Selection of the best of 2022 in congenital heart disease

This paper shows a selection of the most relevant articles in congenital heart diseases in the last year. About intervencional procedures, we comment the latest guidelines in 2021, as well as some interesting papers on the implementation of materials and techniques in the field of percutaneous treatment of congenital heart diseases. In cardiac imaging, we focus on articles related to the revolutionary advance of 4D-NMR and 3D echo in the study of these congenital diseases. Respect pediatrics, there are interesting studies about multisystem inflammatory syndrome linked to SARS-CoV-2 (MIS-C) and also in the field of hypertrophic cardiomyopathy. Related to surgery, we comment the hybrid technique for treating hypoplastic left ventricle. Finally, regarding arrhythmias in congenital heart disease, we focus our attention on atrial fibrillation, due to the peculiarities that exist in this type of patients.

Three dimensional echocardiography and global longitudinal strain in the assessment of left ventricular function in children after PIMS-TS

Background and Aim: Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 (PIMS-TS) is a new dis-ease affecting children, almost alwaysinvolving cardiovascular system and with potential long-term effects still unknown. Method(s): Prospective study enclosed 80 children aged 1-17 years (mean 8.2 years) diagnosed with PIMS-TS between June 2020 and June 2021 who were controlled 6 weeks and 6 months after the disease. In patients with severe cardiac involvement during acute phase (deteriorated left ventricular ejection fraction (LVEF) lt;55% and significantly elevated concentration of NT-pro-BNP (gt;5000 pg/ml) or troponine (gt;500 ng/ml)) the addi-tional check-up after 3 months was scheduled. In all patients at control points three dimensional echocardiography (3D-ECHO) and average global longitudinal strain (GLS) were used to assess left ventricular function. Result(s): In all patients the means of LVEF and average GLS were within normal limits at the time of all check-up points. For the whole group LVEF after 6 weeks was 60.5% (SD: 3.1;51-69%) and GLS 21.2% (SD: 3.9;12.4-29.4%). After 6 months LVEF increased to 63% (SD: 2.4;58-69%) and GLS to 23.6% (SD: 3.2;17.3-33.3%)-both significantly (plt;0.001). In the subgroup of 54 patients with originally mild cardiac involvement LVEF after 6 weeks was 60.7% (SD: 2.6;57-69%) and GLS 21.8% (SD: 3.4;17.3-29.4%). In the subgroup of 26 patients with severe cardiac involvement LVEF after 6 weeks was 59.6% (SD: 3.1;55-67%) and was not significantly different than in subgroup with mild car-diac involvement (p = 0.175) while GLS was significantly lower (19.3%, SD: 3.8;12.4-24.8%;p = 0.009). After 3 months in this group LVEF and GLS did not increase (respectively, 59.9%, 56-67%;p = 0.794 and 20.2%, 13.7-26.9%;p = 0.149). After 6 months LVEF in this subgroup increased to 62.8% (60-68%) and GLS to 22.6% (17.7-27%)-like in patients with mild cardiac involvement (plt;0.001). Conclusion(s): 3D-ECHO and GLS are highly applicable tools for the assessment of cardiac function in children after PIMS-TS. Patients with originally severe cardiovascular involvement have lower average GLS after 6 weeks. 6 months after PIMS-TS patients present significant improvement of left ventricular function. Average GLS seems to be more sensitive test for functional assess-ment than LVEF.

Cardiac dyssynchrony in patients with Paediatric Inflammatory Multisystem Syndrome Temporarily associated with SARS-CoV-2 (PIMS-TS)

