Multimodality imaging evaluation of multilobulated subvalvular aortic aneurysm involving the membranous ventricular septum.

We report a rare clinical case of multilobulated subvalvular aortic aneurysm located in the left ventricular outflow tract, involving the membranous ventricular septum, and associated with mild prolapse of the noncoronary aortic leaflet, emphasizing the importance of multimodality imaging approach, and multidisciplinary discussion.

Ventricular tachycardia oversensing in S-ICD patients: Case-based brief review.

A 76-year-old woman with permanent atrial fibrillation and a mechanical aortic valve came to our attention. Echocardiography showed a 50-55% ejection fraction (EF) with good prosthesis performance. For symptomatic bradyarrhythmia, she received a VVI pacemaker (Proponent MRI L2010 model; Boston Scientific.). During follow-up, frequent symptomatic (presyncopal) episodes of nonsustained episodes of ventricular tachycardia (VT) were detected. Amiodarone proved unsuccessful; she was then offered an upgrade to an implantable cardioverter defibrillator (ICD) and a subcutaneous ICD (S-ICD) was chosen by the patient. A few weeks later, two sustained VT were detected and effectively treated with 80-J shock delivery. In both cases, device interrogation revealed a VT rate around 163 bpm (370 ms cycle length), below the lowest device detection cutoff. The report is a case-based review.

Supravalvular mitral remnant: The other side of the valve.

We report a challenging clinical case of an atypical supravalvular mitral remnant in recent mitral and aortic valve replacement with mechanical valve prostheses, associated with postoperative recurrent inflammatory episodes overlapped with difficult anticoagulation. Negative myocardial scintigraphy was associated with persistence of negative blood cultures. Serial echocardiographic evaluation was performed before and after antimicrobial treatment, and at 3 months follow-up a transesophageal echocardiography showed the persistence of the mass. Diagnostic suspect was finally confirmed.

Implantable-cardioverter-defibrillator after ST-elevation myocardial infarction: when and how frequently is it implanted and what is the rate of appropriate interventions? Insight from Trieste registry.

BACKGROUND: There are limited data on implantable-cardioverter-defibrillator (ICD) implantation after ST-elevation myocardial infarction (STEMI) treated with primary percutaneous coronary intervention (pPCI). Therefore, we evaluated when and how frequently an ICD is implanted after pPCI, the rate of appropriate ICD interventions, and predictors of ICD implantation. METHODS: We analyzed STEMI patients treated with pPCI at the University Hospital of Trieste, Italy, between January 2010 and December 2019. We cross-matched patients' data with those present in the Trieste ICD registry. RESULTS: Among 1805 consecutive patients treated with pPCI, 3.6% underwent ICD implantation during a median follow-up of 6.7 [interquartile range (IQR) 4.3-9.2] years. At 12 months, the mean number of ICD implantations was 2.3/100 patients [95% confidence interval (95% CI) 1.7-3.1] and remained stable over time (at 24 months: 2.5/100 patients, 95% CI 2.0-3.5 and at 36 months: 2.6/100 patients, 95% CI 2.3-3.8); 83.1% of ICDs were implanted for primary prevention, and more than half (55%) were implanted in patients with ejection fraction more than 35% at the moment of STEMI discharge. The rate of appropriate ICD interventions was 16.9% at a median follow-up of 5.7 years (IQR 3.3-8.3 years) after ICD implantation. At 12 months, the mean number of appropriate ICD interventions was 5/100 patients and 7/100 patients after 24 months. In patients with ejection fraction more than 35% at STEMI discharge (median ejection fraction 43%; IQR 40-48), independent predictors of ICD implantation were male sex, anterior STEMI and troponin peak more than 100 000 ng/dl. CONCLUSION: The rate of ICD implantations after pPCI is low; however, the rate of appropriate ICD interventions is high. A relevant subgroup of patients received ICD implantations at follow-up despite a nonsevere ejection fraction at discharge after STEMI. Among these patients, those with high troponin release deserve strict follow-up and full optimal medical treatment.

