[Optimal therapeutic management in the clinical journey of patients with heart failure]. / L'ottimizzazione terapeutica nel percorso del paziente con scompenso cardiaco.

Heart failure (HF) is still characterized by high mortality rates, despite the progress achieved in terms of treatment options. With regard to the treatment of HF with reduced ejection fraction (HFrEF), the 2016 European Society of Cardiology guidelines included in the therapeutic algorithm the angiotensin receptor-neprilysin inhibitor class, whose efficacy in modifying patient prognosis has been extensively proven in many clinical studies. Sacubitril/valsartan, the only representative of this drug class, can effectively affect the natural history of HF, thus reducing cardiovascular mortality (sudden death and death due to worsening cardiac function), total mortality, as well as first and recurrent hospitalization events, by improving renal function, cardiac remodeling, functional capacity and the patient's health-related quality of life.The purpose of this article is to analyze the different phases of the journey of patients with HFrEF (first general practitioner consultation; admission to the emergency department and subsequent hospitalization; referral to a specialist HF clinic) and promotion of a networking approach involving the general practitioner, the hospital and the HF specialist based on common pre-defined diagnostic and therapeutic protocols, that meets patient needs at all stages of the disease (case-specific dosing assessment, drug titration before follow-up and prevention of adverse events).

A case of arrhythmogenic right ventricular cardiomyopathy with biventricular involvement.

We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Surprisingly, a complete echocardiographic evaluation demonstrated the presence of severe right ventricular enlargement with significant wall motion abnormalities, apical aneurysm and reduced systolic function. Cardiac Magnetic Resonance was pathognomonic for a fibro-fatty replacement of both ventricles. We decided for a subcutaneous defibrillator implantation and, after inducing a ventricular fibrillation to test the device status, epsilon wave appeared on the ECG. This clinical scenario depicted an advanced arrhythmogenic right ventricular cardiomyopathy at its first clinical manifestation.

Effects of glucose-insulin-potassium infusion on myocardial perfusion and left ventricular remodeling in patients treated with primary angioplasty for ST-elevation acute myocardial infarction.

The role of glucose-insulin-potassium (GIK) infusion in the management of acute coronary syndrome is controversial. Limited data are available on the effects of adjunctive high-dose GIK (30% glucose, 50 IU of insulin, 80 mEq of potassium chloride infused at 1.5 ml/kg/hour over 24 hours) on myocardial perfusion and left ventricular (LV) remodeling in patients treated with primary percutaneous coronary intervention (PCI) for ST-segment elevation myocardial infarction. In this prospective study, 73 patients were randomized to receive GIK infusion (n = 40) or saline (placebo, n = 33) in addition to standard therapy. The primary end points were myocardial perfusion after PCI and LV remodeling at 6 months. Thrombolysis In Myocardial Infarction frame count and myocardial blush grade were evaluated before and after reperfusion treatment. LV end-diastolic and end-systolic volumes, ejection fraction, and wall motion score index were assessed in each patient after PCI and after 6 months. Although no differences in final Thrombolysis In Myocardial Infarction flow were observed between the 2 groups, myocardial blush grade 3 was more frequently achieved in the GIK group (p <0.05). At 6 months, ventricular remodeling was more often observed in the control group (24% vs 3%, p <0.05). In conclusion, GIK infusion in adjunct to primary PCI in patients with ST-segment elevation myocardial infarction was safe, improved myocardial perfusion after revascularization, and was associated with less LV remodeling at follow-up.

Mid-term efficacy of beraprost, an oral prostacyclin analog, in the treatment of distal CTEPH: a case control study.

BACKGROUND: Prostanoids are a well-established therapy for pulmonary arterial hypertension (PAH), and observational studies suggest their efficacy even in chronic thromboembolic pulmonary hypertension (CTEPH) patients. OBJECTIVE: To compare the effects of 6 months of treatment with beraprost, an orally-active prostacyclin analog, in patients with distal CTEPH and PAH. DESIGN: Case-control study. POPULATION: Sixteen patients with severe pulmonary hypertension (NYHA II-IV), eight with distal CTEPH matched with eight patients with idiopathic PAH for similar effort tolerance. METHODS: All patients were in stable clinical and hemodynamic condition for 3 months with maximal standard therapy. During the titration phase (4 weeks) beraprost was increased to maximal tolerated dose (mean daily dosage: CTEPH 275 +/- 47 microg, PAH 277 +/- 47 microg) in adjunction of standard therapy, patients were followed-up for 6 months. MAIN OUTCOME MEASURES: World Heart Organization (WHO) functional class, exercise capacity measured by distance walked in 6 min, and systolic pulmonary pressure (echocardiography), were evaluated at baseline, and at 1-, 3- and 6-month interval. RESULTS: At 6 months WHO class decreased significantly in both groups (CTEPH from 2.7 +/- 0.6 to 2.0 +/- 0.24, p < 0.05; PAH from 3.0 +/- 0.26 to 2.1 +/- 0.25, p < 0.05), similarly the 6-min walk distance increased significantly from baseline (CTEPH from 312 +/- 31 to 373 +/- 29 m, p < 0.003; PAH from 303 +/- 31 to 347 +/- 29, p < 0.0003). Systolic pulmonary artery pressure showed a trend toward lower value (CTEPH from 85 +/- 7 m to 81 +/- 6 mm Hg, p = NS; PAH from 89 +/- 7 to 82 +/- 5, p = NS). During the observation period we did not have any death. The drug was well-tolerated with minor side-effects. CONCLUSION: In patients with CTEPH beraprost had similar mid-term clinical and hemodynamic improvements than in patients with PAH.

