Comparing outcomes of an 'early' versus 'late' diagnosis of cardiac sarcoidosis following a baseline presentation of high-grade atrioventricular block.

BACKGROUND: There is a paucity of evidence on impact of a delay in Cardiac Sarcoidosis (CS) diagnosis after high-grade atrioventricular-block (AVB) and this study aims to fill this void. METHODS: Consecutive CS patients (n = 77) with high grade AVB referred to one specialist hospital in London between February 2007 to February 2023 were retrospectively reviewed. The median time from AVB to diagnosing CS (112 days) was used to define the Early (n = 38) and Late (n = 39) cohorts. The primary endpoint was a composite of all-cause mortality, cardiac transplantation, ventricular arrhythmic events or heart failure hospitalisation. Secondary endpoints included difference in maintenance prednisolone dose, need for cardiac device upgrade and device complications. RESULTS: The mean age of the cohort was 54.4 (±10.6) years of whom 64 % were male and 81 % Caucasian. After a mean follow up of 54.9 (±45.3) months, the primary endpoint was reached by more patients from the Late cohort (16/39 vs. 6/38, p = 0.02; multivariable HR 6.9; 95 %CI 1.5-32.2, p = 0.01). Early Group were more likely to have received an Implantable Cardioverter Defibrillator or Cardiac Resynchronisation Therapy-defibrillator as index device after AVB (19/38 vs. 6/39; p < 0.01) and had fewer device upgrades (19/38 vs. 30/39, p = 0.01) and a trend towards fewer device complications (1 vs. 5, p = 0.20). The maintenance dose of prednisolone was significantly higher in Late Group [20.7(±9.7) mg vs. 15.3(±7.9) mg, p = 0.02]. CONCLUSION: A late diagnosis of CS was associated with more adverse events, a greater probability of needing a device upgrade and required higher maintenance steroid dose.

Role of serial 18F-fludeoxyglucose positron emission tomography in determining the therapeutic efficacy of immunosuppression and clinical outcome in patients with cardiac sarcoidosis.

BACKGROUND: Myocardial inflammation and perfusion defects detected by 18F-fludeoxyglucose (FDG) and Rubidium-82 positron emission tomography (PET) may be associated with ventricular arrhythmias (VAs) in cardiac sarcoidosis (CS). The role of serial quantitative PET in determining the effect of treatment on myocardial inflammation and clinical outcomes is yet to be defined. METHODS: Newly diagnosed CS patients with active myocardial inflammation (maximum standardised uptake value (SUVmax) ≥ 2.5) were treated with immunosuppression, then underwent repeat FDG-PET, Rubidium-82, and echocardiographic imaging 6-12 months later. Serial changes in SUVmax, SUVmean, inflammatory extent, perfusion defect (PD) extent, metabolism/perfusion mismatch extent, global cardiac metabolic activity, and left ventricular ejection fraction (LVEF) were assessed. The primary endpoint was a composite of all-cause mortality, serious VA and heart-failure (HF) hospitalisation. Event data were recorded from the date of the second FDG-PET. RESULTS: The study population consisted of 113 patients (66% male, age: 55 ± 11 years, LVEF: 54 ± 13%). SUVmax reduced from 4.5 (interquartile range: 3.3-7.1) to 2.7 (2.2-3.6). Overall, 94 (83%) patients saw serial reduction in SUVmax, with 42 (37%) demonstrating complete response (SUVmax <2.5). Following a median of 46 (25-57) months, 28 (25%) patients reached the endpoint (8 deaths, 17 VAs, and 3 HF hospitalisations). PD extent (Hazard ratio 1.03, 95% confidence interval: 1.01-1.05; p = 0.035) was a significant predictor of outcome following treatment, even after accounting for LVEF and change in SUVmean. The risk of adverse events was the greatest in those with a pre-treatment or post-treatment PD extent of >10%. CONCLUSION: In our cohort with active CS, following a treatment-induced reduction in myocardial inflammation, PD extent was the main predictor of adverse events.