Early prognostic stratification and identification of irreversibly shocked patients despite primary percutaneous coronary intervention.

BACKGROUND: Despite prognostic improvements in ST-elevation myocardial infarction (STEMI), patients presenting with cardiogenic shock (CS) have still high mortality. Which are the relevant early prognostic factors despite revascularization in this high-risk population is poorly investigated. METHODS: We analyzed STEMI patients treated with primary percutaneous coronary intervention (PCI) and enrolled at the University Hospital of Trieste between 2012 and 2018. A decision tree based on data available at first medical contact (FMC) was built to stratify patients for 30-day mortality. Multivariate analysis was used to explore independent factors associated with 30-day mortality. RESULTS: Among 1222 STEMI patients consecutively enrolled, 7.5% presented with CS. CS compared with no-CS patients had worse 30-day mortality (33% vs 3%, P < 0.01). Considering data available at FMC, CS patients with a combination of age ≥76 years, anterior STEMI and an expected ischemia time > 3 h and 21 min were at the highest mortality risk, with a 30-day mortality of 85.7%. In CS, age (OR 1.246; 95% CI 1.045-1,141; P = 0.003), final TIMI flow 2-3 (OR 0.058; 95% CI 0.004-0.785; P = 0.032) and Ischemia Time (OR = 1.269; 95% CI 1.001-1.609; P = 0.049) were independently associated with 30-day mortality. CONCLUSIONS: In a contemporary real-world population presenting with CS due to STEMI, age is a relevant negative factor whereas an early and successful PCI is positively correlated with survival. However, a subgroup of elderly patients had severe prognosis despite revascularization. Whether pPCI may have an impact on survival in a very limited number of irreversibly critically ill patients remains uncertain and the identification of irreversibly shocked patients remains nowadays challenging.

COVID-19 impact on ST-elevation myocardial infarction incidence rate in a Italian STEMI network: a U-shaped curve phenomenon.

BACKGROUND: Public health emergencies such as the COVID-19 outbreak may impact on the incidence rate of ST-elevation myocardial infarction (STEMI) in severely affected areas. However, this phenomenon demands attention also in areas where media and patients were focused on the COVID-19 pandemic, but the healthcare system was not overwhelmed by the huge number of COVID-19 patients. METHODS AND RESULTS: In this observational study, we compared the incidence rate of all consecutive STEMI patients admitted at the University Hospital of Trieste, Italy, during March and April 2020 with the same 2 months of the previous 5 years (2015-2019). Patient characteristics were compared between 2020 and 2019.The incidence rate of STEMI admission in March-April 2020 was lower than those in March-April 2015-2019, 36 vs. 56 cases per 100 000 inhabitants/year [relative risk (RR) 0.65, 95% confidence interval (95% CI) 0.42-0.96, P = 0.045]. Considering that the incidence rates were constant in the past years (P = 0.24), the turnaround in 2020 is most likely due to the COVID-19 outbreak. Interestingly, this reduction was a dynamic phenomenon with a U-shaped curve during the 2-month period. System-of-care times were similar between 2020 and 2019; however in 2020, patients presented more frequently signs of heart failure compared to 2019 (Killip class ≥2 in 68% vs. 29%, P = 0.003). CONCLUSION: During the COVID-19 outbreak, we observed a marked reduction in the STEMI incidence rate. This U-shaped phenomenon demands attention because a potential cause for the decrease in STEMI incidence may include the avoidance of medical care. Public campaigns aiming to increase awareness of ischemic symptoms may be needed during community outbreak.

Right Ventricular Dysfunction in Right Coronary Artery Infarction: A Primary PCI Registry Analysis.

