Ventricular Arrhythmias and Sudden Death in Nonischemic Dilated Cardiomyopathy: Matter of Sex or Scar?

BACKGROUND: To evaluate the association between sex and ventricular arrhythmias (VA) or sudden death (SD) in nonischemic dilated cardiomyopathy, including analysis of potential confounders. METHODS AND RESULTS: Retrospective cohort study of consecutive patients with DCM referred for cardiac magnetic resonance at 2 tertiary hospitals. The primary combined end point encompassed sustained VA, appropriate implantable cardioverter defibrillator therapies, resuscitated cardiac arrest, and SD. We included 1165 patients with median follow-up of 36 months (interquartile range 20-58 months). The majority of patients (66%) were males. Males and females had similar left ventricular ejection fraction, but the prevalence of late gadolinium enhancement (LGE) at cardiac magnetic resonance was significantly higher among males (48% vs 30%, P < .001). Males had higher cumulative incidence of the primary end point (8% vs 4%, P = .02), and male sex was a significant predictor of the primary end point at univariate analysis (hazard ratio 1.93, P = .02). However, LGE had a major confounding effect in the association between sex and the primary outcome: the hazard ratio of male sex adjusted for LGE was 1.29 (P = .37). LGE+ females had significantly higher cumulative incidence of the primary end point than LGE- males (13% vs 1.8%, P < .001). CONCLUSIONS: In patients with DCM, the prevalence of LGE is significantly higher among males, implying a major confounding effect in the association between male sex and VA or SD. LGE+ females have significantly higher risk than LGE- males. These data do not support the inclusion of sex into risk stratification algorithms for VA or SD in DCM.

Variations in threshold values for border zone and dense scar produce significant changes in scar parameters obtained by ADAS-3D.

BACKGROUND: ADAS-3D software elaborates cardiac magnetic resonance (CMR) images to obtain a quantitative evaluation of dense scar and border zone (BZ), including BZ channels, which can be useful for ventricular tachycardia ablation and risk stratification. However, most prior reports with ADAS-3D used flexible thresholds (60% ± 5% and 40% ± 5% of maximum pixel signal intensity) to define dense scar and BZ. The impact of such variations of the threshold values on the measurements obtained with ADAS-3D is unknown. OBJECTIVE: This study aimed to quantify the degree of change in ADAS-3D measurements when different thresholds for dense scar and BZ are employed. METHODS: A single-center retrospective observational cohort study including 87 consecutive patients with previous myocardial infarction who underwent CMR was conducted. ADAS-3D software semiautomatically processed CMR sequences. We compared the scar measurements obtained with the 9 possible combinations of thresholds (55%/60%/65% and 35%/40%/45% of maximum pixel signal intensity). RESULTS: The overall comparison between thresholds showed highly significant differences (P < .001) in all scar parameters. Not a single patient maintained the same number of BZ channels with all the thresholds settings. A percentage difference of up to 200% in BZ channel numbers and channel mass was observed in all 36 comparisons. An absolute difference of up to 10 channels was also recorded. Of note, the highest median channel mass (obtained with the thresholds 35-65) was 59-fold higher compared with the lowest one (obtained with the 45-55 cutoffs). CONCLUSION: Variations in threshold values result in statistically significant and high-magnitude changes in the quantification of scar parameters by ADAS-3D.

Impact of Cardiac Magnetic Resonance to Arrhythmic Risk Stratification in Nonischemic Cardiomyopathy.

Left ventricular ejection fraction-based arrhythmic risk stratification in nonischemic cardiomyopathy (NICM) is insufficient and has led to the failure of primary prevention implantable cardioverter defibrillator trials, mainly due to the inability of selecting patients at high risk for sudden cardiac death (SCD). Cardiac magnetic resonance offers unique opportunities for tissue characterization and has gained a central role in arrhythmic risk stratification in NICM. The presence of myocardial scar, denoted by late gadolinium enhancement, is a significant, independent, and strong predictor of ventricular arrhythmias and SCD with high negative predictive value. T1 maps and extracellular volume fraction, which are able to quantify diffuse fibrosis, hold promise as complementary tools but need confirmatory results from large studies.

Prognostic impact of late gadolinium enhancement at the right ventricular insertion points in non-ischaemic dilated cardiomyopathy.

