A Case of Recurrent Glucocorticoid-Induced Pheochromocytoma Crisisb during the Treatment of Urticaria / 대한내과학회지

Pheochromocytoma crisis is a life-threatening endocrine emergency. Stimuli that can elicit a pheochromocytoma crisis include anesthesia, tumor manipulation, and several drugs. Rarely, glucocorticoids can induce a pheochromocytoma crisis. Here, we describe the case of a 65-year-old female who developed an adrenergic crisis with blood pressure fluctuations, dizziness, and seizures after receiving glucocorticoids for the treatment of urticaria. The symptoms led us to speculate that a pheochromocytoma was present. We confirmed the diagnosis based on abdominal imaging and biochemical studies. The patient's symptoms improved after surgical removal of the pheochromocytoma.

Atrial standstill treated with implantation of permanent pacemaker / 대한내과학회지

Persistent atrial standstill is an extremely rare arrhythmia that was first described by Chavez et al. Electrocardiographically, atrial standstill is characterized by bradycardia, the absence of a P wave, and a junctional narrow complex escape rhythm. Atrial standstill is usually classified into two types. The transient type is observed in drug intoxication, such as with digitalis or quinidine, and hyperkalemia. The persistent type is uncommon, often accompanied by syncopal attacks or brain embolism. We report a case of persistent atrial standstill in an 83-year-old man who was treated with implantation of a permanent pacemaker.

Assessing stent restenosis using 64-multidetector computed tomography coronary angiography / 대한내과학회지

BACKGROUND/AIMS: Multidetector computed tomography (MDCT) is considered to be a noninvasive, alternative method for evaluating stent restenosis. However, the diagnostic accuracy of 16-channel MDCT for stent stenosis is reported to have severe limitations because of high-attenuation stent-related artifacts. 64-channel MDCT, which recently became available in clinical practice, has better spatial and temporal resolution than 16-channel MDCT. The diagnostic accuracy of 64-channel MDCT for stent restenosis (in-segment and in-stent) was assessed by comparing it with conventional coronary angiography. METHODS: In-segment and in-stent restenosis (> or =50% in diameter) were evaluated in 96 stent segments in 68 patients [61+/-12 years, 51 (75%) male] using both 64-channel MDCT and conventional coronary angiography. The in-stent analysis was confined to the portion of the artery covered by the stent and the in-segment analysis included the stent and 5 mm proximal or distal to the stent edges. RESULTS: The 64-channel MDCT could evaluate stent restenosis in 93 of 96 (97%) stent segments. Quantitative conventional coronary angiography found in-segment restenosis (> or =50% in diameter) in 16 of 68 (23%) patients and 16 of 96 (17%) segments. For the patients with interpretable stent segments, the sensitivity, specificity, positive predictive value, and negative predictive value of 64-channel MDCT for in-segment restenosis per patient were 63, 96, 83, and 89%, respectively; per segment they were 63, 97, 83, and 93%, respectively; and for in-stent restenosis per stent they were 82, 98, 82, and 98%, respectively. CONCLUSIONS: The diagnostic accuracy of 64-channel MDCT for assessing stent restenosis had high specificity and negative predictive value in the clinical setting. The 64-channel MDCT may be a promising, less-invasive imaging tool for stent restenosis, especially for the purpose of excluding stent restenosis.

Spontaneous Perforation of Sinus of Valsalva without Associated Aortic Pathology

The spontaneous perforation of sinus of Valsalva without congenital anomaly such as aneurysm and trauma has not been reported. We report a 66-year-old female presenting with ST elevation myocardial infarction due to compressing the proximal part of right coronary artery by hematoma attributed to spontaneous perforation of sinus of Vaslava. The patient was stabilized after surgical primary repair.

A Case of Thymic Cyst in the Middle Mediastinum Mimicking Pericardial Cyst

A thymic cyst in the middle mediastinum adjacent to the right pericardium is extremely rare. We report a case of a large thymic cyst in the right cardiophrenic angle, compressing the right atrium, which was presented as a cardiomegaly on the chest radiograph and a pericardial cyst on the echocardiography. The definitive diagnosis was confirmed using surgical resection and biopsy.

