20-year follow-up of rheumatic mitral stenosis patients after percutaneous mitral commissurotomy: invasive transmitral gradient differential as a predictor of events.

Percutaneous mitral valve commissurotomy (PMC) is a viable alternative to mitral valve (MV) surgery in the treatment of patients with rheumatic mitral stenosis (RMS). In this single-center retrospective study of consecutive patients with RMS submitted to PMC from 1991 to 2008, we analyzed clinical, echocardiographic, and hemodynamic data and events during follow-up (FUP) until December 2021. Major adverse cardiovascular events (MACE) were a combined endpoint of all-cause death, cardiovascular hospitalization, and MV re-intervention. A total of 124 patients were enrolled: 108 (87.1%) were female, with a mean age at PMC of 46 [standard deviation (SD) 11] years. PMC was successful in 91.1%, with a mean reduction in invasive transmitral pressure gradient (TMPG) of 8 (SD 7) mmHg at PMC time. During the mean FUP of 20 (SD 6) years, 51 (41.1%) patients had MV re-intervention (86.3% surgery and 13.7% redo-PMC), 37 (29.8%) were hospitalized, and 30 (24.2%) died. Approximately 75% of patients remained MACE-free after 10 years, and this percentage decreased to around 40% after 20 years; at this time mark, about 8 in 10 patients were alive. A reduction of <5 mmHg in TMPG at PMC time was associated with a 2.7-fold greater rate of MACE compared to a reduction of ≥5 mmHg, independent of MV regurgitation after PMC and moderate disease of other valves (adjusted hazard ratio 2,7; 95% confidence interval 1.395-5.298, p=0.003). In this cohort with favorable long-term results after PMC, a reduction of <5 mmHg in TMPG at PMC time was associated with MACE during FUP. More studies are needed to validate this independent predictor.

Screening of cardiac allograft vasculopathy in heart transplant patients with coronary computed tomography angiography.

Although coronary angiography (CA) is the gold standard for coronary allograft vasculopathy (CAV) screening, non-invasive modalities have arisen as potential alternatives, such as coronary computed tomography angiography (CCTA). CCTA also quantifies plaque burden, which may influence medical treatment. From January 2021 to April 2022, we prospectively included heart transplant recipients who performed CCTA as a first-line method for CAV detection in a single center. Clinical, CCTA, and CA data were collected. 38 patients were included, 60.5% men, aged 58±14 years. The most frequent cause of transplantation was dilated cardiomyopathy (42.1%), and the median graft duration was 10 years [interquartile range (IQR) 9]. The median left ventricle ejection fraction was 61.5% (IQR 6). The median calcium score was 17 (IQR 231) and 32 patients (84.2%) proceeded to CCTA: 7, 24, and 1 patients had a graded CAV of 0, 1, and 2, respectively. Most patients (37.5%) had both calcified and non-calcified plaques, and the median number of affected segments was 2 (IQR 3). The remaining six patients had extensive coronary calcification, so CA was performed: 4 had CAV1, 1 had CAV2, and 1 had CAV3. During follow-up (12.2±4.2 months), there were neither deaths nor acute coronary syndromes. After CCTA, therapeutic changes occurred in about 10 (26.3%) of patients, mainly related to anti-lipid intensification; such changes were more frequent in patients with diabetes after heart transplant. In this cohort, CCTA led to therapeutic changes in about one-quarter of patients; more studies are needed to assess how CCT may guide therapy according to plaque burden.

Left Ventricular Mechanical Reverse Remodelling Not Followed by Electrical Reverse Remodelling: A Case Report.

Patients with severely depressed left ventricular ejection fractions (LVEFs) receive implantable cardioverter-defibrillators (ICDs) for the primary prevention of sudden death. However, in some patients, LVEFs may improve or even normalize over time, and these patients would no longer be qualified for ICD implantation based on the original criteria for which they have initially received an ICD. We report a patient with idiopathic dilated cardiomyopathy whose LVEF recovered to normal values after pharmacological therapy. Meanwhile, the patient had life-threatening ventricular fibrillation, aborted by the ICD. We reflect on the pathological features of left ventricular reverse remodelling and ventricular arrhythmogenesis, where the myocardial substrate appears to play an important role. Also, after LVEF improvement in a patient with a cardiac device, there is still a debate on whether we should perform a battery replacement.

