Complete mitochondrial genomes of patients from Thailand with cardiovascular diseases.

Several previous studies have reported that both variation and haplogroups of mitochondrial (mt) DNA were associated with various kinds of diseases, including cardiovascular diseases, in different populations, but such studies have not been carried out in Thailand. Here, we sequenced complete mtDNA genomes from 82 patients diagnosed with three types of cardiovascular disease, i.e., Hypertrophic Cardiomyopathy (HCM) (n = 26), Long Q-T Syndrome (LQTS) (n = 7) and Brugada Syndrome (BrS) (n = 49) and compared these with 750 previously published mitogenome sequences from interviewed normal individuals as a control group. Both patient and control groups are from the same geographic region of northeastern Thailand. We found 9, 2, and 5 novel mutations that were not both damaging and deleterious in HCM, LQTS, and BrS patients, respectively. Haplogroup R9c was significantly associated with HCM (P = 0.0032; OR = 62.42; 95%CI = 6.892-903.4) while haplogroup M12b was significantly associated with LQTS (P = 0.0039; OR = 32.93; 95% CI = 5.784-199.6). None of the haplogroups was found to be significantly associated with BrS. A significantly higher density of mtDNA variants in the rRNA genes was found in patients with HCM and BrS (P < 0.001) than in those with LQTS or the control group. Effects of detected SNPs in either protein coding or tRNA genes of all the mitogenome sequences were also predicted. Interestingly, three SNPs in two tRNA genes (MT-TA m.5618T>C and m.5631G>A heteroplasmic variants in two BrS patients and MT-TQ m.4392C>T novel homoplasmic variant in a HCM patient) were predicted to alter tRNA secondary structure, possibly leading to abnormal tRNA function.

Predicting arrhythmic event score in Brugada syndrome: Worldwide pooled analysis with internal and external validation.

BACKGROUND: Brugada syndrome is an inherited arrhythmic disease associated with major arrhythmic events (MAE). Risk predictive scores were previously developed with various performances. OBJECTIVE: The purpose of this study was to create a novel score-Predicting Arrhythmic evenT (PAT)-with internal and external validation. METHODS: A systematic review was performed to identify risk factors for MAE. The odds ratios (ORs) of each factor were pooled across studies. The PAT scoring scheme was developed based on pooled ORs. The PAT score was internally validated with published 105 Asian patients (follow-up 8.0 ± 4.1 [SD] years) and externally validated with unpublished 164 multiracial patients (82.3% White, 14.6% Asian, 3.2% Black; mean follow-up 8.0 ± 6.9 years) with Brugada syndrome. Performances were assessed and compared with previous scores using receiver operating characteristic curve (ROC) analysis. RESULTS: Sixty-seven studies published between 2002 and 2022 from 26 countries (7358 patients) were included. Pooled ORs were estimated, indicating that 15 of 23 risk factors were significant. The PAT score was then developed accordingly. The PAT score had significantly better discrimination (ROC 0.9671) than the BRUGADA-RISK score (ROC 0.7210; P = .006), Shanghai Score System (ROC 0.7079; P = .003), and Sieira et al score (ROC 0.8174; P = .026) in an external validation cohort. PAT score ≥ 10 predicted the first MAE with 95.5% sensitivity and 89.1% specificity (ROC 0.9460) and the recurrent MAE (ROC 0.7061) with 15.4% sensitivity and 93.3% specificity. CONCLUSION: The PAT score was shown to be useful in predicting MAE for primary prevention in patients with Brugada syndrome.

Prediction of nonviable myocardium by ECG Q-Wave parameters: A 3.0 T cardiovascular magnetic resonance study.

