Reference values of myocardial native T1 and extracellular volume in patients without structural heart disease and had negative 3T cardiac magnetic resonance adenosine stress test.

Background: To establish the reference values of native T1 and extracellular volume (ECV) in patients without structural heart disease and had a negative adenosine stress 3T cardiac magnetic resonance. Methods: Short-axis T1 mapping images were acquired using a modified Look-Locker inversion recovery technique before and after administration of 0.15 mmol/kg gadobutrol to calculate both native T1 and ECV. To compare the agreement between measurement strategies, regions of interest (ROI) were drawn in all 16 segments then averaged to represent mean global native T1. Additionally, an ROI was drawn in the mid-ventricular septum on the same image to represent the mid-ventricular septal native T1. Results: Fifty-one patients (mean 65 years, 65 % women) were included. Mean global native T1 averaged from all 16 segments and a mid-ventricular septal native T1 were not significantly different (1221.2 ± 35.2 vs 1228.4 ± 43.7 ms, p = 0.21). Men had lower mean global native T1 (1195 ± 29.8 vs 1235.5 ± 29.4 ms, p < 0.001) than women. Both mean global and mid-ventricular septal native T1 were not correlated with age (r = 0.21, p = 0.13 and r = 0.18, p = 0.19, respectively). The calculated ECV was 26.6 ± 2.7 %, which was not influenced by either gender or age. Conclusions: We report the first study to validate the native T1 and ECV reference ranges, factors influencing T1, and the validation across measurement methods in older Asian patients without structural heart disease and had a negative adenosine stress test. These references allow for better detection of abnormal myocardial tissue characteristics in clinical practice.

Prevalence of achlorhydria in an Asian population detected using Congo red staining during routine gastroscopy: 22 years' experience from a single centre.

OBJECTIVE: We aimed to study the prevalence of achlorhydria (AC) in a large Asian population. DESIGN: Medical records of patients who underwent oesophagogastroduodenoscopy (OGD) with Congo red staining method at the Vichaiyut Hospital from January 2010 to December 2019 were retrospectively reviewed. RESULTS: A total of 3597 patients was recruited; 223 were excluded due to concurrent use of proton pump inhibitors. Eighteen from 3374 patients (0.53%) had AC. Seven patients were presented with permanent AC (5F, 2M) (median age=69 years; range 58-92). Among 11 patients with temporary AC (5M, 6F: mean age 73.4 years; SD 13.2 years), all had gastrointestinal Helicobacter pylori bacterial infection and were over 45 years old. After successful treatment for H. pylori, AC was absent among patients with temporary AC. If counting only patients over 45 years of age, the prevalence of AC was 0.68% (18/2614). No adverse events arising from Congo red occurred. CONCLUSION: AC is relatively rare. Permanent and temporary AC were found only when they were over 55 and 45 years old, respectively. Staining Congo red on gastric mucosa can be safely and routinely incorporated into the OGD procedure for early detection of AC. We recommended a low-cost screening test such as serum vitamin B levels for screening only in patients aged 50 and over.

Ala97Ser transthyretin amyloidosis-associated polyneuropathy, clinical and neurophysiological profiles in a Thai cohort.

