A Case of Klippel-Trenaunay Syndrome with Acute Submassive Pulmonary Thromboembolism Treated with Thrombolytic Therapy.

Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by varicose veins, cutaneous hemangiomas, soft tissue and bony hypertrophy of limb. Potential complications such as deep venous thrombosis and pulmonary thromboembolism have not been reported in Korea to date. We demonstrate the case of a 48-year-old woman with Klippel-Trenaunay syndrome with extensive varicose veins on right lower limb, hypertrophy of left big toe and basilar artery tip aneurysm, complicated with acute submassive pulmonary thromboembolism treated successfully with intravenous thrombolytic therapy.

A case of severe coronary spasm associated with 5-fluorouracil chemotherapy.

Cardiotoxicity associated with 5-fluorouracil (FU) is an uncommon, but potentially lethal, condition. The case of an 83-year-old man with colon cancer who developed chest pain during 5-FU infusion is presented. The electrocardiogram (ECG) showed pronounced ST elevation in the lateral leads, and the chest pain was resolved after infusion of nitroglycerin. A coronary angiogram (CAG) revealed that the patient had significant atherosclerosis in the proximal left circumflex artery. Coronary artery spasm with fixed stenosis was considered, and a drug-eluting stent was implanted. After 8 hours, the patient complained of recurring chest pain, paralleled by ST elevation on the ECG. The chest pain subsided after administration of intravenous nitroglycerin followed by sublingual nifedipine. Repeated CAG showed patency of the previous stent. This case supports the vasospastic hypothesis of 5-FU cardiac toxicity, indicating that a calcium channel blocker may be effective in the prevention or treatment of 5-FU cardiotoxicity.

Recurrent myocardial infarction due to one subacute and two very late thrombotic events of drug-eluting stent associated with clopidogrel resistance.

Although drug-eluting stent (DES) thrombosis is a recognized complication of coronary intervention, recurrent late stent thrombosis is rarely reported. Clopidogrel resistance or low-responsiveness may be associated with recurrent late stent thrombosis after DES implantation. We present a diabetic patient with hypertension, obesity and heavy smoking history, who suffered 3 recurrent attacks of acute anterior ST-segment elevation myocardial infarction over 6 years due to recurrent thromboses of DES despite prolonged and regular triple antiplatelet therapy. Laboratory evaluation of hypercoagulability yielded negative results, but low responsiveness for clopidogrel was revealed by the VerifyNow P2Y12 assay.

Acute idiopathic hemorrhagic pericarditis with cardiac tamponade as the initial presentation of acquired immune deficiency syndrome.

This paper presents a case of cardiac tamponade with idiopathic hemorrhagic pericarditis as the initial symptom of human immunodeficiency virus (HIV) infection. A 29-year-old male came to the emergency room with a sudden onset of dizziness. Upon arrival, he was hypotensive although not tachycardic, and his jugular venous pressure was not elevated. His chest X-rays revealed a mild cardiomegaly. Transthoracic echocardiography revealed a large amount of pericardial effusion with a diastolic collapse of the right ventricle, a dilated inferior vena cava with little change in respiration, and exaggerated respiratory variation of mitral inflow velocities, representing echocardiographic evidence of cardiac tamponade. After pericardiocentesis, his blood pressure improved to 110/70 mmHg without inotropics support. Serial 12-lead electrocardiograms during hospitalization revealed upwardly concave diffuse ST-segment elevation followed by a T-wave inversion suggestive of acute pericarditis. Pericardial fluid cytology and cultures for bacteria, mycobacteria, adenovirus, and fungus were all negative. HIV enzyme-linked immunosorbent assay (ELISA) was positive and confirmed by Western blot. The CD4 cell count was 168/mm(3). Finally, the diagnosis of cardiac tamponade due to HIV-associated hemorrhagic pericarditis was made. It was concluded that HIV infection should be considered in the diagnosis of unexplained pericardial effusion or cardiac tamponade in Korea.

Atypical takotsubo cardiomyopathy associated with nasal packing for paranasal sinus surgery.

