scholarly journals Clinical Characteristics of Patients with Tetralogy of Fallot who Underwent an Invasive Procedure for Arrhythmias

2021 ◽  
Vol 34 (3) ◽  
pp. 91-99
Author(s):  
Barbara Adelmann de Lima ◽  
Antonio Carlos Gallo da Silva ◽  
Marco Aurélio Lumertz Saffi ◽  
Clóvis Fröemming Junior ◽  
Gabriela Castilhos ◽  
...  
Keyword(s):  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Primary Outcome ◽  
Electrophysiological Study ◽  
Invasive Procedure ◽  
Cyanotic Congenital Heart Disease ◽  
Implantable Cardiac Defibrillator ◽  
Electrophysiological Investigation ◽  
Qrs Duration

Introduction: Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease that has an incidence of sudden cardiac death of 0.2% per year, being arrhythmias the main cause of its occurrence. Objective: To compare characteristics of TOF patients referred for electrophysiological study (EPS) against those that were not (No-EPS). Method: Retrospective cohort with 215 patients (57.2% men; age = 29 ± 4) with corrected TOF (median of three years, ranging from 0.33 to 51) that underwent EPS between 2009-2020. The primary outcome was composed of death, implantable cardiac defibrillator (ICD) requirement and hospitalization. Results: Pre-syncope (EPS = 4.7%, No-EPS = 0%; p = 0.004), syncope (EPS = 7.1%, No-EPS = 1.7%; p = 0.056) and palpitations (EPS = 31%, No-EPS = 5.8%; p < 0.001) were symptoms that justified electrophysiological investigation. ICD was implanted in 24% of EPS and 0.6% of No-EPS (p=0.001). Twenty-six percent of the EPS group presented non-sustained ventricular tachycardia, while 0% in No-EPS (p = 0.012). The EPS group had more atrial fibrillation or atrial Flutter (35.7% vs. 6.9%; p < 0.001). The EPS patients had a wider QRS duration than the no-EPS group (171.12 ± 29.52 ms vs. 147 ± 29.77 ms; p < 0.001). Also, 26.2% of EPS performed ablation to correct macroreentrant atrial tachycardias. The incidence of primary outcome (death + ICD requirement + hospitalization) was higher in patients in the EPS group compared to the No-EPS group (p = 0.001). However, the total of seven deaths occurred during the clinical follow-up, but without differences between the groups (EPS = 4.7% vs. No-EPS = 2.8%; p = 0.480). Conclusion: EPS group had a profile of greater risk, more complex heart disease, and a greater occurrence of the primary outcome when compared to the No-EPS group.

scholarly journals TETRALOGY OF FALLOT IN ADULT;

2017 ◽  
Vol 24 (09) ◽  
pp. 1447-1449
Author(s):  
Abid Rafiq Chaudhry ◽  
Mohammad Tageldeine ◽  
Abdulrahman Abdulaziz Al Mesnid
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Survival Rate ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Blood Flows ◽  
Full Correction

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease inchildren but occurs rarely in adults. The extent of cyanosis depends on the balance of systemicand pulmonary vascular resistance, which depends on the severity of right ventricular outletobstruction.3 The more severe the obstruction, the more blood flows into the left side causingdesaturation and cyanosis. The survival rate of patients who receive surgical full correction isabout 86% at 32 years follow-up and 85% at 36 years follow up.

scholarly journals Coronary risk of patients with valvular heart disease: prospective validation of CT-Valve Score

Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001380
Author(s):  
Rasmus Bo Hasselbalch ◽  
Mia Marie Pries-Heje ◽  
Sarah Louise Kjølhede Holle ◽  
Thomas Engstrøm ◽  
Merete Heitmann ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valvular Heart Disease ◽  
Primary Outcome ◽  
Multislice Ct ◽  
Cost Effective ◽  
University Hospital ◽  
Before And After ◽  
History Of ◽  
Artery Disease

ObjectiveTo prospectively validate the CT-Valve score, a new risk score designed to identify patients with valvular heart disease at a low risk of coronary artery disease (CAD) who could benefit from multislice CT (MSCT) first instead of coronary angiography (CAG).MethodsThis was a prospective cohort study of patients referred for valve surgery in the Capital Region of Denmark and Odense University Hospital from the 1 February 2015 to the 1 February 2017. MSCT was implemented for patients with a CT-Valve score ≤7 at the referring physician’s discretion. Patients with a history of CAD or chronic kidney disease were excluded. The primary outcome was the proportion of patients needing reevaluation with CAG after MSCT and risk of CAD among the patients determined to be low to intermediate risk.ResultsIn total, 1149 patients were included. The median score was 9 (IQR 3) and 339 (30%) had a score ≤7. MSCT was used for 117 patients. Of these 29 (25%) were reevaluated and 9 (7.7%) had CAD. Of the 222 patients with a score ≤7 that did not receive an MSCT, 14 (6%) had significant CAD. The estimated total cost of evaluation among patients with a score ≤7 before implementation was €132 093 compared with €79 073 after, a 40% reduction. Similarly, estimated total radiation before and after was 608 mSv and 362 mSv, a 41% reduction. Follow-up at a median of 32 months (18–48) showed no ischaemic events for patients receiving only MSCT.ConclusionThe CT-Valve score is a valid method for determining risk of CAD among patients with valvular heart disease. Using a score ≤7 as a cut-off for the use of MSCT is safe and cost-effective.

