Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery, but with limited durability, leading to re-intervention. The present single-center, retrospective, cohort study reports the results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. Patients with CHD referred for RV-PA conduit surgical implantation (from October 1997 to January 2022) were included. The primary outcome was conduit failure, defined as a peak gradient above 64 mm Hg, severe regurgitation, or the need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effects linear regression analysis. A total of 252 patients were initially included; 149 patients were eligible for follow-up data collection. After a median follow-up time of 49 months, the primary study end point occurred in 44 (29%) patients. A multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft implantation as protective factors (hazard ratio 0.11, 95% confidence interval [CI] 0.02 to 0.47 and hazard ratio 0.34, 95% CI 0.16 to 0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (odds ratio 4.29, 95% CI 1.41 to 13.01). Long-term use of oral anticoagulants was independently associated with slower progression of peak echocardiographic gradient across the conduits (mixed-effects linear regression p = 0.027). In patients with CHD, the rate of failure of surgically implanted RV-PA conduits is higher in children and after nonhomograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Long-term anticoagulation seems to exert a protective effect.

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: −0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (−0,14 ± 1.24 vs. –0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population.

Background: A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Methods: Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Results: Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. Conclusions: Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.

Objective: The object of the present study is to evaluate factors precluding heart transplantation (HTx) in adult congenital heart disease patients (ACHD) with end-stage heart failure (HF) referred for HTx evaluation. Methods: This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients receiving HTx between 2001 and 2013. HTx refusal due to poor candidacy status for excess risk of mortality after transplantation served as the main study outcome. Results: Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013 (final sample size 60 patients). We compared clinical, anatomical and demographic data of 41 patients suitable for transplantation with 15 patients refused after screening (excluding 4 patients with ongoing screening). Risk factors for refusal were: multiple high risk features (odds ratio [OR]: 3.6; 95% confidence interval [CI]: 1.1 to 12.9; p 0.048); anatomical factors (OR: 14.5; 95% CI: 3.1 to 68.4; p 0.001), out-of-center ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1 to 8.3; p 0.02). Conclusions: In our study risk factors for refusal from HTx are adverse anatomical features, multiple conventional HTx high risk factors and out-of-center referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy are strongly necessary for this growing population.

Abstract Background Patients with repaired Tetralogy of Fallot (ToF) need periodic instrumental assessments, due to complications they can meet during lifetime. Right ventricle (RV) dilation and dysfunction are some examples that explain why those patients regularly undergo Cardiac Magnetic Resonance (CMR), currently the gold standard method to detect these complications. Purpose 3D-echocardiography is an emerging tool to study RV, but there is little data supporting its use in congenital heart diseases. This study aimed at evaluating its accuracy in assessing RV dimensions and systolic function in ToF patients, in comparison to CMR. Methods 34 patients were prospectively enrolled after CMR performance. They all underwent standard 2D-echocardiography and Multi-Beat-ECG-triggered 3D Full-Volume acquisition. RV End Diastolic Volume (RV-EDV), End Systolic Volume and Ejection Fraction (RV-EF) were defined both in 3D-echocardiography and CMR. Results Post-hoc analysis of 3D images was performed through a vendor-independent software in 30 patients, resulting in a feasibility of 88%. A Bland-Altman analysis was performed, showing that 3D-echocardiography underestimates the RV-EDV/BSA of −27.39 mL/m2 on average (CI 95%: −29.46; −25.31, SD: 5.68 mL/m2), compared to CMR, as expected. However, when compared to normality reference, 3D-echocardiography identified RV dilatation with a significant correlation to CMR (V=0.66, p<0.001). Moreover, ROC curves showed that 3D-echo-RV-EDV/BSA is the echocardiographic measure that best correlates to CMR in detecting RV dilatation (AUC 1, CI 95%: 1.00; 1.00), followed by RVOT diameter in PLAX (AUC 0.89, CI 95%: 0.69; 0.99). Youden analysis suggested 77 mL/m2 (Se 95%, Sp 100%; J 0,95) as optimal limit to define RV dilatation in 3D-echocardiography. Regarding the RV-EF, the Bland-Altman analysis showed that 3D-echocardiography overestimates the RV-EF by 1,97% on average (CI 95%: 0.01; 3.95, SD: 5.43), compared to CMR. Secondly, accuracy of different echocardiographic parameters (both 2D and 3D) in detecting a RV systolic impairment was analyzed: ROC curves showed that RV-EF in 3D echocardiography is the measurement that best correlates to CMR in detecting RV dysfunction (AUC 0.92, CI 95%: 0.79–1.00), followed by Fractional Area Change (FAC) (AUC 0.78, CI 95%: 0.60; 0.96). Tricuspid Annular Plane Systolic Excursion (TAPSE) and tricuspid valve annular motion velocities in systole (S'-TDI), instead, showed very poor correlation. Conclusions 3D-echocardiography showed good agreement with CMR in defining RV dimensions and systolic function in ToF patients, even more than the bidimensional measurements cardiologists are used to. This could lead to a wider use of this tool in daily clinical practice, involving a reduction in the execution of the numerous CMR patients usually undergo. Moreover, it could be a valid instrument in patients with contraindication to CMR. Funding Acknowledgement Type of funding sources: None.

