Inferolateral Wall Research Articles

Optimal Echocardiographic Parameters to Improve the Diagnostic Yield of Tc-99m-Bone Avid Tracer Cardiac Scintigraphy for Transthyretin Cardiac Amyloidosis.

Echocardiographic deformation-based ratios and novel multi-parametric scores have been suggested to discriminate transthyretin cardiac amyloidosis (ATTR-CM) from other causes of increased left ventricular wall thickness among patients referred for ATTR-CM evaluation. Their relative predictive accuracy has not been well studied. We sought to (1) identify echocardiographic parameters predictive of ATTR-CM and (2) compare the diagnostic accuracy of these parameters in patients with suspected ATTR-CM referred for technetium-99m-pyrophosphate scintigraphy. Echocardiograms from 598 patients referred to 3 major amyloidosis centers for technetium-99m-pyrophosphate to detect ATTR-CM were analyzed, including longitudinal strain (LS) analysis. Deformation ratios (septal apex to base ratio, relative apical sparing, ejection fraction to global LS), a multi-center European increased wall thickness score, and Mayo Clinic derived ATTR score (transthyretin cardiac amyloidosis score) were calculated. A logistic regression model was used to identify the parameters that best associated with a diagnosis of ATTR-CM. Comparison of the diagnostic capacity of the parameters was performed by receiver operating characteristic curves and the area under the curve (AUC). Over half of the subjects (54.2%) were diagnosed with ATTR-CM (78% were men, median age of 76 years). Age, inferolateral wall thickness, and basal LS were the strongest predictors of ATTR-CM, AUC of 0.87 (95% CI: 0.83, 0.90), superior to the increased wall thickness score AUC of 0.78 (95% CI: 0.73, 0.83; P=0.004). An inferolateral wall thickness of ≥14 mm (AUC: 0.73) was as accurate as the published cut-offs for transthyretin cardiac amyloidosis score and septal apex to base (AUC: 0.72 and 0.69, P=0.8 and P=0.1, respectively), and was superior to ejection fraction to global LS and relative apical sparing (AUC: 0.64 and 0.53, P<0.001, respectively). A cut-off of ≥-8% for average basal LS (AUC: 0.76, CI: 0.72-0.79) had a similar area under the curve to transthyretin cardiac amyloidosis score (TCAS) (P=0.2); outperforming the other indices (P<0.01). Inferolateral wall thickness and average basal LS performed as well as or better than more complex echo ratios and multiparametric scores to predict ATTR-CM.

Cardiovascular Magnetic Resonance Imaging Patterns in Rare Cardiovascular Diseases

Rare cardiovascular diseases (RCDs) have low incidence but major clinical impact. RCDs' classification includes Class I-systemic circulation, Class II-pulmonary circulation, Class III-cardiomyopathies, Class IV-congenital cardiovascular diseases (CVD), Class V-cardiac tumors and CVD in malignancy, Class VI-cardiac arrhythmogenic disorders, Class VII-CVD in pregnancy, Class VIII-unclassified rare CVD. Cardiovascular Magnetic Resonance (CMR) is useful in the diagnosis/management of RCDs, as it performs angiography, function, perfusion, and tissue characterization in the same examination. Edema expressed as a high signal in STIRT2 or increased T2 mapping is common in acute/active inflammatory states. Diffuse subendocardial fibrosis, expressed as diffuse late gadolinium enhancement (LGE), is characteristic of microvascular disease as in systemic sclerosis, small vessel vasculitis, cardiac amyloidosis, and metabolic disorders. Replacement fibrosis, expressed as LGE, in the inferolateral wall of the left ventricle (LV) is typical of neuromuscular disorders. Patchy LGE with concurrent edema is typical of myocarditis, irrespective of the cause. Cardiac hypertrophy is characteristic in hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Anderson-Fabry Disease (AFD), but LGE is located in the IVS, subendocardium and lateral wall in HCM, CA and AFD, respectively. Native T1 mapping is increased in HCM and CA and reduced in AFD. Magnetic resonance angiography provides information on aortopathies, such as Marfan, Turner syndrome and Takayasu vasculitis. LGE in the right ventricle is the typical finding of ARVC, but it may involve LV, leading to the diagnosis of arrhythmogenic cardiomyopathy. Tissue changes in RCDs may be detected only through parametric imaging indices.

