A Case Report of Wet Beriberi Due to Excessive White Rice Consumption in an Elderly Male Patient: A Potentially Forgotten and Underrecognized Disease.

Thiamine deficiency can cause various clinical manifestations. Wet beriberi, a phenotype of thiamine deficiency, is often underdiagnosed in clinical practice due to the nonspecificity of symptoms. An 83-year-old man presented to a primary care clinic with a two-month history of progressing edema in the scrotum and lower extremities. The patient reported a weight gain of 10 kg and was treated with diuretics. However, his condition did not improve even after four weeks. The patient was referred to our hospital for further cardiac evaluation. Transthoracic echocardiographic findings were suggestive of a high cardiac output (CO) state, and the thiamine level was decreased. Further medical interview revealed that the patient had a habit of eating two or three large bowls of white rice with a few side dishes for breakfast and dinner. The hemodynamic evaluation revealed high CO and low systemic vascular resistance. The patient's weight decreased from 56.6 to 52.4 kg in the first 2 days after thiamine administration. Six days later, his weight further decreased to 50.8 kg and edema disappeared completely. Clinicians should be aware that excessive consumption of white rice with few side dishes may lead to thiamine deficiency. This case highlights the importance of considering wet beriberi as a cause of excessive edema with high CO state.

Apical Acute Myocardial Infarction Due to Occluded Posterior Descending Branch of Right Coronary Artery Concomitant With Short Left Anterior Descending Artery: Multi-imaging Modality Assessment.

Regional wall motion abnormality in the left ventricular (LV) apex detected on transthoracic echocardiography is commonly interpreted as the presence of a distal left anterior descending (LAD) artery lesion in clinical practice. Herein, we reported a rare case of apical acute myocardial infarction (AMI) caused by an occluded posterior descending branch of the right coronary artery (RCA), in which the correspondence between coronary arterial anatomy and supplied LV apex was evaluated by multi-imaging modalities. Despite the presence of regional wall motion abnormality in the LV apex, left coronary angiography showed no significant coronary artery diseases. It was of note that LAD terminated before the LV apex. Right coronary angiography showed total occlusion of the posterior descending branch. Cardiac computed tomography (CT) clearly demonstrated that the spontaneously recanalized posterior descending branch extended toward the LV apex. Cardiac magnetic resonance imaging (MRI) clearly revealed regional wall motion abnormality corresponding to myocardial edema in the LV apex. Cardiac CT and MRI were powerful tools in clarifying the correspondence between coronary arterial anatomy and supplied LV apex. Clinicians should be aware that localized apical AMI can occur under the condition of occluded posterior descending branch of RCA concomitant with short LAD.

Deep vein thrombosis in a patient with wild-type transthyretin cardiac amyloidosis.

Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) is a progressive myocardial disease characterized by an increased ventricular wall thickness. ATTRwt-CA is often accompanied by monoclonal gammopathy of undetermined significance (MGUS), as indicated by an abnormal serum-free light chain ratio or detection of monoclonal protein (M-protein) on serum immunofixation electrophoresis. Increasing evidence has emerged regarding the risk of venous thromboembolic disease in patients with MGUS. Herein, we report a case of ATTRwt-CA with M-protein, where the patient developed deep vein thrombosis (DVT). A 72-year-old man presented with gradual progressive swelling of the right lower extremity. He had undergone surgical treatment for bilateral carpal syndrome and lumbar spinal canal stenosis. He was diagnosed with DVT and severe left ventricular (LV) hypertrophy, and was treated with a direct factor Xa inhibitor. Given severe LV hypertrophy, the patient underwent further cardiac examinations. 99mTc-labelled pyrophosphate scintigraphy revealed grade 3 myocardial uptake. Serum immunofixation electrophoresis detected IgA-κ M-protein. An endomyocardial biopsy revealed amyloid deposits. Based on immunohistochemical staining and genetic testing, the patient was diagnosed as having ATTRwt-CA with IgA-κ M-protein. Therefore, clinicians should be aware that ATTRwt-CA is often associated with M-protein. Under these conditions, attention should be paid to both pathological issues. Learning objective: Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) is often accompanied by monoclonal gammopathy of undetermined significance (MGUS), as indicated by an abnormal serum-free light chain ratio or detection of monoclonal protein (M-protein). Meanwhile, increasing evidence has emerged about the risk of deep vein thrombosis (DVT) in patients with MGUS. We report a case of ATTRwt-CA with M-protein, in which DVT developed. Under these conditions, paying attention to both pathological aspects is warranted.

