Temporal implementation of a regional referral pathway in transthyretin cardiac amyloidosis: Emilia-Romagna experience.

AIMS: Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis. We aimed to examine the development of a regional network for the diagnosis and management of ATTR-CA and describe a cohort of patients with ATTR-CA, investigate diagnostic pathways and assess clinical outcomes according to diagnosis periods. METHODS: We performed a survey study analyzing answers from 11 cardiology centers and we conducted a retrospective study including patients with ATTR-CA attending a referral center between 1 January 2012 and 31 December 2022, and categorized by the period of diagnosis (2012-2016 and 2017-2022). RESULTS: Over the years, a growing number of patients reached a diagnosis and were treated in the surveyed nonreferral centers of the region. The retrospective study showed a more significant diagnostic delay in the earlier period rather than the later one [13.4 (5-30.2) vs. 10.6 (5.0-17.9) months, P = 0.04]. Patients diagnosed after 2017 showed a greater survival rate than those diagnosed earlier ( P = 0.02). In the multivariate analysis, the year of diagnosis from 2017 remained independently associated with mortality [hazard ratio (HR) 0.46, 95% confidence interval (CI) 0.28-0.79; P = 0.005]. CONCLUSION: This study emphasized the shift toward noninvasive diagnostic criteria. It revealed a positive impact on patient survival and disease management with the use of disease-modifying therapies and diagnostic developments in more recent years. The findings underscore the importance of disease awareness and networking to reduce diagnostic delays and enhance patient journeys for ATTR-CA.

Left atrial anatomy and function after conversion from atrial fibrillation in hypertrophic hearts.

The aim of the study was to evaluate the influence of left ventricular (LV) hypertrophy on left atrial (LA) electrical and mechanical function after cardioversion atrial fibrillation (A-Fib) of brief duration. Study group A included 100 patients with a first diagnosis of hypertension who had a moderate LV hypertrophy. The patient population included 64 men and 36 women with a mean age of 55 +/-7 years who were hospitalized because of A-Fib and were cardioverted with external DC shock. Control group B included 100 patients without cardiac hypertrophy cardioverted because of lone A-Fib. Atrial function and size were assessed by Doppler echocardiography and the following parameters were measured: transmitral peak A velocity, atrial filling fraction, atrial ejection force, peak E velocity, deceleration time, and isovolumic relaxation time, LA maximal and minimal volume, and LV cardiac mass index. Baseline echocardiography showed that LA diameters and volumes were enlarged in all patients during A-Fib. After the restoration of sinus rhythm LA diameters and volumes decreased and the reduction was more evident in group B compared to group A. LA function as a continuous variable was negatively related to LV mass index (r = -0.77), LA diameter (r = -0.66 and r = -0.69 for the superoinferior diameter), LA maximal volume (r = -0.61) and LA minimal volume (r = -0.55) (all p<0.01). Atrial ejection force as a continuous variable was positively related to age (r =0.78), peak A wave velocity (r =0.71), systolic blood pressure (r =0.51), and IVRT (r =0.41) (all p<0.01). Hypertrophy influenced the recovery of atrial function after cardioversion of A-Fib. Atrial function was reduced in patients with LV hypertrophy even after A-Fib of brief duration.

Left atrial size after cardioversion for atrial fibrillation: effect of external direct current shock.

