Prediction of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging using electrocardiography findings in patients with confirmed cardiac amyloidosis

Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without high voltage R wave in V5 leads on ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invade the right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To predict sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance imaging (MRI), suggesting amyloid invasion, using cardiac rhythm and other ECG findings in patients with cardiac amyloidosis confirmed by biopsy. Methods A total of 26 patients (11 females) with suspected cardiac amyloidosis, showing LV wall thickening by TTE without a high voltage R wave in V5 leads on ECG, underwent cardiac enhanced CT. LV wall thickening on CT in the early phase led to late phase acquisition to detect LE. Five patients (3 females, mean age 73 years) were diagnosed with cardiac amyloidosis: complicated multiple myeloma, 2; senile ATTR (transthyretin) amyloidosis, 1; immunoglobulin light chain (AL) amyloidosis 1; and transthyretin mutation, 1. Four patients underwent cardiac MRI. Results Two patients (cases 1 and 2) had SSS (junctional rhythm), one had atrial tachycardia, and the remaining two (cases 4 and 5) had a normal sinus rhythm. In case 1, ECG showed a left axis QRS wave deviation, no low voltage R wave in limb leads and a mild LA load. Wall thickening in the basal interventricular septum (IVS), LV inferior-posterior wall, LA on CT, LE in the endocardium in whole LV, RV, and RA on CT, and LE in the endocardium in whole LV, RV, LA, and IVS on MRI were observed. In case 2, ECG showed a normal QRS wave axis, no low voltage R wave in limb leads, no LA load, wall thickening in whole LV, RV, LA, and IVS on CT, and unclear (CT) or no (MRI) LE. In case 3, ECG showed a normal QRS wave axis, with low voltage R wave in limb leads, no LA load, wall thickening in LA and basal IVS on CT, LE in LA and basal IVS on CT, and LE in LA only on MRI. In case 4, ECG showed left axis QRS wave deviation, a low voltage R wave in limb leads, and no LA load, wall thickening in the LA and RV moderator band on CT, unclear LE on CT, and LE in whole LV, endocardium in the RV, and whole IVS on MRI. In case 5, ECG showed a right axis QRS wave deviation, low voltage R wave in limb leads, and a mild LA load, wall thickening in the IVS, LV lateral wall, LV anterior wall, RA, RV outflow tract, and RA appendage, and no LE on CT (MRI not performed). Conclusions This pilot study of a few patients with cardiac amyloidosis revealed few links between cardiac rhythm and other ECG findings with sites of wall thickening and abnormal LE. However, a longer-term study of more patients may lead to detecting an association between these variables with this methodology. FUNDunding Acknowledgement Type of funding sources: None.

Myocardial tissue function and characterization in patients with idiopathic cardiac magnetic resonance myopericarditis: evolutionary analysis

Introduction The identification of prognostic markers related to the occurrence of events and recovery of ventricular function may be important in patients with acute myopericarditis (AMP). There is still a lack of data related to tissue characterization by cardiac magnetic resonance (CMR) of AMP, evolution and definition of possible long-term prognostic markers. Purpose To evaluate the myocardial tissue characterization of CMR related to the occurrence of combined events (death from all causes, heart failure and AMP recurrence) and the increase in left ventricular ejection fraction (LVEF) in patients with AMP. Methods Inclusion criteria were chest pain and/or electrocardiographic changes associated with elevated troponin (above the 99th percentile) in the absence of coronary stenosis and diagnosis of AMP by CMR <48 hours of admission confirmed by the presence of edema and/or late enhancement. After a follow-up of up to 24 months, 100 patients remained and in the assessment of the increase in LVEF (increase >5%), 36 cases remained, recalled for a new CMR between 6 and 18 months from the initial event. Results Significant differences in CMR were found between patients who had combined events (n=26) versus no combined events (n=74) in the following characteristics evaluated: initial LVEF (OR=0.938; CI: 0.895–0.984, p=0.008), left ventricular (LV) systolic volume index (OR=1.034; CI: 1.005–1.062, p=0.019), LV diastolic volume index (OR=1.029; CI: 1.002–1.056, p=0.038), presence of hypersignal in T2 (OR=11.325; CI: 2.247–57.075, p=0.003), presence of late anteroseptal enhancement (OR=0.160; CI: 0.037–0.685, p=0.014), basal anteroseptal (OR=0.255; CI: 0.071–0.914, p=0.036) and lateral apical (OR=5.902; CI: 1.236–28.187, p=0.026). In relation to the increase in LVEF, significant differences were found in CMR in the following characteristics evaluated: LVEF (OR=0.870; CI: 0.758–0.988, p=0.047), end systolic volume of the right ventricle (OR=1.047; CI: 1.001–1.096, p=0.047), LV systolic diameter (OR=1.283; CI: 1.034–1.593, p=0.023), LV diastolic diameter (OR=1.225; CI: 1.012–1.482, p=0.038), LV systolic volume index (OR=1.340; CI: 1.066–1.685, p=0.012), LV diastolic volume index (OR=1.111; CI: 1.017–1.213, p=0.019) and right ventricular systolic volume index (OR=1.116; CI: 1.006–1.236, p=0.037). Conclusion We observed a significant association between combined events in the long-term follow-up with initial LVEF, LV systolic and diastolic volume indexes, T2 hypersignal and the presence of mid and basal anteroseptal and lateral apical late enhancement. Already related to the increase in LVEF in evolutionary CMR, we observed a significant association with initial LVEF, end systolic volume of the right ventricle, LV systolic and diastolic diameters, LV systolic and diastolic volume indexes and right ventricle systolic volume index. FUNDunding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): FAPESP

