Pierluigi Festa

Amino-Terminal Fragment of Pro-Brain Natriuretic Hormone Identifies Functional Impairment and Right Ventricular Overload in Operated Tetralogy of Fallot Patients

To evaluate the relationship between plasma concentration of amino-terminal fragment of pro-brain natriuretic peptide (NT-proBNP), functional capacity, and right ventricular overload in survivors of tetralogy of Fallot (TOF) repair, we prospectively studied 70 operated TOF patients (44 males, 21xa0±xa01 years old; meanxa0±xa0SEM) who underwent, during the same day, echocardiography, cardiac magnetic resonance imaging, neurohormonal characterization (plasma NT-proBNP, catecholamines, plasma renin activity, and aldosterone assay), and cardiopulmonary exercise testing. Forty-eight age- and sex-matched healthy volunteers served as the control group. Compared to controls, maximal workload and peak oxygen consumption (VO2/kg) were lower in operated TOF patients (pxa0<xa00.001), whereas NT-proBNP concentration was elevated (pxa0<xa00.001). No difference was found among the other neurohormones. In operated TOF patients, NT-proBNP showed a significant positive correlation with right ventricular (RV) end systolic and end diastolic volumes and RV systolic pressure, and it showed a negative correlation with peak VO2/kg and RV ejection fraction. From multivariable analysis, NT-proBNP concentration was found to be an independent predictor of peak VO2/kg, RV end systolic volume, and RV systolic pressure. These results show an association among RV overload, decrease in functional capacity, and cardiac natriuretic peptide expression in operated TOF patients. NT-proBNP plasma assay may be a useful tool for diagnostic purposes and for decision making in this setting.

Role of Stress Echocardiography in Operated Fallot: Feasibility and Detection of Right Ventricular Response

BACKGROUNDnPatients with repaired tetralogy of Fallot often present residual hemodynamic abnormalities leading to right ventricular (RV) burden. Semisupine exercise echocardiography (Ex-Echo) is a validated method for diagnosis and prognosis in ischemic and valvular heart diseases and has potential for the evaluation of RV burden, pressure, and function. The aims of this study were to assess the effect of exercise on the right ventricle in adults with repaired tetralogy of Fallot and to identify factors associated with decreased RV function at peak exercise in an observational study.nnnMETHODSnA total of 128 patients with repaired tetralogy of Fallot referred to an outpatient congenital heart disease unit were evaluated by Ex-Echo and conventional clinical and diagnostic examinations (i.e., electrocardiography, transthoracic echocardiography, cardiovascular magnetic resonance, cardiopulmonary exercise testing, and N-terminal pro-brain natriuretic peptide assay). The following Ex-Echo parameters were measured at rest and at peak exercise: tricuspid annular plane systolic excursion, RV pressure, and RV fractional area change (FAC).nnnRESULTSnInterpretable images for RV FAC analysis were obtained in 123 of 128 patients. In 91 of 128 with detectable tricuspid valve regurgitation, RV systolic pressure during exercise was evaluated. According to positive or negative RV FAC variation during exercise, 74 patients were respectively defined as responders on stress echocardiography and 49 as nonresponders; the median percentage change between rest and stress was 13.8% (interquartile range, 5.9% to 26.9%) in responders and -13.5% (interquartile range, -25.4% to -7.4%) in nonresponders. Systolic RV systolic pressure increased in a similar manner in the two groups (65xa0±xa036% in responders vs 59xa0±xa039% in nonresponders, Pxa0=xa0.45). Tricuspid annular plane systolic excursion increased significantly during peak exercise in responders from 17.2xa0±xa03.4 mm at rest to 19.7xa0±xa04.3 mm (Pxa0<xa0.0001) but did not in nonresponders (from 16.9xa0±xa04.7 to 18.1xa0±xa04.6xa0mm, Pxa0=xa0.20). Left ventricular end-diastolic volume at rest and left ventricular ejection fraction < 50% were related to the lack of increased RV FAC on exercise.nnnCONCLUSIONSnEx-Echo is feasible in patients with repaired tetralogy of Fallot and allows the integrated assessment of variation in RV systolic pressure, area, and function during exercise, which usefully complement more conventional indices of hemodynamic burden in these patients. Longitudinal follow-up is needed to better delineate the prognostic value of the results of Ex-Echo.

Computational fluid dynamics in a model of the total cavopulmonary connection reconstructed using magnetic resonance images.

The recent developments in imaging techniques have created new opportunities to give an accurate description of the three-dimensional morphology of vessels. Such three-dimensional reconstruction of anatomical structures from medical images has achieved importance in several applications, such as the reconstruction of human bones, spine portions, and vascular districts.

Cardiac magnetic resonance 'virtual catheterization' for the quantification of valvular regurgitations and cardiac shunt.