Endothelial function state following repair of cyanotic congenital heart diseases

2013 ◽  
Vol 25 (2) ◽  
pp. 222-227 ◽  
Author(s):  
Mohammad Reza Sabri ◽  
Hooman Daryoushi ◽  
Mojgan Gharipour
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Endothelial Function ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Vascular Endothelial ◽  
Vascular Endothelial Function ◽  
Flow Mediated Dilatation ◽  
The Mean

AbstractBackgroundRepairing cyanotic congenital heart disease may be associated with preserving endothelial function. The present study aimed to evaluate vascular endothelial function in patients with repaired cyanotic congenital heart disease.MethodsIn a case–control study conducted in 2012 in Isfahan, Iran, 42 consecutive patients aged <35 years who had suffered from different types of cyanotic congenital heart disease and had undergone complete repair of their congenital heart defect were assessed in regard to their endothelial function state by measuring flow-mediated dilatation and other cardiac function indices. They were paired with 42 sex- and age-matched healthy controls.ResultsThe mean flow-mediated dilatation was lower in patients with repaired cyanotic congenital heart disease than in the controls [6.14±2.78 versus 8.16±1.49 respectively (p<0.001)]. Significant adverse correlations were found between flow-mediated dilatation, age, and body mass indexes, in those who underwent repair surgery. In addition, flow-mediated dilatation had a positive association with the shortening fraction, ejection fraction, and tricuspid annular plane systolic excursion value, and it was also inversely associated with carotid intima-media thickness and the myocardial performance index. The mean of the flow-mediated dilatation was significantly higher in the group with tetralogy of Fallot along with complete repair before the age of 2.5 years and also in those patients with total anomalous pulmonary venous connection or transposition of the great arteries repaired with an arterial switch operation before 6 months of age, compared with the other two subgroups. This includes patients with a tetralogy of Fallot defect repaired after 4 years of age and those with complex cyanotic congenital heart disease that was repaired after 2.5 years of age (mean age at repair 9±6.1 years).ConclusionEarly repair of a cyanotic defect can result in the protection of vascular endothelial function and prevent the occurrence of vascular accidents at an older age.

5205Electrocardiographic predictors of appropriate implantable cardioverter defibrillator therapies in patients with tetralogy of Fallot

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Waldmann ◽  
A Bouzeman ◽  
R Koutbi ◽  
F Bessiere ◽  
A Hermida ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Implantable Cardioverter Defibrillator ◽  
Tetralogy Of Fallot ◽  
Predictive Value ◽  
Cumulative Risk ◽  
Cardioverter Defibrillator ◽  
Increased Risk ◽  
Qrs Fragmentation ◽  
Qrs Duration

Abstract Background The electrocardiogram (ECG) is widely available and may contribute to a better risk stratification for sudden cardiac death in patients with tetralogy of Fallot. QRS duration has been consistently associated with outcomes, with a lack of specificity for sudden mortality and a relatively low predictive value. New markers such as QRS fragmentation and vectocardiographic parameters have been recently suggested. Purpose To identify ECG predictors of appropriate therapies in patients with tetralogy of Fallot and implantable cardioverter defibrillator (ICD). Methods The DAI-T4F study is a large ongoing national French registry including all patients with tetralogy of Fallot and ICD (NCT03837574). Information have been collected prospectively since 2010 with annual update. Baseline patient characteristics and clinical events during the follow-up were analyzed with central adjudication. Cox proportional hazard models were used to identify factors associated with appropriate ICD therapies. Results A total of 134 patients (median age 41.7 years, 70.7% males) were enrolled. During a median (IQR) follow-up of 6.1 (2.7–10.2) years, 59 (44.0%) patients received at least one appropriate ICD therapy, giving annual incidence of 5.5% and 7.1% in primary and secondary prevention, respectively (p=0.058). Overall, QRSd ≥180ms (p=0.073), QRS fragmentation (p=0.052), and QRS vector magnitude (vm, p=0.327) were not significantly associated with appropriate ICD therapies, whereas QRS fragmentation in right leads (HR=1.7, 95% CI: 1.1–2.9, p=0.039) and the association of QRSd ≥180ms and overall QRS fragmentation (HR=1.9, 95% CI: 1.1–3.4, p=0.036) were associated with an increased risk of appropriates ICD therapies. In patients with ICD for primary prevention (47 patients, 35.1%), 53.8% had QRS fragmentation, 48.6% had decreased QRS vm, and 41.0% had QRSd ≥180ms. In this group, while non-sustained ventricular tachycardia (NSVT) considered isolated was not associated with ventricular events during follow-up (p=0.069), respective combinations with QRSd ≥180 ms (HR=7.2, 95% CI: 1.6–32.7, p=0.011), QRS fragmentation (HR=3.8, 95% CI: 1.2–12.4, p=0.025), or decreased QRS vm (HR=3.6, 95% CI: 1.1–12.1, p=0.042) were all associated with a higher incidence of appropriate ICD therapies. Positive predictive value and negative predictive value were 0.33 and 0.85, 0.58 and 0.74, and 0.36 and 0.84 in patients with NSVT and QRS ≥180ms, NSVT and QRS fragmentation, and NSVT and decreased QRS vm, respectively. Conclusions Our findings highlight that cumulative risk score derived from ECG may contribute to improve risk stratification in patients with tetralogy of Fallot, in particular QRS fragmentation and QRS vm in association with QRS duration and other traditional risk factors.