Introduction: Paediatric Inflammatory Multisystem Syndrome Temporarily associated with SARS-CoV-2 (PIMS-TS) is commonly associated with cardiovascular compromise. We have previously described the time course and magnitude of left ventricular (LV) systolic dysfunction in children and young adults with PIMSTS. However, it remains unknown if this inflammatory process can cause LV dyssynchrony. We aim to establish whether paediatric patients with PIMS TS develop LV dyssynchrony as assessed by echocardiography. Methods: Comprehensive transthoracic echocardiography in 10 PIMS-TS patients was performed during the acute stage of the initial illness when LV systolic function (3D Ejection Fraction (EF)) was worst and then again at six months post PIMS-TS. At both time points, we compared: 3D EF, LV fractional shortening (FS) and global longitudinal strain (GLS). Intraventricular LV dyssynchrony was assessed byMmode, PW tissue Doppler Velocities (TDI), 2-D speckle tracking and 3D echocardiography, while the interventricular dyssynchrony was also assessed by TDI at both time points. Results: Any improvement in 3D-LV EF at six months post illness (57.8±5.5 %) vs acute phase (51.8± 9.9%) was not statistically significant (p=0.166), whereas the LV FS (29.9± 9.5% vs 36.5± 12.5%, p=0.043) and GLS (-13.8±1.9% vs -18.6±3.1%, p=0.005) were significantly lower during the acute phase of the illness compared to six months later. Regarding dyssynchrony, none of the measures differed at follow up compared with acute phase;the septal to posterior wall motion delay assessed by Mmode (46.1±2.7msec vs 38.6±2.1msec, p=0.417), the basal septal to basal lateral peak velocity delay assessed by TDI (23.2±1.9.msec vs 24±1.9msec, p=0.930), the 2D speckle tracking-derived strain delay index was 1.1±1.2% at the time of the worst LV systolic performance and 0.62±0.26% at 6 months in the recovery period (p=0.219). The 3D echocardiography demonstrated that the 3D systolic dyssynchrony index (SDI) remained similar throughout the follow up period (3.04±1.23% at baseline vs 3.22±1.25% at 6 months, p=0.466). Conclusions: Despite the fact that in patients with PIMS TS cardiac involvement show a decline on LV systolic performance, this does not appear to be associated with LV dyssynchrony as assessed by echocardiography. We recommend larger patient cohort studies to investigate this further.

Pericardial constriction-like echocardiographic findings in elite athletes following mild covid-19 infection: A propensity score-matched analysis

Introduction: The cardiovascular effects of COVID-19 in elite athletes is still a matter of intense scientific debate. Hypothesis: We sought to perform a comprehensive echocardiographic characterization of postCOVID athletes by comparing them to a non-COVID athlete cohort. Methods: 107 elite athletes with COVID-19 were prospectively enrolled (P-CA;23±6 years, 23% female) 107 healthy athletes were selected as a control group using propensity score matching (NCA). All athletes underwent 2D and 3D echocardiography. Left (LV) and right ventricular (RV) enddiastolic volumes (EDVi) and ejection fractions (EF) were quantified. To characterize LV longitudinal deformation, 2D global longitudinal strain (GLS) and the ratio of free wall versus septal longitudinal strain (FWLS/SLS) were also measured. To describe septal flattening (SF-frequently seen in PCA), LV eccentricity index (EI) was calculated.Results: P-CA and N-CA athletes had comparable LV and RVEDVi (P-CA vs N-CA;77±12 vs. 78±13mL/m2;79±16 vs 80±14mL/m2). P-CA had significantly higher LVEF (58±4 vs 56±4%, p<0.001), while LVGLS values did not differ between P-CA and N-CA (-19.0±1.9 vs -18.8±2.2%). EI was significantly higher in P-CA (1.13±0.16 vs 1.01±0.05, p<0.001), which was attributable to a distinct subgroup of P-CA with a prominent SF (n=35, 33%), further provoked by inspiration. In this subgroup, the EI was markedly higher compared to the rest of the P-CA (1.29±0.15 vs 1.04±0.08, p<0.001), LVEDVi was also significantly higher (80±14 vs 75±11 mL/m2, p<0.001), while RVEDVi did not differ (82±16 vs 78±15mL/m2). Moreover, the FWLS/SLS ratio was significantly lower in the SF subgroup (91.7±8.6 vs 97.3±8.2, p<0.01). P-CA with SF experienced symptoms less frequently (1.4±1.3 vs. 2.1±1.5 symptom during the infection, p=0.01). Conclusions: COVID-19 infection might be frequently associated with a constriction-like physiology in elite athletes.

Frequent constriction-like echocardiographic findings in elite athletes following mild COVID-19: In the grasp of SARS-CoV-2?

