Search

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease, and several studies have suggested possible early RV involvement. Aim of the study was to evaluate the 3D echo parameters of the right ventricle (RV) and the metabolomic profile to correlate both with SLE severity. Forty SLE patients, free of cardiovascular disease, were enrolled and the following 3D parameters were evaluated: the RV ejection fraction (RV-EF), longitudinal strain of the interventricular septum (Septal LS), longitudinal strain of the free wall (Free-LS) and the fractional area change (FAC). In addition, a metabolomic analysis was performed. Direct correlations were observed between TAPSE values and the RV 3D parameters. Then, when splitting the population according to the SDI value, it was found that patients with higher cumulative damage (≥3) had significantly lower FAC, RV-EF, Septal LS, and Free-LS values; the latter three parameters showed a significant correlation with the metabolic profile of the patients. Furthermore, the division based on SDI values identified different metabolic profiles related to the degree of RV dysfunction. The RV dysfunction induced by the chronic inflammatory state present in SLE can be identified early by 3D echocardiography. Its severity seems to be related to systemic organ damage and the results associated with a specific metabolic fingerprint constituted by 2,4-dihydroxybutyric acid, 3,4-dihydroxybutyric acid, citric acid, glucose, glutamine, glycine, linoleic acid, oleic acid, phosphate, urea, and valine.

Introduction Apical hypertrophic cardiomyopathy (ApHCM) compared with normal HCM is less frequent, it's more associated with atrial fibrillation (AF) and it has different sudden cardiac death (SCD) risk factors. Authoritative recommendations and guidelines on diagnosis, family screening and especially patient risk stratification don't exist. Taking decisions on ICD implantation can sometimes be challenging. Patient presentation A 66-year-old male with a history of arterial hypertension was admitted to our cardiology department because during the last night, while he was driving his car, he would have lost consciousness having a car accident. He rapidly felt well even though he doesn't remember what happened a few minutes shortly before and immediately after the event. No witnesses. Due to traumatic chest pain he went to emergency department where an electrocardiogram was performed with evidence of negative T waves and ST-segment depression on V3-V6 leads. Diagnosis and management Physical examination didn't show anything relevant. On heart ultrasound he had normal left ventricle ejection fraction with apical hypertrophy (maximum thickness 18 mm). He underwent coronary angiography which showed normal coronaries. During hospitalization a cardiac magnetic resonance was performed; it described apical hypertrophy with some small areas of intra-myocardium delayed enhancement in apex wall. During hospitalizations no arrhythmic alarms were detected. Discussion Pharmacological and electrical treatment options are based on classic HCM aiming to reduce symptoms, incidence of atrial fibrillation, ventricular arrhythmias, and sudden death. Concerning ApHCM, left ventricular outflow tract obstruction is usually absent and therefore pharmacological therapeutic benefits may be lower than in classic HCM. Regarding sudden death prevention, we evaluate ICD implantation according to ESC 5-y HCM SCD risk score which doesn't include potential risk markers for SCD in ApHCM such as apical aneurysm, midcavity gradient and midventricular obstruction with SCD risk underestimation. By the way it's still the most used score being aware that could lead to ICD underutilization. In our case, according to HCM risk score 1.74%, negative family screening, no arrhythmic events for 15 days hospitalization, no previous transient loss of consciousness (TLOC) and dynamics of the car accident, we decided to follow-up the patients in our cardiomyopathies outpatient clinic explaining the TLOC with a sleep disorder.

