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Sample records for acute aortic syndrome

  1. Acute aortic syndromes: current status.

    Science.gov (United States)

    Ridge, Carole A; Litmanovich, Diana E

    2015-05-01

    The term acute aortic syndrome comprises aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer. The most recent developments in acute aortic syndromes include (1) a change in the mindset that each entity is pathologically distinct, with a shift toward considering the acute aortic syndromes as points along a spectrum of aortic disease, (2) the optimization of aortic imaging quality and radiation dose, and (3) surgical or endovascular management. This review article focuses on how these developments pertain to thoracic radiologists.

  2. Acute Type II Aortic Dissection with Severe Aortic Regurgitation and Chronic Descending Aortic Dissection in Pregnant Patient with Marfan Syndrome

    OpenAIRE

    Lee, Seok-Soo; Jung, Tae-Eun; Lee, Dong Hyup

    2012-01-01

    Aortic dilatation and dissection are severe complications during pregnancy that can be fatal to both the mother and the fetus. The risks of these complications are especially high in pregnant patients with Marfan syndrome; however, incidents of descending aortic dissection are very rare. This case report involves a successful Bentall procedure for and recovery from a rare aortic dissection in a pregnant Marfan patient who developed acute type II aortic dissection with severe aortic regurgitat...

  3. Acute Type II Aortic Dissection with Severe Aortic Regurgitation and Chronic Descending Aortic Dissection in Pregnant Patient with Marfan Syndrome.

    Science.gov (United States)

    Lee, Seok-Soo; Jung, Tae-Eun; Lee, Dong Hyup

    2012-12-01

    Aortic dilatation and dissection are severe complications during pregnancy that can be fatal to both the mother and the fetus. The risks of these complications are especially high in pregnant patients with Marfan syndrome; however, incidents of descending aortic dissection are very rare. This case report involves a successful Bentall procedure for and recovery from a rare aortic dissection in a pregnant Marfan patient who developed acute type II aortic dissection with severe aortic regurgitation and chronic descending aortic dissection immediately after Cesarean section. Regular follow-up will be needed to monitor the descending aortic dissection.

  4. Acute aortic dissection mimics acute inferoposterior wall myocardial infarction in a Marfan syndrome patient

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    Phowthongkum, Prasit

    2010-01-01

    A 30-year old man with acute chest pain was diagnosed with acute inferoposterior wall myocardial infarction following electrocardiography. After a failed coronary angiography, an echocardiogram revealed an aortic intimal flap after which acute aortic dissection was diagnosed. The patient received a successful Bentall operation without immediate complication. Retrospective examination then confirmed the diagnosis of Marfan syndrome. This case demonstrates acute aortic dissection may mimic acut...

  5. Acute aortic dissection mimics acute inferoposterior wall myocardial infarction in a Marfan syndrome patient.

    Science.gov (United States)

    Phowthongkum, Prasit

    2010-01-01

    A 30-year old man with acute chest pain was diagnosed with acute inferoposterior wall myocardial infarction following electrocardiography. After a failed coronary angiography, an echocardiogram revealed an aortic intimal flap after which acute aortic dissection was diagnosed. The patient received a successful Bentall operation without immediate complication. Retrospective examination then confirmed the diagnosis of Marfan syndrome. This case demonstrates acute aortic dissection may mimic acute myocardial infarction.

  6. Acute aortic dissection in pregnancy with the marfan syndrome.

    Science.gov (United States)

    Kim, Si Wook; Kim, Dohun; Hong, Jong-Myeon

    2014-06-01

    Acute aortic dissection (AAD) during pregnancy can be fatal to both the pregnant mother and the baby, particularly in patients with the Marfan syndrome. We report a case of the modified Bentall procedure in surgery for AAD in a 31-year-old pregnant woman at 24 weeks of gestation with the Marfan syndrome. The patient recovered well after the operation, but unfortunately, the fetus could not be saved.

  7. Acute Aortic Dissection in Pregnancy with the Marfan Syndrome

    OpenAIRE

    Kim, Si Wook; Kim, Dohun; Hong, Jong-Myeon

    2014-01-01

    Acute aortic dissection (AAD) during pregnancy can be fatal to both the pregnant mother and the baby, particularly in patients with the Marfan syndrome. We report a case of the modified Bentall procedure in surgery for AAD in a 31-year-old pregnant woman at 24 weeks of gestation with the Marfan syndrome. The patient recovered well after the operation, but unfortunately, the fetus could not be saved.

  8. Extended aortic repair using frozen elephant trunk technique for Marfan syndrome with acute aortic dissection.

    Science.gov (United States)

    Uchida, Naomichi; Katayama, Akira; Kuraoka, Masatsugu; Katayama, Keijiro; Takahashi, Shinya; Takasaki, Taiichi; Sueda, Taijiro

    2013-01-01

    The aim of this study was to analyze midterm results of frozen elephant trunk technique for Marfan syndrome with acute aortic dissection. Between February 1999 and August 2011 we performed arch replacement uisng frozen elephant trunk technique for acute aortic dissection in 8 patients with Marfan syndrome containing two complicated type B dissections and six type A dissections.Five patients compromised annulo-aortic ectasia who performed Bentall operation. No patients died in the initial operation. Fate of false lumen on the stent graft border was expressed by CT scan follow-up that were patent in 0, thrombosis in 5 and absorption in 3 patients. One patient who had new aortic dissection 8 years after initial surgery required the Crawford V operation. Ten-years-survival rate was 100% and ten years-event free rate was 67%. Frozen elephant trunk technique was feasible for Marfan syndrome with acute aortic dissection and might become alternative prophylactic treatment to the downstream aorta for acute aortic dissection.

  9. Abdominal Aortic Dissection with Acute Mesenteric Ischemia in a Patient with Marfan Syndrome

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    Chii-Shyan Lay

    2006-07-01

    Full Text Available Marfan syndrome is an autosomal dominant inherited disorder of connective tissue, with various complications manifested primarily in the cardiovascular system. It potentially leads to aortic dissection and rupture, these being the major causes of death. We report a patient who complained of acute abdominal pain, which presented as acute mesenteric ischemia combined with abdominal aortic dissection. Echocardiography showed enlargement of the aortic root and mitral valve prolapse. Abdominal computed tomography scan revealed acute mesenteric ischemia due to abdominal aortic dissection. Finally, the patient underwent surgery of aortic root replacement and had a successful outcome. Therefore, we suggest that for optimal risk assessment and monitoring of patients with Marfan syndrome, both aortic stiffness and the diameter of the superior mesenteric vein compared with that of the superior mesenteric artery are useful screening methods to detect acute mesenteric ischemia secondary to abdominal aortic dissection. Early diagnosis and early treatment can decrease the high mortality rate of patients with Marfan syndrome.

  10. Aortic root reconstruction by aortic valve-sparing operation (David type I reimplantation) in Marfan syndrome accompanied by annuloaortic ectasia and acute type-A aortic dissection.

    Science.gov (United States)

    Inamura, Shunichi; Furuya, Hidekazu; Yagi, Kentarou; Ikeya, Eriko; Yamaguchi, Masaomi; Fujimura, Takabumi; Kanabuchi, Kazuo

    2006-09-20

    To reconstruct the aortic root for aneurysm of the ascending aorta accompanied by aortic regurgitation, annuloaortic ectasia (AAE) and acute type-A dissection with root destruction, the Bentall operation using a prosthetic valve still is the standard procedure today. Valve-sparing procedures have actively been used for aortic root lesions, and have also been attempted in aortic root reconstruction for Marfan syndrome which may have abnormalities in the valve leaflets. We conducted a valve-sparing procedure in a female patient with Marfan syndrome who had AAE accompanied by type-A acute aortic dissection. The patient was a 37-year-old woman complaining of severe pain from the chest to the back. The limbs were long, and funnel breast was observed. Diastolic murmurs were heard. On chest computed tomography, a dissection cavity was present from the ascending aorta to the left common iliac artery, and the root dilated to 55 mm. Grade II aortic regurgitation was observed on ultrasound cardiography. Regarding her family history, her father had died suddenly at 54 years of age. She was diagnosed with type-A acute dissection concurrent with Marfan syndrome and AAE. The structure of the aortic valve was normal, and root reconstruction by a valve-sparing operation and total replacement of the aortic arch was conducted. On postoperative ultrasound cardiography, the aortic regurgitation was within the allowable range, and the shortterm postoperative results were good.

  11. Acute coronary syndrome in a patient with Marfan syndrome following emergent surgical repair of aortic dissection.

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    Bovolato, Francesca Elisa; Isabella, Giambattista; Rampazzo, Debora; Guglielmi, Cosimo; Gerosa, Gino; Iliceto, Sabino; Bilato, Claudio

    2008-06-01

    We report a case of acute coronary syndrome in a patient with suspect Marfan syndrome, 25 days after emergent modified Bentall-De Bono intervention for acute type I peripartum aortic dissection. She was admitted to our intensive care unit because of unstable angina, caused by critical blood flow reduction in a large portion of the myocardium, according to the severity of the symptoms and the electrocardiographic alterations. Coronary angiography showed a sub-occlusive stenosis of the left main coronary artery as a result of the dissection extension to the coronary ostium. Because of the high risk related to heart surgery, the patient was successfully treated by unprotected angioplasty and drug-eluting stent positioning. Short- and mid-term outcomes were favourable. Subsequent tests confirmed the diagnosis of Marfan syndrome. After 2 years of follow-up, the patient remains asymptomatic and in good health. To our knowledge, this is the first report of a successful percutaneous intervention of the left main coronary artery in a patient with Marfan syndrome who had already undergone ascending aortic root and valve replacement by the Bentall-De Bono procedure for acute dissection.

  12. Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    Mandana Master

    2012-01-01

    Full Text Available We report a case of acute aortic dissection in a lady of 28 weeks of gestation with undiagnosed Marfan syndrome. The patient had been seen in our antenatal clinics. Her history documented in her pregnancy record was negative for genetic/congenital abnormalities. There was no family history documented. Subsequently, at 28 weeks of gestation, the patient presented with sudden onset chest, jaw, and back pain. Further history revealed that her father had died at the age of 27 of an aortic dissection. Echocardiography showed aortic root dissection with occlusion of aortic branches. She subsequently underwent an emergency lower segment caesarean section followed by surgical repair of type A dissection. A simultaneous type B dissection was managed conservatively. On later examination, our patient fulfilled the diagnostic criteria for phenotypic expression of Marfan syndrome. Genetic testing also confirmed that she has a mutation of the fibrillin (FBN 1 gene associated with the disease.

  13. [Acute type A aortic dissection with Birt-Hogg-Dubé syndrome; report of a case].

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    Sakaguchi, Masayuki; Gotou, Hirohisa; Nakahara, Takashi; Fuke, Megumi; Nishimura, Kazunori; Kuraishi, Hiroshi; Furuya, Mitsuko

    2014-05-01

    We describe a 76-year-old woman with cardiac tamponade who was admitted to our hospital. She underwent ascending and partial arch aortic replacement to treat acute type A aortic dissection. However, postoperative respiratory failure developed and a chest X-ray revealed right lung pneumothorax. The lung was finally expanded after difficulties with prolonged tube drainage. Chest computed tomography(CT) showed multiple cystic changes in the bilateral lungs. Her sister and her daughter also had a history of spontaneous pneumothorax. We finally diagnosed Birt-Hogg-Dube syndrome after deoxyribonucleic asid(DNA)sequencing of folliculin( FLCN) gene.

  14. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

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    Smith, Katherine; Gros, Bernard

    2017-05-01

    A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of women with Marfan syndrome (n = 36) and without Marfan syndrome (n = 36) were conducted. There were no significant differences in demographics (age, gravidity, parity) between the Marfan and non-Marfan cases. Marfan patients presented with antepartum dissections significantly earlier in pregnancy than those without Marfan syndrome (P = .002). However, there were no significant difference between the 2 groups in maternal mortality, fetal mortality, or obstetric outcomes (mode of delivery and gestational age at delivery). Eight cases described events in Marfan women with an aortic root diameter ≤40 mm. Six events occurred in Marfan women who were managed with beta blockers. Current guidelines rely on aortic root diameter for stratification of Marfan women into risk categories, but we identified several cases that would be missed by these guidelines. Specifically, the existing literature suggest that women with Marfan syndrome should take precautions throughout pregnancy, rather than the third trimester. © 2017 Wiley Periodicals, Inc.

  15. Clinical manifestation as acute coronary syndrome without electrocardiographically ischemia: a clue for aortic dissection

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    Hung Yi Chen

    2015-06-01

    Full Text Available Aortic dissection is a critical condition requiring immediate assessment and management. Clinical presentation is commonly associated with severe chest pain and high blood pressure. However, misdiagnosis is frequent because of various features. We presented a case of 51-year-old woman who complained of dyspnea for 3 d after she experienced back pain for one week. She was presented with severe respiration distress with impending respiration failure on arrival to our hospital. Her chest X-ray showed cardiomegaly with acute pulmonary edema. The laboratory data revealed elevated cardiac enzyme and electrocardiography demonstrated sinus tachycardia. She was hospitalized under the initial diagnosis of acute coronary syndrome. The patient remained hemodynamically stable, and experienced one episode of chest discomfort. After electrocardiography, she was found with bigeminy ventricular premature beats without ST-T change. Follow-up cardiac enzyme demonstrated progressive declined. Cardiac catheterization was performed on the third day of admission, and coronary angiography revealed large intimal flap on aortic root with bilateral coronary artery involvement. Surgical management was arranged after immediate chest computed tomography study.

  16. Aortic dissection with acute malperfusion syndrome: Endovascular fenestration via the funnel technique.

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    Vendrell, Anne; Frandon, Julien; Rodiere, Mathieu; Chavanon, Olivier; Baguet, Jean-Philippe; Bricault, Ivan; Boussat, Bastien; Ferretti, Gilbert Raymond; Thony, Frédéric

    2015-07-01

    To analyze the short- and long-term results of an original aortic fenestration method using the funnel technique during aortic dissection complicated by malperfusion syndrome. The funnel technique consists of deployment of an uncovered aortic stent graft placed from the false to the right lumen through an intimal flap aortic fenestration made by balloon angioplasty. Twenty-eight patients presenting with an aortic dissection (type A, n = 19; type B, n = 9) were treated for malperfusion syndrome owing to dynamic compression (16 renal, 17 bowel, and 13 lower limb ischemia) using the aforementioned technique, and had follow-up evaluation at short term (30 days) and long term (mean: 55 ± 40 months). Eight patients had severe ischemia on arrival (6 bowel, 7 renal, 3 lower limb). Technical success was achieved in 27 of 28 patients (96%), and ischemic symptoms had disappeared in 25 of 28 patients (89%) at short-term follow up. Five patients presented postprocedure complications: 4 minor and 1 major with arterial thrombosis which caused technical failure (3.6%). The 30-day mortality rate was 7% (n = 2), related to bowel ischemia complications. At long-term follow up, 21 patients had a stable thoracic aortic diameter (91%). The funnel technique, in cases of malperfusion syndrome after aortic dissection, safely improves short- and long-term clinical outcome, and could represent an interesting alternative in the management of patients. The hemodynamic efficiency of this technique may account for a lower mortality in our series. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  17. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis

    Directory of Open Access Journals (Sweden)

    Izumi M

    2011-12-01

    Full Text Available Manabu Izumi, Shoko Teraoka, Keisuke Yamashita, Kenji Matsumoto, Tomohiro Muronoi, Yoshimitsu Izawa, Chikara Yonekawa, Masaki Ano, Masayuki SuzukawaDepartment of Emergency and Critical Care Medicine, Jichi Medical University, Tochigi, JapanAbstract: A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.Keywords: intra-aortic thrombus, antiphospholipid antibody syndrome, spinal cord infarction

  18. [Modern aortic surgery in Marfan syndrome--2011].

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    Kallenbach, K; Schwill, S; Karck, M

    2011-09-01

    Marfan syndrome is a hereditary disease with a prevalence of 2-3 in 10,000 births, leading to a fibrillin connective tissue disorder with manifestations in the skeleton, eye, skin, dura mater and in particular the cardiovascular system. Since other syndromes demonstrate similar vascular manifestations, but therapy may differ significantly, diagnosis should be established using the revised Ghent nosology in combination with genotypic analysis in specialized Marfan centres. The formation of aortic root aneurysms with the subsequent risk of acute aortic dissection type A (AADA) or aortic rupture limits life expectancy in patients with Marfan syndrome. Therefore, prophylactic replacement of the aortic root needs to be performed before the catastrophic event of AADA can occur. The goal of surgery is the complete resection of pathological aortic tissue. This can be achieved with excellent results by using a (mechanically) valved conduit that replaces both the aortic valve and the aortic root (Bentall operation). However, the need for lifelong anticoagulation with Coumadin can be avoided using the aortic valve sparing reimplantation technique according to David. The long-term durability of the reconstructed valve is favourable, and further technical improvements may improve longevity. Although results of prospective randomised long-term studies comparing surgical techniques are lacking, the David operation has become the surgical method of choice for aortic root aneurysms, not only at the Heidelberg Marfan Centre. Replacement of the aneurysmal dilated aortic arch is performed under moderate hypothermic circulatory arrest combined with antegrade cerebral perfusion using a heart-lung machine, which we also use in thoracic or thoracoabdominal aneurysms. Close post-operative follow-up in a Marfan centre is pivotal for the early detection of pathological changes on the diseased aorta.

  19. Total Endovascular Aortic Repair in a Patient with Marfan Syndrome.

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    Amako, Mau; Spear, Rafaëlle; Clough, Rachel E; Hertault, Adrien; Azzaoui, Richard; Martin-Gonzalez, Teresa; Sobocinski, Jonathan; Haulon, Stéphan

    2017-02-01

    The aim of this study is to describe a total endovascular aortic repair with branched and fenestrated endografts in a young patient with Marfan syndrome and a chronic aortic dissection. Open surgery is the gold standard to treat aortic dissections in patients with aortic disease and Marfan syndrome. In 2000, a 38-year-old man with Marfan syndrome underwent open ascending aorta repair for an acute type A aortic dissection. One year later, a redo sternotomy was performed for aortic valve replacement. In 2013, the patient presented with endocarditis and pulmonary infection, which necessitated tracheostomy and temporary dialysis. In 2014, the first stage of the endovascular repair was performed using an inner branched endograft to exclude a 77-mm distal arch and descending thoracic aortic aneurysm. In 2015, a 63-mm thoracoabdominal aortic aneurysm was excluded by implantation of a 4-fenestrated endograft. Follow-up after both endovascular repairs was uneventful. Total aortic endovascular repair was successfully performed to treat a patient with arch and thoraco-abdominal aortic aneurysm associated with chronic aortic dissection and Marfan syndrome. The postoperative images confirmed patency of the endograft and its branches, and complete exclusion of the aortic false lumen. Endovascular repair is a treatment option in patients with connective tissue disease who are not candidates for open surgery. Long-term follow-up is required to confirm these favorable early outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. RELEVANCIA DEL DIAGNÓSTICO DIFERENCIAL ENTRE EL SÍNDROME AÓRTICO AGUDO Y EL SÍNDROME CORONARIO AGUDO EN PACIENTES CON DOLOR TORÁCICO Y CRISIS HIPERTENSIVA: REVISIÓN A PROPÓSITO DE 2 CASOS / Relevance of the differential diagnosis between acute aortic syndrome and acute coronary syndrome in patients with thoracic pain and hypertensive crisis: review on 2 case reports

    OpenAIRE

    Borja Simó Sánchez; Maria Pilar Portero Pérez; Jose Ramón Ruiz Arroyo; Jose Antonio Linares Vicente

    2011-01-01

    Acute aortic syndrome is a pathological process with low incidence compared with acute coronary syndrome, although with a worse prognosis in the short term, which is why its early diagnosis and urgent treatment are essential to the favorable evolution of thepatient. Electrocardiographic changes suggestive of myocardial ischemia, with acute evolution, are rare in acute aortic syndrome. Even in the presence of a suggestive thoracic pain and high levels of blood pressure, an adequate differentia...

  1. Recurrent acute pulmonary oedema after aortic and mitral valve surgery due to trachea malacia and obstructive sleep apnoea syndrome

    NARCIS (Netherlands)

    Sankatsing, S. U. C.; Hanselaar, W. E. J. J.; van Steenwijk, R. P.; Van der Sloot, J. A. P.; Broekhuis, E.; Kok, W. E. M.

    2008-01-01

    In this report we describe a patient with recurrent episodes of acute pulmonary oedema after aortic and mitral valve surgery. The first episode of pulmonary oedema was caused by mitral valve dysfunction. The second episode of pulmonary oedema was not clearly associated with a mitral valve problem,

  2. Diagnostic imaging of acute aortic dissection

    International Nuclear Information System (INIS)

    Ohya, Tohru; Kumazaki, Tatsuo

    1991-01-01

    One hundred and nineteen patients with aortic dissection who underwent diagnostic imaging were reviewed and angiographic findings as well as those of CT were analysed. Thirty eight cases (43.1%) had non-contrast opacified false lumen, the type of which we call 'thrombosed type aortic dissection'. A comparative study of the thrombosed type with the patent type of false lumens was made particularly from the stand point of the characteristic diagnostic imagings (CT and angiography). At the same time, the pitfalls of these imagings in thrombosed type aortic dissection were studied. At the onset the average age of thrombosed type was 62.3 years old, while that of the patent type was 57.3. A statistical significance between the two groups was p<0.05. Thrombosed type in all cases was caused by atherosclerosis, whereas patent type was caused by the Marfan's syndrome in 11 cases. Other clinical findings, such as initial symptoms and blood pressure revealed no significant differences between the two groups. Pre-contrast CT in acute thrombosed type aortic dissection showed 'hyperdense crescent sign' in 89.4%. However, in 3 cases with thrombosed type in which the pre-contrast CT showed 'hyperdense crescent sign' contrast-enhanced CT detected no clear evidence of aortic dissection in the same site. This was due to obscurity induced by contrast medium. Angiographic findings of thrombosed type were classified into 3 groups: normal type, stenosed true lumen type and ulcer-like projection type. The incidence of normal type was estimated to be 48.4%, whereas stenosed true lumen type was 24.2% and ulcer-like projection was 27.7%. The present study concluded that thrombosed type is not rare in acute aortic dissection and contrast-enhanced CT as well as pre-contrast CT, is of great value in diagnosing thrombosed type. 'Hyperdense crescent sign' in pre-contrast CT is characteristic of intramural hematoma. (author)

  3. Acute aortic dissection: be aware of misdiagnosis

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    Asteri Theodora

    2009-02-01

    Full Text Available Abstract Background Acute aortic dissection (AAD is a life-threatening condition requiring immediate assessment and therapy. A patient suffering from AAD often presents with an insignificant or irrelevant medical history, giving rise to possible misdiagnosis. The aim of this retrospective study is to address the problem of misdiagnosing AD and the different imaging studies used. Methods From January 2000 to December 2004, 49 patients (41 men and 8 women, aged from 18–75 years old presented to the Emergency Department of our hospital for different reasons and finally diagnosed with AAD. Fifteen of those patients suffered from arterial hypertension, one from giant cell arteritis and another patient from Marfan's syndrome. The diagnosis of AAD was made by chest X-ray, contrast enhanced computed tomography (CT, transthoracic echocardiography (TTE and coronary angiography. Results Initial misdiagnosis occurred in fifteen patients (31% later found to be suffering from AAD. The misdiagnosis was myocardial infarction in 12 patients and cerebral infarction in another three patients. Conclusion Aortic dissection may present with a variety of clinical manifestations, like syncope, chest pain, anuria, pulse deficits, abdominal pain, back pain, or acute congestive heart failure. Nearly a third of the patients found to be suffering from AD, were initially otherwise diagnosed. Key in the management of acute aortic dissection is to maintain a high level of suspicion for this diagnosis.

  4. An alternative surgical approach for the combined treatment of pectus excavatum and acute aortic dissection type-A in Marfan syndrome.

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    Schwill, Simon; Kallenbach, Klaus; Beller, Carsten J; Karck, Matthias

    2011-04-01

    Acute aortic dissection type-A (AADA) is a life-threatening condition especially in patients with Marfan syndrome (MFS) simultaneously suffering from severe pectus excavatum (PE). We report on emergency surgery for combined treatment of PE and AADA in a patient with MFS using an alternative approach. It leads to excellent exposure of the dislocated heart and great vessels enabling Bentall procedure followed by funnel chest repair with modified technique of Adkins and Blades. We achieved favorable functional and cosmetic results. Therefore, we conclude the surgical approach presented is feasible for standard treatment of AADA and consecutive repair of PE.

  5. Aortic and Mitral Calcification Is Marker of Significant Carotid and Limb Atherosclerosis in Patients with First Acute Coronary Syndrome.

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    Sannino, Anna; Losi, Maria-Angela; Giugliano, Giuseppe; Canciello, Grazia; Toscano, Evelina; Giamundo, Alessandra; Scudiero, Fernando; Brevetti, Linda; Scudiero, Laura; Prastaro, Maria; Perrino, Cinzia; Perrone-Filardi, Pasquale; Galderisi, Maurizio; Trimarco, Bruno; Esposito, Giovanni

    2015-12-01

    Atherosclerosis is a systemic disease and coronary artery disease is frequently associated with peripheral artery disease. As aortic and mitral valvular calcification (VC) share some etiopathogenetic mechanisms with atherosclerosis, we analyzed the risk profile and the echocardiographic characteristics of patients admitted for first acute coronary syndrome (ACS) to investigate whether the presence of VC could be a marker of asymptomatic hemodynamically significant peripheral atherosclerosis. A total of 151 patients admitted for ACS without previous history of cardiovascular disease were consecutively enrolled. The presence of VC was identified by echocardiography; a carotid stenosis ≥50% by ultrasound identified carotid artery disease (CarAD); an ankle-brachial index ≤0.9 or ≥1.4 identified lower extremity artery disease (LEAD). Significant peripheral atherosclerosis was defined by the presence of CarAD and/or LEAD. Peripheral atherosclerosis was diagnosed in 82 (54.3%) patients; isolated CarAD in 24, isolated LEAD in 20, both diseases in 38 patients. VC was present in 103 (68.2%) patients. By multivariate analysis, age (OR = 1.059, 95% CI 1.007-1.113, P = 0.025), diabetes mellitus (OR = 5.068, 95% CI 1.480-17.351, P = 0.010), VC (OR = 7.422, 95% CI 2.421-22.880, P < 0.001), and multivessel CAD (OR = 3.317, 95% CI 1.281-8.586, P = 0.013) were the only independent predictors of having peripheral atherosclerosis. C-statistic for VC was not inferior to that obtained by age (0.728, 95% CI 0.649-0.797 vs. 0.800, 95% CI 0.727-0.861, P = 0.101) and to that obtained by the combination of multivessel CAD with diabetes (0.750; 95% CI 0.673-0.817, P = 0.635), and, furthermore, it was higher than that obtained by diabetes alone (0.620, 95% CI 0.538-0.698, P = 0.036). Ruling out the presence of significant peripheral atherosclerosis should be routinely considered in patients with ACS showing VC at echocardiography. © 2015, Wiley Periodicals, Inc.

  6. Surgery for acute Type I aortic dissection without resection of supra-aortic entry sites leads to unfavourable aortic remodelling.

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    Heo, Woon; Song, Suk-Won; Lee, Kwang-Hun; Lee, Shin-Young; Kim, Tae-Hoon; Baek, Min-Young; Yoo, Kyung-Jong

    2018-01-29

    This study aimed to evaluate the impact of remnant re-entries in arch branches on postoperative change in the aortic arch and descending aortic diameters and the rate of major adverse aortic events. Between January 2010 and December 2016, 249 patients underwent surgery for acute Type I aortic dissection. Patients who underwent total arch replacement, had Marfan syndrome or had intramural haematoma were excluded. Seventy-two patients with predischarge and follow-up computed tomography scans were enrolled. Patients with and without re-entries in the arch branches after surgery were assigned to the supra-aortic entry (SAE, n = 21) and no supra-aortic entry (n = 51) groups, respectively. Diameters were measured at 7 levels: the innominate artery, left common carotid artery, left subclavian artery, 20 mm distal to the left subclavian artery, pulmonary artery bifurcation, coeliac axis and maximal diameter of the descending thoracic aorta. Growth rates at the levels of the pulmonary artery bifurcation and 20 mm distal to the left subclavian artery were significantly higher in the SAE group than in the no supra-aortic entry group. The rate of freedom from major adverse aortic events (annual growth >5 mm or maximal diameter of the descending thoracic aorta >50 mm) at 5 years was significantly higher in the no supra-aortic entry group than in the SAE group. Remnant SAE leads to unfavourable aortic remodelling after acute Type I aortic dissection repair. © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  7. Anaesthesia management of acute aortic dissection type B in Marfan ...

    African Journals Online (AJOL)

    Pregnancy in women with Marfan syndrome (MFS) is linked to approximately a 4.4% risk of acute aortic dissection (AAD). The natural history of pregnancy and the ability to deliver a viable fetus depends on the interaction between the pace of changes in the cardiovascular system and the advancement of pregnancy.

  8. Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.

    Science.gov (United States)

    David, Tirone E; David, Carolyn M; Manlhiot, Cedric; Colman, Jack; Crean, Andrew M; Bradley, Timothy

    2015-09-29

    In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  9. RELEVANCIA DEL DIAGNÓSTICO DIFERENCIAL ENTRE EL SÍNDROME AÓRTICO AGUDO Y EL SÍNDROME CORONARIO AGUDO EN PACIENTES CON DOLOR TORÁCICO Y CRISIS HIPERTENSIVA: REVISIÓN A PROPÓSITO DE 2 CASOS / Relevance of the differential diagnosis between acute aortic syndrome and acute coronary syndrome in patients with thoracic pain and hypertensive crisis: review on 2 case reports

    Directory of Open Access Journals (Sweden)

    Borja Simó Sánchez

    2011-11-01

    Full Text Available Acute aortic syndrome is a pathological process with low incidence compared with acute coronary syndrome, although with a worse prognosis in the short term, which is why its early diagnosis and urgent treatment are essential to the favorable evolution of thepatient. Electrocardiographic changes suggestive of myocardial ischemia, with acute evolution, are rare in acute aortic syndrome. Even in the presence of a suggestive thoracic pain and high levels of blood pressure, an adequate differential diagnosis is of utmost importance, since the administration of anticoagulation and antiplatelet therapy in high doses can have a dramatic impact on patient outcome. Two cases are presented in which electrocardiographic changes determined the action to take in patients with acute aortic syndrome.

  10. [Metabolic syndrome and aortic stiffness].

    Science.gov (United States)

    Simková, A; Bulas, J; Murín, J; Kozlíková, K; Janiga, I

    2010-09-01

    The metabolic syndrome (MS) is a cluster of risk factors that move the patient into higher level of risk category of cardiovascular disease and the probability of type 2 diabetes mellitus manifestation. Definition of MS is s based on the presence of selected risk factors as: abdominal obesity (lager waist circumpherence), atherogenic dyslipidemia (low value of HDL-cholesterol and increased level of triglycerides), increased fasting blood glucose (or type 2 DM diagnosis), higher blood pressure or antihypertensive therapy. In 2009 there were created harmonizing criteria for MS definition; the condition for assignment of MS is the presence of any 3 criteria of 5 mentioned above. The underlying disorder of MS is an insulin resistance or prediabetes. The patients with MS more frequently have subclinical (preclinical) target organ disease (TOD) which is the early sings of atherosclerosis. Increased aortic stiffness is one of the preclinical diseases and is defined by pathologically increased carotidofemoral pulse wave velocity in aorta (PWV Ao). With the aim to assess the influence of MS on aortic stiffness we examined the group of women with arterial hypertension and MS and compare them with the group of women without MS. The aortic stiffness was examined by Arteriograph--Tensiomed, the equipment working on the oscillometric principle in detection of pulsations of brachial artery. This method determines the global aortic stiffness based on the analysis of the shape of pulse curve of brachial artery. From the cohort of 49 pts 31 had MS, the subgroups did not differ in age or blood pressure level. The mean number of risk factors per person in MS was 3.7 comparing with 1.7 in those without MS. In the MS group there was more frequently abdominal obesity present (87% vs 44%), increased fasting blood glucose (81% vs 22%) and low HDL-cholesterol level. The pulse wave velocity in aorta, PWV Ao, was significantly higher in patients with MS (mean value 10,19 m/s vs 8,96 m

  11. [Outcomes of acute type A aortic dissection repairs in Iceland].

    Science.gov (United States)

    Geirsson, Arnar; Melvinsdottir, Inga Hlif; Arnorsson, Thorarinn; Myrdal, Gunnar; Gudbjartsson, Tomas

    2016-02-01

    Acute type A aortic dissection is a life-threatening disease associated with significant morbidity and mortality. Treatment is challenging and requires emergency surgery. This study presents for the first time the short- and long-term outcome of acute type A aortic dissection repairs in Iceland. A retrospective review of 45 patients (mean age 60.7 ± 13.9 years, 68.9% male) treated for type A aortic dissection at Landspitali University Hospital between 1992 and 2014. Data was gathered from medical records about known risk factors, presenting symptoms, type of procedure, complications and operative mortality. Out of 45 operations the majority (73.3%) was performed in the second half of the study period. Nearly all patients presented with chest pain and 46.7% were in shock on arrival. Malperfusion syndrome was apparent in 26.7% of cases. A variety of operative methods were used, including hypothermic circulatory arrest in 31.1% of the cases and one-third of patients needed aortic root replacement. Reoperation rate for postoperative bleeding was 29.3% and perioperative stroke occurred in 14.6% of patients. The 30-day mortality rate was 22.2% (10 patients) and 5- and 10-year survival was 71.4 ± 8.2% and 65.4 ± 9.4%, respectively. The short-term outcomes of surgical repair for acute type A aortic dissection in Iceland is comparable to neighbouring countries, including 30-day mortality and long-term survival. Complications, however, are common, especially reoperations for bleeding. 1Department of Cardiothoracic Surgery, Landspitali University Hospital, 2Faculty of Medicine, University of Iceland. Aortic dissection type A, aortic aneurysm, open heart surgery, complications, operative mortality, survival. Correspondence: Arnar Geirsson, arnarge@landspitali.is.

  12. Acute Aortic Arch Perforation During Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis and a Gothic Aortic Arch

    DEFF Research Database (Denmark)

    Millan-Iturbe, Oscar; Sawaya, Fadi J.; Bieliauskas, Gintautas

    2017-01-01

    AS because of its unique anatomic features. This case report describes an acute aortic perforation during delivery of a transcatheter heart valve to treat a severe bicuspid AS with a “gothic aortic arch”; more careful evaluation of the preprocedural multislice computed tomographic scan would have unveiled...

  13. CT diagnosis of acute aortic dissection

    International Nuclear Information System (INIS)

    Ogawa, Noriko; Kobayashi, Takeshi

    1989-01-01

    Sixteen (47.5%) of 35 patients with acute aortic dissection showed a non-opacified crescent in the aorta on an initial contrast CT. Seven of these 16 patients underwent cineangiography soon after the initial CT, and in all 7 patients, neither an intimal tear nor an intimal flap was obtained. All but one of above 16 patients were followed by CT. Mean duration of follow-up was 9.6 months. In 10 of 15 patients with non-opacified false lumen, the false lumen remained non-opacified until the last examination. Moreover, in 6 of these 10 patients, the false lumen shrunk, and in the other 3, it disappeared completely on follow-up CT. On the other hand, in remaining 5 of these 15 patients who were initially diagnosed to have non-opacified false lumen, the false lumen became opacified and enlarged in size on follow-up CT performed in the first 14 weeks. Moreover, in 4 of these 5 patients, the false lumen became opacified in the only first 6 weeks. No matter how intensive care should be paid at least for the first 6 weeks, it seems that patients with aortic dissection which have non-opacified false lumen had good prognosis in comparison to patients with ordinary aortic dissections which have opacified false lumen. We believe aortic dissection with non-opacified false lumen may consist of two type of aortic dissection, one has no intimal tear, the other has some intimal tears and a thrombosed false lumen. In conclusion, CT is the most useful modality in diagnosing acute aortic dissection. The reasons are the incidence of acute aortic dissection with non-opacified false lumen was high, patients with non-opacified false lumen had good prognosis, and it was difficult to diagnose aortic dissection with non-opacified false lumen by conventional cineangiography and/or DSA. (author)

  14. Acute Abdominal Mobile Aortic Thrombus Post Chemotherapy: Two Cases Reports

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Woo Chul; Kim, Jun Ho; Lee, Kyung Hee; Cho, Soon Gu; Jeon, Yong Sun [Dept. of Radiology, Inha University School of Medicine, Incheon (Korea, Republic of)

    2011-08-15

    An acute abdominal mobile aortic thrombus is a very unusual finding, and this is a potential source of arterial embolism. Mobile aortic thrombus mostly occurs in a diffuse atherosclerotic and aneurysmal aorta. Chemotherapy agents have been documented as a possible rare cause of acute arterial thrombus. We report acute abdominal mobile aortic thrombus post-chemotherapy for pancreatic and stomach cancer.

  15. Aortic events in a nationwide Marfan syndrome cohort

    DEFF Research Database (Denmark)

    Groth, Kristian A; Krag, Kirstine Stochholm; Hove, Hanne

    2017-01-01

    BACKGROUND: Marfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan...... syndrome cohort. METHOD: The nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome...

  16. Acute Aortic Arch Perforation During Transcatheter Aortic Valve Replacement in Bicuspid Aortic Stenosis and a Gothic Aortic Arch.

    Science.gov (United States)

    Millan-Iturbe, Oscar; Sawaya, Fadi J; Bieliauskas, Gintautas; Chow, Danny H F; De Backer, Ole; Søndergaard, Lars

    2017-09-01

    Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for high/intermediate-risk patients with severe symptomatic aortic stenosis (AS). Although TAVR is used to treat bicuspid severe AS, the large randomized trials typically excluded bicuspid AS because of its unique anatomic features. This case report describes an acute aortic perforation during delivery of a transcatheter heart valve to treat a severe bicuspid AS with a "gothic aortic arch"; more careful evaluation of the preprocedural multislice computed tomographic scan would have unveiled a sharply angulated aortic arch. This life-threatening complication was successfully treated by thoracic endovascular aortic repair. Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  17. Aortic intramural hematoma : assessment of clinical and radiological features in comparison to acute aortic dissection

    International Nuclear Information System (INIS)

    Yoon, Kwon Ha; Hwang, Jae Cheol; Lee, Jin Seong; Kang, Duk Hyun; Song, Jae Kwan; Song, Koun Sik; Lim, Tae Hwan

    1996-01-01

    To compare the clinical and radiological features of aortic intramural hematoma(IMH) to those of acute aortic dissection(AD). We analyzed the clinical and radiological features of 12 patients with aortic IMH and 43 patients with acute AD. In aortic IMH, the diagnoses were made by means of both CT and transesophageal echocardiography(TEE) and included two surgically proven cases. In acute AD, the diagnoses were made by means of CT and TEE and included 21 surgically proven cases. We compared patients ages, etiologies, the extent of the disease, the presence or absence of aortic branch involvement, complications, and outcomes. Aortic IMH tended to develop in older patients (67.8±7.9 vs. 50.4±13.4, P .05). In aortic IMH, there was no involvement of aortic branches, whereas in acute AD, 14(33%) patients showed involvement of one or more aortic branches. Complications of aortic IMH included pericardial effusion (n=2) and pleural effusion (n=4);in acute AD, pericardial effusion (n=7), pleural effusion (n=4), aortic insufficiency (n=8), cerebral infarction (n=3), renal infarction (n=4) and spinal infarction (n=1) were seen. There was one (8%) death due to aortic IMH and ten (23%) deaths due to acute AD (p<.01). Aortic IMH is characterized by its occurrence in older patients with hypertension, a less frequent incidence of complications, and a more favorable outcome than acute AD

  18. Cauda equina syndrome: an uncommon symptom of aortic diseases.

    Science.gov (United States)

    He, Fuliang; Xing, Tong; Yu, Fang; Li, Hongchuan; Fang, Xiutong; Song, Hongxing

    2015-01-01

    In order to help diagnose and deal with the fetal aortic diseases in time, we retrospectively reviewed 8 patients who presented with cauda equina syndrome (CES) but actually suffered from low spinal nerve ischemia due to aortic diseases. 8 patients were initially diagnosed as CES. 7 patients were confirmed with aortic diseases. 1 patient was confirmed with aortic saddle embolism post emergent laminectomy. Relief of CES symptoms was evaluated during preoperation and follow-up period. 1 patient was diagnosed as aortic dissection and 5 patients as AAA. These 6 patients underwent endovascular aortic repair (EVAR). The CES was relieved in 5-10 d post procedure. The 7th patient was diagnosed with acute abdominal aortic occlusion and then underwent catheter directed thrombolysis with recombinant tissue plasminogen activator (rTPA) for 20 h and CES disappeared. The JOA scores of the 7 patients were recovered from preoperative 15.14±1.21 to 21.00±2.16 within 5-10 d (P<0.01), and evaluated to be 24.12±1.34, 25.88±1.21 and 26.29±1.11 at 3 m-, 6 m- and 12 m-follow-up point, respectively. The 8th patient was initially diagnosed as lumbar spinal stenosis and lumbar disc herniation. The patient underwent emergent vertebral canal decompression and presented with serious CES symptoms. CTA confirmed that the patient had been suffered from aortic saddle embolism (ASE). CES caused by abdominal aortic diseases is a special event with fetal consequences if it is not recognized and treated promptly. Orthopedists and neurosurgeons should pay attentions particularly to this issue to preserve the cauda equina functions to their maximums.

  19. Acute type A aortic dissection in a patient with paraganglioma.

    Science.gov (United States)

    Dos Santos Borrego, Andreia; Carrilho Ferreira, Pedro; Pinto, Fausto J

    2017-10-01

    Acute aortic dissection is the most common acute aortic syndrome. It is more prevalent in males and in the elderly, and has a high mortality. Hypertension is the main risk factor. Diagnosis is based on clinical features, laboratory tests and imaging exams. Treatment is usually surgical, although in some cases an endovascular approach is an alternative. Paraganglioma is an uncommon neuroendocrine tumor. Most produce catecholamines, and so usually manifest with hypertensive crisis, palpitations, headache and sweating. This tumor is diagnosed by measurement of plasma or urinary catecholamines and by computed tomography, magnetic resonance imaging and 123 I-metaiodobenzylguanidine (MIBG) scintigraphy. Surgery is the only potentially curative treatment. Copyright © 2017 Sociedade Portuguesa de Cardiologia. All rights reserved.

  20. Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.

    Science.gov (United States)

    David, Tirone E; Armstrong, Sue; Maganti, Manjula; Colman, Jack; Bradley, Timothy J

    2009-10-01

    The appropriateness of aortic valve-sparing operations in patients with Marfan syndrome has been questioned. This study examines the long-term results of these operations in patients with Marfan syndrome. From 1988 to 2006, 103 consecutive patients with Marfan syndrome (mean age, 37 +/- 12 years) and aortic root aneurysm had aortic valve-sparing operations. Emergency surgery was performed in 11 patients: 8 for acute type A aortic dissection and 3 for unexplained persistent chest pain. Fourteen patients also had mitral valve surgery. The technique of aortic valve reimplantation was used in 77 patients, and aortic root remodeling was used in 26 patients. Patients were followed prospectively and underwent annual echocardiographic studies. The mean follow-up was 7.3 +/- 4.2 years and 100% complete. There was 1 operative death and 5 late deaths. Four of the 6 deaths were due to complications of aortic dissections. The patients' survival at 15 years was 87.2% compared with 95.6% for the general population of Ontario matched for age and sex. Seven patients had important aortic insufficiency: 4 mild to moderate, 2 moderate, and 1 moderate to severe. Freedom from greater than mild aortic insufficiency at 15 years was 79.2%. Three patients, all after aortic root remodeling, had aortic valve replacement, 2 for aortic insufficiency and 1 for endocarditis. At the most recent follow-up, 97 patients were alive: 86 were in functional class I, and 11 were in functional class II. Aortic valve-sparing operations provided excellent clinical outcomes in this series of patients with Marfan syndrome. Postoperatively, complications of aortic dissections were the leading cause of death.

  1. Aortic dissection in patients with Marfan syndrome based on the IRAD data.

    Science.gov (United States)

    de Beaufort, Hector W L; Trimarchi, Santi; Korach, Amit; Di Eusanio, Marco; Gilon, Dan; Montgomery, Daniel G; Evangelista, Arturo; Braverman, Alan C; Chen, Edward P; Isselbacher, Eric M; Gleason, Thomas G; De Vincentiis, Carlo; Sundt, Thoralf M; Patel, Himanshu J; Eagle, Kim A

    2017-11-01

    Between January 1996 and May 2017, the International Registry on Acute Aortic Dissections has collected information on a total of 6,424 consecutive patients with acute aortic dissection, including 258 individuals with a diagnosis of Marfan syndrome. Patients with Marfan syndrome presented at a significantly younger age compared to patients without Marfan syndrome (38.2±13.2 vs . 63.0±14.0 years; PMarfan syndrome cohort, but no larger diameters more distally. The in-hospital mortality in type A dissection was not significantly different in patients with or without Marfan syndrome, despite the differences in age and comorbidities and the lower incidence of aortic rupture in the Marfan syndrome cohort. In contrast, the in-hospital mortality of Marfan syndrome patients with type B dissection appears to be lower than that of patients without Marfan syndrome. The Marfan syndrome cohort that was treated with open surgery for type B dissection seemed to do especially well, with a 0% mortality rate (n=27). Follow-up data for type A and B dissections combined show an estimated five-year survival rate of 80.1% and an estimated reintervention rate of 55.3% in patients with Marfan syndrome. Such a high rate of reinterventions highlights the need for careful surveillance and treatment for patients with Marfan syndrome surviving the acute phase of aortic dissection.

  2. Aortic root surgery in Marfan syndrome.

    Science.gov (United States)

    Sheick-Yousif, Basheer; Sheinfield, Ami; Tager, Salis; Ghosh, Probal; Priesman, Sergey; Smolinsky, Aram K; Raanani, Ehud

    2008-03-01

    As the shortcomings of the Bentall operation and its variants in the Marfan syndrome have become apparent, the recent cusp-sparing techniques (remodeling or reimplantation) bear promise of better mid-term and long-term outcomes. To examine the results of aortic root surgery in patients with Marfan syndrome. During the period March 1994 to September 2007, 220 patients underwent aortic valve-sparing surgery; 20 were Marfan patients (group 1) who were compared with another 20 Marfan patients undergoing composite aortic root replacement (group 2). Fourteen patients had aortic dissection and 26 had thoracic aortic aneurysm. There were 31 males and 9 females with a mean age of 37.9 +/- 13.8 years. In group 1, reimplantation was used in 13 patients, remodeling in 4, and aortic valve repair with sinotubular junction replacement in 3. In group 2, a mechanical valve conduit was used. Mean logistic Euroscore was 12.27 +/- 14.6% for the whole group, five of whom were emergent cases Group 2 had more previous cardiac procedures compared to group 1 (9 vs. 2, P = 0.03) and shorter cross-clamp time (122 +/- 27.1 vs. 153.9 +/- 23.7 minutes, P = 0.0004). Overall mortality was 10%. Early mortality was 10% in group 2 and 5% in group 1 (NS). Mean follow-up time was 25 months for group 2 and 53 months for group 1. Three patients were reoperated; all had undergone the remodeling. Five year freedom from reoperation and death was 86% and 90% in group 2 and 70% and 95% in group 1 (P = 0.6, P = 0.6), respectively. Late survival of patients with Marfan syndrome was similar in both groups. Root reconstruction tends towards a higher incidence of late reoperations if the remodeling technique is used. We now prefer to use the reimplantation technique.

  3. Recommendations for accurate CT diagnosis of suspected acute aortic syndrome (AAS)--on behalf of the British Society of Cardiovascular Imaging (BSCI)/British Society of Cardiovascular CT (BSCCT).

    Science.gov (United States)

    Vardhanabhuti, Varut; Nicol, Edward; Morgan-Hughes, Gareth; Roobottom, Carl A; Roditi, Giles; Hamilton, Mark C K; Bull, Russell K; Pugliese, Franchesca; Williams, Michelle C; Stirrup, James; Padley, Simon; Taylor, Andrew; Davies, L Ceri; Bury, Roger; Harden, Stephen

    2016-01-01

    Accurate and timely assessment of suspected acute aortic syndrome is crucial in this life-threatening condition. Imaging with CT plays a central role in the diagnosis to allow expedited management. Diagnosis can be made using locally available expertise with optimized scanning parameters, making full use of recent advances in CT technology. Each imaging centre must optimize their protocols to allow accurate diagnosis, to optimize radiation dose and in particular to reduce the risk of false-positive diagnosis that may simulate disease. This document outlines the principles for the acquisition of motion-free imaging of the aorta in this context.

  4. [Aortic valve-sparing root reconstruction in Marfan syndrome].

    Science.gov (United States)

    Ogino, H; Sasaki, H; Hanafusa, Y; Hirata, M; Numata, S; Ando, M; Yagihara, T; Kitamura, S

    2002-07-01

    The outcome of aortic valve-sparing root reconstruction in Marfan syndrome was reviewed. Thirteen patients with Marfan syndrome underwent aortic valve-sparing root reconstruction for annuloaortic ectasia or aortic root dissection between 1994 and 1999. The grade of preoperative aortic regurgitation was I in 4, II in 2, III in 5, IV in 2 patients. The procedures of aortic valve-sparing were reimplantation in 7 and remodeling in 5 patients. There was no hospital and late death. Recurrence of aortic regurgitation greater than moderate grade developed in 1 patient immediately after the surgery and in the other 4 patients in the late stage. One patient of them required aortic valve replacement for it. Aortic valve-sparing root reconstruction is applicable in Marfan patients, although the indication should be cautious. Close observation is needed for recurrence of aortic regurgitation.

  5. Outcomes after treatment of acute aortic occlusion.

    Science.gov (United States)

    de Varona Frolov, Serguei R; Acosta Silva, Marcela P; Volo Pérez, Guido; Fiuza Pérez, Maria D

    2015-11-01

    Acute aortic occlusion (AAO) is a rare disease with high morbidity and mortality. The aim of this study was to describe the results of surgical treatment of acute aortic occlusion and risk factors for mortality. Retrospective review of the clinical history of 29 patients diagnosed and operated on for AAO during 28 years. The following variables were analysed: age, sex, tabaco use, diabetes, chronic renal insufficiency, chronic heart failure, atrial fibrillation, arterial hypertension, symptoms, diagnosis and treatment, 30-day mortality and long-term survival. A univariant analysis was performed of variables related to mortality. Twenty-nine patients were included (18 male) with a mean age of 66,2 years. The aetiology was: embolism (EM) in 11 cases and Thrombosis (TR) in 18 cases. The surgical procedures performed included bilateral transfemoral thrombectomy (14 cases), aorto-bifemoral by-pass (8 cases), axilo uni/bifemoral by-pass (5 cases) and aortoiliac and renal tromboendarterectomy (2 cases). Morbidity included: renal failure (14 cases), mesenteric ischemia (4 cases), cardiac complications (7 cases), respiratory complications (5 cases) and loss of extremity (2 cases). The in-hospital mortality was 21% (EM 0%, TR 21%). The estimated survival at 1.3 and 5 years was 60, 50 and 44% respectively. Age (p=0.032), arterial hypertension (p=0.039) and aetiology of the AAO (p=0.039) were related to mortality. Acute aortic occlusion is a medical emergency with high mortality rates. Acute renal failure is the most common postoperative complication. Copyright © 2012 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Diagnostic imaging of acute aortic dissection; Evaluation of thrombosed type aortic dissection by CT and angiography

    Energy Technology Data Exchange (ETDEWEB)

    Ohya, Tohru; Kumazaki, Tatsuo (Nippon Medical School, Tokyo (Japan))

    1991-01-01

    One hundred and nineteen patients with aortic dissection who underwent diagnostic imaging were reviewed and angiographic findings as well as those of CT were analysed. Thirty eight cases (43.1%) had non-contrast opacified false lumen, the type of which we call 'thrombosed type aortic dissection'. A comparative study of the thrombosed type with the patent type of false lumens was made particularly from the stand point of the characteristic diagnostic imagings (CT and angiography). At the same time, the pitfalls of these imagings in thrombosed type aortic dissection were studied. At the onset the average age of thrombosed type was 62.3 years old, while that of the patent type was 57.3. A statistical significance between the two groups was p<0.05. Thrombosed type in all cases was caused by atherosclerosis, whereas patent type was caused by the Marfan's syndrome in 11 cases. Other clinical findings, such as initial symptoms and blood pressure revealed no significant differences between the two groups. Pre-contrast CT in acute thrombosed type aortic dissection showed 'hyperdense crescent sign' in 89.4%. However, in 3 cases with thrombosed type in which the pre-contrast CT showed 'hyperdense crescent sign' contrast-enhanced CT detected no clear evidence of aortic dissection in the same site. This was due to obscurity induced by contrast medium. Angiographic findings of thrombosed type were classified into 3 groups: normal type, stenosed true lumen type and ulcer-like projection type. The incidence of normal type was estimated to be 48.4%, whereas stenosed true lumen type was 24.2% and ulcer-like projection was 27.7%. The present study concluded that thrombosed type is not rare in acute aortic dissection and contrast-enhanced CT as well as pre-contrast CT, is of great value in diagnosing thrombosed type. 'Hyperdense crescent sign' in pre-contrast CT is characteristic of intramural hematoma. (author).

  7. Online network of subspecialty aortic disease experts: Impact of "cloud" technology on management of acute aortic emergencies.

    Science.gov (United States)

    Schoenhagen, Paul; Roselli, Eric E; Harris, C Martin; Eagleton, Matthew; Menon, Venu

    2016-07-01

    For the management of acute aortic syndromes, regional treatment networks have been established to coordinate diagnosis and treatment between local emergency rooms and central specialized centers. Triage of acute aortic syndromes requires definitive imaging, resulting in complex data files. Modern information technology network structures, specifically "cloud" technology, coupled with mobile communication, increasingly support sharing of these data in a network of experts using mobile, online access and communication. Although this network is technically complex, the potential benefit of online sharing of data files between professionals at multiple locations within a treatment network appear obvious; however, clinical experience is limited, and further evaluation is needed. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  8. ACUTE COMPARTMENT SYNDROME

    African Journals Online (AJOL)

    muscle destruction, muscle fibrosis, contractures and permanent disability and at worst case scenario of amputation (3,4). As reported by Frink et al (3) on their study on acute compartment syndrome it can occur even when there is no fracture. Also general surgeons have reported acute compartment syndrome.

  9. Incidence of systemic inflammatory response syndrome after endovascular aortic repair

    DEFF Research Database (Denmark)

    De La Motte, L; Vogt, K; Jensen, Leif Panduro

    2011-01-01

    AIM: The aim of this study was to estimate the incidence of the post-implantation syndrome/systemic inflammatory response syndrome (SIRS) after endovascular aortic repair. METHODS: All patients, undergoing elective primary endovascular repair of an asymptomatic infrarenal abdominal aortic aneurysm...

  10. The risk for type B aortic dissection in Marfan syndrome.

    Science.gov (United States)

    den Hartog, Alexander W; Franken, Romy; Zwinderman, Aeilko H; Timmermans, Janneke; Scholte, Arthur J; van den Berg, Maarten P; de Waard, Vivian; Pals, Gerard; Mulder, Barbara J M; Groenink, Maarten

    2015-01-27

    Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome. The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome. Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome. Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome (mean age 36 ± 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery (hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter ≥27 mm (hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections (hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030). Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  11. Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome.

    Science.gov (United States)

    Rylski, Bartosz; Bavaria, Joseph E; Beyersdorf, Friedhelm; Branchetti, Emanuela; Desai, Nimesh D; Milewski, Rita K; Szeto, Wilson Y; Vallabhajosyula, Prashanth; Siepe, Matthias; Kari, Fabian A

    2014-04-01

    Data on outcomes after Stanford type A aortic dissection in patients with Marfan syndrome are limited. We investigated the primary surgery and long-term results in patients with Marfan syndrome who suffered aortic dissection. Among 1324 consecutive patients with aortic dissection type A, 74 with Marfan syndrome (58% men; median age, 37 years [first and third quartiles, 29 and 48 years]) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft, 30% supracoronary ascending replacement, 15% valve-sparing aortic root replacement; 12% total arch replacement; 3% in-hospital mortality) at 2 tertiary centers in the United States and Europe over the past 25 years. The rate of aortic reintervention with resternotomy was 24% (18 of 74) and of descending aorta (thoracic+abdominal) intervention was 30% (22 of 74) at a median follow-up of 8.4 years (first and third quartiles, 2.2 and 12.7 years). Freedom from need for aortic root reoperation in patients who underwent primarily a composite valved graft or valve-sparing aortic root replacement procedure was 95±3%, 88±5%, and 79±5% and in patients who underwent supracoronary ascending replacement was 83±9%, 60±13%, 20±16% at 5, 10, and 20 years. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement. Emergency surgery for type A dissection in patients with Marfan syndrome is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended because supracoronary ascending replacement is associated with a high need (>40%) for root reintervention.

  12. Aortic events in a nationwide Marfan syndrome cohort.

    Science.gov (United States)

    Groth, Kristian A; Stochholm, Kirstine; Hove, Hanne; Kyhl, Kasper; Gregersen, Pernille A; Vejlstrup, Niels; Østergaard, John R; Gravholt, Claus H; Andersen, Niels H

    2017-02-01

    Marfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan syndrome cohort. The nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome diagnosis prior or after the first aortic event. From the total cohort of 412 patients, 150 (36.4 %) had an aortic event. Fifty percent were event free at age 49.6. Eighty patients (53.3 %) had prophylactic surgery and seventy patients (46.7 %) a dissection. The yearly event rate was 0.02 events/year/patient in the period 1994-2014. Male patients had a significant higher risk of an aortic event at a younger age with a hazard ratio of 1.75 (CI 1.26-2.42, p = 0.001) compared with women. Fifty-three patients (12.9 %) were diagnosed with MFS after their first aortic event which primarily was aortic dissection [n = 44 (83.0 %)]. More than a third of MFS patients experienced an aortic event and male patients had significantly more aortic events than females. More than half of the total number of dissections was in patients undiagnosed with MFS at the time of their event. This emphasizes that diagnosing MFS is lifesaving and improves mortality risk by reducing the risk of aorta dissection.

  13. Utility of hand-held devices in diagnosis and triage of cardiovascular emergencies. Observations during implementation of a PACS-based system in an acute aortic syndrome (AAS) network.

    Science.gov (United States)

    Matar, Ralph; Renapurkar, Rahul; Obuchowski, Nancy; Menon, Venu; Piraino, David; Schoenhagen, Paul

    2015-01-01

    Prompt diagnosis and early referral to specialized centers is critical for patients presenting with cardiovascular emergencies, including acute aortic syndromes (AAS). Prior data has suggested that mobile access to imaging studies with hand-held devices can accelerate diagnosis and management. We conducted a study to determine the diagnostic accuracy of a hand-held device compared to conventional dedicated work-stations for diagnosing a spectrum of cardiovascular emergencies, predominantly acute aortic pathology. This study included 104 cases who underwent computed tomography (CT)-scan during "on-call'' hours between January, 2013 and August, 2014 for suspected AAS. Assessment was performed on a hand-held device independently by two readers using an iPhone5 connected via secure connection to web-based PACS servers. The subsequent interpretation from a dedicated workstation coupled with the diagnosis at the time of discharge was used as the reference standard for determining the presence or absence of an acute abnormality. Sensitivity and Specificity were calculated on a per patient basis. Readers' sensitivity and specificity using the hand-held device to diagnose acute chest pathology were calculated. Hand-held device evaluation was determined to have a sensitivity of 85.2% and a specificity of 98.6% by reader A and a sensitivity of 96.3% and specificity of 100% by reader B. Of 103 cases interpreted by both readers, the readers agreed about the diagnosis in 98 cases (95.1%). This study demonstrates that hand-held devices can be a potential useful tool to assist in diagnosis and triage of patients presenting with cardiovascular emergencies. Further studies are needed to assess the impact of screen size and resolution. Copyright © 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

  14. Consumption coagulopathy in acute aortic dissection: principles of management.

    Science.gov (United States)

    Liu, Yuyong; Han, Lu; Li, Jiachen; Gong, Ming; Zhang, Hongjia; Guan, Xinliang

    2017-06-12

    The effect of acute aortic dissection itself on coagulopathy or surgery-related coagulopathy has never been specifically studied. The aim of the present study was to perioperatively describe consumption coagulopathy in patients with acute aortic dissection. Sixty-six patients with acute type A aortic dissection were enrolled in this study from January 2015 to September 2016. Thirty-six patients with thoracic aortic aneurysms were used as a control group during the same period. Consumption coagulopathy was evaluated using standard laboratory tests, enzyme-linked immunosorbent assay and thromboelastograghy at five perioperative time-points. A significant reduction in clotting factors and fibrinogen was observed at the onset of acute aortic dissection. Enzyme-linked immunosorbent assay and thromboelastograghy also revealed a persistent systemic activation of the coagulation system and the consumption of clotting factors. In contrast, although platelet counts were consistently low, we did not find that platelet function was more impaired in the acute aortic dissection group than the control group. After surgery, clotting factors and fibrinogen were more impaired than platelet function. Thus, we proposed that hemostatic therapy should focus on the rapid and sufficient supplementation of clotting factors and fibrinogen to improve consumption coagulopathy in patients with acute aortic dissection.

  15. Rupture of an Abdominal Aortic Aneurysm in a Young Man with Marfan Syndrome.

    Science.gov (United States)

    Pedersen, Maria Weinkouff; Huynh, Khiem Dinh; Baandrup, Ulrik Thorngren; Nielsen, Dorte Guldbrand; Andersen, Niels Holmark

    2018-04-01

    Abdominal aortic aneurysms (AAAs) are very rare in Marfan syndrome. We present a case with a young nonsmoking and normotensive male with Marfan syndrome, who developed an infrarenal AAA that presented with rupture to the retroperitoneal cavity causing life-threatening bleeding shock. The patient had acute aortic surgery and survived. Five months before this incident, the patient had uneventful elective aortic root replacement (ad modum David) due to an enlarged aortic root. At that time, his abdominal aorta was assessed with a routine ultrasound scan that showed a normal-sized abdominal aorta. This documents that the aneurysm had evolved very rapidly despite young age and absence of risk factors. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Aortic root surgery in Marfan syndrome: Comparison of aortic valve-sparing reimplantation versus composite grafting.

    Science.gov (United States)

    Karck, Matthias; Kallenbach, Klaus; Hagl, Christian; Rhein, Christine; Leyh, Rainer; Haverich, Axel

    2004-02-01

    The objective of this study was to compare the results of aortic valve-sparing reimplantation and aortic root replacement with mechanical valve conduits in patients with Marfan syndrome undergoing operation for aortic root aneurysms. Patients and methods Between March 1979 and April 2002, 119 patients with clinical evidence of Marfan syndrome underwent composite graft replacement with mechanical valve conduits (n = 74) or aortic valve-sparing reimplantation according to David (n = 45). The underlying causes were aortic dissection type A (43 patients) and aneurysms (76 patients). Patients undergoing aortic valve reimplantation were younger compared with patients undergoing composite grafting (28 vs 35 years, P =.002) and had longer intraoperative aortic crossclamp times (125 vs 78 minutes, P valve reimplantation (P =.15). Mean follow-up was 30 months for patients undergoing aortic valve reimplantation and 114 months for patients undergoing composite grafting. Freedom from reoperation and death after 5 years postoperatively was 92% and 89% in patients undergoing composite grafting and 84% and 96% in patients undergoing aortic valve reimplantation (P =.31; P =.54), respectively. Thromboembolic complications or late postoperative bleeding occurred in 17 patients undergoing composite grafting, and an early postoperative event occurred in 1 patient undergoing aortic valve reimplantation. The results of aortic valve reimplantation and composite grafting of the aortic valve and ascending aorta with mechanical valve conduits are similar with regard to early and mid-term postoperative mortality and to the incidence of late reoperations in patients with Marfan syndrome. The low risk of thromboembolic or bleeding complications favors aortic valve reimplantation in these patients.

  17. Combined Repair of Ascending Aortic Pseudoaneurysm and Abdominal Aortic Aneurysm in a Patient with Marfan Syndrome

    OpenAIRE

    Kokotsakis, John N.; Lioulias, Achilleas G.; Foroulis, Christophoros N.; Skouteli, Eleni Anna T.; Milonakis, Michael K.; Bastounis, Elias A.; Boulafendis, Dimitrios G.

    2003-01-01

    Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic ane...

  18. The systemic inflammatory response syndrome predicts short-term outcome after transapical transcatheter aortic valve implantation

    NARCIS (Netherlands)

    Rettig, Thijs C D; Rigter, Sander; Nijenhuis, Vincent J.; Van Kuijk, Jan Peter; Ten Berg, Jurriën M.; Heijmen, Robin H.; Van De Garde, Ewoudt M W; Noordzij, Peter G.

    2015-01-01

    Objective Despite the minimally invasive nature of transcatheter aortic valve implantation (TAVI), the incidence of acute kidney injury (AKI) and mortality is of major concern. Several studies showed that outcome was influenced by the systemic inflammatory response syndrome (SIRS) in patients

  19. Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome

    NARCIS (Netherlands)

    Nollen, Gijs J.; Groenink, Maarten; Tijssen, Jan G. P.; van der Wall, Ernst E.; Mulder, Barbara J. M.

    2004-01-01

    Aim Patients with Marfan syndrome may develop dissection due to progressive dilatation in the entire aorta, which is not always predictable by mere anatomic assessment of the aortic diameter, especially of the descending aorta. The aim of this study was to identify the predictive value of aortic

  20. Acute heart failure syndrome

    African Journals Online (AJOL)

    Heart failure can be defined as a clinical syndrome in which a structural or functional cardiac abnormality impairs the capacity of the ventricle to fill or eject enough blood for the requirements of the body. Acute heart failure syndrome represents a complex, heterogeneous set of clinical conditions, all with the common.

  1. Surgical treatment and thoracic endovascular aortic repair in type A aortic dissection in a pregnant patient with Marfan syndrome.

    Science.gov (United States)

    Sterner, Doerthe; Probst, Chris; Mellert, Friedrich; Schiller, Wolfgang

    2014-07-01

    We report an acute aortic dissection type Stanford A extending down to both iliac arteries affecting a 32-year-old woman suspected to have Marfan syndrome during week 37 of pregnancy. In a multidisciplinary approach, and emergency Cesarean section was performed followed by an abdominal hysterectomy and a valve-sparing aortic root replacement using a reimplantation technique. The aorta was replaced up to the hemi arch. Because of the high suspicion of visceral ischemia as confirmed ex juvantibus, an endovascular stent graft was implanted. Molecular testing revealed a frameshift mutation and confirmed the diagnosis of Marfan syndrome. Both the patient and her healthy child underwent an uneventful recovery. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Overview of current surgical strategies for aortic disease in patients with Marfan syndrome.

    Science.gov (United States)

    Miyahara, Shunsuke; Okita, Yutaka

    2016-09-01

    Marfan syndrome is a heritable, systemic disorder of the connective tissue with a high penetrance, named after Dr. Antoine Marfan. The most clinically important manifestations of this syndrome are cardiovascular pathologies which cause life-threatening events, such as acute aortic dissections, aortic rupture and regurgitation of the aortic valve or other artrioventricular valves leading to heart failure. These events play important roles in the life expectancy of patients with this disorder, especially prior to the development of effective surgical approaches for proximal ascending aortic disease. To prevent such catastrophic aortic events, a lower threshold has been recommended for prophylactic interventions on the aortic root. After prophylactic root replacement, disease in the aorta beyond the root and distal to the arch remains a cause for concern. Multiple surgeries are required throughout a patient's lifetime that can be problematic due to distal lesions complicated by dissection. Many controversies in surgical strategies remain, such as endovascular repair, to manage such complex cases. This review examines the trends in surgical strategies for the treatment of cardiovascular disease in patients with Marfan syndrome, and current perspectives in this field.

  3. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome.

    Science.gov (United States)

    Itagaki, Shinobu; Chikwe, Joanna P; Chiang, Yuting P; Egorova, Natalia N; Adams, David H

    2015-06-09

    Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data. This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome. In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years. The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  4. Aortic Involvement in Pediatric Marfan syndrome: A Review.

