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Introduction Double-chambered right ventricle (DCRV) is a rare congenital heart defect with right ventricular outflow tract (RVOT) obstruction. The right ventricle (RV) is divided into anatomically proximal high-pressure and distal low-pressure chambers by abnormal muscle bundle. DCRV is frequently associated with others congenital heart defects, particularly ventricular septal defects (VSDs). Although its typically presents during childhood and adolescence, it can also present in adulthood. Case Presentation An 84-years-old woman was admitted to our hospital, in emergency department, with a 30-days history of worsening dyspnea and exercise intolerance. She was Ukrainian and did not speak Italian or English. The patient past medical history was unknow except for untreated bilateral glaucoma complicated by blindness. Vital signs were notable for tachycardia, tachypnea (respiratory rate, 28/minute), blood pressure of 118/76 mm Hg, SpO2 of 91%. Physical exam revealed left-sided parasternal systolic murmurs, abolished vesicular murmur at lung bases and jugular vein distension with hepatojugular reflux. The ECG showed atrial fibrillation. Chest X-ray showed moderate cardiomegaly, bilateral pleural effusions, and pulmonary congestion. Transthoracic echocardiography (TTE) was performed and revealed a normal-sized left ventricle with mildly reduced left ventricle ejection fraction (EF 48%), left atrial enlargement, biventricular hypertrophy with asymmetrical interventricular septal hypertrophy. Also, we found massive right atrium and enlarged right ventricle with reduced longitudinal contractility (TAPSE of 13 mm and tricuspid annular tissue Doppler S’ velocity = 7.0 cm/sec). Color flow Doppler in parasternal short-axis view revealed a turbulent systolic flow into the right ventricle. Continuous-wave spectral Doppler analysis showed a peak velocity of 5.6 m/ sec corresponding to a peak gradient of 120 mmHg. Real time 3D-TTE confirmed the of mid-ventricular obstruction due to abnormal trabecular tissue. Therapy including diuretics, beta-blockers and anticoagulants was started. Subsequently, a transesophageal echocardiography (TOE) confirmed the presence of an anomalous mid-ventricular muscle bundle and revealed an associated small sub-aortic ventricular septal defect (VSD) leading to the diagnosis of acute RV failure due to double-chambered RV with VSD and atrial fibrillation. Due to the high risk of complications, patient was considered not amenable for surgery. She was discharged on medical therapy. Conclusions We report a rare case of DCRV and VSD diagnosed in an elderly patient. Due to its rarity, DCRV continues to be misdiagnosed, especially in adulthood. Three-dimensional echocardiography and TOE were most useful tool to define diagnosis and pathophysiology in such an elderly and non-compliant patient.

Background Congenitally corrected transposition of great arteries (ccTGA) is an uncommon complex congenital heart disease with atrio-ventricular and ventriculo-arterial connections discondance. ccTGA may be associated with a situs solitus or situs inversus (34% of cases). Situs inversus is a mirror image of normal with the systemic ventricle situated on the right side. Instead, dextrocardia represents 20% of cases. Case clinic and discussion Came to our observation a 61 years old female, symptomatic for dyspnea on exertion (NYHA II). She had no past medical history of cardiovascular events. In anamnesis two full-term pregnancies without complications. Transthoracic echocardiogram found atrioventricular and ventriculo-arterial discordance in absence of significant valvulopathy. Cardiac computed tomography showed pulmonary veins linked to right atrium, superior and inferior cava veins connected to the left atrium; right atrium with tricuspid valve was connected to a morphologically left ventricle and left atrium with mitral valve was linked to morphologically right ventricle; pulmonary artery was connected to morphologically left ventricle instead aorta with aortic valve was linked to morphologically right ventricle. Cardiac MRI confirmed cctga in situs viscerum inversus, mild subpulmonary stenosis, moderate dilatation of arterial pulmonary trunk, and also intramyocardial late gadolinium enhancement due to fibrosis involving anterior and inferior interventricular junctions. Cardiac Holter monitoring showed sinus rhythm with some brief phases of low atrial rhythm, monomorphic isolated ventricular extrasystoles in absence of significant hyperkinetic or hypokinetic arrhythmias. CcTGA represents approximately 0.5% of all congenital heart disease. If undiagnosed in childhood, people usually become symptomatic during the first decades of life. Dyspnea, syncope and fatigue are the most frequent symptoms detected. Cardiac conduction disorders such as atrioventricular blocks are common due to the abnormal development of cardiac structures. Quality of life and its expectancy are related to the latency of the onset of heart failure symptoms. Only few patients remain asymptomatic beyond 50 years old. Symptoms and signs are frequently due to right sided (systemic) heart dysfunction and tricuspid valve insufficiency. A particular clinical situation worthy of attention is pregnancy because of the hemodynamic imbalance occurring. In fact, cardiac output increases of 40–50% above baseline determining an augmentation of stroke volume and heart rate. For these reasons, echo surveillance is needed every 4-8 weeks because of the increased risk of acute heart failure. An accurate assessment of heart rhythm has to be done due to the known predisposition to bradyarrhythmic and tachyarrhythmic events in ccTGA. Conclusions CcTGA patients require a strict cardiological follow up with echocardiographic assessment and periodic heart rhythm monitoring, in order to early detect worsening of cardiac function and significant abnormalities of the rhythm.

