Search

Introduction Aortic valves with a number of cusps different from two or three are infrequent congenital anomalies that might manifest with valvular disease in young patients. We present two cases of unicuspid and quadricuspid aortic valve diagnosed in our Center. Clinical Cases Case 1. A 23-year-old asymptomatic male was admitted to our Hospital because of the necessity of bone marrow transplantation for Hodgkin lymphoma. He had a previous diagnosis of bicuspid aortic valve and presented a 5/6 mid-systolic harsh murmur, heard best at the right upper sternal border and radiating to the neck. ECG showed sinus rhythm with left ventricular hypertrophy. BNP was in normal range (31 pg/ml). At trans-thoracic echocardiography, there was severe aortic stenosis [peak/mean gradient of 100/67 mmHg with an aortic valve area of 1.04 cm2 (0.54 cm2/m2); V max was 5 m/s] without regurgitation and dilation of the ascending aorta (39 mm), however the morphology of the aortic valve remained undefined. Left ventricle showed hypertrophy (maximum wall thickness = 13 mm) with normal volumes and systolic function. Thus, the patient underwent to 3D-Transesophageal echocardiography, which demonstrated unicuspid aortic valve with a lone commissure between L-NC and anterior calcification. After multidisciplinary discussion, the patient underwent close follow-up and aortic valve replacement was planned after the bone marrow transplantation. Case 2. A 21-year-old male was referred by general practitioner to our outpatient clinic for palpitations. Cardiovascular physical examination and ECG were normal with no evidence of arrhyhtmia or cardiac murmurs. Transthoracic echocardiography revealed central mild aortic valve regurgitation in a suspected quadricuspid aortic valve (i.e., an X-shaped commissure pattern during diastole and a rectangular appearance during systole in the parasternal short-axis view). Left ventricular size and function and ascending aorta dimensions were normal. There was no associated congenital cardiovascular abnormality. 3D-Transesophageal echocardiography confirmed the evidence of a quadricuspid aortic valve. The cusps appeared to be of different size: two equal larger cusps and two equal smaller cusps (Type C, Hurwitz and Robert's classification), thin and mobile. The patient was advised to echocardiographic follow-up at intervals of 12 months. Conclusions We describe two cases of aortic valve congenital anomalies in two coetaneous patients with completely different impacts on hemodynamics. Severe aortic stenosis complicated unicuspid aortic valve whereas quadricuspid aortic valve was associated with mild aortic regurgitation. 3D-Transesophageal echocardiography was essential for accurate morphological characterization of the aortic valves.

Aims Cardiovascular sequelae may occur in patients recovered from COVID-19. Recent studies have detected a considerable incidence of subclinical myocardial dysfunction – assessed with speckle-tracking echocardiography – and of long-COVID symptoms in these patients. This study aimed to define the long-term prognostic role of subclinical myocardial dysfunction and long-COVID condition in patients recovered from COVID-19 pneumonia. Methods and Results We prospectively followed-up 110 patients hospitalized at our Institution due to COVID-19 pneumonia in April 2020 and then recovered from SARS-CoV-2 infection. A 6-month clinical and echocardiographic evaluation was performed, followed by a 21-month clinical follow-up. The primary outcome was major adverse cardiovascular events (MACE), a composite of myocardial infarction, stroke, heart failure hospitalization and all-cause mortality. A subclinical myocardial dysfunction – defined as an impairment of left ventricular global longitudinal strain (≥ -18%) - was identified at 6-month follow-up in 37 patients (34%), was associated with an increased risk of long-term MACE with a good discriminative power (AUC: 0.73) and resulted a strong independent predictor of extended MACE in a multivariate regression analysis (OR 9.29, 95%CI 2.20–39.3, p=0.002). Long-COVID condition was not associated with a worse long-term prognosis, instead. Conclusion In patients recovered from COVID-19 pneumonia, a subclinical myocardial dysfunction is present in one third of the whole population at 6-month follow-up and is associated with a higher risk of MACE at long-term follow-up. Speckle-tracking echocardiography is a promising tool to optimise the risk-stratification in patients recovered from COVID-19 pneumonia, while the definition of a long-COVID condition has not prognostic relevance.

