Anterior Interventricular Septum Research Articles

A CASE REPORT OF AN ASYMPTOMATIC PATIENT WITH TYPE 2 WELLENS’ SYNDROME AND RENAL CARCINOMA

Abstract A 65–year–old male with renal cell carcinoma, previously treated with nephrectomy and recently diagnosed with lung metastases, presented to the cardiology clinic for investigations before starting chemotherapy. He reported a history of ischemic heart disease, a non–ST–elevation myocardial infarction for which he refused coronary angiography in 2005, and a negative maximal stress test for ischemia in 2017. Additionally, the patient reported insulin–dependent type 2 diabetes mellitus and well–controlled arterial hypertension. The patient was referred for a cardiological evaluation due to a reported short–term rhythmic heartbeat without other cardiological symptoms. Cardiological parameters were stable (blood pressure: 140/95 mmHg; heart rate: 42 bpm, and oxygen saturation without oxygen supplementation: 98%). Physical examination was normal. Laboratory investigations did not show elevated levels of high–sensitivity troponin I, and only signs of mild chronic kidney disease were observed. The initial ECG showed sinus bradycardia with deeply inverted T–waves in the extended anterior area (Fig. 1), which were not present in an ECG performed one month earlier. There was no reported chest pain. An echocardiogram examination demonstrated hypertensive heart disease with marked hypokinesia of the mid–apical portion of the anterior wall and anterior interventricular septum, together with a moderate reduction in global systolic function (LV ejection fraction: 40%). The patient was hospitalized, and underwent coronary angiography the following day. He had a low–risk score (GRACE: 95 pts) As shown in Fig. 2, there was evidence of long sub–occlusive stenosis of the proximal part of the anterior descending coronary artery that was treated with angioplasty and a single drug–eluted stent placement, resulting in a good angiographic result. After the procedure, there was a rapid recovery of regional kinetics but a slower normalization of the electrocardiographic morphology (Fig. 3). Comment Wellens‘ syndrome identifies a particular subtype of unstable angina characterized by the appearance of ischemic changes on the ECG,. The clinical risk assessment of this subgroup of patients with ACS, apparently having a more benign prognosis due to their definition as unstable angina with a low GRACE risk score, potentially has an evolution toward a large myocardial infarction if not promptly diagnosed.

VASOSPASTIC INFARCTION STARTED WITH PULSELESS ELECTRICAL ACTIVITY CARDIAC ARREST

Abstract We report the case of a 48–year–old female with a special history of ischemic heart disease. It started in 2021 with an acute anterior ST myocardial infarction: the coronary angiography showed subocclusive stenosis at the first tract of the left anterior descending (LAD), without other coronary stenoses. The patient was treated with primary PCI + DES; the echocardiogram showed EF 30% and akinesia of apex, intermediate segments of anterior wall and interventricular septum. During the same hospitalization the patient experienced recurrence of angina with transient ST elevation in the anterior and inferior leads; a control coronary angiography was unchanged. She was started on therapy with nitrates and diltiazem up to the tolerated dose, statin as well as antiplatelet and anticoagulant therapy due to apical thrombus. She underwent ICD implantation in primary prevention in January 2022. During 2022, there were several accesses to the ED for vasospastic angina despite medical therapy at the maximum tolerated dose. In October 2023 the patient experienced an acute chest pain followed by cardiac arrest, pulseless electrical activity and acute cardiogenic shock. After resuscitation procedures, she underwent an emergency coronary angiography which showed excellent result of the previous PCI, as well as stenosis of the distal edge of the stent resolving after injection of i.c. nitrates. No arrhythmic events at ICD recording and continuous electrocardiographic monitoring in ICU were observed. Cardiac MRI showed areas of subendocardial LGE involving the apex, anterior, anterolateral and septal segments due to ischemic scar; we also observed areas of edema and LGE with an ischemic pattern at the infero–septal and inferior wall in toto, suggesting recent ischemia; EF was 31%. During hospitalization the patient experienced episodes of typical angina with poor response to medical therapy at the maximum tolerated dose. The current clinical case most likely points to acute myocardial infarction due to vasospasm of the right coronary artery with acute pump failure resulting in cardiac arrest and no specific recommendations exist in recent cardiologic guidelines on the strategy to adopt for this acute clinical setting. In the hypothesis of a vasospastic pathogenesis of the problem, counseling for modulation of the stellate ganglion was requested.

UNPREDICTABLE IDENTIFICATION OF A TITIN MUTATION IN A YOUNG PATIENT PRESENTING WITH INFLAMMATORY CARDIOMYOPATHY

Abstract Case Presentation A 23 years old patient, affected by type 1 diabetes, presented with fever, dyspnea and bilateral peripheral edemas. In the last week he received antibiotic treatment for a persistent dry cough and sore throat, and became symptomatic for exertional dyspnea from three days. His mother died at the age of 40 for sudden cardiac death. Blood pressure and oxygen saturation were normal: the ECG showed sinus tachycardia with symmetric negative T waves in all precordial leads. High sensitivity troponin T was 0.01 mcg/L (normal value < 0.014 mcg/L). All remaining blood tests were normal, except for CRP that was slightly elevated. His echocardiogram revealed left ventricular (LV) remodelling and dilation, severely reduced LV ejection fraction (LVEF 20%) with global hypokinesia, more pronounced at the level of the anterior wall, interventricular septum and apex and a dilated right ventricle (RV) with reduced systolic function. Cardiac magnetic resonance (CMR) confirmed severe biventricular disfunction (LVEF 19%; RVEF 23%) and showed a small area of subepicardial inferior junctional late gadolinium enhancement (LGE). The patient underwent an endomyocardial biopsy that showed a lymphocytic virus–negative myocarditis. Given his known type 1 diabetes, steroid treatment was not considered and anakinra was started. The patient was discharged after two weeks with a slight amelioration of the LV function (LVEF 27%), on optimal heart failure therapy and anakinra. At six months he was in NYHA class II and CMR showed slight reduction in LV dimensions and improvement of the LV and RV function (LVEF 39%; RVEF 45%), with no changes in the LGE pattern. A genetic test was performed and revealed two titin gene mutations, both categorized as likely pathogenic (TTN c.[11831T>A]; c.[72510_72517delGACAAATG]), responsible for a genetic form of dilated cardiomyopathy. Conclusion The present case suggests that in young patients presenting with clinical or histological diagnosis of myocarditis, genetic testing for cardiomyopathies should be considered especially in presence of a positive family history for sudden death or an incomplete recovery of the LV function at follow–up. Identification of a gene mutation in these patients allows a more tailored risk stratification of the index case and a proper management of the entire family.

