Mild Ventricular Dilatation Research Articles

Cardiac MRI Predictors of ArrhythmicSudden Cardiac Events in Patients With Fontan Circulation.

Among patients with congenital heart disease, those with single ventricles have the highest risk of early mortality. Sudden cardiac death is an important cause of death in this population. Understanding the risk factors for sudden cardiac events (SCE) in Fontan patients could improve prediction and prevention. The goal of this study was to determine the prevalence of SCE and risk factors for SCE in the Fontan population. The Fontan Outcomes Registry Using CMR Examinations (FORCE) is an international registry collecting clinical and imaging data on Fontan patients. SCE was defined as: 1) cardiac arrest from a shockable rhythm; 2) need for emergent cardioversion/defibrillation; or 3) documented sustained ventricular tachycardia. Univariate and multivariate Cox proportional hazards regression models estimated hazard ratios for predictors of SCE. Our sample included 3,132 patients (41% female). The median age at first cardiac magnetic resonance was 14.6 years. SCE was experienced by 3.5% (n=109) over a median follow-up time of 4.00 years. Of the 109 patients with SCE, 39 (36%) died. On multivariable analysis, NYHA functional class >II (HR: 4.91; P< 0.0001), history of protein-losing enteropathy/plastic bronchitis (HR: 2.37; P=0.0082), single-ventricle end-diastolic volume index >104mL/m2 (HR: 3.15; P< 0.0001), and ejection fraction<50% (HR: 1.73; P=0.0437) were associated with SCE. Kaplan-Meier analysis demonstrated that in patients with none of the above risk factors, the 4-year freedom from SCE was 99.5%. SCE occurred in 3.5% of the study population, and one-third of patients who experienced SCE died. Mild ventricular dysfunction and dilatation by cardiac magnetic resonance, NYHA functional class, and history of protein-losing enteropathy/plastic bronchitis were associated with SCE.

A Barth Syndrome Patient-Derived D75H Point Mutation in TAFAZZIN Drives Progressive Cardiomyopathy in Mice.

Cardiomyopathy is the predominant defect in Barth syndrome (BTHS) and is caused by a mutation of the X-linked Tafazzin (TAZ) gene, which encodes an enzyme responsible for remodeling mitochondrial cardiolipin. Despite the known importance of mitochondrial dysfunction in BTHS, how specific TAZ mutations cause diverse BTHS heart phenotypes remains poorly understood. We generated a patient-tailored CRISPR/Cas9 knock-in mouse allele (TazPM) that phenocopies BTHS clinical traits. As TazPM males express a stable mutant protein, we assessed cardiac metabolic dysfunction and mitochondrial changes and identified temporally altered cardioprotective signaling effectors. Specifically, juvenile TazPM males exhibit mild left ventricular dilation in systole but have unaltered fatty acid/amino acid metabolism and normal adenosine triphosphate (ATP). This occurs in concert with a hyperactive p53 pathway, elevation of cardioprotective antioxidant pathways, and induced autophagy-mediated early senescence in juvenile TazPM hearts. However, adult TazPM males exhibit chronic heart failure with reduced growth and ejection fraction, cardiac fibrosis, reduced ATP, and suppressed fatty acid/amino acid metabolism. This biphasic changeover from a mild-to-severe heart phenotype coincides with p53 suppression, downregulation of cardioprotective antioxidant pathways, and the onset of terminal senescence in adult TazPM hearts. Herein, we report a BTHS genotype/phenotype correlation and reveal that absent Taz acyltransferase function is sufficient to drive progressive cardiomyopathy.

MYOCARDITIS: VIRUS OR DESMOSOME?

