Showing papers by "Aurelio Secinaro published in 2022"

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

Abstract: Abstract Cardiovascular magnetic resonance (CMR) is widely used for diagnostic imaging in the pediatric population. In addition to structural congenital heart disease (CHD), for which published guidelines are available, CMR is also performed for non-structural pediatric heart disease, for which guidelines are not available. This article provides guidelines for the performance and reporting of CMR in the pediatric population for non-structural (“non-congenital”) heart disease, including cardiomyopathies, myocarditis, Kawasaki disease and systemic vasculitides, cardiac tumors, pericardial disease, pulmonary hypertension, heart transplant, and aortopathies. Given important differences in disease pathophysiology and clinical manifestations as well as unique technical challenges related to body size, heart rate, and sedation needs, these guidelines focus on optimization of the CMR examination in infants and children compared to adults. Disease states are discussed, including the goals of CMR examination, disease-specific protocols, and limitations and pitfalls, as well as newer techniques that remain under development.

Radiation safety for cardiovascular computed tomography imaging in paediatric cardiology: a joint expert consensus document of the EACVI, ESCR, AEPC, and ESPR.

Abstract: Children with congenital and acquired heart disease may be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical invasive and non-invasive imaging procedures. Although these imaging procedures are all essential to the care of these complex paediatric population and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of this manuscript is to provide a comprehensive review of radiation dose management and cardiac computed tomography performance in the paediatric population with congenital and acquired heart disease, to encourage informed imaging to achieve indication-appropriate study quality at the lowest achievable dose.

Cardiopulmonary Exercise Testing in Repaired Tetralogy of Fallot: Multiparametric Overview and Correlation with Cardiac Magnetic Resonance and Physical Activity Level

Abstract: Patients with repaired Tetralogy of Fallot (rToF) typically report having preserved subjective exercise tolerance. Chronic pulmonary regurgitation (PR) with varying degrees of right ventricular (RV) dilation as assessed by cardiac magnetic resonance imaging (MRI) is prevalent in rToF and may contribute to clinical compromise. Cardiopulmonary exercise testing (CPET) provides an objective assessment of functional capacity, and the International Physical Activity Questionnaire (IPAQ) can provide additional data on physical activity (PA) achieved. Our aim was to assess the association between CPET values, IPAQ measures, and MRI parameters. All rToF patients who had both an MRI and CPET performed within one year between March 2019 and June 2021 were selected. Clinical data were extracted from electronic records (including demographic, surgical history, New York Heart Association (NYHA) functional class, QRS duration, arrhythmia, MRI parameters, and CPET data). PA level, based on the IPAQ, was assessed at the time of CPET. Eighty-four patients (22.8 ± 8.4 years) showed a reduction in exercise capacity (median peak VO2 30 mL/kg/min (range 25–33); median percent predicted peak VO2 68% (range 61–78)). Peak VO2, correlated with biventricular stroke volumes (RVSV: β = 6.11 (95%CI, 2.38 to 9.85), p = 0.002; LVSV: β = 15.69 (95% CI 10.16 to 21.21), p < 0.0001) and LVEDVi (β = 8.74 (95%CI, 0.66 to 16.83), p = 0.04) on multivariate analysis adjusted for age, gender, and PA level. Other parameters which correlated with stroke volumes included oxygen uptake efficiency slope (OUES) (RVSV: β = 6.88 (95%CI, 1.93 to 11.84), p = 0.008; LVSV: β = 17.86 (95% CI 10.31 to 25.42), p < 0.0001) and peak O2 pulse (RVSV: β = 0.03 (95%CI, 0.01 to 0.05), p = 0.007; LVSV: β = 0.08 (95% CI 0.05 to 0.11), p < 0.0001). On multivariate analysis adjusted for age and gender, PA level correlated significantly with peak VO2/kg (β = 0.02, 95% CI 0.003 to 0.04; p = 0.019). We observed a reduction in objective exercise tolerance in rToF patients. Biventricular stroke volumes and LVEDVi were associated with peak VO2 irrespective of RV size. OUES and peak O2 pulse were also associated with biventricular stroke volumes. While PA level was associated with peak VO2, the incremental value of this parameter should be the focus of future studies.