Background The introduction of Cardiac-MRI (cMRI) in clinical practice has considerably improved risk stratification of patients with hypertrophic cardiomyopathy (HCM). However, the prediction of adverse outcome based on imaging markers remains suboptimal. Right ventricular (RV) involvement is an emerging finding in this disease of unknown clinical value. The identification of accurate and sensible imaging markers to detect morpho-functional alterations of the RV is therefore essential to establish, in the future, any prognostic impact of RV involvement in HCM in order to improve risk stratification. RV free wall longitudinal strain (RV-FWLS) is a promising marker to unveil subclinical RV dysfunction despite normal conventional indices of RV systolic function; however, RV-FWLS has been scarcely explored in HCM patients. Case Summary A 29-year-old man with sarcomeric HCM due to MYBPC3 mutation was referred to our Institution. Trans-Thoracic Echocardiography (TTE) showed left ventricular (LV) apical hypertrophy (27 mm) with an ace of spades morphology and mid-ventricular obstruction (peak gradient 38 mmHg). LV-EF was normal (62%) while LV global longitudinal strain was significantly impaired (-9.3% with a reverse apical sparing pattern). 2nd-degree diastolic dysfunction and left atrial enlargement (maximum volume: 42 ml/m2) were observed. No LV apical aneurysm nor paradoxical diastolic flow at the apex were noted. RV hypertrophy was present (maximum thickness 8 mm) with normal conventional indices of RV systolic function: TAPSE 26 mm, S’ TDI 12 cm/s, FAC 50%. In contrast, RV-FWLS was significantly reduced (–16%). cMRI confirmed normal bi-ventricular function in presence of left and right hypertrophy (maximum wall thickness 28 and 12 mm, respectively) and LV apical thinning. At tissue characterization, elevated native T1 and T2 values were evident in the apex (1071 +/- 45 ms and 54 +/- 6 ms, respectively) and a significant amount of patchy LGE was present in the mid-apical segments of the LV (28% of LV mass) and of the RV. After consideration of clinical and imaging data, an s-ICD for primary prevention was implanted. Discussion The present case highlights known issues and poses new challenges in managing HCM patients. First, it confirms the central role of cMRI, demonstrating its unique capability to spot myocardial fibrosis, guiding our decision to implant an s-ICD in our patient. Second, it draws attention to RV involvement in HCM, an emerging finding in this condition. MYBPC3 mutation has been associated with RV hypertrophy. Both of these conditions, together with patchy RV-LGE, were found in our patient. Notably, RV-FWLS was the only RV systolic index to be impaired and might represent an early and more accurate marker of RV systolic dysfunction compared to other conventional indices, including RV-EF by cMRI. Future studies assessing the prognostic value of RV involvement, including RV-LGE and RV-FWLS, are needed in HCM patients to potentially refine risk stratification in this challenging population.