Transcatheter aortic valve implantation (TAVI) is an established treatment of severe symptomatic aortic valve stenosis and is an effective and less invasive treatment option compared with conventional surgical aortic valve replacement (SAVR) for patients considered inoperable or at high surgical risk. Thromboembolic and bleeding complications negatively impact recovery and survival after TAVI. This case describes one of the few reported TAVI-associated myocardial infarction due to thrombosis of the prosthetic aortic valve. A 82-years-old woman, suffering from severe symptomatic aortic stenosis, underwent TAVI. Before the procedure, coronary artery disease was excluded by invasive coronary angiography. Since the time of the discharge the patient didn't assume any therapy with aspirin or other antithrombotic drugs. A month after TAVI she complained persistent epigastric pain and was admitted to our department for anterior ST elevation myocardial infarction. Coronary angiography highlighted a monovascular coronary artery disease with thrombotic occlusion of the first diagonal and of the distal left anterior descending coronary artery. In consideration of the thrombotic load thyrofiban was started and it was performed a chest contrast CT which confirmed the diagnostic suspicion of bioprosthetic valve thrombosis. She was first treated with unfractionated heparin (UFH) and then oral anticoagulant therapy with warfarin was started. Echocardiograms (echo) showed a severely reduced systolic function (EF 25%) and high trans-prosthetic gradients (mean transprosthetic pressure gradient of 30 mmHg). On the 8th day after hospitalization she complained chest pain with evidence of moderate pericardial effusion. Epistenocardica pericarditis was therefore diagnosed and the patient started acetylsalicylic acid therapy with progressive clinical, instrumental and laboratory improvement. After 10 days of anticoagulation therapy, she underwent a transesophageal echocardiogram which turned out negative for bioprosthetic valve thrombosis. The predischarge transthoracic echocardiography showed severely reduced systolic function (EF 30%) with a mean transprothesic gradient of 12 mmHg; the circumferential pericardial effusion was mild-moderate but not haemodynamically significative. Therapy at discharged included warfarin and aspirin. Ten days after an echo showed a reduction of the pericardial effusion, an improved systolic function (EF 42%) and mean transprothesic gradient not changed significantly since discharge. Prosthetic valve thrombosis has been reported in up to 40% of patients after TAVI and this case hightlights the importance of antithrombotic therapy after this procedure. Therapeutic anticoagulation appears to be the only non-invasive treatment for resolving valve thrombosis but it is not clear how long this therapy should last for the prevention of prosthetic valve thrombosis.

Introduction The Twiddler's syndrome is a rare condition of malfunctioning of a cardiovascular implantable elettronic device (CIED) due to the rotation of the device around its own long axis within its pocket. This brings to the leads wrapping around the device case and consequently dislocating, resulting in a deficit in sensing and pacing. Case Report We present the case of an 82-year-old woman, affect by atrial and conduction tissue disease for which was implanted a dual-chamber PaceMaker (PM). She arrived at the emergency department for asthenia and a traumatic syncopal episode, preceded by intense general malaise, nausea and vomiting. At EKG findings of electroinduced atrial rhythm, electroinduced ventricular rhythm with loss of capture and emergence of spontaneous rhythm, with subsequent resumption of capture. At device telemetry check, rise of ventricular and atrial pacing thresholds together with sporadic ventricular capture losses. Chest×Ray showed entanglement of leads around the PM case and retraction of the ventricular catheter. Therefore, the patient was admitted to the cardiology department, and the surgical pocket was revised with case extraction and leads debridement with subsequent optimization of their position. The next day, following device telemetry check, which showed good electrical parameters, and the follow-up chest X-Ray, which was normal, the patient was discharged. Conclusion All physicians, particularly cardiologists, must pay close attention to the evaluation of patients with CIEDs. First of all, meticulous analysis of the EKG must be performed, which is capable of showing loss of capture and abnormal QRS morphologies due to cardiac pacing at abnormal locations. In addition, it is necessary to perform the telemetry check of the device, infact findings of sensing deficits and/or elevation of pacing thresholds indicate device malfunctioning, which is not necessarily due to leads injuries. Displacement of the leads can be easily detected with a chest X-ray, always remembering to rule out the presence of Twiddler syndrome, however rare it may be.

