Late gadolinium enhancement and outcome of cardiac resynchronization therapy in non-ischemic cardiomyopathy.

BACKGROUND: It is uncertain whether CRT with defibrillator (CRTD) is superior to CRT with pacemaker (CRTP) in NICM. Patients with low arrhythmic risk and high probability of response to CRT might be ideal candidates for CRTP. We aimed to evaluate predictors of ventricular arrhythmias and of echocardiographic response to cardiac resynchronization therapy (CRT) in non-ischemic cardiomyopathy (NICM). METHODS: Multicenter, retrospective observational study of NICM patients with left ventricular ejection fraction (LVEF) ≤35 %, cardiac magnetic resonance with analysis of late gadolinium enhancement (LGE) available and de-novo CRT implant. Echocardiographic response to CRT was defined as an improvement in LVEF ≥10 %. The combined arrhythmic endpoint included sustained ventricular tachycardia, appropriate ICD therapy, resuscitated cardiac arrest and sudden death. RESULTS: We included 167 patients, with a median follow-up of 63 months. LGE was present in 77 (46 %). Response to CRT occurred in 68 % of patients, more frequently in LGE- than in LGE+ (81 % vs 53 %, p < 0.001). Absence of LGE (OR 3.4, p = 0.002), was an independent predictor of response to CRT. The arrhythmic endpoint occurred in 19 patients (11 %). Among LGE- patients there were zero arrhythmic events as compared to a 25 % cumulative incidence in LGE+ (p < 0.001). Presence of LGE (HR 22.5, p < 0.001), was an independent predictor of the arrhythmic endpoint. CONCLUSION: Absence of LGE identifies patients at minimal arrhythmic risk and with high probability of response to CRT. Thus, they might be ideal candidates to CRT-P.

Diagnostic and Prognostic Value of Right Ventricular Fat Quantification from Computed Tomography in Arrhythmogenic Right Ventricular Cardiomyopathy.

Background: In arrhythmogenic right ventricular cardiomyopathy (ARVC) non-invasive scar evaluation is not included among the diagnostic criteria or the predictors of ventricular arrhythmias (VA) and sudden death (SD). Computed tomography (CT) has excellent spatial resolution and allows a clear distinction between myocardium and fat; thus, it has great potential for the evaluation of myocardial scar in ARVC. Objective: The objective of this study is to evaluate the feasibility, and the diagnostic and prognostic value of semi-automated quantification of right ventricular (RV) fat replacement from CT images. Methods: An observational case-control study was carried out including 23 patients with a definite (19) or borderline (4) ARVC diagnosis and 23 age- and sex-matched controls without structural heart disease. All patients underwent contrast-enhanced cardiac CT. RV images were semi-automatically reconstructed with the ADAS-3D software (ADAS3D Medical, Barcelona, Spain). A fibrofatty scar was defined as values of Hounsfield Units (HU) <-10. Within the scar, a border zone (between -10 HU and -50 HU) and dense scar (<-50 HU) were distinguished. Results: All ARVC patients had an RV scar and all scar-related measurements were significantly higher in ARVC cases than in controls (p < 0.001). The total scar area and dense scar area showed no overlapping values between cases and controls, achieving perfect diagnostic performance (sensitivity and specificity of 100%). Among ARVC patients, 16 (70%) had experienced sustained VA or aborted SD. Among all clinical, ECG and imaging parameters, the dense scar area was the only one with a statistically significant association with VA and SD (p = 0.003). Conclusions: In ARVC, RV myocardial fat quantification from CT is feasible and may have considerable diagnostic and prognostic value.

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.

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.

Improved prediction of electrical storm in patients with prior myocardial infarction and implantable cardioverter defibrillator.

AIMS: To evaluate predictors of electrical storm (ES), including chronic total occlusion in an infarct-related coronary artery (infarct-related artery CTO, IRACTO), in a cohort of patients with prior myocardial infarction (MI) and implantable cardioverter-defibrillators (ICD). METHODS: Multicenter observational cohort study including 643 consecutive patients with prior MI and a first ICD implanted between 2005 and 2018 at three tertiary hospitals. All the patients included in the study had undergone a diagnostic coronary angiography before ICD implantation. The variable prior ventricular arrhythmias (VA+) was positive in patients with secondary prevention ICDs and in those with at least one appropriate ICD therapy after primary prevention implantation. RESULTS: During a median follow-up of 42 months 59 patients (9%) suffered ES. The presence of at least one IRACTO not revascularized (IRACTO-NR) was associated with a significantly higher cumulative incidence of ES (14.5% vs 4.8%, p < 0.001). IRACTO-NR maintained a significant association with ES after adjustment for potential confounders (HR 2.3, p = 0.005) and was an independent predictor of ES together with VA+ and LVEF. The best cut-off of LVEF to predict ES was ≤38%. A risk-prediction model based on IRACTO-NR, VA+ and LVEF≤38% identified three categories of ES risk (low, intermediate and high), with progressively increasing cumulative incidence of ES (2.2%, 9% and 20%). CONCLUSION: In a cohort of patients with prior MI and ICD, IRACTO-NR is an independent predictor of ES. A new risk-prediction model allowed the identification of three categories of risk, with potentially important clinical implications.