Iron deficiency status irrespective of anemia: a predictor of unfavorable outcome in chronic heart failure patients.

OBJECTIVE: To assess the prognostic significance of iron deficiency (ID) in a chronic heart failure (CHF) outpatient population. METHODS AND RESULTS: We prospectively evaluated 127 patients with stable CHF and left ventricular ejection fraction ≤45%. Clinical and analytical data as well as information regarding the occurrence of the composite endpoint of overall mortality and nonfatal cardiovascular events were assessed. Among the 127 patients enrolled [81% men, median age: 62 years (25th-75th percentile: 53-68)], 46 (36%) patients had ID. Women, patients with higher plasma brain natriuretic peptide levels (>400 pg/ml) and with right ventricular systolic dysfunction presented ID more frequently (p < 0.05 for all). At 225 ± 139 days of follow-up, the composite endpoint occurred in 15 (12%) patients. It was more frequent in ID (24 vs. 5%, p = 0.001) and anemic patients (25 vs. 8%, p = 0.014). In a Cox regression analysis, ID was associated with a higher likelihood of composite endpoint occurrence (HR 5.00, 95% CI 1.59-15.78, p = 0.006). In a multivariable analysis adjusted for clinical variables, including the presence of anemia, ID remained a significant predictor of the composite endpoint (HR 5.38, 95% CI 1.54-18.87, p = 0.009). CONCLUSION: In a CHF outpatient population, ID carried a higher risk of unfavorable outcome, irrespectively of the presence of anemia.

Cardiopulmonary exercise testing in clinical practice: Principles, applications, and basic interpretation.

Cardiopulmonary exercise testing (CPET) provides a noninvasive and integrated assessment of the response of the respiratory, cardiovascular, and musculoskeletal systems to exercise. This information improves the diagnosis, risk stratification, and therapeutic management of several clinical conditions. Additionally, CPET is the gold standard test for cardiorespiratory fitness quantification and exercise prescription, both in patients with cardiopulmonary disease undergoing cardiac or pulmonary rehabilitation programs and in healthy individuals, such as high-level athletes. In this setting, the relevance of practical knowledge about this exam is useful and of interest to several medical specialties other than cardiology. However, despite its multiple established advantages, CPET remains underused. This article aims to increase awareness of the value of CPET in clinical practice and to inform clinicians about its main indications, applications, and basic interpretation.

Loop diuretic discontinuation in chronic heart failure patients: A retrospective study.

INTRODUCTION AND OBJECTIVES: The use of loop diuretics is central in managing congestion in heart failure (HF), but their impact on prognosis remains unclear. In euvolemic patients, dose reduction is recommended, but there is no recommendation on their discontinuation. This study aims to assess the impact of loop diuretic discontinuation on the prognosis of outpatients with HF with reduced ejection fraction. METHODS: This retrospective cohort study collected data from medical records of patients followed in an outpatient HF clinic at a university hospital center. Patients were included if they had been on loop diuretics and these were discontinued. Demographic, clinical and laboratory data were collected, and number and type of congestive events during the one-year period after discontinuation were recorded. RESULTS: Among 265 patients on loop diuretics, almost half (129) discontinued them at some point. Patients had optimized medical therapy, low median age, low New York Heart Association class, low B-type natriuretic peptide values, normal blood pressure, controlled heart rate and kidney function within normal limits. Among 122 patients with one year of follow-up, 18 (14.8%) had a congestive event. Fifteen events (83.3%) were low-dose diuretic reinitiation at a scheduled visit. There were only three worsening heart failure events (2.5%) during the one-year period. A significant improvement in kidney function from discontinuation to the one-year follow-up appointment was also observed. CONCLUSIONS: In our cohort, loop diuretic discontinuation was possible and safe in a large proportion of patients. The results should be interpreted with caution and cannot be extrapolated to a broader population of HF patients.

An Unexpected Cause of Acute Abdomen Late After Heart Transplantation-A Case Report.