COVID-19 associated multisystem inflammatory syndrome in a neonate with atypical coronary artery involvement.

Abstract: Objective The study aimed to report a COVID-19 associated multisystem inflammatory syndrome in children (MIS-C) in a neonate found to have an atypical diffuse thickening in coronary artery walls, whose diagnosis required a multi-imaging approach. Study Design A neonate presented at birth with multiple organ involvement and coronary artery anomalies. A diagnosis of MIS-C associated to COVID-19 was supported by maternal Sars-CoV-2 infection during pregnancy, and by the presence of both IgG against Sars-CoV-2 and Spike-specific memory B cells response in the neonatal blood. Other plausible causes of the multiple organ involvement were excluded. Result At admission, a severe coronary artery dilatation was identified on echocardiography, supporting the diagnosis of MIS-C Kawasaki-like disease; however, coronary artery internal diameters were found to be normal using cardiac computed tomography angiography. At discharge, comparing the two imaging techniques each other, the correct diagnosis resulted to be an abnormal thickening in coronary arterial walls. These findings suggest that the inflammatory process affecting the coronary arterial wall in MIS-C could result not only in typical coronary artery lesions such as dilatation of the lumen or aneurysms development, but also in abnormal thickening of the coronary artery wall. Conclusion. Our case provides an alert for paediatric cardiologists about the complexity to assess coronary artery involvement in MIS-C, and raises the question of whether an abnormal vascular remodeling, with normal inner diameters, is to be considered like coronary artery dilatation for risk stratification.

Crossed pulmonary arteries: An underestimated cardiovascular variant with a strong association with genetic syndromes—A report of 74 cases with systematic review of the literature

Abstract: Crossed pulmonary arteries (CPAs) represent an uncommon anatomic variant, usually associated with some specific syndromes and conotruncal defects. This finding has been described in 22q11.2 Deletion Syndrome (22q11.2DS). We evaluated the correlation between CPAs and genetic diseases, in order to better define the characteristics of this variant, considered a rare anatomic pattern. An in‐depth analysis of CPAs genotype–phenotype correlations was performed via a literature review. We detected 74 CPAs patients through echocardiography. Of these 74 patients, 35.1% of patients showed additional intracardiac malformations, while 29.7% showed extracardiac vascular anomalies, of which 16.2% were associated with intracardiac defects and 13.5% were not. In all, 62.2% of patients were diagnosed with genetic diseases and 52.2% of them were 22q11.2DS patients. In conclusions, CPAs represent a cardiovascular variant, which is detectable in nonsyndromic individuals, but especially in various genetic syndromes and in particular in 22q11.2DS patients. Data on the real prevalence of this morphology is lacking in literature. Knowledge of this anatomic variant is useful to interpret the unusual course of the pulmonary branches and is helpful information before cardiovascular surgical correction. Moreover, due to the strong association of CPAs with some genetic syndromes, the identification of this anatomic pattern can indicate the utility of a genetic assessment of these patients.

Higher Troponin Levels on Admission are associated With Persistent Cardiac Magnetic Resonance Lesions in Children Developing Myocarditis After mRNA-Based COVID-19 Vaccination