Gene-echocardiography: refining genotype-phenotype correlations in hypertrophic cardiomyopathy.

This study aims to clarify the association between hypertrophic patterns and genetic variants in hypertrophic cardiomyopathy (HCM) patients, contributing to the advancement of personalized management strategies for HCM. A comprehensive evaluation of genetic mutations was conducted in 392 HCM-affected families using Whole Exome Sequencing. Concurrently, relevant echocardiographic data from these individuals were collected. Our study revealed an increased susceptibility to enhanced septal and interventricular septal thickness in HCM patients harbouring gene mutations compared with those without. Mid-septal hypertrophy was found to be associated predominantly with myosin binding protein C3 (MYBPC3) variants, while a higher septum-to-posterior wall ratio correlated with myosin heavy chain 7 (MYH7) variants. Mutations in MYH7, MYBPC3, and other sarcomeric or myofilament genes (troponin I3 [TNNI3], tropomyosin 1 [TPM1], and troponin T2 [TNNT2]) showed a relationship with increased hypertrophy in the anterior wall, interventricular septum, and lateral wall of the left ventricle. In contrast, alpha kinase 3 (ALPK3)-associated hypertrophy chiefly presented in the apical region, while hypertrophy related to titin (TTN) and obscurin (OBSCN) mutations exhibited a uniform distribution across the myocardium. Hypertrophic patterns varied with the type and category of gene mutations, offering valuable diagnostic insights. Our findings underscore a strong link between hypertrophic patterns and genetic variants in HCM, providing a foundation for more accurate genetic testing and personalized management of HCM patients. The novel concept of 'gene-echocardiography' may enhance the precision and efficiency of genetic counselling and testing in HCM.

Cinematic Rendering of Acute Myocardial Infarction.

HomeRadiologyRecently Published PreviousNext Reviews and CommentaryFree AccessImages in RadiologyCinematic Rendering of Acute Myocardial InfarctionXiaoqing Ding , Zhigang GongXiaoqing Ding , Zhigang GongAuthor AffiliationsFrom the Department of Radiology, Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine, No 528, Zhangheng R. Pudong New Area, Shanghai, 201203, China.Address correspondence to X.D. (email: [email protected]).Xiaoqing Ding Zhigang GongPublished Online:Jan 31 2023https://doi.org/10.1148/radiol.221828MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Supplemental material is available for this article.A 43-year-old male patient presented to the emergency department with acute chest pain. The patient condition was stable, and an electrocardiography scan was obtained and showed ST segment changes with troponin I levels of 106.251 ng/mL; however, the patient refused percutaneous catheter intervention. To exclude aortic and pulmonary artery disease, dual-source CT angiography (CTA) of the chest was performed. No aortic or pulmonary artery abnormalities were identified; however, CTA demonstrated suspicious occlusion of left anterior descending coronary artery, focal hypodensity of the anterior wall, and interventricular septum, which is further highlighted with use of cinematic rendering reconstruction technique (Figure, Movie).CT angiography of the chest in a 43-year-old man with acute chest pain. Cinematic rendering of (A) four-chamber heart view and (B) anteroposterior view shows a focal area of decreased attenuation in the myocardium (anterior wall and interventricular septum).Download as PowerPointOpen in Image Viewer Get the Flash Player to see this video.Movie: Cinematic rendering of the heart, show a focal area of decreased attenuation in the myocardium.Download Original Video (20.9 MB)With increasing temporal resolution, it becomes possible to observe changes in myocardial density in the setting of acute myocardial infarction with use of conventional CTA of the chest (1). Cinematic rendering reconstruction technique (2) allows CTA to achieve highly realistic, photograph-level, postprocessed images that show the areas of acute myocardial ischemia more intuitively in three dimensions.Disclosures of conflicts of interest: X.D. No relevant relationships. Z.G. No relevant relationships.

Late gadolinium enhancement in the left ventricular wall is associated with atrial fibrillation in patients with hypertrophic cardiomyopathy.

Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the relation between AF and left ventricular (LV) late gadolinium enhancement (LGE). 55 patients with HCM were retrospectively included. Patients were divided in HCM with AF and HCM without AF. Baseline clinical, echocardiographic and cardiovascular magnetic resonance (CMR) characteristics were collected and compared between groups. In univariable analysis, the factors related to AF development were HCM risk score for sudden cardiac death (SCD) > 2.29% (p = 0.002), left atrium (LA) diameter > 42.5mm (p = 0.014) and LGE in the mid anterior interventricular septum (IVS) (p = 0.021), basal inferior IVS (p = 0.012) and mid inferior IVS (p = 0.012). There were no differences in LV diastolic function and LA strain between groups. Independent predictors of AF were LA diameter (p = 0.022, HR 5.933) and LGE in mid inferior IVS (p = 0.45, HR 3.280). Combining LA diameter (> 42.5mm or < 42.5mm) and LGE in mid inferior IVS (present or absent) in a model with four groups showed a statistically significant difference between groups (p = 0.013 for the model). LGE in mid inferior IVS is an independent predictor for AF occurrence in patients with HCM. Combining both LGE in mid inferior IVS and enlarged LA improves prediction of AF and may be preferred for risk stratification.