INTRODUCTION: The presence of a Q-wave on a 12-lead electrocardiogram (ECG) has been considered a marker of a large myocardial infarction (MI). However, the correlation between the presence of Q-waves and nonviable myocardium is still controversial. The aims of this study were to 1) test QWA, a novel ECG approach, to predict transmural extent and scar volume using a 3.0 Tesla scanner, and 2) assess the accuracy of QWA and transmural extent. METHODS: Consecutive patients with a history of coronary artery disease who came for myocardial viability assessment by CMR were retrospectively enrolled. Q-wave measurements parameters including duration and maximal amplitude were performed from each surface lead. A 3.0 Tesla CMR was performed to assess LGE and viability. RESULTS: Total of 248 patients were enrolled in the study (with presence (n = 76) and absence of pathologic Q-wave (n = 172)). Overall prevalence of pathologic Q-waves was 27.2% (for LAD infarction patients), 20.0 % (for LCX infarction patients), and 16.8% (for RCA infarction patients). Q-wave area demonstrated high performance for predicting the presence of a nonviable segment in LAD territory (AUC 0.85, 0.77-0.92) and a lower, but still significant performance in LCX (0.63, 0.51-0.74) and RCA territory (0.66, 0.55-0.77). Q-wave area greater than 6 ms mV demonstrated high performance in predicting the presence of myocardium scar larger than 10% (AUC 0.82, 0.76-0.89). CONCLUSION: Q-wave area, a novel Q-wave parameter, can predict non-viable myocardial territories and the presence of a significant myocardial scar extension.

Determine Cumulative Radiation Dose and Lifetime Cancer Risk in Marfan Syndrome Patients Who Underwent Computed Tomography Angiography of the Aorta in Northeast Thailand: A 5-Year Retrospective Cohort Study.

OBJECTIVE: To evaluate computed tomography angiography (CTA) data focusing on radiation dose parameters in Thais with Marfan syndrome (MFS) and estimate the distribution of cumulative radiation exposure from CTA surveillance and the risk of cancers. METHODS: Between 1st January 2015 and 31st December 2020, we retrospectively evaluated the cumulative CTA radiation doses of MFS patients who underwent CTA at Khon Kaen University Hospital, a leading teaching hospital and advanced tertiary care institution in northeastern Thailand. We utilized the Radiation Risk Assessment Tool (RadRAT) established at the National Cancer Institute in Bethesda, Maryland, to evaluate the risk of cancer-related CTA radiation. RESULTS: The study recruited 29 adult MFS patients who had CTA of the aorta during a 5-year study period with 89 CTA studies. The mean cumulative CTDI vol is 21.5 ± 14.68 mGy, mean cumulative DLP is 682.2 ± 466.7 mGy.cm, the mean baseline future risk for all cancer is 26,134 ± 7601 per 100,000, and the excess lifetime risk for all cancer is 2080.3 ± 1330 per 100,000. The excess lifetime risk of radiation-induced cancer associated with the CTA surveillance study is significantly lower than the risk of aortic dissection or rupture and lower than the baseline future cancer risk. CONCLUSIONS: We attempted to quantify the radiation-induced cancer risk from CTA surveillance imaging performed for MFS patients in this study, with all patients receiving a low-risk cumulative radiation dose (less than 1 Gy) and all patients having a low excessive lifetime risk of cancer as a result of CTA. The risk-benefit decision must be made at the point of care, and it entails balancing the benefits of surveillance imaging in anticipating rupture and providing practical, safe treatment, therefore avoiding morbidity and mortality.

Comparison of radiation dose and its correlates between coronary computed tomography angiography and invasive coronary angiography in Northeastern Thailand.

BACKGROUND: The number of coronary computed tomography angiography (CCTA) exams is steadily growing. A novel computed tomography (CT) system has been developed to increase image quality while lowering patient radiation. The radiation dose attributed to CCTA has received considerable attention, whereas the dose associated with invasive catheter angiography (ICA) has received less. This study aims to investigate the radiation exposure of CCTA in patients and compare it to ICA. RESULTS: The mean effective dose of CCTA was 2.88 ± 0.85 mSv which was significantly lower than the mean effective dose of ICA (5.61 ± 0.55 mSv), p < 0.0001. The effective dose of CCTA correlated with the weight, height, and BMI, while the effective dose of ICA was associated with patient weight and BMI. The radiation exposure from CCTA has been considerably reduced over the last ten years by almost 2.5 folds. The mean radiation dose from the newer generation CT used in 2019 was significantly lower than that of the single-source CT in 2010 (2.88 ± 0.85 mSv vs. 7.15 ± 3.4 mSv, p < 0.001). CONCLUSIONS: CCTA allows evaluation of CAD with a significantly less effective radiation dose to patients than diagnostic ICA. There was a significant decrease in radiation dose from CCTA over time. Regular measurement of patient doses is an essential step to optimize exposure. It makes operators aware of their performance and allows comparisons with generally accepted practices.