    Science.gov (United States)

    Ekhomu, Omonigho; Naheed, Zahra J

    2015-06-01

    Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

  5. Transcatheter Aortic Valve Replacement for Severe Aortic Regurgitation With Acute Refractory Cardiogenic Shock.

    Science.gov (United States)

    Achkouty, Guy; Amabile, Nicolas; Zannis, Konstantinos; Veugeois, Aurélie; Caussin, Christophe

    2018-03-01

    From January 2013 to January 2017, 686 consecutive patients were referred to our centre for transcatheter aortic valve replacement, including 5 subjects with severe aortic regurgitation and acute refractory cardiogenic shock. These patients were contraindicated for surgical treatment by the heart team because of high surgical risk (median logistic EuroSCORE: 74.6/Society of Thoracic Surgeons score: 37.9). The success rate of valve implantation was 100% through transfemoral access with self-expandable devices. The observed 30-day mortality rate was 20%. Hence, the transcatheter aortic valve replacement procedure might represent a successful and life-saving intervention for treatment of patients with severe aortic regurgitation who present with acute refractory cardiogenic shock. Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  6. Diagnostic and therapy of acute thoracic aortic diseases; Diagnostik und Therapie akuter Erkrankungen der thorakalen Aorta

    Energy Technology Data Exchange (ETDEWEB)

    Schotten, Sebastian; Pitton, Michael B. [Universitaetsmedizin Mainz Univ. (Germany). Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie

    2017-09-15

    Acute diseases of the thoracic aorta represent a relatively rare but life threatening spectrum of pathologies. The non-traumatic diseases are usually summarized by the term ''acute aortic syndrome''. A timely diagnosis and initiation of therapy are cornerstones for the patient outcome. CT has become the standard imaging procedure due do its widespread availability and excellent sensitivity. Furthermore, CT is able to discriminate the variants of acute aortic diseases and to detect the wide spectrum of complications. The volumetric CT dataset is also the basis for planning of interventional procedures. Open surgical repair still represents the standard of care for acute pathologies of the ascending aorta while endovascular therapy, due to minimally invasive character and good outcome, has replaced open surgery in most cases of complicated lesions of the descending aorta.

  7. Acute contained ruptured aortic aneurysm presenting as left vocal fold immobility.

    Science.gov (United States)

    Gnagi, Sharon H; Howard, Brittany E; Hoxworth, Joseph M; Lott, David G

    2015-01-01

    Objective. To recognize intrathoracic abnormalities, including expansion or rupture of aortic aneurysms, as a source of acute onset vocal fold immobility. Methods. A case report and review of the literature. Results. An 85-year-old female with prior history of an aortic aneurysm presented to a tertiary care facility with sudden onset hoarseness. On laryngoscopy, the left vocal fold was immobile in the paramedian position. A CT scan obtained that day revealed a new, large hematoma surrounding the upper descending aortic stent graft consistent with an acute contained ruptured aortic aneurysm. She was referred to the emergency department for evaluation and treatment by vascular surgery. She was counseled regarding surgical options and ultimately decided not to pursue further treatment. Her vocal fold immobility was subsequently treated via office-based injection medialization two weeks after presentation and again 5 months after the initial injection which dramatically improved her voice. Follow-up CT scan at 8 months demonstrated a reduction of the hematoma. The left vocal cord remains immobile to date. Conclusion. Ortner's syndrome, or cardiovocal syndrome, is hoarseness secondary to left recurrent laryngeal nerve palsy caused by cardiovascular pathology. It is a rare condition and, while typically presenting gradually, may also present with acute symptomatology.

  8. Acute Contained Ruptured Aortic Aneurysm Presenting as Left Vocal Fold Immobility

    Directory of Open Access Journals (Sweden)

    Sharon H. Gnagi

    2015-01-01

    Full Text Available Objective. To recognize intrathoracic abnormalities, including expansion or rupture of aortic aneurysms, as a source of acute onset vocal fold immobility. Methods. A case report and review of the literature. Results. An 85-year-old female with prior history of an aortic aneurysm presented to a tertiary care facility with sudden onset hoarseness. On laryngoscopy, the left vocal fold was immobile in the paramedian position. A CT scan obtained that day revealed a new, large hematoma surrounding the upper descending aortic stent graft consistent with an acute contained ruptured aortic aneurysm. She was referred to the emergency department for evaluation and treatment by vascular surgery. She was counseled regarding surgical options and ultimately decided not to pursue further treatment. Her vocal fold immobility was subsequently treated via office-based injection medialization two weeks after presentation and again 5 months after the initial injection which dramatically improved her voice. Follow-up CT scan at 8 months demonstrated a reduction of the hematoma. The left vocal cord remains immobile to date. Conclusion. Ortner’s syndrome, or cardiovocal syndrome, is hoarseness secondary to left recurrent laryngeal nerve palsy caused by cardiovascular pathology. It is a rare condition and, while typically presenting gradually, may also present with acute symptomatology.

  9. Preventative Valve-Sparing Aortic Root Replacement and Pregnancy Outcome in Marfan Syndrome

    OpenAIRE

    Sokol, Vesna; Zlopaša, Gordan; Herman, Mislav; Planinić, Pavao; Micevska, Ana

    2012-01-01

    In Marfan syndrome, with dilatation of the aortic root secondary to an underlying connective tissue defect, pregnancy can cause hemodynamic stress leading to the development of an aortic aneurysm and even a fatal aortic dissection. In the presence of existing aortic root enlargement and a family history of aortic dissection, preventative elective surgery is suggested. Aortic root replacement with or without a valve-sparing procedure is superior to total aortic root replacement with ...

  10. Aortic valve-sparing surgery in Marfan syndrome.

    Science.gov (United States)

    Nachum, Eyal; Shinfeld, Amichay; Kogan, Alexander; Preisman, Sergey; Levin, Shany; Raanani, Ehud

    2013-08-01

    Patients with Marfan syndrome are referred for cardiac surgery due to root aneurysm with or without aortic valve regurgitation. Because these patients are young and frequently present with normal-appearing aortic cusps, valve sparing is often recommended. However, due to the genetic nature of the disease, the durability of such surgery remains uncertain. Between February 2004 and June 2012, 100 patients in our department suffering from aortic aneurysm with aortic valve regurgitation underwent elective aortic valve-sparing surgery. Of them, 30 had Marfan syndrome, were significantly younger (30 +/- 13 vs. 53 +/- 16 years), and had a higher percentage of root aneurysm, compared with ascending aorta aneurysm in their non-Marfan counterparts. We evaluated the safety, durability, clinical and echocardiographic mid-term results of these patients. While no early deaths were reported in either group, there were a few major early complications in both groups. At follow-up (reaching 8 years with a mean of 34 +/- 26 months) there were no late deaths, and few major late complications in the Marfan group. Altogether, 96% and 78% of the patients were in New York Heart Association functional class I-II in the Marfan and non-Marfan groups respectively. None of the Marfan patients needed reoperation on the aortic valve. Freedom from recurrent aortic valve regurgitation > 3+ was 94% in the Marfan patients. Aortic valve-sparing surgery in Marfan symdrome patients is safe and yields good mid-term clinical outcomes.

  11. Acute aortic regurgitation due to infective endocarditis

    Directory of Open Access Journals (Sweden)

    Claudia M Cortés

    2017-10-01

    Full Text Available Acute aortic regurgitation (AAR due to infective endocarditis (IE is a serious disease and usually requires surgical treatment. Our study aims to compare the clinical, echocardiographic, and microbiological characteristics as well as in-hospital outcome of patients with AAR according to the severity of heart failure (HF and to evaluate predictors of in-hospital mortality in a tertiary centre. In a prospective analysis, we compared patients with NYHA functional class I-II HF (G1 vs. functional class III-IV HF (G2. From 06/92 to 07/16, 439 patients with IE were hospitalized; 86 presented AAR: (G1, 39: 45.4% y G2, 47: 54.7%. The G1 had higher prosthetic IE (43.6% vs. 17%, p 0.01. All G2 patients had dyspnoea vs. 30.8% of the G1 (p < 0.0001. There were no differences in clinical, echocardiographic and microbiological characteristics. Surgical treatment was indicated mainly due to infection extension or valvular dysfunction in G1 and HF in G2. In-hospital mortality was 15.4% vs. 27.7% (G1 and G2 respectively p NS. In multivariate analysis, health care-associated acquisition (p 0.001, negative blood cultures (p 0.004, and functional class III-IV HF (p 0.039 were in-hospital mortality predictors. One-fifth of the patients with EI had AAR. Half of them had severe HF which needed emergency surgery and the remaining needed surgery for extension of the infection and / or valvular dysfunction. Both groups remain to have high surgical and in-hospital mortality. Health care-associated acquisition, negative blood cultures and advanced HF were predictors of in-hospital mortality

  12. Combined Repair of Ascending Aortic Pseudoaneurysm and Abdominal Aortic Aneurysm in a Patient with Marfan Syndrome

    Science.gov (United States)

    Kokotsakis, John N.; Lioulias, Achilleas G.; Foroulis, Christophoros N.; Skouteli, Eleni Anna T.; Milonakis, Michael K.; Bastounis, Elias A.; Boulafendis, Dimitrios G.

    2003-01-01

    Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic aneurysm. An aggressive surgical strategy followed by life-long cardiovascular monitoring is warranted in order to prolong the survival of these patients. (Tex Heart Inst J 2003;30:233–5) PMID:12959210

  13. Combined repair of ascending aortic pseudoaneurysm and abdominal aortic aneurysm: in a patient with Marfan syndrome.

    Science.gov (United States)

    Kokotsakis, John N; Lioulias, Achilleas G; Foroulis, Christophoros N; Skouteli, Eleni Anna T; Milonakis, Michael K; Bastounis, Elias A; Boulafendis, Dimitrios G

    2003-01-01

    Pseudoaneurysms of the ascending aorta after the original inclusion/wrap technique of the Bentall procedure present a difficult surgical management problem and are associated with substantial morbidity and mortality. Patients with Marfan syndrome frequently develop aneurysms and dissections that involve multiple aortic segments. We present the case of a Marfan patient who successfully underwent repair of a giant ascending aortic pseudoaneurysm and concomitant repair of an abdominal aortic aneurysm. An aggressive surgical strategy followed by life-long cardiovascular monitoring is warranted in order to prolong the survival of these patients.

  14. Blood groups and acute aortic dissection type III.

    Science.gov (United States)

    Fatic, Nikola; Nikolic, Aleksandar; Vukmirovic, Mihailo; Radojevic, Nemanja; Zornic, Nenad; Banzic, Igor; Ilic, Nikola; Kostic, Dusan; Pajovic, Bogdan

    2017-04-01

    Acute aortic type III dissection is one of the most catastrophic events, with in-hospital mortality ranging between 10% and 12%. The majority of patients are treated medically, but complicated dissections, which represent 15% to 20% of cases, require surgical or thoracic endovascular aortic repair (TEVAR). For the best outcomes adequate blood transfusion support is required. Interest in the relationship between blood type and vascular disease has been established. The aim of our study is to evaluate distribution of blood groups among patients with acute aortic type III dissection and to identify any kind of relationship between blood type and patient's survival. From January 2005 to December 2014, 115 patients with acute aortic type III dissection were enrolled at the Clinic of Vascular and Endovascular Surgery in Belgrade, Serbia and retrospectively analyzed. Patients were separated into two groups. The examination group consisted of patients with a lethal outcome, and the control group consisted of patients who survived. The analysis of the blood groups and RhD typing between groups did not reveal a statistically significant difference ( p = 0.220). Our results indicated no difference between different blood groups and RhD typing with respect to in-hospital mortality of patients with acute aortic dissection type III.

  15. Haemolytic anaemia resulting from the surgical repair of acute type A aortic dissection.

    Science.gov (United States)

    Sekine, Yuji; Yamamoto, Shin; Fujikawa, Takuya; Oshima, Susumu; Ono, Makoto; Sasaguri, Shiro

    2014-02-01

    Haemolytic anaemia after acute aortic dissection surgery is extremely rare. We report 4 cases of haemolytic anaemia with different aetiologies. Four patients underwent emergency operation for acute type A aortic dissection and subsequently developed haemolytic anaemia. Case 1: a 41-year old man underwent hemiarch replacement. We performed total arch replacement 3 years postoperatively, which revealed that haemolytic anaemia was induced by proximal anastomotic stenosis caused by inverted internal felt strip. Case 2: a 28-year old man diagnosed with Marfan syndrome underwent total arch replacement. Five months postoperatively, we noted severe stenosis at the previous distal anastomotic site, which caused the haemolytic anaemia, and performed descending thoracic aortic replacement for a residual dissecting aneurysm. Case 3: a 49-year old man underwent hemiarch replacement. Three years postoperatively, we performed total arch replacement for a residual dissecting aortic arch aneurysm and repaired a kinked graft responsible for haemolytic anaemia. Case 4: a 42-year old man underwent total arch replacement. Eighteen months later, we performed descending thoracic aortic replacement. We repaired a portion of the ascending aorta as haemolityc anaemia was induced by kinking of a total arch replacement redundant graft. All the haemolityc anaemia patients were successfully released after surgical reintervention.

  16. Selective Aortic Arch and Root Replacement in Repair of Acute Type A Aortic Dissection.

    Science.gov (United States)

    Fleischman, Fernando; Elsayed, Ramsey S; Cohen, Robbin G; Tatum, James M; Kumar, S Ram; Kazerouni, Kayvan; Mack, Wendy J; Barr, Mark L; Cunningham, Mark J; Hackmann, Amy E; Baker, Craig J; Starnes, Vaughn A; Bowdish, Michael E

    2018-02-01

    Controversy exists regarding the optimal extent of repair for type A aortic dissection. Our approach is to replace the ascending aorta, and only replace the aortic root or arch when intimal tears are present in those areas. We examined intermediate outcomes with this approach to acute type A aortic dissection repair. Between March 2005 and October 2016, 195 patients underwent repair of acute type A aortic dissection. Repair was categorized by site of proximal and distal anastomosis and extent of repair. Mean follow-up was 31.0 ± 30.9 months. Kaplan-Meier analysis was used to assess survival. Multiple variable Cox proportional hazards modeling was utilized to identify factors associated with overall mortality. Overall survival was 85.1%, 83.9%, 79.1%, and 74.4% at 6, 12, 36, and 60 months, respectively. Eight patients required reintervention. The cumulative incidence of aortic reintervention at 1 year with death as a competing outcome was 3.95%. Multiple variable regression analysis identified factors such as age, preoperative renal failure, concomitant thoracic endograft, postoperative myocardial infarction and sepsis, and need for extracorporeal membrane oxygenation as predictive of overall mortality. Neither proximal or distal extent of repair, nor need for reintervention affected overall survival (proximal: hazard ratio 1.63, 95% confidence interval: 0.75 to 3.51, p = 0.22; distal: hazard ratio 1.12, 95% confidence interval: 0.43 to 2.97, p = 0.81; reintervention: hazard ratio 0.03, 95% confidence interval: 0.002 to 0.490, p < 0.01). A selective approach to root and arch repair in acute type A aortic dissection is safe. If aortic reintervention is needed, survival does not appear to be affected. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  17. Reinforced aortic root reconstruction for acute type A aortic dissection involving the aortic root

    Directory of Open Access Journals (Sweden)

    Han Qing-qi

    2013-06-01

    Full Text Available OBJECTIVE: There are debates regarding the optimal approach for AAAD involving the aortic root. We described a modified reinforced aortic root reconstruction approach for treating AAAD involving the aortic root. METHODS: A total of 161 patients with AAAD involving the aortic root were treated by our modified reinforced aortic root reconstruction approach from January 1998 to December 2008. Key features of our modified approach were placement of an autologous pericardial patch in the false lumen, lining of the sinotubular junction lumen with a polyester vascular ring, and wrapping of the vessel with Teflon strips. Outcome measures included post-operative mortality, survival, complications, and level of aortic regurgitation. RESULTS: A total of 161 patients were included in the study (mean age: 43.3 1 15.5 years. The mean duration of follow-up was 5.1 1 2.96 years (2-12 years. A total of 10 (6.2% and 11 (6.8% patients died during hospitalization and during follow-up, respectively. Thirty-one (19.3% patients experienced postoperative complications. The 1-, 3-, 5-, and 10-year survival rates were 99.3%, 98%, 93.8%, and 75.5%, respectively. There were no instances of recurrent aortic dissection, aortic aneurysm, or pseudoaneurysm during the entire study period. The severity of aortic regurgitation dramatically decreased immediately after surgery (from 28.6% to 0% grade 3-4 and thereafter slightly increased (from 0% to 7.2% at 5 years and 9.1% at 10 years. CONCLUSION: This modified reinforced aortic root reconstruction was feasible, safe and durable/effective, as indicated by its low mortality, low postoperative complications and high survival rate.

  18. [Aortic valve preservation in Marfan syndrome. Initial experience].

    Science.gov (United States)

    Forteza, Alberto; Cortina, Jose M; Sánchez, Violeta; Centeno, Jorge; López, M Jesús; Pérez de la Sota, Enrique; Rufilanchas, Juan J

    2007-05-01

    Preservation of the aortic valve using the technique described by David has been shown to be as effective as the Bentall-De Bono procedure. It avoids both the need for long-term anticoagulation and the complications associated with mechanical prostheses. We report our initial experience using this technique in patients with Marfan syndrome. Between April 2004 and April 2006, we used the David reimplantation technique in 40 patients with an aortic root aneurysm. Eighteen patients had Marfan syndrome. Their median age was 29 years (13-55 years). Echocardiography showed that the median diameter of the aortic sinus was 53 mm (46-59 mm). In 17 patients, aortic valve preservation was possible. No patient died during hospitalization and there were no significant complications. On echocardiography at discharge, no patient had greater than grade-II aortic regurgitation. During a median follow-up period of 8 months (1-24 months), one patient died due to rupture of an abdominal aneurysm. The others are all in New York Heart Association class I. Preservation of the aortic valve by means of valve reimplantation produced excellent results. It avoided both the thromboembolic and hemorrhagic complications associated with prostheses and the need for long-term anticoagulation. If reimplanted valves continue to function adequately over the long term, this technique should become the treatment of choice for aneurysms of the ascending aorta in patients with Marfan syndrome.

  19. Acute respiratory distress syndrome

    OpenAIRE

    Confalonieri, Marco; Salton, Francesco; Fabiano, Francesco

    2017-01-01

    Since its first description, the acute respiratory distress syndrome (ARDS) has been acknowledged to be a major clinical problem in respiratory medicine. From July 2015 to July 2016 almost 300 indexed articles were published on ARDS. This review summarises only eight of them as an arbitrary overview of clinical relevance: definition and epidemiology, risk factors, prevention and treatment. A strict application of definition criteria is crucial, but the diverse resource-setting scenarios foste...

  20. Aortic Disease in the Young: Genetic Aneurysm Syndromes, Connective Tissue Disorders, and Familial Aortic Aneurysms and Dissections

    Directory of Open Access Journals (Sweden)

    Marcelo Cury

    2013-01-01

    Full Text Available There are many genetic syndromes associated with the aortic aneurysmal disease which include Marfan syndrome (MFS, Ehlers-Danlos syndrome (EDS, Loeys-Dietz syndrome (LDS, familial thoracic aortic aneurysms and dissections (TAAD, bicuspid aortic valve disease (BAV, and autosomal dominant polycystic kidney disease (ADPKD. In the absence of familial history and other clinical findings, the proportion of thoracic and abdominal aortic aneurysms and dissections resulting from a genetic predisposition is still unknown. In this study, we propose the review of the current genetic knowledge in the aortic disease, observing, in the results that the causative genes and molecular pathways involved in the pathophysiology of aortic aneurysm disease remain undiscovered and continue to be an area of intensive research.

  1. Effectiveness of treatment for octogenarians with acute abdominal aortic aneurysm

    NARCIS (Netherlands)

    Scheer, Margot L. J.; Pol, Robert A.; Haveman, Jan Willem; Tielliu, Ignace F. J.; Verhoeven, Eric L. G.; Van den Dungen, Jan J. A. M.; Nijsten, Maarten W.; Zeebregts, Clark J.

    Objective: To investigate whether advanced age may be a reason to refrain from treatment in patients with an acute abdominal aortic aneurysm (AAAA). Methods: This was a retrospective cohort study that took place in a tertiary care university hospital with a 45-bed intensive care unit. Two hundred

  2. Influence of distal entry tears in acute type B aortic dissection after thoracic endovascular aortic repair.

    Science.gov (United States)

    Zhu, Chenmou; Huang, Bin; Zhao, Jichun; Ma, Yukui; Yuan, Ding; Yang, Yi; Xiong, Fei; Wang, Tiehao

    2017-08-01

    This study evaluated the clinical influence of distal entry tears in acute type B aortic dissection (ATBAD) after thoracic endovascular aortic repair (TEVAR). From August 2009 to December 2014, the clinical outcomes of 130 patients who underwent TEVAR for ATBAD were retrospectively analyzed. According to whether distal entry tears existed after TEVAR, patients were divided into group A (n = 25, absence of distal entry tears) and group B (n = 105, presence of distal entry tears). We evaluated clinical outcomes, including mortality and morbidity in early and late follow-up, as well as aortic remolding. Late aortic events were defined as aortic dissection-related events occurring >30 days from the initial TEVAR procedures, which consisted of endoleak, retrograde type A aortic dissection, aortic enlargement, late rupture, repeat dissection, and stent-induced new entry tear. The study comprised 130 patients (114 men [87.7%] and 16 women [12.3%)] with a mean age of 53.71 years. The 30-day mortality was 3.1%, and early morbidity included type I endoleak, 3.1%, organ failure, 3.8%; stroke, 3.1%; spinal cord ischemia, 0%; and early rupture 1.5%. The overall survival rate by Kaplan-Meier analysis at 1, 3, and 5 years was 93.8%, 89.5%, and 79.2%, respectively. There were no significant differences in early morbidity and 30-day mortality and late survival between group A and group B. However, group A had a significantly lower rate of late aortic events than group B (P = .028 by log-rank test). Meanwhile, group A had better aortic remolding than group B in complete thrombosis of the thoracic aorta at 12 months postoperatively (100% vs 83.5%; P = .029). This study demonstrated that TEVAR for ATBAD had low perioperative morbidity and mortality and satisfactory midterm outcome. Distal entry tears increase the occurrence of late aortic events and inhibit aortic remolding but do not have a significantly negative effect on late survival. Repairing all entry tears to restore

  3. Acute aortic dissection diagnosed after embalming: macroscopic and microscopic findings.

    Science.gov (United States)

    Savall, Frédéric; Dedouit, Fabrice; Piercecchi-Marti, Marie-Dominique; Leonetti, Georges; Rougé, Daniel; Telmon, Norbert

    2014-09-01

    A 58-year-old man died suddenly in Madagascar and poisoning was suspected. The body was embalmed after death and the general state of preservation was good. We found a major aortic dissection with a large false lumen from the aortic root to the common iliac arteries and a hemopericardium with formalinized blood clot. The intimal tear was on the ascending aorta, and an intramural hemorrhage was noted at the right coronary artery, attesting to a retrograde dissection. Microscopic studies confirmed aortic dissection with extensive intramural hemorrhage and also confirmed the retrograde dissection to the right coronary artery with a reduction of 90% of the true lumen. Classically, aortic dissection occurs in individuals with hypertension and individuals with genetic disorders of collagen formation. The diagnosis is often first established at the postmortem examination. Aortic dissection is therefore dealt with largely in necropsy studies. The usual cause of death is rupture into the pericardial sac. One case of bloodless dissection has been reported but the sudden death was explained by acute myocardial ischemia secondary to dissection of the left coronary artery. In our case, we found major hemopericardium and also intramural hemorrhage at the right coronary artery. We were able to make the diagnosis of aortic dissection and exclude the suspicion of homicide 15 days after death and after embalming. © 2014 American Academy of Forensic Sciences.

  4. Severe acute respiratory syndrome (SARS)

    Science.gov (United States)

    ... their fever and other symptoms are gone. Hand hygiene is the most important part of SARS prevention. ... Coronaviruses, including severe acute respiratory syndrome (SARS) and Middle East respiratory syndrome (MERS). In: Bennett JE, Dolin ...

  5. Transcatheter aortic valve implantation for a failed bio-bentall in patients with Marfan syndrome.

    Science.gov (United States)

    Beigel, Roy; Siegel, Robert J; Kahlon, Ravi S; Jilaihawi, Hasan; Cheng, Wen; Makkar, Raj R

    2014-01-01

    Patients with Marfan syndrome are at risk for ascending aortic dilation and dissection at the level of the aortic sinuses, making aortic root and valve replacement common. Patients undergoing an aortic root replacement with concomitant replacement of the aortic valve with a bioprosthesis (Bio-Bentall) are predisposed to bioprosthesis failure. Transcatheter aortic valve implantation (TAVI) has become an option for aortic valve replacement, avoiding cardiopulmonary bypass and/or median sternotomy. We present the first 2 reported patients with Marfan syndrome who underwent a valve-in-valve TAVI in the setting of a prior Bio-Bentall. © 2014 S. Karger AG, Basel.

  6. Acute Aortic Dissection: Is There Something Better than Physician Gestalt?

    Science.gov (United States)

    Smith, Lane M; Miller, Chadwick D

    2018-03-02

    Acute aortic dissection (AAD) sits at the intersection of rare, deadly, and expensive to diagnose conditions. It is a disease that every emergency physician (EP) considers on a daily basis, but encounters only a handful during a career. Considerable attention has been given to this disease after the American Heart Association, American College of Cardiology, and 10 cosponsoring professional societies published the 2010 thoracic aortic disease guidelines to improve the missed or delayed treatment of AAD (1). This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  7. Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

    Science.gov (United States)

    Shimizu, Hideyuki; Kasahara, Hirofumi; Nemoto, Atsushi; Yamabe, Kentaro; Ueda, Toshihiko; Yozu, Ryohei

    2012-02-01

    We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to 7 years, but new dissection developed in 5 (33.3%) of the 15 patients who were followed up for >7 years after the root repair. Actuarial survival including operative mortality was 88.1 and 65.0% at 10 and 20 years, respectively; groups A and B did not significantly differ. Rates of freedom from all-cause death, new dissection or repeated aortic surgery were 60.1, 44.5 and 26.0% at 5, 10 and 15 years, respectively. Group A was significantly better than group B. Prophylactic aortic root repair apparently reduces the likelihood of overall adverse events, but it cannot guarantee the prevention of further aortic dissection. A multidisciplinary approach is needed for patients with Marfan syndrome.

  8. Preventative valve-sparing aortic root replacement and pregnancy outcome in Marfan syndrome.

    Science.gov (United States)

    Sokol, Vesna; Zlopasa, Gordan; Herman, Mislav; Planinić, Pavao; Micevska, Ana

    2012-06-01

    In Marfan syndrome, with dilatation of the aortic root secondary to an underlying connective tissue defect, pregnancy can cause hemodynamic stress leading to the development of an aortic aneurysm and even a fatal aortic dissection. In the presence of existing aortic root enlargement and a family history of aortic dissection, preventative elective surgery is suggested. Aortic root replacement with or without a valve-sparing procedure is superior to total aortic root replacement with prosthetic valve/tube graft. It provides excellent survival with low rates of aortic - valve related complications.

  9. Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

    OpenAIRE

    Shimizu, Hideyuki; Kasahara, Hirofumi; Nemoto, Atsushi; Yamabe, Kentaro; Ueda, Toshihiko; Yozu, Ryohei

    2011-01-01

    We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to ...

  10. Endovascular treatment of acute type B dissection complicating aortic coarctation.

    Science.gov (United States)

    Kassaian, Seyed Ebrahim; Abbasi, Kyomars; Mousavi, Mehdi; Sahebjam, Mohammad

    2013-01-01

    Surgical treatment poses a high risk to patients with concomitant aortic coarctation and dissection, and an interventional approach could be an alternative. We describe the case of a 52-year-old man with a long history of untreated hypertension and aortic coarctation who emergently presented at our institution with an acute Stanford type B dissection. The patient's elevated serum creatinine level, perfusion deficit in the right lower limb, and hypertension did not respond to medical therapy, and he did not consent to surgery. By endovascular means, we used a self-expandable stent-graft to cover the entry point of the dissection; then, we deployed a balloon-expandable bare-metal stent to correct residual stenosis. To our knowledge, this is the first report of the endovascular treatment of aortic coarctation complicated by type B dissection.

  11. Marfan's syndrome presenting with abdominal aortic aneurysm: A ...

    African Journals Online (AJOL)

    We present the case of a 16-year old student with Marfan's syndrome and abdominal aortic aneurysm who presented with a diagnostic conundrum. He presented with a three months history of progressive painful left upper abdominal mass and back pain. It became severe in the last two weeks before presentation and was ...

  12. Acute Right Coronary Ostial Stenosis during Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Sarwar Umran

    2012-01-01

    Full Text Available We report a rare case of acute right coronary artery stenosis developing in a patient undergoing aortic valve replacement. We present a case report with a brief overview of the literature relating to coronary artery occlusion associated with cardiac valve surgery - the theories and treatments are discussed. A 85 year-old female was admitted under the care of the cardiothoracic team with signs and symptoms of heart failure. Investigations, including cardiac echocardiography and coronary angiography, indicated a critical aortic valve stenosis. Intraoperative right ventricular failure ensued post aortic valve replacement. Subsequent investigations revealed an acute occlusion of the proximal right coronary artery with resultant absence of distal flow supplying the right ventricle. An immediate right coronary artery bypass procedure was performed with resolution of the right ventricular failure. Subsequent weaning off cardiopulmonary bypass was uneventful and the patient continued to make excellent recovery in the postoperative phase. To our knowledge this is one of the few documented cases of intraoperative acute coronary artery occlusion developing during valve surgery. However, surgeons should be aware of the potential for acute occlusion so that early recognition and rapid intervention can be instituted.

  13. Mitral and aortic regurgitation in Behçet's syndrome.

    OpenAIRE

    Peńa, J M; Garcia-Alegria, J; Garcia-Fernandez, F; Arnalich, F; Barbado, F J; Vazquez, J J

    1985-01-01

    A 35-year-old man with definite Behçet's disease developed acute mitral and aortic regurgitation. Valvular disease, we believed, was another manifestation of this disease. Cardiac involvement in Behçet's disease and the role of prednisone therapy are discussed.

  14. Acute Traumatic Aortic Disruption and Right Aortic Arch: A Fatal Diagnosis

    Directory of Open Access Journals (Sweden)

    Shirvan Salaminia

    2016-09-01

    Full Text Available Acute traumatic aortic disruption occurs after forceful deceleration and usually due to motor accidents. Only 10 % to 15 % reach a treatment facility alive and a highly suspicious state is needed for timely diagnosis. Most time they suffer multiple associated lethal injuries. Asymptomatic and isolated right aortic arch is a rare anomaly of the aorta with a prevalence of 0.5% [3]. Its diagnosis is by radiologic studies. We present this patient to remember that the incidental right aortic arch and disruption may interpreted as the left side mediastinal rotation in radiography and so inadvertently lead to late diagnosis and a futile outcome. A 24-year old man was brought to emergency room following a motor accident. He had Glascow Coma Scale Score: 14-15/15 but with stable vital signs. After primary survey chest radiography, emergency abdominal sonography (eFAST and brain CT scanning were requested. Spiral thoracoabdominal CT was also requested about seven hours after admission and when the patient entered an unstable hemodynamic phase. The primary survey was unremarkable. His chest radiography had left mediastinal rotation, which is opposite to what is seen pathologically in the condition of the traumatic aortic disruption, the right mediastinal rotation. His eFAST and brain CT were normal. The patient remained stable until seven hours after admission when the patient becomes unstable. Massive pleural effusion with aortic disruption and a right aortic arch was seen in thoracoabdominal CT. He transferred to the operation room but arrested during transfer. Massive hemothorax was seen during open cardiac massage. Cardiopulmonary resuscitation was unsuccessful. This may raises that in any blunt trauma patient with highly suspicious history for the great vessel injury, it may be better to consider the spiral chest CT scanning as the primary radiologic test for evaluation of the chest trauma and not waste the time or resources with rely simply on a

  15. Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome.

    Science.gov (United States)

    Mommertz, G; Sigala, F; Langer, S; Koeppel, T A; Mess, W H; Schurink, G W H; Jacobs, M J

    2008-02-01

    We assessed the surgical outcome of descending thoracic aortic aneurysm repair (DTAA) and thoracoabdominal aortic aneurym (TAAA) repair in patients with Marfan syndrome. During a six year period, 206 patients underwent DTAA and TAAA repair. In 22 patients, Marfan syndrome was confirmed. The median age was 40 years with a range between 18 and 57 years. The extend of the aneurysms included 6 DTAA (1 with total arch, 2 with distal hemi-arch), 11 type II TAAA (2 with total arch, 3 with distal hemi-arch), 4 type III and one type IV TAAA. All patients suffered from previous type A (n=6) or type B (n=16) aortic dissection and 15 already underwent aortic procedures like Bentall (n=7) and ascending aortic replacement (n=8). All patients were operated on according to the standard protocol with cerebrospinal fluid drainage, distal aortic and selective organ perfusion and monitoring motor evoked potentials. In patients undergoing simultaneous arch replacement (via left thoracotomy), transcranial Doppler and EEG assessed cerebral physiology during antegrade brain perfusion. In four patients circulatory arrest under moderate hypothermia was required. In-hospital mortality did not occur. Major postoperative complications like paraplegia, renal failure, stroke and myocardial infarction were not encountered. Mean pre-operative creatinine level was 125mmol/L, which peaked to a mean maximal level of 130 and returned to 92mmol/L at discharge. Median intubation time was 1.5 days (range 0.33-30 days). Other complications included bleeding requiring surgical intervention (n=1), arrhythmia (n=2), pneumonia (n=2) and respiratory distress syndrome (n=1). At a median follow-up of 38 months all patients were alive. Using CT surveillance, new or false aneurysms were not detected, except in one patient who developed a visceral patch aneurysm six years after open type II repair. Surgical repair of descending and thoracoabdominal aortic aneurysms provides excellent short- and mid-term results in

  16. Sex, pregnancy and aortic disease in Marfan syndrome.

    Science.gov (United States)

    Renard, Marjolijn; Muiño-Mosquera, Laura; Manalo, Elise C; Tufa, Sara; Carlson, Eric J; Keene, Douglas R; De Backer, Julie; Sakai, Lynn Y

    2017-01-01

    Sex-related differences as well as the adverse effect of pregnancy on aortic disease outcome are well-established phenomena in humans with Marfan syndrome (MFS). The underlying mechanisms of these observations are largely unknown. In an initial (pilot) step we aimed to confirm the differences between male and female MFS patients as well as between females with and without previous pregnancy. We then sought to evaluate whether these findings are recapitulated in a pre-clinical model and performed in-depth cardiovascular phenotyping of mutant male and both nulliparous and multiparous female Marfan mice. The effect of 17β-estradiol on fibrillin-1 protein synthesis was compared in vitro using human aortic smooth muscle cells and fibroblasts. Our small retrospective study of aortic dimensions in a cohort of 10 men and 20 women with MFS (10 pregnant and 10 non-pregnant) confirmed that aortic root growth was significantly increased in the pregnant group compared to the non-pregnant group (0.64mm/year vs. 0.12mm/year, p = 0.018). Male MFS patients had significantly larger aortic root diameters compared to the non-pregnant and pregnant females at baseline and follow-up (p = 0.002 and p = 0.007, respectively), but no significant increase in aortic root growth was observed compared to the females after follow-up (p = 0.559 and p = 0.352). In the GT-8/+ MFS mouse model, multiparous female Marfan mice showed increased aortic diameters when compared to nulliparous females. Aortic dilatation in multiparous females was comparable to Marfan male mice. Moreover, increased aortic diameters were associated with more severe fragmentation of the elastic lamellae. In addition, 17β-estradiol was found to promote fibrillin-1 production by human aortic smooth muscle cells. Pregnancy-related changes influence aortic disease severity in otherwise protected female MFS mice and patients. There may be a role for estrogen in the female sex protective effect.

  17. Takayasu arteritis with middle aortic syndrome and mesenteric ischemia treated by aortic stenting

    Directory of Open Access Journals (Sweden)

    Sarah Morrissey, MD, MA, FRCSC

    2017-09-01

    Full Text Available A 48-year-old woman suffering from Takayasu arteritis presented with middle aortic syndrome consisting of abdominal pain, refractory hypertension with pulmonary edema and pleural effusions, and lower limb ischemia. She failed to improve with high-dose steroid therapy and underwent endovascular stenting of two severe stenoses in the supraceliac and infrarenal aorta. Her symptoms resolved and hypertension improved after the procedure.

  18. Metabolic syndrome and acute pancreatitis.

    Science.gov (United States)

    Mikolasevic, I; Milic, S; Orlic, L; Poropat, G; Jakopcic, I; Franjic, N; Klanac, A; Kristo, N; Stimac, D

    2016-07-01

    The aim of our study was to investigate the influence of metabolic syndrome on the course of acute pancreatitis determined by disease severity, the presence of local and systemic complications and survival rate. 609 patients admitted to our hospital in the period from January 1, 2008 up to June 31, 2015 with the diagnosis of acute pancreatitis were analyzed. The diagnosis and the severity of acute pancreatitis were made according to the revised Atlanta classification criteria from 2012. Of 609 patients with acute pancreatitis, 110 fulfilled the criteria for metabolic syndrome. Patients with metabolic syndrome had statistically significantly higher incidence of moderately severe (38.2% vs. 28.5%; p=0.05) and severe (22.7% vs. 12.8%; p=0.01) acute pancreatitis in comparison to those without metabolic syndrome, while patients without metabolic syndrome had higher incidence of mild acute pancreatitis in comparison to those patients with metabolic syndrome (58.7% vs. 39.1%; pacute pancreatitis. Comparing survival rates, patients suffering from metabolic syndrome had a higher death rate compared to patients without metabolic syndrome (16% vs. 4.5%; pacute pancreatitis, as well as higher mortality rate. Copyright © 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  19. Pig specific vascular anatomy allows acute infrarenal aortic occlusion without hind limb ischemia and stepwise occlusion without clinical signs.

    Science.gov (United States)

    Haacke, N; Unger, J K; Haidenhein, C; Russ, M; Hiebl, B; Niehues, S M

    2011-01-01

    In a porcine, aortic graft model we found 5 animals to develop and survive unnoticed, complete infrarenal aortic occlusion and 2 pigs with an acute occlusion but rather unspecific clinical symptoms. We investigated the pigs' vascular system to classify the anatomic capabilities to compensate for an acute abdominal aortic occlusion. Retrospective analysis of CT scans and clinical data to specify unexpected results in a case series of infrarenal occlusion in a porcine model. Collaborative study of experimental and clinical departments. Fifteen healthy female minipigs. All pigs underwent an infrarenal aortic graft intervention. Anesthesia and perioperative management of the animals were preformed along the standard operating procedures of the local Department of Experimental Medicine. All animals received perioperative antibiotics, ASS, and carprofen for postoperative analgesia. Arterial pressure, heart rate, body temperature, and diuresis were monitored during surgery and therapeutic interventions. Contrast media based computed tomography (CT) with total body scans were performed at 1, 4, 10, 12 weeks after surgery. Comparable scans of cardiovascular healthy subjects (humans and pigs) and patients with a Leriche's syndrome were analyzed. Neither acute (within the first 12 h after surgery) nor stepwise total aortic occlusion show unmistakable clinical signs. In pigs, the epigastric artery (EGA) - which is in connection with suprarenal lumbal arteries, subclavian and external iliac artery - is highly developed associated to the high number of mammary glands of about 7 on one side. In humans, the ratio of aortic to EGA-diameter is 1 : 0.15. In minipigs we found a ratio of 1 : 0.43 which changed during aortic occlusion resulting in a ratio of 1 : 0.58. Pigs with a slowly developing occlusion demonstrated an enlargement of the ureteric artery of about 210% completing a sufficient collateral system. While in the human Leriche's syndrome months are needed to enlarge the

  20. Possible extracardiac predictors of aortic dissection in Marfan syndrome

    Science.gov (United States)

    2014-01-01

    Background According to previous studies, aortic diameter alone seems to be insufficient to predict the event of aortic dissection in Marfan syndrome (MFS). Determining the optimal schedule for preventive aortic root replacement (ARR) aortic growth rate is of importance, as well as family history, however, none of them appear to be decisive. Thus, the aim of this study was to search for potential predictors of aortic dissection in MFS. Methods A Marfan Biobank consisting of 79 MFS patients was established. Thirty-nine MFS patients who underwent ARR were assigned into three groups based on the indication for surgery (dissection, annuloaortic ectasia and prophylactic surgery). The prophylactic surgery group was excluded from the study. Transforming growth factor-β (TGF-β) serum levels were measured by ELISA, relative expression of c-Fos, matrix metalloproteinase 3 and 9 (MMP-3 and −9) were assessed by RT-PCR. Clinical parameters, including anthropometric variables - based on the original Ghent criteria were also analyzed. Results Among patients with aortic dissection, TGF-β serum level was elevated (43.78 ± 6.51 vs. 31.64 ± 4.99 ng/l, p < 0.0001), MMP-3 was up-regulated (Ln2α = 1.87, p = 0.062) and striae atrophicae were more common (92% vs. 41% p = 0.027) compared to the annuloaortic ectasia group. Conclusions We found three easily measurable parameters (striae atrophicae, TGF-β serum level, MMP-3) that may help to predict the risk of aortic dissection in MFS. Based on these findings a new classification of MFS, that is benign or malignant is also proposed, which could be taken into consideration in determining the timing of prophylactic ARR. PMID:24720641

  1. Valve-sparing aortic root replacement in Marfan syndrome.

    Science.gov (United States)

    Cameron, Duke E; Vricella, Luca A

    2005-01-01

    Marfan syndrome is the most common inherited connective tissue disorder, affecting approximately 1 in 10,000 live births. The cardinal features of Marfan syndrome are the abnormalities of the skeleton (tall stature, arachnodactyly, and joint hyperelasticity), eye (lens subluxation), and aorta (root aneurysm with proclivity toward rupture and dissection). Aortic catastrophe accounts for most of the premature mortality among Marfan patients, a risk that climbs steeply during adolescence and results in death of half of Marfan patients by the age of 40 years. Most of the improvement in life expectancy that has been achieved in Marfan syndrome is attributable to early recognition of aortic root aneurysms and prophylactic replacement with composite grafts (mechanical valve prostheses within Dacron conduits) before rupture or dissection occurs. Despite the excellent early and late results with composite grafts, there has been growing interest in operative procedures that replace the sinuses but preserve the aortic valve leaflets, to avoid anticoagulation and minimize the risk of prosthesis-related endocarditis. These procedures are still in evolution and late results are not yet known, but as with mitral repair in the setting of myxomatous disease, valve-sparing procedures in Marfan syndrome have weathered a storm of initial criticism and skepticism and are steadily gaining acceptance.

  2. Aortic valve sparing root surgery for Marfan syndrome.

    Science.gov (United States)

    Matalanis, George; Perera, Nisal K

    2017-11-01

    Aortic valve sparing root surgery (AVSRS) is a safe and durable alternative for patients with dilated roots or pure aortic regurgitation (AR), which avoids the risks of anticoagulation or valvular degeneration with prosthetic valves. Notwithstanding the theoretical challenges of greater tissue fragility in Marfan syndrome (MFS), AVSRS has been demonstrated to have equal outcomes in this condition as it does in those without MFS. The benefits of retaining the native aortic valve in this generally younger age group extend beyond those of avoiding the inconvenience and complications of prolonged exposure to anticoagulants and include ease of management for future aortic, cardiac and non-cardiac procedures which are the norm for these patients. The essential principles of AVSRS in MFS do not differ from those for the rest of the population. Successful repair and durable valve function depend on a sound understanding of the close interaction between the structure and function of this exquisitely designed piece of engineering. We are fortunate to have numerous tools in our surgical armamentarium to preserve these valves. It is the purpose of this paper to demystify the complex structure-function interactions of the aortic valve, thereby gaining an intuition for AVSRS. We will also elaborate on specific technical details of established techniques that we have found successful in preserving the normal function of these valves in the long term.

  3. Acute type A aortic dissection: characteristics and outcomes comparing patients with bicuspid versus tricuspid aortic valve.

    Science.gov (United States)

    Etz, Christian D; von Aspern, Konstantin; Hoyer, Alexandro; Girrbach, Felix F; Leontyev, Sergey; Bakhtiary, Farhad; Misfeld, Martin; Mohr, Friedrich W

    2015-07-01

    The aim of this study is to investigate the clinical characteristics and postoperative outcome of patients with a bicuspid aortic valve (BAV) suffering acute dissection in comparison with their tricuspid peers. Between 1995 and 2011, 460 consecutive patients underwent emergency repair for acute type A aortic dissection. In 379 patients without connective tissue disease, the aortic valve morphology could clearly be specified (91.6% tricuspid and 8.4% bicuspid). At the time of dissection, patients with a bicuspid valve were younger (46.7 ± 13 vs 61.6 ± 12 years, P tricuspid valve (bicuspid: 31.3% vs tricuspid: 6.3%, P tricuspid valve patients (P tricuspid patients (P = 0.166). Hospital mortality was 20.3% and not significantly different between the two valve morphologies, even despite the younger age of bicuspid patients: 28.1% among bicuspids vs 19.6% among tricuspids (P = 0.255). Survival after discharge was 63.3% at 10 years for all patients. BAV patients had a significantly better survival with 100% at 10 years compared with 60.2% in tricuspid valve patients (P = 0.011). Mean follow-up among survivors was comparable for bicuspid and tricuspid patients (3.7 and 4.1 years, respectively). Patients with BAV have a distinctive dissection pattern with the entry tear frequently located in the aortic root and-despite their younger age-are subject to substantial hospital mortality. For bicuspid patients suffering from dissection, composite root replacement yields an excellent outcome equal to an age- and gender-matched normal population. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  4. Incidence of systemic inflammatory response syndrome after endovascular aortic repair

    DEFF Research Database (Denmark)

    De La Motte, L; Vogt, K; Jensen, Leif Panduro

    2011-01-01

    AIM: The aim of this study was to estimate the incidence of the post-implantation syndrome/systemic inflammatory response syndrome (SIRS) after endovascular aortic repair. METHODS: All patients, undergoing elective primary endovascular repair of an asymptomatic infrarenal abdominal aortic aneurysm...... during 2007, were retrospectively evaluated for SIRS within the first 5 postoperative days. The only exclusion-criteria were missing data. SIRS was assessed using the criteria defined by the American College of Chest Physicians and Society of Critical Care Medicine Consensus Conference Committee. RESULTS......: Sixty-six patients were included, 40 (60%) met the SIRS criteria within the first 5 postoperative days (95% of the 40 patients met the criteria within 3 days). We found no significant differences between the SIRS and the non-SIRS group in baseline characteristics or other data including volume...

  5. Mutations in a TGF-β Ligand, TGFB3, Cause Syndromic Aortic Aneurysms and Dissections

    OpenAIRE

    Bertoli Avella, Aida; Gillis, Elisabeth; Morisaki, Hiroko; Verhagen, Judith; Graaf, Bianca; Van De Beek, Gerarda; Gallo, Elena; Kruithof, Boudewijn; Venselaar, Hanka; Myers, Loretha; Laga, Steven; Doyle, Alexander; Oswald, Gretchen; Cappellen, Gert; Yamanaka, Itaru

    2015-01-01

    markdownabstractBACKGROUND Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed an important contribution of disturbed transforming growth factor (TGF)-b signaling. OBJECTIVES This study sought to discover a novel gene causing syndromic aortic aneurysms in order ...

  6. Depression following acute coronary syndrome

    DEFF Research Database (Denmark)

    Joergensen, Terese Sara Hoej; Maartensson, Solvej; Ibfelt, Else Helene

    2016-01-01

    .8 % developed a recurrent depression. Most patient characteristics (demographic factors, socioeconomic status, psychosocial factors, health-related behavioural factors, somatic comorbidities, and severity of acute coronary syndrome) were significantly associated with increased HRs for both early and later......PURPOSE: Depression is common following acute coronary syndrome, and thus, it is important to provide knowledge to improve prevention and detection of depression in this patient group. The objectives of this study were to examine: (1) whether indicators of stressors and coping resources were risk...... factors for developing depression early and later after an acute coronary syndrome and (2) whether prior depression modified these associations. METHODS: The study was a register-based cohort study, which includes 87,118 patients with a first time diagnosis of acute coronary syndrome during the period...

  7. Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

    Science.gov (United States)

    Attenhofer Jost, Christine H; Connolly, Heidi M; Scott, Christopher G; Ammash, Naser M; Bowen, Juan M; Schaff, Hartzell V

    2017-01-01

    The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

  8. Spontaneous Thrombosis of a Bicuspid Aortic valve due to Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Sarah Farrell

    2010-08-01

    Full Text Available We present the case of a 51-year-old man who was admitted as an emergency with spontaneous thrombosis of the aortic valve and ascending aorta. At operation he was found to have a congenitally bicuspid aortic valve and subsequent investigation revealed primary antiphospholipid syndrome. He underwent successful removal of the thrombus combined with mechanical replacement of the aortic valve.

  9. Relation between thoracic aortic inflammation and features of plaque vulnerability in the coronary tree in patients with non-ST-segment elevation acute coronary syndrome undergoing percutaneous coronary intervention. An FDG-positron emission tomography and optical coherence tomography study

    Energy Technology Data Exchange (ETDEWEB)

    Taglieri, Nevio; Ghetti, Gabriele; Saia, Francesco; Bacchi Reggiani, Maria Letizia; Rapezzi, Claudio [Alma Mater Studiorum Universita di Bologna, Istituto di Cardiologia, Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Bologna (Italy); Nanni, Cristina; Bonfiglioli, Rachele; Lima, Giacomo Maria; Fanti, Stefano [Alma Mater Studiorum Universita di Bologna, Istituto di Medicina Nucleare, Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Bologna (Italy); Marco, Valeria [CLI Foundation, Rome (Italy); Prati, Francesco [CLI Foundation, Rome (Italy); Ettore Sansavini Health Science Foundation, GVM Care and Research, Cotignola (Italy)

    2017-10-15

    To evaluate the relationship between aortic inflammation as assessed by {sup 18}F-fluorodeoxyglucose-positron emission tomography ({sup 18}F-FDG-PET) and features of plaque vulnerability as assessed by frequency domain-optical coherence tomography (FD-OCT). We enrolled 30 consecutive non-ST-segment elevation acute coronary syndrome patients undergoing percutaneous coronary intervention. All patients underwent three-vessel OCT before intervention and {sup 18}F-FDG-PET before discharge. Univariable and C-reactive protein (CRP)-adjusted linear regression analyses were performed between features of vulnerability [namely:lipid-rich plaques with and without macrophages and thin cap fibroatheromas (TCFA)] and {sup 18}F-FDG uptake in both ascending (AA) and descending aorta (DA) [measured either as averaged mean and maximum target-to-blood ratio (TBR) or as active slices (TBR{sub max} ≥ 1.6)]. Mean age was 62 years, and 26 patients were male. On univariable linear regression analysis TBR{sub mean} and TBR{sub max} in DA was associated with the number of lipid-rich plaques (β = 4.22; 95%CI 0.05-8.39; p = 0.047 and β = 3.72; 95%CI 1.14-6.30; p = 0.006, respectively). TBR{sub max} in DA was also associated with the number of lipid-rich plaques containing macrophages (β = 2.40; 95%CI 0.07-4.72; p = 0.044). A significant CRP adjusted linear association between the TBR{sub max} in DA and the number of lipid-rich plaques was observed (CRP-adjusted β = 3.58; 95%CI -0.91-6.25; p = 0.01). TBR{sub max} in DA showed a trend towards significant CRP-adjusted association with number of lipid-rich plaques with macrophages (CRP-adjusted β = 2.30; 95%CI -0.11-4.71; p = 0.06). We also observed a CRP-adjusted (β = 2.34; 95%CI 0.22-4.47; p = 0.031) linear association between the number of active slices in DA and the number of lipid-rich plaques. No relation was found between FDG uptake in the aorta and the number of TCFAs. In patients with first NSTEACS{sup ,} {sup 18}F-FDG uptake in

  10. Aortic wrapping for stanford type A acute aortic dissection: short and midterm outcome.

    Science.gov (United States)

    Demondion, Pierre; Ramadan, Ramzi; Azmoun, Alexandre; Raoux, François; Angel, Claude; Nottin, Rémi; Deleuze, Philippe

    2014-05-01

    Conventional surgical treatment of Stanford type A acute aortic dissection (AAD) is associated with considerable in-hospital mortality. As regards very elderly or high-risk patients with type A AAD, some may meet the criteria for less invasive surgery likely to prevent the complications associated with aortic replacement. We have retrospectively analyzed a cohort of patients admitted to our center for Stanford type A AAD and having undergone surgery between 2008 and 2012. The outcomes of the patients having had an aortic replacement under cardiopulmonary bypass (group A) have been compared with the outcomes of the patients who underwent off-pump wrapping of the ascending aorta (group B). Among the 54 patients admitted for Stanford type A AAD, 15 with a mean age of 77 years [46 to 94] underwent wrapping of the aorta. Regarding the new standard European system for cardiac operative risk evaluation (EuroSCORE II), the median result in our group B patients was 10.47 [5.02 to 30.07]. In-hospital mortality was 12.80% in group A and 6.6% in group B (p=0.66). For patients who underwent external wrapping of the ascending aorta, follow-up mortality rate was 13.3% with a median follow-up of 15 months [range 0 to 47]. The gold standard in cases of Stanford type A AAD consists of emergency surgical replacement of the dissected ascending aorta. In some cases in which the aortic root is not affected a less invasive surgical approach consisting of wrapping the dissected ascending aorta can be suggested as an alternative. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Marco Confalonieri

    2017-04-01

    Full Text Available Since its first description, the acute respiratory distress syndrome (ARDS has been acknowledged to be a major clinical problem in respiratory medicine. From July 2015 to July 2016 almost 300 indexed articles were published on ARDS. This review summarises only eight of them as an arbitrary overview of clinical relevance: definition and epidemiology, risk factors, prevention and treatment. A strict application of definition criteria is crucial, but the diverse resource-setting scenarios foster geographic variability and contrasting outcome data. A large international multicentre prospective cohort study including 50 countries across five continents reported that ARDS is underdiagnosed, and there is potential for improvement in its management. Furthermore, epidemiological data from low-income countries suggest that a revision of the current definition of ARDS is needed in order to improve its recognition and global clinical outcome. In addition to the well-known risk-factors for ARDS, exposure to high ozone levels and low vitamin D plasma concentrations were found to be predisposing circumstances. Drug-based preventive strategies remain a major challenge, since two recent trials on aspirin and statins failed to reduce the incidence in at-risk patients. A new disease-modifying therapy is awaited: some recent studies promised to improve the prognosis of ARDS, but mortality and disabling complications are still high in survivors in intensive care.

  12. Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Carmen Sílvia Valente Barbas

    2012-01-01

    Full Text Available This paper, based on relevant literature articles and the authors' clinical experience, presents a goal-oriented respiratory management for critically ill patients with acute respiratory distress syndrome (ARDS that can help improve clinicians' ability to care for these patients. Early recognition of ARDS modified risk factors and avoidance of aggravating factors during hospital stay such as nonprotective mechanical ventilation, multiple blood products transfusions, positive fluid balance, ventilator-associated pneumonia, and gastric aspiration can help decrease its incidence. An early extensive clinical, laboratory, and imaging evaluation of “at risk patients” allows a correct diagnosis of ARDS, assessment of comorbidities, and calculation of prognostic indices, so that a careful treatment can be planned. Rapid administration of antibiotics and resuscitative measures in case of sepsis and septic shock associated with protective ventilatory strategies and early short-term paralysis associated with differential ventilatory techniques (recruitment maneuvers with adequate positive end-expiratory pressure titration, prone position, and new extracorporeal membrane oxygenation techniques in severe ARDS can help improve its prognosis. Revaluation of ARDS patients on the third day of evolution (Sequential Organ Failure Assessment (SOFA, biomarkers and response to infection therapy allows changes in the initial treatment plans and can help decrease ARDS mortality.

  13. Aortic aneurysm and craniosynostosis in a family with Cantu syndrome.

    Science.gov (United States)

    Hiraki, Yoko; Miyatake, Satoko; Hayashidani, Michiko; Nishimura, Yutaka; Matsuura, Hiroo; Kamada, Masahiro; Kawagoe, Takuji; Yunoki, Keiji; Okamoto, Nobuhiko; Yofune, Hiroko; Nakashima, Mitsuko; Tsurusaki, Yoshinori; Satisu, Hirotomo; Murakami, Akira; Miyake, Noriko; Nishimura, Gen; Matsumoto, Naomichi

    2014-01-01

    Cantu syndrome is an autosomal dominant overgrowth syndrome associated with facial dysmorphism, congenital hypertrichosis, and cardiomegaly. Some affected individuals show bone undermodeling of variable severity. Recent investigations revealed that the disorder is caused by a mutation in ABCC9, encoding a regulatory SUR2 subunit of an ATP-sensitive potassium channel mainly expressed in cardiac and skeletal muscle as well as vascular smooth muscle. We report here on a Japanese family with this syndrome. An affected boy and his father had a novel missense mutation in ABCC9. Each patient had a coarse face and hypertrichosis. However, cardiomegaly was seen only in the boy, and macrosomia only in the father. Skeletal changes were not evident in either patient. Craniosynostosis in the boy and the development of aortic aneurysm in the father are previously undescribed associations with Cantu syndrome. © 2013 Wiley Periodicals, Inc.

  14. Large aortic aneurysm and dissection in a patient with Marfan's syndrome

    Directory of Open Access Journals (Sweden)

    Fernando Pivatto Júnior

    2015-04-01

    Full Text Available Marfan’s syndrome is an autosomal dominant disorder of connective tissue affecting approximately1 in5000 people. In individuals with this syndrome, more than 90% of deaths from known causes result from cardiovascular complications, such as aortic dissection, aortic regurgitation, and congestive cardiac failure. In this report, we present a patient with a large symptomatic aortic aneurysm and chronic dissection, severe aortic regurgitation and cardiomegaly, treated successfully with resection of the proximal aorta and placement of a mechanic aortic valved graft.

  15. Aortic plaque rupture in the setting of acute lower limb ischemia.

    LENUS (Irish Health Repository)

    O'Donnell, David H

    2012-02-01

    Acute aortic plaque rupture is an uncommon cause of acute lower limb ischemia. The authors report sequence computed tomographic imaging of a distal aortic plaque rupture in a young man with bilateral lower limb complications. Clinical awareness, prompt recognition and imaging, and appropriate treatment of this uncommon condition are necessary to improve patient outcomes.

  16. Acute aortic dissection in patient with suspected pheochromocytoma

    Directory of Open Access Journals (Sweden)

    Lešanović Jelena

    2016-01-01

    Full Text Available Introduction: Aortic dissection is one of the most fatal vascular emergencies. Almost 40% of the patients do not reach hospital in time while more than quarter die in the first 24 hours after the dissection begins. Case Report: A 37-year old man was admitted to our hospital with severe anterior chest pain which had lasted for over a week. Suspected aortic dissection was rapidly confirmed using imaging modalities - MDCT chest scan and TTE, followed by an urgent surgical management - Bentall procedure. MDCT chest scan also discovered adrenal incidentaloma defined as malignant, pheochromocytoma like mass. Due to the critical state of the patient, there was not enough time for further endocrinologic testing. Discussion and conclusion: When treating patients with pheochromocytoma and acute aortic disection, it is crucial to obtain a stable hemodynamic state before the surgery, since they can trigger a severe hypertensive crisis due to high levels of cathecholamines induced chronic vasoconctriction. The most vulnerable periods are the induction of anesthesia and perioperative hemodynamic oscillations, so treating patients with short acting alpha- 1 adrenergic blocking agents preoperatively has proven to be helpful - Phentolamine. Both dissection of aorta and pheochromocytoma present challenges for anesthesiologists and early recognition of symptoms is essential in establishing the diagnosis and reducing the mortality rate.

  17. A rare case of discrete aortic coarctation in Williams-Beuren syndrome. Diagnostic and therapeutic considerations

    OpenAIRE

    Savina Mannarino; Eitan Keizman; Michele Pasotti; Alessia Claudia Codazzi; Elisabetta De Sando; Alessandro Giamberti

    2015-01-01

    Williams-Beuren syndrome (WBS) is a genetic disorder caused by elastin gene deletions, and is characterized by cardiovascular malformations, primarily including supravalvular aortic stenosis and peripheral pulmonary stenosis. We report a case of a neonate who developed severe discrete aortic coarctation, underwent multiple surgical interventions, and was subsequently diagnosed with WBS. Severe discrete aortic coarctation is a rare event in WBS newborns. An abnormally thick aortic wall is pres...

  18. Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome

    Directory of Open Access Journals (Sweden)

    Hyung-Tae Sim

    2015-08-01

    Full Text Available Loeys-Dietz syndrome (LDS is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

  19. The acute radiation syndrome

    International Nuclear Information System (INIS)

    Souhami Filho, L.

    1985-01-01

    Symptoms and signs from medical aspects resulting from whole body exposure, or in the main part, to ionizing radiation are described. The dose-response relationship is studied and the exposure is divided in three parts: central nervous system syndrome, gastrointestinal syndrome and hematopoietic syndrome. Brief comments about the treatment are reported. (M.A.C.) [pt

  20. Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.

    Science.gov (United States)

    Izgi, Cemil; Nyktari, Evangelia; Alpendurada, Francisco; Bruengger, Annina Studer; Pepper, John; Treasure, Tom; Mohiaddin, Raad

    2015-10-15

    Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV). A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up. PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass. Crown Copyright © 2015. Published by Elsevier Ireland Ltd. All rights reserved.

  1. New paradigms in the management of acute type B aortic dissection.

    Science.gov (United States)

    Parisi, Rosario; Secco, Gioel Gabrio; Fattori, Rossella

    2015-11-01

    Type B aortic dissection is a relatively uncommon and multifaceted disease, whose management is ongoing debated. Its wide range of clinical presentations and anatomical features hamper the early identification and medical management. In the past few years, the introduction of endovascular techniques opened new paradigms in comprehension and management of aortic diseases. Aim of this review is to discuss contemporary therapeutic approaches of acute type B aortic dissections highlighting the growing role of thoracic endovascular aortic repair (TEVAR) in focusing its complex physiopathology. Prompt medical therapy followed by endovascular repair should be considered as the gold standard in complicated acute type B aortic dissection. Moreover, recent findings also suggest a potential benefit in case of uncomplicated cases. Management of acute type B aortic dissection is progressively shifting into endovascular approach. However, further studies are warranted to define the optimal treatment strategy in each subset of patients and anatomical features.

  2. Clinical and genetic aspects of Marfan syndrome and familial thoracic aortic aneurysms and dissections

    NARCIS (Netherlands)

    Hilhorst-Hofstee, Yvonne

    2013-01-01

    This thesis concerns the clinical and genetic aspects of familial thoracic aortic aneurysms and dissections, in particular in Marfan syndrome. It includes the Dutch multidisciplinary guidelines for diagnosis and management of Marfan syndrome. These guidelines contain practical directions for

  3. Determination of entry site for acute type A aortic dissection by initial enhanced CT-scan

    Energy Technology Data Exchange (ETDEWEB)

    Mase, Takenori; Narumiya, Chihiro; Aoyama, Takahiko; Nagata, Yoshihisa [Aichi Medical Univ., Nagakute (Japan). School of Medicine

    2002-01-01

    Acute type A aortic dissection presents a surgical emergency because conservative therapy is not effective in the majority of instances. Enhanced CT-scan of the chest is commonly available and is considered to be an optimal diagnostic method for this disease. The operative strategy is to resect the primary tear to close the entry site of the aortic dissection and replace it with a tubular Dacron graft. Therefore, the existence of the entry site is important in determining the operative procedure. Based on the numerical value of the enhanced CT-scan inspection, the present study seeks to preoperatively identify the location of the presumed entry site in aortic dissection. From May 1996 to June 1999, 21 consecutive patients (Marfan's syndrome excluded) with acute type A aortic dissection underwent surgical treatment. Nineteen patients were preoperatively examined by enhanced CT-scan: 11 men and 8 women, with a mean age of 61 years. CT-scan slices used for early diagnosis were of the ascending aorta, aortic arch, descending aorta, and thoracoabdominal aorta. The largest diameters of the whole and true lumen were measured from cross-sectional aortic images with a personal computer, and the areas of the whole and true lumen were obtained by the manual tracing method. The true ratio was calculated for the largest diameter and area of the whole lumen. The nineteen patients were divided into two groups according to the location of the entry site based on the operating views. Seven patients with the entry site in the ascending aorta were classified as group A, and twelve patients with the entry site further in the aortic arch and descending aorta were classified as group B. Comparisons were performed by non-parametric analysis. Moreover, a discriminant analysis was applied to evaluate the classification between the two groups. The ratio of the largest diameter of the true lumen in group A at the level of the ascending and descending aorta was significantly greater than

  4. Determination of entry site for acute type A aortic dissection by initial enhanced CT-scan

    International Nuclear Information System (INIS)

    Mase, Takenori; Narumiya, Chihiro; Aoyama, Takahiko; Nagata, Yoshihisa

    2002-01-01

    Acute type A aortic dissection presents a surgical emergency because conservative therapy is not effective in the majority of instances. Enhanced CT-scan of the chest is commonly available and is considered to be an optimal diagnostic method for this disease. The operative strategy is to resect the primary tear to close the entry site of the aortic dissection and replace it with a tubular Dacron graft. Therefore, the existence of the entry site is important in determining the operative procedure. Based on the numerical value of the enhanced CT-scan inspection, the present study seeks to preoperatively identify the location of the presumed entry site in aortic dissection. From May 1996 to June 1999, 21 consecutive patients (Marfan's syndrome excluded) with acute type A aortic dissection underwent surgical treatment. Nineteen patients were preoperatively examined by enhanced CT-scan: 11 men and 8 women, with a mean age of 61 years. CT-scan slices used for early diagnosis were of the ascending aorta, aortic arch, descending aorta, and thoracoabdominal aorta. The largest diameters of the whole and true lumen were measured from cross-sectional aortic images with a personal computer, and the areas of the whole and true lumen were obtained by the manual tracing method. The true ratio was calculated for the largest diameter and area of the whole lumen. The nineteen patients were divided into two groups according to the location of the entry site based on the operating views. Seven patients with the entry site in the ascending aorta were classified as group A, and twelve patients with the entry site further in the aortic arch and descending aorta were classified as group B. Comparisons were performed by non-parametric analysis. Moreover, a discriminant analysis was applied to evaluate the classification between the two groups. The ratio of the largest diameter of the true lumen in group A at the level of the ascending and descending aorta was significantly greater than that

  5. Life-threatening subdural hematoma after aortic valve replacement in a patient with Heyde syndrome: a case report.

    Science.gov (United States)

    Uchida, Tetsuro; Hamasaki, Azumi; Ohba, Eiichi; Yamashita, Atsushi; Hayashi, Jun; Sadahiro, Mitsuaki

    2017-08-08

    Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding. On the other hand, intracranial hemorrhage complicating Heyde syndrome is extremely rare. A 77-year-old woman presented to our hospital with severe aortic stenosis and severe anemia due to gastrointestinal bleeding and was diagnosed with Heyde syndrome. Although aortic valve replacement was performed without recurrent gastrointestinal bleeding, postoperative life-threatening acute subdural hematoma occurred with a marked midline shift. Despite prompt surgical evacuation of the hematoma, she did not recover consciousness and she died 1 month after the operation. Postoperative subdural hematoma is rare, but it should be kept in mind as a devastating hemorrhagic complication, especially in patients with Heyde syndrome.

  6. 5B.05: MARFAN SYNDROME: ASSESSMENT OF AORTIC DISSECTION RISK BY ANALYSIS OF AORTIC VISCOELASTIC PROPERTIES.

    Science.gov (United States)

    Grillo, A; Pini, A; Marelli, S; Gan, L; Giuliano, A; Trifirò, G; Santini, F; Salvi, L; Salvi, P; Viecca, F; Carretta, R; Parati, G

    2015-06-01

    Marfan syndrome is an autosomal dominant genetic disorder characterized by an abnormal fibrillin-1 synthesis. Aortic root dilation and dissection are the main problems affecting patients prognosis in these patients. Their pharmacological prophylaxis with losartan or with a beta-blocker counteracts the aortic root dilation, but a close follow-up is required to assess therapeutic response rate and to identify non-responders. Unfortunately genotype-phenotype studies do not allow to determine the exact risk profile in these patients and there is no reliable method to accurately predict their risk of aortic dissection. Aim of this study was to evaluate non-invasive markers for identification of Marfan patients at higher risk of aortic complications. We studied 187 Marfan patients (identified according to 2010 Revised Ghent Criteria and positive genetic analysis), age 32.3 ± 16.5 yrs (mean ± SD). 52 patients (27.8%) had undergone surgical ascending aorta replacement (David or Bentall procedure). Central pressure curves were recorded by PulsePen tonometer, and the aortic viscoelastic aortic properties were studied by determination of carotid-femoral pulse wave velocity (PWV). With reference to the age related distribution of PWV values in a normal population, defined according to Arterial-Stiffness-Collaboration, PWV mean values in Marfan patients corresponded to 60th percentile in non- operated patients and to the 67th percentile in those operated. Adult Marfan patients (n = 146) generally displayed a low blood pressure, because of the pharmacological prophylaxis, and were compared with a population of 189 adult healthy subjects (81 males), matched by age (38 ± 13 vs 38 ± 16 yrs.), heart rate (64 ± 9 bpm vs 64 ± 11 bpm) and blood pressure (mean BP = 78 ± 9 mmHg vs 79 ± 4 mmHg) values. Average PWV value was higher than in healthy controls (PWV = 7.0 ± 1.7) both in not operated (PWV = 7.6 ± 1.6; p = 0

  7. Comparison of aortic root replacement in patients with Marfan syndrome.

    Science.gov (United States)

    Bernhardt, Alexander M J; Treede, Hendrik; Rybczynski, Meike; Sheikzadeh, Sara; Kersten, Jan F; Meinertz, Thomas; von Kodolitsch, Yskert; Reichenspurner, Hermann

    2011-11-01

    Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in mid-term and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n=48) or aortic dissection type A (n=10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4-4.2) years. In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p=0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p=0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p=0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p=0.086), especially after aneurysm (p=0.057). After 10 years, freedom from aortic regurgitation ≥II° in the AVS group was 80% for aneurysm and 50% after dissection (p=0.524). The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients. Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights

  8. Rupture of an abdominal aortic aneurysm in a young man with Marfan Syndrome

    DEFF Research Database (Denmark)

    Pedersen, Maria Weinkouff; Huynh, Khiem Dinh; Baandrup, Ulrik Thorngren

    2018-01-01

    Abdominal aortic aneurysms are very rare in Marfan syndrome. We present a case with a young non-smoking and normotensive male with Marfan syndrome, who developed an infrarenal abdominal aortic aneurysm that presented with rupture to the retroperitoneal cavity causing life-threatening bleeding shock...