Abstract This case describes a peculiar aetiology of severe aortic regurgitation and LVOT obstruction in a young adult with bilateral congenital cataract and uncontrolled hypertension, who presented with heart failure and was found to suffer from aortic valve degeneration with fibromuscular metaplasia and cusp delamination.

© Author(s) (or their employer(s)) 2022. No commercial reuse. See rights and permissions. Published by BMJ. INTRODUCTION The first surgical rerouting of systemic venous inflow to the pulmonary circulation without a pumping ventricle was pioneered (in humans) by Dr Francis Fontan in 1968, as a palliative approach in three adult patients with tricuspid atresia. After extensive surgical iterations, nowadays, lateral tunnel baffling or extracardiac total cavopulmonary connection (TCPC) represent the modern staged surgical treatment of congenital heart diseases (CHDs) unsuitable for biventricular circulation either due to single ventricular anatomical cluster or adverse anatomical constraints (atrial isomerism, straddling atrioventricular valves and others) (figure 1). Longterm survival of patients with Fontan circulation (FC) outgrew the expectations and ~95% survival rate at 10 years after Fontan surgery is reported with the more modern surgical innovations. However, such delicate circulatory compromise comes at a cost along the way, and a constellation of cardiac and noncardiac complications is reported in this growing patient population. Large registry data report 10year freedom from death, heart transplantation (HTx), Fontan takedown or conversion, proteinlosing enteropathy (PLE), plastic bronchitis (PB), or New York Heart Association functional class III or IV between 79% and 92% based on underlying ventricular morphology. Fontan failure is defined as a broad, nonspecific term describing dysfunction of the FC that affects a patient’s ability to carry out daily life activities. Aetiologies may include ventricular dysfunction, atrioventricular valve failure, increased pulmonary vascular resistance, recurrent arrhythmia, Fontan pathway obstruction, lymphatic insufficiency or endorgan dysfunction. Fontan failure management is one of the most challenging areas of adult CHD (ACHD) medicine, due to limited evidenceguiding therapies, poor prognosis and potential acute onset (such as in case of arrhythmias or thrombotic event). This review describes a rational approach to management of Fontan failure based on published evidence and expert opinions, covering diverse aspects including specifics of FC, standardised clinical approach to Fontan patients, Fontanassociated liver disease (FALD), cardiorenal syndrome (CRS), arrhythmias, venous and arterial thromboembolism (TE), and lymphatic pathology.