The Thai reference exome (T-REx) variant database.

To maximize the potential of genomics in medicine, it is essential to establish databases of genomic variants for ethno-geographic groups that can be used for filtering and prioritizing candidate pathogenic variants. Populations with non-European ancestry are poorly represented among current genomic variant databases. Here, we report the first high-density survey of genomic variants for the Thai population, the Thai Reference Exome (T-REx) variant database. T-REx comprises exome sequencing data of 1092 unrelated Thai individuals. The targeted exome regions common among four capture platforms cover 30.04 Mbp on autosomes and chromosome X. 345 681 short variants (18.27% of which are novel) and 34 907 copy number variations were found. Principal component analysis on 38 469 single nucleotide variants present worldwide showed that the Thai population is most genetically similar to East and Southeast Asian populations. Moreover, unsupervised clustering revealed six Thai subpopulations consistent with the evidence of gene flow from neighboring populations. The prevalence of common pathogenic variants in T-REx was investigated in detail, which revealed subpopulation-specific patterns, in particular variants associated with erythrocyte disorders such as the HbE variant in HBB and the Viangchan variant in G6PD. T-REx serves as a pivotal addition to the current databases for genomic medicine.

Cardiac computed tomography angiography in the pre-operative assessment of congenital heart disease in Thailand.

INTRODUCTION: Cardiac computed tomography angiography (CCTA) plays a vital role in clinical practice in evaluating patients with congenital heart disease (CHD) when the information from echocardiography is equivocal. AIM: To test the hypothesis that CCTA has significantly value for pre-operative evaluation of congenital heart disease and practicality in the diagnosis and management of congenital heart disease patients at our tertiary care academic hospital. MATERIAL AND METHODS: We studied a total of 78 congenital heart disease patients (median age: 4.5 years) who had undergone CCTA during the period January 2017 to October 2018 at our tertiary care academic hospital. RESULTS: The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was analysed. In each group, the CCTA offered an advantage and provided specific clues for surgical or interventional management. In total, the sensitivity (97.5%), specificity (100%), positive predictive value (100%), negative predictive value (99.38%), and accuracy (99.5%) of CCTA, for which the significant findings were confirmed by surgical or cardiac catheterization, were excellent, with average exposure per CCTA study calculated at 1.41 (0.36-3.28) mSv. CONCLUSIONS: CCTA is an excellent non-invasive modality for the evaluation of congenital heart disease patients, with an important diagnostic and decision-aiding role.

Left Ventricular Thrombi: Insights from Cardiac Magnetic Resonance Imaging.

Objective: Cardiovascular magnetic resonance imaging (CMR) late gadolinium enhancement technique (LGE) detects thrombus rather than anatomical presence based on tissue properties and is theoretically highly accurate. The present study's goal was to compare the diagnostic accuracy obtained with various CMR techniques and transthoracic echocardiography to diagnose left ventricular thrombus and evaluate the prevalence and perspectives of left ventricular (LV) thrombus among patients with impaired systolic left ventricular function. Methods: In a single academic referral center, a retrospective database review of all CMR assessments of the established left ventricular thrombus was carried out in 206 consecutive patients with reduced systolic function for five years. To assess thrombus risk factors, clinical and imaging parameters were analyzed. Sensitivity, specificity, negative predictive value (NPV), positive predictive value (PPV), echocardiography, and cine-CMR sequence accuracy have been identified. LV structural parameters were quantified to detect markers for thrombus and predictors of the additive usefulness of contrast-enhanced thrombus imaging. Comparisons against LGE-CMR were made, which was used as the standard. Results: A 7.8 percent prevalence of left ventricular thrombus was identified by LGE-CMR. Cine-CMR increased the diagnostic efficiency for echocardiographic thrombus identification in this group, with sensitivity increasing from 50 percent by echocardiography to 75 percent by cine-CMR (p = 0.008). Dark blood CMR (DB-CMR) has better sensitivity and accuracy than echocardiography (p < 0.001), comparable to cine-CMR. The transmural infarct size was an independent marker for thrombus after correction for the LVEF and LV volume while considering only CMR parameters. There were significantly higher embolic events (HR = 71.33; CI 8.31-616.06, p < 0.0001) in LV thrombus patients detected by LGE-CMR. Conclusion: CMR imaging was more sensitive to left ventricular thrombi identification compared with transthoracic echocardiography. An additional parameter available from LGE-CMR and shown as an independent risk factor for left ventricular thrombus is the myocardial scar.