Management of Spontaneous Bleeding in COVID-19 Inpatients: Is Embolization Always Needed?

Background: critically ill patients with SARS-CoV-2 infection present a hypercoagulable condition. Anticoagulant therapy is currently recommended to reduce thrombotic risk, leading to potentially severe complications like spontaneous bleeding (SB). Percutaneous transcatheter arterial embolization (PTAE) can be life-saving in critical patients, in addition to medical therapy. We report a major COVID-19 Italian Research Hospital experience during the pandemic, with particular focus on indications and technique of embolization. Methods: We retrospectively included all subjects with SB and with a microbiologically confirmed SARS-CoV-2 infection, over one year of pandemic, selecting two different groups: (a) patients treated with PTAE and medical therapy; (b) patients treated only with medical therapy. Computed tomography (CT) scan findings, clinical conditions, and biological findings were collected. Results: 21/1075 patients presented soft tissue SB with an incidence of 1.95%. 10/21 patients were treated with PTAE and medical therapy with a 30-days survival of 70%. Arterial blush, contrast late enhancement, and dimensions at CT scan were found discriminating for the embolization (p < 0.05). Conclusions: PTAE is an important tool in severely ill, bleeding COVID-19 patients. The decision for PTAE of COVID-19 patients must be carefully weighted with particular attention paid to the clinical and biological condition, hematoma location and volume.

Laminopathies: Should Wenckebach be a cause for concern? A case report

Background LMNA cardiomyopathy is a cause of dilated cardiomyopathy (DCM) characterised by aggressive heart failure, high risk of arrhythmias and sudden cardiac death. We present a case of a male presenting with a LMNA mutation with an aggressive DCM leading to sudden cardiac death (SCD). Case summary A 42-year-old male presented with the feeling of lethargy and intermittent dizziness. ECG demonstrated AV block in keeping with Mobitz type 1, at a rate of 40 b.p.m. and cardiac monitoring showed non-sustained VT. CMR imaging showed preserved left ventricular function (EF 59%) but features suggesting DCM. These included mild LV dilatation with an EDV of 213 mL and late enhancement showing a single mid myocardial focus of high signal over the distal RV insertion point inferiorly and a linear area of high signal over the basal septum. After discussion at cardiology multi-disciplinary meeting a pacemaker was implanted so that beta-blockers could be initiated to suppress the ventricular arrhythmias. A laminopathy was suspected and if this was confirmed from genetic testing the plan was to upgrade to an implantable defibrillator. Due to stability this was decided to be done in an outpatient setting. He unfortunately had an out of hospital VF arrest and died. Post-mortem showed subtle cardiomyopathy in keeping with a DCM. Genetic tests results were returned a few months later which confirmed a pathogenic variant in LMNA. Discussion Because of the complexity of LMNA-related cardiac disease, they should be managed and followed up in centres with special expertise in inherited cardiomyopathy.