Introduction The diagnosis of constrictive pericarditis can be challenging through conventional imaging. Novel imaging techniques have been proposed to improve it. Clinical Case A 74-year-old man with a previous diagnosis of heart failure with preserved ejection fraction and permanent atrial fibrillation (AF) was admitted because of worsening dyspnea, peripheral edema, and ascites refractory to diuretics therapy. ECG revealed low QRS voltages and AF with normal heart rate and laboratory exams detected increased values of BNP and hepatic stasis indices; blood count and CRP were normal. Echocardiography revealed mildly reduced bi-ventricular pump function (left ventricular ejection fraction = 52%, right ventricular fractional area change = 25%) and slightly increased wall thickness. Atria were moderately dilated (left and right atrium 46 and 44 ml/mq, respectively) and mitral and tricuspid annuli were dilated too. There was inferior vena cava plethora, moderate atriogenic tricuspid regurgitation and mild-to-moderate atriogenic mitral regurgitation. A paradoxical interventricular septal motion and significant respiratory changes on trans-mitral (>25%) and trans-tricuspid (>30%) pulsed Doppler were noted; however, there was no "annulus reversus" (lateral and septal e’ 13 and 11 cm/sec, respectively) at TDI analysis. In contrast, 2D-speckle-tracking global longitudinal strain showed impaired deformation ​​of the lateral segments with relative sparing of the septal segments. The latter pattern has been recently described as "strain reversus" or "hot septum sign". The findings were suspected of constrictive pericarditis, but a chest Computed Tomography excluded pericardial calcifications. Cardiac magnetic resonance (CMR) was then performed revealing a "septal shift" at cine-real time sequences analysis. The pericardium was slightly thickened (4-5 mm) with widespread late gadolinium enhancement but without signs of acute inflammation at T2-weighted imaging. Tricuspid regurgitation was confirmed to be "moderate" after phase-contrast imaging. Cardiac catheterization finally revealed normal coronary arteries and confirmed the diagnosis of constrictive pericarditis (square root sign, equalization of bi-ventricular end-diastolic pressures). The patient underwent pericardiectomy and tricuspid and mitral valve repair and was asymptomatic at 6-months follow-up. Conclusions Constrictive pericarditis represents a potentially reversible cause of heart failure but can be easily missed through conventional imaging. In the present case, advanced echocardiography and CMR were essential to come up with an accurate diagnosis, guiding the patient's clinical management with excellent outcome.

Aims Whether pulmonary artery (PA) dimension and coronary artery calcium (CAC) score, as assessed by chest computed tomography (CT), are associated with myocardial injury in patients with coronavirus disease 2019 (COVID-19) is not known. The aim of this study was to explore the risk factors for myocardial injury and death and to investigate whether myocardial injury has an independent association with all-cause mortality in patients with COVID-19. Methods and Results This is a single-centre cohort study including consecutive patients with laboratory-confirmed COVID-19 undergoing chest CT on admission. Myocardial injury was defined as high-sensitivity troponin I >20 ng/L on admission. A total of 332 patients with a median follow-up of 12 days were included. There were 68 (20.5%) deaths; 123 (37%) patients had myocardial injury. PA diameter was higher in patients with myocardial injury compared with patients without myocardial injury [29.0 (25th–75th percentile, 27–32) mm vs. 27.7 (25–30) mm, P < 0.001). PA diameter was independently associated with an increased risk of myocardial injury [adjusted odds ratio 1.10, 95% confidence interval (CI) 1.02–1.19, P = 0.01] and death [adjusted hazard ratio (HR) 1.09, 95% CI 1.02–1.17, P = 0.01]. Compared with patients without myocardial injury, patients with myocardial injury had a lower prevalence of a CAC score of zero (25% vs. 55%, P < 0.001); however, the CAC score did not emerge as a predictor of myocardial injury by multivariable logistic regression. Myocardial injury was independently associated with an increased risk of death by multivariable Cox regression (adjusted HR 2.25, 95% CI 1.27–3.96, P = 0.005). Older age, lower estimated glomerular filtration rate, and lower PaO2/FiO2 ratio on admission were other independent predictors for both myocardial injury and death. Conclusions An increased PA diameter, as assessed by chest CT, is an independent risk factor for myocardial injury and mortality in patients with COVID-19. Myocardial injury is independently associated with an approximately two-fold increased risk of death.

