Diffuse LV Myocardial Fibrosis and its Clinical Associations in Adults With Repaired Tetralogy of Fallot

Broberg, Craig S.; Huang, Jennifer; Hogberg, Ingrid; McLarry, Joel; Woods, Patricia; Burchill, Luke J.; Pantely, George A.; Sahn, David J.; Jerosch‐Herold, Michael

doi:10.1016/j.jcmg.2015.10.006

Cited by 61 publications

(46 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is in agreement with studies in adults with ischemic and valvar heart disease which consistently report significant overlap of health and disease [20]. In contrast to our study Broberg et al found higher ECVs in adult TOF patients than in controls [5, 15]. Importantly, their patients were, on average, 20 years older than ours.…”

Section: Discussionsupporting

confidence: 93%

“…Adult survivors after TOF repair have evidence of increased LV ECV, quantified by CMR T1 mapping [5, 15, 16]. In an earlier pilot study, we found preliminary evidence for a higher LV fibrosis burden in pediatric TOF patients [6].…”

Section: Discussionmentioning

confidence: 97%

“…Importantly, their patients were, on average, 20 years older than ours. As a consequence, Broberg’s patients had had more time for adverse LV remodelling and adverse clinical outcome to occur; even so, 37 out of their 52 TOF patients [15] had ECVs in the normal range and the TOF subgroup had the lowest average ECV of all congenital heart disease patients [5]. The larger RVs and LVs in Broberg’s adult TOF patients as compared to our pediatric patients are further hallmarks of remodeling which, in our study, are associated with CMR markers of fibrosis.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

Riesenkampff

Luining

Seed

et al. 2016

Journal of Cardiovascular Magnetic Resonance

View full text Add to dashboard Cite

BackgroundUnfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors.MethodsIn this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity.ResultsThere was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r = 0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p < 0.05).ConclusionsAs compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males.

show abstract

Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

Riesenkampff

Luining

Seed

et al. 2016

Journal of Cardiovascular Magnetic Resonance

View full text Add to dashboard Cite

show abstract

“…They found that increasing age at death correlated with a higher percentage of RV fibrosis and with increasing myocyte diameter. These data support the CMR findings of Chen et al 21 and Broberg et al 22 and suggest a possible link between RV hypertrophy, reactive fibrosis, and adverse clinical outcomes.…”

Section: Future Worksupporting

confidence: 88%

Myocardial Fibrosis in Congenital Heart Disease

Rathod

Powell

Geva

2016

Circ J

View full text Add to dashboard Cite

“…Furthermore, there is a positive correlation between LV ECV and RV ECV supporting the concept of LV dysfunction in TOF as a result of ventricular interactions 36. Elevated LV ECV in TOF correlates with QRS duration, B-type brain natriuretic peptide and 6 min walk distance among other clinical variables 37. Together, these studies suggest that ECV may serve as a biomarker to predict subclinical deterioration and could be used to refine timing for referral for PVR.…”

Section: Refinement Of Pvr Timingmentioning

confidence: 62%

Approach to residual pulmonary valve dysfunction in adults with repaired tetralogy of Fallot

Kim

Ruckdeschel

2016

Heart

View full text Add to dashboard Cite

Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a result of surgical repair can lead to myriad complications including right ventricular dysfunction, decreased exercise tolerance, right heart failure and symptomatic arrhythmias. The aim of restoring pulmonary valve integrity is to preserve right ventricular size and function with the intent of mitigating the development of symptoms and poor long-term outcomes. Right ventricular size thresholds by cardiac MRI have emerged beyond which reverse right ventricular remodelling after pulmonary valve replacement is less likely. Though pulmonary valve replacement has been shown to improve right ventricular dimensions and symptoms, no consistent improvement in right ventricular ejection fraction or objective measures of exercise capacity have been demonstrated. Furthermore, there are no long-term studies showing that normalisation of right ventricular size results in improved clinical outcomes. New transcatheter techniques of percutaneous pulmonary valve replacement have emerged with good short-term and mid-term outcomes, further adding to the complexity in determining 'when' and 'how' right ventricular outflow tract and pulmonary valve intervention should occur. With improved survival of these patients, the trend towards earlier pulmonary valve replacement at smaller right ventricular size and rapidly evolving transcatheter pulmonary valve techniques, the clinician must balance the goals of preserving right ventricular size and function in an attempt to prevent untoward outcomes with risks of multiple interventions in a patient's lifetime.

show abstract

Diffuse LV Myocardial Fibrosis and its Clinical Associations in Adults With Repaired Tetralogy of Fallot

Cited by 61 publications

References 5 publications

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

Myocardial Fibrosis in Congenital Heart Disease

Approach to residual pulmonary valve dysfunction in adults with repaired tetralogy of Fallot

Contact Info

Product

Resources

About