Arrhythmogenic dysplasia is a disease of genetic aetiology characterised by the progressive replacement of functioning myocardium by fibro-adipose tissue with high arrhythmogenic potential, as well as by morpho-functional impairment. Molecular genetic studies have revealed a deficiency of demosomal proteins such as placoglobin, desmoglein, desmoplazine, placophylline and desmocollin. It usually begins at a young age and in sports subjects. It mainly affects the right ventricle, although over time it can also affect the left sections due to a biventricular alteration of the heart pump. Forms with major left-sided involvement have been described in the literature, but these are rare. A 73-year-old man, a sportsman with over 40 marathons, presented with an episode of sustained VT during exercise in 2011. Admitted to our hospital, he was diagnosed with arrhythmogenic cardiomyopathy and was subsequently implanted with an ICD-DR mod.Teligen 100F. After 3 months, however, there was a new episode of VT, so it was decided to subject the patient to transcatheter ablation with radiofrequency, using an endocardial approach, which proved ineffective. Two months later, a new attempt at radioablation with an endocardial approach. Due to the persistence of arrhythmic symptoms, sustained VT episodes, in February 2012 it was decided to proceed with a third ablation, but adopting an endo-epicardial approach, with apparent resolution of the symptomatology. After severals episodes of atrial fibrillation, he underwent a new ablation with pulmonary vein isolation and ablation line at the level of the cavo-tricuspid isthmus in August 2020. On echocardiogram, the left ventricle had normal dimensions with upper limit thicknesses, diffuse parietal hypokinesis with markedly reduced EF (30%), dilated right ventricle (DTD 48 mm) with parietal hypertrabeculature and free wall hypokinesis (TAPSE 1.4) and dilated right atrium. In May 2020 new relapse of atrial fibrillation successfully cardioverted, maintenance therapy set up with sotalol. In the literature, mainly forms of arrhythmogenic dysplasia in youth (30 years) have been described, whereas older forms appear to be rare. A search of the Medline database (National Library of Medicine, Bethesda, MD) found 5 cases of ARVD among the elderly; all these patients had died and were diagnosed by histology. The diagnosis is multi-parametric and the main aim of therapy is the prevention of sudden cardiac death. Currently, ICD defibrillator implantation is the only effective therapy for the prevention of sudden death. New drug therapies, radiofrequency ablation and defibrillators are likely to have a favourable impact on the survival of these patients and make it not unlikely that elderly patients with ARVD will be present. The possibility of a late onset of the disease should also be suspected.

Introduction Papillary fibroelastomas (PFE) are reported to be the most common tumors of the heart valves and are a possible source of systemic embolism, including myocardial infarction. Case Report We report the case of a 67-year-old female with no background of cardiovascular disease, admitted to our hospital with worsening dyspnea and chest tightness over the prior 10 days. The ECG showed a sustained hemodynamically tolerated monomorphic ventricular tachycardia, successfully treated with antiarrhythmic therapy, and ECG in sinus rhythm revealed signs of previous anterior myocardial infarction. Transthoracic echocardiogram showed a severely depressed systolic function (LVEF 19%), akinesia and thinning of the anterior wall and of all middle-apical segments, complicated by left ventricular aneurysm and endoventricular thrombosis. In the ventricular side of right aortic coronary cusp a mobile, pedunculated mass of 12×8 mm was visualized. A subsequent TEE corroborated the suspicion of PFE. She underwent cardiac MRI which showed areas of transmural late enhancement in all akinetic segments. A coronary angiography revealed sub-occlusion of the mid-anterior descending artery with no angiographic evidence of coronary atherosclerosis elsewhere. In view of her reduced LVEF and history of VT, an ICD was implanted. She started oral anticoagulant therapy and complete resolution of the endoventricular thrombosis was evidenced. After several days of electrical instability, characterized by multiple episodes of ventricular tachycardia, the clinical course was characterized by progressive clinical and electrical improvement and she was discharged in good clinical conditions after four weeks. Due to the severe impairment of the systolic function, not susceptible to surgical remodeling and the high operative risk, no indication for surgical removal of the mass was made. Conclusion Aortic PFE carry a not negligible risk of acute coronary syndrome, electrical storm and sudden death. It should be considered as the cause of myocardial infarction, and not an incidental finding, especially in patients with no angiographic evidence of coronary atherosclerosis.