Mitral Annular Disjunction Assessed Using CMR Imaging: Insights From the UK Biobank Population Study

BackgroundMitral annular disjunction is the atrial displacement of the mural mitral valve leaflet hinge point within the atrioventricular junction. Said to be associated with malignant ventricular arrhythmias and sudden death, its prevalence in the general population is not known. ObjectivesThe purpose of this study was to assess the frequency of occurrence and extent of mitral annular disjunction in a large population cohort. MethodsThe authors assessed the cardiac magnetic resonance (CMR) images in 2,646 Caucasian subjects enrolled in the UK Biobank imaging study, measuring the length of disjunction at 4 points around the mitral annulus, assessing for presence of prolapse or billowing of the leaflets, and for curling motion of the inferolateral left ventricular wall. ResultsFrom 2,607 included participants, the authors found disjunction in 1,990 (76%) cases, most commonly at the anterior and inferior ventricular wall. The authors found inferolateral disjunction, reported as clinically important, in 134 (5%) cases. Prolapse was more frequent in subjects with disjunction (odds ratio [OR]: 2.5; P = 0.02), with positive associations found between systolic curling and disjunction at any site (OR: 3.6; P < 0.01), and systolic curling and prolapse (OR: 71.9; P < 0.01). ConclusionsThis large-scale study shows that disjunction is a common finding when using CMR. Disjunction at the inferolateral ventricular wall, however, was rare. The authors found associations between disjunction and both prolapse and billowing of the mural mitral valve leaflet. These findings support the notion that only extensive inferolateral disjunction, when found, warrants consideration of further investigation, but disjunction elsewhere in the annulus should be considered a normal finding.

Myocarditis following COVID-19 vaccination in adolescents: Cardiac magnetic resonance imaging study.

IntroductionVaccination-associated myocarditis was reported following COVID-19 vaccine initially among persons aged 16 or older and recently among adolescents aged 12–15.ObjectivesTo describe the clinical and cardiac magnetic resonance (CMR) characteristics of adolescents aged 12–15 with myocarditis following the administration of the BNT162b2 mRNA COVID-19 vaccine.MethodsCMR of adolescents (age 12–15) with a clinical diagnosis of myocarditis within 42 days following the first COVID-19 vaccine were analyzed.ResultsA total of 182,605 adolescent were vaccinated, out of which 9 were diagnosed with clinically adjudicated myocarditis while CMR was performed in 5/9 patients (56%). Median age was 15 years (range 13–15), 4/5 (80%) males. All the patients we previously healthy. The ECG upon presentation was abnormal in 3/5 (60%) of patients. All cases were classified as clinically mild and no patient required inotropes or mechanical circulatory support treatment. The median follow-up time, for the 5-included patients, was 206 (IQR 192–229, range 179–233) days. During the follow-up, no re-admissions, deaths, or any other cardiac events have occurred.The median time between the diagnosis to the CMR was 104 days (range 27–149). The median left ventricular ejection fraction was within normal range 65% (range 62–69). Native T1 was available in four patients, the local T1 value was increased in three of them. T2 values were available in two patients and were all within normal range. The median late gadolinium enhancement (LGE) was 2% (range 0–6%) with inferolateral wall being the most common location (3/5). The patterns of the LGE were as following: (i) mid-wall in 3 patients; (ii) epicardial in 1-patient. LGE in the pericardium was present in 2/5 patients with pericardial effusion present in 4/5 patients with a median diameter of 4 mm (range 3–5 mm) at end-systole.ConclusionsCMR findings and clinical course of adolescents with COVID-19 vaccination associated myocarditis, are similar to those of older patients, being relatively mild and potentially implying favorable outcomes.

Dynamic Change of Left Ventricular Mechanics in Patients with Acute Myocarditis with Preserved Left Ventricular Systolic Function: A 2-Year Follow-up Study.

Acute myocarditis mimics acute coronary syndrome due to its clinical course and presentation. This study aimed to evaluate left ventricular longitudinal and circumferential functions during the acute phase and late phase of acute myocarditis with preserved left ventricular ejection fraction using 2-dimensional speckle tracking echocardiography. Forty-one consecutive acute myocarditis patients with preserved left ventricular ejection fraction confirmed by cardiac magnetic resonance imaging underwent 2-dimensional speckle tracking echocardiography within the first week of hospital admission. Findings were compared with age and sex-matched 40 healthy controls. Left ventricular mechanics of the study group were reevaluated by 2-dimensional speckle tracking echocardiography during follow- up (23.85 ± 6.65 months later). Myocardial lesions with late gadolinium enhancement on cardiac magnetic resonance imaging were mostly localized in the subepicardial layer (91.40%) and commonly observed in the inferolateral wall (42.94%). Consistent with the cardiac magnetic resonance imaging findings, 2-dimensional speckle tracking echocardiography showed the localization of the involved myocardial segments with prominent impairment in global longitudinal peak systolic strain and global circumferential strain of the inferolateral wall of the left ventricle. In the acute phase, global longitudinal peak systolic strain (-17.32 ± 2.02 vs -20.59 ± 2.38) and global circumferential strain (-22.33 ± 2.27 vs -24.85 ± 3.19) were found to be lower in patients with acute myocarditis compared to healthy controls (both P &lt; .001). While global circumferential strain was improved in the late phase compared with the acute phase (from -22.28 ± 2.32 to -22.90 ± 2.65; P = .003). Global longitudinal peak systolic strain was not significantly changed during follow-up (from -17.30 ± 2.09 to -17.62 ± 2.19; P = .072). Subtle left ventricular systolic function impairment can be detected by the 2-dimensional speckle tracking echocardiography technique in patients with acute myocarditis with preserved left ventricular ejection fraction and improvement in circumferential function could be observed during follow-up.

