Biatrial Dilatation Research Articles

Differences in cardiac magnetic resonance imaging bi-atrial strain between elite athletes and atrial fibrillation patients

Abstract Background Elite athletes frequently demonstrate outspoken bi-atrial dilation, even exceeding volumes seen in patients with atrial fibrillation (AF). Hence, distinguishing atrial exercise-induced adaptation from maladaptation or atrial pathology cannot be done using volumes only. However, data on bi-atrial strain in athletes compared with AF patients investigated using cardiac magnetic resonance imaging (CMR). Methods We performed a retrospective cross-sectional analysis of CMR data comparing three groups: (1) elite athletes included in the ELITE-cohort at the Amsterdam UMC (> 16 years of age and train ≥ 10 hours/week at (inter)national or Olympic level, (2) paroxysmal- (PAF) and persistent AF patients awaiting PVI and (3) healthy controls. All participants underwent CMR in sinus rhythm. Left- and right atrial (LA/RA) reservoir (RES), conduit (CD) and contractile (CT) strain were analysed by automatic feature tracking (FT). Results We included a total of 340 elite athletes (median age 28 years [17, 67]; female n=145 (42.6%)), 103 AF patients (median age 60 [29, 75], female n=36 (35%), PAF n=74 (71.8%), persistent AF n=29 (28.2%), and 85 healthy controls (median age 28 [16, 72], female n=39 (45.9%). Elite athletes had similar indexed maximal left atrial volumes (LAVI) compared to AF patients (49.6 ml/m2 [41.9, 58.3] vs 47.9 ml/m2 [40.5 ml/m2], p = .25), but larger indexed maximal right atrial volumes (RAVI) (59.3 ml/m2 [50.7, 70.7] vs 44.7 ml/m2 [37.0, 55.7], p < .001). Elite athletes had lower LA RES strain (42.8% [36.7. 49.2]) than controls (47.5% [40.6, 54.3], p < .001) but a higher LA RES than AF patients (34.7% [27.4, 41.6], p < .001). Athletes demonstrated higher LA CD/CT ratio (2.2 [1.8, 2.8]) than healthy controls (1.8 [1.4, 2.2], p < .001) and AF patients (1.4 [1.1, 1.9], p < .001) caused by a higher LA CD in athletes (29.2% [24.7, 34.2] compared to healthy controls (29.1% [24.0, 36.2], p < .001) and AF patients (20.1% [16.2, 25.6], p < .001) and a lower LA CT in both athletes (13.0% [22.1, 15.8] and controls (20.1% [16.2, 25.6], compared to healthy controls (17.2% [14.1, 19.9], p < .001). Elite athletes had lower RA RES strain (51.5% [42.8. 63.4]) than controls (57.3% [48.0, 73.5], p = .001) and higher RA RES than AF patients (44.7% [34.1, 55.0], p < .001). AF patients had lower RA CD (28.3% [20.1, 36.8] than elite athletes (35.9% [28.9, 45.5], p < .001) and healthy controls (41.9% [32.4, 51.1], p < .001). Elite athletes had higher RA CD/CT ratio than AF patients (2.5 [1.9, 3.2] vs 1.9 [1.2, 2.4], p < .001). Conclusion Compared with healthy controls and patients with AF, athletes demonstrate unique bi-atrial characteristics. While LAVI is similar in both elite athletes and AF patients, elite athletes demonstrate larger RAVI and a unique strain profile characterized by a higher bi-atrial CD/CT ratio compared with AF patients, but a reduced bi-atrial RES strain relative to healthy controls yet elevated compared to AF patients.Differences in bi-atrial strain

Atrial Fibrillation in Elite Athletes: A Comprehensive Review of the Literature.

Although the benefits of exercise training have been shown repeatedly in many studies, its relationship with the occurrence of atrial fibrillation (AF) in competitive athletes still remains controversial. In the present review, we sought to demonstrate a comprehensive report of the incidence, pathophysiology, and therapeutic approaches to AF in elite athletes. A 2 to 10 times higher frequency of AF has been shown in many studies in high-intensity endurance athletes compared to individuals who do not exercise. Moreover, a U-shaped relationship between male elite athletes and AF is demonstrated through this finding, while the type and the years of physical activity seem to relate to AF development. A strong correlation seems to exist among the type of exercise (endurance sports), age (>55 years), gender (males), and the time of exercise training, all contributing to an increased risk of AF. The pathophysiology of AF still remains unclear; however, several theories suggest that complex mechanisms are involved, such as bi-atrial dilatation, pulmonary vein stretching, cardiac inflammation, fibrosis, and increased vagal tone. Elite athletes with AF require a comprehensive clinical evaluation and risk factor optimization, similar to the approach taken for nonathletes. Although anticoagulation and rate or rhythm control are cornerstones of AF management, there are still no specific guidelines for elite athletes.

Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome?: A case report and literature review.

Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department as an initial feature when typical clinical symptoms of KD have not be detected. We report the case of a 12-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and signs of shock such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome, while the treatment was not effective. Subsequent chest computerized tomography and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. After a multidisciplinary consultation, a diagnosis of KDSS was made. To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid. Soon afterwards, he showed significant improvement, with the temperature dropped to normal and hypotension corrected about 24 hours post-intravenous immunoglobulin therapy. Polyserous effusions also disappeared before discharge. Follow-up echocardiography revealed normal results. Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.

Moderate/severe biatrial dilation predicts adverse events after ablation in atrial fibrillation with heart failure.

To retrospectively compare the long-term outcomes following atrial fibrillation (AF) ablation between heart failure (HF) with preserved ejection fraction (EF) (HFpEF) and reduced/mildly reduced EF (HFr-mrEF) patients, and to identify novel predictors of adverse clinical events. In total, 1402 AF patients with HF who underwent successful ablation were consecutively enrolled. Adverse clinical events including all-cause death, HF hospitalization, and stroke were followed up. Cox proportional hazards models were used to assess the associations between clinical factors and events. Kaplan-Meier analysis was performed to estimate the cumulative incidences of these events. A receiver operating characteristic curve was used to test the ability of these predictors. During a follow-up period of 42±15months, 265 (18.9%) patients experienced adverse clinical events after ablation. The cumulative incidence of adverse clinical events was significantly higher in HFr-mrEF than in HFpEF (25.4% vs. 15.7%, P<0.001), the similar tendency was observed on all-cause death (10.5% vs. 6.5%, P=0.011) and HF hospitalization (17.2% vs. 10.1%, P<0.001). After multivariate adjustment, non-paroxysmal AF [hazard ratio (HR) 1.922, 95% confidence interval (CI) 1.130-3.268, P=0.016], LAD≥45mm (HR 2.197, 95% CI 1.206-4.003, P<0.001), LVEF (HR 0.959, 95% CI 0.946-0.981, P<0.001), and RAD ≥ 45mm (HR 2.044, 95% CI 1.362-3.238, P<0.001) remained the independent predictors for developing adverse clinical events. A predictive model performed with non-paroxysmal AF, LAD≥45mm and RAD≥45mm yielded an area under curve of 0.728 (95% CI 0.696-0.760, P<0.001). AF patients with HFpEF had better long-term outcomes than those with HFr-mrEF, and moderate/severe biatrial dilation could predict adverse clinical events following catheter ablation in AF and HF patients.

DON’T FORGET ABOUT CARDIAC AMYLOIDOSIS: WHEN RED FLAGS MATTER

Abstract Case Report A 78–year–old man came to our attention for worsening dyspnea, asthenia and frequent lipothymic episodes in the last few weeks. Nothing remarkable was reported in anamnesis except for periodic cardiological check–ups ascribed to hypertensive heart disease and moderate aortic stenosis. Other cardiovascular risk factors were obesity, previous smoking habits and dyslipidemia. A recent 24–hours ECG monitoring revealed slow permanent atrial fibrillation (AF) with 3–seconds pauses, frequent premature ventricular complexes (PVCs) and some PVCs couplets. On arrival, NT–proBNP was elevated (2952 pg/mL), while ECG showed AF with a heart rate of 50/m and low voltages in peripheral leads. Transthoracic echocardiogram revealed a biatrial dilatation, a severe left ventricular (LV) concentric hypertrophy with a preserved LV ejection fraction (EF) and a moderate aortic stenosis. In addition, a restrictive LV filling pattern, a decreased longitudinal myocardial contraction and an apical sparing of longitudinal strain were reported. Invasive coronary angiography was performed with evidence of normal coronary arteries. Given the echocardiographic suspicion of amyloidosis, a 99mTc–Hydroxymethylene diphosphonate (HMDP) scintigraphy was performed, showing a moderate cardiac uptake and an attenuated bone uptake (Perugini Score 2). Serum and urine immunofixation resulted negative. Genetic testing did not find any mutations in Transthyretin (TTR) gene. Therefore, the diagnosis of TTR wild–type amyloidosis was made. Before discharge, putting it all together, we opted to implant a leadless pacemaker in order to protect the patient from life–threatening bradyarrhythmias. Discussion Although it is currently considered rare, there is growing evidence that cardiac amyloidosis may be more common than once thought. First of all, it should be investigated in HFpEF–patients (heart failure with preserved EF) with LV hypertrophy. Despite several red flags (low QRS voltages, conduction disorders, elevated NT–proBNP, severe LV hypertrophy and aortic stenosis) and various medical consultations, our patient had to wait 4 years or more to be diagnosed with cardiac amyloidosis. Inevitably, a diagnostic delay means loss of time in treatment initiation. Earlier diagnosis would enable us to improve our patients’ survival and heart failure hospitalization. Awareness alone is not enough, we must be right on target too.

Pediatric restrictive cardiomyopathy: a case report.

