Prognostic Value of Doppler‐Demonstrated Left versus Biventricular Restrictive Filling Pattern in Thalassemia Major

Abstract

ongestive heart failure is the main cause of death in transfusion-dependent homozygous-thalassaemia (thalassaemia major (TM)) patients. When symptoms of heart failure appear, death usually occurs within 1 year. 1,2 Although cardiac death is the most common cause of death in patients with TM, early markers of cardiac function with prognostic impact on survival have not yet been adequately defined. Deposition of iron in the heart is the major determinant of survival in these patients. 3,4 Left ventricular (LV) restrictive filling pattern (RFP), as assessed by Doppler-echocardiography, is a well-recognized feature of TM patients with normal LV systolic function, 5 although debated by some authors. 6,7 LVRFP, as assessed by Doppler-echocardiography, is characterized by an increase of the peak early to peak late filling velocity ratio (>2) and/or shortened deceleration time of early filling (<150 ms) because of impaired ventricular compliance. 8,9 Doppler-demonstrated LVRFP is an ominous sign in a variety of cardiac diseases with concomitant systolic heart failure, 10-13 but the clinical significance of restrictive physiology in TM patients has not yet been well established. The purpose of this longitudinal study was to investigate the impact of the Doppler-demonstrated LV filling pattern and also the impact of compliance with chelation therapy on survival in a cohort of asymptomatic TM adult patients with normal LV systolic function throughout a 15-year observation period. Methods Patients and Study Protocol All patients gave written informed consent and the protocol of this study was reviewed and approved by the institutional review board. Sixty-five consecutive TM patients, who were followed-up in the Thalassaemia Unit, were considered candidates for the analysis. All patients initially underwent clinical evaluation, chest X-ray, 12-lead ECG and Doppler-echocardiography from May 1989 to August 1989, in order to obtain baseline data, and then they were examined periodically by clinical examination and Doppler-echocardiography up to the end of the data collection (May 2004). The endpoint of the study was death from cardiovas-cular causes. Patients' inclusion criteria were: (1) age ≥14 years at the initial examination, (2) normal LV end-diastolic diameter (=55 mm) and normal LV systolic function (frac-tional shortening =30%), as assessed by echocardiography and (3) absence of any symptom of cardiac and/or pulmonary pathology. Among the 65 patients initially evaluated, 20 were excluded for the following reasons: 11 were <14 years of age; 4 had LV fractional shortening <30%; 5 died from non-car-Background The present study evaluated the prognostic significance of Doppler-demonstrated left ventricular (LV) restrictive filling pattern (RFP) in patients with thalassaemia major (TM), which carries an adverse cardio-vascular prognosis. Methods and Results The study group comprised 45 asymptomatic transfusion-dependent patients with TM and normal LV systolic function. All patients were chelated with desferrioxamine. They wer...

Ratio of Early Transmitral Velocity to Lateral Mitral Annular Early Diastolic Velocity Has the Best Correlation With Wedge Pressure Following Cardiac Surgery

“…9,10 The peak velocities of early (E) and late (A) mitral inflow, the deceleration time of the E wave (DcT), and mitral A duration (MAd) were measured using pulsed-wave Doppler with the sample volume at the tip of the mitral valve leaflets. We also measured the color M-mode Doppler flow propagation velocity of the LV inflow (Vp) as the slope of the first aliasing velocity during early filling, from the mitral valve plane to 4 cm distally into the LV cavity.…”

Cardiac iron overload detection using longitudinal strain in asymptomatic children with beta thalassemia major

“…Diastolic parameters in BTM patients with normal LV systolic function are similar to those observed in conditions with increased preload, probably caused by chronic anemia. Nevertheless, severe myocardial iron overload leads to a restrictive lling pattern of LV [31,32]. An increase in volume and structural remodeling of both right and left atria, even in the absence of LV and RV dysfunction is possibly due to volume overload caused by chronic anemia [33].RV iron overload is tolerated for a long time before RV dysfunction and PHT occur.…”

The Origin and Clinical Significance of the Signal Opposite to the Mitral E-Wave: A Simple and Novel Indicator of Left Ventricular Filling Pressure

“…12,13 Peak E velocities during early diastole were measured from the mitral flow velocity using a pulsed Doppler echocardiography with the sample volume at the tip of the mitral valve leaflets. All Doppler profiles were recorded in an apical four-chamber view as previously described.…”

“…All Doppler profiles were recorded in an apical four-chamber view as previously described. 12,13 Peak E velocities during early diastole were measured from the mitral flow velocity using a pulsed Doppler echocardiography with the sample volume at the tip of the mitral Figure 2. Mitral flow velocity pattern in a healthy subject and in a patient with high left ventricular filling pressure.…”