Influence of Ventricular Wringing on the Preservation of Left Ventricular Ejection Fraction in Cardiac Amyloidosis.

BACKGROUND: The purpose of this work was to determine the influence of myocardial wringing on ventricular function in patients with cardiac amyloidosis (CA). METHODS: Fifteen healthy volunteers (group 1) and 34 patients with CA (17 with left ventricular ejection fractions [LVEFs] ≥ 53% [group 2] and 17 with LVEFs < 53% [group 3]) were evaluated using two-dimensional speckle-tracking echocardiography. A control group of mass-matched patients (n = 20) with left ventricular (LV) hypertrophy and LVEFs ≥ 53% was also included. Longitudinal strain (LS), circumferential strain, and LV twist and torsion were calculated. Deformation index (DefI), a new parameter of wringing, calculated as twist/LS, that takes into account actions that occur simultaneously during LV systole (i.e., longitudinal shortening and twist), was evaluated. Torsional and wringing parameters were calculated according to LVEF. RESULTS: Lower global values of LS and circumferential strain were observed among patients with CA (LS: group 1, -20.6 ± 2.5%; group 2, -11.6 ± 4.1%; group 3, -9.0 ± 3.1%; circumferential strain: group 1, -22.7 ± 4.9%; group 2, -14.4 ± 8.0%; group 3, -13.6 ± 3.8%; P < .001 for both). Torsion did not vary between group 2 and group 1 (2.5 ± 1.1°/cm vs 2.7 ± 0.8°/cm, P = NS). In contrast, DefI was greater in group 2 than in group 1 (-1.8 ± 0.8°/% vs -1.0 ± 0.3°/%, P < .01). Torsion and DefI were lower in group 3 (1.2 ± 0.7°/cm and -1.1 ± 0.6°/%, respectively, P < .001 for both) than in group 2. DefI was similar in patients with LV hypertrophy (-1.7 ± 0.6°/%, P = NS) and group 2. CONCLUSIONS: In patients with CA, preservation of LVEF depends on greater ventricular wringing. DefI, a parameter that integrates the twist and the simultaneous longitudinal shortening of the left ventricle, is a more accurate indicator of the efficacy of this mechanism.

Left anterior descending coronary artery compressed by a giant coronary fistula aneurysm: a case report.

BACKGROUND: Ischaemic chest pain can be originated by different causes. Among all, coronary fistulas are rarely the reason. Such entities are usually asymptomatic and can be diagnosed by echocardiography or coronary angiography. In an even rarer scenario, coronary fistulas might dilate and form an aneurysm. CASE SUMMARY: We report the case of a 62-year-old patient who was initially referred to the emergency department for stable angina. Coronary angiography and computed tomography scan showed a giant aneurysm relating to a coronary fistula with a course from the circumflex coronary artery to the superior vena cava. The aneurysm was critically compressing the left anterior descending coronary artery. It was confirmed and resolved by surgery. DISCUSSION: Giant aneurysms of a coronary fistula are very uncommon entities. We describe a rare case of angina caused by extrinsic compression of the left anterior descending artery from a giant aneurysm of a coronary fistula.

[The portopulmonary hypertension: an overview from diagnosis to treatment]. / Ipertensione portopolmonare: dalla diagnosi alla terapia.

Portopulmonary hypertension (POPH) refers to the condition of pulmonary arterial hypertension (PAH) in association with portal hypertension. Among patients with portal hypertension, reported incidence rates of POPH range from 2 to 10% and long-term prognosis in cases of POPH is poor. Fundamental issues of management of POPH include identification of patients likely to benefit from liver transplantation (LTx) and treatment with specific pulmonary vasodilators before and after LTx. This review presents as an overview of the current knowledge on the epidemiology, diagnosis, treatment and prognosis of patients with portopulmonary hypertension.