scholarly journals Clinical Course Long After Atrial Switch: A Novel Risk Score for Major Clinical Events

2021 ◽  
Vol 10 (5) ◽  
Author(s):  
Odilia I. Woudstra ◽  
Tjitske E. Zandstra ◽  
Rosanne F. Vogel ◽  
Arie P. J. van Dijk ◽  
Hubert W. Vliegen ◽  
...  

Background Patients with transposition of the great arteries corrected by an atrial switch operation experience major clinical events during adulthood, mainly heart failure (HF) and arrhythmias, but data on the emerging risks remain scarce. We assessed the risk for events during the clinical course in adulthood, and provided a novel risk score for event‐free survival. Methods and Results This multicenter study observed 167 patients with transposition of the great arteries corrected by an atrial switch operation (61% Mustard procedure; age, 28 [interquartile range, 24–36] years) for 13 (interquartile range, 9–16) years, during which 16 (10%) patients died, 33 (20%) had HF events, defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF‐related death, and 15 (9%) had symptomatic ventricular arrhythmias. Five‐year risk of mortality, first HF event, and first ventricular arrhythmia increased from 1% each at age 25 years, to 6% (95% CI, 4%–9%), 23% (95% CI, 17%–28%), and 5% (95% CI, 2%–8%), respectively, at age 50 years. Predictors for event‐free survival were examined to construct a prediction model using bootstrapping techniques. A prediction model combining age >30 years, prior ventricular arrhythmia, age >1 year at repair, moderate or greater right ventricular dysfunction, severe tricuspid regurgitation, and mild or greater left ventricular dysfunction discriminated well between patients at low (<5%), intermediate (5%–20%), and high (>20%) 5‐year risk (optimism‐corrected C‐statistic, 0.86 [95% CI, 0.82–0.90]). Observed 5‐ and 10‐year event‐free survival rates in low‐risk patients were 100% and 97%, respectively, compared with only 31% and 8%, respectively, in high‐risk patients. Conclusions The clinical course of patients undergoing atrial switch increasingly consists of major clinical events, especially HF. A novel risk score stratifying patients as low, intermediate, and high risk for event‐free survival provides information on absolute individual risks, which may support decisions for pharmacological and interventional management.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
O Woudstra ◽  
T.E Zandstra ◽  
R.F Vogel ◽  
A.P.J Van Dijk ◽  
H.W Vliegen ◽  
...  

Abstract Background Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce. Purpose To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival. Methods We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. Results We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age &gt;30, prior VA, age &gt;1 year at repair surgery, QRS duration &gt;120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (&lt;5%), medium (5–20%) and high (&gt;20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients. Conclusion The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management. Figure 1. Observed event-free survival of patients with predicted low risk (&lt;5% in 5 years), medium risk (5–20% in 5 years) and high risk (&gt;20% in 5 years). Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund


2021 ◽  
pp. 1-11
Author(s):  
Corinna Lebherz ◽  
Martin Gerhardus ◽  
Astrid Elisabeth Lammers ◽  
Paul Helm ◽  
Oktay Tutarel ◽  
...  

Abstract Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.


Cancers ◽  
2021 ◽  
Vol 13 (14) ◽  
pp. 3494
Author(s):  
Xiaofei Sun ◽  
Zijun Zhen ◽  
Ying Guo ◽  
Yuanhong Gao ◽  
Juan Wang ◽  
...  

Despite aggressive treatment, the prognosis of high-risk NB patients is still poor. This retrospective study investigated the benefits of metronomic maintenance treatment (MT) in high-risk NB patients without ASCT or GD2 antibody therapy. Patients aged ≤ 21 years with newly diagnosed high-risk NB were included. Patients with complete/very good partial remission (CR/VGPR/PR) to conventional treatment received, or not, oral metronomic MT for 1 year. Two hundred and seventeen high-risk NB patients were enrolled. One hundred and eighty-five (85%) had a CR/VGPR/PR to conventional treatment, of the patients with stage 4, 106 receiving and 61 not receiving oral metronomic MT, and the 3-year event-free survival (EFS) rate was 42.5 ± 5.1% and 29.6 ± 6%, respectively (p = 0.017), and overall survival (OS) rate was 71.1 ± 4.7% and 59.4 ± 6.4%, respectively (p = 0.022). A total of 117 high-risk patients with oral metronomic MT had EFS rate of 42.7 ± 4.8%. The toxicity of MT was mild. For high-risk NB patients without ASCT or anti-GD2 antibody therapy, stage 4, MYCN amplication and patients with stage 4 not receiving oral metronomic MT after CR/VGPR/PR were independent adverse prognostic factors. Oral metronomic MT can improve survival in high-risk NB patients in CR/VGPR/PR without ASCT or anti-GD2 antibodies therapy.


2015 ◽  
Vol 65 (10) ◽  
pp. A497
Author(s):  
Carolyn Wilhelm ◽  
Tracey Sisk ◽  
Sharon Roble ◽  
Joanne Chisolm ◽  
John Cheatham ◽  
...  

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Marla J De Jong ◽  
Debra K Moser ◽  
Misook L Chung ◽  
Jia-Rong Wu

Anxiety has been linked to adverse outcomes for patients with cardiac disease but the mechanism for this relationship is unknown. Nonadherence to prescribed medications is common in heart disease, particularly heart failure (HF), and may mediate the relationship between anxiety and outcomes. To determine if nonadherence to prescribed medications mediates any relationship between anxiety and clinical outcomes in patients with HF. Patients (N=147; age 61±11 yrs, 44% female, 59% NYHA class III/IV) with chronic HF were followed 389±324 days for clinical events (composite of death, emergency department visit, or hospitalization). Patients completed the anxiety subscale of the Brief Symptom Inventory at baseline. Objective evidence of medication adherence was measured with the Medication Event Monitoring System. Survival and regression analyses were used to test whether medication nonadherence mediated any association between anxiety and outcomes. Patients with highest anxiety had shorter event-free survival than patients with lower anxiety (Fig. ). After adjusting for age, gender, and NYHA class in Cox regression, high anxiety predicted (OR 2.4; p=.001) clinical events. Anxiety predicted medication doses taken (p=.01) and days correct doses taken (p=.008). Medication doses taken (p=.01) and days dose taken (p=.008) also predicted clinical outcomes. Medication nonadherence mediated the relationship between high anxiety and worse outcomes. This is the first study to show that medication nonadherence links anxiety and clinical outcomes. Interventions that decrease anxiety may improve both medication adherence and outcomes.


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