P747 Echocardiographic evaluation of haemoglobinopathies patients and their correlation with haematologic determinants
Authors: Branco Mano T , Moura Branco L , Aguiar Rosa S , Agapito A , Timoteo A T , Galrinho A , Rio P , Leal A , Gameiro F , Coutinho Cruz M , Ferreira Reis J , Cruz Ferreira R 
Published: January 2020

Background

Although heart disease in haemoglobinopathy have dramatically reduced with modern therapy, cardiac complications are still a leading cause of morbimortality. It became essential to detect cardiac complications in early stages in order to provide specific therapy and to improve prognosis. Transthoracic echocardiography (TTE) has a paramount role in this field.

Purpose

To evaluate cardiac function in haemoglobinopathy patients and to correlate echocardiographic parameters with haematological determinants.

Methods

Retrospective analysis of haemoglobinopathy patients (pts) referred to a tertiary centre for cardiac evaluation and transthoracic echocardiography. Epidemiological, clinical, laboratory and echocardiographic data were analysed. Comparations between echocardiographic data and haemoglobin and serum ferritin levels were performed (independent T test). P value &lt;0.05 was considered statistically significant.

Results

55 pts were included: 58% female, mean age 37.9 ± 10.9 years, 85% with sickle cell disease, 13% (N = 7) with previous thromboembolic event and 20% (N = 11) with documented hemosiderosis. 36% (N = 20) were symptomatic (9 pts with palpitations, 6 pts with thoracic pain and 5 pts with exertion dyspnea – New York Heart Association class II). The mean haemoglobin level was 8.96 ± 1.91 g/dL and serum ferritin 1335.5 ± 2452 ng/mL. All patients had preserved left and right ventricular systolic function (TAPSE 27.1 ± 4.9mm) The mean left ventricular (LV) end-diastolic diameter indexed to body surface area (female 32.2 ± 4.7mm/m2; male: 31.2 ± 4.0mm/m2) and LV mass index (female 105.6 ± 31.4g/m2; male: 134.7 ± 97.3g/m2) were increased in both genders, although interventricular septum thickness was in the superior normal range (mean 9.3 ± 1.6mm). Left atrium enlargement was also present (29.9 ± 8.4mm/m2). The mean global ratio between early mitral inflow velocity and mean (septal and lateral) mitral annular early diastolic velocity (E/e") was 7.5 ±2.3, and 12 pts (21.8%) had impairment of LV diastolic function. Global longitudinal strain (GLS) was assessed in 35 pts, with a mean value of 19.6 ± 2.9%. 40% (14 pts) had GLS &gt; -18%, being the septal wall the most frequently affected. Pulmonary hypertension was rare (3 pts), with mean arterial pulmonary pressure of 27.7 ± 7.0mmHg. An agreement was found between serum ferritin levels and GLS impairment defined as GLS &gt; -18% (2649ng/ml vs 549ng/ml, p = 0.04). Pts with diastolic dysfunction had a trend to higher serum ferritin levels (2753ng/ml vs 1001ng/ml, p = 0.06). The haemoglobin levels did not correlate with echocardiographic parameters.

Conclusion

In the study population of haemoglobinopathy pts, mainly constituted by cardiac asymptomatic individuals, LV diastolic dysfunction and LV global longitudinal strain were impaired in 22% and 40%, respectively. These echocardiographic parameters revealed to be related to serum ferritin levels.