Right ventricular involvement in inferior myocardial infarction (MI) was historically associated with a poor prognosis. However, few studies addressed the impact of right ventricular (RV) dysfunction in the primary percutaneous intervention (pPCI) era. Our aim was to assess the prognostic significance of RV dysfunction in right coronary artery (RCA) related MI treated with pPCI. METHODS: A total of 298 patients with a RCA related MI undergone pPCI between January 2011 and June 2015 were included. RV dysfunction was defined by a RV-FAC <35% at echocardiographic examination and further divided into mild (RV-FAC between 35 and 25%) and moderate-severe (RV-FAC <25%). RV function before discharge was reassessed in 95% of the study cohort. The primary endpoint was overall mortality. Median follow-up was 29 months. RESULTS: In RCA related MI, moderate-severe (HR 5.882, p = 0.002, 95% CI 1.882-18.385) but not mild RV dysfunction independently predicted lower survival at follow-up along with age (HR 1.104, p <0.001, CI 1.045-1.167). Importantly, patients recovering RV function at discharge showed a lower mortality (p = 0.001) vs patients with persistent moderate-severe RV dysfunction) that approached the risk of patients without RV dysfunction at presentation. CONCLUSION: In RCA related MI treated with pPCI, RV dysfunction was one of the strongest independent predictor of lower overall survival. However, patients with only transient RV dysfunction showed a better prognosis compared to patients who had persistent RV dysfunction. The focus on intensive support management of the RV in the first hours after pPCI may be important to overcome the acute phase and to promote RV recovery.

Management of nonischemic-dilated cardiomyopathies in clinical practice: a position paper of the working group on myocardial and pericardial diseases of Italian Society of Cardiology.

: Nonischemic-dilated cardiomyopathy (NIDCM) is an entity that gathers extremely heterogeneous diseases. This awareness, although leading to continuous improvement in survival, has increased the complexity of NIDCM patients' management. Even though the endorsed 'red-flags' approach helps clinicians in pursuing an accurate etiological definition in clinical practice, it is not clear when and how peripheral centers should interact with referral centers with specific expertise in challenging scenarios (e.g. postmyocarditis and genetically determined dilated cardiomyopathy) and with easier access to second-line diagnostic tools and therapies. This position paper will summarize each step in NIDCM management, highlighting the multiple interactions between peripheral and referral centers, from first-line diagnostic workup and therapy to advanced heart failure management and long-term follow-up.

[Diagnostic work-up and clinical management of cardiomyopathies: the operative protocol from the Cardiothoracovascular Department of Trieste, Italy]. / Inquadramento e gestione delle cardiomiopatie: il protocollo della Cardiologia di Trieste.

Cardiomyopathies are primary myocardial disorders, genetically determined, with clinical onset between the third and the fifth decade of life. They represent the main causes of sudden cardiac death and heart failure in the youth. The more common myocardial diseases in clinical practice are dilated cardiomyopathy, arrhythmogenic cardiomyopathy and hypertrophic cardiomyopathy. Next generation sequencing techniques, recently available for genetics researches, together with the diffusion of advanced imaging techniques, permitted in the last years a deeper knowledge of these pathologies. Nevertheless, diagnosis, etiology and several aspects of patients' clinical management remain complex and controversial. This review paper aims to propose some operative flow-charts, derived from scientific evidences and the internal protocol of the Cardiothoracovascular Department of Trieste Hospital, Italian referral Center for cardiomyopathies and heart failure, with more than 30 years of experience in diagnosis and management of patients who suffer from primary myocardial disorders.

A misdiagnosed case of antisynthetase syndrome complicated by myocarditis: when the cardiologist deals with rheumatology.

We report a case of a 56-year-old woman with an alleged diagnosis of an acute coronary syndrome that was later correctly identified in our Cardiology Unit as antisynthetase syndrome (AS) with inflammatory cardiac involvement. In this case report, we focus on clinical features of this rare autoimmune disease aiming to provide useful tips to achieve correct differential diagnosis according to updated international guidelines and recommendations, especially in cases of concurrent disease-related myocarditis.