Cardiac magnetic resonance (CMR) is considered the gold-standard noninvasive technique for the quantification of ventricular volumes by cine-imaging and of vascular flows by velocity-encoded phase contrast (VENC). In routine CMR scans, it is common to found clinical conditions, as valve regurgitations and cardiac shunts, producing a volume overload and significant mismatch between the right and left ventricular stroke volumes (RSV and LSV). In the presence of a valve regurgitation, the volume overload involves the respective ventricular chamber, whereas in cardiac shunts, the location of the volume overload depends on the site of the anatomic defect. Moreover, when a cardiac shunt is present, pulmonary and systemic cardiac outputs are different (Qp/Qsu200a<u200a1 or Qp/Qsu200a>u200a1), whereas in the presence of valve regurgitation, Qp/Qsu200a=u200a1. Therefore, by combining the cine-imaging with the VENC technique, it is possible to investigate the cardiac physiology underlying different pathological conditions producing volume overload, and to quantify this overload (the regurgitant volume and/or shunt volume). In this report, we discussed the technical, theoretical and methodological aspects of this sort of ‘virtual catheterization’ by CMR, providing a simple algorithm to make the correct diagnosis.

Usefulness of Combined Functional Assessment by Cardiac Magnetic Resonance and Tissue Characterization Versus Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy

Current task force criteria (TFC) of cardiac magnetic resonance (CMR) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) were generated by comparing probands (mean age of 44xa0years) to healthy participants of the multi-ethnic study of atherosclerosis (mean age of 60xa0years). These age differences may be a selection bias because right ventricular end-diastolic volume index decreases 4.6% per decade. Moreover, fat infiltration and late gadolinium enhancement were not included. We evaluated the diagnostic accuracy of TFC using the same methodology used by the task force but comparing probands and age- and gender-matched healthy controls and considering also other morphofunctional and tissue abnormalities detected by CMR. Forty-seven probands with previous diagnosis of ARVC/D (excluding probands if CMR was used for diagnosis) were compared with 216 age- and gender-matched healthy controls. TFC had optimal specificity (100%) but poor sensitivity (20% for major and 13% for minor criteria). The presence of any pre- and post-contrast signal abnormalities had 100% specificity and 81% sensitivity. The best diagnostic accuracy (98%) was achieved by the combined evaluation of any right ventricular wall motion abnormality (excluding hypokinesia) with any signal abnormality (including left ventricular fat infiltration and late gadolinium enhancement) yielding a 100% specificity and 96% sensitivity. Left ventricular was involved in 45% of the probands. Current TFC for CMR presented optimal specificity but poor sensitivity to identify patient with ARVC/D. Signal and wall motion parameters of CMR should be considered together to achieve the best diagnostic accuracy for the diagnosis of ARVC/D.

Aortic elasticity indices by magnetic resonance predict progression of ascending aorta dilation.

ObjectivesAortic distensibility and pulse-wave velocity (PWV) are under investigation as parameters by which to evaluate the indication for ascending aorta (AA) replacement. The maximum rate of systolic distension (MRSD) was proposed as a new index of aortic elasticity. The aim of this study was to assess the role of aortic elasticity parameters to predict AA growth rates in patients with AA dilation (AAD).MethodsMagnetic resonance imaging (MRI) was performed annually in 65 patients with AA dilation (median follow-up 17xa0months; 25–75th percentile; range 12–30xa0months). A significant increase in AA diameter was defined as a ≥2-mm increase.ResultsAn increase in AA diameter was found in 42 (68xa0%) patients (AAD+ group) and absent in 20. Median increase was 0.16 (25–75th percentile; range 0.32–0.7) mm/month. The AAD+ group had a lower MRSD (4.6u2009±u20092.2 vs 7.4u2009±u20092.0, pu2009<u20090.001) but the same PWV and distensibility. MRSD showed 93.7xa0% specificity and 75.6xa0% sensitivity for prediction of increase. Patients with MRSDu2009≤u20096 had lower progression-free survival times (pu2009<u20090.002). After a follow-up of 4.1xa0years, patients who underwent surgical therapy had lower MRSD and distensibility than others.ConclusionsMRSD is an index of aorta elastic properties and is a valuable predictor for progression in AAD.Key Points• MRI-derived parameters of aortic wall elasticity predict progression of ascending aorta dilation.• Maximal rate of systolic distension (MRSD) was the best predictor of progression.• Patients with MRSDu2009≤u20096 had lower progression-free survival (PFS) times.• Patients who underwent surgical therapy had lower MRSD and distensibility.• MRI-derived parameters identify patients with fast progression of Ascending Aorta Dilation.

Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance

Fontan palliation allows patients with “single ventricle” circulation to reach adulthood with an acceptable quality of life, although exercise tolerance is significantly reduced. To assess whether controlled respiratory training (CRT) increases cardiorespiratory performance. 16 Adolescent Fontan patients (age 17. 5u2009±u20093.8xa0years) were enrolled. Patients were divided into CRT group (nu2009=u200910) and control group (C group, nu2009=u20096). Maximal cardiopulmonary test (CPT) was repeated at the end of CRT in the CRT group and after an average time of 3xa0months in the C group. In the CRT group a CPT endurance was also performed before and after CRT. In the CRT group there was a significant improvement in cardiovascular and respiratory response to exercise after CRT. Actually, after accounting for baseline values, the CRT group had decreased breathing respiratory reserve (−u200915, 95% CI −22.3 to −u20098.0, pu2009=u20090.001) and increased RR peak (+u20094.8, 95% CI 0.7–8.9, pu2009=u20090.03), VE peak (+u200913.7, 95% CI 5.6–21.7, pu2009=u20090.004), VO2 of predicted (+u20098.5, 95% CI 0.1–17.0, pu2009=u20090.05), VO2 peak (+u20094.3, 95% CI 0.3 to 8.2, pu2009=u20090.04), and VO2 workslope (+u20091.7, 95% CI 0.3–3.1, pu2009=u20090.02) as compared to the control group. Moreover, exercise endurance time increased from 8.45 to 17.7xa0min (pu2009=u20090.01). CRT improves cardiorespiratory performance in post-Fontan patients leading to a better aerobic capacity.

Subaortic ventricular pouch in repaired tetralogy of Fallot mimicking right coronary artery aneurysm

Figure 1. 28-year-old woman with tetralogy of Fallot repair. A and B. view show image suggesting right coronary aneurysm (arrows). C and D dimensional volume rendering (C) and maximum intensity projection re arrows on C) and subaortic pouch (red arrow on D) at the level of the pa show the surgical hypothesis of the subaortic pouch (*). Usual correction the right ventricle is left on the left side of the patch (F). u C e r cars. We illustrate a case of a repaired tetralogy of Fallot resenting with muscular subaortic ventricular pouch that as documented by cardiac computed tomography angiogaphy (CTA) and cardiac magnetic resonance imaging (CMR). A 28-year-old woman was operated at the age of 15 onths for a tetralogy of Fallot. Surgical repair consisted f ventricular septal defect (VSD) closure with a pericarial patch and reconstruction of the right ventricular outflow ract with a transannular patch. She was referred to us for ardiac CTA because of a suspected right coronary aneurysm t TTE (Fig. 1A and B; Online Video 1 and Video 2). She as scheduled, as well, for CMR to evaluate ventricular vol-

Effective Cardiac Index and Systemic-Pulmonary Collaterals Evaluated by Cardiac Magnetic Resonance Late After Fontan Palliation

The regulation of cardiac output in the Fontan circuit is not completely understood. Systemic-pulmonary collaterals (SPCs) are frequent in patients with univentricular heart, and their clinical significance and management remain controversial. The aims of our study were to identify factors associated with SPCs flow at late follow-up after Fontan and evaluate the relation between SPCs flow (QSPCs) and the effective cardiac index (CI). From our cardiac magnetic resonance database, we identified all Fontan patients with a complete set of flow measurements allowing calculation of QSPCs and effective CI. QSPCs was calculated as (left pulmonary veins flowxa0+ right pulmonary veins flow)xa0- (right pulmonary artery flowxa0+ left pulmonary artery flow). Effective CI was calculated as (Aortic flow (QAo)xa0- QSPCs)/BSA. Medical, surgical history, and clinical status were recorded. Sixty-four post-Fontan patients (36xa0male; mean age 19 ± 10xa0years) were included in the study. Median QSPCs was 0.7xa0L/min/m2 (interquartile [IQ] range 0.386-0.983) accounting for a median of 21% (IQ range 13-28) of aortic flow. The effective CI in our population was 2.4 ± 0.6xa0L/min/m2. QSPCs inversely correlate with left pulmonary artery area (rxa0=xa0-0.37, pxa0= 0.004) and total antegrade pulmonary flow (rxa0=xa0-0.32, pxa0= 0.01). QSPCs correlate with indexed aortic flow (rxa0= 0.6, p <0.001) and inversely correlate with effective CI (rxa0=xa0-0.39, pxa0= 0.002). Effective CI inversely correlates with age at study and age at the Fontan palliation (rxa0=xa0-0.35, pxa0= 0.005, and rxa0= -0.29, pxa0= 0.02, respectively) and positively with ventricular ejection fraction (rxa0= 0.3, pxa0= 0.01). In conclusion, SPCs arexa0common in Fontan patients, correlate inversely with effective CI, and are associatedxa0with a reduced antegrade pulmonary flow. In cardiac magnetic resonance evaluation of post-Fontan patients, effective CI should be taken into account rather than the total CI.