AIMS: To evaluate the baseline characteristics and the prognostic implications associated with late gadolinium enhancement limited to the right ventricular insertion points (IP-LGE) or present at both the right ventricular insertion points and the left ventricle (IP&LV-LGE) in non-ischaemic dilated cardiomyopathy (DCM). METHODS AND RESULTS: This is a retrospective observational multicentre cohort study including 1165 consecutive patients with DCM evaluated by cardiac magnetic resonance. The primary endpoint included appropriate defibrillator therapies, sustained ventricular tachycardia, resuscitated cardiac arrest, or sudden death. The secondary outcome encompassed heart failure hospitalizations, heart transplant, left ventricular assist device implantation, and end-stage heart failure death. IP-LGE was found in 72 patients (6%), who had clinical characteristics closer to LGE- than to LGE+ patients. During follow-up (median 36 months), none of the IP-LGE patients experienced the primary endpoint. The cumulative incidence of the primary endpoint was similar between IP-LGE and LGE- patients (P = 1), while IP-LGE had significantly lower cumulative incidence when compared with LGE+ patients (P < 0.001). When compared with IP-LGE patients, the cumulative incidence of the secondary endpoint was similar in LGE- cases (P = 0.86) but tended to be higher in LGE+ patients (P = 0.06). Both clinical characteristics and outcomes were similar between IP&LV-LGE patients and the rest of LGE+ cases. CONCLUSIONS: In a large cohort of DCM patients, IP-LGE was associated with similar outcome when compared with LGE- patients and with significant lower risk of ventricular arrhythmias and sudden death when compared with LGE+ cases. Patients with IP&LV-LGE had clinical characteristics and outcomes similar to the rest of LGE+ cases.

Late gadolinium enhancement and the risk of ventricular arrhythmias and sudden death in NYHA class I patients with non-ischaemic cardiomyopathy.

AIM: To compare the risk of ventricular arrhythmias (VA) and sudden death (SD) between New York Heart Association (NYHA) class I and NYHA class II-III patients with non-ischaemic cardiomyopathy (NICM). METHODS AND RESULTS: Observational retrospective cohort study including patients with NICM who underwent cardiac magnetic resonance at two hospitals. The primary endpoint included appropriate implantable cardioverter defibrillator (ICD) therapies, sustained ventricular tachycardia, resuscitated cardiac arrest and SD. The secondary endpoint included heart failure (HF) hospitalizations, heart transplant, left ventricular assist device implant or HF death. Overall, 698 patients were included, 33% in NYHA class I. During a median follow-up of 31 months, the primary endpoint occurred in 57 patients (8%), with no differences between NYHA class I and NYHA class II-III cases (7% vs. 9%, p = 0.62). Late gadolinium enhancement (LGE) was the only independent predictor of the primary outcome both in NYHA class I and NYHA class II-III patients. LGE+ NYHA class I patients had a similar cumulative incidence of the primary endpoint as compared to LGE+ NYHA class II-III (p = 0.92) and a significantly higher risk as compared to LGE- NYHA class II-III cases (p < 0.001). The risk of the secondary endpoint was significantly higher in patients in NYHA class II-III as compared to those in NYHA class I (hazard ratio 3.2, p = 0.001). CONCLUSIONS: Patients with NICM in NYHA class I are not necessarily at low risk of VA and SD. Actually, LGE+ NYHA class I patients have a high risk. NYHA class I patients with high-risk factors, such as LGE, could benefit from primary prevention ICD at least as much as those in NYHA class II-III with the same risk factors.

Increased Prevalence of Moderate to Severe Mitral and Aortic Valve Dysfunction in Systemic Sclerosis: A Case-control Study.