C61 MULTIDISCIPLINARY MANAGEMENT IN A CASE OF EOSINOPHILIC MYOCARDITIS WITH CHURG STRAUSS SYNDROME: FROM ECG TO ENDOMYOCARDIAL BIOPSY

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss, is a rare multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia (PE). Cardiac involvement may include eosinophilic myocarditis and it is a serious manifestation of EGPA. Case Presentation A 67–year–old woman presented to the emergency department with 2–weeks history of dyspnea, orthopnea and asthenia. She had history of asthma, PE, adjuvant radiotherapy after right mastectomy (July 2021). The patient was diagnosed with new onset atrial fibrillation in the previous month. At admission, the patient was hemodynamically stable and with signs of congestion. Complementary exams showed sinus rhythm and T–wave inversion on lateral leads; PE (2010/uL), elevated troponin and BNP values; and severe biventricular systolic dysfunction with diffuse hypokinesia and apical akinesia. The patient was admitted to the ICU and was treated with intravenous diuretics and levosimendan. Optimal HF therapy was introduced. Serial echocardiography revealed partial recovery of LVEF and blood analysis showed a decrease in troponin levels, with persistent eosinophilia (6330/uL). Computed tomography (CT) excluded significant coronary disease, and showed bilateral basal ground–glass opacities, areas of air–space consolidation and bilateral reticular–nodular pattern. Cardiac magnetic resonance revealed increased T2 values/signs of myocardial edema in anterior wall, interventricular septum and apex and no late gadolinium enhancement, compatible with myocarditis. An endomyocardial biopsy (EMB) was performed and confirmed the diagnosis of eosinophilic myocarditis. Oral corticosteroids were started. Paranasal CT scan showed signs of chronic sinusitis, without polyposis, and antineutrophil cytoplasmic antibodies were positive, making the diagnosis of EGPA, according to ACR criteria. Discussion In a patient presenting with new onset heart failure and with history of asthma and eosinophilia, it is important to suspect eosinophilic myocarditis, as this is a rare but reversible life–threatening condition. EMB plays an important role in the diagnosis and should be done promptly. Conclusion We described a multidisciplinary management of a case of a patient with eosinophilic myocarditis and EGPA, presenting with severe acute biventricular dysfunction.

Determining the Association Between Density of Muscarinic Acetylcholine Receptor M3 in Myocardium and Tunica Media of Coronary Vasculature and Self‐Reported Disease States

Myocardial infarction causes parasympathetic dysfunction in cardiovascular tissue, where central parasympathetic drive decreases but local acetylcholine levels are unchanged. However, muscarinic acetylcholine receptor M3 (AChM3R) is shown to have mediating effects in cardiac tissue and vasculature, such as regulation of heart rate and vasodilation of coronary arteries. The objective of this study is to determine the association between AChM3R levels in the heart wall and in the coronary vasculature with self‐reported disease states from cadaver donor records.Biopsies of the left anterior descending (LAD) artery and the underlying anterior interventricular septum (AIVS) were taken from 14 cadavers as opportunity samples. A 5 mm biopsy of the LAD and its underlying AIVS were harvested 2.54‐3.81cm from the bifurcation point of the left coronary artery, depending on the tissue integrity of the cadaver specimen. The tissues were immunostained for AChM3R (CHRM3 NB100‐58975 at 1:2000) and visualized using a DAB chromogen. Slides were digitally scanned into a virtual image at 20x using the Motic Easyscan (Motic, Inc.). Images were segmented for tunica media and myocardium using Adobe Photoshop CS (Adobe, Inc.). We developed code in Python to calculate the strong positive and positive staining of the tissues. The number of pixels stained was normalized to the tissue area. The donors from which tissue was biopsied were classified into cardiovascular, cerebrovascular, Alzheimer’s disease dementia (AD), or both cerebrovascular and AD disease categories based on self‐reported donor declarations. We used SPSS (v27, IBM, Inc.) to run a Pearson correlation to determine the association of staining positivity in the anatomical regions and a MANOVA to determine significant differences in the amount of pixel positivity in the tunica media and myocardium as a function of disease classification.Strong positive and positive staining of the myocardium were not correlated with strong positive in the tunica media. There was a significant, strong, and positive relationship between strong myocardium staining and positive tunica media staining (r =.727, n=14, p=.003) as well as positive myocardium and positive tunica media staining (r=.674, n=14, p=.008). There is no significant difference between the amount of AChM3R staining and disease classification.The lack of correlation between strong positive in the tunica media and positive and strong positive in the myocardium suggests that changes in the AChM3R of coronary vascular smooth muscle are independent of the AChM3R state in the heart wall. However, significant correlation between strong positive in the myocardium and positive in the tunica media indicates that changes in AChM3R in the myocardium could be dependent on the presence of a baseline amount of AChM3R in the coronary vascular smooth muscle. Categorization of donor disease states may be too broad to provide statistical significance. Many donors reported co‐morbid diseases, which could have affected the influence of cardiovascular disease and dementia on AChM3R modulation. These findings highlight that interventions targeting parasympathetic dysfunction of coronary vasculature may leave the underlying heart wall unaffected. Future studies should consider cardiovascular disease diagnoses to assess AChM3R distribution in the heart wall and coronary vasculature.