Stanford type A acute aortic dissection is a life-threatening cardiological emergency which occurs when an intimal tear in the ascending aorta creates a false lumen within the media refilled by pulsatile blood flow. Some investigators report the immediate mortality to be as high as 40%. Both congenital and acquired conditions can be the cause of an instability of the aortic wall that can lead to dissection. Diagnosis, which requires a high level of suspicion, is usually made with aortic angio-CT. The case we present involves a 62-years old man with a history of hypertension, parossistic atrial fibrillation and familiarity for type B aortic dissection. He called 911 complaining of sudden onset dorsal pain that then irradiated anteriorly to the chest and upwardly to the neck, associated with profuse sweating. An EKG performed at patient's home showed ST elevation in V1 meaningful of right ventricle infarction. Arrived at the Emergency Ward of our hospital arterial pressure was 80/50 mmHg with an HR of 44 bpm. Rapidly, because of the suspect of aortic dissection, an angio-TC was performed showing a type A aortic dissection starting from Valsalva sinuses and extending till common right iliac artery and external left iliac artery including left subclavian artery ostium. The left renal artery originated from false lumen, but patient had a so-called horseshoe kidney. For this reason, the patient was transferred immediately to the cardiac surgery room with indication for Bentall procedure (aortic valve plus ascending aorta replacement with home-made valved Dacron tubular prosthesis), aortic arch repair with supra-aortic vessels debranching and reimplantation and saphenous graft on right coronary artery. During surgery was evident that intimal tear was located immediately after left common carotid artery and right coronary sinus was involved in the dissection thus explaining why right ventricle free wall was visibly hypokinetic. Surgery went without complication and patient entered ICU with circulation supported by a low dose of epinephrine and norepinephrine; respiratory weaning took place in day 1 post-operation. The following days patient developed circumferential pericardial effusion which needed percutaneous subxiphoid drainage despite not being hemodynamically relevant. Echocardiography before discharge showed aortic valve prosthesis and ascending aorta prosthesis well positioned, bi-ventricular normal dimensions and function and organized residual pericardial effusion with fibrin strands. Stanford type A is the most common type of aortic dissection and requires immediate surgical treatment. The Bentall procedure is considered the gold standard for patients needing ascending aorta plus aortic valve replacement. Even though mortality rates remain high for Stanford type A acute aortic dissection, this case shows how is possible to survive from such a life-threatening condition if a prompt and correct diagnosis is made and thanks to flawless surgical operation.

Introduction Rituximab is a chemotherapeutic drug widely used for the treatment of oncologic and rheumatologic diseases. Its cardiotoxic profile has not yet been defined in detail; only a few isolated case reports delineate cardiovascular adverse reactions following its use. In the cases described in literature, the drug was used to treat neoplastic diseases, often in combination with other chemotherapeutic drugs. We present a clinical case of heart failure with reduced ventricular ejection fraction (HFrEF), developed following therapy with Rituximab and a single administration of Cyclophosphamide, in a patient affected by MPO-ANCA-related Microscopic Polyangiitis. Case Report 54-year-old man arrived at the emergency department for weight loss, fever, asthenia, and lower limbs oedema; laboratory findings where diagnostic for acute kidney injury (AKI). His past history was unremarkable. He was admitted to the nephrology department where he was diagnosed with MPO-ANCA-related microscopic polyangiitis. Echocardiography on admission showed normal left systolic ventricular function. He underwent diuretic therapy, intermittent hemodialysis, plasmapheresis and immunosuppressive therapy with steroids, Rituximab (two administration of 375 mg/m2 i.v. one week apart) and Cyclophosphamide (one administration of 300 mg i.v.). Following the achievement of good clinical and hemodynamic compensation, the patient was discharged. However, 5 days later, the patient came back to the hospital, prior to the follow-up that was scheduled for the continuation of immunosuppressive therapy, with symptoms of worsening dyspnea related to acute pulmonary edema. A transthoracic echo showed severe left ventricular systolic dysfunction for global hypokinesia. Cardiac MRI showed hypokinetic dilated cardiomyopathy with severely depressed systolic function (EF 30%) in the absence of lesions compatible with edema and myocardial fibrosis/necrosis. No coronary artery disease was detected with CT coronary angiogram. Given the absence of signs of ischemic cardiopathy or inflammatory related cardiomyopathy, we considered this new onset of HFrEF likely due to cardiotoxicity from chemotherapy drugs, which, in agreement with nephrology colleagues, were discontinued. The patient was started on heart failure medication with Bisoprolol 2,5 mg once daily and Ramipril 2,5 b.i.d., mineralocorticoid receptor antagonist and glifozines were not administered due to the renal injury. Given the HFrEF and numerous features of non-sustained ventricular tachycardia found on EKG monitoring during hospitalization, the patient was discharged with a wearable ICD. He was discharged after 3 weeks in stable clinical conditions with a follow-up appointment in a month. Conclusion Our clinical case provides further evidence regarding the cardiotoxic effect of Rituximab. In contrast with the other cases in literature, Rituximab was used for the treatment of rheumatologic and non-neoplastic pathology, without being combined with several other chemotherapeutics but only with a low-dose administration of Cyclophosphamide. In light of the above, it is worth mentioning that when chemotherapeutics are used, not only in oncology but also in rheumatology, even when their cardiotoxicity profile has not been defined in detail, as in the case of Rituximab, it is essential to use the utmost caution in assessing cardiac function before and after drug administration, so that early detection of cardiotoxicity will be possible.