Abstract: Acute pericarditis/myocarditis is a rare complication of the mRNA-based vaccines and although mostly self-limiting, long-term sequelae remain unclear.We enrolled all patients admitted to the emergency department between September 2021 and February 2022 meeting the CDC work case definition, with symptoms onset after mRNA-based COVID-19 vaccine. Alternative virologic causes were excluded. Clinical data, laboratory values, cardiologic evaluation, electrocardiogram (ECG), and echocardiogram (ECHO) were collected on admission, at discharge, and during follow-up in all patients. Cardiac Magnetic Resonance (CMR) was performed only in those with signs consistent with myocarditis.We observed 13 patients (11M and 2F), median age 15 years, affected by acute pericarditis/myocarditis after COVID-19 mRNA vaccination (11 after Comirnaty® and 2 after Spikevax®). Symptoms'onset occurred at a median of 5 days (range, 1 to 41 days) after receiving mRNA vaccine (13 Prizer 2 Moderna): 4 patients (31%) after the 1st dose, 6 (46%) after the 2nd, and 3 (23%) after 3rd dose. Increased levels of high-sensitive troponin T (hsTnT) (median 519,5 ng/mL) and N-terminal-pro hormone BNP (NT-proBNP) (median 268 pg/mL) and pathognomonic ECG and ECHO abnormalities were detected. On admission, 7 of 13 (54%) presented with myopericarditis, 3 (23%) with myocarditis, and 3 (23%) with pericarditis; CMR was performed in 5 patients upon pediatric cardiologist prescription and findings were consistent with myocarditis. At 12 weeks of follow-up, all but one patient (92%), still presenting mild pericardial effusion at ECHO, were asymptomatic with normal hsTnT and NT-proBNP levels and ECG. On CMR 6 of 9 patients showed persistent, although decreased, myocardial injury. Higher hsTnT levels on admission significantly correlated with persistent CMR lesions.Evidence of persistent CMR lesions highlights the need for a close and standardized follow-up for those patients who present high hsTnT levels on admission.

Syndromic and Non-Syndromic Patients with Repaired Tetralogy of Fallot: Does It Affect the Long-Term Outcome?

Abstract: Background: The impact of genetic syndromes on cardiac magnetic resonance imaging (cMRI) parameters, particularly on right and/or left ventricular dysfunction, associated with clinical parameters following the repair of Tetralogy of Fallot (rToF) is not well known. Therefore, this study aimed to assess the differences in clinical, surgical, and cMRI data in syndromic and non-syndromic rToF patients. Methods: All syndromic rToF patients undergoing a cMRI without general anesthesia between 2010 and 2020 who were able to match with non-syndromic ones for birth date, sex, type of surgery, timing of cMRI, and BSA were selected. Demographic, clinical, surgical, MRI, ECG, and Holter ECG data were collected. Results: A total of one hundred and eight rToF patients equally subdivided into syndromic and non-syndromic, aged 18.7 ± 7.3 years, were studied. Del22q11.2 and Down syndrome (DS) were the most frequent syndromes (42.6% and 31.5%, respectively). Regarding the cMRI parameters considered, left ventricular (LV) dysfunction (LVEF < 50%) was more frequently found in syndromic patients (p = 0.040). In addition, they were older at repair (p = 0.002) but underwent earlier pulmonary valve replacement (PVR) (15.9 ± 5.6 vs. 19.5 ± 6.0 years, p = 0.049). On multivariate Cox regression analysis, adjusted for age at first repair, LV dysfunction remained significantly more associated with DS than del22q11.2 and non-syndromic patients (HR of 5.245; 95% CI 1.709–16.100, p = 0.004). There were only four episodes of non-sustained ventricular tachycardia in our cohort. Conclusions: Among the cMRI parameters commonly taken into consideration in rToF patients, LV dysfunction seemed to be the only one affected by the presence of a genetic syndrome. The percentage of patients performing PVR appears to be similar in both populations, although syndromic patients were older at repair and younger at PVR. Finally, the number of arrhythmic events in rToF patients seems to be low and unaffected by chromosomal abnormalities.