S3593 Eosinophilic Gastritis in Idiopathic Hypereosinophilic Syndrome: A Case Report

Introduction: Idiopathic hypereosinophilic syndrome (IHES) is a group of myeloproliferative disorders causing multi-organ dysfunction with unknown etiology. We report a case of IHES in a patient presenting with gastric wall thickening, multiple organ damage including acute coronary syndrome, encephalopathy, and multiple cortical infarcts. Case Description/Methods: A 47-year-old male patient with past medical history significant for type 2 diabetes mellitus and hypertension presented with nausea, vomiting, abdominal pain, and food intolerance for 7 days. He also complained of intermittent, substernal chest pain for 1-2 days at presentation. His labs were significant for elevated troponin of serum creatinine of 2.85mg/dL and significant leukocytosis (32.80 10^3/cmm). Absolute eosinophilic count was elevated at 14.70 10^3/cmm. Echocardiogram showed hypokinetic mid inferior and inferolateral walls. CT chest/abdomen angiogram was done for concerns of dissection. It showed diffuse gastric wall thickening with upper abdominal retroperitoneal lymphadenopathy and extensive retroperitoneal fat stranding. He was started on antiplatelet agent and heparin drip. Ischemic workup was deferred as troponin plateaued and patient had new-onset altered mental status. MRI brain showed numerous punctate cortical and subcortical foci of restricted diffusion consistent with acute watershed distribution infarcts. In view of possible need for dual anti-platelet therapy and the high concern for gastric malignancy on CT that required tissue biopsy, gastroenterology was consulted for esophagogastroduodenoscopy (EGD). It did not show any gastric mass but diffuse gastritis. Biopsy showed mild chronic gastritis and a focal area showing increased eosinophilic infiltration with eosinophilic cryptitis (Figure). An extensive workup for eosinophilia including infectious etiology returned negative. No secondary causes of hypereosinophilia were identified. Patient was started on 1mg/kg of prednisone with normalization of eosinophils to 0 within 24 hours. Steroids were tapered and patient improved clinically with resolution of altered mental status, abdominal pain, and chest pain. Follow-up EGD has been scheduled to re-evaluate eosinophilic deposits. Discussion: The stomach biopsy showing eosinophilia was the key to diagnosis. Resolution of peripheral eosinophilia with steroids confirms the diagnosis in the absence of a secondary cause. A repeat EGD with biopsy is scheduled to look for histopathological resolution.Figure 1.: Gastric mucosa with increased eosinophilic infiltration within the lamina propria and focal glandular involvement with eosinophils.

S2779 A Drug After My Own Heart: Mesalamine-Induced Perimyocarditis

Introduction: Mesalamine is a commonly used first line treatment to induce remission in mild to moderate Ulcerative Colitis (UC) that many patients tolerate well. However it does not come without risk and can rarely cause serious and life threatening cardiac and lung inflammation including myocarditis. Case Description/Methods: A 33 year old male with recently diagnosed ulcerative pancolitis presented with one day of pleuritic, positional chest pain and exertional dyspnea. Two months prior to presentation he had been diagnosed with moderate pancolonic ulcerative colitis and started on mesalamine 1.2 gm twice daily and mesalamine 1000mg suppository every evening, with significant improvement in his symptoms. On presentation, laboratory evaluation was remarkable for elevated troponin to 0.918 and elevated brain naturetic peptide to 2770. Electrocardiogram showed diffuse PR depression and ST elevations consistent with pericarditis. The patient was admitted for further evaluation. Mesalamine was stopped and he was started on Ibuprofen and colchicine with improvement in his pain. Common viral etiologies were ruled out with polymerase testing. A transthoracic echocardiogram revealed moderate global hypokinesis with a reduced ejection fraction (EF) of 25%. A cardiac MRI showed hyperenhancement throughout basal-to-mid inferior and inferolateral wall with ∼5% scar burden proving the diagnosis of perimyocarditis. At 1 month follow up his EF recovered to 52%. His UC symptoms have returned since stopping therapy though he remains hesitant to start other UC therapy given concerns regarding adverse effects. (Figure) Discussion: Mesalamine induced myocarditis is a rare but potentially lethal complication. It typically occurs within 1-2 weeks of starting the medication but has been reported as early as 48 hours and as late as 4 weeks. The specific pathogenesis of Mesalamine induced myocarditis is unknown. Withdrawal of mesalamine leads to symptomatic improvement in most patients as well as recovery of ejection fraction within weeks of discontinuing the medication, as seen with our patient. Future treatment with mesalamine and other 5-ASA derivatives should be avoided as they can trigger recurrent symptoms. Our case highlights a rare and life threatening complication of a commonly used medication. It also highlights the importance of discussing the potential risks of medications with patients. While rare, these authors recommend discussing the potential for myocarditis when counseling patients on treatment options for UC.Figure 1.: Cardiac MRI with myocardial inflammation.