A 53-old male with arterial hypertension, obesity (BMI 32) and gout presented to our Emergency department symptomatic for dyspnea, complaining about reduced functional capacity (NYHA III), gain of weight e increased abdominal circumference. In the days after we discovered a history of potus (2 liter of wine/day). At a first physical examination we confirmed clinical features of heart failure with pulmonary congestion and ascites. The EKG showed atrial fibrillation, 80 bpm. An echocardiogram showed severely reduced ejection fraction (EF 23%) with diffuse hypokinesia, increased left ventricular mass, deeply increased volumes, with only slightly increased thickness, a mild moderate diastolic dysfunction. Also, the right ventricular was hypokinetic and dilatated. During the hospitalization the patient underwent coronary angiography, which showed no coronary lesion, e cardiac RMN with no pathological features in both T1 and T2 weighted sequences. No increased uptake of contrast in LGE sequences was evidenced. We started diuretic therapy and optimized heart failure therapy with clinical improvement. The management of anticoagulant therapy was difficult because of hepatic dysmetabolic cirrhosis (Child-Pugh Score 8, class B) with contraindication for DOACs treatment. Moreover the patient performed a gastroscopy, which evidenced an ulcerative gastro-duodenitis and erosive gastritis confirming the temporary DOACs contraindication. The patient was discharged in hemodynamic stability, with wearable defibrillator and was taken in charge by a multidisciplinary team. After the normalization of hepatic function the anticoagulation was no longer forbidden so after three weeks we proceeded to successful electrical cardioversion. An echocardiogram was performed after 6 months with almost completely recovery of systolic function (EF 62%) and a slightly reduction of chamber dilatations. The patient denied alcohol consumption throughout the period. This case demonstrates that alcoholic cardiopathy if it's diagnosed at the beginning can be partially reversible. This case presents a challenging clinical setting due to the frequent comorbidities requiring a multidisciplinary approach. Achieving a significant patient compliance is also crucial for long term lifestyle changes.

Introduction Endobronchial ultrasound-guided transbronchial fine needle aspiration (EBUS-TBNA) is a minimally invasive diagnostic method to sample peritracheal and peribronchial le- sions under real-time ultrasound guidance. The most important use of TBNA is the diagnosis and staging of lung cancer and the evaluation of sarcoidosis. Although TBNA is considered generally safe, serious or even fatal complication may develop after the procedure. Here we present a case report of acute purulent myopericarditis complicated by cardiac tamponade occurred after EBUS-TBNA procedure. Case Report A 62-year-old man with preexisting hypertension and previous myocardial infarction and coronary angioplasty was admitted to our hospital for persistent, oppressive and worsening chest pain, which started a few days before admission. Of note, the patient underwent EBUS-TBNA 10 days before admission because he was found to have a tumor in the upper lobe of the left lung and enlargement of multiple mediastinal, right hilar, subcarinal and para-aortic lymph nodes. Two days after the procedure, one week before admission, he was treated with amoxicillin clavulanate and levofloxacin because of fever onset. At the ER, his vital signs showed a blood pressure of 80/60 mmHg and a heart rate of 115 bpm; O2 saturation on room-temperature air was 97%. The ECG at presentation showed concave ST segment elevation in anterolateral and inferior leads. The echocardiography revealed moderate circumferential pericardial effusion without clear tamponade signs. Emergency coronary angiography showed patent coronary arteries. The next morning, the patient developed persistent hypotension (BP 75/50 mmHg). A transthoracic echo showed moderate to large circumferential pericardial effusion with tamponade physiology. He therefore underwent subxiphoid pericardiotomy with drainage of 400 ml of frank pus. Cultures from pericardial fluid were negative for common germs. Cytology was negative for malignancy and acid-fast bacilli. Histological examination of the pericardial tissue was diagnostic for acute fibrinous-purulent pericarditis. The patient was started on broad spectrum antibiotics (piperacillin/tazobactam and ciprofloxacin) with subsequent decrease of inflammation markers and clinical improvement. Control contrast enhanced chest CT scan revealed persistency of a mediastinal pluri-concamerate fluid collection. After a collective discussion with cardiac surgeons, infectious disease specialists and oncologists, given the progressive clinical and instrumental improvement, we decided to discharge the patient with long term oral antibiotics plus colchicine and ibuprofen for pericarditis. Infectious disease and oncological follow up was planned before discharge. Conclusion Complications following TBNA are relatively low, typically around 1.2%-1.4%. To date, only few cases of purulent pericarditis secondary to EBUS-TBNA have been reported in literature. Our case report adds to the existing scant literature regarding cardiac infective bronchoscopic complications and emphasizes concern of weather antibiotic prophylaxis may be needed before respiratory tract invasive procedures. Further research is necessary to better understand the existence of risk factors that could increase the probability to develop such rare but potentially lethal complications.