Restrictive cardiomyopathy (RCM) is a rare childhood cardiomyopathy that is a challenging diagnostic problem for clinicians. We describe a case of an 8-year-old girl with a 2-year history of shortness of breath on exertion. Electrocardiogram and echocardiography showed biatrial enlargement, while cardiac magnetic resonance showed biatrial dilation and normal pericardial thickness. Left and right heart catheterization revealed a left ventricular (LV) end-diastolic pressure (EDP) of 20 mmHg, right ventricular (RV) EDP of 13 mmHg, and pulmonary arterial systolic pressure of 51 mmHg. LV and RV pressure traces showed that LV and RV pressures moved concordantly with respiration, and that the systolic area index was 0.98. Cardiac catheterization data were therefore supportive of RCM. Next-generation sequencing identified a heterozygous variant of the troponin I gene (TNNI3; c.574C>T). Combining these findings led to a diagnosis of RCM. The patient's parents chose conservative treatment, but at the 12-month follow-up she died of worsening heart failure and cerebral infarction. This case emphasizes the need for cardiac catheterization and genetic testing in RCM, and suggests that anticoagulants should be recommended to reduce the risk of thromboembolic events.

Developing a pathway for individuals with heart failure with preserved ejection fraction (hfpef): scoping the population accessing unscheduled care

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): Scottish Government Background The Heart Disease Action Plan’s vision that everyone in Scotland is supported to live well with their heart condition(s) [1]. In 2021 NHS Ayrshire and Arran (NHS A&amp;A) was successful in attaining Scottish Government funding to develop and pilot a care pathway for those with heart failure with preserved ejection fraction (HFpEF). HFpEF is not widely understood by the wider healthcare community, and this population’s access to care is limited. To help inform development of this pathway, a scoping exercise was undertaken to fully understand the needs of the HFpEF population, and to provide baseline data for evaluation of the new pathway. Purpose As part of the scoping exercise, data were examined to help identify and describe the HFpEF population accruing unscheduled care admissions within NHS A&amp;A. It was anticipated that these individuals would be most likely to benefit from the new pathway. Methods Data were obtained using these criteria: - Unscheduled care admission from 1st July to 31st December 2021 (inclusive) - Admissions with HFpEF listed as a primary or secondary cause of admission Demographic, diagnostic, mortality and morbidity information of individuals accruing these admissions were examined to provide a clinical picture of the population. Results During the time period, 81 individuals were admitted to hospital with HFpEF. The mean age of the population was 79.6yrs (31 males [38%] 50 [62%] female) and most (n=26; 32%) were living in areas classed as SIMD 3 [2]. Of the 81, all presented with left ventricular ejection fraction ≥50%, n=59 (73%) had biatrial dilatation, and n=26 (32%) had evidence of right ventricular impairment. Valve disease was present in n=53 (66%). High filling pressures were evident in up to n=39 (48%). The population presented with an average of four co-existing morbidities. Most prevalent were hypertension (n=56; 69%), atrial fibrillation (n=46; 57%), diabetes (n=35; 43%) and chronic kidney disease (n=35; 43%). A total of 160 admissions / 1171 bed days were accrued by the whole population during the time period. Of these admissions, 56 (35%) were ≥ 10 days, however 44 (29%) were 1-day admissions. The primary cause of admission was HFpEF in 64 cases and HFpEF was listed as a secondary cause for a further 37 admissions. A further 24 admissions were due to cardiovascular disease (CVD). To date, n=31 (38%) have died (n=20 during their last 2021 admission, n=11 thereafter). CVD was listed as the primary cause of death in n=11, and in n=7 this was specifically HFpEF. Conclusions These data show the HFpEF population accessing unscheduled care with NHSA&amp;A to be elderly, and mainly female with multimorbidity. Both bed days accrued and mortality rate were high. The data have informed development and implementation of a new care pathway, and it is hoped that evaluation of the pathway will demonstrate a positive impact upon these data.

Predictors of atrial flutter recurrence after atrial fibrillation ablation: the crucial role of structural and morphological left atrial remodeling

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted Grant - Abbott Medical Introduction Recurrence of supraventricular tachyarrhythmias after catheter ablation of atrial fibrillation (AF) is common, representing 30% of patients. Although AF recurrence occurs mostly, 10% of the patients face arrhythmia recurrence through atypical atrial flutter (AFL). The diagnostic and therapeutic management of atypical AFL is challenging. Predicting atypical AFL after AF catheter ablation is critical. Purpose To identify morphological and structural predictors of recurrence in the form of atypical AFL compared to AF based on Late Gadolinium Enhancement Cardiac Magnetic Resonance (LGE-CMR). Methods – From January 2013 to December 2021, 56 patients facing post AF-CA atypical AFL were included in the monocentric and retrospective cohort. 112 patients with AF recurrence were randomly included in the cohort from the general database. Pre-procedure LGE-CMR was post-processed for every patient to assess global and regional left and right atrial (LA, RA) fibrosis quantification using an image intensity ratio &amp;gt; 1.32 as fibrosis. Sphericity was calculated as the percentage of similarity with respect to a sphere, and the volume of LA and RA were calculated. Results – 168 patients who had faced supraventricular arrhythmia recurrence after AF-CA were included in the monocentric retrospective cohort. They were mainly male (72.1%), with a median CHADS-VASc score of 1.4 +/- 1.3, a number of previous CA of 2,2 +/- 0.4, and a biatrial dilation (LA volume index of 53.5 ml/m2, RA volume index of 59.6 ml/m2). An age superior to 65 (OR=6.61, CI95%:2.18-14.41, p&amp;lt;0.01), the number of previous CA procedures (OR=3.03, CI95%:1.17-7.81, p=0.02), a history of management of invasive additional lines (OR=2.48, CI95%:1.01-6.27, p=0.05), an elevated LA sphericity (OR=1.33, CI95%:1.11-1.59, p&amp;lt;0.01) and an increased LA fibrotic burden (OR=1.03, CI95%:1.00-1.07, p=0.04) independently predicted the occurrence of atypical AFL compared with AF recurrence after AF-CA (Figure 1). Specifically, an increased amount of fibrosis in the lateral LA zone area (OR=1.03, CI95%:1.01-1.04, p&amp;lt;0.01)] was an independent predictor of recurrence in the form of atypical AFL compared with AF recurrence (Figure 2). Conclusion The structural LA remodeling of AE, including LA fibrosis and LA sphericity, is a predictor of recurrence in the form of atypical atrial flutter compared with AF recurrence after AF-CA.

