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Introduction Apical Hypertrophic Cardiomyopathy (ApHCM) characterized by persistent diastolic apical contraction results in dynamic apical small-vessel obstruction with microvascular ischemia. Of note, endomyocardial fibrosis (EMF) and calcification are described only in few patients. Case presentation We report 5 cases of ApHCM with apical intramyocardial calcification. They presented characteristic ECG pattern and fibrosis at echocardiogram. All patients presented a preserved ejection fraction (EF), except for one patient with mild reduced EF. Global longitudinal strain was reduced in 3 patients. Diastolic dysfunction was evidenced in 3 patients. Right ventricle involvement was detected in one patient only.On cardiac magnetic resonance, a superficial hypo-intense component, compatible with calcium and a deep layer featured by late gadolinium enhancement (LGE) related to fibrotic tissue, were revealed. LGE was present in all of patients in the apex. One patient presented an apical aneurysm, with high ESC-SCD risk score and ICD implantation. Conclusion EMF pathologic hallmarks were the endocardium and myocardium scarring, evolving to dystrophic calcification. In clinical practice, only a minority of ApHCM patients develops EMF and calcifications. Our clinical series is the largest one in literature. Analyzing patients’ history, a microvascular inflammatory trigger was evident in all of them, particularly severe chronic kidney disease, diabetes, high degree obesity, malaria infection, peripheral microangiopathy and form of thalassemia. This series could demonstrate the pathophysiological relation between apical fibrosis, calcification and microvascular ischemia due to hypertrophy and inflammatory conditions coexistence. A broader case series could evaluate any correlation with their long-term outcome and management strategies.

Chronic myeloid leukemia is a rare myeloproliferative disease, characterized by a chromosomal translocation detected in 95% of cases, defined as "Philadelphia chromosome", encoding for the BCR-ABL fusion protein with continuous activation of the tyrosine kinase domain. Over the last 20 years, treatment has been revolutionized by the use of BCR-ABL tyrosine kinase inhibitors (TKI). Imatinib is the first TKI approved with a good cardiovascular safety profile, while some second-generation (nilotinib and dasatinib) and third-generation (ponatinib) drugs, developed to overcame drug resistance, can be associated with cardiovascular adverse events. The major adverse effect of dasatinib is pulmonary hypertension, reversible after treatment discontinuation. Conversely, nilotinib or ponatinib assumption is associated with a higher incidence of ischemic events, including coronary artery disease, cerebral stroke and peripheral arterial disease. Therefore, the management of patients receiving TKI therapy should include an integrated multidisciplinary evaluation and follow-up, involving highly specialized figures such as a cardiologist, hematologist and/or oncologist and the application of dedicated pathways, in order to prevent the onset or manage cardiovascular complications associated with these drugs.

Aims Although right ventricular (RV) systolic dysfunction seems to be associated with adverse outcome after transcatheter edge-to-edge mitral valve repair (TEER) with the MitraClip system, the prognostic value of RV free wall longitudinal strain (RVFWLS) in this setting has not been yet investigated. The aim of this study is to evaluate RVFWLS as predictor of outcome in patients with severe or moderate-to-severe functional MR undergoing TEER and its prognostic role compared with tricuspid annular plane systolic excursion (TAPSE). Methods and results 102 patients [73 (IQR: 66.8–77.0) years, 73 males (71.6%)] were retrospectively selected from March 2012 to February 2021. Echocardiograms were performed by using General Electric machines. RVFWLS was assessed through RV modified apical 4-chamber view, setting the region of interest to minimum size. Values of RVFWLS > −20% were recognized as abnormal. We considered a composite endpoint including rehospitalization for heart failure (HF) and overall death as primary outcome. Secondary outcomes were overall death, cardiac death and rehospitalization for HF. All patients were assessed at the longest available follow-up [median 22.1 (IQR: 9.7–49.3) months]. Baseline clinical and echocardiographic characteristics are listed in Table 1. Primary outcome was found in 60 (58.8%) patients, while secondary outcomes, i.e. overall death, cardiac death and rehospitalization for HF, were found respectively in 50 (50.0%), 31 (30.4%), and 36 (35.3%) patients. Mean TAPSE was 16.7 ± 4.0 mm and mean RVFWLS was −16.9 ± 6.0%. At univariable analysis both TAPSE (HR: 0.907, CI: 0.848–0.970, P-value: 0.004) and RVFWLS (HR: 0.937, CI: 0.897–0.979, P-value: 0.004) were significantly associated with the primary outcome. Kaplan–Meier survival curves showed that patients with TAPSE −20% also showed a lower survival free from the composite outcome compared with patients with RVFWLS ≤ −20% (Log Rank 0.004). Among patients with preserved RV systolic longitudinal function as indicated by TAPSE ≥ 17 mm, subjects with RVFWLS > −20% had a significantly higher incidence of the composite outcome compare with those with RVFWLS ≤ −20% (Log-Rank = 0.008). Conversely, no difference was found among patients with TAPSE Conclusions RV dysfunction assessed either by TAPSE and RVFWLS is associated with poorer outcome in patients with severe or moderate-to-severe functional MR undergoing TEER. Compared with TAPSE, RVFWLS seems to be superior in identifying patients at higher risk of adverse events during follow-up. Our data encourage the use of this speckle tracking-derived echocardiographic parameter in routinely evaluation of patients with functional MR candidate for TEER.