Higher Troponin Levels on Admission are associated With Persistent Cardiac Magnetic Resonance Lesions in Children Developing Myocarditis After mRNA-Based COVID-19 Vaccination

Abstract: Background: Acute pericarditis/myocarditis is a rare complication of the mRNA-based vaccines and although mostly self-limiting, long-term sequelae remain unclear. Methods: We enrolled all patients admitted to the emergency department between September 2021 and February 2022 meeting the CDC work case definition, with symptoms onset after mRNA-based COVID-19 vaccine. Alternative virologic causes were excluded. Clinical data, laboratory values, cardiologic evaluation, electrocardiogram (ECG), and echocardiogram (ECHO) were collected on admission, at discharge, and during follow-up in all patients. Cardiac Magnetic Resonance (CMR) was performed only in those with signs consistent with myocarditis. Results: We observed 13 patients (11M and 2F), median age 15 years, affected by acute pericarditis/myocarditis after COVID-19 mRNA vaccination (11 after Comirnaty® and 2 after Spikevax®). Symptoms’onset occurred at a median of 5 days (range, 1 to 41 days) after receiving mRNA vaccine (13 Prizer 2 Moderna): 4 patients (31%) after the 1st dose, 6 (46%) after the 2nd, and 3 (23%) after 3rd dose. Increased levels of high-sensitive troponin T (hsTnT) (median 519,5 ng/mL) and N-terminal-pro hormone BNP (NT-proBNP) (median 268 pg/mL) and pathognomonic ECG and ECHO abnormalities were detected. On admission, 7 of 13 (54%) presented with myopericarditis, 3 (23%) with myocarditis, and 3 (23%) with pericarditis; CMR was performed in 5 patients upon pediatric cardiologist prescription and findings were consistent with myocarditis. At 12 weeks of follow-up, all but one patient (92%), still presenting mild pericardial effusion at ECHO, were asymptomatic with normal hsTnT and NT-proBNP levels and ECG. On CMR 6 of 9 patients showed persistent, although decreased, myocardial injury. Higher hsTnT levels on admission significantly correlated with persistent CMR lesions. Conclusion: Evidence of persistent CMR lesions highlights the need for a close and standardized follow-up for those patients who present high hsTnT levels on admission.

Long-Term Cardiovascular Outcome in Children with MIS-C Linked to SARS-CoV-2 Infection—An Italian Multicenter Experience

Abstract: Simple Summary MIS-C is a multisystem inflammatory syndrome that is characterized by severe systemic signs of inflammation and multi-organ failure, including the involvement of the heart. Our study described the long-term cardiovascular outcome in a cohort of pediatric patients with MIS-C, who were admitted to two Italian Pediatric Referral Centers. The number of pediatric patients with an MIS-C diagnosis was 67. Among them, 65% of them had ventricular dysfunction, 66% of them had pericarditis, 35% of them had coronary involvement, and 9% of them showed arrhythmias. Our data described many cases of myocarditis and pericarditis, with mild coronary involvement and a few cases of arrhythmias. This experience showed that cardiac involvement in MIS-C patients is almost the rule, and that LV dysfunction and pericarditis are the most frequent manifestations of it. However, the patients’ clinical course was satisfactory, and during the controls, no additional events or sequelae were observed apart from long-term myocardial scars in 50% of the patients that underwent CMR. Early treatment not only provides a prompt full recovery, but it also has a probably “protective” effect towards late adverse events and long-term complications. Abstract MIS-C is a multisystem inflammatory syndrome that is characterized by multi-organ failure and cardiac involvement. The aim of this study was to describe the long-term cardiovascular outcome in a cohort of MIS-C pediatric patients, who were admitted to two Italian Pediatric Referral Centers. Sixty-seven patients (mean age 8.7 ± 4.7 years, male 60%) were included; 65 (97%) of them showed cardiac involvement. All of the patients completed one month of the follow-up, and 47% completed 1 year of it. ECG abnormalities were present in 65% of them, arrhythmias were present in 9% of them during an acute phase and it disappeared at the point of discharge or later. Pericarditis were detected in 66% of them and disappeared after 6 months. Coronaritis was observed in 35% of the children during an acute phase, and there were no more instances at the 1-year point. An LV dysfunction was present in 65% of the patients at the beginning of the study, with them having a full recovery at the point of discharge and thereafter. Elevated values of the NTproBNP and hsTp were initially detected, which progressively decreased and normalized at the points of discharge and FU. The CMR at the point of FU, there was a presence of long-term myocardial scars in 50% of the patients that were tested. No deaths that were caused by MIS-C during the FU were recorded. Cardiac involvement in MIS-C patients is almost the rule, but the patients’ clinical course was satisfactory, and no additional events or sequelae were observed apart from there being long-term myocardial scars in 50% of the patients that underwent CMR.