742 Myocarditis after SARS-CoV-2 vaccine: is that so simple?

SARS-CoV-2 vaccination is associated with potential side effects, particularly following second vaccine dose. Recent case series have reported a potential association between SARS-CoV-2 vaccination and acute myocarditis, predominantly in young males. We hereby describe a previously healthy 17-year-old man, with no past cardiac history, who presented to the emergency department with persistent chest pain and fever (up to 38 °C). The patient had received the first dose of Cominarty (BioNTech/Pfizer) vaccine 10 days before symptom onset and reported flu-like symptoms and conjunctivitis involving both eyes one week before administration of the first vaccine dose. On that occasion, no COVID test was performed and the patient was treated with anti-inflammatory drugs and antibiotic eye drops. On admission, laboratory tests were performed (Troponin-I Δ 19 500–23 270 ng/l. CRP 23 mg/dl, ESR 43 s, WBC 17 570 cell/mm3) as well as COVID-19 PCR, Serological tests and Autoimmune disorders panel all resulting negative. CT coronary angiogram did not reveal any spontaneous coronary artery dissection or anomalous origin of coronary arteries and Calcium Score was 0. Transthoracic echocardiography showed a depressed LVEF (36%) with concomitant posterior and inferior wall as well as posterior and anterior basal interventricular septum hypokinesia. Endomyocardial biopsy revealed multifocal lymphocytic myocarditis with sub-endomyocardial and interstitial fibrosis. CMR was also performed (1-week after presentation) demonstrating mildly depressed systolic function (LVEF 47%), with hypokinesia of the posterior and inferior wall, increased signal intensity on T2 maps (58 ms, n.v. <55 ms), prolonged native T1 values (1083 ms, n.v. <1030 ms) as well as subepicardial and intramyocardial LGE enhancement of infero-lateral segments reflecting intercellular fibrosis. Thereafter, the patient was discharged with medical therapy including ACE-inhibitor, colchicine, and ibuprofen. Given the close proximity between SARS-CoV-2 vaccine administration and the absence of other predisposing conditions, the aetiology of myocarditis was attributed to the vaccine. In addition, as the patient suffered from flu-like symptoms and conjunctivitis 1 week before the vaccine, a previous paucisymptomatic SARS-CoV-2 infection was suspected and anti-SARS-Coronavirus Nucleocapsid Protein antibody test revealed high antibody levels with low IgG avidity. Given that myocarditic symptoms evolved after complete Sars-Cov2 symptom resolution, our first hypothesis is that the infection is unlikely to be the cause of acute myocarditis in this patient. Indeed, current literature on COVID-related myocarditis reports close temporal association between respiratory symptoms and myocarditis onset. In support to our hypothesis, recent trials have reported that myocarditis more frequently occurs following administration of mRNA vaccines especially in male adolescents and young adults like our patient. However, cardiac side effects typically occur after full vaccination and symptoms appear within three days following the second dose, which does not fully apply to this case. Notwithstanding this, more recent studies have reported myocarditis even after first vaccination dose in patients with previous COVID-19 infection, analogously to the case described. This case suggests a complex interaction between immunological factors and covid infection/vaccination with potential significant implications on the cardiovascular system. From current literature, much uncertainty remains regarding time interval criteria for reliable post-vaccination myocarditis diagnosis, hence large-scale clinical trials are needed to address this issue.

436 A peri-myocarditis unmasks an underlying hypertrophic cardiomyopathy

Abstract Methods and results A 41-year-old black man complaining of severe oppressive chest pain radiated to the back presented to our accident &amp; emergency department (A&amp;E). His symptoms started few days before his hospital admission. Past medical history was remarkable for arterial hypertension in medical therapy and microdrepanocytosis. In A&amp;E, the patient’s physical examination and vital signs were normal, he was normotensive, apyretic with normal oxygen saturation. The ECG showed ST elevation in anterior and lateral leads. Because of his history of arterial hypertension, and the severe chest pain irradiated to the back, an angio-CT was indicated at first. The CT ruled out acute aortic disease. Excluded the acute aortic disease, the patient underwent an urgent coronary angiography. No coronary stenosis was found. Therefore, the patient was admitted in cardiac intensive care unit. The blood test showed an elevation of high sensitive cardiac troponin T (cTnT peak 3093 ng/L) and inflammatory index (leukocytosis 13.65 109/l and protein C reactive peak 347 mg/L). An in-depth anamnestic collection revealed fever with respiratory symptoms about 2 weeks before. The echocardiography demonstrated left ventricular (LV) dysfunction with increased ventricular wall thickness and mild pericardial effusion. No LV outflow tract obstruction was found. A provisional diagnosis of peri-myocarditis was made. During hospitalization, anti-remodelling cardiac therapy was introduced and up titrated. To confirm the provisional diagnosis, a Gadolinium cardiac Magnetic Resonance (CMR) was performed, and it revealed myocardial oedema on basal anterior interventricular septum and multiple areas of late gadolinium enhancement with subepicardial pattern. Moreover, severe LV hypertrophy was confirmed (interventricular septum 19 mm, inferior wall 17 mm). This pattern was consistent to the diagnosis of peri-myocarditis on a hypertrophic cardiomyopathy (HCM). Main infectious causes of peri-myocarditis were investigated, but the results were inconclusive. Unfortunately, genetic test results are still not available. Patient was discharged with recovered LV systolic function and free of symptoms on optimal medical therapy; no ventricular arrhythmias was detected during hospitalization. HCM risk sudden cardiac death (SCD) was lower than 4%. Conclusions Peri-myocarditis can mimic symptoms of an acute coronary syndrome. Furthermore, the inflammation of cardiac muscle and the subsequent interstitial oedema may cause an increase in LV wall thickness. In this setting, the diagnosis of an underlying cardiomyopathy is challenging. This interesting and unusual case highlights the relevance of an accurate diagnostic work up to deliver good clinical practice. In particular, Gadolinium CMR is of paramount importance in this setting.