OBJECTIVE: The aim of this study was to evaluate the effect of external direct current (DC) shock on left atrial (LA) dimension and volumes after cardioversion for atrial fibrillation, and the relation between LA size and atrial function. METHODS: We evaluated 180 patients who were randomly cardioverted with DC shock (90 patients) or drugs (90 patients). Echocardiographic evaluations included LA size and volumes. LA passive and active emptying volumes were calculated, and LA function was measured as atrial ejection force. Changes in LA diameters and volumes were correlate with atrial systolic function. RESULTS: The LA was dilated in all patients during arrhythmia and decreased after the restoration of sinus rhythm. The entity of reduction was different in the 2 groups of patients. LA maximal and minimal volumes were increased after DC shock as compared with patients treated with drugs (LA maximal volume 34 +/- 4 vs 31 +/- 5; P <.01; LA minimal volume 18 +/- 2.6 vs 15 +/- 3.6; P <.01). The atrial function was also depressed after DC shock and the delay in the recovery of atrial contractility was related to LA dilation. Patients treated with drugs had a higher atrial ejection force that was associated with a more marked reduction in LA maximal volume after the restoration of in sinus rhythm. A relationship between LA volumes and atrial ejection force was observed in the group of patients with depressed atrial mechanic function (r = -0.78; P <.001). The active emptying fraction was lower, although not significantly, in this group, whereas the conduit volume was increased. CONCLUSION: External DC shock induced a depressed atrial mechanic function in many patients and this was associated with a persistence of LA dilation.

Evidence of reverse remodeling after long-term biventricular stimulation for resynchronization in patients with wide QRS selected on the basis of echocardiographic electromechanical delays.

BACKGROUND: There is increasing evidence that cardiac resynchronization therapy (CRT) may trigger an inverse remodeling process leading to decreased left ventricular (LV) volumes in patients with heart failure and wide QRS. However, it is still important to simplify patient selection and achieve a widely applicable parameter to better stratify patients who are candidates for CRT. METHODS: Eighteen patients (13 males, 5 females, mean age 67.5 +/- 7.2 years) with advanced heart failure due to ischemic (n = 12) or idiopathic dilated cardiomyopathy (n = 6) and complete left bundle branch block received biventricular pacing. The patients were considered eligible in the presence of echocardiographic evidence of intra- and interventricular asynchrony, defined on the basis of LV electromechanical delay. Investigations were performed before pacemaker implantation (at baseline), the day after, and 3 and 6 months later. RESULTS: Two patients died before the first outpatient examination. There were 15 (83%) responders to reverse remodeling among the remainder. In the overall population, there was a significant and progressive improvement in LV sphericity indexes, ejection fraction, mitral regurgitation area and LV volumes (p < 0.001). The improvement in the interventricular mechanical delay after CRT was significantly correlated with the decrease in LV end-systolic volume (r2 = 0.2558, p = 0.04). CONCLUSIONS: CRT reduces LV volumes in patients with advanced heart failure, complete left bundle branch block and detailed documentation of ventricular asynchrony prior to therapeutic pacing. Broadly applicable Doppler echocardiographic measures may increase the specificity of the long-term response to CRT in terms of LV performance.

Atrial stunning, inflammation and nutritional status after cardioversion from atrial fibrillation.

BACKGROUND: Conversion of atrial fibrillation (A-Fib) to sinus rhythm is associated with transient mechanical dysfunction of left atrium and appendage, termed atrial stunning. OBJECTIVES: The aim of the present study was to evaluate the relationship between nutritional status and atrial stunning after conversion of A-fib. METHODS: Fifty-eight hemodynamically stable patients referred for cardioversion for lone AF were included in this study. To assess nutritional status and inflammation we measured: hemoglobin, erythrocyte mean cell volume, increased transferrin, decreased percent transferrin saturation and ferritin, albumin, CRP and Fe. Usual dietary intake was assessed with the use of a semi-quantitative food frequency questionnaire. LA function was assessed using peak atrial filling velocity, atrial ejection force and peak of emptying and filling velocities of left atrial appendage. RESULTS: Patients were categorized in 4 groups according to value of CRP and Fe. Patients with normal value of CRP and normal value of Fe and patients with elevated CRP but normal value of Fe showed no difference in parameters of atrial function. On contrary patients with reduced value of Fe showed significantly reduced parameters of atrial function. CONCLUSIONS: Data of the present study showed that patients with a reduction of Fe present a marked dysfunction of atrial contractility in comparison with other groups. We cautiously hypothesize an iron mechanism. The hemodynamic stress due to A-Fib can generate highly toxic hydroxyl radicals. These oxygen free radicals probably damage cells by oxidating various cell components and could be important in inducing myocardial stunning after A-Fib.

