Cardiovascular Involvement in Thalassemia Major Patients: WEB-THAL® Data Analysis (A Web-Based Multicentric Database).
Authors: Derchi Giorgio , Formisano Francesco , Lamagna Martina , Galanello Renzo , Bina Patrizio , Cappellini Maria Domenica , Fasulo Maria Rosaria , Piga Antonio , Donato Guido , Quarta Gianni , Melpignano Angela , Tazzer Carla , Forni Gian Luca 
Published: April 2020

Abstract
Prevalence of Left Ventricular Dysfunction (LVD), and Congestive Heart Failure (CHF) have been widely described in Thalassemia Major (TM) patients (pts), according to age, therapy, severity and coexisting diseases. However, so far no data are available in a large population adequately transfused and chelated. To determine the LV involvement in treated TM patients, we studied 524 pts from 5 Thalassemia Centers in Italy (49% males, mean age 30 yrs, range 16 to 56 yrs). In all pts clinical and echocardiographic data were recorded in WEBTHAL® database, a large co-operative Italian project among Thalassemia Centres based on an Internet-shared software for thalassemia. Data were evaluated blind by two independent researchers. Patients were considered having LVD if at least one cardioactive drug and/or history of cardiopathy (heart failure and/or clinically relevant arrhythmias) was recorded in their file (102/524 patients, 19,5%). Tables I and II summarize the echocardiographic results and cardiological treatment.
Table I TM population without LV involvement (n°422 pts) and on cardiological treatment (n° 102 pts) Mean * Statistically significant between groups Pretrasfusional Hb (g/dl) 9,4 Ferritin (μg/dl) mean of last 10yrs 2027,7 LV end diastolic diameter index (mm/m2) 31,8 FS (%) 35,0 EF (%) 60,6 Duration of transfusion period (yrs) 24,5 Pretransfusional Hb (g/dl) 9,5 Ferritin (μg/dl) mean of last 10yrs * 2389,6 LV end diastolic diameter index (mm/m2)* 32,9 FS %* 31,3 EF % 54,4 Duration of transfusion period (yrs)* 26,2 Table 2 Cardiovascular Drugs N° % At least one drug 102 19,5 ACE Inhibitors 81 15,5 Diuretics 39 7,4 Beta-blockers 24 4,6 Antiarrhythmics 16 3,1 Digoxin 8 1,5
The majority of patients (422 subjects, 80.5%) did not take any cardioactive drugs and were considered without LV involvement. Systolic Pulmonary pressure was elevated (PH) (&gt; 25 mmHg) in 10% of pts. ACE-inhibitors were the most used cardiovascular drug (15.5% of the pts), mainly males (n°56 pts, p&lt;0.001). Pts on cardiological treatment showed lower FS%, compared to pts without cardioactive treatment (p&lt; 0.001) and higher LV End Diastolic Diameter Index (32,9 vs 31,2, p&lt; 0.009). Pts with LV involvement had a significatively longer regular trasfusional period compared to patients of matched age and they higher mean ferritin values (Tab1). Female patients had a significantly lower prevalence of LV involvement. In this group EF% and FS% were higher than in males (p&lt; 0,001 and p&lt;0,04, respectively). According to these data, LV involvement is characterized by a large and hypokinetic LV and occurs in 19% of a regularly transfused and chelated TM population. The LV disfunction is likely related to a long exposure to iron load in the heart. The mean values of clinical and echocardiographical variables of TM population without LV involvement can be considered as reference values (Table I).