Abstract Patient Profile Female, 56 yo, arterial hypertension, FH of autoimmune diseases, no history of sudden death. Medical History: • At the age of 46: Hospitalized for chest pain. Coronary angiography: negative. Diagnosis: myocardial ischemia with normal coronary arteries. • 2018: Hospitalized again for angina. ECG showed low voltages, flat T–waves in the inf–lat. leads. Coronary angiography: negative. Biopsy Mild hypertrophy and cytoplasmic vacuolization, minimal increase in interstitial fibrosis, no necrosis or inflammatory infiltrates. Mild nonspecific cardiomyopathy. Viral DNA test positive for HHV–6. Serology: HHV–6 in blood (neg), IgG HHV–6 (pos), IgM (neg). Diagnosis: myocarditis due to HHV–6. • San Camillo Cardiomyopathy Center: Echocardiogram: basal ant–lat. hypokinesia, EF 60%. Therapy: Bisoprolol 3.75 mg, Ramipril 5 mg, Ranolazine 1000 mg. MRI: basal transmural ant–lat. and subepicardial lateral LGE. Oedema and hypokinesia coexists. Subacute myocarditis. MRI at 1 year: mild left ventricular dilation, EF 60%, meso–subepicardial LGE in the ant–lat and inf mid–basal regions without oedema. Serial Holter ECGs: frequent polymorphic ventricular ectopics, couplets, and runs. CPET: normal exercise capacity, pVO2 19.4 (ml/min)/kg (90%). Genetic testing VUS in DSC, probably pathogenic, and VUS in ABCC9, PKP2, DSP (2 mutations) with uncertain clinical significance. • In 2022: Readmitted for angina. Elevated Troponin levels. Coro CT angiography: negative. MRI: EF 45%, akinesia of the apex and apical lat. wall; diffuse LGE and edema. Pericardial effusion. Patient refused biopsy. Discharged after recovery (EF 52%) with the diagnosis of recurrent myocarditis. Empagliflozin added. • Follow up. ECG: progression of repolarization abnormalities (TWI in V1–V3 + inf.–lat. leads). Biopsy: fibroadipose replacement, no necrosis or inflammatory infiltrates. Chronic myocarditis and sequels. Viral tests: negative. CPET and stress echo: VO2peak 20.7 (ml/kg)/min (88%), normal work capacity. Very rare monomorphic PVCs. EF 50%, preserved contractile reserve. Conclusions Diagnosis of left dominant arrhythmogenic cardiomyopathy with a typical pattern for hot phases of myocardial inflammation. The first episode could have been triggered by a viral infection on a predisposing genetic substrate. Nadolol 40 mg x2 was introduced. The possibility of ICD implantation was discussed, and patient refused. A six–month follow–up was planned. Family screening results were negative.

Pulmonary Artery Aneurysm Associated with Sarcoidosis in a 75-year-old with Heart Failure: The Challenges of Diagnosis and Management.

Pulmonary artery aneurysm (PAA) is a rare abnormality of pulmonary vasculature. It can be idiopathic or secondary to various pathologies, frequently with multiple factors leading to its formation. We report the case of a man with concomitant sarcoidosis and PAA. A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis. PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure. Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.

Pediatric ECG-Based Deep Learning to Predict Left Ventricular Dysfunction and Remodeling.

Artificial intelligence-enhanced ECG analysis shows promise to detect ventricular dysfunction and remodeling in adult populations. However, its application to pediatric populations remains underexplored. A convolutional neural network was trained on paired ECG-echocardiograms (≤2 days apart) from patients ≤18 years of age without major congenital heart disease to detect human expert-classified greater than mild left ventricular (LV) dysfunction, hypertrophy, and dilation (individually and as a composite outcome). Model performance was evaluated on single ECG-echocardiogram pairs per patient at Boston Children's Hospital and externally at Mount Sinai Hospital using area under the receiver operating characteristic curve (AUROC) and area under the precision-recall curve (AUPRC). The training cohort comprised 92 377 ECG-echocardiogram pairs (46 261 patients; median age, 8.2 years). Test groups included internal testing (12 631 patients; median age, 8.8 years; 4.6% composite outcomes), emergency department (2830 patients; median age, 7.7 years; 10.0% composite outcomes), and external validation (5088 patients; median age, 4.3 years; 6.1% composite outcomes) cohorts. Model performance was similar on internal test and emergency department cohorts, with model predictions of LV hypertrophy outperforming the pediatric cardiologist expert benchmark. Adding age and sex to the model added no benefit to model performance. When using quantitative outcome cutoffs, model performance was similar between internal testing (composite outcome: AUROC, 0.88, AUPRC, 0.43; LV dysfunction: AUROC, 0.92, AUPRC, 0.23; LV hypertrophy: AUROC, 0.88, AUPRC, 0.28; LV dilation: AUROC, 0.91, AUPRC, 0.47) and external validation (composite outcome: AUROC, 0.86, AUPRC, 0.39; LV dysfunction: AUROC, 0.94, AUPRC, 0.32; LV hypertrophy: AUROC, 0.84, AUPRC, 0.25; LV dilation: AUROC, 0.87, AUPRC, 0.33), with composite outcome negative predictive values of 99.0% and 99.2%, respectively. Saliency mapping highlighted ECG components that influenced model predictions (precordial QRS complexes for all outcomes; T waves for LV dysfunction). High-risk ECG features include lateral T-wave inversion (LV dysfunction), deep S waves in V1 and V2 and tall R waves in V6 (LV hypertrophy), and tall R waves in V4 through V6 (LV dilation). This externally validated algorithm shows promise to inexpensively screen for LV dysfunction and remodeling in children, which may facilitate improved access to care by democratizing the expertise of pediatric cardiologists.

The effects of daily dose of intense exercise on cardiac responses and atrial fibrillation.