From Bicuspid to Quadricuspid Aortic Valve: The Clinical Case of a 38-year-old Woman with Chest Pain.

We report a case of a 38-year-old woman with an alleged diagnosis of bicuspid aortic valve disease that was correctly identified as quadricuspid aortic valve (QAV) disease in our cardiology unit. In this case report, we focus on echocardiographic features of this rare congenital valve disease aiming to provide useful tips to achieve correct differential diagnosis according to the updated echocardiographic international guidelines and recommendations, briefly reviewing other QAV cases reported in the current literature as well. In conclusion, we strongly recommend adhering to practical echocardiographic guidelines to reduce interobserver variability, not to miss the diagnosis of rare congenital defects like the one we reported.

How do cardiologists select patients for dual antiplatelet therapy continuation beyond 1 year after a myocardial infarction? Insights from the EYESHOT Post-MI Study.

BACKGROUND: Current guidelines suggest to consider dual antiplatelet therapy (DAPT) continuation for longer than 12 months in selected patients with myocardial infarction (MI). HYPOTHESIS: We sought to assess the criteria used by cardiologists in daily practice to select patients with a history of MI eligible for DAPT continuation beyond 1 year. METHODS: We analyzed data from the EYESHOT Post-MI, a prospective, observational, nationwide study aimed to evaluate the management of patients presenting to cardiologists 1 to 3 years from the last MI event. RESULTS: Out of the 1633 post-MI patients enrolled in the study between March and December 2017, 557 (34.1%) were on DAPT at the time of enrolment, and 450 (27.6%) were prescribed DAPT after cardiologist assessment. At multivariate analyses, a percutaneous coronary intervention (PCI) with multiple stents and the presence of peripheral artery disease (PAD) resulted as independent predictors of DAPT continuation, while atrial fibrillation was the only independent predictor of DAPT interruption for patients both at the second and the third year from MI at enrolment and the time of discharge/end of the visit. CONCLUSIONS: Risk scores recommended by current guidelines for guiding decisions on DAPT duration are underused and misused in clinical practice. A PCI with multiple stents and a history of PAD resulted as the clinical variables more frequently associated with DAPT continuation beyond 1 year from the index MI.

Prognostic relevance of pericardial effusion in STEMI patients treated by primary percutaneous coronary intervention: a 10-year single-centre experience.

BACKGROUND: Pericardial effusion is frequent in the acute phase of ST-segment elevation myocardial infarction. However, its prognostic role in the era of primary percutaneous coronary intervention is not completely understood. METHODS: We investigated the association between pericardial effusion, assessed by transthoracic echocardiography, and survival in a large cohort of ST-segment elevation myocardial infarction patients treated with primary percutaneous coronary intervention, enrolled in the Trieste primary percutaneous coronary intervention registry from January 2007 to March 2017. Multivariable analysis and a propensity score approach were performed. RESULTS: A total of 1732 ST-segment elevation myocardial infarction patients were included. Median follow-up was 45 (interquartile range 19-79) months. Pericardial effusion was present in 246 patients (14.2%). Thirty-day all-cause mortality was similar between patients with and without pericardial effusion (7.8% vs. 5.4%, P=0.15), whereas crude long-term survival was worse in patients with pericardial effusion (26.2% vs. 17.7%, P≤0.01). However, at multivariable analyses the presence of pericardial effusion was not associated with long-term mortality (hazard ratio 1.26, 95% confidence interval 0.86-1.82, P=0.22). Matching based on propensity scores confirmed the lack of association between pericardial effusion and both 30-day (hazard ratio 1, 95% confidence interval 0.42-2.36, P=1) and long-term (hazard ratio 1.14, 95% confidence interval 0.74-1.78, P=0.53) all-cause mortality. Patients with pericardial effusion experienced a higher incidence of free wall rupture (2.8% vs. 0.5%, P<0.0001) independently of the entity of pericardial effusion. CONCLUSIONS: In acute ST-segment elevation myocardial infarction treated with primary percutaneous coronary intervention, the onset of pericardial effusion after ST-segment elevation myocardial infarction is not independently associated with short and long-term higher mortality. Free wall rupture has to be considered rare compared to the fibrinolytic era and occurs more frequently in patients with pericardial effusion, suggesting a close monitoring of these patients in the early post-primary percutaneous coronary intervention phase.