Effect of coffee consumption, lifestyle and acute life stress in the development of acute lone atrial fibrillation.

OBJECTIVE: The study evaluated the relationships between acute stress, lifestyle and coffee consumption, and acute lone atrial fibrillation (AF). METHODS: The study group included 400 patients with mean age of 54 +/- 11 years, 205 of whom were men. They all presented with a first episode of AF. Patients underwent a series of cognitive tests to evaluate acute psychological stress (mean life acute stress score). Lifestyle and nutritional parameters (diet, alcohol and espresso coffee consumption, smoking and obesity) were investigated. An age-matched and sex-matched control group was selected and compared. RESULTS: Recent stress, high intake of coffee, and obesity were associated with greater risk of AF. Acute stress induces an increase in coffee consumption and changes in lifestyle. The increase in coffee consumption was more marked in nonhabitual drinkers, leading to a higher risk of developing AF [odds ratio (OR) 4.1; 95% confidence interval (CI): 1.98-4.56; P < 0.001]. Spontaneous conversion of AF to sinus rhythm was observed in 191 patients (47%). Patients who experienced AF after an acute stress showed the highest probability of spontaneous conversion. High espresso coffee consumption (OR 0.86; 95% CI: 0.49-1.21; P < 0.01) and obesity (OR 0.88; 95% CI: 0.84-1.20; P < 0.01) were associated with a significantly greater risk of persistent AF. CONCLUSION: Acute stress induced changes in lifestyle, including an increase in coffee consumption, leading to a higher risk of AF. Patients who developed AF after an acute stress showed the highest probability of spontaneous conversion. High espresso coffee consumption and obesity were associated with an increased risk of persistent AF.

Impact of prehospital diagnosis in the management of ST elevation myocardial infarction in the era of primary percutaneous coronary intervention: reduction of treatment delay and mortality.

OBJECTIVES: The aim of the study is to assess, as primary endpoints, in-hospital mortality and percutaneous coronary intervention (PCI) mortality and to compare the outcome in ST elevation myocardial infarction (STEMI) patients with different pathways to a catheterization laboratory in the context of an area with 24 h availability of catheter facilities. METHODS: Three hundred and ninety-nine STEMI patients, referred to an interventional centre for primary PCI, were divided into two groups according to the different pathways to the catheterization laboratory. Group A had 263 patients diagnosed following admission to First Aid. Group B had 136 patients diagnosed in a prehospital setting with telemedicine equipment and transferred directly to the interventional centre by advanced life support (ALS) ambulance. RESULTS: Significantly shorter treatment delay was observed in group B patients than in group A (262 +/- 112 vs. 148 +/- 81 min in group A vs. B, P < 0.001). A significant reduction in total mortality was observed in group B compared with group A (8.7 vs. 3% in group A vs. B, P < 0.05). After multivariate analysis, predictors of in-hospital mortality are age and Killip class (P < 0.01), different pathways to catheterization laboratory, pre-PCI TIMI flow and onset-to-balloon time (P < 0.05). CONCLUSION: The present study shows a reduction in treatment delay and in-hospital mortality by prehospital ECG and direct referral to catheterization laboratory.

[A database in the coronary care unit to assess quality of care of acute myocardial infarction]. / Utilità di un database in unità di terapia intensiva coronarica come strumento di verifica della qualità della cura dell'infarto miocardico acuto.

BACKGROUND: In the context of an operative network for acute coronary syndrome consisting in telemedicine equipment and 24-h catheter facility availability, a database was worked out to verify clinical pathways, timeline, prevalence of cardiovascular risk factors, diagnostic and therapeutic choices, and clinical results. The aim of this study was to evaluate the adherence to guidelines in the real world in order to assess quality of care. METHODS: We implemented a database in the preexisting hospital intranet due to open source interactive pages. From international guidelines we obtained performance measures for each step of the care process, in order to have a continuous and updated evaluation of our healthcare performance. After an accurate analysis of the results of the first 2-year data collection, we modified the performance measurement set to optimize our database. All data were analyzed with statistical program SPSS. RESULTS: A total of 1066 patient characteristics within a period of 5 semesters were analyzed: trends for each of the 11 performance measures were evaluated. In 852 patients clinical and angiographic characteristics were analyzed: cardiovascular risk factors and TIMI risk score for ST-elevation myocardial infarction patients, different pathways and first-aid timeline. Data about pharmacological therapy on admission, during intensive care unit stay and at discharge were also collected. CONCLUSIONS: The present study assesses the utility of continuous monitoring and of verifying quality of care with a dedicated database in order to evaluate adherence to guidelines and to promote optimization of clinical practice.