Aim: The objective of this study was to assess left ventricle (LV) function in patients underwent arterial switch procedure (ASO) for transposition of great arteries (TGA) in long-term follow-up. Methods: We studied 59 asymptomatic patients (43 male) who have undergone single-stage ASO for TGA, aged 13.9 +/- 4.8 years, with a normal LV ejection fraction, compared to healthy peers. We evaluated LV volume, function and myocardial deformation in asymptomatic patients with normal ejection fraction by using speckle-tracking echocardiography (STE). Results: Global longitudinal strain (GLS) was lower in patients compared to healthy peers throughout all age groups (5-9 years: 20.03 +/- 0.65% vs 21.00 +/- 1.30%, p = 0.083; 10-14 years: 19.43 +/- 1.75% vs 21.80 +/- 1.30%, p < 0.0001; 15-19 years: 19.05 +/- 1.65% vs 22.50 +/- 1.30%, p < 0.0001; 20-24 years: 17.90 +/- 0.85% vs 20.90 +/- 1.30%, p < 0.0001; >25 years: 18.60 +/- 0.42% vs 20.60 +/- 1.20%, p = 0.041). At the univariate analysis GLS resulted significantly related only to the presence of restrictive patent foramen ovale at birth (p = 0.0016). At the multivariate analysis GLS was significantly related to prenatal diagnosis, restrictive patent foramen ovale and by-pass time. Conclusion: Children and young adults late after ASO demonstrate normal ejection fraction, but present sub-clinical signs of myocardial dysfunction, such as reduction of longitudinal strain. Our findings support the usefulness of STE to detect it precociously.

Abstract Aims End-stage heart failure (HF) is the leading cause of death in adult congenital heart disease (ACHD) population. Heart transplantation (HTx) improves prognosis in ACHD end-stage HF but candidacy evaluation, referral pattern, and correct listing timing are not fully elucidated in this population. To evaluate factors associated to refusal from Htx in ACHD patients with end-stage HF referred for HTx evaluation. Methods and results This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients undergone HTx between 2000 and 2013. Refusal from HTx served as primary study endpoint. Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013. The main indication to HTx in patients with single ventricle physiology was Fontan failure, while in patients with systemic left ventricle and systemic right ventricle physiology, it was systemic ventricular dysfunction. We compared clinical, anatomical and demographic data of 41 patients accepted for transplantation with 15 patients refused after screening. Risk factors for refusal were: coexistence of multiple high risk features [odds ratio (OR): 3.6; 95% confidence interval (CI): 1.1–12.9; P 0.048]; anatomical factors (OR: 14.5; 95% CI: 3.1–68.4; P 0.001), out-of-centre ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). Survival in patients accepted for HTx was significantly higher than survival in patients declined from HTx with landmark comparison at 20, 40 and 60 months of 87%, 78%, and 72% vs. 70%, 59%, and 20%, respectively. HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1–8.3; P 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, coexistence of multiple conventional HTx high risk factors and out-of-centre referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy and to reduce referral delay in tertiary centre are strongly necessary for this growing population.

OBJECTIVES:Energy demand and supply need to be balanced to preserve myocardial function during paediatric cardiac surgery. After a latent aerobic period, cardiac cells try to maintain energy production by anaerobic metabolism and by extracting oxygen from the given cardioplegic solution. Myocardial oxygen consumption (MVO2) changes gradually during the administration of cardioplegia.METHODS:MVO2 was measured during cardioplegic perfusion in patients younger than 6 months of age (group N: neonates; group I: infants), with a body weight less than 10 kg. Histidine-tryptophan-ketoglutarate crystalloid solution was used for myocardial protection and was administered during a 5-min interval. To measure pO2 values during cardioplegic arrest, a sample of the cardioplegic fluid was taken from the inflow line before infusion. Three fluid samples were taken from the coronary venous effluent 1, 3 and 5 min after the onset of cardioplegia administration. MVO2 was calculated using the Fick principle.RESULTS:The mean age of group N was 0.2 ± 0.09 versus 4.5 ± 1.1 months in group I. The mean weight was 3.1 ± 0.2 versus 5.7 ± 1.6 kg, respectively. MVO2 decreased similarly in both groups (min 1: 0.16 ± 0.07 vs 0.36 ± 0.1 ml/min; min 3: 0.08 ± 0.04 vs 0.17 ± 0.09 ml/min; min 5: 0.05 ± 0.04 vs 0.07 ± 0.05 ml/min).CONCLUSIONS:We studied MVO2 alterations after aortic cross-clamping and during delivery of cardioplegia in neonates and infants undergoing cardiac surgery. Extended cardioplegic perfusion significantly reduces energy turnover in hearts because the balance procedures are both volume- and above all time-dependent. A reduction in MVO2 indicates the necessity of a prolonged cardioplegic perfusion time to achieve optimized myocardial protection.