Racial disparities among patients with cardiac sarcoidosis and arrhythmias in the United States: A propensity matched-analysis from the national inpatient sample database 2016-2020.

BACKGROUND: Cardiac sarcoidosis (CS) is frequently associated with conduction abnormalities and arrhythmias. In this study, we aim to evaluate racial disparities in the frequency of arrhythmias, and associated co-morbidities, among patients with CS. METHODS: White and African American (AA) patients diagnosed with CS were identified and compared from the 2016-2020 National Inpatient Sample (NIS) database whilst adjusting for confounders via logistic regression models. RESULTS: A total of 7,935 patients with CS were included in the study. The propensity-matched sample comprised of 5,570 patients, of whom 2,785 were White and 2,785 were AA. AA patients had a longer mean length of hospital stay (LOS) (7.84 vs. 6.94, p<0.01), a higher mean Charlson Comorbidity Index (CCI) score (3.10 vs. 2.84, p<0.01), and significantly higher incidences of cardiogenic shock [(9.2% vs 6.3%, p<0.01), aOR 1.45 (95% CI 1.17-1.78), p<0.01] and acute kidney injury (AKI) [(34.3% vs. 26.9%, p<0.01), aOR 1.41 (95% CI 1.24-1.61), p<0.01]. From an arrhythmia perspective, AA CS patients were shown to have a lower frequency of: (1) ventricular tachycardia (32.5% vs. 37.9%, p<0.01), (2) ventricular fibrillation (5.4% vs.7.2%, p<0.01), (3) first-degree AV block (1.8% vs. 4.1%, p<0.01), (4) complete AV block (6.3% vs. 14.2%, p<0.01), and (5) atrial fibrillation (31.8% vs. 34.8%, p=0.016) when compared to Whites with CS. Mortality remained higher for AAs (3.8% vs. 2.7%, p=0.024). CONCLUSION: Our study demonstrates a higher incidence of cardiac arrhythmias among White patients but a higher incidence of cardiogenic shock, AKI, mean LOS, and mortality among AA patients with cardiac sarcoidosis.

Two-center validation of Pilot Tone based cardiac triggering of a comprehensive cardiovascular magnetic resonance examination.

The electrocardiogram (ECG) signal is prone to distortions from gradient and radiofrequency interference and the magnetohydrodynamic effect during cardiovascular magnetic resonance imaging (CMR). Although Pilot Tone Cardiac (PTC) triggering has the potential to overcome these limitations, effectiveness across various CMR techniques has yet to be established. To evaluate the performance of PTC triggering in a comprehensive CMR exam. Fifteen volunteers and 20 patients were recruited at two centers. ECG triggered images were collected for comparison in a subset of sequences. The PTC trigger accuracy was evaluated against ECG in cine acquisitions. Two experienced readers scored image quality in PTC-triggered cine, late gadolinium enhancement (LGE), and T1- and T2-weighted dark-blood turbo spin echo (DB-TSE) images. Quantitative cardiac function, flow, and parametric mapping values obtained using PTC and ECG triggered sequences were compared. Breath-held segmented cine used for trigger timing analysis was collected in 15 volunteers and 14 patients. PTC calibration failed in three volunteers and one patient; ECG trigger recording failed in one patient. Out of 1987 total heartbeats, three mismatched trigger PTC-ECG pairs were found. Image quality scores showed no significant difference between PTC and ECG triggering. There was no significant difference found in quantitative measurements in volunteers. In patients, the only significant difference was found in post-contrast T1 (p = 0.04). ICC showed moderate to excellent agreement in all measurements. PTC performance was equivalent to ECG in terms of triggering consistency, image quality, and quantitative image measurements across multiple CMR applications.

Predictors of outcome in a contemporary cardiac sarcoidosis population: Role of brain natriuretic peptide, left ventricular function and myocardial inflammation.