Editorial for "Cardiac Magnetic Resonance Imaging Findings in COVID-19 Vaccine-Related Myocarditis: A Pooled Analysis of 468 Patients".

Samimisedeh et al,1 have published a systematic review and metanalysis of 102 publications with 468 patients with suspected myocarditis following COVID-19 vaccination. An accompanying editorial by Hanneman et al,2 highlights how patients were predominately young, male, and affected after their second dose of vaccine. A total of 79% met the accepted Lake Louise criteria for myocarditis. The authors show that the overall rate of myocarditis is low, ~16 cases per million doses of vaccine, and comparable to other vaccines such as influenza and non-smallpox vaccines.3 The authors can be applauded for demonstrating in a large, pooled cohort that vaccine-related myocarditis is usually mild and self-limiting, with no deaths occurring within the time frame. This provides substantial evidence for clinicians in their counseling of patients with vaccine hesitancy, particularly a younger population who as less likely to develop severe disease. This is in stark contrast to myocarditis related to the COVID-19 virus itself, which portends a poor prognosis due to arrythmias, heart failure and death. One study has shown a 0.2% chance of arrythmias or death within 28 days of a COVID vaccine, compared with 10.3% after a positive COVID test. In another large registry study of COVID-19 positive patients, those with myocarditis compared with those without myocarditis had a mortality rate of 13.4% vs. 4.2%.4 There is evidence cardiac MRI follow-up has prognostic value after an acute diagnosis of myocarditis, with patients with late gadolinium enhancement having complete resolution on a 6-month convalescent scan.5, 6 Early signal in published reports of vaccine-related myocarditis first appeared in mid-2021.7 Therefore, it would have been interesting to review that papers that include 6 to 12 month follow up cardiac MRI studies of cases to see if the degree of late gadolinium enhancement improved or resolved, particularly as it is a self-limiting disease clinically. Cardiac magnetic resonance imaging is non-invasive and excellent investigative tool in diagnosis myocarditis.6 Though cardiac MRI can demonstrate the classic subepicardial basal to mid inferolateral wall late gadolinium enhancement pattern, it does not have enough precision to identify the underlying etiology. Moreover, despite extensive research into vaccine-related myocarditis, we are still unclear as to the exact mechanisms that cause it.8, 9 It would have been interesting to review the studies that included histopathological correlation with endomyocardial biopsy. Finally, there is an emerging signal that myocarditis may represent an early signal of a “hot phase” of inherited or inflammatory cardiomyopathies, and that patients may have a genetic disposition to develop myocarditis.10 This could have been an interesting additional question in this review and will be interesting to follow in the coming years as we learn about the long-term effects of COVID-19 and vaccines to mitigate the risks.

Cardiac metastasis secondary to tongue cancer: An unusual mimicker of ST elevation myocardial infarction

We present a case of a 46-year-old female who presented with acute onset of worsening chest pain for 8 hours. Electrocardiogram (ECG) showed ST elevation in leads II, III and avF with diffuse T wave inversions. On admission, troponin-I was 0.124 ng/mL. Based on the symptoms, ECG changes and elevated troponin, there was a concern for inferior wall ST elevation myocardial infarction (STEMI). The patient was emergently taken for cardiac catheterization. Angiography showed mild non-obstructive coronary artery disease. Transthoracic echocardiogram (TTE) revealed thickened and hypokinetic inferoseptal, inferior and inferolateral walls. It also showed round echodensity in the left ventricular (LV) cavity arising from the thickened infero-lateral wall measuring 1.8x1.5 cm2 suspicious for metastatic intramyocardial tumor. For further evaluation, she underwent cardiac magnetic resonance imaging (MRI). It revealed 3.9 x 2.3 x 3.4 cm mass with imaging findings suggestive of malignant tumor attached to and infiltrating LV inferior myocardium, epicardial fat and pericardium. Based on the angiographic findings, echo and cardiac MRI, her symptoms were attributed to intramyocardial mass causing infiltration or displacement of myocardium. Her chest pain was controlled with tramadol. Our report demonstrates a rare case of cardiac metastasis secondary to tongue cancer presenting as STEMI.

Prevalence of Mitral Annulus Disjunction and Mitral Valve Prolapse in Patients With Idiopathic Ventricular Fibrillation.