  9. Aortic root pathology in Marfan syndrome increases the risk of migraine with aura

    DEFF Research Database (Denmark)

    Koppen, H; Vis, J C; Gooiker, D J

    2012-01-01

    To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor.......To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor....

  10. Mutations in a TGF-β Ligand, TGFB3, Cause Syndromic Aortic Aneurysms and Dissections

    NARCIS (Netherlands)

    A.M. Bertoli Avella (Aida); E. Gillis (Elisabeth); H. Morisaki (Hiroko); J.M.A. Verhagen (Judith ); B.M. de Graaf (Bianca); G. Van De Beek (Gerarda); E. Gallo (Elena); B.P.T. Kruithof (Boudewijn); H. Venselaar (Hanka); L.A. Myers (Loretha); S. Laga (Steven); A.J. Doyle (Alexander); G. Oswald (Gretchen); W.A. van Cappellen (Gert); I. Yamanaka (Itaru); R.M. van der Helm (Robert); H.B. Beverloo (Berna); J.E.M.M. de Klein (Annelies); L.M. Pardo (Luba); M. Lammens (Martin); C. Evers (Christina); K. Devriendt (Koenraad); M. Dumoulein (Michiel); J.M. Timmermans (Janneke); H.T. Brüggenwirth (Hennie); F.W. Verheijen (Frans); I. Rodrigus (Inez); G. Baynam (Gareth); M.J.E. Kempers (Marlies); J. Saenen (Johan); E.M. Van Craenenbroeck (Emeline); K. Minatoya (Kenji); R. Matsukawa (Ritsu); T. Tsukube (Takuro); N. Kubo (Noriaki); R.M.W. Hofstra (Robert); M.-J. Goumans (Marie-José); J.A. Bekkers (Jos); J.W. Roos-Hesselink (Jolien); I.M.B.H. van de Laar (Ingrid); H.C. Dietz (Harry ); L. van Laer (Lut); T. Morisaki (Takayuki); M.W. Wessels (Marja); B.L. Loeys (Bart)

    2015-01-01

    markdownabstractBACKGROUND Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes,

  11. Mutations in a TGF-beta ligand, TGFB3, cause syndromic aortic aneurysms and dissections

    NARCIS (Netherlands)

    Bertoli-Avella, A.M.; Gillis, E.; Morisaki, H.; Verhagen, J.M.A.; Graaf, B.M. de; Beek, G. van de; Gallo, E.; Kruithof, B.P.; Venselaar, H.; Myers, L.A.; Laga, S.; Doyle, A.J.; Oswald, G.; Cappellen, G.W. van; Yamanaka, I.; Helm, R.M. van der; Beverloo, B.; Klein, A.; Pardo, L.; Lammens, M.; Evers, C.; Devriendt, K.; Dumoulein, M.; Timmermans, J.; Bruggenwirth, H.T.; Verheijen, F.; Rodrigus, I.; Baynam, G.; Kempers, M.; Saenen, J.; Craenenbroeck, E.M. Van; Minatoya, K.; Matsukawa, R.; Tsukube, T.; Kubo, N.; Hofstra, R.; Goumans, M.J.; Bekkers, J.A.; Roos-Hesselink, J.W.; Laar, I.M. van de; Dietz, H.C.; Laer, L. Van; Morisaki, T.; Wessels, M.W.; Loeys, B.L.

    2015-01-01

    BACKGROUND: Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed

  12. Progressive Supranuclear Palsy-like Syndrome After Aortic Aneurysm Repair: A Case Series

    Directory of Open Access Journals (Sweden)

    Sirisha Nandipati

    2013-12-01

    Full Text Available The syndrome of progressive supranuclear palsy‐like syndrome is a rare complication of ascending aortic aneurysm repair. We report two patients with videos and present a table of prior reported cases. To our knowledge there is no previously published video of this syndrome. The suspected mechanism is brainstem injury though neuroimaging is often negative for an associated infarct. We hope our report will increase recognition of this syndrome after aortic surgery, especially in patients with visual complaints.

  13. Mild aerobic exercise blocks elastin fiber fragmentation and aortic dilatation in a mouse model of Marfan syndrome associated aortic aneurysm.

    Science.gov (United States)

    Gibson, Christine; Nielsen, Cory; Alex, Ramona; Cooper, Kimbal; Farney, Michael; Gaufin, Douglas; Cui, Jason Z; van Breemen, Cornelis; Broderick, Tom L; Vallejo-Elias, Johana; Esfandiarei, Mitra

    2017-07-01

    Regular low-impact physical activity is generally allowed in patients with Marfan syndrome, a connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene. However, being above average in height encourages young adults with this syndrome to engage in high-intensity contact sports, which unfortunately increases the risk for aortic aneurysm and rupture, the leading cause of death in Marfan syndrome. In this study, we investigated the effects of voluntary (cage-wheel) or forced (treadmill) aerobic exercise at different intensities on aortic function and structure in a mouse model of Marfan syndrome. Four-week-old Marfan and wild-type mice were subjected to voluntary and forced exercise regimens or sedentary lifestyle for 5 mo. Thoracic aortic tissue was isolated and subjected to structural and functional studies. Our data showed that exercise improved aortic wall structure and function in Marfan mice and that the beneficial effect was biphasic, with an optimum at low intensity exercise (55-65% V̇o 2max ) and tapering off at a higher intensity of exercise (85% V̇o 2max ). The mechanism underlying the reduced elastin fragmentation in Marfan mice involved reduction of the expression of matrix metalloproteinases 2 and 9 within the aortic wall. These findings present the first evidence of potential beneficial effects of mild exercise on the structural integrity of the aortic wall in Marfan syndrome associated aneurysm. Our finding that moderate, but not strenuous, exercise protects aortic structure and function in a mouse model of Marfan syndrome could have important implications for the medical care of young Marfan patients. NEW & NOTEWORTHY The present study provides conclusive scientific evidence that daily exercise can improve aortic health in a mouse model of Marfan syndrome associated aortic aneurysm, and it establishes the threshold for the exercise intensity beyond which exercise may not be as protective. These findings establish a platform

  14. Hospital volumes and later year of operation correlates with better outcomes in acute Type A aortic dissection

    DEFF Research Database (Denmark)

    Geirsson, Arnar; Ahlsson, Anders; Franco-Cereceda, Anders

    2018-01-01

    OBJECTIVES: Acute Type A aortic dissection remains a life-threatening disease, but there are indications that its surgical mortality is decreasing. The aim of this report was to study how surgical mortality has changed and what influences those changes. METHODS: Nordic Consortium for Acute Type...... circulatory arrest was used in 85% of cases and its use increased significantly throughout the study. The 30-day mortality decreased from 24% in 2005 to 13% in 2014 ( P  = 0.003). Independent predictors for 30-day mortality were preoperative cardiac arrest, malperfusion syndrome, Penn Class C, Penn Class B...

  15. Pathogenetic Basis of Aortopathy and Aortic Valve Disease

    Science.gov (United States)

    2018-02-19

    Aortopathies; Thoracic Aortic Aneurysm; Aortic Valve Disease; Thoracic Aortic Disease; Thoracic Aortic Dissection; Thoracic Aortic Rupture; Ascending Aortic Disease; Descending Aortic Disease; Ascending Aortic Aneurysm; Descending Aortic Aneurysm; Marfan Syndrome; Loeys-Dietz Syndrome; Ehlers-Danlos Syndrome; Shprintzen-Goldberg Syndrome; Turner Syndrome; PHACE Syndrome; Autosomal Recessive Cutis Laxa; Congenital Contractural Arachnodactyly; Arterial Tortuosity Syndrome

  16. Decreased aortic growth and middle aortic syndrome in patients with neuroblastoma after radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Sutton, Elizabeth J. [Harvard University, Department of Radiology, Mount Auburn Hospital, Cambridge, MA (United States); University of California, San Francisco, Department of Radiology, San Francisco, CA (United States); Tong, Ricky T. [Stanford University, Department of Medicine, Palo Alto, CA (United States); Gillis, Amy M.; Haas-Kogan, Daphne A. [University of California, San Francisco, Department of Radiation Oncology, San Francisco, CA (United States); Henning, Tobias D.; Boddington, Sophie; Sha, Vinil; Gooding, Charles; Coakley, Fergus V.; Daldrup-Link, Heike [University of California, San Francisco, Department of Radiology, San Francisco, CA (United States); Weinberg, Vivian A. [University of California, San Francisco, Comprehensive Cancer Center, Biostatistics Core, San Francisco, CA (United States); Matthay, Katherine [University of California, San Francisco, Department of Pediatrics, San Francisco, CA (United States)

    2009-11-15

    Long-term CT follow-up studies are required in pediatric patients who have received intraoperative radiation therapy (IORT) and external beam radiation therapy (EBRT) to assess vascular toxicities and to determine the exact complication rate. To analyze with CT the effects of radiation therapy (RT) on the growth of the aorta in neuroblastoma patients. Abdominal CT scans of 31 patients with intraabdominal neuroblastoma (stage II-IV), treated with RT (20 IORT{+-}EBRT, 11 EBRT alone), were analyzed retrospectively. The diameter of the abdominal aorta was measured before and after RT. These data were compared to normal and predicted normal aortic diameters of children, according to the model of Fitzgerald, Donaldson and Poznanski (aortic diameter in centimeters = 0.844+0.0599 x age in years), and to the diameters of a control group of children who had not undergone RT. Statistical analyses for the primary aims were performed using the chi-squared test, t-test, Mann-Whitney test, nonparametric Wilcoxon matched-pairs test and analysis of variance for repeated measures. Clinical files and imaging studies were evaluated for signs of late vascular complications of neuroblastoma patients who had received RT. The mean diameter before and after RT and the growth of the aorta were significantly lower than expected in patients with neuroblastoma (P<0.05 for each) and when compared to the growth in a control group with normal and nonirradiated aortas. Among the patients who had received RT, there was no difference due to the type of RT. Seven patients from the IORT{+-}EBRT group developed vascular complications, which included hypertension (five), middle aortic syndrome (two), death due to mesenteric ischemia (one) and critical aortic stenosis, which required aortic bypass surgery (two). Patients with neuroblastoma who had received RT showed impaired growth of the abdominal aorta. Significant long-term vascular complications occurred in seven patients who received IORT

  17. Metabolic syndrome in acute coronary syndrome

    International Nuclear Information System (INIS)

    Bhalli, M.A.; Aamir, M.; Mustafa, G.

    2011-01-01

    Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

  18. Impact of the entry site on late outcome in acute Stanford type B aortic dissection†.

    Science.gov (United States)

    Kitamura, Tadashi; Torii, Shinzo; Oka, Norihiko; Horai, Tetsuya; Itatani, Keiichi; Yoshii, Takeshi; Nakamura, Yuki; Shibata, Miyuki; Tamura, Tomoki; Araki, Haruna; Matsunaga, Yoshikiyo; Sato, Hajime; Miyaji, Kagami

    2015-11-01

    This study aimed to investigate whether the entry site of acute type B aortic dissection affects late outcomes. Inpatient and outpatient records were retrospectively reviewed. We identified 224 cases of acute type B aortic dissection between 1998 and 2013. Of these 224 patients, 168 were men and the age was 64.2 ± 12.6 (range 23-94) years, from which 130 presented with the entry at a location downstream of the distal aortic arch, 67 with the entry at the outer curvature of the distal aortic arch and 27 with the entry at the inner curvature. At the initial presentation, 127 patients had descending false lumen thrombosis. The 30-day mortality rate was 2%, and 8% of patients had malperfusion. The entry at the outer curvature was associated with a higher risk of 30-day mortality. Patients with the entry at a location downstream were significantly older, and had a higher chance for primarily thrombosed descending false lumen and a lower risk of malperfusion. At follow-up (6.0 ± 4.1 years), the actuarial survival rates were 97, 83 and 60%, freedoms from open aortic surgery were 96, 91 and 86%, aortic intervention were 73, 66 and 63% and aortic events were 71, 60 and 52% at 1, 5 and 10 years, respectively. Multivariate logistic regression analysis revealed that the outer curvature entry and maximum aortic diameter were correlated with open aortic surgery, aortic intervention and aortic events. Of the 127 patients with primarily thrombosed false lumen, the outer curvature entry was significantly correlated with aortic events. The primary entry at the outer curvature of the distal aortic arch, as well as the large aortic diameter, is associated with a higher risk of late open aortic surgery, aortic intervention and aortic events in acute type B aortic dissection. Thus, the entry site should be taken into consideration in the establishment of an appropriate treatment indication of type B aortic dissection. © The Author 2015. Published by Oxford University Press on behalf

  19. Reimplantation valve-sparing aortic root replacement in Marfan syndrome using the Valsalva conduit: an intercontinental multicenter study.

    Science.gov (United States)

    Settepani, Fabrizio; Szeto, Wilson Y; Pacini, Davide; De Paulis, Ruggero; Chiariello, Luigi; Di Bartolomeo, Roberto; Gallotti, Roberto; Bavaria, Joseph E

    2007-02-01

    Introduced by DePaulis in 2000, the Gelweave Valsalva graft (Sulzer Vascutek, Refrewshire, Scotland) is a modified Dacron conduit (DuPont, Wilmington, DE), with prefashioned sinuses of Valsalva. The aim of this study was to evaluate the mid-term results of the reimplantation valve-sparing aortic root replacement using the Gelweave Valsalva prosthesis in Marfan syndrome patients. A retrospective review was performed of 35 patients with Marfan syndrome in four centers who underwent the reimplantation valve-sparing aortic root replacement using the Gelweave Valsalva prosthesis. The patients were predominantly men, with a mean age of 36.5 +/- 12.6 years (range, 14 to 62 years). Two patients presented with acute type A dissections and underwent emergent operations. Elective hemiarch reconstruction using hypothermic circulatory arrest was required in 11 patients. Aortic valve cusp repair was performed in 2 patients. There were no operative or hospital deaths, and no patients died during follow-up. The mean follow-up was 19 months (range, 1 to 60 months). Significant (>2+) aortic insufficiency (AI), requiring aortic valve replacement, developed in 3 patients during follow-up that requiring aortic valve replacement. The 5-year freedom from reoperation owing to structural valve deterioration was 88.9% +/- 8.1%. There were no episodes of clinically significant thromboembolism. Reimplantation valve-sparing aortic root replacement with the Gelweave Valsalva prosthesis in Marfan patients provides satisfactory mid-term results, thus encouraging further use of this type of repair. However, long-term results are needed in order to define the durability of this technique.

  20. Unusual presenting of acute aortic dissection due to penetrating atheromatous ulcer.

    Science.gov (United States)

    Atas, Halil; Durmus, Erdal; Sunbul, Murat; Birkan, Yasar; Ozben, Beste

    2014-07-01

    Penetrating atheromatous ulcer (PAU) is an atherosclerotic ulcer penetrating the internal elastic lamina of the aortic wall causing a hematoma within the media layer of aorta. They are commonly located in the descending aorta of the elderly and hypertensive patients. They may rarely be complicated by aortic dissection. We report a relative young normotensive patient presenting with acute aortic dissection due to PAU located in the ascending aorta.

  1. Risk factors for prolonged mechanical ventilation after total aortic arch replacement for acute DeBakey type I aortic dissection.

    Science.gov (United States)

    Li, Cheng-Nan; Chen, Lei; Ge, Yi-Peng; Zhu, Jun-Ming; Liu, Yong-Min; Zheng, Jun; Liu, Wei; Ma, Wei-Guo; Sun, Li-Zhong

    2014-09-01

    EuroSCORE II is an objective risk scoring model. The aim of this study was to assess the performance of EuroSCORE II in the prediction of prolonged mechanical ventilation following total aortic arch replacement for acute DeBakey type I aortic dissection and evaluate the risk factors for prolonged mechanical ventilation. Between February 2009 to February 2012, data from 240 patients who underwent total aortic arch replacement for acute DeBakey type I aortic dissection were collected retrospectively. Mechanical ventilation after the surgery longer than 48 hours was defined as postoperative prolonged mechanical ventilation. EuroSCORE II was applied to predict prolonged mechanical ventilation. A C statistic (receiver operating characteristic curve) was used to test discrimination of the model. Calibration was assessed with a Hosmer-Lemeshow goodness-of-fit statistic. Multiple logistic regression analysis was used to identify the final risk factors of prolonged mechanical ventilation. The overall mortality was 10%. The mean length of mechanical ventilation after total aortic arch replacement was 42.72 ± 51.45 hours. Total 74 patients needed prolonged mechanical ventilation. EuroSCORE II showed poor discriminatory ability (C statistic 0.52) and calibration (Hosmer-Lemeshow, pmechanical ventilation. On multivariate analysis, independent risk factors for postoperative prolonged mechanical ventilation were age ≥ 48.5 years (pmechanical ventilation following total aortic arch replacement for acute DeBakey type I aortic dissection. Preoperative high level of leukocyte, age and surgical period from symptom onset are risk factors for prolonged mechanical ventilation. Copyright © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  2. Prophylactic aortic root surgery in patients with Marfan syndrome : 10 years' experience with a protocol based on body surface area

    NARCIS (Netherlands)

    Aalberts, Jan J. J.; van Tintelen, J. Peter; Hillege, Hans L.; Boonstra, Piet W.; van den Berg, Maarten P.; Waterbolk, T

    Background: Current guidelines recommending prophylactic aortic root replacement in Marfan syndrome are based on absolute diameters of the aortic root. However, aortic root diameter is a function of body surface area (BSA). Here, we report our experience with a protocol for prophylactic aortic root

  3. Acute respiratory distress syndrome

    Science.gov (United States)

    ... edema; Increased-permeability pulmonary edema; ARDS; Acute lung injury ... RS, Gausche-Hill M, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice . 9th ed. Philadelphia, PA: Elsevier; ...

  4. Platypnea-Orthodeoxia Syndrome after Transcatheter Aortic Valve Implantation

    Directory of Open Access Journals (Sweden)

    Andrew K. Roy

    2016-01-01

    Full Text Available Progressive dyspnea and hypoxaemia in the subacute phase after transcatheter aortic valve implantation (TAVI are uncommon and warrant immediate assessment of valve and prosthesis leaflet function to exclude thrombosis, as well as investigation for other causes related to the procedure, such as left ventricular dysfunction, pulmonary embolism, and respiratory sepsis. In this case, we report the observation of a patient presenting two weeks after TAVI with arterial hypoxaemia in an upright position, relieved by lying flat, and coupled with an intracardiac shunt detected on echocardiography in the absence of pulmonary hypertension, raising the suspicion of Platypnea-Orthodeoxia Syndrome (POS. Invasive intracardiac haemodynamic assessment showed a significant right-to-left shunt (Qp/Qs = 0.74, which confirmed the diagnosis, with subsequent closure of the intracardiac defect resulting in immediate relief of symptoms and hypoxaemia. To our knowledge, this is the first reported case of an interatrial defect and shunt causing Platypnea-Orthodeoxia Syndrome after transcatheter aortic valve implantation, resolved by percutaneous device closure.

  5. Severe Acute Respiratory Syndrome- SARS

    Indian Academy of Sciences (India)

    Table of contents. Severe Acute Respiratory Syndrome- SARS · PowerPoint Presentation · Slide 3 · Global pattern of SARS epidemic · Slide 5 · SARS – clinical features · Radiological features of lungs-showing progression of disease · cT Scan of SARS lungs · Imaging type,cost,therapy · SARS – Lung Pathology.

  6. Does altered aortic flow in marfan syndrome relate to aortic root dilatation?

    Science.gov (United States)

    Wang, Hung-Hsuan; Chiu, Hsin-Hui; Tseng, Wen-Yih Isaac; Peng, Hsu-Hsia

    2016-08-01

    To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05-0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R(2)  = 0.50-0.72) in MFS patients. The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow-up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500-508. © 2016 Wiley Periodicals, Inc.

  7. Components of the metabolic syndrome, but not the metabolic syndrome per se, are associated with aortic distensibility.

    Science.gov (United States)

    Tentolouris, Nicholas; Papazafiropoulou, Athanasia; Liatis, Stavros; Moyssakis, Ioannis; Perrea, Despoina; Soldatos, Rigas-Philippos; Katsilambros, Nicholas

    2007-12-01

    Reduction in aortic distensibility occurs early in the atherosclerosis process and carries a poor prognosis. Metabolic syndrome is common and it is associated with increased cardiovascular mortality. The aim of this cross-sectional study was to investigate the association between metabolic syndrome and aortic distensibility. A total of 135 subjects without diabetes were studied. Metabolic syndrome was diagnosed using the NCEP-ATP-III criteria. Aortic distensibility was assessed non-invasively by ultrasonography. Multivariate analysis, after controlling for the components of the metabolic syndrome, and, additionally, for body mass index, pulse pressure, presence of coronary artery disease, use of statins and use of angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers, demonstrated an independent association between aortic distensibility and age (pmetabolic syndrome status. Moreover, there was a suggestive association with albumin-to-creatinine ratio (p=0.06). Metabolic syndrome per se is not associated with reduction in aortic distensibility. From the components of the metabolic syndrome, only blood pressure is a strong predictor of aortic distensibility. In addition, ageing and higher values of albumin-to-creatinine ratio are also associated with low aortic distensibility.

  8. Beta-blockers for preventing aortic dissection in Marfan syndrome.

    Science.gov (United States)

    Koo, Hyun-Kyoung; Lawrence, Kendra Ak; Musini, Vijaya M

    2017-11-07

    Marfan syndrome is a hereditary disorder affecting the connective tissue and is caused by a mutation of the fibrillin-1 (FBN1) gene. It affects multiple systems of the body, most notably the cardiovascular, ocular, skeletal, dural and pulmonary systems. Aortic root dilatation is the most frequent cardiovascular manifestation and its complications, including aortic regurgitation, dissection and rupture are the main cause of morbidity and mortality. To assess the long-term efficacy and safety of beta-blocker therapy as compared to placebo, no treatment or surveillance only in people with Marfan syndrome. We searched the following databases on 28 June 2017; CENTRAL, MEDLINE, Embase, Science Citation Index Expanded and the Conference Proceeding Citation Index - Science in the Web of Science Core Collection. We also searched the Online Metabolic and Molecular Bases of Inherited Disease (OMMBID), ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) on 30 June 2017. We did not impose any restriction on language of publication. All randomised controlled trials (RCTs) of at least one year in duration assessing the effects of beta-blocker monotherapy compared with placebo, no treatment or surveillance only, in people of all ages with a confirmed diagnosis of Marfan syndrome were eligible for inclusion. Two review authors independently screened titles and abstracts for inclusion, extracted data and assessed trial quality. Trial authors were contacted to obtain missing data. Dichotomous outcomes will be reported as relative risk and continuous outcomes as mean differences with 95% confidence intervals. We assessed the quality of evidence using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. One open-label, randomised, single-centre trial including 70 participants with Marfan syndrome (aged 12 to 50 years old) met the inclusion criteria. Participants were randomly assigned to

  9. Risk factors of pre-operational aortic rupture in acute and subacute Stanford type A aortic dissection patients.

    Science.gov (United States)

    Li, Zhuo-Dong; Liu, Yang; Zhu, Jiang; Wang, Jun; Lu, Fang-Lin; Han, Lin; Xu, Zhi-Yun

    2017-12-01

    Aortic rupture is one of the main causes of early death in acute and subacute Stanford type A aortic dissection (ATAAD) patients. This study aimed to analyze potential risk factors for pre-operational aortic rupture in ATAAD patients. We retrospectively reviewed aortic dissection cases treated between May 2013 and May 2016 in Changhai Hospital, Shanghai. A total of 329 patients with ATAAD were included in the final analysis, and 31 patients died of aortic rupture before surgery. Clinical data on basic characteristics, clinical presentation, and biochemical measurements for all 329 patients were analyzed. The in-hospital aortic rupture rate was 9.4% (31/329), and the rupture accounted for 47% (31/66) of all in-hospital deaths of ATAAD patients. Patients who experienced rupture were significantly older (Prisk factors: shock [odds ratio (OR): 8.12; 95% confidence interval (CI), 1.10-59.85, P=0.040], pain requiring medication (OR: 12.67; 95% CI, 2.43-66.09; P=0.003), troponin level >0.7 ng/mL (OR: 9.28; 95% CI, 1.72-50.06; P=0.010), and D-dimer level ≥10 µg/mL (OR: 13.37; 95% CI, 2.18-81.97; P=0.005). Aortic rupture accounted for 47% of all in-hospital deaths among patient with ATAAD. Shock, pain requiring medication, a troponin level >0.7 ng/mL and a D-dimer level ≥10 µg/mL are independent risk factors for aortic rupture in these patients.

  10. Increased frequency of FBN1 truncating and splicing variants in Marfan syndrome patients with aortic events.

    Science.gov (United States)

    Baudhuin, Linnea M; Kotzer, Katrina E; Lagerstedt, Susan A

    2015-03-01

    Marfan syndrome is a systemic disorder that typically involves FBN1 mutations and cardiovascular manifestations. We investigated FBN1 genotype-phenotype correlations with aortic events (aortic dissection and prophylactic aortic surgery) in patients with Marfan syndrome. Genotype and phenotype information from probands (n = 179) with an FBN1 pathogenic or likely pathogenic variant were assessed. A higher frequency of truncating or splicing FBN1 variants was observed in Ghent criteria-positive patients with an aortic event (n = 34) as compared with all other probands (n = 145) without a reported aortic event (79 vs. 39%; P Marfan syndrome patients with FBN1 truncating and splicing variants.Genet Med 17 3, 177-187.

  11. [Valve-sparing aortic root replacement for young female patients with Marfan syndrome].

    Science.gov (United States)

    Nawata, Kan; Morota, T

    2009-10-01

    Annuloaortic ectasia is frequently related with Marfan syndrome, and Bentall procedure or aortic root replacement with valved conduit has been the conventional standard operation for this disease. Recently, some institutes have adopted valve-sparing aortic root replacement (VSARR) instead of Bentall procedure. Young female patients with Marfan syndrome who wishes for childbearing seem to be a group of good candidates of this type of operation, because it let them free from morbidities after artificial valve implantation. Valve-sparing operation should be taken into consideration when the size of Valsalva sinus reaches 45 mm for patients with Marfan syndrome and when it reaches 40 mm for patients with past histories or family histories of aortic dissection or aortic rupture. Since pregnancy is one of the most serious risk factors for aortic events, Valsalva sinus of 40 mm or larger could be the new standard for surgical indication if VSARR is applicable.

  12. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); J. Pepper (John)

    2016-01-01

    textabstractElective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a

  13. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); J. Pepper (John)

    2014-01-01

    textabstractElective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a

  14. Late aortic lymphocele and residual ovary syndrome after gynecological surgery

    Directory of Open Access Journals (Sweden)

    Iuliano Marialetizia

    2007-12-01

    Full Text Available Abstract Background Gynecological surgery, as radical hysterectomy or pelvic and aortic lymphadenectomy, accounts for more than 50% of iatrogenic injuries. In premenopausal women, an hysterectomy with ovarian sparing and concomitant lateral ovarian transposition is frequently performed. However, the fate of the retained ovary is complicated by the residual ovarian syndrome (ROS and one of the most common postoperative complications of the lymphadenectomy procedure is the lymphocele, with an average incidence of 22–48.5%. The differential diagnosis of a postoperative fluid collection includes, in addition to a lymphocele, urinoma, hematoma, seroma or abscess and the computed tomography (CT findings alone is not enough. Case presentation We describe a patient, affected by ROS concomitant with a asymptomatic lymphocele, initially confused with an aortic lymph nodes relapse, after abdominal radical hysterectomy. The patient was subjected to a surgical approach, included a diagnostic open laparoscopy and laparotomy with sovraombelico-pubic incision, wide opening of the pelvic peritoneum and retroperitoneum. Examination of the mass revealed, macroscopically, a ovary with multiloculated cystic masses filled with clear or yellow serous fluid and the layers were composed by flat or cuboidal mesothelial cells. Conclusion The tribute of this case illustrates the atypical appearance with uncertain aetiology after complex imaging. Gynecologist and radiologist should acquaint with the appearance of fluid collection (urinoma, lymphocele, seroma, hematoma, abscess in gynecologic oncology follow-up to properly differentiated from tumor recurrence.

  15. Acute radiation syndrome and chronic radiation syndrome.

    Science.gov (United States)

    Grammaticos, Philip; Giannoula, Evanthia; Fountos, George P

    2013-01-01

    Acute radiation syndrome (ARS) or sickness or poisoning or toxicity is induced after a whole body exposure of men to high doses of radiation between 1-12Gy. First symptoms are from the gastrointestinal system, which together with bone marrow are the most sensitive parts of our body. Chronic radiation syndrome (CRS) may be induced by smaller than 1Gy radiation doses or after a mild form of ARS. Prophylaxis and treatment suggestions are described. In cases of ARS, a large part of the exposed population after proper medical care may survive, while without medical care this part of the population will be lost. Prophylaxis may also save another part of the population.

  16. Emergency Valve-in-Valve Transcatheter Aortic Valve Implantation for the Treatment of Acute Stentless Bioprosthetic Aortic Insufficiency and Cardiogenic Shock

    Directory of Open Access Journals (Sweden)

    Ivan D. Hanson

    2018-01-01

    Full Text Available Bioprosthetic aortic valve degeneration may present as acute, severe aortic regurgitation and cardiogenic shock. Such patients may be unsuitable for emergency valve replacement surgery due to excessive risk of operative mortality but could be treatable with transfemoral valve-in-valve transcatheter aortic valve implantation (TAVI. There is a paucity of data regarding the feasibility of valve-in-valve TAVI in patients presenting with cardiogenic shock due to acute aortic insufficiency from stentless bioprosthetic valve degeneration. We present one such case, highlighting the unique aspects of valve-in-valve TAVI for this challenging patient subset.

  17. Kabuki syndrome in a girl with mosaic 45,X/47,XXX and aortic coarctation.

    Science.gov (United States)

    Chen, Chih-Ping; Lin, Shuan-Pei; Tsai, Fuu-Jen; Chern, Schu-Rern; Wang, Wayseen

    2008-06-01

    To describe the clinical findings of a patient with mosaic 45,X/47,XXX and aortic coarctation. Descriptive case study. Tertiary medical center. A 6-year-old girl with stigmata of Turner syndrome, aortic coarctation, patent ductus arteriosus, and a peculiar facial appearance. None. Cytogenetic analysis. The patient manifested a characteristic Kabuki syndrome facial appearance with long palpebral fissures, everted lateral third of lower eyelids, arched eyebrows, a depressed nasal tip, large dysplastic ears and epicanthic folds. She had undergone cardiac surgery for treatment of aortic coarctation and patent ductus arteriosus. Cytogenetic analysis of the blood lymphocytes revealed a karyotype of mos 45,X,9ph [35 cells]/47,XXX,9ph [5 cells]. This is the first report of mosaic 45,X/47,XXX associated with Kabuki syndrome. We emphasize that Kabuki syndrome, a peculiar facial appearance and aortic coarctation, should be considered in girls with sex chromosome abnormalities.

  18. Endovascular treatment of type B dissection in patients with Marfan syndrome: mid-term outcomes and aortic remodeling.

    Science.gov (United States)

    Eid-Lidt, Guering; Gaspar, Jorge; Meléndez-Ramírez, Gabriela; Cervantes S, Jorge; González-Pacheco, Hector; Dámas de Los Santos, Félix; Meave-González, Aloha; Ramírez Marroquín, Samuel

    2013-12-01

    To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair. MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear. Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%. The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients. Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair. Copyright © 2013 Wiley Periodicals, Inc.

  19. Mutations in the TGF-beta repressor SKI cause Shprintzen-Goldberg syndrome with aortic aneurysm

    NARCIS (Netherlands)

    Doyle, Alexander J.; Doyle, Jefferson J.; Bessling, Seneca L.; Maragh, Samantha; Lindsay, Mark E.; Schepers, Dorien; Gillis, Elisabeth; Mortier, Geert; Homfray, Tessa; Sauls, Kimberly; Norris, Russell A.; Huso, Nicholas D.; Leahy, Dan; Mohr, David W.; Caulfield, Mark J.; Scott, Alan F.; Destrée, Anne; Hennekam, Raoul C.; Arn, Pamela H.; Curry, Cynthia J.; van Laer, Lut; McCallion, Andrew S.; Loeys, Bart L.; Dietz, Harry C.

    2012-01-01

    Elevated transforming growth factor (TGF)-beta signaling has been implicated in the pathogenesis of syndromic presentations of aortic aneurysm, including Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS)(1-4). However, the location and character of many of the causal mutations in LDS intuitively

  20. Upper gastrointestinal obstruction secondary to aortoduodenal syndrome owing to a noninflammatory abdominal aortic aneurysm.

    LENUS (Irish Health Repository)

    Cahill, Kevin

    2012-01-31

    Aortoduodenal syndrome is a rare complication of an abdominal aortic aneurysm wherein the aneurysm sac obstructs the patient\\'s duodenum. It presents with the symptoms of an upper gastrointestinal tract obstruction and requires surgical intervention to relieve it. Previously, gastric bypass surgery was advocated, but now aortic replacement is the mainstay of treatment. We report a case of a 67-year-old woman whose aortoduodenal syndrome was successfully managed and review the literature on this topic.

  1. Massive Thoracoabdominal Aortic Thrombosis in a Patient with Iatrogenic Cushing Syndrome

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Dong Hyun; Lee, Young Min; Kim, Bo Bae; Ki, Young Jae; Kim, Jin Hwa; Chung, Joong Wha; Koh, Young Youp; Kang, Joon Tae; Chae, Seung Seok

    2014-01-01

    Massive thoracoabdominal aortic thrombosis is a rare finding in patients with iatrogenic Cushing syndrome in the absence of any coagulation abnormality. It frequently represents an urgent surgical situation. We report the case of an 82-year-old woman with massive aortic thrombosis secondary to iatrogenic Cushing syndrome. A follow-up computed tomography scan showed a decreased amount of thrombus in the aorta after anticoagulation therapy alone.

  2. Hybrid treatment of penetrating aortic ulcer

    Energy Technology Data Exchange (ETDEWEB)

    Lara, Juan Antonio Herrero; Martins-Romeo, Daniela de Araujo; Escudero, Carlos Caparros; Falcon, Maria del Carmen Prieto; Batista, Vinicius Bianchi, E-mail: jaherrero5@hotmail.com [Unidade de Gestao Clinica (UGC) de Diagnostico por Imagem - Hosppital Universitario Virgen Macarena, Sevilha (Spain); Vazquez, Rosa Maria Lepe [Unit of Radiodiagnosis - Hospital Nuestra Senora de la Merced, Osuna, Sevilha (Spain)

    2015-05-15

    Penetrating atherosclerotic aortic ulcer is a rare entity with poor prognosis in the setting of acute aortic syndrome. In the literature, cases like the present one, located in the aortic arch, starting with chest pain and evolving with dysphonia, are even rarer. The present report emphasizes the role played by computed tomography in the diagnosis of penetrating atherosclerotic ulcer as well as in the differentiation of this condition from other acute aortic syndromes. Additionally, the authors describe a new therapeutic approach represented by a hybrid endovascular surgical procedure for treatment of the disease. (author)

  3. Hybrid treatment of penetrating aortic ulcer

    International Nuclear Information System (INIS)

    Lara, Juan Antonio Herrero; Martins-Romeo, Daniela de Araujo; Escudero, Carlos Caparros; Falcon, Maria del Carmen Prieto; Batista, Vinicius Bianchi; Vazquez, Rosa Maria Lepe

    2015-01-01

    Penetrating atherosclerotic aortic ulcer is a rare entity with poor prognosis in the setting of acute aortic syndrome. In the literature, cases like the present one, located in the aortic arch, starting with chest pain and evolving with dysphonia, are even rarer. The present report emphasizes the role played by computed tomography in the diagnosis of penetrating atherosclerotic ulcer as well as in the differentiation of this condition from other acute aortic syndromes. Additionally, the authors describe a new therapeutic approach represented by a hybrid endovascular surgical procedure for treatment of the disease. (author)

  4. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

    Science.gov (United States)

    Arnold, Raoul; Neu, Marie; Hirtler, Daniel; Gimpel, Charlotte; Markl, Michael; Geiger, Julia

    2017-04-01

    Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all PTurner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections.

  5. Interrupted aortic arch type B in A patient with cat eye syndrome.

    Science.gov (United States)

    Belangero, Sintia Iole Nogueira; Bellucco, Fernanda Teixeira da Silva; Cernach, Mirlene C S P; Hacker, April M; Emanuel, Beverly S; Melaragno, Maria Isabel

    2009-05-01

    We report a patient with cat eye syndrome and interrupted aortic arch type B, a typical finding in the 22q11.2 deletion syndrome. Chromosomal analysis and fluorescent in situ hybridization (FISH) showed a supernumerary bisatellited isodicentric marker chromosome derived from chromosome 22. The segment from 22pter to 22q11.2 in the supernumerary chromosome found in our patient does not overlap with the region deleted in patients with the 22q11.2 deletion syndrome. However, the finding of an interrupted aortic arch type B is unusual in CES, although it is a frequent heart defect in the 22q11 deletion syndrome.

  6. Statins in acute coronary syndromes.

    Science.gov (United States)

    Sposito, Alexandre Russo; Aguiar Filho, Gentil Barreira de; Aarão, Amanda Rezende; Sousa, Francisco Thiago Tomaz de; Bertolami, Marcelo Chiara

    2011-10-01

    Statins are the main resource available to reduce LDL-cholesterol levels. Their continuous use decreases cardiovascular morbidity and mortality due to atherosclerosis. The administration of these medications demonstrated to be effective in primary and secondary prevention clinical trials in low and high risk patients. Specialists believe that a possible benefit of hypolipidemic therapy in preventing complications of atherosclerotic diseases is in the reduction of deposition of atherogenic lipoproteins in vulnerable areas of the vasculature. Experimental studies with statins have shown an enormous variety of other effects that could extend the clinical benefit beyond the lipid profile modification itself. Statin-based therapies benefit other important components of the atherothrombotic process: inflammation, oxidation, coagulation, fibrinolysis, endothelial function, vasoreactivity and platelet function. The demonstration of the effects that do not depend on cholesterol lowering or the pleiotropic effects of statins provides the theoretical basis for their potential role as adjunctive therapy in acute coronary syndromes. Retrospective analyses of a variety of studies indicate the potential benefit of statins during acute coronary events. Recent clinical studies have addressed this important issue in prospective controlled trials showing strong evidence for the administration of statins as adjunctive therapy in acute coronary syndromes.

  7. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms.

    Science.gov (United States)

    Treasure, Tom; Takkenberg, J J M; Pepper, John

    2014-10-01

    Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered.

  8. Prediction of aortic dilation in Turner syndrome - enhancing the use of serial cardiovascular magnetic resonance

    Science.gov (United States)

    2013-01-01

    Background Identification of the subset females with Turner syndrome who face especially high risk of aortic dissection is difficult, and more optimal risk assessment is pivotal in order to improve outcomes. This study aimed to provide comprehensive, dynamic mathematical models of aortic disease in Turner syndrome by use of cardiovascular magnetic resonance (CMR). Methods A prospective framework of long-term aortic follow-up was used, which comprised diameters of the thoracic aorta prospectively assessed at nine positions by CMR at the three points in time (baseline [n = 102, age 38 ± 11 years], follow-up [after 2.4 ± 0.4 years, n = 80] and end-of-study [after 4.8 ± 0.5 years, n = 78]). Mathematical models were created that cohesively integrated all measurements at all positions, from all visits and for all participants, and using these models cohesive risk factor analyses were conducted based on which predictive modeling was performed on which predictive modelling was performed. Results The cohesive models showed that the variables with effect on aortic diameter were aortic coarctation (P aortic valves (P treatment (P = 0.005). Oestrogen replacement therapy had an effect of borderline significance (P = 0.08). From these data, mathematical models were created that enabled preemption of aortic dilation from CMR derived aortic diameters in scenarios both with and without known risk factors. The fit of the models to the actual data was good. Conclusion The presented cohesive model for prediction of aortic diameter in Turner syndrome could help identifying females with rapid growth of aortic diameter, and may enhance clinical decision-making based on serial CMR. PMID:23742092

  9. Coronary ostial involvement in acute aortic dissection: detection with 64-slice cardiac CT.

    LENUS (Irish Health Repository)

    Ryan, E Ronan

    2012-02-01

    A 41-year-old man collapsed after lifting weights at a gym. Following admission to the emergency department, a 64-slice cardiac computed tomography (CT) revealed a Stanford Type A aortic dissection arising from a previous coarctation repair. Multiphasic reconstructions demonstrated an unstable, highly mobile aortic dissection flap that extended proximally to involve the right coronary artery ostium. Our case is an example of the application of electrocardiogram-gated cardiac CT in directly visualizing involvement of the coronary ostia in acute aortic dissection, which may influence surgical management.

  10. Acute Contained Ruptured Aortic Aneurysm Presenting as Left Vocal Fold Immobility

    OpenAIRE

    Gnagi, Sharon H.; Howard, Brittany E.; Hoxworth, Joseph M.; Lott, David G.

    2015-01-01

    Objective. To recognize intrathoracic abnormalities, including expansion or rupture of aortic aneurysms, as a source of acute onset vocal fold immobility. Methods. A case report and review of the literature. Results. An 85-year-old female with prior history of an aortic aneurysm presented to a tertiary care facility with sudden onset hoarseness. On laryngoscopy, the left vocal fold was immobile in the paramedian position. A CT scan obtained that day revealed a new, large hematoma surrounding ...

  11. Whole body perfusion in patients undergoing frozen elephant trunk for type A acute aortic dissections.

    Science.gov (United States)

    Cappabianca, Giangiuseppe; Roscitano, Claudio; Bichi, Samuele; Cricco, Antonio; Parrinello, Matteo; Beghi, Cesare; Albano, Giovanni; Esposito, Giampiero

    2017-03-01

    The Frozen Elephant Trunk (FET) can be adopted in selected type A acute aortic dissections (TAAAD). During FET, a prolonged distal circulatory arrest exposes the spine and visceral organs to potential ischemic injuries. Antegrade distal aortic perfusion (ADAP) could minimize this risk: we describe the technical aspects of the simultaneous use of antegrade cerebral perfusion (ACP) and ADAP achieving a "Whole Body Perfusion" (WBP) during FET.

  12. Aortic valve-sparing operation in Marfan syndrome: what do we know after a decade?

    Science.gov (United States)

    Kallenbach, Klaus; Baraki, Hassina; Khaladj, Nawid; Kamiya, Hiroyuki; Hagl, Christian; Haverich, Axel; Karck, Matthias

    2007-02-01

    We assessed the outcome in patients with Marfan syndrome operated on exclusively with the aortic valve-sparing reimplantation technique for aortic root aneurysms during more than a decade. Between July 1993 and April 2005, the aortic valve-sparing reimplantation technique (David I) was used in 325 patients. In 59 patients with clinical evidence of Marfan syndrome, procedures were done for aortic root aneurysm (n = 55) or aortic dissection type A (n = 4). Their mean age was 30 +/- 12 years (range, 9 to 62 years), and 37 (63%) were male. Additional procedures were arch replacement in 4 patients, coronary artery bypass grafting in 1, mitral valve surgery in 9, and closure of atrial septal defect in 3. Mean follow-up was 54 +/- 37 months (range, 0 to 139 months). No patient died during the first 30 days postoperatively. Mean bypass time was 163 +/- 34 minutes (range, 99 to 248 minutes), and mean aortic cross clamp time was 126 +/- 28 minutes (range, 78 to 202 minutes). Four patients (6.8%) required rethoracotomy for postoperative bleeding. Five late deaths (8.5%) occurred during follow-up. Reoperation of the reconstructed valve was required in 7 patients. Freedom from reoperation was 88% +/- 5% at 5 years and 80% +/- 9% at 10 years. Mean grade of aortic insufficiency was 1.81 preoperatively compared with 0.20 early postoperatively (p valve should encourage use of this technique in patients with Marfan syndrome.

  13. Valve-sparing operation for aortic root aneurysm in patients with Marfan syndrome.

    Science.gov (United States)

    Wang, R; Ma, W G; Tian, L X; Sun, L Z; Chang, Q

    2010-03-01

    We report our experience with aortic valve-sparing procedures in patients with Marfan syndrome and aortic root aneurysm. Between August 2003 and July 2007, we performed aortic valve-sparing procedures in 20 patients with aortic root aneurysm resulting from Marfan syndrome. Mean age was 28 +/- 10 years (range, 10 to 57 years), and there were 9 females and 11 males. A reimplantation technique was used in 9 cases, a remodeling technique in 8 and a patch technique in 3 cases. Additional procedures included total aorta replacement in 1 patient, and aortic arch replacement plus stented elephant trunk in 2 patients. The mean follow-up time was 46 +/- 16 months (range, 17 to 64 months). No in-hospital or late death occurred. Reexploration for bleeding was required in one case on postoperative day 1. No valve-related complications occurred during the follow-up period. At the end of follow-up, trivial or no aortic regurgitation was demonstrated in 14 patients, mild in 4 patients, moderate in 1 and severe in 1. Two patients with moderate and severe aortic regurgitation required reoperation. The early and mid-term results of the valve-sparing operations were favorable, and the durability of the preserved valve should encourage use of this technique in patients with Marfan syndrome.

  14. The hemodynamic effects of acute aortic regurgitation into a stiffened left ventricle resulting from chronic aortic stenosis.

    Science.gov (United States)

    Okafor, Ikechukwu; Raghav, Vrishank; Midha, Prem; Kumar, Gautam; Yoganathan, Ajit

    2016-06-01

    Acute aortic regurgitation (AR) post-chronic aortic stenosis is a prevalent phenomenon occurring in patients who undergo transcatheter aortic valve replacement (TAVR) surgery. The objective of this work was to characterize the effects of left ventricular diastolic stiffness (LVDS) and AR severity on LV performance. Three LVDS models were inserted into a physiological left heart simulator. AR severity was parametrically varied through four levels (ranging from trace to moderate) and compared with a competent aortic valve. Hemodynamic metrics such as average diastolic pressures (DP) and reduction in transmitral flow were measured. AR index was calculated as a function of AR severity and LVDS, and the work required to make up for lost volume due to AR was estimated. In the presence of trace AR, higher LVDS had up to a threefold reduction in transmitral flow (13% compared with 3.5%) and a significant increase in DP (2-fold). The AR index ranged from ∼42 to 16 (no AR to moderate AR), with stiffer LVs having lower values. To compensate for lost volume due to AR, the low, medium, and high LVDS models were found to require 5.1, 5.5, and 6.6 times more work, respectively. This work shows that the LVDS has a significant effect on the LV performance in the presence of AR. Therefore, the LVDS of potential TAVR patients should be assessed to gain an initial indication of their ability to tolerate post-procedural AR.

  15. Predictors of aortic growth in uncomplicated type B aortic dissection from the Acute Dissection Stent Grafting or Best Medical Treatment (ADSORB) database

    DEFF Research Database (Denmark)

    Kamman, Arnoud V; Brunkwall, Jan; Verhoeven, Eric L

    2017-01-01

    BACKGROUND: The high-risk patient cohort of uncomplicated type B aortic dissections (uTBADs) needs to be clarified. We compared uTBAD patients treated with best medical treatment (BMT), with and without aortic growth, from the Acute Dissection Stent Grafting or Best Medical Treatment (ADSORB) trial...... database. Furthermore, we looked for trends in outcome for aortic growth and remodeling after BMT and thoracic endovascular aortic repair (TEVAR) and BMT (TEVAR+BMT). METHODS: BMT patients with available baseline and a 1-year follow-up arterial computed tomography scan were identified. True lumen and false...

  16. Valve-sparing aortic root replacement in Loeys-Dietz syndrome.

    Science.gov (United States)

    Patel, Nishant D; Arnaoutakis, George J; George, Timothy J; Allen, Jeremiah G; Alejo, Diane E; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

    2011-08-01

    Loeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known. We reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations) evidence of LDS who underwent VSRR at our institution. Echocardiographic and clinical data were obtained from hospital and follow-up clinic records. From 2002 to 2009, 31 patients with a firm diagnosis of LDS underwent VSRR for aortic root aneurysm. Mean age was 15 years, and 24 (77%) were children. One (3%) patient had a bicuspid aortic valve. Preoperative sinus diameter was 3.9±0.8 cm (z score 7.0±2.9) and 2 (6%) had greater than 2+ aortic insufficiency. Thirty patients (97%) underwent reimplantation procedures using a Valsalva graft. There were no operative deaths. Mean follow-up was 3.6 years (range, 0 to 7 years). One patient required late repair of a pseudoaneurysm at the distal aortic anastomosis, and 1 had a conversion to a David reimplantation procedure after a Florida sleeve operation. No patient suffered thromboembolism or endocarditis, and 1 (3%) patient experienced greater than 2+ late aortic insufficiency. No patient required late aortic valve repair or replacement. Loeys-Dietz syndrome is an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk. Valve-sparing procedures have encouraging early and midterm results, similar to those in Marfan syndrome, and are an attractive option for young patients. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  17. Valve-sparing aortic root repair in acute type A dissection: how many sinuses have to be repaired for curative surgery?

    Science.gov (United States)

    Urbanski, Paul P; Hijazi, Husam; Dinstak, Witold; Diegeler, Anno

    2013-09-01

    The aim of the study was to evaluate operative and long-term results of valve-sparing aortic root surgery in acute type A dissection. The repair consisted of selective replacement of all dissected and pathological sinuses. Forty-six patients (mean age 62 ± 14; range 29-88 years, 3 with Marfan syndrome), operated on between August 2001 and July 2011 due to acute type A aortic dissection, underwent valve-sparing root repair, resulting in a valve preservation rate of 56% in acute aortic dissection surgery involving the aortic root. Insufficiency grades of 0/1+, 2+, 3+ and 4+ were presented in 16, 17, 12 and 1 patients, respectively. Root repair with resection of the whole of the pathological aortic wall without the use of any glue was performed in all patients. Replacement of 1, 2 or 3 sinuses of Valsalva was performed in 29, 12 and 5 patients, respectively. Concomitant cusp repair was necessary in 7 patients. All perioperative data were collected prospectively and an intention-to-treat analysis was performed. A total of 6 patients (median age 76, range 63-81 years) died, on average 10 months (range 0.9-44) after surgery resulting in an overall survival of 87% at the mean follow-up of 54 ± 37, range 0.9-132 months. The linearized death rate was 2.9%/year, and the actuarial survival rate at 8 years was 85.5 ± 5.6%. No death was related to the aortic valve or aortic root. There were no valve-related events and no patient required reoperation on the proximal aorta/aortic valve during the follow-up. At the last echocardiography (47.8 ± 35.6 months after surgery), 33 patients showed no and 13 patients slight (1+) aortic insufficiency. Curative repair with replacement of all pathological sinuses of Valsalva leads to an excellent long-term outcome. Selected sinus repair is a simple and effective method of curative, valve-sparing root repair in acute aortic dissection because replacement of all sinuses is seldom necessary.

  18. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study

    NARCIS (Netherlands)

    Meijboom, Lilian J.; Vos, Frederiek E.; Timmermans, Janneke; Boers, Godfried H.; Zwinderman, Aeiko H.; Mulder, Barbara J. M.

    2005-01-01

    Aims In women with Marfan syndrome pregnancy presents an increased risk of dilatation, dissection, and rupture of the aorta. The aim of this study was to investigate the influence of pregnancy on growth of the aortic root. Methods and results Between 1993 and 2004 127 women with Marfan syndrome were

  19. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study.

    NARCIS (Netherlands)

    Meijboom, L.J.; Vos, F.E.; Timmermans, J.; Boers, G.H.J.; Zwinderman, A.H.; Mulder, B.J.M.

    2005-01-01

    AIMS: In women with Marfan syndrome pregnancy presents an increased risk of dilatation, dissection, and rupture of the aorta. The aim of this study was to investigate the influence of pregnancy on growth of the aortic root. METHODS AND RESULTS: Between 1993 and 2004 127 women with Marfan syndrome

  20. Aortic calcification and renal cysts demonstrated by CT in a teenager with Alagille syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pombo, F. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Isla, C. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Gayol, A. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain); Bargiela, A. [Dept. of Radiology, Hospital Juan Canalejo, La Coruna (Spain)

    1995-06-01

    Alagille syndrome, or arteriohepatic dysplasia, is a disorder characterized by paucity of intrahepatic bile ducts, peculiar facies and skeletal anomalies. We report a typical case of this syndrome in an 18-year-old girl, in whom abdominal CT showed bilateral renal cysts and aortic wall calcification, findings unreported in the radiological literature. (orig.)

  1. Latest research progress on acute nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Satinder Kakar

    2017-01-01

    Full Text Available Etiology of nephrotic syndrome is somewhat complex in nature. It may range from primary to secondary forms. Nephrotic syndrome patients often need immunosuppressive treatment although it has some side effects and may lead to renal disease which may be acute or severe. This review deals with herbal treatment and other recent approaches for treating symptoms of nephrotic syndrome.

  2. Complete Resolution of a Large Bicuspid Aortic Valve Thrombus with Anticoagulation in Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Rayan Jo Rachwan

    2017-09-01

    Full Text Available Native aortic valve thrombosis in primary antiphospholipid syndrome (APLS is a rare entity. We describe a 38-year-old man who presented with neurological symptoms and a cardiac murmur. Transthoracic echocardiography detected a large bicuspid aortic valve thrombus. Laboratory evaluation showed the presence of antiphospholipid antibodies. Anticoagulation was started, and serial echocardiographic studies showed complete resolution of the aortic valve vegetation after 4 months. The patient improved clinically and had no residual symptoms. This report and review of the literature suggests that vegetations in APLS can be treated successfully with conservative treatment, regardless of their size.

  3. Double valve replacement for acute spontaneous left chordal rupture secondary to chronic aortic incompetence

    Directory of Open Access Journals (Sweden)

    McLenachan Jim

    2006-10-01

    Full Text Available Abstract A 54 years old male with undiagnosed chronic calcific degenerative aortic valve incompetence presented with acute left anterior chordae tendinae rupture resulting in severe left heart failure and cardiogenic shock. He was successfully treated with emergency double valve replacement using mechanical valves. The pathogenesis of acute rupture of the anterior chordae tendinae, without any evidence of infective endocarditis or ischemic heart disease seems to have been attrition of the subvalvular mitral apparatus by the chronic regurgitant jet of aortic incompetence with chronic volume overload. We review the literature with specific focus on the occurrence of this unusual event.

  4. Acute Aortic Dissection Mimicking STEMI in the Catheterization Laboratory: Early Recognition Is Mandatory

    Directory of Open Access Journals (Sweden)

    Alessio Arrivi

    2012-01-01

    Full Text Available Coronary malperfusion due to type A aortic dissection is a life-threatening condition where timely recognition and treatment are mandatory. A 77-year-old woman underwent an acute evolving type A aortic dissection mimicking acute myocardial infarction. Two pathophysiologic mechanisms are discussed: either thrombosis migrating from a previously treated giant aneurism of proximal left anterior descending or a local arterial complication due to left main stenting. Recognition of these occurrences in the catheterization laboratory is important to look immediately for surgery.

  5. Risk factors and early outcomes of acute renal injury after thoracic aortic endograft repair for type B aortic dissection

    Directory of Open Access Journals (Sweden)

    Luo S

    2017-08-01

    Full Text Available Songyuan Luo,* Huanyu Ding,* Jianfang Luo, Wei Li, Bing Ning, Yuan Liu, Wenhui Huang, Ling Xue, Ruixin Fan, Jiyan Chen Cardiology Department, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of Coronary Heart Disease Prevention, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China *These authors contributed equally to this work Background: Thoracic endovascular aortic repair (TEVAR has become an emerging treatment modality for acute type B aortic dissection (TBAD patients in recent years. The risk factors and impacts of acute kidney injury (AKI after percutaneous TEVAR, however, have not been widely established.Methods: We retrospectively studied the clinical records of 305 consecutive patients who admitted to our institution and had TEVAR for TBAD between December 2009 and June 2013. The patients were routinely monitored for their renal functions preoperatively until 7 days after TEVAR. The Kidney Disease Improving Global Guidelines (KDIGO criteria were used for AKI.Results: Of the total 305 consecutive patients, 84 (27.5% developed AKI after TEVAR, comprising 66 (21.6% patients in KDIGO stage 1, 6 (2.0% patients in stage 2 and 12 (3.9% patients in stage 3. From the logistic regression analysis, systolic blood pressure (SBP on admission >140 mmHg (odds ratio [OR], 2.288; 95% CI, 1.319–3.969 and supra-aortic branches graft bypass hybrid surgery (OR, 3.228; 95% CI, 1.526–6.831 were independent risk factors for AKI after TEVAR. Local anesthesia tended to be a protective factor (OR, 0.563; 95% CI, 0.316–1.001. The preoperative renal function, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker or statin administration, volume of contrast agent, range of TBAD and false lumen involving renal artery were not associated with post-operation AKI. The in-hospital mortality and major adverse events were markedly increased with the occurrence of AKI (7.1% vs 0.9%, P=0

  6. [Surgical treatment of the aortic root aneurysm related to Marfan syndrome].

    Science.gov (United States)

    Zheng, Si-hong; Sun, Yan-qing; Meng, Xu; Zhang, Hong; Hou, Xiao-tong; Wang, Jian-gang; Gao, Feng

    2005-08-24

    To review the experience of surgical treatment of aortic root aneurysm of Marfan syndrome. We The clinical data of 84 Marfan syndrome patients, 61 males and 23 females, aged 35 +/- 12 (5 - 62), 41 cases presenting with aortic dissection (Debakey type I in 32 cases and type II in 9), 52 cases with moderate to severe aortic regurgitation, and 9 cases with moderate to severe mitral regurgitation, 43 cases with cardiac function of class I - II, 30 with class III and 11 with class IV according the New York Heart Association (NYHA) standard, who underwent surgical treatment for aortic root aneurysm with a mean diameter of 68 mm +/- 14 mm, were analyzed. Bentall procedure was performed in 68 cases, Wheat procedure in 6, Cabrol procedure in 5, and aortic valve replacement and aortoplasty in 5. Concomitant procedures included mitral value replacement and mitral valvuoplasty in 3 cases respectively. Urgent surgery was conducted in 28 cases, and elective operation in 56 cases. There were 3 in-hospital deaths (3.57%). 76 cases were followed up for a mean duration of 55 +/- 31 months. Three patients underwent reoperation. The cardiac function returned to class I - II except for 2 cases that remained at the class III. Bentall procedure should be the first choice of the surgery for aortic root aneurysm of Marfan syndrome with a low mortality and a good late outcome.

  7. [Surgical results for aortic involvement in Marfan syndrome].

    Science.gov (United States)

    Shiiya, N; Matsuzaki, K; Maruyama, R; Kunihara, T; Murashita, T; Aoki, H; Yasuda, K

    2002-07-01

    From 1991 through 2001, 21 Marfan patients underwent aortic operations in our hospital. They received a total of 36 aortic operations, 31 by ourselves including 4 non-elective operations and 2 operations before 1991. Extent of replacement was Bentall + total arch (4), Bentall (8), valve sparing aortic root (reimplantation) (2), re-anastomosis + coronary aortic bypass grafting (CABG) after Bentall (1), ascending + total arch (3), ascending (1), total arch (1), total thoracoabdominal (10), thoracoabdominal (1), descending thoracic (2), distal arch (1), abdominal (2). Multiple operations were required in 11 patients (2 operations in 7, 3 operations in 4). Eight reoperations in 6 patients were for adjacent lesion, 5 reoperations were for remote lesion, and 2 others were for complication of Bentall (initial operation elsewhere). Among the 8 reoperations for adjacent lesion, 3 were scheduled operation (2 with elephant trunk), 4 were for residual dissection, and 1 was for annulo-aortic ectasia (AAE). Total aortic replacement was achieved in 4 and subtotal replacement excluding the root in 2. There was no hospital mortality. Paraparesis occurred in 1 who died 4.7 years after operation. The remaining patients are currently alive. No other aortic event occurred. Aortic reoperation-free survival was 83% at 5 year and 28% at 10 year.

  8. Mutations in a TGF-β ligand, TGFB3, cause syndromic aortic aneurysms and dissections.

    Science.gov (United States)

    Bertoli-Avella, Aida M; Gillis, Elisabeth; Morisaki, Hiroko; Verhagen, Judith M A; de Graaf, Bianca M; van de Beek, Gerarda; Gallo, Elena; Kruithof, Boudewijn P T; Venselaar, Hanka; Myers, Loretha A; Laga, Steven; Doyle, Alexander J; Oswald, Gretchen; van Cappellen, Gert W A; Yamanaka, Itaru; van der Helm, Robert M; Beverloo, Berna; de Klein, Annelies; Pardo, Luba; Lammens, Martin; Evers, Christina; Devriendt, Koenraad; Dumoulein, Michiel; Timmermans, Janneke; Bruggenwirth, Hennie T; Verheijen, Frans; Rodrigus, Inez; Baynam, Gareth; Kempers, Marlies; Saenen, Johan; Van Craenenbroeck, Emeline M; Minatoya, Kenji; Matsukawa, Ritsu; Tsukube, Takuro; Kubo, Noriaki; Hofstra, Robert; Goumans, Marie Jose; Bekkers, Jos A; Roos-Hesselink, Jolien W; van de Laar, Ingrid M B H; Dietz, Harry C; Van Laer, Lut; Morisaki, Takayuki; Wessels, Marja W; Loeys, Bart L

    2015-04-07

    Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed an important contribution of disturbed transforming growth factor (TGF)-β signaling. This study sought to discover a novel gene causing syndromic aortic aneurysms in order to unravel the underlying pathogenesis. We combined genome-wide linkage analysis, exome sequencing, and candidate gene Sanger sequencing in a total of 470 index cases with thoracic aortic aneurysms. Extensive cardiological examination, including physical examination, electrocardiography, and transthoracic echocardiography was performed. In adults, imaging of the entire aorta using computed tomography or magnetic resonance imaging was done. Here, we report on 43 patients from 11 families with syndromic presentations of aortic aneurysms caused by TGFB3 mutations. We demonstrate that TGFB3 mutations are associated with significant cardiovascular involvement, including thoracic/abdominal aortic aneurysm and dissection, and mitral valve disease. Other systemic features overlap clinically with Loeys-Dietz, Shprintzen-Goldberg, and Marfan syndromes, including cleft palate, bifid uvula, skeletal overgrowth, cervical spine instability and clubfoot deformity. In line with previous observations in aortic wall tissues of patients with mutations in effectors of TGF-β signaling (TGFBR1/2, SMAD3, and TGFB2), we confirm a paradoxical up-regulation of both canonical and noncanonical TGF-β signaling in association with up-regulation of the expression of TGF-β ligands. Our findings emphasize the broad clinical variability associated with TGFB3 mutations and highlight the importance of early recognition of the disease because of high cardiovascular risk. Copyright © 2015 American College of Cardiology Foundation. Published by

  9. Aortic aneurysm and non-Hodgkin’s lymphoma in Marfan syndrome

    Directory of Open Access Journals (Sweden)

    Sujoy Ghosh

    2009-03-01

    Full Text Available The combination of Marfan syndrome with lymphoma is extremely rare. This report describes a case of Marfan syndrome who presented with chest discomfort and was diagnosed to have an aortic aneurysm and an additional incidental mediastinal mass that on further investigation turned out to be a diffuse large B cell lymphoma. We have suggested a hypothesis which can explain the occurrence of lymphoma in Marfan syndrome.

  10. Angiotensin, transforming growth factor β and aortic dilatation in Marfan syndrome: Of mice and humans

    Directory of Open Access Journals (Sweden)

    Christopher Yu

    2018-03-01

    Full Text Available Marfan syndrome is consequent upon mutations in FBN1, which encodes the extracellular matrix microfibrillar protein fibrillin-1. The phenotype is characterised by development of thoracic aortic aneurysm. Current understanding of the pathogenesis of aneurysms in Marfan syndrome focuses upon abnormal vascular smooth muscle cell signalling through the transforming growth factor beta (TGFβ pathway. Angiotensin II (Ang II can directly induce aortic dilatation and also influence TGFβ synthesis and signalling. It has been hypothesised that antagonism of Ang II signalling may protect against aortic dilatation in Marfan syndrome. Experimental studies have been supportive of this hypothesis, however results from multiple clinical trials are conflicting. This paper examines current knowledge about the interactions of Ang II and TGFβ signalling in the vasculature, and critically interprets the experimental and clinical findings against these signalling interactions. Keywords: Aneurysm, Angiotensin blocker, Cell Signalling, Clinical trial

  11. Personal resources and satisfaction with life in Marfan syndrome patients with aortic pathology and in abdominal aortic aneurysm patients.

    Science.gov (United States)

    Stanišić, Michał-Goran; Rzepa, Teresa; Gawrońska, Alicja; Kubaszewski, Przemysław; Putowski, Maciej; Stefaniak, Sebastian; Perek, Bartłomiej

    2018-03-01

    Whether or not the source of aortic pathology is Marfan syndrome (MFS) or other processes leading to development of abdominal aorta aneurysms (AAA), the awareness of pathology may lead to an emotional upset and low assessment of satisfaction with life. To assess, in regard to MFS patients with aortic pathology and to abdominal aortic aneurysm patients: 1) whether or not self-efficacy (SE) and health locus of control (HLoC) affect the patients' satisfaction with life; 2) whether the two groups of patients differ in terms of mental dispositions. The study population consisted of 16 MFS patients with aortic pathology and 16 AAA patients, 9 men and 7 women in each group. The mean age of the MFS patients was 28.5 ±8.214, and of the AAA patients 64.25 ±7.019. The following scales were applied: Generalized Self-Efficacy Scale, Satisfaction With Life Scale, Multidimensional Health Locus of Control Scale. Abdominal aorta aneurysms patients compared to MFS patients gave a higher rating for SE ( MD = 33.94 and MD = 29.56), internal health locus of control ( MD = 25.00 and MD = 21.13), external personal HL o C ( MD = 24.50 and MD = 19.25), external impersonal HLoC ( MD = 23.06 and MD = 18.25), and satisfaction with life ( M = 22.06 and M = 20.13). Internal and external HL o C were significantly lower in MFS patients compared to AAA patients. In patients with aortic diseases, special attention must be paid to the state of personal resources (PR). Interactions made by medical professionals should focus on enhancing PR supporting the patients' self-knowledge on their SE. This will help to improve their satisfaction with life and form a positive attitude to the illness.

  12. Republished review: Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms.

    Science.gov (United States)

    Treasure, Tom; Takkenberg, J J M; Pepper, John

    2016-02-01

    Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  13. Endovascular stent graft treatment of acute thoracic aortic transections due to blunt force trauma.

    LENUS (Irish Health Repository)

    Bjurlin, Marc A

    2012-02-01

    Endovascular stent graft treatment of acute thoracic aortic transections is an encouraging minimally invasive alternative to open surgical repair. Between 2006 and 2008, 16 patients with acute thoracic aortic transections underwent evaluation at our institution. Seven patients who were treated with an endovascular stent graft were reviewed. The mean Glasgow Coma Score was 13.0, probability of survival was .89, and median injury severity score was 32. The mean number of intensive care unit days was 7.7, mean number of ventilator support days was 5.4, and hospital length of stay was 10 days. Mean blood loss was 285 mL, and operative time was 143 minutes. Overall mortality was 14%. Procedure complications were a bleeding arteriotomy site and an endoleak. Endovascular treatment of traumatic thoracic aortic transections appears to demonstrate superior results with respect to mortality, blood loss, operative time, paraplegia, and procedure-related complications when compared with open surgical repair literature.