AIMS: Cardiac sarcoidosis (CS) is a potentially fatal condition that varies in its clinical presentation. Here, we describe baseline characteristics at presentation along with prognosis and predictors of outcome in a sizable and deeply phenotyped contemporary cohort of CS patients. METHODS AND RESULTS: Consecutive CS patients seen at one institution were retrospectively enrolled after undergoing laboratory testing, electrocardiogram, echocardiography, cardiac magnetic resonance (CMR) imaging and 18 F-flourodeoxyglucose positron emission tomography (FDG-PET) at baseline. The composite endpoint consisted of all-cause mortality, aborted sudden cardiac death, major ventricular arrhythmic events, heart failure hospitalization and heart transplantation. A total of 319 CS patients were studied (67% male, 55.4 ± 12 years). During a median follow-up of 2.2 years (range: 1 month-11 years), 8% of patients died, while 33% reached the composite endpoint. The annualized mortality rate was 2.7% and the 5- and 10-year mortality rates were 6.2% and 7.5%, respectively. Multivariate analysis showed serum brain natriuretic peptide (BNP) levels (hazard ratio [HR] 2.41, 95% confidence interval [CI] 1.34-4.31, p = 0.003), CMR left ventricular ejection fraction (LVEF) (HR 0.96, 95% CI 0.94-0.98, p < 0.0001) and maximum standardized uptake value of FDG-PET (HR 1.11, 95% CI 1.04-1.19, p = 0.001) to be independent predictors of outcome. These findings remained robust for different patient subgroups. CONCLUSION: Cardiac sarcoidosis is associated with significant morbidity and mortality, particularly in those with cardiac involvement as the first manifestation. Higher BNP levels, lower LVEF and more active myocardial inflammation were independent predictors of outcomes.

Safety of tracheostomy during extracorporeal membrane oxygenation support: A single-center experience.

BACKGROUND: Some patients on extracorporeal membrane oxygenation (ECMO) require prolonged mechanical ventilation. An early tracheostomy strategy while on ECMO has appeared to be beneficial for these patients. This study aims to explore the safety of tracheostomy in ECMO patients. METHODS: This is a retrospective observational single-center study. RESULTS: Hundred and nine patients underwent tracheostomy (76 percutaneous and 33 surgical) during V-V ECMO support over an 8-year period. Patients with a percutaneous tracheostomy showed a significantly shorter ECMO duration [25.5 (17.3-40.1) vs 37.2 (26.5-53.2) days, p = 0.013] and a shorter ECMO-to-tracheostomy time [13.3 (8.5-19.7) vs 27.8 (16.3-36.9) days, p < 0.001] compared to those who underwent a surgical approach. There was no difference between the two strategies regarding both major and minor/no bleeding (p = 0.756). There was no difference in survival rate between patients who underwent percutaneous or surgical tracheostomy (p = 0.173). Patients who underwent an early tracheostomy (within 10 days from ECMO insertion) showed a significantly shorter hospital stay (p < 0.001) and a shorter duration of V-V ECMO support (p < 0.001). Our series includes 24 patients affected by COVID-19, who did not show significantly higher rates of major bleeding when compared to non-COVID-19 patients (p = 0.297). Within the COVID-19 subgroup, there was no difference in major bleeding rates between surgical and percutaneous approach (p = 1.0). CONCLUSIONS: Percutaneous and surgical tracheostomy during ECMO have a similar safety profile in terms of bleeding risk and mortality. Percutaneous tracheostomy may favor a shorter duration of ECMO support and hospital stay and can be considered a safe alternative to surgical tracheostomy, even in COVID-19 patients, if relevant clinical expertise is available.

A Rare Mechanism for Spontaneous Closure of Muscular Ventricular Septal Defect in Hypertrophic Cardiomyopathy.

