Evaluation of iron overload by cardiac and liver T2* in ß-thalassemia: Correlation with serum ferritin, heart function and liver enzymes.

Introduction: In this study, we aimed to assess the relationship of cardiac and hepatic T2* magnetic resonance imaging (MRI) values as a gold standard for detecting iron overload with serum ferritin level, heart function, and liver enzymes as alternative diagnostic methods. Methods: A total 58 patients with beta-thalassemia major who were all transfusion dependent were evaluated for the study. T2* MRI of heart and liver, echocardiography, serum ferritin level, and liver enzymes measurement were performed. The relationship between T2* MRI findings and other assessments were examined. Cardiac and hepatic T2* findings were categorized as normal, mild, moderate, and severe iron overload. Results: 22% and 11% of the patients were suffering from severe iron overload in heart and liver, respectively. The echocardiographic findings were not significantly different among different iron load categories in heart or liver. ALT level was significantly higher in patient with severe iron overload than those with normal iron load in heart (P =0.005). Also, AST level was significantly lower in normal iron load group than mild, moderate, and severe iron load groups in liver (P <0.05). The serum ferritin level was significantly inversely correlated with cardiac T2* values (r = -0.34, P =0.035) and hepatic T2* values (r = -0.52, P =0.001). Conclusion: Cardiac and hepatic T2* MRI indicated significant correlation with serum ferritin level.

Echocardiographic evaluation of prevalence of pulmonary hypertension in ß-thalassemia major: A cross sectional study.

INTRODUCTION: Pulmonary hypertension is a common complication associated with thalassemia syndromes and it may play an important role in the pathogenesis of right ventricle failure. The true prevalence of pulmonary hypertension in patients with thalassemia major remains unclear and has been reported to be between 2 and 79%. MATERIALS AND METHODS: In total, 70 patients with thalassemia major were initially examined. Patients with valvular left heart disease, congenital heart diseases such as atrial septal defect (ASD) and ventricular septal defect (VSD), left heart failure, and chronic embolism were excluded. All patients with thalassemia major underwent echocardiography. Based on tricuspid regurgitation velocity (TRV), the patients were divided into the following three groups: low, medium, and high risk of pulmonary hypertension. RESULTS: The mean age of the subjects was 24 y; 60.6% of the subjects were males and 39.4% of the subjects were females. Overall, three (4.5%) subjects were considered at a high risk of pulmonary hypertension. The mean hemoglobin level in the patients with a high probability of pulmonary hypertension was 8.2 g/dL and that in the patients with a low or medium probability of pulmonary hypertension was 9.1 g/dL. No significant difference was observed between the groups (p = .059). CONCLUSION: This study showed that, based on new echocardiography criteria, the prevalence of pulmonary hypertension secondary to ß-thalassemia was 4.5% and there was no correlation between TRV and the number of received blood units or disease duration.

Pregnancy outcome in a pregnant patient with idiopathic Pulmonary Arterial Hypertension: a case report and review of the literature.

BACKGROUND: Idiopathic pulmonary arterial hypertension is a rare and progressive condition which is aggravated by the physiologic changes during pregnancy. Because of high mortality rate, most physicians recommend early termination of pregnancy in patients with idiopathic pulmonary arterial hypertension. CASE PRESENTATION: Here we describe a case of a 30-year-old primigravida Caucasian housewife with functional class 1 idiopathic pulmonary arterial hypertension and a positive vasoreactive response to adenosine who had a full-term non-complicated delivery. Right-sided heart catheterization before the pregnancy showed severe pulmonary hypertension with mean pulmonary arterial pressure of 60 mmHg, and pulmonary vascular resistance of 12.2 WU. Vasoreactivity was positive after infusion of 200 µg/kg per minute adenosine. During pregnancy, she did not receive medication other than prophylactic enoxaparin. She had an elective cesarean section under general anesthesia at 39 weeks of gestation without complication and delivered a healthy baby. After delivery, her hemodynamic status was stable. One month postpartum, she was in a stable clinical condition in functional class 1. CONCLUSIONS: In pregnant patients with pulmonary arterial hypertension, decreased mortality has been observed over recent years particularly in patients with well-controlled pulmonary pressure and a positive vasoreactivity test.

Dextrocardia with asymptomatic right atrial appendage aneurysm: a case report.

Right atrial appendage aneurysm (RAAA) is rarely encountered with variable intracardiac anatomy. We report a case of asymptomatic RAAA in a patient with dextrocardia and anomalous origin of RCA from left coronary sinus which was treated successfully by CABG and the aneurysm was completely excised.

Thyrotoxicosis presenting as exertional dyspnea and pulmonary hypertension: Case report and review of literature.

OBJECTIVES: In this case study, we describe a 35-year-old woman with pulmonary hypertension that was secondary to thyrotoxicosis who presented with exertional dyspnea. In the approach to exertional dyspnea, detailed physical examination and laboratory findings helped to get the diagnosis. METHODS: Echocardiography showed right ventricular systolic pressure = 60 mmHg. A suppressed thyroid-stimulating hormone was seen in lab tests. The patient was treated with radioactive iodine. RESULTS: Palpitations and shortness of breath improved, and both her thyroid-stimulating hormone and T4 levels decreased. Echocardiography and the pulmonary pressure were normal after treatment. CONCLUSIONS: Thyrotoxicosis is one of the etiologies of pulmonary hypertension, but the exact etiology and pathogenesis remain unknown. Some patients may have symptomatic pulmonary hypertension that can be resolved by hyperthyroidism treatment.