The Link of Pancreatic Iron with Glucose Metabolism and Cardiac Iron in Thalassemia Intermedia: A Large, Multicenter Observational Study

In thalassemia major, pancreatic iron was demonstrated as a powerful predictor not only for the alterations of glucose metabolism but also for cardiac iron, fibrosis, and complications, supporting a profound link between pancreatic iron and heart disease. We determined for the first time the prevalence of pancreatic iron overload (IO) in thalassemia intermedia (TI) and systematically explored the link between pancreas T2* values and glucose metabolism and cardiac outcomes. We considered 221 beta-TI patients (53.2% females, 42.95 ± 13.74 years) consecutively enrolled in the Extension–Myocardial Iron Overload in Thalassemia project. Magnetic Resonance Imaging was used to quantify IO (T2* technique) and biventricular function and to detect replacement myocardial fibrosis. The glucose metabolism was assessed by the oral glucose tolerance test (OGTT). Pancreatic IO was more frequent in regularly transfused (N = 145) than in nontransfused patients (67.6% vs. 31.6%; p < 0.0001). In the regular transfused group, splenectomy and hepatitis C virus infection were both associated with high pancreatic siderosis. Patients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with altered OGTT. A pancreas T2* < 17.9 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for cardiac iron. Pancreas T2* values were not associated to biventricular function, replacement myocardial fibrosis, or cardiac complications. Our findings suggest that in the presence of pancreatic IO, it would be prudent to initiate or intensify iron chelation therapy to prospectively prevent both disturbances of glucose metabolism and cardiac iron accumulation.

Surgical outcome of Yasui procedure for preserving biventricular function: single centre experience

Background: Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect. Methods: Retrospective chart review analysis of all patients who had Yasui procedure (2008–2020) comparing midterm outcome of one versus two stage repair. Results: Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle–pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality. Conclusion: Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.

Cardiogenic shock complicating multisystem inflammatory syndrome following COVID-19 infection: a case report

Background With the high prevalence of COVID-19 infections worldwide, the multisystem inflammatory syndrome in adults (MIS-A) is becoming an increasingly recognized entity. This syndrome presents in patients several weeks after infection with COVID-19 and is associated with thrombosis, elevated inflammatory markers, hemodynamic compromise and cardiac dysfunction. Treatment is often with steroids and intravenous immunoglobulin (IVIg). The pathologic basis of myocardial injury in MIS-A, however, is not well characterized. In our case report, we obtained endomyocardial biopsy that revealed a pattern of myocardial injury similar to that found in COVID-19 cardiac specimens. Case presentation A 26-year-old male presented with fevers, chills, headache, nausea, vomiting, and diarrhea 5 weeks after his COVID-19 infection. His SARS-CoV-2 PCR was negative and IgG was positive, consistent with prior infection. He was found to be in cardiogenic shock with biventricular failure, requiring inotropes and diuretics. Given concern for acute fulminant myocarditis, an endomyocardial biopsy (EMB) was performed, showing an inflammatory infiltrate consisting predominantly of interstitial macrophages with scant T lymphocytes. The histologic pattern was similar to that of cardiac specimens from COVID-19 patients, helping rule out myocarditis as the prevailing diagnosis. His case was complicated by persistent hypoxemia, and a computed tomography scan revealed pulmonary emboli. He received IVIg, steroids, and anticoagulation with rapid recovery of biventricular function. Conclusions MIS-A should be considered as the diagnosis in patients presenting several weeks after COVID-19 infection with severe inflammation and multi-organ involvement. In our case, EMB facilitated identification of MIS-A and guided therapy. The patient’s biventricular function recovered with IVIg and steroids.