Introduction Danon disease (DD) is a rare multi-systemic disorder firstly described by Danon et al. in 1981 and characterized by a triad consisting with hypertrophic cardiomyopathy, skeletal myopathy, and intellectual disability. It is an inherited dominant X-linked condition, due to mutations in the lysosome-associated membrane protein 2 (LAMP2) gene. Typically, males present a more severe phenotype with earlier onset and rapid heart failure progression with extracardiac manifestations; female patients manifest a milder phenotype with later onset and isolated cardiac involvement. Female carriers may develop dilated cardiomyopathy (DCM). In recent years, it has been described a severe arrhythmogenic phenotype, particularly in women, with a high risk of sudden cardiac death (SCD). Case presentation Case 1 is a 16 y.o. woman referred to our outpatient cardiomyopathies clinic, diagnosed with left ventricle (LV) hypertrophy on the basis of ECG and echocardiography, performed in 2016, in view of cutaneous nevus surgical exeresis. At echocardiographic evaluation we found an asymmetric LV hypertrophy with preserved LV ejection fraction (LVEF 65%). A cMRI was performed, showing mild LV thickening and left ventricular hypertrabeculation morphology, mainly in the LV lateral wall, but not all criteria for LV noncompaction (NC) were satisfied. There was no late gadolinium enhancement (LGE) accumulation. She underwent a genetic screening with inconclusive results, so we started a clinical outpatient follow-up showing no significant arrhythmias at ECG Holter and no limitation of physical activities. In 2021, when she was 21 yo, her father died suddenly and no autoptic examination was performed. A genetic exam was repeated, highlighting a LAMP2 gene mutation. In 2016 her 3 years older sister, Case 2, underwent a familiar screening. She had a long history of mild dyspnea occurring during exertion. Echocardiography showed asymmetric hypertrophy with marked thickening of infero-lateral wall (25 mm,) with normal systolic function (EF 70%). She underwent a cMRI, highlighting diffuse asymmetric hypertrophy (max 24 mm) with LGE in lateral wall and lower septum. First genetic test was nonconclusive but after her father's death another test was performed, demonstrating LAMP2 gene mutation. In 2021 a follow-up cMRI showed markedly reduced myocardial thickness of the apex, with a pattern of noncompaction myocardial. We found a LVEF at lower limits, depressed longitudinal function (MAPSE 8 mm) and manly severe progression of fibrotic degeneration. ECG Holter revealed premature ventricular complex, in absence of nonsustained ventricular tachycardia. Following the evolution of fibrosis, cardioverter defibrillator (ICD) implantation was performed. Case 1 was clinically stable during strict follow-up. Conclusion Cardiac magnetic resonance imaging (cMRI) may be useful in arrhythmic risk stratification, accurate systolic dysfunction evaluation, and proper timing and indication for ICD implantation.

