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BACKGROUND: Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). AIMS: To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO2). METHODS AND RESULTS: All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p < .0001, r = 0.72). RV end-diastolic area > 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO2. Among the Echo data only right atrial strain (RAS) correlated with peak VO2. CONCLUSION: In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO2 and should be added in their follow up.

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INTRODUCTION: Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients. METHODS: We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). RESULTS: After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01). CONCLUSIONS: RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.

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Aortic regurgitation (AR) continues to be an important cause of morbidity and mortality in pediatric patients. Although echocardiographic parameters are well established for the adults, there are no clear cut-off values for AR severity in children. Cardiac magnetic resonance (CMR) imaging is considered a "gold standard" for a quantitative evaluation of the AR, but it is not widely available. This study assesses which echo parameter can accurately define AR severity as assessed by CMR in pediatric patients. A total of 27 pediatric patients (12 ± 3 years, range 6 to 18 years) with different degree of AR underwent echo assessment within an average of 35 days from CMR. CMR included phase-contrast velocity-encoded imaging for the measurement of regurgitant fraction (RF). Severe AR was defined as RF >33%. Echo evaluation included vena contracta, pressure half time, the ratio between the AR jet and the left ventricular outflow tract diameter (jet/left ventricular outflow tract), presence of holodiastolic reversal flow in abdominal aorta, the ratio between the velocity-time integral of the reversal flow over the forward flow in descending aorta (echoRF). Among the studied parameters, the strongest predictor of severe AR, as assessed by CMR, was echoRF. Receiver-operating characteristic curve showed, for a cutoff >0.38, an area under the curve of 0.886 (p <0.0001), a sensitivity of 71%, and a specificity of 100%. Correlation coefficient between echoRF and RF was R = 0.929 (p <0.0001). In conclusion, echoRF is a strong echo-Doppler marker of severe AR in the pediatric population. This parameter should be routinely added in the standard echo evaluation of pediatric patients with AR.

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Arterial hypertension (AH) and diabetes mellitus (DM) are the most common causes of heart deterioration because of their high prevalence in the population. The aim of this study was to evaluate peak left atrial (LA), longitudinal strain (PALS), left ventricular (LV), longitudinal  strain (LS) and global atrial-ventricular strain (GAVS), by speckle-tracking echocardiography (STE), in asymptomatic patients with AH or/and DM and normal LA, LV size and ejection fraction (EF), to analyze their capability to detect early subclinical dysfunction. We enrolled 162 patients affected by AH and/or DM with normal indexed LA volume, LV end-diastolic diameter and a LVEF > 52% (females) or > 54% (males) (60 hypertensives, 52 diabetics and 50 both) and 60 healthy controls. All subjects underwent standard and advanced STE. PALS, LS and GAVS were measured. GAVS was calculated as the algebraic sum of absolute PALS and LS values in four- and two-chambers views. LS, although with lower values in hypertensives, diabetics and both, did not show significant differences between groups. PALS and GAVS were significantly reduced in AH (31.9 ± 10.3% and 49.7 ± 11.2%, respectively) and DM (26.2 ± 7.1% and 42.6 ± 9.8%) compared to controls, and even more if the two coexisted (20.4 ± 6.5% and 37.1 ± 8.4%). PALS had the highest statistical significance and was able to identify subclinical damage independently from LS value. PALS was reduced in patients with AH and/or DM without alteration of standard echo indexes. The value of PALS was independent from LS and was sufficient to identify heart dysfunction in an earlier stage.

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The exact relationship existing among congestion status, brain natriuretic peptide (BNP) changes and acute kidney injury (AKI) has not been elucidated in patients with acute heart failure (AHF). The aims of this study are: to investigate the relation and prognostic role of BNP, AKI and clinical congestion after discharge; to define the exact BNP cut off value or a BNP in-hospital reduction to identify patients with higher risk during vulnerable post-discharge phase. We consecutively enrolled 157 patients with a diagnosis of AHF. BNP and creatinine were measured in all patients, and degree of failure was assessed. AKI was defined as a creatinine increase ≥0.3 mg/dL or eGFR reduction ≥20% during hospitalization. All patients were followed for 1 and 3 months. Of 146 included patients, 110 patients (75%) displayed effective decongestion, 116 (79%) showed a BNP decrease ≥30%, and 28 (19%) developed in-hospital AKI. BNP in-hospital decrease ≥30% was found more often in patients who showed good decongestion in comparison to patients in persistent failure (63 vs 22%; p < 0.001). The ROC curve analyses at 3 months show that both BNP reduction of 30% between admission and discharge and decongestion at discharge identifies patients with a reduced incidence of cardiovascular events (AUC = 0.79, confidence interval 0.68-0.90, sensibility 90%, sensitivity 50% p < 0.001). Kaplan-Meier survival plots show a better outcome in patients with a BNP decrease ≥30% and good decongestion at discharge (p = 0.03). BNP reduction in AHF is associated with decongestion. BNP reduction associated with decongestion at discharge is a favorable prognostic indicator at 90-day survival irrespective of the AKI occurrence.

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