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PP-011 DIFFERENTIAL DIAGNOSIS OF TROMBOPHILIA, LEMIERRE SYNDROME: CASE REPORT
Cardiology Posters / International Journal of Cardiology 140, Supplement 1 (2010) S1–S93
in two weeks. He was dismissed and showed decreased total cholesterol and LDL levels (respectively 237 mg/dl and 201 mg/dl) during the first follow up. Conclusions: Homozygous FH is fatal disease that manifests with cutaneous lesions and hypercholesterolemia since the early ages. Multiple xantomas on extensor faces of extremities are often present and the physician must be worn from these simple lesions to investigate for arterial atherosclerosis, coronary heart disease or aortic valve stenosis. These are common mortality reasons in these patients and early diagnosis may increase survival. Medical treatment must be administered and LDL apheresis is mandatory to decrease cholesterol levels. The patients must be under close follow up and surgical procedures such as CABG or valve replacement must be scheduled when indications are fulfilled.
PP-011 DIFFERENTIAL DIAGNOSIS OF TROMBOPHILIA, LEMIERRE SYNDROME: CASE REPORT Ertan Yucel 1 , Aydan Eroglu 2 , Isik Conkbayir 3 , Sahin Sahinalp 1 1 Department of Cardiovascular Surgery, Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey 2 Department of General Surgery,Ankara University Faculty of Medicine, Ankara, Turkey 3 Department of Radiology, Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey Objective: Lemierre syndrome (LS) is a very rare disease presenting with thrombosis of internal juguler vein (IJV) and septic embolia following orophrangeal infection. The common complaint in LS is pain in the throat and the usual findings are unilateral swelling and decrease in range of motion of neck. Methods: For diagnosis, Doppler ultrasonography, computerized tomography and magnetic resonans imaging can be used. Results: The case presented here is an otherwise healthy 29 years old multipar woman. In this case following postphrangial infection, thrombosis of the right IJV and partial thrombosis of internal carotis arter were found. The laboratory findings of the case revealed high Homocysteine levels and mutation in the metilentetrahidrofolat (MTHFR) 677 CT and MTHFR 1298AC genotype. Moreover, the other laboratory findings confirmed antiphospholipid syndrome. The patient was treated with antibiotics and anticoagulants. Laboratory findings: Hemogram; WBC; 5.9 K/ul, Protein S: 55.980 (58-130%); Hct: 36.7, Antithrombin III: 88.452 (75-125%); MCV: 81.4, Homocysteine-1: 14.2 (5-12); PLT: 160 bin/ul, Homocysteine-2: 8.60 (12-15); Neutrophil: 68%, Fibrinojen: 451.8 mg/dl (180-350); Monocytes: 12%, Sedimentation rate: 50 mm/S. Genetic mutations: Lupus antigen LA1/LA2: <2.0 positive (0.8-1.2); Faktor V Leiden: mutation (–), Anti Nuclear Antibody (ANA): positive; MTHFR* A120C-DL: Heterozygote mutation. Anti-cardiolipin IGG:84 IU/mL (0-48); MTHFR* C677T-DL: Heterozygote mutation. Anti-cardiolipin IGM:142 IU/mL (0-44). *MTHFR: methylenetetrahydrofolate reductase. Conclusions: Due to repetation of the infection this case is LS, a very rare cause of thrombosis with accompanying hereditary trombophilia mutations and antiphospholipid syndrome. The patient was suggested to keep herself from infections and continue taking anticoagulants and stop taking contraceptive pills.