Cytomegalovirus (CMV) infection is a frequent complication after a solid organ transplant, and in 86% of the cases, CMV disease occurred during the first 6 months after transplantation. Invasive CMV infections may be present as ulcerative infections of the upper gastrointestinal tract with esophagitis, gastritis, and ulcerations of the duodenum and the small bowel; however, CMV infections of the pancreatobiliary system, especially papillitis, are rarely observed. We present a case report of a man who underwent a heart transplant 6 years before, with a clinical picture of duodenitis and a simultaneous pseudotumor of major duodenal papilla who developed signs of acute abdomen caused by gastrointestinal CMV infection, successfully treated with medical therapy with valganciclovir. There is an urgent need for developments in CMV and solid organ transplantation to stratify the risk of late-onset CMV disease.

Cutaneous Tuberculosis in Heart Transplant.

Tuberculosis is a disease with a significant global burden in terms of morbidity and mortality. It usually presents as a pulmonary disease but can occasionally have extrapulmonary presentations. Immunosuppressed people are at an increased risk of tuberculosis and more frequently have atypical manifestations of the disease. Cutaneous involvement is estimated to occur in only 2% of extrapulmonary presentations. We report a case of a heart transplant recipient with disseminated tuberculosis who initially presented with cutaneous manifestations in the form of multiple abscesses that were mistaken for a community-acquired bacterial infection. The diagnosis was made after positive nucleic acid amplification testing and cultures for Mycobacterium tuberculosis from the drainage of the abscesses. After initiating antituberculous treatment, the patient had 2 instances of immune reconstitution inflammatory syndrome. A combination of diminished immunosuppression due to discontinuation of mycophenolate mofetil in the setting of acute infection, rifampin drug interactions with cyclosporine, and the beginning of treatment of tuberculosis all contributed to this paradoxical worsening. The patient responded favorably to increased glucocorticoid therapy and showed no signs of treatment failure after 6 months of antituberculous therapy.

Total thyroidectomy in a patient awaiting heart transplant with amiodarone-induced thyrotoxicosis: A case report.

Thyroid function may have a severe impact in cardiac function. Herein, we present the case report of a 53-year-old male patient awaiting heart transplant with amiodarone induced thyrotoxicosis that presented a marked improvement of his cardiac function after total thyroidectomy.

[Prevalence of late complications and survival of patients undergoing cardiac transplantation: a single-center experience]. / Complicações tardias e sobrevivência após transplantação cardíaca: análise de um centro hospitalar.

INTRODUCTION: Heart transplantation is the treatment of choice in severe heart failure despite maximal medical therapy, which has no other surgical alternatives and exhibiting no contraindications. The aim of this study was to analyze the prevalence of late complications and survival of patients undergoing cardiac transplantation at our Hospital Center. METHODS: We evaluated 78 patients (mean age 43 ± 15 years) transplanted at our center between February 1987 and December 2011, with a mean follow-up of 6 years. RESULTS: Of late complications after heart transplantation, allograft vascular disease was detected in 10 patients ( 12.8%), was the one with impact on mortality, being responsible for four deaths. The most frequent complication was hypertension in 54.6% of cases, followed by dyslipidemia (47.4%), renal failure (47.4%), diabetes mellitus (21.8%) and neoplasms (11.5%). Atrial tachyarrhythmias was observed in eight patients (10.3%). The overall survival of our population at first and tenth year after heart transplantation was 81% and 69%, respectively. The mean survival of patients was 15.6 years (Cl 95%: [12,6-18,7]).. There were 23 deaths (29.5%), nine (11.5%) of which occurred within the first 30 days after transplantation. CONCLUSION: Cardiac transplantation remains a valid therapeutic option for patients with end-stage heart disease. Our center had a heart transplant survival rate and incidence of late complications similar to those seen in international registries.

The impact of the COVID-19 pandemic on acute coronary syndrome admissions to a tertiary care hospital in Portugal.