P413 A CASE OF PERIPARTUM DILATED CARDIOMYOPATHY WITH SEVERE BIVENTRICULAR DYSFUNCTION

Abstract Woman, 38 aa, without cardiovascular risk factors with the exception of the tobagic habit; In anamnesis recent Caesarean cut about six months ago for a placenta with the central, complicated by hemorrhagic shock and consequent virgonecontomy. It came to our observation sent by the emergency room, where it had gone by on the onset of about 20 days of heart disease, nightlife dyspnea and fattening dyspnea, associated with the recent appearance of marked edemas declining in the lower limbs bilaterally. At the entrance to the ward, the patient presented himself vigilant, apipperic and tachipnoic. The feedback of turgor of the jugular and of extended edemas extended up to the upper third of the leg bilaterally was also reported. The ECG evidenced a sinus tachycardia and signs of left ventricular overload. The hematochimic tests, including myocardionecrosi markers, were in the norm, with the exception of the pro–bnp which was increased with a value of 5263 pg/ml (cutoff &amp;lt;125 pg/ml). A trastracic echocardiogram was carried out which highlighted a dilated left ventricle with normal parietal thicknesses, global hypocinesia and severely depressed systolic function (FE 28%), a biatrial dilation with dilated and hypocinetic right sections (15mm tapse), to the Doppler color, an insufficiency Mutral valve of moderate and tricuspidal grade of severe grade with an estimated paps of 55 mmhg and a lower cava dilated cava and poorly collapsed with the inspirium. For further diagnostic deepening, a cardiac MRI was performed which confirmed the framework of two–hundred dilation with severe reduction of systolic function (LVEF 25%; RVEF 20%) and showed a post–control myocardial strengthening of the lateral pericardium with extension to be paid by the Subpicardio homosede. A high dosage diuretic therapy and anti–scompense therapy with progressive improvement of the hemodynamic picture was then set. The patient was discharged at home in protected discharge after positioning of wearable defibrillator; It was then checked after 30 days from discharge with a recovery feedback almost complete with the contractile function and followed later over time with periodic follow–ups.

P289 HEART FAILURE DUE TO HIGH OUTPUT ARTERIOVENOUS FISTULA IN A DIALYSIS PATIENT AFFECTED BY AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

Abstract Autosomal dominant polycystic kidney disease (ADPKD) can determine end stage renal disease (ESRD) which require haemodialysis. Surgical creation of arteriovenous fistula (AVF) has been associated with development of left ventricular (LV) dilatation and high output heart failure (HF) and right ventricle (RV) dysfunction at long term follow–up. Databases show an association between ADPKD and idiopathic dilated cardiomyopathy, suggesting that PKD mutations contribute to development of heart failure. 36 ys old female patient affected by ADPKD who required in 2018 bilateral nephrectomy, due to a relapse of severe urinary tract bleeding, and then put in thrice–weekly hemodialysis. Before the procedure in 2016 an echocardiogram showed normal biventricular function, with valvular defects. An AVF, between left humeral artery and the perforator vein of the elbow, was employed as vascular access. 6 months later she was referred to our center for atypical chest pain and palpitations. A transthoracic echocardiogram showed a diffuse hypokinesia with severe left ventricular dysfunction and dilation (EF 25%, VTDi 90ml/mq), a cardiac output of 6,5 l/min, moderate mitral insufficiency, hypokinetic RV, pulmonary artery systolic pressure (PASP) of 56mmHg, severe biatrial dilatation. No signs of congestion. She was admitted to our service. The coronary angiogram was normal, ruling out an underlying ischemic cardiomyopathy. Therapy with b–blocker and ACE–I was started. Doppler ultrasonography of the AVF was performed and showed an high output fistula. Two weeks later at Hospital discharge with medical therapy only, EF improved to 43%. Five months later, the AVF was ligated, after the creation of another more distal one. Echocardiographic re–evaluation one month after the intervention showed a normalization of left ventricular function and volumes ( EF 55%, VDTi 54ml/mq)and of PASP. In 2020 she underwent renaltransplant without complications. The patient is nowadays stable only on beta–blocker for some PVCs, with ongoing annual follow–up. Even if dilated cardiomyopathy and HF in patients affected by APDKD can be caused by genetic predisposition, in our case the main factor was a high output AVF, as showed by complete reversion of left ventricular dysfunction after its ligation.