Peroxisome Proliferator-Activated Receptor Gamma(PPAR-gamma) Agonist Improves Endothelial Function in Diabetic Patients with Metabolic Syndrome: Pivotal Role of NOx and Inflammation

BACKGROUND AND OBJECTIVES: Nitric oxide (NO) is thought to have antiatherosclerotic properties. On the other hand, NO activity is reduced in patients with metabolic syndrome, and endothelial dysfunction is an important early sign of atherosclerosis in patients with metabolic syndrome. The aim of this study was to investigate the effect of pioglitazone on the endothelial function in terms of the plasma NOx (combined nitrate/nitrite), the circulating inflammatory markers and the autonomic nervous system. SUBJECTS AND METHODS: We randomized 40 subjects with metabolic syndrome, and they were assigned to receive 15 mg of pioglitazone per day (the PIO group, n=21) during 12 weeks or they were placed in the placebo group (the PLA group, n=19). We estimate the endothelial function by performing vascular ultrasound. The plasma NOx levels, the levels of the inflammatory markers and the GRK2 levels were measured. RESULTS: After 12 weeks of therapy, flow mediated dilation (FMD) was improved in the PIO group (from 6.7+/-6% to 11.7+/-5%, respectively: p<0.05), but not in the PLA group. The level of plasma NOx was increased in the PIO group (from 67.7+/-30 nmol/dL to 92.9+/-41 nmol/dL, respectively: p<0.001), but not in the PLA group. The plasma levels of hsCRP and IL-6 dropped significantly (from 2.6+/-2.3 mg/L to 1.2+/-1.3 mg/L and 1.7+/-2.1 pg/mL to 0.7+/-0.5 pg/mL, respectively: p<0.05) in the PIO group, but not in the PLA group. The levels of GRK2 (the PLA group from 0.0061+/-0.0023 ng to 0.0075+/-0.0031 ng, and the PIO group from 0.0024+/-0.002 ng to 0.0015+/-0.001 ng, p=ns) didn't dropped significantly. CONCLUSION: Administration of PPAR-gamma agonist in patients suffering with metabolic syndrome improves their endothelial function, enhances the production of NOx and reduces the proinflammatory markers, but this is not related to sympathetic regulation. PPAR-gamma agonist may be able to modulate the progression of atherosclerosis.

Unruptured Aneurysm of the Left Sinus of Valsalva Presenting as Acute Coronary Syndrome: A Case Report

We report here on a case of a 34-year-old man with unruptured aneurysm of the left sinus of Valsalva, and he presented with acute coronary syndrome due to the putative dynamic compression of both the left main coronary artery and the left circumflex coronary artery. The cardiac multislice computed tomography scanning and coronary angiogram revealed the compression of the two coronary arteries by the aneurysm of the left sinus of Valsalva. Aneurysmectomy was performed for surgical repair. After the surgery, the patient stayed asymptomatic during the 6-months of follow-up.

Medical Treatment of Coronary Artery Disease / 한양의대학술지

Coronary heart disease is still highly prevalent worldwide and remains a common cause of mortality. The underlying cause responsible for stable angina is chronic atherosclerotic narrowing of the coronary artery. Most patients with stable angina can be managed with medical treatment with aspirin, beta-blocker, calcium channel blocker (CCB), and nitrate. High-risk patients with previous myocardial infarction (MI), left ventricular dysfunction, and diabetes mellitus should be considered for angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARB) and aggressive statin treatment. Acute coronary syndromes (ACS) are the clinical spectrum that includes unstable angina and non-ST elevation myocardial infarction (UA/NSTEMI) and ST-segment elevation myocardial infarction (STEMI). Because the atherosclerotic plaque instability with subsequent rupture and thrombus formation is a primary mechanism of ACS, antiplatelet and antithrombotic agents are essential for the prevention of coronary events. Combination treatment with antiplatelet agents (aspirin, clopidogrel and cilostazol) and anticoagulants, such as unfractionated heparin and low-molecular-weight heparins (LMWH), provides improved efficacy for the secondary prevention of ACS. The main goal of treatment in STEMI is quick recovery of the culprit vessel patency and maintaining sufficient myocardial perfusion. It can be done by thrombolytic therapy or primary coronary angioplasty.