BackgroundIdiopathic ventricular fibrillation (IVF) is diagnosed in patients with ventricular fibrillation of which the origin is not identified after extensive evaluations. Recent studies suggest an association between mitral annulus disjunction (MAD), mitral valve prolapse (MVP), and ventricular arrhythmias. The prevalence of MAD and MVP in patients with IVF in this regard is not well established. We aimed to explore the prevalence of MAD and MVP in a consecutive cohort of patients with IVF compared with matched controls.Methods and ResultsIn this retrospective, multicenter cohort study, cardiac magnetic resonance images from patients with IVF (ie, negative for ischemia, cardiomyopathy, and channelopathies) and age‐ and sex‐matched control subjects were analyzed for the presence of MAD (≥2 mm) and MVP (>2 mm). In total, 72 patients (mean age 39±14 years, 42% women) and 72 control subjects (mean age 41±11 years, 42% women) were included. MAD in the inferolateral wall was more prevalent in patients with IVF versus healthy controls (7 [11%] versus 1 [1%], P=0.024). MVP was only seen in patients with IVF and not in controls (5 [7%] versus 0 [0%], P=0.016). MAD was observed in both patients with (n=4) and without (n=3) MVP.ConclusionsInferolateral MAD and MVP were significantly more prevalent in patients with IVF compared with healthy controls. The authors advocate that evaluation of the mitral valve region deserves extra attention in the extensive screening of patients with unexplained cardiac arrest. These findings support further exploration of the pathophysiological mechanisms underlying a subset of IVF that associates with MAD and MVP.

The Relationship Between Coronary Dominance and Positive Results in Myocardial Perfusion Imaging.

ObjectiveOur study aims to evaluate the possible relationship between coronary artery dominance and its effect on accurately identifying reversible ischemia of inferior/inferior-lateral wall on cardiac perfusion imaging.BackgroundCoronary artery dominance is conventionally defined by the vessel which gives the rise to the AV nodal artery/posterior descending artery (PDA). Previous studies have explored the potential effect of coronary dominance on the accuracy of single photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI) tests in detecting inferior/inferior-lateral wall ischemia; further evidence is necessary to study that potential effect.MethodsWe conducted a single-center retrospective analysis to explore the potential relationship between coronary artery dominance and inferior/inferior-lateral wall ischemia on SPECT imaging. We identified a cohort of patients with a reversible defect(s) in the inferior and/or inferolateral walls on SPECT MPI who had subsequently undergone invasive coronary angiography. Coronary angiography was used to determine coronary dominance and to confirm the presence/absence of obstructive coronary artery disease in the distribution of the inferior and/or inferolateral wall(s). We correlated the findings on SPECT MPI to coronary angiography to identify true positives and false positive MPIs.ResultsA cohort of 200 patients was identified, patients in the cohort had undergone stress MPI with reversible defects with subsequent invasive coronary angiography. Baseline characteristics including age, BMI and sex were fairly well-balanced between the groups. The mean age was 68 +/- 11 in the right dominant group and 70 +/- 9 in the non right dominant group. One hundred and sixty-one patients (81%) were found to have right dominant circulation and 39 patients (19%) were found to have left or codominant circulation. Of the 161 patients in the right dominant group, 58 patients (36%) were found to have false positive stress MPI. Of the 39 patients in the left or codominant group, 23 patients (59%) were found to have false positive stress MPI. The incidence of false positive stress MPI in the inferior and inferolateral distribution is significantly higher in patients with non-right dominant coronary anatomy (p-value: 0.01).ConclusionNon-right coronary dominant anatomy could have high false positive MPI results in the inferior and inferolateral distribution. Therefore, the interpreting clinicians should exercise caution during the clinical evaluation of these patients.

P101 ARRHYTHMIC MITRAL VALVE PROLAPSE: COULD ELECTROANATOMICAL MAPPING AND CARDIAC MAGNETIC RESONANCE BE GOOD FRIENDS?

Abstract Background There is an increasing awareness of the association between mitral valve prolapse (MVP) and sudden cardiac death (SCD), but risk stratification is still challenging. Mitral annular disjunction (MAD) is a recognized risk factor for arrhythmic complications. A multimodality imaging strategy, including advanced echocardiography, magnetic resonance (CMR) and electromechanical mapping, could improve risk stratification and management by combining anatomical and functional information. Case Review We report a case of a 50–year–old woman without cardiovascular risk factors, complaining for worsening palpitations in the last months. Echocardiogram documented bileaflet MVP with moderate regurgitation, MAD (12 mm in inferolateral wall) and normal ventricular systolic function. 24–hour Holter monitoring revealed a high (13%) premature ventricular complexes burden with ventricular bigeminy and non–sustained ventricular tachycardia. Stress test showed a reduction of the arrhythmias during exercise. CMR confirmed MAD but no late gadolinium enhancement areas were detected. The patient underwent electrophysiological study with electroanatomic mapping (CARTO). The latter revealed multifocal fragmented and delayed low voltage potentials close to the posterior papillary muscle and the posterior anular mitral area, respectively. Both locations were the targets of endoventricular radiofrequency ablation. However, due to her high arrhythmic risk profile the patient implanted a cardioverter defibrillator. Discussion In arrhythmic risk stratification of MVP with MAD, CMR could have technical limitations due to diffuse fibrosis patterns or irregular cardiac rhythm that could undermine a detailed and complete evaluation. Thus, electroanatomical mapping during electrophysiological testing could offer additional and complementary information that could improve risk stratification and further management.