Takotsubo cardiomyopathy (TC) is characterized by reversible left ventricular (LV) apical ballooning and no significant coronary artery stenosis. New variants of TC with localized wall motion abnormality or inversed pattern with hyperdynamic apex have been reported. We present the case of a 24-year-old female with atypical presentation of TC occurring in the setting of paranasal sinus surgery under local anaesthesia with post-surgical nasal packing. She did not demonstrate ST-segment elevation on electrocardiogram, but transient moderate LV systolic dysfunction and localized wall motion abnormality affecting basal to mid-ventricular anterior and anteroseptal wall. She rapidly and completely recovered without sequelae.

A case of Leclercia adecarboxylata endocarditis in a woman with endometrial cancer.

Leclercia adecarboxylata, a motile, gram-negative bacillus of the enterobacteriaceae family, is rarely recovered as a pathogen. We report an exceptionally rare case of L. adecarboxylata endocarditis complicated by embolization of the kidney and spleen in a 48-year-old woman with endometrial cancer. After 4 weeks of antimicrobial treatment, she recovered without sequelae.

Sulfasalazine induces haem oxygenase-1 via ROS-dependent Nrf2 signalling, leading to control of neointimal hyperplasia.

AIMS: Inflammation, and the subsequent proliferative activity of vascular smooth muscle cells (VSMCs), is one of the major pathophysiological mechanisms associated with neointimal hyperplasia following vascular injury. Although sulfasalazine (SSZ) has been used as an anti-inflammatory and immune-modulatory agent in various inflammatory diseases, its primary targets and therapeutic effects on vascular disease have not yet been determined. We investigated whether SSZ could suppress VSMC growth and prevent neointimal hyperplasia. METHODS AND RESULTS: SSZ was found to have pro-apoptotic and anti-proliferative activity in cultured VSMCs. Unexpectedly, these effects were not mediated by nuclear factor kappa B (NF-kappaB) inhibition, which has been suggested to be the anti-inflammatory mechanism associated with the effects of SSZ. Instead, cell-cycle arrest of the VSMCs was observed, which was mediated by induction of haem oxygenase-1 (HO-1) followed by an increased expression of p21(waf1/Cip1). The underlying mechanism for SSZ-induced HO-1 expression was by reactive oxygen species (ROS)-dependent nuclear translocation and activation of nuclear factor erythroid-2-related factor 2 (Nrf2). In a rat carotid artery balloon injury model, administration of SSZ significantly suppressed neointimal growth. In a series of reverse experiments, inhibition of HO-1 by shRNA, ROS by N-acetylcysteine (NAC) or Nrf2 by dominant-negative Nrf2 abrogated the beneficial effects of SSZ. CONCLUSION: Our data demonstrate that SSZ inhibits VSMC proliferation in vitro and in vivo through a novel signalling pathway and may be a promising therapeutic option for the treatment of proliferative vascular disease.

Spontaneous rupture of idiopathic thymic abscess with a markedly increased CA-125 level.

We report a rare case of spontaneous rupture of idiopathic thymic abscess into the pleural cavity. A 64-year-old woman was admitted to hospital with pleuritic retrosternal chest pain. Chest roentgenograms disclosed a small amount of bilateral pleural effusion, the examination of which exposed a sterile serous exudate with a markedly increased CA-125 level. Chest computed tomography revealed a large anterior mediastinal cystic mass with bilateral pleural effusions. Following complete resection of the mass, the histological examination revealed cavitary lesion with necrotic thymic tissue and inflammatory infiltrate surrounded by fibrous wall. The immunohistochemical staining for CA-125 displayed strong positivity at the Hassall's corpuscles. Cyst fluid also revealed a highly elevated CA-125 level. Her serum CA-125 concentration two months after surgery had fallen to 28 IU/L. She is now doing well without recurrence of the cyst five months after surgery.

Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney complex.