Episodic Myocardial Injury: Consider Desmoplakin Cardiomyopathy!

<h3>Introduction</h3> Recurrent myopericarditis, characterized by repeated episodes of pericardial and myocardial inflammation, can be a diagnostic conundrum. We present one such case ultimately found to have a desmoplakin (DSP) gene mutation. <h3>Case Report</h3> Our patient was a previously well adolescent male with a several year history of episodic chest pain and significant troponin elevation. He had mild left ventricular (LV) systolic dysfunction by echocardiography, with no ventricular dilation, pericardial effusion or valvar dysfunction. He had intermittent ventricular bigeminy. Coronary arteries were normal. Cardiac MRI revealed late gadolinium enhancement involving the anterior and lateral LV walls and interventricular septum. Myocardial biopsy revealed patchy interstitial fibrosis. Genetic testing revealed a likely pathogenic DSP mutation, c.123C>G (p.Tyr41*), predicted to cause loss of normal protein function. The variant is uncommon in the general population and several downstream nonsense variants have been associated with cardiomyopathy, supporting pathogenicity. <h3>Summary</h3> DSP is an essential protein in desmosomes, intercellular complexes responsible for maintaining cell-cell adhesion. Desmosomal mutations can cause arrhythmogenic cardiomyopathy, in which cardiac myocytes undergo fibroadiposis resulting in ventricular dysfunction, arrhythmias and sudden death. New evidence supports that DSP mutations can cause an inflammatory phenotype distinct from typical arrhythmogenic cardiomyopathy, characterized by predominant LV involvement, recurrent episodes of myocardial injury and fibrosis preceding systolic dysfunction. Our patient fits the novel description of DSP cardiomyopathy. His management plan includes reverse remodeling agents, surveillance for arrhythmia and cardiac dysfunction, restriction from competitive sports, and cascade genetic screening. Our case highlights the role of cardiomyopathy/arrhythmia genetic panels in patients with recurrent, unexplained myocardial inflammation.

Assessment of no-reflow phenomenon in patients with acute ST-elevation myocardial infarction

The problems concerning assessment of the state of myocardial perfusion in patients with acute ST elevation myocardial infarction after successful revascularization still remain of current importance. Contrast-enhanced echocardiography remains the least studied and most promising ultrasound technology for the diagnosis of the no-reflow phenomenon. The study was aimed at evaluating echocardiographic and angiographic characteristics of the no-reflow phenomenon detected by means of contrast-enhanced echocardiography in patients with ST-segment elevation myocardial infarction. The study included a total of forty-three 40-to-82-year-old patients in acute period of myocardial infarction. The patients were divided into two groups: 32 patients with satisfactory myocardial reperfusion after revascularization according to the findings of contrast-enhanced echocardiography and 11 patients with impaired perfusion. The patients in the group with impaired perfusion demonstrated a greater size of the left ventricular (LV) asynergy (40.1±2.2% vs 27.4±8.5%, p<0.001), more frequent LV dilatation (LV end-systolic volume 67.3±20.3 ml vs 51.8±17.2 ml, p=0.015), decreased LV contractility (LV ejection fraction 39.5±3.4% vs 47.2±4.9%, p < 0.001), and significant mitral regurgitation (45.5% vs 3.1%, p=0.011) with a decrease in DP/DT (979.9±363.4 mmHg/s vs 1565.7±502.8 mmHg/s, p<0.001) were more often detected in this group. Coronary angiography showed no perfusion disorders after revascularization in more than a quarter of these patients. In the group with impaired perfusion, more frequently revealed were single-vascular lesions (46.9% vs 9.1%, p=0.033), lesions of the anterior interventricular artery (90.9% vs 40.6%, p=0.004), and acute occlusion (100% vs 68.8%, p=0.043); compliance by the SYNTAX score in this group was higher (18.9±3.7 vs 9.9±5.7, p<0.001). In patients with acute myocardial infarction after successfully performed revascularization, perfusion disorders revealed by the findings of contrast-enhanced echocardiography were accompanied by more pronounced echo signs of left-ventricular dysfunction, higher values of the SYNTAX score and significantly more frequently revealed lesions of the anterior interventricular septum as compared with the patients with recovered perfusion.

Systematic quantification of histologic ventricular fibrosis in isolated mitral valve prolapse and sudden cardiac death

Cardiac fibrosis in mitral valve prolapse (MVP) is implicated in the development of sudden cardiac death (SCD); however, the pattern remains poorly characterized. The purpose of this study was to systematically quantify left and right ventricular fibrosis in individuals with isolated MVP and SCD (iMVP-SCD), whereby other potential causes of death are excluded, compared to a control cohort. Individuals with iMVP-SCD were identified from the Victorian Institute of Forensic Medicine, Australia, and matched for age, sex, and body mass index to control cases with noncardiac death. Cardiac tissue sections were analyzed to determine collagen deposition in the left ventricular free wall (anterior, lateral, and posterior portions), interventricular septum, and right ventricle. Within the iMVP-SCD cases, the endocardial-to-epicardial distribution of fibrosis within the left ventricle was specifically characterized. Seventeen cases with iMVP-SCD were matched 1:1 with 17 controls, yielding 149 samples and 1788 histologic regions. The iMVP-SCD group had increased left ventricular (anterior, lateral, and posterior; all P <.001) and interventricular septum fibrosis (P <.001), but similar amounts of right ventricular fibrosis (P = .62) compared to controls. In iMVP-SCD, left ventricular fibrosis was significantly higher in the lateral and posterior walls compared to the anterior wall and interventricular septum (all P <.001). Within the lateral and posterior walls, iMVP-SCD cases had a significant endocardial-to-epicardial gradient of cardiac fibrosis (P <.01) similar to other known conditions that cause cardiac remodeling. Our study indicates that nonuniform left ventricular remodeling with both localized and generalized left ventricular fibrosis is important in the pathogenesis of SCD in individuals with MVP.