A Case of New-Onset Atrial Tachyarrhythmias With Apical Hypertrophic Cardiomyopathy and Bronchiectasis in a Very Elderly Patient: A Therapeutic Dilemma.

Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease that is genetically transmitted as an autosomal dominant trait. Even apical HCM (ApHCM) induces atrial fibrillation (AF) based on underlying left ventricle (LV) diastolic dysfunction, where anticoagulation therapy is recommended. However, anticoagulation for AF in patients at high risk of bleeding is a double-edged sword. A 98-year-old woman living in a nursing home presented to our hospital with sudden-onset dyspnea and palpitation persisting for two hours. The patient had a history of apical HCM and bronchiectasis. An electrocardiogram showed a regular tachycardia with a heart rate of 130 bpm, suggesting atrial flutter with 2:1 atrioventricular conduction. Intravenous verapamil (5 mg) resulted in the conversion into AF, and subsequent cibenzoline (70 mg) failed to restore sinus rhythm. Given the impossibility of continuous anticoagulation, electrical cardioversion was planned. Electrical cardioversion was successful in converting AF into sinus rhythm. Given the very high risk of hemoptysis, anticoagulation was avoided. This case gives an insight into how to manage a practical therapeutic problem, which is the coexistence of AF and bronchiectasis. A variety of individual factors should be considered for clinical decision-making and management of patients with concomitant HCM and AF.

Takotsubo Syndrome After Alcohol Withdrawal in a Patient With Suspected Alcoholic Cardiomyopathy.

Takotsubo syndrome is a non-ischemic cardiomyopathy characterized by transient left ventricular (LV) apical ballooning, which typically occurs after exposure to emotional or physical stress in elderly women. An 85-year-old woman with hypertension presented with a recent onset of palpitation and exertional dyspnea. The patient had a long-standing history of alcohol consumption, and transthoracic echocardiography revealed diffuse LV hypokinesia including apical area with an ejection fraction of 30%. The patient was suspected of alcoholic cardiomyopathy and was recommended to quit alcohol consumption. Six weeks after the first admission, the patient presented to the emergency department with a three-day history of dyspnea. Based on newly developed negative T-waves and LV apical akinesia in the absence of significant coronary artery disease, the patient was diagnosed with takotsubo syndrome combined with suspected alcoholic cardiomyopathy. Clinicians should be aware that takotsubo syndrome can occur even in the presence of reduced LV ejection fraction, leading to further LV systolic dysfunction.

Assessing a Myocardial Area at Risk in Non-ST Elevation Acute Myocardial Infarction Without Wall Motion Abnormalities Using Cardiac Magnetic Resonance and Radionuclide Imaging.

Evaluation of a myocardial area at risk is clinically important because it contributes to clinical decision-making and management of patients with acute myocardial infarction (AMI). Herein, we reported a case of non-ST-elevation AMI (non-STEMI) without wall motion abnormalities on echocardiography, in which the myocardial area at risk was evaluated by two modalities; cardiac magnetic resonance (CMR) and radionuclide imaging. Coronary angiography revealed significant luminal stenosis in the diagonal branch and the obtuse marginal branch. It remained unclear which branch was the culprit. T2-weighted CMR revealed myocardial edema in the left ventricular anterolateral area. Based on the extent of myocardial edema, the patient was diagnosed with non-STEMI in the area corresponding to the diagonal branch. The area exhibiting impaired fatty acid metabolism on iodine-123-beta-methyl-p-iodophenyl penta-decanoic acid (123I-BMIPP) imaging matched well with the area showing myocardial edema on T2-weighted CMR. In conclusion, both CMR and BMIPP imaging are powerful tools in identifying a myocardial area at risk even in non-STEMI without wall motion abnormalities. This should contribute to clinical decision-making and management of patients with AMI.

