Basal Wall Research Articles

358 A POSSIBLE RARE CASE OF VENTRICULAR LIPOMATOSIS: A STIMULUS FOR A PANORAMA OF FATTY CARDIAC LESIONS

Abstract We exhibit the case of a 75 years-old man who undertook cardio-oncological assessment for Non-Hodgkin Lymphoma: baseline echo revealed typical features of lipomatous hypertrophy of the interatrial septum (LHIS) and an apical hypoechoic left ventricle (LV) lesion. Five years before, the patient performed MRI (Magnetic Resonance Imaging), which confirmed LHIS, but superabundant epicardial fat extended to the right ventricle and the apical junction between the two ventricles, both characterized by normal ejection fraction, and surrounded the origin of the pulmonary trunk. LV lateral and inferior walls presented lipomatous metaplasia foci and no history or ECG sign of previous myocardial injury could be pointed out. A slight band of late gadolinium enhancement at the basal infero-lateral wall of LV was highlighted. The patient referred episodes of fainting: the Holter-ECG showed pronounced bradycardia which couldn't be explained by other secondary causes. So a bicameral pace-maker was implanted. During our ambulatory evaluation, we decided to repeat MRI, which showed no difference in comparison with the previous one, so excluding malignant degeneration of the fatty mass surrounding the heart. Despite older age and the lack of sudden cardiac death family history we searched for pathognomonic arrhythmogenic cardiomyopathy (ACM) mutations. In consideration of the new evidence of the metabolic risk associated with abundant epicardial fat (EF) we asked for updated laboratory dosage of plasma glucose, LDL cholesterol and triglycerides and we recommended stricter control of blood pressure. From 4% to 52% of cardiac mass is made up of fat but only EF interacts with myocardium and coronary arteries. Anomalous growth of fat in the heart can present itself as LHIS, excessive lipomatous infiltration or cardiac lipomas (real capsulated tumors) but also ACM is histologically characterized by fibrofatty replacement of cardiomyocites. Overabundant and inflammed EF is now considered a surrogate of metabolic risk and is associated with multivessel coronary disease, the finding of chronic total occlusions (CTO) and microvascular angina. Identifying fatty heart lesions and defining their clinical and prognostic role remains an intriguing challenge. We suggest a general binary way to follow: the “mechanic one” for lipomas or lipomatous infiltration, that is paying attention to compression phenomena or the generation of arrhythmias, and the “metabolic one”, in other words optimizing cardiovascular prevention when EF is too much. A careful research of imaging features of ACM can't be omitted.

66 MYOCARDIAL EDEMA CONTRIBUTES TO INTERSTITIAL EXPANSION AND ASSOCIATES WITH MECHANICAL AND ELECTROCARDIOGRAPHIC CHANGES IN TAKOTSUBO SYNDROME: A CMR T1 AND T2 MAPPING STUDY

Abstract Background myocardial edema has a central role in takotsubo syndrome (TTS). Relationships between edema, mechanical and electrical abnormalities in TTS are still elusive. Methods n=32 hospitalized TTS patients and n=23 controls were analyzed. We performed cardiac magnetic resonance (CMR) with tissue mapping for the assessment of edema and interstitial expansion, global longitudinal (GLS) and circumferential strain (GCS) were evaluated by CMR feature-tracking. Twelve-lead ECGs were recorded at the time of CMR in TTS patients, aVR voltage, negative T-wave voltage and QTc length were calculated. Results mean age of the TTS cohort was 72±12-year-old, 94% women, undergoing CMR at a median of 4 days (interquartile range 2, 6) after hospital admission. Compared with controls, patients had increased LV mass (55±11 g/m2 vs 46±8 g/m2, p<0.001), worse left ventricular ejection fraction (LVEF), GLS and GCS (all p<0.001), higher septal native T1 (1116±73 msec vs 970±23 msec, p<0.001), T2 (56±5 msec vs 46±2 msec, p<0.001) and extra-cellular volume (ECV) fraction (32±5% vs 24±1%, p<0.001). TTS patients had higher apicobasal gradient of T2 values (12±6 msec vs 2±6 msec, p<0.001), basal LV wall displayed higher native T1, T2 and ECV (all p<0.002) but similar circumferential strain against controls (-23±3% vs -24±4%, p=0.351). In the TTS cohort, septal T2 value showed significant correlations with native T1 (r=0.609, p<0.001), ECV (r=0.689, p<0.001), LVEF (r=-0.459, p=0.008) and aVR voltage (r=-0.437, p<0.05). Negative T waves voltage and QTc length significantly correlated with apicobasal T2 mapping gradient (r=0.499, p=0.007 and r=0.372, p=0.047 respectively) but not with other tissue mapping measurements. Conclusions CMR T1 and T2 mapping demonstrated increased myocardial water content conditioning interstitial expansion in acute TTS. Myocardial edema was detected even outside areas of abnormal wall motion, its burden and distribution associated with mechanical and electrocardiographic changes, making it a potential prognostic marker and therapeutic target in TTS.