P177 A SHOT TO THE HEART

Abstract We report the case of a 50–year–old man who, during a wild bird hunt, was accidentally fired with a shotgun loaded with pellets which hit the chest, the neck and the face of the hunter. The patient was taken to the emergency room, tachypnoic and suffering from chest pain, systolic blood pressure was 150/70 mmHg, SpO2 88%, analgesic therapy, liquids administration and O2 therapy were performed with moderate response. On physical examination there was a reduced vesicular murmur in middle field on the right, no heart murmurs; noticeable edema of face and neck soft tissues and absence of evident jugular turgor. The ECG showed sinus rhythm, HR 100/min, morphology at the limit for ST slightly rigid in the inferior site. He underwent a total body CT in which a shotgun pellet was found located at the heart base near the right atrium, with associated pericardial fluid, with a maximum thickness of about 10 mm, with blood density. A shotgun pellet was also found in the apical segment of the right upper lung lobe with associated parenchymal ground glass (the pellet site of passage) without pneumothorax and pleural effusion. CT also showed multiple hunting pellets in the soft tissues of the face and of the neck without significant lesions. The echocardiogram confirmed the presence of circumferential pericardial effusion of mild degree, but progressively worsening, mainly represented along the cardiac apex and the inferolateral wall, but without signs of cardiac tamponade (right atrium within limits, absence of significant Doppler variation in blood flow across cardiac valves). The hospital where the patient arrived was at a distance of 30 minutes from the cardiac surgery, therefore the patient was urgently transferred to the cardiac surgery room of the hub (distance of 30 minutes). Fortunately, the patient maintained hemodynamic stability and arrived in the operating room with good blood pressure, but with a pre–tamponade effusion. The man quickly underwentcardiac surgery, the pericardial effusion was drained and the responsible breach was found in at the atrio–caval junction and successfully sutured. The patient was discharged in the 6th day and currently is in good clinical condition.

C40 THREE–DIMENSIONAL FINGER TEST: A NEW ECHOCARDIOGRAPHIC METHOD TO LOCATE THE BEST ACCESS SITE DURING NEOCHORD PROCEDURE

Abstract Background The NeoChord procedure is a trans–ventricular, beating–heart chordal implantation for severe degenerative mitral valve regurgitation due to prolapse or flail leaflet and it is performed using a dedicated device (DS 1000 system, NeoChord, Inc. St. Louis Park, MN). The use of the transesophageal echocardiography (TEE) is crucial to guide the procedure. Bi–dimensional (2D) imaging completed with simultaneous biplane view during surgeon finger pushing on the LV wall (finger test) is currently used to choose the LV access, which is usually on the mid–distal infero–lateral wall (ILW), between the papillary muscles (PMs) at the inferior level of their insertion on LV wall. This simulation helps the operators to evaluate the safe distance to PMs to minimize the risk of damaging the sub–valvular apparatus during the insertion of the device. We aimed to compare a new 3D method with the conventional one in terms of safety and better localization of the desired entry site. Methods During the procedure finger test has been performed with conventional 2D imaging and simultaneous biplane method. It has been completed using the real time 3D TEE placing the sample box in the bi–commissural view of the LV including the PMs and the apex. The resulting 3D volume was subsequently edited to visualize the LV from above (surgical view) to localize the bulge of the operator finger pushing on the desired segment of the LV wall. We asked the first operator, the second operator and the cardiac surgery fellow, separately, to evaluate location of their finger pushing, in terms of desired position and safety of access, both with 2D method and the 3D method to estimate the inter–operator concordance. Results From March 2019 to September 2021 42 consecutive cases have been performed using finger test completed with 3D method without complications related to the trans–ventricular access. Regarding the choice of the right and safe entry site, the percentage of agreement between operators was higher using LV real time 3D rendering compared to the conventional finger test [82 + 21% Vs 59% + 29%, IC 95%, p: 0,04]. Conclusion Three–dimensional finger test is easy to perform and decreases inter–operator variability of image interpretation facilitating the surgeons to choose the best entry site in term of anatomical localization and safety.

P313 TAKOTSUBO SYNDROME – FOCAL VARIANT

Abstract Takotsubo syndrome (TTS) is a cardiomyopathy induced by physical and/or emotional stress, characterised by transient changes of the regional motion generally extending beyond the territory distribution of one single coronary vessel. The classic form of TTS presents, in 43.7% of cases, with ST segment elevation and in about 80% with cardiac apical ballooning. In 1.5% of TTS cases, however, regional changes in motion are observed which may mimic an acute coronary syndrome. We describe the case of a 77–year–old woman with hypertension and hypercholesterolemia. The patient came to the ER due to polytrauma secondary to an accidental fall. During hospitalisation, the patient complained of a sudden onset of a retrosternal aching chest pain radiating to the back and right shoulder, associated with neurovegetative symptoms. An electrocardiogram showed a normofrequent sinus rhythm and T–negative waves in the infero–lateral region. Upon cardiology consultation, a transthoracic echocardiogram was performed showing a left ventricle with akinesia of the middle and apical segments of the inferolateral wall with compensatory hyperkinesis of the other segments. When an acute coronary syndrome was suspected, urgent coronary imaging was performed, showing coronary arteries without significant stenosis, while ventriculography confirmed the alterations in ventricular motion. Blood chemistry tests showed a phasic increase in myocardial necrosis enzymes and, in particular, in HS troponin values (max value 2714 ng/L). Therapy with an ace–inhibitor and a beta blocker was introduced; seriate echocardiographic controls showed a progressive normalisation of the alterations in motion. A TTS diagnosis was therefore made, according to InterTAK 2018 diagnostic criteria. This case is of particular interest as it is a prime example of a rare variant of TTS: the clinical presentation, the ECG alterations and the regional akinesia may lead to a clinical suspicion of ACS. A good knowledge of the TTS, including the focal variant, enables the cardiologist to make a correct diagnosis and to initiate suitable therapy.