PP-012 VENOUS THROMBOEMOLISM, THROMBOPHYLIA, YOUNG ADULT PATIENTS Pavel Chechulov 1 , Vladimir Soroka 1 , Sergey Kapustin 2 , Anna Demyanenko 1 1 Department of Vascular Surgery, Emergency Research Institute, St. Petersburg, Russia 2 Hematology institute, St. Petersburg, Russia Objective: Deep vein thrombosis (DVT) and pulmonary embolism (PE) are two conditions that have the common name – venous thromboembolism (VTE). It’s one of the most dangerous diseases that are poorly understood till nowadays. The main course of VTE between young adults (<45 years old) is associated with thrombophylic conditions. Methods: Retrospective analysis of the 367 patients with an unprovoked
S45
episode of VTE who were treated in our hospital since 2000 to 2009 years. Males - 241 (65%), females 126 (35%), mean age 35±2 years. We analysed 3 main genetic factors in 52 (14.7%) patients (FV Leiden, PRTG20210A, MTHFR C677T). Results: DVT was diagnosed in 247 patients. The prevalence of iliofemoral thrombosis had place in 209 (84.6%) of these patients. The presence of factor (F) V Leiden was in 23% of patients (OR=1.6, 95% Cl: 1.0-2.6; p=0.049) with majority of the heterozygote mutation. The prothrombin gene mutation was in 80.8% (OR=4.3, 95% CI: 1.7-10.9; p<0.001). MTHFR 677T was registered in 46.5% cases (OR=2.1, 95% CI: 1.2-3.7; p=0.01). PE had place in 20 (5.4%) of these patients. Lethal complication we observed in 13 (3.24%) cases. The male gender was an independent predictor of VTE (> 1.7 times), PE (>5 times) and lethal complications (>2.25 times) in compare with female gender. Conclusions: The main predictor of venouse thromboembolism is the young males who have more complications and the worse prognosis. Relatively high percent of the prothrombin G20210A mutation didn’t correlate with prognosis and severity of complications.
PP-013 FAMILIAL HYPERCHOLESTEROLEMIA PRESENTING AS BILATERAL MASTOID XANTHOMA Mehmet Ali Sendur 1 , Gulay Sain Guven 1 , Hikmet Yorgun 2 , Ahmet Hakan Ates 2 , Hamza Sunman 2 , Muhammet Dural 2 , Ulvi Yalcin 2 , Kudret Aytemir 2 1 Hacettepe University, Department of Internal Medicine, Ankara, Turkey 2 Hacettepe University, Department of Cardiology, Ankara, Turkey Objective: Familial hypercholesterolemia (FH) is an autosomal dominant disorder that causes severe elevations in total cholesterol and low-density lipoprotein cholesterol. In patients with hypercholesterolemia, xanthoma formation is frequently seen over subcutaneous tissue of extensor surfaces and tendons that have received minor trauma and friction. Intracranial xanthomas are rare: only rare cases have been reported in the literature, and most of these were unilateral. Methods: This 23-year-old man presented to our hospital with tinnitus, vertigo, headache and ataxia. Physical examination revealed that common tendinous xanthoms. There was also a strong family history of hypercholesterolemia. Results: Analysis of the patient’s blood showed a raised fasting cholesterol level of 590 mg/dl, low-density lipoprotein level of 510 mgl/dL, and highdensity lipoprotein level of 26 mg/dl. A computed tomography scan showed that there was a mass of 6.8×4.1 cm in diameter extending from the right mastoid region upward into posterior fossa. A similar mass of 3×5 cm was also seen that arose from the left mastoid and extended up into the left serebellar hemisphere. The patient underwent a right temporal craniotomy with radical mastoidectomy and tumor excision. Histological examination demonstrated fragments of fibrous tissue infiltrated by large numbers of foamy histiocytes that exhibited an abundance of cholesterol clefts and were surrounded by multinucleated giant cells The patient recovered well after surgery was treated with statin, ezetimib and niacin. Lipid apheresis was begun every two weeks. At 18 months postoperatively there was no worsening of his symptoms and has no xanthomas. Conclusions: Familial hypercholesterolemia is associated with a high risk for premature coronary artery disease, health professionals should be alert to the signs found during a physical examination and to the laboratory values suggestive of FH. Bilateral xanthoma of the temporal bone is an extremely rare, benign condition but lifelong follow-up is essential to monitor the progress of the disease.
