The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in ß-thalassemia major.

Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in ß-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 29 ± 11 years) with ß-thalassemia and normal left ventricular function and 90 age and sex-matched healthy controls. Patients and controls underwent a thorough cardiac echocardiographic study and measurements of the b-type (NT-proBNP) and atrial natriuretic peptides (proANP). Patients underwent 24-hr Holter recordings for arrhythmia monitoring. In the patient group, atria were affected early during the course of the disease, prior to diastolic and systolic left ventricular dysfunction. The E/E'ratio (E Doppler mitral fast inflow to the corresponding tissue Doppler E) continually increased with age (P < 0.05) and reached levels indicating left ventricular diastolic dysfunction (E/E' > 15) in the third decade whereas indexes of active and passive atrial function decreased gradually throughout life. In controls, the E/E' ratio continually increased with age but with later (fifth decade) appearance of diastolic dysfunction and a compensatory increase in atrial active function. Both natriuretic peptides were significantly increased in patients compared to controls (558 ± 141 and 2,580 ± 1,830 fmol/mL for NT-proBNP and proANP versus 332 ± 106 and 1,331 ± 1,134 fmol/mL, respectively). Atrial fibrillation was found in a subgroup of 23 (26%) patients, older in age with mild diastolic function and enlarged, depressed atria. In conclusion, atrial mechanical depression seems to be a very early sign of cardiac damage. It may become echocardiographically evident even before diastolic and systolic dysfunction and is associated to supraventricular arrhythmias.

Dislocation of an infected and abandoned pacemaker lead.

We present a case of pacemaker lead dislodgment in an 83-year-old patient with a pacemaker infection. Initially, the generator and the proximal part of the leads were removed, and the remaining leads were severed and abandoned. Twenty-five days later, dislodgment of both abandoned leads and systemic infection were documented. The leads were then surgically removed without further complications.

NT-proBNP levels and diastolic dysfunction in beta-thalassaemia major patients.

BACKGROUND: The early diagnosis and treatment of heart failure in beta-thalassaemic patients is related to survival. Iron alone or in combination with other factors causes diastolic dysfunction, which usually precedes systolic dysfunction. NT-proBNP is a sensitive biomarker of ventricular dysfunction. AIM: To evaluate NT-proBNP in non heart failure beta-thalassaemic patients. METHODS: Fifty-two beta-thalassaemia major patients (mean age: 27.2+/-12.5 years) with normal LV systolic function, underwent NT-proBNP measurement and a thorough Doppler-echocardiographic and pulsed tissue-Doppler study, 4 days following blood transfusion. Fifty-two age matched healthy controls were also studied. RESULTS: NT-proBNP and E/E' ratio were increased in thalassaemic patients compared with controls [(469+/-171 vs 262+/-51 pmol/l, p<0.001) and (10.8+/-4.0 vs 6.6+/-1.1, p<0.001)] and were well correlated (r: 0.54, p<0.01). Although NT-proBNP levels were increased in patients with higher E/E' ratios (E/E' <8: 354+/-119, 8-15: 516+/-177, >15: 565+/-114 pmol/l, ANOVA p: 0.002) this increase only became statistically significant in the 3rd decade of life, while E/E' ratio increased in the 4th decade. CONCLUSION: NT-proBNP increases in beta-thalassaemia major patients and is related to age and LV diastolic dysfunction. NT-proBNP appears to be an early biomarker of LV diastolic dysfunction, compared with the conventional Echo-Doppler indexes.