PO-02-197 PREDICTORS OF ATRIAL FLUTTER RECURRENCE AFTER ATRIAL FIBRILLATION ABLATION: THE CRUCIAL ROLE OF STRUCTURAL AND MORPHOLOGICAL LEFT ATRIAL REMODELING

Recurrence of supraventricular tachyarrhythmias after catheter ablation of atrial fibrillation (AF) is common, representing 30% of patients. Although AF recurrence occurs mostly, 10% of the patients face arrhythmia recurrence through atypical atrial flutter (AFL). The diagnostic and therapeutic management of atypical AFL is challenging. Predicting atypical AFL after AF catheter ablation is critical. To identify morphological and structural predictors of recurrence in the form of atypical AFL compared to AF based on Late Gadolinium Enhancement Cardiac Magnetic Resonance (LGE-CMR). From January 2013 to December 2021, 56 patients facing post-AF-CA atypical AFL were included in the monocentric and retrospective cohort. 112 patients with AF recurrence were randomly included in the cohort from the general database. Pre-procedure LGE-CMR was post-processed for every patient to assess global and regional left and right atrial (LA, RA) fibrosis quantification using an image intensity ratio > 1.32 as fibrosis. Sphericity was calculated as the percentage of similarity with respect to a sphere, and the volume of LA and RA were calculated. 168 patients who had faced supraventricular arrhythmia recurrence after AF-CA were included in the monocentric retrospective cohort. They were mainly male (72.1%), with a median CHADS-VASc score of 1.4 +/- 1.3, a number of previous CA of 2,2 +/- 0.4, and a biatrial dilation (LA volume index of 53.5 ml/m2, RA volume index of 59.6 ml/m2). An age superior to 65 (OR=6.61, CI95%:2.18-14.41, p<0.01), the number of previous CA procedures (OR=3.03, CI95%:1.17-7.81, p=0.02), a history of management of invasive additional lines (OR=2.48, CI95%:1.01-6.27, p=0.05), an elevated LA sphericity (OR=1.33, CI95%:1.11-1.59, p<0.01) and an increased LA fibrotic burden (OR=1.03, CI95%:1.00-1.07, p=0.04) independently predicted the occurrence of atypical AFL compared with AF recurrence after AF-CA (Figure). Specifically, an increased amount of fibrosis in the lateral LA zone area (OR=1.03, CI95%:1.01-1.04, p<0.01)] was an independent predictor of recurrence in the form of atypical AFL compared with AF recurrence (Table). The structural LA remodeling of AE, including LA fibrosis and LA sphericity, is a predictor of recurrence in the form of atypical atrial flutter compared with AF recurrence after AF-CA.

PO-01-087 A RARE CASE OF BI-ATRIAL FDG-UPTAKE: PRESENTING AS NEW ONSET ATRIAL FIBRILLATION

Isolated atrial myocarditis is rare, with presenting symptoms that are non-specific. Prior reports have suggested association with atrial arrythmias and increased risk of stroke due to atrial dysfunction. Fluorodeoxyglucose (FDG)-positron emission tomography (PET) CT scan is considered as a goal standard test for the diagnosis of myocarditis. To present a case highlighting that isolated atrial myocarditis had higher incidence atrial arrythmias and oral anticoagulation should be considered in such cases. N/A A 55yo male with no prior medical history presented with non-specific symptoms of fatigue, shortness of breath and frequent palpitations. He underwent 2D echocardiogram showed LVEF of 50-55% with mild bi-atrial dilatation with no regional wall motion abnormalities and was diagnosed with incidental atrial fibrillation during the procedure. He was referred to cardiac electrophysiology for further work up. During his initial EP clinic visit, 12 lead ECG demonstrated normal sinus rhythm with premature atrial contractions and premature ventricular contractions. Due to symptomatic, dual chamber arrythmias, cardiac MRI (CMR) and FDG-PET CT was ordered to rule out myocarditis. The CMR was negative for any LGE however, high-fat dietary preparation FDG-PET CT scan revealed uptake on bi-atrial walls (figure) and interatrial septum with SUV max of 3.1. There was no LV myocardial FDG uptake noted. Patient was diagnosed with isolated atrial myocarditis and was started on dronedarone, OAC (CHADS2VASc =0) for stroke prophylaxis and immunosuppressive therapy (methotrexate and low dose prednisone). Invasive atrial myocardial biopsy was not considered due good response to therapy. Isolated atrial myocarditis is an uncommon presentation of acute myocarditis and is associated with atrial arrythmias. FDG PET-CT or CMR imaging may be useful to aid diagnosis. Long-term response to immunosuppressive therapy may need further investigations. OAC should be considered for stroke prophylaxis, despite low CHADS2VASc due to unknown long-term implications of atrial dysfunction.