Transcatheter closure of patent foramen ovale (PFO) and secundum type atrial septal defect (ASD) are common transcatheter procedures. Although they share many technical details, these procedures are targeting two different clinical indications. PFO closure is usually considered to prevent recurrent embolic stroke/systemic arterial embolization, ASD closure is indicated in patients with large left-to-right shunt, right ventricular volume overload, and normal pulmonary vascular resistance. Multimodality imaging plays a key role for patient selection, periprocedural monitoring, and follow-up surveillance. In addition to routine cardiovascular examinations, advanced neuroimaging studies, transcranial-Doppler, and interventional transesophageal echocardiography/intracardiac echocardiography are now increasingly used to deliver safely and effectively such procedures. Long-standing collaboration between interventional cardiologist, neuroradiologist, and cardiac imager is essential and it requires a standardized approach to image acquisition and interpretation. Periprocedural monitoring should be performed by experienced operators with deep understanding of technical details of transcatheter intervention. This review summarizes the specific role of different imaging modalities for PFO and ASD transcatheter closure, describing important pre-procedural and intra-procedural details and providing examples of procedural pitfall and complications.

Background: There are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS). Methods: We performed a retrospective cohort study of 109 patients with pathogenic variants in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right-coronary artery (RCA) were measured by computed tomography angiography. Results: The overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68% in patients with a native aortic root (group 1) and patients with previous aortic-root replacement (group 2), respectively. Previous aortic dissection or aortic intervention, longer time from aortic-root replacement, higher systemic score, significant mitral valve involvement, and diffuse aortic disease were correlated with CAA. During a mean follow-up of 8.5 +/- 7.6 years, 4 patients developed pseudoaneurysms of the coronary anastomoses, requiring surgery. Conclusions: CAAs are common in adult patients with MFS and are associated with a more severe aortic phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates that coronary artery size should be regularly followed, mostly after aortic-root replacement and in patients with severe aortic phenotypes. Large multicentre studies are warranted to elucidate the most appropriate surveillance plan.

Abstract Clinical case A 53 years woman in good health and un uneventful clinical history except for a mild hypercholesteremia was evaluated for palpitations. At the clinical examination she had a systolic murmur 3/6 Levine, with no signs of heart failure. The ECG showed normal sinus rhythm with a normal heart rate (62bpm), normal atrio-ventricular and intraventricular conduction and normal repolarisation, one supraventricular premature beat. The echocardiography showed normal biventricular dimension and function, no valvular heart diseases, no septal defects, regular aortic dimensions. A giant right coronary was evidenced (Figure, panel a) with an arteriovenous fistula originating from the right coronary artery and draining through the coronary sinus into the right atrium (Figure, panel b). The CT coronary angiogram evidenced an dilated right coronary artery communicating with the coronary sinus (arteriovenous fistula) draining into the right atrium. A smaller arteriovenous fistula was evidenced between the circumflex artery (slightly dilated) and the great cardiac vein. (Figure, panel c-e) The Treadmill test didn’t evidence an induced ischemia; however the patient didn’t perform a maximal exercise (double product 20400mmHb*bpm). Moreover, during the first steps of recovery frequent supraventricular premature beats were registered with phases of bigeminies followed by a junctional rhythm phase. Thus, a Gated myocardial Perfusion SPECT was performed evidenced a mild stress induced ischemia of the inferolateral and apical left ventricle wall with normal rest perfusion and normal left ventricle volumes (125ml during exercise and 134ml at rest) with a normal ejection fraction ( > 65%). (Figure, panel f)An elective coronarography was planned. The patient is on therapy with beta-blockers and aspirin. The patient is asymptomatic for angina. Antibiotic prophylaxis was recommended for dental, gastrointestinal, or urologic procedures. Discussion: Coronary arteriovenous fistula (CAVF), first described in 1865 by Krausein (1), are a rare congenital heart disease representing less than 0.5% of all congenital heart diseases with an extremely rare prevalence 0.002% in the general population (2). Moreover, therapy of CAVF is still controversial with previous data showing a relatively high rate of myocardial infarction after surgical repair (3). The recent AHA/ACC guideline for the management of adults with Congenital heart disease recommend a review by a knowledgeable team that may include congenital or noncongenital cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical closure (4) Conclusion: CAVF is a very rare congenital heart defect and might be asymptomatic and evidenced by hazard in adults patients. Therapy strategy demands a multidisciplinary team evaluation and should be be individualized according to the clinic presentation, the presence or absence of myocardial ischemia or ventricular dysfunction. Abstract P642 Figure.

