Screening for cardiac amyloidosis in patients with tenosynovial red flags: A collaboration between family medicine and cardiology.

BACKGROUND: Amyloid deposition in tenosynovial structures precedes cardiac involvement up to 20 years. Therefore, a cardiological screening in patients with a history of tenosynovial manifestations of cardiac amyloidosis (CA) could lead to an increased number of early diagnoses. METHODS: Patients with tenosynovial manifestations of CA (carpal tunnel syndrome, atraumatic biceps tendon rupture, lumbar spinal stenosis) have been identified by general practitioners and evaluated in a Referral Center for CA. Patients with a high suspicion of CA underwent the CA diagnostic pathway. RESULTS: Among 50 General Practitioners (GP) contacted, 10 (20%) agreed to participate in the study for a total of 5615 patients ≥60 years. One hundred forty-five patients met the inclusion criteria, 2 of them already had a diagnosis of CA, and 57 agreed to undergo a cardiological evaluation (electrocardiography, echocardiography, NTproBNP assay). The median age was 73 [67-80] years and 31 (54%) were women. Eight patients were suggested to start the CA diagnostic pathway, five of them underwent a complete diagnostic evaluation for CA, three refused to complete the diagnostic exams and no new diagnoses were made. CONCLUSION: A screening program for CA in patients with tenosynovial manifestations identified by general practitioners is feasible, but may not yield a high rate of new diagnosis. In this study, we identified two patients who already had a diagnosis of CA, and among patients at high risk for CA, 37% refused to complete the diagnostic pathway. Increased awareness of CA among patients might increase participation and diagnostic yield in screening studies. Further validation of this protocol is needed to evaluate its diagnostic performance.

Mesalazine-induced myopericarditis: a case series.

Background: Inflammatory bowel diseases (IBD) are characterized by chronic inflammation of the gastrointestinal tract but can have multiorgan involvement. Mesalazine (5-ASA) is a key therapeutic agent in IBD. Mesalazine has rare but potentially life-threatening side effects such as cardiac injury. Case summary: We present two cases of myopericarditis, documented also with cardiac magnetic resonance, that we attributed to 5-ASA hypersensitivity: the first is a young woman with ulcerative colitis who developed myopericarditis after the initiation of 5-ASA, with a good clinical response after discontinuation; the second is a 79-year-old man who developed symptoms of heart failure after the diagnosis of IBD and the introduction of 5-ASA. Discussion: Mesalazine may cause rare but potentially life-threatening cardiac injury, which can be difficult to distinguish from acute IBD-induced cardiac inflammation.

Transient stress-related hyperviscosity and endothelial dysfunction in Takotsubo syndrome: a time course study.

Takotsubo syndrome (TTS) is an acute and usually reversible heart failure syndrome, frequently associated with emotional or physical stress. Its pathophysiology remains largely unclear, although several mechanisms related to catecholaminergic storm have been proposed. In this study we analyzed during the acute phase of TTS and at follow-up both hemorheological parameters and biomarkers of endothelial damage, whose time course has never been fully explored. In 50 TTS women, we analyzed several hemorheological parameters [whole blood viscosity (WBV) at 0.512 s-1 and at 94.5 s-1, plasma viscosity (PLV), erythrocyte deformability and aggregation index] as well as biomarkers of endothelial dysfunction [von Willebrand Factor (vWF), Plasminogen activator inhibitor-1 and factor VIII levels] during the acute phase and after a median 6 months follow-up. These variables were also assessed in 50 age-matched healthy women. Respect to follow-up, in the acute phase of TTS we observed higher values of white blood cell count, fibrinogen, WBV at low and high shear rates, PLV, erythrocyte aggregation index and lower values of erythrocyte elongation index. Moreover, all biomarkers of endothelial dysfunction resulted significantly higher in the acute phase. During follow-up WBV at 94.5 s-1, erythrocyte elongation index and vWF resulted significantly altered with respect to controls. The results of this study confirm the role of hyperviscosity and endothelial dysfunction in TTS pathophysiology. Moreover, they suggest the persistence of alterations of erythrocyte deformability and endothelial dysfunction even beyond the acute phase that could be the target of therapeutic strategies also during follow-up.

Sex-related differences in clinical presentation and all-cause mortality in patients with cardiac transthyretin amyloidosis and light chain amyloidosis.

