The concepts underlying hypertrophic cardiomyopathy (HCM) pathogenesis have evolved greatly over the last 60 years since the pioneering work of the British pathologist Donald Teare, presenting the autopsy findings of “asymmetric hypertrophy of the heart in young adults”. Advances in human genome analysis and cardiac imaging techniques have enriched our understanding of the complex architecture of the malady and shaped the way we perceive the illness continuum. Presently, HCM is acknowledged as “a disease of the sarcomere”, where the relationship between genotype and phenotype is not straightforward but subject to various genetic and nongenetic influences. The focus of this review is to discuss key aspects related to molecular mechanisms and imaging aspects that have prompted genotype–phenotype correlations, which will hopefully empower patient-tailored health interventions.

Several studies showed that right ventricular (RV) dysfunction is a powerful predictor in heart failure (HF). Advanced echocardiographic techniques such as speckle-tracking imaging and three-dimensional (3D) echocardiography proved to be accurate tools for RV assessment, but their clinical significance remains to be clarified. The aim of this study was to evaluate the role of two-dimensional (2D) RV strain and 3D ejection fraction (RVEF) in predicting adverse outcome in patients with non-ischemic dilated cardiomyopathy (DCM). We prospectively screened 81 patients with DCM and sinus rhythm, 50 of whom were enrolled and underwent comprehensive echocardiography, including RV strain and 3D RV volumetric assessment. Patients were followed for a composite endpoint of cardiac death, nonfatal cardiac arrest and acute worsening of HF requiring hospitalization. After a median follow-up of 16 months, 29 patients reached the primary endpoint. Patients with events had more impaired RV global longitudinal strain (− 10.5 ± 4.5% vs. − 14.3 ± 5.2%, p = 0.009), RV free wall longitudinal strain (− 12.9 ± 8.7% vs. − 17.5 ± 7.1%, p = 0.046) and 3D RVEF (38 ± 8% vs. 47 ± 9%, p = 0.001). By Cox proportional hazards multivariable analysis, RV global longitudinal strain and RVEF were independent predictors of outcome after adjustment for age and NYHA class. RVEF remained the only independent predictor of events after further correction for echocardiographic risk factors. By receiver-operating characteristic analysis, the optimal RVEF cut-off value for event prediction was 43.4% (area under the curve = 0.768, p = 0.001). Subjects with RVEF > 43.4% showed more favourable outcome compared to those with RVEF < 43.4% (log-rank test, p < 0.001). In conclusion, 3D RVEF is an independent predictor of major adverse cardiovascular events in patients with DCM.

Background: Right ventricular (RV) pulmonary artery coupling (RVPAC) is a predictor of outcome in left-sided heart failure (HF). Several echocardiographic estimates for RVPAC have been proposed. Aims:This study aimed to compare multiple non-invasive methods to calculate RVPAC and to assess its prognostic role in patients with dilated cardiomyopathy (DCM). Methods:We prospectively enrolled 60 stable patients with DCM. RVPAC was estimated using five methods: as the tricuspid annular plane excursion/pulmonary artery systolic pressure (PASP) ratio; as the RV global longitudinal strain/PASP ratio; as the RV free wall strain (RVFW-LS)/PASP ratio; as the three-dimensional (3D) RV ejection fraction (RVEF)/PASP ratio; and as the 3D RV stroke volume (SV)/end-systolic volume (ESV) ratio. Patients were followed for a mean period of 18 (9) months for the endpoint of HF rehospitalizations.Results: Twenty-nine patients (48%) reached the endpoint. All RVPAC estimates were more impaired in those patients reaching the endpoint (P <0.001 for all) and all predicted rehospitalizations in unadjusted analysis. RVFW-LS/PASP and RVEF/PASP remained independent predictors of events, after adjustment for clinical and echocardiographic confounders. Using cut-offs obtained from receiver operating characteristic (ROC) analysis, we found that patients with RVFW-LS/PASP >-0.40 and patients with RVEF/PASP <1.30 had a higher risk of HF rehospitalization (log-rank P = 0.001 and P = 0.002, respectively). Conclusion:RVFW-LS/PASP and RVEF/PASP as non-invasive estimates of RVPAC are independent predictors of HF rehospitalization in patients with DCM.

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There is little information on the incidence and prognostic significance of arterial hypertension (HTN) in acute coronary syndromes (ACSs), especially in the east European countries. We sought to investigate a registry of ACS patients in Romania, in order to better elucidate whether hypertensive patients are at higher risk of death and deserve a tailored approach for management and follow-up. The data of this study are a framework of the International Survey of Acute Coronary Syndromes in Transitional Countries (ISACS-TC) (ClinicalTrials.gov, NCT01218776). The present analysis focused on 2286 retrospective patients admitted to 23 hospitals in Romania with a diagnosis of ACS. Among 1450 hypertensive patients, 64.5% were admitted with a diagnosis of STelevation myocardial infarction (STEMI), while the remaining was admitted with a diagnosis of non-STEMI (NSTEMI). When compared with non-hypertensive patients, hypertensive patients were older (mean age 60.3 vs. 66.7 years, P , 0.001), were prevalently female (25.8% vs. 35.5%, P , 0.001), and had higher rates of cardiovascular risk factors as well as higher rates of prior myocardial infarction (11.2% vs. 18.3%, P , 0.001). Additionally, they had higher rates of prior stroke (4.2% vs. 11.7%, P , 0.001) and chronic heart failure (11.5% vs. 18.4%, P , 0.001). Despite this adverse clinical profile, hypertensive patients were less likely be to be admitted with Killip class ≥2 (23.1% vs. 26.6%, P , 0.001) but they were more likely to be discharged with NYHA class ≥III (10.6% vs. 7.1%, P , 0.006). There were significant higher rates of unadjusted in-hospital mortality among hypertensive older (.65 years) patients with both STEMI and NSTEMI. Hypertensive ACS patients in Romania represent a higher risk group, since they are more often discharged with NYHA class ≥ III, are older and have an adverse clinical profile. In the elderly, the outcomes of the hypertensive patients are worse than non-hypertensive patients.