Multiparametric cardiac magnetic resonance assessmenti in sickle-beta-thalassemia

Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A., ApoPharma Inc.). Background. Sickle β-thalassemia (Sβ-thal) is a hereditary hemoglobinopathy resulting from the combined heterozygosity for sickle cell and β-thalassemia genes. Cardiac involvement in Sβ-thal patients has been poorly investigated. Aim. We aimed to evaluate myocardial iron overload and cardiac function by cardiovascular magnetic resonance (CMR) in patients with Sβ-thal. Methods. One hundred and eleven Sβ-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell disease (SCD) patients. Biatrial and biventricular function CMR parameters of Sβ-thal patients were compared with those of 111 healthy volunteers, matched by gender and age. Myocardial iron overload (MIO) was assessed by T2* technique. Cine images were acquired to quantify biventricular function. Macroscopic myocardial fibrosis was evaluated by late gadolinium enhancement (LGE) technique. Results. In Sβ-thal and SCD patients morphological and functional MR parameters were not significantly different, except for left atrial area and SVI (p = 0.023 and p = 0.048, respectively) that were significantly higher in SCD patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sβ-thal patients showed significantly higher bi-atrial and biventricular parameters except for LVEF that was significantly lower (Fig.1). Conclusions. The CMR analysis confirmed that Sβ-thal and SCD patients are phenotypically similar. Since Sβ-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sβ-thal/SCD-specific bi-atrial and biventricular reference values.

Short-Term CPAP Improves Biventricular Function in Patients with Moderate-Severe OSA and Cardiometabolic Comorbidities

Obstructive sleep apnea (OSA) is the most common form of sleep-disordered breathing, exhibiting an increasing prevalence and several cardiovascular complications. Continuous positive airway pressure (CPAP) is the gold-standard treatment for moderate-severe OSA, but it is associated with poor patient adherence. We performed a prospective study that included 57 patients with newly diagnosed moderate-severe OSA, prior to CPAP initiation. The objective of our study was to assess the impact of short-term CPAP on ventricular function in patients with moderate-severe OSA and cardiometabolic comorbidities. The patients underwent a clinical exam, ambulatory blood pressure monitoring and comprehensive echocardiographic assessment at baseline and after 8 weeks of CPAP. Hypertension, obesity and diabetes were highly prevalent among patients with moderate-severe OSA. Baseline echocardiographic parameters did not significantly differ between patients with moderate and severe OSA. Short-term CPAP improved left ventricular global longitudinal strain (LV-GLS), isovolumetric relaxation time, transmitral E wave amplitude, transmitral E/A ratio, right ventricular (RV) diameter, RV wall thickness, RV systolic excursion velocity (RV S‘) and tricuspid annular plane systolic excursion (TAPSE). Short-term CPAP improves biventricular function, especially the LV-GLS, which is a more sensitive marker of CPAP-induced changes in LV systolic function, compared to LVEF. All these benefits are dependent on CPAP adherence.

The acute effects of an ultramarathon on biventricular function and ventricular arrhythmias in master athletes

Funding Acknowledgements Type of funding sources: None. Background. Endurance sports practice has significantly increased over the last decades, with a growing proportion of participants older than 40 years. Although the benefits of moderate regular exercise are well known, concerns exist regarding the potential negative effects induced by extreme endurance sport. The aim of this study was to analyze the acute effects of an ultramarathon race on the ECG, biventricular function and ventricular arrhythmias in a population of master athletes. Methods. Master athletes participating in an ultramarathon (50 km, 600 meters of elevation gain) with no history of heart disease were recruited. A single-lead ECG was recorded continuously from the day before to the end of the race. Echocardiography and 12-lead resting ECG were performed before and at the end of the race. Results. The study sample consisted of 68 healthy non-professional master athletes. Compared with baseline, R-wave amplitude in V1 and QTc duration were higher after the race (p &lt; 0.001). Exercise-induced isolated premature ventricular beats were observed in 7% of athletes; none showed non-sustained ventricular tachycardia before or during the race. Left ventricular ejection fraction, global longitudinal strain (GLS) and twisting did not significantly differ before and after the race. After the race, no significant differences were found in right ventricular inflow and outflow tract dimensions, fractional area change, s’ and GLS. Conclusions. In master endurance athletes running an ultra-marathon, exercise-induced ventricular dysfunction or relevant ventricular arrhythmias were not detected. These results did not confirm the hypothesis of a detrimental acute effect of strenuous exercise on the heart. Abstract Figure.