The authors report the case of multiple right-sided myxomas in a 42-year-old man with Carney complex. He had previously been diagnosed as Cushing's syndrome and undergone resection of pituitary adenoma at the age of 21. After 10 years, bilateral adrenalectomy had been performed with recurrence of Cushing's syndrome. Recently, he complained of palpitation and intracardiac masses were detected. On physical examination, he had nevi on the lips and in the oral cavity. A tiny eyelid nodule was noted and histopathological analysis confirmed the diagnosis of skin myxoma. Thyroid ultrasonography revealed multiple hypoechoic nodules, which were confirmed pathologically as follicular adenomas. Scrotal ultrasonography also revealed small multiple testicular tumors. Echocardiography demonstrated intracardiac masses in right atrium and right ventricle. Right atriotomy revealed a right atrial mass attached to the annulus of the tricuspid valve and another mass arising from the interatrial septum. In right ventricle, a polypoid mass arose from the anterior leaflet of the tricuspid valve. Also, there were multiple tiny to small masses on the interatrial septum. The masses were completely excised and the tricuspid valve was repaired with annuloplasty. Histopathological analysis confirmed myxomas with foci of extramedullary hematopoiesis and ossification.

Stent-related cardiac events after non-cardiac surgery: drug-eluting stent vs. bare metal stent.

For evaluation of post-operative stent associated cardiac events after drug-eluting stent (DES) implantation we analyzed data of 138 patients who underwent non-cardiac surgery after DES implantation and compared the data with those of 101 patients who underwent non-cardiac surgery after bare metal stent (BMS) implantation. Three patients (2.2%) in DES group developed post-operative cardiac events and none in BMS group (p=0.2). One patient died due to suspected stent thrombosis and the other two had myocardial infarction due to angiographically proven stent thrombosis. The time interval between stent implantation and surgery in those with cardiac events was 6, 264, and 367 days, respectively. Our data shows that peri-operative stent-related thrombotic complications can occur long after DES implantation and careful peri-operative monitoring for cardiac events is needed in patients receiving non-cardiac surgery after DES implantation.

Hypereosinophilia presenting as eosinophilic vasculitis and multiple peripheral artery occlusions without organ involvement.

We report here a case with hypereosinophilia and peripheral artery occlusion. A 32-yr-old Korean woman presented to us with lower extremity swelling and pain. Angiography revealed that multiple lower extremity arteries were occlusive. The biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin lesion and pain were improved and the eosinophil count was dramatically decreased. After discharge, eosinophil count gradually increased again. Cyanosis and pain of her fingers recurred. She had been treated with cyclophosphamide pulse therapy. Her eosinophilia was decreased, but the cyanosis and tingling sense were progressive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive eosinophilic infiltration. The serum TNF-alpah was markedly increased. These results suggest that CD40L (a member of TNF-alpah superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged.

Activated forkhead transcription factor inhibits neointimal hyperplasia after angioplasty through induction of p27.

OBJECTIVE: We examined the effects of FKHRL1 (forkhead transcription factor in rhabdomyosarcoma like-1) overexpression on vascular smooth muscle cell (VSMC) proliferation, apoptosis, and cell cycle, in vitro, and the role of FKHRL1 and p27 in the pathophysiology of neointimal growth after balloon angioplasty, in vivo. Furthermore, we tested whether FKHRL1 overexpression can inhibit neointimal hyperplasia in a rat carotid artery model. METHODS AND RESULTS: Adenovirus expressing the constitutively active FKHRL1 (FKHRL1-TM; triple mutant) with 3 Akt phosphorylation sites mutated was transfected to subconfluent VSMCs. FKHRL1 overexpression in cultured VSMCs increased p27 expression, leading to G1 phase cell-cycle arrest and increased apoptosis. In vivo, the phosphorylation of FKHRL1 increased significantly 3 hours after balloon injury and decreased thereafter, with the subsequent downregulation of p27. Although the phosphorylation of FKHRL1 was greatest at 3 hours, the downregulation of p27 showed a temporal delay, only slightly starting to decrease after 3 hours and reaching a nadir at 72 hours after balloon injury. Gene transfer of FKHRL1-TM increased p27, decreased proliferation, and increased apoptosis of VSMCs, which resulted in a marked reduction in neointima formation (intima-to-media ratio: 0.31+/-0.13 versus 1.17+/-0.28, for FKHRL1-TM versus Adv-GFP; P<0.001). CONCLUSIONS: Balloon angioplasty leads to the phosphorylation of FKHRL1 and decreased expression of p27, thereby promoting a proliferative phenotype in VSMCs in vitro and in vivo. This study reveals the importance of FKHRL1 in proliferation and viability of VSMCs and suggests that it may serve as a molecular target for interventions to reduce neointima formation after angioplasty.