Heart Failure in a Patient With Preexisting Giant Hiatal Hernia.

Hiatal hernia is one of common conditions in the elderly. Most patients with hiatal hernia are asymptomatic. However, some reports have described cardiac complications such as heart failure or arrhythmias due to cardiac compression. We report a diagnostically challenging case of heart failure accompanied by preexisting giant hiatal hernia, atrio-ventricular block (AVB)-related bradycardia and aortic valve stenosis (AS). Initial transthoracic echocardiogram (TTE) at the time of 2:1 AVB revealed moderate AS with no cardiac compression by a giant hiatal hernia. Repeated TTE after the correction of heart rate with pacemaker showed decreased peak velocity and mean pressure gradient. The true severity of AS was considered mild under the hemodynamically stable condition. Eventually, AVB-related bradycardia was identified to be responsible for heart failure rather than AS or giant hiatal hernia. The number of diagnostic occasions of heart failure is being increasing especially in the elderly. This case highlights the importance of careful evaluation to determine the major cause of heart failure accompanied by multiple comorbidities.

A Super-elderly Case of Suspected New-onset Vasospastic Angina Complicated by Myocardial Bridge.

A 90-year-old man experienced chest oppressive sensation at 12:30 AM for the first time. Electrocardiography showed a newly developed inversion of the terminal T-wave in the V2-5 leads. A left coronary angiogram showed no significant atherosclerotic stenosis. A myocardial bridge was found in the mid-left anterior descending artery (LAD). Myocardial scintigraphy with 123I beta-methyl 15-para-iodophenyl 3(R,S)-methylpentadecanoic acid revealed a reduced uptake in the apical and septal areas. Based on these findings, we suspected new-onset vasospastic angina complicated by a myocardial bridge in the territory of the LAD. He remained in good condition without recurrent anginal attacks after nifedipine was started before bedtime.

Female "Paradox" in Atrial Fibrillation-Role of Left Truncation Due to Competing Risks.

Female sex in patients with atrial fibrillation (AF) is a controversial and paradoxical risk factor for stroke-controversial because it increases the risk of stroke only among older women of some ethnicities and paradoxical because it appears to contradict male predominance in cardiovascular diseases. However, the underlying mechanism remains unclear. We conducted simulations to examine the hypothesis that this sex difference is generated non-causally through left truncation due to competing risks (CR) such as coronary artery diseases, which occur more frequently among men than among women and share common unobserved causes with stroke. We modeled the hazards of stroke and CR with correlated heterogeneous risk. We assumed that some people died of CR before AF diagnosis and calculated the hazard ratio of female sex in the left-truncated AF population. In this situation, female sex became a risk factor for stroke in the absence of causal roles. The hazard ratio was attenuated in young populations without left truncation and in populations with low CR and high stroke incidence, which is consistent with real-world observations. This study demonstrated that spurious risk factors can be identified through left truncation due to correlated CR. Female sex in patients with AF may be a paradoxical risk factor for stroke.

Association of Serum Levels of Cholesterol Absorption and Synthesis Markers with the Presence of Cardiovascular Disease: The CACHE Study CVD Analysis.

AIM: Serum levels of cholesterol absorption and synthesis markers have been associated with cardiovascular risk in the United States and European countries. In this study, we examined the relevance of these biomarkers and the presence of cardiovascular disease (CVD) in Japanese individuals. METHODS: The CACHE consortium, comprising of 13 research groups in Japan possessing data on campesterol, an absorption marker, and lathosterol, a synthesis marker measured by gas chromatography, compiled the clinical data using the REDCap system. RESULTS: Among the 2,944 individuals in the CACHE population, those with missing campesterol or lathosterol data were excluded. This cross-sectional study was able to analyze data from 2,895 individuals, including 339 coronary artery disease (CAD) patients, 108 cerebrovascular disease (CeVD) patients, and 88 peripheral artery disease (PAD) patients. The median age was 57 years, 43% were female, and the median low-density lipoprotein cholesterol and triglyceride levels were 118 mg/dL and 98 mg/dL, respectively. We assessed the associations of campesterol, lathosterol, and the ratio of campesterol to lathosterol (Campe/Latho ratio) with the odds of CVD using multivariable-adjusted nonlinear regression models. The prevalence of CVD, especially CAD, showed positive, inverse, and positive associations with campesterol, lathosterol, and the Campe/Latho ratio, respectively. These associations remained significant even after excluding individuals using statins and/or ezetimibe. The associations of the cholesterol biomarkers with PAD were determined weaker than those with CAD. Contrarily, no significant association was noted between cholesterol metabolism biomarkers and CeVD. CONCLUSION: This study showed that both high cholesterol absorption and low cholesterol synthesis biomarker levels were associated with high odds of CVD, especially CAD.

