scholarly journals Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort

2021 ◽  
Vol 9 ◽  
Author(s):  
Po-Yuan Wang ◽  
Wei-Chieh Tseng ◽  
Chun-Min Fu ◽  
Mei-Hwan Wu ◽  
Jou-Kou Wang ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Ventricular Function ◽  
Age At Diagnosis ◽  
Term Survival ◽  
Function Recovery ◽  
Asian Cohort ◽  
Waitlist Mortality ◽  
Diagnostic Age

Background: Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy. The epidemiological profiles and prognosticators of clinical outcomes in Asian populations are not well elucidated.Methods: Data of 104 children aged <18 years with a diagnosis of primary DCM from January 1990 to December 2019 in our institutional database were retrospectively investigated. Relevant demographic, echocardiographic, and clinical variables were recorded for analysis. A P <0.05 was considered statistically significant.Results: The median age at diagnosis was 1.4 years (interquartile range = 0.3–9.1 years), and 52.9% were males. During a median follow-up duration of 4.8 years, 48 patients (46.2%) were placed on the transplantation waitlist, and 52.1% of them eventually received heart transplants. An exceptionally high overall waitlist mortality rate was noted (27.1%), which was even higher (43.5%) if the diagnostic age was <3 years. The 1-, 5-, and 10-year transplant-free were 61.1, 48.0, and 42.8%. Age at diagnosis >3 years and severe mitral regurgitation at initial diagnosis were independent risk factors for death or transplantation (hazard ratios = 2.93 and 3.31, respectively; for both, P <0.001). In total, 11 patients (10.6%) experienced ventricular function recovery after a median follow-up of 2.5 (interquartile range = 1.65–5) years. Younger age at diagnosis was associated a higher probability of ventricular function recovery.Conclusions: Despite donor shortage for heart transplantation and subsequently high waitlist mortality, our data from an Asian cohort indicated that transplant-free long-term survival was comparable with that noted in reports from Western populations. Although younger patients had exceptionally higher waitlist mortality, lower diagnostic age was associated with better long-term survival and higher likelihood of ventricular function recovery.

Abstract 14915: Incidence, Predictors and Clinical Impact of Left Ventricular Functional Recovery Following Coronary Artery Bypass Grafting in Patients With Ischemic Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Masaro Nakae ◽  
Satoshi Kainuma ◽  
Koichi Toda ◽  
Shigeru Miyagawa ◽  
Daisuke Yoshioka ◽  
...  
Keyword(s):  
Ischemic Cardiomyopathy ◽  
Artery Bypass ◽  
Left Ventricular ◽  
Lv Function ◽  
Term Survival ◽  
Function Recovery ◽  
Lv Remodeling ◽  
Relationship Of

Introduction: CABG is considered the standard treatment for patients with ischemic cardiomyopathy (ICM). However, it remains unknown who would achieve postoperative LV function recovery after CABG. Furthermore, the relationship of postoperative LV function recovery with long-term outcomes remains unclear. Hypothesis: In patients with ICM who undergo CABG, postoperative LV function recovery, which would be influenced by degree of LV remodeling at baseline, is associated with improved outcomes. Methods: This multicenter retrospective study comprised 490 cases with LVEF of ≤40% who underwent CABG between 1993 and 2015. Clinical follow-up was completed in 467 cases (95%), with a mean follow-up of 65±46 months (range, 0-266). A total of postoperative echocardiographic assessments were carried with an average number of 3.7±2.4. LV function recovery was defined as LVEF ≥35% at more than one exam. Association of LV function recovery with mortality after adjustments for clinically relevant covariates was estimated using Cox proportional hazard model. Pre- and intraoperative associates of LV function recovery were identified using logistic regression model. Results: During follow-up, there were 203 mortalities (41%) and overall 10-year survival was 45%. LV function recovery was found in 368 cases (75%), while not in 122 (25%). Overall 10-year survival was significantly higher in patients who achieved LV function recovery as compared with those who did not achieve it (52% vs. 23%). Multivariate analysis identified LV function recovery was independently associated with decreased overall mortality (adjusted HR 0.40, p<0.0001). Preoperative LVDs (adjusted OR 0.92, p<0.0001), eGFR (adjusted OR 1.12, p=0.016) and revascularization using bilateral internal thoracic arteries (BITA) (adjusted OR 2.81, p=0.018) are the independent predictors of LV function recovery. Conclusions: Among patients with ICM who underwent CABG, 75% achieved substantial postoperative LV function recovery, in association with better long-term survival, as compared with the remaining 25% of patients who did not achieve it. Preoperative less LV remodeling and preserved renal function as well as revascularization with use of BITA might be associated with LV function recovery.

scholarly journals The Evaluation of Residual Disease By Digital PCR, and TKI Duration Are Critical Predictive Factors for Molecular Recurrence after for Stopping Imatinib First-Line in Chronic Phase CML Patients: Results of the STIM2 Study.

