Hye Bin Gwag

Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience

Background/Aims The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis. Methods We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy. Results Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447). Conclusions The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.

Immunoglobulin G4 Non-Related Sclerosing Disease with Intracardiac Mass Mimicking Mitral Stenosis: Case Report

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.

ASPIRIN VERSUS CLOPIDOGREL FOLLOWING DUAL ANTIPLATELET THERAPY ON THE ERA OF DRUG-ELUTING STENTS

Dual antiplatelet therapy (DAPT) for 12 months is recommended in patients with drug-eluting stent (DES) implantation. However, there are few studies compared between aspirin and clopidogrel as a monotherapy after DAPT. We compared the efficacy and safety of clopidogrel versus aspirin following 12-

A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma

Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.

Uric Acid Level Has a U-shaped Association with Clinical Outcomes in Patients with Vasospastic Angina

No data are available on the association of serum uric acid and vasospastic angina (VSA) which has endothelial dysfunction as a possible pathophysiologic mechanism. Low uric acid level might cause adverse outcomes in VSA in connection with endothelial dysfunction. We enrolled 818 VSA patients whose uric acid level was measured at admission. Patients were categorized according to tertiles of uric acid level: group I, ≤ 4.8 mg/dL; group II, 4.9–5.9 mg/dL; and group III, ≥ 6.0 mg/dL. Primary outcome was major adverse cardiac events (MACEs), defined as a composite of cardiac death, acute myocardial infarction (MI), ischemic stroke, coronary revascularization, and rehospitalization for angina. Median follow-up duration was 49.2 months. Median uric acid values were 4.1 mg/dL for group I, 5.4 mg/dL for group II, and 6.7 mg/dL for group III. In the overall population, group II had a significantly lower incidence of MACE compared to group I (47 [17.1%] vs. 66 [24.6%]; hazard ratio [HR], 1.52; 95% confidence interval [CI], 1.02–2.26; P = 0.040) and a tendency of lower incidence of MACEs compared to Group III (47 [17.1%] vs. 62 [22.5%]; HR, 1.44; 95% CI, 0.98–2.13; P = 0.067). Among group I patients, those who received nitrates had a higher incidence of MACEs than those without nitrate therapy (P < 0.001). Low uric acid level was associated with adverse clinical outcomes, while high uric acid level had a trend toward an increase in it. Use of nitrate in patients with low uric acid level might have adverse effects on clinical outcomes of VSA.

Gender differences in long-term clinical outcomes and prognostic factors in patients with vasospastic angina

BACKGROUND Men are more likely to suffer from vasospastic angina (VSA) than women; however, gender differences in the long-term prognosis of VSA patients have not been fully elucidated. We sought to investigate clinical outcomes and predictive factors of VSA patients according to gender. METHODS A total of 986 patients (838 men and 148 women) with a positive result on intracoronary ergonovine provocation test between January 2003 and December 2014 were analyzed. The primary outcome was major adverse cardiac events (MACE), defined as a composite of cardiac death, acute myocardial infarction, revascularization, or rehospitalization due to recurrent angina. RESULTS Women were younger and showed a lower prevalence of smoking or fixed coronary stenosis than men. The risk for MACE was similar between male and female patients (hazard ratio [HR], 0.95; 95% confidence interval [CI], 0.65-1.39; p=0.79). In multivariable prediction models for MACE, high-sensitivity C-reactive protein (hs-CRP) level was a significant predictor of MACE in male patients (HR, 1.95; 95% CI, 1.25-3.06; p=0.003), but there was no significant predictor in female patients. There was a significant interaction between hs-CRP level and MACE rate across genders (interaction p=0.02). CONCLUSIONS Long-term clinical outcome was not different between genders. Hs-CRP was an important predictor of long-term clinical outcomes in male patients with VSA, but not in female patients.

Clinical implications of low-dose aspirin on vasospastic angina patients without significant coronary artery stenosis; a propensity score-matched analysis

BACKGROUND High-dose aspirin has been reported to exacerbate coronary artery spasm in patients with vasospastic angina. We investigated clinical implications of low-dose aspirin on vasospastic angina patients without significant coronary artery stenosis. METHODS We included patients without significant coronary artery stenosis on coronary angiography (CAG) and with positive results on intracoronary ergonovine provocation test between January 2003 and December 2014. A total of 777 patients were divided into two groups according to prescription of low-dose aspirin at discharge: aspirin group (n=321) and non-aspirin group (n=456). The major adverse cardiovascular events (MACE), defined as composite outcomes of cardiac death, acute myocardial infarction, revascularization, or rehospitalization requiring CAG or medication change due to recurrent angina were compared. RESULTS The aspirin group had significantly higher incidence of MACE (22.8% versus 12.1%; p=0.04) and had higher tendency for rehospitalization (20.6% versus 11.2%; p=0.08). All-cause mortality and cardiac death were similar between the two groups. After propensity score matching, the aspirin group had greater risk of MACE (hazard ratio [HR] 1.54; 95% confidence interval [CI], 1.04-2.28; p=0.037) and rehospitalization requiring CAG (HR, 1.33; 95% CI, 1.13-4.20; p=0.03), and a higher tendency for rehospitalization (HR, 1.40; 95% CI, 0.94-2.09; p=0.12). CONCLUSION In vasospastic angina without significant coronary artery stenosis, patients taking low-dose aspirin are at higher risk of MACE, driven primarily by tendency toward rehospitalization. Low-dose aspirin might be used with caution in vasospastic angina patients without significant coronary artery stenosis.