In the context of hypertrophic cardiomyopathy (HCM), a ventricular septal defect (VSD) is a rare finding. We present the case of a large spontaneously closed muscular VSD in a patient with HCM. We describe the role of cardiovascular magnetic resonance in the assessment of a VSD and its differential diagnosis in HCM. (Level of Difficulty: Advanced.).

Two-center validation of Pilot Tone Based Cardiac Triggering of a Comprehensive Cardiovascular Magnetic Resonance Examination.

Background: The electrocardiogram (ECG) signal is prone to distortions from gradient and radiofrequency interference and the magnetohydrodynamic effect during cardiovascular magnetic resonance imaging (CMR). Although Pilot Tone Cardiac (PTC) triggering has the potential to overcome these limitations, effectiveness across various CMR techniques has yet to be established. Purpose: To evaluate the performance of PTC triggering in a comprehensive CMR exam. Methods: Fifteen volunteers and twenty patients were recruited at two centers. ECG triggered images were collected for comparison in a subset of sequences. The PTC trigger accuracy was evaluated against ECG in cine acquisitions. Two experienced readers scored image quality in PTC-triggered cine, late gadolinium enhancement (LGE), and T1- and T2-weighted dark-blood turbo spin echo (DB-TSE) images. Quantitative cardiac function, flow, and parametric mapping values obtained using PTC and ECG triggered sequences were compared. Results: Breath-held segmented cine used for trigger timing analysis was collected in 15 volunteers and 14 patients. PTC calibration failed in three volunteers and one patient; ECG trigger recording failed in one patient. Out of 1987 total heartbeats, three mismatched trigger PTC-ECG pairs were found. Image quality scores showed no significant difference between PTC and ECG triggering. There was no significant difference found in quantitative measurements in volunteers. In patients, the only significant difference was found in post-contrast T1 (p = 0.04). ICC showed moderate to excellent agreement in all measurements. Conclusion: PTC performance was equivalent to ECG in terms of triggering consistency, image quality, and quantitative image measurements across multiple CMR applications.

A rare case of extensive biventricular cardiac sarcoidosis with reversible torrential tricuspid regurgitation.

Reversal of torrential tricuspid regurgitation is rarely seen. We describe a case in which effective immunosuppression alongside conventional heart failure therapies lead to reversibility of torrential tricuspid regurgitation in a patient with cardiac sarcoidosis. We also discuss the diagnostic challenge in distinguishing cardiac sarcoidosis from other myocardial diseases in a patient presenting with biventricular failure.

Diagnosis of cardiac sarcoidosis in patients presenting with cardiac arrest or life-threatening arrhythmias.

OBJECTIVE: Cardiac sarcoidosis (CS) may present with cardiac arrest or life-threatening arrhythmias. There are limited data on this subgroup of patients with CS. Advanced imaging including cardiovascular magnetic resonance (CMR) and cardiac 18-fluorodeoxyglucose (FDG) positron emission tomography (PET) are used for diagnosis. This study aimed to describe advanced imaging patterns suggestive of CS among patients presenting with cardiac arrest or life-threatening arrhythmias. METHODS: An imaging database of a CS referral centre (Royal Brompton Hospital, London) was screened for patients presenting with cardiac arrest or life-threatening arrhythmias and having imaging features of suspected CS. Patients diagnosed with definite or probable/possible CS were included. RESULTS: Study population included 60 patients (median age 49 years) with male predominance (76.7%). The left ventricle was usually non-dilated with mildly reduced ejection fraction (53.4±14.8%). CMR studies showed extensive late gadolinium enhancement (LGE) with 5 (4-8) myocardial segments per patient affected; the right ventricular (RV) side of the septum (28/45) and basal anteroseptum (28/45) were most frequently involved. Myocardial inflammation by FDG-PET was detected in 45 out of 58 patients vs 11 out of 33 patients with oedema imaging available on CMR. When PET was treated as reference to detect myocardial inflammation, CMR oedema imaging was 33.3% sensitive and 77% specific. CONCLUSIONS: In patients with CS presenting with cardiac arrest or life-threatening arrhythmias, LGE was located in areas where the cardiac conduction system travels (basal anteroseptal wall and RV side of the septum). While CMR was the imaging technique that raised possibility of cardiac scarring, oedema imaging had low sensitivity to detect myocardial inflammation compared with FDG-PET.

Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement.

AIMS: Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. METHODS AND RESULTS: The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. CONCLUSIONS: 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

Diagnostic and Prognostic Value of Cardiovascular Magnetic Resonance in Neuromuscular Cardiomyopathies.

Neuromuscular diseases (NMD) encompass a broad spectrum of diseases with variable type of cardiac involvement and there is lack of clinical data on Cardiovascular Magnetic Resonance (CMR) phenotypes or even prognostic value of CMR in NMD. We explored the diagnostic and prognostic value of CMR in NMD-related cardiomyopathies. The study included retrospective analysis of a cohort of 111 patients with various forms of NMD; mitochondrial: n = 14, Friedreich's ataxia (FA): n = 27, myotonic dystrophy: n = 27, Becker/Duchenne's muscular dystrophy (BMD/DMD): n = 15, Duchenne's carriers: n = 6, other: n = 22. Biventricular volumes and function and myocardial late gadolinium enhancement (LGE) pattern and extent were assessed by CMR. Patients were followed-up for the composite clinical endpoint of death, heart failure development or need for permanent pacemaker/intracardiac defibrillator. The major NMD subtypes, i.e. FA, mitochondrial, BMD/DMD, and myotonic dystrophy had significant differences in the incidence of LGE (56%, 21%, 62% & 30% respectively, chi2 = 9.86, p = 0.042) and type of cardiomyopathy phenotype (chi2 = 13.8, p = 0.008), extent/pattern (p = 0.006) and progression rate of LGE (p = 0.006). In survival analysis the composite clinical endpoint differed significantly between NMD subtypes (p = 0.031), while the subgroup with LGE + and LVEF < 50% had the worst prognosis (Log-rank p = 0.0034). We present data from a unique cohort of NMD patients and provide evidence on the incidence, patterns, and the prognostic value of LGE in NMD-related cardiomyopathy. LGE is variably present in NMD subtypes and correlates with LV remodelling, dysfunction, and clinical outcomes in patients with NMD.

Cardiac Decompression by Pericardiectomy for Constrictive Pericarditis: Multimodality Imaging to Identify Patients at Risk for Prolonged Inotropic Support.

BACKGROUND: Post-pericardiectomy right ventricular (RV) failure has been reported but it remains not well-studied. To investigate imaging parameters that could predict RV function and the outcome of patients post-pericardiectomy. METHODS: We analysed data from a total of 53 CP patients undergoing pericardiectomy. Preoperative, early and at 6 months postoperative echocardiographic (echo) imaging datasets were analysed and correlated with preoperative cardiac magnetic resonance (CMR), cardiac computed tomography scans and histology. The primary endpoint of the study was RV functional status early postoperatively and at 6 months. Secondary endpoint was the need for prolonged inotropic support. RESULTS: A cause of CP was identified in 26 patients (49%). Inotropic support ≥ 48 hours was required in n = 28 (53%) of patients and was correlated with lower preoperative RV areas by echo or RV volumes by CMR (p < 0.05 for all). A pericardial score based on pericardial thickness/calcification and epicardial fat thickness had good diagnostic accuracy to identify patients requiring prolonged use of inotropes (area under the curve, 0.825; 95% confidence interval, 0.674-0.976). Pericardiectomy resulted in RV decompression and impaired RV function early postoperatively (fractional area change: 40.5% ± 8.8% preoperatively vs. 31.4% ± 10.4% early postoperatively vs. 42.5% ± 10.2% at 6 months, p < 0.001). CONCLUSIONS: We show that a smaller RV cavity size and a pericardial scoring system are associated with prolonged inotropic support in CP patients undergoing pericardiectomy. RV systolic impairment post decompression is present in most patients, but it is only transient.