Prognostic relevance of pericardial effusion in STEMI patients treated by primary percutaneous coronary intervention: a 10-year single-centre experience.

BACKGROUND: Pericardial effusion is frequent in the acute phase of ST-segment elevation myocardial infarction. However, its prognostic role in the era of primary percutaneous coronary intervention is not completely understood. METHODS: We investigated the association between pericardial effusion, assessed by transthoracic echocardiography, and survival in a large cohort of ST-segment elevation myocardial infarction patients treated with primary percutaneous coronary intervention, enrolled in the Trieste primary percutaneous coronary intervention registry from January 2007 to March 2017. Multivariable analysis and a propensity score approach were performed. RESULTS: A total of 1732 ST-segment elevation myocardial infarction patients were included. Median follow-up was 45 (interquartile range 19-79) months. Pericardial effusion was present in 246 patients (14.2%). Thirty-day all-cause mortality was similar between patients with and without pericardial effusion (7.8% vs. 5.4%, P=0.15), whereas crude long-term survival was worse in patients with pericardial effusion (26.2% vs. 17.7%, P≤0.01). However, at multivariable analyses the presence of pericardial effusion was not associated with long-term mortality (hazard ratio 1.26, 95% confidence interval 0.86-1.82, P=0.22). Matching based on propensity scores confirmed the lack of association between pericardial effusion and both 30-day (hazard ratio 1, 95% confidence interval 0.42-2.36, P=1) and long-term (hazard ratio 1.14, 95% confidence interval 0.74-1.78, P=0.53) all-cause mortality. Patients with pericardial effusion experienced a higher incidence of free wall rupture (2.8% vs. 0.5%, P<0.0001) independently of the entity of pericardial effusion. CONCLUSIONS: In acute ST-segment elevation myocardial infarction treated with primary percutaneous coronary intervention, the onset of pericardial effusion after ST-segment elevation myocardial infarction is not independently associated with short and long-term higher mortality. Free wall rupture has to be considered rare compared to the fibrinolytic era and occurs more frequently in patients with pericardial effusion, suggesting a close monitoring of these patients in the early post-primary percutaneous coronary intervention phase.

Clinical management of dilated cardiomyopathy: current knowledge and future perspectives.

Dilated cardiomyopathy (DCM) is a primary heart muscle disease characterized by a progressive dilation and dysfunction of either the left or both ventricles. The management of DCM is currently challenging for clinicians. The persistent lack of knowledge about the etiology and pathophysiology of this disease continues to determine important fields of uncertainty in managing this condition. Molecular cardiology and genetics currently represent the most crucial horizon of increasing knowledge. Understanding the mechanisms underlying the disease allows clinicians to treat this disease more effectively and to further improve outcomes of DCM patients through advancements in etiologic characterization, prognostic stratification and individualized therapy. Left ventricular reverse remodeling predicts a lower rate of major cardiac adverse events independently from other factors. Optimized medical treatment and device implantation are pivotal in inducing left ventricular reverse remodeling. Newly identified targets, such as angiotensin-neprilysin inhibition, phosphodiesterase inhibition and calcium sensitizing are important in improving prognosis in patients affected by DCM.

[Dilated cardiomyopathy: a dynamic disease - clinical course, reverse remodeling and prognostic stratification]. / La cardiomiopatia dilatativa come malattia dinamica: storia naturale, rimodellamento inverso e stratificazione prognostica.