Incidence and Outcome of Unexpected Non-Cardiac Abnormality on Cardiac Magnetic Resonance Imaging: A Report from Northeastern Thailand.

Objective: To ascertain non-cardiac abnormality (NCA) incidence in patients undergoing clinical cardiovascular magnetic resonance imaging (CMR) and determine such patients' clinical importance. Methods: Consecutive patients undertaking CMR study from January 2012 to June 2017 for various cardiovascular diseases were enrolled. To assess NCA's therapeutic importance, all incidental findings that were not expected from the patient's history were analyzed. A careful review of medical history determines the information on mortality. Results: Three hundred and eighty-two consecutive patients (mean age 58 ± 11 years) who underwent CMR for different clinical indications were enrolled in the present study. Potentially significant results have been identified as abnormalities that require further clinical or radiological follow-up or therapy. On CMR, 118 NCA (30.9%) were found. In 25 patients, potential clinically significant NCAs, such as aortic aneurysm (n = 3), aortic dissection (n = 2), pulmonary thromboembolism (n = 2), and malignancy (n = 18), were identified (6.54%). In terms of one-year mortality data, in a patient without NCA, we observed a significantly higher survival rate than those with NCA (p = 0.0085) and a higher mortality rate in a patient with clinically significant NCA than a patient with irrelevant NCA (p = 0.02). Survival, as assessed via Kaplan‒Meier analysis, disclosed significantly higher mortality in the patients with clinically significant NCA than patients with irrelevant NCA (HR = 11.20, CI = 4.71-26.60, p < 0.001). Conclusions: We concluded that it is vital for the CMR study to determine the relevance of NCA, especially in the cholangiocarcinoma endemic region such as northeastern Thailand. Eventually, to reorganize the patients according to appropriate management, clinical correlation and prognosis must be summarily established.

Importance of computed tomography pulmonary angiography for predict 30-day mortality in acute pulmonary embolism patients.

OBJECTIVE: The purpose of the present study was to assess the implications of different parameters of computed tomography pulmonary angiography (CTPA) to predict 30-day mortality in acute pulmonary embolism (APE) patients. MATERIAL AND METHOD: Patients who had clinical suspicion of APE and underwent CTPA were recruited in a retrospective cohort study. The findings of the CTPA included the parameters of right ventricular dysfunction (RVD), the severity of obstruction to the pulmonary artery by CT obstruction index, and the ratio of pulmonary trunk diameter and aorta. The endpoint of the study was established as the 30-day mortality associated with APE. RESULTS: A total of 238 patients with a confirmed APE diagnosis with CTPA were included in the study; 26 (10.9 %) of those patients died within 30 days. In patients with cancer and the Pulmonary Embolism Severity Index (PESI) class 5, the mortality rate was significantly higher. Compared with survivors, the mean CT obstruction index in the non-survivor group was significantly higher (p < 0.001). Higher mortality was associated with all RVD parameters identified by CTPA, such as the RV/LV ratio (p < 0.001), interventricular septum deviation grade 3 (p < 0.001), increased RV diameter (p < 0.001), and IVC contrast reflux (p < 0.001). The highest adjusted odds ratio was RV diameter at 1.094, followed by PESI and the CT obstruction index at 1.040. CONCLUSION: CTPA-detected RVD parameters and CT obstruction index can predict a 30-day mortality rate in APE patients and be used for risk stratification. In APE patients, the RV diameter of 53 mm or greater and the CT obstruction index >70% is associated with increased 30-day mortality.

The Importance of Cardiac T2* Magnetic Resonance Imaging for Monitoring Cardiac Siderosis in Thalassemia Major Patients.