PP-014 COMPLETE RESOLUTION OF VERY LATE STENT THROMBOSIS WITH SYSTEMIC THROMBOLYSIS: TWO CASES Omer Yiginer, Ejder Kardesoglu, Bekir Y. Cingozbay, Namik Ozmen, Mustafa Aparci, Omer Uz, Zafer Isilak, Bekir S. Cebeci Department of Cardiology, GMMA Haydarpasa Hospital, Istanbul, Turkey Objective: Because of its poor outcome, coronary stent thrombosis (ST) is a severe complication occuring following both bare metal stent (BMS) and also drug eluting stent (DES) deployement. Although many treatment strategies including thrombus aspiration, balon angioplasty, and stenting
in two weeks. He was dismissed and showed decreased total cholesterol and LDL levels (respectively 237 mg/dl and 201 mg/dl) during the first follow up. Conclusions: Homozygous FH is fatal disease that manifests with cutaneous lesions and hypercholesterolemia since the early ages. Multiple xantomas on extensor faces of extremities are often present and the physician must be worn from these simple lesions to investigate for arterial atherosclerosis, coronary heart disease or aortic valve stenosis. These are common mortality reasons in these patients and early diagnosis may increase survival. Medical treatment must be administered and LDL apheresis is mandatory to decrease cholesterol levels. The patients must be under close follow up and surgical procedures such as CABG or valve replacement must be scheduled when indications are fulfilled.
PP-011 DIFFERENTIAL DIAGNOSIS OF TROMBOPHILIA, LEMIERRE SYNDROME: CASE REPORT Ertan Yucel 1 , Aydan Eroglu 2 , Isik Conkbayir 3 , Sahin Sahinalp 1 1 Department of Cardiovascular Surgery, Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey 2 Department of General Surgery,Ankara University Faculty of Medicine, Ankara, Turkey 3 Department of Radiology, Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey Objective: Lemierre syndrome (LS) is a very rare disease presenting with thrombosis of internal juguler vein (IJV) and septic embolia following orophrangeal infection. The common complaint in LS is pain in the throat and the usual findings are unilateral swelling and decrease in range of motion of neck. Methods: For diagnosis, Doppler ultrasonography, computerized tomography and magnetic resonans imaging can be used. Results: The case presented here is an otherwise healthy 29 years old multipar woman. In this case following postphrangial infection, thrombosis of the right IJV and partial thrombosis of internal carotis arter were found. The laboratory findings of the case revealed high Homocysteine levels and mutation in the metilentetrahidrofolat (MTHFR) 677 CT and MTHFR 1298AC genotype. Moreover, the other laboratory findings confirmed antiphospholipid syndrome. The patient was treated with antibiotics and anticoagulants. Laboratory findings: Hemogram; WBC; 5.9 K/ul, Protein S: 55.980 (58-130%); Hct: 36.7, Antithrombin III: 88.452 (75-125%); MCV: 81.4, Homocysteine-1: 14.2 (5-12); PLT: 160 bin/ul, Homocysteine-2: 8.60 (12-15); Neutrophil: 68%, Fibrinojen: 451.8 mg/dl (180-350); Monocytes: 12%, Sedimentation rate: 50 mm/S. Genetic mutations: Lupus antigen LA1/LA2: <2.0 positive (0.8-1.2); Faktor V Leiden: mutation (–), Anti Nuclear Antibody (ANA): positive; MTHFR* A120C-DL: Heterozygote mutation. Anti-cardiolipin IGG:84 IU/mL (0-48); MTHFR* C677T-DL: Heterozygote mutation. Anti-cardiolipin IGM:142 IU/mL (0-44). *MTHFR: methylenetetrahydrofolate reductase. Conclusions: Due to repetation of the infection this case is LS, a very rare cause of thrombosis with accompanying hereditary trombophilia mutations and antiphospholipid syndrome. The patient was suggested to keep herself from infections and continue taking anticoagulants and stop taking contraceptive pills.
