The Prognostic Role of Hypertrabeculation By Cardiac Magnetic Resonance in Thalassemia Intermedia Patients

Abstract

Background. Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depends on the selected CMR criterion. The recently proposed Piga’s criterion (NC/C ratio threshold of >2.5, Am J Haem 2012) seems to have a low specificity to identify the true LVNC in TI. Anyway, the Piga’s criterion could well detect easy a negative heart remodeling in TI patients.Purpose: To assess prospectively whether the Piga's criterion has a prognostic role for adverse cardiovascular outcomes in TI patients.Methods. We studied prospectively by CMR 168 TI patients (81 males, mean age 38.32 ±11.61 years) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network. Eight patients were excluded because a cardiac complication was present at the first CMR.Using Piga's criterion the study population was divided into two groups: patients with Piga's positive criterion (n=18, 11.2%) and with Piga's negative criterion (n=143, 88.8%).Results. Mean follow-up time was 57.50 ± 21.87 months. Sixteen cardiac new events were recorded: 1 heart failure (HF), 10 supraventricular arrhythmias and 5 pulmonary hypertension (PH). The patients with Piga’s positive criterion had a significant higher risk of developing arrhythmias (hazard ratio-HR= 5.35 ; 95%CI=1.51-18.98; P=0.009). The figure shows the Kaplan-Meier survival curve.The positivity for the Piga’s criterion was not predictive for PH or for cardiac complications globally considered.Conclusions. Based on our data a NC/C ratio >2.5 provides [Display omitted] DisclosuresPepe:Chiesi Farmaceutici and ApoPharma Inc.: Other: Alessia Pepe is the PI of the MIOT project, that receives no profit support from Chiesi Farmaceutici S.p.A. and ApoPharma Inc..