BACKGROUND: Ala97Ser transthyretin amyloidosis-associated polyneuropathy (ATTRA97S-PN) is a rare form of inherited polyneuropathy, usually manifesting with late-onset (> 50) progressive polyneuropathy. This mutation is mostly prevalent in Taiwanese and Han-Chinese individuals. The aim of this study was to describe the clinical and comprehensive neurophysiological profiles of ATTRA97S-PN in Thai patients. METHODS: The clinical profiles and serial neurophysiologic studies (nerve conduction study (NCS), quantitative sensory test (QST), and comprehensive autonomic function test (AFT)) of symptomatic ATTRA97S-PN patients who had been followed-up at King Chulalongkorn Memorial Hospital during 2010-2020 were retrospectively reviewed. RESULTS: Nine symptomatic patients (55.6 % were male) from four unrelated families were included. All were Thais of mixed Thai Chinese descent. The mean age of onset was 48.3 (32-60) years. The mean age at diagnosis was 54.8 (33-66) years. Three patients developed early-onset (< 40y) polyneuropathy. The mean Neuropathy Impairment Score was 41.33 (10-92) at diagnosis. Sensory (9/9) and autonomic (9/9) neuropathies were more frequent than motor neuropathy (5/9), which appeared in the late stage of disease. Hypoesthesia in the feet, and gastrointestinal autonomic symptoms were frequently reported as the initial symptoms. The course of neuropathy progressed over years to decades. The worsening of neuropathy tended to progress faster once motor nerves were affected in both clinical and neurophysiological aspects. Concurrent cardiac amyloidosis was found in 6/9 patients. NCS showed length-dependent sensorimotor axonal polyneuropathy in 5/9 patients, and median neuropathy at the wrist (mostly bilateral) in 7/9 patients. QST showed abnormalities in the vibratory detection threshold, the cold detection threshold and the heat pain sensation in 8/9, 8/9 and 7/7 tested patients, respectively. AFT results were abnormal in all. The mean composite autonomic severity score was 5 (3-9). CONCLUSIONS: This clinical study is the first of ATTRA97S-PN in Thai patients. The mixed polyneuropathy-cardiopathy phenotype was the most common manifestation. In this cohort, the age of onset was lower, and the course of neuropathy was relatively longer, than that in previous studies. Some patients may develop early-onset polyneuropathy. This mutation has not yet been documented in any population other than Han Chinese-related populations, probably suggesting a founder effect. Further studies are warranted.

Very late presentation of anomalous origin of the left coronary artery from the pulmonary artery: case report.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. The enlarged right coronary artery provides retrograde collaterals to supply the left ventricle then preferentially directs into the lower pressure pulmonary artery system causing coronary steal phenomenon. Few patients who survive through adulthood without surgery must have abundant, well-formed functioning collaterals with adequate perfusion of the left ventricle. We present the oldest reported patient with ALCAPA to undergo corrective surgery. CASE PRESENTATION: A 79-year-old woman presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination discovered a soft continuous murmur at the left upper chest. Transthoracic echocardiography demonstrated an unusual, tubular-like structure inside the interventricular septum with a turbulent flow from color Doppler. Moreover, there was a severe mitral regurgitation from posterior mitral leaflet restriction associated with ventricular remodeling in combination with mitral annular dilatation. Coronary angiography and coronary computed tomography angiography established the diagnostic hallmark of ALCAPA syndrome. Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia suggesting adequate collateral circulation. Remarkably, there was a left coronary ostial stenosis, which served as a protective mechanism against myocardia ischemia by limiting the steal effect. The patient successfully underwent the ligation of anomalous artery at its origin in combination with bioprosthetic mitral valve replacement. Her postoperative course was uneventful. CONCLUSIONS: This case utilized multimodality imaging for delineating the course of abnormal vessels and helping to formulate therapeutic decision.

Effect of the 2010 task force criteria on reclassification of cardiovascular magnetic resonance criteria for arrhythmogenic right ventricular cardiomyopathy.

BACKGROUND: We sought to evaluate the effect of application of the revised 2010 Task Force Criteria (TFC) on the prevalence of major and minor Cardiovascular Magnetic Resonance (CMR) criteria for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) versus application of the original 1994 TFC. We also assessed the utility of MRI to identify alternative diagnoses for patients referred for ARVC evaluation. METHODS: 968 consecutive patients referred to our institution for CMR with clinical suspicion of ARVC from 1995 to 2010, were evaluated for the presence of major and minor CMR criteria per the 1994 and 2010 ARVC TFC. CMR criteria included right ventricle (RV) dilatation, reduced RV ejection fraction, RV aneurysm, or regional RV wall motion abnormalities. When quantitative measures of RV size and function were not available, and in whom abnormal size or function was reported, a repeat quantitative analysis by 2 qualified CMR physicians in consensus. RESULTS: Of 968 patients, 220 (22.7%) fulfilled either a major or a minor 1994 TFC, and 25 (2.6%) fulfilled any of the 2010 TFC criterion. Among patients meeting any 1994 criteria, only 25 (11.4%) met at least one 2010 criterion. All patients who fulfilled a 2010 criteria also satisfied at least one 1994 criterion. Per the 2010 TFC, 21 (2.2%) patients met major criteria and 4 (0.4%) patients fulfilled at least one minor criterion. Eight patients meeting 1994 minor criteria were reclassified as satisfying 2010 major criteria, while 4 patients fulfilling 1994 major criteria were reclassified to only minor or no criteria under the 2010 TFC.Eighty-nine (9.2%) patients had alternative cardiac diagnoses, including 43 (4.4%) with clinically significant potential ARVC mimics. These included cardiac sarcoidosis, RV volume overload conditions, and other cardiomyopathies. CONCLUSIONS: Application of the 2010 TFC resulted in reduction of total patients meeting any diagnostic CMR criteria for ARVC from 22.7% to 2.6% versus the 1994 TFC. CMR identified alternative cardiac diagnoses in 9.2% of patients, and 4.4% of the diagnoses were potential mimics of ARVC.