584 AN UNUSUAL CASE OF A STRESSED HEART

Abstract A 53-year-old woman was admitted to our emergency department complaining severe stabbing and oppressive retrosternal pain radiating posteriorly to left scapulae and left arm The onset was followed by general malaise, nausea, vomiting which she attributed to a period of intense emotional stress. She denied common cardiovascular risk factors, but in 2019 had a suspected acute coronary syndrome with subsequent finding of normal coronary artery at angiography in another institute. Her past medical history included severe asthma on multiple drug therapy with regular follow-up. Physical exam was normal. Electrocardiogram (ECG) showed subtle diphasic-negative T wave in anterior leads with mild ST depression in V4-V5, with patient still symptomatic. Lab examinations showed significantly increased troponins levels (hsTnI 664 ng/L, reference < 34 ng/L) with other lab values within normal range. A transthoracic echocardiogram at the bedside revealed mildly reduced systolic function with hypo-akinesis of the mid-apical anteroseptal and anterior left ventricular myocardial wall. Therefore, acute coronary syndrome was suspected and heparin and aspirin at a loading dose were administered. Still symptomatic, the patient was admitted in the ICU. In the same day a left heart catheterization revealed normal aspect of epicardial coronary arteries and ventriculography showed preserved basal and apical myocardial wall motion but hypo-akinesis of mid-cavitary walls, suggesting a possible mid-ventricular variant of Takotsubo syndrome (Figure 1). The patient was still symptomatic for stabbing chest pain after coronarography. To rule out the suspicion of acute aortic syndrome and pulmonary embolism, she also underwent a contrast-enhanced chest CT scan, that was fortunately normal. During her hospital stay, the patient had no arrhythmic and hemodynamic complications. Peak troponin level was 5027 ng/L. At serial ECGs, she developed diffuse deep T-wave inversion and a mild prolonged QT interval. Echocardiogram was repeated after 4 days showing complete normalization of left ventricular wall motion and restored ejection fraction also. From the traditional “apical ballooning” Takotsubo Syndrome with apical akinesia and basal hypercontractility, different variants have been described. The mid-ventricular variant, seen in our patient was found in only 14.6% of the cases in the International Takotsubo Registry, followed by basal “reverse” and focal variants (2.2% and 1.5%, respectively).

48 A CASE OF RECURRENT VENTRICULAR TACHYCARDIA IN A PATIENT WITH GRANULOMATOSIS WITH POLYANGIITIS WITH A CHALLEGING IDENTIFICATION OF ARRHYTHMIC SUBSTRATE: THE ROLE OF MULTIMODALITY IMAGING

Abstract Background Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. Cardiac involvement is rare and generally includes pericarditis, myocarditis and aortic valve disease. We report a case of recurrent sustained ventricular tachycardia (VTs) in a patient with GPA. Case summary A 67-year-old man with past medical history of GPA presented palpitations, chest pain and episodes of presyncope. Electrocardiographic tracing upon ED presentation showed sustained monomorphic VT (fig.1) associated with global hypokinesia of the left ventricle and moderately reduced ejection fraction at transthoracic echocardiography (TTE). As the patient was hemodynamically stable, prompt treatment with amiodarone was adopted with successful recovery of sinus rhythm. After a few hours, TTE was normal. However, as troponin I was markedly elevated, coronarography was performed and coronary disease was ruled out. Contrast-enhanced cardiac magnetic resonance imaging (ceCMR) revealed late gadolinium enhancement in basal anteroseptal mid wall, suggestive of myocardial scar tissue with non-ischemic pattern. No sustained ventricular arrhythmias were induced during electrophysiological study (EPS), even with an aggressive protocol (up to 4 extra stimuli and ventricular refractoriness) and electro-anatomical mapping of both ventricles failed to show any arrhythmic substrate. An implantable cardioverter defibrillator (ICD) was then implanted. After 4 months the patient was admitted for multiple episodes of sustained VT (with cycle length in the ICD monitoring window) refractory to drugs and three external defibrillations. Repeated EPS failed to induce sustained arrhythmias. High-density electroanatomical mapping of the right ventricle, however, showed fragmented potentials in the mid-posterior septal wall (fig. 2-3). After myocardial biopsy of the region of interest, radiofrequency catheter ablation of the area was performed with regression of the potentials. The results of endomyocardial biopsy demonstrated fibrosis. At 5 months of follow-up, the patient didn't relapse. Discussion Multimodality imaging is of paramount importance in identifying arrhythmogenic substrate and in characterizing the specific anatomical–functional components of VT circuits. In our patient high-density electroanatomical mapping was able to identify fragmented potentials and define the ablation target area, despite the mismatch with ceCMR.