Compressed Sensing Cardiac Cine Imaging Compared with Standard Balanced Steady-State Free Precession Cine Imaging in a Pediatric Population.

Abstract: Purpose To compare real-time compressed sensing (CS) and standard balanced steady-state free precession (bSSFP) cardiac cine imaging in children. Materials and Methods Twenty children (mean age, 15 years ± 5 [SD], range, 7-21 years; 10 male participants) with biventricular congenital heart disease (n = 11) or cardiomyopathy (n = 9) were prospectively included. Examinations were performed with 1.5-T imagers by using both bSSFP and CS sequences in all participants. Quantification of ventricular volumes and function was performed for all images by two readers blinded to patient diagnosis and type of sequence. Values were correlated with phase-contrast flow measurements by one reader. Intra- and interreader agreement were analyzed. Results There were no significant differences between ventricular parameters measured on CS compared with those of bSSFP (P > .05) for reader 1. Only ejection fraction showed a significant difference (P = .02) for reader 2. Intrareader agreement was considerable for both sequences (bSSFP: mean difference range, +1 to -2.6; maximum CI, +7.9, -13; bias range, 0.1%-4.1%; intraclass correlation coefficient [ICC] range, 0.931-0.997. CS: mean difference range, +7.4 to -5.6; maximum CI, +37.2, -48.8; bias range, 0.5%-7.5%; ICC range, 0.717-0.997). Interreader agreement was acceptable but less robust, especially for CS (bSSFP: mean difference range, +2.6 to -5.6; maximum CI, +60.7, -65.3; bias range, 1.6%-6.2%; ICC range, 0.726-0.951. CS: mean difference range, +10.7 to -9.1; maximum CI, +87.5, -84.6; bias range, 1.1%-17.3%; ICC range, 0.509-0.849). The mean acquisition time was shorter for CS (20 seconds; range, 17-25 seconds) compared with that for bSSFP (160 seconds; range, 130-190 seconds) (P < .001). Conclusion CS cardiac cine imaging provided equivalent ventricular volume and function measurements with shorter acquisition times compared with those of bSSFP and may prove suitable for the pediatric population.Keywords: Compressed Sensing, Balanced Steady-State Free Precession, Cine Imaging, Cardiovascular MRI, Pediatrics, Cardiac, Heart, Cardiomyopathies, Congenital, Segmentation© RSNA, 2022.

Cardiac Imaging in Patients After Fontan Palliation: Which Test and When?

Abstract: The Fontan operation represents the final stage of a series of palliative surgical procedures for children born with complex congenital heart disease, where a “usual” biventricular physiology cannot be restored. The palliation results in the direct connection of the systemic venous returns to the pulmonary arterial circulation without an interposed ventricle. In this unique physiology, systemic venous hypertension and intrathoracic pressures changes due to respiratory mechanics play the main role for propelling blood through the pulmonary vasculature. Although the Fontan operation has dramatically improved survival in patients with a single ventricle congenital heart disease, significant morbidity is still a concern. Patients with Fontan physiology are in fact suffering from a multitude of complications mainly due to the increased systemic venous pressure. Consequently, these patients need close clinical and imaging monitoring, where cardiac exams play a key role. In this article, we review the main cardiac imaging modalities available, summarizing their main strengths and limitations in this peculiar setting. The main purpose is to provide a practical approach for all clinicians involved in the care of these patients, even for those less experienced in cardiac imaging.

