Echocardiographic abnormalities in adolescent and adult Saudi patients with sickle cell disease

OBJECTIVE: Cardiovascular complications in sickle cell disease (SCD) have been well documented but cardiac involvement in Saudi patients with SCD is not known. We sought to identify cardiac abnormalities by echocardiography in adolescent and adult Saudi patients with stable SCD.

METHODS: Sixty-five consecutive patients with SCD followed at King Khalid University Hospital, Riyadh, were prospectively studied from January 2005 to December 2005. All patients underwent echocardiographic examination to determine chamber dimensions, left ventricular function, valvular anomalies and pulmonary artery pressure. Data were compared to normal age and gender-matched controls. Hematological data were also collected from the patients and correlated with the echocardiographic results.

RESULTS: Twenty-eight males and 37 females were evaluated. The mean age of the group was 24.5 ± 9.2 (range 14-44) years. The most common abnormality found was pulmonary hypertension (PH) present in 25 (38%) patients. The majority of these patients had mild PH and only 6 (9%) patients had pulmonary artery systolic pressure (PASP) more than 40 mm Hg. Older age, lower level of fetal hemoglobin and high serum ferritin were associated with increased PASP. Other abnormalities present included dilated left atrium in 17 (26%) patients, dilated right atrium 13 (20%), dilated left ventricle 10 (15%), valvular anomalies 21 (32%) and reduced ejection fraction in 4 (6%) patients.

CONCLUSION: Cardiac abnormalities are found in a significant proportion of Saudi patients with SCD. Pulmonary hypertension is the most common finding while other abnormalities are less frequent.