Medical Treatment of Coronary Artery Disease / 한양의대학술지

Coronary heart disease is still highly prevalent worldwide and remains a common cause of mortality. The underlying cause responsible for stable angina is chronic atherosclerotic narrowing of the coronary artery. Most patients with stable angina can be managed with medical treatment with aspirin, beta-blocker, calcium channel blocker (CCB), and nitrate. High-risk patients with previous myocardial infarction (MI), left ventricular dysfunction, and diabetes mellitus should be considered for angiotensin-converting enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARB) and aggressive statin treatment. Acute coronary syndromes (ACS) are the clinical spectrum that includes unstable angina and non-ST elevation myocardial infarction (UA/NSTEMI) and ST-segment elevation myocardial infarction (STEMI). Because the atherosclerotic plaque instability with subsequent rupture and thrombus formation is a primary mechanism of ACS, antiplatelet and antithrombotic agents are essential for the prevention of coronary events. Combination treatment with antiplatelet agents (aspirin, clopidogrel and cilostazol) and anticoagulants, such as unfractionated heparin and low-molecular-weight heparins (LMWH), provides improved efficacy for the secondary prevention of ACS. The main goal of treatment in STEMI is quick recovery of the culprit vessel patency and maintaining sufficient myocardial perfusion. It can be done by thrombolytic therapy or primary coronary angioplasty.

The Usefulness of 2-Dimensional Longitudinal Strain for Prediction of the Postoperative Left Ventricular Systolic Function in Patients with Valvular Heart Disease Causing Volume Overloading

BACKGROUND AND OBJECTIVES: Prediction of the postoperative left ventricular (LV) function in valvular heart disease that will cause LV volume overloading, such as chronic mitral regurgitation (MR) and aortic regurgitation (AR), remains elusive. We sought to test if 2-dimensional peak negative longitudinal strain (LS2D) was useful for prediction of the postoperative LV function in relation to this disease entity. SUBJECTS AND METHODS: Newly developed speckle tracking imaging was performed preoperatively to measure the LS2D in 26 and 22 patients with MR and AR, respectively. A favorable response after the operation (FR) was defined according to the change in the LV ejection fraction (EF): 1) a LVEF > or =55% both pre- and post-operation, 2) a postoperative LVEF > or =55% with a pre-operative LVEF between or =45%, or 3) an increase in the LVEF >10% with a pre-operative LVEF <45%. RESULTS: Follow-up echocardiography was performed at an average of 6.7+/-2.3 months after the operation. FR was confirmed in 36 patients (LVEF from 55.4+/-10.1 to 58.3+/-5.0%, p=0.06), with the remaining 12 showing an unfavorable response (LVEF from 52.5+/-7.6 to 45.1+/-5.4%, p<0.01). There was no significant difference in the baseline characteristics, including underlying etiologies, operation techniques, and cardiopulmonary bypass time, and LV volumes and EF between the two groups. The only difference was the LS2D, which was significantly larger in the FR group (-19.8+/-3.9 versus -16.2+/-2.9%, p<0.01). An LS2D of -18.3% could predict an unfavorable response of the LVEF following an operation, with a sensitivity and specificity of 75 and 75%, respectively. CONCLUSION: The preoperative LS2D is a useful predictor of the postoperative left ventricular function in AR and MR.

Late Stent Thrombosis Associated with Late Stent Malapposition after Drug-Eluting Stenting: A Case Report

We report here on one patient who suffered from acute myocardial infarction that was due to late stent thrombosis, and this was associated with late stent malapposition very late (21 months) after the deployment of a paclitaxel-eluting stent and shortly after (7 days) the discontinuation of the aspirin therapy. The intravascular ultrasound examination revealed that the late stent thrombosis was accompanied by late stent malapposition. This is a report on late stent thrombosis associated with late stent malapposition after the successful implantation of a paclitaxel-eluting stent (PES).

A Case of Shoshin Beriberi Presenting as Acute Coronary Syndrome with Shock: Shoshin Beriberi Mimicking Acute Coronary Syndrome

Cardiac beriberi is caused by thiamine deficiency. Shoshin beriberi is a rare and fulminant form of cardiac beriberi characterized by hypotension, high output heart failure, lactic acidosis and anuria. Without early recognition and immediate treatment, most of these patients will be fatal. Therefore clinical diagnosis of shoshin beriberi is most important in emergency situation. We report a case of shoshin beriberi with clinical features mimicking acute coronary syndrome. Fifty year old male patient with chronic alcoholism was presented with shock, hypoxia, right heart failure and severe acidosis. Electrocardiogram showed abnormal Q in V1-3 and mild ST elevation and level of troponin I was slightly elevated. All manifestations including lactic acidosis were dramatically subsided in 18 hours by thiamine infusion. Even in developed country, shoshin beriberi can be occurred in patients with malnutrition and/or chronic alcoholism and should be differentiated with acute coronary syndrome.