433 A PRO-ARRHYTHMIC DOUBLE HIT

Abstract A 42-year-old man went to the emergency room complaining about dyspnea and palpitations. He reported an episode of atrial fibrillation (AF) with symptoms of dizziness 8 years before. He had a family history of ischemic cardiopathy, his father has suffered from AF since a young age.Acute course: The ECG revealed AF at high ventricular frequency. The echocardiography demonstrated mild reduction of the left ventricular function (EF 45%) with diffuse hypokinesis, and mitral valve prolapse (MVP) with mild mitral regurgitation. The blood tests showed a troponin peak of 750 ng/L (n.v. 0–34 ng/L). The suspect was acute coronary syndrome. Thus, he was treated with dual antiplatet therapy and low doses of beta-blockers, and he underwent coronarography, which demonstrated no significant lesions. So, after a transesophageal echocardiography that excluded thrombosis, and a loading dose of amiodarone, a successful electrical cardioversion was performed. The discharge diagnosis was AF with mild left ventricular disfunction. Three months later: He came back to the emergency room because of suffering some days of progressive epigastric and retrosternal pain radiating to the shoulders. The ECG and the echocardiography were unchanged, the troponin-I values were 79 ng/L-74 ng/L. Therefore, he underwent cardiac magnetic resonance (CMR), which showed normal biventricular dimensions and function (LVEF 64%, LVEDV 80 ml/mq), MVP with mitral annular disjunction (MAD) and curling of the infero-lateral basal wall (Figure A). The native T1, T2 and ECV values were increased, and there was a wide left ventricular subepicardial late gadolinium enhancement (LGE). The conclusion was MVP and active myocarditis. Moreover, he underwent endomyocardial biopsy that revealed focal lymphocytic infiltration, PCRs for common cardiotropic viruses or bacteria were negative. Six months later: The basal ECG was characterized by low QRS voltages in limb leads (Figure B). The CMR revealed a wide left ventricular non-ischemic fibrosis with a ring-like pattern (Figure C). The 24-hours Holter ECG showed premature ventricular beats with a right bundle branch block/superior and inferior axis morphology (Figure D), in keeping with MVP. The next step was the genetic testing that resulted positive for a Filamin-C (FLNC) mutation associated with cardiomyopathy. The final diagnosis was “Hot-phase of Left Ventricular Arrhythmogenic Cardiomyopathy (ACM), MVP and paroxysmal AF”. Discussion: The ACM is a rare hereditary heart muscle disease, and the “hot phase” represents its uncommon clinical presentation. Mutations in FLNC gene could be involved in the pathogenesis of ACM. Moreover, there are few cases in literature about the correlation between FLNC mutations and MVP. FLNC mutation may be a cross-sectional substrate for both these pro-arrhythmic conditions.

347 ARRHYTHMIC MITRAL VALVE PROLAPSE AND MITRAL ANNULUS DISJUNCTION: WHEN PRIMARY PREVENTION IMPLANTATION OF ICD IS POSSIBLE?

Abstract Mitral valve prolapse (MVP) is the most common valvular heart disease. Although MVP is generally considered benign, it can be associated with important complications, including sudden cardiac death (SCD). MVP is often related to mitral annular disjunction (MAD), a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. It is associated with an increased risk of arrhythmias and is an integral component of the arrhythmic MVP (AMVP) complex. Here we present a case report of a patient with MAD, Barlow's syndrome with bileaflet mitral prolapse and a history of recurrent non sustained ventricular tachycardia (NSVT) who underwent subcutaneous cardioverter defibrillator (ICD) implant in primary prevention. A 49 year old woman, with a history of moderate mitral regurgitation and posterior leaflet prolapse, was sent to our hospital by her cardiologist for the evidence on holter ecg of several tracts of non sustained ventricular arrhythmias. Transesophageal echocardiogram revealed the presence of moderate mitral regurgitation, bileaflet prolapse (mainly of the redundant posterior leaflet), dilation of the left atrium and positive Pickelhaube sign, suggestive of MAD. After few days was performed cardiac magnetic resonance imaging with evidence of mitral annulus disjunction associated with bileaflet prolapse and moderate mitral regurgitation. Delayed gadolinium enhanced (DGE) signal was found in the posterior basal wall. During hospitalization, she presented daily episodes of high frequency (>180 bpm) NSVT with right bundle block (RBBB) morphology and several premature ventricular complex. It was therefore decided to discharge the patient with Life Vest and five months later, after asking second opinion to an Italian high specialization center, we decided to place a subcutaneous ICD in primary prevention for the high arrhythmic risk of the patient. Arrhythmic mitral valve prolapse (AMVP) is an estimated cause of sudden cardiac death. It is known that patients with MVP and MAD have a high arrhythmic risk but there aren't trials that demonstrate the benefits of primary ICD implantation. The recent “EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction “ affirms that can be reasonable, primary ICD implant, in patients with AMVP, 1 high risk features (VT (hemodynamically tolerated), NSVT, unexplained syncope) and 2 or more phenotypic risk features (—T-wave inversion in the inferior leads, repetitive documented polymorphic PVCs, MAD phenotype, redundant MV leaflet, enlarged left atrium or ejection fraction≤50%, LGE). For this reason, our patient underwent subcoutanues ICD implant. Therefore, we believe that further studies, to develop guidelines for risk stratification of patients with evident MVP, would be beneficial in reducing the incidence of potentially fatal events in these patients.