Effects of levosimendan on left ventricular diastolic function after primary angioplasty for acute anterior myocardial infarction: a Doppler echocardiographic study.

BACKGROUND: Levosimendan is a new Ca-sensitizing drug with combined positive inotropic and vasodilatory effects that offers new therapeutic possibilities in patients with severe heart failure. Compared with other inotropic agents, animal studies demonstrated that levosimendan does not impair left ventricular diastolic function. OBJECTIVE: We sought to evaluate the effects of levosimendan on left ventricular diastolic function, using conventional transmitral Doppler and Doppler tissue imaging parameters, in patients with anterior acute myocardial infarction undergoing primary angioplasty. METHODS: After a successful primary angioplasty, we randomized 52 consecutive patients with anterior acute myocardial infarction to levosimendan or placebo infusion and analyzed the diastolic function using conventional transmitral Doppler flow and Doppler tissue imaging at mitral annulus. RESULTS: At 24 hours after the index intervention, patients treated with levosimendan (n = 26) showed a significant reduction of the isovolumetric relaxation time (114.6 +/- 15.1-69.2 +/- 5.6 milliseconds; P = .001) and the ratio between the early diastolic flow and early tissue velocity (E/E') (21.4 +/- 10.7-12.8 +/- 7.3; P = .04), and a significant increase of the ratio between the early and late diastolic flow (E/A) (0.86 +/- 0.33-1.52 +/- 0.88; P = .03) and E' (6.4-7.9 cm/s; P = .001). On the other hand, only a significant increase in E/A ratio (0.97 +/- 0.32-1.64 +/- 0.51; P = .002) was observed in the placebo group (n = 26). CONCLUSIONS: Levosimendan, after primary angioplasty in patients with anterior acute myocardial infarction, appears to improve the Doppler echocardiographic parameters of left ventricular diastolic function.

Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.

INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital systemic angiodysplasia with multiple vascular malformations in the skin, gastrointestinal tract and, less often, in other internal organs and the brain. CASE REPORT: A 36-year-old man with past history of BRBNS was admitted to our hospital for progressive dyspnea and fatigue. Primary pulmonary hypertension (PPH) was diagnosed. He then developed acute abdominal pain and dyspnea, dying in a few hours due to sudden cardiac arrest. Postmortem examination demonstrated angiomatous lesions located in the skin, small bowel, heart, lungs, liver and thyroid. The lesions were slightly raised, soft and compressible and microscopically consisted of dilated vascular channels lined by a flattened endothelium. The vascular wall was formed by several layers of smooth muscle cells, intermixed with abundant aggregates of elastic lamellae and thin collagen fibers. Luminal thrombi were a frequent finding. In the small bowel, we identified the presence of an abnormally large artery directly opening into a thin-walled venous channel. The most striking finding in the lungs was the presence of thrombi of varying age in the lumen of segmental and elastic arteries, as well as muscular arteries and arterioles. Severe medial hypertrophy of muscular arteries and muscolarization of arterioles were also present. Intimal proliferative lesions and plexiform lesions were never observed. CONCLUSION: The pulmonary findings are consistent with recurrent thromboembolic events from shunts in the visceral lesions. To our knowledge, this is the first report of BRBNS with visceral arterovenous (AV) fistulae complicated by thromboembolic pulmonary hypertension (PH).

New positive inotropic agents in the treatment of left ventricular dysfunction.

Three major classes of inotropic agents have been clinically evaluated in patients with left ventricular dysfunction: a) agents that increase the intracellular concentration of cyclic adenosine monophosphate by stimulating the beta-adrenergic receptor or inhibiting phosphodiesterase; b) drugs that increase the intracellular sodium concentration; c) the new calcium-sensitizing drugs. This review will focus on the newest drug for each of the above-mentioned classes of inotropic agents. Moreover, we present a new protocol which provides the use of levosimendan in patients with post-ischemic left ventricular dysfunction.