.1–2.8, p = 0.01) and native T1 > 1056.42 ms (HR 2.36, 95% CI 0.9–6.4, p = 0.07). In multivariate cox regression, CMR score model comprising these three variables independently predicted mortality in HFpEF when compared to NTproBNP (HR 4 vs HR 1.65). In non-amyloid HFpEF cases, only native T1 > 1056.42 ms demonstrated higher mortality (AUC 0.833, p < 0.01). In patients with HFpEF, multiparametric CMR aids prognostication. Our results show that left ventricular fibrosis and hypertrophy quantified by CMR are associated with all-cause mortality in patients with HFpEF.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
O.M Aldaas ◽  
F Lupercio ◽  
C.L Malladi ◽  
P.S Mylavarapu ◽  
D Darden ◽  
...  

Abstract Background Catheter ablation improves clinical outcomes in symptomatic atrial fibrillation (AF) patients with heart failure (HF) with reduced ejection fraction (HFrEF). However, the role of catheter ablation in HF patients with a preserved ejection fraction (HFpEF) is less clear. Purpose To determine the efficacy of catheter ablation of AF in patients with HFpEF relative to those with HFrEF. Methods We performed an extensive literature search and systematic review of studies that compared AF recurrence at one year after catheter ablation of AF in patients with HFpEF versus those with HFrEF. Risk ratio (RR) 95% confidence intervals were measured using the Mantel-Haenszel method for dichotomous variables, where a RR&lt;1.0 favors the HFpEF group. Results Four studies with a total of 563 patients were included, of which 312 had HFpEF and 251 had HFrEF. All patients included were undergoing first time catheter ablation of AF. Patients with HFpEF experienced similar recurrence of AF one year after ablation on or off antiarrhythmic drugs compared to those with HFrEF (RR 0.87; 95% CI 0.69–1.10, p=0.24), as shown in Figure 1. Recurrence of AF was assessed with electrocardiography, Holter monitoring, and/or event monitoring at scheduled follow-up visits and final follow-up. Conclusion Based on the results of this meta-analysis, catheter ablation of AF in patients with HFpEF appears as efficacious in maintaining sinus rhythm as in those with HFrEF. Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Eunsil Koh ◽  
Noeul Kang ◽  
Jin-Young Lee ◽  
Duk-Kyung Kim ◽  
Young Soo Do ◽  
...  

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis mainly affecting small-sized arteries. Involvement of medium-sized vessels is very rare in EGPA. Here we present the case of a patient with EGPA who showed multiple hepatic aneurysms and distal gangrene. Case presentation A known EGPA patient visited to the emergency room (ER) with abrupt squeezing abdominal pain. She had suffered from gangrene in the fingertips of both hands for 1 year because of arterial thrombosis associated with hypereosinophilia. However, her absolute eosinophil count in the ER was 1120 cells/µL. An abdomen-pelvis CT demonstrated subcapsular hematoma in the right hepatic lobe. A celiac angiogram demonstrated multiple sized aneurysms in both hepatic lobes and some aneurysms in S7 and S8 were huge, more than 1 cm in size. The shape of the small aneurysms resembled a string of beads, as in polyarteritis nodosa. Given the clinical situation, emergency embolization was performed. Before this patient visited to the ER, she had been treated with a high dose of systemic corticosteroid, azathioprine, and cyclophosphamide. After addition of mepolizumab, the eosinophil count remained stable state with a near zero percentage of total white blood cell count. Conclusions Aneurysm and gangrene resulting from the involvement of medium-sized vessels can occur in EGPA. Destruction of vessels might occur even if eosinophil count is below 1500 cells/µL. If involvement of medium-sized arteries is suspected, thorough investigation to identify the involved organs and prompt management are needed to prevent fatal complications.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Kumpei Ueda ◽  
Shungo Hikoso ◽  
Daisaku D Nakatani ◽  
Shunsuke Tamaki ◽  
Masamichi Yano ◽  
...  

