Regurgitation In Tetralogy Of Fallot Research Articles

Discussion to: Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants

Discussion to: Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants

ObjectiveWe sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). MethodsA single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. ResultsOf 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045). ConclusionsPatients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.

Reversal of Right Ventricular Remodeling After Correction of Pulmonary Regurgitation in Tetralogy of Fallot

In the sheep model with pathophysiologic changes similar to patients with repaired TOF, severe PR leads to fibrotic changes in the RV. Pulmonary valve replacement reverses these fibrotic changes. Early valve replacement led to a quick RV recovery, and in time there was no difference in outcome between early and late valve replacement. These data support the benefit of valve replacement for RV function and suggest that there is a margin in the timing of the surgery. The fibrotic changes correlated well with the circulating biomarker PICP, which can have an added value in the clinical follow-up of patients with repaired TOF.

Time to reconsider when to re-valve for free pulmonary regurgitation in tetralogy of Fallot?

Time to reconsider when to re-valve for free pulmonary regurgitation in tetralogy of Fallot?

Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI

Quantification of pulmonary regurgitation (PR) is essential in the management of patients with repaired tetralogy of Fallot (TOF). We sought to evaluate the accuracy of first-line Doppler echocardiography in comparison with cardiac magnetic resonance imaging (MRI) to identify hemodynamic significant PR. Paired cardiac MRI and echocardiographic studies (n = 97) in patients with repaired TOF were retrospectively analyzed. Pressure half time (PHT) and pulmonary regurgitation index (PRi) were measured using continuous wave Doppler. The ratio of the color flow Doppler regurgitation jet width to pulmonary valve (PV) annulus (jet/annulus ratio) and diastolic to systolic time velocity integral (DSTVI; pulsed wave Doppler) were assessed. Accuracy of echocardiographic measurements was tested to identify significant PR as determined by phase-contrast MRI (PR fraction [PRF] ≥ 20%). Mean PRF was 29.4 ± 15.7%. PHT < 100 ms had a sensitivity of 93%, specificity 75%, positive predictive value (PPV) 92% and negative predictive value (NPV) 78% for identifying significant PR (C-statistic 0.82). PRi < 0.77 had sensitivity and specificity of 66% and 54%, respectively (C-statistic 0.63). Jet/annulus ratio ≥1/3 had sensitivity 96%, specificity 75%, PPV 92% and NPV 82% (C-statistic 0.87). DSTVI had sensitivity 84%, specificity 33%, PPV 84% and NPV 40%, (C-statistic 0.56). Combined jet/annulus ratio ≥1/3 and PHT < 100 ms was highly accurate in identifying PRF ≥ 20%, with sensitivity 97% and specificity 100%. PHT and jet/annulus ratio on Doppler echocardiography, especially when combined, are highly accurate in identifying significant PR and therefore seem useful in the follow-up of patients with repaired TOF.

Pulmonary Regurgitation in Tetralogy of Fallot: Can Circulatory Modeling Help Us Avert Right Ventricular Failure?

Pulmonary Regurgitation in Tetralogy of Fallot: Can Circulatory Modeling Help Us Avert Right Ventricular Failure?

NT-proBNP as a biomarker of right ventricular dilatation and pulmonary regurgitation in Tetralogy of Fallot

Background After surgical correction of Tetralogy of Fallot (TOF), residual pulmonary regurgitation (PR), right ventricular (RV) dilatation and RV failure have been associated with adverse clinical events and are considered criteria for pulmonary valve replacement. Cardiovascular magnetic resonance imaging (CMR) is the gold-standard for evaluation of these parameters. However it is an expensive technique which is not readily available in all hospitals and outpatient clinics. NT-proBNP is a biomarker which may overcome these CMR limitations and might be used as a cost-effective biomarker for monitoring patients with TF. The aim of this study is to evaluate whether NTproBNP plasma levels may help as a biomarker for monitoring ventricular dilatation, pulmonary valve regurgitation and heart failure in patients with repaired TF

Hybrid Procedure for Acute Severe Aortic Regurgitation in an Uncorrected Adult Tetralogy of Fallot

Adult survivors of surgically uncorrected complex tetralogy of Fallot [TOF] with multiple aorto-pulmonary coronary arteries [MAPCAs] are unusual and the development of severe aortic regurgitation [AR] due to endocarditis is a rare and difficult problem to manage. We report a successful hybrid approach utilising temporary balloon occlusion of the collaterals during the cross-clamp time as a strategy to allow adequate brain perfusion and safe aortic root replacement.

Prevalence and optimal management strategy for aortic regurgitation in tetralogy of Fallot

Aortic regurgitation (AR) in the tetralogy of Fallot (TOF) is not frequent, but when present it impacts significantly on surgical management. Furthermore, the incidence of late AR development has been increasing, along with surgical interest in current practices. Pre- and post-operative studies on 427 patients (TOF, 374; TOF/PA (TOF with pulmonary atresia), 53) who survived corrective operation were reviewed. AR (> or =mild) was detected in 28. Nine had AR preoperatively, while 25 (including six with preoperative AR) exhibited AR post-operatively. In the 19 who developed AR post-operatively, the aortic root diameter (AoRoD) and indexed AoRoD (%AoRoD) were 42+/-11 mm and 166+/-36%, increased from the preoperative values of 30+/-10mm and 149+/-24%. AR-free rate at 20 years was 95.1% of all cases studied, 84.3 vs 96.5% in TOF/PA vs classic TOF (P<0.0001), and 82.2 vs 97.0% in bulboventricular VSD vs infracristal VSD (P<0.0001). Older age at repair, and bulboventricular VSD were identified as risk factors for the progression of AR. Aortic valvuloplasty (AVP; n=5) or replacement (AVR; n=4) was performed nine times in eight patients before (n=1), during (n=4), or late after TOF repair (n=4); all showed improvement of NYHA class. Survival- and reoperation-free survival curves showed no significant difference between patients with or without AR. After repair of TOF, careful observation for a late progression of AR is needed for the optimal timing of surgical intervention, especially in patients who repaired at higher age with a dilated aortic root or in patients with bulboventricular VSD.

The murmur of pulmonic regurgitation in tetralogy of Fallot with absent pulmonic valve.

Absent pulmonic valve (APV) in tetralogy of Fallot produces a pulmonic regurgitation murmur (PRM) which is usually late in onset after A2, low pitched, and of crescendo-decrescendo character. We have seen three adult patients with tetralogy of Fallot with APV and have done intracardiac sound and pressure studies in two. The PRM was loudest in the RV outflow tract (RVOT), where the onset was earlier than the murmur recorded on the chest wall. The crescendo portion of the PRM occurred during an abnormally slow decline in the RVOT pressure pulse after the crossover of PA and RVOT pressures. The RVOT pressure reached its minimum 30 msec after the RV body pressure, resulting in a pressure gradient between the two. The PRM peaked 30 msec later in the RV body than in the RVOT. The delayed precordial onset of the PRM after A2 is likely due to failure of transmission of early vibrations through the chest wall. The morphology of the PRM in tetralogy of Fallot with APV may be related to delayed relaxation with altered diastolic compliance of the RVOT which is subjected to a large regurgitant volume from the massively dilated pulmonary arteries.