  14. Antiphospholipid Antibody Syndrome With Valvular Vegetations in Acute Q Fever.

    Science.gov (United States)

    Million, Matthieu; Thuny, Franck; Bardin, Nathalie; Angelakis, Emmanouil; Edouard, Sophie; Bessis, Simon; Guimard, Thomas; Weitten, Thierry; Martin-Barbaz, François; Texereau, Michèle; Ayouz, Khelifa; Protopopescu, Camelia; Carrieri, Patrizia; Habib, Gilbert; Raoult, Didier

    2016-03-01

    Coxiella burnetii endocarditis is considered to be a late complication of Q fever in patients with preexisting valvular heart disease (VHD). We observed a large transient aortic vegetation in a patient with acute Q fever and high levels of IgG anticardiolipin antibodies (IgG aCL). Therefore, we sought to determine how commonly acute Q fever could cause valvular vegetations associated with antiphospholipid antibody syndrome, which would be a new clinical entity. We performed a consecutive case series between January 2007 and April 2014 at the French National Referral Center for Q fever. Age, sex, history of VHD, immunosuppression, and IgG aCL assessed by enzyme-linked immunosorbent assay were tested as potential predictors. Of the 759 patients with acute Q fever and available echocardiographic results, 9 (1.2%) were considered to have acute Q fever endocarditis, none of whom had a previously known VHD. After multiple adjustment, very high IgG aCL levels (>100 immunoglobulin G-type phospholipid units; relative risk [RR], 24.9 [95% confidence interval {CI}, 4.5-140.2]; P = .002) and immunosuppression (RR, 10.1 [95% CI, 3.0-32.4]; P = .002) were independently associated with acute Q fever endocarditis. Antiphospholipid antibody syndrome with valvular vegetations in acute Q fever is a new clinical entity. This would suggest the value of systematically testing for C. burnetii in antiphospholipid-associated cardiac valve disease, and performing early echocardiography and antiphospholipid dosages in patients with acute Q fever. © The Author 2015. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.

  15. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Arnold, Raoul [University Medical Center Heidelberg, Department of Congenital Heart Disease and Pediatric Cardiology, Heidelberg (Germany); Neu, Marie [University Medical Center, Department of Pediatric Hematology/Oncology/Hemostaseology, Mainz (Germany); Hirtler, Daniel [University of Freiburg, Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center, Freiburg im Breisgau (Germany); Gimpel, Charlotte [Center for Pediatrics, Medical Center - University of Freiburg, Department of General Pediatrics, Adolescent Medicine and Neonatology, Freiburg im Breisgau (Germany); Markl, Michael [Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago, IL (United States); Northwestern University, Department of Biomedical Engineering, McCormick School of Engineering, Chicago, IL (United States); Geiger, Julia [Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago, IL (United States); University Children' s Hospital, Department of Radiology, Zuerich (Switzerland)

    2017-04-15

    Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all P<0.03). Patients with abnormal helical or vortical flow in the ascending aorta had significantly larger diameters of the ascending aorta (P<0.03). Peak systolic wall shear stress, pulsatility index and oscillatory shear index were significantly lower in Turner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections. (orig.)

  16. Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.

    Science.gov (United States)

    Takata, Munenori; Amiya, Eisuke; Watanabe, Masafumi; Omori, Kazuko; Imai, Yasushi; Fujita, Daishi; Nishimura, Hiroshi; Kato, Masayoshi; Morota, Tetsuro; Nawata, Kan; Ozeki, Atsuko; Watanabe, Aya; Kawarasaki, Shuichi; Hosoya, Yumiko; Nakao, Tomoko; Maemura, Koji; Nagai, Ryozo; Hirata, Yasunobu; Komuro, Issei

    2014-07-01

    Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA). The mean value of flow-mediated dilation was 6.5 ± 2.4 %. Flow-mediated dilation had a negative correlation with the diameter of the ascending thoracic aorta (AscAd)/BSA (R = -0.39, p = 0.020) and multivariate analysis revealed that flow-mediated dilation was an independent factor predicting AscAd/BSA, whereas other segments of the aorta had no association. Furthermore, Brinkman index had a somewhat greater influence on flow-mediated dilation (R = -0.42, p = 0.008). Although subjects who smoked tended to have a larger AscAd compared with non-smokers (AscA/BSA: 17.3 ± 1.8 versus 15.2 ± 3.0 mm/m(2), p = 0.013), there was no significant change in flow-mediated dilation, suggesting that smoking might affect aortic dilation via an independent pathway. Common atherogenic risks, such as impairment of flow-mediated dilation and smoking status, affected aortic dilation in subjects with Marfan syndrome.

  17. Presentation of an uncommon form of aortic dissection and rupture in Marifoan syndrome

    International Nuclear Information System (INIS)

    Delgado, I.; Ruiz, R.; Villanueva, J.M.; Fernandez Cueto, J.L.

    1995-01-01

    In Marfan syndrome, aneurysmatic enlargement of ascending aorta and dissection starting at the root are the most common cardiovascular complications. We present an infrequent case of a 15-year-old patient with a typical case of Marfan syndrome. CT disclosed an aorta and aortic arch of normal size with dissection originating distally with respect to the point where left subclavian artery arises. The disecction extended to descending aorta and to iliac and femoral arteries. Aortic rupture occurred in the arch, with massive hemothorax. The CT findings were confirmed at necropsy. 9 refs

  18. Medical image of the week: acute aortic dissection

    Directory of Open Access Journals (Sweden)

    Desai H

    2015-06-01

    Full Text Available No abstract available. Article truncated after 150 words. An 85-year-old gentleman with the past medical history significant for hypertension, smoking, and coronary artery disease presented to the emergency department (ED with complains of sudden onset of chest pain. His pain was described as squeezing and radiating to the back, associated with nausea and vomiting. His chest pain improved with nitroglycerin in ED. Chest x-ray showed a tortuous aortic knob and widened mediastinum. He underwent a CT angiogram, which showed, Stanford Type B aortic dissection, from distal aortic arch to renal arteries (Figure 1. He was managed in the hospital conservatively with tight blood pressure control given the type of dissection and no surgical intervention was done. He was uneventfully discharged with follow up arranged with vascular surgery. Aortic dissection is classified by Stanford Criteria as Type A which involves the ascending aorta and arch and Type B when it involves the descending aorta. Type A dissection is a ...

  19. How I do it: transapical cannulation for acute type-A aortic dissection

    Directory of Open Access Journals (Sweden)

    Alexiou Christos

    2008-01-01

    Full Text Available Abstract Aortic dissection is the most frequently diagnosed lethal disease of the aorta. Half of all patients with acute type-A aortic dissection die within 48 hours of presentation. There is still debate as to the optimal site of arterial cannulation for establishing cardiopulmonary bypass in patients with type-A aortic dissection. Femoral artery cannulation with retrograde perfusion is the most common method but because of the risk of malperfusion of vital organs and atheroembolism related to it different sites such as the axillary artery, the innominate artery and the aortic arch are used. Cannulation of these sites is not without risks of atheroembolism, neurovascular complications and can be time consuming. Another yet to be popularised option is the transapical aortic cannulation (TAC described in this article. TAC consists of the insertion of the arterial cannula through the apex of the left ventricle and the aortic valve to lie in the sinus of Valsalva. Trans-oesophageal guidance is necessary to ensure correct placement of the cannula. TAC is an excellent method of establishing cardiopulmonary bypass as it is quick, provides a more physiological method of delivering antegrade arterial flow and is the only method to assure perfusion of the true lumen.

  20. Postoperative peri-axillary seroma following axillary artery cannulation for surgical treatment of acute type A aortic dissection

    Directory of Open Access Journals (Sweden)

    Katsanos Konstantinos

    2010-05-01

    Full Text Available Abstract The arterial cannulation site for optimal tissue perfusion and cerebral protection during cardiopulmonary bypass (CPB for surgical treatment of acute type A aortic dissection remains controversial. Right axillary artery cannulation confers significant advantages, because it provides antegrade arterial perfusion during cardiopulmonary bypass, and allows continuous antegrade cerebral perfusion during hypothermic circulatory arrest, thereby minimizing global cerebral ischemia. However, right axillary artery cannulation has been associated with serious complications, including problems with systemic perfusion during cardiopulmonary bypass, problems with postoperative patency of the artery due to stenosis, thrombosis or dissection, and brachial plexus injury. We herein present the case of a 36-year-old Caucasian man with known Marfan syndrome and acute type A aortic dissection, who had direct right axillary artery cannulation for surgery of the ascending aorta. Postoperatively, the patient developed an axillary perigraft seroma. As this complication has, not, to our knowledge, been reported before in cardiothoracic surgery, we describe this unusual complication and discuss conservative and surgical treatment options.

  1. Prognostic Implications of Acute Renal Failure after Surgery for Type A Acute Aortic Dissection.

    Science.gov (United States)

    Sansone, Fabrizio; Morgante, Alessandro; Ceresa, Fabrizio; Salamone, Giovanni; Patanè, Francesco

    2015-06-01

    "Type A" acute aortic dissection (AAAD) is the most challenging among the emergency operations in cardiac surgery. The aim of this study was the evaluation of the role of acute renal failure (ARF) in postoperative survival of patients operated for AAAD. From February 2010 to April 2012, 37 consecutive patients were operated at our department for AAAD. We studied our population by subdividing the patients within groups according to the presence of ARF requiring continuous veno-venous hemofiltration (CVVH) and according to hypothermic circulatory arrest (HCA) times and degrees. The overall 30-day mortality was 27% (50% group A with ARF, 13% group B no ARF). Acute renal failure requiring CVVH was 37.8%. Multivariate analysis revealed a significant association with 30-day mortality (odds ratio 6.6 and p = 0.020). Preoperative oliguria [urine output less than 30 ml/h (odds ratio 4.7 p = 0.039)], CPB greater than 180 minutes (odds ratio 6.5 p = 0.023) and postoperative bleeding requiring a surgical reopening (odds ratio 12.2 and p = 0.021) were the variables significantly associated with acute kidney injury. The data obtained from our analysis bring out the high incidence of renal injuries after surgery for AAAD, and indicate a negative impact on renal injuries of a preoperative oliguria, longer Cardiopulmonary bypass (CBP)/HCA times, and postoperative bleeding requiring a surgical revision. Our data also suggest a better 30-day survival and better renal outcomes in case of shorter HCA and lesser degree of hypothermia. The option of lesser and shorter hypothermia may be very useful, especially for the elderly patients and octogenarians.

  2. Prophylactic antidepressant treatment following acute coronary syndrome

    DEFF Research Database (Denmark)

    Christiansen, Ole G; Madsen, Michael T; Simonsen, Erik

    2017-01-01

    the current evidence of primary prophylactic treatment of depression in patients after acute coronary syndrome. The study protocol was prospectively registered at PROSPERO (registration number CRD42015025587). A systematic review were conducted and reported according to Preferred Reporting Items......Major depressive disorder is significantly increased in patients following acute coronary syndrome resulting in twofold increased mortality compared with patients without depression. The depression diagnosis is often missed leading to considerable undertreatment. This systematic review assesses...

  3. A rare case of discrete aortic coarctation in Williams-Beuren syndrome. Diagnostic and therapeutic considerations

    Directory of Open Access Journals (Sweden)

    Savina Mannarino

    2015-11-01

    Full Text Available Williams-Beuren syndrome (WBS is a genetic disorder caused by elastin gene deletions, and is characterized by cardiovascular malformations, primarily including supravalvular aortic stenosis and peripheral pulmonary stenosis. We report a case of a neonate who developed severe discrete aortic coarctation, underwent multiple surgical interventions, and was subsequently diagnosed with WBS. Severe discrete aortic coarctation is a rare event in WBS newborns. An abnormally thick aortic wall is present in these patients and is the basis of the failure of the classical approach towards coarctation repair, which consists of end-to-end anastomosis as first surgical choice. Our case, and a very few similar previously documented cases, have all demonstrated recoarctation, which only aortic patch implantation was able to successfully repair. In light of this, we would also like to underline the importance of early WBS diagnosis. Therefore, even in mild syndromic phenotype such as low birth weight or facial dysmorphism that raise the suspicion of a genetic syndrome, it is advisable to perform fluorescent in situ hybridization analysis rather than merely karyotypic one.

  4. Factors influencing prognosis in patients with marfan syndrome after aortic surgery.

    Science.gov (United States)

    Gao, Linggen; Zhou, Xianliang; Zhang, Lin; Wen, Dan; Chang, Qian; Wu, Yongbo; Sun, Lizhong; Hui, Rutai

    2011-08-01

    Aortic aneurysm formation leading eventually to aortic rupture or dissection in early adult life is a fatal outcome of Marfan syndrome (MFS). Advances in the treatment of the syndrome have improved prognosis, but the long-term reoperation rate is still high. It remains unknown which factors influence the long-term prognosis, including the reoperation and mortality rates, in surgically treated Chinese patients with MFS. The authors studied 125 such patients to investigate factors influencing prognosis after aortic surgery. A retrospective clinical investigation. An academic medical center. One hundred twenty-five Marfan patients who had undergone aortic surgery. None. The indications for aortic surgery were aortic aneurysm and/or dissection in the 125 Marfan patients. The most commonly performed procedure was the Bentall in 92 patients. Sixteen patients underwent total arch replacement combined with stented elephant trunk implantation. Ten patients underwent the David procedure. Overall in-hospital and 30-day mortality rate was 1.6%. The survival rate was 97.5%, 91.4%, and 74.2% at 1, 5, and 10 years after surgery, respectively. The reoperation rate was 2.5%, 12.9%, and 32.9% at 1, 5, and 10 years after surgery, respectively. Multivariate analysis revealed that increased systolic blood pressure (Sys BP) was the predictor of death (p < 0.05), and body mass index and smoking were significant predictors of reoperation (p < 0.05). The present findings report the factors influencing the prognosis of Chinese patients with MFS after aortic surgical procedures. Managing these risk factors may enable health care professionals to improve the prognosis of MFS patients after aortic surgical procedures. Copyright © 2011 Elsevier Inc. All rights reserved.

  5. Acute intestinal pseudo-obstruction (Ogilvie's syndrome).

    Science.gov (United States)

    Maloney, Nell; Vargas, H David

    2005-05-01

    Acute colonic pseudo-obstruction (ACPO), also known as Ogilvie's syndrome, is a condition characterized by massive colonic distension in the absence of mechanical obstruction. Patients presenting with Ogilvie's syndrome have underlying medical and surgical conditions predisposing them to the syndrome. Ogilvie's syndrome can often be managed by conservative therapy. However, unrecognized and untreated, the continued distension associated with Ogilvie's syndrome can lead to perforation that is associated with a high mortality rate. In this article, the pathophysiology, epidemiology, and treatment options are reviewed.

  6. Acute radiation syndrome in human

    International Nuclear Information System (INIS)

    Guskova, A.K.

    1992-01-01

    The combination of the different types of irradiation dramatically changes the clinical course of acute radiation syndrome (ARS) in the case of short term exposure. The recent experience has been compared mostly with the data on the atomic bomb victims in Hiroshima and Nagasaki. The comparison of the injuries from different radiation exposures resulted in the possibility to receive summarized data and the actual basis of the observed difference. The situation with total relatively uniform irradiation is observed when human position is at long distance from powerful radiation sources or when he changes his position, as seen in atomic bomb survivors, the results of nuclear weapon tests and the patients in the Chernobyl accident. This is connected with the ARS of bone, marrow and intestine. The situation characterized by the clinical signs of the large area of skin and mucosa injuries was observed in nuclear weapon tests and the Chernobyl accident. In the case of the more localized and less severe beta injuries of skin and mucosa, the long term effects may be important. The majority of accidents in peaceful period are related to the uneven exposure from near sources, and the situation of the combination of external and internal irradiation is related to uneven irradiation and the predominance of internal exposure. (K.I.)

  7. [Hyperglycaemia and Acute Coronary Syndrome].

    Science.gov (United States)

    Grembiale, A; Cloro, C; Iorio, F; Cufone, S; Succurro, E; Arturi, F

    2012-01-01

    Hyperglycaemia in patients with Acute Coronary Syndrome (ACS)is common, and is an independent predictor of mortality and morbidity in patients both with and without diabetes mellitus. Hyperglycaemia may be a marker of pre-existing diabetes or glucose intolerance or may also represent a transient stress response mediated through the autonomic nervous system with release of adrenal corticosteroids and catecholamines. Several evidences suggest that an intensive control of hyperglycaemia results in a significant improvement of the adverse outcomes in the short and long term. In fact, an intensive metabolic treatment can counteract the negative effects of hyperglycaemia. However, the main difficulty to intensive glucose control in patients with ACS remains hypoglycaemia that is associated with an increased risk of mortality and myocardial re-infarction. No definitive data are available about the beneficial effects of insulin intensive treatment. Therefore, randomized multicenter clinical trials will be needed to definitively establish whether intensive glucose control will reduce the associated increased mortality rate and higher rates of complications in hospitalized ACS patients with hyperglycaemia.

  8. Florida Sleeve Procedure Is Durable and Improves Aortic Valve Function in Marfan Syndrome Patients.

    Science.gov (United States)

    Aalaei-Andabili, Seyed Hossein; Martin, Tomas; Hess, Philip; Klodell, Charles; Karimi, Ashkan; Arnaoutakis, George; Lee, Teng; Beaver, Thomas

    2017-09-01

    The Florida sleeve (FS) procedure was developed as a simplified approach for repair of functional type I aortic insufficiency secondary to aortic root aneurysm. We evaluated postoperative aortic valve function, long-term survival, and freedom from reoperation in Marfan syndrome patients who underwent the FS procedure at our center. All Marfan syndrome patients undergoing FS procedure from May 2002 to December 2014 were included. Echocardiography assessment included left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD), ejection fraction, and degree of aortic insufficiency (none = 0, minimal = 1, mild = 2, moderate = 3, severe = 4). Social Security Death Index and primary care physicians' report were used for long-term follow-up. Thirty-seven Marfan syndrome patients, 21 (56.8%) men and 16 (43%) women with mean age of 35.08 ± 13.45 years underwent FL repair at our center. There was no in-hospital or 30-day death or stroke. Two patients required reoperation due to bleeding. Patients' survival rate was 94% at 1 to 8 years. Freedom from reoperation was 100% at 8 years. Twenty-five patients had postoperative follow-up echocardiography at 1 week. Aortic insufficiency grade significantly decreased after the procedure (preoperative mean ± SD: 1.76 ± 1.2 versus 1-week postoperative mean ± SD: 0.48 ± 0.71, p Marfan syndrome patients with immediate improvement in aortic valve function. Long-term survival and freedom from reoperation rates are encouraging. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  9. [Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].

    Science.gov (United States)

    Mariucci, Elisabetta; Guidarini, Marta; Donti, Andrea; Lovato, Luigi; Wischmeijer, Anita; Angeli, Emanuela; Gargiulo, Gaetano D; Picchio, Fernando M; Bonvicini, Marco

    2015-12-01

    Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term. This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography. After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life. Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.

  10. Elevated expression levels of lysyl oxidases protect against aortic aneurysm progression in Marfan syndrome

    NARCIS (Netherlands)

    Busnadiego, O.; Gorbenko del Blanco, D.; González-Santamaría, J.; Habashi, J.P.; Calderon, J.F.; Sandoval, P.; Bedja, D.; Guinea-Viniegra, J.; Lopez-Cabrera, M.; Rosell-Garcia, T.; Snabel, J.M.; Hanemaaijer, R.; Forteza, A.; Dietz, H.C.; Egea, G.; Rodriguez-Pascual, F.

    2015-01-01

    Patients with Marfan syndrome (MFS) are at high risk of life-threatening aortic dissections. The condition is caused by mutations in the gene encoding fibrillin-1, an essential component in the formation of elastic fibers. While experimental findings in animal models of the disease have shown the

  11. Modified Sleeve Technique in Aortic Valve-Sparing Operation for Marfan Syndrome.

    Science.gov (United States)

    Wu, Yung-Szu; Hsieh, Shih-Rong; Wang, Chung-Chi; Tsai, Chung-Lin

    2018-03-22

    We devised a simple modification of the Florida Sleeve procedure to perform aortic valve-sparing surgery. This technique is simple, quick, effective, and safe. We used this technique in operations performed on two young patients with Marfan syndrome. The initial and short-term results were satisfactory.

  12. Marfan syndrome in children and adolescents: an adjusted nomogram for screening aortic root dilatation

    NARCIS (Netherlands)

    Rozendaal, L.; Groenink, M.; Naeff, M. S.; Hennekam, R. C.; Hart, A. A.; van der Wall, E. E.; Mulder, B. J.

    1998-01-01

    OBJECTIVE: To construct an adjusted nomogram for the echocardiographic screening of aortic root diameter in children with possible Marfan disease. DESIGN: In 91 children (42 boys, 49 girls, age range 3.2 to 18.4 years) undergoing Marfan screening from 1983 until 1996, the diagnosis Marfan syndrome

  13. Aortic root pathology in Marfan syndrome increases the risk of migraine with aura

    NARCIS (Netherlands)

    Koppen, H.; Vis, J. C.; Gooiker, D. J.; Knudsen, S.; Bouma, B. J.; Tijssen, J. G. P.; de Mol, B. A. J. M.; Mulder, B. J. M.; Russell, M. B.; Ferrari, M. D.

    2012-01-01

    To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor. In a multicentre study 123 MFS patients (n = 52 with AR, n = 71 without AR), 82 age- and sex-matched controls and 51 patients

  14. Middle aortic syndrome as a cause of heart failure in children and its management.

    OpenAIRE

    Gupta, S; Goswami, B; Ghosh, D C; Sen Gupta, A N

    1981-01-01

    Two cases of middle aortic syndrome in children are described along with two other cases reported earlier. In childhood, this disease may present as incipient or overt cardiac failure. Surgical treatment should be undertaken based on an objective assessment of the severity of the stricture and after taking into account the future growth of the child.

  15. Ascending aortic aneurysm and diaphragmatic hernia in a case of Marfan syndrome.

    Science.gov (United States)

    Kothari, Jignesh; Hinduja, Manish; Baria, Kinnaresh; Pandya, Himani

    2017-06-01

    Marfan syndrome commonly affects the skeletal, ocular, and cardiovascular systems. Involvement of the gastrointestinal system is known but uncommon. Intervention depends upon the system involved and the severity of symptoms. Special awareness is required for the diagnosis and management of gastrointestinal involvement in these patients. We report a rare case of simultaneous surgical repair of an ascending aortic aneurysm and a type IV hiatal hernia in a 35-year-old man with Marfan syndrome.

  16. Our experience in the diagnosis of aortic dissection by multislice computed tomography

    International Nuclear Information System (INIS)

    Llerena Rojas, Luis R; Mendoza Rodriguez, Vladimir; Olivares Aquiles, Eddy

    2011-01-01

    Aortic dissection (AD) is the most frequent and life-threatening acute aortic syndrome. Currently the more used method for the aortic study is the multislice computed tomography. The purpose of this paper is to expose the more relevant features in 22 patients with AD consecutively studied by multislice computed tomography

  17. Cardiorenal Syndrome in Acute Heart Failure Syndromes

    Directory of Open Access Journals (Sweden)

    Mohammad Sarraf

    2011-01-01

    Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

  18. Life-Threatening Postpneumonectomy Syndrome Complicated with Right Aortic Arch after Left Pneumonectomy.

    Science.gov (United States)

    Karasaki, Takahiro; Tanaka, Makoto

    2015-01-01

    A 54-year-old man with right aortic arch underwent left lower lobectomy and lingular segmentectomy, followed by complete pneumonectomy, for refractory nontuberculous mycobacterial infection. Three months after the pneumonectomy, he developed acute respiratory distress. Computed tomography showed an excessive mediastinal shift with an extremely narrowed bronchus intermedius and right lower bronchus compressed between the right pulmonary artery and the right descending aorta. Soon after the nearly obstructed bronchus intermedius was observed by bronchoscopy, he began to exhibit frequent hypoxic attacks, perhaps due to mucosal edema. Emergent surgical repositioning of the mediastinum and decompression of the bronchus was indicated. After complete adhesiolysis of the left thoracic cavity was performed, to maintain the proper mediastinal position, considering the emergent setting, an open wound thoracostomy was created and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. Thoracoplasty was performed three months later, and he was eventually discharged without any dressings needed. Mediastinal repositioning under thoracostomy should be avoided in elective cases because of its extremely high invasiveness. However, in the case of life-threatening postpneumonectomy syndrome in an emergent setting, mediastinal repositioning under thoracostomy may be an option to save life, which every thoracic surgeon could attempt.

  19. Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions.

    Science.gov (United States)

    Song, Howard K; Preiss, Liliana R; Maslen, Cheryl L; Kroner, Barbara; Devereux, Richard B; Roman, Mary J; Holmes, Kathryn W; Tolunay, H Eser; Desvigne-Nickens, Patrice; Asch, Federico M; Milewski, Rita K; Bavaria, Joseph; LeMaire, Scott A

    2014-05-01

    The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR). AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less. AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.

  20. Valve Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

    Science.gov (United States)

    Song, Howard K.; Preiss, Liliana R.; Maslen, Cheryl L.; Kroner, Barbara; Devereux, Richard B.; Roman, Mary J.; Holmes, Kathryn W.; Tolunay, H. Eser; Desvigne-Nickens, Patrice; Asch, Federico M.; Milewski, Rita K.; Bavaria, Joseph; LeMaire, Scott A.

    2016-01-01

    Background The long-term outcomes of aortic valve sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. We sought to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). Methods At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 have undergone aortic root replacement. Patients who have undergone AVS procedures were compared to those who have undergone aortic valve replacing (AVR) procedures. Results AVS root replacement was performed in 43.5% of MFS patients and the frequency of AVS increased over the past 5 years. AVS patients were younger at the time of surgery (31.0 vs. 36.3 years, p=0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients. AVR patients were more likely to have had aortic valve dysfunction and aortic dissection as a primary indication for surgery. After mean follow-up of 6.2 (SD=3.6) years, none of the 87 AVS patients have required reoperation; in contrast, after mean follow up of 10.5 (SD=7.6) years, 11.5% of AVR patients have required aortic root reoperation. Aortic valve function has been durable with 95.8% of AVS patients with aortic insufficiency graded as mild or less. Conclusions AVS root replacement is performed commonly in the MFS population. The durability of the aortic repair and aortic valve function have been excellent to date. These results justify the continued use of the procedure in the elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future. PMID:25296451

  1. Antegrade versus retrograde cerebral perfusion in relation to postoperative complications following aortic arch surgery for acute aortic dissection type A.

    Science.gov (United States)

    Apostolakis, Efstratios; Koletsis, Efstratios N; Dedeilias, Panagiotis; Kokotsakis, John N; Sakellaropoulos, George; Psevdi, Argini; Bolos, Konstantin; Dougenis, Dimitrios

    2008-01-01

    Aortic arch surgery is impossible without the temporary interruption of brain perfusion and therefore is associated with high incidence of neurologic injury. The deep hypothermic circulatory arrest (HCA), in combination with antegrade or retrograde cerebral perfusion (RCP), is a well-established method of brain protection in aortic arch surgery. In this retrospective study, we compare the two methods of brain perfusion. From 1998 to 2006, 48 consecutive patients were urgently operated for acute type A aortic dissection and underwent arch replacement under deep hypothermic circulatory arrest (DHCA). All distal anastomoses were performed with open aorta, and the arch was replaced totally in 15 cases and partially in the remaining 33 cases. Our patient cohort is divided into those protected with antegrade cerebral perfusion (ACP) (group A, n = 23) and those protected with RCP (group B, n = 25). No significant difference was found between groups A and B with respect to cardiopulmonary bypass-time, brain-ischemia time, cerebral-perfusion time, permanent neurologic dysfunction, and mortality. The incidence of temporary neurologic dysfunction was 16.0% for group A and 43.50% for group B (p = 0.04). The mean extubation time was 3.39 +/- 1.40 days for group A and 4.96 +/- 1.83 days for group B (p = 0.0018). The mean ICU-stay was 4.4 +/- 2.3 days for group A and 6.9 +/- 2.84 days for group B (p = 0.0017). The hospital-stay was 14.38 +/- 4.06 days for group A and 19.65 +/- 6.91 days for group B (p = 0.0026). The antegrade perfusion seems to be related with significantly lower incidence of temporary neurological complications, earlier extubation, shorter ICU-stay, and hospitalization, and hence lower total cost.

  2. Acute resistance exercise using free weights on aortic wave reflection characteristics.

    Science.gov (United States)

    Tai, Yu Lun; Gerhart, Hayden; Mayo, Xián; Kingsley, J Derek

    2018-01-01

    Aortic wave reflection characteristics such as the augmentation index (AIx), wasted left ventricular pressure energy (ΔE w ) and aortic haemodynamics, such as aortic systolic blood pressure (ASBP), strongly predict cardiovascular events. The effects of acute resistance exercise (ARE) using free-weight exercises on these characteristics are unknown. Therefore, we sought to determine the effects of acute free-weight resistance exercise on aortic wave reflection characteristics and aortic haemodynamics in resistance-trained individuals. Fifteen young, healthy resistance-trained (9 ± 3 years) individuals performed two randomized sessions consisting of an acute bout of free-weight resistance exercise (ARE) or a quiet control (CON). The ARE consisted of three sets of 10 repetitions at 75% one repetition maximum for squat, bench press and deadlift. In CON, the participants rested in the supine position for 30 min. Measurements were made at baseline before sessions and 10 min after sessions. A two-way ANOVA was used to compare the effects of condition across time. There were no significant interactions for aortic or brachial blood pressures. Compared to rest, there were significant increases in augmentation pressure (rest: 5·7 ± 3·0 mmHg; recovery: 10·4 ± 5·7 mmHg, P = 0·002), AIx (rest: 116·8 ± 4·2%; recovery: 123·2 ± 8·4%, P = 0·002), AIx normalized at 75 bpm (rest: 5·2 ± 7·6%; recovery: 27·3 ± 13·2%, P<0·0001), ΔE w (rest: 1215 ± 674 dynes s cm -2 ; recovery: 2096 ± 1182 dynes s cm -2 , P = 0·008), and there was a significant decrease in transit time of the reflected wave (rest: 150·7 ± 5·8 ms; recovery 145·5 ± 5·6 ms, P<0·001) during recovery from ARE compared to CON. These data suggest that ARE using free-weight exercises may have no effect on aortic and brachial blood pressure but may significantly alter aortic wave reflection characteristics. © 2016 Scandinavian Society of Clinical Physiology and

  3. Effect of the Antioxidant Lipoic Acid in Aortic Phenotype in a Marfan Syndrome Mouse Model

    Directory of Open Access Journals (Sweden)

    Maria C. Guido

    2018-01-01

    Full Text Available Marfan syndrome (MFS cardiovascular manifestations such as aortic aneurysms and cardiomyopathy carry substantial morbidity/mortality. We investigated the effects of lipoic acid, an antioxidant, on ROS production and aortic remodeling in a MFS mgΔloxPneo mouse model. MFS and WT (wild-type 1-month-old mice were allocated to 3 groups: untreated, treated with losartan, and treated with lipoic acid. At 6 months old, echocardiography, ROS production, and morphological analysis of aortas were performed. Aortic ROS generation in 6-month-old MFS animals was higher at advanced stages of disease in MFS. An unprecedented finding in MFS mice analyzed by OCT was the occurrence of focal inhomogeneous regions in the aortic arch, either collagen-rich extremely thickened or collagen-poor hypotrophic regions. MFS animals treated with lipoic acid showed markedly reduced ROS production and lower ERK1/2 phosphorylation; meanwhile, aortic dilation and elastic fiber breakdown were unaltered. Of note, lipoic acid treatment associated with the absence of focal inhomogeneous regions in MFS animals. Losartan reduced aortic dilation and elastic fiber breakdown despite no change in ROS generation. In conclusion, oxidant generation by itself seems neutral with respect to aneurysm progression in MFS; however, lipoic acid-mediated reduction of inhomogeneous regions may potentially associate with less anisotropy and reduced chance of dissection/rupture.

  4. [Images in cardiology after clinical observation - aortic dissection in Marfan syndrome].

    Science.gov (United States)

    Cabanelas, Nuno; Nobre, Angelo; Guerra, Nuno; Gallego, Javier; Ferreira, Ricardo; Carvalheiro, Catarina; Roque, João; Peres, Marisa; Siopa, Luís; Martins, Vítor Paulo; Silva, Graça; Cravino, João

    2011-09-01

    Stanford type A aortic dissection is a rare phenomenon with high short-term mortality and clinical manifestations that can make differential diagnosis a lengthy process requiring several diagnostic examinations. Based on a case report, the aim is to highlight the importance of physical examination in the initial management of these patients and of rapid access to a surgical center. A brief review follows on the diagnosis and treatment of ascending aortic dissection, and its specific nature in Marfan syndrome. A 33-year-old man was admitted to the emergency department of a district hospital with chest and back pain associated with vomiting, 20 hours after symptom onset. Initial physical examination revealed an aortic systolic murmur and musculoskeletal morphological abnormalities compatible with Marfan syndrome. Given suspected aortic dissection, a transthoracic echocardiogram was immediately performed, which showed an extensive intimal flap originating at the sinotubular junction. He was transferred to the cardiothoracic surgery department of a referral hospital where he was treated by a Bentall procedure. In this case, careful physical examination during initial assessment raised the suspicion that this patient was in a high-risk group for aortic dissection, thus avoiding unnecessary and lengthy exams. This diagnosis requires emergent surgical treatment, and so direct contact in real time between those making in the diagnosis and the surgeon is essential, as well as protocols governing immediate access to a surgical center. Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  5. Allometric considerations when assessing aortic aneurysms in Turner syndrome: Implications for activity recommendations and medical decision-making.

    Science.gov (United States)

    Corbitt, Holly; Maslen, Cheryl; Prakash, Siddharth; Morris, Shaine A; Silberbach, Michael

    2018-02-01

    In Turner syndrome, the potential to form thoracic aortic aneurysms requires routine patient monitoring. However, the short stature that typically occurs complicates the assessment of severity and risk because the relationship of body size to aortic dimensions is different in Turner syndrome compared to the general population. Three allometric formula have been proposed to adjust aortic dimensions, all employing body surface area: aortic size index, Turner syndrome-specific Z-scores, and Z-scores based on a general pediatric and young adult population. In order to understand the differences between these formula we evaluated the relationship between age and aortic size index and compared Turner syndrome-specific Z-scores and pediatric/young adult based Z-scores in a group of girls and women with Turner syndrome. Our results suggest that the aortic size index is highly age-dependent for those under 15 years; and that Turner-specific Z-scores are significantly lower than Z-scores referenced to the general population. Higher Z-scores derived from the general reference population could result in stigmatization, inappropriate restriction from sports, and increasing the risk of unneeded medical or operative treatments. We propose that when estimating aortic dissection risk clinicians use Turner syndrome-specific Z-score for those under fifteen years of age. © 2017 Wiley Periodicals, Inc.

  6. Acute aortic dissection in a young healthy athlete with androgenic anabolic steroid use: A case report

    Directory of Open Access Journals (Sweden)

    Barman M, Djamel B, Mathews J

    2014-07-01

    Full Text Available Background: Acute aortic dissection can occur at the time of intense physical exertion in strength-trained athletes like weight lifters, bodybuilders, throwers, and wrestlers. Rapid rise in blood pressure and history of hypertension are the most common causes of aortic dissection in athletes. It is a very tragic event because of its high mortality rate of about 32% in young patients. We report a case of aortic dissection in a young weightlifter with a history of anabolic steroid usage with an extensive intimal tear of the aorta at Sino tubular junction and arch. All athletes must be assessed for predisposing factors for aortic dissection, and all patients should be encouraged to undergo appropriate diagnostic studies, like echocardiography and blood pressure monitoring while weightlifting to recognize possible predisposing factors for aortic dissection. Athletes who do have a problem should be encouraged to avoid or limit their exercise or activity by their cardiologist. It is vital that this disastrous event be prevented in young people. In conclusion, although a rare occurrence, AD should be considered in symptomatic patients with any family history of early cardiac deaths, a history suggestive of a connective tissue disorder (that is, multiple joint surgeries or who practice weightlifting.

  7. Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

    Science.gov (United States)

    Kunkala, Meghana R; Schaff, Hartzell V; Li, Zhuo; Volguina, Irina; Dietz, Harry C; LeMaire, Scott A; Coselli, Joseph S; Connolly, Heidi

    2013-09-10

    Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤ 2 would benefit from prophylactic MV intervention.

  8. Acute aortic rupture in a dog with spirocercosis following the administration of medetomidine : clinical communication

    Directory of Open Access Journals (Sweden)

    K.E. Joubert

    2005-06-01

    Full Text Available Spirocercosis is an emerging disease in veterinary medicine. A strong suspicion of spirocercosis is usually evident after a thorough clinical examination and radiography of the chest has been performed. Lesions seen on radiography include an oesophageal mass, spondylitis and oesophageal air. Unfortunately, radiography is not diagnostic and additional diagnostic procedures are required to confirm the diagnosis. Endoscopy is commonly performed to diagnose the condition. The dog presented in this study had radiographic and clinical signs consistent with spirocercosis and definitive diagnosis was required. Shortly after sedation with medetomidine, the dog went into cardiac arrest and failed to respond to resuscitative measures. On post mortem, the diagnosis of spirocercosis was confirmed and the cause of death was identified as acute aortic rupture. Aortic aneurysms are not an uncommon finding and cause of acute death in dogs with spirocercosis. The acute rupture of the aorta in this case is most probably the result of cardiovascular changes associated with the administration of medetomidine. Medetomidine causes an acute rise in systemic vascular resistance with hypertension. The increase in shear stress across the weakened aortic wall resulted in rupture. Caution with the use of medetomidine in patients with spirocercosis is advised.

  9. Three-year-old child with middle aortic syndrome treated by endovascular stent implantation.

    Science.gov (United States)

    Moszura, Tomasz; Goreczny, Sebastian; Dryzek, Pawel; Niwald, Marek

    2013-04-01

    Middle aortic syndrome (MAS) is an extremely rare anomaly and represents both a diagnostic and therapeutic challenge, particularly in young children. A case of a 3.5 year-old child with MAS and arterial hypertension is reported, where owing to the patient's young age and the length of the hypoplastic aortic segment, surgical correction with end-to-end anastomosis was not feasible. Instead of palliative bypass grafting between the thoracic and abdominal aorta, successful percutaneous balloon angioplasty and stenting of the lesion was performed with the assistance of three-dimensional rotational angiography.

  10. Serum beta-2 microglobulin levels for predicting acute kidney injury complicating aortic valve replacement.

    Science.gov (United States)

    Zaleska-Kociecka, Marta; Skrobisz, Anna; Wojtkowska, Izabela; Grabowski, Maciej; Dabrowski, Maciej; Kusmierski, Krzysztof; Piotrowska, Katarzyna; Imiela, Jacek; Stepinska, Janina

    2017-10-01

    Acute kidney injury complicating both transcatheter and surgical aortic valve replacement is associated with high rates of morbidity and mortality. The aim of this study was to investigate the role of serum beta 2 (β2) microglobulin, cystatin C and neutrophil gelatinase-associated lipocalin levels in detecting periprocedural acute kidney injury. Eighty consecutive patients who were 70 years of age or older and who were having surgical (n = 40) or transcatheter (n = 40) aortic valve replacement were recruited in a prospective study. The biomarkers were tested before the procedure, 6 times afterwards, at discharge and at a 6-month follow-up visit. The baseline β2-microglobulin level was the strongest predictor of acute kidney injury as a complication of transcatheter aortic valve replacement [odds ratio (OR) 5.277, P = 0.009]. Its level 24 h after the procedure reached the largest area under the curve (AUC) of 0.880 (P regression analysis, the levels of β2-microglobulin and cystatin C 24 h after the procedure were significantly associated with acute kidney injury after transcatheter valve replacement (OR 38.15, P = 0.044; OR 1782, P = 0.019, respectively). In the surgical aortic valve replacement group, the highest AUCs belonged to β2-microglobulin and cystatin C at 24 h (AUC = 0.808, P = 0.003 and AUC = 0.854, P = 0.001, respectively). Their higher values were also associated with acute kidney injury (OR 17.2, P = 0.018; OR 965.6, P = 0.02, respectively). A persistent increase in the postoperative levels of β2-microglobulin following acute kidney injury was associated with the progression of chronic kidney disease for 6 months after both transcatheter (OR 6.56, P = 0.030) and surgical (OR 7.67, P = 0.03) aortic valve replacements. Serum β2-microglobulin had the potential to predict acute kidney injury complicating transcatheter valve replacement and to diagnose it as early as 24 h after both the

  11. Clinical Examination for Acute Aortic Dissection: A Systematic Review and Meta-analysis.

    Science.gov (United States)

    Ohle, Robert; Kareemi, Hashim Khaliq; Wells, George; Perry, Jeffrey J

    2018-04-01

    Acute aortic dissection is a life-threatening condition due to a tear in the aortic wall. It is difficult to diagnose and if missed carries a significant mortality. We conducted a librarian-assisted systematic review of PubMed, MEDLINE, Embase, and the Cochrane database from 1968 to July 2016. Titles and abstracts were reviewed and data were extracted by two independent reviewers (agreement measured by kappa). Studies were combined if low clinical and statistical heterogeneity (I 2  < 30%). Study quality was assessed using the QUADAS-2 tool. Bivariate random effects meta analyses using Revman 5 and SAS 9.3 were performed. We identified 792 records: 60 were selected for full text review, nine studies with 2,400 participants were included (QUADAS-2 low risk of bias, κ = 0.89 [for full-text review]). Prevalence of aortic dissection ranged from 21.9% to 76.1% (mean ± SD = 39.1% ± 17.1%). The clinical findings increasing probability of aortic dissection were 1) neurologic deficit (n = 3, specificity = 95%, positive likelihood ratio [LR+] = 4.4, 95% confidence interval [CI] = 3.3-5.7, I 2  = 0%) and 2) hypotension (n = 4, specificity = 95%, LR+ = 2.9 95% CI = 1.8-4.6, I 2  = 42%), and decreasing probability were the absence of a widened mediastinum (n = 4, sensitivity = 76%-95%, negative likelihood ratio [LR-] = 0.14-0.60, I 2  = 93%) and an American Heart Association aortic dissection detection (AHA ADD) risk score < 1 (n = 1, sensitivity = 91%, LR- = 0.22, 95% CI = 0.15-0.33). Suspicion for acute aortic dissection should be raised with hypotension, pulse, or neurologic deficit. Conversely, a low AHA ADD score decreases suspicion. Clinical gestalt informed by high- and low-risk features together with an absence of an alternative diagnosis should drive investigation for acute aortic dissection. © 2017 by the Society for Academic Emergency Medicine.

  12. Interrupted Aortic Arch Type B in A Patient with Cat Eye Syndrome

    OpenAIRE

    Belangero, Sintia Iole Nogueira; Bellucco, Fernanda Teixeira da Silva; Cernach, Mirlene C. S. P.; Hacker, April M.; Emanuel, Beverly S.; Melaragno, Maria Isabel

    2009-01-01

    We report a patient with cat eye syndrome and interrupted aortic arch type B, a typical finding in the 22q11.2 deletion syndrome. Chromosomal analysis and fluorescent in situ hybridization (FISH) showed a supernumerary bisatellited isodicentric marker chromosome derived from chromosome 22. The segment from 22pter to 22q11.2 in the supernumerary chromosome found in our patient does not overlap with the region deleted in patients with the 22q11.2 deletion syndrome. However, the finding of an in...

  13. Acute pulmonary edema due to stress cardiomyopathy in a patient with aortic stenosis: a case report

    OpenAIRE

    Bayer, Monika F

    2009-01-01

    Introduction Stress cardiomyopathy is a condition of chest pain, breathlessness, abnormal heart rhythms and sometimes congestive heart failure or shock precipitated by intense mental or physical stress. Case presentation A 64-year-old male with a known diagnosis of moderate-to-severe aortic stenosis and advised that valve replacement was not urgent, presented with acute pulmonary edema following extraordinary mental distress. The patient was misdiagnosed as having a "massive heart attack" and...

  14. Inflammation: a trigger for acute coronary syndrome

    International Nuclear Information System (INIS)

    SAGER, Hendrik B.; NAHRENDORF, Matthias

    2016-01-01

    Atherosclerosis is a chronic inflammatory disease of the vessel wall and a major cause of death worldwide. One of atherosclerosis’ most dreadful complications are acute coronary syndromes that comprise ST-segment elevation myocardial infarction, non-ST-segment elevation myocardial infarction, and unstable angina. We now understand that inflammation substantially contributes to the initiation, progression, and destabilization of atherosclerosis. In this review, we will focus on the role of inflammatory leukocytes, which are the cellular protagonists of vascular inflammation, in triggering disease progression and, ultimately, the destabilization that causes acute coronary syndromes.

  15. [Acute pseudoobstruction of the colon (Ogilvie syndrome)].

    Science.gov (United States)

    Lausen, M; Fiedler, L; Hauenstein, K H; Farthmann, E H

    1985-02-01

    Pseudo-obstruction of the colon was observed in six patients. The cardinal feature is acute distension of the large bowel without distal obstruction. Apart from few idiopathic cases, the syndrome usually is associated with postoperative, posttraumatic or metabolic disorders of extraintestinal origin. Without treatment, increasing distension leads to cecal perforation with a high mortality. Treatment is conservative initially. If decompression by colonoscopy fails, cecostomy or right hemicolectomy are mandatory. The pathophysiologic mechanism of acute colonic pseudoobstruction is unknown. Whether Ogilvie's syndrome is a genuine clinical entity or a complication of associated diseases is still on question.

  16. AP-1 Oligodeoxynucleotides Reduce Aortic Elastolysis in a Murine Model of Marfan Syndrome.

    Science.gov (United States)

    Arif, Rawa; Zaradzki, Marcin; Remes, Anca; Seppelt, Philipp; Kunze, Reiner; Schröder, Hannes; Schwill, Simon; Ensminger, Stephan M; Robinson, Peter N; Karck, Matthias; Müller, Oliver J; Hecker, Markus; Wagner, Andreas H; Kallenbach, Klaus

    2017-12-15

    Marfan syndrome is characterized by high expression of matrix metalloproteinases (MMPs) in aortic smooth muscle cells (AoSMCs) associated with medial elastolysis and aortic root aneurysm. We aimed to reduce aortic elastolysis through decrease of MMP expression with decoy oligodeoxynucleotides (dODNs) neutralizing the transcription factor activating factor-1 (AP-1). AP-1 abundance in nuclear extracts as well as MMP-2 and MMP-9 expression were significantly increased in isolated mAoSMC of mgR/mgR Marfan mice compared to wild-type cells. Exposure to AP-1 neutralizing dODNs resulted in a significant reduction of basal and interleukin-1β-stimulated MMP expression and activity in mAoSMCs. Moreover, increased migration and formation of superoxide radical anions was substantially decreased in mAoSMCs by AP-1 dODN treatment. Aortic grafts from donor Marfan mice were treated with AP-1- dODN ex vivo and implanted as infrarenal aortic interposition grafts in mgR/mgR mice. Pretreatment of aortic grafts with AP-1 dODN led to reduced elastolysis, macrophage infiltration, and MMP activity. Permeability of the endothelial monolayer was increased for dODN in mgR/mgR aortae with observed loss of tight junction proteins ZO-1 and occludin, enabling dODN to reach the tunica media. Targeting AP-1 activity offers a new potential strategy to treat the vascular phenotype associated with Marfan syndrome. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

  17. Aortic valve-sparing in 37 patients with Marfan syndrome: midterm results with David operation.

    Science.gov (United States)

    Forteza, Alberto; De Diego, Javier; Centeno, Jorge; López, Maria Jesus; Pérez, Enrique; Martín, Carlos; Sánchez, Violeta; Rufilanchas, Juan J; Cortina, Jose

    2010-01-01

    We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years. Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 +/- 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 +/- 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 +/- 16 months (range, 1 to 61 months). There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation. Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. Endovascular treatment of acute type B dissection complicating graft-bypass repair for aortic coarctation

    Directory of Open Access Journals (Sweden)

    Serkan Ertugay

    Full Text Available Objectives: The early dissection of the descending aorta after the repair of aortic coarctation is very rare. Herein, we present a special endovascular technique used for acute type B dissection complicating graft bypass for aortic coarctation. Methods: The 48 year-old male patient with the diagnosis of adult type aortic coarctation had bypass procedure between the aortic arch and the descending aorta. Six weeks after the first operation, the patient was readmitted with severe back pain and had the diagnosis of acute type B dissection which involved the descending aorta at the distal part of the graft anastomosis. Results: Two separate stent-grafts were deployed respectively 31 × 150 mm and 34 × 200 mm (C-TAG™ WL Gore&Asc., Flagstaff, AZ, USA. The previous Dacron bypass graft was used as a proximal landing zone for the first stent-graft. The distal landing zone for the second stent was the area between the celiac trunk and superior mesenteric artery. Therefore, the covered stent-graft was implanted to the celiac trunk (Viabahn™ 7 × 50 mm WL Gore&Asc, AZ, USA to maintain its patency before the deployment of the second graft. The segment of coarctation was closed with a vascular plug (Amplatzer™ vascular plug II to prevent persistent perfusion of aneurysmal false lumen. Conclusion: The endovascular approach offers multiple less invasive options based on a patient-specific problem. Keywords: Aortic coarctation, Bypass graft, Endovascular, Vascular plug

  19. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome

    Science.gov (United States)

    2014-01-01

    Background Bicuspid aortic valve (BAV) is the most frequent congenital heart disease with frequent involvement in thoracic aortic dilatation, aneurysm and dissection. Although BAV and Marfan syndrome (MFS) share some clinical features, and some MFS patients with BAV display mutations in FBN1, the gene encoding fibrillin-1, the genetic background of isolated BAV is poorly defined. Methods Ten consecutive BAV patients [8 men, age range 24–42 years] without MFS were clinically characterized. BAV phenotype and function, together with evaluation of aortic morphology, were comprehensively assessed by Doppler echocardiography. Direct sequencing of each FBN1 exon with flanking intron sequences was performed on eight patients. Results We detected three FBN1 mutations in two patients (aged 24 and 25 years) displaying aortic root aneurysm ≥50 mm and moderate aortic regurgitation. In particular, one patient had two mutations (p.Arg2726Trp and p.Arg636Gly) one of which has been previously associated with variable Marfanoid phenotypes. The other patient showed a pArg529Gln substitution reported to be associated with an incomplete MFS phenotype. Conclusions The present findings enlarge the clinical spectrum of isolated BAV to include patients with BAV without MFS who have involvement of FBN1 gene. These results underscore the importance of accurate phenotyping of BAV aortopathy and of clinical characterization of BAV patients, including investigation of systemic connective tissue manifestations and genetic testing. PMID:24564502

  20. Sandwich Technique for Endovascular Repair of Acute Type A Aortic Dissection.

    Science.gov (United States)

    Gao, Feng; Zeng, Qian; Lin, Fangming; Ge, Xiaohu

    2017-10-01

    To describe a new endovascular procedure for acute type A aortic dissection (TAAD) repair. Between 2013 and 2016, 12 patients (average age 54±9.6 years; 10 men) with acute TAAD (mean EURO score 11.4%±3.2%, range 5-17) and unfit for surgery underwent thoracic endovascular aortic repair (TEVAR) with 2 periscope grafts to preserve blood supply to supra-aortic branches plus bypass grafting as needed. If the ascending aorta was dilated to >40 mm, sternotomy was performed to wrap the ascending aorta and reduce its diameter to accommodate the aortic stent-grafts. All patients were successfully treated. Seven patients required bypass grafting, and most of the patients had periscope grafts to the innominate/right common carotid artery and left common carotid artery; only 3 patients had the left subclavian artery preserved. All patients exhibited good hemodynamics and normal pressures after the procedure. The mean procedure time and blood loss were 4.5±1.0 hours and 217±111.5 mL, respectively. Two patients treated emergently died shortly after surgery from multiorgan failure. The average follow-up duration was 17±14.5 months (range 2-42) in the 10 survivors. The remaining patients recovered and none experienced stent-graft thrombosis, stroke, or peripheral artery embolism during follow-up. A procedure that combines sandwich/periscope grafting with TEVAR, wrapping of the aorta, and supra-arch bypass grafting can be used to treat patients with acute TAAD.

  1. Preoperative renal function and surgical outcomes in patients with acute type A aortic dissection†.

    Science.gov (United States)

    Imasaka, Ken-ichi; Tayama, Eiki; Tomita, Yukihiro

    2015-04-01

    There are few data concerning the impact of preoperative renal function, assessed using estimated glomerular filtration rate, on surgical outcomes following acute type A aortic dissection. We investigated the accuracy of estimated glomerular filtration rate (in ml/min/1.73 m(2)) in predicting in-hospital mortality and postoperative renal replacement therapy in such cases. We reviewed 114 consecutive patients with non-dialysis-dependent renal dysfunction who underwent thoracic aortic surgery for acute type A aortic dissection between 1997 and 2012. Preoperative renal function was categorized as normal (estimated glomerular filtration rate >90; n = 15) or as mild (60-89; n = 39), moderate I (45-59; n = 39), moderate II (30-44; n = 14) or severe (15-29; n = 7) renal dysfunction. In-hospital mortality was 14.9%. Eighteen (15.8%) of 114 patients required renal replacement therapy. A more severe stage stratified by preoperative estimated glomerular filtration rate levels could effectively predict postoperative renal replacement therapy (area under the receiver operating characteristic curve 0.786). The best cut-off value of estimated glomerular filtration rate for predicting postoperative renal replacement therapy was 60 (sensitivity 95%, specificity 59%). On multiple regression analysis, the independent preoperative and intraoperative risk factors for postoperative renal replacement therapy were estimated glomerular filtration rate (P replacement (P replacement therapy (OR, 5.47; P regression analysis. Preoperative estimated glomerular filtration rate can effectively predict the need for renal replacement therapy after surgery for acute type A aortic dissection. However, it is not an effective diagnostic tool to predict in-hospital mortality. The complexity of the characteristics of patients who undergo surgical procedures may make prediction of surgical outcomes difficult. Risk models to predict hospital mortality and morbidities are needed to assist clinicians in

  2. Serum Biomarker Identification by Mass Spectrometry in Acute Aortic Dissection

    Directory of Open Access Journals (Sweden)

    Yong Ren

    2017-12-01

    Full Text Available Background/Aims: Aortic dissection (AD is also known as intramural hematoma. This study aimed to screen peripheral blood biomarkers of small molecule metabolites for AD using high-performance liquid chromatography-mass spectrometry (HPLC-MS. Methods: Sera from 25 healthy subjects, 25 patients with well-established AD, and 25 patients with well-established hypertension were investigated by HPLC-MS to detect metabolites, screen differentially expressed metabolites, and analyze metabolic pathways. Results: Twenty-six and four metabolites were significantly up- and down-regulated in the hypertensive patients compared with the healthy subjects; 165 metabolites were significantly up-regulated and 109 significantly down-regulated in the AD patients compared with the hypertensive patients. Of these metabolites, 35 were up-regulated and 105 down-regulated only in AD patients. The metabolites that were differentially expressed in AD are mainly involved in tryptophan, histidine, glycerophospholipid, ether lipid, and choline metabolic pathways. As AD alters the peripheral blood metabolome, analysis of peripheral blood metabolites can be used in auxiliary diagnosis of AD. Conclusion: Eight metabolites are potential biomarkers for AD, 3 of which were differentially expressed and can be used for auxiliary diagnosis of AD and evaluation of treatment effectiveness.

  3. Serum Biomarker Identification by Mass Spectrometry in Acute Aortic Dissection.

    Science.gov (United States)

    Ren, Yong; Tang, Qizhu; Liu, Wenwei; Tang, Yongqian; Zhu, Rui; Li, Bin

    2017-01-01

    Aortic dissection (AD) is also known as intramural hematoma. This study aimed to screen peripheral blood biomarkers of small molecule metabolites for AD using high-performance liquid chromatography-mass spectrometry (HPLC-MS). Sera from 25 healthy subjects, 25 patients with well-established AD, and 25 patients with well-established hypertension were investigated by HPLC-MS to detect metabolites, screen differentially expressed metabolites, and analyze metabolic pathways. Twenty-six and four metabolites were significantly up- and down-regulated in the hypertensive patients compared with the healthy subjects; 165 metabolites were significantly up-regulated and 109 significantly down-regulated in the AD patients compared with the hypertensive patients. Of these metabolites, 35 were up-regulated and 105 down-regulated only in AD patients. The metabolites that were differentially expressed in AD are mainly involved in tryptophan, histidine, glycerophospholipid, ether lipid, and choline metabolic pathways. As AD alters the peripheral blood metabolome, analysis of peripheral blood metabolites can be used in auxiliary diagnosis of AD. Eight metabolites are potential biomarkers for AD, 3 of which were differentially expressed and can be used for auxiliary diagnosis of AD and evaluation of treatment effectiveness. © 2017 The Author(s). Published by S. Karger AG, Basel.

  4. Effects of atypical pneumonia agents on progression of atherosclerosis and acute coronary syndrome.

    Science.gov (United States)

    Iriz, Erkan; Cirak, Meltem Yalinay; Engin, Evren Doruk; Zor, Mustafa Hakan; Erer, Dilek; Imren, Yildirim; Turet, Sevgi; Halit, Velit

    2007-12-01

    The aim of this study was to investigate the presence of various atypical pneumonia agents (Chlamydia pneumoniae, cytomegalovirus, Mycoplasma pneumoniae), which are considered to have a role in the ethiopathogenesis of atherosclerosis, in aortic biopsies without macroscopically visible plaque and in internal thoracic artery biopsies. Thirty-three patients (group 1), who had undergone coronary bypass operation and 10 non-atherosclerotic patients (group 2), were included in the study. Seventy-six tissue biopsies were taken. Biopsies from the patients in group 1 a were obtained from the atheroma plaque-free aortic tissue and 33 biopsies (group Ib) were obtained from their internal thoracic arteries. Following DNA extraction, nested PCR was used to detect Chlamydia pneumoniae DNA, and real time PCR was used to detect cytomegalovirus and Mycoplasma pneumoniae DNA. Blood parameters (lipid profile, CRP, fibrinogen) of the patients and operation characteristics were recorded. Chlamydia pneumoniae DNA was detected in 5 of 33 biopsy samples from coronary bypass patients, whereas none of the control patients (group 1b and group 2) were positive for this agent (P = 0.001). Neither CMV nor Mycoplasma pneumoniae was detected in IMA and aortic biopsies of both bypass and control patients. Elevated total cholesterol levels (P = 0.02) and positive CRP (P = 0.001) was found in C. pneumoniae positive patients. Prevalence of acute coronary syndrome was significantly higher in C. pneumoniae detected patients compared (P = 0.00 1). Detection of C. pneumoniae DNA in the atheroma free aortic biopsies might indicate that this micro-organism intervened in the progression of atheroma plaque. There was a strong relationship between the detection of this micro-organism in the aortic wall and acute coronary syndrome. The absence of DNA of the corresponding micro-organisms in the IMA wall may show its resistance to infective agents and in turn to atherosclerosis, which is a result of the

  5. Perturbed sympatho-vagal balance in Turner syndrome - relation to aortic dilation

    DEFF Research Database (Denmark)

    Trolle, Christian; Mortensen, Kristian Havmand; Andersen, Niels Holmark

    Objective: The risk of aortic dissection is 100 fold increased in Turner syndrome (TS). Increased blood pressure (BP) and heart rate is present as well as an increased risk of ischemic heart disease and diabetes. This study aimed to prospectively assess heart rate variability (HRV) in TS and its...... relation to aortic dimensions. Methods: Adults with TS (n=91, aged 37.4±10.4 years) recruited through the Danish National Society of Turner Syndrome Contact Group and an endocrine outpatient clinic were examined thrice (mean follow-up of 4.7±0.5 years). Healthy controls (n=64, aged 39.4±12.1 years) were...

  6. Apical Hypertrophic Cardiomyopathy Mimicking Acute Coronary Syndrome

    Directory of Open Access Journals (Sweden)

    Lütfü Bekar

    2013-03-01

    Full Text Available Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy and it can be mistaken for coronary artery disease due to the symptoms and electrocardiography findings. In this report, we aimed to present a patient referred to our clinic with complaints of chest pain and electrocardiography findings who had been misdiagnosed as non-ST elevation acute coronary syndrome.

  7. Acute cardiac failure in neuroleptic malignant syndrome.

    LENUS (Irish Health Repository)

    Sparrow, Patrick

    2012-02-03

    We present a case of rapid onset acute cardiac failure developing as part of neuroleptic malignant syndrome in a 35-year-old woman following treatment with thioridazine and lithium. Post mortem histology of cardiac and skeletal muscle showed similar changes of focal cellular necrosis and vacuolation suggesting a common disease process.

  8. Imaging in severe acute respiratory syndrome (SARS)

    International Nuclear Information System (INIS)

    Antonio, G.E.; Wong, K.T.; Chu, W.C.W.; Hui, D.S.C.; Cheng, F.W.T.; Yuen, E.H.Y.; Chung, S.S.C.; Fok, T.F.; Sung, J.J.Y.; Ahuja, A.T.

    2003-01-01

    Severe acute respiratory syndrome (SARS) is a highly infectious disease caused by a novel coronavirus, and has become pandemic within a short period of time. Imaging plays an important role in the diagnosis, management and follow-up of patients with SARS. The current status of imaging in SARS is presented in this review

  9. Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures.

    Science.gov (United States)

    Price, Joel; Magruder, J Trent; Young, Allen; Grimm, Joshua C; Patel, Nishant D; Alejo, Diane; Dietz, Harry C; Vricella, Luca A; Cameron, Duke E

    2016-02-01

    Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined. Adult patients with Marfan syndrome who had Bentall or aortic valve-sparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact. One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n = 98; 69 reimplantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P = .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P = .003), more aortic dissections (25.4% vs 4.1%; P Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  10. Aortic root operations for Marfan syndrome: a comparison of the Bentall and valve-sparing procedures.

    Science.gov (United States)

    Patel, Nishant D; Weiss, Eric S; Alejo, Diane E; Nwakanma, Lois U; Williams, Jason A; Dietz, Harry C; Spevak, Philip J; Gott, Vincent L; Vricella, Luca A; Cameron, Duke E

    2008-06-01

    We compared results of the Bentall procedure with valve-sparing aortic root replacement (VSRR) for aortic root aneurysm in Marfan syndrome. Marfan syndrome patients who had the Bentall procedure or VSRR at our institution between April 1997 and September 2006 were identified. Follow-up information was obtained from hospital charts and contact with patients or their physicians. Kaplan-Meier survival and propensity score analyses were performed. One hundred forty Marfan syndrome patients had either the Bentall procedure (n = 56) or VSRR (n = 84; 40 remodeling and 44 reimplantation). Bentall patients were older than VSRR patients (38 versus 29 years; p = 0.0001) and had more aortic dissections (16% versus 1%; p = 0.0012); more urgent/emergent surgery (20% versus 2%; p = 0.0008); larger preoperative sinus diameter (5.7 versus 5.1 cm; p = 0.0004); and more preoperative 3+/4+ aortic insufficiency (59% versus 10%; p valve replacement was 90% for VSRR patients. Eight-year survival was 90% for Bentall and 100% for VSRR patients (p = 0.01). Propensity-adjusted regression showed that the Bentall procedure did not predict mortality (p = 1.00) and did not protect from reoperation (odds ratio = 0.28; 95% confidence interval: 0.01 to 4.33; p = 0.36). The Bentall procedure and VSRR have similar operative results in Marfan syndrome. The procedures are distinguished by higher rates of thromboembolism among Bentall patients and higher rates of reoperation among VSRR patients. Lower late survival among Bentall patients probably reflects the preferential use of the Bentall procedure for higher risk patients.

  11. Demonstration of ascending aorta in hypoplastic left heart syndrome with aortic atresia by balloon occlusion aortography

    International Nuclear Information System (INIS)

    Lo, R.N.S.; Leung, M.P.; Lau, K.C.

    1987-01-01

    Balloon occlusion angiography in the descending aorta produced clear retrograde visualization of the hypoplastic ascending aorta and related structures in eighteen neonates with aortic atresia. Transient bradycardia was the only complication observed. It is technically simpler than retrograde cannulation of the aorta via an arteriotomy and should be the method of choice when cardiac catheterization is required in patients with the hypoplastic left heart syndrome. (orig.)

  12. Radiobiology of the acute radiation syndrome

    International Nuclear Information System (INIS)

    Garau, M. M.; Calduch, A. L.; Lopez, E. C.

    2011-01-01

    Acute radiation syndrome or acute radiation sickness is classically subdivided into three sub syndromes: the hematopoietic, gastrointestinal and neurovascular syndrome but many other tissues can be damaged. The time course and severity of clinical signs and symptoms are a function of the overall body volume irradiated, the inhomogeneity of dose exposure, the particle type, the absorbed dose and the dose rate. Classical pathophysiology explain the failure of each of these organs and the timing of appearance of their signs and symptoms due to radiation-induced cytocidal effects of a great number of parenchymal cells of hierarchically organized tissues. Contemporaneously, many other radiation-induced effects has been described and all of them may lead to tissue injury with their corresponding signs and symptoms that can be expressed after short or long period of time. Radiation-induced multi-organ involvement is thought to be due to radiation-induced systemic inflammatory response mediated by released pro-inflammatory cytokines. (authors)

  13. Late Pseudocoarctation Syndrome After Stent-Graft Implantation For Traumatic Aortic Rupture

    International Nuclear Information System (INIS)

    Letocart, Vincent; Fau, Georges; Tirouvanziam, Ashok; Toquet, Claire; Al Habash, Oussama; Guerin, Patrice; Rousseau, Hervé; Crochet, Dominique

    2013-01-01

    The present observation illustrates an unusual complication occurring after stent-grafting (S-graft) for aortic isthmus rupture. A 22-year-old patient, treated by S-graft in the emergency department for traumatic aortic rupture, was readmitted 10 months later with pseudocoarctation syndrome. A membrane was found inside the stent-graft that had induced a pseudo-dissection, which caused the pseudocoarctation syndrome. Surgical treatment consisted of removing the stent-graft and membrane and replacing it with a vascular implant. The patient’s clinical course was fair. The suggested mechanism was circumferential neoendothelialization of the stent-graft. Dehiscence caused the superior part of the membrane to drop into the lumen of the stent-graft creating a “false channel” that compressed the “true lumen” and induced “pseudocoarctation” syndrome. The cause of the extensive neointimalization remains unexplained. Thoracic aortic stent-grafts require regular follow-up monitoring by angioscan or angio-magnetic resonance imaging.

  14. Late Pseudocoarctation Syndrome After Stent-Graft Implantation For Traumatic Aortic Rupture

    Energy Technology Data Exchange (ETDEWEB)

    Letocart, Vincent, E-mail: vincent.letocart@chu-nantes.fr; Fau, Georges, E-mail: georges.fau@chu-nantes.fr; Tirouvanziam, Ashok, E-mail: ashok.tirouvanziam@chu-nantes.fr [University Hospital of Nantes, Institut du Thorax (France); Toquet, Claire, E-mail: claire.toquet@chu-nantes.fr [University Hospital of Nantes, Department of Pathology (France); Al Habash, Oussama, E-mail: oussama.alhabash@chu-nantes.fr; Guerin, Patrice, E-mail: patrice.guerin@chu-nantes.fr [University Hospital of Nantes, Institut du Thorax (France); Rousseau, Herve, E-mail: rousseau.h@chu-toulouse.fr [University Hospital of Toulouse, Department of Radiology (France); Crochet, Dominique, E-mail: dominique.crochet@chu-nantes.fr [University Hospital of Nantes, Institut du Thorax (France)

    2013-06-15

    The present observation illustrates an unusual complication occurring after stent-grafting (S-graft) for aortic isthmus rupture. A 22-year-old patient, treated by S-graft in the emergency department for traumatic aortic rupture, was readmitted 10 months later with pseudocoarctation syndrome. A membrane was found inside the stent-graft that had induced a pseudo-dissection, which caused the pseudocoarctation syndrome. Surgical treatment consisted of removing the stent-graft and membrane and replacing it with a vascular implant. The patient's clinical course was fair. The suggested mechanism was circumferential neoendothelialization of the stent-graft. Dehiscence caused the superior part of the membrane to drop into the lumen of the stent-graft creating a 'false channel' that compressed the 'true lumen' and induced 'pseudocoarctation' syndrome. The cause of the extensive neointimalization remains unexplained. Thoracic aortic stent-grafts require regular follow-up monitoring by angioscan or angio-magnetic resonance imaging.

  15. CASE OF EFFECTIVE REPERFUSION THERAPY IN PATIENTS WITH ACUTE CEREBROVASCULAR ACCIDENT AND ACUTE CORONARY SYNDROME

    Directory of Open Access Journals (Sweden)

    L. V. Timchenko

    2015-01-01

    Full Text Available ABSTRACT. Currently, acute cerebrovascular accident is an extremely important medical and social issue. Stroke is a major cause of disability in the population and takes a leading place among the causes of death.Modern high­tech endovascular treatment of the ischemic stroke (IS and acute coronary syndrome (ACS includes effective methods of reperfusion of the brain and myocardium within first hours of the disease.In this paper, we report two clinical examples of high­tech reperfusion endovascular treatment in the same patient at an intervals of 1 year and 6 months in the development of clinical picture of the stroke and ACS in Regional Clinical hospital № 1 n.a. Prof. S.V. Ochapovsky.In the first case, intra­aortic TLT of two cerebral arteries (ACA and MCA was performed simultaneously with delayed recanalization and complete regression of focal neurological symptoms. And in the second case, selective thrombolytic therapy was performed in the acute phase of stroke, which developed few hours after PTCA RCA in ACS, with a good clinical effect. 