Atrial fibrillation (AF) is a supraventricular tachyarrhythmia that is strongly associated with cardiovascular (CV) disease and sedentary lifestyles. Despite the benefits of exercise on overall health, AF incidence in high-level endurance athletes rivals that of CV disease patients, suggesting a J-shaped relationship with AF. To investigate the dependence of AF vulnerability on exercise, we varied daily swim durations (120, 180 or 240minday-1 ) in 7-week-old male CD1mice. We assessed mice after performing equivalent amounts of cumulative work during swimming (i.e. ∼700L O2 kg-1 ), as determined from O2 consumption rates ( ). The mean during exercise increased progressively throughout the training period and was indistinguishable between the swim groups. Consistent with similar improvements in aerobic conditioning induced by swimming, skeletal muscle mitochondria content increased (P=0.027) indistinguishably between exercise groups. Physiological ventricular remodelling, characterized by mild hypertrophy and left ventricular dilatation, was also similar between exercised mice without evidence of ventricular arrhythmia inducibility. By contrast, prolongation of daily swim durations caused progressive and vagal-dependent heart rate reductions (P=0.008), as well as increased (P=0.005) AF vulnerability. As expected, vagal inhibition prolonged (P=0.013) atrial refractoriness, leading to reduced AF vulnerability, although still inducible in the 180 and 240min swim groups. Accordingly, daily swim dose progressively increased atrial hypertrophy (P=0.003), fibrosis (P<0.001) and macrophage accumulation (P=0.006) without differentially affecting the ventricular tissue properties. Thus, increasing daily exercise duration drives progressively adverse atrial-specific remodelling and vagal-dependent AF vulnerability despite robust and beneficial aerobic conditioning and physiological remodelling of ventricles and skeletal muscle. KEY POINTS: Previous studies have suggested that a J-shaped dose-response relationship exists between physical activity and cardiovascular health outcomes, with moderate exercise providing protection against many cardiovascular disease conditions, whereas chronic endurance exercise can promote atrial fibrillation (AF). We found that AF vulnerability increased alongside elevated atrial hypertrophy, fibrosis and inflammation as daily swim exercise durations in mice were prolonged (i.e. ≥180minday-1 for 6weeks). The MET-hweek-1 (based on O2 measurements during swimming) needed to induce increased AF vulnerability mirrored the levels linked to AF in athletes. These adverse atria effects associated with excessive daily exercise occurred despite improved aerobic conditioning, skeletal muscle adaptation and physiological ventricular remodelling. We suggest that atrial-specific changes observed with exercise arise from excessive elevations in venous filling pressures during prolonged exercise bouts, which we argue has implications for all AF patients because elevated atrial pressures occur in most cardiovascular disease conditions as well as ageing which are linked to AF.

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed. Postoperative adverse events included paravalvular or transvalvular leak, endocarditis, explant, thromboembolism, valve thrombosis, valve-related bleeding, hemolysis, and structural valve degeneration. Progression of valve gradients was assessed from discharge to 30 days and one year. Of 24 patients with median age of 26 years (interquartile range [IQR]: 17-33; range: 4-60 years), 22 (91.7%) patients had previously undergone tetralogy of Fallot repair and 2 (8.3%) patients had undergone double-outlet right ventricle repair in the neonatal period or infancy. All patients had at least mild right ventricular (RV) dilatation (median RV end-diastolic volume index 161.4, IQR: 152.3-183.5 mL/m2) and at least moderate pulmonary insufficiency (95.8%) or stenosis (8.3%). Median cardiopulmonary bypass and cross-clamp times were 71 (IQR: 63-101) min and 66 (IQR: 60-114) min, respectively. At a median postoperative follow-up of 2.5 years (IQR: 1.4-2.6; range: 1.0-3.0 years), there were no mortalities, valve-related reoperations, or adverse events. Postoperative valve gradients and the severity of pulmonary regurgitation did not change significantly over time. At short-term follow-up, the bioprosthesis in this study demonstrated excellent safety and effectiveness for PVR. Further studies with longer follow-up are warranted.