Cardiomyopathy Due to Propionic Acidemia: Clinical Features and Therapeutic Implication

Abstract: Purpose Propionic acidemia (PA) is an organic acidemia caused by an inherited deficiency of the enzyme Propionyl CoA carboxylase. Cardiac involvement as QTc interval prolongation and heart muscle disease, typically as dilated cardiomyopathy, is known. To date, the characteristics of cardiomyopathy (CMP) and its treatment are poorly understood. Aims: define cardiovascular (CV) profile in patients with PA and response to CV therapy. Methods Single center retrospective analysis of patients with PA followed at Bambino Gesù Children's Hospital in Rome. Each patient's clinical, CV data (ECG, echocardiogram, cardiac magnetic resonance [MRI], NTproBNP, troponinT) and CV therapy were collected: at the first CV contact, at CMP onset, after 1 year and at last follow-up. Death, CV death, CV involvement, liver transplantation (LT) were considered as clinical outcomes. Results 26 patients (20 M) were included. Mean age at PA diagnosis was 83 days and at first CV contact was 8.48 ± 6.4 yrs. Over the time, 42% reported CV manifestation (38.5% CMP), 23% underwent LT, 46% died. Mean age at death was 8 ± 8 years. Mean age at CMP onset was 12 ± 6 years. 1 patient experienced sudden aborted cardiac death (SCD - defibrillated VF) at the age of 7. QTc interval was increased (>460ms) in the 43% (mean QTc 452 ms±43 ms). 8 cardiac MRIs were performed in 7 pts. MRI data showed LV dilation and eccentric remodelling: average indexed telediastolic volume 107 ml/m2; average indexed LV mass 71 g/m2. In no case were significant areas of late myocardial enhancement after contrast medium infusion. Therapeutically, 100% of patients followed for CMP were treated with therapeutic dose of ACE inhibitors, 70% with beta blocker, 80% with antialdosteronic agents, and 30% with ivabradine. After 1 year of treatment, LVEF increased from a mean value of 35.9% (± 11) to 52% (± 2.7; p< 0.05). 3 patients underwent LT for severe LV disfunction. They were all treated with maximal CV therapy before LT. At last follow up, 12 mos from LT for the first patient and 18 mos for the second, LVEF reached 47% and 54% from 35% at baseline. The third patient died at 3 months from LT for pneumonia. Conclusion CMP and SCD affect the prognosis of PA, especially in patients without metabolic decompensation. CV therapy can improve cardiac function. LT can be considered also in case of severe impaired LV function, after heart failure treatment has been optimized. Propionic acidemia (PA) is an organic acidemia caused by an inherited deficiency of the enzyme Propionyl CoA carboxylase. Cardiac involvement as QTc interval prolongation and heart muscle disease, typically as dilated cardiomyopathy, is known. To date, the characteristics of cardiomyopathy (CMP) and its treatment are poorly understood. Aims: define cardiovascular (CV) profile in patients with PA and response to CV therapy. Single center retrospective analysis of patients with PA followed at Bambino Gesù Children's Hospital in Rome. Each patient's clinical, CV data (ECG, echocardiogram, cardiac magnetic resonance [MRI], NTproBNP, troponinT) and CV therapy were collected: at the first CV contact, at CMP onset, after 1 year and at last follow-up. Death, CV death, CV involvement, liver transplantation (LT) were considered as clinical outcomes. 26 patients (20 M) were included. Mean age at PA diagnosis was 83 days and at first CV contact was 8.48 ± 6.4 yrs. Over the time, 42% reported CV manifestation (38.5% CMP), 23% underwent LT, 46% died. Mean age at death was 8 ± 8 years. Mean age at CMP onset was 12 ± 6 years. 1 patient experienced sudden aborted cardiac death (SCD - defibrillated VF) at the age of 7. QTc interval was increased (>460ms) in the 43% (mean QTc 452 ms±43 ms). 8 cardiac MRIs were performed in 7 pts. MRI data showed LV dilation and eccentric remodelling: average indexed telediastolic volume 107 ml/m2; average indexed LV mass 71 g/m2. In no case were significant areas of late myocardial enhancement after contrast medium infusion. Therapeutically, 100% of patients followed for CMP were treated with therapeutic dose of ACE inhibitors, 70% with beta blocker, 80% with antialdosteronic agents, and 30% with ivabradine. After 1 year of treatment, LVEF increased from a mean value of 35.9% (± 11) to 52% (± 2.7; p< 0.05). 3 patients underwent LT for severe LV disfunction. They were all treated with maximal CV therapy before LT. At last follow up, 12 mos from LT for the first patient and 18 mos for the second, LVEF reached 47% and 54% from 35% at baseline. The third patient died at 3 months from LT for pneumonia. CMP and SCD affect the prognosis of PA, especially in patients without metabolic decompensation. CV therapy can improve cardiac function. LT can be considered also in case of severe impaired LV function, after heart failure treatment has been optimized.