Background and Aim: To evaluate long term clinical outcomes and a cross-sectional quality of life study, in congenital patients after right ventricular outflow tract (RVOT) reconstruction with cryopreserved pulmonary homograft (HG). Methods: 110 patients (pts) undergone RVOT reconstruction with cryopreserved pulmonary HG from January 1995 to September 2014 were included in this retrospective review. Homograft's failure was diagnosed in presence of pulmonary valve peak gradient ≥50mmHg and/or moderate to severe degree of valve regurgitation. Mean diameter of HG implanted was 22 mm and donors’ mean age 39.1 ± 16.0years. Follow-up data included echocardiogram, cardiac MRI and clinical evaluation. The Short-Form-36 was presented to patients to assess perceived quality of life. Results: Mean age at surgery was 24.5 ± 15 years. Mean follow-up was10.1 ± 5 years. There was no perioperative mortality. At follow-up, 2 pts (1.8%) died due to non cardiac comorbidities. Homograft failure was observed in 6 pts (5,4%): 5 required percutaneous valve implantation, with a mean interval from the main procedure of 5.3 ± 2.1years and 1 pt required HG replacement after 7years for pseudoaneurysm formation. Freedom from reoperation was 92% and 84% at 5 and 15 years respectively. At follow-up reduction of RV dilatation was observed in 71%pts with ED-volume 98.5 ± 26.1 ml/mq, ES-volume 50.1 ± 16.1 ml/mq, RVEF50.7 ± 6.9% and LVEF59.2 ± 6.9%. HG initial diameter was not a significant prognostic factor for HG dysfunction. Conclusions: Cryopreserved pulmonary homograft represent a valid option in case of right ventricular outflow tract reconstruction, as it is associated with low failure rate and no mortality in the long term.