OBJECTIVE: To investigate the prevalence, severity, and associated clinical factors of mitral and aortic valvular involvement in patients with systemic sclerosis (SSc). METHODS: Our case-control study included 172 patients with SSc and 172 non-SSc adults without known cardiac disease matched by age, sex, and prevalence of cardiovascular (CV) risk factors. The screening of mitral and aortic valvular involvement was performed by transthoracic Doppler echocardiogram. The prevalence of aortic stenosis (AS) was also compared with that reported in a population-based study performed in our community during the same period. RESULTS: Patients with SSc showed an almost 5-fold increased prevalence of moderate to severe mitroaortic valve dysfunction compared to non-SSc controls (OR 4.60, 95% CI 1.51-13.98; P = 0.003). The most common lesion was mitral regurgitation (MR), which was observed in 5.2% of patients, followed by AS in 3.5%, and aortic regurgitation (AR) in 1.7%. Analyzing the different types of valvular lesion separately, we observed a significantly higher frequency of MR compared to controls (OR 4.69, 95% CI 1.12-22.04; P = 0.032), as well as a higher frequency of AS in the 65-75 (OR 7.51, 95% CI 1.22-46.23, P = 0.01) and 76-85 age groups (OR 3.53, 95% CI 1.03-12.22, P = 0.043) when compared to the general population in our community. CONCLUSION: We found an increased prevalence of moderate to severe MR and AS in SSc compared to age-matched non-SSc controls with similar CV comorbidities. While results from this study do not allow for establishing a direct causal relationship, they strongly support the contribution of SSc-specific factors in the development of these complications.

Improved Risk Stratification for Ventricular Arrhythmias and Sudden Death in Patients With Nonischemic Dilated Cardiomyopathy.

BACKGROUND: Risk stratification for ventricular arrhythmias (VA) and sudden death in nonischemic dilated cardiomyopathy (DCM) remains suboptimal. OBJECTIVES: The goal of this study was to provide an improved risk stratification algorithm for VA and sudden death in DCM. METHODS: This was a retrospective cohort study of consecutive patients with DCM who underwent cardiac magnetic resonance with late gadolinium enhancement (LGE) at 2 tertiary referral centers. The combined arrhythmic endpoint included appropriate implantable cardioverter-defibrillator therapies, sustained ventricular tachycardia, resuscitated cardiac arrest, and sudden death. RESULTS: In 1,165 patients with a median follow-up of 36 months, LGE was an independent and strong predictor of the arrhythmic endpoint (hazard ratio: 9.7; p < 0.001). This association was consistent across all strata of left ventricular ejection fraction (LVEF). Epicardial LGE, transmural LGE, and combined septal and free-wall LGE were all associated with heightened risk. A simple algorithm combining LGE and 3 LVEF strata (i.e., ≤20%, 21% to 35%, >35%) was significantly superior to LVEF with the 35% cutoff (Harrell's C statistic: 0.8 vs. 0.69; area under the curve: 0.82 vs. 0.7; p < 0.001) and reclassified the arrhythmic risk of 34% of patients with DCM. LGE-negative patients with LVEF 21% to 35% had low risk (annual event rate 0.7%), whereas those with high-risk LGE distributions and LVEF >35% had significantly higher risk (annual event rate 3%; p = 0.007). CONCLUSIONS: In a large cohort of patients with DCM, LGE was found to be a significant, consistent, and strong predictor of VA or sudden death. Specific high-risk LGE distributions were identified. A new clinical algorithm integrating LGE and LVEF significantly improved the risk stratification for VA and sudden death, with relevant implications for implantable cardioverter-defibrillator allocation.

Genotype-phenotype correlation of LMNA variants involving the Arg541 residue: a case report with multimodality imaging and literature review.

We present a case of atypical LMNA cardiomyopathy associated with the pathogenic variant p.Arg541Ser. The patient had early-onset severe ventricular arrhythmias but atrioventricular conduction was normal. Segmental motion abnormalities and a large transmural scar, mainly apical and lateral, were found at cardiac magnetic resonance, corresponding to areas of severe wall thinning at computed tomography and of low voltages at electroanatomic mapping. Ventricular tachycardia ablation was successful in controlling ventricular arrhythmias. Few other cases described patients with pathogenic variants in the Arg541 residue, and they displayed similar atypical features, suggesting a genotype-phenotype correlation which may have specific prognostic and therapeutic implications.

[Clinical predictors of left main coronary artery disease in high-risk patients with a first episode of non-ST-segment elevation acute coronary syndrome]. / Variables clínicas predictoras de enfermedad del tronco común en pacientes de alto riesgo con un primer episodio de síndrome coronario agudo sin elevación del segmento ST.