Serum Values of Cholesterol Absorption and Synthesis Biomarkers in Japanese Healthy Subjects: The CACHE Study HEALTHY Analysis.

AIM: Blood cholesterol absorption and synthesis biomarkers predict cardiovascular risk. This study aimed to determine the values of serum non-cholesterol sterol markers [lathosterol (Latho), campesterol (Campe), and sitosterol (Sito)] in healthy individuals and factors affecting these markers. METHODS: The CACHE Consortium compiled clinical data, including serum Latho (cholesterol synthesis marker), and Campe and Sito (cholesterol absorption markers), by a gas chromatography method in 2944 individuals. Healthy subjects were selected by excluding those with prior cardiovascular disease, diabetes mellitus, hypertension, chronic kidney disease, familial hypercholesterolemia, sitosterolemia, current smokers, those with low (＜17 kg/m2) or high (≥ 30 kg/m2) body mass index (BMI), and those with treatment for dyslipidemia or hyperuricemia. Nonlinear regression stratified by sex was used to examine the associations of cholesterol metabolism markers with age, BMI, and serum lipid levels. RESULTS: Of 479 individuals selected, 59.4% were female; the median age was 48 years in females and 50 years in males. The three markers showed positively skewed distributions, and sex differences were present. Age was associated positively with Latho, inversely with Campe, but not significantly with Sito. BMI was associated positively with Latho, but not significantly with Campe or Sito. High-density lipoprotein cholesterol (HDL-C) was positively associated with Campe and Sito, but not significantly with Latho. Non-HDL-C was positively associated with the three markers. CONCLUSION: Our study results in the healthy subjects help to interpret the non-cholesterol sterol markers for cardiovascular risk assessment in patients with cardiovascular risk factors.

Association between Familial Hypercholesterolemia and Serum Levels of Cholesterol Synthesis and Absorption Markers: The CACHE Study FH Analysis.

AIM: Serum levels of cholesterol absorption and synthesis markers are known to be associated with cardiovascular risk. Familial hypercholesterolemia (FH) is a well-known inherited disorder presenting elevated low-density lipoprotein cholesterol (LDL-C) and total cholesterol (TC) levels and premature coronary disease. In this study, we aim to examine the differences in terms of serum markers of cholesterol metabolism between FH and non-FH individuals and to examine their associations with serum lipid levels. METHODS: In this study, we utilized data on serum markers of cholesterol metabolism, namely, lathosterol (Latho, synthesis marker), campesterol (Campe, absorption marker), and sitosterol (Sito, absorption marker) measured by gas chromatography of the CACHE consortium, which comprised of 13 research groups in Japan. Clinical data were compiled using REDCap system. Among the 2944 individuals in the CACHE population, we selected individuals without lipid-lowering medications and hemodialysis patients for this CACHE study FH analysis. Multivariable adjustment was performed to assess the associations. RESULTS: In this study, we analyzed data from 51 FH patients and 1924 non-FH individuals. After adjustment for possible confounders, the FH group was shown to have significantly higher Campe and Sito concentrations and insignificantly higher Latho concentrations than the non-FH group. These marker concentrations showed nonlinear associations with TC in the FH group. Campe/Latho and Sito/Latho ratios were significantly higher in the FH group than in the non-FH group. CONCLUSION: FH group had significantly elevated serum Campe and Sito concentrations and insignificantly elevated Latho concentrations; thus, intestinal cholesterol absorption relative to hepatic cholesterol synthesis was suggested to be elevated in patients with FH. Serum Latho, Campe, and Sito concentrations showed nonlinear associations with TC in the FH group.

