Search

Residual severe coronary artery (CA) lesion (SCL) in children after cardiac surgery involving the CA is a major concern.To evaluate the value of exercise electrocardiogram (eECG) for risk-based screening of SCL.We analysed 135 maximal eECG from 115 children (mean age 13.6±3.7 years) who underwent concomitant CA imaging. SCL was defined as a stenosis exceeding 50%.Underlying congenital heart diseases were transposition of the great arteries (TGA) (n = 116), CA pathway anomaly (n = 13) and left CA from the pulmonary artery (n = 6). Eleven SCLs were identified in 10 patients, of which 3 had a known untreated non-severe lesion and 4 had no lesions on previous imaging. In multivariable analysis, risks markers for SCL were effort chest pain (OR: 4.72, 95% CI: 1.23-18.17; P=0.024), intramural pathway (OR: 4.37, 95% CI: 1.14-16.81; P=0.032). Yacoubs C-type CA was added as a risk marker for patients with TGA (P=0.0009). All patients with SCL had a positive eECG (sensitivity: 100%, 95% CI: 72-100). Specificity was 81% (95% CI: 73-87). In the low-risk group (0 risk markers), 3/95 patients had SCL (3%), and the post-test probability of SCL with positive eECG (PPr+) was 15% (95% CI: 8-21). In the high-risk group (≥1 risk marker) comprising 8/40 SCLs (20%), PPr+ was 53% (95% CI: 35-67).Most SCL tended to develop gradually, years after surgery. Provided it is near maximal, a negative eECG appears sufficient to exclude SCL. In the high-risk group, PPr+ exceeded 50%.

Clinical and prognostic role of cardiac magnetic resonance (CMR) in adult population with hypertrophic cardiomyopathy (HCM) have been largely assessed. We sought to investigate the role of CMR for predicting cardiovascular events in children with HCM.CMR was performed in 116 patients with HCM (37 sarcomeric mutations, 31 other mutations, mean age 10.4 ± 4.3 yrs). CMR protocol included cine imaging for evaluation of morphology and function and late gadolinium enhancement (LGE). Hard cardiac events (sustained VT, resuscitated cardiac arrest, sudden cardiac death, end-stage heart failure, heart transplant and appropriate ICD intervention) were recorded through a median follow-up of 4 (1-7) years.During follow-up 21 heart cardiac events occurred. At maximal-rank statistic the optimal cut-point for LGE extent for predicting events was ≥2%. Syncope, non-sustained ventricular tachycardia (NSVT) and LGE extent ≥2% were independent predictors of events. At Harrel's C statistic combination of LGE extent ≥2% and syncope was the strongest model for predicting events. HR of patients with LGE extent ≥2% and no history of syncope was 3.6 (1.1-12.2) that increased to 37.6 (5.4-161) in those with LGE extent ≥2% and syncope. The median time dependent AUC of LGE extent (0.88, 95% CI 0.86-0.89) was significantly higher than that of syncope (0.63, 95% CI 0.61-0.66, p 0.0001) and NSVT (0.52, 95% CI 0.50-0.53, p 0.0001).In children with HCM, LGE and syncope were independent predictors of hard cardiac events at follow-up.