Background Large cardiovascular (CV) trials enrolling patients with type 2 diabetes showed that sodium glucose co-transporter-2 inhibitors (SGLT2i) significantly decreased heart failure (HF) hospitalization, both in patients with or without a history of HF. Accordingly, DAPA-HF (Dapagliflozin and Prevention of Adverse Outcomes in Heart Failure) demonstrated the efficacy of dapagliflozin, for the reduction of CV death/HF hospitalization in patients with HF with reduced ejection fraction (HFrEF) regardless of type 2 diabetes status. However, there are still few real-word data and it is still not well known how early the clinical benefits are after the introduction of the drug into therapy; consequently, we aimed to evaluate the effect of dapagliflozin three months after its introduction in therapy in our real-world population. Methods From February 2022 to September 2022 we introduced Dapagliflozin in 23 HFrEF patients’ therapy and we collected data of 11 patients (66.78±3.96 years; 89% men) at 3-months-FU. Specifically, on the first visit we collected the clinical, laboratory and echocardiographic parameters and dapagliflozin was added to optimal medical therapy of patients; then, the patients were evaluated after 3 months (follow-up). Results At follow-up, all patients were free from side effects and we did not record statistically significant differences in laboratory parameters and/or blood pressure values. As regards the echocardiographic parameters, there was an improvement in FE (28.11±2.95 vs 37.00±5.71%, p0.17), PAPS (46.89±3.94 vs 37.63±5.27mmHg, p0.17), and LVEDVind (75.34±10.58 vs 57.20±13.55 ml/m2, p0.30), although not statistically significant. Moreover, we observed a statistically significant reduction in the diameter of the inferior vena cava (18.89±1.78 vs 11.5±1.15 mm, p Conclusions Dapagliflozin improved symptoms, and quality of life in patients with HFrEF of our real world population already after 12 weeks, accordingly with previous data of DEFINE-HF trial. Moreover, already after 3 months was possible to record improvements in the echocardiographic parameters, even if they are not statistically significant. Certainly, it will be necessary to continue with the study to evaluate these results on a larger sample.