INTRODUCTION AND OBJECTIVES: The coronavirus SARS-CoV-2 (COVID-19) pandemic has been an unmatched challenge to global healthcare. Although the majority of patients admitted with acute coronary syndrome (ACS) may not be infected with COVID-19, the quarantine and public health emergency measures may have affected this particular high risk group. The objective of this study is to assess the impact of the early period of the COVID-19 pandemic on ACS admissions and clinical course in a tertiary care hospital in Portugal's most affected region. METHODS: This retrospective, case-control study included patients admitted with a diagnosis of ACS during March and April 2020 (pandemic group) and in the same period in 2019 (control group). Clinical course and complications were also assessed. RESULTS: During the pandemic, there were fewer ACS admissions but presentation was more severe, with a larger proportion of acute ST-elevation myocardial infarctions (54.9% vs. 38.8%, p=0.047), higher maximum troponin levels and greater prevalence of left ventricular systolic dysfunction at discharge (58.0% vs. 35.0%, p=0.01). In this population, although not statistically significant, it was observed a delay between the onset of symptoms and percutaneous coronary intervention, which may traduce a deferred search for urgent medical care during the pandemic. CONCLUSION: The lockdown phase of COVID-19 pandemic was associated with fewer and more severe ACS in a Tertiary Care Hospital in Portugal's most affected region by the pandemic.

Hyperkalemia as a limiting factor of neurohormonal blockade/modulation in everyday clinical practice.

INTRODUCTION AND OBJECTIVES: Neurohormonal blockade (NB)/modulation is the combination of two renin-angiotensin-aldosterone system inhibitors (RAASi) with a beta blocker. It is the core therapy for heart failure with reduced ejection fraction (HFrEF). While improving long term prognosis, it also induces hyperkalemia (serum K+ >5.0 mEq/L) due to RAASi effects. This may cause lethal arrhythmias and increase mortality in the short term. Thus, hyperkalemia frequently leads to withholding or reducing the intensity of neurohormonal blockade/modulation, which is associated with worsening long term prognosis. We assessed the relevance of hyperkalemia as a limiting factor of neurohormonal blockade/modulation in real life clinical conditions. METHODS: We reviewed the medical records of HFrEF patients attending a HF clinic at a tertiary Portuguese hospital during 2018 (n=240). The number of patients not tolerating maximal neurohormonal blockade/modulation due to hyperkalemia was determined. The incidence and characteristics of hyperkalemia episodes were also assessed. RESULTS: Only six patients (3%) achieved maximal doses of neurohormonal blockade/modulation. Hyperkalemia was the limiting factor in 48 (20%) patients. A total of 185 hyperkalemia episodes occurred in 100 (42%) patients. Forty-five (24%) episodes were moderate or severe (serum K+ >5.5 mEq/L). In these HFrEF patients, the co-existence of hypertension, diabetes or renal failure was associated with the occurrence of hyperkalemia. CONCLUSIONS: In daily clinical practice, hyperkalemia is frequent and limits neurohormonal blockade/modulation by leading to the withholding or reducing of the intensity of RAAS inhibition. Considering the negative prognostic impact associated with sub-optimal neurohormonal blockade/modulation, addressing hyperkalemia is an important issue when treating HFrEF patients.

Comparison of levosimendan, NO, and inhaled iloprost for pulmonary hypertension reversibility assessment in heart transplant candidates.

AIMS: Assessing reversibility of pulmonary vascular changes through vasoreactivity testing (VRT) optimizes end-stage heart failure patient selection for heart transplant. All efforts should be made to unload the left ventricle and reduce pulmonary vascular resistance to effectively exclude irreversible pulmonary hypertension. METHODS AND RESULTS: We reviewed our centre's cardiac transplant registry database (2009-2017) for VRT and compared haemodynamic responses with 40 ppm inhaled NO (n = 14), 14-17 µg inhaled iloprost (n = 7), and 24 h 0.1 µg/kg/min intravenous levosimendan (n = 14). Response to levosimendan was assessed by repeat right heart catheterization within 72 h. Baseline clinical and haemodynamic features were similar between groups. VRT was well tolerated in all patients. All drugs effectively reduced pulmonary artery pressures and transpulmonary gradient while increasing cardiac index, although levosimendan had a greater impact on cardiac index increase (P = 0.036). Levosimendan was the only drug that reduced pulmonary artery wedge pressure (P = 0.004) and central venous pressures (P < 0.001) and increased both left and right ventricular stroke work indexes (P = 0.020 and P = 0.042, respectively) and cardiac power index (P < 0.001) compared with NO and iloprost. Right ventricular end-diastolic pressures and central venous pressure were only decreased by levosimendan. The rate of positive responses (≥10 mmHg decrease or final mean pulmonary artery pressure ≤40 mmHg with increased/unaltered cardiac index) was lower with inhaled iloprost (14%) than with either levosimendan or NO (71% and 64%, respectively; P < 0.05). CONCLUSIONS: Levosimendan may be a safe and effective alternative for pulmonary hypertension reversibility assessment or a valuable pre-test medical optimization tool in end-stage heart failure patient assessment for heart transplantation offering extended haemodynamic benefits. Whether it increases the rate of positive responses or allows a better selection of candidates to heart transplantation remains to be established.