  16. Neuroleptic-induced acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Francisco Garcia Soriano

    Full Text Available CONTEXT: A case of neuroleptic malignant syndrome and acute respiratory distress syndrome is presented and discussed with emphasis on the role of muscle relaxation, creatine kinase, and respiratory function tests. CASE REPORT: A 41-year-old man presented right otalgia and peripheral facial paralysis. A computed tomography scan of the skull showed a hyperdense area, 2 cm in diameter, in the pathway of the anterior intercommunicating cerebral artery. Preoperative examination revealed: pH 7.4, PaCO2 40 torr, PaO2 80 torr (room air, Hb 13.8 g/dl, blood urea nitrogen 3.2 mmol/l, and creatinine 90 mmol/l. The chest x-ray was normal. The patient had not eaten during the 12-hour period prior to anesthesia induction. Intravenous halothane, fentanyl 0.5 mg and droperidol 25 mg were used for anesthesia. After the first six hours, the PaO2 was 65 torr (normal PaCO2 with FiO2 50% (PaO2/FiO2 130, and remained at this level until the end of the operation 4 hours later, maintaining PaCO2 at 35 torr. A thrombosed aneurysm was detected and resected, and the ends of the artery were closed with clips. No vasospasm was present. This case illustrates that neuroleptic drugs can cause neuroleptic malignant syndrome associated with acute respiratory distress syndrome. Neuroleptic malignant syndrome is a disease that is difficult to diagnose. Acute respiratory distress syndrome is another manifestation of neuroleptic malignant syndrome that has not been recognized in previous reports: it may be produced by neuroleptic drugs independent of the manifestation of neuroleptic malignant syndrome. Some considerations regarding the cause and effect relationship between acute respiratory distress syndrome and neuroleptic drugs are discussed. Intensive care unit physicians should consider the possibility that patients receiving neuroleptic drugs could develop respiratory failure in the absence of other factors that might explain the syndrome.

  17. Aortic root surgery in Marfan syndrome: Bentall procedure with the composite mechanical valved conduit versus aortic valve reimplantation with Valsalva graft.

    Science.gov (United States)

    Nardi, Paolo; Pellegrino, Antonio; Versaci, Francesco; Mantione, Ludmilla; Polisca, Patrizio; Iorio, Fiore S; Chiariello, Luigi

    2010-09-01

    The aim of the study is to compare mid-term results of Bentall aortic root replacement with composite mechanical valved conduit and aortic valve reimplantation procedure using the Valsalva graft for the treatment of aortic root aneurysm in patients with Marfan syndrome. We retrospectively compared data of 23 patients (mean age 38 + or - 14 years) who had undergone the Bentall procedure (group B) to those of 24 patients (mean age 36 + or - 12 years) who had undergone aortic valve reimplantation (group R) during a 14-year period. Follow-up (mean duration 65 + or - 44 months) was 100% complete. There were no operative deaths in either group. In group B, as compared with group R, preoperative aortic insufficiency (3.2 + or - 1.1/4 vs. 1.7 + or - 1.4/4, P Marfan patients, the Bentall procedure is associated with excellent mid-term outcome. The reimplantation technique, adopted for less dilated aortas, provides similarly satisfactory results. The Valsalva graft seems, with time, to allow a stable aortic valve function.

  18. [Clinical demonstrations: Heart rupture in acute myocardial infarct. Infectious endocarditis. Wolff-Parkinson-White syndrome].

    Science.gov (United States)

    Nager, F

    1984-12-08

    This clinical demonstration includes three topics of clinical cardiology: myocardial rupture in acute myocardial infarction, infective endocarditis, and WPW-syndrome with paroxysmal supraventricular tachycardia. In the first part three cases with septal perforation or papillary muscle rupture are demonstrated. Our experience with myocardial rupture (free wall, septum, papillary muscle) during the last six years is summarized with special reference to the significance and the differential diagnosis of systolic regurgitant murmurs after myocardial infarction. Special features of acute mitral incompetence (papillary muscle dysfunction) in myocardial infarction are outlined and diagnostic guidelines for differentiation between septal perforation and papillary muscle rupture are discussed. In the second part two patients with aortic (e.g. mitral) valve rupture in the course of infective endocarditis are presented. The synoptic comparison of these two patients is related to the results of our own clinical studies on the changing pattern of infective endocarditis (epidemiologically, clinically) during the last three decades. The clinical picture of acute aortic valve rupture is outlined and the bedside signs indicating catastrophic complications of infective endocarditis are summarized. In the third part the odyssey of a patient with WPW-syndrome and consecutive paroxysmal supraventricular tachycardia is described. Progress in electrophysiological analysis of the re-entry circles in preexcitation syndromes is outlined.

  19. Metabolic syndrome and its effect on aortic stiffness in premenopausal women.

    Science.gov (United States)

    Simkova, A; Bulas, J; Balogova, S; Reptova, A; Kisa, B; Luha, J; Kinova, S

    2013-01-01

    Metabolic syndrome (MS) is a cluster of proatherogenic risk factors (RF) (abdominal obesity, atherogenic dyslipidemia, impaired fasting glucose or type 2 diabetes mellitus, higher blood pressure or antihypertensive therapy) that move patients into a higher risk for development of cardiovascular disease (CVD) and type 2 diabetes. The preclinical (subclinical) target organ diseases (SOD) are early signs of atherosclerosis. An increased aortic stiffness characterised by an increased pulse wave velocity in aorta (PWV Ao) is one of SOD.The aim of the present study was to assess the impact of metabolic syndrome (MS) on aortic wall stiffness and the risk profile in premenopausal women. The aortic stiffness was measured using Arteriograph-Tensiomed, based on oscillometric measurement and analysis of the shape of brachial pulse wave, giving the PWV Ao. The results of measurements characterise a global aortic stiffness. We examined 81 premenopausal women (without history of CVD). The MS (according to the 2009 "harmonizing" definition) was present in 31 women (mean age 41.5 y), in the control group, there were 50 women (39 y). The most frequent components of MS were abdominal obesity (93 % vs 42%), arterial hypertension (68 % vs 10 %) and dyslipidemia (29 % vs 8 %). The PWV Ao was significantly higher in women with MS (9.26 m/s) compared to the control group (7.44 m/s). The aortic stiffness in women with MS compared to controls was significantly higher despite a presumed general protective hormonal effect on cardiovascular system in women with child-bearing potential (Tab. 4, Ref. 25).

  20. Using D-Dimer to Diagnose Painless Acute Aortic Dissection: A Case Report

    Directory of Open Access Journals (Sweden)

    Caroline Barniol

    2011-01-01

    Case Presentation. We report the case of a 66-year-old Caucasian male who presented a syncope immediately followed by a left-arm weakness while driving his car. Clinical examination was normal, but bilateral jugular vein distension was noted. Electrocardiogram and chest radiography were unremarkable. Among blood tests performed, troponin I test result was negative, and D-dimer test concentration was >4000 ng/mL. Since D-dimer test result was positive, chest computer tomography angiogram was performed and found a thoracic aortic dissection. Conclusion. Our case report shows that acute aortic dissection diagnosis is difficult and must be associated with the interpretation of various clinical signs and D-dimer measurement. It could be helpful for the emergency physician to have a pretest probability D-dimer like in pulmonary embolism diagnosis.

  1. Evaluation of patients with acute vestibular syndrome.

    Science.gov (United States)

    Thabet, Elsaeid

    2008-03-01

    Acute vestibular syndrome is characterized by a rapid unilateral injury to either peripheral or central vestibular structures. It consists of severe vertigo, nausea and vomiting, spontaneous nystagmus, and postural instability. In many cases, a peripheral etiology is considered although it may be due to an underlying serious central pathology. The present study was designed to investigate the feasibility of differentiating the cause of acute vestibular syndrome in such patients using clinical, audiovestibular and radiologic tools. We performed a case series study of patients complaining of acute vertigo at a university referring center for hearing and balance disorders. Thirty patients with history of acute vertigo within 3 days onset with no history of previous otological or neurological disorders. Eighteen patients were due to acute peripheral vestibular lesion, 1 due to psychiatric illness under antidepressant drugs and 11 were of central vestibular lesion. The most important step in the diagnosis of acute vertigo is a thorough and detailed history. The common error of carrying out investigations in place of a detailed history is to be avoided. The clinical evaluation has the highest sensitivity and specificity in differentiating central from peripheral vestibular lesions.

  2. Emergency endovascular management of acute thoracic aortic pathology A safe and feasible option.

    Science.gov (United States)

    Ahmed, Z; McHugh, S M; Elmallah, A; Colgan, M P; O'Callaghan, A; O'Neill, S M; Madhavan, P; Martin, Z

    2017-12-01

    Endovascular repair has revolutionised the emergency treatment of thoracic aortic disease. We report our 10 year experience using this treatment in emergency cases. A prospectively maintained vascular database was analysed. Patients' medical records and CT images stored on the hospital PACS system were also reviewed. Statistical analysis was done using IBM SPSS V21. There were a total of 59 thoracic aortic stenting procedures of which 33 (60% males with a mean age of 58 yrs) were performed for emergency thoracic pathologies: traumatic transection (n = 10), ruptured aneurysm (n = 6), non-traumatic dissection (n = 8) and penetrating aortic ulcer (n = 9). All patients had self-expanding endografts implanted. Two patients required debranching procedures before the endovascular treatment. Thirty-day mortality was 12.1% (4/33). 70% of patients received a single device. There were 7 procedure related complications, 6 requiring re-intervention: thoracotomy and drainage in 2 patients, proximal graft extension in 2, open drainage of groin haematoma in 1 and open repair of a common femoral artery pseudo-aneurysm in one patient. In total 23 patients were transferred from 11 centres nationwide. There were no mortalities or other complications related to patient transfer from peripheral centres. Although acute thoracic aortic pathology is life threatening, appropriate blood pressure management and treatment of associated injuries can result in favourable outcomes. Endovascular repair is a safe and effective treatment option which enables patients to be treated with reduced morbidity and mortality. Transfer of patients with acute pathology to a tertiary centre can safely be performed. Copyright © 2016 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.

  3. Acute glomerulonephritis mimicking nephrotic syndrome

    African Journals Online (AJOL)

    2016-01-04

    Jan 4, 2016 ... Abstract: Background: Acute post infectious glomerulonephritis. (APIGN) describes a wide range of glomerulonephritis character- ized by an immunologic response of the kidney to varieties of infec- tious agent commonly bacteria. It is characterized by an abrupt on- set of haematuria, moderate oedema ...

  4. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome

    Directory of Open Access Journals (Sweden)

    Lu-Hang Liu

    2018-04-01

    Full Text Available Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Key Words: FBN1, losartan, neonatal Marfan syndrome

  5. Induction of Macrophage Chemotaxis by Aortic Extracts from Patients with Marfan Syndrome Is Related to Elastin Binding Protein

    Science.gov (United States)

    Guo, Gao; Gehle, Petra; Doelken, Sandra; Martin-Ventura, José Luis; von Kodolitsch, Yskert; Hetzer, Roland; Robinson, Peter N.

    2011-01-01

    Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and dissection are the major determinants of premature death in untreated patients. In previous work, we showed that extracts of aortic tissues from the mgR mouse model of Marfan syndrome showed increased chemotactic stimulatory activity related to the elastin-binding protein. Aortic samples were collected from 6 patients with Marfan syndrome and 8 with isolated aneurysms of the ascending aorta. Control samples were obtained from 11 organ donors without known vascular or connective tissue diseases. Soluble proteins extracted from the aortic samples of the two patient groups were compared against buffer controls and against the aortic samples from controls with respect to the ability to induce macrophage chemotaxis as measured using a modified Boyden chamber, as well as the reactivity to a monoclonal antibody BA4 against bioactive elastin peptides using ELISA. Samples from Marfan patients displayed a statistically significant increase in chemotactic inductive activity compared to control samples. Additionally, reactivity to BA4 was significantly increased. Similar statistically significant increases were identified for the samples from patients with idiopathic thoracic aortic aneurysm. There was a significant correlation between the chemotactic index and BA4 reactivity, and the increases in chemotactic activity of extracts from Marfan patients could be inhibited by pretreatment with lactose, VGVAPG peptides, or BA4, which indicates the involvement of EBP in mediating the effects. Our results demonstrate that aortic extracts of patients with Marfan syndrome can elicit macrophage chemotaxis, similar to our previous study on aortic extracts of the mgR mouse model of Marfan syndrome (Guo et al., Circulation 2006; 114:1855-62). PMID:21647416

  6. Induction of macrophage chemotaxis by aortic extracts from patients with Marfan syndrome is related to elastin binding protein.

    Directory of Open Access Journals (Sweden)

    Gao Guo

    Full Text Available Marfan syndrome is an autosomal dominantly inherited disorder of connective tissue with prominent skeletal, ocular, and cardiovascular manifestations. Aortic aneurysm and dissection are the major determinants of premature death in untreated patients. In previous work, we showed that extracts of aortic tissues from the mgR mouse model of Marfan syndrome showed increased chemotactic stimulatory activity related to the elastin-binding protein. Aortic samples were collected from 6 patients with Marfan syndrome and 8 with isolated aneurysms of the ascending aorta. Control samples were obtained from 11 organ donors without known vascular or connective tissue diseases. Soluble proteins extracted from the aortic samples of the two patient groups were compared against buffer controls and against the aortic samples from controls with respect to the ability to induce macrophage chemotaxis as measured using a modified Boyden chamber, as well as the reactivity to a monoclonal antibody BA4 against bioactive elastin peptides using ELISA. Samples from Marfan patients displayed a statistically significant increase in chemotactic inductive activity compared to control samples. Additionally, reactivity to BA4 was significantly increased. Similar statistically significant increases were identified for the samples from patients with idiopathic thoracic aortic aneurysm. There was a significant correlation between the chemotactic index and BA4 reactivity, and the increases in chemotactic activity of extracts from Marfan patients could be inhibited by pretreatment with lactose, VGVAPG peptides, or BA4, which indicates the involvement of EBP in mediating the effects. Our results demonstrate that aortic extracts of patients with Marfan syndrome can elicit macrophage chemotaxis, similar to our previous study on aortic extracts of the mgR mouse model of Marfan syndrome (Guo et al., Circulation 2006; 114:1855-62.

  7. Bicuspid aortic valve syndrome and fibrillinopathies: potential impact on clinical approach

    Directory of Open Access Journals (Sweden)

    Rosina De Cario

    2014-01-01

    Full Text Available Bicuspid aortic valve (BAV is a common heterogeneous disorder whose natural history is determined by hemodynamic valvular impairment and/or increased prevalence of aortic abnormalities ranging from dilatation to aneurysm and dissection. BAV-related aortopathy is frequently associated with relevant aortic pathologic changes leading to structural alterations, characteristic degenerative lesions and histological changes of the aorta very similar to those identified and described in patients with Marfan syndrome (MFS, an inherited connective tissue disorder associated with mutations in fibrillin 1 (FBN1 gene in more than 90% of patients. Recently, a 4-fold increase in the prevalence of BAV in MFS patients has been reported. Subsequently, pathogenetic FBN1 mutations in patients with BAV and aortic dilatation/aneurysm in whom MFS and other more severe type 1 fibrillinopathies were clinically excluded have been identified. In this review we discuss how this evidence, together with that of the wide heterogeneity in pathogenetic mechanisms of BAV-related aortopathy, may impact the clinical management of BAV.

  8. Acute gastrointestinal bleeding following aortic valve replacement in a patient with Heyde's sindrome. Case report.

    Science.gov (United States)

    De Palma, G D; Salvatori, F; Masone, S; Simeoli, I; Rega, M; Celiento, M; Persico, G

    2007-09-01

    A 58-year old man was admitted to the hospital because of melena. He had a 1-year history of mechanical aortic valve replacement and coronary stent placement because of myocardial infarction and he was taking warfarin and clopidogrel. Esophagogastroduodenoscopy and colonoscopy were negative for bleeding. Capsule endoscopy showed bleeding diffuse angiodysplasia of the small bowel. The patient was treated with octreotide 20 mg, at monthly interval. After 25 months there had been no recurrence of gastrointestinal bleeding. The case suggests that mechanical valve replacement may not prevent gastrointestinal bleeding in Heyde syndrome and that octreotide treatment should be considered in these cases.

  9. The Provisional Extension To Induce Complete Attachment (PETTICOAT) technique to promote distal aortic remodelling in repair of acute DeBakey type I aortic dissection: preliminary results.

    Science.gov (United States)

    Hsu, Hung-Lung; Chen, Yin-Yin; Huang, Chun-Yang; Huang, Jih-Hsin; Chen, Jer-Shen

    2016-07-01

    To report our preliminary results of an aggressive technique, the Provisional Extension To Induce Complete Attachment (PETTICOAT), in repair of acute DeBakey type I aortic dissection. From April 2014 to November 2014, 18 patients with acute DeBakey type I aortic dissection were reviewed retrospectively. Nine patients underwent open repair combined with proximal stent grafting and distal bare stenting (PETTICOAT group). For comparison, another 9 patients underwent open repair combined with proximal stent grafting (NON-PETTICOAT group) were included. Open repair entailed ascending aorta plus total arch replacement under circulatory arrest, with variable aortic root work. Mortality and morbidity were recorded, and computed tomography was performed to evaluate the aortic remodelling at 6 months postoperatively. Preoperative parameters were similar. In the PETTICOAT group, one early mortality was noted. One complication of cardiac tamponade and sternal wound infection led to reopen surgeries. In the NON-PETTICOAT group, one case of transient ischaemic attack took place. Compared with the NON-PETTICOAT group, a significant increase in diameter of true lumen (median, 0.6 vs 0.1 mm, P University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  10. Hybrid endovascular treatment of an aortic root and thoracoabdominal aneurysm in a high-risk patient with Marfan syndrome.

    Science.gov (United States)

    Gelpi, Guido; Mazzaccaro, Daniela; Romagnoni, Claudia; Contino, Monica; Antona, Carlo

    2013-05-01

    This report describes the hybrid endovascular treatment of an aortic root dilatation and a thoracoabdominal aneurysm in a high-risk patient with Marfan syndrome. A 50-year-old male, in hemodialysis for polycystic kidney and polycystic liver, was referred to our department for aortic root dilatation of 5 cm and a 6.3-cm thoracoabdominal aneurysm . He already underwent surgical repair of abdominal aortic aneurysm 10 years ago, complicated by pseudoaneurysm of the proximal anastomosis that had been treated in another center, with an endoprosthesis. The patient underwent aortic root replacement, aortic valve sparing operation, and rerouting of the superior mesenteric artery and celiac trunk to the ascending aorta. The thoracoabdominal aneurysm was excluded with an endoprosthesis few days after the surgical step. The 12-month computed tomography scan confirmed the complete exclusion of the thoracoabdominal aneurysm.

  11. Posterior leukoencephalopathy syndrome in poststretococcal acute glomerulonephritis

    International Nuclear Information System (INIS)

    Bazzino Borzone, F.; Pandolfo Arias, M.; Protasio Palomino, L.; Pujadas Ferrer, M.; Cerisola Cardozo, A.; Gonzalez, G.; Caggiani Malzone, M.; Rubio Santoro, I.

    2005-01-01

    Reversible posterior leukoencephalopathy (LEPR) is a clinical entity that affects radiation usually the white matter of the cerebral hemispheres. It is frequently associated with acute arterial hypertension and immunosuppressive therapy, among other causes. The clinical presentation is varied, with headache, nausea, vomiting, impaired consciousness and abnormal behavior, seizures and visual disturbances, symptoms that often regress. Computed tomography (CT) and magnetic resonance imaging (MRI) images show white matter edema predominantly in posterior regions of the brain. We present a 10 year old boy with leprosy in the course of a nephrotic syndrome secondary to acute diffuse glomerunefritis (GNDA) poststreptococcal. (author) [es

  12. Quantification of aortic and cutaneous elastin and collagen morphology in Marfan syndrome by multiphoton microscopy.

    Science.gov (United States)

    Cui, Jason Z; Tehrani, Arash Y; Jett, Kimberly A; Bernatchez, Pascal; van Breemen, Cornelis; Esfandiarei, Mitra

    2014-09-01

    In a mouse model of Marfan syndrome, conventional Verhoeff-Van Gieson staining displays severe fragmentation, disorganization and loss of the aortic elastic fiber integrity. However, this method involves chemical fixatives and staining, which may alter the native morphology of elastin and collagen. Thus far, quantitative analysis of fiber damage in aorta and skin in Marfan syndrome has not yet been explored. In this study, we have used an advanced noninvasive and label-free imaging technique, multiphoton microscopy to quantify fiber fragmentation, disorganization, and total volumetric density of aortic and cutaneous elastin and collagen in a mouse model of Marfan syndrome. Aorta and skin samples were harvested from Marfan and control mice aged 3-, 6- and 9-month. Elastin and collagen were identified based on two-photon excitation fluorescence and second-harmonic-generation signals, respectively, without exogenous label. Measurement of fiber length indicated significant fragmentation in Marfan vs. control. Fast Fourier transform algorithm analysis demonstrated markedly lower fiber organization in Marfan mice. Significantly reduced volumetric density of elastin and collagen and thinner skin dermis were observed in Marfan mice. Cutaneous content of elastic fibers and thickness of dermis in 3-month Marfan resembled those in the oldest control mice. Our findings of early signs of fiber degradation and thinning of skin dermis support the potential development of a novel non-invasive approach for early diagnosis of Marfan syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

  13. Osteoprotegerin in Turner syndrome - relationship to aortic diameter

    DEFF Research Database (Denmark)

    Trolle, Christian; Mortensen, Kristian Havmand; Bjerre, Mette

    2015-01-01

    BACKGROUND: Cardiovascular disease is a cardinal trait of Turner syndrome (TS), causing half of the 3-fold excess mortality. Since osteoprotegerin (OPG) is as a potential biomarker of cardiovascular disease, this cross-sectional and prospective study aimed at elucidating OPG levels in TS and its...

  14. Preventing the aortic complications of Marfan syndrome: a case-example of translational genomic medicine

    Science.gov (United States)

    Li-Wan-Po, Alain; Loeys, Bart; Farndon, Peter; Latham, David; Bradley, Caroline

    2011-01-01

    The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation. It relies on defined clinical criteria with confirmation based on FBN1 mutation testing. Surgical advances have prolonged life in Marfan syndrome. First-line prophylaxis of complications with β-adrenoceptor blockers became established on the basis that reduction of aortic pressure and heart rate would help. Over-activity of proteinases, first suggested in 1980, has since been confirmed by evidence of over-expression of matrix metalloproteinases (MMP), notably MMP-2 and MMP-9. The search for MMP inhibitors led to the evaluation of doxycycline, and both animal studies and small trials, provided early evidence that this widely used antimicrobial agent was useful. Identification of the importance of TGF-β led to evaluation of angiotensin II type I receptor (AT1R) blockers with highly promising results. Combination prophylactic therapy would appear rational. Pharmacological and genomic research has provided good evidence that therapy with losartan and doxycycline would prevent the aortic complications of Marfan syndrome. If on-going well designed trials confirm their efficacy, the outlook for Marfan syndrome patients would be improved considerably. PMID:21276043

  15. Acute heart failure syndrome | Ker | South African Family Practice

    African Journals Online (AJOL)

    Keywords: acute heart failure syndrome, acute coronary syndrome, adult respiratory distress syndrome (ARDS). Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians ...

  16. Case report of a rarely seen long-segment middle aortic syndrome.

    Science.gov (United States)

    Yakut, Kahraman; Erdoğan, İlkay

    2017-03-01

    Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture. In order to draw attention to this disease, presently described is case of high blood pressure detected during routine examination of a child who had no complaint, and discovery of long-segment stenosis in the abdominal aorta identified with echocardiography and conventional angiography.

  17. Abnormal aortic arch morphology in Turner syndrome patients is a risk factor for hypertension.

    Science.gov (United States)

    De Groote, Katya; Devos, Daniël; Van Herck, Koen; Demulier, Laurent; Buysse, Wesley; De Schepper, Jean; De Wolf, Daniël

    2015-09-01

    Hypertension in Turner syndrome (TS) is a multifactorial, highly prevalent and significant problem that warrants timely diagnosis and rigorous treatment. The objective of this study was to investigate the association between abnormal aortic arch morphology and hypertension in adult TS patients. This was a single centre retrospective study in 74 adult TS patients (age 29.41 ± 8.91 years) who underwent a routine cardiac MRI. Patients were assigned to the hypertensive group (N = 31) if blood pressure exceeded 140/90 mmHg and/or if they were treated with antihypertensive medication. Aortic arch morphology was evaluated on MRI images and initially assigned as normal (N = 54) or abnormal (N = 20), based on the curve of the transverse arch and the distance between the left common carotid-left subclavian artery. We additionally used a new more objective method to describe aortic arch abnormality in TS by determination of the relative position of the highest point of the transverse arch (AoHP). Logistic regression analysis showed that hypertension is significantly and independently associated with age, BMI and abnormal arch morphology, with a larger effect size for the new AoHP method than for the classical method. TS patients with hypertension and abnormal arch morphology more often had dilatation of the ascending aorta. There is a significant association between abnormal arch morphology and hypertension in TS patients, independent of age and BMI, and not related to other structural heart disease. We suggest that aortic arch morphology should be included in the risk stratification for hypertension in TS and propose a new quantitative method to express aortic arch morphology.

  18. [Marfan syndrome: clinical and pathomorphological restructurings after surgical treatment of aortic aneurysm].

    Science.gov (United States)

    Zhuraev, R K; Zerbino, D D

    2014-01-01

    The authors examined the state of patients suffering from Marfan syndrome (MS) who endured operation for ascending aorta aneurysm with replacement of the ascending aorta and aortic valve (Bentall operation), studying alterations of the skeleton, face, heart and eyes, as well as pathomorphological restructurings in the aortic wall. The study was carried out 7.0 ± 4.2 years after the operation. We examined a total of 39 patients with MS - 27 (69.2%) men and 12 (30.8%) women aged from 22 to 70 years old (average age - 42.1 ± 13.4 years). All patients were operated on for dissecting aneurysm of the ascending aorta accompanied by a considerable degree of aortic valve insufficiency or aortic ostium stenosis. The mean diameter of the aorta at the level of the sinuses of Valsalva amounted to 7.0 ± 1.3 cm (minimal - 5.0 cm, maximal - 12.0 cm), the Z-score prior to operation was 12.7 ± 6.5. The time form making the diagnosis of MS to surgical intervention for aortic aneurysm amounted to 9.6 ± 5.9 years. The condition after operative treatment in all patients was satisfactory, with the haemodynamic indices stable: systolic AP - 133.5 ± 19.1 mm Hg, diastolic AP 85.1 ± 12.9 mm Hg, heart rate 74.8 ± 7.2 bpm. The average systemic score for the symptoms and tests of MS patients amounted to 8.2 ± 3.3 points. Pathohistological alterations of the aorta in patients with Marfan syndrome consisted in pronounced restructuring of the wall with deep irreversible alternative changes. The pathological process extended in the middle aortic layer all alone the length, but not only in the portions of rupture and dissection. The main pathomorphological signs in MS were as follows: focal accumulations of mucoid substances, dystrophic alterations of smooth-muscle cells, ribbon-like anuclear zones, formation of cystlike cavities, alterations of elastic fibres - fragmentation, hyperelastosis, multiplication, thinning and straightening, zones of elastolysis.

  19. ADVANCIS Score Predicts Acute Kidney Injury After Percutaneous Coronary Intervention for Acute Coronary Syndrome.

    Science.gov (United States)

    Fan, Pei-Chun; Chen, Tien-Hsing; Lee, Cheng-Chia; Tsai, Tsung-Yu; Chen, Yung-Chang; Chang, Chih-Hsiang

    2018-01-01

    Acute kidney injury (AKI), a common and crucial complication of acute coronary syndrome (ACS) after receiving percutaneous coronary intervention (PCI), is associated with increased mortality and adverse outcomes. This study aimed to develop and validate a risk prediction model for incident AKI after PCI for ACS. We included 82,186 patients admitted for ACS and receiving PCI between 1997 and 2011 from the Taiwan National Health Insurance Research Database and randomly divided them into a training cohort (n = 57,630) and validation cohort (n = 24,656) for risk model development and validation, respectively. Risk factor analysis revealed that age, diabetes mellitus, ventilator use, prior AKI, number of intervened vessels, chronic kidney disease (CKD), intra-aortic balloon pump (IABP) use, cardiogenic shock, female sex, prior stroke, peripheral arterial disease, hypertension, and heart failure were significant risk factors for incident AKI after PCI for ACS. The reduced model, ADVANCIS, comprised 8 clinical parameters (age, diabetes mellitus, ventilator use, prior AKI, number of intervened vessels, CKD, IABP use, cardiogenic shock), with a score scale ranging from 0 to 22, and performed comparably with the full model (area under the receiver operating characteristic curve, 87.4% vs 87.9%). An ADVANCIS score of ≥6 was associated with higher in-hospital mortality risk. In conclusion, the ADVANCIS score is a novel, simple, robust tool for predicting the risk of incident AKI after PCI for ACS, and it can aid in risk stratification to monitor patient care.

  20. Aortic Aneurysm Statistics

    Science.gov (United States)

    ... people with inherited connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, get thoracic aortic aneurysms. ... Smoking . Some inherited connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, can also increase your ...

  1. Elevated expression levels of lysyl oxidases protect against aortic aneurysm progression in Marfan syndrome.

    Science.gov (United States)

    Busnadiego, O; Gorbenko Del Blanco, D; González-Santamaría, J; Habashi, J P; Calderon, J F; Sandoval, P; Bedja, D; Guinea-Viniegra, J; Lopez-Cabrera, M; Rosell-Garcia, T; Snabel, J M; Hanemaaijer, R; Forteza, A; Dietz, H C; Egea, G; Rodriguez-Pascual, F

    2015-08-01

    Patients with Marfan syndrome (MFS) are at high risk of life-threatening aortic dissections. The condition is caused by mutations in the gene encoding fibrillin-1, an essential component in the formation of elastic fibers. While experimental findings in animal models of the disease have shown the involvement of transforming growth factor-β (TGF-β)- and angiotensin II-dependent pathways, alterations in the vascular extracellular matrix (ECM) may also play a role in the onset and progression of the aortic disease. Lysyl oxidases (LOX) are extracellular enzymes, which initiates the formation of covalent cross-linking of collagens and elastin, thereby contributing to the maturation of the ECM. Here we have explored the role of LOX in the formation of aortic aneurysms in MFS. We show that aortic tissue from MFS patients and MFS mouse model (Fbn1(C1039G/+)) displayed enhanced expression of the members of the LOX family, LOX and LOX-like 1 (LOXL1), and this is associated with the formation of mature collagen fibers. Administration of a LOX inhibitor for 8weeks blocked collagen accumulation and aggravated elastic fiber impairment, and these effects correlated with the induction of a strong and rapidly progressing aortic dilatation, and with premature death in the more severe MFS mouse model, Fbn1(mgR/mgR), without any significant effect on wild type animals. This detrimental effect occurred preferentially in the ascending portion of the aorta, with little or no involvement of the aortic root, and was associated to an overactivation of both canonical and non-canonical TGF-β signaling pathways. The blockade of angiotensin II type I receptor with losartan restored TGF-β signaling activation, normalized elastic fiber impairment and prevented the aortic dilatation induced by LOX inhibition in Fbn1(C1039G/+) mice. Our data indicate that LOX enzymes and LOX-mediated collagen accumulation play a critical protective role in aneurysm formation in MFS. Copyright © 2015 Elsevier

  2. Mid-term results of different aortic valve-sparing procedures in Marfan syndrome.

    Science.gov (United States)

    Schmidtke, Claudia; Karluss, Antje; Sier, Holger; Hüppe, Michael; Brauer, Kirk; Sievers, Hans-H

    2012-03-01

    Marfan patients with aortic root aneurysm are typically treated with the Bentall procedure, though aortic valve-sparing procedures (AVSPs) are also possible. The study aim was to compare the authors' experience with two such techniques performed at their institution, namely a reimplantation according to David (David I) and remodeling according to Yacoub. Between 1996 and 2009, a total of 37 Marfan patients underwent an AVSP at the authors' institution. Of these patients, 25 (mean age 32 +/- 14.9 years) underwent surgery according to David (group D), and 12 (mean age 35 +/- 10.9 years) according to Yacoub (group Y). The patients underwent both clinical and echocardiographic follow up examinations at a mean of 42.0 +/- 36.4 months after surgery. One patient from each group had moved abroad and was lost to follow up. The remaining 35 patients were alive at follow up, and none presented with any major neurological or bleeding complications. In addition, no significant differences were noted between the groups in terms of NYHA classification, left ventricular function, or left ventricular diameter. At follow up, aortic valve function was also comparable between groups, with a peak/mean gradient of 9.4 +/- 6.4/5.3 +/- 3.5 mmHg and 5.1 +/- 3.3/2.8 +/- 1.5 mmHg for groups D and Y, respectively (p = 0.081/0.058). The measured mean grades of aortic valve regurgitation were comparable in groups D and Y (0.6 +/- 0.7 and 1.1 +/- 0.6, respectively; p = 0.055). However, aortic root dimensions obtained via M-mode were smaller in group D patients (29.6 +/- 2.3 mm) than in group Y patients (36.1 +/- 6.6 mm) (p = 0.027). Only three patients from group Y required reoperation on the aortic valve due to valvular regurgitation (p = 0.028); two of these had presented with aortic dissection at the first operation. Both types of AVSP can be performed with comparably good interim clinical results, and also low mortality and morbidity, in patients with Marfan syndrome.

  3. [Emergency diagnosis of the acute vestibular syndrome].

    Science.gov (United States)

    Tamás, T László; Garai, Tibor; Király, István; Mike, Andrea; Nagy, Csaba; Paukovics, Ágnes; Schmidt, Péter; Szatmári, Ferenc; Tompos, Tamás; Vadvári, Árpád; Szirmai, Ágnes

    2017-12-01

    To diagnose acute vestibular syndrome (AVS) in a prospective study by a new bedside test (providing 1A evidence) based on oculomotor analysis and assessment of hearing loss. To assess the frequency of central and peripheral causes of acute vestibular syndrome in the emergency room. To establish the diagnostic accuracy of acute cranial computed tomography as compared to oculomotor analysis done by video oculography goggles and audiometry. Between 1st March 2016 and 1st March 2017 we documented 125 patients (62 women, 63 men, average age 53 years) in the emergency room of the Petz Aladár County Teaching Hospital using the above bedside and instrumental testing. Diagnosis was verified by cranial magnetic resonance imaging. According to the results of the instrumental examination in AVS in 67% we found a peripheral cause and in 33% a central pathology. In 62% isolated posterior circulation stroke manifested itself by isolated vertigo without additional focal signs and the acute cranial computed tomography showed negative results in 96%. The instrumental examination increased diagnostic accuracy by making the diagnosis of isolated inferior semicircular canal vestibular neuritis possible. The new bedside oculomotor test is suitable for the diagnosis of posterior circulation stroke manifesting with isolated vertigo in early cases, when the routine neuroradiologic methods have a lower sensitivity or are not available. Orv Hetil. 2017; 158(51): 2029-2040.

  4. Mutations in a TGF-β Ligand, TGFB3, Cause Syndromic Aortic Aneurysms and Dissections

    Science.gov (United States)

    Bertoli-Avella, Aida M.; Gillis, Elisabeth; Morisaki, Hiroko; Verhagen, Judith M.A.; de Graaf, Bianca M.; van de Beek, Gerarda; Gallo, Elena; Kruithof, Boudewijn P.T.; Venselaar, Hanka; Myers, Loretha A.; Laga, Steven; Doyle, Alexander J.; Oswald, Gretchen; van Cappellen, Gert W.A.; Yamanaka, Itaru; van der Helm, Robert M.; Beverloo, Berna; de Klein, Annelies; Pardo, Luba; Lammens, Martin; Evers, Christina; Devriendt, Koenraad; Dumoulein, Michiel; Timmermans, Janneke; Bruggenwirth, Hennie T.; Verheijen, Frans; Rodrigus, Inez; Baynam, Gareth; Kempers, Marlies; Saenen, Johan; Van Craenenbroeck, Emeline M.; Minatoya, Kenji; Matsukawa, Ritsu; Tsukube, Takuro; Kubo, Noriaki; Hofstra, Robert; Goumans, Marie Jose; Bekkers, Jos A.; Roos-Hesselink, Jolien W.; van de Laar, Ingrid M.B.H.; Dietz, Harry C.; Van Laer, Lut; Morisaki, Takayuki; Wessels, Marja W.; Loeys, Bart L.

    2015-01-01

    Background Aneurysms affecting the aorta are a common condition associated with high mortality as a result of aortic dissection or rupture. Investigations of the pathogenic mechanisms involved in syndromic types of thoracic aortic aneurysms, such as Marfan and Loeys-Dietz syndromes, have revealed an important contribution of disturbed transforming growth factor (TGF)-β signaling. Objectives This study sought to discover a novel gene causing syndromic aortic aneurysms in order to unravel the underlying pathogenesis. Methods We combined genome-wide linkage analysis, exome sequencing, and candidate gene Sanger sequencing in a total of 470 index cases with thoracic aortic aneurysms. Extensive cardiological examination, including physical examination, electrocardiography, and transthoracic echocardiography was performed. In adults, imaging of the entire aorta using computed tomography or magnetic resonance imaging was done. Results Here, we report on 43 patients from 11 families with syndromic presentations of aortic aneurysms caused by TGFB3 mutations. We demonstrate that TGFB3 mutations are associated with significant cardiovascular involvement, including thoracic/abdominal aortic aneurysm and dissection, and mitral valve disease. Other systemic features overlap clinically with Loeys-Dietz, Shprintzen-Goldberg, and Marfan syndromes, including cleft palate, bifid uvula, skeletal overgrowth, cervical spine instability and clubfoot deformity. In line with previous observations in aortic wall tissues of patients with mutations in effectors of TGF-β signaling (TGFBR1/2, SMAD3, and TGFB2), we confirm a paradoxical up-regulation of both canonical and noncanonical TGF-β signaling in association with up-regulation of the expression of TGF-β ligands. Conclusions Our findings emphasize the broad clinical variability associated with TGFB3 mutations and highlight the importance of early recognition of the disease because of high cardiovascular risk. PMID:25835445

  5. Losartan in combination with propranolol slows the aortic root dilatation in neonatal Marfan syndrome.

    Science.gov (United States)

    Liu, Lu-Hang; Lin, Shan-Miao; Lin, Dar-Shong; Chen, Ming-Ren

    2018-04-01

    Neonatal Marfan syndrome, in contrast to classical Marfan syndrome, is characterized by rapidly progressive multi-valvular cardiac disease and death from congestive heart failure, typically within the first year of life. Due to the rarity of this condition, treatment for neonatal Marfan syndrome has not been well studied. In this report, a combination of losartan and propranolol reduced the aortic root dilatation rate after three months of losartan therapy. Genetic analysis in this patient revealed a mutation in exon 25 of the FBN1 gene, which typically results in a shorter life expectancy. However, the patient's heart failure was controlled by losartan, propranolol and other anti-congestive medications, which may have prolonged his survival. Copyright © 2017. Published by Elsevier B.V.

  6. A single-center experience of hemofiltration treatment for acute aortic dissection (Stanford type A) complicated with postoperative acute renal failure.

    Science.gov (United States)

    Qi, Peng; Zhang, Xi-Quan; Pang, Xin-Yan; Cao, Guang-Qing; Fang, Chang-Cun; Wu, Shu-Ming

    2015-01-01

    To investigate the effect of continuous venovenous hemofiltration (CVVH) for aortic dissection patients with acute renal failure after surgery in retrospective manner. A total of thirty-seven aortic dissection patients with postoperative acute renal failure accepted CVVH therapy. The effect of CVVH was evaluated by analyzing clinical condition changes and laboratory examination results. After treatment of CVVH, renal function and clinical symptoms were significantly improved in thirty patients. Eight of the thirty patients got completely renal function recovery within two weeks after CVVH therapy; and twenty-two of the thirty patients got completely renal function recovery within four weeks after CVVH therapy. Nevertheless, seven patients got no benefit from CVVH therapy with poor prognosis. CVVH is an effective treatment to most aortic dissection patients with postoperative acute renal failure. The effect of CVVH was correlated with original renal function, early CVVH therapy, and continuous intensive care.

  7. Treatment of Acute Aortic Type B Dissection with Stent-Grafts

    International Nuclear Information System (INIS)

    Hausegger, K.A.; Tiesenhausen, K.; Schedlbauer, P.; Oberwalder, P.; Tauss, J.; Rigler, B.

    2001-01-01

    Purpose: To evaluate the feasibility of endoluminal stent-grafts in the treatment of acute type B aortic dissections.Methods: In five patients with acute aortic type B dissections, sealing of the primary intimal tear with an endoluminal stent-graft was attempted. Indication for treatment was aneurysm formation in two patients and persistent pain in three patients. One of the latter also had an unstable dissection flap compromising the ostium of the superior mesenteric artery. The distance from the intimal tear to the left subclavian artery was <0.5 cm in four patients, who had typical type B dissections. In one patient with an atypical dissection the distance from the primary tear to the left subclavian artery was 4 cm. This patient had no re-entry tear. Talent tube grafts (World Medical Manufacturing Cooperation, Sunrise, FL, USA) were used in all patients.Results: Stent-graft insertion with sealing of the primary tear was successful in all patients. The proximal covered portion of the stent-graft was placed across the left subclavian artery in four patients (1x transposition of the left subclavian artery). Left arm perfusion was preserved via a subclavian steal phenomenon in the patients in whom the stent-graft covered the orifice of the left subclavian artery. The only procedural complication we observed was an asymptomatic segmental renal infarction in one patient. In the thoracic aorta thrombosis of the false aortic lumen occurred in all patients. In one patient the false lumen of the abdominal aorta thrombosed after 4 weeks; in the other three patients the status of the abdominal aorta remained unchanged compared with the situation prior to stent-graft insertion. As a late complication formation of a secondary aneurysm of the thoracic aorta was observed at the distal end of the stent-graft 3 months after the primary intervention. This aneurysm was treated by coaxial insertion of an additional stent-graft without complications.Conclusion: Endoluminal treatment

  8. Acute hypotension induced by aortic clamp vs. PTH provokes distinct proximal tubule Na+ transporter redistribution patterns

    DEFF Research Database (Denmark)

    Leong, Patrick K K; Yang, Li E; Lin, Harrison W

    2004-01-01

    Renal parathyroid hormone (PTH) action is often studied at high doses (100 microg PTH/kg) that lower mean arterial pressure significantly, albeit transiently, complicating interpretation of studies. Little is known about the effect of acute hypotension on proximal tubule Na(+) transporters....... This study aimed to determine the effects of acute hypotension, induced by aortic clamp or by high-dose PTH (100 microg PTH/kg), on renal hemodynamics and proximal tubule Na/H exchanger isoform 3 (NHE3) and type IIa Na-P(i) cotransporter protein (NaPi2) distribution. Subcellular distribution was analyzed...... clearance. There was, however, no significant change in glomerular filtration rate (GFR) or subcellular distribution of NHE3 and NaPi2. In contrast, high-dose PTH rapidly (

  9. Shock complicating type A acute aortic dissection: Clinical correlates, management, and outcomes.

    Science.gov (United States)

    Bossone, Eduardo; Pyeritz, Reed E; Braverman, Alan C; Peterson, Mark D; Ehrlich, Marek; O'Gara, Patrick; Suzuki, Toru; Trimarchi, Santi; Gilon, Dan; Greason, Kevin; Desai, Nimesh D; Montgomery, Daniel G; Isselbacher, Eric M; Nienaber, Christoph A; Eagle, Kim A

    2016-06-01

    Shock is among the most dreaded and common complications of type A acute aortic dissection (TAAAD). However, clinical correlates, management, and short- and long-term outcomes of TAAAD patients presenting with shock in real-world clinical practice are not known. We evaluated 2,704 patients with TAAAD enrolled in the International Registry of Acute Aortic Dissection between January 1, 1996, and August 18, 2012. On admission, 407 (15.1%) TAAAD patients presented with shock. Most in-hospital complications (coma, myocardial or mesenteric ischemia or infarction, and cardiac tamponade) were more frequent in shock patients. In-hospital mortality was significantly higher in TAAAD patients with than without shock (30.2% vs 23.9%, P=.007), regardless of surgical or medical treatment. Most shock patients underwent surgical repair, with medically managed patients demonstrating older age and more complications at presentation. Estimates using Kaplan-Meier survival analysis indicated that most (89%) TAAAD patients with shock discharged alive from the hospital survived 5years, a rate similar to that of TAAAD patients without shock (82%, P=.609). Shock occurred in 1 of 7 TAAAD patients and was associated with higher rates of in-hospital adverse events and mortality. However, TAAAD survivors with or without shock showed similar long-term mortality. Successful early and aggressive management of shock in TAAAD patients has the potential for improving long-term survival in this patient population. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Hypothermic circulatory arrest for acute traumatic aortic rupture associated with shock.

    Science.gov (United States)

    Kawahito, Koji; Adachi, Hideo

    2011-07-01

    Hypothermic circulatory arrest is considered to be a contraindication in acute traumatic aortic rupture (TAR) because full heparinization and hypothermia may lead to fatal bleeding if concomitant hemorrhagic injuries are present. However, in extremely emergent situations, rapid volume infusion via cardiotomy vacuums and the institution of hypothermic circulatory arrest appears to be the only method for saving patients with uncontrollable bleeding. In this study, we evaluate the feasibility of hypothermic circulatory arrest for treating patients with TAR with hemorrhagic shock. Ten patients (nine men and one woman; mean age, 35 ± 18 years), with acute TAR caused by blunt chest trauma and in a shock state, underwent surgery between 1999 and 2007. All ten patients exhibited rupture of the isthmus, and polytraumatism was frequent. Any life-threatening blood loss from other injuries was surgically treated before aortic surgery. All patients were operated on with standard cardiopulmonary bypass under hypothermic circulatory arrest. All patients received prosthetic graft replacement. The overall hospital mortality was 10.0% (1/10). One patient died of intraabdominal and pulmonary bleeding during surgery, and the other nine were discharged without complications. There was no evidence of any new or increased hemorrhagic complications during heparinization in the nine surviving patients. Mean operation and circulatory arrest times were 305 ± 44 min and 27 ± 7 min, respectively. Hypothermic circulatory arrest is feasible for saving TAR patients with unstable hemodynamics resulting from rupture, provided associated injuries are properly treated in advance.

  11. Ischemic acute pancreatitis with pancreatic pseudocyst in a patient with abdominal aortic aneurysm and generalized atheromatosis - case report.

    Science.gov (United States)

    Cocota, Ileana; Badea, Radu; Scridon, Traian; Dumitrascu, Dan L

    2015-03-21

    Ischemic pancreatitis is a rare medical entity. The pancreatic tissue is susceptible to ischemia with the possibility of developing acute pancreatitis. The abdominal aortic aneurysm can be one possible cause of pancreatic hypoperfusion. We report the case of a 68-year-old man, Caucasian, with a history of a cluster of severe cardiovascular conditions, who presented epigastric pain of variable intensity for about 2 weeks. The pain occurred after intense physical effort, and was associated with anorexia and asthenia. The palpation revealed epigastric pain and palpable pulsatile mass above the umbilicus. Laboratory tests showed increased serum and urine amylases. The abdominal contrast-enhanced CT scan evidenced acute lesions of the pancreas and a caudal pancreatic pseudocyst of 39x24 mm. An abdominal aortic aneurysm was also described (which extended from the kidney level to the bilateral femoral level) with a maximum diameter of 60.5 mm and generalized atheromatosis. By corroborating clinical, anamnestic, laboratory and imaging data, the case was diagnosed as moderately severe acute ischemic pancreatitis, pancreatic pseudocyst, abdominal aortic aneurysm, generalized atheromatosis. The pancreatic pseudocyst was resorbed in eight months. Surgery for the abdominal aneurysm was performed after the resorption of the pseudocyst. The patient died after aortic surgery because of a septic complication. Ischemic pancreatitis is a rare condition but should be considered in a patient with upper abdominal pain and elevated amylase in the context of an abdominal aortic aneurysm and generalized atheromatosis.

  12. Gender bias in acute coronary syndromes.

    Science.gov (United States)

    Bugiardini, Raffaele; Estrada, Jose L Navarro; Nikus, Kjell; Hall, Alistair S; Manfrini, Olivia

    2010-03-01

    The major aim of this review was to ascertain whether effective evidence-based treatments for acute coronary syndromes (ACS) are underutilized in women in various geographic areas compared with men. The focus of our review was the relative use of effective treatments in patients with coronary angiographic evidence of obstructive coronary disease, defined as a lumen stenosis >50% of the adjacent non-diseased arterial diameter. We searched MEDLINE, and the Cochrane Database between January 1998 and May 2008. Only a few of the published clinical registries on ACS provide data on treatments dichotomized by confirmed coronary angiographic disease. Consequently, we also accessed individual patient-level data from 3 established ACS registries: the Finnish TACOS (Tampere Acute COronary Syndrome), the British EMMACE 2 (Evaluation of Methods and Management of Acute Coronary Events) and the Argentine PACS-ITALSIA (Prognosis in Acute Coronary Syndromes and the ITALian hospital Sindrome Isquemico Agudo). Despite presenting with higher risk characteristics and having higher in-hospital and 6 months risk of death, women with ACS and obstructive coronary artery disease were apparently treated less aggressively with secondary preventive drugs than were men, being less likely to receive aspirin, beta-blockers and statins at discharge. Overall, coronary revascularization appears to be performed in a similar proportion of women and men - once angiography has been performed and the coronary anatomy is known. However, substantial geographic variation exists in the relative rate of coronary angiography in men and women. In United Kingdom coronary revascularization tends to be done less frequently in women. Our study, therefore, demonstrates a gender bias in the delivery of secondary drug treatments for ACS, even for patients with documented significant coronary disease.

  13. Acute respiratory failure following ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Antonello Nicolini

    2013-03-01

    Full Text Available Ovarian hyperstimulation syndrome is a serious and potentially life-threatening physiological complication that may be encountered in patients who undergo controlled ovarian hyperstimulation cycles. The syndrome is typically associated with regimes of exogenous gonadotropins, but it can be seen, albeit rarely, when clomiphene is administered during the induction phase. Although this syndrome is widely described in scientific literature and is well known by obstetricians, the knowledge of this pathological and potentially life-threatening condition is generally less than satisfactory among physicians. The dramatic increase in therapeutic strategies to treat infertility has pushed this condition into the realm of acute care therapy. The potential complications of this syndrome, including pulmonary involvement, should be considered and identified so as to allow a more appropriate diagnosis and management. We describe a case of a woman with an extremely severe (Stage 6 ovarian hyperstimulation syndrome who presented ascites, bilateral pleural effusion and severe respiratory failure treated with non-invasive ventilation. The patient was admitted to the intensive care unit because of severe respiratory failure, ascites, and bilateral pleural effusion due to ovarian hyperstimulation syndrome. Treatment included non-invasive ventilation and three thoracentesis procedures, plus the administration of albumin, colloid solutions and high-dose furosemid. Severe form of ovarian hyperstimulation syndrome is observed in 0.5-5% of the women treated, and intensive care may be required for management of thromboembolic complications, renal failure and severe respiratory failure. Pulmonary intensive care may involve thoracentesis, oxygen supplementation and, in more severe cases, assisted ventilation. To our knowledge, there have been only two studies in English language medical literature that describe severe respiratory failure treated with non

  14. [Acute cor pulmonale in acute respiratory distress syndrome].

    Science.gov (United States)

    Zhang, Feng; Cao, Quan; Zuo, Xiangrong

    2017-03-01

    Acute respiratory distress syndrome (ARDS) is a severe respiratory condition that is characterized by rapidly progressive hypoxemia with noncardiogenic pulmonary edema. Despite the improvement of therapeutic methods, the mortality of ARDS is in the range of 40%-50% all over the world. Some studies have shown that a significant number of patients with ARDS had acute cor pulmonale (ACP), and ACP is independently associated with the mortality of patients with ARDS, which has attracted wide attention in recent years. This paper reviewed recent related studies, summarized the prevalence, pathogenesis and diagnostic approaches of ACP in ARDS, especially echocardiography which was considered as a cornerstone for ACP diagnosis, and elucidated the beneficial effects of right ventricular protective ventilatory strategy and prone-positioning on the pulmonary vasculature and right heart, in order to provide a novel idea for the therapy of ACP in ARDS.

  15. Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome

    Directory of Open Access Journals (Sweden)

    Jong Uk Lee

    2016-04-01

    Full Text Available Williams syndrome (WS is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS and peripheral pulmonary artery stenosis (PPAS are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.

  16. Endovascular Repair of Aortic Dissection in Marfan Syndrome: Current Status and Future Perspectives

    Directory of Open Access Journals (Sweden)

    Rosario Parisi

    2015-07-01

    Full Text Available Over the last decades, improvement of medical and surgical therapy has increased life expectancy in Marfan patients. Consequently, the number of such patients requiring secondary interventions on the descending thoracic aorta due to new or residual dissections, and distal aneurysm formation has substantially enlarged. Surgical and endovascular procedures represent two valuable options of treatment, both associated with advantages and drawbacks. The aim of the present manuscript was to review endovascular outcomes in Marfan syndrome and to assess the potential role of Thoracic Endovascular Aortic Repair (TEVAR in this subset of patients.

  17. [Effect of native aortic valve sparing aortic root reconstruction surgery on short- and long-term prognosis in Marfan syndrome patients:a meta-analysis].

    Science.gov (United States)

    Hu, Rui; Wang, Zhiwei; Hu, Xiaoping; Wu, Hongbing; Zhou, Zhen

    2014-05-01

    This meta-analysis was performed to analyze the effect of preserving the native aortic valve on short- and long-term prognosis post aortic root reconstruction surgery for patients with Marfan syndrome. Database including Pubmed,Embase, Cochrane library, CNKI, Wanfang,VIP and CBM were searched to collect studies comparing clinical results of valve sparing surgery with composite valve graft surgery for patients with Marfan syndrome. Study quality was assessed by Newcastle-Ottawa Scale and publication bias was assessed by visual inspection of the funnel plot together with Egger test. Clinical outcomes data was extracted from the manuscripts and analyzed with Revman 5.0 supplied by Cochrane collaboration. Seven clinical trials with 690 patients were included. Meta- analysis demonstrated that valve sparing surgery was associated with a lower incidence of re-exploration (RR = 0.51, 95%CI:0.29- 0.90, P 0.05). Valve sparing aortic root reconstruction surgery is a superior procedure to composite valve graft surgery in term of improving the short- and long-term prognosis for patients with Marfan syndrome.

  18. Dietary fatty acids on aortic root calcification in mice with metabolic syndrome.

    Science.gov (United States)

    Naranjo, Maria C; Bermudez, Beatriz; Garcia, Indara; Lopez, Sergio; Abia, Rocio; Muriana, Francisco J G; Montserrat-de la Paz, Sergio

    2017-04-19

    Metabolic syndrome (MetS) is associated with obesity, dyslipidemia, type 2 diabetes, and chronic low-grade inflammation. The aim of this study was to determine the role of high-fat low-cholesterol diets (HFLCDs) rich in SFAs (HFLCD-SFAs), MUFAs (HFLCD-MUFAs) or MUFAs plus omega-3 long-chain PUFAs (HFLCD-PUFAs) on vascular calcification by the modulation of the RANKL/RANK/OPG system in the aortic roots of Lep ob/ob LDLR -/- mice. Animals fed with HFLCD-SFAs had increased weight and a greater atheroma plaque size, calcification, and RANKL/CATHK expression in the aortic root than mice on MUFA-enriched diets, with an increasing OPG expression in the aortic roots of the latter. Our study demonstrates that compared to dietary SFAs, MUFAs from olive oil protect against atherosclerosis by interfering with vascular calcification via the RANKL/RANK/OPG system in the setting of MetS. These findings open opportunities for developing novel nutritional strategies with olive oil as the most important dietary source of MUFAs (notably oleic acid) to prevent cardiovascular complications in MetS.

  19. Benefits and medium-term outcome of the Sorin Pericarbon Freedom stentless aortic prosthesis in cases of acute bacterial endocarditis.

    Science.gov (United States)

    Stefanelli, Guglielmo; Pirro, Fabrizio; Meli, Marco; Trevisan, Davide; Telesca, Mariassunta; Campisano, Barbara; Mussini, Cristina; Barbieri, Andrea

    2016-10-01

    The aim of this study is to evaluate the ease of use and the advantages of Sorin Pericarbon Freedom (SPF) stentless valve in cases of acute bacterial endocarditis and to check the intermediate-term results after the implant of SPF with respect to resistance to infection, valve deterioration and durability. Between June 2003 and February 2015, 26 patients with active aortic valve bacterial endocarditis underwent aortic valve replacement with SPF pericardial stentless aortic prosthesis. The mean age was 57 ± 18 years; 73% of the patients were in preoperative NYHA class III and VI. Mean Logistic EuroSCORE was 14.2 ± 12.7. Endocarditis occurred in 18 patients with native valves, and in 9 patients with prosthetic valves (4 mechanical aortic valve prostheses; 5 aortic bioprostheses). Aortic root abscesses were observed in 16 cases (61.5%). Surgery was emergent in 3 cases (11.5%). Redo surgery was performed in 9 cases (35%). Cumulative follow-up was 126.8 patient-years (mean 4.9 ± 3.3 years). Operative hospital mortality was 0% for all patients. Residual mean prosthetic gradient at discharge was 9.4 ± 3.6 mmHg. Neither residual aortic incompetence nor residual abscess cavity was observed at discharge. Mean ejection fraction at discharge was 54 ± 8% (Min; Max: 35%; 65%). A total of 4 patients died at follow-up, all for non-cardiac causes. One patient was lost to follow-up. Two patients (8%) underwent non-valve-related reoperation with 0% mortality. Residual mean gradient at follow-up was 7.2 ± 2.1 mmHg. Three patients (17%) presented with mild/moderate aortic incompetence and 89% of patients were in NYHA Class I-II at follow-up. At 9 years, actuarial freedom from valve-related reoperation and from structural valve deterioration was 100%. The SPF aortic prosthesis is a true pericardial stentless prosthesis suitable for the treatment of acute bacterial endocarditis. Intermediate-time results in terms of freedom from reoperation, structural valve deterioration and

  20. Surgical treatment of ascending aortic complications in Marfan syndrome: early and long-term outcomes.

    Science.gov (United States)

    Favaloro, Roberto R; Casabé, J Horacio; Segura, Mónica; Abud, José; Casas, Jorge; López, Claudio; Dulbecco, Eduardo; Raffaelli, Héctor

    2008-08-01

    Findings in 54 patients (mean age 39 years, range 18-66 years, 25% female) were analyzed. Of these patients, 21 had dissection of the ascending aorta (15 acute and six chronic) and 33 had aneurysm of the ascending aorta. Surgery was classified as emergency surgery in three cases, as urgent in 15, and as scheduled surgery in 36. The Bentall-De Bono procedure was performed in 39 patients, aortic valve reimplantation was carried out in nine, Cabrol's operation was performed in three, and a homograft was used in three. The mean diameter of the ascending aorta was 66.6 mm. Overall, in-hospital mortality was 3.7% (33.3% for emergency surgery vs. 2.8% for scheduled surgery; P< .001). During the mean follow-up period of 4 years (range, 2 months-14 years), seven patients died, including four who died due to type-B aortic dissection. The actuarial survival rate at 2, 5 and 10 years was 94%, 83% and 75%, respectively, with 88%, 67% and 43% of patients, respectively, not requiring reoperation. Elective aortic root replacement was associated with a low risk and a good survival rate.

  1. [Maternal and fetal outcomes with aortic dissection in pregnant patients with Marfan syndrome].

    Science.gov (United States)

    Yang, Puyu; Zhang, Jun; Li, Yanna; Wang, Hui; Zheng, Jun

    2015-05-01

    To evaluate the clinical characteristics of aortic dissection in pregnant patients with Marfan syndrome and the maternal and fetal outcomes in cardiovascular surgery. Seven pregnant women with Marfan syndrome with aortic dissection were identified, who were treated in Beijing Anzhen Hospital Affiliated to Capital Medical University between January 2012 and September 2014. Patient charts were reviewed for cardiovascular surgery, occurrence of complications, clinical features and the maternal and fetal outcomes. (1) Among 7 patients, 4 cases were diagnosed as type A aortic dissection and 3 were cases diagnosed as type B aortic dissection. The diagnosis mainly depends on CT angiography. New York Heart Association (NYHA) classify into 5 of level II, 1 of level III, 1 of leveI IV. Except for 1 patient with cardiac tamponade lead to heart failure, the remaining 6 cases had no complications. (2) Three patients underwent heart surgery with cardiopulmonary bypass in second trimester and two patients underwent heart surgery in third trimester. Two patients terminated pregnancy before heart surgery (one of whom underwent artificial abortion, one of whom underwent cesarean section in second trimester). (3) The methods of cardiovascular surgeries were as follow: 3 of Bentall+Sun', 1 of Bentall+Sun'+ right coronary artery bypass grafting, 1 of Bentall, 1 of the whole chest aorta replacement surgery, and 1 of femoral artery catheter chest aorta with membrane mesh stent implantation. The diameter of aortic roots measured during operation were 5 cm in 2 cases, 7 cm in 2 cases and 10 cm in 2 cases respectively. Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery, 1 was terminated pregnancy due to intrauterine fetal death after cardiovascular surgery, and 1 was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy after cardiovascular surgery. (4) Among the 7 cases, 3 were conducted cesarean sections during

  2. Modelling and numerical simulation of the in vivo mechanical response of the ascending aortic aneurysm in Marfan syndrome.

    Science.gov (United States)

    García-Herrera, Claudio M; Celentano, Diego J; Herrera, Emilio A

    2017-03-01

    Marfan syndrome (MFS) is a genetic disorder that affects connective tissue, impairing cardiovascular structures and function, such as heart valves and aorta. Thus, patients with Marfan disease have a higher risk of developing circulatory problems associated with mitral and aortic valves prolapse, manifested as dilated aorta and aortic aneurysm. However, little is known about the biomechanical characteristics of these structures affected with MFS. This study presents the modelling and simulation of the mechanical response of human ascending aortic aneurysms in MFS under in vivo conditions with intraluminal pressures within normotensive and hypertensive ranges. We obtained ascending aortic segments from five adults with MFS subjected to a vascular prosthesis implantation replacing an aortic aneurysm. We characterised the arterial samples via ex vivo tensile test measurements that enable fitting the material parameters of a hyperelastic isotropic constitutive model. Then, these material parameters were used in a numerical simulation of an ascending aortic aneurysm subjected to in vivo normotensive and hypertensive conditions. In addition, we assessed different constraints related to the movement of the aortic root. Overall, our results provide not only a realistic description of the mechanical behaviour of the vessel, but also useful data about stress/stretch-based criteria to predict vascular rupture. This knowledge may be included in the clinical assessment to determine risk and indicate surgical intervention.

  3. Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome: Postnatal Outcomes of the First 100 Patients

    Science.gov (United States)

    Freud, Lindsay R.; McElhinney, Doff B.; Marshall, Audrey C.; Marx, Gerald R.; Friedman, Kevin G.; del Nido, Pedro J.; Emani, Sitaram M.; Lafranchi, Terra; Silva, Virginia; Wilkins-Haug, Louise E.; Benson, Carol B.; Lock, James E.; Tworetzky, Wayne

    2015-01-01

    Background Fetal aortic valvuloplasty (FAV) can be performed for severe mid-gestation aortic stenosis (AS) in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after FAV. The postnatal outcomes and survival of the BV patients, compared to those managed as HLHS, have not been reported. Methods and Results We included 100 patients who underwent FAV for severe mid-gestation AS with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and LV volume, were significantly larger in the BV group at the time of birth (p-values <0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank p=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic and/or mitral valve replacement. On most recent echocardiogram, the median LV end-diastolic volume z-score was +1.7 (range: -1.3, +8.2), and 80% had normal ejection fraction. Conclusions Short- and intermediate-term survival among patients who underwent FAV and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and on-going assessment is warranted. PMID:25052401

  4. Surgical management of aortic root disease in Marfan syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Benedetto, Umberto; Melina, Giovanni; Takkenberg, Johanna J M; Roscitano, Antonino; Angeloni, Emiliano; Sinatra, Riccardo

    2011-06-01

    Surgical treatment of aortic root aneurysm in Marfan syndrome (MFS) patients. To compare results of total root replacement versus valve-sparing aortic root replacement in MFS patients. PubMed, Embase and Cochrane library were searched from January 1966 until February 2010 looking for papers reporting on aortic root operations in MFS patients. 530 studies were retrieved. Finally, 11 publications were enrolled. Inclusion criteria were observational studies reporting valve-related morbidity and mortality after total root replacement (TTR) and/or valve-sparing root replacement (VSRR) in patients with MFS and study size n≥30, reflecting the centre's experience. Data obtained from papers reporting both TRR and VSRR cohorts were analysed separately. In case of multiple publications, the most recent and complete report was selected. If the total number of patient-years was not provided, we calculated it by multiplying the number of hospital survivors with the mean follow-up duration of that study. Overall, 1,385 patients were analysed (972 patients had TTR and 413 patients had VSRR). Reintervention rate was 0.3%/year (95% CI 0.1 to 0.5) versus 1.3%/year (95% CI 0.3 to 2.2) (p=0.02) and thromboembolic events rate was 0.7%/year (95% CI 0.5 to 0.9) versus 0.3%/year (95% CI 0.1 to 0.6) (p=0.01) after TRR and VSRR, respectively. When composite valve-related events were compared, no difference existed between the two surgical strategies (p=0.41). Among patients undergoing VSRR, reimplantation was associated with a reduced rate of reintervention compared with remodelling (0.7%/year vs 2.4%/year, p=0.02). VSRR may represent a valuable option for patients with MFS with aortic aneurysm. However, this technique should be used with caution in patients with valve characteristics at risk for decreased durability.

  5. Usefulness of intra-aortic balloon pump counterpulsation in patients with cardiogenic shock from acute myocardial infarction

    NARCIS (Netherlands)

    Cheng, Jin M.; Valk, Suzanne D. A.; den Uil, Corstiaan A.; van der Ent, Martin; Lagrand, Wim K.; van de Sande, Meike; van Domburg, Ron T.; Simoons, Maarten L.

    2009-01-01

    Although intra-aortic balloon pump (IABP) counterpulsation is increasingly being used for the treatment of patients with cardiogenic shock from acute myocardial infarction, data on the long-term outcomes are lacking. The aim of the present study was to evaluate the 30-day and long-term mortality and

  6. Electrocardiography changes in acute aortic dissection-association with troponin leak, coronary anatomy, and prognosis.

    Science.gov (United States)

    Pourafkari, Leili; Tajlil, Arezou; Ghaffari, Samad; Chavoshi, Mohammadreza; Kolahdouzan, Kasra; Parvizi, Rezayat; Parizad, Raziyeh; Nader, Nader D

    2016-08-01

    Electrocardiography (ECG) offers some information that may be used to prognosticate acute type A aortic dissection (AAOD) for short- and long-term mortality. We retrospectively analyzed the electrocardiograms of patients with AAOD admitted from March 2004 to March 2015. The frequency of ECG findings and their prognostic value on hospital and follow-up mortality were investigated. Findings pertaining to coronary involvement and troponin level were also examined. A total of 120 men and 64 women were admitted. Acute ischemic changes were reported in 38.0%, whereas T inversion was the most common recorded abnormality, which occurred in 38.6%. Acute ST-elevation myocardial infarction was detected in 16.3%. Troponin increased in 36.6%; 21.9% of the patients underwent coronary angiography among which 70% were normal. Coronary involvement or troponin increase was not different in patients with acute ECG changes. During hospitalization, 45.7% of the patients died. In multivariate analyses, ST elevation in lead aVR was associated with higher hospital death (odds ratio, 5.30; 95% confidence interval, 1.09-25.73; P = .038), whereas QRS greater than 120 milliseconds was associated with long-term mortality (hazard ratio, 2.45; 95% confidence interval, 1.25-3.76; P = .006). Acute ischemic ECG changes are common in AAOD, and a completely normal ECG is infrequently encountered. Acute ECG changes were not associated with the increased troponin or the presence of coronary lesions in angiography. Published by Elsevier Inc.