Morphine Does Not Affect Myocardial Salvage in ST-Segment Elevation Myocardial Infarction.

Recent studies have proposed intravenous (IV) morphine is associated with delayed action of antiplatelet agents in acute myocardial infarction. However, it is unknown whether morphine results in increased myocardial damage in ST-segment elevation myocardial infarction (STEMI) patients undergoing primary percutaneous coronary intervention (PCI). We investigated myocardial salvage index (MSI) to determine whether IV morphine affects myocardial injury adversely in STEMI patients undergoing primary PCI. 299 STEMI patients underwent contrast-enhanced magnetic resonance imaging a median of 3 days after PCI. Infarct size was measured on delayed-enhancement imaging, and area at risk was quantified on T2-weighted imaging. MSI was calculated as ‘[area at risk–infarct size] X 100 / area at risk’. IV morphine was administrated in 32.1% of patients. Patients treated with morphine had shorter symptom to balloon time and higher prevalence of Thrombolysis in Myocardial Infarction flow grade 0 or 1. The morphine group showed a trend toward larger MSI and infarct size and significantly greater area at risk than the non-morphine group. After propensity score matching (90 pairs), MSI was similar between the morphine and non-morphine group (46.1% versus 43.5%, P = .11), and infarct size and area at risk showed no difference. In propensity score-matched analysis, IV morphine prior to primary PCI in STEMI patients did not cause adverse impacts on myocardial salvage.

Staged hybrid procedure versus radiofrequency catheter ablation in the treatment of atrial fibrillation

The treatment effect of the hybrid procedure, consisting of a thoracoscopic ablation followed by an endocardial radiofrequency catheter ablation (RFCA), is unclear. A total of 117 ablation-naïve patients who underwent either the staged hybrid procedure (n = 72) or RFCA alone (n = 105) for drug-refractory, non-valvular persistent or long-standing persistent atrial fibrillation (AF) were enrolled. The primary outcome is occurrence of total atrial arrhythmia, defined as a composite of AF, sustained atrial tachycardia (AT), and atypical atrial flutter (AFL) after index procedure. The mean age was 52.7 years. Eighty-four percentage of the patients were male. Patients with prior history of stroke and long-standing persistent AF were more prevalent in the hybrid group than RFCA group. The left atrial volume index was larger in the hybrid group (P<0.001). During 2.1 years of median follow-up, the incidence of total atrial arrhythmia was not different between the two groups (32.5% vs. 35.7%; adjusted hazard ratio: 0.64; 95% confidence interval: 0.36–1.14; P = 0.13). The AF recurrence was significantly lower in the hybrid group than in the RFCA group (29.6% vs. 34.9%; adjusted HR: 0.53; 95% CI: 0.29–0.99; P = 0.046). The hospital stay was longer in the hybrid group than in the RFCA group (11 days vs. 4 days; P<0.001). A staged hybrid procedure may be an alternative choice for drug-refractory persistent AF, but it is no more effective than RFCA alone to eliminate atrial arrhythmias. Considering the long-length of stay and the morbidity, careful consideration should be given in selection of treatment strategy.

Which antiarrhythmic drug to choose after electrical cardioversion: A study on non-valvular atrial fibrillation patients

The relative efficacy of antiarrhythmic drugs (AADs) after electrical cardioversion are not well established. This study aimed to investigate the efficacies of different AADs for maintaining sinus rhythm (SR) after electrical cardioversion for atrial fibrillation (AF). We selected patients from a retrospective registry including patients admitted for cardioversion between January 2012 and June 2016. The primary outcome was time to AF recurrence during the first year after cardioversion. The secondary outcomes included AF recurrence within 1 month, and first readmission due to heart failure, stroke, or additional non-pharmacological rhythm control. A total of 265 patients were divided into the 4 groups according to AAD type: flecainide (n = 33), propafenone (n = 64), amiodarone (n = 128), and dronedarone (n = 40). During the first year after cardioversion, the AF recurrence-free survival was similar between all AAD groups (69.7% vs. 67.2% vs. 71.9% vs. 80.0%, p = 0.439). About half of all recurrences occurred during the first month. There was no difference in any of the secondary outcomes, although the amiodarone group showed a trend toward more non-pharmacological rhythm control. AAD type was not associated with recurrence in multivariate analysis. In this study, half of all patients received amiodarone after electrical cardioversion. Flecainide, propafenone, amiodarone, and dronedarone showed similar efficacies for maintaining SR after electrical cardioversion. Thus, it might be reasonable to reconsider amiodarone use after cardioversion, since it did not show superior efficacy to the other drugs considered and is associated with potential side effects.