INTRODUCTION AND OBJECTIVES: Risk stratification in non-ST-elevation acute coronary syndrome makes use of clinical variables that can identify patients at an increased risk of complications. Our objective was to identify clinical variables that predict significant stenosis (i.e., >50%) of the left main coronary artery in high-risk patients who have had a first episode of non-ST-elevation acute coronary syndrome but who do not have a history of coronary artery disease. METHODS: The study included 102 high-risk patients with no history of coronary artery disease who were admitted because of non-ST-elevation acute coronary syndrome. All underwent coronary angiography. Patients were divided into two groups: those with significant left main coronary artery stenosis (n=14) and those without (n=88). RESULTS: Univariate analysis showed that the variables significantly associated with left main coronary artery stenosis were age >65 years (57.1% vs 15.9%, P=.002), diabetes mellitus (71.4% vs 33.0%, P=.006), chronic renal failure (28.6% vs 5.7%, P=.019), left heart failure (71.4% vs 6.8%, P< .0001), cardiogenic shock (21.4% vs 1.1%, P=.008), and a low left ventricular ejection fraction at admission (49.9% [14.7%] vs 58.8% [9.9%], P=.044). In the multivariate analysis, the only significant independent predictor of left main coronary artery disease was left heart failure. CONCLUSIONS: The presence of left heart failure at initial assessment of high-risk patients with non-ST-elevation acute coronary syndrome but without a history of coronary artery disease could be a useful predictor of significant left main coronary artery disease.

Giant thrombus trapped in foramen ovale with pulmonary embolus and stroke.

We describe the case of a young man who, while he was in coma because of a traffic accident, had first a pulmonary embolus and immediately afterwards had a systemic (cerebral) embolus. A transesophageal echocardiographic image revealed a giant thrombi trapped in foramen ovale protruding in right and left ventricles, diagnosing, thus, a paradoxical embolism. The relationship between patent foramen ovale and pulmonary embolism has been reported in some series. Elevated right-chamber pressure caused by pulmonary hypertension could favor the establishment of a right-to-left shunt, causing, in some cases, paradoxical embolisms. We review the clinical implications.

[Post-anoxic encephalopathy after an episode of aborted sudden cardiac death]. / Encefalopatía postanoxémica tras un episodio de muerte súbita cardíaca recuperada.

INTRODUCTION AND OBJECTIVES: The presence of post-anoxic encephalopathy after an episode of aborted sudden cardiac death is a serious medical and social problem. PATIENTS AND METHOD: Of 193 patients admitted to the coronary unit during a 12-year period with aborted sudden cardiac death, 104 died (54%) and 89 survived the episode (46%). We compared the characteristics and 6-month prognosis between survivors according to the presence or absence of post-anoxic encephalopathy. RESULTS: Of 89 survivors, 38 patients were discharged alive with post-anoxic encephalopathy (42%) and 51 were discharged without neurological disturbances (58%). Patients with post-anoxic encephalopathy were older (65 13.6 vs. 59 12.9 years; p = 0.04). Mean time to care for cardiac arrest was 11.14.9 min in the post-anoxic encephalopathy group, versus 3.43 min (p < 0.01). The first documented rhythm was VF/VT in 57% and asystolia in 29% in the post-anoxic encephalopathy group, vs. 88% and 3% (p = 0.02). More than half (58%) of the patients in the post-anoxic encephalopathy group had coronary artery disease, vs. 49% (p = NS). Slightly more than half (52%) of the patients in the post-anoxic encephalopathy group had infectious complications, vs. 21% (p = 0.01). After 6 months of follow-up, mortality in patients discharged with post-anoxic encephalopathy was 62%, vs. 10% (p < 0.01); 21% of then showed significant functional improvement and 18% remained neurologically stable. CONCLUSIONS: Most patients admitted to a coronary unit with aborted sudden cardiac death died during hospitalization. Almost half of the survivors had post-anoxic encephalopathy at discharge; of these patients, 62% died within the following 6 months, and functional status improved only in a minority of them.

Ablación de taquicardia ventricular guiada por cardiorresonancia magnética / Ventricular tachycardia ablation guided by cardiac magnetic resonance

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Miocardiopatía dilatada y fosfolipidosis inducida por hidroxicloroquina: de los cuerpos curvilíneos a la sospecha clínica / Dilated cardiomyopathy and hydroxychloroquine-induced phospholipidosis: from curvilinear bodies to clinical suspicion

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