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We assessed the efficacy and safety of ranolazine in real-world patients with hypertrophic cardiomyopathy (HCM).Ranolazine is an anti-anginal drug that inhibits the late phase of the inward sodium current. In a small prospective trial, ranolazine reduced the arrhythmic burden and improved biomarker profile in HCM patients. However, systematic reports reflecting real-world use in this setting are lacking.Changes in clinical and instrumental features, symptoms and arrhythmic burden were evaluated in 119 patients with HCM before and during treatment with ranolazine at a national referral centre for HCM.Patients were treated with ranolazine for 2 [1-4] years; 83 (70%) achieved a dosage ≥1000 mg per day. Treatment interruption was necessary in 24 patients (20%) due to side effects (n = 10, 8%) or disopyramide initiation (n = 8, 7%). Seventy patients (59%) were treated with ranolazine for relief of angina. Among them, 51 (73%) had total symptomatic relief and 47 patients (67%) showed ≥2 Canadian Cardiovascular society (CCS) angina grade improvement. Sixteen patients (13%) were treated for recurrent ventricular arrhythmias, including 4 with a clear ischemic trigger, who experienced no further arrhythmic episodes while on ranolazine. Finally, 33 patients (28%) were treated for heart failure associated with severe diastolic dysfunction: no symptomatic benefit could be observed in this group.Ranolazine was safe and well tolerated in patients with HCM. The use of ranolazine may be considered in patients with HCM and microvascular angina.

Pre-partecipation ECG screening of large populations has a significant socioeconomic impact. Technological progress now allows for high-tech-low-cost ECG screening using validated smartphone-based devices capable of guiding to the correct performance of a 12‑lead ECG by layman with no medical background.We enrolled 728 (364, 52% males) individuals, aged 12-13 years who underwent ECG screening with a smartphone 12‑lead ECG during school hours by layman volunteers. Correct electrodes placement was provided by a validated image-processing algorithm by the smartphone camera in the App. ECG interpretation was via a telecardiology platform and alterations classified following current standards.A total of 741 ECGs were recorded, of which 13(2%) were technically not interpretable. Mean PR, QRS and QTc were: 145 ± 22, 85 ± 19 and 387 ± 57 msec. No QTc prolongation was observed. Mean QRS axis was 15°; 26 (4%) patients presented an iRBB. T-wave inversion from V1-V3 was present in 145 (21%) subjects. Twenty-one(3%) patients were referred to second level examination: deep Q-waves in inferior leads in 12(1.6%), ventricular ectopics in 5(0.7%), anterior T-waves inversions V1-V4 in 3(0.4%); extreme right axis deviation in 1(0.3%). Second line investigations did not provide any definitive diagnosis. Total project costs (material equipment and human cost) was 14.460€, 19.51€ per individual. The potential net saving with respect to current pre-participation screening cost was 19%.Layman 12‑lead Smartphone-ECG population screening proved feasible and effective, with a rate of non-interpretable ECG of5%. Potential cost-saving in ECG screening and recording was 19%, providing an appealing opportunity when large campaigns should be addressed also in developing countries.

The implantable cardioverter defibrillator(ICD) has revolutionized the management of patients with hypertrophic cardiomyopathy (HCM) at risk of sudden cardiac death (SCD). However, the identification of ideal candidates remains challenging. We aimed to describe the long-term impact of the ICD for primary prevention in patients with HCM based on stringent (high SCD risk) vs lenient indications (need for pacing/personal choice).Data from two Italian HCM Cardiomyopathy Units were retrospectively analyzed. Only patients1 follow-up visits were divided into two groups according to ICD candidacy:stringent (high SCD risk) and lenient (need for pacing, patients' choice, physician advice despite lack of high SCD risk). Major cardiac events (composite of appropriate shock/intervention and SCD) was the primary endpoint. A safety endpoint was defined as a composite of inappropriate shocks and device-related complications.Of 2009 patients, 252(12.5%) received an ICD, including 27(1.3%) in secondary prevention and 225(11.2%) in primary prevention (age at implantation 49 ± 16 years; men 65.3%). Among those in primary prevention, 167(74.2%) had stringent, while 58(25.8%) had lenient indications. At 5 ± 4 years, only stringent ICD patients experienced major cardiac events (2.84%/year, 5-year cumulative incidence: 8.1%, 95%CI [3.5-14.1%]). ICD-related complications were similar across stringent and lenient subgroups. However, patients implanted60 years had a significantly higher risk of adverse events.One third of ICD recipients with HCM in primary prevention received a lenient implantation and had no appropriate intervention. ICD implantation due to systematic upgrade in patients requiring pacing and increased risk perception may offer little advantage and increase complication rates.

