Prevalence and predictors of cardiac and liver iron overload in patients with thalassemia: A multicenter study based on real-world data.

Prevalence of cardiac and liver iron overload in patients with thalassemia in real-world practice may vary among different regions especially in the era of widely-used iron chelation therapy. The aim of this study was to determine the prevalence of cardiac and liver iron overload in and the management patterns of patients with thalassemia in real-world practice in Thailand. We established a multicenter registry for patients with thalassemia who underwent magnetic resonance imaging (MRI) as part of their clinical evaluation. All enrolled patients underwent cardiac and liver MRI for assessment of iron overload. There were a total of 405 patients enrolled in this study. The mean age of patients was 18.8±12.5years and 46.7% were male. Two hundred ninety-six (73.1%) of patients received regular blood transfusion. Prevalence of cardiac iron overload (CIO) and liver iron overload (LIO) was 5.2% and 56.8%, respectively. Independent predictors for iron overload from laboratory information were serum ferritin and transaminase for both CIO and LIO. Serum ferritin can be used as a screening tool to rule-out CIO and to diagnose LIO. Iron chelation therapy was given in 74.6%; 15.3% as a combination therapy.

Outcomes of straightforward extracardiac Fontan operation in advanced-age group.

INTRODUCTION: Fontan operation is a palliative procedure used in patients with univentricular heart (UVH). There have been numerous modifications to the technique. The appropriate strategy for treating patients with UVH in Thailand is still a major concern. OBJECTIVE: To describe the outcomes of patients after straightforward Fontan operation at advanced-age and evaluate their post-surgical cardiac performance. MATERIAL AND METHOD: A cross sectional study was performed in post-Fontan operation patients at Rajvithi Hospital between 2000 and 2009. Post-operative cardiac performance was analyzed using the echocardiogram and treadmill exercise stress test (EST). RESULTS: Thirty-nine patients were enrolled in the present study. The mean age was 11.7 years and the mean age at operation was 7.4 years. The predominant systemic chamber was morphologic left ventricle. Straightforward Fontan operation was performed in 60% of cases. Mean of Fontan index, mean of McGoon ratio, and mean of Nakata index were 2.63, 2.32, and 414.15 sqmm/sqm, respectively. Mean of the PVR and PAP were 1.98 U/m2 and 11.05 mmHg. Eighteen percent of patients died in the early post-operative period. Most of the patients died from septicemia. The mean EFwas 58.43%. Systolic dysfunction in 17.9% of the cases was abnormal, whereas diastolic dysfunction was present in half of the cases. Thirty-nine percent had MPI abnormality. During EST three cases developed arrhythmias and 3 cases had hypotension. Patients were categorized into 3 groups (Group 1: normal systolic and diastolic functions, Group 2: diastolic dysfunction, Group 3: impairment of both systole and diastole). Pre-operative cardiac catheterization parameters, surgical data and cardiac performance showed no statistical significance. However, there was a significant correlation between those with abnormal cardiac performance and arrhythmias or hypotension during EST (p = 0.003). CONCLUSION: The selection of suitable cases and good pre-operative evaluation could decrease the morbidity and mortality in patients undergoing the Fontan procedure. This study also found a correlation between abnormal cardiac performance and transient cardiac arrhythmia during exercise. The evaluation of cardiac performance and EST remains to be performed for following-up of patients who have undergone the Fontan operation, even for the asymptomatic cases.

A comparison of oral chloral hydrate and sublingual midazolam sedation for echocardiogram in children.

OBJECTIVE: To compare the efficacy and safety of oral chloral hydrate and sublingual midazolam to sedate the children undergoing echocardiography. MATERIAL AND METHOD: A double-blind, randomized trial study in the children judged to require sedation prior echocardiogram were performed. Two hundred sixty-four patients between 6 months and 5 years of age were randomized to chloral hydrate or midazolam groups. Either 50 mg/kg of chloral hydrate orally or 0.3 mg/kg of midazolam sublingually was given in each groups. If the child was not responded within 30 minutes after the first dose, another half dose of each drug for the second dose will be required. The action duration time, sedation score level and the ability to complete echocardiogram were collected. RESULTS: Both groups were comparable with respect to age, sex, body weight, underlying heart disease, baseline O2 saturation and functional heart classification. The children in chloral hydrate group needed the second dose for sedation more than midazolam group (10.6%, 5.3% p = 0.111). The onset, action duration and total study time were significantly shorter in midazolam than in chloral hydrate group (p < 0.001). The number of the patients who had the action duration within the optimal time (< 45 min) were significantly more cases in midazolam than in chloral hydrate group (93.1%, 43.5% p < 0.001). Success rate of echocardiogram was 99.2% in each group. There was no difference in echocardiographic time performed in both groups. The children in chloral hydrate group had deeper in level of sedation (p < 0.001). Both groups showed no significant difference in term of the ability to complete echocardiographic examination. The reaction of the children to take the medication and the number of the patients who had systemic O2 saturation change more than 5%from the baseline were higher in chloral hydrate group significantly (14.4%, 4.5% p = 0.006 and 9.9%, 3.1% p = 0.025). CONCLUSION: Sublingual midazolam at the dose of 0.3 mg/kg can be used to sedate the children at age group between 6 months to 5 years who undergoing echocardiogram with comparable rate of success and safety as 50 mg/kg of chloral hydrate orally. The less depth in the level of consciousness after sedation with midazolam compare to chloral hydrate may be advantage in a high risk patient to avoid deep sedation but may be disadvantage in case who need more comprehensive echocardiographic evaluation.