Aims Mitral annulus disjunction (MAD) has been associated with sudden cardiac death in selected patients with arrhythmic presentation, while its clinical significance in unselected cohorts remains unknown. Our purpose was to assess the prevalence and clinical significance of MAD in consecutive patients referred to cardiovascular-magnetic-resonance (CMR). Methods and results Our population included 103 consecutive patients undergoing CMR at our Institution, between August and September 2021. MAD was defined as a ≥ 1 mm atrial displacement of the mitral leaflet hinge point in standard long-axis cine images during end-systole. MAD analysis was performed in 97 patients (feasibility = 94%) and resulted positive in 49 (51%). MAD—patients were more often males (75% vs. 57%; P = 0.045) and affected by ischaemic (35% vs. 12%, P = 0.01) and non-ischaemic cardiomyopathy (38% vs. 16%, P = 0.026) compared to MAD+ patients. No significant differences were found in terms of age, history of ventricular arrhythmias, bi-ventricular and bi-atrial volumes, bi-ventricular ejection fraction, native T1 and T2 mapping values, extracellular volume, and prevalence of late gadolinium enhancement (P > 0.05 for all) between MAD + vs. MAD—patients. MAD extent was higher in patients with mitral valve prolapse (MVP; n = 7), (3.5 ± 1.5 mm in MVP+ vs. 2.0 ± 1.0 mm in MVP– patients; P = 0.004). No significant differences were conversely found in MAD extent between patients with and without ventricular arrhythmias (2.5 ± 1.1 mm vs. 2.3 ± 1.1 mm; P = 0.815). Conclusions Our findings suggest a high prevalence of MAD in unselected cohorts of patients, with no clinical significance. Prospective studies are needed to further elucidate the interplay between MAD and malignant ventricular arrhythmias in unselected cohorts of patients.

Aims Repetitive Levosimendan treatment in advanced heart failure patients has not been investigated yet via myocardial work indices (MWI), which could more accurately detect the effects of this both inotropic and vasodilatory drug. The aims of this study were (1) to describe variations of myocardial work indices, as a consequence of repetitive Levosimendan infusions and (2) to assess the prognostic value of myocardial work parameters in these patients. Methods and results Fourteen patients with advanced heart failure treated with intermittent in-hospital levosimendan infusions were prospectively included. Clinical, laboratory, and echocardiographic assessment were performed before and after every Levosimendan infusion. The primary endpoint was a composite of any episode of decompensated HF, urgent HF rehospitalization, cardiogenic shock, cardiac arrest and cardiovascular death at 4–6 weeks follow-up after each planned infusion. During follow-up (mean: 150 ± 99 days) a total of 37 infusions were performed and a total of 11 cardiovascular events occurred. Global constructive work (GCW), global work efficiency (GWE), and global work index (GWI) increased after Levosimendan infusion in 62.2%, 73.0%, and 70.3% of cases, with significant differences between patients with and without outcomes [delta GCW: −7.36 mmHg% (134.12) vs. 113.81 mmHg% (204.41), P = 0.007; delta GWE: −3.27% (8.38) vs. 4.30% (5.58), P = 0.002]. Delta value of GWE showed the largest area under curve (AUC: 0.82, 95% CI: 0.64–1.00, P = 0.002) for outcome prediction with a cut-off point of 0.5%. Independent prognostic value of GWE variation was confirmed in multivariable regression models (OR: 0.825, 95% CI: 0.702–0.970, P = 0.02). Conclusions GWE and GCW provided incremental and independent prognostic value at short-term follow-up over traditional echocardiographic parameters. The differentiation of patients into ‘workers’, whose GWE improved after Levosimendan infusion, and ‘non-workers’, who failed to improve their GWE, permitted to identify patients at higher risk of forthcoming cardiovascular events. Monitoring these patients with MWI may have relevant clinical implications.

Papillary muscle rupture is a rare complication of myocardial infarction. Here we describe a case of an apparently spontaneous rupture of papillary muscle, not associated with coronary obstruction. The patient was a 73-year-old man admitted to our hospital for diabetic ketoacidosis complicated by acute pulmonary edema and cardiogenic shock. Transthoracic and transesophageal echocardiography showed partial rupture of papillary muscle leading to severe mitral regurgitation. Urgent cardiac surgery with mitral valve replacement was performed.