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 462-462 ◽  
Author(s):  
Franck Emmanuel Nicolini ◽  
Stephanie Dulucq ◽  
Joelle Guilhot ◽  
Gabriel Etienne ◽  
Francois-Xavier Mahon
Keyword(s):  
Board Of Directors ◽  
Predictive Factors ◽  
De Novo ◽  
Age At Diagnosis ◽  
First Line ◽  
Advisory Committees ◽  
Term Survival ◽  
Risk Of Relapse

Abstract Background TKI discontinuation is now a critical goal of CML management and several studies have demonstrated the feasibility of stopping safely imatinib (IM). A sustained deep molecular response (DMR) on long-term TKI therapy seems critical prior to attempting treatment-free remission (TFR). Results from different studies have been reported recently, but they failed to identify robust and reproducible predictive factors allowing a better selection of candidate patients for successful TFR. Aims We prospectively asked whether the use of IM first-line in de novo CP-CML patients and a systematic screen of patients with digital droplet quantitative PCR (ddPCR) for BCR-ABL1 would impact on the TFR rates. Methods In a national prospective phase II trial (Eudract # NCT01343173), we enrolled newly diagnosed CP-CML patients, in sustained MR4.5 (≥2 yrs on at least 5 consecutive points) according to the ELN criteria, checked centrally. Further molecular follow-up was performed in local laboratories, all ELN standardized and aligned on the international scale (IS). Molecular recurrence was defined as the positivity of BCR-ABL1/ABL1 transcripts with at least 1-log increase between 2 consecutive assessments or as loss of MMR on a single assessment. Duplex ddPCR was performed on screening samples after RT, using EAC primers and probes. Pre-mixes were transferred in aDG8 cartridge, and generation of droplets was made on the QX200 droplet generator. Each emulsion was transferred into a 96 wells ddPCR plate and amplified on the Biorad-C1000 Touch Thermocycler. Each patient was analyzed in duplicate. ddPCR was considered positive when the BCR-ABL1/ABL1 % was ≥3 SD. BCR-ABL1/ABL1 ratios were aligned on the IS, a conversion factor was calculated for the RT-ddPCR as previously described (S. Branford et al., Blood 2008). Impact of variables on the risk of relapse were assessed through univariate and multivariate analyses using SAS program. Results The median follow-up after IM cessation was 23.5 (1-64) Mo. One patient died from unrelated cause, and 107 experienced a loss of MMR. The molecular recurrence-free survival (RFS) was 52% (95%CI: 45-59%) at 6 months, and 50% (95%CI: 43-57%) at 24 months. The Sokal and the EUTOS long-term survival (ELTS) scores failed to discriminate patients but a trend was observed for ELTS score (p=0.0547). Other variables analysed such as gender (p=0.6246), age at diagnosis (p=0.966), age at IM cessation (p=0.6197), interval diagnosis-IM (p=0.9621), interval IM initiation-DMR (p=0.9978) did not impact on the rate of relapse. In contrast, IM and DMR duration before IM cessation significantly impacted on the rate of relapse, as this interval is redundant with IM duration (p=0.008). ddPCR was applied to 174 out of 218 patients at IM cessation. A cut-off of 0.0023%IS ratio (= median of positive ratios) was also associated with a higher risk of relapse. The RFS estimate according to this cut-off is shown in Figure 1. In a multivariate analysis, the duration of IM (≥74.8 months) and ddPCR (≥0.0023%IS) were the two identified significant predictive factors with respectively p=0.0366 [HR=0.635, 95%CI: 0.415-0.972] and p=0.0081 [HR=0.635, 95%CI: 0.415-0.972)]. No association was observed between a ddPCR ≥0.0023%IS and gender, Sokal or ELTS score, age at diagnosis, age at IM discontinuation, duration of IM treatment, DMR duration or the time-lapse for obtaining DMR. Conclusion We conclude that the duration of IM and the residual leukemic cell load as determined by a sensitive technique such as ddPCR are key factors for predicting TFR for de novo CP-CML patients who have been treated with IM front-line. Disclosures Nicolini: Sun Pharma Ltd: Consultancy; BMS: Consultancy, Speakers Bureau; Incyte Biosciences: Consultancy, Speakers Bureau. Dulucq:Incyte: Consultancy; BMS: Consultancy. Etienne:Pfizer: Membership on an entity's Board of Directors or advisory committees, Other: Travel, Speakers Bureau; Incyte: Honoraria, Patents & Royalties, Speakers Bureau; BMS: Membership on an entity's Board of Directors or advisory committees, Other: Travel, Speakers Bureau; Novartis: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Mahon:Incyte: Speakers Bureau; Pfizer: Speakers Bureau; Novartis: Speakers Bureau; BMS: Speakers Bureau.