Role of 3 Tesla Magnetic Resonance Imaging in the Assessment of Infiltrative Cardiomyopathies.

The aim of the present study was to assess the role of 3 Tesla (3T) magnetic resonance imaging (MRI) in the assessment of infiltrative cardiomyopathy (ICM). Cardiac MRI was performed on a 3T MRI machine for 15 patients who had clinical or echocardiographic signs of infiltrative cardiomyopathy. Each scan was assessed on a set of anatomical and functional parameters. The patterns of left ventricular (LV) late gadolinium enhancement (LGE) were also analyzed. Bi-atrial dilatation was noted in 14 patients, consistent with a restrictive phenotype. All 15 patients had diastolic dysfunction with reduced LV diastolic ventricular filling and prolonged peak filling times. Eleven patients had a decreased peak filling rate. Twelve patients had systolic dysfunction with reduced ejection fraction (EF). Ten patients had contractile dysfunction in the form of global LVhypokinesia. On delayed contrast imaging, four patients showed no abnormal LGE. Two patients showed diffuse subendocardial enhancement. Two patients showed patchy subendocardial enhancement. Six patients showed patchy mid-myocardial enhancement. One patient showed diffuse mid-myocardial enhancement. Three patients showed patchy subepicardial enhancement. Two patients showed patchy transmural enhancement. Three patients showed reversed myocardial nulling. All 15 patients received a provisional diagnosis of infiltrative cardiomyopathy on the basis of cardiac MRI findings. Sarcoidosis was given as a probable cause in four patients, amyloidosis in three patients, an infectious cause in two patients, and drug-induced cardiomyopathy in one patient. In five patients, no obvious cause could be identified. Infiltrative cardiomyopathies, although relatively uncommon, pose significant challenges in diagnosis and treatment. Cardiac MRI has become the gold standard for non-invasive diagnosis of all infiltrative cardiomyopathies.

Novel filamin C (FLNC) variant causes a severe form of familial mixed hypertrophic-restrictive cardiomyopathy.

Variants of filamin C (FLNC) have been identified as rare genetic substrate for hypertrophic cardiomyopathy (HCM). Data on the clinical course of FLNC-related HCM are conflicting with some studies suggesting mild phenotypes whereas other studies have reported more severe outcomes. In this study, we present a novel FLNC variant (Ile1937Asn) that was identified in a large family of French-Canadian descent with excellent segregation data. FLNC-Ile1937Asn is a novel missense variant characterized by full penetrance and poor clinical outcomes. End stage heart failure requiring transplantation occurred in 43% and sudden cardiac death in 29% of affected family members. Other particular features of FLNC-Ile1937Asn include an early disease onset (mean age of 19 years) and the development of a marked atrial myopathy (severe biatrial dilatation with remodeling and multiple complex atrial arrhythmias) that was present in all gene carriers. The FLNC-Ile1937Asn variant is a novel, pathogenic mutation resulting in a severe form of HCM with full disease penetrance. The variant is associated with a high proportion of end-stage heart failure, heart transplantation, and disease-related mortality. Close follow-up and appropriate risk stratification of affected individuals at specialized heart centers is recommended.

Machine learning identifies pathophysiologically and prognostically informative phenotypes among patients with mitral regurgitation undergoing transcatheter edge-to-edge repair.

Patients with mitral regurgitation (MR) present with considerable heterogeneity in cardiac damage depending on underlying aetiology, disease progression, and comorbidities. This study aims to capture their cardiopulmonary complexity by employing a machine-learning (ML)-based phenotyping approach. Data were obtained from 1426 patients undergoing mitral valve transcatheter edge-to-edge repair (MV TEER) for MR. The ML model was developed using 609 patients (derivation cohort) and validated on 817 patients from two external institutions. Phenotyping was based on echocardiographic data, and ML-derived phenotypes were correlated with 5-year outcomes. Unsupervised agglomerative clustering revealed four phenotypes among the derivation cohort: Cluster 1 showed preserved left ventricular ejection fraction (LVEF; 56.5 ± 7.79%) and regular left ventricular end-systolic diameter (LVESD; 35.2 ± 7.52 mm); 5-year survival in Cluster 1, hereinafter serving as a reference, was 60.9%. Cluster 2 presented with preserved LVEF (55.7 ± 7.82%) but showed the largest mitral valve effective regurgitant orifice area (0.623 ± 0.360 cm2) and highest systolic pulmonary artery pressures (68.4 ± 16.2 mmHg); 5-year survival ranged at 43.7% (P-value: 0.032). Cluster 3 was characterized by impaired LVEF (31.0 ± 10.4%) and enlarged LVESD (53.2 ± 10.9 mm); 5-year survival was reduced to 38.3% (P-value: <0.001). The poorest 5-year survival (23.8%; P-value: <0.001) was observed in Cluster 4 with biatrial dilatation (left atrial volume: 312 ± 113 mL; right atrial area: 46.0 ± 8.83 cm2) although LVEF was only slightly reduced (51.5 ± 11.0%). Importantly, the prognostic significance of ML-derived phenotypes was externally confirmed. ML-enabled phenotyping captures the complexity of extra-mitral valve cardiac damage, which does not necessarily occur in a sequential fashion. This novel phenotyping approach can refine risk stratification in patients undergoing MV TEER in the future.

