Left ventricular dysfunction by strain echocardiography in thalassemia patients: a pilot study

Background: To evaluate the myocardial function and its correlation with serum ferritin and the number of transfusions in beta-thalassemia major patients by using standard echocardiography and left ventricular strain imaging.Methods: This was a cross-sectional exploration study comprised of 56 beta-thalassemia patients conducted at a tertiary-care center in India between September 2016 and August 2017. Patients with age less than 18 years, diagnosed with thalassemia major, recipients of >20 units of blood transfusions, and normal Left Ventricular (LV) function by 2D-echocardiography were included in the study. Severity of iron overload was determined by using serum ferritin levels and LV strain imaging parameters were evaluated by using strain values of 17 LV segments.Results: A total of 56 beta-thalassemia patients were included in the study. Of these, 29(51.8%) patients were boys and 27(48.2%) patients were girls with a mean age of 7.8±1.84 years. Average serum ferritin level was found to be 4089.83 ng/dl. Strain values of the basal lateral wall of the left ventricle were significantly abnormal in patients who received more (>80) transfusions compared with those who received lesser transfusions (p=0.025 and p=0.045), respectively. Patients with serum ferritin >6000 ng/ml had impaired strain (p=0.03).Conclusions: Conventional echocardiographic parameters and Left Ventricular Ejection Fraction (LVEF) do not provide adequate information about LV dysfunction. Systolic strain index imaging of the LV indicated the presence of early LV systolic dysfunction in patients who received a greater number of blood transfusions and patients with higher serum ferritin levels

Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva-a single center experience with a therapeutic conundrum

Background: Coronary artery anomalies are rare congenital abnormalities, most often found incidentally on conventional coronary angiography and CT angiography (CTA). CTA better delineates the origin and course of anomalous coronaries. Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva (ACAOS) has a prevalence of 1% with a very few having an interarterial (malignant) course. There is limited literature, especially in the Indian population, dealing with this topic.Methods: In this retrospective observational study, angiographic data of 8500 consecutive patients from June 2011 to December 2019 at a large tertiary care hospital in western India was analyzed. Patients diagnosed with ACAOS underwent CTA for delineation of the exact anatomy. Those with a non-malignant course with evidence of ischemia clinically or on stress myocardial perfusion imaging (MPI), underwent PCI. Others with a non-malignant course were medically managed. Patients with malignant (interarterial) course were revascularized by coronary artery bypass graft (CABG) surgery in case of LCA involvement or positive MPI test. Asymptomatic patients with negative MPI were managed medically. Clinical follow-up over 12 months of patients undergoing PCI and those with a malignant course showed no major adverse cardiovascular events (MACE).Results: Of the 8500 patients studied, 74 (0.87%) had ACAOS. Of these, 51 (68.9%) patients had anomalous origin of right coronary artery (RCA) from the left aortic sinus, 21 (28.4%) had anomalous origin of the circumflex artery (Cx) from the right aortic sinus and two patients (2.7%) had an anomalous origin of the left main coronary artery (LCA) from the right aortic sinus. Interarterial course was found in five (6.7%) patients. Of these, four patients underwent CABG-one asymptomatic patient with LCA from right aortic sinus and three with positive MPI in anomalous RCA. All five patients with malignant course and ten patients who underwent PCI were free of MACE over 12 months’ clinical follow up.Conclusions: ACAOS is a rare anomaly and if interarterial course is excluded, then PCI is feasible in selected cases with significant stenosis. Patients with malignant course with inducible ischemia or LCA involvement should undergo surgical revascularisation