A Case of Left Ventricular Pseudoaneurysm Extending to Lateral Side of Left Atrium after Myocardial Infarction

Left ventricular pseudoaneurysm is a rare but fatal complication of acute myocardial infarction. It occurs as a consequence of rupture of the ventricular free wall and is confined by a portion of pericardium. The pseudoaneurysm extended to lateral side of the left atrium is rare. We report a case of left ventricular pseudoaneurysm extended to lateral side of the left atrium in a 83-year-old man.

Clinical Characteristics of Patients with Cardiac Involvement in Behcet's Disease

BACKGROUND AND OBJECTIVES: Despite the sporadic case reports on the cardiac involvement of Behcet's disease, any systemic investigation has not been reported on. We sought to investigate the frequency and clinical characteristics of the patients with the cardiac manifestations of Behcet's disease. SUBJECTS AND METHODS: We retrospectively analyzed the clinical data of 217 patients (mean age: 39+/-11 years) with Behcet's disease who were diagnosed in Asan Medical Center from January 1990 to September 2000. The patients were subgrouped into the mucocutaneous, cardiac, vascular, central nervous system, gastrointestinal and ocular group according to the involved organ. RESULTS: Among 217 patients, nineteen (8.8%) were found to have cardiac involvement: aortic regurgitation was found in ten patients (53%), right ventricular thrombi in six (32%), acute myocardial infarction in two (11%), and myocarditis in one (5%). The incidence of initial oro-genital ulcers at the first clinical presentation was significantly lower in the cardiac Behcet's group than in the other subgroups (52% vs 90%, p=0.001). The event-free survival rate during a mean follow-up duration of 67+/-46 months was also significantly lower in the cardiac Behcet's group (63% vs 14%, p=0.0001). CONCLUSION: Aortic regurgitation and right ventricular thrombus are two common types of cardiac Behcet's disease. Although its incidence is low, cardiac Bechcet's disease has a characteristic pattern for the clinical presentation, and it has a worse prognosis compared with the other subgroups of Behcet's disease that have other solid organ involvement.

Primary Sirolimus-Eluting Stent Implantation for Patients with Acute ST-Segment Elevation Myocardial Infarction

BACKGROUND AND OBJECTIVES: Sirolimus-eluting stents (SES) have been shown to significantly inhibit neointimal hyperplasia, resulting in reduced restenosis compared with bare metal stents (BMS). However, the efficacy and safety of SES implantation for patients with acute ST-segment elevation myocardial infarction (STEMI) remain unclear. SUBJECTS AND METHODS: Primary stenting was performed using SES in 74 patients (mean age: 58.0+/-12.7 years, 59 males) and BMS in 88 patients (mean age: 59.3+/-10.7 years, 63 males) between April 2003 and July 2004. We retrospectively compared the incidence of 6-month angiographic restenosis and the major adverse cardiac events (MACE) defined as cardiac death, non-fatal myocardial infarction and target lesion revascularization (TLR), between the SES group and the BMS group. RESULTS: The SES group had smaller vessels (3.04+/-0.47 mm vs. 3.24+/-0.56 mm, respectively, p=0.02) and a longer stent length (33.7+/-14.3 mm vs. 25.0+/-9.6 mm, p=0.00). The procedural success rate (87.8% vs. 92.0%, respectively, p=0.37) and the peak creatine kinase-MB (239+/-196 ng/mL vs. 274+/-188 ng/mL, p=0.26) were similar. The 6-month angiographic restenosis rate (0.0% vs. 30.4%, respectively, p=0.00) and late loss (-0.03+/-0.55 mm vs. 1.28+/-0.58 mm, p=0.00) were significantly lower in the SES group compared with the BMS group. Stent thrombosis developed in only 1 case of the SES group (1.4% vs. 0.0%, respectively, p=0.45). At 6 months, SES implantation significantly reduced the incidence of MACE (6.9% vs. 19.5%, respectively, p=0.04), because of a reduction in the incidence of TLR (1.4% vs. 11.5%, p=0.01). Likewise, the MACE-free survival rate was significantly higher in the SES group (93.06% vs. 80.46%, respectively, p=0.03). CONCLUSION: Compared with the BMS, the SES was effective in reducing the incidence of 6-month angiographic restenosis and MACE without any increased risk of stent thrombosis in the patients with STEMI who received primary stenting.