We aimed to ascertain whether sex-related differences are relevant to clinical presentation, cardiac phenotype and all-cause mortality in different types of cardiac amyloidosis, a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Women with wtATTR, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men, otherwise, bio-humoral parameters, NYHA class, and ECG characteristics were similar. Comparing females and male with wtATTR, hATTR and AL, no differences in Kaplan-Meier curves for all-cause mortality were observed with regard to sex, p-value >0.05. In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, all-cause mortality was not affected. Nevertheless, women with wtATTR had echocardiographic signs of more advanced disease and higher NAC score at diagnosis suggesting a possible later recognition of disease compared to men.

Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy Plus Apical Ballooning: Management With VA-ECMO and Myectomy.

A patient with known obstructive hypertrophic cardiomyopathy developed worsening left ventricular outflow tract obstruction, severe mitral regurgitation, and apical ballooning leading to cardiogenic shock, a combination in which treatment of each component could worsen the others. Emergency veno-arterial extracorporeal membrane oxygenation, levosimendan, and noradrenaline transiently restored adequate systemic perfusion and gas exchange. Surgical myectomy offered a more definitive solution. (Level of Difficulty: Intermediate.).

Endocarditis with spondylodiscitis: clinical characteristics and prognosis.

BACKGROUND: The association of infective endocarditis (IE) with spondylodiscitis (SD) was first reported in 1965, but few data are available about this issue. This study aimed to evaluate the prevalence of SD in patients with IE, and to determine the clinical features and the prognostic impact of this association. METHODS: We retrospectively analysed 363 consecutive patients admitted to our Department with non-device-related IE. Radiologically confirmed SD was revealed in 29 patients (8%). Long-term follow-up (average: 3 years) was obtained by structured telephone interviews; in 95 cases (13 of whom had been affected by SD), follow-up echocardiographic evaluation was also available. RESULTS: At univariable analysis, the combination of IE with SD was associated with male gender (p = 0.017), diabetes (p = 0.028), drug abuse (p = 0.009), Streptococcus Viridans (p = 0.009) and Enterococcus (p = 0.015) infections. At multivariable analysis, all these factors independently correlated with presence of SD in patients with IE. Mortality was similar in patients with and without SD. IE relapses at 3 years were associated with the presence of SD (p = 0.003), Staphylococcus aureus infection (p < 0.001), and drug abuse (p < 0.001) but, at multivariable analysis, only drug abuse was an independent predictor of IE relapses (p < 0.001; HR 6.8, 95% CI 1.6-29). At echocardiographic follow-up, SD was not associated with worsening left ventricular systolic function or valvular dysfunction. CONCLUSIONS: The association of IE with SD is not rare. Hence, patients with IE should be screened for metastatic infection of the vertebral column, especially if they have risk factors for it. However, SD does not appear to worsen the prognosis of patients with IE, either in-hospital or long-term.

Predictors of mortality and adverse events in patients with infective endocarditis: a retrospective real world study in a surgical centre.

PURPOSE: Mortality in infective endocarditis (IE) is still high, and the long term prognosis remains uncertain. This study aimed to identify predictors of long-term mortality for any cause, adverse event rate, relapse rate, valvular and ventricular dysfunction at follow-up, in a real-world surgical centre. METHODS: We retrospectively analyzed 363 consecutive episodes of IE (123 women, 34%) admitted to our department with a definite diagnosis of non-device-related IE. Median follow-up duration was 2.9 years. Primary endpoints were predictors of mortality, recurrent endocarditis, and major non-fatal adverse events (hospitalization for any cardiovascular cause, pace-maker implantation, new onset of atrial fibrillation, sternal dehiscence), and ventricular and valvular dysfunction at follow-up. RESULTS: Multivariate analysis independent predictors of mortality showed age (HR per unit 1.031, p < 0.003), drug abuse (HR 3.5, p < 0.002), EUROSCORE II (HR per unit 1.017, p < 0.0006) and double valve infection (HR 2.3, p < 0.001) to be independent predictors of mortality, while streptococcal infection remained associated with a better prognosis (HR 0.5, p < 0.04). Major non-fatal adverse events were associated with age (HR 1.4, p < 0.022). New episodes of infection were correlated with S aureus infection (HR 4.8, p < 0.001), right-sided endocarditis (HR 7.4, p < 0.001), spondylodiscitis (HR 6.8, p < 0.004) and intravenous drug abuse (HR 10.3, p < 0.001). After multivariate analysis, only drug abuse was an independent predictor of new episodes of endocarditis (HR 8.5, p < 0.001). Echocardiographic follow-up, available in 95 cases, showed a worsening of left ventricular systolic function (p < 0.007); severe valvular dysfunction at follow-up was reported only in 4 patients, all of them had mitral IE (p < 0.03). CONCLUSIONS: The present study highlights some clinical, readily available factors that can be useful to stratify the prognosis of patients with IE.