Surgical reconstruction for unilateral iliac artery lesions in patients younger than 50 years

VASA ◽  
2011 ◽  
Vol 40 (6) ◽  
pp. 474-481 ◽  
Author(s):  
Radak ◽  
Babic ◽  
Ilijevski ◽  
Jocic ◽  
Aleksic ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Limb Salvage ◽  
Iliac Artery ◽  
Graft Patency ◽  
Long Term Results ◽  
Surgical Reconstruction ◽  
Safe Procedure ◽  
Term Survival

Background: To evaluate safety, short and long-term graft patency, clinical success rates, and factors associated with patency, limb salvage and mortality after surgical reconstruction in patients younger than 50 years of age who had undergone unilateral iliac artery bypass surgery. Patients and methods: From January 2000 to January 2010, 65 consecutive reconstructive vascular operations were performed in 22 women and 43 men of age < 50 years with unilateral iliac atherosclerotic lesions and claudication or chronic limb ischemia. All patients were followed at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. Results: There was in-hospital vascular graft thrombosis in four (6.1 %) patients. No in-hospital deaths occurred. Median follow-up was 49.6 ± 33 months. Primary patency rates at 1-, 3-, 5-, and 10-year were 92.2 %, 85.6 %, 73.6 %, and 56.5 %, respectively. Seven patients passed away during follow-up of which four patients due to coronary artery disease, two patients due to cerebrovascular disease and one patient due to malignancy. Limb salvage rate after 1-, 3-, 5-, and 10-year follow-up was 100 %, 100 %, 96.3 %, and 91.2 %, respectively. Cox regression analysis including age, sex, risk factors for vascular disease, indication for treatment, preoperative ABI, lesion length, graft diameter and type of pre-procedural lesion (stenosis/occlusion), showed that only age (beta - 0.281, expected beta 0.755, p = 0.007) and presence of diabetes mellitus during index surgery (beta - 1.292, expected beta 0.275, p = 0.026) were found to be significant predictors of diminishing graft patency during the follow-up. Presence of diabetes mellitus during index surgery (beta - 1.246, expected beta 0.291, p = 0.034) was the only variable predicting mortality. Conclusions: Surgical treatment for unilateral iliac lesions in patients with premature atherosclerosis is a safe procedure with a low operative risk and acceptable long-term results. Diabetes mellitus and age at index surgery are predictive for low graft patency. Presence of diabetes is associated with decreased long-term survival.

Role of Closed Mitral Commissurotomy for Mitral Stenosis: Mid- and Long-term Surgical Outcome of 36 Patients

2005 ◽  
Vol 8 (1) ◽  
pp. 55 ◽  
Author(s):  
Azman Ates ◽  
Yahya �nl� ◽  
Ibrahim Yekeler ◽  
Bilgehan Erkut ◽  
Yavuz Balci ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Mitral Stenosis ◽  
Operating Time ◽  
Term Survival ◽  
Long Term Outcome ◽  
Mitral Commissurotomy ◽  
Closed Mitral Commissurotomy