Prognostic stratification of women with chronic heart failure referred for heart transplantation: relevance of gender as compared with gender-related characteristics.

BACKGROUND: Few studies are available regarding prognostic stratification of women with severe chronic heart failure (CHF). Although women seem to have a better outcome than men, this may be due to favorable baseline characteristics. METHODS: We analyzed a cohort of CHF patients referred for heart transplantation (HT) who underwent clinical/laboratory/instrumental evaluation. Women and men were frequency matched for baseline age (53 +/- 14 vs 53 +/- 9 years, p = 0.92), left ventricular ejection fraction (33 +/- 10 vs 31 +/- 8%, p = 0.90) and ischemic etiology (17 vs 22%, p = 0.50). RESULTS: A total of 198 patients were analyzed (109 women matched to 89 men). In addition to matching parameters, prevalence of severe symptoms, diabetes and hypertension were also comparable (p > or = 0.25). After 3 years, cardiovascular death or need for HT (CD/HT) event-free survival was 78 +/- 4% in women and 50 +/- 6% in men (p = 0.005). On multivariate analysis, female gender was associated with a lower risk of CD/HT (relative risk [RR] 0.52; 95% confidence interval [CI] 0.30 to 0.89; p = 0.017), independently of symptoms, blood pressure (BP), left ventricular end-diastolic diameter (LVEDD) and mitral regurgitation (MR). Nevertheless, CD/HT event-free survival at 3 years was 49 +/- 9% for women with New York Heart Association (NYHA) Class III or IV status, who presented with either severe MR, mean BP < or =60 mm Hg or LVEDD > or =35 mm/m2. CONCLUSIONS: In advanced CHF, women patients seem to have a better prognosis irrespective of baseline characteristics, supporting the hypothesis that female gender is protective against myocardial injury. However, women with severe symptoms accompanied by either hypotension, severe left ventricular enlargement or MR are at high risk and deserve cautious follow-up and consideration for HT.

Role of severe functional mitral regurgitation in predicting electrical remodeling in idiopathic dilated cardiomyopathy.

OBJECTIVE: To investigate incidence and predictors of clinically relevant QRS widening (predefined as > or = 10% with respect to baseline) in idiopathic dilated cardiomyopathy (IDC) and particularly the prognostic role of functional mitral regurgitation (MR). Although QRS widening in left ventricular systolic dysfunction carries relevant prognostic and therapeutic implications, its incidence and predictors in patients with IDC remain unknown. METHODS: We analyzed 114 patients with IDC receiving optimized medical treatment (age 52 +/- 10 years; 44% males; 36% New York Heart Association class III-IV) who underwent clinical, echocardiographic, hemodynamic, and laboratory evaluations and at least two electrocardiograms > or = 6 months after the index evaluation. RESULTS: During follow-up (median 20 months), 19 (17%) patients developed clinically relevant QRS widening, corresponding to an incidence of 8% per year. At multivariable analysis, the presence of echocardiographically detected severe MR (P = 0.029) and mean right atrial pressure (RAP) by right heart catheterization (P = 0.021) independently predicted clinically relevant QRS widening. CONCLUSIONS: Clinically relevant QRS widening is relatively frequent in IDC despite optimized medical treatment, and is independently predicted by MR severity and high RAP. IDC patients presenting either of these risk-factors might benefit from strict follow-up, which could also allow timely detection of the onset of indications for cardiac resynchronization therapy.