Sacubitril/valsartan in everyday clinical practice: an observational study based on the experience of a heart failure clinic.

BACKGROUND: Heart failure (HF) is a growing public health problem. Sacubitril/valsartan is now recommended to be used in persistently symptomatic patients with left ventricular ejection fraction (LVEF) <40%, replacing angiotensin-converting enzyme inhibitors (ACEis)/angiotensin receptor blockers (ARBs). In the present study, we aimed to characterise the challenges of sacubitril/valsartan use in everyday clinical practice. METHODS: We assessed the medical records of patients with HF and reduced ejection fraction eligible for sacubitril/valsartan attending a HF clinic at a Portuguese University Hospital during 2018 (n=152). The number of eligible patients receiving the drug and the reasons for not prescribing sacubitril/valsartan were evaluated. Additionally, we assessed the tolerability of maximal doses of sacubitril/valsartan. New York Heart Association functional class (NYHA class) and LVEF before and after up-titration to maximal tolerated sacubitril/valsartan dose were compared. Median follow-up was 41 months. RESULTS: Of the 152 included patients, 75 (49%) were prescribed the drug. The two main reasons for non-prescription were patient financial barriers (31%) and hypotension (27%). Only 33% of patients on sacubitril/valsartan did reach maximal dose. Hypotension was the main limiting factor for dose optimisation. Duration of sacubitril/valsartan treatment showed a positive association with LVEF improvement during follow-up (6.6% absolute LVEF increase/year). NYHA functional class improved significantly from baseline through the end of follow-up. CONCLUSIONS: In every-day clinical practice, although sacubitril/valsartan was associated with a marked improvement in NYHA class and in LVEF, important financial and clinical barriers to the implementation of this therapy were identified.

Temporal trends of lipid control in very high cardiovascular risk patients.

INTRODUCTION: Since 2011, the European guidelines have included a specific low-density lipoprotein cholesterol (LDL-C) target, <70 mg/dl, for very high cardiovascular risk (CVR) patients. However, registries have shown unsatisfactory results in obtaining this level of adequate lipid control. OBJECTIVES: To assess temporal trends in the use of lipid-lowering therapy (LLT) and attainment of adequate control in very high CVR patients since 2011. METHODS: We performed a retrospective observational study including very high CVR patients admitted in two periods: the first two years since the 2011 guidelines (2011/2012) and five years later (2016/2017). Lipid values, LLT, clinical variables and adequate lipid control rates were analyzed. RESULTS: A total of 1314 patients were reviewed (2011/2012: 638; 2016/2017: 676). Overall, 443 patients (33.7%) were not under LLT and only a slight improvement in drug prescription was observed from 2011/2012 to 2016/2017. In LLT users, the proportion of high-intensity LLT increased significantly in the later years (6.4% vs. 24.0%; p<0.001), but this was not associated with adequate lipid control. Overall, mean LDL-C was 95.4±37.2 mg/dl and adequate control was achieved in 320 patients (24.4%), without significant differences between 2011/2012 and 2016/2017 (p=0.282). Independent predictors of adequate control were male gender, older age, diabetes, chronic kidney disease, prior acute coronary syndrome, prior stroke and LLT, while stable coronary artery disease was associated with higher risk of failure. CONCLUSION: Even after the introduction of specific LDL-C targets, these are still not reached in most patients. Over a five-year period, LLT prescription only improved slightly, while adequate lipid control rates remained unchanged.

Survival of patients with familial dilated cardiomyopathy on optimal heart failure therapy.

