[Abnormal autonomic cardiovascular responses in patients with sickle cell anemia]

To evaluate the presence of anomalies of the autonomic reflex cardiovascular response in patients with chronic sickle-cell anaemia.

The study was extended to 30 patients with sickle-cell anaemia, 10 patients with iron-lack anaemia and 30 healthy subjects. Age and sex distribution was similar in each group. To be included in the study, patients should have had no painful crisis or blood transfusion in the 6 months previous to the assay. Clinico-laboratory survey, chest x-ray and EKG were performed in every case. Blood cell count and abnormal haemoglobin study on cellulose acetate were carried out as well. The evaluation of reflex autonomic responses was performed by means of active orthostatism, cold pressor test, Valsalva maneuver and urine catecholamine output. The statistical analysis was performed with the variance analysis (ANOVA) for multiple groups.

The following abnormalities were found: 12 patients had haemoglobin SS, 8 had haemoglobin SS and F, 3 had haemoglobin SC, 2 had haemoglobin S and beta-thalassaemia, and 5 had combined haemoglobin SS,F and A2. Systolic pressure and heart frequency in the supine position were similar in all groups. Diastolic pressure was lower in the sickle-cell anaemia group with respect to the normals. Patients with sickle-cell disease had lower heart frequency in the active orthostatism test with regard to the other groups, along with paradoxal changes in systolic pressure and lesser increase of the diastolic pressure. Significantly lower response to the cold pressor test was seen in the sickle-cell patients as compared with the iron-lack cases and the normal controls. Reduced sympathetic tachycardia was seen with the Valsalva maneuver, whereas the bradycardia was similar to the other groups. The urine noradrenaline in output was significantly lower in the sickle-cell patients, it was normal in the other groups (p < 0.01).

These results suggest a defective sympathetic activity of heart and arteries in patients with sickle-cell anaemia.