The COVID-19 pandemic had a major impact on the sports community as well. Despite the vast majority of athletes experiencing mild symptoms, potential cardiac involvement and complications have to be explored to support a safe return to play. Accordingly, we were aimed at a comprehensive echocardiographic characterization of post-COVID athletes (P-CA) by comparing them to a propensity-matched healthy, non-COVID athlete (NCA) cohort. One hundred and seven elite athletes with COVID-19 were prospectively enrolled after an appropriate quarantine period and formed the P-CA group (23±6 years, 23% female). From our retrospective database comprising 425 elite athletes, 107 age-, gender-, body surface area-, and weekly training hours-matched subjects were selected as a reference group using propensity score matching (N-CA group). All athletes underwent a comprehensive clinical investigation protocol comprising 2D and 3D echocardiography. Left (LV) and right ventricular (RV) end-diastolic volumes (EDVi) and ejection fractions (EF) were quantified using dedicated softwares. To characterize LV longitudinal deformation, 2D global longitudinal strain (GLS) and the ratio of free wall versus septal longitudinal strain (FWLS/SLS) were also calculated. In order to describe septal flattening (SF-frequently seen in P-CA), LV eccentricity index (EI) was measured. P-CA and N-CA athletes had comparable LV and RV EDVi (P-CA vs NCA;77±12 vs 78±13mL/m2;79±16 vs 80±14mL/m2, respectively). P-CA group had significantly higher LV EF (58±4 vs 56±4%, p<0.001) and GLS (-18.2±1.8 vs -17.6±2.2%, p<0.05). Eccentricity index was significantly lower in P-CA (0.89±0.10 vs 0.99±0.04, p<0.001), which was attributable to a distinct subgroup of P-CA athletes with a prominent SF (n=34, 32%), further provoked by inspiration. In this subgroup, the eccentricity index was markedly lower compared to the rest of the P-CA group (0.79±0.07 vs 0.95±0.07, p<0.001). In the SF subgroup, LV EDVi was significantly higher (80±14 vs 75±11 mL/m2, p<0.001), while RV EDVi did not differ (82±16 vs 78±15mL/m2). Moreover, the FWLS/SLS ratio was significantly lower in the SF subgroup (0.92±0.09 vs 0.97±0.08, p<0.01). Interestingly, P-CA athletes with SF experienced fatigue (17 vs 34%, p<0.05) or chest pain (0 vs 15%, p=) less frequently during the course of the infection;however, the presence of a mild pericardial effusion was more common (41 vs 12%, p<0.01). Elite athletes following COVID-19 showed distinct morphological and functional cardiac changes compared to a propensity score-matched control athlete group. These results are mainly driven by a subgroup, which presented with some echocardiographic features characteristic of constrictive pericarditis (septal flattening, lower FWLS/SLS ratio, pericardial effusion). Follow-up of athletes and further, higher case number studies are warranted to determine the clinical significance and potential effects on exercise capacity of these findings.

Echocardiographic Findings and Correlation with Laboratory Values in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19.

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p < 0.001), E/A ratio was significantly lower, and averaged E/e' ratio was significantly higher (median 1.5 vs. 1.8, p < 0.05; 8.9 vs. 6.3, p < 0.001 respectively) in patients compared to control. Patients were divided into 2 groups according to 3D LV ejection fraction (LVEF) on the echo-1: Group 1; LVEF < 55%, 26 patients, and group 2; LVEF ≥ 55%, 24 patients. E/e' ratio was significantly higher in group 1 than group 2 and control at discharge (median 7.4 vs. 6.9, p = 0.005; 7.4 vs. 6.3, p < 0.001 respectively). Coronary ectasia was detected in 2 patients (z score: 2.53, 2.6 in the right coronary artery), and resolved at discharge. Compared with group 2, group 1 had significantly higher troponin-I (median 658 vs. 65 ng/L; p < 0.001), NT-pro BNP (median 14,233 vs. 1824 ng/L; p = 0.001), procalcitonin (median 10.9 vs. 2.1 µg/L; p = 0.009), ferritin (median 1234 vs. 308 µg/L; p = 0.003). The most common findings were ventricular systolic dysfunction recovering during hospitalization, and persisting LV diastolic dysfunction in the reduced LVEF group at discharge. Coronary artery involvement was rare in the acute phase of the disease. Also, in MIS-C patients, the correlation between LV systolic dysfunction and markers of inflammation and cardiac biomarkers should be considered.

Prognostic Value of Right Ventricular Ejection Fraction Assessed by 3D Echocardiography in COVID-19 Patients.

