Burden and Impact of Arrhythmias in Repaired Tetralogy of Fallot

Pinsker, Bret L.; Serfas, John D.; Krasuski, Richard A.

doi:10.1007/s11886-022-01638-z

Cited by 4 publications

(2 citation statements)

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“…Although patients with repaired TOF have a high chance of surviving into adulthood, the transition into middle life is accompanied by severe morbidity and increased mortality rates ( Dennis et al, 2017 ). Both atrial and ventricular arrhythmias are a common source of morbidity and mortality following TOF repair ( Pinsker et al, 2022 ). Additional causes of mortality for individuals with TOF are heart failure and sudden cardiac death ( Norgaard et al, 1996 ; Oechslin et al, 2000 ); and ( Dennis et al, 2017 ).…”

Section: Introductionmentioning

confidence: 99%

Genetic insights into non-syndromic Tetralogy of Fallot

Althali

Hentges

2022

Front. Physiol.

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Congenital heart defects (CHD) include structural abnormalities of the heart or/and great vessels that are present at birth. CHD affects around 1% of all newborns worldwide. Tetralogy of Fallot (TOF) is the most prevalent cyanotic congenital cardiac abnormality, affecting three out of every 10,000 live infants with a prevalence rate of 5–10% of all congenital cardiac defects. The four hallmark characteristics of TOF are: right ventricular hypertrophy, pulmonary stenosis, ventricular septal defect, and overriding aorta. Approximately 20% of cases of TOF are associated with a known disease or chromosomal abnormality, with the remaining 80% of TOF cases being non-syndromic, with no known aetiology. Relatively few TOF patients have been studied, and little is known about critical causative genes for non-syndromic TOF. However, rare genetic variants have been identified as significant risk factors for CHD, and are likely to cause some cases of TOF. Therefore, this review aims to provide an update on well-characterized genes and the most recent variants identified for non-syndromic TOF.

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Section: Introductionmentioning

confidence: 99%

Genetic insights into non-syndromic Tetralogy of Fallot

Althali

Hentges

2022

Front. Physiol.

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“…6 Arrhythmias portend complications such as palpitations, sudden cardiac arrest (SCA), congestive heart failure (CHF), hospitalization, and stroke. 7,8 The consequences of residual hemodynamic derangements, placement of patch material, and progressive pulmonary regurgitation create a milieu favorable for arrhythmogenesis. 9 Arrhythmias are a well-described and common occurrence in patients with repaired TOF both periprocedurally and in the long-term.…”

Section: Introductionmentioning

confidence: 99%

Predictors of Arrhythmia in Adults with Repaired Tetralogy of Fallot

Moak

Falk

Aldous

et al. 2023

Preprint

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Introduction: Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease in adults, has excellent long-term survival. However, many patients (30-45%) develop late arrhythmias. Previous studies have identified predictors of arrhythmia (atrial or ventricular) using clinical markers that predate arrhythmia onset for many years. Our objective was to develop predictive models for incident atrial arrhythmias or any arrhythmia (atrial and ventricular) within two years of clinical evaluation and diagnostic testing. Methods: A single-center retrospective cohort study of 188 adults with repaired TOF. We included only patients with results from ECG and echocardiogram data in the required time interval (3-24 months before first arrhythmia for cases; 24 months of follow-up for controls). Predictive multivariable models for risk of atrial arrhythmia using logistic regression with a least absolute shrinkage and selection operator (LASSO) penalty were developed. Results: Of 41 demographic, surgical, and diagnostic variables, six were selected as having predictive value for atrial arrhythmia based on cross validation. The factors with the greatest predictive value in decreasing order were moderate / severe tricuspid regurgitation (adjusted odds ratio (OR) 149.42), QRS fragmentation (OR 28.08), severe pulmonary regurgitation (OR 8.22), RV systolic dysfunction (OR 2.95), 1 degree AV block, and age at time of surgical repair. Conclusion: Predictors for atrial or any arrhythmia in our study were similar, suggesting a common pathophysiologic substrate, i.e., abnormal right ventricle anatomical function and electrophysiologic properties (conduction and repolarization).

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