Global and segmental left ventricular function in beta-thalassemia.

The systolic and diastolic left ventricular (LV) function was studied by M-mode echocardiography in 60 patients with beta-thalassemia (mean age +/- SD, 17.1 +/- 7.5 years) and 30 healthy controls (15.4 +/- 3.8 years). In thalassemic patients, echocardiograms were obtained 48 h posttransfusion, with a mean hemoglobin level of 12.4 +/- 0.9 g/dl. To examination time, thalassemic patients had received 30-774 blood units (318 +/- 176). Congestive heart failure (CHF) was present in 14 thalassemic patients (19.6 +/- 3.4 years), while 46 (16.3 +/- 8.2 years) had no clinical signs of CHF. Global LV function study showed enlarged LV dimensions in thalassemic patients with CHF (p less than 0.001) and similar cavity size in controls and patients without CHF (p = NS). The same was true for velocity measurements, while diastolic LV indices had similar values in all groups (p = NS). Segmental LV function study showed no significant differences in systolic and diastolic LV posterior wall behavior between thalassemic patients and controls, and even more, between thalassemic patients with and without CHF, while it was independent of iron load. These findings indicate that global and segmental LV function in thalassemic patients remain within normal limits until the final stages of the disease. CHF onset marks the deterioration of LV systolic performance, while global and segmental diastolic indices do not change significantly. The above findings question the role of iron overload in the development of CHF in beta-thalassemia.

Iron overload and left ventricular performance in beta thalassemia.

The effect of iron overload on left ventricular (LV) performance was studied in 60 patients with beta-thalassemia. Patients were divided into 3 groups according to the number of blood units (BU) received. Clinically, 14 patients were in advanced classes (III and IV) of congestive heart failure (CHF). LV performance was extensively studied by M-mode echocardiography, and the results were correlated to BU transfused and CHF presence. E point-septal separation, LV systolic and diastolic dimensions had greater values in CHF patients (p less than 0.0001). The percentage shortening of the internal LV diameter and the peak velocity of circumferential fiber shortening were reduced in CHF patients (p less than 0.01 and 0.0001), while the percentage thickening of the LV posterior wall (PW) and interventricular septum (IVS), were independent of CHF presence. LVPW relaxation and indices of the overall diastolic LV function had similar values in all groups. The relation of all systolic and diastolic indices to BU was low (r less than 0,6) and in most cases indifferent (p:NS). A special finding was observed in 4 CHF patients, where segmental IVS dyskinesia contrasted with a satisfactory LVPW motion. The above results indicate that CHF in beta-thalassemia is not the consequence of volume and iron overload, but that these factors are predisposing towards the development of a specific type of cardiomyopathy.

Autonomic neuropathy in leprosy.

The integrity of the autonomic control of the cardiovascular system was studied in 21 patients with lepromatous leprosy and in ten normal people using several simple tests based on cardiovascular reflexes. Impairment of both parasympathetic and sympathetic function was demonstrated in the lepromatous patients.

Sinus node coronary arteries studied with angiography.

Coronary angiograms of 309 consecutive patients undergoing coronary angiography were reviewed to investigate the blood supply to the sinus node area. Blood was supplied from the right coronary artery in 59% of cases, from the left coronary artery in 38%, and from both coronary arteries in 3%. The posterior sinus node artery was demonstrated in 32 patients (27% of the 119 patients with the sinus node artery originating from the left circumflex and 10.5% of all patients).