Man-machine interaction-based phenotyping identifies pathophysiologically and prognostically informative clusters among patients with mitral regurgitation undergoing transcatheter edge-to-edge repair

Abstract Background Depending on etiology, extent of disease progression, and comorbidities, patients with severe mitral regurgitation (MR) typically present with considerable heterogeneity. Purpose This study therefore sought to improve diagnostic and prognostic resolution in patients undergoing mitral valve transcatheter edge-to-edge repair (MV TEER) for severe MR by developing a man-machine interaction-based phenotyping approach. Methods All 609 patients from this single-center registry underwent MV TEER for severe MR between 2009 and 2020. Unsupervised agglomerative clustering was applied to preprocedural echocardiography data, and an artificial neural network (ANN) was subsequently trained for future patient-to-cluster assignment. Primary outcome measure was postprocedural 5-year survival Results Cluster analysis revealed four pathophysiologically and prognostically informative phenotypes: Cluster 1 was constituted by patients (n=188) presenting with preserved left ventricular ejection fraction (LVEF; 56.5±7.79%) and regular left ventricular end-systolic diameter (LVESD; 35.2±7.52 mm). 5-year survival in patients from cluster 1, hereinafter serving as a reference, was 60.9% (95% CI: 53.3–69.7%). Patients from cluster 2 (n=102) also presented with preserved LVEF (55.7±7.82%) and regular LVESD (34.9±7.68 mm), but showed the largest mitral valve effective regurgitant orifice area (0.623±0.360 cm2) and highest systolic pulmonary artery pressures (68.4±16.2 mmHg). Consequently, their 5-year survival ranged at 43.7% (95% CI: 33.2–57.6%; p-value: 0.032). Patients from cluster 3 (n=270) were predominantly characterized by impaired left ventricular systolic function (LVEF: 31.0±10.4%) and dilated left ventricular diameters (LVESD: 53.2±10.9 mm), and their 5-year survival was reduced to 38.3% (95% CI: 31.9–46.1%; p-value: &amp;lt;0.001). Poorest 5-year survival (23.8% [95% CI: 12.8–44.3%]; p-value: &amp;lt;0.001) was observed in patients from cluster 4 (n=49) with biatrial dilatation (left atrial volume: 312±113 mL; right atrial area: 46.0±8.83 cm2) although LVEF was only slightly reduced (51.5±11.0%). All patients from cluster 4 were diagnosed with atrial fibrillation. An ANN could precisely predict cluster assignment (accuracy: 85.2%), detecting patients from high-risk clusters 3 and 4 with excellent specificity (95.0% and 99.4%, respectively). Conclusion Assigning patients to clusters using a multiparametric phenotypic approach can facilitate risk stratification in future clinical practice. Our unsupervised machine learning-based classification system differs from previous approaches for risk stratification, because we do neither hypothesize a linear sequence of accumulated pathologies caused by severe MR (potentially ignoring the aggravating impact of comorbidities), nor do we stratify patients into low- and high-risk cohorts in accordance with a single variable's dichotomy (prone to oversimplification). Funding Acknowledgement Type of funding sources: None.

Extent and determinants of dynamic mitral regurgitation unmasked by handgrip exercise in patients with primary mitral regurgitation

Abstract Background Bicycle exercise is the exercise modality of choice to unmask the dynamic nature of mitral regurgitation (MR). Handgrip exercise may serve as alternative exercise intervention, as it can be performed physically accomplished by the majority of patients. Until now, there are only scarce data on the prevalence and magnitude of dynamic MR during handgrip exercise. Purpose To assess extent and predictors of exercise-induced dynamic MR during handgrip exercise in patients with primary MR. Methods We prospectively included patients with primary MR and at least mild severity that underwent echocardiography at rest and during three minutes of handgrip exercise according to a standardized protocol. Results The final patient cohort included 166 patients. Mean age was 72±12 years, 57% were female and 53% had atrial fibrillation. At rest, MR severity was graded as mild in 57% of patients, moderate MR in 27% and severe in 16%. Handgrip exercise led to an increase in at least one grade in MR severity in 31 patients (19%), while 15 patients (9%) experienced a decrease of MR severity (Fig. 1B). Twenty patients (14%) of those with non-severe MR at rest, developed severe MR during handgrip exercise (Fig. 1B). According to a pre-defined cutoff of increase in effective orifice regurgitant area (EROA) &amp;gt;10 mm2 during exercise, 19% of patients revealed a marked exercise-induced increase in MR, irrespective of the severity of MR at rest. Patients with marked exercise-induced increases in MR severity (EROA &amp;gt;10 mm2) had elevated left- (53±22ml/m2 vs. 44±19ml/m2; p=0.036), and right atrial volumes (43±19ml/m2 vs. 34±18ml/m2; p=0.014), more often pathology of the anterior mitral leaflet (e.g. prolapse; flail leaflet) (65% vs. 33%; p&amp;lt;0.001), and increased mitral annulus diameter (38±5mm vs. 36±4mm; p=0.003). The exercise-induced changes in EROA correlated with changes in estimated systolic pulmonary pressure from rest to exercise (r=0.438 (0.287 to 0.568); p&amp;lt;0.001) (Fig. 1C). Conclusion(s) In patients with primary MR, handgrip echocardiography unmasks marked exercise-induced increases of MR severity in every fifth patient independent of the severity at rest. A relevant number of patients with non-severe MR at rest developed severe MR during exercise. Dynamic MR is associated with bi-atrial dilatation, increased mitral annulus diameter, and pathology of the anterior mitral valve leaflet. Moreover, changes in MR severity during handgrip exercise correlate with changes in pulmonary pressures. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): This work was supported by the Forschungskommission of the Medical Faculty of the Heinrich Heine University Düsseldorf Maximilian Spieker for a Clinician Scientist Track.