Abstract 18941: Pediatric Electrocardiogram-Based Deep Learning to Predict Left Ventricular Dysfunction and Remodeling

Background: Artificial intelligence-enhanced ECG algorithms show promise to detect a range of adult phenotypes, inclusive of ventricular dysfunction and remodeling. However, it remains unclear whether similar approaches provide meaningful and generalizable predictions in children. Methods: We trained a convolutional neural network on paired ECG-echos (≤ 2 days apart) from patients ≤ 18 years old without major congenital heart disease to detect human expert classified greater than mild left ventricular (LV) dysfunction, hypertrophy, and dilation (individually and as a composite outcome). Model performance was assessed by calculating area under the receiver operating curve (AUROC) in an internal test set and external setting (emergency department). Results: The main cohort comprised of 119,787 ECG-echo pairs (61,722 patients; median age 8.3 [IQR, 2.9-13.8] years; 55% male). Training, internal test, and external test sets comprised of 92,377, 24,343, and 3,067 ECG-echo pairs with 8.2%, 7.7%, and 10.0% composite outcome rates, respectively. During internal testing, AUROCs of 0.84 [95% CI, 0.83-0.85], 0.87 [95% CI, 0.85-0.88], 0.86 [95% CI, 0.84-0.87], and 0.84 [95% CI, 0.83-0.86] were achieved for the LV composite outcome, dysfunction, hypertrophy, and dilation, respectively (Figure). During external testing, AUROCs of 0.80 [95% CI, 0.77-0.83], 0.84 [95% CI, 0.80-0.88], 0.77 [95% CI, 0.72-0.82], and 0.86 [95% CI, 0.82-0.89] were achieved, respectively (Figure). Similar model performance was achieved when using quantitative z-score cutoffs for each outcome. Exploratory saliency mapping provides insight into notable ECG components that influence model predictions. Conclusions: Our artificial intelligence-enhanced pediatric ECG algorithm shows promise to inexpensively screen for and diagnose LV dysfunction or remodeling in children. Prospective trials to guide model implementation for supporting clinical decision making are warranted.

Association of right atrial strain and long-term outcome in severe secondary tricuspid regurgitation

ObjectiveSevere secondary tricuspid regurgitation (STR) causes significant right atrial (RA) volume overload, resulting in structural and functional RA-remodelling. This study evaluated whether patients with severe STR and reduced RA function,...

FRI244 Autoimmune Polyglandular Syndrome Type 2 And Peripartum Cardiomyopathy: A Case Report

Abstract Disclosure: A.G. Jimenez: None. J.D. Christensen: None. P. Surampudi: None. S. Phatak: None. J. Yoon: None. Background: Peripartum cardiomyopathy (PPCM) is a diagnosis of exclusion with evolving understanding of pathophysiology. There is limited consideration of the autoimmune processes contributing to PPCM. Case Presentation: A 20-year-old female had a recent delivery complicated by placenta previa, hemorrhage requiring transfusion, and pelvic infection. She presented 2 weeks postpartum with altered mental status and abdominal pain, found to be in septic shock, acute respiratory failure, acute liver injury initially thought to be secondary to retained products of conception. Subsequently, she was diagnosed with moderate-to-severe PPCM as she developed signs of heart failure, low ejection fraction &amp;lt;35%, mild ventricular dilatation, cardiogenic shock, hemorrhagic pericardial effusion with tamponade. She had no other prior cardiovascular history. She was briefly started on bromocriptine for treatment of suspected prolactin-mediated PPCM, in addition to digoxin, as her blood pressure was unable to sustain other treatments for PPCM. However, she hemodynamically decompensated and required persistent pressor support, leading to evaluation for other causes. Due to her prior history of hypothyroidism, an adrenal evaluation with a cosyntropin stimulation test was done. It demonstrated an undetectable cortisol at 0, 30, 60 minutes, with an elevated ACTH (1256, n=7-63 ng/dL), consistent with primary adrenal insufficiency (PAI). Workup for PAI did not reveal any infectious, infiltrative, vascular, pharmacological, or neoplastic etiology. Lab workup for concomitant hypophysitis was significant for low IGF-1 (Z-score -5.8, n= -2.0 to +2.0), but otherwise had expected elevated prolactin (121 ng/mL, n=5.2-26.5 ng/mL), suppressed LH (0.3 mIU/mL) and FSH (2.4 mIU/mL), and normal TSH (3.87 mIU/mL, n=0.27-4.20 mIU/mL) on levothyroxine 100 mcg. She had normal parathyroid hormone and hemoglobin A1c levels with no history of chronic candidiasis. Further workup showed positive antibodies to adrenal 21-hydroxylase enzyme, thyroid peroxidase, and glutamic acid decarboxylase 65, suggestive of autoimmune polyglandular syndrome (APS) type 2. She was started on glucocorticoids and mineralocorticoids for adrenal insufficiency resulting in a significant clinical improvement. She was subsequently discharged in stable condition. Conclusion: Due to increased rates of mortality in PPCM (4-11%), thought should be given to nontraditional etiologies of cardiac failure. Although rare, adrenal insufficiency must be considered in those with moderate-to-severe PPCM who continue to decompensate despite treatments. Untreated adrenal insufficiency can result in fatal consequences if not immediately treated. APS type 2 should be considered even in those without any confirmed family history of autoimmune disease. Those with autoimmune tendencies may be at higher risk of PPCM. Presentation: Friday, June 16, 2023

Utility of Follow-Up Echocardiograms in Uncomplicated Surgical Secundum Atrial Septal Defect Closures: Preliminary Analysis.

