711 Postgrad Med J: first published as 10.1136/pgmj.2004.031096 on 4 November 2005. Downloaded from ORIGINAL ARTICLE Systolic and diastolic function in middle aged patients with sickle b thalassaemia. An echocardiographic study I Moyssakis, R Tzanetea, P Tsaftaridis, I Rombos, D P Papadopoulos, V Kalotychou, A Aessopos ............................................................................................................................... Postgrad Med J 2005;81:711–714. doi: 10.1136/pgmj.2004.031096 Objective: To evaluate the right and left ventricular systolic and diastolic function in middle aged patients with sickle b thalassaemia. Methods: Forty three patients with sickle b thalassaemia were recruited for echocardiographic study while 55 controls, matched for age and sex, served as the control group. Parameters measured included: See end of article for dimensions and wall thickness of left (LV) and right (RV) ventricle and left atrium, LV mass, and cardiac authors’ affiliations ....................... index. LV and RV contractility variables—ejection fraction, circumferential fibre shortening velocity, end systolic stress, end systolic stress/volume index ratio, mitral and tricuspid annulus systolic excursion, and Correspondence to: Tei index—were also calculated. The study also evaluated parameters of RV and LV diastolic function Dr D P Papadopoulos, Laiko General Hospital of including early and late atrioventricular flow velocities (E and A wave respectively), E/A ratio, Athens, Department of deceleration time (DT), isovolumic relaxation time (IVRT) as well as pulmonary and hepatic veins systolic to Cardiology, 6–8 Glykonos diastolic (S/D) ratio. Street St-10675, Athens, Greece; jimpapdoc@ Results: Chamber enlargement, greater LV mass index, cardiac index, and RV wall thickness were found in yahoo.com the anaemic group compared with controls. The LV and RV contractility variables of the patients were similar to controls. Conversely the LV and RV Tei index was significantly greater in the patient group. Submitted Diastolic dysfunction was present in the anaemic patients resulting from the increased LV and RV A-wave, 29 November 2004 Accepted the longer LVIVRT, RVIVRT, and RVDT, as well as the higher hepatic and pulmonary veins S/D ratio. 23 February 2005 Conclusions: The results show that in middle aged patients with sickle b thalassaemia the diastolic function ....................... is abnormal in both ventricles but still more in RV, whereas the systolic function remains unchanged. ickle cell disease is a hereditary haemolytic anaemia and a/b chain synthesis to confirm the thalassaemic characterised by the synthesis of the haemoglobin S genotype. All patients were Sb+ type, with haemoglobin S(HbS, sickle cell haemoglobin). Homozygous sickle cell concentrations higher than 70 g/l; they had painful crises and disease (SS) results from the inheritance of two sickle cell red cell transfusions rarely (less than five per year) while genes (bs). Coinheritance of a bs gene and a b thalassaemia none was receiving drugs such as digoxin, diuretics, or gene (bthal) results in different types of sickle b thalassaemia vasodilators. The study protocol was approved by the (Sb) depending on the mutation, which is carried by the b institutional committee on human research and all subjects http://pmj.bmj.com/ thalassaemia gene, and the amount of HbA synthesised. If gave informed consent. the bs gene is inherited with a b+ thalassaemia gene, in which some b chains are present, the genotype is Sb+ and if the b Echocardiographic evaluation thalassaemia gene is b0, in which b chains are absent, the Comprehensive echocardiographic examination with pulsed, genotype is Sb0. It is well known that whether the interacting continuous, and colour Doppler was performed with a Hewlet b thalassaemia gene is bo the clinical picture is similar to Packard Sonos 1000 ultrasound System, using a 2.5 MHz sickle cell anaemia whereas, when the interacting b transducer by the same cardiologist and was recorded on on September 28, 2021 by guest. Protected copyright. thalassaemia gene is of b+ type it has milder clinical signs tape. The measurements were performed, independently, by and symptoms.1 Cardiac abnormalities in sickle cell disease two cardiologists unaware of the subject’s status. From the have been well described but mainly in sickle cell anaemia.2 two dimensional guided M-mode echocardiogram, LV end However, little is known about heart involvement in sickle b systolic and end diastolic diameters as well as interventri- thalassaemia. cular septum and posterior wall thickness at end diastole The purpose of this study was to investigate the changes in were measured for the calculation of fractional shortening systolic and diastolic function of the left and right ventricle (FS) and LV mass with the Penn convention formula.34 (LV and RV respectively) in middle aged patients with sickle Measurements of LV mass were divided by body surface