  7. Valve-sparing David I procedure in acute aortic type A dissection: a 20-year experience with more than 100 patients.

    Science.gov (United States)

    Beckmann, Erik; Martens, Andreas; Pertz, Jana; Kaufeld, Tim; Umminger, Julia; Hanke, Jasmin S; Schmitto, Jan D; Cebotari, Serghei; Haverich, Axel; Shrestha, Malakh Lal

    2017-08-01

    The aortic valve-sparing David procedure has been applied to the elective treatment of patients with aortic aneurysms with excellent results. The use of this technique in patients with acute aortic dissection type A (AADA) is still a matter of debate. We present our long-term experience with 109 patients with AADA who had the valve-sparing David I procedure. Between July 1993 and October 2015, 109 patients with AADA had the valve-sparing David I procedure at our centre. We conducted a retrospective review with follow-up. The mean age was 54 ± 12 years; 78 (72%) patients were men. Marfan syndrome was present in 6 (5%) patients and bicuspid aortic valve in 3 (3%). Only 4 (4%) patients received the isolated David procedure; 50 (46%) underwent additional proximal, 13 (12%) subtotal and 42 (39%) total aortic arch replacement. The in-hospital mortality rate was 11% ( n  = 12). Intraoperative/discharge echocardiography showed aortic insufficiency ≤ I° in 93 of 97 patients (96%). Mean follow-up time was 8.3 ± 5.7 years. The survival rate after discharge at 1, 5 and 10 years was 94%, 90% and 78%, respectively. Thirteen percent ( n  = 13) of patients underwent valve-related reoperation. Freedom from valve-related reoperation at 1, 5 and 10 years was 96%, 88% and 85%, respectively. Compared to patients who underwent the David I procedure for any reason other than AADA, there were no significant differences in long-term survival rates ( P  = 0.29) and freedom from a valve-related reoperation ( P  = 0.39). The valve-sparing David I procedure has acceptable long-term results even in emergent operations for AADA and is not inferior when performed in elective settings. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  8. Phenotype in girls and women with turner syndrome: Association between dysmorphic features, karyotype and cardio-aortic malformations.

    Science.gov (United States)

    Noordman, Iris; Duijnhouwer, Anthonie; Kapusta, Livia; Kempers, Marlies; Roeleveld, Nel; Schokking, Michiel; Smeets, Dominique; Freriks, Kim; Timmers, Henri; van Alfen-van der Velden, Janiëlle

    2018-01-12

    Turner syndrome (TS) is a genetic disorder characterized by the (partial) absence or a structural aberration of the second sex chromosome and is associated with a variety of phenotypes with specific physical features and cardio-aortic malformations. The objective of this study was to gain a better insight into the differences in dysmorphic features between girls and women with TS and to explore the association between these features, karyotype and cardio-aortic malformations. This prospective study investigated 14 dysmorphic features of TS girls and women using a checklist. Three major phenotypic patterns were recognized (severe phenotype, lymphatic phenotype and skeletal phenotype). Patient data including karyotype and cardio-aortic malformations (bicuspid aortic valve (BAV) and aortic coarctation (COA)) were collected. Associations between the prevalence of dysmorphic features, karyotype and cardio-aortic malformations were analysed using chi 2 -test and odds ratios. A total of 202 patients (84 girls and 118 women) were analysed prospectively. Differences in prevalence of dysmorphic features were found between girls and women. A strong association was found between monosomy 45,X and the phenotypic patterns. Furthermore, an association was found between COA and lymphatic phenotype, but no association was found between karyotype and cardio-aortic malformations. This study uncovered a difference in dysmorphic features between girls and women. Monosomy 45,X is associated with a more severe phenotype, lymphatic phenotype and skeletal phenotype. All patients with TS should be screened for cardio-aortic malformations, because in contrast to previous reports, karyotype and cardio-aortic malformations showed no significant association. Copyright © 2018. Published by Elsevier Masson SAS.

  9. Rivaroxaban in patients with a recent acute coronary syndrome

    DEFF Research Database (Denmark)

    Mega, Jessica L; Braunwald, Eugene; Wiviott, Stephen D

    2012-01-01

    Acute coronary syndromes arise from coronary atherosclerosis with superimposed thrombosis. Since factor Xa plays a central role in thrombosis, the inhibition of factor Xa with low-dose rivaroxaban might improve cardiovascular outcomes in patients with a recent acute coronary syndrome....

  10. Chinese herbal medicine for severe acute respiratory syndrome

    DEFF Research Database (Denmark)

    Liu, Jianping; Manheimer, Eric; Shi, Yi

    2004-01-01

    To review randomized controlled trials (RCTs) evaluating the effects of Chinese herbal medicine for treating severe acute respiratory syndrome (SARS) systematically.......To review randomized controlled trials (RCTs) evaluating the effects of Chinese herbal medicine for treating severe acute respiratory syndrome (SARS) systematically....

  11. Continuous glucose monitoring in acute coronary syndrome.

    Science.gov (United States)

    Rodríguez-Quintanilla, Karina Alejandra; Lavalle-González, Fernando Javier; Mancillas-Adame, Leonardo Guadalupe; Zapata-Garrido, Alfonso Javier; Villarreal-Pérez, Jesús Zacarías; Tamez-Pérez, Héctor Eloy

    2013-01-01

    Diabetes mellitus is an independent risk factor for cardiovascular disease. To compare the efficacy of devices for continuous glucose monitoring and capillary glucose monitoring in hospitalized patients with acute coronary syndrome using the following parameters: time to achieve normoglycemia, period of time in normoglycemia, and episodes of hypoglycemia. We performed a pilot, non-randomized, unblinded clinical trial that included 16 patients with acute coronary artery syndrome, a capillary or venous blood glucose ≥ 140 mg/dl, and treatment with a continuous infusion of fast acting human insulin. These patients were randomized into 2 groups: a conventional group, in which capillary measurement and recording as well as insulin adjustment were made every 4h, and an intervention group, in which measurement and recording as well as insulin adjustment were made every hour with a subcutaneous continuous monitoring system. Student's t-test was applied for mean differences and the X(2) test for qualitative variables. We observed a statistically significant difference in the mean time for achieving normoglycemia, favoring the conventional group with a P = 0.02. Continuous monitoring systems are as useful as capillary monitoring for achieving normoglycemia. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  12. Pathogenesis and symptomatics of the acute radiation syndrome

    International Nuclear Information System (INIS)

    Fliedner, T.M.; Haen, M.; Carbonell, F.

    1980-01-01

    The pathogenesis and symptomatics of the acute radiation syndrome are discussed. Diagnosis and therapy would be impossible without detailed knowledge in these fields. The concept of acute radiation syndrome is explained, and a pathophysiological analysis of the various forms of radiation syndrome - haematological, intestinal and affecting the central nervous system is attempted. The developments in the diagnosis and therapy of acute radiation syndrome since its first description - 35 years ago - are reviewed. Today, whole-body doses of 100 rd and more can be treated by radiotherapy. (orig./MG) [de

  13. The diagnosis of atherosclerotic aortic ulcer by electron beam CT

    International Nuclear Information System (INIS)

    Zhi Aihua; Dai Ruping; Jiang Shiliang

    2007-01-01

    Objective: To evaluate the clinical value of electron beam computed tomography (EBCT) in the diagnosis of atherosclerotic aortic ulcer. Methods: Sixty-eight consecutive patients (55 men and 13 women, aged 40-85 years, mean 65.12 ± 9.55 years) with atherosclerotic aortic ulcer, who underwent EBCT scans from December 2001 to December 2004, were studied retrospectively. Contrast-enhanced continuous volume scanning (CVS) was performed by Imatron C-150XP EBCT scanner with 6 mm or 3 mm slice thickness and 100 milliseconds acquisition time. The scan was started 18-30 s after the injection of 80-100 ml contrast medium at the rate of 3.5-4.5 ml/s. Results: In sixty-eight patients with atherosclerotic aortic ulcer, 50 patients had acute aortic syndromes, 36 had intramural hematomas, 15 had atherosclerotic aortic aneurysms, 3 had aortic dissections. 46 patients with progresive ulcer usually had acute aortic syndrome while 22 patients with stable ulcer didn't (P<0.01). Atherosclerotic aortic ulcer was seen more frequently in the aorta arch than other portions of the aorta (P<0.01). Conclusion: EBCT is a very useful tool for the detection and follow-up of atherosclerotic aortic ulcer. (authors)

  14. Participation of Arachidonic Acid Metabolism in the Aortic Aneurysm Formation in Patients with Marfan Syndrome

    Directory of Open Access Journals (Sweden)

    María E. Soto

    2018-02-01

    Full Text Available Marfan syndrome (MFS is a pleiotropic genetic disease involving the cardiovascular system where a fibrillin-1 mutation is present. This mutation is associated with accelerated activation of transforming growth factor β (TGFβ1 which contributes to the formation of aneurysms in the root of the aorta. There is an imbalance in the synthesis of thromboxane A2 (TXA2 and prostacyclin, that is a consequence of a differential protein expression of the isoforms of cyclooxygenases (COXs, suggesting an alteration of arachidonic acid (AA metabolism. The aim of this study was to analyze the participation of AA metabolism associated with inflammatory factors in the dilation and dissection of the aortic aneurysm in patients with MFS. A decrease in AA (p = 0.02, an increase in oleic acid (OA, TGFβ1, tumor necrosis factor alpha (TNFα, prostaglandin E2 (PGE2 (p < 0.05, and COXs activity (p = 0.002 was found. The expressions of phospholipase A2 (PLA2, cytochrome P450 (CYP450 4A, 5-lipoxygenase (5-LOX, COX2 and TXA2R (p < 0.05 showed a significant increase in the aortic aneurysm of patients with MFS compared to control subjects. COX1, 6-keto-prostaglandin 1 alpha (6-keto-PG1α and 8-isoprostane did not show significant changes. Histological examination of the aortas showed an increase of cystic necrosis, elastic fibers and collagen in MFS. The results suggest that there are inflammatory factors coupled to genetic factors that predispose to aortic endothelial dysfunction in the aortic tissue of patients with MFS. There is a decrease in the percentage of AA, associated with an increase of PLA2, COX2/TXA2R, CYP450 4A, and 5-LOX which leads to a greater synthesis of PGE2 than of 6-keto-PGF1α, thus contributing to the formation of the aortic aneurysm. The evident loss of the homeostasis in these mechanisms confirms that there is a participation of the AA pathway in the aneurysm progression in MFS.

  15. Painless Aortic Dissection—Diagnostic Dilemma With Fatal Outcomes: What Do We Learn?

    Directory of Open Access Journals (Sweden)

    Saeeda Fatima MD

    2017-07-01

    Full Text Available Aortic dissection is the most catastrophic clinical condition that involves the aorta. It has a high mortality as well as high rate of misdiagnosis due to frequent unusual presentation. Typically, it presents with acute chest, back, and tearing abdominal pain. However, it can present atypically with minimal or no pain, making diagnosis difficult. Physicians should always suspect acute aortic dissection in patients with certain clinical conditions like difficult-to-control hypertension, giant cell arteritis, bicuspid aortic valve, intracranial aneurysms, simple renal cysts, family history of aortic disease, and Marfan syndrome, especially when a patient presents with ischemic symptoms involving multiple organ without an obvious cause.

  16. Hemiarch versus total aortic arch replacement in acute type A dissection: a systematic review and meta-analysis.

    Science.gov (United States)

    Poon, Shi Sum; Theologou, Thomas; Harrington, Deborah; Kuduvalli, Manoj; Oo, Aung; Field, Mark

    2016-05-01

    Despite recent advances in aortic surgery, acute type A aortic dissection remains a surgical emergency associated with high mortality and morbidity. Appropriate management is crucial to achieve satisfactory outcomes but the optimal surgical approach is controversial. The present systematic review and meta-analysis sought to access cumulative data from comparative studies between hemiarch and total aortic arch replacement in patients with acute type A aortic dissection. A systematic review of the literature using six databases. Eligible studies include comparative studies on hemiarch versus total arch replacement reporting short, medium and long term outcomes. A meta-analysis was performed on eligible studies reporting outcome of interest to quantify the effects of hemiarch replacement on mortality and morbidity risk compared to total arch replacement. Fourteen retrospective studies met the inclusion criteria and 2,221 patients were included in the final analysis. Pooled analysis showed that hemiarch replacement was associated with a lower risk of post-operative renal dialysis [risk ratio (RR) =0.72; 95% confidence interval (CI): 0.56-0.94; P=0.02; I(2)=0%]. There was no significant difference in terms of in-hospital mortality between the two groups (RR =0.84; 95% CI: 0.65-1.09; P=0.20; I(2)=0%). Cardiopulmonary bypass, aortic cross clamp and circulatory arrest times were significantly longer in total arch replacement. During follow up, no significant difference was reported from current studies between the two operative approaches in terms of aortic re-intervention and freedom from aortic reoperation. Within the context of publication bias by high volume aortic centres and non-randomized data sets, there was no difference in mortality outcomes between the two groups. This analysis serves to demonstrate that for those centers doing sufficient total aortic arch activity to allow for publication, excellent and equivalent outcomes are achievable. Conclusions on

  17. Subclinical nephritic syndrome in children cohabiting with pediatric patients, Presenting acute nephritic syndrome

    OpenAIRE

    Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil

    2012-01-01

    Introduction: subclinical nephritic syndrome is the presence of hematuria, hypocomplementemiaand/or proteinuria without the presence of signs and/or symptoms.Objective: to determine the incidence of subclinical nephritic syndrome in childrenliving with pediatric patients diagnosed with acute nephritic syndrome.Methods: family visit to identify children living together in the two previous months, with pediatric patients hospitalized with acute nephritic syndrome, at Hospital InfantilNapoleon F...

  18. Colchicine Acutely Suppresses Local Cardiac Production of Inflammatory Cytokines in Patients With an Acute Coronary Syndrome

    Science.gov (United States)

    Martínez, Gonzalo J; Robertson, Stacy; Barraclough, Jennifer; Xia, Qiong; Mallat, Ziad; Bursill, Christina; Celermajer, David S; Patel, Sanjay

    2015-01-01

    Background Interleukin (IL)-1β, IL-18, and downstream IL-6 are key inflammatory cytokines in the pathogenesis of coronary artery disease. Colchicine is believed to block the NLRP3 inflammasome, a cytosolic complex responsible for the production of IL-1β and IL-18. In vivo effects of colchicine on cardiac cytokine release have not been previously studied. This study aimed to (1) assess the local cardiac production of inflammatory cytokines in patients with acute coronary syndromes (ACS), stable coronary artery disease and in controls; and (2) determine whether acute administration of colchicine inhibits their production. Methods and Results Forty ACS patients, 33 with stable coronary artery disease, and 10 controls, were included. ACS and stable coronary artery disease patients were randomized to oral colchicine treatment (1 mg followed by 0.5 mg 1 hour later) or no colchicine, 6 to 24 hours prior to cardiac catheterization. Blood samples from the coronary sinus, aortic root (arterial), and lower right atrium (venous) were collected and tested for IL-1β, IL-18, and IL-6 using ELISA. In ACS patients, coronary sinus levels of IL-1β, IL-18, and IL-6 were significantly higher than arterial and venous levels (P=0.017, Colchicine administration significantly reduced transcoronary gradients of all 3 cytokines in ACS patients by 40% to 88% (P=0.028, 0.032, and 0.032, for IL-1β, IL-18, and IL-6, respectively). Conclusions ACS patients exhibit increased local cardiac production of inflammatory cytokines. Short-term colchicine administration rapidly and significantly reduces levels of these cytokines. PMID:26304941

  19. Surgical reconstruction of aortic root in Marfan syndrome patients: a systematic review.

    Science.gov (United States)

    Hu, Rui; Wang, Zhiwei; Hu, Xiaoping; Wu, Hongbing; Wu, Zhiyong; Zhou, Zhen

    2014-07-01

    Several recent studies have compared the clinical results of valve-sparing (VS) surgery and composite valve graft (CVG) surgery in the aortic root reconstruction of Marfan syndrome (MS) patients. The study aim was to investigate whether it is appropriate to preserve the native aortic valve in root surgery of MFS patients when taking the short-term and long-term prognoses into consideration. A thorough literature search of PubMed, Embase and Cochrane library was conducted to identify studies comparing the outcomes of VS and CVG surgery in MFS patients. The Newcastle-Ottawa Scale evaluation scheme was used to assess the methodological quality of the included articles. Data were extracted from reports and analyzed using Revman 5.0, supplied by Cochrane collaboration. Six clinical trials incorporating 539 patients were included. Compared to CVG, VS surgery was associated with a lower risk for re-exploration (RR 0.48, 95% CI 0.24-0.97; p = 0.04), thromboembolic events (RR 0.17, 95% CI 0.05-0.57; p = 0.004) and endocarditis (RR 0.31, 95% CI 0.11-0.94; p = 0.04). Despite an inherent incidence of aortic regurgitation, VS surgery resulted in an elevation of long-term survival rate; however, no statistical differences were found between groups with regards to reoperation (RR 1.07, 95% CI 0.35-3.27; p = 0.91). Root reconstruction with VS surgery can effectively improve the prognosis of MFS patients and provide a promising alternative for surgical treatment. However, the results must be interpreted with caution due to the retrospective nature of the included studies; large-scale prospective control trials are needed to confirm these findings.

  20. Disease Beyond the Arch: A Systematic Review of Middle Aortic Syndrome in Childhood.

    Science.gov (United States)

    Rumman, Rawan K; Nickel, Cheri; Matsuda-Abedini, Mina; Lorenzo, Armando J; Langlois, Valerie; Radhakrishnan, Seetha; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

    2015-07-01

    Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options. A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes. Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking. MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition. © American Journal of Hypertension, Ltd 2015. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  1. Consecutive operative procedures in patients with Marfan syndrome up to 28 years after initial aortic root surgery.

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    Puluca, Nazan; Burri, Melchior; Cleuziou, Julie; Krane, Markus; Lange, Rüdiger

    2018-02-28

    Most patients (75%) with Marfan syndrome present with aortic root dilatation that may require surgical intervention. However, associated cardiovascular disorders are not limited to the aortic root. These patients frequently require consecutive operations on the remaining thoracic aorta or the heart valves. Our intent was to characterize the spectrum of such procedures. Data from all patients with Marfan syndrome undergoing aortic root surgery at our centre between 1988 and 2016 were analysed retrospectively. Overall, 73 patients (26 women) were selected for the study. The median age at 1st operation was 30 years (3-68 years). Indications for aortic root surgery were aneurysm (78%) and dissection (22%). Initially, 33 Bentall procedures and 40 valve-sparing root replacement procedures were performed, with a 97% rate of follow-up completion. The median follow up was 8 years (0-28 years). Survival at 1, 10 and 15 years was 100%, 85% and 82%, respectively. During follow-up monitoring, 48 subsequent procedures were performed in 33 patients (aorta, 23; aortic valve, 11; mitral valve, 7 and combined procedures, 7). The 30-day mortality rate after subsequent procedures was 4.2%. Freedom from subsequent operation and death (combined end-point) after 5, 10 and 15 years was 70%, 53% and 34%, respectively. After the initial surgery, subsequent procedures required in the setting of Marfan syndrome most often involve the remaining native aorta, followed by the aortic and mitral valves. The continued need for additional operative procedures remains high, even decades after the initial surgeries are undertaken. Therefore, long-term patient monitoring at specialized centres is imperative.

  2. Abdominal compartment syndrome caused by ruptured abdominal aortic aneurysm in vena cava

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    Filipović Aleksandar

    2006-01-01

    Full Text Available Background. Abdominal compartment syndrome (ACS is a rapid increase in intra-abdominal pressure associated with multi-organs dysfunction. It is caused mostly by abdominal bleeding und massive volume compensation. Case report. We reported a 76-year-old patient admitted to the hospital with aortic abdominal aneurysm, 13.7 cm in diameter, ruptured in vena cava, which caused intraabdominal hypertension, the liver and kidney dysfunction, as well as circulation, respiration and metabolic disorders. Intraabdominal pressure was measured by bladder manometry. Central venous pressure and systemic arterial pressure were monitored continuously. Clinical signs were thrill and typical abdominal bruit. Aorto-caval fistula was diagnosed by the use of contrast computerized tomography. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 × 9 mm Dacron prothesis were performed. Haemodynamic changes were mostly corrected during the surgery. The complete correction of haemodynamics, liver, kidney, respiration and metabolic changes was established in the next few weeks. Conclusion. The ACS was caused by rupture of abdominal aortic aneurysm in vena cava followed by edema of the abdominal organs, retroperitoneum, abdominal wall and ascites. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 × 9 mm Dacron prothesis solved aortocaval fistula as well as all the organs and metabolic dysfunctions caused by ACS.

  3. Acute respiratory distress syndrome: evaluation and management.

    Science.gov (United States)

    Cortés, I; Peñuelas, O; Esteban, A

    2012-03-01

    Acute respiratory distress syndrome (ARDS) is a life-threatening condition that affects patients admitted in the Intensive Care Units (ICUs) under mechanical ventilation. ARDS is a process of non-hydrostatic pulmonary edema and hypoxemia associated with a variety of conditions, resulting in a direct (e.g., pneumonia) or indirect (e.g., sepsis) lung injury and is associated with a significant morbidity and mortality. A large body of clinical and basic research has focused in ventilatory strategies and novel pharmacological therapies but, nowadays, treatment is mainly supportive. Mechanical ventilation is the hallmark of the management of these patients. In the last decades, the recognition that mechanical ventilation can contribute to harming the lung has changed the goals of this therapy and has driven research to focus in ventilatory strategies that mitigate lung injury. This review emphasizes clinical aspects in the evaluation and management of ARDS in the ICUs and updates the latest advances in these therapies.

  4. [Inflammatory biomarkers in ischemic acute coronary syndrome].

    Science.gov (United States)

    Domínguez-Rodríguez, Alberto; Abreu-González, Pedro

    2015-10-01

    Diagnosing acute coronary syndrome (ACS) in the emergency department is often a complex process. Inflammatory markers might be useful for the rapid assessment of a patient's overall risk and might also help predict future episodes. The clinical use of these biomarkers could potentially lower the number of emergency visits and help in the prevention of future adverse events. The aim of this review was to evaluate the clinical utility of markers of cardiovascular inflammation in emergency patients with ACS. Based on a critical analysis of a selection of the literature, we concluded that none of the biomarkers of cardiovascular inflammation would at present be useful for stratifying risk in emergency situations, aiding prognosis, or guiding therapy for patients with ACS.

  5. Pathogenesis of Acute Respiratory Distress Syndrome

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    A. M. Golubev

    2012-01-01

    Full Text Available Acute respiratory distress syndrome (ARDS is a common complication of many diseases. Its polyetiological pattern determines the specific features of lung morphological changes and the clinical course of ARDS. Objective: to analyze the pathogenesis of ARDS in the context of the general pathological processes underlying its development. Material and methods. More than 200 lungs from the people who had died from severe concomitant injury or ARDS-complicated pneumonia were investigated. More than 150 rat experiments simulated various types of lung injury: ventilator-induced lung injury with different ventilation parameters; reperfusion injuries (systemic circulation blockade due to 12-minute vascular fascicle ligation, followed by the recovery of cardiac performance and breathing; microcirculatory disorder (injection of a thromboplastin solution into the jugular vein; blood loss; betaine-pepsin aspiration; and closed chest injury. Different parts of the right and left lungs were histologically examined 1 and 3 hours and 1 and 3 days after initiation of the experiment. Lung pieces were fixed in 10% neutral formalin solution and embedded in paraffin. Histological sections were stained with hematoxylin and eosin and using the van Gieson and Weigert procedures; the Schiff test was used. Results. The influence of aggression factors (trauma, blood loss, aspiration, infection, etc. results in damage to the lung and particularly air-blood barrier structures (endothelium, alveolar epithelium, their basement membrane. In turn the alteration of cellular and extracellular structures is followed by the increased permeability of hemomicrocirculatory bed vessels, leading to the development of non-cardiogenic (interstitial, alveolar pulmonary edema that is a central component in the pathogenesis of ARDS. Conclusion. The diagnosis of the early manifestations of ARDS must account for the nature of an aggression factor, the signs confirming the alteration of the lung

  6. Transcatheter double stent implantation for treatment of middle aortic coarctation syndrome.

    Science.gov (United States)

    McMahon, Colin J; Lambert, Imelda; Walsh, Kevin P

    2013-10-01

    A 13-year-old boy presented with severe systemic hypertension. His upper limb blood pressure measured 190/100 mm Hg and lower limb blood pressure measured 98/64 mm Hg. The brachial pulses were bounding and femoral pulses were not palpable. Echocardiography and magnetic resonance angiography confirmed middle aortic syndrome. There was severe diffuse thoraco-abdominal coarctation with continuous Doppler run-off. Cardiac catheterization was undertaken and using a retrograde approach two Advanta V12 stents were implanted in the complex thoraco-abdominal coarctation. The gradient across the coarctation was reduced from 80 to 40 mm Hg gradient with a significant improvement in the luminal diameter of the aorta. His upper limb blood pressure reduced to 142/78 mm Hg six weeks later. Copyright © 2013 Wiley Periodicals, Inc.

  7. Acute Respiratory Distress: from syndrome to disease.

    Science.gov (United States)

    Cardinal-Fernández, P; Correger, E; Villanueva, J; Rios, F

    2016-04-01

    The acute respiratory distress syndrome (ARDS) is currently one of the most important critical entities given its high incidence, rate of mortality, long-term sequelae and non-specific pharmacological treatment. The histological hallmark of ARDS is diffuse alveolar damage (DAD). Approximately 50% of ARDS patients present DAD, the rest is made up of a heterogeneous group of histological patterns, many of which correspond to a well-recognized disease. For that reason, if these patterns could be diagnosed, patients could benefit from a treatment. Recently, the effect of DAD in clinical and analytical evolution of ARDS has been demonstrated, so the classical approach to ARDS as an entity defined solely by clinical, radiological and gasometrical variables should be reconsidered. This narrative review aims to examine the need to evolve from the concept of ARDS as a syndrome to ARDS as a specific disease. So we have raised 4 critical questions: a) What is a disease?; b) what is DAD?; c) how is DAD considered according to ARDS definition?, and d) what is the relationship between ARDS and DAD? Copyright © 2015 Elsevier España, S.L.U. and SEMICYUC. All rights reserved.

  8. The pathophysiology of the acute compartment syndrome.

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    Tollens, T; Janzing, H; Broos, P

    1998-08-01

    The acute compartment syndrome is a condition in which increased pressure within a limited space compromises the circulation and function of the tissues therein, resulting in tissue ischaemia, necrosis and nerve damage. This rise in tissue pressure originates in a decrease of the compartment size or increase of the intracompartmental volume by oedema and/or haemorrhage. Following the arterio-venous gradient theory, capillary blood flow may be impaired through increased venous pressure, decreased arterial pressure and increased peripheral vascular resistance. Often, compartment syndromes develop during reperfusion following a period of ischaemia. During ischaemia, there is a gradual depletion of intracellular stores of high energy phosphate bonds and glycogen stores. There is a buildup of products of glycolysis, particularly lactic acid, with accompanying hydrogen ion accumulation as well as an increase in intracellular reducing agents. Reperfusion may, instead of restoring normal muscle metabolic activity, cause harmful effects by washing out necessary precursors for adenine nucleotide resynthesis. Production of oxygen free radicals occurs with ensuing lipid peroxidation, and calcium influx occurs upon reoxygenation with resultant disruption of oxidative rephosphorylation in the mitochondria. Furthermore, several lines of evidence suggest that white blood cells are important in the pathogenesis of reperfusion injury. Upregulation of both neutrophil receptors and endothelial leucocyte adhesion molecules leads to the sequestration of white blood cells in the muscle with prolongation of the reperfusion injury. This subsequently results in damage to remote organs such as lungs, liver, heart and kidneys.

  9. Prognostic value of sustained elevated C-reactive protein levels in patients with acute aortic intramural hematoma.

    Science.gov (United States)

    Kitai, Takeshi; Kaji, Shuichiro; Kim, Kitae; Ehara, Natsuhiko; Tani, Tomoko; Kinoshita, Makoto; Furukawa, Yutaka

    2014-01-01

    The appropriate management of aortic intramural hematoma is still controversial, because a variety of aortic events can arise during follow-up in some patients. However, simplified identification of these patients remains challenging. The present study aimed to determine the prognostic significance of serial C-reactive protein measurements for the prediction of adverse events in patients with acute aortic intramural hematoma. A total of 180 patients with aortic intramural hematoma were retrospectively reviewed. The C-reactive protein data were obtained at admission and 2 days, 1 week, and 2 weeks from the onset, and the maximum value was obtained during the acute phase. Adverse aorta-related events were defined by a composite of aortic rupture, aortic aneurysm, and surgical or endovascular aortic repair. The C-reactive protein value was 3.0 ± 4.6, 8.7 ± 5.9, 9.0 ± 5.5, and 5.7 ± 4.5 mg/dL on admission and 2 days, 1 week, and 2 weeks from the onset, respectively. The maximal value of C-reactive protein was 12.4 ± 6.3 mg/dL at a mean of 4 days from the onset. Patients with elevated C-reactive protein levels (≥7.2 mg/dL) at 2 weeks had significantly greater rates of aorta-related events (P analysis, an elevated C-reactive protein level at 2 weeks (hazard ratio, 3.16; P value compared with the development of an ulcer-like projection (chi-square, 16.94 for ulcer-like projection only vs 34.32 with the addition of C-reactive protein at 2 weeks, P < .001). C-reactive protein was a simple and useful marker providing incremental prognostic information compared with the development of an ulcer-like projection in patients with aortic intramural hematoma. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  10. ANALYSIS OF PRE-HOSPITAL TREATMENT OF ACUTE CORONARY SYNDROME

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    O. V. Reshetko

    2007-01-01

    Full Text Available Aim. To evaluate the pre-hospital treatment of patients with acute coronary syndromes (acute myocardial infarction and unstable angina in 2001 and 2006.Material and methods. Retrospective pre-hospital treatment survey was performed in 1114 patients with acute coronary syndrome (acute myocardial infarction (AMI or unstable angina (UA in 2001 and 2006.Results. For acute myocardial infarction use of aspirin, β-blockers, heparin was 0%, 0%, 81,5% in 2001 and 23,9%, 8%, 13,4% in 2006, respectively. Use of aspirin, β-blockers, heparin in unstable angina were 0%, 16,2%, 12,3% in 2001 and 3,4%, 1,6%, 0,5% in 2006, respectively. Fibrinolytic therapy was not provided. Polypragmasia reduced in 2006 in comparison with 2001.Conclusions. This survey demonstrates the discordance between existing current practice and guidelines for acute coronary syndrome.

  11. Acute respiratory distress syndrome: epidemiology and management approaches

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    Walkey AJ

    2012-07-01

    Full Text Available Allan J Walkey,1 Ross Summer,1 Vu Ho,1 Philip Alkana21The Pulmonary Center, Boston University School of Medicine, Boston, MA, USA; 2Asthma Research Center, Brigham and Women's Hospital, Boston, MA, USAAbstract: Acute lung injury and the more severe acute respiratory distress syndrome represent a spectrum of lung disease characterized by the sudden onset of inflammatory pulmonary edema secondary to myriad local or systemic insults. The present article provides a review of current evidence in the epidemiology and treatment of acute lung injury and acute respiratory distress syndrome, with a focus on significant knowledge gaps that may be addressed through epidemiologic methods.Keywords: acute lung injury, acute respiratory distress syndrome, review, epidemiology

  12. Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis.

    Science.gov (United States)

    Al-Kindi, Sadeer G; Amer Al-Aiti, M; Yang, Michael; Josephson, Richard A

    2017-01-01

    Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can have multisystem involvement, though cardiac involvement is very rare. The case is described of a 53-year-old woman who presented with acute aortic and mitral valve regurgitation requiring surgical intervention. Pathology from the excised aortic valve showed geographic necrosis concerning for GPA. Subsequent rheumatologic testing was positive for anti-serine proteinase 3 (PR3) antibody, consistent with GPA. A year after the valve surgery the patient was found to have a vegetation of the mitral valve and elevated PR3 antibody levels, and was successfully treated with an intensification of immunosuppression. The cardiac involvement of GPA is discussed and big data analyzed to identify the epidemiology of valvular involvement. In conclusion, GPA can have multiple different valvular manifestations with a high recurrence rate. GPA should be considered in patients who present with acute valvular disease without any clear precipitant.

  13. [Anesthetic Management of a Patient Complicated with Marfan Syndrome and Suffering from Stanford Type A Aortic Dissection during Pregnancy].

    Science.gov (United States)

    Uozaki, Nako; Mizuno, Kaori; Shiraishi, Yoshito; Doi, Matsuyuki; Sato, Shigehito

    2015-04-01

    We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery. Although intra-aortic balloon pumping and percutaneous cardiopulmonary support were required after weaning from the cardiopulmonary bypass, she was discharged on post-operative day (POD) 40 and the baby was discharged on POD 60, without signs of cerebral palsy. Unfortunately, this patient died on POD 57, due to heart failure. We discuss how to determine the priority of surgeries for patients who require emergency surgery for cardiovascular disease during pregnancy.

  14. The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome.

    Science.gov (United States)

    Benke, Kálmán; Ágg, Bence; Pólos, Miklós; Sayour, Alex Ali; Radovits, Tamás; Bartha, Elektra; Nagy, Péter; Rákóczi, Balázs; Koller, Ákos; Szokolai, Viola; Hedberg, Julianna; Merkely, Béla; Nagy, Zsolt B; Szabolcs, Zoltán

    2017-07-17

    Marfan syndrome is a genetic disease, presenting with dysfunction of connective tissues leading to lesions in the cardiovascular and skeletal muscle system. Within these symptoms, the most typical is weakness of the connective tissue in the aorta, manifesting as aortic dilatation (aneurysm). This could, in turn, become annuloaortic ectasia, or life-threatening dissection. As a result, life-saving and preventative cardiac surgical interventions are frequent among Marfan syndrome patients. Aortic aneurysm could turn into annuloaortic ectasia or life-threatening dissection, thus life-saving and preventive cardiac surgical interventions are frequent among patients with Marfan syndrome. We hypothesized that patients with Marfan syndrome have different level of anxiety, depression and satisfaction with life compared to that of the non-clinical patient population. Patients diagnosed with Marfan syndrome were divided into 3 groups: those scheduled for prophylactic surgery, those needing acute surgery, and those without need for surgery (n = 9, 19, 17, respectively). To examine the psychological features of the patients, Spielberger's anxiety (STAI) test, Beck's Depression questionnaire (BDI), the Berne Questionnaire of Subjective Well-being, and the Satisfaction with Life scale were applied. A significant difference was found in trait anxiety between healthy individuals and patients with Marfan syndrome after acute life-saving surgery (p Marfan syndrome patients was 48.56 (standard deviation (SD): 5.8) as compared to the STAI population mean score of 43.72 (SD: 8.53). No difference was found between groups on the BDI (p > 0.1). Finally, a significant, medium size effect was found between patient groups on the Joy in Living scale (F (2.39) = 3.51, p = 0.040, η 2  = 0.15). Involving psychiatric and mental-health care, in addition to existing surgical treatment interventions, is essential for more successful recovery of patients with Marfan syndrome.

  15. [A re-operative case of bentall operation and aortic arch replacement using a stent graft for a Marfan syndrome, post sternum turnover and post mitral valve replacement].

    Science.gov (United States)

    Sasaki, H; Aomi, S; Noji, S; Uwabe, K; Kihara, S; Kurihara, H; Koyanagi, H

    2001-09-01

    A 36-year-old male with Marfan syndrome succesfully underwent Bentall operation and aortic arch replacement using a stent graft as an elephant trunk. He had received MVR with sternum turn over 14 years previously. Median sternotomy was performed. Under circulatory arrest with rertograde cerebral perfusion we performed Bentall operation and aortic arch replacement using a stent graft. The sternum was cured well. Retractive breathing was not detected. This surgical procedure was effective for cardiovascular disease with Marfan syndrome.

  16. Transcatheter aortic valve implantation for bicuspid aortic valve stenosis: Acute and intermediate-term outcomes in a high volume institution

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    Anže Djordjević

    2017-03-01

    Full Text Available Background: We report our experience with transcatheter aortic valve implantation (TAVI in patients with bicuspid aortic valve (BAV stenosis.Methods: Perioperative and intermediate-term follow-up data were retrospectively analysed. All procedures were performed within the premises of an experienced high-volume TAVI centre.Results: Tirty-three consecutive BAV patients (age 55 to 87 years underwent TAVI. Mean logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE was 23,2 ± 19,3. Transapical Edwards Sapien® valve was implanted in the majority of patients (87.9 %. Nine patients (27.3 % required post-ballooning of the implanted valve for moderate to severe paravalvular leak, 3 patients (9 % required a second valve implantation for persistent severe paravalvular leak, and 2 (6 % required conversion to conventional surgery. Post-operative mild aortic regurgitation (AR was presented in 12 patients (36.4% and AR = 2 in 3 %. No AR > 2 was observed. Te device success rate according to the valve academic research consortium (VARC criteria was 82 %. Similar BAV anatomy, calcium distribution, type and size of implanted valve were noticed in patients with and without residual AR. Tere was no thirty-day mortality. Two-year estimated survival was 70 % (CI: 52.7–93.1 and was similar in patients with and without post-procedural residual paravalvular leak.Conclusions: TAVI in BAV stenosis is feasible but, even in experienced centres, is technically more challenging and is associated with a higher rate of post-dilatation, re-valving, and conversion to conventional surgery. Results should be re-tested in light of the recent introduction of second-generation TAVI prostheses.

  17. Lack of neurologic improvement after aortic repair for acute type A aortic dissection complicated by cerebral malperfusion: predictors and association with survival.

    Science.gov (United States)

    Morimoto, Naoto; Okada, Kenji; Okita, Yutaka

    2011-12-01

    Surgical treatment of acute type A aortic dissection complicated by cerebral malperfusion remains challenging. This study evaluated predictors of lack of neurologic improvement after aortic repair for acute type A dissection complicated by cerebral malperfusion and assessed relationship with survival. We retrospectively reviewed 41 consecutive patients operated on between 1999 and 2008 for acute type A dissection complicated by cerebral malperfusion. Lack of postoperative neurologic improvement was defined as a difference between baseline and postoperative National Institutes of Health Stroke Scale scores of 3 points or less. Lack of neurologic improvement was seen in 15 patients (37%). Logistic regression analysis, baseline National Institutes of Health Stroke Scale score (odds ratio, 6.7; 95% confidence interval, 1.4-32.4; P = .02), and time to surgery (odds ratio, 14.6; 95% confidence interval, 2.7-8.5; P = .002) were significantly associated with lack of neurologic improvement. In receiver operating characteristic analysis, National Institutes of Health Stroke Scale score greater than 11 and time to surgery longer than 9.1 hours were best cutoffs for predicting lack of neurologic improvement. Thirty-day mortality was 14.6%. All early deaths were caused by large hemispheric infarction. Postoperative computed tomography or magnetic resonance imaging revealed cerebral infarction in 21 patients (51%). Five-year survival was significantly lower in patients without neurologic improvement (33% ± 12% vs 84% ± 7%, log-rank P lack of improvement, which was associated with poor survival. Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  18. [The nutrition of acute phase in patients with metabolic syndrome].

    Science.gov (United States)

    Tsutsumi, Rie; Sebe, Mayu

    2016-03-01

    In this session, we describe the acute phase in patients with metabolic syndrome from two sides; acute disease that occurs higher in patients with metabolic syndrome such as colonary heart disease and stroke, and acute aggravation of diabetes such as diabetic ketoacidosis and hyperosmolar hyperglycemic syndrome. The electrolyte imbalance is frequently detected in critical ill patients. It is reported that the extreme abnormalities of ionized calcium concentrations are independent predictors of mortality. In addition, from clinical database MIMIC-Ⅱ,calcium supplementation improves clinical outcome in intensive care unit patients. Although metabolic syndrome; lifestyle-related disease, is a chronic disease, the possibility of falling into acute disease by having it becomes very high and improvement of electrolyte imbalance, especially hypocalcaemia is expected to effective on clinical outcome.

  19. Impact of copeptin on diagnosis of acute coronary syndrome

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    Zeinab H. El Sayed

    2014-07-01

    Conclusion: In suspected acute coronary syndrome, determination of copeptin and cardiac troponin I provides a remarkable negative predictive value, which aids in early and safe ruling out of myocardial infarction.

  20. A Case with Repeated Recurrent Acute Coronary Syndrome due to Pseudoephedrine Use: Kounis Syndrome

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    Metin Çeliker

    2014-01-01

    Full Text Available Allergic reaction-associated acute coronary syndrome picture is defined as Kounis syndrome. Although drug use is the most common cause of allergic reaction, foods and environmental factors may also play a role in the etiology. Herein, a case with acute coronary syndrome that developed two times at 8-month interval due to pseudoephedrine use for upper respiratory tract infection is presented.

  1. Persistent Fifth Aortic Arch Associated with 22q11.2 Deletion Syndrome

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    Meng-Luen Lee

    2006-01-01

    Conclusion: Congenital conotruncal malformations, including tetralogy of Fallot with pulmonary atresia or stenosis, and aortic arch anomalies including a persistent fifth aortic arch or a right aortic arch, should lead to suspicion of chromosome 22q11.2 deletion when manifested together with any one of the other four cardinal phenotypic features.

  2. Acute Radiation Syndrome. Consequences and outcomes

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    Okladnikova, N.D.; Pesternikova, V.S.; Sumina, M.V.; Azizova, T.V.; Yurkov, N.N. [Branch No 1 State Research Center of Russia, Ozyorsk (Russian Federation). Inst. of Biophysics

    2000-05-01

    The consequences and outcomes of an Acute Radiation Syndrome (ARS), induced by external gamma radiation for 59 persons (49 men and 10 women) have been estimated. All incidents have taken place more than 40 years ago in the yearly years of adjustment of an atomic industry (1950-1953-38 persons, 1954-1958-21 persons). According to the degree of severity ARS 5 groups are selected: the severest degree - 7 individuals (average dose in group 43.8{+-}12.8 Sv), severe - 4 individuals (9.3{+-}1.5 Sv), medium - 14 individuals (2.2{+-}0.8 Sv), a light degree - 15 individuals (0.93{+-}0.13 Sv), ''erased'' from - 19 individuals (0.85{+-}0.07 Sv). In all cases, except for lethal (the severest degree), the characteristics of morphological composition of the peripheral blood were restored in the first year after ARS and now correspond to physiological standard. In 2 cases the moderate hypoplasia of granulocytopoiesis was diagnosed. A marker of the acute exposure was the chromosome aberrations in lymphocytes of the peripheral blood. The frequency of chromosome aberrations correlates with severity degree of ARS (from 3-7 up to 35-50 stable aberrations per 100 cells). In cases of ARS with severe degree the early development of a cerebral atherosclerosis is detected. The radiation cataract was diagnosed in 5 patients (an exposure doses 4.0-9.8 Sv, a period of development 2-5 years). During the first years after ARS in 80% of cases the complete labour rehabilitation is reached. Of 53 patients with known vital status by 45 year of monitoring 19 persons (35.8%) have died, of these in 2 cases the causes of death are not determined. In remaining cases the causes of death were ARS of severest degree (7 persons), Ischemic Heart Disease (5 persons), malignant tumors (4 persons), accidents and traumas (2 persons). (author)

  3. Nuclear cardiology in acute coronary syndromes

    International Nuclear Information System (INIS)

    Bulow, H.; Schwaiger, M.

    2005-01-01

    Acute coronary syndromes are a frequent manifestation of a coronary artery disease, usually being associated with chest pain and presenting as a medical emergency. Since a considerable number of patients with chest pain, however, have a non cardiac etiology of trier pain, properly triaging these patients represents a diagnostic challenge for physicians in the emergency department. As the available diagnostic procedures have limited accuracy, many different diagnostic strategies have been evaluated. Among these, radionuclide myocardial perfusion imaging (MPI) at rest or in combination with stress procedures has been investigated in many trails. MPI has been proven to be useful, especially in a patient population with a low to intermediate probability of an ischemic event. Perfusion scintigraphy has a high sensitivity in the detection of myocardial infarction and reveals an excellent negative predictive value, allowing a safe discharge strategy of patients with a negative scan result. Moreover, it enables risk stratification and provides incremental and independent prognostic information regarding short to long term future cardiac adverse events. Several cost effectiveness studies have shown that perfusion imaging leads to lower overall direct costs, mainly by a reduction of unnecessary hospital admissions and diagnostic angiograms, without worsening of the clinical outcome of these patients. As a possible study endpoint, myocardial perfusion imaging in the acute setting enables the quantification of salvaged myocardium and therefore the evaluation of treatment efficacy. Besides perfusion agents, several infarcts avid radiopharmaceuticals have been developed, which in part show promising results. However, larger randomized trials evaluating these tracers in clinical settings are needed to warrant routine clinical application

  4. The Recent Severe Acute Respiratory Syndrome (SARS) Epidemic ...

    African Journals Online (AJOL)

    Severe Acute Respiratory syndrome (SARS) took the world by storm in the later part of February 2003.It is a syndrome characterized by fever, cough, sore throat , shortness of breath and malaise which may deteriorate very rapidly to respiratory failure and death. The symptoms of SARS are quite similar to those of common ...

  5. acute compartment syndrome of the thigh without associated fracture

    African Journals Online (AJOL)

    exercise and contact sport like rugby and soccer (7-11). Other cases were in association with arterial injury (4,. 5, 12). The diagnosis of acute compartment syndrome of the thigh requires a high index of suspicion as it is not a common complication of thigh injury (5). Like compartment syndrome elsewhere, thigh compartment.

  6. Bentall operation, total aortic replacement and mitral valve replacement for a young adult with Marfan syndrome: a case of three-staged operation.

    Science.gov (United States)

    Inui, K; Shimazaki, Y; Watanabe, T; Kuraoka, S; Minowa, T; Miura, M; Oshikiri, S; Toyama, H

    1998-08-01

    In Marfan syndrome, the most common cardiovascular abnormalities are dilatation of the aorta and aortic valve regurgitation in adult patients. Mitral valve dysfunction is the most common cause of morbidity and mortality in infants and children with Marfan syndrome, and is not frequently operated on in adult Marfan patients who undergo surgery for diseases of the aortic root and total aorta. This report describes a successfully three-staged operation for a 24 year-old man with Marfan syndrome who underwent an emergent Bentall operation and aortic arch replacement, total aortic replacement and mitral valve replacement over 2 years. Mitral valve regurgitation was mild but increased after the second operation. The graft was tightly adhesive and invasive to the sternum. Endoscopic view was helpful to avoid graft damage at resternotomy. The postoperative course was uneventful in each operation. Microscopic examination of the mitral valve leaflets showed abnormal increase of mucopolysaccharides, and disruption and fragmentation of elastic fibers.

  7. The non-syndromic familial thoracic aortic aneurysms and dissections maps to 15q21 locus

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    Chandok Gurangad

    2010-10-01

    Full Text Available Abstract Background Thoracic aortic aneurysms and dissections (TAAD is a critical condition that often goes undiagnosed with fatal consequences. While majority of the cases are sporadic, more than 20% are inherited as a single gene disorder. The most common familial TAA is Marfan syndrome (MFS, which is primarily caused by mutations in fibrillin-1 (FBN1 gene. Patients with FBN1 mutations are at higher risk for dissection compared to other patients with similar size aneurysms. Methods Fifteen family members were genotyped using Affymetrix-10K genechips. A genome-wide association study was carried out using an autosomal dominant model of inheritance with incomplete penetrance. Mutation screening of all exons and exon-intron boundaries of FBN1 gene which reside near the peak Lod score was carried out by direct sequencing. Results The index case presented with agonizing substernal pain and was found to have TAAD by transthoracic echocardiogram. The family history was significant for 3 first degree relatives with TAA. Nine additional family members were diagnosed with TAA by echocardiography examinations. The affected individuals had no syndromic features. A genome-wide analysis of linkage mapped the disease gene to a single locus on chromosome 15q21 with a peak Lod score of 3.6 at fibrillin-1 (FBN1 gene locus (odds ratio > 4000:1 in favour of linkage, strongly suggesting that FBN1 is the causative gene. No mutation was identified within the exons and exon-intron boundaries of FBN1 gene that segregated with the disease. Haplotype analysis identified additional mutation carriers who had previously unknown status due to borderline dilation of the ascending aorta. Conclusions A familial non-syndromic TAAD is strongly associated with the FBN1 gene locus and has a malignant disease course often seen in MFS patients. This finding indicates the importance of obtaining detailed family history and echocardiographic screening of extended relatives of patients

  8. MRI assessment of local acute radiation syndrome

    International Nuclear Information System (INIS)

    Weber-Donat, G.; Potet, J.; Baccialone, J.; Teriitehau, C.; Amabile, J.C.; Laroche, P.; Lahutte-Auboin, M.; Bey, E.

    2012-01-01

    To describe local acute radiation syndrome and its radiological imaging characteristics. We performed a retrospective study of patients who had suffered skin and deeper radiation damage who were investigated by magnetic resonance imaging (MRI). We compared the clinical findings, C-reactive protein (CRP) levels and MRI results. A total of 22 MRI examinations were performed between 2005 and 2010 in 7 patients; 6 patients had increased CRP levels and MRI abnormalities. They were treated by surgery and local cellular therapy. One patient had no CRP or MRI abnormalities, and had a spontaneous good outcome. Eighteen abnormal MR examinations demonstrated high STIR signal and/or abnormal enhancement in the dermis and muscle tissues. Three MRI examinations demonstrated skeletal abnormalities, consistent with radionecrosis. The four normal MRI examinations were associated only with minor clinical manifestations such as pain and pigmentation disorders. MRI seems to be a useful and promising imaging investigation in radiation burns management i.e. initial lesion evaluation, treatment evaluation and complication diagnosis. MRI findings correlated perfectly with clinical stage and no false negative examinations were obtained. In particular, the association between normal MRI and low CRP level seems to be related to good outcome without specific treatment. (orig.)

  9. Novel oral anticoagulants in acute coronary syndrome.

    Science.gov (United States)

    Costopoulos, Charis; Niespialowska-Steuden, Maria; Kukreja, Neville; Gorog, Diana A

    2013-09-10

    Coronary artery disease (CAD) is a leading cause of morbidity and mortality worldwide with a prevalence that has now reached pandemic levels as a consequence of the rapid modernization of the developing world. Its presentation as an acute coronary syndrome (ACS) is a frequent reason for hospital admission and of profound implications for personal, societal and global health. Despite improvements in the management of ACS with anti-platelet and anticoagulant therapy and revascularization techniques, many patients continue to suffer recurrent ischemic events. The need to reduce future cardiovascular events has led to the development of novel therapies to prevent coronary thrombosis, targeting thrombin-mediated pathways. These include direct Xa inhibitors (apixaban, rivaroxaban and darexaban), direct thrombin inhibitors (dabigatran) and PAR 1 antagonists (vorapaxar and atopaxar). This article critically reviews the comparative mechanisms of action, the risks and benefits, together with the clinical evidence base for the use of these novel oral agents in the management of ACS patients. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  10. MRI assessment of local acute radiation syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Weber-Donat, G.; Potet, J.; Baccialone, J.; Teriitehau, C. [Military Hospital Percy, Radiology Department, Clamart (France); Amabile, J.C.; Laroche, P. [Military Hospital Percy, Army Institute of Radioprotection, Clamart (France); Lahutte-Auboin, M. [Military Hospital Val-de-Grace, Radiology Department, Paris (France); Bey, E. [Military Hospital Percy, Plastic and Reconstructive Surgery Department, Clamart (France)

    2012-12-15

    To describe local acute radiation syndrome and its radiological imaging characteristics. We performed a retrospective study of patients who had suffered skin and deeper radiation damage who were investigated by magnetic resonance imaging (MRI). We compared the clinical findings, C-reactive protein (CRP) levels and MRI results. A total of 22 MRI examinations were performed between 2005 and 2010 in 7 patients; 6 patients had increased CRP levels and MRI abnormalities. They were treated by surgery and local cellular therapy. One patient had no CRP or MRI abnormalities, and had a spontaneous good outcome. Eighteen abnormal MR examinations demonstrated high STIR signal and/or abnormal enhancement in the dermis and muscle tissues. Three MRI examinations demonstrated skeletal abnormalities, consistent with radionecrosis. The four normal MRI examinations were associated only with minor clinical manifestations such as pain and pigmentation disorders. MRI seems to be a useful and promising imaging investigation in radiation burns management i.e. initial lesion evaluation, treatment evaluation and complication diagnosis. MRI findings correlated perfectly with clinical stage and no false negative examinations were obtained. In particular, the association between normal MRI and low CRP level seems to be related to good outcome without specific treatment. (orig.)

  11. Chilaiditi's syndrome as an acute abdomen | Jambo | Port Harcourt ...

    African Journals Online (AJOL)

    The interposed loop of bowel reduced spontaneously following conservative management. Conclusion: As Chilaiditi syndrome is a rare disease entity, a high index of suspicion is required to make an accurate diagnosis. It should be considered as one of the differential diagnoses of an acute abdomen. Keywords: Acute ...

  12. Acute coronary syndromes amongst type 2 diabetics with ischaemic ...

    African Journals Online (AJOL)

    Majority had three coronary artery disease (CAD) risk factors: obesity 86%, elevated LDL 73% and hypertension 60%. Therapy in use was OHA 43%, insulin 42%, insulin and OHA 1%; prophylactic aspirin 14.7% and statins 8.4%. Thirty four (35.8%) were classified as acute coronary syndrome (ACS); 29 ( 30.5%) acute ...

  13. Impact of copeptin on diagnosis of acute coronary syndrome

    African Journals Online (AJOL)

    Zeinab H. El Sayed

    2014-03-17

    Mar 17, 2014 ... Abstract Background: Acute coronary syndrome remains the principal cause of death, so the early diagnosis is of great importance. Cardiac troponin is the preferred biomarker for acute myo- cardial infarction. Cardiac chest pain immediately increased copeptin secretion. The combination of copeptin and ...

  14. Impact of copeptin on diagnosis of acute coronary syndrome | El ...

    African Journals Online (AJOL)

    Background: Acute coronary syndrome remains the principal cause of death, so the early diagnosis is of great importance. Cardiac troponin is the preferred biomarker for acute myocardial infarction. Cardiac chest pain immediately increased copeptin secretion. The combination of copeptin and cardiac troponin I is being ...

  15. German Registry for Acute Aortic Dissection Type A (GERAADA)--new software design, parameters and their definitions.

    Science.gov (United States)

    Weigang, E; Görgen, C; Kallenbach, K; Dapunt, O; Karck, M

    2011-03-01

    The working group for aortic surgery and interventional vascular surgery of the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) initiated the web-based German Registry for Acute Aortic Dissection type A (GERAADA). It is the project's aim to collect standardized data from a large pool of patients with acute aortic dissections type A (AADA) to gain a deeper insight and knowledge to improve surgical therapies and perioperative management for these patients in the future. In addition to new medical insights, the working group has gained more experience over the last 4 years in how to collect valid and high-quality data. This experience led us to revise the database completely. In this article we describe the new version of GERAADA, providing an overview as well as defining the parameters, and explaining the new features. This overview fulfills a request by the users of GERAADA in the participating centers. Since its inception, 50 cardiac centers in Germany, Austria and Switzerland have provided over 2000 records and the first statistical results have been published. GERAADA's new design allows it to stay abreast of changes in medicine and to focus on the essentials necessary for statistically relevant results, while keeping the work load low for the data providers at each cardiac center. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy.

    Science.gov (United States)

    Lacro, Ronald V; Guey, Lin T; Dietz, Harry C; Pearson, Gail D; Yetman, Anji T; Gelb, Bruce D; Loeys, Bart L; Benson, D Woodrow; Bradley, Timothy J; De Backer, Julie; Forbus, Geoffrey A; Klein, Gloria L; Lai, Wyman W; Levine, Jami C; Lewin, Mark B; Markham, Larry W; Paridon, Stephen M; Pierpont, Mary Ella; Radojewski, Elizabeth; Selamet Tierney, Elif Seda; Sharkey, Angela M; Wechsler, Stephanie Burns; Mahony, Lynn

    2013-05-01

    The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area-adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome. Copyright © 2013 Mosby, Inc. All rights reserved.

  17. Redox stress in Marfan syndrome: Dissecting the role of the NADPH oxidase NOX4 in aortic aneurysm.

    Science.gov (United States)

    Jiménez-Altayó, Francesc; Meirelles, Thayna; Crosas-Molist, Eva; Sorolla, M Alba; Del Blanco, Darya Gorbenko; López-Luque, Judit; Mas-Stachurska, Aleksandra; Siegert, Ana-Maria; Bonorino, Fabio; Barberà, Laura; García, Carolina; Condom, Enric; Sitges, Marta; Rodríguez-Pascual, Fernando; Laurindo, Francisco; Schröder, Katrin; Ros, Joaquim; Fabregat, Isabel; Egea, Gustavo

    2018-04-01

    Marfan syndrome (MFS) is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix fibrillin-containing microfibrils and dysfunction of TGF-β signaling. Here we identify the molecular targets of redox stress in aortic aneurysms from MFS patients, and investigate the role of NOX4, whose expression is strongly induced by TGF-β, in aneurysm formation and progression in a murine model of MFS. Working models included aortae and cultured vascular smooth muscle cells (VSMC) from MFS patients, and a NOX4-deficient Marfan mouse model (Fbn1 C1039G/+ -Nox4 -/- ). Increased tyrosine nitration and reactive oxygen species levels were found in the tunica media of human aortic aneurysms and in cultured VSMC. Proteomic analysis identified nitrated and carbonylated proteins, which included smooth muscle α-actin (αSMA) and annexin A2. NOX4 immunostaining increased in the tunica media of human Marfan aorta and was transcriptionally overexpressed in VSMC. Fbn1 C1039G/+ -Nox4 -/- mice aortas showed a reduction of fragmented elastic fibers, which was accompanied by an amelioration in the Marfan-associated enlargement of the aortic root. Increase in the contractile phenotype marker calponin in the tunica media of MFS mice aortas was abrogated in Fbn1 C1039G/+ -Nox4 -/- mice. Endothelial dysfunction evaluated by myography in the Marfan ascending aorta was prevented by the absence of Nox4 or catalase-induced H 2 O 2 decomposition. We conclude that redox stress occurs in MFS, whose targets are actin-based cytoskeleton members and regulators of extracellular matrix homeostasis. Likewise, NOX4 have an impact in the progression of the aortic dilation in MFS and in the structural organization of the aortic tunica media, the VSMC phenotypic modulation, and endothelial function. Copyright © 2018 Elsevier Inc. All rights reserved.

  18. Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: a prospective, multicenter, comparative study.

    Science.gov (United States)

    Coselli, Joseph S; Volguina, Irina V; LeMaire, Scott A; Sundt, Thoralf M; Connolly, Heidi M; Stephens, Elizabeth H; Schaff, Hartzell V; Milewicz, Dianna M; Vricella, Luca A; Dietz, Harry C; Minard, Charles G; Miller, D Craig

    2014-06-01

    To compare the 1-year results after aortic valve-sparing (AVS) or valve-replacing (AVR) aortic root replacement from a prospective, international registry of 316 patients with Marfan syndrome (MFS). Patients underwent AVS (n = 239, 76%) or AVR (n = 77, 24%) aortic root replacement at 19 participating centers from 2005 to 2010. One-year follow-up data were complete for 312 patients (99%), with imaging findings available for 293 (94%). The time-to-events were compared between groups using Kaplan-Meier curves and Cox proportional hazards models. Two patients (0.6%)--1 in each group--died within 30 days. No significant differences were found in early major adverse valve-related events (MAVRE; P = .6). Two AVS patients required early reoperation for coronary artery complications. The 1-year survival rates were similar in the AVR (97%) and AVS (98%) groups; the procedure type was not significantly associated with any valve-related events. At 1 year and beyond, aortic regurgitation of at least moderate severity (≥2+) was present in 16 patients in the AVS group (7%) but in no patients in the AVR group (P = .02). One AVS patient required late AVR. AVS aortic root replacement was not associated with greater 30-day mortality or morbidity rates than AVR root replacement. At 1 year, no differences were found in survival, valve-related morbidity, or MAVRE between the AVS and AVR groups. Of concern, 7% of AVS patients developed grade ≥2+ aortic regurgitation, emphasizing the importance of 5 to 10 years of follow-up to learn the long-term durability of AVS versus AVR root replacement in patients with MFS. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  19. Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with Marfan syndrome.

    Science.gov (United States)

    Ma, Wei-Guo; Zhang, Wei; Zhu, Jun-Ming; Ziganshin, Bulat A; Zhi, Ai-Hua; Zheng, Jun; Liu, Yong-Min; Elefteriades, John A; Sun, Li-Zhong

    2017-10-01

    The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FET and total arch replacement (TAR) for TAAD in patients with MFS. The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 ± 9.7 years) undergoing FET + TAR for TAAD. Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0% (96 of 99), averaging 6.3 ± 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046). The frozen elephant trunk and total arch replacement procedure can be safely performed

  20. A successfully thrombolysed acute inferior myocardial infarction due to type A aortic dissection with lethal consequences: the importance of early cardiac echocardiography

    Directory of Open Access Journals (Sweden)

    Bousoula Eleni

    2011-08-01

    Full Text Available Abstract Thrombolysis, a standard therapy for ST elevation myocardial infarction (STEMI in non-PCI-capable hospitals, may be catastrophic for patients with aortic dissection leading to further expansion, rupture and uncontrolled bleeding. Stanford type A aortic dissection, rarely may mimic myocardial infarction. We report a case of a patient with an inferior STEMI thrombolysed with tenecteplase and followed by clinical and electrocardiographic evidence of successful reperfusion, which was found later to be a lethal acute aortic dissection. Prognostic implications of early diagnosis applying transthoracic echocardiography (TTE are described.

  1. Ortner’s syndrome: Cardiovocal syndrome caused by aortic arch ps

    Directory of Open Access Journals (Sweden)

    Adil H. Al Kindi

    2016-10-01

    Ortner’s syndrome describes vocal changes caused by cardiovascular pathology. It should be included in the differential diagnosis of patients with cardiovascular risk factors presenting with hoarseness. This case demonstrates the use of endovascular stents to treat the causative pathology with resolution of symptoms. In expert hands, it represents low risk, minimally invasive therapeutic strategy with excellent early results in patients who are high risk for open procedure.

  2. CT of suspected thoracic acute aortic injury in the emergency department: is routine abdominopelvic imaging worth the additional collective radiation dose?

    Science.gov (United States)

    Haji-Momenian, Shawn; Rischall, Jonathan; Okey, Neil; Taffel, Myles; Khati, Nadia; Zeman, Robert

    2017-02-01

    This study aimed to determine the incidence of non-traumatic acute aortic injury (AAI) extending from the chest into the abdomen or pelvis in emergency department (ED) patients with acute aortic syndrome (AAS), to estimate the effective dose of the abdominopelvic portion of these CT exams, and to compare the number needed to screen (NNS) with the collective population radiation dose of imaging those stations. All patients (n = 238) presenting to the ED with AAS between March 2014 and June 2015 who were imaged per CT AAI protocol (noncontrast and contrast-enhanced CT angiography of the chest, abdomen, and pelvis) were retrospectively identified in this IRB-approved HIPAA-compliant study. The Stanford classification for positive cases of AAI was further subclassified based on chest, abdominal, or pelvic involvement. The dose length product (DLP) of each exam was used to estimate the dose of the abdominal and pelvic stations and the collective effective dose for the population. There were five cases of aortic dissection (AD) and two of intramural hematoma (IMH), with an AAI incidence of 2.9/100. Three cases of AAI were confined to the chest. Two cases of AAI were confined to the chest and abdomen, and two cases involved the chest, abdomen, and pelvis. There was only one case of AAI involving the ascending aorta that extended into the abdomen or pelvis. The number needed to screen to identify (a) AAI extending from the chest into the abdomen or pelvis was 59.5 and (b) Stanford A AAI extending into the abdomen or pelvis was 238. The estimated mean effective dose for the abdominopelvic stations were unenhanced abdomen 2.3 mSv, unenhanced pelvis 3.3 mSv, abdominal CTA 2.5 mSv, and pelvic CTA 3.6 mSv. The collective effective doses to the abdomen and pelvis with unenhanced CT and CTA in 59.5 patients and 238 patients were 761.6 and 3046.4 mSv, respectively. While the estimated mean effective dose for imaging of the abdominopelvic stations are low, the collective

  3. Decision-making in aortic root surgery in Marfan syndrome: bleeding, thromboembolism and risk of reintervention after valve-sparing or mechanical aortic root replacement.

    Science.gov (United States)

    Schoenhoff, Florian S; Langhammer, Bettina; Wustmann, Kerstin; Reineke, David; Kadner, Alexander; Carrel, Thierry

    2015-12-01

    Valve-sparing root replacement (VSRR) is thought to reduce the rate of thromboembolic and bleeding events compared with aortic root replacement using a mechanical aortic root replacement (MRR) with a composite graft by avoiding oral anticoagulation. But as VSRR carries a certain risk for subsequent reinterventions, decision-making in the individual patient can be challenging. Of 100 Marfan syndrome (MFS) patients who underwent 169 aortic surgeries and were followed at our institution since 1995, 59 consecutive patients without a history of dissection or prior aortic surgery underwent elective VSRR or MRR and were retrospectively analysed. VSRR was performed in 29 (David n = 24, Yacoub n = 5) and MRR in 30 patients. The mean age was 33 ± 15 years. The mean follow-up after VSRR was 6.5 ± 4 years (180 patient-years) compared with 8.8 ± 9 years (274 patient-years) after MRR. Reoperation rates after root remodelling (Yacoub) were significantly higher than after the reimplantation (David) procedure (60 vs 4.2%, P = 0.01). The need for reinterventions after the reimplantation procedure (0.8% per patient-year) was not significantly higher than after MRR (P = 0.44) but follow-up after VSRR was significantly shorter (P = 0.03). There was neither significant morbidity nor mortality associated with root reoperations. There were no neurological events after VSRR compared with four stroke/intracranial bleeding events in the MRR group (log-rank, P = 0.11), translating into an event rate of 1.46% per patient-year following MRR. The calculated annual failure rate after VSRR using the reimplantation technique was lower than the annual risk for thromboembolic or bleeding events. Since the perioperative risk of reinterventions following VSRR is low, patients might benefit from VSRR even if redo surgery may become necessary during follow-up. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  4. Transesophageal echocardiographic evaluation of an intraoperative retrograde acute aortic dissection: case report

    Directory of Open Access Journals (Sweden)

    Reiter Charles G

    2006-04-01

    Full Text Available Abstract Background We report an intraoperative retrograde dissection of the aorta and its subsequent evaluation by transesophageal echocardiography (TEE. Case presentation A 78 year old woman with an ascending aortic aneurysm without dissection and coronary artery disease was brought to the operating room for aneurysm repair and coronary artery bypass grafting. After initiation of cardiopulmonary bypass through a femoral artery cannula, aortic dissection was noted and subsequently imaged by TEE. Conclusion Retrograde aortic dissection through the femoral artery is life-threatening. Intraoperative TEE can be used to diagnose this uncommon event, and should be considered after initiation of bypass.

  5. Systemic inflammation, coagulopathy, and acute renal insufficiency following endovascular thoracoabdominal aortic aneurysm repair.

    Science.gov (United States)

    Chang, Catherine K; Chuter, Timothy A M; Niemann, Claus U; Shlipak, Michael G; Cohen, Mitchell J; Reilly, Linda M; Hiramoto, Jade S

    2009-05-01

    To characterize the inflammatory and coagulopathic response after endovascular thoracoabdominal aortic aneurysm (TAAA) repair and to evaluate the effect of the response on postoperative renal function. From July 2005 to June 2008, 42 patients underwent elective endovascular repair of a TAAA using custom designed multi-branched stent-grafts at a single academic institution. Four patients were excluded from the analysis. White blood cell count (WBC), platelet count, prothrombin time (PT), and creatinine were measured in all patients. In the last nine patients, interleukin-6 (IL-6), protein C, Factor V, d-dimers, cystatin C, and neutrophil gelatinase-associated lipocalin (NGAL) levels were also measured. Change in lab values were expressed as a percentage of baseline values. The 30-day mortality rate was 5% (2/38). All patients (n = 38) had a higher WBC (mean +/- SD: 139 +/- 80%, P acute renal insufficiency (>50% rise in creatinine). Patients with renal insufficiency had significantly larger changes in WBC (178 +/- 100% vs 121 +/- 64%, P = .04) and platelet count (64 +/- 17% vs 52 +/- 12%, P = .02) compared with those without renal insufficiency. All patients (n = 9) had significant increases in NGAL (182 +/- 115%, P = .008) after stent-graft insertion. Six of nine patients (67%) had increased cystatin C (35 +/- 43%, P = .04) after stent-graft insertion, with a greater rise in those with postoperative renal insufficiency (87 +/- 32% vs 8 +/- 13%, P = .02). IL-6 levels were markedly increased in all patients (n = 9) after repair (9840 +/- 6160%, P = .008). Protein C (35 +/- 10%, P = .008) and Factor V levels (28 +/- 20%, P = .008) were uniformly decreased, while d-dimers were elevated after repair in all patients (310 +/- 213%, P = .008). Leukocytosis and thrombocytopenia were uniform following endovascular TAAA repair, and the severity of the response correlated with post-operative renal dysfunction. Elevation of a sensitive marker of renal injury (NGAL) suggests that

  6. Molecular genetic analysis of individuals with Williams syndrome and supravalvar aortic stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Smoot, L.B.; Lacro, R.V.; Kunkel, L.M. [Children`s Hospital, Boston, MA (United States); Pober, B. [Yale Univ., New Haven, CT (United States)

    1994-09-01

    Mutations at the elastin locus (chromosome 7q11.23) have been demonstrated in individuals with Williams syndrome (WS) and familial supravalvar aortic stenosis (SVAS). Relationships between elastin mutations and vascular and/or neurodevelopmental pathology have yet to be defined. In determining phenotype-genotype correlations in WS/SVAS, we examined 35 individuals with sporadic WS, families with SVAS affecting multiple members, and sporadic cases of isolated obstructive vascular disease. Full length elastin cDNA was used to probe a human genomic library from which multiple elastin genomic clones have been isolated and ordered relative to the elastin gene, covering a minimum of 35 kb. (Additional genomic clones are being obtained by {open_quote}walking{close_quote} 5{prime} and 3{prime} to elastin.) Elastin genomic clones were used as probes in fluorescent in situ hybridization of metaphase chromosomes from WS/SVAS patients. Preliminary analysis confirms elastin deletions in WS patients, but have not yet been demonstrated in patients with isolated vascular disease using this technique. Results of deletional analysis in individuals representing a wide spectrum of phenotypes will be presented.