Objective: Cardiac T2* magnetic resonance imaging (MRI) has recently attracted considerable attention as a non-invasive method for detecting iron overload in various organs in thalassemia major patients. This study aimed to identify the prevalence of cardiac siderosis in thalassemia major patients and evaluate cardiac T2* MRI for monitoring cardiac siderosis before and after patients receive iron chelation therapy and its relation to serum ferritin, left ventricular ejection fraction, and liver iron concentration. The information gathered would be used for the direct monitoring, detection, and treatment of complications early on. Methods: A total of 119 thalassemia major patients were recruited in the present study. The cardiac T2* MRI was compared to serum ferritin levels, liver iron concentration (LIC), and left ventricular ejection fraction. All patients were classified into four groups based on their cardiac siderosis as having normal, marginal, mild to moderate, or severe cardiac iron overload. At the follow-up at years one, three, and five, the cardiac T2* MRI, LIC, serum ferritin, and left ventricular ejection fraction (LVEF) were determined. Results: The prevalence of cardiac siderosis with cardiac T2* MRI ≤ 25 ms was 17.6% (n = 21). There was no correlation between cardiac T2* MRI and serum ferritin, liver iron concentration, and LVEF (p = 0.39, 0.54, and 0.09, respectively). During one year to five years' follow-up periods, cardiac T2* MRI (ms) in patients with severe cardiac siderosis had significantly improved from 8.5 ± 1.49 at baseline to 33.9 ± 1.9 at five years (p < 0.0001). Patients with severe, mild-moderate, marginal, and no cardiac siderosis had median LIC (mg/g dw) of 23.9 ± 6.5, 21.6 ± 13.3, 25.3 ± 7.7, and 19.9 ± 5.5 at baseline, respectively. Conclusions: This study supports the use of cardiac T2* MRI to monitor cardiac iron overload in patients who have had multiple blood transfusions. Early diagnosis and treatment of patients at risk of cardiac siderosis is a reasonable method of reducing the substantial cardiac mortality burden associated with myocardial siderosis. Cardiac T2* MRI is the best test that can identify at-risk patients who can be managed with optimization of their chelation therapy.

Prevalence of intracardiac thrombi on cardiac computed tomography angiography: Outcome and impact on consequent management.

OBJECTIVE: Intracardiac thrombi are intermittently come across on cardiac computed tomography angiography (CCTA). This study aimed to examine the prevalence, outcome, and prognosis in patients with incidental found left-sided cardiac thrombi on CCTA. MATERIAL AND METHODS: The Ethics Committee approved the present study of the Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. A retrospective review of CCTA was performed for incidental left-sided cardiac thrombi. RESULTS: A total of 1080 CCTAs were enrolled with the prevalence of incidental left-sided cardiac thrombi is 4.53%. Of the 49 patients with CCTA incidental left-sided cardiac thrombi, 16 had left atrial thrombi, and 33 had left ventricular thrombi. All thrombi were undetermined before the CCTA, and their identification subsequently generated anticoagulation treatment. In 10 patients, embolic complications happened, 4 of which were fatal. Patients with incidental detected left-sided intracardiac thrombi seen by CCTA had more embolic event than patients who did not discover left-sided intracardiac thrombi by CCTA (HR = 8.07; 95% CI 1.48-44.06; p = 0.016). CONCLUSIONS: Incidental left-sided cardiac thrombi on CCTA guided to management adjustments and seemed to present substantial mortality and morbidity in the present study. Physicians who interpret CCTA should ensure a dedicated effort not to disregard these prospective pitfalls.

Diagnosis and prognostic significance of anomalous origin of coronary artery from the opposite sinus of Valsalva assess by dual-source coronary computed tomography angiography.

BACKGROUND: Clinically recognized coronary anomalies in adults are infrequent and characteristically establish on autopsy. The clinical importance coronary arteries consist of those with anomalous origin of the coronary artery from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course (IAC). We have endeavored to attribute variable risk based on morphological appearances of the ACAOS by dual-source coronary computed tomography angiography (DSCTA). MATERIAL AND METHODS: ACAOS patients who undergoing DSCTA over a 5-year period were identified and assess morphologic characteristics. Medical records were reviewed for major adverse cardiovascular events (MACEs). RESULTS: A total of 1126 patients who undergoing DSCTA were evaluated. Twenty-four patients with ACAOS with IAC were recognized with a prevalence of 2.1% of study population. Twenty patients had anomalous origin of right coronary artery from left sinus of Valsalva (RCA-LSV) and 4 patients had anomalous origin of left coronary from right sinus of Valsalva (LCA-RSV). A significant increased prevalence of MACEs in were observed in intramural, slit-like and high inter-arterial course morphology group. Median follow-up time was 13 months (IQR 3-18  months). During follow-up 2 died, 4 had PCI, 13 had myocardial infarction and 7 had surgical treatment. CONCLUSIONS: Intramural, slit-like and high type inter-arterial course morphology of ACAOS with IAC are high risk features for MACEs which can be identified by DSCTA.