Coincidence of Events Causing Paradoxical Hypotension in a Patient with an Intra-Aortic Balloon Pump.

The left ventricular outflow tract is a region of the left ventricle that lies between the anterior leaflet of the mitral valve and the ventricular septum. Dynamic left ventricular outflow tract obstruction (LVOTO) has classically been observed in patients with hypertrophic obstructive cardiomyopathy (HOCM) where it occurs secondary to asymmetric septal hypertrophy and systolic anterior motion (SAM) of the mitral valve. However, there are some instances that lead to hypercontractility of the myocardium, and with a combination of other physiological conditions, result in SAM of a mitral valve leaflet, leading to LVOTO in the absence of phenotype.We present such a case of an acute inferolateral wall myocardial infarction that was complicated by cardiogenic shock, requiring an intra-aortic balloon pump (IABP) and inotropic support which paradoxically provoked LVOTO. A reduction in IABP counterpulsation from 1:1 to 1:3 and the addition of intravenous fluids and a beta blocker resulted in significant improvement in blood pressure with rapid tapering of pressors.Inotropes and IABP, although helpful in cardiogenic shock, have the potential to induce or worsen the LVOTO, which can lead to a vicious cycle of worsening hypotension and increasing adrenergic drive that further deteriorates myocardial viability. Timely diagnosis with an echocardiogram and the withdrawal of inotropic and IABP support has the potential to improve haemodynamics and, thereby, outcome.LEARNING POINTSDynamic left ventricular outflow tract obstruction (LVOTO) should be one of the differentials in patients with cardiogenic shock, especially if it is refractory in the setting of an intra-aortic balloon pump.The diagnosis of LVOTO by echocardiography should result in immediate implementation of therapy to eliminate the factors that can potentially intensify the obstruction.

Association of ANK2 Mutation and Massive Myocardial Calcification: A Case Report

<h3>Introduction</h3> A 35-year-old man with no prior history of cardiovascular diseases was admitted with progressive exertional chest discomfort. Physical examination revealed irregular heart rhythm and an ejection murmur at the aortic area. EKG showed atrial fibrillation, and chest X-ray, calcification within the cardiac silhouette. Laboratory tests were all within normal ranges, including serological and inflammatory tests. <h3>Case Report</h3> An echocardiogram demonstrated diffuse myocardium calcification and intense mitral- aortic fibrocalcification, causing severe stenosis and moderate regurgitation, with a dilated left ventricle (LV) and moderate systolic dysfunction, akinesia of the inferolateral walls and hypokinesia of the anterior walls. A 128-row multidetector CT showed massive calcification affecting the aortic and mitral valves, the right ventricle, the left atrium wall and all the segments of the LV, preserving only the basal septum. Cardiovascular magnetic resonance imaging 1.5T revealed diffuse myocardial thickening, matching the areas seen on CT (figure). These areas were hypointense on the SSFP cine images and on T1- and T2-weighted images and had no late gadolinium enhancement (LGE). However, there was LGE in the underlying myocardium, in a non-ischemic distribution. Pericardium and coronary arteries were preserved. Genetic analysis identified a rare heterozygous variant of uncertain significance, c.10702C>T(p.Arg3568Trp), in ANK2 gene. <h3>Summary</h3> Ankyrin-B, also known as Ankyrin-2, is encoded by the ANK2 gene, which plays critical roles in the localization and stabilization of ion transporters and ion channels in cardiomyocytes. The Ankyrin-B syndrome has been associated to long QT syndrome and torsades depointes, but there are no reports of myocardial calcification. The patient was found to be a candidate for cardiac transplantation and is currently on the waiting list.