Progressive Heart Failure and Death as the Initial Manifestation of NK/T-Cell Lymphoma: A Case Report and Literature Review

Natural killer/T-cell (NK/T-cell) lymphoma is a rare-type non-Hodgkin lymphoma derived from NK cells or cytotoxic T cells. Here, we present a case of a 40-year-old woman who experienced quick-developed global heart failure and then was diagnosed with NK/T-cell lymphoma through lymphoid biopsy. Neither transthoracic echocardiography nor any radiological images detected a mass in her heart or pericardium. Elevated plasma troponin level and diffused patchy areas of gadolinium late enhancement on cardiac magnetic resonance were compatible with myocarditis. Considering the persistently elevated cytokine level, systemic inflammation symptoms, acute respiratory distress syndrome, and cardiac dysfunction, a cytokine storm secondary to NK/T-cell lymphoma was considered. Due to the refractory malignant arrhythmia, the patient died soon after being admitted to our hospital.

Cardiac manifestations and short-term outcomes of Multisystem Inflammatory Syndrome in Middle Eastern Children during the COVID-19 pandemic: A case series

We herein report on a series of 4 patients presented to our tertiary care center with features of multisystem inflammatory syndrome in children (MIS-C) and cardiac involvement. Two of our patients had recent exposure to a COVID-19 positive patient, 1 had recent documented infection, and another had no known positive contact. All patients tested positive for Severe acute respiratory syndrome coronavirus 2 Immunoglobulin G (SARS-CoV-2 IgG) antibody at the time of presentation. All of them fulfilled the diagnostic criteria according to World Health Organization Centers for Disease Control or the British guidelines for MISC (fever for ≥3 days, multisystem involvement (at least 2), elevated markers of inflammation and no other alternative diagnosis). (1, 2,3) Cardiac involvement was variable ranging from isolated ectasia of the coronary arteries to full blown pan-carditis: severe biventricular dysfunction, multi-valvar involvement, and pericardial effusion. All our patient received Intravenous immunoglobulin IVIG (2 g/kg), methylprednisolone, and aspirin and some required inotropic support and ICU admission. Remarkably, all our patients showed significant improvement in their cardiac disease within few days as evident on serial echocardiographic evaluation. However, we stress the need for long term follow up as one of our patients demonstrated mild LV myocardial scarring as evident by gadolinium late enhancement on a Cardiac MRI.

Prognostic assessment following acute myocarditis requires convalescent scanning: neither peak troponin nor index scan T2 signal predicts convalescent late gadolinium enhancement

Funding Acknowledgements Type of funding sources: None. Background Cardiovascular magnetic resonance (CMR) is a key diagnostic investigation in acute myocarditis (1) and permits quantification of late gadolinium enhancement (LGE) and myocardial oedema. Follow-up CMR imaging is recommended to check for persistence of scar and oedema (2). Persistent late gadolinium enhancement is associated with a worse prognosis (3). It is not known whether all patients require follow-up scanning or whether the initial scan can provide useful information to identify which patients need convalescent assessment. Purpose In this study we considered whether extent of troponin elevation, extent of T2 elevation and initial late gadolinium enhancement burden predicted long-term late gadolinium enhancement at follow-up. Methods Index and follow-up CMR scans of consecutive patients presenting with a diagnosis of acute myocarditis between 2019 and 2020 across three hospitals were included. Inclusion criteria were: follow-up scan within 9 months of the index scan, CMR with LGE imaging and T2 mapping, and acute myocarditis being the primary diagnosis of the index scan. Myocardial T2 values in the area affected by myocarditis and percentage of LV myocardium showing late enhancement (using a threshold-based full height half width or manual region of interest strategy) were extracted. Results 20 patients were included in the study (80% male; mean age 37 years). Mean interval between the index and follow-up scan was 4.1 months. Peak troponin level during the acute illness was not associated with the proportion of LV myocardium affected by LGE in the index scan (R^2 &lt;0.01) (Figure 1A). Myocardial T2 values in the first scan were not associated with the proportional resolution in LGE between the index and follow-up scans (R^2 0.02) (Figure 1B). The mean change in LGE was -61.7% (+/-22.8%) but the initial LGE burden did not predict the proportional degree of improvement in LGE between scans (R^2 &lt;0.01)(Figure 1C). Conclusions The extent of troponin elevation and initial CMR phenotype was not a good predictor of the burden of long-term late gadolinium enhancement. Although most cases showed improvement in LGE scar burden between index and follow-up imaging, neither peak troponin level during the acute episode, nor T2 values at the first CMR scan were predictive of initial or change in scar burden. Serial CMR assessment is required to identify those patients who have residual long-term scarring.