2D speckle tracking echocardiography (2DSTE) is superior to standard echocardiography in the assessment of subtle right ventricle (RV) systolic dysfunction. In this study we aimed to: 1) test the hypothesis that 2DSTE may unveil subtle RV systolic dysfunction in patients with Fabry disease; 2) investigate whether the physiologic difference between the 3-segment (RV-FWS) and the 6-segment (RV-GLS) RV strain (∆RV strain) is preserved in Fabry patients.Standard echocardiography and 2DSTE were performed in 49 Fabry patients and 49 age- and sex-matched healthy controls. Fabry patients were divided in two groups according to the presence/absence of left ventricular hypertrophy (LVH+: left ventricular wall thickness 12 mm, 49% of total Fabry patients). RV systolic function assessed by standard echocardiography was normal in the majority of Fabry patients (92%) while RV-GLS and RV-FWS were impaired in about 40%. RV-GLS and RV-FWS were significantly worse in patients LVH+ vs LVH- and vs controls (RV-GLS: LVH+ vs LVH-: -18.4 ± -4.3% vs -23.8 ± -3.1% p0.001; LVH+ vs controls: -18.4 ± -4.3% vs -23.9 ± -2.8% p0.001; RV-FWS: LVH+ vs LVH-: -21.8 ± -5.3% vs -26.7 ± -3.8% p = 0.002, LVH+ vs controls -21.8 ± -5.3% vs -26.8 ± -3.9% p0.001). No difference was found between LVH- patients and controls in both RV-GLS (p = 0.65) and RV-FWS (p = 0.79). ∆RV strain was similar among the groups.In Fabry cardiomyopathy impaired RV-GLS and RV-FWS is a common finding, while RV strain is preserved in Fabry patients without overt cardiac involvement. The physiologic difference between RV-FWS and RV-GLS is maintained in Fabry patients, regardless of the presence of cardiomyopathy.

Funding Acknowledgements Type of funding sources: None. Background The right ventricle-pulmonary artery (RV-PA) coupling is a strong prognostic marker in several clinical settings, but only few studies focused on its role in repaired Tetralogy of Fallot (rToF) with pulmonary regurgitation (PR). Aim of this study was to assess whether differences exist in RV-PA coupling, estimated as by echocardiography, between patients with rToF and PR with (i-PVR) or without (ni-PVR) indication for pulmonary valve replacement (PVR). 3 Materials and Methods The study population included 40 rToF patients allocated to two groups: 20 i-PVR and 20 ni-PVR; 40 healthy controls were also included. All subjects underwent echocardiogram, while Cardiac magnetic resonance (CMR) was available in 27/40 rToF patients. RV-PA coupling was assessed by echocardiographic TAPSE/PASP and RV stroke volume/RV end systolic volume (RVSV/RVESV) by CMR. Results TAPSE was similar in i-PVR vs ni-PVR (19.0 ± 3.4 vs 18.8 ± 2.7 mm, p = 0.85) while RV-PA coupling was significantly worse in i-PVR vs ni-PVR (TAPSE/PASP 0.8 ± 0.3 vs 1.1 ± 0.5 mm/mmHg, p = 0.009) as well as in i-PVR vs controls (p = 0.02) while there was no difference between ni-PVR and controls (p = 0.29). CMR data confirmed the echo results, with a significant difference in RV-PA coupling between i-PVR and ni-PVR (RVSV/RVESV 0.9 ± 0.2 vs 1.2 ± 0.3 mL/min/mL, p = 0.01). Conclusions This study shows the presence of worse RV-PA uncoupling, despite normal RV systolic function, in rToF patients with indication to PVR. RV-PA coupling could be a sensitive marker of a progressive maladaptive RV response to long-standing volume overload in rToF prior to the onset of clinical symptoms and RV systolic dysfunction. Abstract Figure. example of an i-PVR patient

ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).MethodsIn this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.ResultsShort PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ2 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78.ConclusionsStandard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL ≥1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis.