Reverse Takotsubo Stress Cardiomyopathy During Liver Transplantation

Reverse Takotsubo Stress Cardiomyopathy During Liver Transplantation

Lodging resistance of rice plants studied from the perspective of culm mechanical properties, carbon framework, free volume, and chemical composition

In this study, four varieties of rice were cultivated on the same farmland under same conditions and for same duration. However, their lodging resistance was found to be obviously different from each other. Herein, three key factors that highly influenced the lodging resistance were identified. First, in terms of morphological trait, in contrast to the generally believed theory that the overall thickness of the fresh culm wall governs the strength, the thickness of the depressed region of the dried basal culm wall largely determined the mechanical properties by acting as the weak link. This depressed region represents the vulnerable part with high syneresis rate. Second, the culm and its carbon framework exhibited sufficient strength and rigidity for both support and stability of the rice stem. The constraint of high lodging resistance of rice plants is attributed to the culm flexibility. Furthermore, the results of the positron annihilation lifetime spectroscopy corroborate that the most amorphous part and the highest-fraction free volume in the culm carbon framework were found for samples that exhibited high lodging resistance. This result confirmed the significant influence of the culm flexibility on lodging resistance. Third, a higher level of nitrogen element content in the basal culm can benefit its growth and development, which may contribute to an increase in lodging resistance of rice plants.

Abstract 12215: A Case of Post-Vaccine Myocarditis Mimicking Long Covid

Myocarditis is a rare complication following mRNA-based COVID vaccinations. While the risk appears to be greatest in adolescent males, increased rates of myocarditis following COVID vaccination have been documented in both sexes and across multiple age groups.In this case, a 59-year-old woman with history of mild COVID infection developed unexplained fatigue, diminished exercise capacity, and intermittent chest discomfort several days after receiving her first dose of the Pfizer-BioNTech COVID-19 vaccine. She was seen in the emergency department at the onset of symptoms and found to have a dynamic troponin elevation peaking at 177 ng/L. A comprehensive workup including regadenoson stress testing, CT scan of the chest with contrast, and ambulatory cardiac monitoring failed to demonstrate an etiology. She subsequently received a second dose of the COVID-19 vaccine which resulted in worsening cardiopulmonary symptoms that persisted over the next several months. Her symptoms were initially attributed to long COVID, and she was provided supportive care and an SSRI for anxiety. After reevaluation in the Cardiology clinic, the patient underwent cardiac MRI revealing edema in the basal lateral wall and late gadolinium enhancement in a subepicardial distribution, suggestive of myocardial inflammation. She was treated with prednisone 50 mg daily and colchicine 0.6 mg twice daily resulting in resolution of chest pain. A repeat MRI performed two weeks later showed complete resolution of myocardial edema with residual subepicardial LGE in the basal lateral wall consistent with prior myocarditis.This case demonstrates the pitfalls of relying on a diagnosis of ‘long COVID’ to explain concerning cardiac symptoms of uncertain etiology and highlights post-vaccine myocarditis as an important differential in the COVID-19 era. At this time, it is unclear how many patients experience persistent cardiopulmonary symptoms following COVID vaccination and whether a subset of these patients have undiagnosed myocarditis. It is important for clinicians to be alert to the possibility of post-vaccine myocarditis in patients presenting with persistent symptoms following vaccination, regardless of demographic profile.

