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Background Since 2014, European Guidelines have recommended using a novel clinical risk prediction model, called the HCM Risk-SCD, to identify which patients with hypertrophic cardiomyopathy (HCM) who are at higher risk for sudden cardiac death (SCD) and would benefit most from having a prophylactic cardioverter defibrillator (ICD) implantable. In recent years, evidence has called into question the effectiveness of this model in patient selection. Material and Methods Data from consecutive patients with HCM and ICD who were followed at our Cardiology Department from January 2000 to January 2022 were retrospectively collected. Results Among 702 HCM patients, 52 (7%) received an ICD (female, 31%; mean age at implantation, 49 ± 20; S-ICD, 13%; single-chamber ICD, 50%). Of them, 7 (13%) patients were implanted for secondary prevention while 45 (87%) for primary prevention. Out of the 45 primary prevention patients, 7 (16%) experienced 8 appropriate shocks (AS) while 10 (22%) had 15 inappropriate shocks (IS), during a mean follow up of 8 ± 5 years. Overall, mean HCM Risk-SCD score was 6.8 ± 4.7. Patients with AS showed a non-significant higher risk score when compared to non-AS subjects (7.1 ± 5.4 vs 4.8 ± 5.1, p=0.27). By stratifying study population according to pre-established and clinically adopted HCM Risk-SCD score cut-off (such as < 4 vs. > or = 4), we failed to highlight any significant difference in term of AS between groups. Indeed, out of 10 (22%) patients with HCM Risk-SCD score < 4, 3 (7%) experienced AS, while among 35 (78%) patients with HCM Risk-SCD score > or = 4, 4 (9%) had AS (30% vs. 11%, p=0.17). Conclusions The HCM Risk-SCD score does not seem to be able to predict the occurrence of life-threatening ventricular arrhythmias and AS in HCM patients. Our data highlight the need to elaborate a new score in order to improve SCD risk stratification and better select patients for ICD implantation.

A 76–year–old Caucasian woman presented to our hospital with progressive exertional fatigue and weight–loss over 2 months.Her past medical history revealed a recent local–hospital admission because of atrioventricular third–degree block, requiring permanent pacemaker implan­tation. At presentation her cardiovascular exam was normal.Laboratory studies revealed elevated LDH and PCR; electrolytes, cardiac biomarkers and complete blood count were normal. ECG demonstrated AF and ventricular stimulation.Transtoracich echocardiography showed LV hypertrophy in the basal segments, with normal ejection fraction and a reduced LV–GLS with a relative apical sparing pattern.There was a large, well–circumscribed mass arising from the right atrium, with a contextual moderate pericardial effusion (Fig1).Vasculitic, thrombophilic and infectious work–up were negative.The patient was immunocompetent. CMR was proposed but could not be performed due to patient claustrophobia. ECG gated cardiac CT was carried out for a better differential diagnosis of the mass, as well as to look for possible embolization or primary tumours, in the hypothesis of cardiac metastases. A CT total body showed a large ill–defined mass invading the interatrial and interventricular septum with projections into the left ventricle. Not enlarged lymph nodes were detected with neither hepatosplenomegaly nor abdominal lymphadenopathy. A total body 18FDG PET revealed significant tracer uptake in the heart and pericardium without extracardiac uptake, consistent with a malignant cardiac mass (Fig2).LV endomyocardial biopsy was attempted: pathology revealed large B–cell lymphoma expressing CD20, Bcl–2, Bcl–6, Mum–1, C–myc and with a high Ki67 proliferation rate. The patient underwent six cycles of chemotherapy(R–POCH).Echocardiography imaging after the second cycle showed significant reduction in myocardial wall thickness and concomitant improvement in LV–GLS. Repeated PET–CT scans 6 months post–chemotherapy showed complete remission with no evidence of FDG–avid lymphoma (Fig3). Primary cardiac lymphoma (PCL) is an extranodal non–Hodgkin’s B–cell lymphoma exclusively located in the heart or pericardium, accounting for 1–2% of primary cardiac tumours. PCL often presents with non–specific clinical symptoms making the diagnosis challenging. Multimodality approach and myocardial biopsy are essential in the diagnostic work–up. Early diagnosis and treatment are crucial since a good response to chemotherapy is expected.

Background Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular (LV) stiffness, leading to raised ventricular filling pressures and restrictive diastolic dysfunction (RDD), in itself independently associated with an increased risk of a clinical deterioration. We assessed whether echocardiography evaluation of diastolic function in HCM could yield prognostic value and be useful in HCM risk stratification. Methods We retrospectively reviewed clinical and imaging (echocardiography and cardiovascular magnetic resonance, CMR) data from 361 consecutive patients from two HCM Italian referral centers (Mauriziano Hospital, Turin; University Hospital of Trieste, Trieste). Among them, 28 patients were classified after Doppler echocardiography examination as restrictive (E/A > 1.5, DecT E < 140 ms, E/e’ > 10). The primary endpoint was a large cardiovascular composite consisting of sudden cardiac death (SCD), major ventricular arrythmias (MVAs), heart transplant (HT) and hospitalization for heart failure (HF). Other secondary endpoints were investigated as follows: 1) a secondary arrhythmic endpoint consisting of SCD and MVAs, 2) a key secondary composite of HF, HT, non–SCD. A Bayesian Model Averaging (BMA) was then performed to overcome the bias determined by the limited number of observations. Results Over a mean follow up of 7 ± 3 years, 61 (17%) patients experienced at least one primary composite endpoint event. Of them, 10 were identified with a restrictive diastolic pattern. When BMA was performed, late gadolinium enhancement (LGE) presence at CMR and RDD were significantly associated with the primary composite outcome (respectively, PI = 100, HR = 3.55, pd = 1.00; PI = 74.5, HR = 3.33, pd = 0.99). Left atrium (LA) diameter was not strongly associated with the arrhythmic endpoint, whereas it was significantly associated with the key secondary composite outcome, as well as with increased filling pressures (E/e’) (respectively, PI = 94.5, HR = 1.10, pd = 1.00; PI = 86.8, HR = 1.08, pd = 0.99). Conclusions Restrictive diastolic dysfunction is an independent predictor of poor prognosis in early stages of HCM in absence of systolic dysfunction. In particular, RDD predicted outcomes related to heart failure better then LA diameter and E/e’.