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.