Association of Major Histocompatibility Complex Class I Related Chain A/B Positive Microparticles with Acute Myocardial Infarction and Disease Severity.

BACKGROUND: Various cell types undergo activation and stress during atherosclerosis resulting in the development of acute myocardial infarction (AMI) in coronary artery disease (CAD). Major histocompatibility complex class I related chain A and B (MICA/B) can be expressed on the surface of activated and stressed cells and released into blood circulation in several forms including microparticles (MICA/B+ MPs) from various cell types. We aimed to investigate the association of these MICA/B+ MPs with the presence of AMI. Fifty-one AMI and 46 age-matched control subjects were recruited. METHODS: Levels of MICA/B+ MPs derived from various parent cells including endothelial cells, platelets, monocytes, neutrophils, and T lymphocytes were determined by flow cytometry. RESULTS: The levels and proportion of MICA/B+ MPs from all types of cell origin were significantly increased in AMI patients compared to those of the controls. A multivariate regression model showed an independent association between MICA/B+ MPs and AMI (OR = 11.6; 95% CI = 2.8, 47.3). Interestingly, based on the disease severity, we found that the levels of MICA/B+ MPs were significantly elevated in the ST-segment elevation myocardial infarction (STEMI) compared to the non-STEMI (NSTEMI) patients. Moreover, an independent association of MICA/B+ MPs with the occurrence of STEMI was also demonstrated (OR = 4.1; 95% CI = 1.5, 16.7). CONCLUSIONS: These results suggest that MICA/B+ MPs are associated with AMI and disease severity. They may act as mediators contributing to the pathological process of AMI. Alternatively, they are the results of various cell activations contributing to AMI.

Common and rare susceptibility genetic variants predisposing to Brugada syndrome in Thailand.

BACKGROUND: Mutations in SCN5A are rarely found in Thai patients with Brugada syndrome (BrS). Recent evidence suggested that common genetic variations may underlie BrS in a complex inheritance model. OBJECTIVE: The purpose of this study was to find common and rare/low-frequency genetic variants predisposing to BrS in persons in Thailand. METHODS: We conducted a genome-wide association study (GWAS) to explore the association of common variants in 154 Thai BrS cases and 432 controls. We sequenced SCN5A in 131 cases and 205 controls. Variants were classified according to current guidelines, and case-control association testing was performed for rare and low-frequency variants. RESULTS: Two loci were significantly associated with BrS. The first was near SCN5A/SCN10A (lead marker rs10428132; odds ratio [OR] 2.4; P = 3 × 10-10). Conditional analysis identified a novel independent signal in the same locus (rs6767797; OR 2.3; P = 2.7 × 10-10). The second locus was near HEY2 (lead marker rs3734634; OR 2.5; P = 7 × 10-9). Rare (minor allele frequency [MAF] <0.0001) coding variants in SCN5A were found in 8 of the 131 cases (6.1% in cases vs 2.0% in controls; P = .046; OR 3.3; 95% confident interval [CI] 1.0-11.1), but an enrichment of low-frequency (MAF<0.001 and >0.0001) variants also was observed in cases, with 1 variant (SCN5A: p.Arg965Cys) detected in 4.6% of Thai BrS patients vs 0.5% in controls (P = 0.015; OR 9.8; 95% CI 1.2-82.3). CONCLUSION: The genetic basis of BrS in Thailand includes a wide spectrum of variant frequencies and effect sizes. As previously shown in European and Japanese populations, common variants near SCN5A and HEY2 are associated with BrS in the Thai population, confirming the transethnic transferability of these 2 major BrS loci.

The value of magnetic resonance imaging in evaluation of myocardial and liver iron overload in a thalassaemia endemic population: a report from Northeastern Thailand.