Purpose: To evaluate long-term survival and valve-related complications as well as prognostic factors for mid- and long-term outcome after closed mitral commissurotomy, covering a follow-up period of 14 years. Material and Methods: Between 1989 and 2003, 36 patients (28 women and 8 men, mean age 28.8 6.1 years) underwent closed mitral commissurotomy at our institution. The majority of patients were in New York Heart Association (NYHA) functional class IIB, III, or IV. Indication for closed mitral commissurotomy was mitral stenosis. Closed mitral commissurotomy was undertaken with a Tubbs dilator in all cases. Median operating time was 2.5 hours 30 minutes. Results: After closed mitral commissurotomy, the mitral valve areas of these patients were increased substantially, from 0.9 to 2.11 cm2. No further operation after initial closed mitral commissurotomy was required in 86% of the patients (n = 31), and NYHA functional classification was improved in 94% (n = 34). Postoperative complications and operative mortality were not seen. Follow-up revealed restenosis in 8.5% (n = 3) of the patients, minimal mitral regurgitation in 22.2% (n = 8), and grade 3 mitral regurgitation in 5.5% (n = 2) patients. No early mortality occurred in closed mitral commissurotomy patients. Reoperation was essential for 5 patients following closed mitral commissurotomy; 2 procedures were open mitral commissurotomies and 3 were mitral valve replacements. No mortality occurred in these patients. Conclusions: The mitral valve area was significantly increased and the mean mitral valve gradient was reduced in patients after closed mitral commissurotomy. Closed mitral commissurotomy is a safe alternative to open mitral commissurotomy and balloon mitral commissurotomy in selected patients.

Long-term follow-up of cardiac resynchronization therapy patients with non-ischemic dilated cardiomyopathy assessed by radionuclide angiography

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Valzania ◽  
R Bonfiglioli ◽  
F Fallani ◽  
J Frisoni ◽  
M Biffi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Dilated Cardiomyopathy ◽  
Radionuclide Angiography ◽  
Cardiac Resynchronization ◽  
Resynchronization Therapy ◽  
Long Term Follow Up ◽  
Non Ischemic Dilated Cardiomyopathy ◽  
Lv Ejection Fraction

Abstract Background While the beneficial effects of cardiac resynchronization therapy (CRT) have been widely investigated soon after CRT implantation, relatively few data are available on long-term clinical outcomes of CRT recipients. Aim To investigate long-term outcomes of CRT patients with non-ischemic dilated cardiomyopathy stratified as responders and non-responders according to radionuclide angiography. Methods Consecutive heart failure patients with non-ischemic dilated cardiomyopathy undergoing CRT implantation at our University Hospital between 2007 and 2013 were enrolled. All patients were assessed with equilibrium Tc99 radionuclide angiography at baseline and after 3 months of CRT. Left ventricular (LV) ejection fraction was computed on the basis of relative end-diastolic and end-systolic counts, and intraventricular dyssynchrony was derived by Fourier phase analysis. Response to CRT was defined by an absolute increase in LV ejection fraction (LVEF) ≥5% at 3-month follow-up. Clinical outcome was assessed after 10 years through hospital records review. Results Forty-seven patients (83% men, 63±11 years) were included in the study. At 3 months, 25 (53%) patients were identified as CRT responders according to LVEF increase (from 26±8 to 38±12%, p&lt;0.001). In these patients, LV dyssynchrony decreased from 59±30° to 29±18° (p&lt;0.001). Twenty-two (47%) patients were defined as non-responders. No significant changes in LVEF and LV dyssynchrony (50±30° vs. 38±19°, p=0.07) were observed in non-responders. At long-term follow-up (11±2 years), all-cause and cardiac mortality rates were 24% and 12% in responders vs. 32% and 27% in non-responders, respectively (p=ns). Heart transplantation was performed in 3 patients. One (4%) patient among CRT responders compared with 6 (27%) patients among non-responders died of worsening heart failure (p=0.03). Conclusions Although late overall mortality of non-ischemic CRT recipients was not significantly different between mid-term responders and non-responders, CRT responders were at lower risk of worsening heart failure death. Funding Acknowledgement Type of funding source: None

scholarly journals The Long-Term Follow-Up of Patients with Cystine Stones: A Single-Center Experience for 13 Years

2021 ◽  
Vol 10 (7) ◽  
pp. 1336
Author(s):  
Toshifumi Takahashi ◽  
Shinya Somiya ◽  
Katsuhiro Ito ◽  
Toru Kanno ◽  
Yoshihito Higashi ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Median Number ◽  
Small Sample ◽  
Age At Diagnosis ◽  
Surgical Interventions ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Cystine Stones