Clinical and Molecular Characteristics of Patients with PLN R14del Cardiomyopathy: State-of-the-Art Review

The deletion of the arginine 14 codon (R14del) in the phospholamban (PLN) gene is a rare cause of arrhythmogenic cardiomyopathy (ACM) and is associated with prevalent ventricular arrhythmias, heart failure, and sudden cardiac death. The pathophysiological mechanism which culminates in the ACM phenotype is multifactorial and mainly based on the alteration of the endoplasmic reticulum proteostasis, mitochondrial dysfunction and compromised Ca2+ cytosolic homeostasis. The symptoms of this condition are usually non-specific and consist of arrhythmia-related or heart failure-related manifestation; however, some peculiar diagnostic clues were detected, such as the T-wave inversion in the lateral leads, low QRS complexes voltages, mid-wall or epicardial fibrosis of the inferolateral wall of the left ventricle, and their presence should raise the suspicion of this condition. The risk stratification for sudden cardiac death is mandatory and several predictors were identified in recent years. However, the management of affected patients is often challenging due to the absence of specific prediction tools and therapies. This review aims to provide the current state of the art of PLN R14del cardiomyopathy, focusing on its pathophysiology, clinical manifestation, risk stratification for sudden cardiac death, and management.

A subset of arachnoid granulations in humans drain to the venous circulation via intradural lymphatic vascular channels.

The discovery of dural lymphatics has spurred interest in the mechanisms of drainage of interstitial fluid from the CNS, the anatomical components involved in clearance of macromolecules from the brain, mechanisms of entry and exit of immune components, and how these pathways may be involved in neurodegenerative diseases and cancer metastasis. In this study the authors describe connections between a subset of arachnoid granulations (AGs) and the venous circulation via intradural vascular channels (IVCs), which stain positively with established lymphatic markers. The authors postulate that the AGs may serve as a component of the human brain's lymphatic system. AGs and IVCs were examined by high-resolution dissection under stereoscope bilaterally in 8 fresh and formalin-fixed human cadaveric heads. The superior sagittal sinus (SSS) and adjacent dura mater were immunostained with antibodies against Lyve-1 (lymphatic marker), podoplanin (lymphatic marker), CD45 (panhematopoietic marker), and DAPI (nuclear marker). AGs can be classified as intradural or interdural, depending on their location and site of drainage. Interdural AGs are distinct from the dura, adhere to arachnoid membranes, and occasionally open directly in the inferolateral wall or floor of the SSS, although some cross the infradural folds of the dura's inner layer to meet with intradural AGs and IVCs. Intradural AGs are located within the leaflets of the dura. The total number of openings from the AGs, lateral lacunae, and cortical veins into the SSS was 45 ± 5.62 per head. On average each cadaveric head contained 6 ± 1.30 intradural AGs. Some intradural AGs do not directly open into the SSS and use IVCs to connect to the venous circulation. Using immunostaining methods, the authors demonstrate that these tubular channels stain positively with vascular and lymphatic markers (Lyve-1, podoplanin). AGs consist of two subtypes with differing modes of drainage into the SSS. A subset of AGs located intradurally use tubular channels, which stain positively with vascular and lymphatic markers to connect to the venous lacunae and ultimately to the SSS. The present study suggests that AGs may function as a component of brain lymphatics. This finding has important clinical implications for cancer metastasis to and from the CNS and may shed light on mechanisms of altered clearance of macromolecules in the setting of neurodegenerative diseases.

Myocardial Injury Pattern at MRI in COVID-19 Vaccine-Associated Myocarditis.

BackgroundThere is limited data on the pattern and severity of myocardial injury in patients with COVID-19 vaccination associated myocarditis.PurposeTo describe myocardial injury following COVID-19 vaccination and to compare these findings to other causes of myocarditis.MethodsIn this retrospective cohort study, consecutive adult patients with myocarditis with at least one T1-based and at least one T2-based abnormality on cardiac MRI performed at a tertiary referral hospital between 2019-2021 were included. Patients were classified into one of three groups: myocarditis following COVID-19 vaccination, myocarditis following COVID-19 illness, and other myocarditis not associated COVID-19 vaccination or illness.ResultsOf the 92 included patients, 21 (22%) had myocarditis following COVID-19 vaccination (mean age 31 years ±14 [standard deviation]; 17 men; mRNA-1273 in 12 [57%] and BNT162b2 in 9 [43%]). Ten patients (11%) had myocarditis following COVID-19 illness (mean age 51 years ±14; 3 men), and 61 (66%) had other myocarditis (mean age 44 years ±18; 36 men). MRI findings in vaccine associated myocarditis included late gadolinium enhancement (LGE) in 17 (81%) and left ventricular dysfunction in 6 (29%). Compared with other causes of myocarditis, patients with vaccine associated myocarditis had higher left ventricular ejection fraction and less extensive LGE, even after controlling for age, sex, and duration between symptom onset and MRI. The most frequent location of LGE in all groups was subepicardial at the basal inferolateral wall, although septal involvement was less common in vaccine associated myocarditis. At short-term follow-up (median 22 [IQR 7-48] days), all patients with vaccine associated myocarditis were asymptomatic with no adverse events.ConclusionsCardiac MRI demonstrated a similar pattern of myocardial injury in vaccine associated myocarditis compared to other causes, although abnormalities were less severe, with less frequent septal involvement, and no adverse events over short-term follow-up.See also the editorial by Raman and Neubauer.