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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.

We present the case of a 56-year-old man affected by heart failure (HF) with reduced ejection fraction (HFrEF) and permanent atrial fibrillation, who previously underwent a CRT-D implant with left ventricular stimulation delivered by an epicardial pacing lead due to an unfavourable CS anatomy. We upgraded this system to left bundle branch pacing. The LBB lead was connected to the atrial plug in of the device in order to deliver multisite left ventricular pacing. The device was programmed in DDDR mode and the delay between the two LV pacing sites was chosen (modifying atrioventricular delay) after multimodality assessment (both electrocardiographic and echocardiographic) to obtain the best resynchronization and systolic performance.

Background Cardiac-MRI is the gold-standard non-invasive imaging modality to evaluate cardiac masses. Case Summary A 58-year-old man underwent surgical excision of a sacral chordoma. At the time of surgery, there was neoplastic infiltration of bones and soft tissues (immunophenotype: CKMNF116+, EMA+, S100 +/-) and radiotherapy was performed. The patient underwent superior-lobectomy of the left lung six years later because of pulmonary metastasis of the chordoma. Nine years later, the patient was asymptomatic and referred to us for Cardiac-MRI because a suspected cardiac mass at echocardiography. At Cardiac-MRI, we found normal bi-ventricular volumes, wall thickness, wall motion and ejection fraction. Atria, heart valves and pericardium were normal too. In ssFP-cine images, an oval, hypointense mass with sharp margins (26×18 mm) was evident in the context of the posteromedial papillary muscle (Panel, A-B). Compared to the myocardium, the lesion was hyperintense at T2-weighted (Panel, C), native T1-(Panel, D) and T2-mapping sequences (Panel, E). After contrast injection, there was no increased mass signal at first-pass perfusion (Panel, F), whereas the late sequences revealed a soft, heterogeneous enhancement of the central core of the lesion, and a bright peripheral rim (Panel, G-H). These findings were interpreted as a cardiac metastasis of the chordoma and systematic treatment with imatinib was started. Discussion Six cases of cardiac metastasis secondary to chordoma were previously described. The present case is the first to provide advanced, multiparametric Cardiac-MRI assessment and represents the first description of a location within the context of a papillary muscle. Cardiac-MRI was essential to identify cardiac metastasis, leading patient's management and targeting appropriate treatment.

Aims Left atrial appendage (LAA) occlusion with first generation Ultraseal device (Cardia, Eagan, Minnesota) has been shown to be a feasible therapeutic option in patients with non-valvular atrial fibrillation (NVAF). However, there is a paucity of data regarding the novel Ultraseal device. To assess feasibility and safety of second generation LAA closure Ultraseal device in patients with NVAF. Methods and results All patients with NVAF undergoing second generation Ultraseal device implantation between February 2018 and September 2020 were included in a multicentre international registry. Periprocedural and post-discharge events were collected up to 6-months follow-up. Primary efficacy endpoints of interest were device success and technical success; primary safety endpoint was in-hospital major adverse events occurrence. A total of 52 patients were included: mean age 75 ± 8, 69.2% male, mean HAS-BLED 3 ± 1. The device was successfully implanted in all patients. Technical success was achieved in 51 patients (98.0%). In-hospital major adverse event occurred in three patients (5.8%). The incidence of 6-months all-cause death and major bleeding was 11.6% and 2.1%, respectively. No strokes, transient ischaemic attacks, systemic embolisms or device embolization were reported. Conclusions Second generation Ultraseal Device implantation was associated with high procedural success rate and a low incidence of peri-procedural complications. Larger studies with longer follow-up are warranted to evaluate the safety and the efficacy of this device, especially at medium and long term follow-up.

Aims Left ventricle outflow tract (LVOT) obstruction is a feared complication of transcatheter mitral valve replacement (TMVR) procedures. Multimodal imaging evaluation is the key to identify at-risk patient and select the best management. Methods and results An 83-year-old woman with a history of mitral valve replacement with a 27-mm Carpentier-Edwards bioprosthesis (Edwards Lifesciences, Irvine, CA) for rheumatic heart disease was admitted to our department complaining worsening effort dyspnoea. Clinical evaluation revealed a grade 3/6 holosystolic murmur. She underwent combined transthoracic and transesophageal echocardiography (TEE) which demonstrated mitral bioprosthesis degeneration leading to severe stenosis (mean gradient = 13 mmHg, PHT-derived area = 0.9 cm2) and moderate regurgitation, with preserved biventricular function, and severe pulmonary hypertension (pulmonary artery systolic pression = 65 mmHg). The patient presented a high estimated risk for redo-surgery (Society of Thoracic Surgeons score estimated mortality: 6%) due to her complex medical history, including advanced chronic kidney disease and permanent atrial fibrillation; therefore, she underwent evaluation for valve-in-valve TMVR. Cardiac computed tomography (CT) revealed bioprosthetic anterior leaflet in tight contact with the septum in systole; predicted neo-LVOT using virtual 26-mm Sapien S3 (Edwards Lifesciences, Irvine, CA) valve was 150 mm2, conferring a high risk of fixed LVOTO. A 26-mm Sapien S3 valve was selected based on CT derived surgical valve ID of 24 mm. After Heart Team discussion, we performed a modified LAMPOON technique to achieve tip-to-base laceration of the bioprosthetic leaflet beside the LVOT, in order to prevent LVOT obstruction. Briefly, after transseptal puncture through right femoral vein access (16 Fr), we crossed the mitral prosthesis using a balloon wedge end-hole catheter, through which we advanced a 300 cm 0.014-inch wire into the ascending aorta, where it was snared from left arterial femoral access (8 Fr) and covered by a micro-catheter. The wire was previously kinked mid-shaft to form a ‘flying-V’ that was focally denuded and positioned at the target bioprosthetic leaflet’s tip using TEE and fluoroscopy. The guidewire was pulled toward the valve ring and electrified at 70 W with continuous 5% dextrose flush until adequate tip-to-base leaflet laceration. Thereafter, we successfully implant a 26-mm Sapien S3 valve. Maximal LVOT gradient post implant was 5 mmHg. The patient was discharged on post-operative day two and she recovered well, reporting functional and symptomatic improvement at 6-month follow-up. Conclusions our case highlights the importance of multimodality imaging and careful procedural planning to succeed even in complex valve-in-valve TMVR procedures. Transcatheter electrosurgery is an emerging tool for percutaneous structural heart interventions.

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.

Increased sizes and dysfunction of the left atrium have been related to adverse outcomes. 3D-echocardiography is more accurate than 2D-echocardiography in estimating LA volumes and ejection fraction. However, the use of 3DE for LA analysis is limited by the absence of established reference values. We performed a systematic review and meta-analysis to provide reference ranges of LA maximum and minimum volumes indexed for body surface area (LAVi max and LAVi min, respectively), and LA-EF assessed by 3DE in healthy adults. Data search was conducted from inception through September 15, 2021, using the following Medical Subject Heading terms: left atrial/atrium, three-dimensional/3D echocardiography. The study protocol was registered in the PROSPERO database (CRD42021252428). 15 studies including 4,226 healthy adults (51% males) and reporting 3DE values of LAVi max, LAVi min and LA-EF were selected. LAVi max, LAVi min and LA-EF mean and reference values were equal to 25.18 ml/m

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.