  7. Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.

    Science.gov (United States)

    Laraja, Kristin; Sadhwani, Anjali; Tworetzky, Wayne; Marshall, Audrey C; Gauvreau, Kimberlee; Freud, Lindsay; Hass, Cara; Dunbar-Masterson, Carolyn; Ware, Janice; Lafranchi, Terra; Wilkins-Haug, Louise; Newburger, Jane W

    2017-05-01

    To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Anesthetic Implications of Emergent Cesarean Section in a Parturient with Marfan Syndrome Complicated by Ascending Aortic Aneurysm and Heart Failure

    Directory of Open Access Journals (Sweden)

    Young Sung Kim

    2014-07-01

    Full Text Available Cardiovascular comorbidities to the Marfan syndrome may induce hemodynamic instability especially in the parturients during labor or delivery. For anesthesiologists, it is challenging to maintain hemodynamic stability during Cesarean section in those patients with Marfan syndrome. Remifentanil is an ultra-short-acting opioid with rapid onset and offset of action which provides cardiovascular stability during surgery. Together with remifentanil, the use of a laryngeal mask airway can reduce the risk of hypertensive response followed by tracheal intubation. We describe the successful administration of remifentanil and application of laryngeal mask airway for emergent Cesarean section performed under general anesthesia in a patient with Marfan syndrome complicated by ascending aortic aneurysm and heart failure. The use of remifentanil (loading dose of 1 μg/kg for 1 min, 2 min before induction; thereafter continuous infusion dose of 0.1 μg/kg/min was useful to maintain hemodynamic stability of the parturient throughout the surgery without neonatal respiratory depression. Keywords: Ascending Aortic Aneurysm; Cesarean section; Laryngeal mask airway; Marfan syndrome; Remifentanil

  9. Enhanced caspase activity contributes to aortic wall remodeling and early aneurysm development in a murine model of Marfan syndrome.

    Science.gov (United States)

    Emrich, Fabian C; Okamura, Homare; Dalal, Alex R; Penov, Kiril; Merk, Denis R; Raaz, Uwe; Hennigs, Jan K; Chin, Jocelyn T; Miller, Miquell O; Pedroza, Albert J; Craig, Juliana K; Koyano, Tiffany K; Blankenberg, Francis G; Connolly, Andrew J; Mohr, Friedrich W; Alvira, Cristina M; Rabinovitch, Marlene; Fischbein, Michael P

    2015-01-01

    Rupture and dissection of aortic root aneurysms remain the leading causes of death in patients with the Marfan syndrome, a hereditary connective tissue disorder that affects 1 in 5000 individuals worldwide. In the present study, we use a Marfan mouse model (Fbn1(C1039G/+)) to investigate the biological importance of apoptosis during aneurysm development in Marfan syndrome. Using in vivo single-photon emission computed tomographic-imaging and ex vivo autoradiography for Tc99m-annexin, we discovered increased apoptosis in the Fbn1(C1039G/+) ascending aorta during early aneurysm development peaking at 4 weeks. Immunofluorescence colocalization studies identified smooth muscle cells (SMCs) as the apoptotic cell population. As biological proof of concept that early aortic wall apoptosis plays a role in aneurysm development in Marfan syndrome, Fbn1(C1039G/+) mice were treated daily from 2 to 6 weeks with either (1) a pan-caspase inhibitor, Q-VD-OPh (20 mg/kg), or (2) vehicle control intraperitoneally. Q-VD-OPh treatment led to a significant reduction in aneurysm size and decreased extracellular matrix degradation in the aortic wall compared with control mice. In vitro studies using Fbn1(C1039G/+) ascending SMCs showed that apoptotic SMCs have increased elastolytic potential compared with viable cells, mostly because of caspase activity. Moreover, in vitro (1) cell membrane isolation, (2) immunofluorescence staining, and (3) scanning electron microscopy studies illustrate that caspases are expressed on the exterior cell surface of apoptotic SMCs. Caspase inhibition attenuates aneurysm development in an Fbn1(C1039G/+) Marfan mouse model. Mechanistically, during apoptosis, caspases are expressed on the cell surface of SMCs and likely contribute to elastin degradation and aneurysm development in Marfan syndrome. © 2014 American Heart Association, Inc.

  10. The clinical aspects of the acute facet syndrome

    DEFF Research Database (Denmark)

    Hestbaek, Lise; Kongsted, Alice; Jensen, Tue Secher

    2009-01-01

    to agree on the number, frequency and duration of chiropractic treatment. RESULTS: Thirty-four chiropractors from nine European countries participated. They described the characteristics of an acute, uncomplicated facet syndrome as follows: local, ipsilateral pain, occasionally extending into the thigh...... that there would be no signs of neurologic involvement or antalgic posture and no aggravation of pain from sitting, flexion or coughing/sneezing. CONCLUSION: The chiropractors attending the workshop described the characteristics of an acute, uncomplicated lumbar facet syndrome in much the same way as chronic pain......ABSTRACT: BACKGROUND: The term 'acute facet syndrome' is widely used and accepted amongst chiropractors, but poorly described in the literature, as most of the present literature relates to chronic facet joint pain. Therefore, research into the degree of consensus on the subject amongst a large...

  11. Acute renal failure likely due to acute nephritic syndrome associated with typhoid fever.

    Science.gov (United States)

    Hayashi, Manabu; Kouzu, Hideshi; Nishihara, Masahiro; Takahashi, Tohru; Furuhashi, Masato; Sakamoto, Ken-ichi; Satoh, Naotoshi; Nishitani, Takahiro; Shikano, Yasukuni

    2005-10-01

    A 45-year-old woman was admitted to our hospital because of high fever and malaise. She also presented abnormal urine findings including macrohematuria and proteinuria with transient renal insufficiency. Salmonella Typhi was isolated from her blood and stool culture, and then she was diagnosed as having typhoid fever. Salmonella enterica serotype typhi infection can be complicated by renal involvement, although rare. In Japan, few cases have been reported about acute nephritic syndrome in typhoid fever. Here, we report a case of endemic acquired typhoid fever associated with acute renal failure probably due to acute nephritic syndrome successfully treated with levofloxacin.

  12. Tachyarrhythmias, bradyarrhythmias and acute coronary syndromes

    Directory of Open Access Journals (Sweden)

    Trappe Hans-Joachim

    2010-01-01

    Full Text Available The incidence of bradyarrhythmias in patients with acute coronary syndrome (ACS is 0.3% to 18%. It is caused by sinus node dysfunction (SND, high-degree atrioventricular (AV block, or bundle branch blocks. SND presents as sinus bradycardia or sinus arrest. First-degree AV block occurs in 4% to 13% of patients with ACS and is caused by rhythm disturbances in the atrium, AV node, bundle of His, or the Tawara system. First- or second-degree AV block is seen very frequently within 24 h of the beginning of ACS; these arrhythmias are frequently transient and usually disappear after 72 h. Third-degree AV blocks are also frequently transient in patients with infero-posterior myocardial infarction (MI and permanent in anterior MI patients. Left anterior fascicular block occurs in 5% of ACS; left posterior fascicular block is observed less frequently (incidence < 0.5%. Complete bundle branch block is present in 10% to 15% of ACS patients; right bundle branch block is more common (2/3 than left bundle branch block (1/3. In patients with bradyarrhythmia, intravenous (IV atropine (1-3 mg is helpful in 70% to 80% of ACS patients and will lead to an increased heart rate. The need for pacemaker stimulation (PS is different in patients with inferior MI (IMI and anterior MI (AMI. Whereas bradyarrhythmias are frequently transient in patients with IMI and therefore do not need permanent PS, there is usually a need for permanent PS in patients with AMI. In these patients bradyarrhythmias are mainly caused by septal necrosis. In patients with ACS and ventricular arrhythmias (VTA amiodarone is the drug of choice; this drug is highly effective even in patients with defibrillation-resistant out-of-hospital cardiac arrest. There is general agreement that defibrillation and advanced life support is essential and is the treatment of choice for patients with ventricular flutter/fibrillation. If defibrillation is not available in patients with cardiac arrest due to VTA

  13. Pediatric acute respiratory distress syndrome: Host factors in Down syndrome and the general population

    NARCIS (Netherlands)

    Bruijn, M.

    2013-01-01

    We find that Down syndrome is an important risk factor for developing acute respiratory distress syndrome (ARDS) in children, but the reason why remains to be elucidated. In addition, we find several differences between adult and pediatric ARDS. The association between C-reactive protein (CRP)

  14. Cleaved caspase-3 in lung epithelium of children who died with acute respiratory distress syndrome

    NARCIS (Netherlands)

    Bem, Reinout A.; van der Loos, Chris M.; van Woensel, Job B. M.; Bos, Albert P.

    2010-01-01

    OBJECTIVE: To investigate the extent of cleaved caspase-3 immunostaining in lung epithelial cells in children with acute respiratory distress syndrome. DESIGN: Observational study in sixteen children who died with acute respiratory distress syndrome and diffuse alveolar damage. SETTING: Pediatric

  15. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study

    DEFF Research Database (Denmark)

    Cleemann, Line; Mortensen, Kristian H; Holm, Kirsten

    2010-01-01

    subjects. The mean age of the patients was 17 +/- 3.3 years. Magnetic resonance imaging was performed for all the patients. The thoracic aortic diameters of the patients were measured at nine positions. Adjustment for body surface area (BSA) was performed. The outcome for the patients was measured in terms...... of absolute and BSA-adjusted aortic dilation. In TS, both the absolute and the BSA-adjusted mean aortic diameters were smaller than or comparable with those of the control subjects. However, individual aortic dilation at one to four positions was found in four TS patients according to the uncorrected data...... and in five TS patients after BSA-adjustment. The aortic diameters correlated with height, weight, body mass index (BMI), and BSA at all positions (R = 0.34-0.60; all p

  16. Early Referral to Coronary Artery Bypass Grafting Following Acute Coronary Syndrome, Trends and Outcomes from the Acute Coronary Syndrome Israeli Survey (ACSIS) 2000-2010.

    Science.gov (United States)

    Klempfner, Robert; Barac, Yaron D; Younis, Arwa; Kopel, Eran; Younis, Anan; Ronen, Goldkorn; Maor, Elad; Arbel, Yaron; Rott, David; Goldenberg, Ilan; Aravot, Dan

    2018-02-01

    Limited information exists on whether changes in medical practices over the study decades have affected the outcomes of acute coronary syndrome (ACS) patients who undergo early coronary artery bypass surgery (CABG) during index hospitalisation. Data on trends for early CABG referral and associated outcomes were obtained among 11,485 ACS patients enrolled in the biennial Acute Coronary Syndrome Israeli Surveys (ACSIS) 2000-2010. Among 11,485 patients, 566 (5%) were referred to early CABG. These patients displayed higher risk characteristics, including Killip class >II, anterior myocardial infarction, greater left ventricular dysfunction, and more frequent use of mechanical ventilation and intra-aortic balloon pump (all ptrend over the study decade (6.7% - 1.7%; p<0.001). One year survival was similar between patients referred to early CABG during the late (years: 2006-2010) vs. early (years: 2000-2005) period (85.7% vs. 90%; log-rank p-value=0.15), whereas, among patients who didn't undergo early CABG, and underwent percutaneous coronary intervention (PCI) or medical management only, enrolment during the late periods was associated with a significant survival benefit (91.5% vs. 88.1%; log-rank p-value<0.001). Over the study decade there was a significant decline in referral for early CABG, without a difference in the one-year mortality between the early and non-early CABG group. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  17. Acute Pulmonary Embolism Mimics Acute Coronary Syndrome in Older Patient

    Directory of Open Access Journals (Sweden)

    Chun-Chieh Liu

    2009-12-01

    Full Text Available Acute pulmonary embolism is a fatal disease and an often missed diagnosis. There are no specific symptoms or signs. Accurate diagnosis followed by effective therapy can reduce mortality. We report on a 67-year-old man who underwent lumbar laminectomy and developed an acute anterior compressive-like chest pain and jaw numbness rather than dyspnea on the fifth postoperative day. Owing to refractory chest pain with suspicious posterior myocardial infarction or unstable angina on surface electrocardiogram, the patient received emergency coronary catheterization, which demonstrated normal coronary arteries. Further investigation provided a final diagnosis of acute pulmonary embolism. Acute pulmonary embolism with simultaneous recent neuro-surgery was a therapeutic dilemma because of the risk of postoperative hemorrhage threatening neurologic function. After treatment with enoxaparin and close monitoring of his neurologic condition, his symptoms were eliminated. Clinicians must keep in mind a differential diagnosis of pulmonary embolism in a postoperative high-risk patient.

  18. Idiopathic Atypical Haemolytic Uraemic Syndrome presenting with acute dystonia

    LENUS (Irish Health Repository)

    Maduemem, Rizwan K E

    2017-09-01

    Hemolytic Uremic Syndrome (HUS), a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The atypical HUS (aHUS) results from over activation of complement system with formation of micro thrombi and damage to endothelial cells resulting in renal impairment in 50 % and death in 25 %, commonly in untreated patients. We report an intriguing case of aHUS presenting with acute onset of movement disorder and fluctuating delirium.

  19. Visceral Myopathy Presenting as Acute Appendicitis and Ogilvie Syndrome

    OpenAIRE

    Kharbuja, Punyaram; Thakur, Raghvendra; Suo, Jian

    2013-01-01

    Background. Visceral myopathy is rare pathological condition of gastrointestinal tract with uncertain clinical presentation and unknown etiology. It often presents with symptoms of chronic intestinal pseudoobstruction of colon. We report a case of visceral myopathy which presented to us as acute appendicitis and Ogilvie syndrome, and we managed it surgically. Method and Result. A case report of 20-year female clinically presented as acute appendicitis and we performed laparoscopic exploratio...

  20. Distal biceps brachii tendon rupture resulting in acute compartment syndrome.

    Science.gov (United States)

    Grandizio, Louis C; Suk, Michael; Feltham, Glen T

    2013-11-01

    Distal biceps brachii tendon rupture is an uncommon injury. Compartment syndrome of the upper arm is rarely described in the literature. The diagnosis of upper arm compartment syndrome requires a high index of suspicion, and emergent surgical treatment with fasciotomy in the acute setting is necessary to avoid devastating neurovascular complications. This article reports a case of acute compartment syndrome of the anterior compartment of the upper arm after a complete rupture of the distal biceps brachii tendon. A healthy 45-year-old man presented with increasing arm pain; paresthesia in the lateral antebrachial cutaneous nerve distribution; and a tense, swollen anterior compartment of his upper arm. Side port catheter absolute pressure measurement was 83 mm Hg with a diastolic blood pressure of 92 mm Hg. The patient underwent an emergent fasciotomy and was found to have a complete rupture of his distal biceps brachii tendon. He subsequently underwent distal biceps tendon repair and delayed primary closure of his incision. Postoperatively, his paresthesia improved and he has no neurological deficit. There is a paucity of case reports describing compartment syndrome after rupture of either the proximal or distal end of the biceps brachii tendon, and none of the reports describe compartment syndrome of the upper arm after rupture of the distal biceps tendon. This article highlights an unusual complication of an uncommon injury and reviews diagnostic and treatment principles for the management of acute compartment syndrome of the upper arm. Copyright 2013, SLACK Incorporated.

  1. Neutrophil gelatinase-associated lipocalin and acute kidney injury in endovascular aneurysm repair or open aortic repair: a pilot study.

    Science.gov (United States)

    Rampoldi, Benedetta; Tessarolo, Serena; Giubbilini, Paola; Gaia, Paola; Corino, Samantha D; Mazza, Sarah; Rigolini, Roberta; Poli, Marco Dei; Vianello, Elena; Romanelli, Massimiliano M Corsi; Costa, Elena

    2018-02-15

    Acute kidney injury (AKI) occurs frequently after abdominal aortic surgery and there is currently no effective marker able to detect early onset. The aim of this study is to evaluate the ability of neutrophil gelatinase-associated lipocalin (NGAL) to early identify the development of acute renal damage in patients undergoing endovascular aneurysm repair (EVAR) or open aortic repair (OAR). Serial samples of blood and urine were obtained from 25 patients undergoing both EVAR and OAR. Seven male subjects with AKI and 18 subjects with no-AKI (17 males, 1 female) were included in the study. We determined concentrations of serum creatinine (sCr) and urinary, serum and whole blood NGAL (uNGAL, sNGAL, bNGAL) collected at baseline, and after 4 and 18 hours. AKI was defined according to the RIFLE criteria (risk, injury, failure, loss of kidney function, and end-stage kidney disease): increase by 50% in sCr or reduction of at least 25% of estimated glomerular filtration rate (eGFR) from baseline. Seven patients developed AKI in the stage Risk. There was no significant difference in sNGAL concentrations in the AKI group as compared to no-AKI group. However, the uNGAL/uCreatinine ratio and bNGAL concentrations were significantly higher after 18 hours in the AKI group (no-AKI 1.69 (0.91 - 2.47) vs AKI 3.2 (2.08 - 5.92) ng/mg for uNGAL/uCreatinine ratio, P = 0.036; and no-AKI 83 (59 - 131) vs AKI 164 (126 - 263) ng/mL for bNGAL, P = 0.029). Our results suggest that uNGAL, sNGAL and bNGAL, after abdominal aortic surgery, are not suitable as early biomarkers of AKI.

  2. Acute and chronic pain syndromes in multiple sclerosis

    DEFF Research Database (Denmark)

    Stenager, E; Knudsen, L; Jensen, K

    1991-01-01

    A representative sample of 117 patients with definite multiple sclerosis (MS) was interviewed on pain syndromes. Chronic syndromes lasting more than one month included dysaestesthesia, low back pain, spasms, tonic seizures, tightening and painful sensations in the extremities. Acute syndromes...... with pain at the time of the examination increased with age and duration of disease. Patients with pain were significantly more often spastic and significantly more often sought alternative treatment forms. No difference was found for mean age, sex, physical impairment, duration of disease from onset of MS...

  3. Coronary Emboli in a Young Patient with Mechanical Aortic Valve: A Rare Cause of Acute Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Arash Gholoobi

    2016-03-01

    Full Text Available Coronary artery embolism is an uncommon cause of Acute Myocardial Infarction (AMI. Herein, we reported a 24-year-old male who was admitted with acute infero-posterior myocardial infarction and cerebral Transient Ischemic Attack (TIA. He had undergone mechanical Aortic Valve Replacement (AVR surgery 6 years ago. Surprisingly, the patient had decided to stop taking his medication (warfarin 20 days earlier without any medical advice. Coronary angiography revealed a thrombus located at the distal part of the left circumflex artery. Discontinuation of anticoagulant therapy in the presence of mechanical valve prosthesis, clinical evidence of coincidental TIA, and lack of atherosclerotic risk factors were highly suggestive of coronary thromboembolism as the cause of AMI. Overall, this case report emphasized the necessity of continuous education in patients with mechanical heart valves to prevent such undesired events.

  4. Gender-Related Differences in Presentation and Outcome of Acute Type a Aortic Dissection

    Directory of Open Access Journals (Sweden)

    Leili Pourafkari

    2017-09-01

    Full Text Available Background: Although acute type-A aortic dissection (AAOD is more common in males, the course of the disease may differ in females. Objectives: We aimed to investigate the gender-related differences in patients with AAOD with regard to clinical presentation, short-term and long-term mortality. Patients and Methods: Medical records of 192 patients with a confirmed diagnosis of AAOD in a University Heart Center from March 2004 through October 2015 were evaluated. Clinical information, hospital mortality, and long-term survival were explored with regard to gender. Propensity matching was performed to adjust for baseline differences. Kaplan-Meier survival analysis was used to determine the impact of gender on long-term mortality. Results: Among 192 patients, 71 (37.0% were females. The women were older (65.7 ± 13.8 vs. 53.9 ± 15.1 years, P < 0.001. Hypertension (77.5% vs. 56.7%; P = 0.006 and diabetes mellitus (12.7% vs. 0.1%; P = 0.001 were more common among women than men, while active smoking was significantly more prevalent in men (46.7% vs. 9.9%; P < 0.001. The frequency of various clinical presentations was not different between the two groups. Among men, 71.7% were surgically managed compared to 67.6% among women. In-hospital mortality was 50.7% in women and 42.1% in men and remained statistically similar after matching. For long-term survival female to male hazard ratio (HR was 1.24 with 95% CI: 0.85 - 1.81 (P = 0.257, which basically remained unchanged after propensity matching, HR was 1.13, 95%CI: 0.73 - 1.73 (P = 0.300. Conclusions: In patients admitted with AAOD, there was no gender-related differences in clinical presentation and type of management. Similarly, hospital and long-term mortality did not differ with regard to gender.

  5. Hybrid approach in an acute type B aortic dissection in a female patient after having a renal transplant.

    Science.gov (United States)

    Janczak, Dariusz; Krajewska, Magdalena; Garcarek, Jerzy; Gancarek, Jerzy; Chabowski, Mariusz

    2014-04-01

    This study describes our experiences with a 44-year-old woman who developed acute type B aortic dissection and elected emergency surgery 3 years after a renal transplant. This led to acute ischemia in the right lower extremity. The first stage of surgery was to implant an extra-anatomic (pretracheal) bypass with a GORE-TEX prosthesis from the brachiocephalic trunk to the left common carotid artery. The second stage was implanting a stent graft into the aortic arch that covered the left common carotid artery and the left subclavian artery. The third stage was to insert a stent graft that involved the entire thoracic aorta and proximal segment of the abdominal aorta to the celiac trunk, with the right axillary and left femoral approach. The fourth stage was an extra-anatomic (suprapubic) bypass with the GORE-TEX prosthesis from the left femoral artery to the right femoral artery. Surgery resulted in normal blood supply to the organs and restored renal function.

  6. Management and Outcomes of Childhood Renal Artery Stenosis and Middle Aortic Syndrome.

    Science.gov (United States)

    Rumman, Rawan K; Matsuda-Abedini, Mina; Langlois, Valerie; Radhakrishnan, Seetha; Lorenzo, Armando J; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

    2018-01-24

    Renal artery stenosis (RAS) in isolation or in conjunction with middle aortic syndrome (MAS), are important vascular causes of childhood hypertension. Few longitudinal studies have assessed the risk of surgical or endovascular intervention, and outcomes by etiology or extent of vascular disease. In a retrospective study of 93 children seen over 30 years with RAS and/or MAS, data on vascular involvement (isolated RAS vs RAS with MAS), etiology (unknown, inflammatory, or genetic), and management were collected. Time to first intervention (endovascular or surgical) was assessed by Cox regression. Mixed-effects analysis examined the longitudinal change in blood pressure after intervention compared to antihypertensive medications alone. Children were 7.0±5.4 years old. Etiology was unknown in 50%, genetic in 26% and inflammatory in 24% of children. Children had isolated RAS (49%) or MAS with or without RAS (51%). Overall, 70% were managed with surgical or endovascular intervention. After adjusting for age, sex, and systolic blood pressure, children with unknown etiology had a higher risk of intervention compared to those with genetic and inflammatory diseases (HR 3.1, 95% CI 1.7,5.6). Children with RAS and MAS were less likely to receive intervention (HR 0.4, 95% CI 0.2,0.8) than isolated RAS. Over a median follow-up of 2 years, 65% remained hypertensive. The longitudinal changes in systolic blood pressure did not differ by etiology, or between interventional and medical management. Hypertension persists despite endovascular or surgical management of childhood RAS and MAS highlighting the importance of close monitoring and ongoing medical management. © American Journal of Hypertension, Ltd 2018. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  7. Cardiovascular Structure and Function in Children With Middle Aortic Syndrome and Renal Artery Stenosis.

    Science.gov (United States)

    Rumman, Rawan K; Slorach, Cameron; Hui, Wei; Matsuda-Abedini, Mina; Langlois, Valerie; Radhakrishnan, Seetha; Lorenzo, Armando J; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

    2017-12-01

    Middle aortic syndrome (MAS) is a narrowing of the abdominal aorta, often in conjunction with renal artery stenosis (RAS). Structure and function of the cardiovascular system are not well understood. In a prospective cross-sectional study, 35 children with MAS or RAS or both (MAS/RAS) were compared with 140 age-, sex-, and body surface area-matched healthy children. Vascular assessment included carotid intima-media thickness and carotid distensibility using B-mode ultrasound and central and peripheral pulse wave velocities using applanation tonometry. Left ventricular structure and function were assessed by 2-dimensional and speckle-tracking echocardiography. Children with MAS or RAS were 12.5±3.0 years old at enrollment, and 50% were men. Carotid intima-media thickness (0.54±0.10 versus 0.44±0.05 mm; P children with disease compared with healthy children; however, after adjustment for systolic blood pressure z score, only carotid intima-media thickness remained significantly higher in the MAS/RAS group compared with the controls (β=0.07 [0.03, 0.10]). Peripheral pulse wave velocities and carotid distensibility were normal. Children with disease had significantly increased left ventricular mass and changes in diastolic function (lower E/a ratio and lower e' velocities). Systolic parameters, including ejection fraction, global longitudinal and circumferential strain, were similar to controls. Our findings demonstrate that children with MAS or RAS have evidence of carotid and left ventricular remodeling, without peripheral arterial involvement, which suggests a localized disease process. Left ventricular systolic function is preserved; however, subtle changes in diastolic function are observed. Carotid vessel changes are consistent with a 5- to 10-year aging, which underscores the importance of blood pressure control. © 2017 American Heart Association, Inc.

  8. Relationship of metabolic syndrome with incident aortic valve calcium and aortic valve calcium progression: the Multi-Ethnic Study of Atherosclerosis (MESA).

    Science.gov (United States)

    Katz, Ronit; Budoff, Matthew J; Takasu, Junichiro; Shavelle, David M; Bertoni, Alain; Blumenthal, Roger S; Ouyang, Pamela; Wong, Nathan D; O'Brien, Kevin D

    2009-04-01

    Metabolic syndrome (MetS) has been associated with increased prevalence of aortic valve calcium (AVC) and with increased progression of aortic stenosis. The purpose of this study was to determine whether MetS is associated with increased risks for the development of new ("incident") AVC or for progression of established AVC as assessed by CT. The relationships of MetS or its components as well as of diabetes to risks for incident AVC or AVC progression were studied among participants with CT scans performed at baseline and at either year 2 or year 3 examinations in the Multi-Ethnic Study of Atherosclerosis (MESA). Of 5,723 MESA participants meeting criteria for inclusion, 1,674 had MetS by Adult Treatment Panel III criteria, whereas 761 had diabetes. Among the 5,123 participants without baseline AVC, risks for incident AVC, adjusted for time between scans, age, sex, race/ethnicity, LDL cholesterol, lipid-lowering medications, and smoking, were increased significantly for MetS (odds ratio [OR] 1.67 [95% CI 1.21-2.31]) or diabetes (2.06 [1.39-3.06]). In addition, there was an increase in incident AVC risk with increasing number of MetS components. Similar results were found using the International Diabetes Federation MetS criteria. Among the 600 participants (10.5%) with baseline AVC, neither MetS nor diabetes was associated with AVC progression. In the MESA cohort, MetS was associated with a significant increase in incident ("new") AVC, raising the possibility that MetS may be a potential therapeutic target to prevent AVC development.

  9. The effects of contrast media volume on acute kidney injury after transcatheter aortic valve replacement: a systematic review and meta-analysis.

    Science.gov (United States)

    Thongprayoon, Charat; Cheungpasitporn, Wisit; Podboy, Alexander J; Gillaspie, Erin A; Greason, Kevin L; Kashani, Kianoush B

    2016-11-01

    The goal of this systematic review was to assess the effects of contrast media volume on transcatheter aortic valve replacement-related acute kidney injury. A literature search was performed using Medline, EMbase, the Cochrane Database of Systematic Reviews, and clinicaltrials.gov from the inception of these databases through December 2015. Studies that reported relative risk, odds ratio, or hazard ratio comparing the risks of acute kidney injury following transcatheter aortic valve replacement in patients who received high contrast media volume were included. Pooled risk ratio (RR) and 95% confidence intervals (95% CI) were calculated using a random-effect, generic inverse variance method. Four cohort studies composed of 891 patients were included in the analyses to assess the risk of acute kidney injury after transcatheter aortic valve replacement in patients who received high contrast media volume. The pooled RR of acute kidney injury after transcatheter aortic valve replacement in patients who received a large volume of contrast media was 1.41 (95% CI, 0.87 to 2.28) compared with low contrast media volume. The meta-analysis was limited to studies using standard acute kidney injury definitions, and the pooled RR of acute kidney injury in patients who received high contrast media volume is 1.12 (95% CI, 0.78 to 1.62). Our meta-analysis shows no significant association between contrast media volume and risk of acute kidney injury after transcatheter aortic valve replacement. © 2016 Chinese Cochrane Center, West China Hospital of Sichuan University and John Wiley & Sons Australia, Ltd.

  10. Impact of Acute Kidney Injury on Short- and Long-term Outcomes After Transcatheter Aortic Valve Implantation.

    Science.gov (United States)

    Nunes Filho, Antonio C B; Katz, Marcelo; Campos, Carlos M; Carvalho, Luiz A; Siqueira, Dimytri A; Tumelero, Rogério T; Portella, Antenor L F; Esteves, Vinícius; Perin, Marco A; Sarmento-Leite, Rogério; Lemos Neto, Pedro A; Tarasoutchi, Flavio; Bezerra, Hiram G; de Brito, Fábio S

    2018-01-18

    Acute kidney injury (AKI) is frequently observed after transcatheter aortic valve implantation (TAVI) and is associated with higher mortality. However, the impact of AKI on long-term outcomes remains controversial. Therefore, we sought to evaluate the impact of AKI on short- and long-term outcomes following TAVI using the Valve Academic Research Consortium 2 criteria. Consecutive patients (n = 794) with severe aortic stenosis who underwent TAVI were included in a multicenter Brazilian registry. Logistic regression analysis was used to identify predictors of AKI. Four-year outcomes were determined as Kaplan-Meier survival curves, and an adjusted landmark analysis was used to test the impact of AKI on mortality among survivors at 12 months. The incidence of AKI after TAVI was 18%. Independent predictors of AKI were age, diabetes mellitus, major or life-threatening bleeding and valve malpositioning. Acute kidney injury was independently associated with higher risk of all-cause death (adjusted HR, 2.8; 95%CI, 2.0-3.9; P cardiovascular mortality (adjusted HR, 2.9; 95%CI, 1.9-4.4; P < .001) over the entire follow-up period. However, when considering only survivors at 12 months, there was no difference in both clinical endpoints (adjusted HR, 1.2; 95%CI, 0.5-2.4; P = .71, and HR, 0.7; 95%CI, 0.2-2.1; P = .57, respectively). Acute kidney injury is a frequent complication after TAVI. Older age, diabetes, major or life-threatening bleeding, and valve malpositioning were independent predictors of AKI. Acute kidney injury is associated with worse short- and long-term outcomes. However, the major impact of AKI on mortality is limited to the first year after TAVI. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  11. Guillain-Barre Syndrome Presenting as Acute Abdomen

    Directory of Open Access Journals (Sweden)

    Faruk incecik

    2015-09-01

    Full Text Available Guillain-Barr and eacute; syndrome (GBS is the most common cause of acute flaccid paralysis in childhood. Symmetric weakness, headache, respiratory symptom, neuropathic pain, muscle pain, paresthesia, and facial palsy were the most common clinical presentations. We report 13-year-old boy with GBS who presented with acute abdominal pain. This is the first report, to our knowledge, first presented of acute abdomen of a pediatric patient with GBS. [Cukurova Med J 2015; 40(3.000: 601-603

  12. Aortic valve-sparing operation after correction of heart displacement due to pectus excavatum using Nuss procedure in a Marfan syndrome patient.

    Science.gov (United States)

    Fukunaga, Naoto; Yuzaki, Mitsuru; Hamakawa, Hiroshi; Nasu, Michihiro; Takahashi, Yutaka; Okada, Yukikatsu

    2012-01-01

    Cardiovascular surgery in the setting of chest wall deformities is a clinical challenge. Pectus excavatum, for example, can cause heart displacement to the left thoracic cavity, following the poor operative field. This report highlights a case in which a successful aortic valve-sparing operation via conventional median sternotomy after correction of the heart displacement due to pectus excavatum using Nuss procedure in Marfan syndrome. This technique can be one surgical option in Marfan syndrome patients with pectus excavatum and thoracic aortic aneurysm under close follow up.

  13. Metabolic syndrome and the risk of adverse cardiovascular events after an acute coronary syndrome.

    Science.gov (United States)

    Cavallari, Ilaria; Cannon, Christopher P; Braunwald, Eugene; Goodrich, Erica L; Im, KyungAh; Lukas, Mary Ann; O'Donoghue, Michelle L

    2018-01-01

    Background The incremental prognostic value of assessing the metabolic syndrome has been disputed. Little is known regarding its prognostic value in patients after an acute coronary syndrome. Design and methods The presence of metabolic syndrome (2005 International Diabetes Federation) was assessed at baseline in SOLID-TIMI 52, a trial of patients within 30 days of acute coronary syndrome (median follow-up 2.5 years). The primary endpoint was major coronary events (coronary heart disease death, myocardial infarction or urgent coronary revascularization). Results At baseline, 61.6% ( n = 7537) of patients met the definition of metabolic syndrome, 34.7% (n = 4247) had diabetes and 29.3% had both ( n = 3584). The presence of metabolic syndrome was associated with increased risk of major coronary events (adjusted hazard ratio (adjHR) 1.29, p definition, only diabetes (adjHR 1.48, p metabolic syndrome was numerically but not significantly associated with the risk of major coronary events (adjHR 1.13, p = 0.06). Conversely, diabetes was a strong independent predictor of major coronary events in the absence of metabolic syndrome (adjHR 1.57, p metabolic syndrome identified patients at highest risk of adverse outcomes but the incremental value of metabolic syndrome was not significant relative to diabetes alone (adjHR 1.07, p = 0.54). Conclusions After acute coronary syndrome, diabetes is a strong and independent predictor of adverse outcomes. Assessment of the metabolic syndrome provides only marginal incremental value once the presence or absence of diabetes is established.

  14. Evaluation of acute compartment syndrome of extremities in ...

    African Journals Online (AJOL)

    The aim of this study was to analyze the clinical signs of diagnosis, treatment and followup of the pediatric population presenting with acute compartment syndrome in the emergency room at an Institutional Level II trauma center. The study is a prospective case series. Thirty-two samples were selected from Institutional ...

  15. Acute colonic pseudo obstruction (Ogilvie\\'s syndrome): Case report ...

    African Journals Online (AJOL)

    Ogilvie\\'s syndrome was first described by Sir Heneage Ogilvie in 1948 and is a rare disorder comprising acute colonic pseudo obstruction (ACPO) with gross dilation of the caecum and right hemicolon (occasionally extending to the rectum) without an anatomic lesion that obstructs the aboral flow of intestinal contents.

  16. Acute Coronary Syndrome In Zanzibar, Tanzania | Budha | Tropical ...

    African Journals Online (AJOL)

    A descriptive study was carried out to determine patients characteristics, prevalence of risk factors, presenting symptom, provisional diagnosis, final diagnosis and outcome of acute coronary syndrome (ACS). Thirteen cases were collected between July 2001 to July 2002 and September 2003 to April 2004. Eleven (84.6%) ...

  17. Infective endocarditis presenting as acute coronary syndrome | El ...

    African Journals Online (AJOL)

    We report tow cases of infective endocarditis (IE) presenting as acute coronary syndrome (ACS). Case 1: A 60-year-old man with the diagnosis of mitral IE complicated by an ST segment elevation myocardial infarction. Primary percutaneous coronary intervention with aspiration of the thrombus at the distal leftanterior ...

  18. Acute kidney injury and hepatorenal syndrome in cirrhosis

    DEFF Research Database (Denmark)

    Egerod Israelsen, Mads; Gluud, Lise Lotte; Krag, Aleksander

    2015-01-01

    Cirrhosis is the eighth leading cause of "years of lost life" in the United States and accounts for approximately 1% to 2% of all deaths in Europe. Patients with cirrhosis have a high risk of developing acute kidney injury. The clinical characteristics of hepatorenal syndrome (HRS) are similar...

  19. Evaluation of acute compartment syndrome of extremities in ...

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    necrotic tissue in the histological examination. In our study, we also observed patchy necrosis of muscle fibers with perifascicular oedema3. We observed ischemic contractures in about 18.7% of our cases. In a series of 24 children with acute compartment syndrome, chronic contractures were reported only in 4.2% cases3.

  20. Developing diagnostic guidelines for the acute radiation syndrome

    International Nuclear Information System (INIS)

    Densow, D.; Fliedner, T.M.; Kindler, H.

    1996-01-01

    Diagnostic guidelines seem to be promising for improving medical care. One aspect of a diagnostic guideline for the acute radiation syndrome has been tested against an extensive case history database. Subsequently, the guideline has been optimized for a small set of case histories. The improved performance has been proven by a test against the rest of the case history database

  1. Recurrent Metachronous Acute Compartment Syndrome in the Legs ...

    African Journals Online (AJOL)

    METHODS: A seven-year-old boy presented with four separate episodes of acute compartment syndrome of the legs within three years. The recurrent nature of the illness prompted detail review of the records of the child. RESULTS: There was involvement of each leg twice between the ages of three and seven years ...

  2. Addison's disease presenting as acute chest syndrome: Case report ...

    African Journals Online (AJOL)

    Addison's disease presenting as acute chest syndrome: Case report and review of literature. MR Akpa, OJ Odia. Abstract. No Abstract. Nigerian Journal of Medicine Vol. 15 (4) October-December 2006: 451-452. Full Text: EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

  3. Acute lymphoblastic leukemia in children with Down syndrome

    DEFF Research Database (Denmark)

    Buitenkamp, Trudy D; Izraeli, Shai; Zimmermann, Martin

    2014-01-01

    Children with Down syndrome (DS) have an increased risk of B-cell precursor (BCP) acute lymphoblastic leukemia (ALL). The prognostic factors and outcome of DS-ALL patients treated in contemporary protocols are uncertain. We studied 653 DS-ALL patients enrolled in 16 international trials from 1995...

  4. Chernobyl 1996. New materials concerning acute radiation syndrome around Chernobyl

    International Nuclear Information System (INIS)

    Lupandin, Vladimir M.

    2016-01-01

    In 1996 we have succeeded in getting reliable data on radiation situation in the Khoiniki district. These were data of Civil Defence Headquarters of the Khoiniki district, remaining in private notes of the Chief of the Headquarters. Including the other data or materials, radiation syndromes in particular the acute are described. (J.P.N.)

  5. Acute coronary syndrome caused by anomalous origin of the right ...

    African Journals Online (AJOL)

    Acute coronary syndrome caused by anomalous origin of the right coronary artery from the left sinus of Valsalva. AS Assiri. Abstract. No Abstract. West African Journal of Medicine Vol. 24(3) 2005: 278-279. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT.

  6. Ward Round - Late Presentation of Acute Compartment Syndrome in ...

    African Journals Online (AJOL)

    following the course of ibuprofen mentioned. Twelve days after admission he started to complain of increasing pain and tightness in his left thigh. Sensation and motor function. Ward Round - Late Presentation of Acute. Compartment Syndrome in the Thigh. University of Malawi, College of Medicine, Department of Surgery,.

  7. Complex aortic and bilateral renal artery aneurysm repair in a young patient with multiple arterial aneurysm syndrome

    Directory of Open Access Journals (Sweden)

    Carlos A. Hinojosa, MD, MSc

    2016-09-01

    Full Text Available Aneurysmal disease affecting the aorta and visceral vessels in young patients is uncommon and typically associated with connective tissue disorders. We describe the case of a 17-year-old girl who presented with acute onset of abdominal pain; computed tomography scan revealed aortic and bilateral renal artery aneurysms and a perirenal hematoma. She was taken to the angiography suite; rupture of the right renal artery aneurysm was identified and immediately treated successfully with coil embolization. The left renal artery aneurysm was repaired with ex-vivo renal autotransplantation; 2 years later, the aorta and right renal artery underwent surgical reconstruction.

  8. [Acute colonic pseudo-obstruction or Ogilvie syndrome].

    Science.gov (United States)

    Kiss, L; Nica, C

    2000-01-01

    Acute colonic pseudo-obstruction is a clinical condition with the symptoms, signs and radiological appearances of acute large bowel obstruction but without any apparent mechanical cause. The mortality rate for patients undergoing surgery was 57%. The incidence of caecal-perforation was 1 for 7 patients. The pathogenesis of the syndrome is unknown but in more then 80% of cases many conditions that have been associated with this syndrome. The efficacy of prepulsid agents for the treatment of syndrome remains to be assessed in a controlled study. In our patients clinical observation, radiologic studies and direct observation during several hours of surgery had shown a complete lack of propulsive motility within of the large bowel. This can be related to the ganglionitis and later to the aganglionosis of the mesenteric plexus.

  9. Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

    Science.gov (United States)

    Nicolo, Francesco; Romeo, Francesco; Lio, Antonio; Bovio, Emanuele; Scafuri, Antonio; Bassano, Carlo; Polisca, Patrizio; Pellegrino, Antonio; Nardi, Paolo; Chiariello, Luigi; Ruvolo, Giovanni

    2017-07-01

    The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation. Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29). No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33). The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

  10. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

    Science.gov (United States)

    Ritter, Alyssa; Atzinger, Carrie; Hays, Brandon; James, Jeanne; Shikany, Amy; Neilson, Derek; Martin, Lisa; Weaver, Kathryn Nicole

    2017-06-01

    Hypermobile Ehlers-Danlos syndrome (hEDS) is a common inherited connective tissue disorder characterized by joint hypermobility. The natural history of aortic root dilation (AoD), a potential complication of EDS, has not been well characterized in this population. We describe the natural history of aortic root size in a large cohort of patients with hEDS. A cohort of 325 patients with HEDS was identified at Cincinnati Children's Hospital Medical Center (CCHMC), including 163 patients from a previous study. Medical records were reviewed and each participant's height, weight, and aortic dimensions from up to four echocardiograms were documented. Aortic root z-scores were calculated using two established formulas based on age (Boston or Devereux). Overall prevalence of AoD and prevalence by age were calculated and longitudinal regression was performed. The prevalence of AoD with a z-score ≥ 2.0 was 14.2% (46/325) and with a z-score of ≥3.0 was 5.5% (18/325). No significant increases in z-score were seen over time for patients with multiple echocardiograms. Participants under the age of 15 years had an average decline of 0.1 standard deviations (SDs)/year. No significant change was found after 15 of age. Between the ages of 15 and 21 years, Boston z-scores were 0.96 higher than Devereux z-scores. The nearly 1 z-score unit difference between formulas indicates caution prior to diagnosing AoD in patients with hEDS. In light of the low prevalence and lack of progression of AoD, routine echocardiograms may not be warranted for pediatric patients with hEDS. © 2017 Wiley Periodicals, Inc.

  11. Identification Bracelet Precipitated Acute Compartment Syndrome during Intravenous Infusion in an Obtunded Patient

    Directory of Open Access Journals (Sweden)

    Wahib Zafar

    2016-01-01

    Full Text Available Acute compartment syndrome is a serious condition requiring immediate medical care. A lack of urgent medical treatment can result in serious complications such as loss of function and even amputation. While the pathophysiology of acute compartment syndrome is well understood, numerous potential causes are still being discovered. A rare cause of acute compartment syndrome is IV infiltration. We present a case of acute compartment syndrome resulting from intravenous infusion due to proximal placement of a patient identification bracelet. We conclude that both routine evaluation for IV infiltration and proximal placement of IV lines are essential for prevention of acute compartment syndrome.

  12. Intermittent acute aortic valve regurgitation: A case report of a prosthetic valve dysfunction

    NARCIS (Netherlands)

    S.E. Karagiannis (Stefanos); G. Karatasakis (George); K. Spargias (Konstantinos); L. Louka; D. Poldermans (Don); D.V. Cokkinos (Dennis)

    2008-01-01

    textabstractComplications of any mechanical prosthesis include thrombus or pannus formation. In our case report we demonstrate that prosthetic aortic valve regurgitation due to pannus formation may be intermittent and non-cyclic in pattern and therefore not obvious at the time of original clinical

  13. 78 FR 79300 - Cardiovascular Devices; Reclassification of Intra-Aortic Balloon and Control Systems for Acute...

    Science.gov (United States)

    2013-12-30

    .... FDA-2013-N-0581] Cardiovascular Devices; Reclassification of Intra-Aortic Balloon and Control Systems... of these devices into class II. List of Subjects in 21 CFR Part 870 Medical devices, Cardiovascular... Commissioner of Food and Drugs, 21 CFR part 870 is amended as follows: PART 870--CARDIOVASCULAR DEVICES 0 1...

  14. Evidence, lack of evidence, controversy, and debate in the provision and performance of the surgery of acute type A aortic dissection

    DEFF Research Database (Denmark)

    Bonser, Robert S; Ranasinghe, Aaron M; Loubani, Mahmoud

    2011-01-01

    Acute type A aortic dissection is a lethal condition requiring emergency surgery. It has diverse presentations, and the diagnosis can be missed or delayed. Once diagnosed, decisions with regard to initial management, transfer, appropriateness of surgery, timing of operation, and intervention for ...

  15. Compliance of the normal-sized aorta in adolescents with Marfan syndrome: comparison of MR measurements of aortic distensibility and pulse wave velocity

    International Nuclear Information System (INIS)

    Eichhorn, J.G.; Ruediger, H.J.; Gorenflo, M.; Khalil, M.; Ulmer, H.; Krissak, R.; Kauczor, H.U.; Ley, S.; Universitaetsklinik Heidelberg; Arnold, R.; Universitaetskinderklinik Freiburg; Boese, J.; Siemens AG, Medical Solutions, Forchheim; Krug, R.; Fink, C.

    2007-01-01

    Purpose: To compare the aortic compliance of the normal-sized aorta of adolescents with Marfan syndrome and healthy controls using MR measurements of the aortic distensibility and pulse wave velocity. Materials and Methods: Fourteen patients (median age: 15 [9-21] years) and 11 healthy subjects (23 [12-32] years) were examined at 1.5 T. The MR protocol included 2D steady-state free precession (SSFP)-CINE MRI of the aortic distensibility and PC-MRI of the pulse wave velocity. All measurements were positioned perpendicular to the descending aorta at the level of the diaphragm for assessing the changes in the aortic cross-sectional areas and additionally above and below this plane for assessing the pulse wave velocity. In addition contrast-enhanced 3D-MR angiography was performed in adolescents with Marfan syndrome to exclude morphologic changes and to prove normal-sized aorta. Results: Compared with control subjects, adolescents with Marfan syndrome had significantly decreased distensibility and significantly increased pulse wave velocity (χ 2 -test, p = 0.0002) using an age-related non-linear regression analysis. The related aortic compliance was significantly decreased (χ 2 -test, p = 0.0002). There was a good correlation between the two methods (r = 0.86). A low intraobserver variability was found for both methods (≤ 2 %). (orig.)

  16. The role of levosimendan in acute heart failure complicating acute coronary syndrome

    DEFF Research Database (Denmark)

    Nieminen, Markku S; Buerke, M.; Cohen-Solal, A.

    2016-01-01

    Acute heart failure and/or cardiogenic shock are frequently triggered by ischemic coronary events. Yet, there is a paucity of randomized data on the management of patients with heart failure complicating acute coronary syndrome, as acute coronary syndrome and cardiogenic shock have frequently been...... defined as exclusion criteria in trials and registries. As a consequence, guideline recommendations are mostly driven by observational studies, even though these patients have a particularly poor prognosis compared to heart failure patients without signs of coronary artery disease. In acute heart failure...... adrenergic inotropes as a first line therapy for all ACS-AHF patients who are under beta-blockade and or when urinary output is insufficient after diuretics. Levosimendan can be used alone or in combination with other inotropic or vasopressor agents, but requires monitoring due to the risk of hypotension. (C...

  17. Risk Factors for Mortality and Outcomes in Pediatric Acute Lung Injury/Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Panico, Flávia F; Troster, Eduardo J; Oliveira, Cindy S; Faria, Aline; Lucena, Michelle; João, Paulo R D; Saad, Everardo D; Foronda, Flávia A K; Delgado, Artur F; de Carvalho, Werther Brunow

    2015-09-01

    Children admitted to PICUs often present with or develop respiratory failure that requires mechanical ventilation. We prospectively identified children admitted to three general PICUs, with the goal of identifying risk factors for mortality. Prospective multicenter observational study. Three general PICUs, two in São Paulo and one in Curitiba, Brazil. Children aged between 1 month and 15 years, consecutively admitted between August 2008 and July 2010, with acute lung injury or acute respiratory distress syndrome that developed at least 12 hours after invasive or noninvasive mechanical ventilation. None. We used logistic regression models to explore the relationship between death and independent variables. Of 3,046 patients admitted to the three PICUs, 1,658 patients underwent mechanical ventilation, and 84 fulfilled the acute lung injury/acute respiratory distress syndrome inclusion criteria and were analyzed. Nearly 60% were boys, and the median age was 31 months. Pressure control/assist control was the initial mode of mechanical ventilation in 86% of cases, and the median durations of mechanical ventilation and PICU stay were 12 and 15 days, respectively. None of the eight patients with acute lung injury died, whereas 33 of 76 of the remaining patients with acute respiratory distress syndrome died, for an overall mortality rate of 39.3% (95% CI, 28.8-50.6%). In different multivariate logistic regression model, the number of organ dysfunctions at admission, peak inspiratory pressure, airway pressure gradient on day 1, and the mean airway pressure gradient over the first 7 days of mechanical ventilation were significantly associated with mortality. Mortality is high in pediatric acute lung injury/acute respiratory distress syndrome. Mechanical ventilation-associated risk factors for death among such patients are potential targets for intervention.

  18. A Rare Case of Acute Coronary Syndrome in a Patient With Turner Syndrome.

    Science.gov (United States)

    Kemaloglu, Tugba; Ozer, Nihat; Fikri Yapici, Mehmet

    2016-05-01

    In Turner syndrome, cardiovascular complications are the most important causes of early mortality. Congenital cardiovascular abnormalities are found in approximately one third of Turner syndrome patients. Developments in diagnosis and treatment have decreased the rate of mortality related to these abnormalities. In recent years, many papers have mentioned that coronary artery disease developing at early ages in patients with Turner syndrome causes sudden deaths. The patient, a 27-year-old female was admitted to the emergency room with chest pain at rest. She was diagnosed with Turner Syndrome in her teenage years due to amenorrhea. Patients with ECG changes and cardiac enzyme elevations were treated with acute coronary syndrome. The young woman with Turner Syndrome have several risk factors for early Coronary Artery Disease development. In such cases, dramatic results like sudden death or heart attack at an early age may occur in cases of insufficient follow-up and treatment.

  19. Acute radiation syndrome caused by accidental radiation exposure - therapeutic principles

    Directory of Open Access Journals (Sweden)

    Dörr Harald

    2011-11-01

    Full Text Available Abstract Fortunately radiation accidents are infrequent occurrences, but since they have the potential of large scale events like the nuclear accidents of Chernobyl and Fukushima, preparatory planning of the medical management of radiation accident victims is very important. Radiation accidents can result in different types of radiation exposure for which the diagnostic and therapeutic measures, as well as the outcomes, differ. The clinical course of acute radiation syndrome depends on the absorbed radiation dose and its distribution. Multi-organ-involvement and multi-organ-failure need be taken into account. The most vulnerable organ system to radiation exposure is the hematopoietic system. In addition to hematopoietic syndrome, radiation induced damage to the skin plays an important role in diagnostics and the treatment of radiation accident victims. The most important therapeutic principles with special reference to hematopoietic syndrome and cutaneous radiation syndrome are reviewed.

  20. Contemporary Management of Acute Aortic Occlusion Has Evolved but Outcomes Have Not Significantly Improved.

    Science.gov (United States)

    Robinson, William P; Patel, Rupal K; Columbo, Jesse A; Flahive, Julie; Aiello, Francesco A; Baril, Donald T; Schanzer, Andres; Messina, Louis M

    2016-07-01

    Most existing series of acute aortic occlusion (AAO) predate the changes in surgical and endovascular therapy of the last 2 decades. We examined the contemporary management and outcomes of AAO. We reviewed consecutive patients with AAO at a tertiary referral center from 2004 to 2012. Outcomes were stratified and compared according to etiology and procedure performed. AAO in 29 patients was due to in situ thrombosis in 21 (72%) and embolism in 8 (28%) patients. Vascular patients with embolism were on average older (77 ± 7 vs. 66 ± 12 years, P = 0.02) and had higher rates of atrial fibrillation (100% vs. 20%, P = 0.0002) and congestive heart failure (75% vs. 0%, P = 0.0001) in comparison with those with in situ thrombosis. Neurologic deficit was present in 16 (55%) patients. Six patients (21%) presented with bilateral paresis/paralysis secondary to spinal cord or lumbosacral plexus ischemia, and primary neurologic etiology was investigated before vascular consultation was obtained in 4 of these 6 patients. Of the 29 patients, 28 (97%) underwent revascularization including transfemoral embolectomy (n = 6), transperitoneal aortoiliac thrombectomy (n = 2), axillobifemoral bypass (n = 10), aortobifemoral bypass (n = 6), and endovascular therapy including thrombolysis, angioplasty ± stenting (n = 4). In-hospital mortality was 31% and did not vary significantly according to etiology (embolism 38% vs. in situ thrombosis 29%, P = 0.67). In-hospital mortality varied widely according to procedure (transfemoral embolectomy 50%, aortoiliac thrombectomy 100%, axillobifemoral bypass 30%, aortobifemoral bypass 0%, and endovascular therapy 25%, P = 0.08). Major morbidity (59%), length of stay (8.6 ± 8.0 days), and discharge to a rehabilitation facility (50%) did not vary by etiology or procedure. At a media follow-up of 361 ± 460 days (range 3-2014), overall survival was 42%. There were no amputations among 20 survivors of initial

  1. Prone position in patients with acute respiratory distress syndrome

    Science.gov (United States)

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making. PMID:27925054

  2. Generalized pustular psoriasis complicated by acute respiratory distress syndrome.

    Science.gov (United States)

    Abou-Samra, T; Constantin, J-M; Amarger, S; Mansard, S; Souteyrand, P; Bazin, J-E; D'Incan, M

    2004-02-01

    Psoriasis has a chronic and relatively benign course. However, severe complications are possible. One rare complication is acute interstitial pneumonitis. This entity should be suspected when a patient presents with dyspnoea and high fever. Knowledge of this pathology is crucial, for although it is essential to rule out aetiologies requiring specific management such as microbial infection or drug-related syndromes, diagnosis should not be delayed as its severe clinical course is improved by corticosteroids. We report two patients with an acute respiratory distress syndrome arising during the course of pustular psoriasis. Repeated bacteriological testing in lungs and blood remained negative. In both cases lung involvement was severe, requiring artificial ventilation. Dramatic clinical resolution was obtained by using corticosteroids. Besides infectious causes and drug hypersensitivity to methotrexate or acitretin, acute respiratory distress syndrome, sometimes due to a pulmonary capillary leak syndrome, is a rare cause of pneumonitis in the course of psoriasis, and may be fatal. Its pathogenesis is unknown. However, animal models suggest a role for T-helper (Th) 1 lymphocytes, known to be activated in psoriasis, and a role for tumour necrosis factor-alpha, a major Th1 cytokine, in alveolar damage.

  3. Prone position in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making.

  4. Acute myocardial infarction in young adults with Antiphospholipid syndrome: report of two cases and literature review

    OpenAIRE

    Abid, Leila; Frikha, Faten; Bahloul, Zouhir; Kammoun, Samir

    2011-01-01

    Abstract Acute myocardial infarction (AMI) is rarely associated with antiphospholipid syndrome. The treatment of these patients is a clinical challenge. We report the observations of 2 young adults (1 woman and 1 man), admitted in our acute care unit for acute myocardial infarction (AMI). A coagulopathy work-up concludes the existence of antiphospholipid syndrome (APS) in the 2 cases. APS syndrome was considered primary in 2 cases. All patients presented an intense inflammatory syndrome (high...

  5. [Acute Stress and Broken Heart Syndrome. A Case Report].

    Science.gov (United States)

    Vergel, Juliana; Tamayo-Orozco, Sebastián; Vallejo-Gómez, Andrés Felipe; Posada, María Teresa; Restrepo, Diana

    Stress has been associated with an acute heart failure syndrome of important morbidity and mortality. Case report and non-systematic review of the relevant literature. A 65-year-old woman with a history of an untreated generalized anxiety disorder, whom after the violent death of her son presented with oppressive chest pain irradiated to neck and left superior extremity, lasting for more than 30minutes, initial clinical suspect suggests acute coronary syndrome. Tako-tsubo cardiomyopathy is characterized by a reversible left ventricular dysfunction and wall movement abnormalities, without any compromise of the coronary arteries, associated to high plasma levels of catecholamines which in most cases correlates with an acute stress of emotional or physical type. Tako-tsubo cardiomyopathy has to be considered by physicians among the differential diagnosis when facing a patient with suspected acute coronary syndrome, especially in post-menopausal women with a history of psychiatric comorbidities such as a generalized anxiety disorder. Copyright © 2016 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

  6. Contemporary management and outcomes of acute type A aortic dissection: An analysis of the STS adult cardiac surgery database.

    Science.gov (United States)

    Lee, Teng C; Kon, Zachary; Cheema, Faisal H; Grau-Sepulveda, Maria V; Englum, Brian; Kim, Sunghee; Chaudhuri, Paramita S; Thourani, Vinod H; Ailawadi, Gorav; Hughes, G Chad; Williams, Matthew L; Brennan, J Matthew; Svensson, Lars; Gammie, James S

    2018-01-01

    Management of acute type A aortic dissection (AAAD) is challenging and operative strategies are varied. We used the STS Adult Cardiac Surgery Database (STS ACSD) to describe contemporary surgical strategies and outcomes for AAAD. Between July 2011 and September 2012, 2982 patients with AAAD underwent operations at 640 centers in North America. In this cohort, median age was 60 years old, 66% were male, and 80% had hypertension. The most common arterial cannulation strategies included femoral (36%), axillary (27%), and direct aortic (19%). The median perfusion and cross-clamp times were 181 and 102 min, respectively. The lowest temperature on bypass showed significant variation. Hypothermic circulatory arrest (HCA) was used in 78% of cases. Among those undergoing HCA, brain protection strategies included antegrade cerebral perfusion (31%), retrograde cerebral perfusion (25%), both (4%), and none (40%). Median HCA plus cerebral perfusion time was 40 min. Major complications included prolonged ventilation (53%), reoperation (19%), renal failure (18%), permanent stroke (11%), and paralysis (3%). Operative mortality was 17%. The median intensive care unit and hospital length of stays were 4.7 and 9.0 days, respectively. Among 640 centers, the median number of cases performed during the study period was three. Resuscitation, unresponsive state, cardiogenic shock, inotrope use, age >70, diabetes, and female sex were found to be independent predictors of mortality. These data describe contemporary patient characteristics, operative strategies, and outcomes for AAAD in North America. Mortality and morbidity for AAAD remain high. © 2018 Wiley Periodicals, Inc.

  7. Laparoscopic treatment of acute non traumatic abdomen syndrome

    International Nuclear Information System (INIS)

    Rodriguez, G.; Tellechea, M.; Antunez, C.; Perdomo, M.; Gateno, N.

    2003-01-01

    Laparoscopic treatment of acute non traumatic abdomen syndrome may be safely performed in the majority of cases.Results obtained cover the treatment of 155 patients through video laparoscopic approach, between February 1997 and March 2001.Twenty two patients were excluded because the laparoscopic performed was solely for the purpose of diagnosis.Seventy two patients were women and 61 men.Average age was 45 years old, the youngest being 7 and the oldest 86.Surgery indicators were 67 acute appendicitis; 51 acute cholecystitis; 8 diffuse peritonitis (5 due to perforated gastroduodenal ulcus, 1 of appendicular origin, another of biliary origin and still another as perforative complication of diverticular colopathy); 5 acute pancreatitis of biliary origin; 1 peri diverticular abscess and 1 bilioma.Twelve patients had to be convert to open surgery (9%).Global morbidity was 6% (4 post appendicectomy abscesses; 1 sub hepatic biblio ma, a biliary peritonitis and two infections of parietal abscesses.Mortality was null

  8. [Clinical and laboratory characteristics of the acetonemia syndrome in children with severe acute intestinal infection].

    Science.gov (United States)

    Zriachkin, N I; Chudakova, T K; Buchkova, T N

    2012-12-01

    A total of 55 children suffering from acute intestinal infection severe in age from 3 months to 7 years; of these, 37 patients with atsetonemicheskim syndrome (AS). Found that the development AS in children with acute intestinal infections severe, aggravate the disease. In children with acute intestinal infection with the syndrome, the duration of atsetonemicheskim main symptoms of intoxication in the 1,2-1,5 times longer than those of children suffering from acute intestinal infection without atsetonemicheskogo syndrome.

  9. Aortic regurgitation after valve-sparing aortic root replacement: modes of failure.

    Science.gov (United States)

    Oka, Takanori; Okita, Yutaka; Matsumori, Masamichi; Okada, Kenji; Minami, Hitoshi; Munakata, Hiroshi; Inoue, Takeshi; Tanaka, Akiko; Sakamoto, Toshihito; Omura, Atsushi; Nomura, Takuo

    2011-11-01

    Despite the positive clinical results of valve-sparing aortic root replacement, little is known about the causes of reoperations and the modes of failure. From October 1999 to June 2010, 101 patients underwent valve-sparing aortic root replacement using the David reimplantation technique. The definition of aortic root repair failure included the following: (1) intraoperative conversion to the Bentall procedure; (2) reoperation performed because of aortic regurgitation; and (3) aortic regurgitation equal to or greater than a moderate degree at the follow-up. Sixteen patients were considered to have repair failure. Three patients required intraoperative conversion to valve replacement, 3 required reoperation within 3 months, and another 8 required reoperation during postoperative follow-up. At initial surgery 5 patients had moderate to severe aortic regurgitation, 6 patients had acute aortic dissections, 3 had Marfan syndrome, 2 had status post Ross operations, 3 had bicuspid aortic valves, and 1 had aortitis. Five patients had undergone cusp repair, including Arantius plication in 3 and plication at the commissure in 2. The causes of early failure in 6 patients included cusp perforation (3), cusp prolapse (3), and severe hemolysis (1). The causes of late failure in 10 patients included cusp prolapse (4), commissure dehiscence (3), torn cusp (2), and cusp retraction (1). Patients had valve replacements at a mean of 23 ± 20.9 months after reimplantation and survived. Causes of early failure after valve-sparing root replacement included technical failure, cusp lesions, and steep learning curve. Late failure was caused by aortic root wall degeneration due to gelatin-resorcin-formalin glue, cusp degeneration, or progression of cusp prolapse. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  10. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

    NARCIS (Netherlands)

    Lacro, R.V.; Guey, L.T.; Dietz, H.C.; Pearson, G.D.; Yetman, A.T.; Gelb, B.D.; Loeys, B.L.; Benson, D.W.; Bradley, T.J.; Backer, J. de; Forbus, G.A.; Klein, G.L.; Lai, W.W.; Levine, J.C.; Lewin, M.B.; Markham, L.W.; Paridon, S.M.; Pierpont, M.E.; Radojewski, E.; Selamet Tierney, E.S.; Sharkey, A.M.; Wechsler, S.B.; Mahony, L.; et al.,

    2013-01-01

    BACKGROUND: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and

  11. Acute Aortic Dissection in a Third Trimester Pregnancy without Risk Factors

    Directory of Open Access Journals (Sweden)

    Elizabeth L Walters

    2011-05-01

    Full Text Available Spontaneous aortic dissection in pregnancy is rare and life threatening for both the mother and the fetus. Most commonly, it is associated with connective tissue disorders, cardiac valve variants, or trauma. We present the case of a 23-year-old previously healthy woman, 36 weeks pregnant with a syncopal episode after dyspnea and vomiting. She subsequently developed cardiac arrest and underwent aggressive resuscitation, emergent thoracotomy, and cesarean delivery without recovery. On autopsy, she was found to have an aortic dissection of the ascending aorta. This case is presented to raise awareness and review the literature and the clinical approach to critical care for pregnant patients. [West J Emerg Med. 2011;12(4:571–574.

  12. Short report Neutrophil gelatinase-associated lipocalcin (NGAL) as a biomarker of dialysis-dependent acute kidney injury following infrarenal aortic surgery

    DEFF Research Database (Denmark)

    Jørgensen, Helene Korvenius; Stæhr, Jannie Bisgaard; Gilsaa, Torben

    2013-01-01

    Background: Acute kidney injury (AKI) is common following abdominal aortic surgery. NGAL might be useful in the early diagnosis of AKI since it responds rapidly to ischaemic damage. Methods: Twenty patients undergoing elective infrarenal aortic surgery. U-NGAL was measured before surgery and 24, 48...... and 72 hours postoperatively. Results: No significant rise in U-NGAL was seen in patients without AKI at any time. All patients who developed dialysisdependent AKI had a significant increase in U-NGAL. Conclusion: U-NGAL did not increase solely in response to the surgical trauma. U-NGAL may be a useful...

  13. Pathophysiology and Management of Acute Respiratory Distress Syndrome in Children.

    Science.gov (United States)

    Heidemann, Sabrina M; Nair, Alison; Bulut, Yonca; Sapru, Anil

    2017-10-01

    Acute respiratory distress syndrome (ARDS) is a syndrome of noncardiogenic pulmonary edema and hypoxia that accompanies up to 30% of deaths in pediatric intensive care units. Pediatric ARDS (PARDS) is diagnosed by the presence of hypoxia, defined by oxygenation index or Pao 2 /Fio 2 ratio cutoffs, and new chest infiltrate occurring within 7 days of a known insult. Hallmarks of ARDS include hypoxemia and decreased lung compliance, increased work of breathing, and impaired gas exchange. Mortality is often accompanied by multiple organ failure. Although many modalities to treat PARDS have been investigated, supportive therapies and lung protective ventilator support remain the mainstay. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Acute carpal tunnel syndrome in a patient with haemophilia.

    Science.gov (United States)

    Mayne, Alistair Ivan William; Howard, Anthony; Kent, Matthew; Banks, Joanne

    2012-07-03

    Acute carpal tunnel syndrome (CTS) is a rare surgical condition usually resulting from wrist trauma. We present the case of a young haemophilic man who developed acute CTS following trivial injury. The patient was initially managed conservatively but symptom progression resulted in carpal tunnel decompression. A literature review and management approach are presented. This is an important complication of haemophilia to be aware of as prompt conservative management can obviate the need for surgery. This case is useful in (a) highlighting the importance of considering a patient's medical history when formulating differential diagnoses and (b) outlining a management approach to this condition.

  15. Embolized prostatic brachytherapy seeds mimicking acute chest pain syndromes

    Directory of Open Access Journals (Sweden)

    Nirmal Guragai

    2017-01-01

    Full Text Available A 59-year-old male with a history of nonobstructive coronary artery disease, diabetes mellitus, hypertension, and prostate cancer presented to the hospital with 1-day history of pleuritic chest pain. Initial workup for acute coronary event was unremarkable. Chest X-ray revealed multiple small radial densities which were linear and hyperdense, consistent with embolization of metallic seeds to the pulmonary circulation. The patient was noted to have had radioactive metallic seeds implanted for prostate cancer 6 months ago. Diagnosis of pulmonary embolization of prostatic seeds is challenging as they frequently present with chest pain mimicking acute coronary syndromes.

  16. [Prognostic factors for in-hospital mortality in patients with acute kidney injury requiring continuous renal replacement therapy undergoing surgery for acute Stanford type A aortic dissection].