Relationship between Diabetes Mellitus and Serum Lathosterol and Campesterol Levels: The CACHE Study DM Analysis.

AIM: Risk of cardiovascular disease is increased in patients with diabetes mellitus (DM). Cholesterol metabolism (hepatic synthesis and intestinal absorption) is known to be associated with cardiovascular risk. Next, we examined the association of DM with cholesterol absorption/synthesis. METHODS: The CACHE Consortium, which is comprised of 13 research groups in Japan possessing data of lathosterol (Latho, synthesis marker) and campesterol (Campe, absorption marker) measured by gas chromatography, compiled the clinical data using the REDCap system. Among the 3597 records, data from 2944 individuals were used for several analyses including this study. RESULTS: This study analyzed data from eligible 2182 individuals including 830 patients with DM; 42.2% were female, median age was 59 years, and median HbA1c of patients with DM was 7.0%. There was no difference in Latho between DM and non-DM individuals. Campe and Campe/Latho ratio were significantly lower in DM individuals than in non-DM individuals. When the associations of glycemic control markers with these markers were analyzed with multivariable-adjusted regression model using restricted cubic splines, Campe and Campe/Latho ratio showed inverse associations with glucose levels and HbA1c. However, Latho showed an inverted U-shaped association with plasma glucose, whereas Latho showed a U-shaped association with HbA1c. These associations remained even after excluding statin and/or ezetimibe users. CONCLUSION: We demonstrated that DM and hyperglycemia were independent factors for lower cholesterol absorption marker levels regardless of statin/ezetimibe use.

A retrospective investigation to establish new screening approach for the detection of patients at high risk of Fabry disease in male left ventricular hypertrophy patients.

BACKGROUND: The prevalence of Fabry disease (FD) in male patients with left ventricular hypertrophy (LVH) is about 1%. From the perspective of performing more efficient screening with measurement of α-galactosidase (α-Gal) activity, it is important to raise the pretest probability. METHODS: We retrospectively investigated the prevalence of FD in 701 male patients with LVH who already had been screened by measurement of α-Gal activity in eight hospitals. From the viewpoint of enzymatic screening, we validated previously reported clinical features of FD including the electrocardiographic and echocardiographic characteristics with comparing each clinical determinant between patients with FD and non-FD patients. We finally aimed to establish a new screening approach for the detection of patients at high risk of FD. RESULTS: There were five FD patients (0.7%) in the 701 male patients with LVH. Those five patients with FD all had the cardiac variant type and age at detection of LVH was ≥35 years in all patients. In LVH patients with LV ejection fraction (EF) ≥ 50%, Pend-Q interval < 40 msec, SV1 + RV5 > 4.0 mV, and diffuse LVH were important determinants of FD. In LVH patients with LVEF < 50%, asymmetric septal hypertrophy and posterior wall motion abnormality seemed to be associated with FD. CONCLUSIONS: In our retrospective study, the prevalence of FD in male patients with LVH was found to be 0.7%. We established the efficient combinations of clinical determinants using age at detection of LVH, Pend-Q interval, high voltage, and LVH pattern in an echocardiogram.

Association of Kidney Function with Serum Levels of Cholesterol Absorption and Synthesis Markers: The CACHE Study CKD Analysis.

AIM: Serum levels of cholesterol absorption and synthesis markers are known to be associated with cardiovascular risk. Individuals with reduced kidney function or chronic kidney disease (CKD) are at an increased risk for cardiovascular disease. Hence, we examined the relationship between estimated glomerular filtration rate (eGFR) and serum markers of cholesterol absorption and synthesis. METHODS: The CACHE (Cholesterol Absorption and Cholesterol synthesis in High-risk patiEnts) Consortium, comprised of 13 research groups in Japan possessing data of lathosterol (Latho, synthesis marker) and campesterol (Campe, absorption marker) measured via gas chromatography, compiled the clinical data using the REDCap system. Among the 3597 records, data from 2944 individuals were utilized for five analyses including this CKD analysis. RESULTS: This study analyzed data from 2200 individuals including 522 hemodialysis patients; 42.3% were female, the median age was 58 years, and the median eGFR was 68.9 mL/min/1.73 m2. Latho, Campe, and Campe/Latho ratio were significantly different when compared across CKD stages. When the associations of eGFR with these markers were assessed with multivariable nonlinear regression models, Latho, Campe, and Campe/Latho ratio showed positive, inverse, and inverse associations with eGFR. These associations were significantly modified by sex, the presence/absence of diabetes mellitus, and the presence/absence of statin use. CONCLUSION: We showed that individuals with lower eGFR have lower cholesterol synthesis marker levels and higher cholesterol absorption marker levels in this large sample.