Association between betaARK1 Level of Circulating Mononuclear Leukocytes and Left Ventrcular mass in Non-treated Hypertensive Patients

BACKGROUND: Beta-adrenergic receptor Kinase 1(betaARK1) is a serine/threonine kinase attached, which inhibits the coupling of beta-adrenergic receptor with G-protein. Myocardial betaARK1 level is usually elevated in heart failure and hypertrophy, but it is not known whether the circulating betaARK1 level is related with the degree of cardiac hypertrophy. This study was performed to evaluate the association of the betaARK1 level in circulating mononuclear leukocytes(MNL) in untreated hypertension with left ventricular mass in hypertensive patients. Method: Nineteen non-treated hypertensive patients were included for this study. High blood pressure was confirmed when systolic BP is over 150 mmHg or diastoli BP is over 95 mmHg. Echocardiography was performed to evaluate the degree of hypertrophy by measuring the left ventricular mass index(LVMI) and relative wall thickness(RWT), and test the LV function by measuring the ejection fraction(EF) according to ASE guideline. At the same time, blood was collected from each patient and MNL were isolated by gradient centrifuge with Ficoll-400. Total RNA was purified from MNL and semi-quantitative RT-PCR was performed. After reverse transcription, PCR was done with primers for human betaARK1 and GAPDH as external control. betaARK1 levels were expressed by ratio to GAPDH level and estimated the relations with clinical and Echocardiographic parameters. Result: We studied confirmed 19 hypertensive patients(10 men and 9 women, mean age of 50.6 years). Echocardiographically measured indices(mean+/-SD) were as follows; LVMI(137.3+/-30.6g/m2), PWT(0.53+/-0.09) and EF(54.6+/-8.5%). Ratio of betaARK1 levels to GAPDH was from 0.10 to 0.96 (0.62+/-0.25). betaARK1 levels were correlated with LVMI(correlation coefficient: r=.502, p=.029) and RWT(r=.627, p=.004). But Systolic BP(r=0.009, p=.93), diastolic BP(r=.07, p=.85) or EF(r=.045, p=.84) were not related to level of betaARK1. CONCLUSIONS: The betaARK1 level of circulating MNL was correlated well with the degree of the cardiac hypertrophy estimated by LVMI and RWT. This data suggests that activation of sympatho-adrenal system would exert a major role in developing cardiac hypertrophy and we can expect the decreased responsiveness to catecholamine in the heart of hypertensive patients. betaARK1 in circulating MNL might be used as a predictor or marker for LV hypertrophy in hypertensive patients.

A Case of Pure Unroofed Coronary Sinus without Persistent Left Superior Vena Cava

We report a case of 51-year-old woman with pure unroofed coronary sinus without persistent left superior vena cava and other cardiac anomaly. She presented with dyspnea on exertion during several years. Her chest film showed prominent cardiomegaly and dilated hilar vessels. Cardiac rhythm was atrial fibrillation. Transthoracic echocardiography demonstrated the enlarged coronary sinus with defect toward left atrium on parasternal long axis view and significant flow from coronary sinus into right atrium on subxyphoid view, and its other findings were dilated right ventricle and right atrium, paradoxical septal motion, moderate tricuspid regurgitation and mild mitral regurgitation, which were mimicking of large secundum atrial septal defect. Radionuclide cardioangiography and cardiac catheterization showed the existence of significant shunt. There was no evidence of persistent left superior vena cava on chest CT. Closure of Coronary sinus opening was done. Thereafter her symptoms of congestive heart failure were much improved. We think that careful examination of 2-D echocardiography can be valuable tool for diagnosis of unroofed coronary sinus in adult patient.

Hemiazygos Continuation of Left Inferior Vena Cava Draining into the Right Atrium via Persistent Left Superior Vena Cava: A Variant of Polysplenia Syndrome Mimicking Aortic Dissection

We report a case of a 58 year-old male with polysplenia and left inferior vena cava draining into the right atrium via hemiazygous vein; the left superior vena cava and the coronary sinus in order. He presented dyspnea on exertion and atrial fibrillation. Originally, through findings of mediastinal widening in chest X-ray and the double lumen of the descending aorta in transesophageal echocardiography, he was erroneously diagnosed with aortic dissection. The anomalous venous connection was discovered via spiral CT and venography. We also found three to five small spleens via CT. We emphasize that normal left superior vena cava mimic aortic dissection on chest X-rays and transesophageal echocardiographys.