Prognostic Role of the Progression of Late Gadolinium Enhancement in Hypertrophic Cardiomyopathy.

In hypertrophic cardiomyopathy (HCM), late gadolinium enhancement (LGE) extent ≥15% of left ventricular mass is considered a prognostic risk factor. LGE extent increases over time and the clinical role of the progression of LGE over time (LGE rate) was not prospectively evaluated. We sought to evaluate the prognostic role of the LGE rate in HCM. We enrolled 105 patients with HCM who underwent cardiac magnetic resonance (CMR) at baseline (CMR-I) and after ≥2 years of follow-up (CMR-II). LGE rate was defined as the ratio between the increase of LGE extent (grams) and the time interval (months) between examinations. A combined end point of sudden cardiac death, resuscitated cardiac arrest, appropriate Implanted Cardioverter Defibrillator (ICD) intervention, and sustained ventricular tachycardia was used (hard events). The percentage of patients with LGE extent ≥15% increased from 9% to 20% from CMR-I to CMR-II (p = 0.03). During a median follow-up of 52 months, 25 hard events were recorded. The presence of LGE ≥15% at CMR-II allowed a significant reclassification of the risk of patients than at LGE ≥15% at CMR-I (net reclassification improvement 0.21, p = 0.046). On the MaxStat analysis, the optimal prognostic cut point for LGE rate was >0.07 g/month. On the Kaplan-Meier curve, patients with LGE rate >0.07 had worse prognosis than those without (p <0.0001). LGE rate >0.07 allowed a significant reclassification of the risk compared with LGE ≥15% at CMR-I and at CMR-II (net reclassification improvement 0.49, p = 0.003). In the multivariable models, LGE rate >0.07 was the best independent predictor of hard events. In conclusion, CMR should be repeated after 2 years to reclassify the risk for sudden death of those patients. A high LGE rate may be considered a novel prognostic factor in HCM.

Diagnostic Role of Native T1 Mapping Compared to Conventional Magnetic Resonance Techniques in Cardiac Disease in a Real-Life Cohort.

We sought to compare native T1 mapping to conventional late gadolinium enhancement (LGE) and T2-STIR techniques in a cohort of consecutive patients undergoing cardiac MRI (CMR). CMR was performed in 323 patients, 206 males (64%), mean age 54 ± 8 years, and in 27 age- and sex- matched healthy controls. In T2-STIR images, myocardial hyperintensity suggesting edema was found in 41 patients (27%). LGE images were positive in 206 patients (64%). T1 mapping was abnormal in 171 (49%). In 206 patients (64%), a matching between LGE and native T1 was found. T1 was abnormal in 32 out of 41 (78%) with edema in T2-STIR images. Overall, LGE and/or T2-STIR were abnormal in 209 patients, whereas native T1 was abnormal in 154 (52%). Conventional techniques and T1 mapping were concordant in 208 patients (64%). In 39 patients, T1 mapping was positive despite negative conventional techniques (12%). T1 mapping was able in conditions with diffuse myocardial damage such as cardiac amyloidosis, scleroderma, and Fabry disease (additive role in 42%). In contrast, T1 mapping was less effective in cardiac disease with regional distribution of myocardial damage such as myocardial infarction, HCM, and myocarditis. In conclusion, conventional LGE/T2-STIR and T1 mapping are complementary techniques and should be used together in every CMR examination.

Dark papillary muscles sign: a novel prognostic marker for cardiac magnetic resonance.

OBJECTIVES: The prognostic role of left ventricular (LV) papillary muscle abnormalities in patients with preserved LV systolic ejection fraction (LVEF) is unknown. We sought to evaluate the prognosis role of LV papillary muscle abnormalities by CMR in patients with ventricular arrhythmias, preserved LVEF with no cardiac disease. METHODS: A total of 391 patients with > 500/24 h premature ventricular complexes and/or with non-sustained ventricular tachycardia (NSVT), preserved LVEF, and no cardiac disease were enrolled. Different features of LV papillary muscles were considered: supernumerary muscles, papillary thickness, the attachment, late gadolinium enhancement (LGE). Dark-Paps was defined as end-systolic signal hypointensity of both papillary muscles in early post-contrast cine CMR images. Mitral valve prolapse, mitral annular disjunction (MAD), and myocardial LGE were considered. RESULTS: Dark-Paps was found in 79 (20%) patients and was more frequent in females. It was associated with higher prevalence of mitral valve prolapse and MAD. During a median follow-up of 2534 days, 22 hard cardiac events occurred. At Kaplan-Meier curve analysis, patients with Dark-Paps were at higher risk of events than those without (p < 0.0001). Dark-Paps was significantly associated with hard cardiac events in all the multivariate models. Dark-Paps improved prognostic estimation when added to NSVT (p = 0.0006), to LGE (p = 0.005) and to a model including NSVT+LGE (p = 0.014). Dark-Paps allowed a significant net reclassification when added to NSVT (NRI 0.30, p = 0.03), to LGE (NRI 0.25, p = 0.04), and to NSVT + LGE (NRI 0.32, p  = 0.02). CONCLUSIONS: In LV papillary muscles, Dark-Paps is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. KEY POINTS: • Papillary muscle abnormalities are seen in patients with ventricular arrhythmias and preserved left ventricular ejection fraction. • Early post-contrast hypointensity of papillary muscles in end-systolic cine images (Dark-Paps) is a novel prognostic marker in patients with ventricular arrhythmias and preserved ejection fraction. • Dark-Paps had an additive prognostic role over late gadolinium enhancement and non-sustained ventricular tachycardia.