Background Heart failure (HF) alternates phases of stability and phases of exacerbation, with a progressive decline in the patient's functional capacity and quality of life; the need to anticipate and improve the effectiveness of management of HF exacerbation has led to the development of several remote monitoring tools. We report our experience with CardioMEMS HF system (implantable device to monitor changes in pulmonary artery diastolic pressure (PAPd) as early indicator of the onset of worsening HF) in order to optimize the pharmaceutical treatments strategy (e.g. Levosimendan infusion) and to assess the impact on hospital resources consumption and costs. Methods We enrolled 7 patients (69.00±4.88 years; 30% female) with end-stage HF, implanted with CardioMEMS and daily monitored remotely, in order to optimize both tailored adjustment of home therapy and infusions of Levosimendan. More in detail, if the cardiologist detected a tendency for PAPd to rise, patients were contacted for home therapeutic changes. If no further changes were possible, the patient was hospitalized for the infusion of Levosimendan. In order to calculate the impact of this remote monitoring strategy on resources consumption, we collected data on hospitalizations (e.g. causes, numbers, length, high-cost drugs and costs) taking into account the same number of months pre and post-CardioMEMS implant for each patient. Results Following the implantation of CardioMEMS we observed a 45% reduction in the total number of hospitalizations and a 62% reduction in the days of hospitalization (from a total of 421 days before implantation to a total of 159 days post implantation in the observation period). From an economic point of view, a significant hospital cost reduction was recorded in terms of both hospitalization costs (HF related re-hospitalization and CardioMEMS's implant related cost) and drugs infusion costs (hospital stay and drug costs); more specifically, the total savings for the 7 patients are around € 236,000 and total days of hospitalization avoided are around 500 days including the hospitalizations avoided for drugs infusion. Accordingly, was recorded an improvement in patients’ quality of life measured with EQ5D (pre-implantation 75.17±2.06 vs post-implantation 108.60±8.70, p 0.0078). Conclusions Our preliminary results support the usefulness of this system in the remote management of the HF patients and in the re-hospitalization reduction both for exacerbation and drug management. In fact, the parameters’ monitoring through the CardioMEMS device allows a personalized management of drug therapy; more precisely, considering the drug Levosimendan, instead of a periodic standard timing for infusion, a patient-tailored timing of infusion was applied. In conclusion, our innovative strategy contributes to achieve the organizational efficiency of the healthcare facilities, as well as to the adequate use and allocation of financial and human resources with a better outcome for HF patients.

Sacubitril/valsartan improves outcome in patients with heart failure (HF) with reduced left ventricular (LV) ejection fraction (EF, HFrEF). However, little is known about possible mechanisms underlying this favourable effect.To assess changes in echocardiographically-derived hemodynamic profiles induced by sacubitril/valsartan and their impact on outcome.In this multicenter, open-label study, 727 HFrEF outpatients underwent comprehensive echocardiography at baseline (before starting sacubitril/valsartan) and after 12 months. Estimated LV filling pressure (E/e') and cardiac index (CI, l/min/mAt baseline, 29% had profile-A, 15% had profile-B, 32% profile-C, and 24% profile-D. After 12 months, the hemodynamic profile improved in 53% of patients (all profile-A achievers, or profile-D patients achieving either C or B profile), while it remained unchanged in 39% patients and worsened in 9%. Prevalence of improved profile progressively increased with increasing dose of sacubitril/valsartan (P 0.0001). After the second echocardiography, patients were followed up 12.6 ± 7.6 months: event-rate was lower in patients with improved profile (12.3%, 95%CI: 9.4-16.1) compared to patients in whom hemodynamic profile remained unchanged (29.9%, 24.0-37.3) or worsened (31.2%, 20.7-46.9, P 0.0001). Improved hemodynamic profile was associated with favourable outcome independent of LVEF and other covariates (HR 0.65, 95%CI: 0.45-0.95, P 0.05).In HFrEF patients, the beneficial prognostic effects of sacubitril/valsartan are associated with improvement in hemodynamic conditions.

The aim of this study was to evaluate the effects of Sacubitril/Valsartan (S/V) on clinical, laboratory and echocardiographic parameters and outcomes in a real-world population with heart failure with reduced ejection fraction (HFrEF). This was a prospective observational study enrolling patients with HFrEF undergoing treatment with S/V. The primary outcome was the composite of cardiac death and HF rehospitalization at 12 months follow-up; secondary outcomes were all-cause death, cardiac death and the occurrence of rehospitalization for worsening HF. The clinical outcome was compared with a retrospective cohort of 90 HFrEF patients treated with standard medical therapy. The study included 90 patients (66.1 ± 11.7 years) treated with S/V. The adjusted regression analysis showed a significantly lower risk for the primary outcome (HR:0.31; 95%CI, 0.11–0.83; p = 0.019) and for HF rehospitalization (HR:0.27; 95%CI, 0.08–0.94; p = 0.039) in S/V patients as compared to the control group. A significant improvement in NYHA class, left ventricular ejection fraction, left ventricular end systolic volume and systolic pulmonary arterial pressure was observed up to 6 months. S/V did not affect negatively renal function and was associated with a significantly lower dose of furosemide dose prescribed at 6- and 12-month follow-up. In this study, S/V reduced the risk of HF rehospitalization and cardiac death at 1 year in patients with HFrEF. S/V improved NYHA class, echocardiographic parameters and need of furosemide, and preserved renal function.