Cardiovascular beriberi: rare cause of reversible pulmonary hypertension / 영남의대학술지

Cardiovascular beriberi is caused by thiamine deficiency and usually presents as high cardiac output failure associated with predominantly right-sided heart failure and rapid recovery after treatment with thiamine. Because of its rarity in developed countries, the diagnosis can often be delayed and missed. We recently experienced a case of cardiovascular beriberi with pulmonary hypertension which successfully treated with thiamine infusion. A 50-year-old man with chronic heavy alcoholics was refered to our department for dyspnea with mental change. Echocardiography showed marked right ventricular (RV) dilatation and flattening of the interventricular septum with a D-shaped deformation of the left ventricle. Moderate tricuspid valve regurgitation was found and estimated RV systolic pressure was 52 mm Hg. Because of his confused mentality and history of chronic alcohol intake, neurological disorder due to thiamine deficiency was suspected and intravenous thiamine was administered and he continuously received a daily dose of 100 mg of thiamine. Follow up echocardiography showed marked reduction of RV dilatation and improvement of a D-shaped deformation of the left ventricle. He finally diagnosed as cardiovascular beriberi on the basis of dramatic response to intravenous thiamine. Thiamine deficiency can cause reversible pulmonary hypertension, and can still be encountered in the clinical setting. Thus high index of suspicion is critically needed for diagnosis.

A Case of Thymic Carcinoma Misdiagnosed as Epipericardial Fat Necrosis / 대한내과학회지

Epipericardial fat necrosis (EPFN) is an uncommon benign condition of unknown etiology. It presents as an unexplained acute severe pleuritic chest pain that is associated with the presence of a well-defined juxtacardiac mass usually located in or near the cardiophrenic angle. Although its typical clinical manifestations and chest computed tomography findings might lead to successful diagnosis of this rare disease, an unusual mass location such as the anterosuperior mediastinum should be considered and biopsies should be performed. We herein report a case of thymic carcinoma that was suspected initially to be EPFN.

A Case of Life-Threatening Supraventricular Tachycardia Related to Flecainide Toxicity / 대한내과학회지

Flecainide acetate is a potent class Ic anti-arrhythmic drug with a major sodium channel-blocking effect. Flecainide toxicity can cause myocardial impairment and precipitate circulatory collapse, particularly in patients with renal failure. Electrical and hemodynamic deterioration during flecainide toxicity may not respond to conventional treatments. We report the successful management of flecainide toxicity using extracorporeal membrane oxygenation (ECMO), hemoperfusion, and bicarbonate administration maintaining alkalinity.

Erratum: Etiologies and Predictors of False-Positive Diagnosis of ST-Segment Elevation Myocardial Infarction

On page 370, Article Title has been incorrectly marked Etiologies and Predictors of ST-Segment Elevation Myocardial Infarction. The correct title is Etiologies and Predictors of False-Positive Diagnosis of ST-Segment Elevation Myocardial Infarction.

Complication Rate of Transfemoral Endomyocardial Biopsy with Fluoroscopic and Two-dimensional Echocardiographic Guidance: A 10-Year Experience of 228 Consecutive Procedures

Endomyocardial biopsy (EMB) is one of the reliable methods for the diagnosis of various cardiac diseases. However, EMB can cause various complications. The purpose of this study is to evaluate the complication of transfemoral EMB with both fluoroscopic and two-dimensional (2-D) echocardiographic guidance. A total of 228 patients (148 men; 46.0+/-14.6 yr-old) who underwent EMB at Kyungpook National University Hospital from January 2002 to June 2012 were included. EMB was performed via the right femoral approach with the guidance of both echocardiography and fluoroscopy. Overall, EMB-related complications occurred in 21 patients (9.2%) including one case (0.4%) with cardiac tamponade requiring emergent pericardiocentesis, four cases (1.8%) with small pericardial effusion without pericardiocentesis, two cases (0.9%) with hemodynamically unstable ventricular tachycardia (VT), one case (0.4%) with nonsustained VT, one case (0.4%) with tricuspid regurgitation, twelve cases (5.3%) with right bundle branch block. There was no occurrence of either EMB-related death or cardiac surgery. Left ventricular ejection fraction was significantly lower (32.0+/-18.7% vs 42.0+/-19.1%, P=0.023) and left ventricular end-diastolic dimension was larger (60.0+/-10.0 mm vs 54.2+/-10.2 mm, P=0.013) in patients with EMB related complications than in those without. It is concluded that transfemoral EMB with fluoroscopic and 2-D echocardiographic guidance is a safe procedure with low complication rate.

Fabry Cardiomyopathy

Fabry disease is a progressive X-linked disorder of glycosphingolipid metabolism caused by a deficiency of the alpha-galactosidase lysosomal enzyme. The partial or complete deficiency of the lysosomal enzyme leads to an accumulation of neutral glycosphingolipids in the vascular endothelium and visceral tissues throughout the body. In the heart, glycosphingolipids deposition causes progressive left ventricular hypertrophy (LVH). We report a case of Fabry disease which was suspected based upon two-dimensional echocardiographic finding of LVH. A 44-year-old man was admitted to evaluation of aggravated exertional dyspnea of two weeks duration. He had been diagnosed with end-stage renal disease of unknown etiology at age 41 followed by renal transplantation that year. He had been treated with oral immunosuppressive agents. On hospital day two, transthoracic echocardiography revealed concentric LVH. Left ventricular systolic function was preserved but diastolic dysfunction was present. Fabry disease was confirmed by demonstration of a low plasma alpha-galactosidase A (alpha-Gal A) activity. Analysis of genomic DNA showed alpha-Gal A gene mutation. The patient was diagnosed with Fabry disease.

Etiologies and Predictors of ST-Segment Elevation Myocardial Infarction

BACKGROUND AND OBJECTIVES: Rapid diagnosis of ST-segment elevation myocardial infarction (STEMI) is essential for the appropriate management of patients. We investigated the prevalence, etiologies and predictors of false-positive diagnosis of STEMI and subsequent inappropriate catheterization laboratory activation in patients with presumptive diagnosis of STEMI. SUBJECTS AND METHODS: Four hundred fifty-five consecutive patients (62+/-13 years, 345 males) with presumptive diagnosis of STEMI between August 2008 and November 2010 were included. RESULTS: A false-positive diagnosis of STEMI was made in 34 patients (7.5%) with no indication of coronary artery lesion. Common causes for the false-positive diagnosis were coronary spasm in 10 patients, left ventricular hypertrophy in 5 patients, myocarditis in 4 patients, early repolarization in 3 patients, and previous myocardial infarction and stress-induced cardiomyopathy in 2 patients each. In multivariate logistic regression analysis, symptom-to-door time >12 hours {odds ratio (OR) 4.995, 95% confidence interval (CI) 1.384-18.030, p=0.014}, presenting symptom other than chest pain (OR 7.709, 95% CI 1.255-39.922, p=0.027), absence of Q wave (OR 9.082, CI 2.631-31.351, p<0.001) and absence of reciprocal changes on electrocardiography (ECG) (OR 17.987, CI 5.295-61.106, p<0.001) were independent predictors of false-positive diagnosis of STEMI. CONCLUSION: In patients whom STEMI was planned for primary coronary intervention, the false-positive diagnosis of STEMI was not rare. Correct interpretation of ECGs and consideration of ST-segment elevation in conditions other than STEMI may reduce inappropriate catheterization laboratory activation.