Developmental Alterations of Cerebral Gray and White Matter in Children with Congenital Cyanotic Heart Disease

2018 ◽  
Vol 17 (05) ◽  
pp. 161-167
Author(s):  
Juan Leon-Wyss ◽  
Cynthia Rosario ◽  
Janet Toribio ◽  
Herwin Speckter ◽  
Bernd Foerster ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
White Matter ◽  
Oxygen Saturation ◽  
Congenital Heart ◽  
Diffusion Tensor ◽  
Cyanotic Congenital Heart Disease ◽  
Congenital Cyanotic Heart Disease ◽  
Diffusion Tensor Imaging Dti

AbstractThis article examines the relation between oxygen saturation and T2 star time in cyanotic congenital heart disease and its correlation to cerebral gray and white matter alterations. Magnetic resonance imaging was performed in 25 patients (mean age: 52.2 months) and 32 controls. Gray and white matter volumes, as well as fractional anisotropy and longitudinal diffusivity, were significantly reduced in patients. The reduction longitudinal diffusivity correlated to oxygen saturation and T2 star time of gray matter (p < 0.05). This diffusion tensor imaging (DTI) parameter was most affected in cyanotic congenital heart disease and because is the only parameter showing significant correlation to reduced oxygenation, it should be included more often in the follow-up of these patients over time.

scholarly journals Association of Pulmonary Valve Morphology Differences With Outcomes in Tetralogy of Fallot Repair With Right Ventricular Outflow Tract Incision

2021 ◽  
Vol 8 ◽  
Author(s):  
Jinyang Liu ◽  
Xianchao Jiang ◽  
Bo Peng ◽  
Shoujun Li ◽  
Jun Yan ◽  
...  
Keyword(s):  
Propensity Score ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Primary Outcome ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Complete Repair

Background: Current observational studies may not have large samples to investigate the relationship between pulmonary valve (PV) morphology differences and outcomes after complete repair for tetralogy of Fallot (TOF) by right ventricular outflow tract (RVOT) incision. This study aimed to assess the impact of PV morphology differences on outcomes after complete repair for TOF.Methods: This is a retrospective cohort study. Consecutive patients who underwent TOF repair with RVOT incision at Fuwai Hospital from January 2012 to December 2017 were included and compared according to PV morphology differences (unicuspid or bicuspid was abnormal morphology, while the tricuspid valve was normal morphology). The primary outcome was defined as a composite of death, or reintervention, or significant annular peak gradient (APG), or significant pulmonary regurgitation (PR), whichever occurred first. Multivariable Cox model analysis was used to assess the relationships between PV morphology differences and outcomes. Subgroup analysis and Propensity-score analysis were performed as sensitivity analyses to assess the robustness of our results.Results: The cohort included a total of 1,861 patients with primary diagnosis of TOF, with 1,688 undergoing CR-TOF with RVOT incision. The median age was 318 days [interquartile range (IQR): 223–534 days], a median weight of 8.9 kg (IQR: 7.6–10.5 kg) and 60.0% (1,011) were male. Complete follow-up data were available for 1,673 CR-TOF patients with a median follow-up duration of 49 months. Adjusted risks for the primary outcome and significant APG were lower for patients with normal PV morphology at follow up [adjusted hazard ratio (HR): 0.68; 95% CI: 0.46–0.98; adjusted HR: 0.22; 95% CI: 0.07–0.71, respectively]. The trend for the primary outcome during follow-up remained unchanged, even in subgroups and propensity score matching analyses.Conclusions: In this analysis of data from a large TOF cohort, patients with normal tricuspid PVs were associated with a decreased risk of the primary outcome and a lower risk of significant APG, as compared with patients with abnormal unicuspid or bicuspid PVs.

Cyanotic congenital heart disease in adolescentsand adults. Events at follow-up

1998 ◽  
Vol 5 (8) ◽  
pp. 947
Author(s):  
A. Agapito ◽  
L. Sousa ◽  
J. Aranha ◽  
M. Sousa ◽  
M. Oliveira ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease
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