Aims The aim of this study was to evaluate the effect of microsomal triglyceride transfer protein inhibitor (lomitapide) in patients with homozygous familial hypercholesterolaemia. Methods and results In 12 homozygous familial hypercholesterolaemia patients treated with lipid-lowering drugs ± biweekly lipoprotein apheresis sessions (nine patients), daily lomitapide was added. The lipid profile (total cholesterol, low-density lipoprotein cholesterol, triglycerides, high-density lipoprotein cholesterol) before and after lomitapide treatment was evaluated. The follow-up period with lomitapide treatment was 3–24 months (13.8 ± 7.9). The median baseline low-density lipoprotein cholesterol level was 900 mg/dl (348–1070), after lipid-lowering drugs therapy was 383.5 mg/dl (214–866) and after lipid-lowering drugs + time-averaged level was 288 mg/dl (183.7–716.6). The addition of lomitapide lowered low-density lipoprotein cholesterol levels further by 56.8% compared to lipid-lowering drugs alone (mean reduction 262, 95% confidence interval (105.5–418.7), p = 0.005) and by 54% (mean reduction 182.9, 95% confidence interval (−342 – −23), p = 0.031) comparing to lipid-lowering drugs + lipoprotein apheresis (time-averaged level). The time-averaged level of low-density lipoprotein cholesterol in lipid-lowering drugs + lipoprotein apheresis patients compared with lipid-lowering drugs + lomitapide was 54% in favour of lomitapide ( p = 0.031). Conclusions Treatment with lomitapide in homozygous familial hypercholesterolaemia patients has a beneficial effect with a constant decrease of low-density lipoprotein cholesterol by 57% compared with classical lipid-lowering therapy and by 54% compared with classical lipid-lowering therapy and time-averaged level of low-density lipoprotein cholesterol.
Background Myocardial iron overload in patients with thalassemia major (TM) is one of the most important complications. The purpose of the study was to identify advanced echocardiography parameters for early identification of myocardial dysfunction during follow-up of patients with TM. Methods Forty TM patients who were 41 ± 5 years old were included in the study and divided into two groups according to cardiac magnetic resonance T2* results (Group 1: Τ2* > 25 ms, Group 2: Τ2* ≤ 25 ms). Liver T2* parameters were also measured. Conventional and deformational echocardiographic parameters were measured at baseline and approximately 2 years later. Results Thirty-two patients had Τ2* = 34 ± 4 ms (Group 1), and 8 had Τ2* = 17 ± 9 ms (Group 2). Blood consumption was 185 ± 60 and 199 ± 37 ml/kg/yr (p = 0.64), and liver T2* was 4 ± 5 and 17 ± 21 ms (p = 0.01) in Groups 1 and 2, respectively. At baseline, Group 1 had better left ventricular global longitudinal strain (GLS) (− 22 ± 3 vs. − 18 ± 5, p = 0.01) and similar left ventricular ejection fraction (LVEF) (62 ± 5% vs. 58 ± 10%, p = 0.086) than Group 2. At the 28 ± 11-month follow-up, LVEF, GLS, and T2* values in Group 1 (63 ± 3%, − 21 ± 3%, 34 ± 4 ms) and Group 2 (56 ± 11%, − 17 ± 4%, 17 ± 9 ms) did not change significantly compared to their corresponding baseline values. In 8 patients from Group 1, a worsening (> 15%) in LS (p = 0.001) was detected during follow-up, with a marginal reduction in LVEF. Conclusions GLS seems to be an efficient echocardiographic parameter for detecting hemochromatosis-related cardiac dysfunction earlier than LVEF. It also seems to be affected by other factors (free radical oxygen, immunogenetic mechanisms or viral infections) in a minority of patients, underscoring the multifactorial etiology of cardiomyopathy.