Though less common in the current era, surgical closure of secundum atrial septal defects (ASD2) is still performed in certain clinical situations. Guidelines currently recommend lifelong follow-up with transthoracic echocardiograms (TTE) for patients who have undergone a surgical ASD2 closure. The goal of this study was to determine the utility of follow-up TTE in patients who underwent an uncomplicated ASD2 closure. Chart review was performed on patients who had a surgical ASD2 closure between April 1, 1996, and August 30, 2021. Patients were excluded if they had other congenital heart disease, had a diagnosis of a residual ASD2, atrial/ventricular arrhythmias, pulmonary hypertension, heart failure, or did not have a follow-up TTE > 6months after the procedure. The most recent TTEs and clinic notes were evaluated. A total of 30 patients met the criteria. The median age at ASD2 surgery was 4.0years (IQ; 1.9-10.5). ASD2 was closed via patch repair in 16 patients and primarily closed in 14 patients. The most recent TTE was performed a median of 9.5years (IQ; 4.0, 14.7) after ASD2 closure. Two patients had mild right atrial and ventricular dilation, one patient had mild right atrial dilation, and one patient had mild right ventricular dilation. All other patients had qualitatively normal right-sided chamber sizes. All patients had normal biventricular function (left ventricular fractional shortening (median 36% (IQ; 33, 42)), no evidence of residual atrial shunts, and no evidence of pulmonary hypertension. No patient was on any cardiac medications at last clinic visit. Four patients were discharged from cardiology clinic and 10 patients were lost to follow-up. There were no deaths. Twenty-four patients had 46 repeat echocardiograms > 1year after ASD2 with no change in clinical management. In patients who underwent an uncomplicated ASD2 closure, there were no significant abnormalities noted on follow-up TTEs. The need for repeat lifetime TTEs and their frequency, in this uncomplicated population, should be reassessed if larger studies with longer follow-up confirm these initial findings.

HGG-11. APPLICATION OF SHUNT IN PEDIATRIC DIFFUSE INTRINSIC PONTINE GLIOMA COMPLICATED WITH HYDROCEPHALUS: A 10-YEAR EXPERIENCE FROM A SINGLE CENTER

Abstract OBJECTIVE Currently, there is no unified treatment protocol for pediatric diffuse intrinsic pontine glioma with hydrocephalus. The present study aims to explore the clinical characteristics and the best intervention modality for pediatric diffuse intrinsic pontine glioma with hydrocephalus. METHODS The detailed data of children with diffuse intrinsic pontine glioma admitted to the Department of Pediatrics of Guangdong Sanjiu Brain Hospital from January 2010 to December 2021 were retrospectively analyzed. The effect of radiotherapy and shunt on the prognosis of pediatric diffuse intrinsic pontine glioma complicated with hydrocephalus was evaluated. RESULTS 165 children with DIPG aged ≤18 years were included in the analysis, of whom 9 (5.5%) had mild ventricular dilatation, 48 (35%) had hydrocephalus, and 11 (22.9%) underwent cerebrospinal fluid shunting, of whom 10 (90.9%) underwent ventriculoperitoneal shunt and 1 (9.1%) underwent endoscopic third ventriculostomy. After shunt and radiotherapy, 53 cases of hydrocephalus could be evaluated, of which 37 patients (69.8%) were significantly relieved, and 16 (30.2%) were not. 99 children had tumor progression after treatment, including 41 cases (41.4%) with hydrocephalus and 5 cases (12.2%) with ventriculoperitoneal shunt. A total of 138 children were included in the survival analysis, which revealed that hydrocephalus, mild ventricular dilatation, and no ventricular abnormality had no effect on the prognosis (p=0.944). The treatment of hydrocephalus at the initial diagnosis did not affect the prognosis, p=0.405. The median survival of the hydrocephalus remission group post-radiotherapy was 12 months, and 8 months for the non-remission group, p=0.042. The median survival was 20 months in the shunt group and 8 months in the untreated group (p=0.005). CONCLUSIONS Asymptomatic ventricular enlargement in pediatric diffuse intrinsic pontine gliomas does not require surgical intervention. Hydrocephalus remission post-radiotherapy is a favorable prognostic indicator. Cerebrospinal fluid shunt could improve the prognosis of children with hydrocephalus when the tumor progresses post-radiotherapy.