Cardiac Metastasis of Sacral Chordoma.

Chordoma is a rare tumor, usually diagnosed when the disease is advanced. Despite its slow growth, it is locally aggressive and has a poor long-term prognosis. Surgery is the mainstay treatment. Although cardiac metastases are very rare, the heart is frequently involved in systemic neoplastic diseases. This report describes a typical case of metastatic chordoma: the age at first diagnosis, the site of the primary tumor, and the slow growth of the cardiac metastasis were all typical features. Surgical excision of the mass from the right ventricular outflow tract is described together with echocardiographic, radiologic, and histopathologic characteristics of the metastatic chordoma.

Embolic risk stratification and prognostic impact of early surgery in left-sided infective endocarditis.

BACKGROUND: In patients with left-sided infective endocarditis (IE) and heart failure associated with large vegetations, early surgery prevents embolic events. However, optimal timing of surgery for other indications is still unresolved particularly when the presence of large vegetations represents the sole indication. METHODS: We retrospectively analyzed 308 consecutive patients admitted to our department with definite left-sided IE. Of these patients, 243 (79%) underwent cardiac surgery (complicated IE), 34 patients with uncomplicated IE received medical treatment, 24 were not operated due to prohibitive general conditions and 7 refused surgery. Long-term follow-up was obtained by structured telephone interviews. RESULTS: During the 6-year follow-up (average 121.8 weeks ± 76), patients not operated because of general conditions or refusal had the worst prognosis, while outcome in operated patients for complicated IE was comparable to that of uncomplicated IE treated medically. Early (<2 weeks from diagnosis) surgery was associated with better survival compared to delayed surgery (HR 0.58, p = 0.23). Embolic events were detected at admission in 38% of cases; Staphylococcus Aureus etiology and vegetation size were independently associated with embolism (OR 2.4, p = 0.01; OR 1, p=0.008 respectively). CONCLUSIONS: Compared to uncomplicated medically-treated patients, complicated IE showed comparable survival when managed aggressively by surgical intervention, whereas a conservative approach was associated with an adverse prognosis. Staphylococcus Aureus infection and vegetation size were independent predictors of systemic embolism. Our data support aggressive surgical management of complicated IE patients and highlight the importance of etiological characterization in clinical decision-making.

Primary cardiac sarcoma: a case report of a therapeutic challenge.

BACKGROUND: Primary cardiac sarcomas are very rare and the prognosis is poor both because the diagnosis is typically made at an advanced stage of the disease and because data are insufficient to identify a standard treatment. Surgical resection is the cornerstone of therapy with the need to develop new therapeutic strategies. CASE SUMMARY: We present a case of a young man admitted to the emergency department due to worsening dyspnoea. A left-sided sarcoma was diagnosed and treated with surgery, chemo- and radiation therapy, and subsequently with heart transplant for local recurrence of the disease. Endomyocardial biopsy made during the routine follow-up period was complicated by pericardial tamponade and cardiogenic shock and the patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of left ventricular function (left ventricular ejection fraction of 55%). After 1 year a kidney transplant was performed. After 42 months from diagnosis, the patient is in good general condition. DISCUSSION: Primary cardiac sarcomas are treated with surgery to reach R0 (free resection margins) and with chemo- and radiation therapy with adjuvant purposes. Auto-transplantation is also performed, while conventional heart transplant must be customized on an individual basis, after excluding metastases. A multidisciplinary assessment should be performed and the single patient treated with a personalized approach, in relation to his performance status, location of the mass, and stage of the disease.

Two-dimensional and three-dimensional left ventricular deformation analysis: a study in competitive athletes.