Abstract 15827: A Case of Ventricular Arrhythmia as Presentation for Cardiac Involvement of Polymyositis

Introduction: Polymyositis is an autoimmune systemic disease which is characterized by chronic weakness and inflammation in skeletal muscle predominantly. Cardiac involvement has been reported and is a poor prognostic factor. Clinical course 61-year-old male with biopsy proven polymyositis with history of cardiac arrest secondary to ventricular fibrillation and dual chamber automatic implantable cardioverter defibrillator (AICD) implanted for secondary prevention. He had recurrent PVC-induced ventricular fibrillation causing AICD shocks. Eventually, he underwent electrophysiology study and successful ablation of PVC targeted to the basal lateral left ventricle.Transthoracic echocardiogram showed unremarkable systolic and akinetic basal inferolateral wall segment.He underwent PET-CT of myocardium which showed focal FDG uptake of the lateral basal left ventricular could indicate focal myocarditis.Endomyocardial biopsy did not show any evidence of myocarditis.Eventually he had recurrent rapid monomorphic ventricular tachycardia and was briefly placed on amiodarone. He was placed on immunosuppressive therapy managed by Rheumatology with etanercept, rituximab and IVIG. No recurrence of ventricular arrythmia has occurred since. Amiodarone has been discontinued. Discussion: Myocardial involvement of polymyositis is not uncommon and has been noted to increase morbidity and mortality.The most common reported manifestations include congestive heart failure, conduction abnormalities which can range from ventricular arrhythmia to complete heart block and coronary artery disease. ConclusionIt is very important to recognize polymyositis as a cause for ventricular arrhythmias in the right clinical setting as management with immunosuppressive therapy can decrease the burden in ventricular arrhythmias.

Abstract 13713: Myocarditis and Coronary Vasospasm in a Patient With Antiphospholipid Syndrome and MINOCA

Background : The prevalence of MINOCA in patients with antiphospholipid syndrome (APLS) is estimated to be 20%. Frequent causes in this population include coronary vasospasm, microcirculatory dysfunction and takotsubo cardiomyopathy. Objective : This case reveals myocarditis and coronary vasospasm as newly described complications of APLS associated MINOCA and illustrates the use of CMR to differentiate ischemic injury from myocarditis in this population. Case : A 40-year-old female presented with two-days of left-sided, substernal, pleuritic, and radiating chest pain associated with dyspnea. Admission ECG showed 0.5mm ST elevations in the inferior leads with ST depression in aVL. She had an initial high sensitivity troponin of 1531 ng/L, NT-proBNP of 18,357 pg/mL, baseline creatinine of 1.53 mg/dL, platelets of 95,000/μL, hemoglobin of 8.4 g/dL, and a subtherapeutic INR of 1.1 on warfarin. An emergent catheterization showed no coronary artery disease or evidence of SCAD. TTE showed hypokinesis of inferior and lateral walls with an LVEF of 45% and CTA chest ruled out pulmonary embolism. The patient’s admission was complicated by rising troponins to a peak of 9790 ng/L, oliguric renal failure requiring continuous renal replacement therapy, and fever. Patient was medically managed for presumed myocardial infarction and repeat TTE two days after catheterization showed a recovered LVEF of 59%. CMR showed a transmural infarct of the basal and inferior walls with signs of microvascular obstruction and diffuse patchy late gadolinium enhancement of the LV midwall, consistent with myocarditis. An infectious workup for myocarditis was negative. Patient was discharged home on warfarin, aspirin, and amlodipine. Conclusion : MINOCA is more common in patients with APLS, although coronary vasospasm and myocarditis have not been described in the same patient. CMR is useful to differentiate the etiology of ischemic injury following successful diagnosis of MINOCA.

Abstract 15095: A Rare Cardiomyopathy Caught Offsides: Discovery of a New Phenotype

A 51-year-old male with Wolff-Parkinson-White (WPW) syndrome, atrial flutter, and third-degree heart block necessitating pacemaker placement presented with asymptomatic atrial flutter. The patient’s father and paternal aunt were diagnosed with WPW and died suddenly at approximate ages of 40 and 60 years, respectively. An echocardiogram showed RV dilation and moderate to severe RV systolic dysfunction with normal LV ejection fraction. Cardiac CT demonstrated thinning of the RV free wall and free wall outpouchings during systole (Figure), as well as moderate asymmetric LV hypertrophy, aneurysmal changes of the basal inferior wall, and dyskinesis of the LV apex. At this time, the differential for RV dysfunction included group 1 pulmonary hypertension, ischemic cardiomyopathy, sarcoidosis, and arrhythmogenic right ventricular cardiomyopathy (ARVC). Subsequent testing excluded all diagnoses other than ARVC. Additionally, 2010 Task Force Criteria for ARVC were not met. However, genetic testing revealed p.Arg302Gln mutation in the PRKAG2 gene. AMP-activated protein kinase (AMPK) is a highly conserved kinase encoded by the PRKAG2 gene that is responsible for controlling cellular energy homeostasis. This PRKAG2 mutation, within the spectrum of genetic mutations resulting in the rare autosomal dominant PRKAG2 syndrome, is characterized by glycogen storage cardiomyopathy and a familial form of WPW, supraventricular arrhythmias, and left ventricular hypertrophy. To the best of our knowledge, this is the first report of PRKAG2 syndrome with prominent abnormalities of the RV, and it expands the scope of PRKAG2 syndrome. Management included implantation of an AICD and ongoing careful monitoring for arrhythmia and heart failure. The patient’s siblings and children were referred for genetic counseling. This case highlights the importance of a broad differential diagnosis and exhaustive evaluation of patients presenting with right ventricular abnormalities.