Whether early vs. delayed referral to septal reduction therapies (SRT, alcohol septal ablation or surgical myectomy) bears prognostic relevance in hypertrophic obstructive cardiomyopathy (HOCM) is unresolved. We analyzed the impact of SRT timing on the outcome of HOCM patients.We followed 126 patients for 5 ± 4 years after SRT (mean age 53 ± 15 years; 55 post-ASA and 71 post-SM). Based on time-to-treatment (TTT; from HOCM diagnosis to SRT), patients were divided into three groups: "3" years, N = 50; "3-5" years, N = 25; "5" years, N = 51.Patients with TTT 5 years were younger at diagnosis and more often had atrial fibrillation (AF). Left ventricular outflow tract (LVOT) gradients were comparable in the 3 TTT groups. Two patients died peri-operatively, all with TTT 5. Long-term, 8 patients died (3 suddenly and 5 due to heart failure). Mortality increased progressively with TTT (2% vs. 4% vs. 12% for TTT "3", "3-5", and "5" years, p for trend = 0.039). Independent predictors of disease progression (new-onset AF, worsening to NYHA III/IV symptoms, re-intervention or death) were TTT ("3-5" vs. "3" years: HR: 4.988, 95%CI: 1.394-17.843; "5" vs. "3" years: HR: 3.420, 95%CI: 1.258-9.293, overall p-value = 0.025), AF at baseline (HR: 1.896, 95%CI: 1.002-3.589, p = 0.036) and LVOT gradient (HR per mm Hg increase: 1.022, 95%CI: 1.007-1.024, p = 0.023).Delay in SRT referral has significant impact on long-term outcome of patients with HOCM, particularly when5 years from first detection of gradient, even when successful relief of symptoms and gradient is achieved. Earlier interventions are associated with lower complication rates and better prognosis, suggesting the importance of timely SRT to maximize treatment benefit and prevent late HOCM-related complications.

Ventricular or supraventricular ectopic beats or atrial fibrillation may be the first presentation of uncommon cardiac disease, both acquired and genetically determined. In some patients, these manifestations can be the first sign of the underlying cardiac disorder. In others, however, they are also important as prognostic indicators, reflecting electrical instability and risk. Most cardiology clinics are busy environments where the implementation of complex diagnostic algorithms is not feasible. However, it is equally impossible to reach a final diagnosis, among the thousands of rare diseases that involve the heart, moving from a first line clinical and instrumental examination. Cardiac and extra-cardiac red flags, an accurate family and clinical history and ECG interpretation may be of help in identifying a rare disease. Advanced imaging and laboratory testing at experienced referral centers is then necessary to reach a final diagnosis, but the first step in the right direction, based on these simple elements, is the most important. We here review arrhythmic presentations of rare or relatively rare diseases, and suggest a simple "rule out-rule in" approach to help direct clinical suspicion and minimize risk of neglect.

Background MHealth technologies are revolutionizing cardiovascular medicine. However, a low-cost, user-friendly smartphone-based electrocardiograph is still lacking. D-Heart® is a portable device that enables the acquisition of the ECG on multiple leads which streams via Bluetooth to any smartphone. Because of the potential impact of this technology in low-income settings, we determined the accuracy of D-Heart® tracings in the stratification of ECG morphological abnormalities, compared with 12-lead ECGs. Methods Consecutive African patients referred to the Ziguinchor Regional Hospital (Senegal) were enrolled (n=117; 69 males, age 39±11years). D-Heart® recordings (3 peripheral leads plus V5) were obtained immediately followed by 12 lead ECGs and were assessed blindly by 2 independent observers. Global burden of ECG abnormalities was defined by a semi-quantitative score based on the sum of 9 criteria, identifying four classes of increasing severity. Results D-Heart® and 12-lead ECG tracings were respectively classified as: normal: 72 (61%) vs 69 (59%); mildly abnormal: 42 (36%) vs 45 (38%); moderately abnormal: 3 (3%) vs 3 (3%). None had markedly abnormal tracings. Cohen's weighted kappa (k w ) test demonstrated a concordance of 0,952 (p w =0,893; p Conclusions D-Heart® proved effective and accurate stratification of ECG abnormalities comparable to the 12-lead electrocardiographs, thereby opening new perspectives for low-cost community cardiovascular screening programs in low-income settings.

Background: Subcutaneous ICD (S-ICD) is a promising option for Hypertrophic Cardiomyopathy (HCM) patients at risk of Sudden Cardiac Death (SCD). However, its effectiveness in terminating ventricular arrhythmias in HCM is yet unresolved.Methods: Consecutive HCM patients referred for S-ICD implantation were prospectively enrolled. Patients underwent one or two attempts of VF induction by the programmer. Successful conversion was defined as any 65 J shock that terminated VF (not requiring rescue shocks). Clinical and instrumental parameterswere analyzed to study predictors of conversion failure.Results: Fifty HCMpatients (34males, 40 +/- 16 years) with amean BMI of 25.2 +/- 4.4 kg/m(2) were evaluated. Mean ESC SCD risk of was 6.5 +/- 3.9% and maximal LV wall thickness (LVMWT) was 26 +/- 6 mm. In 2/50 patients no arrhythmias were inducible, while in 7 (14%) only sustained ventricular tachycardia was induced and cardioverted. In the remaining 41 (82%) patients, 73 VF episodes were induced (1 episode in 14 and > 1 in 27 patients). Of these, 4 (6%) spontaneously converted. In 68/69 (98%) the S-ICD successfully cardioverted, but failed in 1 (2%) patient, who needed rescue defibrillation. This patient was severely obese (BMI 36) and LVMWT of 25 mm. VF was re-induced and successfully converted by the 80 J reversed polarity S-ICD.Conclusions: Acute DT at 65 J at the implant showed the effectiveness of S-ICD in the recognition and termination of VT/VF in all HCM patients except one. Extreme LVH did not affect the performance of the device, whereas severe obesity was likely responsible for the single 65 J failure. (C) 2017 Elsevier Ireland Ltd. All rights reserved.