Background: An elevated pulmonary artery wedge pressure (PAWP), a surrogate of left ventricular filling pressure, is associated with poor outcomes in patients with heart failure (HF). In addition, obesity paradox is well recognized in HF patients and body mass index (BMI) also provides a prognostic information. However, there is little information available on the prognostic value of the combination of the echocardiographic derived PAWP and BMI in patients with HF with preserved ejection fraction (HFpEF). Methods and Results: Patients data were extracted from The Prospective mUlticenteR obServational stUdy of patIenTs with Heart Failure with Preserved Ejection Fraction (PURSUIT HFpEF) study, which is a prospective multicenter observational registry for acute decompensated heart failure (ADHF) patients with HFpEF. We analyzed 548 patients after exclusion of patients undergoing hemodialysis, patients with in-hospital death, missing follow-up data, or missing data to calculate PAWP or BMI. Body weight measurement and echocardiography were performed just before discharge. PAWP was calculated using the Nagueh formula [PAWP = 1.24* (E/e’) + 1.9] with e’ = [(e’ septal + e’ lateral ) /2]. During a mean follow up period of 1.5±0.8 years, 86 patients had all-cause death (ACD). Multivariate Cox analysis showed that both PAWP (p=0.020) and BMI (p=0.0001) were significantly associated with ACD, independently of age and previous history of HF hospitalization, after the adjustment with gender, left ventricular ejection fraction, NT-proBNP and estimated glomerular filtration rate. Kaplan-Meier curve analysis revealed that there was a significant difference in the risk of ACD when patients were stratified into 3 groups based on the median values of PAWP (17.3) and BMI (21.4). Conclusions: The combination of the echocardiographic derived PAWP and BMI might be useful for stratifying ADHF patients with HFpEF at risk for the total mortality.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Gianluigi Savarese ◽  
Camilla Hage ◽  
Ulf Dahlström ◽  
Pasquale Perrone-Filardi ◽  
Lars H Lund

Introduction: Changes in N-terminal pro brain natriuretic peptide (NT-proBNP) have been demonstrated to correlate with outcomes in patients with heart failure (HF) and reduced ejection fraction (EF). However the prognostic value of a change in NT-proBNP in patients with heart failure and preserved ejection fraction (HFPEF) is unknown. Hypothesis: To assess the impact of changes in NT-proBNP on all-cause mortality, HF hospitalization and their composite in an unselected population of patients with HFPEF. Methods: 643 outpatients (age 72+12 years; 41% females) with HFPEF (ejection fraction ≥40%) enrolled in the Swedish Heart Failure Registry between 2005 and 2012 and reporting NT-proBNP levels assessment at initial registration and at follow-up were prospectively studied. Patients were divided into 2 groups according the median value of NT-proBNP absolute change that was 0 pg/ml. Median follow-up from first measurement was 2.25 years (IQR: 1.43 to 3.81). Adjusted Cox’s regression models were performed using total mortality, HF hospitalization (with censoring at death) and their composite as outcomes. Results: After adjustments for 19 baseline variables including baseline NT-proBNP, as compared with an increase in NT-proBNP levels at 6 months (NT-proBNP change>0 pg/ml), a reduction in NT-proBNP levels (NT-proBNP change<0 pg/ml) was associated with a 45.2% reduction in risk of all-cause death (HR: 0.548; 95% CI: 0.378 to 0.796; p:0.002), a 50.1% reduction in risk of HF hospitalization (HR: 0.49; 95% CI: 0.362 to 0.689; p<0.001) and a 42.6% reduction in risk of the composite outcome (HR: 0.574; 95% CI: 0.435 to 0.758; p<0.001)(Figure). Conclusions: Reductions in NT-proBNP levels over time are independently associated with an improved prognosis in HFPEF patients. Changes in NT-proBNP could represent a surrogate outcome in phase 2 HFPEF trials.


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