Incidence of acute myocarditis and pericarditis during the coronavirus disease 2019 pandemic: comparison with the prepandemic period.

BACKGROUND: Myocarditis and pericarditis have been proposed to account for a proportion of cardiac injury during SARS-CoV-2 infection. The impact of COVID-19 the pandemic on the incidence of this acute inflammatory cardiac disease was not systematically evaluated. AIM: To examine the incidence and prevalence of inflammatory heart disorders prior to and during the COVID-19 pandemic. METHODS: We compared the incidence and prevalence of acute inflammatory heart diseases (myocarditis, pericarditis) in the provinces of Pisa, Lucca and Livorno in two time intervals: prior to (PRECOVID, from 1 June 2018 to 31 May 2019) and during the COVID-19 pandemic (COVID, from 1 June 2020 to May 2021). RESULTS: Overall 259 cases of inflammatory heart disease (myocarditis and/or pericarditis) occurred in the areas of interest. The annual incidence was of 11.3 cases per 100 000 inhabitants. Particularly, 138 cases occurred in the pre-COVID, and 121 in the COVID period. The annual incidence of inflammatory heart disease was not significantly different (12.1/100 000 in PRECOVID vs 10.3/100 000 in COVID, P = 0.22). The annual incidence of myocarditis was significantly higher in PRECOVID than in COVID, respectively 8.1/100 000/year vs. 5.9/100 000/year (P = 0.047) consisting of a net reduction of 27% of cases. Particularly the incidence of myocarditis was significantly lower in COVID than in PRECOVID in the class of age 18-24 years. Despite this, myocarditis of the COVID period had more wall motion abnormalities and greater LGE extent. The annual incidence of pericarditis was, instead, not significantly different (4.03/100 000 vs, 4.47/100 000, P = 0.61). CONCLUSION: Despite a possible etiologic role of SARS-CoV-2 and an expectable increased incidence of myocarditis and pericarditis, data of this preliminary study, with a geographically limited sample size, suggest a decrease in acute myocarditis and a stable incidence of pericarditis and of myopericarditis/perimyocarditis.

Magnetic Resonance for Differential Diagnosis of Left Ventricular Hypertrophy: Diagnostic and Prognostic Implications.

BACKGROUND: Left ventricular hypertrophy (LVH) may be due to different causes, ranging from benign secondary forms to severe cardiomyopathies. Transthoracic Echocardiography (TTE) and ECG are the first-level examinations for LVH diagnosis. Cardiac magnetic resonance (CMR) accurately defines LVH type, extent and severity. OBJECTIVES: to evaluate the diagnostic and prognostic role of CMR in patients with TTE and/or ECG evidence of LVH. METHODS: We performed CMR in 300 consecutive patients with echocardiographic and/or ECG signs of LVH. RESULTS: Overall, 275 patients had TTE evidence of LVH, with initial suspicion of hypertrophic cardiomyopathy (HCM) in 132 (44%), cardiac amyloidosis in 41 (14%), hypertensive LVH in 48 (16%), aortic stenosis in 4 (1%), and undetermined LVH in 50 (16%). The initial echocardiographic diagnostic suspicion of LVH was confirmed in 172 patients (57.3%) and changed in 128 patients (42.7%, p < 0.0001): the diagnosis of HCM increased from 44% to 71% of patients; hypertensive and undetermined LVH decreased significantly (respectively to 4% and 5%). CMR allowed for a diagnosis in 41 out of 50 (82%) patients with undetermined LVH at TTE. CMR also identified HCM in 17 out of 25 patients with apparently normal echocardiography but with ECG criteria for LVH. Finally, the reclassification of the diagnosis by CMR was associated with a change in survival risk of patients: after CMR reclassification, no events occurred in patients with undetermined or hypertensive LVH. CONCLUSIONS: CMR changed echocardiographic suspicion in almost half of patients with LVH. In the subgroup of patients with abnormal ECG, CMR identified LVH (particularly HCM) in 80% of patients. This study highlights the indication of CMR to better characterize the type, extent and severity of LVH detected at echocardiography and suspected with ECG.