Morphometric characterization of collagen and fat in normal ventricular myocardium

ObjectiveWe used automated image analysis software to determine the proportion of collagen, fat, and myocytes across six histological regions of normal ventricular myocardium. MethodsTwenty-nine non-cardiac death cases referred to our national cardiac pathology center were included in this study. Whole hearts were macroscopically and microscopically normal following expert histopathological evaluation. Tissue sections from the right ventricular outflow tract, right ventricle (RV), anterior interventricular septum (IVS), posterior IVS, anterior left ventricle (LV), and posterior LV were stained with Picrosirius red for collagen and scanned using a high-resolution slide scanner. Quantification of collagen, fat, and myocyte proportions was performed using Visiopharm software after automated exclusion of perivascular collagen. ResultsThe majority of decedents were male (25/29; 86%) with a mean age at death of 32.1 ± 9.9 (range 18-54) and mean BMI 28.7 ± 7.3. We report predicted values (collagen %, fat %, myocytes %) for cardiac tissue composition within the RV, IVS, and LV (including epicardial and endocardial layers). The proportion of collagen and fat were higher in the RV compared with the LV (ratios 1.61 [1.45-1.78]; 2.63 [1.99-3.48], respectively) and RV compared with the IVS (ratios 1.77 [1.60-1.97]; 8.41[6.35-11.13], respectively). The ratio of epicardial versus endocardial fat was increased in both ventricles (RV: ratio 4.49 [3.67-5.49]; LV: ratio 3.46 [2.49-4.81]). In multivariable analysis, there was no significant association between collagen or fat proportion and sex (p=0.12; p=0.08, respectively), age at death (p=0.36; p=0.23, respectively), or BMI (p=0.45; p=0.43, respectively). ConclusionsOur findings provide location and sex-specific proportions of myocardial histological tissue composition that may aid quantitative evaluation of pathology in future studies.

109 Anderson Fabry disease in CMR: twins by traditional tissue characterization, different by parametric mapping

Abstract Anderson Fabry disease (AFD) is an X-linked recessive lysosomal storage disease, caused by intracellular accumulation of glycosphingolipids due to deficiency of the enzyme α-galactosidase. The cardiac involvement carries a worse prognosis. Myocardial hypertrophy is the most common manifestation due to the intracellular accumulation of glycosphingolipids in myocytes. With disease progression the deposits are replaced by fibrosis although recent data suggest a chronic inflammation also independently to glycosphingolipids accumulation. Cardiac magnetic resonance (CMR) by tissue characterization using parametric mapping is a unique technique in evaluating AFD progression and in determining enzyme treatment indications and evolution under therapy. Heterozygous females are not asymptomatic carriers of the AFD mutation, they have a variable clinical presentation; disease expression in females is the result of random inactivation of the X chromosome. Female patients have a . This case provides an example of Fabry disease in a couple of sisters with a third sister with a diagnosis of AFD with cardiac, hepatic, and renal involvement. The youngest sister with a positive genetic test was symptomatic for chest pain, without any significant coronary arteries disease by coro CT. The ECG showed atrial fibrillation and sign for LVH confirmed by echocardiography. The traditional CMR study by SSFP cine images and LGE technique showed an increased thickness in the basal infero-lateral wall (15 mm) and the anterior interventricular septum (14 mm) with midwall fibrosis in the basal infero-lateral wall (Fig. 1 A, B). The oldest sister with a positive genetic test was asyntomatic in AF. ECG and ECOC were aspecific. The traditional CMR study by SSFP cine images and LGE technique showed the same finding of the youngest sister (Fig. 2 A, B) By T1 mapping based on the normal cut off values of the Lab the older sister reported a short T1 global value (915 ms) and short T1 values in all segment with exception of lateral wall, mid and distal inferior segments and distal septum (Figure 1 C). Despite the presence of positive LGE in the basal infero-lateral wall the T1 value was normal in this segment due to a pseudo normalization related to the sphingolipid accumulation. Based on the CMR report she started the enzyme replacement therapy and the CMR follow was planned at 1 year. Conversely, the younger woman reported a long T1 value only in the basal infero-lateral wall with normal T1 value in all the other segments (Figure 2 C). The positive LGE in the basal infero-later segment (oedema/fibrosis) justifies the long T1 value in this no thickness segment. Based on the CMR report she did not start enzyme replacement therapy. Conclusion The case of this family show how quantitative parametric mapping could be a unique tool for the clinicians to adjust the therapy and plan the follow up of patients affected by AFD with cardiac involvement. Abstract 109 Figure.