Introduction: Cystine stone development is relatively uncommon among patients with urolithiasis, and most studies have reported only on small sample sizes and short follow-up periods. We evaluated clinical courses and treatment outcomes of patients with cystine stones with long-term follow-up at our center. Methods: We retrospectively analyzed 22 patients diagnosed with cystine stones between January 1989 and May 2019. Results: The median follow-up was 160 (range 6–340) months, and the median patient age at diagnosis was 46 (range 12–82) years. All patients underwent surgical interventions at the first visit (4 extracorporeal shockwave lithotripsy, 5 ureteroscopy, and 13 percutaneous nephrolithotripsy). The median number of stone events and surgical interventions per year was 0.45 (range 0–2.6) and 0.19 (range 0–1.3) after initial surgical intervention. The median time to stone events and surgical intervention was 2 years and 3.25 years, respectively. There was a significant difference in time to stone events and second surgical intervention when patients were divided at 50 years of age at diagnosis (p = 0.02, 0.04, respectively). Conclusions: Only age at a diagnosis under 50 was significantly associated with recurrent stone events and intervention. Adequate follow-up and treatment are needed to manage patients with cystine stones safely.

Anemia increases long-term mortality in patients undergoing conventional coronary angiography – the ECAT registry

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Balcer ◽  
I Dykun ◽  
S Hendricks ◽  
F Al-Rashid ◽  
M Totzeck ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Regression Analysis ◽  
Coronary Artery ◽  
Coronary Angiography ◽  
Conventional Coronary Angiography ◽  
Term Survival ◽  
Coronary Syndrome ◽  
Artery Disease

Abstract Background Anemia is a frequent comorbidity in patients with coronary artery disease (CAD). Besides a complemental effect on myocardial oxygen undersupply of CAD and anemia, available data suggests that it may independently impact the prognosis in CAD patients. We aimed to determine the association of anemia with long-term survival in a longitudinal registry of patients undergoing conventional coronary angiography. Methods The present analysis is based on the ECAD registry of patients undergoing conventional coronary angiography at the Department of Cardiology and Vascular Medicine at the University Clinic Essen between 2004 and 2019. For this analysis, we excluded all patients with missing hemoglobin levels at baseline admission or missing follow-up information. Anemia was defined as a hemoglobin level of &lt;13.0g/dl for male and &lt;12.0g/dl for female patients according to the world health organization's definition. Cox regression analysis was used to determine the association of anemia with morality, stratifying by clinical presentation of patients. Hazard ratio and 95% confidence interval are depicted for presence vs. absence of anemia. Results Overall, data from 28,917 patient admissions (mean age: 65.3±13.2 years, 69% male) were included in our analysis (22,570 patients without and 6,347 patients with anemia). Prevalence of anemia increased by age group (age &lt;50 years: 16.0%, age ≥80 years: 27.7%). During a mean follow-up of 3.2±3.4 years, 4,792 deaths of any cause occurred (16.6%). In patients with anemia, mortality was relevantly higher as compared to patients without anemia (13.4% vs. 28.0% for patients without and with anemia, respectively, p&lt;0.0001, figure 1). In univariate regression analysis, anemia was associated with 2.4-fold increased mortality risk (2.27–2.55, p&lt;0.0001). Effect sizes remained stable upon adjustment for traditional risk factors (2.38 [2.18–2.61], p&lt;0.0001). Mortality risk accountable to anemia was significantly higher for patients receiving coronary interventions (2.62 [2.35–2.92], p&lt;0.0001) as compared to purely diagnostic coronary angiography examinations (2.31 [2.15–2.47], p&lt;0.0001). Likewise, survival probability was slightly worse for patients with anemia in acute coronary syndrome (2.70 [2.29–3.12], p&lt;0.0001) compared to chronic coronary syndrome (2.60 [2.17–3.12], p&lt;0.0001). Interestingly, within the ACS entity, association of anemia with mortality was relevantly lower in STEMI patients (1.64 [1.10–2.44], p=0.014) as compared to NSTEMI and IAP (NSTEMI: 2.68 [2.09–3.44], p&lt;0.0001; IAP: 2.67 [2.06–3.47], p&lt;0.0001). Conclusion In this large registry of patients undergoing conventional coronary angiography, anemia was a frequent comorbidity. Anemia relevantly influences log-term survival, especially in patients receiving percutaneous coronary interventions. Our results confirm the important role of anemia for prognosis in patients with coronary artery disease, demonstrating the need for specific treatment options. Figure 1. Kaplan Meier analysis Funding Acknowledgement Type of funding source: None

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