Abstract 15548: Rare Association of Arrhythmogenic Left Ventricular Cardiomyopathy Phenotype of Titin Mutation of Uncertain Significance

Case Presentation: A 67-year-old female presented to the office with exertional dyspnea, atypical chest pain, palpitations, and dizziness. She had a past medical history of hypertension, hyperlipidemia, mitral valve prolapse, and moderate mitral regurgitation. Her EKG identified multiple PVCs requiring the placement of a Holter monitor, a PVC burden of 31,441 beats (15%) was captured with variable morphology. TTE showed mild global hypokinesis of the left ventricle and ejection fraction (EF) of 40%. Coronary arteries were normal on cardiac catheterization. Cardiac magnetic resonance revealed epicardial late gadolinium enhancement suggestive of fatty infiltration of the distal lateral wall of the left ventricle (LV) indicative of Arrhythmogenic left ventricular cardiomyopathy (ALVC). The basal anterolateral wall of the LV appeared thin and dyskinetic, and the right ventricle was normal in size and function. Optimal medical therapy was initiated with metoprolol, sacubitril/valsartan, dapagliflozin, and spironolactone. A dual-chamber ICD was placed for primary prevention. Over time with medical management, her PVC burden reduced, and LVEF improved to 50%. A multigene panel for cardiomyopathies identified a mutation in titin (TTN) gene variant of unknown significance. Discussion: Previously thought to affect only the right ventricle, arrhythmogenic cardiomyopathy (ACM) is a rare genetic disease characterized by fibrofatty replacement of the ventricular myocardium. ALVC refers to the predominant LV dominant phenotype of this disease. The most frequent genetic mutations occur in the genes plakophilin (PKP2), desmoplakin (DSP), and desmoglein (DSG2). However, we report a case of ALVC in the setting of titin (TTN) gene variant, which is a gene usually implicated in dilated cardiomyopathy. However, the clinical significance of the TTN gene variant identified in our patient remains unclear as more studies are needed to link it to the phenotypic findings.

Different mechanisms of mitral regurgitation in hypertrophic cardiomyopathy: A clinical case and literature review.

Abnormalities of the mitral valve (MV) apparatus are typical features of hypertrophic cardiomyopathy (HCM). These abnormalities include leaflet elongation, thick leaflets, displacement of papillary muscle, and systolic anterior motion (SAM) of the MV anterior leaflet. Mitral valve chordal rupture associated with HCM is a rare but serious issue capable of change the clinical apparence and the prognosis of the patient. A 57-year-old lady with a history of diabetes, dyslipidemia, and a previous single episode of atrial fibrillation (treated with pharmacological cardioversion), presented to the Emergency Department for worsening dyspnea (New York Heart Association Classification class IV). A trans-thoracic echocardiogram (TTE) showed a significant, septal, and asymmetric left ventricular hypertrophy (basal anteroseptal wall diastolic thickness of 19 mm) with normal left ventricle systolic function. A SAM of AML was evident together with a left ventricular outflow tract gradient of 56 mmHg at rest, rising to 136 mmHg during the Valsalva maneuver. In addition, there was evidence of moderate to severe mitral regurgitation (MR) with an anteriorly directed jet, not very typical of MR related to SAM. A 2D-3D trans-esophageal echocardiogram (2D-3D TEE) revealed a combined MR mechanism based on PML degenerative prolapse with P2-flail from ruptured chordae with related eccentric anteriorly directed regurgitant jet, together with a second regurgitant posteriorly directed jet, related to SAM of AML. The patient underwent MV repair together with septal myectomy, with a good final outcome. Pre-operative echocardiography (both TTE and 2D-3D TEE) is an essential tool in order to detect different MV abnormalities in patients with HCM. These types of patients should never be treated by septal reduction alone. Surgical MV repair or replacement, together with septal myectomy, may be the preferred approach.

