The predictive role of cardiac magnetic resonance imaging in determining thalassemia patients with intermediately to highly probable pulmonary hypertension

Abstract

Objectives:We sought to determine the cardiac magnetic resonance (CMR) indicators of intermediately to highly probable pulmonary hypertension (IHpPH) in patients with thalassemia referred for myocardial iron overload assessments to prevent further cardiac complications. Methods:The study population consisted of 152 patients with thalassemia (major or intermedia) (49.3% women, mean age = 33 ± 10.1 years) who underwent non-contrast CMR and echocardiographic examinations on the same day. Functional, T2*, and global strain parameters via a feature-tracking method were extracted from CMR. The probability of PH was defined based on the tricuspid regurgitation velocity and echocardiographic parameters. The catheterization-derived hemodynamic data of patients with moderate to high probable PH was registered.Results: Twenty-two (14.5%) patients suffered from IHpPH. The multivariate logistic regression analysis revealed that the right ventricular end-systolic volume index (RVESVI) was the strongest of all the CMR parameters for the prediction of IHpPH (OR:1.044, 95% CI: 1.021-1.067). The other powerful IHpPH predictor was age (OR: 1.066, 95% CI: 1.009-1.126). A cutoff point of greater than 47 ml for RVESVI (AUC: .801, 95% CI: .728-.861) was found to predict IHpPH with 73.91% sensitivity and 70.31% specificity. The single most robust CMR-derived strain parameter for IHpPH prediction was the right ventricular global longitudinal strain (OR: .887, 95% CI: .818-.961). A p value of less than 0.05 was considered significant. Conclusions:Both CMR functional and global strain parameters were strong predictors of IHpPH in our patients with thalassemia. K E Y W O R D Scardiac magnetic resonance (CMR), intermediately to highly probable pulmonary hypertension (IHpPH), pulmonary hypertension (PH), thalassemia INTRODUCTIONThalassemia, considered the most typical of all hereditary disorders, results in the diminished or absent synthesis of hemoglobin chains. 1Globally, there are approximately 270 million carriers of various hemoglobinopathies, of whom 30% are carriers of β-thalassemia. 2 Cardiac complications persist as the main cause of morbidity and the leading cause of mortality in these patients. [3][4][5][6] Heart injury due to myocardial iron overload (MIO) is not completely reversible. There are multiple causes of cardiac disease, especially in older thalassemia

The relationship of myocardial and liver T2* values with cardiac function and laboratory findings in transfusion-dependent thalassemia major patients: A retrospective cardiac MRI study

“…On the other hand, according to some previous studies, RV dysfunction is the most significant predictor of pulmonary hypertension in thalassemia patients, one of the leading causes of mortality. 39 We should consider that iron overload is not the only cause of cardiac disease in beta-thalassemia patients. Nutritional deficiencies, genetic variations, pulmonary hypertension, changes in cardiac function due to fibrosis, and a decrease in vascular compliance are other causes that should be mentioned in the heart disease pathway in beta-thalassemia patients.…”

“…On the other hand, according to some previous studies, RV dysfunction is the most significant predictor of pulmonary hypertension in thalassemia patients, one of the leading causes of mortality. 39 …”

Feature tracking cardiac magnetic resonance imaging to assess cardiac manifestations of systemic diseases

“…Strain levels may manifest the deficiencies and help in the early recognition of the myocardial dysfunction to improve survival and prevent additional problems. Biventricular and RV GLS could distinguish thalassemia major patients in regards to the presence of myocardial iron overload and intermediately to highly probable pulmonary hypertension [ 79 , 80 ]. CMR obtained T2 mapping is the gold standard parameter to assess the myocardial iron overload.…”