Cardiac magnetic resonance in cocaine-induced myocardial damage: cocaine, heart, and magnetic resonance.

The use of cocaine constitutes a major health problem. Cocaine use is associated with acute and chronic complications that might involve any system, the most common being the cardiovascular system. The precise incidence of cocaine-induced cardiomyopathy remains mysterious and probably underreported. Cocaine use should be considered in young patients presenting with chest pain or heart failure without other underlying risk factors. Cocaine-related cardiovascular complications can be acute or chronic and include ischemic and non-ischemic events. Frequent cocaine users have a seven-fold higher risk of myocardial infarction. In addition to its ischemic effects, other cardiovascular complications of cocaine use and abuse are hypertensive crises, aortic dissection or aortic rupture, cerebral hemorrhage, arrhythmias and sudden cardiac death, myocarditis, dilated cardiomyopathy, heart failure, and endocarditis. The mechanism of cocaine's cardiovascular toxicity relates to its sympathomimetic effect, to the block of voltage-dependent K+ and Na2+ channels, and a hypersensitivity reaction to drug or contaminants, such as amphetamine, sugars, or talc. Cardiac magnetic resonance (CMR) can provide a valuable assessment of cocaine-induced myocardial damage both in acute and chronic cardiac complications: it gives prognostic information in clinically relevant settings, and it identifies silent myocardial damage in asymptomatic patients. Indeed, CMR study should be considered in symptomatic cocaine users to assess the extent and evolution of myocardial injury. Furthermore, it was suggested to repeat CMR after 4-8 months of appropriate management to evaluate myocardial response to abstinence and medical therapy.

Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy.

AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC. METHODS AND RESULTS: In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow-up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6-32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2-10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9-9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events. CONCLUSIONS: The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.

Prognostic Value of Magnetic Resonance Phenotype in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.

BACKGROUND: Cardiac magnetic resonance (CMR) is widely used to assess tissue and functional abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC). Recently, a ARVC risk score was proposed to predict the 5-year risk of malignant ventricular arrhythmias in patients with ARVC. However, CMR features such as fibrosis, fat infiltration, and left ventricular (LV) involvement were not considered. OBJECTIVES: The authors sought to evaluate the prognostic role of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different CMR presentations. METHODS: A total of 140 patients with definite ARVC were enrolled (mean age 42 ± 17 years, 97 males) in this multicenter prospective registry. As per study design, CMR was performed in all the patients at enrollment. The novel 5-year ARVC risk score was retrospectively calculated using the patient's characteristics at the time of enrollment. During a median follow-up of 5 years (2 to 8 years), the combined endpoint of sudden cardiac death, appropriate implantable cardioverter-defibrillator intervention, and aborted cardiac arrest was considered. RESULTS: CMR was completely negative in 14 patients (10%), isolated right ventricular (RV) involvement was found in 58 (41%), biventricular in 52 (37%), and LV dominant in 16 (12%). During the follow-up, 48 patients (34%) had major events, but none occurred in patients with negative CMR. At Kaplan-Meier analysis, patients with LV involvement (LV dominant and biventricular) had a worse prognosis than those with lone RV (p < 0.0001). At multivariate analysis, the LV involvement, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major events. The estimated 5-year risk was able to predict the observed risk in patients with lone RV but underestimated the risk in those with LV involvement. CONCLUSIONS: Different CMR presentations of ARVC are associated with different prognoses. The 5-year ARVC risk score is valid for the estimation of risk in patients with lone-RV presentation but underestimated the risk when LV is involved.

Prognostic Role of Cardiac Magnetic Resonance in Arrhythmogenic Right Ventricular Cardiomyopathy.

We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles). During the follow-up time 35 patients had hard cardiac events (sudden cardiac death, appropriate implantable cardioverter defibrillator shock and resuscitated cardiac arrest), and 34 of them occurred in patients with abnormal-CMR (negative predictive value = 96.9%). At the multivariate Cox-regression analysis LV involvement at CMR (fat infiltration and/or late gadolinium enhancement), and episode of nonsustained ventricular tachycardia (NSVT) were independent predictors of cardiac events in both the whole population (LV involvement: HR 3.69, 95% CI 1.57-8.65, p = 0.0002; NSVT: HR 5.8, 95% CI 2.82-11.9, p < 0.0001), and in the group of patients with definite ARVC (LV involvement: HR 3.03, 95% CI 1.15 to 8.02, p = 0.02; NSVT: HR 12.1, 95% CI 4.02-36.5, p < 0.0001). In conclusion, CMR evidence of LV involvement is a strong independent predictor of cardiac events in patients with definite, borderline or possible ARVC diagnosis. Abnormal CMR has very high negative predictive value for hard cardiac events.