Mitral valve prolapse (MVP) is the most common valvular heart disease. Although MVP is generally considered benign, it can be associated with important complications, including sudden cardiac death (SCD). MVP is often related to mitral annular disjunction (MAD), a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. It is associated with an increased risk of arrhythmias and is an integral component of the arrhythmic MVP (AMVP) complex. Here we present a case report of a patient with MAD, Barlow's syndrome with bileaflet mitral prolapse and a history of recurrent non sustained ventricular tachycardia (NSVT) who underwent subcutaneous cardioverter defibrillator (ICD) implant in primary prevention. A 49 year old woman, with a history of moderate mitral regurgitation and posterior leaflet prolapse, was sent to our hospital by her cardiologist for the evidence on holter ecg of several tracts of non sustained ventricular arrhythmias. Transesophageal echocardiogram revealed the presence of moderate mitral regurgitation, bileaflet prolapse (mainly of the redundant posterior leaflet), dilation of the left atrium and positive Pickelhaube sign, suggestive of MAD. After few days was performed cardiac magnetic resonance imaging with evidence of mitral annulus disjunction associated with bileaflet prolapse and moderate mitral regurgitation. Delayed gadolinium enhanced (DGE) signal was found in the posterior basal wall. During hospitalization, she presented daily episodes of high frequency (>180 bpm) NSVT with right bundle block (RBBB) morphology and several premature ventricular complex. It was therefore decided to discharge the patient with Life Vest and five months later, after asking second opinion to an Italian high specialization center, we decided to place a subcutaneous ICD in primary prevention for the high arrhythmic risk of the patient. Arrhythmic mitral valve prolapse (AMVP) is an estimated cause of sudden cardiac death. It is known that patients with MVP and MAD have a high arrhythmic risk but there aren't trials that demonstrate the benefits of primary ICD implantation. The recent “EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction “ affirms that can be reasonable, primary ICD implant, in patients with AMVP, 1 high risk features (VT (hemodynamically tolerated), NSVT, unexplained syncope) and 2 or more phenotypic risk features (—T-wave inversion in the inferior leads, repetitive documented polymorphic PVCs, MAD phenotype, redundant MV leaflet, enlarged left atrium or ejection fraction≤50%, LGE). For this reason, our patient underwent subcoutanues ICD implant. Therefore, we believe that further studies, to develop guidelines for risk stratification of patients with evident MVP, would be beneficial in reducing the incidence of potentially fatal events in these patients.

Background Traditional cardiovascular risk factors (RFs) and coronary artery disease (CAD) do not always run parallel. We investigated functional-metabolic correlations of CAD, RFs, or neither in the CAPIRE (Coronary Atherosclerosis in Outlier Subjects: Protective and Novel Individual Risk Factors Evaluation) 2 × 2 phenotypic observational study. Methods Two hundred and fortyone subjects were included based on RF burden, presence/absence of CAD (assessed by computed tomography angiography), age and sex. Participants displayed one of four phenotypes: CAD with ≥3 RFs, no-CAD with ≥3 RFs, CAD with ≤1 RF and no-CAD with ≤1 RF. Metabolites were identified by gas chromatography–mass spectrometry and pathways by metabolite set enrichment analysis. Results Characteristic patterns and specific pathways emerged for each phenotypic group: amino sugars for CAD/high-RF; urea cycle for no-CAD/high-RF; glutathione for CAD/low-RF; glycine and serine for no-CAD/low-RF. Presence of CAD correlated with ammonia recycling; absence of CAD with the transfer of acetyl groups into mitochondria; high-risk profile with alanine metabolism (all p The comparative case-control analyses showed a statistically significant difference for the two pathways of phenylalanine, tyrosine and tryptophan biosynthesis and phenylalanine metabolism in the CAD/Low-RF vs NoCAD/Low-RF comparison. Conclusions The present 2 × 2 observational study identified specific metabolic pathways for each of the four phenotypes, providing novel functional insights, particularly on CAD with low RF profiles and on the absence of CAD despite high-risk factor profiles.

Aims In breast cancer (BC) patients treated with anthracyclines-based therapies, we aim at assessing whether adjuvant drugs impact cardiac function differently and whether their cardiotoxicity has a regional pattern. Methods and results In a multicentre study, 146 BC patients (56 ± 11 years) were prospectively enrolled and divided into three groups according to the received treatments: AC/EC-Group (doxorubicin or epirubicin + cyclophosphamide), AC/EC/Tax-Group (AC/EC + taxanes), FEC/Tax-Group (fluorouracil + EC + taxanes). Fifty-six patients of the total cohort also received trastuzumab. Left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) were calculated before starting chemotherapy (T0), at 3 months (T3), at 6 (T6), and 12 months (T12). A ≥10% drop of EF, while remaining within the normal range, was reached at T6 in 25.3% of patients from the whole cohort with an early decrease only in FEC/Tax-Group (P = 0.04). A ≥15% GLS reduction was observed in many more (61.6%) patients. GLS decreased early both in the whole population (P Conclusions The decrease of GLS is more precocious and pronounced in BC patients who received FEC + taxanes. Cardiac function further worsens after 6 months of adjuvant trastuzumab. All LV segments are damaged, with the anterior septum and the apex showing the greatest impairments.