An uncommon case of complete AV block

BackgroundGranulomatosis with polyangiitis (GPA) is a rare systemic inflammatory disorder characterized by vasculitis of the small vessels, as well as necrotizing granulomatous lesions, affecting mainly upper and lower respiratory tracts, lungs and kidneys. Cardiac involvement has traditionally been a rare manifestation of GPA, with misleading clinical presentation until late stages. Cardiac conducting tissue involvement is a rare and potentially life-threatening complication.Case presentationWe report the case of a 45-year-old man diagnosed with GPA with typical symptoms, but also complete atrioventricular (AV) block at the onset of the disease. The echocardiogram was unremarkable but the cardiac magnetic resonance (CMR) showed evidence of inflammation of the basal and septal ventricle walls. Despite effective immunosuppressive therapy, a permanent pacemaker was required for recurring complete AV block.DiscussionConduction system abnormalities are a rare manifestation of GPA, due to granulomatous lesions within the conduction system, or arteritis of the atrioventricular nodal artery. Patients are often asymptomatic, so careful and regular screening for cardiac involvement in this multi-system condition is required, often with echocardiogram, electrocardiogram (ECG) monitoring and CMR. Early immunosuppressive treatment may reverse a complete AV block but a pacemaker implantation may sometimes be necessary.

Migration and 3D Traction Force Measurements inside Compliant Microchannels.

Cells migrate in vivo through channel-like tracks. While polydimethylsiloxane devices emulate such tracks in vitro, their channel walls are impermeable and have supraphysiological stiffness. Existing hydrogel-based platforms address these issues but cannot provide high-throughput analysis of cell motility in independently controllable stiffness and confinement. We herein develop polyacrylamide (PA)-based microchannels of physiological stiffness and prescribed dimensions for high-throughput analysis of cell migration and identify a biphasic dependence of speed upon confinement and stiffness. By utilizing novel four-walled microchannels with heterogeneous stiffness, we reveal the distinct contributions of apicolateral versus basal microchannel wall stiffness to confined versus unconfined migration. While the basal wall stiffness dictates unconfined migration, apicolateral stiffness controls confined migration. By tracking nanobeads embedded within channel walls, we innovate three-dimensional traction force measurements around spatially confining cells at subcellular resolution. Our unique and highly customizable device fabrication strategy provides a physiologically relevant in vitro platform to study confined cells.

Evaluation of Right Ventricular Function and Myocardial Microstructure in Fetal Hypoplastic Left Heart Syndrome.

Right ventricular (RV) function is one of the critical factors affecting the prognosis of fetuses with hypoplastic left heart syndrome (HLHS). Our study objectives included assessment of cardiac function and comprehensive measurement of cardiac microstructure. We retrospectively studied 42 fetuses diagnosed as HLHS by echocardiography. Myocardial deformation of the right ventricular wall was calculated automatically in offline software. Postmortem cardiac imaging for three control fetal hearts and four HLHS specimens was performed by a 9.4T DTI scanner. Myocardial deformation parameters of the RV (including strain, strain rate, and velocity) were significantly lower in HLHS fetuses (all p < 0.01). FA values increased (0.18 ± 0.01 vs. 0.21 ± 0.02; p < 0.01) in HLHS fetuses, but MD reduced (1.3 ± 0.15 vs. 0.88 ± 0.13; p < 0.001). The HLHS fetuses’ RV lateral base wall (−7.31 ± 51.91 vs. −6.85 ± 31.34; p = 0.25), middle wall (1.71 ± 50.92 vs. −9.38 ± 28.18; p < 0.001), and apical wall (−6.19 ± 46.61 vs. −11.16 ± 29.86, p < 0.001) had HA gradient ascent but HA gradient descent in the anteroseptal wall (p < 0.001) and inferoseptal wall (p < 0.001). RV basal lateral wall HA degrees were correlated with RVGLS (R2 = 0.97, p = 0.02). MD values were positively correlated with RVGLS (R2 = 0.93, p = 0.04). Our study found morphological and functional changes of the RV in HLHS fetuses, and cardiac function was related to the orientation patterns of myocardial fibers. It may provide insight into understanding the underlying mechanisms of impaired RV performance in HLHS.

Takotsubo Cardiomyopathy Following Traumatic Hand Amputation: A Case Report.

IntroductionTakotsubo or stress cardiomyopathy is a syndrome of transient left ventricular systolic dysfunction seen in the absence of obstructive coronary artery disease.Case ReportWe describe a case of stress cardiomyopathy diagnosed in the emergency department (ED) using point-of-care ultrasound associated with traumatic hand amputation. The patient suffered a near-complete amputation of the right hand while using a circular saw, subsequently complicated by brief cardiac arrest with rapid return of spontaneous circulation. Point-of-care ultrasonography in the ED revealed the classic findings of takotsubo cardiomyopathy, including apical ballooning of the left ventricle and hyperkinesis of the basal walls with a severely reduced ejection fraction. After formalization of the amputation and cardiovascular evaluation, the patient was discharged from the hospital in stable condition 10 days later.ConclusionEmergency physicians should be aware of the possibility of stress cardiomyopathy as a cause for acute decompensation, even in isolated extremity trauma.

