Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy.

Whereas it is well known that idiopathic HCM can present in newborns and infants, little information is available on HT in this very young age group. We report a series of 17 infants with idiopathic HCM, including two neonates with rapidly progressive severe HF for whom HT was necessary. When HF manifests in a newborn/infant with idiopathic HCM and extreme cavity size reduction, the possibility of a rapidly progressive clinical course should be anticipated and HT may become the only available therapeutic solution.

Frequency, determinants, and clinical relevance of acute coronary syndrome-like electrocardiographic findings in patients with acute aortic syndrome.

We investigated frequency/characteristics of acute coronary syndrome-like (ACS-like) electrocardiographic (ECG) profiles among patients with a final diagnosis of acute aortic syndrome (AAS), and explored pathophysiologic determinants and prognostic relevance within each Stanford subtype. We blindly reviewed presentation electrocardiograms of 233 consecutive patients with final diagnosis of AAS (164 Stanford type A) at a regional treatment center. Prevalence of ACS-like ECG findings was 27% (type A, 26%, type B, 29%); most were non-ST-elevation myocardial infarction-like. Patients with ACS-like ECG findings more often had coronary ostia involvement (p=0.002), pleural effusion (p=0.02), significant aortic regurgitation (p=0.01), and troponin positivity (p=0.001). ACS-like ECG profile in type A disease was independently associated with coronary ostia involvement (odds ratio [OR] 5.27, 95% confidence interval [CI] 1.75 to 15.88). ACS-like ECG profile predicted in-hospital mortality (OR 2.90, 95% CI 1.24 to 6.12), as did age (each incremental 10-year: OR 1.59, 95% CI 1.14 to 2.22), and syncope at presentation (OR 2.90, 95% CI 1.16 to 7.24). In conclusion, about 25% of our AAS patients (in either Stanford subtype) presented ACS-like ECG patterns-often with non-ST-elevation myocardial infarction characteristics-which could cause misdiagnosis. ACS-like ECG profile was associated with more complicated disease, and in type A disease was a strong independent predictor of in-hospital mortality.

[CircuLite Synergy ventricular assist device: a new approach to end-stage congestive heart failure]. / Sistema di assistenza ventricolare CircuLite Synergy: un nuovo approccio all'insufficienza cardiaca terminale.

BACKGROUND: The Synergy system, a miniature partial circulatory support device, is implanted by an off-pump, minimally invasive surgical approach. The system has been optimized to improve performance in an EU clinical trial for chronic ambulatory heart failure. This therefore offers the possibility of treating elderly chronic heart failure patients who might not usually be considered for long-term circulatory support. METHODS: From June 2007 to December 2012, 63 patients were implanted with the Synergy system (12 patients ≥70 years) using four different releases of the device. Briefly, the system draws blood through the inflow cannula from the left atrium into the micro-pump (placed in a right subclavicular pocket) and pumps it through an outflow graft to the right subclavian artery. In this paper, we present an intermediate analysis of the clinical trial as performed on April 30, 2013, leading to the placing of the CE mark. RESULTS: Mean duration of support is ongoing at 230 days (range 23-1387). Follow-up showed improved hemodynamic response, with additional improvements in 6-min walk distance (299 ± 144 to 420 ± 119 m) and Minnesota Living with Heart Failure Questionnaire (69.5 ± 20.4 to 49.2 ± 24.3). Older patients had longer mean durations of support (337 vs 188 days). On average, elderly and younger patients showed similar improvements in hemodynamics and 6-min walk distance (107 ± 120 vs 130 ± 121 m). Major adverse cardiac events included bleeding (n=4) with one bleeding related to renal failure resulting in death. CONCLUSIONS: Clinical use of the Synergy device was associated with a significant functional improvement. Very low adverse event rates were reported with the latest device release. Older patients had smaller body sizes and worse renal function than younger patients. Both groups experienced similar hemodynamic benefits and functional improvements. The risk of bleeding and renal dysfunction appears to be increased in the elderly, though still within acceptable ranges compared to other full support devices. Minimally invasive long-term circulatory support devices, like Synergy, offer a new treatment option that might be available even for the elderly chronic heart failure population.

[Electrocardiographic diagnosis of acute myocardial infarction in the aera of primary percutaneous coronary intervention and hub and spoke networks]. / La diagnosi elettrocardiografica di infarto miocardico acuto nell'era dell'angioplastica primaria e delle reti hub and spoke.

More than a century after it was invented, standard ECG is enjoying a renaissance of sorts. With regard to acute ischemic heart disease, this phenomenon is due mainly to the availability of large databases that in an ordered and predefined manner collect patient ECG patterns along side their clinical and coronary angiography details as well as outcome data. The present review critically analyses the diagnostic role of standard ECG in acute coronary syndromes with or without ST-segment elevation (STEMI and NSTEMI, respectively) and focuses on interpretation pitfalls and patterns that can contribute to therapeutic decision-making. In front of a patient with a clinical presentation suggestive of acute myocardial infarction the ECG can help answer many questions. In case a STEMI is suspected: are we sure we can exclude an infarction? (the problem of false negatives); are we sure it is a real infarction and not a false positive? Which is the obstructed coronary artery and at what level? Has there been reperfusion? In case an NSTEMI is suspected: are we sure it is a real myocardial infarction, rather than a pulmonary embolism or an aortic dissection? Are we sure it is NSTEMI rather than a "masked" dorsal STEMI? Which coronary substrate and what ischemia extension can we hypothesize in this patient? In particular, is the substrate of such high risk suggesting an emergency invasive approach?