    Science.gov (United States)

    Jiao, R; Liu, N

    2017-04-01

    Objective: To evaluate prognostic factors for in-hospital mortality in patients with acute kidney injury (AKI) requiring continuous renal replacement therapy (CRRT) undergoing surgery for acute Stanford type A aortic dissection. Methods: Retrospective analysis were conducted for 60 patients diagnosed with AKI requiring CRRT undergoing surgery for acute Stanford type A aortic dissection at Beijing Anzhen Hospital, Capital Medical University from March 2015 to September 2016. There were 43 male and 17 female patients with an mean age of (50±14) years. Demographic characteristics, diagnosis, perioperative periodrelated data, clinical parameters during CRRT were collected to set up a database. The patients were divided into survival group and non-survival group according to in-hospital mortality. The prognostic factors of mortality in-hospital after AKI requiring CRRT were analyzed by multivariate Logistic regression analysis regression. Results: In the 60 adult patients who had received CRRT, 21 patients (35.0%) died. There were significant differences between died and survival patients on proportion of age>60 years (χ(2)=6.851, P =0.003), lactic acid levels at 12-hour after CRRT ( t =-3.631, P =0.004), lactic acid levels at 24 hours after CRRT ( t =-2.986, P =0.032), proportion of body mass index >25 kg/m(2) (χ(2)=5.660, P =0.041), cardiopulmonary bypass time ( t =-2.720, P =0.001). Multivariate Logistic regression analysis revealed that age≥60 years ( OR =16.450, 95% CI: 2.172 to 84.589); high lactic acid levels at 12-hour after CRRT ( OR =1.719, 95% CI: 1.998 to 2.960) and long cardiopulmonary bypass time ( OR =1.028, 95% CI: 1.004 to 1.052) (all P acid levels at 12-hour after CRRT and long cardiopulmonary bypass time were independent prognostic factors of patients with AKI requiring CRRT after aortic surgery. Proper identification and management shall improve the prognosis of patients.

  17. Preliminary Evidence for Aortopathy and an X-Linked Parent-of-Origin Effect on Aortic Valve Malformation in a Mouse Model of Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Robert B. Hinton

    2015-07-01

    Full Text Available Turner syndrome (TS, most frequently caused by X-monosomy (45,X, is characterized in part by cardiovascular abnormalities, including aortopathy and bicuspid aortic valve (BAV. There is a need for animal models that recapitulate the cardiovascular manifestations of TS. Extracellular matrix (ECM organization and morphometrics of the aortic valve and proximal aorta were examined in adult 39,XO mice (where the parental origin of the single X was paternal (39,XPO or maternal (39,XMO and 40,XX controls. Aortic valve morphology was normal (tricuspid in all of the 39,XPO and 40,XX mice studied, but abnormal (bicuspid or quadricuspid in 15% of 39,XMO mice. Smooth muscle cell orientation in the ascending aorta was abnormal in all 39,XPO and 39,XMO mice examined, but smooth muscle actin was decreased in 39,XMO mice only. Aortic dilation was present with reduced penetrance in 39,XO mice. The 39,XO mouse demonstrates aortopathy and an X-linked parent-of-origin effect on aortic valve malformation, and the candidate gene FAM9B is polymorphically expressed in control and diseased human aortic valves. The 39,XO mouse model may be valuable for examining the mechanisms underlying the cardiovascular findings in TS, and suggest there are important genetic modifiers on the X chromosome that modulate risk for nonsyndromic BAV and aortopathy.

  18. Hematological observations on two cases of acute radiation syndrome

    International Nuclear Information System (INIS)

    Jiang Benrong; Wang Guilin; Huang Shimin

    1990-01-01

    The hematological changes of two cases of acute radiation syndrome were observed. The physical doses of patients Liang and Yan were 3.5 Gy and 2.6 Gy respectively. According to the changes in WBC and platelet counts and the absolute count of lymphocytes and in comparison with the hematological data of the victims of Y-12 accident in USA in 1958 and those of previous accidents in China, Liang suffered from a moderate or moderate to severe degree, and Yan suffered from a moderate or moderate mild degree of hemopoietic form of acute radiation syndrome. This estimation was consistent with their clinical course and physical doses. Some blood cells appeared in the cytoplasm of megakaryocytes in bone marrow smears of those two cases. The mechanism of this phenomenon is discussed and its clinical significance remains to be studied

  19. Bilateral acute lupus pneumonitis in a case of rhupus syndrome

    Directory of Open Access Journals (Sweden)

    Supriya Sarkar

    2012-01-01

    Full Text Available Rhupus syndrome, the overlap of rheumatoid arthritis (RA and systemic lupus erythematosus (SLE, is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.

  20. Acute Respiratory Distress Syndrome Complicating Strongyloides stercoralis Hyperinfection

    Directory of Open Access Journals (Sweden)

    Ming-Ju Tsai

    2011-03-01

    Full Text Available Strongyloidiasis is endemic in tropic and subtropic areas, but is currently seldom encountered in developed area like Taiwan. We present an elder man with acute respiratory distress syndrome complicating Strongyloides stercoralis hyperinfection. There was no significant clue initially for diagnosing this patient as having S. stercoralis hyperinfection. Neither peripheral eosinophilia nor significant hemoptysis was noted. Bronchoscopy played a critical role to define the unexpected cause of his progressive pulmonary infiltrates. The correct diagnosis was soon made by recognition of the worm in bronchioloalveolar lavage cytology, and specific treatment was initiated promptly. For a septic patient with progressive pulmonary infiltrates, bronchoscopic studies including cytology may be necessary for defining the cause. Hyperinfection strongyloidiasis should be considered as a cause of acute respiratory distress syndrome in immunocompromised patient, especially with the presence of chronic gastrointestinal symptoms.

  1. Acute toxic neuropathy mimicking guillain barre syndrome

    Directory of Open Access Journals (Sweden)

    Muhammed Jasim Abdul Jalal

    2015-01-01

    Full Text Available Case: A 30 year old male presented with numbness of palms and soles followed by weakness of upper limbs and lower limbs of 5 days duration, which was ascending and progressive. Three months back he was treated for oral and genital ulcers with oral steroids. His ulcers improved and shifted to indigenous medication. His clinical examination showed polyneuropathy. CSF study did not show albuminocytological dissociation. Nerve conduction study showed demyelinating polyneuropathy. His blood samples and the ayurvedic drug samples were sent for toxicological analysis. Inference: Acute toxic neuropathy - Arsenic

  2. Aortic and Cardiac Structure and Function Using High-Resolution Echocardiography and Optical Coherence Tomography in a Mouse Model of Marfan Syndrome.

    Directory of Open Access Journals (Sweden)

    Ling Lee

    Full Text Available Marfan syndrome (MFS is an autosomal-dominant disorder of connective tissue caused by mutations in the fibrillin-1 (FBN1 gene. Mortality is often due to aortic dissection and rupture. We investigated the structural and functional properties of the heart and aorta in a [Fbn1C1039G/+] MFS mouse using high-resolution ultrasound (echo and optical coherence tomography (OCT. Echo was performed on 6- and 12-month old wild type (WT and MFS mice (n = 8. In vivo pulse wave velocity (PWV, aortic root diameter, ejection fraction, stroke volume, left ventricular (LV wall thickness, LV mass and mitral valve early and atrial velocities (E/A ratio were measured by high resolution echocardiography. OCT was performed on 12-month old WT and MFS fixed mouse hearts to measure ventricular volume and mass. The PWV was significantly increased in 6-mo MFS vs. WT (366.6 ± 19.9 vs. 205.2 ± 18.1 cm/s; p = 0.003 and 12-mo MFS vs. WT (459.5 ± 42.3 vs. 205.3 ± 30.3 cm/s; p< 0.0001. PWV increased with age in MFS mice only. We also found a significantly enlarged aortic root and decreased E/A ratio in MFS mice compared with WT for both age groups. The [Fbn1C1039G/+] mouse model of MFS replicates many of the anomalies of Marfan patients including significant aortic dilation, central aortic stiffness, LV systolic and diastolic dysfunction. This is the first demonstration of the direct measurement in vivo of pulse wave velocity non-invasively in the aortic arch of MFS mice, a robust measure of aortic stiffness and a critical clinical parameter for the assessment of pathology in the Marfan syndrome.

  3. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  4. Acute Compartment Syndrome of the Thigh in Combat Casualties

    Science.gov (United States)

    2013-03-01

    anesthesia (12); (c) internal hemorrhage or fluid extravasation such as coagulopathy and intramuscular hematoma (13, 14), false aneurysm (15), hamstring ...clinical signs and symptoms alone (27, 28). These signs include pain out of proportion, weakness and passive pain on stretch of the muscles in the...thigh. J. Orthop. Trauma 16:436–438, 2002. 6. Rooser, B., Bengtson, S., Hagglund, G. Acute compartment syndrome from anterior thigh muscle contusion: a

  5. Women and the management of acute coronary syndrome

    Czech Academy of Sciences Publication Activity Database

    Ošťádal, P.; Ošťádal, Bohuslav

    2012-01-01

    Roč. 90, č. 9 (2012), s. 1151-1159 ISSN 0008-4212 R&D Projects: GA MŠk(CZ) 1M0510 Grant - others:GA MZd(CZ) NT12153 Institutional research plan: CEZ:AV0Z50110509 Keywords : sex differences * acute coronary syndrome * women Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery Impact factor: 1.556, year: 2012

  6. Periodontal status and Prevotella intermedia antibody in acute coronary syndrome.

    Science.gov (United States)

    Soejima, Hirofumi; Oe, Yoko; Nakayama, Hideki; Matsuo, Katsuhiko; Fukunaga, Takashi; Sugamura, Koichi; Kawano, Hiroaki; Sugiyama, Seigo; Shinohara, Masanori; Izumi, Yuichi; Ogawa, Hisao

    2009-11-12

    We performed periodontal examination and measured serum antibody levels against Prevotella intermedia in patients with acute coronary syndrome (ACS). Composite periodontal risk scores were significantly higher in the ACS group than in the coronary artery disease (CAD) group. Serum antibody levels were higher in the ACS group than in the CAD group and those were significantly correlated with the composite periodontal risk scores. These results provided important information about the status of P. intermedia infection in patients with ACS.

  7. Network analysis reveals distinct clinical syndromes underlying acute mountain sickness.

    Directory of Open Access Journals (Sweden)

    David P Hall

    Full Text Available Acute mountain sickness (AMS is a common problem among visitors at high altitude, and may progress to life-threatening pulmonary and cerebral oedema in a minority of cases. International consensus defines AMS as a constellation of subjective, non-specific symptoms. Specifically, headache, sleep disturbance, fatigue and dizziness are given equal diagnostic weighting. Different pathophysiological mechanisms are now thought to underlie headache and sleep disturbance during acute exposure to high altitude. Hence, these symptoms may not belong together as a single syndrome. Using a novel visual analogue scale (VAS, we sought to undertake a systematic exploration of the symptomatology of AMS using an unbiased, data-driven approach originally designed for analysis of gene expression. Symptom scores were collected from 292 subjects during 1110 subject-days at altitudes between 3650 m and 5200 m on Apex expeditions to Bolivia and Kilimanjaro. Three distinct patterns of symptoms were consistently identified. Although fatigue is a ubiquitous finding, sleep disturbance and headache are each commonly reported without the other. The commonest pattern of symptoms was sleep disturbance and fatigue, with little or no headache. In subjects reporting severe headache, 40% did not report sleep disturbance. Sleep disturbance correlates poorly with other symptoms of AMS (Mean Spearman correlation 0.25. These results challenge the accepted paradigm that AMS is a single disease process and describe at least two distinct syndromes following acute ascent to high altitude. This approach to analysing symptom patterns has potential utility in other clinical syndromes.

  8. Acute respiratory distress syndrome after orthotopic liver transplantation.

    Science.gov (United States)

    Zhao, Wei; Ge, Xupeng; Sun, Kai; Agopian, Vatche G; Wang, Yuelan; Yan, Min; Busuttil, Ronald W; Steadman, Randolph H; Xia, Victor W

    2016-02-01

    Acute respiratory distress syndrome (ARDS) is a devastating complication with substantial mortality. The aims of this study were to identify the incidence, preoperative and intraoperative risk factors, and impact of ARDS on outcomes in patients after orthotopic liver transplantation (OLT). Adult OLT patients between January 2004 and October 2013 at our center were included. Postoperative ARDS was determined using the criteria proposed by the Berlin Definition. Multivariate logistic models were used to identify preoperative and intraoperative risk factors for ARDS. Of 1726 patients during the study period, 71 (4.1%) developed ARDS. In the preoperative model, encephalopathy (odds ratio [OR], 2.22; P = .022), preoperative requirement of intubation (OR, 2.06; P = .020), and total bilirubin (OR, 1.02; P = .003) were independent risk factors. In the intraoperative model, large pressor bolus was the sole risk factor for ARDS (OR, 2.69; P = .001). Postoperatively, patients with ARDS had a 2-fold increase in 1-year mortality, mechanical ventilation time, and length of hospital stay. Acute respiratory distress syndrome occurred at a rate of 4.1% following OLT in adult patients and was associated with preoperative encephalopathy, requirement of intubation, and total bilirubin and intraoperative large boluses of pressors. Acute respiratory distress syndrome was associated with increased mortality, longer ventilation time, and hospital stay. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Trastuzumab-Induced Myocardiotoxicity Mimicking Acute Coronary Syndrome

    Directory of Open Access Journals (Sweden)

    K.B. Ribeiro

    2012-03-01

    Full Text Available Trastuzumab is an important biological agent in the treatment of HER2-positive breast cancer, with effects on response rates, progression-free survival, overall survival and quality of life. Although this drug is well tolerated in terms of adverse effects, trastuzumab-associated myocardiotoxicity has been described to have an incidence of 0.6–4.5% and in rare cases, the drug can trigger severe congestive heart failure with progression to death or even mimic acute coronary syndrome with complete left bundle branch blockade. In this paper is reported a case of trastuzumab-associated myocardiotoxicity manifesting as acute coronary syndrome in a 69-year-old female. The patient is currently undergoing a conservative clinical treatment that restricts overexertion.The majority of clinical studies report trastuzumab-induced cardiotoxicity as a rare event, and, when present, characterized by mild to moderate clinical signs, the ease of reversibility with pharmacological measures and the temporary discontinuation of the medication. Conversely, it is vital for the oncologist/cardiologist to consider the possibility that trastuzumab-induced cardiotoxicity may manifest itself as a severe clinical case, mimicking acute coronary syndrome, justifying careful risk stratification and adequate cardiac monitoring, especially in high-risk patients.

  10. Acute and chronic pain syndromes in multiple sclerosis

    DEFF Research Database (Denmark)

    Stenager, E; Knudsen, L; Jensen, K

    1991-01-01

    A representative sample of 117 patients with definite multiple sclerosis (MS) was interviewed on pain syndromes. Chronic syndromes lasting more than one month included dysaestesthesia, low back pain, spasms, tonic seizures, tightening and painful sensations in the extremities. Acute syndromes...... with pain at the time of the examination increased with age and duration of disease. Patients with pain were significantly more often spastic and significantly more often sought alternative treatment forms. No difference was found for mean age, sex, physical impairment, duration of disease from onset of MS...... included neuralgia, L'Hermitte's sign and pain associated with optic neuritis. Thirty-five per cent were pain-free. Of the remaining patients had 45% pain at the time of the examination, 32% indicated pain among the most severe symptoms of MS and 23% had pain at the onset of MS. The number of patients...

  11. Acute and chronic pain syndromes in multiple sclerosis

    DEFF Research Database (Denmark)

    Stenager, E; Knudsen, L; Jensen, K

    1991-01-01

    with pain at the time of the examination increased with age and duration of disease. Patients with pain were significantly more often spastic and significantly more often sought alternative treatment forms. No difference was found for mean age, sex, physical impairment, duration of disease from onset of MS......A representative sample of 117 patients with definite multiple sclerosis (MS) was interviewed on pain syndromes. Chronic syndromes lasting more than one month included dysaestesthesia, low back pain, spasms, tonic seizures, tightening and painful sensations in the extremities. Acute syndromes...... included neuralgia, L'Hermitte's sign and pain associated with optic neuritis. Thirty-five per cent were pain-free. Of the remaining patients had 45% pain at the time of the examination, 32% indicated pain among the most severe symptoms of MS and 23% had pain at the onset of MS. The number of patients...

  12. Urgent carotid stenting before cardiac surgery in a young male patient with acute ischemic stroke caused by aortic and carotid dissection

    Directory of Open Access Journals (Sweden)

    Popović Rade

    2016-01-01

    Full Text Available Introduction. Acute aortic dissection (AD is the most common life-threatening disorder affecting the aorta. Neurological symptoms are present in 17-40% of cases. The management of these patients is controversial. Case report. We presented a 37-year-old man admitted for complaining of left-sided weak-ness. Symptoms appeared two hours before admission. The patient had no headache, neither thoracic pain. Neurological examination showed mild confusion, left-sided hemiplegia, National Institutes of Health Stroke Scale (NIHSS score was 10. Ischemic stroke was suspected, brain multislice computed tomography (MSCT and angiography were performed and right intrapetrous internal carotid artery dissection noted. Subsequent color Doppler ultrasound of the carotid arteries showed dissection of the right common carotid artery (CCA. The patient underwent thoracic and abdominal MSCT aortography which showed ascending aortic dissection from the aortic root, propagating in the brachiocephalic artery and the right CCA. Digital subtraction angiography was performed subsequently and two stents were successfully implanted in the brachiocephalic artery and the right CCA prior to cardiac surgery, only 6 hours after admission. The ascending aorta was reconstructed with graft interposition and the aortic valve re-suspended. The patient was hemodynamically stable and with no neurologic deficit after surgery. Unfortinately, at the operative day 6, mediastinitis developed and after intensive treatment the patients died 35 days after admission. Conclusion. In young patients with suspected stroke and oscillatory neurological impairment urgent MSCT angiography of the brain and neck and/or Doppler sonography of the carotid and vertebral artery are mandatory to exclude carotid and aortic dissection. The prompt diagnosis permits urgent carotid stenting and cardiosurgery. To the best of our knowledge, this is the first published case of immediate carotid stenting in acute ischemic

  13. [Characteristics of traditional Chinese medicine syndromes in patients with acute ischemic stroke of yin or yang syndrome: a multicenter trial].

    Science.gov (United States)

    You, Jin-song; Huang, Yan; Cai, Ye-feng; Guo, Jian-wen; Liang, Wei-xiong; Huang, Pei-xin; Liu, Mao-cai

    2008-04-01

    To explore the composition characteristics of traditional Chinese medicine (TCM) syndromes in patients with acute ischemic stroke of yin or yang syndrome by investigating the characteristics of TCM syndromes at different periods after onset. One thousand two hundred and forty-six patients with acute ischemic stroke were admitted in twenty hospitals. According to the "diagnostic criteria of syndrome differentiation of stroke", the characteristics of syndromes in the patients were investigated at the periods of 1-3 days, 4-10 days and 11-30 days after they had ischemic stroke. General distribution of six basic syndromes was compared between the patients with yin syndrome and the patients with yang syndrome at the three periods. The six basic syndromes were wind syndrome, pathogenic fire syndrome, phlegm syndrome, blood stasis syndrome, qi deficiency syndrome, and syndrome of yin deficiency and yang hyperactivity. The percentages of wind, pathogenic fire, and phlegm syndromes in the patients were decreased at the period of 11-30 days as compared with the period of 1-3 days (87.1% vs 79.3%, 52.1% vs 38.7% and 67.1% vs 57.4% respectively, P0.05). There were no differences in the distribution of yin and yang syndromes among the three periods (P>0.05). The percentages of syndromes of wind, pathogenic fire, phlegm, and yin deficiency and yang hyperactivity were higher (Pfour or five syndromes were higher, and the percentages of single-syndromes and complex syndromes of two syndromes were lower in patients with yang syndrome than in patients with yin syndrome (P<0.05, P<0.01). The most frequent complex syndromes in patients with yin syndrome were complex syndrome of wind, phlegm, blood stasis and qi deficiency, and complex syndrome of wind, phlegm and qi deficiency; while the most frequent complex syndromes in patients with yang syndrome were complex syndrome of wind, pathogenic fire, phlegm and qi deficiency, and complex syndrome of wind, pathogenic fire and phlegm. The

  14. Marijuana use in acute coronary syndromes.

    Science.gov (United States)

    Draz, Eman I; Oreby, Mervat M; Elsheikh, Eman A; Khedr, Lamia A; Atlam, Salwa A

    2017-09-01

    Cannabis is one of the most widely used illicit substances worldwide, and it has the highest prevalence among drugs used in Egypt. The aims were to evaluate whether the use of cannabis is a risk factor of acute coronary heart disease in low-risk, young males and to compare the cardiac pathological changes between cannabis exposed and non-exposed ischemic patients. This was a cross-sectional study that was performed on 138 male patients, aged ≤ 40 years, with acute myocardial infarction who were admitted to the Cardiac Care Unit at the University Hospital. Urine samples were submitted for toxicological analysis using a homogenous enzyme immunoassay technique to determine the substance of use. The patients were divided into three groups: group 1 (n = 23), cannabis-positive only patients; group 2 (n = 28), patients positive for any other substance of use; and group 3 (n = 34), patients negative for any substance of use. Smoking was prominent, whereas group 1 had no other risk factors. In groups 1 and 2, ST-segment elevation myocardial infarction (STEMI) was dominant, whereas no ST-segment elevation myocardial infarction (NSTEMI) was prominent in group 3. Ischemic resting wall motion abnormalities were presented in 47.8% of group 1 and in only 11.8% of group 3. None of group 1 had normal coronaries, whereas 14.3% of group 3 had normal coronaries. Significant changes in echocardiography and angiography were observed between group 1 and other groups. Cannabis smoking could be a potential risk factor for the development of cardiac ischemia.

  15. Re-interventions on the thoracic and thoracoabdominal aorta in patients with Marfan syndrome

    OpenAIRE

    Schoenhoff, Florian S.; Carrel, Thierry P.

    2017-01-01

    The advent of multi-gene panel genetic testing and the discovery of new syndromic and non-syndromic forms of connective tissue disorders have established thoracic aortic aneurysms as a genetically mediated disease. Surgical results in patients with Marfan syndrome (MFS) provide an important benchmark for this patient population. Prophylactic aortic root surgery prevents acute dissection and has contributed to the improved survival of MFS patients. In the majority of patients, re-interventions...

  16. Increased prevalence of bicuspid aortic valve in Turner syndrome links with karyotype: the crucial importance of detailed cardiovascular screening.

    Science.gov (United States)

    Klásková, Eva; Zapletalová, Jiřina; Kaprálová, Sabina; Šnajderová, Marta; Lebl, Jan; Tüdös, Zbyněk; Pavlíček, Jan; Černá, Jana; Mihál, Vladimír; Stará, Veronika; Procházka, Martin

    2017-03-01

    Bicuspid aortic valve (BAV) represents one of the strongest risk factors for aortic dissection in Turner syndrome (TS). An exact relation between the occurrence of BAV and a particular karyotype has not been established yet. The aim of this study was to determine the association between karyotype and prevalence of BAV. Sixty-seven TS patients aged between 6.6 and 32.5 years underwent cardiac magnetic resonance imaging (MRI) study. They were divided into four cytogenetic subgroups-45,X karyotype (n=27); 45,X/46,XX mosaicism (n=17); structural abnormalities of the X chromosome (n=10); and 45,X/structural abnormality of the X chromosome mosaicism (n=13). Prevalence of BAV and odds ratio (OR) compared with the general population in the whole study group, and statistical comparison of prevalences of BAV among the individual subgroups were determined. Prevalence of BAV in the whole study group was established as 28.4% [OR 208.3 (95% CI - 103.8-418.0); p-valuekaryotype had the highest prevalence of BAV - 40.7%, p-valuekaryotype significantly predisposed to BAV (p-value=0.05). The 45,X karyotype is associated with the highest prevalence of BAV. Also, the presence of the 45,X cell line in any mosaic karyotype increases the probability of BAV.

  17. Abdominal aortic calcification quantified by the Morphological Atherosclerotic Calcification Distribution (MACD index is associated with features of the metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Barascuk Natasha

    2011-12-01

    Full Text Available Abstract Background Abdominal aortic calcifications (AAC predict cardiovascular mortality. A new scoring model for AAC, the Morphological Atherosclerotic Calcification Distribution (MACD index may contribute with additional information to the commonly used Aortic Calcification Severity (AC24 score, when predicting death from cardiovascular disease (CVD. In this study we investigated associations of MACD and AC24 with traditional metabolic-syndrome associated risk factors at baseline and after 8.3 years follow-up, to identify biological parameters that may account for the differential performance of these indices. Methods Three hundred and eight healthy women aged 48 to 76 years, were followed for 8.3 ± 0.3 years. AAC was quantified using lumbar radiographs. Baseline data included age, weight, blood pressure, blood lipids, and glucose levels. Pearson correlation coefficients were used to test for relationships. Results At baseline and across all patients, MACD correlated with blood glucose (r2 = 0.1, P Conclusion Patterns of calcification identified by the MACD, but not the AC24 index, appear to contain useful biological information perhaps explaining part of the improved identification of risk of cardiovascular death of the MACD index. Correlations of MACD but not the AC24 with glucose levels at baseline suggest that hyperglycemia may contribute to unique patterns of calcification indicated by the MACD.

  18. Acute Testicular Ischemia following Endovascular Abdominal Aortic Aneurysm Repair Identified in the Emergency Department

    Directory of Open Access Journals (Sweden)

    Nathan Finnerty

    2014-01-01

    Full Text Available Endovascular aneurysm repair (EVAR is perhaps the most widely utilized surgical procedure for patients with large abdominal aortic aneurysms. This procedure is minimally invasive and reduces inpatient hospitalization requirements. The case involves a 72-year-old male who presented to the emergency department with right testicular ischemia two days following EVAR. Given the minimal inpatient hospitalization associated with this procedure, emergency physicians are likely to encounter associated complications. Ischemic and thromboembolic events following EVAR are extremely rare but require prompt vascular surgery intervention to minimize morbidity and mortality.

  19. [Valve-sparing Replacement in Patients with Aortic Root Dilatation].

    Science.gov (United States)

    Yamazaki, Kazuhiro; Minatoya, Kenji; Ueda, Ryoma; Takehara, Masato; Sakamoto, Kazuhisa; Ide, Yujiro; Kanemitsu, Hideo; Ueyama, Koji; Ikeda, Tadashi

    2018-01-01

    Valve-sparing root replacement is increasingly used to overcome drawbacks associated with valvular prostheses. In our institution, 7 patients underwent valve-sparing root replacement from August 2016 to July 2017. The mean age was 45 years (range, 14~69 years). Three patients had Marfan syndrome and 1 had Loeys-Dietz syndrome with acute aortic dissection. All patients underwent surgery with reimplantation technique using a Valsalva graft. Two patients required repair of aortic valve leaflet prolapse. All patients had an excellent clinical course, with mild or no aortic regurgitation and a decrease in end-diastolic volume on echocardiography. These results support the continued use of valve-sparing root replacement in selected patients.

  20. Acute respiratory distress syndrome assessment after traumatic brain injury

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    Shahrooz Kazemi

    2016-01-01

    Full Text Available Background: Acute respiratory distress syndrome (ARDS is one of the most important complications associated with traumatic brain injury (TBI. ARDS is caused by inflammation of the lungs and hypoxic damage with lung physiology abnormalities associated with acute respiratory distress syndrome. Aim of this study is to determine the epidemiology of ARDS and the prevalence of risk factors. Methods: This prospective study performed on patients with acute traumatic head injury hospitalization in the intensive care unit of the Shohaday-e Haftom-e-Tir Hospital (September 2012 to September 2013 done. About 12 months, the data were evaluated. Information including age, sex, education, employment, drug and alcohol addiction, were collected and analyzed. The inclusion criteria were head traumatic patients and exclusion was the patients with chest trauma. Questionnaire was designed with doctors supervision of neurosurgery. Then the collected data were analysis. Results: In this study, the incidence of ARDS was 23.8% and prevalence of metabolic acidosis was 31.4%. Most injury with metabolic acidosis was Subarachnoid hemorrhage (SAH 48 (60% and Subdural hemorrhage (SDH was Next Level with 39 (48% Correlation between Glasgow Coma Scale (GCS and Respiratory Distress Syndrome (ARDS were significantly decreased (P< 0.0001. The level of consciousness in patients with skull fractures significantly lower than those without fractures (P= 0.009 [(2.3±4.6 vs (4.02±7.07]. Prevalence of metabolic acidosis during hospitalization was 80 patients (31.4%. Conclusion: Acute respiratory distress syndrome is a common complication of traumatic brain injury. Management and treatment is essential to reduce the mortality. In this study it was found the age of patients with ARDS was higher than patients without complications. ARDS risk factor for high blood pressure was higher in men. Most victims were pedestrians. The most common injury associated with ARDS was SDH. Our analysis

  1. Acute coronary syndromes in patients with pre-existing moderate to severe valvular disease of the heart: lessons from the Euro-Heart Survey of acute coronary syndromes.

    Science.gov (United States)

    Hasdai, David; Lev, Eli I; Behar, Solomon; Boyko, Valentina; Danchin, Nicholas; Vahanian, Alec; Battler, Alexander

    2003-04-01

    To determine the frequency of pre-existing valvular disease (VD) among patients with acute coronary syndromes (ACS) and to compare the clinical characteristics, clinical course, treatment, and outcomes of ACS patients with and without pre-existing VD. The Euro Heart Survey ACS prospectively enrolled 10,484 ACS patients in 103 hospitals in 25 countries across Europe and the Mediterranean basin. Of the 10,207 patients with data on VD status, 489 (4.8%) had a diagnosis of pre-existing VD: 3.7% of 4339 ST-segment-elevation-ACS patients, 5.2% of 5210 non-ST-segment-elevation-ACS patients, and 10.8% of 658 undetermined-electrocardiogram-ACS patients. Moderate/severe mitral regurgitation had been diagnosed in 54.0% (48.7% without and 5.3% with concomitant mitral stenosis), and moderate/severe aortic stenosis occurred in 31.7% (26.4% without and 5.3% with concomitant aortic regurgitation). Patients with pre-existing VD had worse baseline clinical and demographic characteristics, were more likely to present with heart failure and less likely to have typical angina, and had a more complicated in-hospital course (heart failure, atrial arrhythmias, and renal failure). They were more likely to receive inotropic agents, diuretics, amiodarone, and warfarin, and less likely to receive antiplatelet agents and beta-adrenergic blockers. As compared to patients without VD, the adjusted risk (95% confidence interval) of in-hospital death for VD patients was 1.55 (0.85, 2.80), 1.92 (1.03, 3.59), and 1.77 (0.75, 4.17) for ST-segment-elevation-ACS, non-ST-segment-elevation-ACS, and undetermined-electrocardiogram-ACS, respectively. Patients with ACS and pre-existing VD constitute about 5% of all ACS patients; they have high-risk features and poor prognosis. There is a need to better define their optimal treatment, in order to improve their prognosis.

  2. Inhaled nitric oxide for acute respiratory distress syndrome (ARDS) and acute lung injury in children and adults

    DEFF Research Database (Denmark)

    Afshari, Arash; Brok, Jesper; Møller, Ann

    2010-01-01

    Acute hypoxaemic respiratory failure (AHRF), defined as acute lung injury (ALI) and acute respiratory distress syndrome (ARDS), are critical conditions. AHRF results from a number of systemic conditions and is associated with high mortality and morbidity in all ages. Inhaled nitric oxide (INO) has...

  3. Message concerning Severe Acute Respiratory Syndrome ("SARS")

    CERN Multimedia

    2003-01-01

    IMPORTANT REMINDER If you have just come back from one of the regions identified by the WHO as being infected with SARS, it is essential to monitor your state of health for ten days after your return. The syndrome manifests itself in the rapid onset of a high fever combined with respiratory problems (coughing, breathlessness, breathing difficulty). Should these signs appear, you must contact the CERN Medical Service as quickly as possible on number 73802 or 73186 during normal working hours, and the fire brigade at all other times on number 74444, indicating that you have just returned from one of the WHO-identified areas with recent local transmission.China: Beijing, Hong Kong (Special Administrative Region), Guangdong Province, Inner Mongolia, Shanxi Province, Tianjin ProvinceTaiwan:TaipeiMoreover, until further notice the CERN Management requests that all trips to these various regions of the world be reduced to a strict minimum and then only with the consent of the Division Leader concerned. Anyone comin...

  4. Comparing American, European and Asian practice guidelines for aortic diseases.

    Science.gov (United States)

    Rozado, Jose; Martin, Maria; Pascual, Isaac; Hernandez-Vaquero, Daniel; Moris, Cesar

    2017-05-01

    The aortic disease comprises a group of different pathologies of high prevalence, seriousness and ever changing by the medical and surgical investigations. Therefore cardiovascular scientific societies in USA, Europe and Asia have created Task Force on practice guidelines (PG) to develop, update and revise PG for aortic diseases. These documents issue recommendations on the diagnosis and management of different aortic diseases. The three societies agree on the recommendations about diagnostic tests and on the value of computed tomography and magnetic resonance as the main tools for the diagnosis and follow-up of aortic disease. Concerning to acute aortic syndromes (AAS), American and European GPs recognize intramural hematoma (IMH) as a type of AAS with surgery indication; however Asian guidelines consider IMH a pathological process different from AAS and indicate medical treatment. In thoracic aortic aneurysms (TAA), all express the need for an adequate control of cardiovascular risk factors, emphasizing strict control of blood pressure, smoking cessation and recommend the use of beta-blockers and statins. The threshold for asymptomatic repair is 5.5 cm in European and American and 6 cm for Asian PG, with lower thresholds in Marfan and bicuspid aortic valve (BAV). As regards the abdominal aortic aneurysms (AAA), the PGs recognize the adequate control of cardiovascular risk factors, but there are differences in class of recommendation on statins, angiotensin-converting enzyme inhibitors or beta-blockers to prevent progression of AAA. For intervention, the threshold diameter in asymptomatic is 5.5 cm but can be reduced to 5 cm in women as recommended by Asian PG. Moreover the specific diseases such as Marfan, BAV, pregnancy or atherosclerosis aortic present specific recommendations with small differences between PGs. In conclusion, PGs are interesting and appropriate documents at present. They issue recommendations based on evidence that help the clinician and

  5. Nonpulmonary treatments for pediatric acute respiratory distress syndrome: proceedings from the Pediatric Acute Lung Injury Consensus Conference.

    Science.gov (United States)

    Valentine, Stacey L; Nadkarni, Vinay M; Curley, Martha A Q

    2015-06-01

    To describe the recommendations from the Pediatric Acute Lung Injury Consensus Conference on nonpulmonary treatments in pediatric acute respiratory distress syndrome. Consensus conference of experts in pediatric acute lung injury. A panel of 27 experts met over the course of 2 years to develop a taxonomy to define pediatric acute respiratory distress syndrome and to make recommendations regarding treatment and research priorities. The nonpulmonary subgroup comprised three experts. When published data were lacking, a modified Delphi approach emphasizing strong professional agreement was utilized. The Pediatric Acute Lung Injury Consensus Conference experts developed and voted on a total of 151 recommendations addressing the topics related to pediatric acute respiratory distress syndrome, 30 of which related to nonpulmonary treatment. All 30 recommendations had strong agreement. Patients with pediatric acute respiratory distress syndrome should receive 1) minimal yet effective targeted sedation to facilitate mechanical ventilation; 2) neuromuscular blockade, if sedation alone is inadequate to achieve effective mechanical ventilation; 3) a nutrition plan to facilitate their recovery, maintain their growth, and meet their metabolic needs; 4) goal-directed fluid management to maintain adequate intravascular volume, end-organ perfusion, and optimal delivery of oxygen; and 5) goal-directed RBC transfusion to maintain adequate oxygen delivery. Future clinical trials in pediatric acute respiratory distress syndrome should report sedation, neuromuscular blockade, nutrition, fluid management, and transfusion exposures to allow comparison across studies. The Consensus Conference developed pediatric-specific definitions for pediatric acute respiratory distress syndrome and recommendations regarding treatment and future research priorities. These recommendations for nonpulmonary treatment in pediatric acute respiratory distress syndrome are intended to promote optimization and

  6. Pregnancy and delivery in a patient with aortic prosthesis for Leriche syndrome

    OpenAIRE

    Sass,Nelson; Poli,Antonio Henrique Moura; Torloni,Maria Regina; Gibim,Paula Gabriela Monteagudo; Andrade,Januário de; Izukwa,Nilo Mitsuro

    2003-01-01

    CONTEXT: Leriche syndrome is a thrombotic obliteration of the bifurcation of the aorta, a rare condition that usually affects older men as a result of atherosclerosis. Women of childbearing age rarely need a vascular prosthesis (as a result of Leriche syndrome or other conditions) and there is no literature on an association between Leriche syndrome/vascular prosthesis and pregnancy/labor/delivery. CASE REPORT: A case of pregnancy and delivery in a 38-year-old patient with Leriche syndrome an...

  7. Vitamin D Deficiency Is Associated with Increased Osteocalcin Levels in Acute Aortic Dissection: A Pilot Study on Elderly Patients

    Directory of Open Access Journals (Sweden)

    Elena Vianello

    2017-01-01

    Full Text Available An imbalance between degradation and reconstruction of the aortic wall is one of the leading causes of acute aortic dissection (AAD. Vitamin D seems an intriguing molecule to explore in the field of AAD since it improves endothelial function and protects smooth muscle cells from inflammation-induced remodeling, calcification, and loss of function, all events which are strongly related to the aging process. We quantified 25-hydroxy vitamin D, calcium, parathormone, bone alkaline phosphatase, and osteocalcin levels in 24 elderly AAD patients to identify a potential pathological implication of these molecules in AAD. Median 25-hydroxy vitamin D (10.75 ng/mL, 25th–75th percentiles: 6.86–19.23 ng/mL and calcium levels (8.70 mg/dL, 25th–75th percentiles: 7.30–8.80 mg/dL suggested hypovitaminosis D and a moderate hypocalcemia. Thirty-eight percent of AAD patients had severe (<10 ng/mL, 38% moderate (10–20 ng/mL, and 24% mild 25-hydroxy vitamin D deficiency (20–30 ng/mL. A significant inverse correlation was observed between 25OHD and osteocalcin levels. All the other molecules were unchanged. A condition of hypovitaminosis D associated to an increase in osteocalcin levels is present in AAD patients. The identification of these molecules as new factors involved in AAD may be helpful to identify individuals at high risk as well to study preventing strategies.

  8. [Status of intra-aortic balloon pump application and related factors in Chinese patients with acute myocardial infarction].

    Science.gov (United States)

    Wu, N Q; Gao, Z; Zheng, Y; Li, W; Li, J N; Xu, H Y; Cheng, X S; Wu, Y; Wang, Y; Wang, Z J; Yang, J G; Yang, Y J

    2018-01-24

    Objective: To investigate the current status of application of intra-aortic balloon pump(IABP) and analyze the factors which might impact the application of IABP in patients with acute myocardial infarction complicated with cardiac shock in China. Methods: In China Acute Myocardial Infarction (CAMI) Registry,a nationwide, multicenter(107 hospitals), prospective study, 26 592 patients with acute myocardial infarction were enrolled consecutively between January 1, 2013 and September 30, 2014. After excluding of 30 cases due to missing important data,26 562 cases were analyzed.The application status of IABP was analyzed and multivariate logistic regression analysis was performed to determine the factors related to IABP application. Results: A total of 785(3.0%) patients with acute myocardial infarction received IABP implantation, and 381(49.9%) patients belonged to preventive application of IABP before primary percutaneous coronary intervention,and 6(0.8%) patients were complicated with mechanical complications.There were 118(15.0%) patients with cardiac shock received IABP implantation, which accounted for 12.0%(118/984) of all patients with cardiac shock.Multivariate logistic regression analysis showed that the clinical independent factors of the decision of IABP insertion included dyslipidemia ( OR= 2.858, 95% CI 1.397-5.846, P= 0.004),left ventricular ejection fraction ( OR= 0.977, 95% CI 0.961-0.994, P= 0.009),usage of dopamine ( OR= 2.817, 95% CI 1.495-5.308, P= 0.001), left main disease ( OR= 2.817, 95% CI 1.495-5.308, P= 0.001), GRACE score ( OR= 1.006, 95% CI 1.000-1.011, P= 0.034), receiving primary percutaneous coronary intervention ( OR= 4.508, 95% CI 1.673-12.146, P= 0.003), teritiary hospitals ( OR= 2.562, 95% CI 1.498-4.384, P= 0.001), and higher education of the patients ( OR= 2.183, 95% CI 1.056-4.509, P= 0.016). Conclusions: Among the Chinese acute myocardial infarction patients who received IABP implantation, nearly half application of IABP are

  9. A Fever in Acute Aortic Dissection is Caused by Endogenous Mediators that Influence the Extrinsic Coagulation Pathway and Do Not Elevate Procalcitonin.

    Science.gov (United States)

    Inoue Arita, Yoshie; Akutsu, Koichi; Yamamoto, Takeshi; Kawanaka, Hidekazu; Kitamura, Mitsunobu; Murata, Hiroshige; Miyachi, Hideki; Hosokawa, Yusuke; Tanaka, Keiji; Shimizu, Wataru

    2016-01-01

    Objective A fever is observed in approximately one-third of cases of acute aortic dissection (AAD); however, the causes remain unclear. We investigated the mechanism of a fever in AAD by measuring the serum concentrations of inflammatory markers, mediators of coagulation and fibrinolysis, and procalcitonin, a marker of bacterial infection. Methods We retrospectively studied 43 patients with medically treated AAD without apparent infection. Patients were divided into those with (Group A; n=19) and without (Group B; n=24) a maximum body temperature >38°C. We established which patients fulfilled the criteria for systemic inflammatory response syndrome (SIRS), and its relationship with a fever was examined. Mediators of inflammation, coagulation and fibrinolysis were compared by a univariate analysis. Factors independently associated with a fever were established by a multivariate analysis. Results The criteria for SIRS were fulfilled in a greater proportion of patients in Group A (79%) than in Group B (42%, p=0.001). There was no difference in the procalcitonin concentration between Groups A and B (0.15±0.17 ng/mL vs. 0.11±0.12 ng/mL, respectively; p=0.572). Serum procalcitonin concentrations lay within the normal range in all patients in whom it was measured, which showed that the fever was caused by endogenous mediators. On the multivariate analysis, there was a borderline significant relationship between a fever and the prothrombin time-International Normalized Ratio (p=0.065), likely reflecting the extrinsic pathway activity initiated by tissue factor. Conclusion Our findings suggest that a fever in AAD could be caused by SIRS, provoked by endogenous mediators that influence the extrinsic coagulation pathway without elevating the serum procalcitonin concentration.

  10. Acute Chest Syndrome in Children with Sickle Cell Disease

    Science.gov (United States)

    Bakshi, Nitya; Krishnamurti, Lakshmanan

    2017-01-01

    Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray. The spectrum of clinical manifestations can range from mild respiratory illness to acute respiratory distress syndrome. The presence of severe hypoxemia is a useful predictor of severity and outcome. The etiology of ACS is often multifactorial. One of the proposed mechanisms involves increased adhesion of sickle red cells to pulmonary microvasculature in the presence of hypoxia. Other commonly associated etiologies include infection, pulmonary fat embolism, and infarction. Infection is a common cause in children, whereas adults usually present with pain crises. Several risk factors have been identified in children to be associated with increased incidence of ACS. These include younger age, severe SCD genotypes (SS or Sβ0 thalassemia), lower fetal hemoglobin concentrations, higher steady-state hemoglobin levels, higher steady-state white blood cell counts, history of asthma, and tobacco smoke exposure. Opiate overdose and resulting hypoventilation can also trigger ACS. Prompt diagnosis and management with intravenous fluids, analgesics, aggressive incentive spirometry, supplemental oxygen or respiratory support, antibiotics, and transfusion therapy, are key to the prevention of clinical deterioration. Bronchodilators should be considered if there is history of asthma or in the presence of acute bronchospasm. Treatment with hydroxyurea should be considered for prevention of recurrent episodes. This review evaluates the

  11. Apical ballooning syndrome complicated by acute severe mitral regurgitation with left ventricular outflow obstruction – Case report

    Directory of Open Access Journals (Sweden)

    Celermajer David S

    2007-02-01

    Full Text Available Abstract Background Apical ballooning syndrome (or Takotsubo cardiomyopathy is a syndrome of transient left ventricular apical ballooning. Although first described in Japanese patients, it is now well reported in the Caucasian population. The syndrome mimicks an acute myocardial infarction but is characterised by the absence of obstructive coronary disease. We describe a serious and poorly understood complication of Takotsubo cardiomyopathy. Case Presentation We present the case of a 65 year-old lady referred to us from a rural hospital where she was treated with thrombolytic therapy for a presumed acute anterior myocardial infarction. Four hours after thrombolysis she developed acute pulmonary oedema and a new systolic murmur. It was presumed she had acute mitral regurgitation secondary to a ruptured papillary muscle, ischaemic dysfunction or an acute ventricular septal defect. Echocardiogram revealed severe mitral regurgitation, left ventricular apical ballooning, and systolic anterior motion of the mitral valve with significant left ventricular outflow tract gradient (60–70 mmHg. Coronary angiography revealed no obstructive coronary lesions. She had an intra-aortic balloon pump inserted with no improvement in her parlous haemodynamic state. We elected to replace her mitral valve to correct the outflow tract gradient and mitral regurgitation. Intra-operatively the mitral valve was mildly myxomatous but there were no structural abnormalities. She had a mechanical mitral valve replacement with a 29 mm St Jude valve. Post-operatively, her left ventricular outflow obstruction resolved and ventricular function returned to normal over the subsequent 10 days. She recovered well. Conclusion This case represents a serious and poorly understood association of Takotsubo cardiomyopathy with acute pulmonary oedema, severe mitral regurgitaton and systolic anterior motion of the mitral valve with significant left ventricular outflow tract obstruction. The

  12. Acute Symptomatic Abdominal Aortic Aneurysm Secondary to Endovascular Stent Graft Associated Type II Endoleak

    Directory of Open Access Journals (Sweden)

    Karen Ka Leung Chan

    2006-07-01

    Full Text Available Endovascular abdominal aneurysm repair (EVAR is popular because of its low invasiveness and feasibility for high-risk patients. Endoleak is common after EVAR and is characterized by blood flow within the aneurysm sac but outside the stent graft. Type II or collateral endoleak commonly results from retrograde filling of the aneurysm from collateral visceral vessels, lumbar, inferior mesenteric, accessory renal or sacral arteries. Collateral leaks are generally thought to be benign and over half of the early leaks will seal spontaneously. Sporadically, collateral endoleak could lead to aneurysm sac pressurization and place the patient at ongoing risk of rupture. Herein, we report an uncommon case of early post-stent graft placement symptomatic abdominal aortic aneurysm associated with type II endoleak.

  13. Cardiac troponin T in acute coronary syndrome with renal insufficiency.

    Science.gov (United States)

    Chew, Huck Chin

    2008-08-01

    Cardiac troponin levels are frequently elevated in patients with chronic renal failure, hence diagnosis of myocardial necrosis is difficult. The prevalence of elevated serum troponin T was determined and its diagnostic value in acute coronary syndrome was assessed in patients with chronic renal insufficiency. A retrospective cross-sectional analysis was performed in 227 patients with chronic renal insufficiency and a diagnosis of unstable angina, non-ST or ST-segment elevation myocardial infarction. All patients had baseline serum troponin T levels measured at previous visits; the baseline troponin T level was raised in 53.3%. Cardiac troponin T levels did not correlate with creatinine levels, and were not affected by dialysis. Mortality after an acute coronary event was high (46.3%). Because of the elevated baseline cardiac troponin T levels, detection of acute coronary syndrome in patients with chronic renal failure requires evaluation of serial cardiac enzyme measurements and serial 12-lead electrocardiograms. Early and definitive cardiac interventions may contribute towards decreasing the mortality rate in this group of patients.

  14. Pleural effusions in acute idiopathic pericarditis and postcardiac injury syndrome.

    Science.gov (United States)

    Porcel, José M

    2017-07-01

    Pleural effusions are frequent in the context of acute idiopathic pericarditis and following pericardiotomy, but they have seldom been characterized. This review summarizes their most relevant clinical features. In acute idiopathic pericarditis, pleural effusions tend to be left-sided and, if bilateral, they are usually larger on the left. Less than 5% are unilateral right-sided. About 90% of the effusions occupy less than one-third of the hemithorax, and 99% meet Light's exudative criteria with a predominance of lymphocytes in three fourths of the cases. Although postcardiac injury syndrome (PCIS)-related effusions share similar characteristics, they present some differential features: more than 15% are unilateral on the right (except for Dressler syndrome), one-fourth opacify half or more of the hemithorax, and nearly two thirds are bloody. The combination of nonsteroidal anti-inflammatory drugs and colchicine, along with therapeutic thoracenteses for moderate-to-large effusions, is the mainstay treatment approach. The postoperative use of colchicine is also a reasonable option for preventing PCIS in patients who have undergone cardiac surgery. Pleural effusions because of pericardial diseases remain a clinical diagnosis. If unilateral right-sided, massive, or transudative effusions are seen, an alternative diagnosis to acute pericarditis should be considered.

  15. Diagnosis and management of acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Baker Hamilton

    2013-09-01

    La prévalence des maladies cardiovasculaires connaît une croissance rapide dans les pays en développement, entraînant une incidence croissante du syndrome coronarien aigu (SCA. Les modalités de diagnostic et de traitement de cette maladie continuent d’évoluer, et il convient de tenir compte des ressources locales lors de la réalisation d’un diagnostic et la détermination des options thérapeutiques. Cet article constitue un guide à la prise en charge du SCA fondé sur l’expérience, et fournit des recommandations spécifiques destinées aux médecins hospitaliers travaillant dans les pays à bas et moyen revenu. Le diagnostic du SCA, y compris les SCA sans élévation du ST et avec élévation du ST, se concentre sur la stratification du risque, la vigilance relative aux manifestations subtiles ou atypiques, et la prise en considération d’autres causes des douleurs poitrinaires. Le processus de diagnostic implique l’évaluation des facteurs de risque, la connaissance des antécédents médicaux défavorables et les conclusions de l’examen physique (des variantes étant susceptibles d’exister dans les différentes populations, ainsi que l’utilisation de tests de diagnostic appropriés. Il est recommandé d’utiliser de l’aspirine à titre de traitement initial, parallèlement à un antiagrégant plaquettaire supplémentaire. Le prasugrel est préféré au clopidogrel si le patient présente un SCA avec élévation du ST et qu’une intervention coronaire percutanée (ICP est prévue. La bivalidurine devrait être le premier choix pour éviter la coagulation dans les SCA avec élévation du ST, suivie de l’enoxaparine (qui ne nécessite pas de perfusion, puis d’héparine non fractionnée. Pour les patients présentant un SCA sans élévation du ST et en cas de risque de saignement accru, le fondaparinux devrait être envisagé à la place de l’énoxaparine. Les patients souffrant de dyspnée, présentant des signes d

  16. Comparison of cine-MRI and transthoracic echocardiography for the assessment of aortic root diameters in patients with suspected Marfan syndrome

    International Nuclear Information System (INIS)

    Bannas, P.; Derlin, T.; Yamamura, J.; Lund, G.; Adam, G.; Rybczynski, M.; Sheikhzadeh, S.; Kodolitsch, Y. von; Groth, M.

    2015-01-01

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r=0.929), but a statistically significant bias of -1.0 mm (p<0.001). The mean absolute diameter for sinuses of Valsalva obtained by cine-MRI was 32.3 ± 5.8 mm as compared to 33.4 ± 5.4 mm obtained by echocardiography. Interobserver agreement of measurements of the sinuses of Valsalva was higher for cine-MRI than for echocardiography (p=0.029). Despite small, but statistically significant differences in terms of agreement and reproducibility, cine-MRI and echocardiographic measurements of aortic root diameters provide comparable results without a significant clinical difference. Therefore both techniques may be used for monitoring of the aortic root in patients with Marfan syndrome.

  17. Comparison of cine-MRI and transthoracic echocardiography for the assessment of aortic root diameters in patients with suspected Marfan syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Bannas, P.; Derlin, T.; Yamamura, J.; Lund, G.; Adam, G. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of Diagnostic and Interventional Radiology; Rybczynski, M.; Sheikhzadeh, S.; Kodolitsch, Y. von [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of General and Interventional Cardiology; Groth, M. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Section for Pediatric Radiology

    2015-11-15

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r=0.929), but a statistically significant bias of -1.0 mm (p<0.001). The mean absolute diameter for sinuses of Valsalva obtained by cine-MRI was 32.3 ± 5.8 mm as compared to 33.4 ± 5.4 mm obtained by echocardiography. Interobserver agreement of measurements of the sinuses of Valsalva was higher for cine-MRI than for echocardiography (p=0.029). Despite small, but statistically significant differences in terms of agreement and reproducibility, cine-MRI and echocardiographic measurements of aortic root diameters provide comparable results without a significant clinical difference. Therefore both techniques may be used for monitoring of the aortic root in patients with Marfan syndrome.

  18. Thrombosis and antiphospholipid antibody syndrome during acute Q fever

    Science.gov (United States)

    Million, Matthieu; Bardin, Nathalie; Bessis, Simon; Nouiakh, Nadia; Douliery, Charlaine; Edouard, Sophie; Angelakis, Emmanouil; Bosseray, Annick; Epaulard, Olivier; Branger, Stéphanie; Chaudier, Bernard; Blanc-Laserre, Karine; Ferreira-Maldent, Nicole; Demonchy, Elisa; Roblot, France; Reynes, Jacques; Djossou, Felix; Protopopescu, Camelia; Carrieri, Patrizia; Camoin-Jau, Laurence; Mege, Jean-Louis; Raoult, Didier

    2017-01-01

    Abstract Q fever is a neglected and potentially fatal disease. During acute Q fever, antiphospholipid antibodies are very prevalent and have been associated with fever, thrombocytopenia, acquired heart valve disease, and progression to chronic endocarditis. However, thrombosis, the main clinical criterion of the 2006 updated classification of the antiphospholipid syndrome, has not been assessed in this context. To test whether thrombosis is associated with antiphospholipid antibodies and whether the criteria for antiphospholipid syndrome can be met in patients with acute Q fever, we conducted a cross-sectional study at the French National Referral Center for Q fever. Patients included were diagnosed with acute Q fever in our Center between January 2007 and December 2015. Each patient's history and clinical characteristics were recorded with a standardized questionnaire. Predictive factors associated with thrombosis were assessed using a rare events logistic regression model. IgG anticardiolipin antibodies (IgG aCL) assessed by an enzyme-linked immunosorbent assay were tested on the Q fever diagnostic serum. A dose-dependent relationship between IgG aCL levels and thrombosis was tested using a receiver operating characteristic (ROC) analysis. Of the 664 patients identified for inclusion in the study, 313 (47.1%) had positive IgG aCL and 13 (1.9%) were diagnosed with thrombosis. Three patients fulfilled the antiphospholipid syndrome criteria. After multiple adjustments, only positive IgG aCL (relative risk, 14.46 [1.85–113.14], P = .011) were independently associated with thrombosis. ROC analysis identified a dose-dependent relationship between IgG aCL levels and occurrence of thrombosis (area under curve, 0.83, 95%CI [0.73–0.93], P antiphospholipid antibodies are associated with thrombosis, thrombocytopenia, and acquired valvular heart disease. Antiphospholipid antibodies should be systematically assessed in acute Q fever patients. Hydroxychloroquine

  19. [Sudden death and cardiovascular complications in Marfan syndrome: impact of surgical intervention].

    Science.gov (United States)

    Ohtsubo, Satoshi; Itoh, Tsuyoshi

    2005-07-01

    Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated. Major cause of death include acute aortic dissection, aortic rupture, and sudden death, which resulted from congenital vascular fragility. Such life-threatening complications in Marfan syndrome can be managed effectively, by routine aortic imaging, beta-adrenergic blockade, and prophylactic replacement of the aortic root before the diameter exceeds 5.0 to 5.5 mm. Valve preserving aortic root reconstruction yielded improved postoperative quality of life compared with Bentall operation, by reducing late complications related to anticoagulants. It should be carried out before onset of aortic regurgitation for long-term native valve durability.

  20. [Acute states in gastroenterology: spontaneous bacterial peritonitis and the acute intestinal pseudoobstruction syndrome].

    Science.gov (United States)

    Lukás, K

    2001-07-19

    Our article concentrates on two acute states, which develop less dramatically but their after-effects may be very serious: Spontaneous bacterial peritonitis and Ogilvie's syndrome. Spontaneous bacterial peritonitis is a bacterial infection of the ascitic fluid without any intraperitoneal source of infection. Ascites is a condition of the disease but need not be clinically manifested. Spontaneous bacterial peritonitis comes usually during heavy hepatic impairment. Diagnosis can be set according: 1. Positive cultivation of ascitic fluid, 2. PMN levels higher than 250/mm3, 3. No infection, which may require a surgical intervention is apparent. Liver disease, which brings about the spontaneous bacterial peritonitis can be: 1. Chronic (e.g. alcoholic cirrhosis), 2. Subacute (e.g. alcoholic hepatitis), 3. Acute (e.g. fulminant hepatic failure). Mortality of this form of peritonitis can reach up to 46%. The most frequent etiological factor is alcohol and viral hepatitis, the most frequent agents are E. coli and Klebsiella pneumoniae. The disease is most effectively cured by cefalosporins of the third generation. With inadequate treatment, prognosis may be poor. Intestinal pseudoobstruction syndrome has clinical symptomatology of a serious impairment with ileus without signs of any mechanical intestinal obstruction. Syndrome can be classified according to its development: 1. Acute form--acute intestinal pseudoobstruction syndrome--Ogilvie's syndrome, 2. Chronic form--chronic intestinal pseudoobstruction syndrome. Pathogenic mechanism of the syndrome is not known. The disease is related to immobility, administration of some drugs, electrolyte imbalance and concomitant diseases (most frequently malignant tumors). Clinical symptomatology dominates nausea, vomiting, diffuse abdominal pain, constipation or diarrhoea. For diagnostics the first step should be termination of all medication, which could have causing affects, then taking native abdominal X-ray picture where gaseous

  1. [Case in which renal function improved following stent-graft placement in the aorta two months after the onset of stanford type B acute aortic dissection].

    Science.gov (United States)

    Fusamae, Juri; Nishino, Tomoya; Uramatsu, Tadashi; Obata, Yoko; Furusu, Akira; Sakamoto, Ichiro; Kohno, Shigeru

    2011-01-01

    A 48-year-old man was admitted to the Department of Cardiovascular Surgery in our hospital after developing Stanford type B acute aortic dissection with a patent false lumen in July 2008. Conservative treatment involving rest and antihypertensive therapy was provided following admission. Urine volume decreased from day 9, and serum creatinine increased to 7.7 mg/dL. As it was suspected that the reduced renal blood flow was caused by progression of aortic dissection, contrast-enhanced computed tomography (CT)was performed. The left kidney showed reduced enhancement and the right kidney was heterogeneously enhanced. The dissection had extended to the left renal artery, and the reduced renal blood flow caused by narrowing of the left renal artery was thought to have caused the renal dysfunction. As elevated urea nitrogen and serum creatinine levels and hyperkalemia persisted, hemodialysis was performed a total of four times. Although the patient was subsequently withdrawn from dialysis, he continued to display severe renal dysfunction and was transferred to our department on day 28 for the treatment of renal failure. Conservative treatment was continued, but the maximum diameter of the thoracic aorta gradually increased, and stent placement at the entry of aortic dissection was indicated. On day 86, two stent-grafts were placed for entries at the distal site of the descending aorta and the distal site of the aortic arch. Postoperative abdominal contrast-enhanced CT showed expansion of the true lumen, and blood flow and contrast enhancement improved in both kidneys. Postoperatively, serum creatinine gradually decreased, improving to 1.16 mg/dL on day 96. Renography in the third month after stent-graft placement showed improved renal function in both kidneys. These findings suggest that even at approximately 2 months after the onset of acute renal failure associated with aortic dissection, renal function can be improved by restoring blood flow in the renal arteries.

  2. Prediction of acute coronary syndromes by urinary proteome analysis.

    Directory of Open Access Journals (Sweden)

    Nay M Htun

    Full Text Available Identification of individuals who are at risk of suffering from acute coronary syndromes (ACS may allow to introduce preventative measures. We aimed to identify ACS-related urinary peptides, that combined as a pattern can be used as prognostic biomarker. Proteomic data of 252 individuals enrolled in four prospective studies from Australia, Europe and North America were analyzed. 126 of these had suffered from ACS within a period of up to 5 years post urine sampling (cases. Proteomic analysis of 84 cases and 84 matched controls resulted in the discovery of 75 ACS-related urinary peptides. Combining these to a peptide pattern, we established a prognostic biomarker named Acute Coronary Syndrome Predictor 75 (ACSP75. ACSP75 demonstrated reasonable prognostic discrimination (c-statistic = 0.664, which was similar to Framingham risk scoring (c-statistics = 0.644 in a validation cohort of 42 cases and 42 controls. However, generating by a composite algorithm named Acute Coronary Syndrome Composite Predictor (ACSCP, combining the biomarker pattern ACSP75 with the previously established urinary proteomic biomarker CAD238 characterizing coronary artery disease as the underlying aetiology, and age as a risk factor, further improved discrimination (c-statistic = 0.751 resulting in an added prognostic value over Framingham risk scoring expressed by an integrated discrimination improvement of 0.273 ± 0.048 (P < 0.0001 and net reclassification improvement of 0.405 ± 0.113 (P = 0.0007. In conclusion, we demonstrate that urinary peptide biomarkers have the potential to predict future ACS events in asymptomatic patients. Further large scale studies are warranted to determine the role of urinary biomarkers in clinical practice.

  3. Metabolic syndrome in the survivors of childhood acute lymphoblastic leukaemia.

    Science.gov (United States)

    Abu-Ouf, Noran M; Jan, Mohammed M

    2015-01-01

    Metabolic syndrome is a common complication encountered in children surviving acute lymphoblastic leukaemia (ALL). Affected patients develop obesity, insulin resistance, hypertension, and hyperlipidemia. Metabolic syndrome is a consequence of multiple factors, particularly hormonal imbalance induced by various ALL treatments. This review aims to evaluate the risk factors and mechanisms leading to the development of metabolic syndrome. Further research is needed to improve our understanding of the mechanisms leading to insulin resistance and the associated endothelial and adipose tissue dysfunction. Future studies should also examine other possible contributing factors, such as environmental and genetic factors. Understanding these factors will help in guiding modifications of the current ALL treatment protocols in order to prevent the development of this syndrome and hence improve the quality of life of ALL survivors. Until this is achieved, clinicians should continue to identify patients at risk early and use a therapeutic approach that combines dietary restrictions and enhanced physical activity. Copyright © 2014 Asian Oceanian Association for the Study of Obesity. Published by Elsevier Ltd. All rights reserved.

  4. [Organ damage and cardiorenal syndrome in acute heart failure].

    Science.gov (United States)

    Casado Cerrada, Jesús; Pérez Calvo, Juan Ignacio

    2014-03-01

    Heart failure is a complex syndrome that affects almost all organs and systems of the body. Signs and symptoms of organ dysfunction, in particular kidney dysfunction, may be accentuated or become evident for the first time during acute decompensation of heart failure. Cardiorenal syndrome has been defined as the simultaneous dysfunction of both the heart and the kidney, regardless of which of the two organs may have suffered the initial damage and regardless also of their previous functional status. Research into the mechanisms regulating the complex relationship between the two organs is prompting the search for new biomarkers to help physicians detect renal damage in subclinical stages. Hence, a preventive approach to renal dysfunction may be adopted in the clinical setting in the near future. This article provides a general overview of cardiorenal syndrome and an update of the physiopathological mechanisms involved. Special emphasis is placed on the role of visceral congestion as an emergent mechanism in this syndrome. Copyright © 2014 Elsevier España, S.L. All rights reserved.

  5. A case report of acute myelogenous leukemia with Turner Syndrome.

    Science.gov (United States)

    Siddiqui, Nadir; Ali Baig, Mirza Faris; Khan, Bilal Ahmed

    2017-09-01

    Turner Syndrome was diagnosed in a 45 years old female, known case of Acute Myeloid Leukaemia (AML) with maturation, on Bone Marrow biopsy. She presented with blurred vision, vertigo, exertional dyspnoea and insomnia. She did not show the typical features of Turner syndrome, but her cytogenetis confirmed the diagnosis. Bone marrow biopsy showed diffuse infiltration of blast cells with cellularity around 80-85% and haematopoietic suppression. Karyotype analysis showed: 45 X, -X, t (8; 21) (q22; q22) [According to The International System for Human Cytogenetic Nomenclature (ISCN)]. Turner syndrome is caused by partial or complete absence of second X chromosome in a female. It is known to have Cardiovascular and Reproductive complications but it is rare to find haematologic malignancies. There are few similar reported cases of AML associated with Turner syndrome, therefore this is a unique case presented to Jinnah Postgraduate Medical Center, Karachi, Pakistan and further research should be done to identify more similar cases to explore the prognostic significance of this association.

  6. [Acute respiratory distress syndrome: a review of the Berlin definition].

    Science.gov (United States)

    de Luis Cabezón, N; Sánchez Castro, I; Bengoetxea Uriarte, U X; Rodrigo Casanova, M P; García Peña, J M; Aguilera Celorrio, L

    2014-01-01

    Acute Respiratory Distress Syndrome (ARDS) is due to many causes. The absence of a universal definition up until now has led to a series of practical problems for a definitive diagnosis. The incidences of ARDS and Acute Lung Injury (ALI) vary widely in the current literature. The American-European Consensus Conference definition has been applied since its publication in 1994 and has helped to improve knowledge about ARDS. However, 18 years later, in 2011, the European Intensive Medicine Society, requested a team of international experts to meet in Berlin to review the ARDS definition. The purpose of the Berlin definition is not to use it as a prognostic tool, but to improve coherence between research and clinical practice. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  7. Fractional flow reserve in acute coronary syndromes: A review

    Directory of Open Access Journals (Sweden)

    Nikunj R. Shah

    2014-12-01

    Full Text Available Fractional flow reserve (FFR assessment provides anatomical and physiological information that is often used to tailor treatment strategies in coronary artery disease. Whilst robust data validates FFR use in stable ischaemic heart disease, its use in acute coronary syndromes (ACS is less well investigated. We critically review the current data surrounding FFR use across the spectrum of ACS including culprit and non-culprit artery analysis. With adenosine being conventionally used to induce maximal hyperaemia during FFR assessment, co-existent clinical conditions may preclude its use during acute myocardial infarction. Therefore, we include a current review of instantaneous wave free ratio as a novel vasodilator independent method of assessing lesion severity as an alternative strategy to guide revascularisation in ACS.

  8. Obesity and nutrition in acute respiratory distress syndrome.

    Science.gov (United States)

    Stapleton, Renee D; Suratt, Benjamin T

    2014-12-01

    This article discusses obesity, its contribution to clinical outcomes, and the current literature on nutrition. More than one third of Americans are obese. Literature suggests that, among critically ill patients, the relationship between obesity and outcomes is complex. Obese patients may be at greater risk of developing acute respiratory distress syndrome (ARDS) than normal weight patients. Although obesity may confer greater morbidity in intensive care, it seems to decrease mortality. ARDS is a catabolic state; patients demonstrate a profound inflammatory response, multiple organ dysfunction, and hypermetabolism, often with malnutrition. The concept of pharmaconutrition has emerged. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Mechanical ventilation in the acute respiratory distress syndrome.

    Science.gov (United States)

    Epelbaum, Oleg; Aronow, Wilbert S

    2017-08-01

    The management of the acute respiratory distress syndrome (ARDS) patient is fundamental to the field of intensive care medicine, and it presents unique challenges owing to the specialized mechanical ventilation techniques that such patients require. ARDS is a highly lethal disease, and there is compelling evidence that mechanical ventilation itself, if applied in an injurious fashion, can be a contributor to ARDS mortality. Therefore, it is imperative for any clinician central to the care of ARDS patients to understand the fundamental framework that underpins the approach to mechanical ventilation in this special scenario. The current review summarizes the major components of the mechanical ventilation strategy as it applies to ARDS.

  10. Collagen XVIII Mutation in Knobloch Syndrome with Acute Lymphoblastic Leukemia

    Science.gov (United States)

    Mahajan, Vinit B.; Olney, Ann Haskins; Garrett, Penny; Chary, Ajit; Dragan, Ecaterina; Lerner, Gary; Murray, Jeffrey; Bassuk, Alexander G.

    2010-01-01

    Knobloch syndrome (KNO) is caused by mutations in the collagen XIII gene (COL18A1) and patients develop encephalocele and vitreoretinal degeneration. Here we report an El Salvadorian family where two sisters showed features of KNO. One of the siblings also developed acute lymphoblastic leukemia. DNA sequencing of COL18A1revealed a homozygous, 2-base pair deletion (c3514-3515delCT) in exon 41, which leads to abnormal collagen XVIII and deficiency of its proteolytic cleavage product endostatin. KNO patients with mutations in COL18A1 may be at risk for endostatin-related conditions including malignancy. PMID:20799329

  11. Yttrium Y 90 Anti-CD45 Monoclonal Antibody BC8 Followed by Donor Stem Cell Transplant in Treating Patients With High-