Dilated cardiomyopathy (DCM) is a relatively rare primary heart muscle disease with genetic or post-inflammatory etiology. In the last decade, the incidence and prevalence of the disease have significantly increased as a consequence of an earlier diagnosis supported by extensive familial screening programs and by the improvement in diagnostic techniques. Moreover, current therapeutic strategies have deeply modified the prognosis of DCM with a dramatic reduction in mortality. A significant number of patients with DCM present an impressive response to pharmacological and non-pharmacological therapy in terms of left ventricular reverse remodeling (reduction in ventricular size with improvement of systolic function), which confers a more favorable prognosis in the long term. However, the identification of patients with an increased likelihood of improvement after therapeutic optimization remains a challenging issue; in particular the assessment of arrhythmic risk carries important implications. Finally, the long-term follow-up of patients showing a significant left ventricular functional recovery under optimal treatment is still poorly known. Hence, the aim of the present review is to provide an insight into the clinical evolution/long-term follow-up of DCM, which should be actually considered a dynamic process rather than a static and chronic disease. Left ventricular reverse remodeling should be considered a key therapeutic goal, mostly associated with a long-standing recovery, but cannot be considered the expression of permanent "healing", confirming the need for a systematic and careful follow-up over time in this setting.

Pulmonary artery aneurysm and sarcoidosis.

Pulmonary artery aneurysm unassociated to congenital heart disease and pulmonary hypertension is exceedingly rare. Its pathogenesis and correct management remain unknown. Sarcoidosis is a systemic disease that can exceptionally involve large vessels, leading to stenosis and dilatation. Pulmonary artery aneurysm has never been described in association with sarcoidosis. Surgical approach should prevent aneurysm rupture, but it is not known when surgery should be preferred to strict medical follow-up. In this report we present a case of large pulmonary artery aneurysm associated to systemic sarcoidosis underlining problematic management of diseases 'forgotten' by evidence based medicine.

Fatal myocarditis: morphologic and clinical features.

BACKGROUND: Autopsy studies report a frequency of myocarditis ranging from 0.11 to 5.5% in the general population, reaching almost 50% in selected groups. Myocarditis is often undiagnosed and the incidence of fatal course myocarditis has never been evaluated. The aim of our study was to assess the frequency of fatal course myocarditis in a consecutive series of autopsies and to describe the clinical, histological and morphologic features of the disease. METHODS: From January 1, 1995 to January 31, 1996, 2560 autopsies were performed, and 143 cases of active myocarditis were diagnosed (5.6%). RESULTS: In 39 cases (1.5%; 12 males; 4/39 aged < or = 35 years) active myocarditis was identified as the final cause of death. Only in 1 case was myocarditis suspected ante-mortem. The histological pattern was lymphocytic in 64% of cases. A mixed inflammatory infiltration was found in 33% and a granulomatous infiltration in 3%. In 49% of cases myocarditis was localized in both ventricles and the interventricular septum. The clinical presentation of myocarditis was heart failure in 18/39 patients (46%), cardiac arrest in 4/39 patients (10%) and syncope and chest pain in 1/39 patient (3%). The mean creatine phosphokinase levels were 890 +/- 2742 IU/I (assessed in 11/39 patients, 28%) but they were increased only in 7/39 (18%). ECG (performed in 29/39 patients, 74%) showed sinus rhythm in 16/39 patients (55%, > 100 b/min in 41%), atrioventricular or interventricular conduction defects in 10/39 patients (34%) and a pathological Q wave in 4/39 patients (14%). At echocardiography (performed in 7/39 patients, 18%), right and/or left ventricular dysfunction was found to be present in 5 cases (71%) and a pericardial effusion in 4 cases (57%). CONCLUSIONS: Myocarditis is underdiagnosed ante-mortem. A high index of clinical suspicion is mandatory for prompt diagnosis and treatment of this fatal disease seen also in the young.