The relationship between personality, socio-economic factors, acute life stress and the development, spontaneous conversion and recurrences of acute lone atrial fibrillation.

AIMS: The present study was designed to establish the relationship between personality factors, socio-economic factors and acute life stress with development, spontaneous cardioversion and recurrences of acute lone atrial fibrillation. METHODS: The study group consisted of 116 patients with lone atrial fibrillation cardioverted within 48h of the onset of arrhythmia; they underwent a series of cognitive tests to evaluate acute psychological stress and personality type. The socio-economic status and other covariates (alcohol consumption, smoking, and body mass index) were investigated. A control group, age- and sex-matched, was selected and compared. In the logistic regression analysis, the presence of spontaneous conversion to sinus rhythm was used as the dependent variable. Independent variables were indicator variables representing categories of stress, Type A behaviour pattern, coffee consumption and body mass index. Variables considered for logistic analysis were only those with independent prognostic value. RESULTS: Type A behaviour pattern was found in 23 (20%) patients with atrial fibrillation and in 11 (9%) controls (P<0.001). The mean score among patients with atrial fibrillation was 8+/-2.7, while in control subjects it was 5.5+/-2. The mean acute life stress score among patients with atrial fibrillation was 56+/-33, while in controls it was 34+/-27 (P<0.01). Spontaneous conversion of atrial fibrillation to sinus rhythm was observed in 72 patients (63%). In univariate analysis alcohol consumption, income, education and smoking habits did not affect spontaneous conversion. High coffee consumption (OR 0.3 95% CI 0.11-0.49; P<0.008) and high body mass index were associated with a significantly greater risk of atrial fibrillation (OR 1.5 95% CI 1.2-1.7). CONCLUSIONS: Type A behaviour pattern and acute life stress affect the development and spontaneous conversion of atrial fibrillation. Patients with acute stress showed the highest probability of spontaneous conversion followed by patients with Type A behaviour. Other socio-economic factors affect spontaneous conversion and recurrences of lone atrial fibrillation to a lesser extent.

Influence of regression of left ventricular hypertrophy on left atrial size and function in patients with moderate hypertension.

OBJECTIVES: The aim of the study was to evaluate the effect of regression of left ventricular (LV) hypertrophy on left atrial (LA) size and function in patients treated with telmisartan, an angiotensin II receptor blocker. METHODS: Patients population included 80 patients with mild-moderate LV hypertrophy treated with telmisartan. Patients were followed over a period of 12 months from the start of telmisartan treatment. LA size was measured during systole from the parasternal long-axis view from M-mode. Atrial function was assessed by Doppler-echocardiography and the following parameters were measured: transmitral peak A velocity, atrial filling fraction, atrial ejection force (AEF), peak E velocity, deceleration time and isovolumic relaxation time, LA maximal and minimal volume, and LV cardiac mass index (LVMI). RESULTS: All patients had an increased LVMI and decrease during follow-up. LA dimensions were greater at baseline and reduced after 1 year of treatment. LA volume indexes maximal volume, minimal volume and P volume were reduced compared with baseline value (maximal volume from 35+/-5 to 32+/-5, p<0.05; minimal volumes from 14+/-2 to 10+/-4, p<0.05). AEF, a parameter of atrial systolic function, increased from 12+/-3 to 15+/-2.4 (p<0.01). The reduction of LA volumes correlate with reduction of LVMI (LA maximal volume and LVMI r = 0.45; p<0.01; LA minimal volume and LVMI r = 0.34; p<0.05). A positive correlation was also found between LV mass index and P volume (r = 0.41; p<0.01), LV mass index and LA active emptying volume (r = 0.39; p<0.01), and LV mass index and LA total emptying volume (r = 0.38; p<0.05). CONCLUSIONS: The present study suggests that regression of LV hypertrophy due to telmisartan is associated with reduction of LA volumes that expresses variation of LV end-diastolic pressure. The reduction of LV end-diastolic pressure is associated with an increase in diastolic filling and with a significant reduction of active and passive emptying contribution of left atrium to LV stroke volume.