New Onset Cardiac Murmur and Exertional Dyspnea in an Apparently Healthy Child: A Rare Localization of Obstructive Myxoma in the Right Ventricle Outflow Tract without Pulmonary Embolization—A Case Report and Literature Review

Abstract: Myxomas are slowly growing benign neoplasms which are rare in children. Up to 80% can be located in the left atrium and generate symptoms such as embolism, cardiac failure, fever and weight loss. Rarely, myxomas can be detected in the right ventricle outflow tract, causing arrhythmias, pulmonary emboli and sudden death. We report the case of a 13-year-old healthy child brought to the Emergency Department (ED) of the Children’s Hospital Bambino Gesù, Rome, for recent dyspnea, chest pain on exertion and new onset cardiac murmur. Patient underwent medical examination and echocardiogram with the finding of a rounded and lobulated voluminous mass in the right ventricle outflow tract (RVOT) which caused severe obstruction. The contrast computed tomography (CT) scan confirmed the presence of a heterogeneously enhancing soft-tissue mass occupying the RVOT with no evidence of pulmonary embolization. The mass was surgically excised, and the pathologic examination confirmed our suspicion of myxoma. Our experience suggests that myxoma can have mild clinical symptoms, the presentation may be non-specific, and diagnosis can be a challenge Careful examination and a diagnostic imaging workup, primarily with the transthoracic echocardiogram, are needful to make a rapid differential diagnosis and to better manage surgical treatment and follow-up.

255 impact of severe valvular heart disease in adult congenital heart disease patients

Abstract: The clinical impact of valvular heart disease (VHD) in adult congenital heart disease (ACHD) patients is unascertained. Aim of our study was to assess the prevalence and clinical impact of severe VHD (S-VHD) in a real-world contemporary cohort of ACHD patients. Patients followed-up at our ACHD Outpatient Clinic were enrolled. Clinical characteristics and echocardiographic data were prospectively entered into a digitalized medical records database. VHD at the first access was evaluated and graded according to VHD guidelines. Clinical data at follow-up were collected. A total of 390 patients were included and S-VHD was present in 101 (25.9%) patients. Over a median follow-up time of 26 months (IQR: 12-48), the primary composite endpoint occurred in 76 patients (19.5%). The cumulative primary endpoint-free survival was significantly lower in patients with S-VHD vs patients with non-severe VHD (Log rank p <0.001). At multivariable analysis, age and atrial fibrillation at first visit (p=0.029 and p=0.006 respectively), lower %Sat O2, higher NYHA class (p=0.005 for both), lower LVEF (p=0.008) and S-VHD (p=0.015) were independently associated to the primary endpoint. The likelihood ratio test demonstrated that S-VHD added significant prognostic value (p=0.017) to a multivariate model including age, severe CHD, atrial fibrillation, Sat O2, NYHA, LVEF and right ventricle systolic pressure>45 mmHg. In ACHD patients, the presence of S-VHD is independently associated with the occurrence of cardiovascular mortality and hospitalization. The prognostic value of S-VHD is incremental above other established prognostic markers.