Extent and determinants of pulmonary hypertension unmasked by handgrip exercise in patients with mitral regurgitation

Abstract Background Pulmonary hypertension (PH) is an established prognosticator in patients with mitral regurgitation (MR). However, a substantial proportion of patients with MR presents without PH at rest but may develop PH during exercise. Previous studies already demonstrated the prevalence and prognostic impact of PH during bicycle exercise. Until now, there are no data on exercise PH assessed by handgrip exercise. Purpose To assess prevalence and predictors of exercise-induced PH during handgrip exercise in patients with MR. Methods We prospectively included patients with MR and at least mild severity that underwent echocardiography at rest and during three minutes of handgrip exercise according to a standardized protocol. PH was defined by systolic pulmonary artery pressure (SPAP) &amp;gt;50 mmHg. Results The final patient cohort included 371 patients with primary and secondary MR. Mean age was 74±11 years, 48% were female and 59% had atrial fibrillation. Median NT-proBNP was 2017 (845–4976) ng/l. Thirty-eight percent of patients had primary MR, while 62% presented with secondary MR. At rest, MR severity was graded as mild in 56% of patients, moderate in 32% and severe in 12%. Mean SPAP at rest was 40±12 mmHg, and 63 patients (17%) revealed PH at rest (SPAP &amp;gt;50 mmHg). Handgrip exercise leads to an increase in SPAP by 7±12 mmHg. Thus, during exercise 137 patients (37%) exhibited SPAP &amp;gt;50 mmHg. Together, 110 patients (32%) of those patients without PH at rest, showed PH during handgrip exercise. Left- (OR 1.021 (1.010 to 1.033); p&amp;lt;0.001), and right atrial volume index (OR 1.020 (1.009 to 1.035); p&amp;lt;0.001), RV fractional area change (OR 0.972 (0.949 to 0.994); p=0.013), tricuspid regurgitation (OR 1.863 (1.434 to 2.445); p&amp;lt;0.001) and SPAP at rest (OR 1.131 (1.100 to 1.167); p&amp;lt;0.001) were the predictors of exercise PH. Moreover, there was a linear correlation between the change in MR severity assessed by effective regurgitant orifice area (r=0.359; p&amp;lt;0.001) and regurgitation volume (r=0.370; p&amp;lt;0.001) and the change in SPAP. Patients with exercise PH and patients with PH at rest were more symptomatic according to NYHA functional class than patients without PH (p=0.008) (Fig. 1). Conclusion(s) In patients with primary and secondary MR, handgrip echocardiography unmasks exercise PH in every third patient without PH at rest. Bi-atrial dilatation, right ventricular function, tricuspid regurgitation and SPAP at rest were predictors of exercise PH, while changes in MR severity during handgrip exercise correlate with changes in pulmonary pressures. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): This work was supported by the Forschungskommission of the Medical Faculty of the Heinrich Heine University Düsseldorf to Maximilian Spieker for a Clinician Scientist Track.

The "Mickey Mouse Heart"

The "Mickey Mouse Heart" Abstract. The "Mickey Mouse Heart" describes patients with a combination of HFpEF, massive biatrial dilatation and dilatation of the mitral- and tricuspid annulus, atrial fibrillation and severe mitral- and tricuspid regurgitation. Most frequently elderly patients with HFpEF are affected by this syndrome. The underlying molecular mechanisms or risk factors for the typical biatrial dilatation are unknown. The guideline-directed heart failure therapy as well as the evaluation of early rhythm control for atrial fibrillation and the treatment of mitral- and tricuspid regurgitation are recommended.

Heart Failure Due to Cardiac Transthyretin Amyloidosis

A 90-year-old male patient presented with complaints and physical examination compatible with decompensated heart failure. Electrocardiogram showed atrial fibrillation and low voltage in limb leads, and echocardiogram identified biatrial dilatation, in addition to mild increases in ventricular wall thickness and diameter. Magnetic resonance imaging confirmed suspicion of cardiac amyloidosis. With negative serum and urinary protein electrophoresis and positive pyrophosphate cardiac scintigraphy, diagnosis of transthyretin cardiac amyloidosis was confirmed. Transthyretin gene mutations were not identified on sequencing, characterizing wild-type transthyretin [...]