Eosinophilic heart disease: diagnostic and prognostic assessment by cardiac magnetic resonance.

AIMS: Eosinophilic heart disease (EHD) is a rare cardiac condition with a wide spectrum of phenotypes. The diagnostic and prognostic value of cardiac magnetic resonance (CMR) in EHD remains unknown. METHODS AND RESULTS: This was a retrospective analysis of 250 patients with eosinophilia referred for a CMR scan (period 2000-2020). CMR data sets and clinical/laboratory data were collected. Patients were followed up for a mean of 24 months (range 1-224) for the composite endpoint of death, acute coronary syndrome, hospitalization for acute heart failure, malignant ventricular arrhythmias, or the need for implantable cardiac defibrillator/pacemaker. The main objectives were to explore the diagnostic value of CMR in EHD; relationships between cardiac function, late gadolinium enhancement (LGE), and EHD phenotypes; and the prognostic value of fibrosis and oedema by CMR. The prevalence of findings compatible with EHD was 39% (patients with cardiac symptoms: 57% vs. screening: 20%, P < 0.001). EHD phenotypes included subendocardial LGE (n = 58), mid-wall/subepicardial LGE (n = 26), pericarditis (n = 5) or dilated cardiomyopathy (n = 8). Myocardial oedema was present in 10% of patients. Intracardiac thrombi (7%) were associated with EHD phenotype (χ2=47.3, P = 1.3×10-8). LGE extent correlated with LVEDVi (rho = 0.268, P = 5.3×10-5) and LVEF (rho=-0.415, P = 8.6×10-11). A CMR scan positive for EHD [hazard ratio (HR) = 5.61, 95% confidence interval (CI): 1.82-17.89, P = 0.0026] or a subendocardial LGE pattern (HR = 5.13, 95% CI: 1.29-20.38, P = 0.020) were independently associated with the composite clinical endpoint. CONCLUSION: The diagnostic yield of CMR screening in patients with persistent eosinophilia, even if asymptomatic, is high. The extent of subendocardial fibrosis correlates with LV remodelling and independently predicts clinical outcomes in patients with eosinophilia.

A case report of a primary cardiac lymphoma causing superior vena cava obstruction: the value of multimodality imaging in the clinical workup.

BACKGROUND: This is a case report of a primary cardiac lymphoma with an unusual clinical presentation. We hereby illustrate the characteristic features of cardiac lymphomas by multimodality imaging and particularly cardiac magnetic resonance (CMR) that can help reach a timely diagnosis non-invasively and guide treatment decisions. CASE SUMMARY: A 58-year-old woman, without significant past medical history, presented with a 3-week history of shortness of breath associated with facial and neck swelling. Transthoracic echocardiogram confirmed the presence of a cardiac mass in the right atrium. Cardiac magnetic resonance helped to characterize the mass, assess its haemodynamic significance and relation to cardiac structures, and reach a non-invasive diagnosis that was crucial for guiding treatment decisions and interventions. DISCUSSION: Cardiac masses have distinct imaging features that can help differentiate malignant from benign cardiac tumours. More specifically, primary cardiac lymphomas can be relatively easy diagnosed by CMR in most cases thanks to their characteristic imaging appearance.

Extensive cardiac infiltrative melanoma.

We report an unusual clinical case of a 66-year-old patient with cardiac involvement from a metastatic melanoma, causing the formation of a large right atrial mass with extensive infiltration of the right atrial free wall, the interatrial septum, the coronary sinus, and up to the mitral annulus and posterior wall of the right ventricle, unamendable to complete surgical excision. As secondary cardiac tumors are not part of routine daily clinical practice, we thought that this clinical case would be a good educational opportunity for the practicing clinicians, both specialists and nonspecialists.