Factors Influencing Cardiac Sympathetic Nervous Function in Patients With Severe Aortic Stenosis: Assessment by 123I-Metaiodobenzylguanidine Myocardial Scintigraphy.

BACKGROUND: Numerous studies have shown that 123I-metaiodobenzylguanidine (MIBG) scintigraphy, an index of cardiac sympathetic nervous (CSN) activity, is useful for predicting prognosis in patients with heart failure. However, the factors influencing the CSN activity of patients with severe aortic stenosis (AS) remain unclear. METHODS: We enrolled 91 patients with severe AS who underwent 123I-MIBG scintigraphy, coronary computed tomography (CCT), and transthoracic echocardiography. When CCT angiography (CCTA) showed an obstructive epicardial artery, invasive coronary angiography was performed within 1 week of CCTA. RESULTS: There were 21 male and 70 female patients with a mean age of 84±5 years. Eighty-five (85) patients (93%) had hypertension and 13 patients (14%) had diabetes. Two (2) patients (2%) had previous myocardial infarction and eight (9%) had a previous coronary intervention. All patients had severe AS: aortic valve area was 0.63±0.18 cm2 and the mean pressure gradient was 56±19 mmHg. Regarding 123I-MIBG parameters, early heart-to-mediastinum (H/M) ratio was 3.1±0.5, delayed H/M ratio was 2.8±0.6, and the washout rate (WR) was 35%±13%. Multivariable linear regression analysis showed that coronary artery disease (ß=-0.30, p=0.002) was an independent predictor of delayed H/M ratio, and that aortic valve area (ß=-0.20, p=0.048) was an independent predictor of WR. CONCLUSIONS: Our findings suggest that coronary artery disease is an independent predictor of delayed H/M ratio, and aortic valve area is an independent predictor of WR in patients with severe AS.

The Monocyte to High-Density Lipoprotein Cholesterol Ratio Is Associated with Left Ventricular Diastolic Function in Patients with No Significant Perfusion Abnormality.

The monocyte to high-density lipoprotein cholesterol (HDL-C) ratio has been considered to be a prognostic marker. Whether this ratio is associated with left ventricular (LV) diastolic function remains undetermined. We tested the hypothesis that the monocyte to HDL-C ratio is associated with LV diastolic parameters derived from gated myocardial perfusion single-photon emission computed tomography (SPECT) in patients with no significant perfusion abnormality.The study population included 196 patients with no significant perfusion abnormalities and preserved ejection fraction. The peak filling rate (PFR) and one-third mean filling rate (1/3 MFR) were obtained as LV diastolic parameters using gated SPECT. Monocyte counts and plasma HDL-C levels were also examined.Significant associations were observed between the monocyte to HDL-C ratio and PFR (r = -0.20; P = 0.005) and 1/3 MFR (r = -0.19; P = 0.009). Multivariate linear regression analysis was performed to determine factors associated with LV diastolic parameters. Age (ß = -0.27; P < 0.001), LV end-diastolic volume (ß = -0.19; P = 0.034), and monocyte to HDL-C ratio (ß = -0.15; P = 0.027) were determined to be significantly associated with PFR. Moreover, age (ß = -0.13; P = 0.007), LV mass index (ß = -0.18; P = 0.037), and the monocyte to HDL-C ratio (ß = -0.13; P = 0.045) were significantly associated with 1/3 MFR.These results demonstrated that the monocyte to HDL-C ratio is associated with LV diastolic function, as derived from gated SPECT in patients with no significant perfusion abnormality.