PURPOSE: To assess the ability of four-dimensional (4D) flow MRI to quantify flow volume of the Fontan circuit, including the frequency and hemodynamic contribution of systemic-to-pulmonary venovenous collateral vessels. MATERIALS AND METHODS: In this retrospective study, patients with Fontan circulation were included from three institutions (2017–2021). Flow measurements were performed at several locations along the circuit by two readers, and collateral shunt volumes were quantified. The frequency of venovenous collaterals and structural defects were tabulated from concurrent MR angiography, contemporaneous CT, or catheter angiography and related to Fontan clinical status. Statistical analysis included Pearson and Spearman correlation and Bland-Altman analysis. RESULTS: Seventy-five patients (mean age, 20 years; range, 5–58 years; 46 female and 29 male patients) were included. Interobserver agreement was high for aortic output, pulmonary arteries, pulmonary veins, superior vena cava (Glenn shunt), and inferior vena cava (Fontan conduit) (range, ρ = 0.913–0.975). Calculated shunt volume also showed strong agreement, on the basis of the difference between aortic and pulmonary flow (ρ = 0.935). A total of 37 of 75 (49%) of the patients exhibited shunts exceeding 1.00 L/min, 81% (30 of 37) of whom had pulmonary venous or atrial flow volume step-ups and corresponding venovenous collaterals. A total of 12% of patients (nine of 75) exhibited a high-output state (>4 L/min/m(2)), most of whom had venovenous shunts exceeding 30% of cardiac output. CONCLUSION: Fontan flow and venovenous shunting can be reliably quantified at 4D flow MRI; high-output states were found in a higher proportion of patients than expected, among whom venovenous collaterals were common and constituted a substantial proportion of cardiac output. Keywords: Pediatrics, MR Angiography, Cardiac, Technology Assessment, Hemodynamics/Flow Dynamics, Congenital Supplemental material is available for this article. © RSNA, 2021

Background Progressive right heart chambers dilatation is frequent in the adult congenital heart disease (ACHD) population. We evaluated the immediate and mid-term response of right heart chambers to surgery performed in adulthood for lesions associated with right heart chambers enlargement. Methods Thirty-six adult patients with lesions associated with right heart chambers enlargement submitted to surgery were studied . We collected echocardiographic data of right ventricle (RV) mid-diameter, right atrial volume indexed, RV systolic pressure, and tricuspid annular plane systolic excursion (TAPSE) prior to surgery (T0), at 2 to 5 days (T1), and 3 to 6 months (T2) after surgery. Results At T1, we observed a significant decrease of RV mid-diameter (47.2 ± 8.4 vs. 39.6 ± 7.4 mm, P Conclusions Positive right heart chambers remodeling occurs as early as in the immediate post-operative period in most ACHD patients operated for lesions associated with right heart chambers enlargement.

A large proportion of patients with coronary artery disease treated with elective percutaneous coronary intervention have high residual platelet reactivity1 and endothelial dysfunction, which might represent the link to the occurrence of ischemic events.2 This is even more the case for patients with diabetes mellitus, in whom more potent P2Y12 receptor inhibitors have been proposed with promising results in terms of reduced platelet reactivity.3 Nevertheless, whether this enhanced platelet inhibition alsomight be beneficial to the endothelial function is yet unknown. CLOTILDIA (Clopidogrel High Dose Versus Ticagrelor for Antiplatelet Maintenance in Diabetic Patients)4 was a single-center, prospective, randomized, open label, crossover study, enrolling patients with type2 diabetes mellitus and stable coronary artery disease treated with percutaneous coronary intervention and drug-eluting stent implantation. Patients were recruited at least 1month after percutaneous coronary intervention (3.8±2.1 months), while they were still on dual-antiplatelet therapy with aspirin (100 mg/d) and clopidogrel (75 mg/d). At study entry (T0), with the use ofa computer-based randomization system, patients were assigned randomlyto receive either 90 mg ticagrelor twice daily or clopidogrel 150 mg once daily for 14 …

We report the case of a 65-year-old man referred to our department because of a complex coronary aneurysmal disease presenting as subacute ST-segment elevation myocardial infarction due to intraluminal thromboembolism. The patient's past medical history (recurrent episodes of lymphadenopathies, parotid swelling, prostatitis, and dacryoadenitis) raised the suspicion of a unifying systemic disorder with coronary involvement. An extensive infectious and autoimmune screening was performed, leading to the final diagnosis of IgG4-related disease. Our case highlights the importance to include this rare and recently described disease in the diagnostic workup of acquired coronary aneurysms.