BACKGROUND: Familial dilated cardiomyopathy (FDCM) is characterized by clinical and genetic heterogeneity. There are still few survival studies concerning this subgroup of patients. AIM: To determine the prognosis of patients with FDCM on optimal medical therapy and attending a heart failure clinic. METHODS: This is a prospective study including patients with FDCM, defined according to the guidelines of the European Society of Cardiology. Cardiovascular morbidity and all-cause mortality were evaluated. RESULTS: Thirty-six patients, 23 (64%) men, were followed for 3.8 +/- 2.5 years. Age at baseline was 42 +/- 14 years and 67% were in NYHA class II. In 22% heart failure symptoms first occurred after a respiratory infection, and in 6%, after pregnancy/delivery. Most patients were in sinus rhythm (89%) and 33% had left bundle branch block (LBBB). Baseline left ventricular (LV) ejection fraction was 28 +/- 9%, LV end-diastolic diameter was 68 +/- 8 mm and left atrial dimension was 46 +/- 9 mm. Baseline serum sodium was 140 +/- 3 mEq/l. All patients were taking angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-II receptor blockers (ARBs), 81% beta-blockers and 47% spironolactone. During follow-up, 5 patients died, 4 underwent heart transplantation and one received an implantable cardioverter-defibrillator. Five-year survival was 68%. CONCLUSIONS: Five-year survival of our patients with FDCM, under optimal medical therapy, was similar to that of other forms of nonischemic DCM reported in the literature.

I-123-MIBG cardiac uptake imaging, in familial dilated cardiomyopathy.

UNLABELLED: The pathological significance of myocardial adrenergic activity in patients with heart failure is well documented. No previous study has assessed the usefulness of I123-metaiodobenzylguanidine (123I-MIBG) cardiac uptake imaging for the evaluation of familial dilated cardiomyopathy (DCM). OBJECTIVE: To evaluate cardiac adrenergic activity, using 123I-MIBG cardiac uptake imaging, in members of a genotyped family with DCM. METHODS: Clinical evaluation, 12-lead ECG, 2D echocardiogram, heart rate variability analysis by 24h Holter, plasma B-type natriuretic peptide (BNP) measurements and 123I-MIBG cardiac imaging were performed in all participants. Anterior projection planar images and single photon emission computed tomographies of the thorax were obtained 20 min and 4 hours after the intravenous administration of 370 MBq of 123I-MIBG (early and late images). Heart/mediastinal (H/M) ratio and myocardial washout (MW) rate were obtained based on the anterior planar images. In polar maps, segmental uptake of 123I-MIBG was evaluated using a 4-grade visual score: grade 1 - uptake > 75% of maximum myocardial uptake (MMU); grade 2 - uptake 51-75% of MMU; grade 3 - uptake 26-50% of MMU; grade 4 - uptake < or = 25% of MMU. RESULTS: Eleven adults were included: 4 with DCM, 4 with isolated left ventricular enlargement (LVE), and 3 with normal echocardiogram. Patients with DCM and LVE presented higher MW rates, lower H/M ratios and higher visual score grades than those with normal 2D echocardiograms. One patient with a normal echocardiogram but carrying the disease locus also presented an abnormal MIBG cardiac scintigram. CONCLUSION: Patients with the phenotypic expression of the disease (DCM and LVE) and even carriers of the DCM gene with normal echocardiograms may present an abnormal MIBG cardiac scintigram, probably reflecting cardiac adrenergic hyperactivity. If confirmed in larger numbers, this method may be useful for the evaluation of DCM families.

Effectiveness and safety of levosimendan in clinical practice.

