Rationale and Aim of the Study Cardiac involvement is very common in transfusion dependent beta-thalassemia (TDT) and is mainly related to cardiac iron overload and myocardial damage with progressive systolic and diastolic dysfunction of the left and right ventricles. It has also been observed that in patients with TDT, cardiac fibrosis may not be directly related with iron deposition and is associated with a higher risk of cardiovascular complications. CMR is the gold standard for the evaluation of iron overload with T2* sequences and for the detection of cardiac fibrosis with late gadolinium enhancement (LGE) Apart from the use of imaging techniques, it is well known that the fibrosis and inflammation biomarkers, Galectin-3 (Gal-3) and ST2, are involved in the early stages and progression of various fibrosis-related diseases, such as heart failure, liver cirrhosis, renal and pulmonary fibrosis. To date, the role of Gal-3 and ST-2 as markers of cardiac fibrosis and myocardial damage and their possible association with myocardial damage detected by CMR, has never been studied in patients with TDT. The aim of the study was to evaluate the role of Gal-3 and ST-2 in the characterization of myocardial involvement, especially cardiac fibrosis in a population of TDT patients. Methods Twenty-six patients with a confirmed diagnosis of TDT, based on βglobin genotype, transfusion history and clinical evaluation, undergoing periodic blood transfusion and iron-chelation therapy were enrolled. None presented overt clinical signs of heart failure, CMR with T2* technique, haematochemical routine, ferritinemia assay, Gal-3 and ST-2 assay and dynamic ECG Holter were performed on all patients. Results Both Gal-3 and ST-2 have a positive correlation with systemic markers of inflammation, such as erythrocyte sedimentation rate (ESR) (10 0.65 p 0.042 and 26 0.58 p 0.002, respectively). A Gal-3 value > 17.9 ng/mL is associated with inflammatory comorbidities such as diabetes (p 0.010) and with cardiac iron overload evaluated using CMR T2 * technique (p 0.007) (Fig.1). A positive correlation was also observed with medium values of ferritine (rho 0.46 p 0.017). There was a statistically significant positive association between an ST-2 value > 35 ng/mL and cardiac fibrosis detected by CMR (p 0.020) (Fig. 2). Higher ST-2 values have been detected in patients with cardiac fibrosis when compared to those without (42 ± 14 vs 28 ±8 p 0.006). Conclusions The present study demonstrates that, in a small population of TDT patients undergoing periodic blood transfusion and iron-chelation therapy with no overt clinical signs of heart failure, Gal-3 appears to be a marker of cardiac iron overload detected with CMR T2* sequences and systemic inflammation associated with higher ESR values and inflammatory comorbidities such as diabetes mellitus. ST-2 is a marker of cardiac fibrosis detected by CMR LGE.
Introduction Long QT Syndrome (LQTS) is a rare cardiac channelopathy characterized by a high risk of lethal arrhythmias. Pregnancy and post–partum period may induce an increased vulnerability, so an appropriate prevention is needed. Case presentation A 31–year–old primigravid woman at 36 weeks of pregnancy was referred to our Cardiomyopathy Unit located in a tertiary centre for LQTS screening. The patient had a family history of LQTS (mother and brother) and Brugada syndrome (cousin). The 12–lead ECG (Figure 1) showed a long QT interval (QT corrected: 508 msec), while the transthoracic echocardiogram was unremarkable. The genetic test performed is still ongoing. The patient reported sporadic episodes of palpitations at rest and the following 24–hour ambulatory ECG revealed a very high burden of polymorphic ventricular arrhythmias, with about sixteen thousand premature ventricular complexes and a non–sustained ventricular tachycardia. Therefore, due to the patient’s high–risk for life–threatening arrhythmic events, metoprolol 25 mg o.d. was initiated and a wearable cardioverter defibrillator (WCD) with remote monitoring was provided during pregnancy and labour. After 3 weeks a caesarean delivery in epidural analgesia was performed and managed by a pregnancy heart team including a cardiologist, gynaecologist, obstetrician, and anaesthetist in close cardiac monitoring. No maternal or infant complications occurred, without arrhythmic episodes detected by the device. The WCD will be maintained also in the post–partum period with a narrow follow–up of the patient. Discussion In pregnant high–risk LQTS patients, beta–blockers are the first line therapeutic option, but they do not able to interrupt lethal arrythmias in out–of–hospital setting. The implantable cardiac defibrillator is not the best choice, due to the concerns about radiation exposure and anaesthesia during pregnancy. Moreover, lead–associated thrombosis and infections during pregnancy were reported in literature. The WCD is the ideal option in these cases. It is an external device suited for transient higher arrhythmic risk periods. Moreover, it allows a remote monitoring of arrhythmic burden (figure 2) which may help clinicians in guiding the follow–up and the therapeutic management of the patient. One drawback is the need of continuous patient’s compliance. Finally, a pregnancy heart team is mandatory in presence of these rare cardiac syndromes.
Funding Acknowledgements Type of funding sources: None. Background Cardiac involvement is common and the leading cause of death in transfusion dependent beta-thalassemia (TDT) patients. It is mainly related with cardiac iron overload and myocardial fibrosis, leading to a progressive cardiac damage which evolves towards a severe deterioration of systo-diastolic cardiac function. Moreover, myocardial fibrosis may not be directly related with the degree of cardiac iron deposition and it is associated with a high risk of cardiovascular complications. To date, Cardiac Magnetic Resonance (CMR) is the gold standard for the evaluation of iron overload with T2* sequences and for the detection of myocardial fibrosis with late gadolinium enhancement (LGE). However, CMR is not always feasible. Left Ventricular Global Longitudinal Strain (LV GLS) with Speckle Tracking Echocardiography (STE) is a non-invasive imaging tool, which may play a key role in the early recognition of subclinical myocardial damage and dysfunction (figure 1). This may help clinicians in guiding the diagnostic algorithm and the therapeutic approach. Purpose The aim of this study was the evaluation of the role of LV GLS with STE in the characterization of myocardial involvement in a population of TDT patients. Methods An observational, cross-sectional, monocentric study was conducted by the enrollment of 38 patients with TDT from the outpatient clinic of hematology of our hospital. Each patient underwent a thorough clinical evaluation including laboratory testing, transthoracic echocardiogram (TTE) with LV GLS-STE and contrast enhanced CMR with T2* technique and LGE sequences. A T2* value < 20 msec was set to define the presence of iron overload. Results The mean age of patients was 38 ± 10 years with a slight prevalence of female sex (53% vs 47% respectively). Cardiovascular risk factors were rarely present, the most common was arterial hypertension in about 12% of patients. The TTE pointed out a left ventricular ejection fraction within the normal range (mean value of 59 ± 5%). As regards LV GLS, the mean value was −19,8 ± 2,9%. Cardiac iron overload was observed in 18% of patients, while about 15% of patients had cardiac fibrosis. A mild clinical but not statistically significant difference was observed between LV GLS values in patients with cardiac iron overload as compared to patients without (−18,3 ± 2,3% vs −20,1 ± 3,0%; p value=0,147). A statistically significant difference was documented between LV GLS values in patient with cardiac fibrosis as compared to patient without LGE (−16.4 ± 1.7% vs −19,8 ± 2,4%; p value=0,004). A LV GLS cut-off value of −18,3% was found by the analysis of ROC curve with an AUC of 0,904 (95% CI: 0,798–1,000) (figure 2). Conclusions In a small population of TDT patients we observed that LV GLS with STE may be a promising tool in the early identification of cardiac damage to provide useful informations for diagnostic and therapeutic approach.
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