Successful staged separation of total angular craniopagus.

Abstract: BACKGROUND Total craniopagus is an exceedingly rare condition in which surgical treatment is complex and potentially fatal. Over the last decades, a multistep surgical approach, which allows development of venous collateral circulation, has fostered a dramatic improvement of successful separation rates and neurological outcomes. Most of the experience derives from management of vertical craniopagus, the angular form being rarer and less amenable to successful surgical separation. METHODS We present a case of total angular craniopagus twins observed at our Institution. Specific features included a large occipital fusion area with a bone defect, complete separation of brain and arterial vessels and a complex configuration of dural venous sinuses. The superior sagittal sinus of each twin preferentially drained to a single transverse sinus through a shared torcular. RESULTS After an extensive diagnostic phase, including neuroimaging, tridimensional and virtual reality modelling, neurological, neurophysiological and rehabilitation assessment, a detailed multistep surgical plan, was proposed to a wide multidisciplinary team. The venous system was managed by taking advantage of the fact each twin's superior sagittal sinus was drained preferentially by the transverse sinus on the twin's left. The transverse sinuses were thus separated accordingly. CONCLUSIONS Successful separation was achieved in three surgical steps over one year, with an excellent outcome for both twins.

Right ventricle-pulmonary artery coupling in repaired tetralogy of fallot with pulmonary regurgitation: clinical implications

Abstract: 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

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Abstract: Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure. Regardless of the strategy, outcomes have altered the natural history of the disease, with a complete repair rate of approximately 80% and low early and late mortality rates. Given this heterogeneity of pulmonary vasculature, a tailored approach should be adopted for each patient, using all diagnostic methods currently offered by technical developments.

Impact of severe valvular heart disease in adult congenital heart disease patients

Abstract: Background The clinical impact of valvular heart disease (VHD) in adult congenital heart disease (ACHD) patients is unascertained. Aim of our study was to assess the prevalence and clinical impact of severe VHD (S-VHD) in a real-world contemporary cohort of ACHD patients. Materials and methods Consecutive patients followed-up at our ACHD Outpatient Clinic from September 2014 to February 2021 were enrolled. Clinical characteristics and echocardiographic data were prospectively entered into a digitalized medical records database. VHD at the first evaluation was assessed and graded according to VHD guidelines. Clinical data at follow-up were collected. The study endpoint was the occurrence of cardiac mortality and/or unplanned cardiac hospitalization during follow-up. Results A total of 390 patients (median age 34 years, 49% males) were included and S-VHD was present in 101 (25.9%) patients. Over a median follow-up time of 26 months (IQR: 12–48), the study composite endpoint occurred in 76 patients (19.5%). The cumulative endpoint-free survival was significantly lower in patients with S-VHD vs. patients with non-severe VHD (Log rank p < 0.001). At multivariable analysis, age and atrial fibrillation at first visit (p = 0.029 and p = 0.006 respectively), lower %Sat O2, higher NYHA class (p = 0.005 for both), lower LVEF (p = 0.008), and S-VHD (p = 0.015) were independently associated to the study endpoint. The likelihood ratio test demonstrated that S-VHD added significant prognostic value (p = 0.017) to a multivariate model including age, severe CHD, atrial fibrillation, %Sat O2, NYHA, LVEF, and right ventricle systolic pressure > 45 mmHg. Conclusion In ACHD patients, the presence of S-VHD is independently associated with the occurrence of cardiovascular mortality and hospitalization. The prognostic value of S-VHD is incremental above other established prognostic markers.