P1482 Surviving free wall rupture

Abstract Introduction Free wall cardiac rupture (CR) is a rare event accounting for 0.1–0.3% of the patients suffering acute myocardial infarction. Its outcome is very poor and it is the third most common cause of early mortality after hospitalization for ST Elevation Myocardial Infarction (STEMI). Purpose We report a case of a 63 years-old woman surviving a free wall rupture after ST Elevation Myocardial Infarction. Methods The patient was referred to our cath-lab to undergo primary PCI in ST elevation myocardial infarction. Results Coronary angiography showed long thrombotic occlusion of left anterior descendent (LAD) artery and critical stenosis of posterior descending artery. The CULPRIT lesion on the anterior descending artery was treated with angioplasty and implantation of three drug eluting stents (3.0x31 mm; 2.75x15 mm; 2.5x30 mm). Twenty four hours later the patient developed a double cardiac arrest with pulseless electrical activity, which were immediately managed with ALS protocol. The patient recovered both time within a couple of minutes and point-of-care transthoracic echocardiogram (TTE) showed a newly developed circumferential pericardial effusion (maximum diastolic diameter 9 mm), associated to a significant thinning of the anterior interventricular septum. A fibrin clot was tamponating a suspected free wall rupture. Emergent coronary angiography showed an in-stent thrombosis but failed to restore adequate blood flow in the LAD artery. In the following days, the patients developed cardiogenic shock handled with i.v. dobutamine and intra-aortic balloon pump (IABP). Only after day 10 hemodynamic parameters started to improve gradually, allowing IABP removal and finally discharge on day 30. A second TTE, performed on day 7, confirmed massive necrosis of the anterior wall with severely reduced ejection fraction (EF 22%), pericardial thrombus and aneurismatic evolution of the apex and the mid-anterior septum. To support our finding we performed a cardiac magnetic resonance which confirmed missing ventricular wall at the anterior apex. It also showed transmural late gadolinium enhancement (LGE) of the anterior mid-apical septum and of the apex and a huge pericardial thrombus encompassing the whole mid-anterior septum (Figure 1, a-f). Before discharge the patient underwent two cardiac surgery visits which contraindicated surgical treatment in the acute phase. Therefore she was sent to cardiac rehabilitation program. Six months later, the patient finally underwent cardiac surgery and the covered free wall rupture was confirmed in the operating theatre. Conclusion This is a very rare case of covered free wall rupture, treated 6 months after the acute event. Multimodality imaging was essential to confirm the diagnosis and to guide the following management. Abstract P1482 Figure 1

4093Beyond global longitudinal strain: early impairment of apical function after antracyclines therapy

Abstract Purpose To confirm GLS diagnostic sensitivity as parameter for the evaluation of LV systolic function in women with breast cancer who underwent chemotherapy including anthracyclines and to identify a pattern of decreased 2D speckle tracking regional longitudinal strain through the analysis of polar maps obtained with AFI technology. Methods We enrolled 60 female patients (age with 56.5±12 years) with breast cancer before the beginning of chemotherapy. The study protocol included clinical examination, ECG with QTc calculation, lab test (BNP and troponin I) and echocardiography with TDI and speckle tracking analysis (STI), that were performed before the beginning of the chemotherapy (basal) and after 3, 6 and 12 months. Echocardiography evaluation included the following parameters: LV end-diastolic and end-systolic volumes, LV ejection fraction (EF), average TDI S' at the mitral annulus, Global Longitudinal Strain (GLS), regional longitudinal strain, E/A ratio, E/E' ratio and sPAP. For each patient we analyzed the bull's eye maps before the beginning of the therapy (basal value) and when GLS showed the lowest values during the FU (FU value), to identify the pattern of regional longitudinal strain alterations. We compared basal and FU strain values for each of the 17 LV segments and the difference between them (delta) was calculated according to the formula [(FU LS –basal LS, (%)]. Results During the FU, systolic blood pressure, systolic pulse pressure and BNP values increased from the basal assessment to the 3 and 6 months FU. Similarly, a progressive worsening of GLS values has been observed (basal −20.4±2.6%, 3 months FU −18.2±2.5%, 6 months FU −17.7±2.9, 12 months FU −17.6±3, p value &lt;0.001). Through the analysis of polar maps, we observed that regional strain values worsened significantly in all the LV segments but the most evident impairment was reported in the apical cap (−22.8±3.9 vs −17.1±3.8; p&lt;0.001, Δ=−5,78%) and in the apical segment of the anterior interventricular septum (−23.4±4.5 vs −17±6.3; p&lt;0.001, Δ=−6,2%), as reported in Figure. Conclusion GLS is able to identify LV systolic dysfunction that EF is not able to detect. However, since that it describes the global function of LV, GLS could result as normal (18–20%) when strain impairment of some LV segments is counterbalanced by the compensatory strain increase of other segments, determining an misdiagnosis of myocardial damage. Regional strain and particularly the Δ-strain, seem to suggest that anthracyclines induce a damage more evident in the apical cap and in the apical segment of the interventricular septum and this pattern could be typical in these patients. Thus, polar maps analysis could be provide additional information about cardiac damage in this population.

Efficacy and safety of transthoracic echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation for the treatment of patients with obstructive hypertrophic cardiomyopathy