PP-012 VENOUS THROMBOEMOLISM, THROMBOPHYLIA, YOUNG ADULT PATIENTS Pavel Chechulov 1 , Vladimir Soroka 1 , Sergey Kapustin 2 , Anna Demyanenko 1 1 Department of Vascular Surgery, Emergency Research Institute, St. Petersburg, Russia 2 Hematology institute, St. Petersburg, Russia Objective: Deep vein thrombosis (DVT) and pulmonary embolism (PE) are two conditions that have the common name – venous thromboembolism (VTE). It’s one of the most dangerous diseases that are poorly understood till nowadays. The main course of VTE between young adults (<45 years old) is associated with thrombophylic conditions. Methods: Retrospective analysis of the 367 patients with an unprovoked
S45
episode of VTE who were treated in our hospital since 2000 to 2009 years. Males - 241 (65%), females 126 (35%), mean age 35±2 years. We analysed 3 main genetic factors in 52 (14.7%) patients (FV Leiden, PRTG20210A, MTHFR C677T). Results: DVT was diagnosed in 247 patients. The prevalence of iliofemoral thrombosis had place in 209 (84.6%) of these patients. The presence of factor (F) V Leiden was in 23% of patients (OR=1.6, 95% Cl: 1.0-2.6; p=0.049) with majority of the heterozygote mutation. The prothrombin gene mutation was in 80.8% (OR=4.3, 95% CI: 1.7-10.9; p<0.001). MTHFR 677T was registered in 46.5% cases (OR=2.1, 95% CI: 1.2-3.7; p=0.01). PE had place in 20 (5.4%) of these patients. Lethal complication we observed in 13 (3.24%) cases. The male gender was an independent predictor of VTE (> 1.7 times), PE (>5 times) and lethal complications (>2.25 times) in compare with female gender. Conclusions: The main predictor of venouse thromboembolism is the young males who have more complications and the worse prognosis. Relatively high percent of the prothrombin G20210A mutation didn’t correlate with prognosis and severity of complications.
PP-013 FAMILIAL HYPERCHOLESTEROLEMIA PRESENTING AS BILATERAL MASTOID XANTHOMA Mehmet Ali Sendur 1 , Gulay Sain Guven 1 , Hikmet Yorgun 2 , Ahmet Hakan Ates 2 , Hamza Sunman 2 , Muhammet Dural 2 , Ulvi Yalcin 2 , Kudret Aytemir 2 1 Hacettepe University, Department of Internal Medicine, Ankara, Turkey 2 Hacettepe University, Department of Cardiology, Ankara, Turkey Objective: Familial hypercholesterolemia (FH) is an autosomal dominant disorder that causes severe elevations in total cholesterol and low-density lipoprotein cholesterol. In patients with hypercholesterolemia, xanthoma formation is frequently seen over subcutaneous tissue of extensor surfaces and tendons that have received minor trauma and friction. Intracranial xanthomas are rare: only rare cases have been reported in the literature, and most of these were unilateral. Methods: This 23-year-old man presented to our hospital with tinnitus, vertigo, headache and ataxia. Physical examination revealed that common tendinous xanthoms. There was also a strong family history of hypercholesterolemia. Results: Analysis of the patient’s blood showed a raised fasting cholesterol level of 590 mg/dl, low-density lipoprotein level of 510 mgl/dL, and highdensity lipoprotein level of 26 mg/dl. A computed tomography scan showed that there was a mass of 6.8×4.1 cm in diameter extending from the right mastoid region upward into posterior fossa. A similar mass of 3×5 cm was also seen that arose from the left mastoid and extended up into the left serebellar hemisphere. The patient underwent a right temporal craniotomy with radical mastoidectomy and tumor excision. Histological examination demonstrated fragments of fibrous tissue infiltrated by large numbers of foamy histiocytes that exhibited an abundance of cholesterol clefts and were surrounded by multinucleated giant cells The patient recovered well after surgery was treated with statin, ezetimib and niacin. Lipid apheresis was begun every two weeks. At 18 months postoperatively there was no worsening of his symptoms and has no xanthomas. Conclusions: Familial hypercholesterolemia is associated with a high risk for premature coronary artery disease, health professionals should be alert to the signs found during a physical examination and to the laboratory values suggestive of FH. Bilateral xanthoma of the temporal bone is an extremely rare, benign condition but lifelong follow-up is essential to monitor the progress of the disease.
PP-014 COMPLETE RESOLUTION OF VERY LATE STENT THROMBOSIS WITH SYSTEMIC THROMBOLYSIS: TWO CASES Omer Yiginer, Ejder Kardesoglu, Bekir Y. Cingozbay, Namik Ozmen, Mustafa Aparci, Omer Uz, Zafer Isilak, Bekir S. Cebeci Department of Cardiology, GMMA Haydarpasa Hospital, Istanbul, Turkey Objective: Because of its poor outcome, coronary stent thrombosis (ST) is a severe complication occuring following both bare metal stent (BMS) and also drug eluting stent (DES) deployement. Although many treatment strategies including thrombus aspiration, balon angioplasty, and stenting