area b thalassaemia. to obtain LV mass index. The LV ejection fraction (EF) was determined by the biplane Simpson’s method. The LV stroke METHODS volume was also calculated as the product of the cross Study population sectional area of the LV outflow tract and velocity time Forty three consecutive patients between 45 and 66 years (15 integral of the LV outflow velocity.5 The LV cardiac output men, 28 women, aged mean (SD) 54 (10) years) with sickle b was derived as the product of the stroke volume and thalassaemia and no underlying heart disease and 55 (18 heart rate and, divided by body surface area; the LV cardiac men, 37 women, aged 53 (11) years) healthy volunteers were index was calculated. Furthermore, we measured several recruited in our study. Patients with sickle b thalassaemia were recruited from the haemoglobinopathies unit of Laiko Abbreviations: RV, right ventricle; LV, left ventricle; DT, deceleration General Hospital, Athens, Greece. Inclusion criteria were, time; FS, fractional shortening; EF, ejection fraction; TAPSE, tricuspid diagnosis of sickle b thalassaemia, made by solubility annular plane systolic excursion; IVRT, isovolumic relaxation time; ESS, screening test for sickling, haemoglobin electrophoresis, end systolic stress; MAPSE, mitral annular plane systolic excursion www.postgradmedj.com 712 Moyssakis, Tzanetea, Tsaftaridis, et al Postgrad Med J: first published as 10.1136/pgmj.2004.031096 on 4 November 2005. Downloaded from Table 1 Clinical characteristics of the study population Characteristics SbT Controls p Value Age (y) 54 (10) 53 (11) NS Men/women 15/28 18/37 NS Heart rate (b/min) 81 (13) 76 (11) NS SAP (mm/Hg) 121 (13) 126 (17) NS DAP (mm Hg) 69 (9) 78 (7) ,0.05 Packed cell volume (%) 28.70 (4.30) 43.40 (5.20) ,0.01 Haemoglobin (g/l) 95.8 (8.6) 146.0 (15.0) ,0.01 Serum ferritin (mg/l) 782 (346) 81 (29) ,0.01 SAP, systolic arterial pressure; DAP, diastolic arterial pressure; SbT, sickle b thalassaemia. parmaeters of LV systolic function such as the circumferential RESULTS fibre shortening velocity (Vcf) as the ratio FS to ejection As table 1 shows, the patients with sickle b thalassaemia had period, the meridional end systolic stress (ESS), the ratio end lower levels of diastolic blood pressure, packed cell volume, systolic stress/end systolic volume index (ESS/ESVI), and the and haemoglobin and increased concentrations of serum mitral annular plane systolic excursion (MAPSE).6–9 RV end ferritin compared with controls. diastolic diameter and thickness of the free wall was Chamber enlargement (left atrium, LV, and RV), greater LV measured and the RV systolic function was evaluated from mass index (109 (8) compared with 86 (19) g/m2,p,0.01), the tricuspid annular plane systolic excursion (TAPSE).10 11 cardiac index (4.80 (1.80) compared with 3.70 (1.20) l/min/ We also evaluated the myocardial performance index (Tei m2,p,0.01) and RV wall thickness (0.32 (0.07) compared index) of LV and RV.12 13 Using pulsed Doppler from the with 0.29 (0.06) cm, p,0.05) were noted in the anaemic mitral and tricuspid inflow velocity curves the following group (table 2). parameters were calculated: peak early velocity (E-wave), There was no difference in the LV systolic function peak velocity at the time of atrial contraction (A-wave), E/A variables such as the EF, ESS, ESS/ESVI, Vcf, and MAPSE ratio, deceleration time (DT) of the peak early velocity, and between patients and controls. Similarly, the RV systolic the isovolumic relaxation time (IVRT). For the calculation of function was not impaired assessed by the TAPSE. Conversely the RV diastolic variables at least three beats from the end the Tei index expressing combined systolic and diastolic inspiration and three beats from the end expiration were performance was increased for both ventricles in the anaemic recorded and their values were averaged.14–16 Flow velocities group (0.39 (0.07) compared with 0.36 (0.06), p,0.05 for LV of the hepatic veins and upper pulmonary veins were also and 0.37 (0.11) compared with 0.32 (0.09), p,0.05 for RV) recorded for systolic to diastolic forward flow velocity ratio distinguishing global LV and RV dysfunction (table 3). (S/D) calculation. The intraobserver and interobserver mean The diastolic function was also impaired in anaemic percentage error (absolute difference between two measure- patients compared with controls. Specifically, the LV and ments divided by the mean and expressed in percentage) of RV A-wave was increased (53 (12) v 44 (15) cm, p,0.01 and Doppler measurements were respectively (for the LV: 3.1% 47 (9) v 40 (8), p,0.01 respectively), the LV and RV IVRT was and 3.3% for E-wave, 3.3% and 3.6% for A-wave, 3.5% and longer (83 (17) v 76 (11) ms, p,0.05 and 52 (13) v 46 4.2% for DT, 3.5% and 4.3% for IVRT whereas for the RV: (11) ms, p,0.05 respectively) and the RV DT was also longer 6.3% and 6.9% for E-wave, 6.5% and 7% for A-wave, 5.6% and (198 (39) v175 (34) ms, p,0.01) in the group of patients.