More than 2 decades ago, para-Hisian pacing was introduced to assess the pattern of retrograde conduction during electrophysiological studies. Although there is no ideal maneuver for every patient and condition, para-Hisian pacing is a valuable and handy strategy to differentiate between retrograde conduction over the atrioventricular node and the accessory pathways. The dynamic behavior of para-Hisian pacing, in a region with unique anatomical features, can produce various activation patterns and intriguing electrophysiological phenomena. Although the demonstration of a retrograde nodal activation pattern during para-Hisian pacing does not rule out the presence of an accessory pathway, evidence of retrograde conduction over an accessory pathway does not prove its active role in the culprit tachycardia. Multipolar His bundle recordings, detailed atrial mapping, and recognition of the truly captured structures and the impact of temporal changes of autonomic tone or pacing rates, are essential keys for accurate interpretation of this maneuver that may ultimately guide judicious catheter ablation of the arrhythmic substrate. This review aims to summarize the practical usefulness and potential pitfalls of the para-Hisian pacing maneuver, focusing on the interpretation of electrocardiograms and intracardiac recordings.

This case concerns a 24‐year‐old female who developed malignant ventricular tachyarrhythmia a few weeks after pacemaker implantation for complete heart block. Apparently, right ventricular pacing caused significant repolarization abnormalities in both native and paced rhythms with marked QT prolongation and substantial electrical instability. This case highlights other intriguing phenomena in the puzzle of cardiac repolarization and how pacing therapy may alter this complex process providing arrhythmic substrate in vulnerable subjects. Though such arrhythmic events are clinically rare, vulnerable patients or with suspected myocardial disease that may cause QT prolongation should be carefully followed in the course of pacing therapy.

Background Myocardial injury is frequent among patients hospitalized with coronavirus disease-2019 (COVID-19) and is associated with a poor prognosis. However, the mechanisms of myocardial...

Aims Left atrial appendage (LAA) closure is considered an effective option in patients with non-valvular atrial fibrillation (NVAF) and contraindications to long-term oral anticoagulant (OAC) therapy. However, there are some concerns about safety of currently available devices. Our aim is to provide an initial assessment on feasibility and safety of the novel LAA closure Ultraseal device in patients with NVAF and contraindications to long-term OAC therapy. Methods Twenty-three consecutive patients with NVAF undergoing Ultraseal device implantation between July 2016 and February 2018 at two institutions were included. All patients performed transesophageal echocardiography and computed tomography angiography prior to LAA closure. Results Procedural success was achieved in all patients except two who experienced incorrect device deployment with incomplete LAA closure. Procedure duration halved from first to last procedure performed. The only periprocedural adverse events observed were a myocardial infarction and an in-hospital death due pneumonia. At mean follow-up (166 ± 80 days) all other patients were alive and free from major bleedings and ischaemic strokes. Conclusions Our results suggest that the Ultraseal device is a feasible option for LAA occlusion. Notably, the learning curve in this registry was fast, paralleled by extremely low complication rates. These results should be considered hypothesis generating and larger studies are mandatory.

Background: Radial artery occlusion (RAO) is a thrombotic complication of transradial catheterization that can lead to permanent occlusion of the radial artery. Sheath-vessel diameter ratio, postprocedure compression time, occlusive hemostasis, and insufficient anticoagulation are all predictors of RAO. However, excessive anticoagulation can lead to longer time to achieve complete hemostasis and less patent hemostasis rate. This study was designed to assess the relationship among residual anticoagulation at the end of a percutaneous coronary procedure and the risk of RAO. Methods: Eight hundred thirty-seven patients undergoing transradial catheterization were enrolled. Activated clotting time (ACT) was measured before sheath removal. Patients were divided into 3 groups according to ACT values (ACT 250 s), patent hemostasis with reverse Barbeau test was attempted in all patients, and compression device removed as soon as possible. Within 24 hours, patency of radial artery was checked by Doppler using reverse Barbeau technique. Results: Incidence of RAO was higher for the extreme ACT values. Patent hemostasis were less frequently obtained and time to hemostasis significantly longer for increasing ACT values ( P =0.004 for trend and P =0.001) while adjusted probability for RAO confirmed U-shaped relationship with ACT values. Conclusions: The level of anticoagulation is strongly related to incidence of RAO and should be measured objectively by ACT. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT02762344.

