Optimal method for early detection of cardiac disorders in thalassemia major patients: magnetic resonance imaging or echocardiography?

BACKGROUND: Heart failure resulting from myocardial iron deposition is the most important cause of death in beta-thalassemia major (TM) patients. Cardiac T2*magnetic resonance imaging (MRI), echocardiography, and serum ferritin level serve as diagnostic methods for detecting myocardial iron overload. In this study, we aimed to evaluate the relationship between the above-mentioned methods. METHODS: T2*MRI and echocardiographic measurement of left ventricular (LV) systolic and diastolic function were performed in 63 patients. Serum ferritin level was measured. The relationships between all assessments were evaluated. RESULTS: There were 40 women and 23 men with a mean age of 23.7+/-5.1 years (range, 15-35 years). There was no statistically significant correlation between serum ferritin level and LV systolic and diastolic function (P=0.994 and P=0.475, respectively). T2*MRI results had a significant correlation with ferritin level; 63.6% of patients with serum ferritin level >2,000 ng/mL had abnormal cardiac MRI, while none of the patients with ferritin level <1,000 ng/mL had abnormal cardiac MRI (P=0.001). There was no significant correlation between MRI findings and LV systolic function (P=1.00). However, we detected a significant difference between LV diastolic function and cardiac siderosis (P=0.03) CONCLUSION: MRI findings are a good predictor of future cardiac dysfunction, even in asymptomatic TM patients; however, diastolic dysfunction may happen prior to cardiac siderosis in some patients, and echocardiography is able to diagnose this diastolic dysfunction while T2*MRI shows normal findings.

relationship between coronary artery aneurysm and QT interval dispersion in acute phase of Kawasaki disease

QT dispersion [QTd] has been proposed as a marker of ventricular repolarization inhomogeneity and several investigations have proved the relationship between it and cardiac ischemia, ventricular arrhythmia and sudden cardiac death. The aim of this study was to assess the relation between coronary artery involvement and QTd, and QTc dispersion [QTcd] in the acute phase of Kawasaki disease [KD]. We studied 65 patients with acute KD. Patients were divided into 3 groups. Group one consisted of 48 patients without coronary artery involvement. Group two comprised 13 patients with small to medium size aneurysm. In Group three there were 4 patients with giant aneurysm or multiple small to medium size aneurysms or thrombosis in coronary arteries. For each patient 12 lead ECG was obtained, and QT, QTc, QTd, QTcd, and RR interval were calculated. There were 40 males and 25 females with a mean age of 41.4 +/- 31.1 months. There was no significant difference in QT, QTc, RR measurements between 3 groups. QTd was greater in group 3 versus group 1 and 2, but the difference was not statistically significant [P=0.06]. QTcd was significantly greater in group 3 than in groups 1 and 2 [75.02 +/- 11.53 ms versus 46.82 +/- 15.39 ms and 48.88 +/- 10. 55 ms respectively [P = 0.04]]. The sensitivity of QTcd >/=60 ms to detect the patients with severe coronary arteries involvement was 100%, the specificity was 93.4%, positive predictive value was 50%, negative predictive value was 100%, and accuracy was 93.8%. QTcd can be used as a predictive factor for diagnosis of severe coronary arteries involvement in the acute phase of KD

Short and long-term outcomes after balloon pulmonary valvuloplasty in congenital pulmonary stenosis

Balloon pulmonary valvuloplasty [BPV] has emerged as the treatment of choice for patients with valvular pulmonary stenosis [PS]. We report here our short and long-term outcomes of BPV in 64 patients with isolated native PS. From February 1996 to February 2006, sixty-four patients with PS [pressure gradients ?40 mm Hg] were enrolled in this retrospective study. The hemodynamic data at catheterization revealed that the RV - PA pressure gradient before BPV ranged from 40 to 240 mmHg [mean +/- SD=93.2 +/- 43.4 mmHg]. The above gradient immediately after BPV ranged from 5 to 163 mmHg [mean +/- SD=30.3 +/- 27.7 mmHg], and the difference was significant [p=0.0037]. Twenty-three patients had regular follow-up. The duration of follow- up ranged from 1-120 months with a mean of 38.5 +/- 31.3 months. The transvalvar pressure gradient during the above period ranged from 10 to 140 mmHg with a mean of 35.9 +/- 27.9 mmHg and showed a significant difference [p=0.0032] with the pressure gradients before BPV. BPV provides short and mid-term relief of pulmonary valve obstruction in the majority of patients