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Background Pericardial agenesis is a rare congenital heart disease characterized by a variable clinical presentation. Case summary A 32-year-old man was sent by an occupational health physician to our health care centre because of pathological electrocardiogram (ECG). On transthoracic echocardiogram, we had some difficulty to obtain a good quality of four-chamber apical view that was shifted upper and laterally towards the left anterior axillary line. Nonetheless, an abnormal diastolic expansion of the apex of the left ventricle (LV) that had an otherwise normal systolic function was detected. A chest X-ray confirmed the leftward shift of the heart, with the elongation of the left border of cardiac silhouette and cardiac MRI, finally revealed the absence of left-sided pericardium associated with a leftward dislocation of the heart and a dysmorphism of the LV apex that appeared rounded and curved. The non-invasive work-up was completed with 48 h long Holter ECG that was unremarkable. The exercise test was also negative for both inducible myocardial ischaemia and arrhythmias. Patient was scheduled for loop-recorder implantation, and a 6-month clinical follow-up was advised. Discussion Pericardial agenesis is a rare congenital heart disease associated with an increased risk of cardiac arrhythmias and type A aortic dissection, however its clinical course could be also completely unremarkable. The diagnosis is challenging, and cardiac MRI remains the gold standard imaging modality. In complete left-sided and asymptomatic forms, no treatment is needed. Prognosis is not well established due to both the rarity of disease and extreme variability of clinical presentation. [ABSTRACT FROM AUTHOR]

Aims Bicuspid aortic valve (BAV) may be complicated by aortic aneurysms and dissection. This study aimed to evaluate the prognostic efficacy of markers from cardiac imaging, as well as genetic and new biomarkers, to early predict aortic complications. Methods and results We re-evaluated after a mean time of 48 ± 11 months 47 BAV patients who had undergone previous echocardiography for evaluation of aortic stiffness and 2D aortic longitudinal strain (LS) (by speckle-tracking analysis), and who had given a blood sample for the assessment of a single-nucleotide polymorphism of elastin gene (ELN rs2 071307) and quantification of elastin soluble fragments (ESF). Surgical treatment of aortic aneurysm/dissection was the primary endpoint, and an aortic dimension increase (of one or more aortic segments) ≥1 mm/year was the secondary endpoint. Nine patients underwent surgical treatment of ascending aorta (AA) aneurysms. Out of the 38 patients who did not need surgical intervention, 16 showed an increase of aortic root and/or AA dimension ≥1 mm/year. At multivariate Cox regression analysis, an impaired AA LS was an independent predictor of aortic surgery [ P  =   0.04; hazard ratio (HR) 0.961; 95% confidence interval (CI) 0.924–0.984] and aortic dilatation (P  =   0.007; HR 0.960; 95% CI 0.932–0.989). An increased quantity of ESF was correlated (P  =   0.015) with the primary endpoint at univariate Cox regression analysis but it did not keep statistical significance at multivariate analysis. Conclusion In BAV patients, impairment of elastic properties of the AA, as assessed by 2D LS, is an effective predictor of aortic complications. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate short- and long-term outcome in a single prospective cohort of Takotsubo syndrome (TTS) patients, trying to early identify those with better prognosis and to assess the prevalence of left ventricular ejection fraction (LVEF) recovery over time., Methods: Forty-nine patients prospectively enrolled underwent to assessment of demographic, clinical, and echocardiographic characteristics, and later were followed to identify the outcomes during a mean follow-up (FU) of 93 months. At the end of the FU phase, a subgroup of patients underwent to a clinical and echocardiographic re-evaluation. As major adverse cardiac events (MACE) were considered: Intra and extra-hospital death, re-hospitalizations for acute heart failure or atrial fibrillation (AF) or acute myocardial infarction (AMI) and TTS recurrence; minor events were considered minor symptoms of heart failure and angina not requiring hospitalization., Results: The prevalence of re-hospitalizations (MACE) over time was: 41% ( n = 12) for HF; 6.8% ( n = 2) for AMI; 3.4% ( n = 1) for TTS relapse and 20% ( n = 6) for AF. Minor events were the symptoms of chest pain and dyspnea not requiring hospitalization in 6 (20.7%) and 12 (34.4%) patients, respectively. LVEF at the time of admission was predictor for MACE. Stratifying patients on the LVEF admission median value (40%). Patients with LVEF <40% at admission had a significantly lower survival free from adverse cardiac events compared to patients with LVEF ≥40%. Twenty-seven (93%) patients underwent to a clinical reassessment with electrocardiogram and echocardiographic examination. LVEF value showed a statistically significant increase ( P = 0.004) at the end of FU., Conclusions: At admission, "high-risk" patients (LVEF <40%) can be easily detected, allowing an appropriate pharmacological and/or mechanical support strategy and a more "careful" FU., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Journal of Cardiovascular Echography.)