BACKGROUND: In previous randomized studies levosimendan improved hemodynamics and clinical course, with a still unclear effect on prognosis. There are, however, few data regarding its effects when used in daily practice. AIMS: We evaluated the clinical effectiveness and safety of levosimendan in the treatment of acute systolic heart failure (SHF) in daily practice conditions. METHODS: In this prospective, multicenter, nonrandomized trial, a continuous infusion of levosimendan (0.05 microg/kg/min-0.2 microg/kg/min) was administered for 24 hours. An optional loading dose of 12 microg/kg over 10 minutes was used. The primary combined endpoint of clinical effectiveness (as defined by a eight-variable clinical score) and safety (defined by the absence of serious adverse events) was assessed at 24 hours after the beginning of treatment; a second similar primary combined endpoint was assessed at 5 days. RESULTS: One hundred and twenty-nine consecutive patients requiring inotropes despite optimal oral background heart failure therapy were recruited. The primary endpoint was reached in 80.6% at 24 hours and in 79.7% at 5 days. During the six months before levosimendan the number of patient days of hospitalization for heart failure was 14.9 +/- 14.6 versus 3.1 +/- 7.6 during the six months following levosimendan (p < 0.001). CONCLUSIONS: In daily practice, levosimendan was clinically effective and safe in 80.6% and 79.7% of patients with acute SHF at 24 hours and 5 days respectively after the beginning of treatment. A marked reduction in the number of days of hospitalization for heart failure was also seen during the subsequent six months.

Genetic variants identified by target next-generation sequencing in heart transplant patients with dilated cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Dilated cardiomyopathy (DCM) is a myocardial disease that can progress to a terminal stage, requiring heart transplantation. In this work we aim to contribute to knowledge of genetic variants in adult patients undergoing heart transplantation due to end-stage DCM, reporting the results obtained in our single-center tertiary hospital series using target next-generation sequencing (NGS). METHODS AND RESULTS: Genetic variants were screened in 15 genes, preselected based on variants previously identified in DCM patients. Thirteen unrelated patients were included, nine (69%) male, mean age at diagnosis 33±13 years, eight (62%) with familial DCM. Nine genetic variants were identified in six (46%) patients: five in LMNA, two in LBD3, one in TNNT2 and one in TCAP. These variants were new in most patients. The majority were classified as of uncertain significance. Two patients were double and triple heterozygotes in the LBD3 and LMNA genes, respectively. CONCLUSION: Our results highlight the potential of NGS in the genetic characterization of DCM patients. LMNA is one of the most frequently mutated genes and should be included in all target gene assessments of end-stage DCM patients until more data are available.

Left ventricular reverse remodeling in dilated cardiomyopathy- maintained subclinical myocardial systolic and diastolic dysfunction.

In idiopathic dilated cardiomyopathy (DCM), myocardial deformational parameters and their relationships remain incompletely characterized. We measured those parameters in patients with DCM, during left ventricular reverse remodeling (LVRR). Prospective study of 50 DCM patients (in sinus rhythm), with left ventricular ejection fraction (EF) <40%. LVRR was defined as an increase of ten units of EF and decrease of diastolic left ventricular diameter (LVDD) in the absence of resynchronization therapy. Performed morphological analysis, myocardial performance quantification (LV and RV Tei indexes) and LV averaged peak systolic longitudinal strain (SSR long) and circumferential strain (SSR circ). At baseline, mean EF was 25.4 ± 9.8%, LVDD was 62.4 ± 7.4 mm, LVDD/BSA of 34.2 ± 4.5 mm/m2 and 34% had MR grade >II/IV. LVRR occurred in 34% of patients within 17.6 ± 15.6 months and was associated with a reduced rate of death or heart failure hospitalization (5.9% vs. 33.3; p = 0.03). Patients with LVRR had a final EF of 48.9 ± 7.9% (Δ LV EF of 22.4%) and there was a significant decrease (p < 0.05) in: LVDD/BSA, LV systolic diameter/BSA, LV diastolic volume, LV systolic volume, LV mass; an increase (p < 0.05) in sphericity index. However, measures of diastolic function (LA volume/BSA, e'velocity and' E/e'ratio), final LV and RV Tei indexes were not significantly different from baseline. Additionally, final SSR circ and SSR long values were not different from basal. Patients who recovered EF >50% (n = 10), SSR circ and SSR long were inferior to normal. Improvement in EF occurred in one-third of DCM pts and was associated with a decrease of major cardiac events. There was an improvement of diastolic and systolic volumes and in sphericity index, confirming truly LV reverse reshaping. However, myocardial performance indexes, SSR long and SSR circ in reverse-remodeled DCM were still abnormal, suggesting a maintained myocardial systolic and diastolic dysfunction.