More than 2 decades ago, para-Hisian pacing was introduced to assess the pattern of retrograde conduction during electrophysiological studies. Although there is no ideal maneuver for every patient and condition, para-Hisian pacing is a valuable and handy strategy to differentiate between retrograde conduction over the atrioventricular node and the accessory pathways. The dynamic behavior of para-Hisian pacing, in a region with unique anatomical features, can produce various activation patterns and intriguing electrophysiological phenomena. Although the demonstration of a retrograde nodal activation pattern during para-Hisian pacing does not rule out the presence of an accessory pathway, evidence of retrograde conduction over an accessory pathway does not prove its active role in the culprit tachycardia. Multipolar His bundle recordings, detailed atrial mapping, and recognition of the truly captured structures and the impact of temporal changes of autonomic tone or pacing rates, are essential keys for accurate interpretation of this maneuver that may ultimately guide judicious catheter ablation of the arrhythmic substrate. This review aims to summarize the practical usefulness and potential pitfalls of the para-Hisian pacing maneuver, focusing on the interpretation of electrocardiograms and intracardiac recordings.

Junctional and AV nodal reentrant tachycardia share common electrocardiographic features, but they differ in their management and outcomes after catheter ablation. This case concerns a 60-year-old female who presented with recurrent episodes of a relatively slow, regular supraventricular arrhythmia. Electrocardiographic features of the arrhythmia were discordant regarding its underlying mechanism. However, careful analysis of 12-lead electrocardiograms, with focus on the effect of spontaneous premature beats, pointed out the arrhythmia etiology. Electrophysiological study and pacing maneuvers defined the arrhythmic substrate that was successfully treated by catheter ablation.

The subcutaneous implantable cardioverter defibrillator (S-ICD) is a valuable alternative to the conventional trans-venous ICD (TV-ICD) for the prevention of sudden cardiac death (SCD). Prospective registries showed that the S-ICD is safe and effective in treating ventricular tachyarrhythmias in high-risk patients without pacing indications. While in earlier studies patients implanted with S-ICDs were young and mostly affected by channelopathies, contemporary S-ICD cohorts include patients with severely impaired left ventricular function and significant comorbidities. This review focuses on S-ICD evidence-based use and highlights current gaps between guidelines recommendations and real-world clinical practice.

Coronary artery disease and aortic stenosis frequently coexist. The pathophysiology of both conditions is similar where atherosclerosis is the hallmark feature. Risk factors for aortic stenosis are also similar for coronary artery disease. The standard therapy in the past decades has been coronary artery bypass grafting and aortic valve replacement; however, with the introduction of transcatheter aortic valve implantation their management is probably going to shift towards a percutaneous strategy.

Background: There is controversy about the outcome of patients with acute myocarditis (AM), and data are lacking on how patients admitted with suspected AM are managed. We report characteristics, in-hospital management, and long-term outcome of patients with AM based on a retrospective multicenter registry from 19 Italian hospitals. Methods: A total of 684 patients with suspected AM and recent onset of symptoms (70 years of age and those >50 years of age without coronary angiography were excluded. The final study population comprised 443 patients (median age, 34 years; 19.4% female) with AM diagnosed by either endomyocardial biopsy or increased troponin plus edema and late gadolinium enhancement at cardiac magnetic resonance. Results: At presentation, 118 patients (26.6%) had left ventricular ejection fraction P P P =0.18). After a median time of 196 days, 200 patients had follow-up cardiac magnetic resonance, and 8 of 55 (14.5%) with complications at presentation had left ventricular ejection fraction Conclusions: In this contemporary study, overall serious adverse events after AM were lower than previously reported. However, patients with left ventricular ejection fraction

Angiosarcoma, a rare malignant primary cardiac tumour, shows extremely aggressive growth and poor prognosis. Few small population studies are available; therefore, clinical cases add important information to its knowledge. We report a rare case of aggressive angiosarcoma involving both caval veins. A 60-year-old man sought assistance to the emergency department because of chest pain and …