Background: RVEF (right ventricular ejection fraction) measured by three-dimensional echocardiography (3DE) has been used in evaluating right ventricular (RV) function and can provide useful prognostic information in other various cardiovascular diseases. However, the prognostic value of 3D-RVEF in coronavirus disease 2019 (COVID-19) remains unknown. We aimed to investigate whether 3D-RVEF can predict the mortality of COVID-19 patients. Methods: A cohort of 128 COVID-19-confirmed patients who had undergone echocardiography were studied. Thirty-one healthy volunteers were also enrolled as controls. COVID-19 patients were divided into three subgroups (general, severe, and critical) according to COVID-19 severity-of-illness. Conventional RV structure and function parameters, RV free wall longitudinal strain (FWLS) and 3D-RVEF were acquired. RVFWLS was measured by two-dimensional speckle tracking echocardiography. RVEF was acquired by 3DE. Results: Compared with controls, 2D-RVFWLS and 3D-RVEF were both significantly decreased in COVID-19 patients (-27.2 ± 4.4% vs. -22.9 ± 4.8%, P < 0.001; 53.7 ± 4.5% vs. 48.5 ± 5.8%, P < 0.001). Critical patients were more likely to have a higher incidence of acute cardiac injury and acute respiratory distress syndrome (ARDS), and worse prognosis than general and severe patients. The critical patients exhibited larger right-heart chambers, worse RV fractional area change (RVFAC), 2D-RVFWLS, and 3D-RVEF and higher proportion of pulmonary hypertension than general and severe patients. Eighteen patients died during a median follow-up of 91 days. The multivariate Cox regression analysis revealed the acute cardiac injury, ARDS, RVFAC, RVFWLS, and 3D-RVEF were independent predictors of death. 3D-RVEF (chi-square to improve 18.3; P < 0.001), RVFAC (chi-square to improve 4.5; P = 0.034) and 2D-RVFWLS (chi-square to improve 5.1; P = 0.024) all provided additional prognostic value of higher mortality over clinical risk factors. Moreover, the incremental predictive value of 3D-RVEF was significantly (P < 0.05) higher than RVFAC and RVFWLS. Conclusion: 3D-RVEF was the most robust independent predictor of mortality in COVID-19 patients and provided a higher predictive value over conventional RV function parameters and RVFWLS, which may be helpful to identify COVID-19 patients at a higher risk of death.

Left and right ventricular dysfunction in patients with COVID-19-associated myocardial injury.

PURPOSE: SARS-COV-2 infection can develop into a multi-organ disease. Although pathophysiological mechanisms of COVID-19-associated myocardial injury have been studied throughout the pandemic course in 2019, its morphological characterisation is still unclear. With this study, we aimed to characterise echocardiographic patterns of ventricular function in patients with COVID-19-associated myocardial injury. METHODS: We prospectively assessed 32 patients hospitalised with COVID-19 and presence or absence of elevated high sensitive troponin T (hsTNT+ vs. hsTNT-) by comprehensive three-dimensional (3D) and strain echocardiography. RESULTS: A minority (34.3%) of patients had normal ventricular function, whereas 65.7% had left and/or right ventricular dysfunction defined by impaired left and/or right ventricular ejection fraction and strain measurements. Concomitant biventricular dysfunction was common in hsTNT+ patients. We observed impaired left ventricular (LV) global longitudinal strain (GLS) in patients with myocardial injury (-13.9% vs. -17.7% for hsTNT+ vs. hsTNT-, p = 0.005) but preserved LV ejection fraction (52% vs. 59%, p = 0.074). Further, in these patients, right ventricular (RV) systolic function was impaired with lower RV ejection fraction (40% vs. 49%, p = 0.001) and reduced RV free wall strain (-18.5% vs. -28.3%, p = 0.003). Myocardial dysfunction partially recovered in hsTNT + patients after 52 days of follow-up. In particular, LV-GLS and RV-FWS significantly improved from baseline to follow-up (LV-GLS: -13.9% to -16.5%, p = 0.013; RV-FWS: -18.5% to -22.3%, p = 0.037). CONCLUSION: In patients with COVID-19-associated myocardial injury, comprehensive 3D and strain echocardiography revealed LV dysfunction by GLS and RV dysfunction, which partially resolved at 2-month follow-up. TRIAL REGISTRATION: COVID-19 Registry of the LMU University Hospital Munich (CORKUM), WHO trial ID DRKS00021225.