Objective: To investigate the safety and efficacy of transthoracic echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) in patients with hypertrophic obstructive cardiomyopathy (HOCM). Methods: Nine HOCM patients with interventricular septal thickness ≥15 mm and ≤25 mm who were treated with PIMSRA between October 2016 to March 2017 in the Hypertrophic Cardiomyopathy Center of Xijing Hospital of Air Force Military Medical University were enrolled,and the clinical data were retrospectively analyzed.Interventricular septum thickness, left ventricular outflow tract diameter and maximum gradient were measured by transthoracic echocardiography immediately after procedure, at 1 month, 3 months and 6 months after operation.Symptoms and New York Heart Association (NYHA) functional class were assessed, and provoked left ventricular outflow tract gradient and exercise time were measured after 6 months. Results: The anterior interventricular septum ((21.5±2.6) mm vs. (24.7±2.7) mm, P<0.05) and posterior interventricular septum (21.1±2.5) mm vs. (22.6±3.3) mm, P<0.05) were significantly increased,left ventricular outflow tract diameter was widened ((8.2±3.4) mm vs. (4.8±2.2) mm, P<0.05), left ventricular outflow tract gradient ((26.8±19.6) mmHg (1 mmHg=0.133 kPa) vs. (83.3±32.4) mmHg, P<0.05) and mitral regurgitation (2.0±1.9) ml vs. (3.2±3.0) ml, P<0.05) were significantly decreased immediately after ablation compared with pre-operation values. Anterior interventricular septum, posterior interventricular septum and left ventricular outflow tract gradient further decreased after 1 month ((17.5±2.0) mm vs. (24.7±2.7) mm, P<0.05; (16.9±2.1) mm vs. (22.6±3.3) mm, P<0.05; (11.6±4.0) mmHg vs. (26.8±19.6) mmHg, P<0.05, respectively) compared with values immediately after ablation. Anterior interventricular septum and posterior interventricular septum decreased after 3 and 6 months ((14.8±1.7) mm and (13.4±2.0) mm vs. (17.5±2.0) mm, all P<0.05; (12.9±1.9) mm and (12.3±2.4) mm vs. (16.9±2.1) mm, all P<0.05, respectively) compared with values at 1 month after ablation.There were no significantly difference in left ventricular outflow tract gradient at 3 and 6 months post procedure compared with 1 month after ablation (all P>0.05). Compared with pre-operation, provoked left ventricular outflow tract gradient decreased ((25.5±11.4) mmHg vs. (147.8±58.0) mmHg, P<0.01), and total exercise time increased ((9.3±1.6) minutes vs. (6.7±1.6) minutes, P=0.03) at 6 months after operation.The symptoms were disappeared in 5 patients. There were 2 cases with NYHA class Ⅱ and 7 cases with NYHA class Ⅲ before operation,while there were 6 patients with NYHA classⅠ and 3 patients with NYHA class Ⅱ at 6 months after operation (P<0.01). Conclusion: Transthoracic echocardiography-guided PIMSRA is a safe and effective new treatment approach for patients with obstructive hypertrophic cardiomyopathy.

Outcomes of simultaneous unipolar radiofrequency catheter ablation for intramural septal ventricular tachycardia in nonischemic cardiomyopathy

Radiofrequency (RF) ablation of intramural septal ventricular tachycardia (VT) in patients with nonischemic cardiomyopathy (NICM) is challenging. The purpose of this study was to investigate the outcomes of simultaneous unipolar RF ablation for intramural septal VT in NICM. We included patients with NICM and mid-myocardial septal substrate referred for VT ablation. After failed prolonged sequential unipolar RF lesions, simultaneous unipolar RF was delivered using 2 open-irrigated catheters at the site of earliest activation and/or best entrainment or pace mapping and at an anatomically adjacent/opposite site (up to 40 W for up to 3 minutes; RF energy independently titrated for each catheter to achieve an impedance drop of at least 15% from the baseline values). A total of 6 patients (mean age 62±13 years; mean left ventricular ejection fraction 38%±17%) were included. The clinical VTs were mapped at the anterior interventricular septum in 2 (33%) patients and at the inferior septum in 4 (67%). In all patients, prolonged sequential unipolar RF at the best activation/entrainment/pace-mapping site and at an anatomically opposite/adjacent site failed to eliminate VT. In 3 cases (50%), late VT termination with VT reinducibility was observed after sequential unipolar RF. Simultaneous unipolar ablation was then delivered, resulting in VT elimination and noninducibility in all patients. No procedural complications and no steam pops were observed. After a median follow-up of 20 months (range 13-20 months), 4 patients (67%) remained free of VT recurrence. In patients with NICM and intramural septal VT refractory to conventional RF ablation, simultaneous unipolar RF ablation is a safe and effective alternative ablation approach to improve long-term VT control.

Percutaneous Intramyocardial Septal Radiofrequency Ablation for Hypertrophic Obstructive Cardiomyopathy

BackgroundIn patients with disabling symptoms caused by hypertrophic obstructive cardiomyopathy (HOCM), echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) could be a less invasive treatment option. ObjectivesThis study aimed to investigate the safety and efficacy of the PIMSRA for left ventricular outflow tract (LVOT) gradient reduction in HOCM. MethodsThe study enrolled 15 patients with HOCM. These patients underwent electrocardiography, imaging, and blood biochemistry examination over 6 months of follow-up. ResultsAt 6 months of follow-up, patients showed significant reductions in peak LVOT gradients (resting gradient: from 88.00 [66.00] mm Hg to 11.00 [6.00] mm Hg; p = 0.001; stress-induced gradient: from 117.00 [81.00] mm Hg to 25.00 [20.00] mm Hg; p = 0.005) and interventricular septum (IVS) thickness (anterior IVS: from 25.00 [21.00] mm to 14.00 [12.00] mm; p = 0.001; posterior IVS: from 24.00 [21.00] mm to 14.00 [11.50] mm; p = 0.001). The reductions in IVS thickness and LVOT gradients were associated with improvement in New York Heart Association functional classification (from 3.00 [2.00] to 1.00 [1.00]; p < 0.001), total exercise time (from 6.00 [5.50] min to 9.00 [8.00] min; p = 0.007), and pro B-type natriuretic peptide levels (from 924.00 [370.45] pg/ml to 137.45 [75.73] pg/ml; p = 0.028). No patient had bundle branch block or complete heart block. ConclusionsPIMSRA is a safe and effective treatment approach for severe, symptomatic HOCM and results in sustained improvement in exercise capacity, persistent reduction in LVOT gradient, and sustained improvement in cardiac function.