Two-dimensional (2D) speckle-tracking echocardiography (STE) has clarified functional adaptations accompanying the morphological features of 'athlete's heart'. However, 2D STE has some limitations, potentially overcome by three-dimensional (3D) STE. Unfortunately, discrepancies between 2D- and 3D STE have been described. We therefore sought to evaluate whether dimensional and functional differences exist between athletes and controls and whether 2D and 3D left ventricular (LV) strains differ in athletes. One hundred sixty-one individuals (91 athletes, 70 controls) were analysed. Athletes were members of professional sports teams. 2D and 3D echocardiography and STE were used to assess LV size and function. Bland-Altman analysis was used to estimate the level of agreement between 2D and 3D STE. Athletes had greater 2D and 3D-derived LV dimensions and LV mass (p < 0.0001 for all), while 2D- and 3D-derived LV ejection fraction did not differ as compared with controls (p = 0.82 and p = 0.89, respectively). Longitudinal, radial, and circumferential strains did not differ between athletes and controls, neither by 2D nor by 3D STE. Three-dimensional longitudinal and circumferential strain values were lower (p < 0.0001 for both) while 3D radial strain was greater, as compared with 2D STE (p < 0.001). Bland-Altman plots demonstrated the presence of an absolute systematic error between 2D and 3D STE to analyse LV myocardial deformation. 3D STE is a useful and feasible technique for the assessment of myocardial deformation with the potential to overcome the limitations of 2D imaging. However, discrepancies exist between 2D and 3D-derived strain suggesting that 2D and 3D STE are not interchangeable.

Echocardiographic assessment of left ventricular systolic function: from ejection fraction to torsion.

Assessment of left ventricular (LV) systolic function is the cornerstone of the echocardiographic examination. There are many echocardiographic parameters that can be used for clinical and research purposes, each one with its pros and cons. The LV ejection fraction is the most used one due to its feasibility and predictability, but it also has many limits, related to both the imaging technique used for calculation and to the definition itself. LV longitudinal function is expression of subendocardial fibers contraction. Because the subendocardium is often involved early in many pathological processes, its analysis has been a fertile field for the development of sensitive parameters. Longitudinal function can be evaluated in many ways, such as M-mode echocardiography, tissue Doppler imaging, and speckle tracking echocardiography. This latter is a relatively new tool to assess LV function through measurement of myocardial strain, with a high temporal and spatial resolution and a better inter- and intra-observer reproducibility compared to Doppler strain. It is angle independent, not affected by translation cardiac movements, and can assess simultaneously the entire myocardium along all the three-dimensional geometrical (longitudinal, circumferential, and radial) axes. Speckle tracking echocardiography also allows the analysis of LV torsion. The aim of this paper was to review the main echocardiographic parameters of LV systolic function and to describe its pros and cons.

Increased left atrial size is associated with reduced atrial stiffness and preserved reservoir function in athlete's heart.

Left atrial (LA) fibrosis with increased stiffness has been assumed to be the substrates for occurrence of atrial arrhythmias in athletes. However, this hypothesis has not yet been confirmed in humans. Aim of this study was, therefore, to assess LA remodeling and stiffness in competitive athletes. 150 competitive athletes and 90 age and sex-matched sedentary subjects were analyzed by speckle-tracking echocardiography to measure peak atrial longitudinal strain (PALS) and peak atrial contraction strain (PACS). LA stiffness was determined using E/e' ratio in conjunction with PALS. Left ventricular (LV) stiffness was also calculated. LA volume index was greater in athletes as compared with controls (24.6 ± 7.3 vs. 18.4 ± 7.8 mL/m(2), p < .0001). LA PALS, LA PACS, and E/e' ratio were lower in athletes in comparison with controls (p < .05, p ≤ .001, and p < .0001, respectively). Despite greater LA size, competitive athletes had lower LA stiffness as compared with controls (0.13 ± 0.04 vs. 0.16 ± 0.06, p ≤ .001). In addition, LV stiffness was lower in athletes (0.84 ± 0.27 vs. 1.07 ± 0.46, p ≤ .001). The only independent predictor of LA stiffness was LV stiffness (ß = 0.46, p < .0001), while the only independent predictor of LA volume index was LV end-systolic volume index (ß = 0.25, p = .002). Competitive athletes showed greater LA size associated with lower stiffness as compared with controls. Thus, LA remodeling in the context of the athlete's heart is not associated with increased LA stiffness. These findings support the benign nature of LA remodeling in athletes, occurring as a physiological adaptation to exercise conditioning.