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Bedside evaluation using HHE is helpful for assessing LV chamber and walls dimensions, LV regional function, and morphological abnormalities of the valves. The device can be used by cardiology trainees with limited experience in echocardiography but only in combination with a standard examination.

Background: The aim of this study was to explore the rare variants in a cohort of Romanian index cases with hypertrophic cardiomyopathy (HCM). Methods: Forty-five unrelated probands with HCM were screened by targeted next generation sequencing (NGS) of 47 core and emerging genes connected with HCM. Results: We identified 95 variants with allele frequency < 0.1% in population databases. MYBPC3 and TTN had the largest number of rare variants (17 variants each). A definite genetic etiology was found in 6 probands (13.3%), while inconclusive results due to either known or novel variants were established in 31 cases (68.9%). All disease-causing variants were detected in sarcomeric genes (MYBPC3 and MYH7 with two cases each, and one case in TNNI3 and TPM1 respectively). Multiple variants were detected in 27 subjects (60%), but no proband carried more than one causal variant. Of note, almost half of the rare variants were novel. Conclusions: Herein we reported for the first time the rare variants identified in core and putative genes associated with HCM in a cohort of Romanian unrelated adult patients. The clinical significance of most detected variants is yet to be established, additional studies based on segregation analysis being required for definite classification.

Objective: To evaluate the impact of superobesity, defined as body mass index (BMI) ≥50, on cardiac structure and function. Methods: Using echocardiography, we studied 198 asymptomatic patients (mean age 48 ± 13 years, 29.3% were men) with a BMI ≥40. Insulin resistance was measured using the Homeostasis Model Assessment of insulin resistance (HOMA-IR). Patients were divided into 2 groups: morbidly obese (BMI ≥40 and <50; n = 160) and superobese (BMI ≥50; n = 38). Results: There were no significant differences in age, gender, hypertension and diabetes between groups. Superobese patients had higher LV mass (66.0 ± 14.7 vs. 59.9 ± 11.9 g/m^2.7, p = 0.007), left ventricular (LV) end-diastolic (33.8 ± 7.7 vs. 31.5 ± 7.1 ml/m^2.7, p = 0.041) and end-systolic (12.2 ± 3.6 vs. 10.9 ± 2.8 ml/m^2.7, p = 0.016) volumes, left atrial volume (13.8 ± 4.5 vs. 12.2 ± 3.9 ml/m^2.7, p = 0.029), peak velocity of transmitral flow in early diastole/early diastolic peak myocardial velocity ratio (9.1 ± 2.6 vs. 8.2 ± 2.2, p = 0.03) and HOMA-IR (9.7 ± 7.3 vs. 7.3 ± 6.5, p = 0.047). LV ejection fraction was similar. Conclusions: Superobesity is associated with insulin resistance and a worse impact on cardiac remodeling and LV diastolic function than morbid obesity. Prospective studies are needed to evaluate whether such further classification of morbid obesity could stratify the cardiovascular risk in these patients more accurately.

Hypertrophic cardiomyopathy (HCM) is a genetic disease and the most frequent primary cardiomyopathy, affecting 1:500 of the general population. Integrated multimodality imaging, including transthoracic echocardiography, 2-and 3-dimensional transesophageal echocardiography, stress echocardiography, and cardiac magnetic resonance, has provided answers to questions on the management of HCM, leading to standardized protocols. The late 1990s brought the news of a nonsurgical treatment of obstruction in HCM. It is now increasingly evident that septal ablation cannot address all the mechanisms of the left ventricular outflow tract (LVOT) gradient, especially mitral valve involvement. According to American and European guidelines, surgical septal myectomy is the current gold standard treatment. However, deep septal myectomy requires specific operator and institutional experience; therefore, it should not be performed in small community hospitals but only in centers of excellence for HCM treatment. The so-called Ferrazzi technique involves cutting the fibrotic secondary chordae of the mitral valve (MV) and thus helps avoid a deep myectomy by moving the anterior mitral leaflet and the coaptation point of the MV posteriorly away from the septum. This technique, together with careful mobilization of the papillary muscles, helped us achieve excellent results since November 2015, with no mortality, resolution of the LVOT gradient, and MV preservation in all 72 patients. Owing to recent advances in the surgical treatment of hypertrophic obstructive cardiomyopathy, addressing not only the septum but also the MV, the procedure of a deep myectomy has been simplified and mitral regurgitation adequately corrected. KEY WORDS hypertrophic cardiomyopathy, secondary chordae of the mitral valve, septal myectomy 11 Tendera M, Wycisk A, Schneeweiss A, et al. Effect of sotalol on arrhythmias and exercise tolerance in patients with hypertrophic cardiomyopathy. Cardiology. 1993; 82: 335-342. 12 Maron MS, Spirito P, Maron BJ. Case for earlier surgical myectomy in patients with obstructive hypertrophic cardiomyopathy. Circulation. 2018; 138: 2076-2078. 13 Maron BJ, Rowin EJ, Casey SA, Maron MS. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice. JAMA Cardiol. 2016; 1: 98-105. 14 Vriesendrop PA, Schinkel AF, Soliman OI, Kofflard MJ, de Jong PL, van Herwerden LA, et al. A contemporary European experience with surgical septal myectomy and anterior mitral vave extension in obstructive cardiomyopathy. Eur Heart J. 2012; 33: 2080-2087. 15 Kim LK, Swaminathan RV, Looser P, et al. Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy: US Nationwide Inpatient Database, 2003-2011. JAMA Cardiol. 2016; 1: 324-332. 16 Desai MY, Smedira NG, Bhonsale A, et al. Symptom assessment and exercise impairment in surgical decision making in hypertrophic obstructive cardiomyopathy: relations...