Introduction Left anterior descending velocity reserve (LADVR) by transthoracic echocardiography (TTE) has been proposed for cardiovascular risk stratification in observational prospective studies. Aim of the current study was to interrogate the prognostic consistency and coherence of the existing LADVR data by the means of meta-analysis of relevant studies. Methods A systematic research through electronic databases was performed for prospective studies with patients with known or suspected coronary artery disease (CAD) who had LADVR data. The exposure was abnormal values of LADVR as defined in each study and the outcome was the occurrence of cardiovascular event or death (CE-D). Statistical index considered were the risk ratio (RR) for CE-D of patients with abnormal vs. normal LADVR, as obtained from Cox proportional hazard models. A meta-analysis of these studies using random-effects model was performed to evaluate the pooled prognostic value of abnormal LADVR. Results Fifteen studies with 13050 patients (59.7% male; mean age 64.2 years; mean follow-up 25.1 months) were included in this meta-analysis. Every study used adjustments for every established risk factor for CE-D (age, hypertension, diabetes, dyslipidemia, smoking habits, wall motion abnormalities during stress echo). The abnormal value of LADVR was associated with an increased risk of CE-D (RR=3.33, 95% CI: 2.54–4.37, p-value <0.001). Moderate heterogeneity was observed between studies (Q=35.83, p-value=0.001, I2=60.9%) which was further investigated with sensitivity analysis, subgroup analysis and meta-regression. Conclusions Meta-analytic data for the cardiovascular risk stratification based on dichotomous LADVR data provide robust evidence for efficient prognostic yield. The current results support the broader clinical application of the LADVR. LADVR meta-analysis forestplot Funding Acknowledgement Type of funding source: None
Funding Acknowledgements Type of funding sources: None. Background Intra-aortic balloon pump (IABP) can be used as circulatory support in order to stabilize haemodynamically compromised patients as either a bridge to therapy or to further mechanical support. Based on the current literature its use should be limited to up to two weeks and there are not enough data regarding its long term use’s efficacy and possible complications. Purpose To review the possible complications of the long-term use of IABP Methods We restrospectively analysed the data from 24 consecutive patients with end-stage heart failure (ESHF) who received long-term IABP support and recorded the complications during their hospitalization as well as their outcome. Results 24 patients (14 male and 10 female) were included. In 5 of them ESHF was attributed to ischemic cardiomyopathy and the in 19 to dilated cardiomyopathy. Their mean age was 45.6+/-14 years. The mean duration of IABP support was 70.2 days (minimum 30days maximum 192 days). The mean ejection fraction (EF) was 20%. Regarding the pharmacological therapy, 12/24 patients were on dobutamine, 4/24 on dobutamine and milrinone and 8/24 on dobutamine and noradrenaline. Regarding the clinical course of these patients, 7/24 underwent heart transplantation, 2/24 managed to wean from IABP, 5/24 received left lentrivular assist device (LVAD), 6/24 received biventricular assist veice (BiVAD) and 4/24 died. In terms of complications they were recorded as following : infection 7/24, bleeding 3/24, thrombosis 4/24, heparin-induced thrombocytopenia(HIT) 5/24, hematoma 4/24, ischemia 0/24 and rupture 1/24. Conclusions Although not indicated by the current guidelines, long term IABP can be used as a relatively safe circulatory support method.
Background Inappropriate shocks have been reported in approximately 1/3 of patients with implantable cardiac defibrillators (ICDs). We report an unusual case of inappropriate ICD shocks due to atrial fibrillation (AF) caused by a missed atrial septal defect (ASD) in a patient with a modified Bentall procedure. Case summary A 67-year-old Caucasian male, with an ICD and a history of a modified Bentall procedure 24 years ago, reported to our outpatient clinic with recurrent inappropriate ICD shocks due to episodes of fast AF. The transthoracic echocardiographic exam revealed 2 large aneurysms at the ostia of the coronary arteries. We performed further evaluation with transesophageal echocardiogram (TOE) and computed tomography (CT) angiography. The aneurysms measured on CT were 3.14*2.29 cm on the right ostium and 1.9*0.99 cm on the left. A large secundum-type atrial septal defect (ASD) of 1.5 cm was revealed that was missed in all previous echocardiographic studies. The therapeutic options of surgical closure of the ASD and repair of the aneurysms or a more conservative approach with percutaneous closure of the ASD and closer follow-up were discussed with the patient. The patient declined the surgical option due to high complication risk, and closure of the ASD with an Amplatzer device was performed 3 months later. A 3 -year Follow-up was uneventful. Conclusion It is of major importance to comprehensively and thoroughly assess patients before and after a surgical intervention to not miss other treatable conditions preoperatively and complications in the postoperative period.
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