P299 CONDUCTION DISORDERS AS THE FIRST HALLMARK OF ISOLATED CARDIAC SARCOIDOSIS IN A HIGHLY ACTIVE INDIVIDUAL: A CASE REPORT

Abstract A 45–year–old highly active Caucasian individual was admitted to the emergency department complaining of slightly reduced physical performances: he reported a lack of increase of heart rate beyond 80–100 bpm at fitness tracker monitoring during physical activity during the previous week. He used to train 3–4 times/week. He had no previous medical concerns and he didn’t take any medication. Basal electrocardiogram (ECG) showed 2:1 atrioventricular block (AVB) and left axis deviation, with the exception of his 2:1 AVB, the other features on the ECG are common training related changes. Transthoracic echocardiogram was normal. Treadmill exercise test revealed paroxysmal 2:1 AVB and Mobitz I AVB at peak effort, then first degree AVB and isolated and coupled right infundibular premature ventricular complexes (PVC) in the recovery phase: exercise related AV–conduction improvement suggested supra–nodal AVB. Cardiac magnetic resonance (CMR) imaging reported two areas of late gadolinium enhancement (LGE) with midwall/subepicardial distribution: the first one in the basal anterior septal wall extending to basal anterior wall and the second one involving the basal and medium segment of inferior septum and inferior wall. LGE quantification was 6.0 g/m2 (9.5%). Short tau inversion recovery (STIR) acquisitions were negative for oedema (Figure 1). Subsequent FDG–PET imaging confirmed high uptake in the basal anterior septal wall, the anterior wall and in the basal inferior septal area (Figure 2). No other organ involvement was detected. Considering the FDG–PET, LGE and AV–conduction abnormalities, the patient was clinically diagnosed with symptomatic isolated CS. However, a definitive histologic diagnosis was not possible as no myocardial biopsy was performed. High dose corticosteroid therapy was startedand after few days of treatment ECG monitoring revealed an imrovement of cardiac conduction. The treadmill test was repeated and showed conduction improvement during effort, however the test was suspended due to the occurrence of ventricular couples and ventricular bigeminy during effort. For arrhythmic risk assessment, electrophysiological study (EPS) was performed and no arrhythmias were induced. We decided to defer ICD implantation. However, a loop–recorder for continuous arrhythmias monitoring was implanted. At three months, a new CMR was performed and was identical to the previous one, with no significant LGE reduction or increase detected.

P78 AN ANTEGRADE RECANALIZATION OF LEFT ANTERIOR DESCENDING CHRONIC TOTAL OCCLUSION WITH ORBITAL ATHERECTOMY IN PATIENT WITH SEVER LEFT VENTRICULAR DISFUNCTION

Abstract We share the case of a 71–year old man, affected by hypertension, presented in Emergency Room (ER) with chest pain. No clinical evidence of heart failure. EKG showed fast atrial fibrillation with QS complex and ST elevation in precordial leads. Echocardiogram confirmed sept and apex akinesia. Troponin I peak was 72pg/ml. In ER electrical cardioversion was efficacy performed. He was referred for invasive coronary angiography, that showed a left anterior descending (LAD) chronic total occlusion (CTO), with Rentrop grade 2 collaterals providing retrograde flow from the right coronary artery (RCA), a severe stenosis of ramus intermedius (RI) and a moderate stenosis of circumflex. A stress–echocardiogram demonstrated vitality of medio–basal anterior wall. Subsequent, recanalization procedure of LAD CTO was planned. J–CTO score was 1 (tapered calcific, lesion, &amp;lt;20mm). double radial access and ante grade approach was chosen. An EBU3.5 guiding catheter was engaged in the left coronary artery, and 8000 units of heparin administered. The iFR of Cx was 0.99, indicative of non significant myocardial ischemia. During hemodynamic evaluation of RI, a ventricular fibrillation occurred, treated by DC–SHOCK and PCI with stenting with no polymer, eluted with Biolimus A9 (3.0x24mm) with good result. The occlusion was crossed via an antegrade approach with an Asahi Gaia II wire and with microcatheter support. Gaia II was changed in favor of Asai Sion blu, and the IVUS catheter was enable of cross the lesion. Efficacy orbital atherectomy with Diamondback 260 was performed (5 ante and retro grade run at 80Krpm and a retro grade run at 120 Krpm. Ivus was able to cross the lesion. Efficacy pre–dilatation with semi and non compliant balloon (2.5–3.5mm) was performed and then stented with a 3.0 × 36 mm Bioloimu A9–eluting stent. Finally, IVUS confirmed correct stent expansion, achieving an excellent angiographic result. Patient was discharged with triple anti–thrombotic therapy, with indication to “life jacket” till the assessment at 1 month.