THE ASSOCIATION OF HYPERTENSION AND OBESITY: WHAT IMPACT ON THE LONGITUDINAL SYSTOLIC FUNCTION OF THE LEFT VENTRICLE

Objective: Hypertension and obesity are well known each result in heart failure with preserved ejection fraction. Indeed, there is ample evidence that the accumulation of adipose tissue in obese subjects negatively affects the left atrial and ventricular structure, as well as diastolic and systolic function. Predisposing factors for heart failure with preserved LVEF are advanced age, hypertension, diabetes, dyslipidemia and obesity. The development of the 2D strain has made it possible to make an early diagnosis of ventricular dysfunction in patients with cardiovascular risk factors. Design and method: This work consists of performing in a series of 128 hypertensive patients divided into two subgroups: 58 obese patients and 70 patients with normal BMI. A complete cardiographic echo study was performed in both subgroups, including LVEF by Simpson biplane method, calculation of indexed left ventricular mass and parietal relative thickness, analysis of diastolic function and finally study of longitudinal LV deformation by speckle tracking technique. Results: We note in this work that dyslipidemia and diabetes were significantly more prevalent in the HTA+obesity arm. The average blood pressure figures were slightly higher in the HTA+obesity arm. LVH was clearly predominant in the HTA+obesity arm with a more consequent decrease in the longitudinal contraction index. In obese hypertensive patients, LVH was most often concentric (53.4%). An increase in filling pressures was found in 11 obese hypertensive patients compared to only 4 non-obese hypertensive patients (p = 0.0001), with a good correlation with the decrease in GLS. These results suggest that increased BMI is closely associated with atrioventricular interaction in patients with hypertension, with a perfect correlation with impairment of longitudinal systolic function and diastolic function compared to the control group. Conclusions: Although the pathophysiological mechanism behind obesity is disputed, several possible explanations have been proposed: obesity has been considered a state of chronic volume overload, increased blood volume, neurohormonal activation, thus increasing oxidative stress. Therefore, obesity is associated with mild ventricular dilation (eccentric remodeling). However, this finding is in contrast to other studies that associate obesity with a concentric rather than eccentric remodeling of the LV.

P120 NEW MALIGNANT MUTATION IN HYPERTROPHIC CARDIOMYOPATHY

Abstract A 62–year–old man diagnosed with non–obstructive hypertrophic cardiomyopathy and dual–chamber pacemaker wearer since the age of 40, for third–degree atrioventricular block, came to our attention for atrial fibrillation (AF) episodes (one complicated by cardiogenic shock and multiorgan failure) and flutter with high ventricular response. For a progressive worsening of the systolic function, and the onset of multiple episodes of sustained ventricular tachycardia, an upgrade to the defibrillator was performed, which, in the following months, intervened appropriately for recurrences of ventricular arrhythmias. The patient was admitted to our ward for exacerbation of heart failure. The echocardiography showed moderate concentric hypertrophy with mild ventricular dilatation and ejection fraction 33%, inferior and inferoseptal wall, and apex akinesia with thrombotic apposition. Due to a sudden deterioration of the clinical conditions, the patient died. After genetic counseling the patient‘s family agreed to genetic testing in the proband. This highlighted the presence of a missense mutation, i.e. the substitution of the cysteine ​​residue with a serine, in the codon 905 of the MYH7 gene, encoding the cardiac isoform of myosin heavy chain. The mutation is probably pathogenetic as it falls in the region where most of the missense variants are clustered and statistically associated with the HCM phenotype. Only in one previous case of HCM was detected a mutation of the same codon, Cys905Phe. Several studies have reported that AF tends to be more prevalent in patients carrying MYH7 mutations and is associated with a substantial risk of heart failure–related mortality, stroke, and severe functional disability. Knowledge of the entire spectrum of MYH7 mutations, especially those related to a high arrhythmic burden or SCD, combined with an accurate clinical and molecular characterization of the patient, can improve the genotype–phenotype correlation by clarifying the mechanism of HCM and allowing a better clinical and therapeutic decision–making of patients.