PURPOSE: Patients with chronic haemolytic anaemia, such as in thalassaemia, require repeated blood transfusions, which leads to iron overload and cellular damage, especially in the heart and liver. Classically, serum ferritin and liver biopsy have been used to monitor patient response to chelation therapy. Magnetic resonance imaging (MRI) has proven to be effective in detecting and quantifying iron in the heart and liver. The aim of the paper is to evaluate the accuracy of the MRI T2* procedure in the assessment of liver iron concentration and myocardial iron overload. MATERIAL AND METHODS: In 210 cases of monthly transfused patients, hepatic and myocardial iron overload was measured by multi-breath-hold MRI T2* and compared to serum ferritin (a traditional marker of iron overload). RESULTS: No significant correlation was observed between serum ferritin level and cardiac T2* MRI (p = 0.68, r = 0.06). However, a significant correlation was observed between serum ferritin and liver iron concentration evaluated by MRI (p = 0.04, r = 0.68). CONCLUSION: Routine evaluation of liver and heart iron content using MRI T2* is suggested to better evaluate the haemosiderosis status in thalassaemic patients.

Value of cardiac computed tomography angiography in pre-operative assessment of infective endocarditis.

BACKGROUND: Substantial development of cardiac computed tomography angiography (CTA) technology in the last decade has commanded to increase usage of this modality for assessing infective endocarditis (IE). The objective of this study is to evaluate the sensitivity and specificity of preoperative cardiac CTA imaging as opposed to transthoracic echocardiography (TTE) in the assessment of complications associated to IE, with comparison to surgical findings. METHODS: Among 52 patients with surgically proven IE in our database, 24 underwent contrast-enhanced ECG cardiac CTA and were included in the study and all of them also underwent TTE. RESULTS: For the detection of pseudoaneurysm/abscess in both native and prosthetic valves, cardiac CTA demonstrated significantly higher sensitivity (91.5% vs. 15.8%, p < 0.0001) with similar specificity (81.25). Cardiac CTA demonstrated similar sensitivity and specificity in identifying vegetation and valvular dehiscence in all patients. CONCLUSIONS: Preoperative cardiac CTA can be seen as complementary to TTE in assessing complications such as pseudoaneurysm or abscess of the patients with IE.

Retraction: Value of cardiac magnetic resonance imaging in systemic sclerosis.

[This retracts the article DOI: 10.5114/reum.2018.75520.].

Value of cardiac magnetic resonance imaging in systemic sclerosis.

OBJECTIVES: To assess the prevalence and patterns of cardiac abnormalities as detected by cardiac magnetic resonance imaging (MRI) in systemic sclerosis. MATERIAL AND METHODS: Twenty-six consecutive patients with systemic sclerosis underwent cardiac MRI to determine morphological, functional, perfusion at rest, and delayed enhancement abnormalities. RESULTS: At least one abnormality on cardiac MRI was observed in 19/26 (73%) patients. Increased myocardial signal intensity in T2 was observed in 10 patients (38.5%), thinning of the left ventricular myocardium in 1 patient (3.5%), and pericardial effusion in 12 patients (46%). Left and right ventricular ejection fractions were altered in 10 patients (38.5%) and 11 patients (42%), respectively. Myocardial delayed contrast enhancement was found in 11 (42%) patients. No perfusion defects at rest were found. Patients with limited systemic sclerosis had similar cardiac MRI abnormalities to patients with diffuse systemic sclerosis. Four out of 11 patients (36.4%) without pulmonary arterial hypertension had right ventricular dilatation. CONCLUSIONS: The present study shows that cardiac MRI is an accurate and reliable technique to diagnose cardiac involvement in systemic sclerosis and to analyze precisely its mechanisms, including inflammatory, microvascular and fibrotic components. As it is non-invasive, quantitative and highly sensitive, cardiac MRI appears to be a method of choice to determine the natural history of untreated patients or to accurately monitor the effects of treatment. Moreover, it could provide powerful prognostic factors in both groups. Compared to echocardiography, cardiac MRI appears to provide additional information by visualizing myocardial fibrosis and inflammation. Finally, the present study has shown that RV dilatation is not specific for pulmonary arterial hypertension and could correspond to a specific heart involvement in systemic sclerosis.