Background-The clinical course of patients with hypertrophic cardiomyopathy and advanced heart failure (HF) subtended by progressive left ventricular dysfunction has received limited attention. Our aim was to assess the outcome of HF and impact of treatment options including the implantable cardioverter-defibrillator and heart transplantation (HT) in patients with hypertrophic cardiomyopathy evaluated at 2 Italian referral centers >3 decades. Methods and Results-All-cause mortality and a combined end point including death, HT, or appropriate implantable cardioverter-defibrillator shock were assessed in 71 consecutive patients with HF not related to outflow obstruction (7% of the entire hypertrophic cardiomyopathy cohort) followed up for 6.1±6.9 years after development of New York Heart Association class III to IV symptoms. At enrollment, left ventricular ejection fraction was <50% in 55 patients and >50% in 16; all had restrictive left ventricular filling. During follow-up, 35 patients died (49%%; 5-year rate, 49%) and 53 met the combined end point (75%; 5-year rate, 62%). Most events occurred in the 3 years after HF onset (17% per year compared with only 3% per year subsequently). Appropriate implantable cardioverter-defibrillator shocks occurred in 11 of 34 implanted patients. Of 37 patients evaluated for HT, 14 were transplanted, 10 listed, and 13 excluded; 2 early post-HT deaths occurred in patients with elevated pulmonary vascular resistance. Eleven of the 14 HT patients were alive at 10±8 years. Conclusions-In hypertrophic cardiomyopathy, advanced HF not associated with outflow obstruction portends a severely unfavorable prognosis, particularly in the first 3 years after onset of symptoms, despite frequently preserved systolic function in about one quarter of the patients. Outcome of HT is favorable but requires early consideration, as the window of opportunity may be short. (Circ Heart

Treatment of chronic diseases, such as heart failure, requires complex protocols based on early diagnosis; self-monitoring of symptoms, vital signs and physical activity; regular medication intake; and education of patients and caregivers about relevant aspects of the disease. Smartphones and mobile health applications could be very helpful in improving the efficacy of such protocols, but several barriers make it difficult to fully exploit their technological potential and produce clear clinical evidence of their effectiveness. App suppliers do not help users distinguish between useless/dangerous apps and valid solutions. The latter are few and often characterised by rapid obsolescence, lack of interactivity and lack of authoritative information. Systematic reviews can help physicians and researchers find and assess the ‘best candidate solutions’ in a repeatable manner and pave the way for well-grounded and fruitful discussion on their clinical effectiveness. To this purpose, the authors assess 10 apps for heart failure self-care using the Intercontinental Marketing Statistics score and other criteria, discuss the clinical effectiveness of existing solutions and identify barriers to their use in practice and drivers for change.

In spite of its successful surgical cure, Cushing's syndrome results in a significantly decreased arterial distensibility when compared with the control group, which might explain these differences in blood pressure levels. It underlines a significantly higher cardiovascular risk, notwithstanding both the normalisation of cortisol secretion and the very early age of the patients.

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We present the case of a 67-year-old woman affected by hypertension and hypercholesterolemia. She had a history of alcoholism, anxiety disorder with panic attacks, and paroxysmal atrial fibrillation. Despite a previous transient ischemic attack and her thromboembolic risk (CHA 2 DS 2 -VASc score: 5), she had not been taking anticoagulants at home without a reasonable motivation. Moreover, around eighteen years prior, she had breast cancer and underwent surgical intervention, radiotherapy and several years of hormone-therapy. However, in the following years, she had pelvic bone metastasis needing multiple orthopaedic procedures.The patient was admitted to the Emergency Department with complaints of diarrhoea lasting one week and palpitations associated with dyspnoea in the last 24-hours.Laboratory tests showed severe hypokalaemia (2.8 meq/l), hypomagnesemia (0.4 mg/dl) and elevation, although not significant, of troponin I (0.09 ng/ml -NV ≤ 0.07).ECG showed atrial fibrillation with fast ventricular rate (140/min) and diffuse inverted T-wave, while echocardiography revealed a normal left ventricular function (EF ≈ 60%).A few hours after intravenous replacement of K + and Mg 2+ , the patient showed spontaneous restoration of sinus rhythm. However, suddenly, she became confused and developed generalized motor and non-motor seizures, causing aggravation of diarrhoea and electrolyte disorders. An urgent cerebral CT scan was performed, which excluded acute cerebral events.Contextually, the patient had severe hypotension (systolic blood pressure < 90 mmHg). A significant elevation of troponin I (34.9 ng/ ml) and lactates with low bicarbonates were detected. ECG was normal; however, the echocardiography revealed a severe left ventricular dysfunction (EF: ≈ 20%) due to akinesia of all basal segment and hyperkinesia of apex. Considering the clinical scenario and that regional wall motion abnormalities extended beyond a single epicardial vascular distribution, a basal Takotsubo syndrome (TTS) was hypothesized.After adequate refilling with bolus fluids and intravenous replacement of bicarbonates and electrolytes, the hemodynamic status improved.Simultaneously, in the following 48-hours neurologists tried multiplies oral and intravenous antiepileptics drugs (oxcarbazepine, levetiracetam, sodium valproate and phenytoin) without success, suggesting a non-convulsive status epilepticus, which was confirmed by electroencephalogram ( Fig. 1A). Finally, a deep sedation with propofol was induced and the patient was assisted by mechanical ventilation. In the following days, we assisted in the resolution of the cardiological and neurological states, and the patient was then extubated.