P325 CARDIAC SARCOIDOSIS PRESENTING WITH ATRIOVENTRICULAR BLOCK AND SUBSEQUENT CARDIAC ARREST: A CASE REPORT

Abstract A 53–year–old male patient with no significant past medical history and no familiar history for cardiac diseases presented to the emergency department for asymptomatic bradycardia. The ECG showed complete atrioventricular block in the absence of pathological findings on the echocardiogram and no coronary lesions were found on coronary angiography. The patient underwent pacemaker (PM) implantation. A few months later the patient suffered from cardiac arrest in sustained ventricular tachycardia, effectively treated with DC shock. The echocardiogram showed mild right ventricular dilation and dysfunction with regional wall motion abnormalities, especially in the subtricuspid site. Cardiac magnetic resonance imaging (MRI) showed focal areas of delayed enhancement with a non–ischaemic pattern and distribution with a hook sign, highly suggestive of cardiac sarcoidosis. FDG PET showed cardiac, hepatic, splenic, and lymph nodal uptake. In the suspicion of systemic sarcoidosis, an hepatic biopsy was performed, being the liver the most accessible site. The histological examination showed the presence of non–caseating granulomas. Therefore, a diagnosis of Systemic Sarcoidosis (SS) with Cardiac involvement was made and the PM was upgraded to CRT–D in secondary prevention. The patient was discharged with cortisone therapy. Three months after discharge, a new total body PET–CT scan was performed and showed, in comparison to the previous one, the absence of areas of significant hypermetabolism in the cardiac, hepatic and bone fields. Cardiac sarcoidosis (CS) is a rare inflammatory granulomatous myocardial disease of unknown etiology that should be suspected in young patients with atrioventricular conduction disturbances and complex ventricular arrhythmias. The diagnostic approach is challenging: imaging techniques (MRI, PET) can be suggestive of CS but biopsy with histological demonstration of the non–caseous granuloma is mandatory. Endomyocardial biopsy (which has suboptimal sensitivity due to focal lesion) could be avoided if there is a more accessible and less invasive extracardiac site for biopsy. Early diagnosis relies on multidisciplinary collaboration and is essential for staging and long–term management of the disease and prevention of sudden cardiac death. Cortisone therapy remains the mainstay of treatment.

Characterization of dorsal recumbency syndrome associated with woody breast in broiler flocks from Ontario, Canada

A dorsal recumbency syndrome (DRS) has been recently described in market-age broiler chickens. Affected broilers fall onto their backs, and are unable to right themselves, and eventually die of cardiopulmonary insufficiency. These broilers are referred to as turtle chickens. A previous report and anecdotal evidence suggest that breast myopathies, such as woody breast (WB), may be associated with DRS due to impaired contractility of the pectoral muscles. In this study, we aimed to provide additional evidence to document DRS in broilers, and its possible association with breast myopathies. A total of 64 broilers (Ross 708), 33 DRS-affected and 31 controls, were culled between 42 and 48 d of age from 3 different commercial farms over 4 visits. All broilers underwent postmortem analysis; breast muscles were scored grossly and/or histologically to determine the presence and severity of myopathies, and sera were used to determine the level of aspartate aminotransferase (AST) and creatine kinase (CK). A gross diagnosis of WB was moderately associated with DRS broilers, and DRS broilers displayed a greater microscopic severity of lesions (P < 0.001) in the Pectoralis major, as typically observed with WB. Levels of AST and CK were greater (P < 0.001) in the sera of DRS-affected compared to control broilers, consistent with muscular damage. The frequency of cardiac changes, such as mild hydropericardium and right ventricular dilation, or severity of microscopic pulmonary lesions, such as edema, were not significantly different between the 2 groups. The odds of DRS increased with the histology score of the P. major (OR=1.37, 95% CI 1.02-1.85). The data presented in this study support an association between DRS and muscular damage of the P. major, suggesting that WB may predispose broilers to DRS. DRS might be a cause of broiler death, and this syndrome could be responsible for significant financial loss to the farmers and to the whole poultry industry.

Abstract 12675: A Modified Classification Scheme for Chaga's Cardiomyopathy Incorporating Cardiac Magnetic Resonance Imaging

Introduction: The public health concerns from Chaga’s disease warrant improved cardiovascular imaging efforts. We present a novel and modified Chaga’s staging classification incorporating cardiac magnetic resonance imaging (CMR) to aid in diagnosing cardiomyopathies (CMs). (Figure 1) Case Presentation: An 18 year old asymptomatic military basic trainee screened serology positive for Trypanosoma Cruzi. A native of South Texas, he had frequent exposure to many triatome bugs without any history of chagoma or bites. Electrocardiogram showed sinus bradycardia, first degree atrioventricular delay, right bundle branch block with a left anterior fascicular block. Transthoracic echocardiogram (TTE) demonstrated a preserved left ventricular ejection fraction (LVEF), no wall motion abnormalities or valvular disease. Cardiopulmonary exercise testing demonstrated the trainee prematurely reaching anaerobic threshold. CMR was performed to evaluate specifically for delayed gadolinium enhancement and chamber size derangements for prognostication and military disposition purposes. CMR demonstrated mild left ventricular dilation, LVEF 65%, no late gadolinium enhancement, normal T1 relaxation time of 932 milliseconds, and normal T2 weighted images. The exposure history, positive serologies, electrocardiogram, and early ventricular remodeling were consistent with a Chaga’s dilated CM revealed with CMR improved spatial resolution. Case Outcome: Military standards currently prohibit cardiomyopathies from entering active duty service. The trainee was discharged and referred for medical therapy. Conclusions: We propose that CMR be incorporated into the Chaga’s classification schemes to (1) identify structural disease not apparent by TTE, (2) recognize fibrotic or inflammatory myocardial involvement which is prognosis altering, and (3) diagnose early involvement of CM which could benefit from earlier anti-parasitic therapy.