Serum NT-proBNP in the early detection of doxorubicin-induced cardiac dysfunction.

AIM: Cardiac dysfunction is a major limitation of anthracycline treatment in cancer patients. There are several useful serum markers in other types of cardiomyopathy, including N-terminal pro-brain-natriuretic peptide (NT-proBNP), troponin-T and creatine kinase MB isoform. We investigated the potential application of these serum biomarkers in cancer patients receiving treatment with anthracycline. METHODS: We collected data from 52 female breast cancer patients receiving doxorubicin and cyclophosphamide every 3 weeks for four cycles. Cardiac function evaluations by echocardiography were done at baseline and at the end of the fourth cycle of chemotherapy. Patients' blood samples were serially measured for cardiac biomarkers. RESULTS: The mean cumulative dose of doxorubicin in this study was 237 mg/m(2) . No symptomatic heart failure was detected during the study period. However, there were significant asymptomatic reductions of left ventricular ejection fraction (LVEF) from mean ± SD 70.7 ± 6% at baseline to 67.0 ± 5% (P < 0.001). By clinical toxicity criteria the LVEF decline was grade I in 18% and grade II in 4%. After one dose of chemotherapy, a significant rise of serum NT-proBNP occurred in patients who subsequently developed an LVEF reduction compared with patients with normal LVEF (P = 0.04). A correlation analysis demonstrated that the reduction of fractional shortening was significantly associated with elevated NT-proBNP (r = -0.016, P = 0.014). CONCLUSION: Asymptomatic reductions in cardiac function are common in breast cancer patients treated with doxorubicin. NT-proBNP may serve as a convenient serum biomarker for the early detection of cardiotoxicity induced by anthracycline.

Comparison of dual-source 64-slice adenosine stress CT perfusion with stress-gated SPECT-MPI for evaluation of left ventricular function and volumes.

BACKGROUND: Evaluation of left ventricular (LV) volumes and ejection fraction (LVEF) represent important components of pharmacologic stress imaging with either myocardial CT perfusion (CTP) or gated single-photon emission CT (SPECT) myocardial perfusion imaging (SPECT-MPI). OBJECTIVES: We compared measurements of left ventricular function and volumes obtained with CTP and SPECT-MPI. METHODS: Forty-seven patients (mean age, 62 ± 11 years; male, n = 39) underwent stress CTP and SPECT-MPI. LVEF (in %), end-systolic volume (ESV; in mL), and end-diastolic volume (EDV; in mL) derived from stress CTP images were compared with SPECT-MPI. RESULTS: Stress CTP was in good agreement with SPECT-MPI for quantification of LVEF (r = 0.91), EDV (r = 0.75), and ESV (r = 0.83; all P < 0.001). The mean LVEF measured by stress CTP (66% ± 17%) was similar to SPECT-MPI (64% ± 15%). Similar values were also derived for mean EDV (123 ± 30 mL vs 120 ± 34 mL) and ESV (44 ± 28 mL vs 51 ± 34 mL) for CTP and SPECT-MPI, respectively. Good agreement was also shown between both techniques for the assessment of regional wall motion with identical wall motion scores in 95.3% of the segments (κ = 0.79). CONCLUSIONS: LVEF and LV volume parameters as determined by dual-source 64-slice adenosine stress CTP show a high correlation with values obtained with stress-gated SPECT-MPI.