Assessment of longue term follow-up after surgical repair of Complete Atrioventricular Septal Defect (cAVSD). Evaluation of 3D versus standard 2D echocardiographic accuracy during the last follow-up. 43 patients were included in this study. Mean age at surgical repair was 7 months, 48.8% of patients were male; 67.4% had Down Syndrome. Mean time follow-up was 13 ± 6 years. During the last follow-up 2D and 3D echo evaluation were performed. All patients were alive and had a NYHA class I at the last follow-up. Survival free from new cardiac surgery at 13 years was 86%. During follow-up a progressive moderate to severe/severe mitral regurgitation (MR) was observed in 6 patients (16.3%) while 4 patients developed a progressive left ventricular outflow tract obstruction (LVOTO); overall 5 patients underwent new cardiac surgery (3 for MR and 2 for LVOTO). Echocardiographic evaluation showed: LV end diastolic volume 55.7 ± 13.0ml/m2, LV ejection fraction 64 ± 5%, left atrium volume 24.9 ± 13.9ml/m2, right ventricle systolic pression 21.0 ± 2.0 and a LVOT gradient 14.6 ± 16.4 mmHg. Mitral valve evaluation is evidenced in Table 1. Predictor factors of severe MR during follow-up were the cleft area and depth and the presence of anterior leaflet prolapse. Cohen’s kappa evidenced a good correlation between 2D and 3D VC evaluation with a higher sensibility of 3D evaluation to identify severe MR, data confirmed by the ROC curve analysis (Figure A and B). Clinical outcome after cAVSD surgical repair is excellent with a low rate of reintervention. 3D echo evaluation is more sensitive to identify both predictors and severity of MR ì. LAST Follow-up echocardiography 2D evaluation 3D evaluation Mitral Valve evaluation Vena contracta, mm 4.8 ± 2.5 5.3 ± 2.5 Regurgitant volume, ml 25 ± 20 ERO, cm2 0.17 ± 0.13 Mean gradient, mmHg 4 ± 2.4 Residual cleft, n(%) A1 A2 A3 30 (90.7) 4 (9.3) 33 (76.8) 6 (13.9) Cleft area,cm2 0.19 ± 0.21 Cleft depth, mm 5 .0 ± 2.9 Valve thickening, n(%) 14 (13.6) Leaflet Prolapse, n (%) Anterior Leaflet Prolapse, n (%) Posterior Leaflet Prolapse, n (%) 30 (69.7) 25 (58.1) 10 (23.2) Single papilary, n(%) 10 (23.2) Abstract P1599 Figure.

This study aimed to investigate the potential association between imaging features and cardiovascular outcomes in patients with Loeys–Dietz syndrome (LDS). We performed a retrospective cohort study of 36 patients with LDS and described cardiovascular events and imaging data. We observed different clinical courses in patients with LDS, irrespective of the causative gene. Angular or elongated aortic arch geometry correlated with aortic dissection (R = .39, p = .02), occurrence of the first cardiovascular event before 45 years of age (R = .36, p = .03), and the number of operations (R = 0.47, p = .004), but not with age (R = −.05, p = .79) or the causative gene (R = −0.04, p = .79). Relative absences of cardiovascular events at ages 20, 40, and 60 were 100, 75, and 56%, respectively, in patients with “romanesque” aortic arches, and 74, 39, and 21%, respectively, in patients with “gothic” and “elongated” aortic arches (p = .03). Angular or elongated aortic arch geometry is associated with early‐onset of disease and a worse cardiovascular outcome in LDS patients. Large multicenter studies are warranted to elucidate the impact of aortic arch morphology evaluation in clinical practice.

A 6-year-old African boy with multi-viral infection including parvovirus B19 and severe acute respiratory syndrome coronavirus 2 was admitted for persistent fever associated with respiratory distress and myocarditis complicated by cardiogenic shock needing ventilatory and inotropic support. Coronary aneurysms were also documented in the acute phase. Blood tests were suggestive of macrophage activation syndrome. He was treated with intravenous immunoglobulins, aspirin, diuretics, dexamethasone, hydroxychloroquine, and prophylactic low molecular weight heparin. Normalization of cardiac performance and coronary diameters was noticed within the first days. Cardiac magnetic resonance imaging, performed 20 days after the hospitalization, evidenced mild myocardial interstitial oedema with no focal necrosis, suggesting a mechanism of cardiac stunning related to cytokines storm rather than direct viral injury of cardiomyocytes.

Aim: The aim of the study was to assess predictors of outcome in patients hospitalized for dilated cardiomyopathy (DCM) and severe left ventricular dysfunction. Patients& methods: 83 pediatric patients hospitalized for heart failure due to DCM with coexistent left ventricular dysfunction were enrolled. Results: Overall, 5-year survival free from heart transplantation was 69.8%. Normalization of left ventricular function was achieved in 39.8% of patients during follow-up: younger age, less necessity of inotropic support and other than idiopathic DCM predicted left ventricular function, while familial history for cardiac disease or sudden death and inotropic support during hospitalization were associated with poorer outcome. Conclusion: Almost 40% of patients with DCM experienced a complete normalization of cardiac function. Outcome was extremely variable according to the type of DCM.