Recent SARS-CoV-2 infection: too early to vaccinate?

Recent SARS-CoV-2 infection: too early to vaccinate?

Utility of Cardiac Magnetic Resonance Imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot

Arrhythmias are the leading cause of morbidity and mortality in repaired tetralogy of Fallot (TOF), and over 20% of these patients will develop a sustained atrial arrhythmia during their lifetimes. Cardiac magnetic resonance imaging (cMRI) is frequently performed in TOF, although its ability to identify patients at risk of atrial arrhythmias is uncertain. Adult TOF patients (n=175) with no history of atrial arrhythmia who underwent cMRI between 2003 and 2020 at a single tertiary care center were identified. Clinical characteristics and imaging findings were evaluated to identify a predilection for atrial arrhythmias using Kaplan-Meier survival analysis and log-rank testing. Multivariable Cox regression was used to determine independent predictors of atrial arrhythmias. Over a median follow-up of 3.6years, 29 patients (17%) developed atrial arrhythmias. Independent predictors of atrial arrhythmia included age (hazard ratio [HR] 1.06 per 1-year increase, 95% confidence interval [CI] 1.02 to 1.09, p=0.002), diabetes mellitus (HR 4.26, 95% CI 1.26 to 14.41, p=0.020), indexed right ventricular end-diastolic volume (RVEDVi), (HR 1.20 per 10-ml/m2 increase, 95% CI 1.05 to 1.39, p=0.010), and moderate or greater tricuspid regurgitation (TR) (HR 6.32, 95% CI 2.15 to 18.60, p=0.001). Utilizing Kaplan-Meier analysis, patients with at least mild right ventricular dilation (RVEDVi >100ml/m2, p=0.047) and greater than or equal to moderate TR (p <0.001) were found to be significantly more likely to develop atrial arrhythmias. In conclusion, cMRI can help to identify TOF patients at increased risk for atrial arrhythmia beyond standard clinical and imaging data by better quantifying RVEDVi and degree of TR.

Correlation of intraprocedural and follow up parameters for mitral regurgitation grading after percutaneous edge-to-edge repair

Abstract Background Percutaneous edge-to-edge mitral repair has merged as an effective therapy for moderate-to-severe mitral regurgitation (MR) in high surgical risk patients. Transesophageal echocardiogram (TEE) is crucial for procedure guiding and immediate result evaluation, whereas transthoracic echocardiogram (TTE) is largely used in follow up. However, there is no consensus on the best intraprocedural parameter to evaluate residual MR. Purpose To evaluate the predictive value of different MR parameters from intraprocedural TEE with grading in consecutive TTE during the follow up. Methods All the consecutive patients who underwent percutaneous mitral repair with the MitraClip system between 2010 and 2020 in our tertiary university hospital were considered for this study. Immediate posprocedural MR parameters (number of jets, summatory and maximum vena contracta (VC), summatory and maximum 3D effective regurgitation orifice (ERO) and pulmonary vein (PV) flow parameters) were reassessed when possible blindly to the follow up MR grading in sequential TTE. Results We included 88 patients (64.8% males) with a mean age of 76±10 years. Baseline MR was graded as moderate-to-severe in 13 (14.8%) and severe in 75 (85.2%). The most frequent MR etiology was secondary (44.3%) followed by primary (35.2%) and mixed (20.5%). Patients presented with mild left ventricular systolic dysfunction (LVEF 44.5±15.3%) and dilatation (LVEDVi 71.8 [51.5–102.8] mL/m2). MR grading distribution remained stable at 1 and 6 months follow up TTE. Among all the aforementioned criteria only summatory and maximum VC remained significant for different MR grade prediction. Thus, these values were able to identify MR ≥3 at 1 and 6 months (Table). Moreover, on ROC analysis maximum VC demonstrated an excellent discriminatory power to identify significant MR at 6 months (Figure). Thereby, a cut-off point of 0.45 cm was able to predict MR ≥3 with 88% sensitivity and 89% specificity. Conclusion Among intraprocedural TEE parameters to evaluate residual MR in percutaneous edge-to-edge mitral repair, maximum and summatory VC appeared to be the more reliable to predict significant insufficiency in the follow up. Funding Acknowledgement Type of funding sources: None. Table 1. Differences in intraprocedural TEE VC in relation with significant MR in follow-up TTEFigure 1. ROC curve of maximum VC for prediction of significant MR at 6 months